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ACTA ENDOCRINOLOGICA (BUC)
The International Journal of Romanian Society of Endocrinology / Registered in 1938in ISI Thomson Master Journal List
Acta Endocrinologica(Bucharest) is live in PubMed Central


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Acta Endocrinologica (Buc)
Bavadiya G, Roy A, Sarkar KK, Shekhda KM, Chatterjee A, Shah C, Chakrabarty A
Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Presenting as Cushing Syndrome in a Child and Review of Literature
Acta Endo (Buc) 2020, 16 (3): 362-365doi: 10.4183/aeb.2020.362
Cushing syndrome in the paediatric age group is
very difficult to diagnose due to atypical presenting features
in children. Primary pigmented nodular adrenocortical
disease (PPNAD) is a rare cause of ACTH-independent
Cushing syndrome in children and it has characteristic gross
and microscopic pathologic features. We report a case of
PPNAD in a 16-year-old boy who was evaluated in our
hospital with chief complaints of poor height velocity and
rapid weight gain for 2-3 years before presentation. Proper
evaluation showed ACTH-independent Cushing syndrome
with normal imaging. Total bilateral adrenalectomy was
performed followed by hormones replacement. 6 months
after surgery, significant acceleration of height velocity
was noticed. Patient also lost body weight and developed
secondary sexual characteristics.
Keywords: Cushing syndrome, Primary Pigmented Nodular Adrenocortical Disease, Height velocity
Correspondence: Kalyan Mansukhbhai Shekhda D.N.B. (Medicine), M.R.C.P. (UK), Southend University Hospital NHS Foundation
Trust, Diabetes and Endocrinology, Prittlewell chase, Westcliff-on-Sea, SS0 0RY, United Kingdom of Great Britain and Northern Ireland, E-mail: kalokly@gmail.com