Corticoresistant nephrotic syndrome associated with T-cell deficiency in two sisters

The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes an...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1983-01, Vol.71 (1), p.93-96
Main Authors: Tabin, R, Guignard, J P, Gautier, E, Dubrit, M, Jeannet, M, Golaz, J, Moretta, L
Format: Article
Language:English
Subjects:
Child
Child, Preschool
Consanguinity
Female
Glomerulosclerosis, Focal Segmental - genetics
Glomerulosclerosis, Focal Segmental - immunology
Humans
Immunologic Deficiency Syndromes - genetics
Leukocyte Count
Lymphopenia - genetics
Nephrotic Syndrome - drug therapy
Nephrotic Syndrome - genetics
Nephrotic Syndrome - immunology
Prednisone - therapeutic use
Rosette Formation
T-Lymphocytes - immunology
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Summary:The coexistence in two sisters, born to related parents, of a corticoresistant nephrotic syndrome, lymphopenia, an immune deficit, short stature, and photophobia is described. The immune deficit is mainly cellular; studies of lymphocyte markers demonstrate a pronounced deficiency of T lymphocytes and Fc-mu receptor-bearing cells. It is suggested that a thorough examination of number and function of T cells should be performed in patients with a familial corticoresistant nephrotic syndrome and recurrent infectious episodes before considering immunosuppressive treatment.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.71.1.93