Niedobór pseudocholinesterazy
Zapobieganie i profilaktyka

Niedobór pseudocholinesterazy jest klinicznie niemym schorzeniem ujawniającym się podczas ekspozycji na estry choliny, zwłaszcza w trakcie znieczulenia ogólnego. Diagnostyka opiera się na oznaczeniu aktywności enzymu w osoczu oraz badaniach genetycznych, w tym PCR identyfikującym nietypowe allele genu pseudocholinesterazy. Wartości aktywności enzymu mogą być obniżone nawet o 24-33% u kobiet w ciąży i w okresie poporodowym, co zwiększa ryzyko powikłań. Pacjenci z niedoborem powinni nosić identyfikatory medyczne (MedicAlert) i posiadać kartę informacyjną, a także informować lekarzy o schorzeniu przed każdym zabiegiem. W rodzinach z historią niedoboru zalecane jest poradnictwo genetyczne oraz badania przesiewowe, ze względu na autosomalny recesywny charakter dziedziczenia.

Informowanie o niedoborze pseudocholinesterazy

Niedobór pseudocholinesterazy jest stanem klinicznie niemym, który ujawnia się dopiero podczas ekspozycji na zewnętrzne źródła estrów choliny, szczególnie w trakcie znieczulenia ogólnego.1 Osoby ze zdiagnozowanym niedoborem pseudocholinesterazy powinny bezwzględnie informować swojego lekarza oraz anestezjologa o swoim schorzeniu przed każdym zabiegiem operacyjnym.23 Karta medyczna pacjenta powinna być zaktualizowana, aby odzwierciedlać diagnozę niedoboru pseudocholinesterazy.4

Jeśli w rodzinie występował niedobór pseudocholinesterazy lub którykolwiek z członków rodziny miał problemy ze znieczuleniem, należy poinformować o tym lekarza przed zabiegiem wymagającym znieczulenia.56 Ocena ryzyka niedoboru pseudocholinesterazy pozwala anestezjologowi na uniknięcie stosowania określonych środków zwiotczających mięśnie, w razie potrzeby.7

Noszenie identyfikatorów medycznych

Osoby z rozpoznanym niedoborem pseudocholinesterazy powinny nosić bransoletkę lub naszyjnik medyczny (tzw. MedicAlert) oraz posiadać kartę informacyjną w portfelu.89 Tego typu identyfikatory medyczne pozwalają pracownikom służby zdrowia na rozpoznanie ryzyka związanego z niedoborem pseudocholinesterazy, szczególnie w sytuacjach nagłych.1011 Noszenie takich identyfikatorów jest szczególnie istotne w przypadku konieczności przeprowadzenia nieplanowanej operacji lub w sytuacji wypadku.12

Pacjenci powinni również otrzymać kartę ostrzegawczą, która będzie alertować lekarzy o tej nieprawidłowości biochemicznej, szczególnie jeśli defekt ma pochodzenie genetyczne i jest trwały.13

Badania przesiewowe i profilaktyczne

W przypadku występowania niedoboru pseudocholinesterazy w rodzinie, można zapobiec problemom podczas znieczulenia poprzez wykonanie badań przed zabiegiem.14 Badania przesiewowe w kierunku aktywności pseudocholinesterazy są zalecane, jeśli pacjent lub bliski krewny doświadczył przedłużonego paraliżu i bezdechu po zastosowaniu sukcynylocholiny podczas znieczulenia.15

Badania genetyczne i laboratoryjne

Ponieważ znana jest sekwencja DNA genu pseudocholinesterazy i jego struktura aminokwasowa, nietypowe allele mogą być obecnie identyfikowane za pomocą badań amplifikacji PCR przy użyciu DNA wyekstrahowanego z leukocytów we krwi.16 Konsultacja z genetykiem może pomóc w identyfikacji specyficznych atypowych alleli genotypu przyczyniających się do niedoboru pseudocholinesterazy.17

Badania laboratoryjne mogą obejmować oznaczenie liczby dibukainy lub poziomu pseudocholinesterazy w osoczu.18 Diagnostyka może zostać potwierdzona poprzez pomiar aktywności pseudocholinesterazy w osoczu.19

Badania krewnych

Członkowie rodzin pacjentów z niedoborem pseudocholinesterazy są zachęcani do poddania się badaniom laboratoryjnym ze względu na silny komponent genetyczny związany z dziedziczeniem nieprawidłowego wariantu genu pseudocholinesterazy.2021 Po rozpoznaniu niedoboru pseudocholinesterazy u pacjenta, ważne jest, aby określić poziom pseudocholinesterazy u pacjenta i wszystkich członków rodziny.2223

Członkowie rodzin osób dotkniętych tym schorzeniem powinni rozważyć poradnictwo genetyczne i badania w celu określenia ryzyka nosicielstwa lub przekazania wadliwego genu.24 Jest to szczególnie istotne, biorąc pod uwagę autosomalny recesywny sposób dziedziczenia niedoboru pseudocholinesterazy.25

Unikanie leków ryzyka

Podstawowym działaniem profilaktycznym dla osób z niedoborem pseudocholinesterazy jest unikanie określonych leków, które mogą wywołać przedłużone zwiotczenie mięśniowe i potencjalnie prowadzić do niewydolności oddechowej.2627

Leki przeciwwskazane

U pacjentów z niedoborem pseudocholinesterazy należy unikać stosowania następujących środków:28

  • Sukcynylocholiny (depolaryzujący środek zwiotczający mięśnie)2930
  • Miwakurium (niedepolaryzujący środek zwiotczający mięśnie)3132
  • Estrów miejscowych anestetyków, takich jak prokaina3334

Osoby z niedoborem pseudocholinesterazy, które wymagają znieczulenia, mogą uniknąć przedłużonego paraliżu poprzez zastosowanie innych środków zwiotczających mięśnie o odmiennym mechanizmie metabolizmu.35

Alternatywne środki zwiotczające

Dla pacjentów z niedoborem pseudocholinesterazy bezpieczne są inne niedepolaryzujące środki zwiotczające mięśnie, takie jak:3637

W przypadku elektrowstrząsów (ECT) u pacjentów z niedoborem pseudocholinesterazy, podanie rokuronium z następczym odwróceniem blokady nerwowo-mięśniowej sugammadeksem wydaje się lepszą opcją niż sukcynylocholina.45 Zalety stosowania rokuronium z sugammadeksem dla ECT, w tym szybkie ustąpienie zwiotczenia mięśniowego i mniejsza liczba działań niepożądanych, mogą przewyższać wysokie koszty ich stosowania.46

Postępowanie u osób z podwyższonym ryzykiem

Szczególnej uwagi wymagają określone grupy pacjentów z potencjalnie podwyższonym ryzykiem niedoboru pseudocholinesterazy, w tym kobiety w ciąży i osoby z określonymi schorzeniami.

Kobiety w ciąży

Aktywność enzymatyczna pseudocholinesterazy jest zmniejszona o 24% w czasie ciąży, 25% w pierwszym dniu po porodzie i 33% w trzecim dniu po porodzie. Dlatego wybór odpowiedniego planu znieczulenia dla kobiet w ciąży jest ważny, ponieważ normalne zmniejszenie poziomów enzymu w osoczu w połączeniu z wariantem genetycznym może okazać się śmiertelne w przypadku ekspozycji na sukcynylocholinę lub miejscowy anestetyk estrowy, taki jak chloroprokaina.47

Pacjenci z chorobami współtowarzyszącymi

Osoby z nabytym niedoborem pseudocholinesterazy mogą zmniejszyć ryzyko poprzez leczenie chorób podstawowych, takich jak niedożywienie lub przewlekła choroba nerek (CKD).48 W przypadku nabytego niedoboru pseudocholinesterazy, unikanie ekspozycji na określone substancje chemiczne i zarządzanie podstawowymi stanami zdrowotnymi, takimi jak choroba wątroby, może pomóc w utrzymaniu poziomów aktywności enzymu.49

Dokładna ocena przedoperacyjna powinna obejmować szczegółową historię przyjmowania leków. Zencirci zgłosił niedawno nabyty niedobór pseudocholinesterazy związany z sertraliną i przedłużone porażenie mięśni po podaniu miwakurium.50

Osoby z grup etnicznych o podwyższonym ryzyku

W niektórych społecznościach częstość występowania niedoboru pseudocholinesterazy może być wyższa. Na przykład społeczność Arya Vysya w Indiach ma wyższy odsetek osób dotkniętych homozygotyczną mutacją, z częstością występowania 2-4%.51 Sugeruje się, aby osoby z tej społeczności z historią niedoboru pseudocholinesterazy informowały swojego lekarza przed poddaniem się znieczuleniu do operacji, aby uniknąć potencjalnie poważnych niepożądanych skutków.52

Postępowanie w przypadku podejrzenia niedoboru

Wczesne rozpoznanie niedoboru pseudocholinesterazy jest kluczowe dla zapobiegania powikłaniom podczas znieczulenia u osób dotkniętych tym schorzeniem lub osób z grupy wysokiego ryzyka.53

Rozpoznanie kliniczne

Niedobór pseudocholinesterazy należy uwzględnić w diagnostyce różnicowej opóźnionego wybudzenia po znieczuleniu ogólnym.54 Połączenie utrzymującego się paraliżu pomimo wskaźników coraz lżejszej sedacji resztkowej lub dyskomfortu (tj. zwiększających się wskaźników EEG, tachykardii i nadciśnienia) powinno natychmiast wzbudzić podejrzenia nieprawidłowej odpowiedzi na miwakurium lub sukcynylocholinę.55

Po podejrzeniu, diagnozę kliniczną można potwierdzić za pomocą stymulatora nerwów obwodowych i badań laboratoryjnych, podczas gdy badania genetyczne mogą pomóc w określeniu etiologii choroby.56

Postępowanie w przypadku przedłużonego paraliżu

Jeśli podejrzewa się, że niedobór pseudocholinesterazy powoduje nieoczekiwany przedłużony bezdech po podaniu sukcynylocholiny, bardzo ważne są następujące środki:57

  • Kontrolowana wentylacja mechaniczna z wystarczającą sedacją, aż do całkowitego zmetabolizowania sukcynylocholiny58
  • Określenie poziomów pseudocholinesterazy u pacjenta i wszystkich członków rodziny59
  • Unikanie dalszej ekspozycji na sukcynylocholinę, miwakurium i aminoestrowe środki znieczulenia miejscowego60
  • Powiadomienie lekarzy podczas każdej kolejnej wizyty w szpitalu, że pacjent ma niedobór pseudocholinesterazy61

Podstawą leczenia u tych pacjentów jest kontynuacja wentylacji mechanicznej z sedacją i kontynuowanie monitorowania nerwów obwodowych, aż do ustąpienia paraliżu i powrotu aktywności mięśniowej do normy.62

Profilaktyczne przetaczanie osocza

Profilaktyczne przetaczanie świeżo mrożonego osocza może wzmocnić endogenną aktywność pseudocholinesterazy w osoczu pacjenta. Praktyka ta nie jest zalecana ze względu na ryzyko jatrogennych powikłań infekcji wirusowych. Jednakże okołooperacyjne przetaczanie świeżo mrożonego osocza podawanego w celu skorygowania koagulopatii może maskować podstawowy niedobór pseudocholinesterazy.63

Edukacja pacjentów

Właściwa edukacja pacjentów na temat nowej diagnozy niedoboru pseudocholinesterazy jest kluczowa dla zapobiegania przyszłym incydentom przedłużonego paraliżu.64 Pacjenci powinni być świadomi, że ich wrażliwość na sukcynylocholinę naraża ich na szkody i stanowi ryzyko zdrowotne związane ze znieczuleniem.65

Komunikacja z personelem medycznym

Osoby cierpiące na niedobór pseudocholinesterazy powinny poinformować swojego lekarza lub farmaceutę podczas przepisywania leków.66 Inną metodą zapobiegania ponownemu wystąpieniu jest umieszczenie miwakurium i sukcynylocholiny jako źródeł alergii lub niepożądanych reakcji pacjenta.67

Ponownemu wystąpieniu można również zapobiec poprzez dokładnie przeprowadzoną ocenę przedoperacyjną przez anestezjologa. Pytania dotyczące wcześniejszych operacji i problemów ze znieczuleniem w przeszłości mogą ujawnić historię niedoboru pseudocholinesterazy u pacjentów.68

Rozpoznanie w ocenie przedoperacyjnej

Ważne jest, aby zapytać potencjalnych kandydatów do operacji, czy mieli złe reakcje na środki znieczulające w przeszłości. Jeśli powrót siły mięśniowej był opóźniony, to prawdopodobnie pacjent ma niedobór pseudocholinesterazy.69

Chociaż testy diagnostyczne w kierunku niedoboru pseudocholinesterazy nie są częścią rutynowego przedoperacyjnego badania, a pacjenci są zazwyczaj bezobjawowi w codziennym życiu, wczesna diagnoza niedoboru pseudocholinesterazy ma kluczowe znaczenie.70 Pozwala uniknąć niepotrzebnej ekspozycji na inne leki (na przykład nalokson, flumazenil i fizostygminę) i zapewnić doskonałe rokowanie po rozpoczęciu leczenia wspomagającego.71

W niektórych ośrodkach pomiar poziomów pseudocholinesterazy jest częścią rutynowej oceny przed zabiegami, co pozwala na odpowiedni dobór środków zwiotczających mięśnie i zapobiega powikłaniom.72

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

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

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

Materiały źródłowe

  • #1 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. […] 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. […] 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.
  • #2 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    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. […] Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene.
  • #3 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #4 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #5 Health Information Library | Rumah Sakit Pusat Pertamina
    https://rspp.co.id/dcontent.html?id=CON-20200770&n=Pseudocholinesterase%20deficiency
    If you have a family history of pseudocholinesterase deficiency or have a family member who had any problem with anesthesia, tell your health care provider before getting a medical procedure that requires anesthesia. […] 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.
  • #6 Pseudocholinesterase deficiency
    https://www.mymlc.com/health-information/diseases-and-conditions/p/pseudocholinesterase-deficiency/
    If you have a family history of pseudocholinesterase deficiency or have a family member who had any problem with anesthesia, tell your doctor before getting a medical procedure that requires general anesthesia. […] Evaluating your risk of pseudocholinesterase deficiency allows your anesthesiologist to avoid certain drugs, if needed.
  • #7 Health Information Library | Rumah Sakit Pusat Pertamina
    https://rspp.co.id/dcontent.html?id=CON-20200770&n=Pseudocholinesterase%20deficiency
    If you have a family history of pseudocholinesterase deficiency or have a family member who had any problem with anesthesia, tell your health care provider before getting a medical procedure that requires anesthesia. […] 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.
  • #8 Pseudocholinesterase deficiency – Diagnosis & treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/pseudocholinesterase-deficiency/diagnosis-treatment/drc-20354545
    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’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.
  • #9 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    You cant 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. […] 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. […] You may be able to reduce your risk for acquired pseudocholinesterase deficiency by treating underlying health conditions like malnutrition or chronic kidney disease (CKD). You can also ask your healthcare provider about switching medications if youre taking something that lowers your production of pseudocholinesterase enzyme. […] If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: […] Consider wearing a medical ID bracelet that informs healthcare providers about your condition. This can be helpful if you ever need emergency surgery.
  • #10 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. […] If this condition is recognized by the anesthesiologist early, then there is rarely a problem, as the patient can be kept intubated and sedated until the muscle relaxation resolves.
  • #11 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency results in the slower metabolism of medications used in anesthesia like succinylcholine, mivacurium, procaine, etc. […] A patients sensitivity towards succinylcholine makes him or her susceptible to harm and puts him or her at a health risk from anaesthesia. […] If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] 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. […] If your surgeon/doctor is unaware of your pseudocholinesterase deficiency, then you may suffer from loss of breathing.
  • #12 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency
    Pseudocholinesterase deficiency results in the slower metabolism of medications used in anesthesia like succinylcholine, mivacurium, procaine, etc. […] A patient’s sensitivity towards succinylcholine makes him or her susceptible to harm and puts him or her at a health risk from anesthesia. […] If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] 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. […] If your surgeon/doctor is unaware of your pseudocholinesterase deficiency, then you may suffer from loss of breathing.
  • #13
    https://link.springer.com/article/10.1007/BF03002224
    Pseudocholinesterase is essential for the rapid breakdown of succinylcholine. If pseudocholinesterase is quantitatively or qualitatively deficient, prolonged apnoea may result after administration of succinylcholine. […] Knowledge of a low pseudocholinesterase level will certainly be of interest. […] If it is of genetic origin, it is a permanent defect and such persons should receive a warning card to alert physicians regarding this biochemical abnormality. […] Tous les individus chez qui on dpiste un trouble des pseudocholinesterases devraient porter une carte ou un disque davertissement.
  • #14 Health Information Library | Rumah Sakit Pusat Pertamina
    https://rspp.co.id/dcontent.html?id=CON-20200770&n=Pseudocholinesterase%20deficiency
    If you have a family history of pseudocholinesterase deficiency or have a family member who had any problem with anesthesia, tell your health care provider before getting a medical procedure that requires anesthesia. […] 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.
  • #15 Cholinesterase Tests – Testing.com
    https://www.testing.com/tests/cholinesterase-test/
    Sometimes to identify individuals with inherited pseudocholinesterase deficiency before they are given anesthesia with the muscle relaxant succinylcholine or to help determine the cause of prolonged apnea after surgical anesthesia. […] Pre-operative screening for pseudocholinesterase activity is advised if a person or a close relative has experienced prolonged paralysis and apnea after the use of succinylcholine for anesthesia during an operation. […] Pseudocholinesterase testing can be performed prior to surgery on those with a family history of prolonged apnea after use of succinylcholine to determine if they are at risk of complications related to this drug.
  • #16 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. […] 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. […] 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.
  • #17 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. […] 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. […] 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.
  • #18 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #19 2025 Critical Care Congress
    https://congress2025.eventscribe.net/ajaxcalls/PosterInfo.asp?PosterID=718320
    PChE deficiency is rare and results in prolonged paralysis and apnea when NMBAs are administered. […] The condition is inherited in an autosomal recessive pattern or may be acquired due to liver disease, renal failure, malnutrition, and pregnancy. […] It can be discovered when respiratory paralysis persists after administration of NMBAs. […] The diagnosis is confirmed by measuring the plasma PChE activity assay. […] Management includes supportive care and mechanical ventilation. […] Thus, early recognition is imperative for prevention of complications during anesthesia in affected or high risk individuals.
  • #20 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    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. […] Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene.
  • #21 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #22 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    Pseudocholinesterase deficiency, arising from inherited, acquired, and iatrogenic causes, produces prolonged neuromuscular blockade after succinylcholine administration used for endotracheal intubation. […] Because prolonged apnea due to pseudocholinesterase deficiency is triggered after mivacurium or succinylcholine administration for muscle relaxation, pseudocholinesterase deficiency is clinically asymptomatic in patients without any previous history of receiving succinylcholine or mivacurium. […] Therefore, it was important to determine the pseudocholinesterase levels in the patient and all family members. […] Taking this case into consideration, after succinylcholine administration, anesthesiologists should apply a peripheral nerve stimulator to assess the extent of muscle relaxation reversal or carefully observe the return of spontaneous respiration, and then determine whether to administer an additional non-depolarizing muscle relaxant.
  • #23 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #24 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://www.medicoverhospitals.in/diseases/pseudocholinesterase-deficiency/
    While genetic causes cannot be prevented, awareness and proactive management can significantly reduce the risks associated with pseudocholinesterase deficiency. […] Family members of affected individuals should consider genetic counseling and testing to determine their risk of carrying or passing on the defective gene. […] For those with acquired pseudocholinesterase deficiency, avoiding exposure to certain chemicals and managing underlying health conditions like liver disease can help maintain enzyme activity levels.
  • #25 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #26 Pseudocholinesterase Deficiency – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK541032/
    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. […] Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene.
  • #27 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #28 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    You cant 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. […] 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. […] You may be able to reduce your risk for acquired pseudocholinesterase deficiency by treating underlying health conditions like malnutrition or chronic kidney disease (CKD). You can also ask your healthcare provider about switching medications if youre taking something that lowers your production of pseudocholinesterase enzyme. […] If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: […] Consider wearing a medical ID bracelet that informs healthcare providers about your condition. This can be helpful if you ever need emergency surgery.
  • #29 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #30 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency is usually diagnosed retrospectively after a patient has received general anesthesia with succinylcholine or mivacurium used for endotracheal intubation. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. This involves properly educating patients on this new diagnosis. Another failsafe method to prevent reoccurrence is to list mivacurium and succinylcholine as sources of patient allergies or adverse reactions. Reoccurrence can also be deterred by a thoroughly conducted preoperative assessment by the anesthesia provider. Questions aimed at previous surgeries and issues with anesthesia in the past can elicit a history of pseudocholinesterase deficiency from patients.
  • #31 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #32 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency is usually diagnosed retrospectively after a patient has received general anesthesia with succinylcholine or mivacurium used for endotracheal intubation. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. This involves properly educating patients on this new diagnosis. Another failsafe method to prevent reoccurrence is to list mivacurium and succinylcholine as sources of patient allergies or adverse reactions. Reoccurrence can also be deterred by a thoroughly conducted preoperative assessment by the anesthesia provider. Questions aimed at previous surgeries and issues with anesthesia in the past can elicit a history of pseudocholinesterase deficiency from patients.
  • #33 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. […] Therefore, these drugs should not be administered to patients having pseudocholinesterase deficiency. […] The enzymatic activity is said to be reduced by 24 % during pregnancy, 25 % on day one postpartum, and 33 % on day three postpartum, respectively. Therefore, selecting an appropriate anesthetic plan for the pregnant woman is important because normal reductions in the plasma enzyme levels combined with a genetic variant could prove fatal if exposed to Succinylcholine or a local ester anesthetic like Chloroprocaine. […] 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.
  • #34 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency results in the slower metabolism of medications used in anesthesia like succinylcholine, mivacurium, procaine, etc. […] A patients sensitivity towards succinylcholine makes him or her susceptible to harm and puts him or her at a health risk from anaesthesia. […] If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] 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. […] If your surgeon/doctor is unaware of your pseudocholinesterase deficiency, then you may suffer from loss of breathing.
  • #35 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. […] Pseudocholinesterase individuals in need of anesthesia can avoid paralysis by undergoing treatment with different neuromuscular blockers, such as atracurium and rocuronium. […] Considering the various genetic variants, medical conditions, and yet unknown molecular mechanisms that are linked to pseudocholinesterase deficiency and thereby bring about prolonged anesthesia-induced paralysis, anesthesia providers should take great care when prescribing choline ester anesthetics. […] Pseudocholinesterase deficiency is rare, but anesthesia providers should be equipped to detect it in advance and prevent its potentially harmful consequences.
  • #36 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #37 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #38 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    You cant 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. […] 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. […] You may be able to reduce your risk for acquired pseudocholinesterase deficiency by treating underlying health conditions like malnutrition or chronic kidney disease (CKD). You can also ask your healthcare provider about switching medications if youre taking something that lowers your production of pseudocholinesterase enzyme. […] If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: […] Consider wearing a medical ID bracelet that informs healthcare providers about your condition. This can be helpful if you ever need emergency surgery.
  • #39 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. […] If this condition is recognized by the anesthesiologist early, then there is rarely a problem, as the patient can be kept intubated and sedated until the muscle relaxation resolves.
  • #40 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #41 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #42 Pseudocholinesterase Deficiency | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/27928
    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. Family members of patients with pseudocholinesterase deficiency are encouraged to undergo laboratory testing due to a strong genetic component associated with inheriting an abnormal variant of the pseudocholinesterase gene. […] 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.
  • #43 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #44 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    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. […] Pseudocholinesterase deficiency is inherited in an autosomal recessive manner; however, given the large genetic component, it is recommended that immediate family members get tested. Testing can be done with a dibucaine number as mentioned above, or providers can send for a plasma pseudocholinesterase level.
  • #45 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
    ROC administration followed by sugammadex reversal of neuromuscular blockade seems to be better than succinylcholine for ECT, particularly in patients with pseudocholinesterase deficiency. […] 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.
  • #46 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
    ROC administration followed by sugammadex reversal of neuromuscular blockade seems to be better than succinylcholine for ECT, particularly in patients with pseudocholinesterase deficiency. […] 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.
  • #47 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. […] Therefore, these drugs should not be administered to patients having pseudocholinesterase deficiency. […] The enzymatic activity is said to be reduced by 24 % during pregnancy, 25 % on day one postpartum, and 33 % on day three postpartum, respectively. Therefore, selecting an appropriate anesthetic plan for the pregnant woman is important because normal reductions in the plasma enzyme levels combined with a genetic variant could prove fatal if exposed to Succinylcholine or a local ester anesthetic like Chloroprocaine. […] 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.
  • #48 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/pseudocholinesterase-deficiency
    You cant 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. […] 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. […] You may be able to reduce your risk for acquired pseudocholinesterase deficiency by treating underlying health conditions like malnutrition or chronic kidney disease (CKD). You can also ask your healthcare provider about switching medications if youre taking something that lowers your production of pseudocholinesterase enzyme. […] If you have a pseudocholinesterase deficiency diagnosis, you should avoid certain anesthetic medications, including: […] Consider wearing a medical ID bracelet that informs healthcare providers about your condition. This can be helpful if you ever need emergency surgery.
  • #49 Pseudocholinesterase Deficiency: Symptoms & Treatment
    https://www.medicoverhospitals.in/diseases/pseudocholinesterase-deficiency/
    While genetic causes cannot be prevented, awareness and proactive management can significantly reduce the risks associated with pseudocholinesterase deficiency. […] Family members of affected individuals should consider genetic counseling and testing to determine their risk of carrying or passing on the defective gene. […] For those with acquired pseudocholinesterase deficiency, avoiding exposure to certain chemicals and managing underlying health conditions like liver disease can help maintain enzyme activity levels.
  • #50
    https://journals.lww.com/jica/fulltext/2022/01020/revisiting_pseudocholinesterase_deficiency__the.1.aspx
    Prevention is the best cure and this old adage could not be truer for a patient with PChE deficiency. A thorough preoperative evaluation can guide the anesthesiologist to the possibility of PChE deficiency. A detailed history should include medical history for acquired causes, any previous anesthesia exposure requiring extended mechanical ventilation due to neuromuscular blockade in the patient as well as any family history of relatives in susceptible populations. […] Preoperative evaluation should include a careful history of drug intake. Recently, Zencirci has reported sertraline-associated acquired PChE deficiency and prolonged muscular paralysis following the administration of mivacurium. […] 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. The patient may be advised to wear a MedicAlert bracelet and the immediate family members should be encouraged to get tested keeping in mind the genetic inheritance of PChE deficiency.
  • #51 Pseudocholinesterase Deficiency in an Indian Community | Journal of Pharmacy Practice and Community Medicine
    https://jppcm.org/archives/article/60.html
    Arya Vysya community people of India having deficiency of pseudocholinesterase is the most affected people than any other with homozygous mutation incidence rate of 2-4%. […] It was suggested that the Arya Vysya Community people with a history of pseudocholinesterase deficiency should tell his or her doctor if when they undergo anesthesia for surgery to avoid potentially serious unwanted adverse effects.
  • #52 Pseudocholinesterase Deficiency in an Indian Community | Journal of Pharmacy Practice and Community Medicine
    https://jppcm.org/archives/article/60.html
    Arya Vysya community people of India having deficiency of pseudocholinesterase is the most affected people than any other with homozygous mutation incidence rate of 2-4%. […] It was suggested that the Arya Vysya Community people with a history of pseudocholinesterase deficiency should tell his or her doctor if when they undergo anesthesia for surgery to avoid potentially serious unwanted adverse effects.
  • #53 2025 Critical Care Congress
    https://congress2025.eventscribe.net/ajaxcalls/PosterInfo.asp?PosterID=718320
    PChE deficiency is rare and results in prolonged paralysis and apnea when NMBAs are administered. […] The condition is inherited in an autosomal recessive pattern or may be acquired due to liver disease, renal failure, malnutrition, and pregnancy. […] It can be discovered when respiratory paralysis persists after administration of NMBAs. […] The diagnosis is confirmed by measuring the plasma PChE activity assay. […] Management includes supportive care and mechanical ventilation. […] Thus, early recognition is imperative for prevention of complications during anesthesia in affected or high risk individuals.
  • #54 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. […] Diagnostic tests for pseudocholinesterase deficiency are not part of a routine preoperative work-up, and patients are typically asymptomatic in their daily lives. […] 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. […] To ensure a timely diagnosis, pseudocholinesterase deficiency must be included in the differential diagnosis of delayed emergence, and the combination of sustained paralysis despite indicators of increasingly light residual sedation or distress (that is, increasing EEG indices, tachycardia, and hypertension) should immediately raise suspicions of an abnormal response to mivacurium or succinylcholine. […] Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests, while genetic testing can help determine the etiology of disease.
  • #55 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. […] Diagnostic tests for pseudocholinesterase deficiency are not part of a routine preoperative work-up, and patients are typically asymptomatic in their daily lives. […] 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. […] To ensure a timely diagnosis, pseudocholinesterase deficiency must be included in the differential diagnosis of delayed emergence, and the combination of sustained paralysis despite indicators of increasingly light residual sedation or distress (that is, increasing EEG indices, tachycardia, and hypertension) should immediately raise suspicions of an abnormal response to mivacurium or succinylcholine. […] Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests, while genetic testing can help determine the etiology of disease.
  • #56 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. […] Diagnostic tests for pseudocholinesterase deficiency are not part of a routine preoperative work-up, and patients are typically asymptomatic in their daily lives. […] 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. […] To ensure a timely diagnosis, pseudocholinesterase deficiency must be included in the differential diagnosis of delayed emergence, and the combination of sustained paralysis despite indicators of increasingly light residual sedation or distress (that is, increasing EEG indices, tachycardia, and hypertension) should immediately raise suspicions of an abnormal response to mivacurium or succinylcholine. […] Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests, while genetic testing can help determine the etiology of disease.
  • #57 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #58 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #59 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #60 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #61 Delayed recovery from paralysis by succinylcholine in patient with preoperatively unrecognized and inherited pseudocholinesterase deficiency
    https://ekja.org/journal/view.php?number=7771
    In conclusion, if pseudocholinesterase deficiency is suspected of causing unexpected prolonged apnea after succinylcholine administration, the following measures are very important: 1) controlled mechanical ventilation with sufficient sedation until succinylcholine is completely metabolized; 2) determination of pseudocholinesterase levels in the patient and all family members; 3) avoidance of further exposure to succinylcholine, mivacurium, and aminoester local anesthetics; and 4) notification given to medical doctors on any subsequent hospital visit that a patient has pseudocholinesterase deficiency.
  • #62 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. […] Another effective method to prevent its incidence is to list Mivacurium and Succinylcholine drugs as sources of patient allergies. Reoccurrence can also be reduced by a thoroughly conducted preoperative assessment by the anesthesia and healthcare provider. Patients suffering from Pseudocholinesterase deficiency should also be properly educated about the adverse reactions of these drugs.
  • #63 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. […] 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. […] 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.
  • #64 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency is usually diagnosed retrospectively after a patient has received general anesthesia with succinylcholine or mivacurium used for endotracheal intubation. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. This involves properly educating patients on this new diagnosis. Another failsafe method to prevent reoccurrence is to list mivacurium and succinylcholine as sources of patient allergies or adverse reactions. Reoccurrence can also be deterred by a thoroughly conducted preoperative assessment by the anesthesia provider. Questions aimed at previous surgeries and issues with anesthesia in the past can elicit a history of pseudocholinesterase deficiency from patients.
  • #65 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency results in the slower metabolism of medications used in anesthesia like succinylcholine, mivacurium, procaine, etc. […] A patients sensitivity towards succinylcholine makes him or her susceptible to harm and puts him or her at a health risk from anaesthesia. […] If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] 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. […] If your surgeon/doctor is unaware of your pseudocholinesterase deficiency, then you may suffer from loss of breathing.
  • #66 Pseudocholinesterase Deficiency | Apollo Hospitals
    https://www.apollohospitals.com/diseases-and-conditions/pseudocholinesterase-deficiency/
    Pseudocholinesterase deficiency results in the slower metabolism of medications used in anesthesia like succinylcholine, mivacurium, procaine, etc. […] A patients sensitivity towards succinylcholine makes him or her susceptible to harm and puts him or her at a health risk from anaesthesia. […] If you suffer from PCE deficiency, your first and foremost step should be informing your doctor or physician when they prescribe medications for you. […] 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. […] If your surgeon/doctor is unaware of your pseudocholinesterase deficiency, then you may suffer from loss of breathing.
  • #67 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency is usually diagnosed retrospectively after a patient has received general anesthesia with succinylcholine or mivacurium used for endotracheal intubation. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. This involves properly educating patients on this new diagnosis. Another failsafe method to prevent reoccurrence is to list mivacurium and succinylcholine as sources of patient allergies or adverse reactions. Reoccurrence can also be deterred by a thoroughly conducted preoperative assessment by the anesthesia provider. Questions aimed at previous surgeries and issues with anesthesia in the past can elicit a history of pseudocholinesterase deficiency from patients.
  • #68 Pseudocholinesterase Deficiency and Patient Perspectives
    https://clinmedjournals.org/articles/ijaa/international-journal-of-anesthetics-and-anesthesiology-ijaa-8-124.php
    Pseudocholinesterase deficiency is usually diagnosed retrospectively after a patient has received general anesthesia with succinylcholine or mivacurium used for endotracheal intubation. […] To prevent future incidence of prolonged paralysis, patients with pseudocholinesterase deficiency should avoid succinylcholine and mivacurium. This involves properly educating patients on this new diagnosis. Another failsafe method to prevent reoccurrence is to list mivacurium and succinylcholine as sources of patient allergies or adverse reactions. Reoccurrence can also be deterred by a thoroughly conducted preoperative assessment by the anesthesia provider. Questions aimed at previous surgeries and issues with anesthesia in the past can elicit a history of pseudocholinesterase deficiency from patients.
  • #69 Pseudocholinesterase deficiency – GPnotebook
    https://gpnotebook.com/en-GB/pages/surgery/pseudocholinesterase-deficiency
    It is important to ask potential surgical candidates if they have had bad reactions to anaesthetics in the past. If recovery of muscle power was delayed then it is likely the patient has pseudocholinesterase deficiency. […] Patients who are given suxamethonium for surgery will remain paralysed for much longer than normal and may need ventilation for up to 24 hr. […] Once the patient has recovered, a genetic study should be carried out on the patient and relatives.
  • #70 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. […] Diagnostic tests for pseudocholinesterase deficiency are not part of a routine preoperative work-up, and patients are typically asymptomatic in their daily lives. […] 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. […] To ensure a timely diagnosis, pseudocholinesterase deficiency must be included in the differential diagnosis of delayed emergence, and the combination of sustained paralysis despite indicators of increasingly light residual sedation or distress (that is, increasing EEG indices, tachycardia, and hypertension) should immediately raise suspicions of an abnormal response to mivacurium or succinylcholine. […] Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests, while genetic testing can help determine the etiology of disease.
  • #71 Hereditary pseudocholinesterase deficiency in a 4-year-old girl: a case report | Journal of Medical Case Reports | Full Text
    https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-025-05183-5
    Pseudocholinesterase deficiency is an important, albeit rare, differential diagnosis of delayed emergence from general anesthesia. […] Diagnostic tests for pseudocholinesterase deficiency are not part of a routine preoperative work-up, and patients are typically asymptomatic in their daily lives. […] 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. […] To ensure a timely diagnosis, pseudocholinesterase deficiency must be included in the differential diagnosis of delayed emergence, and the combination of sustained paralysis despite indicators of increasingly light residual sedation or distress (that is, increasing EEG indices, tachycardia, and hypertension) should immediately raise suspicions of an abnormal response to mivacurium or succinylcholine. […] Once suspected, a clinical diagnosis can be supported using a peripheral nerve stimulator, and confirmed using laboratory tests, while genetic testing can help determine the etiology of disease.
  • #72
    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. […] 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. […] 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.