November 1978

Microphallus, Growth Hormone Deficiency, and Hypoglycemia in Russell-Silver Syndrome

Author Affiliations

Department of Medical Genetics Children's Orthopedic Hospital and Medical Center 4800 Sand Point Way NE, Box 5371 Seattle, WA 98105

Am J Dis Child. 1978;132(11):1149. doi:10.1001/archpedi.1978.02120360105028

Sir.—We read with interest the letter of Sujansky and Riccardi (132:214, 1978) in response to the article by Marks and Bergeson (131:447-451, 1977) on the genital anomalies seen in Russell-Silver syndrome since we have followed up a very similar child for the last four years. The child was initially diagnosed as having Russell-Silver syndrome without asymmetry because of intrauterine growth retardation (birth weight at 36 weeks' gestation, 1,049 g; birth length, 36 cm), clinodactyly, partial soft-tissue syndactyly, microphallus (at 17 months of age, his penis measured 1.1 cm; no hypospadias was present), cryptorchidism, and a few episodes of poorly recorded hypoglycemia (Figure). However, at age 2 years and 9 months, Patient at 2 years of age. Note microphallus. endocrine evaluation disclosed that he had severe hypoglycemia with seizures, hypopituitarism including growth hormone deficiency, hypothyroidism, hypoadrenalism, and apparent hypogonadotropism (serum luteinizing hormone level, under 5 ImU/ml; follicle-stimulating hormone level, 6

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