Splenomegaly and Variceal Bleeding in a Ten-year-old HIV-infected Girl With Noncirrhotic Portal Hypertension

Abstract

Noncirrhotic portal hypertension is an uncommon liver disease of unknown origin, increasingly described in HIV-infected adults. Prolonged antiretroviral exposure, in particular to didanosine, and thrombophilic predisposition have been suggested as potential pathogenic factors. Data are limited in children. We describe a 10-year-old HIV-infected girl with noncirrhotic portal hypertension who presented with progressive spleen enlargement and variceal bleeding.

Paediatric European Network for Treatment of AIDS (PENTA) guidelines for treatment of paediatric HIV‐1 infection 2015: optimizing health in preparation for adult life

“…Noncirrhotic portal hypertension has been reported as a rare complication of exposure to ddI in adults 126, 127, 128 and children 129, 130, may be associated with a genetic predisposition 126 and can become evident after ddI has been discontinued. PENTA does not support the use of d4T or ddI in first‐ or second‐line ART.…”

“…For infants with first‐line failure, ddI may be substituted for TDF; however, recent case reports of noncirrhotic portal hypertension in HIV‐infected adolescents following prolonged exposure to ddI are of concern 126, 127, 128, 129, 130. Therefore, ddI exposure should be kept to a minimum with substitution of an alternative agent at the earliest opportunity.…”

Noncirrhotic Portal Hypertension in Perinatally HIV-infected Adolescents Treated With Didanosine-containing Antiretroviral Regimens in Childhood

“…Recently, two casereports of perinatally HIV-infected adolescents, 1 female and 1 boy with prolonged exposure to didanosine were described, presenting with variceal bleeding based on NCPH. 12,13 Also in our series, the 6 adolescent patients were perinatally infected and had been treated with didanosine with a median of 5.5 years (range 4-10). Previously, didanosine has been suggested as a potential risk factor for the development of NCPH in adults, although an exact mechanism to explain the selective impact of this antiretroviral drug in NCPH is as yet unknown although endothelial damage upon prolonged exposure to NRTIs has been proposed.…”

“…Complications of portal hypertension, notably splenomegaly, esophageal varices (n=6) and ascites (n=4) developed during clinical follow-up at relatively young age (median 16 years, range[13][14][15][16][17][18][19][20]. In 2 of the patients these complications could be explained by the presence of extrahepatic portal vein thrombosis, whereas in the others the NCPH progressed in a short period to portal hypertension without evidence of any thrombosis.…”

“…5,9 Symptomatic noncirrhotic portal hypertension (NCPH), defined as the presence of portal hypertension in the absence of cirrhotic change 15 has to date been reported in around 140 HIVinfected adults and 2 HIV-infected children. [10][11][12][13][14] Early diagnosis is necessary to reduce the emergence of severe complication, such as variceal bleeding, portal thrombosis and ascites in relation to portal hypertension.…”