PEDIATRICS Vol. 35 No. 1 January 1965, pp. 70-75
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CONGENITAL HYPOPLASIA OF THE ADRENAL GLANDS

Report of Two Cases in Sisters with Necropsy

Anadil Roselli M.D.1 and Luiz Torres Barbosa M.D.2

1 Pathologic Anatomy Service; Hospital Servidores do Estado (HSE), Rio de Janeiro, Brazil
2 The Pediatric Service; Hospital Servidores do Estado (HSE), Rio de Janeiro, Brazil

The cases of two sisters with extreme bilateral adrenal hypoplasia, verified at necropsy, have been presented. Two other siblings died in infancy with symptoms which make it very likely that they also had adrenal hypoplasia. A review of the medical literature revealed 23 cases in which necropsy had been performed. These included 15 males and 8 females as well as one set of twins and 3 siblings. Although the incidence of reported cases of adrenal hypoplasia is low, the authors suggest that many cases may have been missed in the past. The clinical picture and possible etiology of this condition have been discussed.

Submitted on August 16, 1963
Accepted on June 16, 1964