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Biomedical subjects

E Gratz

Publications and source records attributed to E Gratz.

11 recordsLinked to original sources

Septo-optic dysplasia in two siblings.

We treated two siblings, a sister and brother, who had features of septo-optic dysplasia, including bilateral optic nerve hypoplasia, absent septum pellucidum, and partial pituitary insufficiency. Additionally, midline central nervous system abnormalities of the corpus callosum and cerebellum were shown. The familial occurrence of this disorder raises the possibility of autosomal-recessive inheritance.

Abnormalities, Multiple↗

Clinical correlation with anti-peripheral-nerve myelin antibodies in Guillain-Barré syndrome.

Anti-peripheral-nerve myelin antibodies (anti-PNM Ab) can be detected in the serum of all patients with acute-phase Guillain-Barré syndrome (GBS) thus far tested. Correlation of the titer of this antibody with the clinical course would help to establish a role for the humoral immune system in the pathophysiology of GBS. In this study, anti-PNM Ab levels were measured in serial serum samples of 7 patients with GBS with an assay that detects antibodies bound to peripheral nerve myelin antigens by fixation of the first component of complement. Although the titers of anti-PNM Ab detected in these patients varied between 0 and 256 U/ml, the antibody titer was always highest on admission (35 to 256 U/ml) and rapidly declined during a one-to-three-week period. Disappearance of antibodies or very low levels of them correlated with cessation of progression and considerable clinical improvement as documented by increased pulmonary vital capacity and muscular strength. Low but measurable antibody titers (5 to 12 U/ml) were frequently found up to four months following the acute neurological deficit. The close temporal relationship between anti-PNM Ab titer and the clinical course in GBS suggests that antibody most likely participates through complement activation in peripheral nerve demyelination.

Adolescent↗

Weber--Christian syndrome in infancy.

A review of Weber-Christian syndrome in infancy is presented along with the report of two cases. Both infants had low serum properdin levels, and one patient showed remissions of his disease when he was T-lymphocytopenic. This suggests that the T-lymphocyte may have an important role in the pathogenesis of Weber--Christian syndrome.

Adipose Tissue↗