An interesting case report (LA Beste et al. NEJM 2016; 374: 73-8) reviews the presentation of a previously healthy 54 year old with ascites. He initially indicated that he was taking 100 IU per day of vitamin A (his current dose), but later on directed questioning admitted that he had averaged 98,500 IU/day for prior 6 months.
The clinical-problem solving case reviews useful pointers about portal hypertension and in particularly noncirrhotic portal hypertension. Vitamin A is a rare cause of noncirrhotic portal hypertension.
Other causes of noncirrhotic portal hypertension:
- Prehepatic level: portal vein or splenic vein thrombosis, splanchnic arteriovenous malformation
- Intrahepatic level: hepatic vasculitis, HIV infection, infiltrative disease, and medications
- Posthepatic: Budd-Chiari syndrome, IVC obstruction, restrictive cardiac disease.
- Worldwide, schistosomiasis is the most common reason.
- When other causes have been excluded, idiopathic noncirrhotic portal hypertension may be diagnosed, “especially in patients with chronic infection, thrombophilia, and immunologic conditions such as SLE.” In one series of 69 patients, the diagnosis of idiopathic noncirrhotic portal hypertension was delayed for more than a year in 25% of cases and 7% received an erroneous diagnosis of cryptogenic cirrhosis.
- When ascites is due to cirrhosis, other signs of liver disease are typically present, including jaundice and laboratory findings (low albumin, coagulopathy, hyperbilirubinemia) as well as absence of cirrhosis on biopsy.
- Serum retinol levels poorly reflect total body stores of vitamin A (& was normal in this patient)
- Vitamin A supplementation in appropriate doses can prevent blindness in areas where food stores are not secure. But, consuming excessive doses can lead to being a case report.
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