Gastroenterology
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Case Reports
Transjugular intrahepatic portosystemic shunt improves oxygenation in hepatopulmonary syndrome.
Hepatopulmonary syndrome is a complication of chronic liver disease in which arterial hypoxemia results from abnormalities in pulmonary blood flow. Severe hypoxemia can lead to clinical deterioration and death. Although the etiology is unknown, portal hypertension seems to be an important factor in the development of hepatopulmonary syndrome. ⋯ We described a patient with hepatopulmonary syndrome who noted improvement in symptoms of dyspnea after the placement of a transjugular intrahepatic portosystemic shunt. Arterial oxygenation and calculated shunt fraction improved significantly during the follow-up period, and liver transplantation was subsequently performed without difficulty. Portal decompression using transjugular intrahepatic portosystemic shunt may represent a palliative therapy for hepatopulmonary syndrome in patients awaiting liver transplantation.
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Case Reports
Hyperammonemic coma due to parenteral nutrition in a woman with heterozygous ornithine transcarbamylase deficiency.
Ornithine transcarbamylase deficiency is an X-linked disorder of the urea cycle that can cause hyperammonemic encephalopathy in hemizygous males and heterozygous females. Affected females typically limit protein intake in their diet. This case report describes a 36-year-old woman with ulcerative colitis who went into hyperammonemic coma after administration of total parenteral nutrition. ⋯ Heterozygous ornithine transcarbamylase deficiency was diagnosed based on a positive allopurinol tolerance test result after elevated levels of plasma glutamine and low plasma citrulline were detected. The protein load associated with parenteral alimentation resulted in symptomatic expression of this partial enzyme deficiency in this unique case. Partial ornithine transcarbamylase deficiency must always be considered in adult women and men with hyperammonemia who have normal liver function test results.