[Recessive osteopetrosis. Identification of a form of medium severity].

Background: Several distinct forms of osteopetrosis have been identified. Some of the autosomally recessive inherited forms are benign, much like the autosomal dominant form. Others are more malignant.

[Hemiballism. Description of a clinical case in childhood].

Biballism is an infrequent hyperkinetic disorder characterized by involuntary, intermittent, violent, uncontrollable contractions of the proximal muscles of the limbs. Biballism is classically ascribed to a lesion in the controlateral subthalamic nucleus or its connections but other causes have been reported. These include infections (bacterial, viral parasitic), cerebrovascular lesions, tumors, toxics, and systemic disease (systemic lupus erythematosus).

[Budd-Chiari syndrome in children. Apropos of 7 cases].

Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients.

[Asphyxiating thoracic dysplasia associated with hepatic ductal hypoplasia, agenesis of the corpus callosum and Dandy-Walker syndrome].

The authors report on a case of a newborn with asphyxiating thoracic dysplasia who died 36 h after birth. This chondrodysplasia was associated with hepatic ductular hypoplasia, agenesis of the corpus callosum and Dandy-Walker malformation. To our knowledge, such an association has not previously been reported in the literature.

[Transient dilatation of intra- and extra-hepatic bile ducts. A case].

A case of transient dilatation of intra and extrahepatic bile ducts in the course of an infectious disease is described in a three-year-old girl. The ultrasonic bile duct abnormalities disappeared after three months. The mechanism involved remains uncertain; the hypothesis of a transient inflammatory obstruction is evoked.