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 (CGD) is a rare congenital disorder characterized by repeated bacterial and fungal infections. Aside from a high incidence of liver abscess, little is known about hepatic involvement in CGD. The aim of this study was to describe the spectrum of liver abnormalities seen in CGD. The charts of 194 patients with CGD followed at the NIH were reviewed, with a focus on liver abnormalities. Liver enzyme elevations occurred on at least one occasion in 73% of patients during a mean of 8.9 years of follow-up. ALT elevations were generally transient. Although transient alkaline phosphatase (ALP) elevations were also common, persistent ALP elevations lasting up to 17.6 years were seen in 25% of patients. Liver abscess occurred in 35% of patients. Drug-induced hepatotoxicity was documented in 15% of patients but likely occurred more frequently. Hepatomegaly was found in 34% and splenomegaly in 56% of patients. Liver histology showed granulomata in 75% and lobular hepatitis in 90% of specimens. Venopathy of the portal vein was common (80%) and associated with splenomegaly. Venopathy of the central vein was also common (63%) and was associated with the number of abscess episodes. Nodular regenerative hyperplasia (NRH) was seen in 9 patients, including 6 of 12 autopsy specimens. C hronic granulomatous disease (CGD) is a rare inherited disorder occurring in 1 in 250,000 individuals. 1 The underlying defect is a loss or inactivation of a component of the reduced NADPH complex, resulting in a defective oxygen metabolic burst with inadequate production of superoxide and peroxide and, perhaps most importantly, an inability of phagocytic cells to kill certain bacteria and fungi. 2 These defects predispose affected individuals to recurrent infectious complications and significantly reduced long-term survival. 3 Four principal CGD genotypes have been described, with important differences in phenotypic expression. Because the gp91 phox genotype is X-linked and accounts for 70% of patients in large cohort studies, CGD has a significant male predominance. 4 The other described genotypes, p22 phox , p47 phox , and p67 phox , are autosomal recessive. 4 In addition to being more common, X-linked CGD (gp91 phox ) portends a worse prognosis with a higher annual mortality compared with the other genotypes. 1 The reduced NADPH is composed of cell membrane (p22 phox , gp91 phox ) or cytoplasmic moieties (p47 phox , p67 phox ). 1 Although pulmonary infections predominate in CGD, with 79% of a 368-patient registry reporting a history of pneumonia, other sites are also commonly affected, including suppurative adenitis (53%), subcutaneous abscess From the

SUMMARY ObjectiveTo determine whether antiplatelet agents are associated with endoscopic sphincterotomy-related haemorrhage as few well-controlled data exist on this controversial issue. MethodsA case-control study in a tertiary care setting included cases with bleeding following endoscopic sphincterotomy, matched with 2-3 controls selected according to age AE 15 years, sex, and procedural date AE 2 years. Cases and controls were compared for possible risk factors of postendoscopic sphincterotomy bleeding (presence of a coagulopathy and cholangitis). The main outcome measurement was the association between the use of antiplatelet medications and postendoscopic sphincterotomy bleeding after adjustment for possible confounding. ResultsThe 40 cases [mean age 68 AE 17 (s.d.) years, 50% female] and 86 controls [68 AE 16 years, 50% female] were comparable except for differences noted in International Normalized Ratio (INR) (>2 in four cases vs. two controls), and pre-endoscopic sphincterotomy cholangitis (45% vs. 20%). Amongst cases, 13% were on aspirin and 3% on clopidogrel; 17% of controls took aspirin, and 4% a non-steroidal anti-inflammatory drug. 53% of cases bled immediately; the remainder haemorrhaged at 2 AE 3 days. After adjustment for an elevated INR and cholangitis, exposure to antiplatelet agents was not significantly associated with procedure-related bleeding (odds ratio ¼ 0.41, 95% CI [ 0.13; 1.31]). ConclusionThis case-control study provides controlled data suggesting that antiplatelet agents do not significantly increase the risk of clinicallyimportant bleeding related to endoscopic sphincterotomy. The low prevalences of non-steroidal anti-inflammatory drugs and clopidogrel use limit any definite conclusion on their elective use before endoscopic sphincterotomy.