A Case of Neonatal McCune‐Albright Syndrome with Cushing Syndrome and Hyperthyroidism
- 1 October 1991
- journal article
- case report
- Published by Wiley in Acta Paediatrica
- Vol. 80 (10) , 984-987
- https://doi.org/10.1111/j.1651-2227.1991.tb11769.x
Abstract
We describe a female newborn infant with McCune‐Albright syndrome. In addition to the cutaneous pigmentation, she had apparent manifestations of hyperthyroidism and Cushing syndrome since birth. X‐ray examinations showed many scattered lucencies in multiple bones. Endocrinological findings were as follows: serum T4 276 nmol/l; free T4 125 pmol/l; TSH 2210 nmol/l; plasma ACTH < 10 pg/ml; urinary free cortisol 865 nmol/day; estradiol 0.36 nmol/l. Regardless of treatment with antithyroid drugs and an inhibitor of 3β‐hydroxysteroid dehydrogenase, the patient died of cardiac failure at the age of 4 months. Autopsy findings included a follicle cyst in the right ovary and multinodular hyperplasia in the thyroid and both adrenals. To our knowledge such a severe neonatal form of McCune‐Albright syndrome has not been described in the literature.Keywords
This publication has 9 references indexed in Scilit:
- McCune-Albright SyndromePublished by American Medical Association (AMA) ,1986
- McCune-Albright syndrome. Long-term follow-upPublished by American Medical Association (AMA) ,1986
- The McCune-Albright syndromeActa Endocrinologica, 1986
- The McCune‐Albright syndrome: a lethal gene surviving by mosaicismClinical Genetics, 1986
- Fibrous dysplasia of boneThe Journal of Pediatrics, 1977
- Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancyThe Journal of Pediatrics, 1975
- Fibrous Dysplasia of Bone and Primary HyperparathyroidismAnnals of Internal Medicine, 1972
- McCune-Albright's syndrome following adrenalectomy for Cushing's syndrome in infancyThe Journal of Pediatrics, 1968
- Polyostotic Fibrous Dysplasia And AcromegalyArchives of internal medicine (1960), 1964