eISSN: 2083-8441
ISSN: 2081-237X
Pediatric Endocrinology Diabetes and Metabolism
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3/2020
vol. 26
 
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abstract:
Case report

Cushing’s syndrome due to primary pigmented nodular adrenal disease in two brothers with Carney complex

Bhawna Attri
1
,
Anshita Aggarwal
1
,
Sahil Mattoo
1
,
Bindu Kulshreshtha
1

1.
Department of Endocrinology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr. Ram Manohar Lohia Hospital, New Delhi, Delhi, India
Pediatr Endocrinol Diabetes Metab 2020; 26 (3): 155–158
Online publish date: 2020/09/01
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Two brothers (19 and 18 years old, respectively) presented with weight gain and stunted growth since the age of 10 years. They had spotty skin pigmentation over the face along with florid features of Cushing’s syndrome with low bone density, renal calculi, dyslipidaemia, hypertension, and dilated cardiomyopathy. They underwent evaluation of the hypothalamic-pituitary-adrenals axis, which suggested ACTH-independent Cushing’s syndrome, and the abdominal CT imaging revealed normal adrenals. The diagnosis of Familial Cushing’s syndrome with primary pigmented nodular adrenal disease and Carney complex was made. Bilateral adrenalectomy was carried out in both of them with resolution of the features of hypercortisolaemia post-operatively.
keywords:

PPNAD, Carney complex, Cushing’s syndrome

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