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J Israël

Publications and source records attributed to J Israël.

5 recordsLinked to original sources

[Congenital generalized cutis laxa].

Congenital cutis laxa is a rare disorder of the elastic tissue in which lax skin gives a premature senile appearance. We report a new case of this disease associated with craniosynostosis. In the literature, the genetics of cutis laxa are not clear. Fleischmajer and Matus (18) considers an inherited autosomal recessive or an incomplete autosomal dominant trait, Mehregan (33) reports an autosomal recessive mode of transmission and Byers (10) an X-linked form. Among these cases, the most serious visceral involvement is the development of pulmonary emphysema. However, in cutis laxa with development retardation, variety of minor injury of the skeleton, the prognosis seems appears to be better.

Cutis Laxa↗

[The use of new immunoglobulin preparation, enriched in IgA and IgM (IgGAM)].

The clinical results obtained with fraction IgGAM are reported. Different types of antibody deficiency syndromes have successfully been treated : 8 cases of Bruton-type agammaglobulinemia. In one of these case a tenacious Pseudomonas infection cleared off during the treatment. Two cases of non sex-linked familial agammaglobulinemia. Three cases of isolated IgM deficiency. Five cases of isolated IgA deficiency. Five cases with Soothill type IgA deficiency associated with high IgE levels. Five cases of septicemia of the new-born. Three cases with acquired agammaglobulinemia and in the premature infant (5 cases). No side-effects nor appearance of anti-IgA antibodies have been observed.

Adult↗