[Successful treatment of chronic active hepatitis with high-dose intravenous immunoglobulins in agammaglobulinemia].
BACKGROUND
Rapid progression of chronic active hepatitis can occur in patients with hypogammaglobulinemia. This report describes the successful use of i.v. immunoglobulins to treat chronic hepatitis in a child with agammaglobulinemia.


METHODS
A 17 month-old boy was admitted because he had suffered from recurrent infections since the age of 6 months. His family history was normal. Clinical and laboratory investigations showed hepatomegaly, agammaglobulinemia with absence of IgG, IgA, IgM and IgE, absence of beta cells, normal T cells, normal T cell proliferation and normal levels of complement, elevated ALAT (70 and 200 IU/ml) and ASAT (60 and 188 IU/ml). Liver biopsy showed typical features of chronic active hepatitis. The cause of this hepatitis (B and C virus, EBV, autoimmune markers) was not found. The patient was first given gammaglobulins (80 mg/kg) every week, subcutaneously, for 9 weeks, which did not change his transaminasemia. A second course of gamma-globulins, 400 mg/kg every 3 weeks, intravenously, for 6 months, resulted in a transient normalization of transaminases for 3 months. Definitive normalization was only obtained when the patient was given i.v. gammaglobulins (400 mg/kg/week) which gave a residual level of blood IgG of 10 g/l. This apparent cessation of hepatitis activity was confirmed by a second liver biopsy. The patient is now given i.v. gammaglobulins, twice a month, producing a residual blood IgG concentration of 5 g/l.


CONCLUSIONS
The activity of this chronic hepatitis is closely correlated with the residual blood IgG concentration. Gammaglobulins could help neutralize virus extra-cellularly, although the viral origin of this hepatitis has not been-demonstrated.
Bejaoui
M
M
Service de pédiatrie, hôpital Charles-Nicolle, Tunis, Tunisie.
Gharbi
A
A
Bouguerra
A
A
Dellagi
K
K
Lakhoua
R
R
D000361:Agammaglobulinemia / Q000188:drug therapy*; D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D004305:Dose-Response Relationship, Drug; D006521:Hepatitis, Chronic / Q000097:blood / Q000473:pathology / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007223:Infant; D008297:Male
D016756:Immunoglobulins, Intravenous; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase
Guérison d'une hépatite chronique active par immunoglobulines intraveineuses à fortes doses au cours d'une agammaglobulinémie.