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August 1967

Cytomegalic Adrenal Hypoplasia in a 4½-Year-Old Boy

Author Affiliations

Sydney, Australia
From the Department of Pediatric Pathology, Prince of Wales Hospital (Dr. Baker), the Department of Pediatrics, School of Pediatrics, University of New South Wales (Dr. Wise), Randwick, New South Wales, Australia, and Ryde, New South Wales, Australia (Dr. Mezger).

Am J Dis Child. 1967;114(2):180-185. doi:10.1001/archpedi.1967.02090230110014

KARENYI1 (1961) described two types of adrenal hypoplasia in infancy. He called one the anencephalic or secondary type because of the morphological similarity to the hypoplastic adrenals seen with anencephaly, a "miniature adult" type of adrenal. He called the other the primary or cytomegalic type because of the presence of large cortical cells with abnormal nuclear and cytoplasmic staining. He found in the literature papers describing five deaths from adrenal hypofunction in which the cytomegalic type of adrenal histology was described.2-6 All these infants develop symptoms before the age of 1 month, and the longest survival time was six months. One child had a sibling in whom adrenal hypofunction was proved and this child had survived with replacement therapy. The descriptions of the adrenal histology were very similar in all cases, the characteristics being groups of large eosinophilic cortical cells with pyknotic or poorly hematoxyphilic nuclei. The deeper