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Biomedical subjects

S Snyderman

Publications and source records attributed to S Snyderman.

3 recordsLinked to original sources

Familial congenital short small bowel with associated defects. A long-term survival.

In 1974, Royer et al. described a familial syndrome consisting of a short and sluggish small bowel, malrotation of the gut, and pyloric stenosis. These authors stressed the uniformly fatal outcome of their four cases, as well as other possibly unrecognized cases previously described in the literature. The present report deals with two more familial cases, of which one represents a long-term survivor of the syndrome. The intensive work of maintaining nutrition, controlling infection, and managing the complications of associated defects are described.

Abnormalities, Multiple↗

An immunodeficient child with inflammatory bowel disease: involvement of cyclic nucleotides and effects of lithium.

A 3-year-old male with inflammatory bowel disease and hypogammaglobulinemia was found to have decreased T lymphocyte function. His serum was shown to depress normal T cell proliferative responses to phytohemagglutinin. Incorporation of lithium chloride to in vitro cultures enhanced autologous lymphocyte responses to phytohemagglutinin. Since lithium acts by inhibiting cAMP production, the child's lymphocytes were postulated to have increased levels of cAMP. Both lymphocytes and serum were shown to contain elevated levels of cAMP. In vivo therapy with lithium citrate was initiated and enhanced T cell numbers and function were observed concomitantly. Serum cAMP was also reduced to normal levels. The patient showed initially marked clinical improvement as assessed by mood, weight gain, and diminution of diarrhea. This clinical improvement was unfortunately not sustained despite the continued improvement in immune parameters and cAMP levels.

Agammaglobulinemia↗