Case Report
Copyright ©The Author(s) 2015.
World J Hepatol. May 8, 2015; 7(7): 1007-1011
Published online May 8, 2015. doi: 10.4254/wjh.v7.i7.1007
Table 1 Laboratory investigations and imaging at time of hospitalization
Laboratory investigation/imagingValues
BMPWithin normal limits (Cr 1.0 mg/dL)
CBCWithin normal limits (platelets 158 K/μL)
Iron panelWithin normal limits
Hepatic panelAST 30 U/L
ALT 9 U/L
Alkaline phosphatase 52 U/L
Total Bilirubin 0.9 mg/dL
Urine orotic acid1.4 mmol/mol of creatinine
Ammonia level155 mcg/dL
Carnitine level, zinc, manganese, vitamin B12, vitamin A, vitamin DWithin normal limits
Abdominal ultrasoundNo evidence of liver disease or cirrhosis
MRI brainNo acute or chronic intracranial abnormalities
Table 2 Etiology of hyperammonemia of non-hepatic origin[21]
AgeCategoryExamples
AdultsStressful events-
Partial enzyme deficiency-
InfectionUrinary tract infections (urease-producing organism, such as proteus mirabilis)
PediatricsMedicationsValproate
Topiramate
Salicylates such as aspirin
Acetazolamide
Zonisamide
High-dose chemotherapy (5-fluorouracil)
InfectionUrinary tract infections (urease-producing organism, such as proteus mirabilis)
Organic acidemiasIsovalericacidemia, propionic acidemia, methylmalonicacidemia, glutaricacidemia type II, multiple carboxylase deficiency, beta-ketothiolase deficiency
Congenital lactic acidosisPyruvate dehydrogenase deficiency
Pyruvate carboxylase deficiency
Mitochondrial disorders
Fatty acid oxidation defectsAcyl CoA dehydrogenase deficiency
Systemic carnitine deficiency
Dibasic aminoacid transport defectsLysinuric protein intolerance
Hyperammonemia-hyperornithinemia-homocitrullinuria
MiscellaneousTransient hyperammonemia of the newborn asphyxia
Reye syndrome
Lactic acidosis