[Histiocytosis X and sclerosing cholangitis].
The case of a seven-year-old with multifocal (type II) Langheran's cell histiocytosis since the age of two years is reported. Despite therapy, biliary cirrhosis with portal hypertension developed gradually. Histologic studies of liver biopsy specimens, computed tomography, and transhepatic cholangiography disclosed dilatation of the bile ducts, suggesting sclerosing cholangitis, a specific complication of Langherans' cell histiocytosis which precipitates the development of biliary cirrhosis.
Soua
H
H
Service de Pédiatrie, Hôpital de Mahdia, Tunisie.
Pousse
H
H
Ladeb
F
F
Korbi
S
S
Ayadi
A
A
Boussoffara
R
R
Belkhir
Y
Y
Descos
B
B
Sfar
M T
MT
Harbi
A
A
D001706:Biopsy; D002648:Child; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D014057:Tomography, X-Ray Computed
Histiocytose X et cholangite sclérosante.