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| __NOTOC__ | | __NOTOC__ |
| {{Glucose-6-phosphate dehydrogenase deficiency}} | | {{Glucose-6-phosphate dehydrogenase deficiency}} |
| {{CMG}}; {{AE}}{{MA}} [mailto:malihash@bidmc.harvard.edu] | | {{CMG}}; {{AE}}{{MA}} |
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| ==Overview== | | ==Overview== |
| There is no treatment for [disease name]; the mainstay of therapy is supportive care.
| | The mainstay of treatment for G6PD deficiency is avoidance of the foods such as [[Fava bean|fava beans]] and drugs that cause [[hemolysis]]. Pharmacologic medical therapy is recommended among patients with chronic [[hemolysis]]. [[Blood transfusion]] can be considered in the acute phase of [[hemolysis]]. |
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| OR
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| Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
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| OR
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| The majority of cases of [disease name] are self-limited and require only supportive care.
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| OR
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| [Disease name] is a medical emergency and requires prompt treatment.
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| OR
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| The mainstay of treatment for [disease name] is [therapy].
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| OR
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| The optimal therapy for [malignancy name] depends on the stage at diagnosis.
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| OR
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| [Therapy] is recommended among all patients who develop [disease name]. | |
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| OR
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| Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3]. | |
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| OR
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| Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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| OR
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| Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
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| OR
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| Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
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| ==Medical Therapy== | | ==Medical Therapy== |
| * The mainstay of treatment for is [therapy]. | | * The mainstay of treatment for G6PD deficiency is avoidance of the foods such as [[Fava bean|fava beans]] and drugs that cause hemolysis.<ref name="pmid7949118">{{cite journal |vauthors=Beutler E |title=G6PD deficiency |journal=Blood |volume=84 |issue=11 |pages=3613–36 |date=December 1994 |pmid=7949118 |doi= |url=}}</ref> |
| | * Neonatal [[jaundice]]: |
| | ** Mild: no treatment |
| | ** Intermediate: [[phototherapy]] |
| | ** Severe: [[exchange transfusions|exchange transfusion]] |
| | * [[Blood transfusion]] in acute phase of [[hemolysis]] |
| | * [[Dialysis]] in [[Acute kidney injury|acute kidney failure]] |
| | * [[Folic Acid|Folic acid]] for patients with chronic [[hemolysis]]<ref name="pmid27040960">{{cite journal |vauthors=Luzzatto L, Nannelli C, Notaro R |title=Glucose-6-Phosphate Dehydrogenase Deficiency |journal=Hematol. Oncol. Clin. North Am. |volume=30 |issue=2 |pages=373–93 |date=April 2016 |pmid=27040960 |doi=10.1016/j.hoc.2015.11.006 |url=}}</ref> |
| | * |
| | * |
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| *Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3]. | | * |
| *Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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| *Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
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| *Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
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| ===Disease Name=== | | ===Disease Name=== |
| | | * '''Adult''' |
| * '''1 Stage 1 - Name of stage'''
| | ** [[Folic Acid|Folic acid]]: 1 mg daily |
| ** 1.1 '''Specific Organ system involved 1'''
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| *** 1.1.1 '''Adult'''
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| **** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''
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| **** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
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| **** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
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| **** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| **** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| **** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
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| *** 1.1.2 '''Pediatric'''
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| **** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
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| ***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
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| ***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
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| ***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
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| ***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
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| ***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
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| ****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
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| ***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
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| ***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
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| ***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
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| ***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
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| ** 1.2 '''Specific Organ system involved 2'''
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| *** 1.2.1 '''Adult'''
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| **** Preferred regimen (1): [[drug name]] 500 mg PO q8h
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| *** 1.2.2 '''Pediatric'''
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| **** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
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| * 2 '''Stage 2 - Name of stage'''
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| ** 2.1 '''Specific Organ system involved 1 '''
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| **: '''Note (1):'''
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| **: '''Note (2)''':
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| **: '''Note (3):'''
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| *** 2.1.1 '''Adult'''
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| **** Parenteral regimen
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| ***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
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| ***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
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| ***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
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| **** Oral regimen
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| ***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
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| ***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
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| ***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
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| ***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| ***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| ***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
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| *** 2.1.2 '''Pediatric'''
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| **** Parenteral regimen
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| ***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
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| ***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
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| ***** Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
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| **** Oral regimen
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| ***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
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| ***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
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| ***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
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| ***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
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| ***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
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| ***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
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| ** 2.2 '<nowiki/>'''''Other Organ system involved 2''''''
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| **: '''Note (1):'''
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| **: '''Note (2)''':
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| **: '''Note (3):'''
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| *** 2.2.1 '''Adult'''
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| **** Parenteral regimen | |
| ***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
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| ***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
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| ***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
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| **** Oral regimen
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| ***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
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| ***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
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| ***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
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| ***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
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| ***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
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| ***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
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| *** 2.2.2 '''Pediatric'''
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| **** Parenteral regimen
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| ***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
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| ***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
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| ***** Alternative regimen (2): [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
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| **** Oral regimen
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| ***** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
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| ***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
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| ***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
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| ***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
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| ***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
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| ***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
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| ==References== | | ==References== |
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| {{WH}} | | {{WH}} |
| {{WS}} | | {{WS}} |
| [[Category: (name of the system)]]
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| <nowiki>{Glucose-6-phosphate dehydrogenase deficiency}}</nowiki>
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| {{CMG}}; '''Associate Editor(s)-In-Chief:''' [[Priyamvada Singh|Priyamvada Singh, M.D.]] [mailto:psingh13579@gmail.com]
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| Please help WikiDoc by adding content here. It's easy! Click [[Help:How to Edit a Page|here]] to learn about editing.
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| ==Overview==
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| '''Glucose-6-phosphate dehydrogenase (G6PD) deficiency''' is an [[Sex-linked|X-linked recessive]] [[hereditary disease]] featuring abnormally low levels of the [[G6PD]] enzyme, which plays an important role in [[red blood cell]] function. Individuals with the disease may exhibit non-immune [[hemolytic anemia]] in response to a number of causes. It is closely linked to '''[[favism]]''', a disorder characterized by a hemolytic reaction to consumption of [[Vicia faba|broad bean]]s, with a name derived from the [[Italian language|Italian]] name of the broad bean (''fava''). Sometimes the name, [[favism]], is alternatively used to refer to the enzyme
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| deficiency as a whole.
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| ==Treatment==
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| * In the acute phase of hemolysis, [[blood transfusion]]s might be necessary. Blood transfusion is an important symptomatic measure, as the transfused red cells are generally not G6PD deficient.
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| * [[Dialysis]] in [[acute renal failure]]
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| * Some patients benefit from removal of the [[spleen]] ([[splenectomy]]), as this is an important site of [[red blood cell]] destruction. * [[Folic acid]] should be used in any disorder featuring a high [[red blood cell]] turnover.
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| * Although [[vitamin E]] and [[selenium]] have antioxidant properties, their use does not decrease the severity of [[G6PD deficiency]].
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| ====Contraindicated medications====
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| {{MedCondContrAbs
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| |MedCond = Glucose-6-phosphate dehydrogenase (G6PD) deficiency |Pegloticase}}
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| ==References==
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| {{reflist|2}}
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| [[Category:Disease]]
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| [[Category:Hematology]]
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