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
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]
AdultsStressful events-
Partial enzyme deficiency-
InfectionUrinary tract infections (urease-producing organism, such as proteus mirabilis)
Salicylates such as aspirin
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
MiscellaneousTransient hyperammonemia of the newborn asphyxia
Reye syndrome
Lactic acidosis