The Journal of Clinical Endocrinology & Metabolism Vol. 92, No. 3 754
Copyright © 2007 by The Endocrine Society
Calcified Adrenals Associated with Perinatal Adrenal Hemorrhage and Adrenal Insufficiency
Shira Perl,
Linda Kotz,
Meg Keil,
Nicholas J. Patronas and
Constantine A. Stratakis
Molecular and Clinical Hematology Branch (S.P.), National Institute of Diabetes, Digestive, and Kidney Disease; Section on Endocrinology and Genetics (L.K., M.K., C.A.S.), Developmental Endocrinology Branch, National Institute of Child Health and Human Development; Inter-Institute Endocrinology Training Program (S.P., C.A.S.), National Institute of Child Health and Human Development/National Institute of Diabetes, Digestive, and Kidney Disease; and Department of Diagnostic Radiology (N.J.P.), Warren Grant Magnuson Clinical Center, National Institutes of Health, Bethesda, Maryland 20892
Address all correspondence and requests for reprints to: Constantine A. Stratakis, M.D., D.Sc., Section on Endocrinology and Genetics, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10 (CRC-East), Room 1-3330, 10 Center Drive MSC 1103, Bethesda, Maryland 20892. E-mail: stratakc{at}mail.nih.gov.
A 6-yr-old girl was referred for evaluation of adrenal insufficiency.
She was born via a difficult and prolonged labor after a 40-wk pregnancy complicated by gestational diabetes. She was then admitted to the neonatal intensive care unit for hypoglycemia; by the sixth day, hyperkalemia and hyponatremia were noted, and adrenal insufficiency was suspected. An abdominal ultrasound demonstrated bilateral adrenal hemorrhage, but she was not consistently treated with hormone replacement.
In our institute, cortisol levels remained undetectable in response to ACTH, and plasma renin activity was elevated. Computed tomography of the abdomen demonstrated grossly calcified adrenal glands (Fig. 1
). She was treated with proper hormonal replacement.
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FIG. 1. Bilateral adrenal calcifications on axial (A) and coronal (B) images of a computed tomography scan of the patient with no associated masses or other lesions. The arrows on both panels indicate the location of the right and left adrenal glands, respectively. P, Posterior.
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Adrenal hemorrhage, a well-recognized obstetrical complication of the newborn (1, 2), is an increasingly rare cause of adrenal insufficiency due to the improvements in care during labor and delivery. The last case-series of adrenal hemorrhage (eight in total, three of which were bilateral) was reported in 1973 (3). Risk factors included large birth weight, hypoxia, septicemia, coagulation defects, and thromboembolism. The adrenal glands became grossly calcified in all seven surviving patients, confirming an observation that dates back to 1935 (4). Other causes of adrenal calcifications include tuberculosis, Addison’s, Wolman’s (familial xanthomatosis), and Niemann-Pick diseases, and masses including hemorrhagic and teratomatous cysts, ganglioneuromas, pheochromocytomas, neuroblastomas, and adrenocortical cancer. These were excluded in our patient. Adrenal calcification may also be an incidental finding (5), although it is also attributed to perinatal adrenal hemorrhage.
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Footnotes
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The clinical investigations reported here were part of an intramural grant from the National Institute of Child Health and Human Development to Dr. C. A. Stratakis (grant no. 1 Z01 HD000642-04); the authors also wish to thank Dr. Samuel M. Freedman (Hollywood, FL) for referring this patient to their studies.
Disclosure Statement: The authors have nothing to disclose.
Received September 14, 2006.
Accepted December 6, 2006.
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References
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- Khuri FJ, Alton DJ, Hardy BE, Cook GT, Churchill BM 1980 Adrenal hemorrhage in neonates: report of 5 cases and review of the literature. J Urol 124:684–687[Medline]
- Kuhn J, Jewett T, Munschauer R 1971 The clinical and radiological features of massive neonatal adrenal hemorrhage. Radiology 99:647–652[Medline]
- Black J, Innes Smith D 1973 Natural history of adrenal hemorrhage in the newborn. Arch Dis Child 48:183–190[Abstract/Free Full Text]
- Snelling CE, Erb IH 1935 Hemorrhage and subsequent calcification of the suprarenal. J Pediatr 6:22–23
- Krill Jr CE, Ryan SP 1997 Adrenal calcification: an incidental finding or not? Pediatr Radiol 27:821–823[CrossRef][Medline]
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