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November 1977

Adrenal Calcification in Beckwith-Wiedemann Syndrome

Author Affiliations

Department of Pediatrics Okayama National Hospital, 2-13-1 Okayama City, Japan

Am J Dis Child. 1977;131(11):1296-1297. doi:10.1001/archpedi.1977.02120240114025

The features of the Beckwith-Wiedemann syndrome are characterized by exomphalos, macroglossia, macrosomia, visceromegaly, ear lobe anomaly, nevus flammeus, hemihypertrophy, and other abnormalities. Adrenal calcifications can be seen in a variety of pediatric conditions. More than 171 children with the Beckwith-Wiedemann syndrome have been described in the pediatric literature, but none of these patients have been reported as having adrenal calcification.1 The present communication describes two girls with the Beckwith-Wiedemann syndrome who were demonstrated to have adrenal calcifications at ages 7 and 4 years, respectively.

Report of Cases.—Case 1.—This 7-day-old girl was hospitalized because of macrosomia (4.8 kg), macroglossia, and a history of poor feedings. She had been born at term, following a traction delivery. On admission macrosomia, macroglossia, peculiar facies, relative microcephaly, navel hernia, and visceromegaly were striking. Serum bilirubin level on the seventh day of life was 18.8 mg/100 ml. A diagnosis of cretinism was suspected