Niedobór pseudocholinesterazy
Leczenie

Niedobór pseudocholinesterazy to rzadkie zaburzenie enzymatyczne, które prowadzi do przedłużonego zwiotczenia mięśniowego po podaniu standardowych dawek środków zwiotczających metabolizowanych przez ten enzym, takich jak sukcynylocholina i miwakurium. Diagnostyka obejmuje oznaczenie poziomu pseudocholinesterazy w surowicy oraz badania genetyczne, a postępowanie przedzabiegowe polega na unikaniu tych leków i stosowaniu alternatywnych zwiotczających, np. rokuronium, wekuronium, atrakurium czy cisatrakurium. W przypadku przedłużonej blokady nerwowo-mięśniowej konieczne jest wspomaganie wentylacji mechanicznej oraz sedacja, a odwracanie blokady inhibitorami cholinesterazy (np. neostygminą) jest przeciwwskazane ze względu na ryzyko nasilenia zwiotczenia. Metody takie jak transfuzje świeżo mrożonego osocza (FFP) czy koncentratu krwinek czerwonych są kontrowersyjne i niezalecane rutynowo ze względu na zmienność aktywności enzymu i ryzyko powikłań.

Leczenie pseudocholinesterazy/” title=”niedobór pseudocholinesterazy” class=”to-tag” data-termid=”103109″>niedoboru pseudocholinesterazy

Niedobór pseudocholinesterazy to rzadkie zaburzenie enzymatyczne, które może być dziedziczone genetycznie lub nabyte w wyniku chorób, urazów czy stosowania niektórych leków. Charakteryzuje się ono zmniejszoną zdolnością do metabolizowania i inaktywacji określonych leków zwłaszcza środków zwiotczających mięśnie, co prowadzi do przedłużonego zwiotczenia mięśniowego po standardowych dawkach tych preparatów12. Obecnie nie istnieje skuteczna metoda leczenia przyczynowego tego zaburzenia, a postępowanie koncentruje się głównie na leczeniu objawowym i profilaktyce34.

Postępowanie w przypadku rozpoznanego niedoboru pseudocholinesterazy

U pacjentów z rozpoznanym niedoborem pseudocholinesterazy kluczowe znaczenie ma odpowiednie postępowanie przed planowanymi zabiegami wymagającymi znieczulenia5. W takich przypadkach należy zastosować następujące środki zapobiegawcze:

  • Unikanie stosowania sukcynylocholiny i miwakurium – środków zwiotczających, które są metabolizowane przez pseudocholinesterazę67
  • Wybór alternatywnych środków zwiotczających, które nie są zależne od pseudocholinesterazy, takich jak atrakurium, rokuronium lub wekuronium68
  • Pacjenci powinni informować personel medyczny o swoim stanie przed jakimkolwiek zabiegiem9
  • Zaleca się noszenie bransoletki lub naszyjnika medycznego oraz posiadanie karty informacyjnej o schorzeniu, co ma szczególne znaczenie w sytuacjach nagłych1011

Leczenie przedłużonego zwiotczenia mięśniowego

W przypadku wystąpienia przedłużonego zwiotczenia mięśniowego po zastosowaniu środków zwiotczających u pacjentów z niedoborem pseudocholinesterazy, postępowanie obejmuje12:

  • Mechaniczna wentylacja – podstawową metodą leczenia jest wspomaganie oddychania poprzez zastosowanie wentylacji mechanicznej do czasu samoczynnego ustąpienia blokady nerwowo-mięśniowej1312
  • Sedacja – pacjenci powinni pozostawać w sedacji podczas okresu zwiotczenia, aby zmniejszyć ryzyko świadomości przy braku funkcji motorycznych1412
  • Monitorowanie funkcji nerwowo-mięśniowej – powtarzana ocena powrotu funkcji motorycznych przy użyciu stymulacji nerwów12
  • Oczekiwanie na samoistne ustąpienie – powrót do zdrowia następuje w wyniku biernej dyfuzji sukcynylocholiny z połączenia nerwowo-mięśniowego13

Metody potencjalnego przyspieszenia ustąpienia blokady nerwowo-mięśniowej

Istnieją pewne metody, które teoretycznie mogą skrócić czas trwania blokady nerwowo-mięśniowej, jednak ich skuteczność jest ograniczona i budzą one kontrowersje1415:

  • Świeżo mrożone osocze (FFP) – może dostarczyć egzogennej pseudocholinesterazy i potencjalnie zwiększyć aktywność enzymu, jednak praktyka ta nie jest rutynowo zalecana ze względu na ryzyko powikłań infekcyjnych1316
  • Krew pełna lub koncentrat krwinek czerwonych – również zawierają pseudocholinesterazę i mogą poprawić aktywność enzymatyczną1416
  • Ludzka surowicza cholinesteraza – dostępna w niektórych krajach europejskich, jednak jej stosowanie nie jest powszechne ze względu na ograniczoną skuteczność16

Należy podkreślić, że transfuzje FFP i krwi pełnej nie są rekomendowanymi metodami leczenia, ponieważ poziom aktywności enzymatycznej może się różnić w zależności od jednostki, a brak jest ustalonych wytycznych dotyczących liczby jednostek potrzebnych do odwrócenia blokady mięśniowej16. Ponadto, stanowi to nieoptymalne wykorzystanie cennych produktów krwiopochodnych i wiąże się z ryzykiem infekcji jatrogennych17.

Przeciwwskazane interwencje farmakologiczne

Niektóre interwencje farmakologiczne są przeciwwskazane w leczeniu przedłużonej blokady nerwowo-mięśniowej u pacjentów z niedoborem pseudocholinesterazy1819:

  • Inhibitory cholinesterazy – leki takie jak neostygmina i fizostygmina, które są powszechnie stosowane w anestezjologii, mogą antagonizować i hamować aktywność pseudocholinesterazy17
  • Odwracanie blokady farmakologicznej – podawanie inhibitorów cholinesterazy, takich jak neostygmina, jest kontrowersyjne w przypadku odwracania bezdechu związanego z sukcynylocholiną u pacjentów z niedoborem pseudocholinesterazy. Efekty mogą być przejściowe i potencjalnie mogą prowadzić do nasilenia blokady nerwowo-mięśniowej19

Efekty stosowania inhibitorów cholinesterazy mogą być przejściowe i potencjalnie mogą prowadzić do nasilenia blokady nerwowo-mięśniowej1720. Dlatego zaleca się zachowawcze podejście z sedacją i wentylacją mechaniczną do czasu samoczynnego powrotu funkcji mięśniowej12.

Alternatywne środki zwiotczające w znieczuleniu ogólnym

U pacjentów z niedoborem pseudocholinesterazy, którzy wymagają znieczulenia ogólnego ze zwiotczeniem mięśniowym, zaleca się stosowanie alternatywnych środków zwiotczających, które nie są metabolizowane przez pseudocholinesterazę76:

  • Rokuronium – jeden z najczęściej stosowanych środków zwiotczających u osób z niedoborem pseudocholinesterazy7
  • Wekuronium – bezpieczny środek zwiotczający o działaniu niedepolaryzującym6
  • Atrakurium – środek zwiotczający o działaniu niedepolaryzującym, który jest metabolizowany niezależnie od pseudocholinesterazy6
  • Cisatrakurium – również bezpieczny do stosowania u pacjentów z niedoborem pseudocholinesterazy17

Specjalistyczne zastosowania środków zwiotczających

W szczególnych przypadkach, takich jak elektrowstrząsy (ECT), które wymagają krótkotrwałego zwiotczenia mięśniowego, również zaleca się stosowanie alternatywnych środków2122:

  • Rokuronium z sugammadeksem – skojarzenie to okazało się skuteczną i bezpieczną alternatywą w sytuacjach, gdy sukcynylocholina jest przeciwwskazana2324
  • Sugammadeks – umożliwia szybkie odwrócenie blokady mięśniowej wywołanej przez rokuronium, co jest szczególnie korzystne u pacjentów z niedoborem pseudocholinesterazy2526

Korzyści wynikające z zastosowania rokuronium z sugammadeksem podczas ECT, w tym szybki powrót ze zwiotczenia mięśniowego i mniejsza liczba działań niepożądanych, mogą przewyższać wysoki koszt jego stosowania24. Przewaga rokuronium-sugammadeksu nad sukcynylocholiną jest jeszcze bardziej widoczna u osób z niedoborem pseudocholinesterazy, ponieważ w takich przypadkach można oczekiwać znacznego wydłużenia spontanicznego powrotu do zdrowia po działaniu środków zwiotczających24.

Leki do unikania u pacjentów z niedoborem pseudocholinesterazy

Pacjenci z niedoborem pseudocholinesterazy powinni unikać stosowania następujących leków727:

  • Sukcynylocholina – depolaryzujący środek zwiotczający7
  • Miwakurium – niedepolaryzujący środek zwiotczający7
  • Miejscowe środki znieczulające typu estrowego:

Badania diagnostyczne i konsultacje specjalistyczne

W przypadku podejrzenia niedoboru pseudocholinesterazy, zaleca się przeprowadzenie odpowiednich badań diagnostycznych i konsultacji specjalistycznych19:

  • Konsultacja genetyczna – może pomóc w identyfikacji specyficznych alleli genotypowych przyczyniających się do niedoboru pseudocholinesterazy19
  • Badania molekularne – sekwencja DNA genu pseudocholinesterazy i jego struktura aminokwasowa są znane, dzięki czemu nietypowe allele mogą być identyfikowane poprzez badania amplifikacji PCR z wykorzystaniem DNA wyekstrahowanego z leukocytów w próbce krwi19
  • Określenie poziomu pseudocholinesterazy w surowicy – może być częścią rutynowej przedoperacyjnej diagnostyki laboratoryjnej25

Wykrycie poziomu pseudocholinesterazy w ramach przedoperacyjnej oceny może pomóc w zmniejszeniu powikłań związanych ze znieczuleniem2628.

Rokowanie

Rokowanie u pacjentów z niedoborem pseudocholinesterazy jest zazwyczaj dobre, pod warunkiem odpowiedniego postępowania2930:

  • Pełny powrót do zdrowia – pacjenci z rozpoznanym niedoborem pseudocholinesterazy mogą oczekiwać pełnego powrotu do zdrowia po podaniu sukcynylocholiny lub miwakurium, pod warunkiem zapewnienia odpowiedniej wentylacji mechanicznej i czujnego monitorowania20
  • Powrót do spontanicznego oddychania – zwykle następuje w ciągu kilku godzin31
  • Brak długoterminowych skutków – niedobór pseudocholinesterazy zazwyczaj ma łagodny przebieg i nie powoduje długotrwałych konsekwencji po odpowiednim leczeniu30

Profilaktyka i edukacja pacjentów

Kluczową rolę w zarządzaniu niedoborem pseudocholinesterazy odgrywa edukacja pacjentów i profilaktyka432:

  • Informowanie personelu medycznego – pacjenci powinni zawsze informować lekarzy i innych pracowników służby zdrowia o swoim stanie, szczególnie przed zabiegami chirurgicznymi9
  • Identyfikatory medyczne – noszenie bransoletki lub naszyjnika medycznego oraz posiadanie karty informacyjnej o schorzeniu3311
  • Testowanie członków rodziny – w przypadku rodzinnego występowania niedoboru pseudocholinesterazy, zaleca się testowanie członków rodziny przed zabiegami wymagającymi znieczulenia4
  • Świadomość leków przeciwwskazanych – pacjenci powinni być świadomi, jakich leków należy unikać15

Właściwa edukacja pacjentów i współpraca między pacjentem a zespołem medycznym stanowią podstawę skutecznego zarządzania niedoborem pseudocholinesterazy i zapobiegania potencjalnym powikłaniom3435.

Nowe kierunki badań

Prowadzone są badania nad nowymi metodami leczenia niedoboru pseudocholinesterazy. W miarę pogłębiania wiedzy na temat genetyki i biologii tego schorzenia, na horyzoncie pojawiają się lepsze opcje terapeutyczne35. Istnieje potrzeba dalszych badań w celu potwierdzenia skuteczności wspomnianych powyżej środków zapobiegawczych i terapeutycznych36.

Zrozumienie implikacji anestezjologicznych niedoboru pseudocholinesterazy pomaga pracownikom służby zdrowia zapewnić bezpieczną i skuteczną opiekę, co jest szczególnie istotne dla pacjentów podczas zabiegów chirurgicznych35.

Kolejne rozdziały

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Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 12.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Pseudocholinesterase Deficiency: Anesthesia Considerations – Radius Anesthesia of Michigan
    https://radiusmichigan.com/pseudocholinesterase-deficiency-anesthesia/
    Pseudocholinesterase deficiency is characterized by the reduced ability to metabolize and thereby terminate the effects of certain muscle relaxants, resulting in prolonged muscular paralysis from standard doses. […] Treatment for a patient with this condition who is exposed to one of these choline esters includes mechanical ventilation until the effects of the neuromuscular blocking agent resolve spontaneously. […] While no cure exists for pseudocholinesterase deficiency, various interventions in cases of exposure can successfully reverse paralysis and apnea: these include administration of whole blood and the use of human serum cholinesterase. […] Pseudocholinesterase individuals in need of anesthesia can avoid paralysis by undergoing treatment with different neuromuscular blockers, such as atracurium and rocuronium.
  • #2 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. […] 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. […] Patients with known pseudocholinesterase deficiency may wear a medic-alert bracelet that will notify healthcare workers of increased risk from administration of succinylcholine. […] Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #3 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://www.medicoverhospitals.in/diseases/pseudocholinesterase-deficiency/
    Currently, there is no cure for pseudocholinesterase deficiency. However, several management strategies can help mitigate its effects, especially in surgical settings. […] It’s crucial for individuals diagnosed with pseudocholinesterase deficiency to inform their healthcare providers before undergoing surgery. Anesthesiologists can adjust the choice of drugs to avoid those metabolized by the cholinesterase enzyme, thereby reducing the risk of complications. […] In cases where standard anesthetics are used, close monitoring in a post-operative setting is necessary. Mechanical ventilation and supportive care may be required until muscle function and breathing return to normal. […] Certain drugs, such as neostigmine, may be administered to counteract the prolonged effects of muscle relaxants. These medications work by inhibiting the breakdown of acetylcholine, thereby compensating for the deficient pseudocholinesterase activity. […] Treatment focuses on careful management during anesthesia and may include using alternative medications that do not rely on cholinesterase for metabolism.
  • #4 Pseudocholinesterase deficiency – Symptoms & causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pseudocholinesterase-deficiency/symptoms-causes/syc-20354543
    Pseudocholinesterase deficiency can be caused by a gene change (mutation) that is inherited. This condition can also be caused by illness, injury or certain medications. […] There is no cure for pseudocholinesterase deficiency. But if you’re diagnosed with the disorder, your health care provider can use other types of muscle relaxants that won’t cause muscle paralysis that lasts longer than expected. […] If you have a family history of pseudocholinesterase deficiency, you can prevent problems during anesthesia by having testing before the procedure. […] Evaluating your risk of pseudocholinesterase deficiency allows your health care provider to avoid certain muscle relaxants, if needed.
  • #5 Pseudocholinesterase deficiency | Altru Health System
    https://www.altru.org/health-library/conditions/pseudocholinesterase-deficiency
    There is no cure for pseudocholinesterase deficiency. But if you’re diagnosed with the disorder, your health care provider can use other types of muscle relaxants that won’t cause muscle paralysis that lasts longer than expected. […] 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.
  • #6 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium. Other nondepolarizing neuromuscular blockers such as atracurium, rocuronium, and vecuronium safe for use in future anesthetics.
  • #7 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    If you receive general anesthesia and develop symptoms of pseudocholinesterase deficiency, you’ll likely need immediate medical care. Your healthcare team will monitor you while you recover. If necessary, they’ll keep you under anesthesia and use mechanical ventilation to help you breathe until you can breathe on your own again. […] If you have the gene mutation, your provider will avoid choline esters and choose another type of anesthetic instead. Rocuronium is one of the most used anesthetic medications for people with pseudocholinesterase deficiency. […] If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: Succinylcholine, Mivacurium, Procaine, Chloroprocaine, Benzocaine, Tetracaine. […] There’s 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.
  • #8 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Reluctance to use FFPs is not only due to the risk versus benefit assessment, but also due in part to the unreliability of transfusions in blockade reversal. […] Pharmacological blockade reversal is also not advised; agents such as neostigmine and physostigmine, which are commonly used anesthetic agents, which antagonize and inhibit pseudocholinesterase activity. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] Fortunately, there are other paralytic alternatives that have different metabolism mechanisms, such as rocuronium, vecuronium, atracurium and cisatracurium that will not significantly prolong neuromuscular blockade.
  • #9 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency
    There is no conventional form of medical treatment for pseudocholinesterase deficiency. If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] If muscle relaxants or anesthesia are administered to you, these can put your life at risk. You may require mechanical ventilation to help breathing that may be hampered by relaxed respiratory muscles. Wearing a medical alert bracelet/necklace that indicates PCE deficiency will enable healthcare workers to realize your condition in the event of an accident/injury/surgery.
  • #10 Pseudocholinesterase deficiency | Altru Health System
    https://www.altru.org/health-library/conditions/pseudocholinesterase-deficiency
    With pseudocholinesterase deficiency, you may also be sensitive to other medications. These can include local numbing medications, also called local anesthetics. Examples are procaine, tetracaine, benzocaine and cocaine. […] If you’ve been diagnosed with pseudocholinesterase deficiency, wear a medical alert bracelet or necklace and carry a wallet card. This lets health care professionals know of your risk, especially in an emergency.
  • #11 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. […] 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. […] Patients with known pseudocholinesterase deficiency may wear a medic-alert bracelet that will notify healthcare workers of increased risk from administration of succinylcholine. […] Mechanical ventilation and close clinical monitoring are required to prevent hypoxic respiratory failure.
  • #12 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    Individuals with pseudocholinesterase deficiency are often only diagnosed after experiencing prolonged neuromuscular paralysis following standard doses of succinylcholine and mivacurium. The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. Repeated evaluation for the return of motor function can be assessed through the use of nerve stimulation. Patients should also remain sedated during this period to reduce the risk of awareness while awaiting the return of motor function. Conservative, supportive treatment with sedation and mechanical ventilation until recovery, which is usually only several hours, is felt to involve less risk than reversal with transfusion of plasma or use of other medications, which have not been reliable previously, to try to reverse the block.
  • #13 Pseudocholinesterase Deficiency Treatment & Management: Medical Care
    https://emedicine.medscape.com/article/247019-treatment
    Pseudocholinesterase deficiency is a clinically silent condition seen in individuals who are not exposed to exogenous sources of choline esters. […] Patients with prolonged paralysis following administration of succinylcholine can be treated in the following ways: […] Prophylactic transfusion of fresh frozen plasma can augment the patient’s endogenous plasma pseudocholinesterase activity. This practice is not recommended because of the risk of iatrogenic viral infectious complications. However, perioperative transfusion of fresh frozen plasma administered to correct a coagulopathy may mask an underlying pseudocholinesterase deficiency. […] Mechanical ventilatory support is the mainstay of treatment until respiratory muscle paralysis spontaneously resolves. Recovery eventually occurs as a result of passive diffusion of succinylcholine away from the neuromuscular junction.
  • #14 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency, commonly referred to as Butyrylcholinesterase deficiency, is a rare, inherited, or acquired condition that results in decreased or absent enzymatic activity. Treatment is mainly supportive with continued mechanical ventilation until return of muscle function because paralysis can last from minutes to hours and re-sedation to limit patient discomfort. […] The mainstay of treatment is continued mechanical ventilation with sedation and continued peripheral nerve monitoring until paralysis ends and spontaneity of muscle activity returns. […] It has been theorized that fresh frozen plasma (FFPs) or packed red blood cells (pRBCs) can be administered to shorten the duration of the prolonged block. […] When FFPs or pRBCs are given, the patient is being provided with exogenous pseudocholinesterase.
  • #15 What Is Pseudocholinesterase Deficiency?
    https://www.icliniq.com/articles/genetic-disorders/pseudocholinesterase-deficiency
    There is no known treatment for pseudocholinesterase deficiency. However, there are interventions and precautions that, if taken, can speed up the onset of recovery in an individual exposed to Succinylcholine or Mivacurium. The mainstay of treatment in these patients is to continue mechanical ventilation with sedation and continue peripheral nerve monitoring until paralysis ends and the muscle activity returns to normal. […] Administration of whole blood, fresh frozen plasma, packed red blood cells, and human serum cholinesterase is an intervention that can be done to shorten the duration of the block. […] The golden rule is to let the individual recover spontaneously. It has been theorized in the literature that the administration of whole blood has successfully reversed prolonged apnea and paralysis after the administration of Succinylcholine in patients with pseudocholinesterase deficiency. […] Note: Patients with pseudocholinesterase deficiency should avoid drugs like Succinylcholine and Mivacurium to prevent the future occurrence of prolonged paralysis.
  • #16 Pseudocholinesterase Deficiency – OpenAnesthesia
    https://www.openanesthesia.org/keywords/pseudocholinesterase-deficiency/
    There are no current treatments in the United States for PChE deficiency. The recommended management is supportive care, keeping the patient sedated and ventilated until spontaneous recovery. […] Human plasma cholinesterase is available in Europe but is not commonly used. It has not shown to be very reliable. […] Plasma contains pseudocholinesterase. Transfusion of fresh frozen plasma (FFP) has been shown to increase a patients enzyme level and activity. […] Whole blood has pseudocholinesterase as well and can improve enzyme activity. […] Transfusions of FFP and whole blood are not recommended treatments. The level of enzyme activity will vary per unit and there are no set guidelines on the number of units needed to reverse muscle blockade. This is not a good use of valuable blood products and poses a risk of iatrogenic infections.
  • #17 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Reluctance to use FFPs is not only due to the risk versus benefit assessment, but also due in part to the unreliability of transfusions in blockade reversal. […] Pharmacological blockade reversal is also not advised; agents such as neostigmine and physostigmine, which are commonly used anesthetic agents, which antagonize and inhibit pseudocholinesterase activity. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] Fortunately, there are other paralytic alternatives that have different metabolism mechanisms, such as rocuronium, vecuronium, atracurium and cisatracurium that will not significantly prolong neuromuscular blockade.
  • #18 Pseudocholinesterase Deficiency – OpenAnesthesia
    https://www.openanesthesia.org/keywords/pseudocholinesterase-deficiency/
    Administration of an anticholinesterase is not recommended. Administration has been shown to potentially transiently reverse the apneic effects of succinylcholine. However, this is followed by an intensified neuromuscular blockade. […] The potential for PChE enzyme deficiency makes it vital to check for recovery from succinylcholine before administering any other paralytic and waking up a patient.
  • #19 Pseudocholinesterase Deficiency Treatment & Management: Medical Care
    https://emedicine.medscape.com/article/247019-treatment
    Administration of cholinesterase inhibitors such as neostigmine is controversial for reversing succinylcholine-related apnea in patients who are pseudocholinesterase deficient. The effects may be transient, possibly followed by intensified neuromuscular blockade. […] Consultation with a geneticist may help identify the specific atypical genotype alleles contributing to pseudocholinesterase deficiency. […] Because the DNA sequence of the pseudocholinesterase gene and its amino acid structure is known, atypical alleles now can be identified by polymerase chain reaction (PCR) amplification studies using DNA extracted from leukocytes in a blood sample.
  • #20
    https://journals.lww.com/jica/fulltext/2022/01020/revisiting_pseudocholinesterase_deficiency__the.1.aspx
    This issue describes an interesting case of an unusual presentation of pseudocholinesterase (PChE) deficiency in a parturient undergoing emergent cesarean section by Upadaya and Garg. […] However, when diagnosed, it is treatable with ventilatory support till spontaneous recovery. […] Patients diagnosed with PChE deficiency are expected to make a full recovery after administration of succinylcholine or mivacurium provided adequate mechanical ventilation and vigilant monitoring is provided to ensure the spontaneous return of motor function. […] Some authors advocate shortening the duration of the prolonged block by providing exogenous PChE through administration of fresh frozen plasma or packed red blood cells. […] Pharmacological blockade reversal with neostigmine and physostigmine is not recommended as these drugs can also inhibit the activity of PChE enzyme leading to further prolongation of the paralysis as reported by Upadaya and Garg.
  • #21 Pseudocholinesterase deficiency and electroconvulsive therapy – PubMed
    https://pubmed.ncbi.nlm.nih.gov/17805000/
    Clinically significant pseudocholinesterase deficiency is a relatively uncommon disorder, but when present, it presents clinicians with challenges regarding medication administration. This is especially true in cases of patients receiving electroconvulsive therapy (ECT), as the presence of pseudocholinesterase deficiency limits the use of certain muscle relaxants. […] Subsequent ECT treatments were then managed with an alternative muscle relaxant which was not dependent on pseudocholinesterase for termination of action.
  • #22 Hereditary pseudocholinesterase deficiency discovery after electroconvulsive therapy | BMJ Case Reports
    https://casereports.bmj.com/content/14/1/e239206
    Inherited pseudocholinesterase deficiency refers to an uncommon defect in the butyrylcholinesterase enzyme which can result in prolonged muscle paralysis due to delayed breakdown of choline ester paralytic anaesthetic agents. […] Implications of pseudocholinesterase deficiency for ECT treatment and anaesthetic strategies are discussed.
  • #23
    https://journals.lww.com/ectjournal/fulltext/2011/03000/rocuronium_antagonized_by_sugammadex_for_series_of.47.aspx
    We report the anesthetic management of a patient with catatonic schizophrenia and pseudocholinesterase deficiency, using the nondepolarizing neuromuscular blocking drug, rocuronium, reversed by its specific reversal agent, sugammadex, for a series of electroconvulsive therapy sessions. […] Rocuronium and sugammadex were used every 48 hours for 8 consecutive times and proved to be an effective and safe combination in a situation where succinylcholine was contraindicated.
  • #24 Comparison between succinylcholine and rocuronium as neuromuscular blocking agents for electroconvulsive therapy in a patient with pseudocholinesterase deficiency | JA Clinical Reports | Full Text
    https://jaclinicalreports.springeropen.com/articles/10.1186/s40981-015-0009-2
    We report here the anesthetic management of a patient with schizophrenia and pseudocholinesterase deficiency. Electroconvulsive therapy was performed using succinylcholine and rocuronium as the neuromuscular blocking agents in the first seven and latter six treatments, respectively. The recovery time from muscle relaxation after succinylcholine administration was remarkably longer than that after rocuronium-sugammadex administration. Rocuronium and sugammadex appear to be useful in situations in which succinylcholine is contraindicated. […] Hence, we decided to use rocuronium instead of SCC for future ECTs. […] In the eighth treatment session, anesthesia was induced with propofol followed by 17 mg (0.3 mg/kg) ROC under neuromuscular monitoring. […] The advantages of use of ROC-sugammadex for ECT, including fast recovery from muscle relaxation and fewer side effects, may outweigh the high cost of its usage. The superiority of ROC-sugammadex for ECT is even more obvious in subjects with pseudocholinesterase deficiency, since spontaneous recovery from muscle relaxant effects after SCC is expected to be markedly prolonged in such cases. […] ROC administration followed by sugammadex reversal of neuromuscular blockade seems to be better than succinylcholine for ECT, particularly in patients with pseudocholinesterase deficiency.
  • #25
    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. […] We performed modified ECT with propofol and rocuronium instead of succinylcholine as usual. Sugammadex was used for speeding up the recovery. […] We suggest further investigation of this topic because screening for PCE levels in pre-ECT assessment may reduce complications of modified ECT with anesthesia. […] Detection of patients’ serum pseudocholinesterase levels is part of our routine preoperative laboratory workup. […] Since we had detected the patient’s severe PCE deficiency, we used rocuronium – a non-depolarizing muscle relaxant – instead of succinylcholine.
  • #26
    https://arsiv.dusunenadamdergisi.org/ing/fArticledetails4370.html?MkID=1286
    The availability of sugammadex enables the rapid termination of the muscle blockade effected by rocuronium. […] Thanks to the detection of PCE levels is part of our pre-ECT assessments, it was possible for us to select rocuronium as the muscle relaxant to prevent complications. […] Thus we suggest the detection of PCE levels to be a part of routine pre-ECT laboratory assessments.
  • #27 Can I use Local Anesthetic on a patient with pseudocholinesterase deficiency? | University OMF
    https://universityomf.ca/research_post/can-i-use-local-anesthetic-on-a-patient-with-pseudocholinesterase-deficiency/
    This article provides a comprehensive review of pseudocholinesterase deficiency, including its pathophysiology, genetic basis, available testing and drugs to avoid. […] The authors review acquired conditions, drugs that influence enzyme activity and possible treatments of the disease. […] The use of succinylcholine, mivacurium and ester local anesthetics must be avoided in patients with pseudocholinesterase deficiency because these patients may not be able to metabolize such anesthetics. […] Dentists should be aware of the importance of avoiding ester local anesthetics in patients with pseudocholinesterase deficiency.
  • #28 Modified Electroconvulsive Therapy in Pseudocholinesterase Deficiency: A Case Report | European Psychiatry | Cambridge Core
    https://www.cambridge.org/core/journals/european-psychiatry/article/modified-electroconvulsive-therapy-in-pseudocholinesterase-deficiency-a-case-report/B1761F82051DB6BB222224E46CF867DD
    Pseudocholinesterase (PCHE) deficiency is an inherited condition, in which recovery from anesthetic agents like succinylcholine and mivacurium is slow and complicated with prolonged paralysis of respiratory muscles in susceptible patients. […] After the detection of PCHE deficiency (PCHE level: 126 U/L), we performed the modified ECT with propophol and rocuronium instead of succinylcholine as usual. Sugammadex 100 mg was used for fastening the recovery. […] Screening for PCHE levels in the pre-ECT assessments is efficacious in order to decrease the complications of the ECT procedure.
  • #29 Pseudocholinesterase deficiency – Wikipedia
    https://en.wikipedia.org/wiki/Pseudocholinesterase_deficiency
    Patients with known pseudocholinesterase deficiency may wear a medic-alert bracelet that will notify healthcare workers of increased risk from administration of succinylcholine, and use a non-depolarising neuromuscular-blocking drug for general anesthesia, such as rocuronium. […] Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures.
  • #30 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 will almost always present unexpectedly and must be included in the differential diagnosis of delayed emergence. […] 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. A causal treatment does not currently exist. However, the disorder typically has a benign course, and an excellent prognosis, if supportive management, including prolonged post-operative ventilatory support, can be provided.
  • #31 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. […] 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. 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. […] With pseudocholinesterase deficiency, you may also be sensitive to other medications. These can include local numbing medications, also called local anesthetics. Examples are procaine, tetracaine, benzocaine and cocaine. […] If you’ve been diagnosed with pseudocholinesterase deficiency, wear a medical alert bracelet or necklace and carry a wallet card. This lets health care professionals know of your risk, especially in an emergency.
  • #32 Pseudocholinesterase deficiency
    https://www.mymlc.com/health-information/diseases-and-conditions/p/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency can be caused by a genetic defect (mutation) that either is inherited or is acquired due to illness, injury or certain medications. […] There is no cure for pseudocholinesterase deficiency. But if you’re diagnosed with the condition, your doctor can use other types of muscle relaxants that won’t cause prolonged muscle relaxation. […] If you have pseudocholinesterase deficiency, your anesthesiologist can avoid giving you drugs that may trigger prolonged muscle relaxation and choose other muscle relaxants instead. […] There is no cure for pseudocholinesterase deficiency. If you have the condition and you receive muscle relaxants that prolong your anesthesia recovery, you’ll likely need medical assistance. If needed, mechanical ventilation support and sedation are provided while you recover and start breathing on your own. […] If you’ve been diagnosed with pseudocholinesterase deficiency, wear a medical alert bracelet or necklace. This lets health care professionals know of your risk, especially in an emergency.
  • #33 Pseudocholinesterase deficiency | UM Health-Sparrow
    https://www.uofmhealthsparrow.org/departments-conditions/conditions/pseudocholinesterase-deficiency
    There is no cure for pseudocholinesterase deficiency. But if you’re diagnosed with the disorder, your health care provider can use other types of muscle relaxants that won’t cause muscle paralysis that lasts longer than expected. […] 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. […] 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. 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. […] If you’ve been diagnosed with pseudocholinesterase deficiency, wear a medical alert bracelet or necklace and carry a wallet card. This lets health care professionals know of your risk, especially in an emergency.
  • #34 Pseudocholinesterase Deficiency – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group
    https://www.acibademhealthpoint.com/cat/health-library/pseudocholinesterase-deficiency/
    Managing pseudocholinesterase deficiency needs a plan made just for each person. Doctors and patients work together to create a care plan. This plan helps tackle the unique challenges of this rare condition. […] It’s important to know the anesthetic implications of this condition. Before surgery, patients must tell their anesthesiologists about their condition. This helps the medical team choose the right anesthetics and watch the patient closely during and after surgery. […] The table below shows some key points for treating and managing pseudocholinesterase deficiency: Management Strategy Description Preoperative screening Identify patients with pseudocholinesterase deficiency before surgery Anesthetic drug choices Select anesthetics that are not metabolized by pseudocholinesterase Postoperative monitoring Closely observe patients for prolonged paralysis or respiratory depression Patient education Inform patients about their condition and provide resources for support Medical alert identification Encourage patients to wear medical alert bracelets or carry cards.
  • #35 Pseudocholinesterase Deficiency – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Group
    https://www.acibademhealthpoint.com/cat/health-library/pseudocholinesterase-deficiency/
    Research is also underway to find new treatments for pseudocholinesterase deficiency. As we learn more about the genetics and biology of this condition, better treatment options are on the horizon. […] Understanding the anesthetic implications of pseudocholinesterase deficiency helps healthcare providers give safe and effective care. This is important for patients during surgical procedures. […] Treatment focuses on managing anesthesia and avoiding certain drugs. Patients should tell their doctors about their condition. This ensures the right anesthetic drug choices and postoperative monitoring. A personalized care plan and teamwork among healthcare professionals are key.
  • #36 A Case of Pseudocholinesterase Deficiency in Patient Underwent General Anesthesia with Flexible Bronchoscopy, International Journal of Anesthesia and Clinical Medicine, Science Publishing Group
    https://www.sciencepublishinggroup.com/article/10.11648/j.ijacm.20231102.13
    Pseudocholinesterase deficiency is a rare clinical condition primarily associated with genetic alterations, but it can also be caused by certain diseases and medication factors. Patients with this condition experience significantly prolonged muscle paralysis when succinylcholine or mivacurium is used during general anesthesia, due to the decreased enzyme levels. […] The diagnosis of pseudocholinesterase deficiency is typically made after the administration of succinylcholine or mivacurium. Inquiring about the patient’s family history is also crucial for proper diagnosis and intervention. […] This article presents a case of delayed recovery in a patient with pseudocholinesterase deficiency following painless flexible bronchoscopy. It also summarizes the causes, clinical manifestations, diagnosis, and treatment of pseudocholinesterase deficiency-related delayed emergence. It is hoped that this article will contribute to timely recognition and management of such cases, thereby preventing any potential adverse outcomes for patients. Furthermore, since pseudocholinesterase deficiency is relatively rare, further research is needed to confirm the effectiveness of the preventive and therapeutic measures mentioned in this article.