35 research outputs found
Hepatic abnormalities in patients with chronic granulomatous disease
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. CONCLUSION:
Liver enzyme abnormalities occur frequently in patients with CGD. In addition to
liver abscesses and granulomata, drug hepatotoxicity is likely underappreciated.
Vascular lesions such as venopathy and--to a lesser extent--NRH are common. The
cause and clinical consequences of venopathy await prospective evaluation
Neutrophil Chemotaxis Defect in IgA Deficiency Evaluated by Migration Agarose Method
The chemotactic and random mobility functions of twelve selectively IgA-deficient patients were evaluated by a method using agarose gel. A severe polymorphonuclear cellular chemotactic defect was found in ten out of twelve patients, but only five of them also showed a marked associated impairment of random locomotory function. Futhermore, in one subject, levamisole therapy resulted in a dramatic improvement of both chemotactic and random mobility functions. These results are discussed in the paper with respect to the possible pathogenetic implications