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April 1982

Robinow's Syndrome: Partial Primary Hypogonadism in Pubertal Boys, With Persistence of Micropenis

Author Affiliations

From the Division of Pediatric Endocrinology, Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore (Drs Lee, Migeon, and Brown), and the Division of Medical Genetics and Birth Defects, Department of Pediatrics, Wright State University School of Medicine, Dayton, Ohio (Dr Robinow).

Am J Dis Child. 1982;136(4):327-330. doi:10.1001/archpedi.1982.03970400045014

• We studied four patients with Robinow's syndrome. New findings included the following: (1) normal pubertal virilization with persistence of micropenis; (2) elevated basal serum follicle-stimulating hormone levels and a hyperresponse of serum luteinizing hormone to gonadorelin hydrochloride (Factrel) stimulation among postpubertal male patients, suggesting partial primary hypogonadism; (3) normal 5α-reductase and androgen receptor activity in genital skin fibroblasts; and (4) normal to borderline adult height.

(Am J Dis Child 1982;136:327-330)