Hepatic Involvement and Portal Hypertension Predict Mortality in Chronic Granulomatous Disease

Abstract

Background-Chronic granulomatous disease (CGD) is a rare genetic disorder, predisposing affected individuals to recurrent infectious complications and reduced survival. Liver involvement in CGD includes vascular abnormalities, which may lead to non-cirrhotic portal hypertension.

Chronic Granulomatous Disease

“…High rates of portal venopathy and nodular regenerative hyperplasia may contribute to portal hypertension, splenomegaly, and splenic sequestration (44). This latter point is noteworthy, because the decline in platelet count is linked to splenomegaly and is also a strong predictor of mortality in CGD (45). Chronic drug effects, liver enzyme elevations, and recurrent infections are obvious risks for liver dysfunction.…”

Hépatite auto-immune associée à une granulomatose septique chronique chez une fille de 2 ans

“…Le suivi des enfants atteints de la GSC associée à des manifestations hépatiques et, en particulier, à une hépatite auto-immune doit être régulier. L'évolution vers une cirrhose a été rapportée [9] avec développement d'une hypertension portale qui assombrit le pronostic et contribue à augmenter la morbidité et la mortalité [20].…”

Hematopoietic Stem Cells Therapeutic Applications

“…An increased alkaline phosphatase level, a history of liver abscesses, and a decrease in platelet count reflecting portal hypertension are adverse prognostic indicators (Feld et al, 2008). These patients might also be considered for early transplantation.…”