DDSEP® 8 Quick Quiz

March 2017 Quiz 2

Publish date: March 1, 2017

Q2: A 52-year-old man with history of recurrent variceal bleeding presents for evaluation. He has an HIV infection that is controlled, with undetectable virus and CD4 count of 423 cells/mcL. He has no known underlying liver disease. He is currently on etravirine, emtricitabine, and tenofovir. He has previously taken didanosine. His physical exam is unremarkable and his laboratory data reveals a normal CBC, normal INR, and normal liver enzymes. Testing for hepatitis B and C and autoimmune liver disease, as well as iron overload and other etiologies of chronic liver disease are all negative. Ultrasound of the abdomen notes a normal-appearing liver and patent portal and hepatic veins. A liver biopsy demonstrates mildly dilated portal veins and mild fibrosis of the portal venous walls. There is no evidence of cirrhosis on the liver biopsy.

Which of the following statements is true regarding noncirrhotic portal hypertension in this patient?

Clinical presentation with ascites is more common than variceal bleeding.

A history of didanosine use is consistent with his clinical presentation.

Presentation with normal liver function is atypical in this setting.

The underlying pathology is related to progressive sinusoidal fibrosis.

This patient is at low risk for developing portal vein thrombosis.

Q2: Answer: B

This patient, with no imaging or laboratory findings to suggest cirrhosis, most likely has noncirrhotic portal hypertension (NCPH). There is now a well-described association between HIV and NCPH with the prevalence of NCPH in HIV estimated to be –0.5% to 1%. Patients typically are unaware of any underlying liver disease until presentation with variceal bleeding. Variceal bleeding is a much more common manifestation of NCPH than ascites. Clinical presentation with normal hepatic enzymes and normal hepatic synthetic function is a very typical feature in these patients. Although the exact etiology is not fully understood, NCPH in HIV is likely related to HAART, particularly didanosine use, hypercoagulability, microbial translocation from the gut, and direct effects of HIV. NCPH is a presinusoidal lesion, and liver biopsy may reveal paucity of portal vasculature and focal obliteration of small portal veins. Portal vein thrombosis in patients with HIV and NCPH is common and has been observed in 25%-75% of patients.

Reference

1. Vispo E., Morello J., Rodriguez-Novoa S., Soriano V. Noncirrhotic portal hypertension in HIV infection. Curr Opin Infect Dis. 2011;24:12-8.
2. Khanna R., Sarin S.K. Noncirrhotic portal hypertension – Diagnosis and management. J Hepatol. 2014;60:421-41.

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