Niedobór pseudocholinesterazy
Objawy

Niedobór pseudocholinesterazy (BChE) to rzadkie zaburzenie genetyczne lub nabyte, prowadzące do zmniejszonej aktywności enzymu odpowiedzialnego za metabolizm leków zwiotczających mięśnie, takich jak sukcynylocholina i miwakurium. Klinicznie objawia się przedłużonym zwiotczeniem mięśni, czasowym paraliżem mięśni oddechowych oraz koniecznością mechanicznej wentylacji. U heterozygot obserwuje się około 30% wydłużenie czasu blokady nerwowo-mięśniowej, co przekłada się na zwiotczenie trwające od kilkudziesięciu minut do kilku godzin, natomiast u homozygot czas ten może wynosić od 2 do 3 godzin, a w skrajnych przypadkach nawet do 8 godzin lub dłużej. Mechanizm polega na spowolnionym metabolizmie sukcynylocholiny, co powoduje utrzymanie wysokiego stężenia leku w złączu nerwowo-mięśniowym i przedłużoną blokadę neuromięśniową. Nabyte formy niedoboru mogą być związane z chorobami wątroby, nerek, ciążą, nowotworami czy stanami zapalnymi, które obniżają aktywność enzymu.

Objawy i progresja niedoboru pseudocholinesterazy

Niedobór pseudocholinesterazy to rzadkie zaburzenie genetyczne lub nabyte, charakteryzujące się nieprawidłową aktywnością enzymu pseudocholinesterazy (butyrylocholinesterazy, BChE), który odpowiada za metabolizm określonych leków używanych podczas znieczulenia ogólnego. Osoby z tym schorzeniem wykazują nadwrażliwość na estry cholinowe, szczególnie na leki zwiotczające mięśnie, takie jak sukcynylocholina i miwakurium.123

Charakterystyka objawów klinicznych

U większości pacjentów z niedoborem pseudocholinesterazy brak jest widocznych objawów klinicznych w codziennym życiu. Zaburzenie to pozostaje najczęściej nierozpoznane, dopóki pacjent nie zostanie poddany znieczuleniu ogólnemu z zastosowaniem zwiotczających leków mięśniowych, takich jak sukcynylocholina lub miwakurium.456

Główne objawy kliniczne niedoboru pseudocholinesterazy obejmują:

  • Przedłużone zwiotczenie mięśni, utrzymujące się znacznie dłużej niż oczekiwano po standardowej dawce leków zwiotczających78
  • Czasowy paraliż mięśni oddechowych9
  • Niezdolność do samodzielnego oddychania, wymagająca mechanicznej wentylacji10
  • Przedłużone utrzymywanie się apnei oddechowej11

Czas trwania i nasilenie objawów

Czas trwania objawów niedoboru pseudocholinesterazy różni się znacząco w zależności od stopnia niedoboru enzymu oraz statusu zygotyczności pacjenta.1213

  • Heterozygoty (osoby z jedną nieprawidłową kopią genu) doświadczają około 30% wydłużenia czasu trwania blokady nerwowo-mięśniowej po standardowym dawkowaniu sukcynylocholiny, co przekłada się na zwiotczenie mięśni trwające od kilkudziesięciu minut do kilku godzin1415
  • Homozygoty (osoby z dwiema nieprawidłowymi kopiami genu) mogą doświadczać zwiotczenia mięśni trwającego znacznie dłużej, zazwyczaj od 2 do 3 godzin, a w skrajnych przypadkach nawet do 8 godzin161718
  • U pacjentów z całkowitym brakiem aktywności enzymu BChE, przedłużenie zwiotczenia mięśni może trwać nawet powyżej 8 godzin1920

W przypadku pacjentów z nabytym niedoborem pseudocholinesterazy, czas trwania i nasilenie objawów mogą być również uzależnione od choroby podstawowej, która doprowadziła do obniżenia aktywności enzymu.21

Fizjologiczne podłoże objawów

Mechanizm powstawania objawów niedoboru pseudocholinesterazy jest związany z nieefektywnym metabolizmem leków zwiotczających. U osób z prawidłową aktywnością enzymu, sukcynylocholina jest szybko metabolizowana w osoczu krwi przez pseudocholinesterazę, co prowadzi do krótkotrwałego działania leku (zazwyczaj 5-10 minut). Natomiast u pacjentów z niedoborem pseudocholinesterazy, metabolizm leku jest znacznie spowolniony, co skutkuje przedłużonym działaniem zwiotczającym.2223

Sukcynylocholina jest lekiem depolaryzującym płytkę nerwowo-mięśniową, co blokuje możliwość przekazywania impulsów z nerwów do mięśni. W warunkach normalnych efekt ten ustępuje szybko, ale przy niedoborze pseudocholinesterazy wyższe stężenie niemetabolizowanej sukcynylocholiny utrzymuje się w obrębie złącza nerwowo-mięśniowego, powodując przedłużoną blokadę neuromięśniową.2425

Powikłania i następstwa kliniczne

Niedobór pseudocholinesterazy, choć sam w sobie nie jest bezpośrednio zagrażający życiu, może prowadzić do poważnych powikłań, szczególnie w kontekście znieczulenia ogólnego i zabiegów chirurgicznych.2627

Bezpośrednie powikłania oddechowe

Głównym powikłaniem niedoboru pseudocholinesterazy jest ryzyko wystąpienia niewydolności oddechowej na skutek przedłużonej blokady nerwowo-mięśniowej wywołanej przez sukcynylocholinę lub miwakurium.2829

  • Przedłużona apnea (bezdech) wymagająca mechanicznej wentylacji30
  • Niewydolność oddechowa, jeśli nie zostanie zastosowane odpowiednie wsparcie oddechowe31
  • Hipoksja w przypadku zbyt wczesnego usunięcia rurki intubacyjnej32

Powikłania związane z diagnostyką i leczeniem

W przypadku nierozpoznania niedoboru pseudocholinesterazy mogą wystąpić dodatkowe powikłania:3334

  • Ryzyko błędnej diagnozy – objawy mogą być mylnie interpretowane jako przedawkowanie opioidów, udar pnia mózgu lub pozostałości działania leków sedacyjnych35
  • Pacjent może pozostawać przytomny, ale całkowicie sparaliżowany, nie mogąc komunikować się z personelem medycznym36
  • Przedłużona zależność od respiratora po znieczuleniu37
  • Niepokój i dyskomfort psychiczny związany z przedłużonym paraliżem przy zachowanej świadomości38

Szczególne sytuacje kliniczne

Niedobór pseudocholinesterazy może stanowić szczególne zagrożenie w określonych sytuacjach klinicznych:3940

  • Zabiegi nagłe, takie jak cięcie cesarskie, gdzie istotne jest szybkie zwiotczenie i odwrócenie działania leków41
  • Anestezja w warunkach ambulatoryjnych, gdzie przedłużony czas wybudzania może powodować problemy logistyczne42
  • W przypadku osób z niezdiagnozowanym niedoborem pseudocholinesterazy, ekspozycja na kokainę (również ester) może prowadzić do nagłej śmierci sercowej w warunkach pozamedycznych4344

Również stosowanie lokalnych anestetyków będących estrami (prokaina, chloroprokaina, tetrakaina) może powodować zwiększone ryzyko efektów toksycznych u pacjentów z niedoborem pseudocholinesterazy.4546

Czynniki wpływające na nasilenie objawów

Nasilenie objawów niedoboru pseudocholinesterazy zależy od wielu czynników, zarówno genetycznych, jak i nabytych.4748

Czynniki genetyczne

Głównym czynnikiem determinującym nasilenie objawów jest status genetyczny pacjenta:4950

  • Homozygotyczność – osoby z dwiema nieprawidłowymi kopiami genu BCHE wykazują najdłuższe i najbardziej nasilone objawy zwiotczenia mięśni po podaniu sukcynylocholiny5152
  • Heterozygotyczność – osoby z jedną nieprawidłową kopią genu doświadczają łagodniejszych i krócej trwających objawów5354
  • Warianty genetyczne – różne mutacje genu BCHE mogą prowadzić do różnego stopnia deficytu aktywności enzymatycznej55

Czynniki nabyte

Poza genetycznymi formami niedoboru pseudocholinesterazy, istnieją również nabyte formy tego zaburzenia, związane z chorobami współistniejącymi lub czynnikami zewnętrznymi:5657

  • Choroby wątroby – obniżona produkcja enzymu ze względu na dysfunkcję wątroby58
  • Niewydolność nerek i hemodializa – mogą wpływać na aktywność enzymu59
  • Ciąża – fizjologicznie obniżona aktywność pseudocholinesterazy60
  • Choroby nowotworowe – mogą prowadzić do wtórnego niedoboru pseudocholinesterazy61
  • Ciężkie oparzenia, zawał serca, niewydolność serca – stany kliniczne mogące wpływać na aktywność enzymu62
  • Niedożywienie – może zmniejszać produkcję enzymu63
  • Przewlekłe infekcje – mogą powodować wtórny niedobór pseudocholinesterazy64
  • Zaawansowany wiek – może wpływać na efektywność normalnej aktywności BChE65

Obecność tych czynników może nasilać objawy u osób z genetyczną predyspozycją do niedoboru pseudocholinesterazy lub samodzielnie wywoływać nabytą formę tego zaburzenia.66

Rokowanie i przebieg naturalny

Niedobór pseudocholinesterazy, choć może prowadzić do poważnych komplikacji podczas znieczulenia, ma generalnie dobre rokowanie przy właściwym postępowaniu medycznym.676869

Samoistny powrót funkcji mięśniowych

U pacjentów z rozpoznanym niedoborem pseudocholinesterazy po ekspozycji na sukcynylocholinę lub miwakurium oczekuje się pełnego powrotu do zdrowia po samoistnym powrocie funkcji motorycznych.707172

  • Pacjenci zawsze odzyskują pełną funkcję mięśniową, chociaż czas powrotu do normy może być znacznie wydłużony73
  • Powrót funkcji mięśniowych następuje spontanicznie, gdy lek zostanie ostatecznie zmetabolizowany przez organizm74
  • Nie ma trwałych następstw neurologicznych czy mięśniowych związanych z przedłużonym zwiotczeniem75

Wsparcie terapeutyczne i monitorowanie

Kluczowym elementem zapewniającym dobre rokowanie jest odpowiednie wsparcie medyczne w okresie przedłużonego zwiotczenia mięśniowego:767778

  • Mechaniczna wentylacja do czasu samodzielnego powrotu oddychania79
  • Sedacja w celu zmniejszenia dyskomfortu i niepokoju pacjenta8081
  • Ścisłe monitorowanie kliniczne w celu zapobiegania hipoksycznej niewydolności oddechowej82
  • Stymulacja nerwów w celu oceny powrotu funkcji mięśniowych83

Przy zastosowaniu odpowiedniego wsparcia oddechowego i monitorowania rokowanie jest doskonałe.8485

Długoterminowe konsekwencje i zapobieganie nawrotom

Po zdiagnozowaniu niedoboru pseudocholinesterazy, kluczowym elementem długoterminowego postępowania jest profilaktyka powikłań w przyszłości:8687

  • Pacjent powinien być poinformowany o swoim schorzeniu i potencjalnych zagrożeniach związanych ze znieczuleniem88
  • W przypadku konieczności przeprowadzenia znieczulenia ogólnego, anestezjolog powinien unikać stosowania sukcynylocholiny i miwakurium8990
  • Należy rozważyć alternatywne środki zwiotczające, które nie są metabolizowane przez pseudocholinesterazę91
  • Warto przeprowadzić badania genetyczne wśród członków rodziny, szczególnie w przypadku dziedzicznej formy niedoboru92
  • Pacjent powinien nosić identyfikator medyczny informujący o niedoborze pseudocholinesterazy93

Należy również pamiętać, że osoby z niedoborem pseudocholinesterazy mogą wykazywać zwiększoną wrażliwość na inne leki, w tym lokalne środki znieczulające z grupy estrów (prokaina, chloroprokaina) oraz niektóre pestycydy.949596

Przy odpowiednim postępowaniu medycznym i unikaniu czynników wyzwalających, osoby z niedoborem pseudocholinesterazy mogą prowadzić całkowicie normalne życie bez jakichkolwiek objawów czy ograniczeń.97

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Pseudocholinesterase deficiency – Symptoms & causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pseudocholinesterase-deficiency/symptoms-causes/syc-20354543
    Pseudocholinesterase deficiency is a rare disorder that makes you sensitive to certain muscle relaxants succinylcholine or mivacurium used during general anesthesia. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can’t move or breathe on your own. The length of time may vary widely among people with this disorder. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after getting succinylcholine. This prevents you from moving or breathing on your own for a few hours longer than expected. How long it takes your body to metabolize the drug depends on how much pseudocholinesterase enzyme you produce and how well it functions.
  • #2 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a condition that makes a person extremely sensitive to certain anesthetic medications. People with pseudocholinesterase deficiency may not be able to move or breathe on their own for several hours after receiving these medications. Mechanical ventilation supports your breathing until you recover. […] People with pseudocholinesterase deficiency usually dont know they have it until they receive choline esters during general anesthesia. Hallmark symptoms include: Extreme muscle relaxation that lasts several hours longer than expected. Temporary paralysis of your respiratory muscles. […] It can take several hours for pseudocholinesterase deficiency symptoms to improve. This timeline varies from person to person. […] If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, youll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, theyll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] Theres no cure for pseudocholinesterase deficiency. But knowing whether you have it can help you avoid symptoms like temporary muscle paralysis and breathing issues after surgery.
  • #3 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, refers to a rare acquired or inherited defect in the pseudocholinesterase enzyme produced by the liver. […] Patients with defective forms of pseudocholinesterase will have a reduced ability to metabolize these two muscle relaxants and will present with prolonged muscular paralysis from standard doses of succinylcholine and mivacurium. […] Heterozygotes will present with an approximate 30 percent increase in the duration of the neuromuscular blockade after standard succinylcholine dosing. Homozygotes, however, can present with neuromuscular blockade for a clinically significant greater duration (2 to 3 hrs). […] With an inherited deficiency, the defective form of the enzyme is unable to metabolize succinylcholine and mivacurium to the same degree, leading to prolonged neuromuscular paralysis for those who inherited atypical pseudocholinesterase enzyme.
  • #4 Pseudocholinesterase deficiency // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/pseudocholinesterase-deficiency
    For most people with pseudocholinesterase deficiency, no signs or symptoms of the condition occur until you get the muscle relaxant succinylcholine. This medication is used as part of anesthesia. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can’t move or breathe on your own. The length of time may vary widely among people with this disorder. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after getting succinylcholine. This prevents you from moving or breathing on your own for a few hours longer than expected. How long it takes your body to metabolize the drug depends on how much pseudocholinesterase enzyme you produce and how well it functions.
  • #5 Pseudocholinesterase deficiency: MedlinePlus GeneticsLock
    https://medlineplus.gov/genetics/condition/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is a condition that results in increased sensitivity to certain muscle relaxant drugs used during general anesthesia, called choline esters. […] However, people with pseudocholinesterase deficiency may not be able to move or breathe on their own for a few hours after the drugs are administered. Affected individuals must be supported with a machine to help them breathe (mechanical ventilation) until the drugs are cleared from the body. […] The condition causes no other signs or symptoms and is usually not discovered until an abnormal drug reaction occurs.
  • #6 Pseudocholinesterase Deficiency – Norton & Elaine Sarnoff Center for Jewish Genetics
    https://www.jewishgenetics.org/genetic-disorders/search-genetic-disorders/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency happens when people dont have enough of an enzyme called pseudocholinesterase. This enzyme is important for breaking down certain drugs used during surgery, like succinylcholine and mivacurium, which help relax the muscles. […] If someone has pseudocholinesterase deficiency, these drugs dont work well for them. This can lead to problems because the muscles that control breathing might stop working properly, making them unable to breathe (apnea). […] Carriers generally do not have symptoms, but they may sometimes experience a short period of breathing paralysis following anesthesia.
  • #7 Pseudocholinesterase deficiency | Altru Health System
    https://www.altru.org/health-library/conditions/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a rare disorder that makes you sensitive to certain muscle relaxants succinylcholine or mivacurium used during general anesthesia. […] With pseudocholinesterase deficiency, the body muscles stay relaxed for a longer time than expected. […] This temporary loss of the ability to move your muscles (paralysis) makes you unable to breathe or move on your own. This can last for several hours. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can’t move or breathe on your own. The length of time may vary widely among people with this disorder. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after getting succinylcholine. This prevents you from moving or breathing on your own for a few hours longer than expected.
  • #8 Mayo Clinic Health Library – Pseudocholinesterase deficiency | Swiss Medical Network
    https://www.swissmedical.net/de/healtcare-library/con-20200770
    This causes sensitivity to certain muscle relaxants used during anesthesia. Muscles may stay relaxed for too long, interfering with movement and breathing. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can’t move or breathe on your own. The length of time may vary widely among people with this disorder. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after getting succinylcholine. This prevents you from moving or breathing on your own for a few hours longer than expected. How long it takes your body to metabolize the drug depends on how much pseudocholinesterase enzyme you produce and how well it functions. […] If you have pseudocholinesterase deficiency, the health care provider who gives you anesthesia (anesthesiologist) can avoid succinylcholine that may trigger prolonged muscle relaxation. The anesthesiologist can choose other muscle relaxants instead.
  • #9 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a condition that makes a person extremely sensitive to certain anesthetic medications. People with pseudocholinesterase deficiency may not be able to move or breathe on their own for several hours after receiving these medications. Mechanical ventilation supports your breathing until you recover. […] People with pseudocholinesterase deficiency usually dont know they have it until they receive choline esters during general anesthesia. Hallmark symptoms include: Extreme muscle relaxation that lasts several hours longer than expected. Temporary paralysis of your respiratory muscles. […] It can take several hours for pseudocholinesterase deficiency symptoms to improve. This timeline varies from person to person. […] If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, youll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, theyll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] Theres no cure for pseudocholinesterase deficiency. But knowing whether you have it can help you avoid symptoms like temporary muscle paralysis and breathing issues after surgery.
  • #10 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #11 Orphanet: Hereditary butyrylcholinesterase deficiency
    https://www.orpha.net/en/disease/detail/132
    Butyrylcholinesterase (BChE) deficiency is a metabolic disorder characterised by prolonged apnoea after the use of certain anaesthetic drugs, including the muscle relaxants succinylcholine or mivacurium and other ester local anaesthetics. The duration of the prolonged apnoea varies significantly depending on the extent of the enzyme deficiency. […] Individuals with undetectable levels of BChE activity display a severe prolongation lasting more than 8 hours. The prevalence of this severe form is estimated at 1 in 100 000 individuals. […] Affected individuals are asymptomatic unless exposed to neuromuscular blocking agents, however, prolonged respiratory paralysis following anaesthesia makes mechanical ventilation essential until the excess aesthetic agent is metabolised permitting normal neuromuscular function.
  • #12 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Duration of neuromuscular blockade is dependant upon whether the patient is homozygous or heterozygous for the defective enzyme. Heterozygotes will experience a muscular blockade of moderate duration, while homozygotes can have muscular paralysis longer than 2 hours. […] Patients diagnosed with pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. […] The primary complication of pseudocholinesterase deficiency is respiratory failure following the prolonged neuromuscular paralysis after administration of succinylcholine or mivacurium.
  • #13 Pseudocholinesterase deficiency
    https://www.mymlc.com/health-information/diseases-and-conditions/p/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is a rare condition that makes you sensitive to certain muscle relaxants ? succinylcholine or mivacurium ? used during anesthesia. The drugs are designed to briefly relax your muscles during a medical procedure. With pseudocholinesterase deficiency, the body muscles stay relaxed for a longer time. Paralysis of the respiratory muscles causes you to be unable to breathe on your own. […] For most people with pseudocholinesterase deficiency, no signs or symptoms of the condition occur until they’re exposed to the muscle relaxants succinylcholine or mivacurium. These drugs are used as part of anesthesia. Then signs and symptoms include muscle relaxation or muscle paralysis that lasts for several hours, with the inability to breathe on your own. The length of muscle relaxation may vary widely among people with this deficiency.
  • #14 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, refers to a rare acquired or inherited defect in the pseudocholinesterase enzyme produced by the liver. […] Patients with defective forms of pseudocholinesterase will have a reduced ability to metabolize these two muscle relaxants and will present with prolonged muscular paralysis from standard doses of succinylcholine and mivacurium. […] Heterozygotes will present with an approximate 30 percent increase in the duration of the neuromuscular blockade after standard succinylcholine dosing. Homozygotes, however, can present with neuromuscular blockade for a clinically significant greater duration (2 to 3 hrs). […] With an inherited deficiency, the defective form of the enzyme is unable to metabolize succinylcholine and mivacurium to the same degree, leading to prolonged neuromuscular paralysis for those who inherited atypical pseudocholinesterase enzyme.
  • #15 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    In a literature review consisting of 40 case reports, Hackett and Sakai found the length of paralysis ranged from 50 minutes to 10 hours. […] Duration of blockade is linked to zygosity. Heterozygotes present with a 30% increase in the duration of blockade, in minutes to hours. Homozygotes exhibit an average of at least 2 hours of prolonged blockade. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium.
  • #16 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #17 Recognizing Pseudocholinesterase Deficiency in the Post-operative Patient: Diagnosis and Management in the ICU
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8274813/
    This diagnosis was supported by the patients spontaneous recovery within hours. […] Inherited pseudocholinesterase deficiency severity depends on the homozygosity vs. heterozygosity of the patient. Heterozygotes for the deficient enzyme occur in about 1 in 500 persons, and homozygotes for the deficient enzyme occur in about 1 in 2,000 5,000 persons. Homozygotes have more prolonged paralysis with succinylcholine than heterozygotes.
  • #18 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    In most cases of pseudocholinesterase deficiency, no signs or symptoms of the condition are evident. This condition is first suspected after prolonged recovery from paralysis following general anesthesia in which succinylcholine or mivacurium is administered. […] Physical findings in pseudocholinesterase deficiency are typically limited to the postoperative period and may include: Persistent muscle flaccidity, Absent or shallow spontaneous respirations, Delayed return of motor function after anesthesia. […] In individuals with an inherited form of pseudocholinesterase deficiency, only a single atypical allele is carried in a heterozygous fashion, resulting in a partial deficiency in enzyme activity, which manifests as a slightly prolonged duration of paralysis longer than 5 minutes but shorter than 1 hour following administration of succinylcholine. Severe forms of inherited pseudocholinesterase deficiency may exhibit prolonged muscle paralysis for as long as 8 hours following a single dose of succinylcholine.
  • #19 Orphanet: Hereditary butyrylcholinesterase deficiency
    https://www.orpha.net/en/disease/detail/132
    Butyrylcholinesterase (BChE) deficiency is a metabolic disorder characterised by prolonged apnoea after the use of certain anaesthetic drugs, including the muscle relaxants succinylcholine or mivacurium and other ester local anaesthetics. The duration of the prolonged apnoea varies significantly depending on the extent of the enzyme deficiency. […] Individuals with undetectable levels of BChE activity display a severe prolongation lasting more than 8 hours. The prevalence of this severe form is estimated at 1 in 100 000 individuals. […] Affected individuals are asymptomatic unless exposed to neuromuscular blocking agents, however, prolonged respiratory paralysis following anaesthesia makes mechanical ventilation essential until the excess aesthetic agent is metabolised permitting normal neuromuscular function.
  • #20 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    In a literature review consisting of 40 case reports, Hackett and Sakai found the length of paralysis ranged from 50 minutes to 10 hours. […] Duration of blockade is linked to zygosity. Heterozygotes present with a 30% increase in the duration of blockade, in minutes to hours. Homozygotes exhibit an average of at least 2 hours of prolonged blockade. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium.
  • #21 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #22 Recognizing Pseudocholinesterase Deficiency in the Post-operative Patient: Diagnosis and Management in the ICU
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8274813/
    Succinylcholine is a paralytic drug commonly used during general surgery. It is a depolarizing paralytic, meaning that it achieves its paralytic effect by overstimulating the neuromuscular endplate and thereby blocking neuromuscular junction-released acetylcholine from causing muscular contraction. The onset of action is typically within seconds of injection and usually declines and clears over a course of a few minutes. Clearance of the drug is dependent on the enzyme pseudocholinesterase, which degrades succinylcholine. Genetic or acquired deficiency of pseudocholinesterase therefore results in unexpected prolongation of succinylcholine clearance and prolonged flaccid paralysis. […] Depending on whether the patient is heterozygous or homozygous for the genetic enzyme deficiency, the duration of extended flaccid paralysis can last up to a few hours.
  • #23 cholinesterase_inhibitors [TUSOM | Pharmwiki]
    https://tmedweb.tulane.edu/pharmwiki/doku.php/cholinesterase_inhibitors
    Genetic mutations of pseudocholinesterase can result in reduced enzymatic activity or pseudocholinesterase deficiency. This is an otherwise silent medical condition until patients are given the muscle relaxant succinylcholine during a surgical procedure. Patients with pseudocholinesterase deficiency will exhibit a more intense and greatly prolonged neuromuscular block (e.g. from a normal duration of block of 5 minutes to as long as 8 hours). If not identified, a patient with a pseudocholinesterase deficiency who is given succinylcholine can become hypoxic and potentially die if artificial ventilation is not maintained. […] Pseudocholinesterase deficiency can also increase the risk of systemic toxicity produced by ester-type local anesthetics (e.g. during dental procedures). Such patients are typically given an amide-type local anesthetic instead.
  • #24 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #25 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, refers to a rare acquired or inherited defect in the pseudocholinesterase enzyme produced by the liver. […] Patients with defective forms of pseudocholinesterase will have a reduced ability to metabolize these two muscle relaxants and will present with prolonged muscular paralysis from standard doses of succinylcholine and mivacurium. […] Heterozygotes will present with an approximate 30 percent increase in the duration of the neuromuscular blockade after standard succinylcholine dosing. Homozygotes, however, can present with neuromuscular blockade for a clinically significant greater duration (2 to 3 hrs). […] With an inherited deficiency, the defective form of the enzyme is unable to metabolize succinylcholine and mivacurium to the same degree, leading to prolonged neuromuscular paralysis for those who inherited atypical pseudocholinesterase enzyme.
  • #26 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #27 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Duration of neuromuscular blockade is dependant upon whether the patient is homozygous or heterozygous for the defective enzyme. Heterozygotes will experience a muscular blockade of moderate duration, while homozygotes can have muscular paralysis longer than 2 hours. […] Patients diagnosed with pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. […] The primary complication of pseudocholinesterase deficiency is respiratory failure following the prolonged neuromuscular paralysis after administration of succinylcholine or mivacurium.
  • #28 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #29 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #30 Orphanet: Hereditary butyrylcholinesterase deficiency
    https://www.orpha.net/en/disease/detail/132
    Butyrylcholinesterase (BChE) deficiency is a metabolic disorder characterised by prolonged apnoea after the use of certain anaesthetic drugs, including the muscle relaxants succinylcholine or mivacurium and other ester local anaesthetics. The duration of the prolonged apnoea varies significantly depending on the extent of the enzyme deficiency. […] Individuals with undetectable levels of BChE activity display a severe prolongation lasting more than 8 hours. The prevalence of this severe form is estimated at 1 in 100 000 individuals. […] Affected individuals are asymptomatic unless exposed to neuromuscular blocking agents, however, prolonged respiratory paralysis following anaesthesia makes mechanical ventilation essential until the excess aesthetic agent is metabolised permitting normal neuromuscular function.
  • #31 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #32 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #33 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #34 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #35 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #36 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #37 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #38 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php?jid=ijaa
    Duration of blockade is linked to zygosity. Heterozygotes present with a 30% increase in the duration of blockade, in minutes to hours. Homozygotes exhibit an average of at least 2 hours of prolonged blockade. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium.
  • #39 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #40 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #41 Pseudocholinesterase Deficiency Clinical Presentation: History, Physical Examination, Complications
    https://emedicine.medscape.com/article/247019-clinical
    Complications of pseudocholinesterase deficiency may include: Prolonged ventilator dependence after anesthesia, Potential for misdiagnosis as opioid overdose, brainstem stroke, or residual sedation, Cocaine exposure in undiagnosed individuals may cause sudden cardiac death, Risk of adverse outcomes in emergency surgeries, such as cesarean section, where rapid paralysis and reversal are critical.
  • #42 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. Insufficient enzyme activity alters the metabolism of choline esters, such as succinylcholine and mivacurium, and patients typically present with prolonged post-anesthetic apnea and paralysis. […] A healthy 4-year-old girl of Northern European descent underwent general anesthesia for tonsillotomy, adenoidectomy, and bilateral tympanocentesis. Previously unknown pseudocholinesterase deficiency presented as delayed emergence with sustained apnea and paralysis following administration of mivacurium, necessitating transfer to the pediatric intensive care unit for prolonged post-operative ventilatory support and monitoring. […] Pseudocholinesterase deficiency will almost always present unexpectedly and must be included in the differential diagnosis of delayed emergence.
  • #43 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #44 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
  • #45 cholinesterase_inhibitors [TUSOM | Pharmwiki]
    https://tmedweb.tulane.edu/pharmwiki/doku.php/cholinesterase_inhibitors
    Genetic mutations of pseudocholinesterase can result in reduced enzymatic activity or pseudocholinesterase deficiency. This is an otherwise silent medical condition until patients are given the muscle relaxant succinylcholine during a surgical procedure. Patients with pseudocholinesterase deficiency will exhibit a more intense and greatly prolonged neuromuscular block (e.g. from a normal duration of block of 5 minutes to as long as 8 hours). If not identified, a patient with a pseudocholinesterase deficiency who is given succinylcholine can become hypoxic and potentially die if artificial ventilation is not maintained. […] Pseudocholinesterase deficiency can also increase the risk of systemic toxicity produced by ester-type local anesthetics (e.g. during dental procedures). Such patients are typically given an amide-type local anesthetic instead.
  • #46 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #47 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, refers to a rare acquired or inherited defect in the pseudocholinesterase enzyme produced by the liver. […] Patients with defective forms of pseudocholinesterase will have a reduced ability to metabolize these two muscle relaxants and will present with prolonged muscular paralysis from standard doses of succinylcholine and mivacurium. […] Heterozygotes will present with an approximate 30 percent increase in the duration of the neuromuscular blockade after standard succinylcholine dosing. Homozygotes, however, can present with neuromuscular blockade for a clinically significant greater duration (2 to 3 hrs). […] With an inherited deficiency, the defective form of the enzyme is unable to metabolize succinylcholine and mivacurium to the same degree, leading to prolonged neuromuscular paralysis for those who inherited atypical pseudocholinesterase enzyme.
  • #48
    https://homeopathyaajtak.com/diseases/p/pseudocholinesterase-deficiency-144
    The cause of pseudocholinesterase deficiency or PD is an inherited gene passed down from both of your parents. If you have one faulty gene, you won’t have the condition, but you may have problems with anesthesia drugs. You can pass the faulty gene to your children. But to have pseudocholinesterase deficiency, your child must inherit a faulty gene from both parents. […] Your risk for pseudocholinesterase deficiency or PD is higher if someone in your family has the condition.
  • #49 Recognizing Pseudocholinesterase Deficiency in the Post-operative Patient: Diagnosis and Management in the ICU
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8274813/
    This diagnosis was supported by the patients spontaneous recovery within hours. […] Inherited pseudocholinesterase deficiency severity depends on the homozygosity vs. heterozygosity of the patient. Heterozygotes for the deficient enzyme occur in about 1 in 500 persons, and homozygotes for the deficient enzyme occur in about 1 in 2,000 5,000 persons. Homozygotes have more prolonged paralysis with succinylcholine than heterozygotes.
  • #50 Genetic Testing – pseudocholinesterase, deficiency …, (Pseudocholinesterase deficiency) – Gen BCHE. – IVAMI
    https://www.ivami.com/en/genetic-testing-human-gene-mutations-diseases-neoplasias-and-pharmacogenetics/3684-genetic-testing-pseudocholinesterase-deficiency-pseudocholinesterase-deficiency-gen-bche
    Pseudocholinesterase deficiency may also have non – genetic causes. In these cases, the alteration is called pseudocholinesterase acquired deficiency, which is not inherited. […] When the disease is due to genetic causes, it inherited with an autosomal recessive pattern, meaning that two copies of the gene in each cell have mutations. […] In some cases, carriers of the BCHE gene mutations take longer than usual to remove choline ester of body drugs, but less time than individuals with two copies of the altered gene in each cell.
  • #51 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, refers to a rare acquired or inherited defect in the pseudocholinesterase enzyme produced by the liver. […] Patients with defective forms of pseudocholinesterase will have a reduced ability to metabolize these two muscle relaxants and will present with prolonged muscular paralysis from standard doses of succinylcholine and mivacurium. […] Heterozygotes will present with an approximate 30 percent increase in the duration of the neuromuscular blockade after standard succinylcholine dosing. Homozygotes, however, can present with neuromuscular blockade for a clinically significant greater duration (2 to 3 hrs). […] With an inherited deficiency, the defective form of the enzyme is unable to metabolize succinylcholine and mivacurium to the same degree, leading to prolonged neuromuscular paralysis for those who inherited atypical pseudocholinesterase enzyme.
  • #52 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #53 Genetic Testing – pseudocholinesterase, deficiency …, (Pseudocholinesterase deficiency) – Gen BCHE. – IVAMI
    https://www.ivami.com/en/genetic-testing-human-gene-mutations-diseases-neoplasias-and-pharmacogenetics/3684-genetic-testing-pseudocholinesterase-deficiency-pseudocholinesterase-deficiency-gen-bche
    Pseudocholinesterase deficiency may also have non – genetic causes. In these cases, the alteration is called pseudocholinesterase acquired deficiency, which is not inherited. […] When the disease is due to genetic causes, it inherited with an autosomal recessive pattern, meaning that two copies of the gene in each cell have mutations. […] In some cases, carriers of the BCHE gene mutations take longer than usual to remove choline ester of body drugs, but less time than individuals with two copies of the altered gene in each cell.
  • #54 Pseudocholinesterase Deficiency – Norton & Elaine Sarnoff Center for Jewish Genetics
    https://www.jewishgenetics.org/genetic-disorders/search-genetic-disorders/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency happens when people dont have enough of an enzyme called pseudocholinesterase. This enzyme is important for breaking down certain drugs used during surgery, like succinylcholine and mivacurium, which help relax the muscles. […] If someone has pseudocholinesterase deficiency, these drugs dont work well for them. This can lead to problems because the muscles that control breathing might stop working properly, making them unable to breathe (apnea). […] Carriers generally do not have symptoms, but they may sometimes experience a short period of breathing paralysis following anesthesia.
  • #55
    https://arsiv.dusunenadamdergisi.org/ing/fArticledetails4370.html?MkID=1286
    Pseudocholinesterase (PCE) deficiency is an inherited condition in which recovery from anesthetic agents like succinylcholine and mivacurium is slow and complicated by prolonged paralysis of respiratory muscles in susceptible patients. […] Main outcome of the condition is prolonged paralysis and apnea after anesthetic procedures. […] The PCE level of the patient in the case outlined above was the lowest of the values we have ever detected in our ECT center, and as far as we know it was the lowest value reported in the ECT literature. […] Prolonged apnea is an expected problem when succinylcholine is used in ECT in cases of PCE deficiency and mechanical ventilation is needed continuously until the succinylcholine paralysis ends. […] We did not detect any acquired cause for the PCE deficiency in our case, so we can conclude that it was a genetic variant of PCE deficiency. […] Therefore, we can also expect the incidence of acquired cases of PCE deficiency to be increased in these patients compared to the general population.
  • #56 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #57 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #58 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #59 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #60 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #61 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #62 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #63 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #64 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is usually identified when a patient experiences long-lasting muscle weakness after being administered standard doses of certain medications like succinylcholine or mivacurium. […] Individuals with certain health conditions like cancer, severe burns, heart attack, heart failure, pregnancy, liver disease, hemodialysis, and chronic infections may develop a secondary form of pseudocholinesterase deficiency. These conditions may cause reduced levels of the pseudocholinesterase enzyme, making the patient more likely to experience long-lasting muscle weakness with standard doses of succinylcholine or mivacurium. […] Its important to note that there are no unique physical signs of pseudocholinesterase deficiency. People with this condition may seem perfectly healthy (if its inherited) or they may show signs related to their other health conditions (if its acquired).
  • #65 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #66 PPT – P seudocholinesterase Deficiency PowerPoint Presentation, free download – ID:1901818
    https://www.slideserve.com/sherry/p-seudocholinesterase-deficiency
    Pseudocholinesterase Deficiency. Etiology and considerations. Objectives. 1. Review physiology, diagnosis, prevalence, and effects of pseudocholinesterase deficiency […] Reportedly 80% of patients presenting with symptoms will have atypical pseudocholinesterase present. […] Onset of symptoms usually occurs when 75% suppression of the wild type is present. Can occur with as little as 50% depression, depending on comorbidities and coexisting conditions. […] Each can decrease the effectiveness of normal BChE: Advancing Age, Renal failure, Malnutrition, Hepatic failure, Pregnancy. […] Homozygous: variable paralysis, from 1-4 hours or more. […] Congenital deficiency: Avoid Succinylcholine with known congenital deficiency. Avoid Tetracaine, Chloroprocaine, and Procaine (OB patients). Consider NDMR in patients with potential for attenuated pseudocholinesterase activity. […] Pseudocholinesterase deficiency can be: 1. Drug, environment, or comorbidity induced (affecting quality) 2. Congenital (affecting quantity of true BChE). Heterozygous carriers -slightly prolonged paralysis. Homozygous silent type -most prolonged paralysis.
  • #67 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Duration of neuromuscular blockade is dependant upon whether the patient is homozygous or heterozygous for the defective enzyme. Heterozygotes will experience a muscular blockade of moderate duration, while homozygotes can have muscular paralysis longer than 2 hours. […] Patients diagnosed with pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. […] The primary complication of pseudocholinesterase deficiency is respiratory failure following the prolonged neuromuscular paralysis after administration of succinylcholine or mivacurium.
  • #68 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #69 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #70 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Duration of neuromuscular blockade is dependant upon whether the patient is homozygous or heterozygous for the defective enzyme. Heterozygotes will experience a muscular blockade of moderate duration, while homozygotes can have muscular paralysis longer than 2 hours. […] Patients diagnosed with pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. […] The primary complication of pseudocholinesterase deficiency is respiratory failure following the prolonged neuromuscular paralysis after administration of succinylcholine or mivacurium.
  • #71 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #72 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is treated with respiratory support using a mechanical ventilator and sedation to reduce anxiety. Nerve stimulation is used to check if muscle function is returning. The preferred treatment approach is to provide support with sedation and mechanical ventilation until recovery takes place, which usually occurs within several hours. […] If someone is diagnosed with Pseudocholinesterase Deficiency after being exposed to certain drugs, they can fully recover as muscle function naturally returns. However, during this time, they will need mechanical ventilation and close observation by their medical team to prevent hypoxic respiratory failure.
  • #73 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    Duration of neuromuscular blockade is dependant upon whether the patient is homozygous or heterozygous for the defective enzyme. Heterozygotes will experience a muscular blockade of moderate duration, while homozygotes can have muscular paralysis longer than 2 hours. […] Patients diagnosed with pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. […] The primary complication of pseudocholinesterase deficiency is respiratory failure following the prolonged neuromuscular paralysis after administration of succinylcholine or mivacurium.
  • #74 Recognizing Pseudocholinesterase Deficiency in the Post-operative Patient: Diagnosis and Management in the ICU
    https://pmc.ncbi.nlm.nih.gov/articles/PMC8274813/
    This diagnosis was supported by the patients spontaneous recovery within hours. […] Inherited pseudocholinesterase deficiency severity depends on the homozygosity vs. heterozygosity of the patient. Heterozygotes for the deficient enzyme occur in about 1 in 500 persons, and homozygotes for the deficient enzyme occur in about 1 in 2,000 5,000 persons. Homozygotes have more prolonged paralysis with succinylcholine than heterozygotes.
  • #75 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
  • #76 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #77 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Prolonged paralysis […] The patient experiences prolonged paralysis of the respiratory muscles, requiring an extended period of time during which the patient must be mechanically ventilated. […] If this happens, there are two major complications that can arise. First, the patient may lie awake and paralyzed while medical providers try to determine the cause of the patient’s unresponsiveness. Second, the breathing tube may be removed before the patient is strong enough to breathe properly, potentially causing respiratory arrest. […] The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures. […] In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
  • #78 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is treated with respiratory support using a mechanical ventilator and sedation to reduce anxiety. Nerve stimulation is used to check if muscle function is returning. The preferred treatment approach is to provide support with sedation and mechanical ventilation until recovery takes place, which usually occurs within several hours. […] If someone is diagnosed with Pseudocholinesterase Deficiency after being exposed to certain drugs, they can fully recover as muscle function naturally returns. However, during this time, they will need mechanical ventilation and close observation by their medical team to prevent hypoxic respiratory failure.
  • #79 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a condition that makes a person extremely sensitive to certain anesthetic medications. People with pseudocholinesterase deficiency may not be able to move or breathe on their own for several hours after receiving these medications. Mechanical ventilation supports your breathing until you recover. […] People with pseudocholinesterase deficiency usually dont know they have it until they receive choline esters during general anesthesia. Hallmark symptoms include: Extreme muscle relaxation that lasts several hours longer than expected. Temporary paralysis of your respiratory muscles. […] It can take several hours for pseudocholinesterase deficiency symptoms to improve. This timeline varies from person to person. […] If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, youll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, theyll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] Theres no cure for pseudocholinesterase deficiency. But knowing whether you have it can help you avoid symptoms like temporary muscle paralysis and breathing issues after surgery.
  • #80 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is treated with respiratory support using a mechanical ventilator and sedation to reduce anxiety. Nerve stimulation is used to check if muscle function is returning. The preferred treatment approach is to provide support with sedation and mechanical ventilation until recovery takes place, which usually occurs within several hours. […] If someone is diagnosed with Pseudocholinesterase Deficiency after being exposed to certain drugs, they can fully recover as muscle function naturally returns. However, during this time, they will need mechanical ventilation and close observation by their medical team to prevent hypoxic respiratory failure.
  • #81 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php?jid=ijaa
    Duration of blockade is linked to zygosity. Heterozygotes present with a 30% increase in the duration of blockade, in minutes to hours. Homozygotes exhibit an average of at least 2 hours of prolonged blockade. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium.
  • #82 Pseudocholinesterase Deficiency: Background, Etiology, Pathophysiology
    https://emedicine.medscape.com/article/247019-overview
    Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited enzyme abnormality that results in abnormally slow metabolic degradation of exogenous choline ester drugs such as succinylcholine and mivacurium. If there is a deficiency in the plasma activity of pseudocholinesterase, prolonged muscular paralysis may occur, resulting in an extended need for mechanical ventilation. […] This condition is recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine. The mainstay of treatment in these cases is ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle. […] Pseudocholinesterase deficiency can result in higher levels of intact succinylcholine molecules reaching receptors in the neuromuscular junction, causing the duration of paralytic effect to continue for as long as 8 hours. […] Patients with diagnosed pseudocholinesterase deficiency after exposure to succinylcholine or mivacurium are expected to make a full recovery, following the spontaneous return of motor function. Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #83 Pseudocholinesterase Deficiency – MD Searchlight
    https://mdsearchlight.com/health/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency is treated with respiratory support using a mechanical ventilator and sedation to reduce anxiety. Nerve stimulation is used to check if muscle function is returning. The preferred treatment approach is to provide support with sedation and mechanical ventilation until recovery takes place, which usually occurs within several hours. […] If someone is diagnosed with Pseudocholinesterase Deficiency after being exposed to certain drugs, they can fully recover as muscle function naturally returns. However, during this time, they will need mechanical ventilation and close observation by their medical team to prevent hypoxic respiratory failure.
  • #84 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
  • #85 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    A timely working diagnosis of pseudocholinesterase deficiency is crucial, because it can help avoid unnecessary exposure to other medication (for example, naloxone, flumazenil, and physostigmine), and because the prognosis of pseudocholinesterase deficiency is excellent once supportive management has been initiated. Treatment of perioperative complications usually involves continued mechanical ventilation until the culprit agent has been metabolized and any residual paralysis has subsided. […] In summary, pseudocholinesterase deficiency is a rare disorder that commonly presents as prolonged apnea and paralysis following general anesthesia after induction with succinylcholine or mivacurium.
  • #86 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a condition that makes a person extremely sensitive to certain anesthetic medications. People with pseudocholinesterase deficiency may not be able to move or breathe on their own for several hours after receiving these medications. Mechanical ventilation supports your breathing until you recover. […] People with pseudocholinesterase deficiency usually dont know they have it until they receive choline esters during general anesthesia. Hallmark symptoms include: Extreme muscle relaxation that lasts several hours longer than expected. Temporary paralysis of your respiratory muscles. […] It can take several hours for pseudocholinesterase deficiency symptoms to improve. This timeline varies from person to person. […] If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, youll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, theyll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] Theres no cure for pseudocholinesterase deficiency. But knowing whether you have it can help you avoid symptoms like temporary muscle paralysis and breathing issues after surgery.
  • #87 Pseudocholinesterase deficiency – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pseudocholinesterase-deficiency/diagnosis-treatment/drc-20354545
    Pseudocholinesterase deficiency may be suspected when you have problems recovering muscle control and breathing after you get the muscle relaxant succinylcholine as part of anesthesia. […] If you have pseudocholinesterase deficiency, the health care provider who gives you anesthesia (anesthesiologist) can avoid succinylcholine that may trigger prolonged muscle relaxation. […] There is no cure for pseudocholinesterase deficiency. If you have the disorder and you receive a muscle relaxant that prolongs your anesthesia recovery, you’ll likely need medical assistance. […] With pseudocholinesterase deficiency, you may also be sensitive to other medications.
  • #88 My father has pseudocholinesterase deficiency. Can I also get it?
    https://www.icliniq.com/qa/pseudocholinesterase-deficiency/do-i-have-pseudocholinesterase-deficiency-based-on-the-blood-results
    You may have atypical pseudocholinesterase deficiency (a rare genetic disorder that makes people extremely sensitive to certain muscle relaxants used during general anesthesia) due to a heterozygous mutation, which is hereditary. […] Pseudocholinesterase is an enzyme responsible for the metabolism of certain anesthetic drugs like succinylcholine and ester group of local anesthetics. […] But still, some drugs can be avoided if you require anesthesia. It is not like you can not have a surgery. If you have a surgery in the future, please tell your anesthetist about this, so he will be more careful in choosing drugs.
  • #89 Pseudocholinesterase deficiency // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/pseudocholinesterase-deficiency
    If you have pseudocholinesterase deficiency, the health care provider who gives you anesthesia (anesthesiologist) can avoid succinylcholine that may trigger prolonged muscle relaxation. The anesthesiologist can choose other muscle relaxants instead. […] If you have the disorder and you receive a muscle relaxant that prolongs your anesthesia recovery, you’ll likely need medical assistance. If needed, a machine that takes over the work of breathing (mechanical ventilation support) and sedation are provided while you recover and start breathing on your own. This may take several hours.
  • #90 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php?jid=ijaa
    Duration of blockade is linked to zygosity. Heterozygotes present with a 30% increase in the duration of blockade, in minutes to hours. Homozygotes exhibit an average of at least 2 hours of prolonged blockade. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium.
  • #91 Mayo Clinic Health Library – Pseudocholinesterase deficiency | Swiss Medical Network
    https://www.swissmedical.net/de/healtcare-library/con-20200770
    This causes sensitivity to certain muscle relaxants used during anesthesia. Muscles may stay relaxed for too long, interfering with movement and breathing. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected. During that time, you can’t move or breathe on your own. The length of time may vary widely among people with this disorder. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long after getting succinylcholine. This prevents you from moving or breathing on your own for a few hours longer than expected. How long it takes your body to metabolize the drug depends on how much pseudocholinesterase enzyme you produce and how well it functions. […] If you have pseudocholinesterase deficiency, the health care provider who gives you anesthesia (anesthesiologist) can avoid succinylcholine that may trigger prolonged muscle relaxation. The anesthesiologist can choose other muscle relaxants instead.
  • #92
    https://www.aaaai.org/allergist-resources/ask-the-expert/answers/2022/succinylcholine
    There is a well-known familial condition called pseudocholinesterase deficiency which is associated with prolonged apnea after succinylcholine administration. […] BChE activity can be determined for pre-operative screening and detection of at-risk of prolonged apnea in persons receiving succinylcholine. […] Given the current literature, I recommend performing genetic testing for pseudocholinesterase deficiency if the symptoms were prolonged apnea in family members.
  • #93 My father has pseudocholinesterase deficiency. Can I also get it?
    https://www.icliniq.com/qa/pseudocholinesterase-deficiency/do-i-have-pseudocholinesterase-deficiency-based-on-the-blood-results
    You may have atypical pseudocholinesterase deficiency (a rare genetic disorder that makes people extremely sensitive to certain muscle relaxants used during general anesthesia) due to a heterozygous mutation, which is hereditary. […] Pseudocholinesterase is an enzyme responsible for the metabolism of certain anesthetic drugs like succinylcholine and ester group of local anesthetics. […] But still, some drugs can be avoided if you require anesthesia. It is not like you can not have a surgery. If you have a surgery in the future, please tell your anesthetist about this, so he will be more careful in choosing drugs.
  • #94 Pseudocholinesterase deficiency – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pseudocholinesterase-deficiency/diagnosis-treatment/drc-20354545
    Pseudocholinesterase deficiency may be suspected when you have problems recovering muscle control and breathing after you get the muscle relaxant succinylcholine as part of anesthesia. […] If you have pseudocholinesterase deficiency, the health care provider who gives you anesthesia (anesthesiologist) can avoid succinylcholine that may trigger prolonged muscle relaxation. […] There is no cure for pseudocholinesterase deficiency. If you have the disorder and you receive a muscle relaxant that prolongs your anesthesia recovery, you’ll likely need medical assistance. […] With pseudocholinesterase deficiency, you may also be sensitive to other medications.
  • #95 cholinesterase_inhibitors [TUSOM | Pharmwiki]
    https://tmedweb.tulane.edu/pharmwiki/doku.php/cholinesterase_inhibitors
    Genetic mutations of pseudocholinesterase can result in reduced enzymatic activity or pseudocholinesterase deficiency. This is an otherwise silent medical condition until patients are given the muscle relaxant succinylcholine during a surgical procedure. Patients with pseudocholinesterase deficiency will exhibit a more intense and greatly prolonged neuromuscular block (e.g. from a normal duration of block of 5 minutes to as long as 8 hours). If not identified, a patient with a pseudocholinesterase deficiency who is given succinylcholine can become hypoxic and potentially die if artificial ventilation is not maintained. […] Pseudocholinesterase deficiency can also increase the risk of systemic toxicity produced by ester-type local anesthetics (e.g. during dental procedures). Such patients are typically given an amide-type local anesthetic instead.
  • #96 Genetic Testing – pseudocholinesterase, deficiency …, (Pseudocholinesterase deficiency) – Gen BCHE. – IVAMI
    https://www.ivami.com/en/genetic-testing-human-gene-mutations-diseases-neoplasias-and-pharmacogenetics/3684-genetic-testing-pseudocholinesterase-deficiency-pseudocholinesterase-deficiency-gen-bche
    Pseudocholinesterase deficiency is a condition that results in an increased sensitivity to certain muscle relaxants drugs used in general anesthesia, called choline esters. These fast acting medications such as succinylcholine and mivacurium are supplied to relax skeletal muscles, including the muscles involved in breathing. […] However, people with deficiency pseudocholinesterase may not be able to move or breathe on their own over a couple of hours after administering medications. Affected individuals require mechanical ventilation until the medication is eliminated from the body. People with pseudocholinesterase deficiency may also be more sensitive to other drugs, including procaine local anesthetic and specific agricultural pesticides. […] Lack of functional pseudocholinesterase impairs the body’s ability to efficiently decompose drugs choline ester, causing abnormally prolonged effects of drugs.
  • #97 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency is a condition that makes a person extremely sensitive to certain anesthetic medications. People with pseudocholinesterase deficiency may not be able to move or breathe on their own for several hours after receiving these medications. Mechanical ventilation supports your breathing until you recover. […] People with pseudocholinesterase deficiency usually dont know they have it until they receive choline esters during general anesthesia. Hallmark symptoms include: Extreme muscle relaxation that lasts several hours longer than expected. Temporary paralysis of your respiratory muscles. […] It can take several hours for pseudocholinesterase deficiency symptoms to improve. This timeline varies from person to person. […] If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, youll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, theyll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] Theres no cure for pseudocholinesterase deficiency. But knowing whether you have it can help you avoid symptoms like temporary muscle paralysis and breathing issues after surgery.