Niedobór pseudocholinesterazy
Charakterystyka, pielęgnacja i opieka
Niedobór pseudocholinesterazy (butyrylocholinesterazy) to rzadkie zaburzenie enzymatyczne, dziedziczone autosomalnie recesywnie lub nabyte, charakteryzujące się zmniejszoną aktywnością enzymu wątrobowego odpowiedzialnego za metabolizm estrów cholinowych, takich jak sukcynylocholina i miwakurium. Klinicznie manifestuje się przedłużonym zwiotczeniem mięśni i zaburzeniami oddychania po podaniu tych leków zwiotczających podczas znieczulenia ogólnego. Diagnostyka opiera się na obserwacji klinicznej oraz monitorowaniu funkcji nerwowo-mięśniowej za pomocą stymulatora nerwów obwodowych (TOF). Aktywność pseudocholinesterazy w osoczu w normie wynosi 4000-13500 U/L, a jej obniżenie potwierdza rozpoznanie. Leczenie jest wyłącznie objawowe i polega na wsparciu wentylacji mechanicznej do czasu ustąpienia blokady nerwowo-mięśniowej, przy jednoczesnym zapewnieniu sedacji i monitorowaniu parametrów życiowych oraz funkcji mięśniowych.
- Wprowadzenie do niedoboru pseudocholinesterazy
- Opieka pielęgniarska nad pacjentem z niedoborem pseudocholinesterazy
- Rozpoznanie i wczesna identyfikacja
- Postępowanie w przypadku rozpoznania niedoboru
- Sedacja i komfort pacjenta
- Monitorowanie i ocena funkcji nerwowo-mięśniowej
- Wentylacja mechaniczna i wsparcie oddechowe
- Edukacja pacjenta i rodziny
- Informacje o schorzeniu i jego konsekwencjach
- Zalecenia dotyczące przyszłych zabiegów medycznych
- Wsparcie psychologiczne
- Zapobieganie powikłaniom długotrwałego unieruchomienia
- Współpraca zespołu interdyscyplinarnego
- Rola pielęgniarki w zespole interdyscyplinarnym
- Współpraca z anestezjologiem
- Rola genetyka w diagnostyce
- Postępowanie w sytuacjach szczególnych
- Dokumentacja medyczna i identyfikacja pacjentów z niedoborem pseudocholinesterazy
- Podsumowanie zasad opieki nad pacjentem z niedoborem pseudocholinesterazy
Wprowadzenie do niedoboru pseudocholinesterazy
Niedobór pseudocholinesterazy (ang. pseudocholinesterase deficiency), znany również jako niedobór butyrylocholinesterazy, to rzadkie zaburzenie charakteryzujące się nieprawidłową lub zmniejszoną aktywnością enzymu pseudocholinesterazy produkowanego przez wątrobę. Zaburzenie to może być wrodzone (dziedziczone autosomalnie recesywnie) lub nabyte. Głównym problemem klinicznym związanym z tym niedoborem jest nadwrażliwość na określone leki zwiotczające mięśnie stosowane podczas znieczulenia ogólnego, co prowadzi do przedłużonego zwiotczenia mięśni i zaburzeń oddychania.12
U pacjentów z niedoborem pseudocholinesterazy występuje nieprawidłowy metabolizm estrów cholinowych, takich jak sukcynylocholina, miwakurium oraz estrowe leki znieczulenia miejscowego. W warunkach prawidłowych enzym ten rozkłada te substancje do nieaktywnych metabolitów, jednak przy jego niedoborze dochodzi do przedłużonego działania leków i związanych z tym powikłań.34
Opieka pielęgniarska nad pacjentem z niedoborem pseudocholinesterazy
Rozpoznanie i wczesna identyfikacja
Podejrzenie niedoboru pseudocholinesterazy pojawia się, gdy pacjent ma problemy z odzyskaniem kontroli nad mięśniami i oddychaniem po otrzymaniu leków zwiotczających, takich jak sukcynylocholina lub miwakurium podczas znieczulenia ogólnego. Niedobór ten często pozostaje niezdiagnozowany, dopóki pacjent nie zostanie poddany zabiegowi chirurgicznemu, podczas którego otrzyma te leki.56
Personel pielęgniarski powinien zwrócić szczególną uwagę na następujące objawy:7
- Przedłużone zwiotczenie mięśni po zakończeniu operacji
- Brak samodzielnego oddychania
- Brak możliwości poruszania się przez kilka godzin po zakończeniu znieczulenia
Ważne jest monitorowanie stanu neurologicznego pacjenta przy użyciu stymulatora nerwów obwodowych, który może potwierdzić kliniczne podejrzenie niedoboru pseudocholinesterazy.8
Postępowanie w przypadku rozpoznania niedoboru
Podstawą leczenia pacjentów z niedoborem pseudocholinesterazy jest wspomaganie oddychania za pomocą wentylacji mechanicznej do czasu samoistnego ustąpienia blokady nerwowo-mięśniowej. Nie istnieje leczenie przyczynowe tego zaburzenia.910
Zespół pielęgniarski powinien zapewnić:1112
- Ciągłe monitorowanie parametrów życiowych: tętna, ciśnienia tętniczego, saturacji
- Utrzymanie drożności dróg oddechowych
- Wsparcie wentylacji mechanicznej aż do powrotu spontanicznego oddychania
- Sedację w celu zapewnienia komfortu pacjenta i ograniczenia dyskomfortu związanego z przedłużoną intubacją
- Regularne kontrolowanie funkcji nerwowo-mięśniowej za pomocą stymulatora nerwów obwodowych
- Dokumentowanie wszystkich parametrów i obserwacji
Sedacja i komfort pacjenta
Szczególnie istotnym aspektem opieki nad pacjentem z niedoborem pseudocholinesterazy jest właściwe stosowanie sedacji. Pacjenci, którzy doświadczają przedłużonego paraliżu po podaniu sukcynylocholiny lub miwakurium, pozostają świadomi, lecz nie mogą się poruszać ani komunikować. Ten stan może wywołać silny lęk i dyskomfort.13
Personel pielęgniarski powinien:14
- Zapewnić odpowiednią sedację, aby zmniejszyć lęk i dyskomfort
- Podawać leki przeciwbólowe zgodnie ze zleceniem lekarskim
- Monitorować głębokość sedacji, aby zapewnić komfort pacjenta bez nadmiernego tłumienia funkcji życiowych
- Upewnić się, że pacjent jest regularnie oceniany pod kątem potrzeby dostosowania dawek leków sedacyjnych
Monitorowanie i ocena funkcji nerwowo-mięśniowej
Dokładne monitorowanie stanu pacjenta jest kluczowym elementem opieki. Personel pielęgniarski musi ściśle współpracować z zespołem anestezjologicznym, aby określić moment, gdy pacjent odzyskuje funkcje mięśniowe.15
Ocena funkcji nerwowo-mięśniowej obejmuje:1617
- Regularne stosowanie stymulatora nerwów obwodowych (TOF – train-of-four)
- Ocenę siły mięśniowej gdy pacjent zaczyna odzyskiwać funkcje ruchowe
- Monitorowanie parametrów oddychania, aby określić moment, gdy pacjent może być odłączony od respiratora
- Dokumentowanie wszystkich pomiarów i obserwacji
Wentylacja mechaniczna i wsparcie oddechowe
Podstawowym elementem leczenia pacjentów z niedoborem pseudocholinesterazy, którzy doświadczyli przedłużonego zwiotczenia po podaniu sukcynylocholiny lub miwakurium, jest wsparcie oddechowe.1819
Opieka pielęgniarska w zakresie wentylacji mechanicznej obejmuje:2021
- Zapewnienie prawidłowego funkcjonowania respiratora
- Monitorowanie parametrów wentylacji (objętość oddechowa, częstość oddechów, FiO2, PEEP)
- Regularne odsysanie wydzieliny z dróg oddechowych
- Pielęgnację jamy ustnej i rurki intubacyjnej
- Zmianę pozycji ciała pacjenta w celu zapobiegania odleżynom i powikłaniom oddechowym
- Przygotowanie do ekstubacji, gdy pacjent odzyska zdolność do samodzielnego oddychania
Czas trwania wsparcia oddechowego może wynosić od kilku do kilkunastu godzin, w zależności od nasilenia niedoboru pseudocholinesterazy i dawki zastosowanego leku zwiotczającego.2223
Edukacja pacjenta i rodziny
Edukacja pacjenta i jego rodziny jest kluczowym elementem opieki pielęgniarskiej w przypadku zdiagnozowania niedoboru pseudocholinesterazy. Właściwe przekazanie informacji może zapobiec przyszłym powikłaniom.24
Informacje o schorzeniu i jego konsekwencjach
Pacjent i jego rodzina powinni otrzymać kompleksowe informacje na temat:2526
- Natury niedoboru pseudocholinesterazy i jego genetycznego podłoża
- Możliwości dziedziczenia schorzenia przez członków rodziny
- Znaczenia badań genetycznych dla krewnych pacjenta
- Konsekwencji niedoboru podczas przyszłych zabiegów chirurgicznych
- Leków, których należy unikać ze względu na ryzyko przedłużonego zwiotczenia
Zalecenia dotyczące przyszłych zabiegów medycznych
Pacjent powinien być poinformowany o konieczności informowania wszystkich pracowników ochrony zdrowia o swoim stanie, szczególnie przed zabiegami chirurgicznymi.2728
- Noszenie bransoletki lub naszyjnika medycznego z informacją o niedoborze pseudocholinesterazy
- Posiadanie przy sobie karty informacyjnej dotyczącej schorzenia
- Informowanie o niedoborze wszystkich lekarzy, a szczególnie anestezjologów przed każdym zabiegiem
- Unikanie leków, które mogą wywołać przedłużone zwiotczenie: sukcynylocholiny, miwakurium, prokaina, chloroprokaina, benzokaina, tetrakaina
Pacjent powinien otrzymać pisemną informację z listą leków, których należy unikać oraz listę bezpiecznych alternatyw dla leków zwiotczających.3132
Wsparcie psychologiczne
Doświadczenie przedłużonego paraliżu może być traumatyczne dla pacjenta. Wsparcie psychologiczne jest istotnym elementem opieki pielęgniarskiej.33
Personel pielęgniarski powinien:34
- Wyjaśnić, że stan zwiotczenia jest tymczasowy i ustąpi samoistnie
- Zapewnić spokojne środowisko i komfortową opiekę
- Umożliwić pacjentowi wyrażenie obaw i lęków po odzyskaniu funkcji mięśniowych
- W razie potrzeby skierować pacjenta do specjalisty psychologa
Zapobieganie powikłaniom długotrwałego unieruchomienia
Przedłużone unieruchomienie pacjenta z niedoborem pseudocholinesterazy może prowadzić do rozwoju powikłań. Personel pielęgniarski powinien wdrożyć odpowiednie działania profilaktyczne.35
Działania zapobiegawcze obejmują:36
- Zmianę pozycji ciała co 2 godziny w celu zapobiegania odleżynom
- Stosowanie materacy przeciwodleżynowych
- Utrzymanie prawidłowej higieny ciała
- Profilaktykę przeciwzakrzepową (farmakologiczną i/lub mechaniczną)
- Monitorowanie stanu skóry, szczególnie w miejscach narażonych na ucisk
- Zapewnienie odpowiedniego nawodnienia i odżywienia
Współpraca zespołu interdyscyplinarnego
Opieka nad pacjentem z niedoborem pseudocholinesterazy wymaga ścisłej współpracy między różnymi specjalistami w zespole medycznym.3738
Rola pielęgniarki w zespole interdyscyplinarnym
Pielęgniarka pełni kluczową rolę w zespole, odpowiadając za:39
- Ciągłe monitorowanie stanu pacjenta i natychmiastowe raportowanie zmian
- Koordynację opieki między różnymi specjalistami
- Dokumentację wszystkich obserwacji i interwencji
- Edukację pacjenta i rodziny
- Komunikację między zespołem medycznym a pacjentem i rodziną
Współpraca z anestezjologiem
Anestezjolog odgrywa kluczową rolę w zarządzaniu stanem pacjenta z niedoborem pseudocholinesterazy. Pielęgniarka powinna ściśle współpracować z anestezjologiem w zakresie:4041
- Monitorowania funkcji nerwowo-mięśniowej
- Dostosowywania parametrów wentylacji mechanicznej
- Określenia momentu, gdy pacjent może być ekstubowany
- Doboru odpowiednich leków sedacyjnych i przeciwbólowych
Rola genetyka w diagnostyce
W przypadku podejrzenia wrodzonego niedoboru pseudocholinesterazy, konsultacja z genetykiem może pomóc w identyfikacji konkretnych alleli atypowych przyczyniających się do niedoboru.4243
Badania genetyczne mogą obejmować:44
- Sekwencjonowanie genu BCHE (gen kodujący pseudocholinesterazę)
- Badania PCR w celu identyfikacji atypowych alleli
- Poradnictwo genetyczne dla rodziny pacjenta
Postępowanie w sytuacjach szczególnych
Niedobór pseudocholinesterazy u kobiet ciężarnych
Opieka nad kobietą ciężarną z niedoborem pseudocholinesterazy wymaga szczególnej uwagi ze względu na możliwe implikacje dla matki i dziecka.4546
Postępowanie powinno obejmować:47
- Dokładną ocenę przedoperacyjną i zaplanowanie znieczulenia z unikaniem sukcynylocholiny i miwakurium
- Ścisłą współpracę między anestezjologiem a położnikiem
- Przygotowanie do możliwości przedłużonego wsparcia oddechowego po cesarskim cięciu
- Monitorowanie noworodka pod kątem objawów niedoboru pseudocholinesterazy
Nabyty niedobór pseudocholinesterazy
Oprócz postaci dziedzicznej, niedobór pseudocholinesterazy może być nabyty w wyniku różnych stanów chorobowych lub stosowania niektórych leków.4849
Personel pielęgniarski powinien być świadomy czynników ryzyka nabytego niedoboru pseudocholinesterazy:50
- Choroby wątroby
- Nowotwory złośliwe
- Niewydolność nerek
- Ciąża
- Stosowanie niektórych leków (np. wysokie dawki cyklofosfamidu)
Dokumentacja medyczna i identyfikacja pacjentów z niedoborem pseudocholinesterazy
Właściwa dokumentacja i identyfikacja pacjentów z niedoborem pseudocholinesterazy jest kluczowa dla zapewnienia bezpieczeństwa podczas przyszłych procedur medycznych.5152
Personel pielęgniarski powinien zadbać o:5354
- Dokładne udokumentowanie rozpoznania niedoboru pseudocholinesterazy w historii choroby pacjenta
- Zaznaczenie informacji o niedoborze w sekcji „Alergie” w dokumentacji medycznej
- Przekazanie pacjentowi pisemnej informacji o jego stanie i lekach, których należy unikać
- Zalecenie noszenia bransoletki lub naszyjnika medycznego z informacją o niedoborze
- Poinformowanie pacjenta o konieczności informowania wszystkich lekarzy o niedoborze pseudocholinesterazy przed każdą procedurą medyczną
Badania laboratoryjne potwierdzające rozpoznanie
Po ustąpieniu przedłużonego zwiotczenia, które nasunęło podejrzenie niedoboru pseudocholinesterazy, wskazane jest wykonanie badań laboratoryjnych potwierdzających rozpoznanie.5556
Badania mogą obejmować:57
- Pomiar aktywności pseudocholinesterazy w osoczu (prawidłowe wartości: 4000-13500 U/L)
- Liczbę dibukainową (określającą jakościowy wariant enzymu)
- Badania genetyczne w kierunku mutacji genu BCHE
Podsumowanie zasad opieki nad pacjentem z niedoborem pseudocholinesterazy
Opieka nad pacjentem z niedoborem pseudocholinesterazy wymaga kompleksowego podejścia i ścisłej współpracy całego zespołu medycznego.5859
Kluczowe elementy opieki pielęgniarskiej obejmują:6061
- Wczesne rozpoznanie przedłużonego zwiotczenia mięśni
- Zapewnienie odpowiedniego wsparcia oddechowego za pomocą wentylacji mechanicznej
- Monitorowanie funkcji nerwowo-mięśniowej za pomocą stymulatora nerwów obwodowych
- Stosowanie odpowiedniej sedacji w celu zapewnienia komfortu pacjenta
- Zapobieganie powikłaniom długotrwałego unieruchomienia
- Edukację pacjenta i rodziny na temat schorzenia i jego konsekwencji
- Właściwą dokumentację rozpoznania i zalecenie noszenia identyfikatora medycznego
Niedobór pseudocholinesterazy, choć rzadki, stanowi poważne wyzwanie anestezjologiczne, które przy właściwym rozpoznaniu i postępowaniu może być skutecznie zarządzane, zapewniając pacjentowi pełny powrót do zdrowia.6263
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Materiały źródłowe
- #1 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://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. […] This activity reviews the evaluation and management of pseudocholinesterase deficiency, and highlights the role of the interprofessional team in managing patients with this condition. […] Explain the importance of improving care coordination amongst the interprofessional team to enhance the delivery of care for patients with pseudocholinesterase deficiency. […] The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. […] Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium.
- #2 Pseudocholinesterase Deficiency and Patient Perspectiveshttps://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. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] It is imperative that patients are re-sedated in a timely manner to limit patient discomfort. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested.
- #3 Digital Commons @ Otterbeinhttps://digitalcommons.otterbein.edu/stu_msn/56/
Pseudocholinesterase deficiency is a rare genetic or acquired variation in the metabolism of choline esters such as the neuromuscular blockers succinylcholine, mivacurium, and ester local anesthetics. […] This condition is rare but must be known and understood by the clinician in order to provide the safest patient care possible. […] The purpose of this discussion is to further educate the practitioner on the pathophysiology of pseudocholinesterase deficiency, as well as signs and symptoms, and patient management strategies to improve patient outcomes.
- #4 Residual Muscle Weakness after Succinylcholine Infusion: Clinical Presentation, Diagnosis and Treatmenthttps://austinpublishinggroup.com/anesthesia-analgesia/fulltext/ajaa-v2-id1022.php
Pseudocholinesterase deficiency is synthesized by the liver and exists in plasma. It breaks down Succinylcholine to inactive metabolites. […] The lab results of the 2nd patient did show decreased pseudocholinesterase level (though dubicaine number is unavailable), which indicated decreased enzyme quantity. […] The presence of chronic malignancy increases the likelihood of pseudocholinesterase abnormality, including both quality and quantity, not to mention the possibility of genetic variant of pseudocholinesterase. […] The detection of decreased pseudocholinesterase level may warrant further lab test such as dubicaine number or genetic test for pseudocholinesterase. […] It is prudent to apply neuromuscular monitoring (such as acceleromyography with train-of-four nerve stimulation) during succinylcholine infusion even when the surgery is considered a short procedure.
- #5 Pseudocholinesterase deficiency – Diagnosis & treatment – Mayo Clinichttps://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. […] 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.
- #6 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK541032/
Pseudocholinesterase deficiency is a clinical condition that is often only discovered after exposure to succinylcholine or mivacurium. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. […] Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] While a rare condition, pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #7 Pseudocholinesterase deficiency | Altru Health Systemhttps://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. […] You may need help breathing with a mechanical ventilator until you can start breathing on your own. […] 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. […] Signs and symptoms of pseudocholinesterase deficiency include muscle relaxation or muscle paralysis that lasts several hours longer than expected.
- #8 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Texthttps://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. […] Pseudocholinesterase deficiency will almost always present unexpectedly and must be included in the differential diagnosis of delayed emergence. Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests. Genetic testing can help determine the etiology of disease. […] Evaluation of patients neuromuscular status using a peripheral nerve stimulator should routinely be performed following administration of mivacurium or succinylcholine. It is a simple, quick, reliable, and inexpensive way of ensuring that pseudocholinesterase deficiency will not be overlooked.
- #9 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://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. […] This activity reviews the evaluation and management of pseudocholinesterase deficiency, and highlights the role of the interprofessional team in managing patients with this condition. […] Explain the importance of improving care coordination amongst the interprofessional team to enhance the delivery of care for patients with pseudocholinesterase deficiency. […] The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. […] Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium.
- #10 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #11 Pseudocholinesterase Deficiency and Patient Perspectiveshttps://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. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] It is imperative that patients are re-sedated in a timely manner to limit patient discomfort. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested.
- #12 Pseudocholinesterase Deficiency Treatment & Management: Medical Carehttps://emedicine.medscape.com/article/247019-treatment
Patients with prolonged paralysis following administration of succinylcholine can be treated in the following ways: […] 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. […] 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.
- #13 Pseudocholinesterase Deficiency and Patient Perspectiveshttps://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. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] It is imperative that patients are re-sedated in a timely manner to limit patient discomfort. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested.
- #14 What Is Pseudocholinesterase Deficiency?https://www.icliniq.com/articles/genetic-disorders/pseudocholinesterase-deficiency
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. […] Note: Patients with pseudocholinesterase deficiency should avoid drugs like Succinylcholine and Mivacurium to prevent the future occurrence of prolonged paralysis. […] Patients suffering from Pseudocholinesterase deficiency should also be properly educated about the adverse reactions of these drugs.
- #15 Pseudocholinesterase Deficiency – MD Searchlighthttps://mdsearchlight.com/health/pseudocholinesterase-deficiency/
For those patients who have already been diagnosed with pseudocholinesterase deficiency, the approach is to avoid using the drugs succinylcholine and mivacurium, which block nerve signals to muscles. Instead, doctors recommend using other safe alternatives for future anesthetic procedures such as atracurium, rocuronium, and vecuronium. […] If you have been diagnosed with a condition called pseudocholinesterase deficiency, its vital to inform your doctor and the person who gives you anesthesia before any surgery. This is because your condition needs to be recorded in your medical history. […] 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. This approach is considered less risky than alternatives such as transfusion of plasma or the use of other medications to reverse the paralysis, which have proven to be unreliable.
- #16 Residual Muscle Weakness after Succinylcholine Infusion: Clinical Presentation, Diagnosis and Treatmenthttps://austinpublishinggroup.com/anesthesia-analgesia/fulltext/ajaa-v2-id1022.php
Pseudocholinesterase deficiency is synthesized by the liver and exists in plasma. It breaks down Succinylcholine to inactive metabolites. […] The lab results of the 2nd patient did show decreased pseudocholinesterase level (though dubicaine number is unavailable), which indicated decreased enzyme quantity. […] The presence of chronic malignancy increases the likelihood of pseudocholinesterase abnormality, including both quality and quantity, not to mention the possibility of genetic variant of pseudocholinesterase. […] The detection of decreased pseudocholinesterase level may warrant further lab test such as dubicaine number or genetic test for pseudocholinesterase. […] It is prudent to apply neuromuscular monitoring (such as acceleromyography with train-of-four nerve stimulation) during succinylcholine infusion even when the surgery is considered a short procedure.
- #17 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Texthttps://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. […] Pseudocholinesterase deficiency will almost always present unexpectedly and must be included in the differential diagnosis of delayed emergence. Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests. Genetic testing can help determine the etiology of disease. […] Evaluation of patients neuromuscular status using a peripheral nerve stimulator should routinely be performed following administration of mivacurium or succinylcholine. It is a simple, quick, reliable, and inexpensive way of ensuring that pseudocholinesterase deficiency will not be overlooked.
- #18 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #19 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/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. This activity reviews the evaluation and management of pseudocholinesterase deficiency, and highlights the role of the interprofessional team in managing patients with this condition. […] Explain the importance of improving care coordination amongst the interprofessional team to enhance the delivery of care for patients with pseudocholinesterase deficiency. […] The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. […] Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #20 Pseudocholinesterase deficiency // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/pseudocholinesterase-deficiency
Pseudocholinesterase deficiency can be caused by a gene change (mutation) that is inherited. […] 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. […] 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.
- #21 Mayo Clinic Health Library – Pseudocholinesterase deficiency | Swiss Medical Networkhttps://www.swissmedical.net/de/healtcare-library/con-20200770
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.
- #22 Pseudocholinesterase deficiency | Beacon Health Systemhttps://www.beaconhealthsystem.org/library/diseases-and-conditions/pseudocholinesterase-deficiency?content_id=CON-20200770
This causes sensitivity to certain muscle relaxants used during anesthesia. Muscles may stay relaxed for too long, interfering with movement and breathing. […] 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.
- #23 Pseudocholinesterase Deficiency – MD Searchlighthttps://mdsearchlight.com/health/pseudocholinesterase-deficiency/
Pseudocholinesterase deficiency is a rare condition that can be inherited or acquired. Its a defect in an enzyme produced by the liver called pseudocholinesterase. […] People with pseudocholinesterase deficiency are usually diagnosed after they experience extended muscle paralysis after having standard doses of certain medications like succinylcholine and mivacurium. The main treatment for this condition is respiratory support with the help of a mechanical ventilator. This system helps the patient breathe until their own muscle function begins to recover naturally. […] The preferred treatment is to provide support with sedation (medications to help the patient relax or sleep) and mechanical ventilation (machine-assisted breathing), until recovery takes place usually occurring within several hours.
- #24 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/books/NBK541032/
Pseudocholinesterase deficiency is a clinical condition that is often only discovered after exposure to succinylcholine or mivacurium. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. […] Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] While a rare condition, pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #25 What Is Pseudocholinesterase Deficiency?https://www.icliniq.com/articles/genetic-disorders/pseudocholinesterase-deficiency
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. […] Note: Patients with pseudocholinesterase deficiency should avoid drugs like Succinylcholine and Mivacurium to prevent the future occurrence of prolonged paralysis. […] Patients suffering from Pseudocholinesterase deficiency should also be properly educated about the adverse reactions of these drugs.
- #26 Decoding Pseudocholinesterase Deficiency: Understanding, Diagnosing, and the Role of Genetic Testinghttps://sequencing.com/education-center/medical/pseudocholinesterase-deficiency?srsltid=AfmBOooYUnjnddphvIosCPF0PA1nFCYIUgxdfGdNjueSQxcNyjAtlKPZ
Understanding, diagnosing, and managing pseudocholinesterase deficiency is essential for healthcare providers to ensure the safety and well-being of their patients. Genetic testing plays a crucial role in identifying the genetic causes of the condition, developing animal models for research, and screening and counseling at-risk individuals.
- #27 Pseudocholinesterase Deficiency: Symptoms & Treatmenthttps://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: Succinylcholine, Mivacurium, Procaine, Chloroprocaine, Benzocaine, Tetracaine. […] If you receive a pseudocholinesterase deficiency diagnosis, letting your healthcare providers know is the best thing you can do especially those you see for surgical procedures.
- #28 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #29 Pseudocholinesterase deficiencyhttps://johnsonmemorial.org/jmh-health/disease-conditions/con-20200770
Pseudocholinesterase deficiency can be caused by a gene change (mutation) that is inherited. […] 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 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.
- #30 Pseudocholinesterase deficiencyhttps://ask-ahd.ahdubai.com/con-20200770
This causes sensitivity to certain muscle relaxants used during anesthesia. Muscles may stay relaxed for too long, interfering with movement and breathing. […] Pseudocholinesterase deficiency causes the muscles to stay relaxed for too long, which prevents you from moving or breathing on your own for a few hours after receiving the drug. […] 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.
- #31https://cacgas.com/forms
1) Add „Pseudocholinesterase deficiency” to the „Allergies” of the patient. This will ensure it follows patient throughout hospitalization. It will also be added to their wrist band for this, and future admissions. […] 2) Launch the „Psudocholinesterase deficiency” order set: This will instruct nursing to give a copy of the” „PROLONGED NMB letter” to the patient (family) on discharge, and also add a copy of the letter to the chart. […] 3) Complete and print the PROLONGED NMB LETTER. […] At surgery Centers, please complete the PROLONGED NMB LETTER, and give one copy to patient, one copy for chart.
- #32 AANA Journal June 2016: Unknown Pseudocholinesterase Deficiency in a Patient Undergoing TIVA with Planned Motor Evoked Potential Monitoring: A Case Reporthttp://www.onlinedigeditions.com/article/Unknown+Pseudocholinesterase+Deficiency+in+a+Patient+Undergoing+TIVA+with+Planned+Motor+Evoked+Potential+Monitoring%3A+A+Case+Report/2493563/304411/article.html
Pseudocholinesterase abnormalities are a genetic cause of aberrant metabolism of the depolarizing muscle relaxant succinylcholine. […] This case study highlights the intraoperative and postoperative management of an elderly patient with an unknown pseudocholinesterase deficiency. […] In the presence of an unknown pseudocholinesterase deficiency, the duration of paralysis after succinylcholine administration may be significantly increased, resulting in the inability to monitor evoked potentials. […] The lack of response led to a presumptive diagnosis of pseudocholinesterase deficiency. […] The patient was educated regarding his atypical enzyme and given a letter regarding his pseudocholinesterase deficiency for future anesthesia experiences. […] The inability to elicit a TOF response led the student registered nurse anesthetist to an early presumptive diagnosis of pseudocholinesterase deficiency.
- #33 Pseudocholinesterase Deficiency – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Grouphttps://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. […] 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. […] After surgery, postoperative monitoring is key for these patients. They might need to stay in the post-anesthesia care unit (PACU) longer to fully recover. Monitoring should include checks on breathing, oxygen levels, and muscle strength. […] Understanding the anesthetic implications of pseudocholinesterase deficiency helps healthcare providers give safe and effective care. This is important for patients during surgical procedures.
- #34 Pseudocholinesterase Deficiency and Patient Perspectiveshttps://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. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. […] It is imperative that patients are re-sedated in a timely manner to limit patient discomfort. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested.
- #35https://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. […] It is thus important to revisit PChE deficiency, and start a conversation on this life-threatening but relatively easy-to-manage complication following succinylcholine administration. […] Although PChE deficiency is a rare disorder, it is a serious condition since it is difficult to diagnose clinically preoperatively and can be potentially fatal. However, when diagnosed, it is treatable with ventilatory support till spontaneous recovery. […] A thorough preoperative evaluation can guide the anesthesiologist to the possibility of PChE deficiency. […] It is, therefore, prudent that a detailed preoperative evaluation for any suggestive history or acquired causes of PChE deficiency should be done. Keep a high index of suspicion in the event of delayed or inadequate recovery from neuromuscular block. Plasma enzyme assay will help confirm the diagnosis. […] 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.
- #36 Pseudocholinesterase deficiency // Middlesex Healthhttps://middlesexhealth.org/learning-center/diseases-and-conditions/pseudocholinesterase-deficiency
Pseudocholinesterase deficiency can be caused by a gene change (mutation) that is inherited. […] 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. […] 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.
- #37 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/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. This activity reviews the evaluation and management of pseudocholinesterase deficiency, and highlights the role of the interprofessional team in managing patients with this condition. […] Explain the importance of improving care coordination amongst the interprofessional team to enhance the delivery of care for patients with pseudocholinesterase deficiency. […] The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. […] Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #38 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #39 SMA Abstract: Pseudocholinesterase Deficiency: A Case Studyhttps://sma.org/abstracts/pseudocholinesterase-deficiency/
Upon completion of this lecture, learners should be better prepared to: describe the management of patients with pseudocholinesterase deficiency; […] Upon completion of this lecture, learners should be better prepared to: explain the importance of identifying patients with pseudocholinesterase deficiency. […] It is important to quickly recognize this rare condition as patient care post-surgery will need to include mechanical ventilation until the medication has been fully metabolized. […] Management of pseudocholinesterase deficiency is typically conservative treatment.
- #40https://www.apicareonline.com/index.php/APIC/article/view/296
The management of an undetected pseudocholinesterase deficiency in a parturient who underwent urgent cesarean section has been presented. […] Upon persistent prolonged neuromuscular blockade sugammadex was administered. […] the importance of suspecting pseudocholinesterase deficiency and the need of neuromuscular monitoring have been argued in this case report.
- #41https://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. […] Since the measurement of PCE levels of all patients eligible for ECT is part of our pre-ECT assessment procedure, we could detect the deficiency before the ECT procedure. […] 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 patientsâ serum pseudocholinesterase levels is part of our routine preoperative laboratory workup. […] 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.
- #42 Pseudocholinesterase Deficiency Treatment & Management: Medical Carehttps://emedicine.medscape.com/article/247019-treatment
Patients with prolonged paralysis following administration of succinylcholine can be treated in the following ways: […] 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. […] 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.
- #43 Pseudocholinesterase Deficiency: Symptoms & Treatmenthttps://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. […] 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. […] You can’t prevent inherited pseudocholinesterase deficiency. But if it runs in your family, you can get tested for the BCHE gene mutation before undergoing general anesthesia. This can help you avoid surgical complications.
- #44 Decoding Pseudocholinesterase Deficiency: Understanding, Diagnosing, and the Role of Genetic Testinghttps://sequencing.com/education-center/medical/pseudocholinesterase-deficiency?srsltid=AfmBOooYUnjnddphvIosCPF0PA1nFCYIUgxdfGdNjueSQxcNyjAtlKPZ
Pseudocholinesterase deficiency is a rare genetic disorder that affects the bodys ability to break down certain drugs, such as muscle relaxants used during anesthesia. This can lead to prolonged neuromuscular block and other complications. […] Understanding, diagnosing, and managing this condition is essential for healthcare providers, especially in the context of surgical procedures. […] For patients with known or suspected pseudocholinesterase deficiency, it is crucial to carefully manage their post-operative care. This may include maintaining intubated status until the patients ability to draw proper tidal volumes returns. Additionally, healthcare providers should be aware of alternative anesthetic agents that can be used in patients with this condition. […] Genetic testing can help identify the specific gene mutations responsible for pseudocholinesterase deficiency. This can be particularly useful for individuals with a family history of the condition or those who have experienced prolonged neuromuscular block after anesthesia. Knowing the genetic cause can help healthcare providers make informed decisions about anesthesia management and post-operative care.
- #45 Pseudocholinesterase Deficiency – Acibadem Health Point – ACIBADEM Hospitals – Acibadem Health Grouphttps://www.acibademhealthpoint.com/cat/health-library/pseudocholinesterase-deficiency/
Its very important to manage anesthesia carefully for pregnant women with pseudocholinesterase deficiency. This helps reduce risks and ensures a safe delivery. Anesthesiologists and obstetricians should work together to create a plan that fits the patients needs and medical history. […] By making lifestyle changes and teaching family, those with pseudocholinesterase deficiency can manage their health better. Remember, knowledge is power when it comes to managing rare genetic disorders like pseudocholinesterase deficiency.
- #46 What Is Pseudocholinesterase Deficiency?https://www.icliniq.com/articles/genetic-disorders/pseudocholinesterase-deficiency
Pseudocholinesterase deficiency, also known as butyrylcholinesterase deficiency, is an inherited or acquired condition in which the metabolism of certain choline ester drugs such as Succinylcholine, Mivacurium, and ester-linked local anesthetics is altered. […] People with a deficiency of pseudocholinesterase enzymes suffer from prolonged paralysis and apnea after administering drugs like Succinylcholine or Mivacurium. Therefore, these drugs should not be administered to patients having pseudocholinesterase deficiency. […] Pseudocholinesterase deficiency, whether inherited or acquired, should be considered by providers that administer Succinylcholine, including anesthesia, intensive care unit (ICU), emergency department, and perioperative personnel, including the nurses to the patients. Special screening should be done for pregnant women who have either genetic or acquired variants of the condition.
- #47https://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. […] It is thus important to revisit PChE deficiency, and start a conversation on this life-threatening but relatively easy-to-manage complication following succinylcholine administration. […] Although PChE deficiency is a rare disorder, it is a serious condition since it is difficult to diagnose clinically preoperatively and can be potentially fatal. However, when diagnosed, it is treatable with ventilatory support till spontaneous recovery. […] A thorough preoperative evaluation can guide the anesthesiologist to the possibility of PChE deficiency. […] It is, therefore, prudent that a detailed preoperative evaluation for any suggestive history or acquired causes of PChE deficiency should be done. Keep a high index of suspicion in the event of delayed or inadequate recovery from neuromuscular block. Plasma enzyme assay will help confirm the diagnosis. […] 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.
- #48 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #49 Acquired pseudocholinesterase deficiency after high-dose cyclophosphamide | Bone Marrow Transplantationhttps://www.nature.com/articles/1702097
Succinylcholine, a depolarizing neuromuscular blocking agent used in anesthesia is hydrolyzed in the plasma by the enzyme pseudocholinesterase (PSC). […] Conditions associated with reduced PSC activity lead to sustained action of succinylcholine and result in prolonged apnea. […] This case highlights the potential risk of drug-induced PSC deficiency and cautions the use of depolarizing muscular relaxants soon after high-dose cyclophosphamide.
- #50 Mayo Clinic Health Library – Pseudocholinesterase deficiency | Swiss Medical Networkhttps://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. […] 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 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.
- #51https://cacgas.com/forms
1) Add „Pseudocholinesterase deficiency” to the „Allergies” of the patient. This will ensure it follows patient throughout hospitalization. It will also be added to their wrist band for this, and future admissions. […] 2) Launch the „Psudocholinesterase deficiency” order set: This will instruct nursing to give a copy of the” „PROLONGED NMB letter” to the patient (family) on discharge, and also add a copy of the letter to the chart. […] 3) Complete and print the PROLONGED NMB LETTER. […] At surgery Centers, please complete the PROLONGED NMB LETTER, and give one copy to patient, one copy for chart.
- #52 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #53 Health Information Library | Rumah Sakit Pusat Pertaminahttps://rspp.co.id/dcontent.html?id=CON-20200770&n=Pseudocholinesterase%20deficiency
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.
- #54 Pseudocholinesterase deficiencyhttps://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 used during anesthesia. […] 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. […] 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.
- #55 Residual Muscle Weakness after Succinylcholine Infusion: Clinical Presentation, Diagnosis and Treatmenthttps://austinpublishinggroup.com/anesthesia-analgesia/fulltext/ajaa-v2-id1022.php
Pseudocholinesterase deficiency is synthesized by the liver and exists in plasma. It breaks down Succinylcholine to inactive metabolites. […] The lab results of the 2nd patient did show decreased pseudocholinesterase level (though dubicaine number is unavailable), which indicated decreased enzyme quantity. […] The presence of chronic malignancy increases the likelihood of pseudocholinesterase abnormality, including both quality and quantity, not to mention the possibility of genetic variant of pseudocholinesterase. […] The detection of decreased pseudocholinesterase level may warrant further lab test such as dubicaine number or genetic test for pseudocholinesterase. […] It is prudent to apply neuromuscular monitoring (such as acceleromyography with train-of-four nerve stimulation) during succinylcholine infusion even when the surgery is considered a short procedure.
- #56 2025 Critical Care Congresshttps://congress2025.eventscribe.net/ajaxcalls/PosterInfo.asp?PosterID=718320
PChE deficiency is rare and results in prolonged paralysis and apnea when NMBAs are administered. […] He was kept intubated, sedated, and transferred to the ICU. […] The diagnosis is confirmed by measuring the plasma PChE activity assay. Normal levels range from 4,000 13,500 U/L while reduced levels indicate deficiency. […] Management includes supportive care and mechanical ventilation. The prognosis is excellent with early detection and recovery is spontaneous. Thus, early recognition is imperative for prevention of complications during anesthesia in affected or high risk individuals.
- #57https://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. […] Since the measurement of PCE levels of all patients eligible for ECT is part of our pre-ECT assessment procedure, we could detect the deficiency before the ECT procedure. […] 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 patientsâ serum pseudocholinesterase levels is part of our routine preoperative laboratory workup. […] 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.
- #58 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelfhttps://www.ncbi.nlm.nih.gov/sites/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. This activity reviews the evaluation and management of pseudocholinesterase deficiency, and highlights the role of the interprofessional team in managing patients with this condition. […] Explain the importance of improving care coordination amongst the interprofessional team to enhance the delivery of care for patients with pseudocholinesterase deficiency. […] The mainstay of treatment involves respiratory support with mechanical ventilation until the spontaneous resolution of neuromuscular blockade. […] Management for those previously diagnosed with pseudocholinesterase deficiency includes the avoidance of depolarizing the neuromuscular blocker succinylcholine and the non-depolarizing muscular blocker mivacurium. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #59 Pseudocholinesterase Deficiency | Treatment & Management | Point of Carehttps://www.statpearls.com/point-of-care/27928
Pseudocholinesterase deficiency can be acquired and/or be inherited. […] 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. […] 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. […] Individuals diagnosed with pseudocholinesterase deficiency should inform their doctor and anesthesia provider of their condition before any surgery. The patient’s medical record should be updated to reflect the diagnosis of pseudocholinesterase deficiency. Future anesthetics should avoid administration of succinylcholine and mivacurium to avoid prolonged neuromuscular blockade and possibly respiratory failure. […] Pseudocholinesterase deficiency is best addressed via an interprofessional healthcare team approach, with physicians, specialists, specialty trained nursing staff, and pharmacists, working and communicating to achieve the best possible patient outcomes.
- #60 Pseudocholinesterase Deficiency: A Case Report and Literature Reviewhttps://file.scirp.org/Html/21-1920056_22247.htm
Pseudocholinesterase deficiency is a rare cause of prolonged paralysis, regarding which any clinician utilizing paralytics should be educated. […] The management of patients with prolonged paralysis due to PChE deficiency is mainly conservative. Patients are maintained with mechanical ventilation under sedation while the effect of the paralytic is cleared. […] If PChE deficiency is suspected, the patient and family should be counseled with recommendation of further diagnostic tests and a Medic Alert bracelet should be issued.
- #61 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Texthttps://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. 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.
- #62https://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. […] It is thus important to revisit PChE deficiency, and start a conversation on this life-threatening but relatively easy-to-manage complication following succinylcholine administration. […] Although PChE deficiency is a rare disorder, it is a serious condition since it is difficult to diagnose clinically preoperatively and can be potentially fatal. However, when diagnosed, it is treatable with ventilatory support till spontaneous recovery. […] A thorough preoperative evaluation can guide the anesthesiologist to the possibility of PChE deficiency. […] It is, therefore, prudent that a detailed preoperative evaluation for any suggestive history or acquired causes of PChE deficiency should be done. Keep a high index of suspicion in the event of delayed or inadequate recovery from neuromuscular block. Plasma enzyme assay will help confirm the diagnosis. […] 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.
- #63 Pseudocholinesterase Deficiency: Symptoms & Treatmenthttps://www.medicoverhospitals.in/diseases/pseudocholinesterase-deficiency/
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. […] Treatment focuses on careful management during anesthesia and may include using alternative medications that do not rely on cholinesterase for metabolism.