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Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Presenting as ACTH-Independent Cushing's Syndrome: A Case Report

Laura Buceta Cuéllar, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Raquel Nebot Martín, María del Carmen de Mingo Alemany, Sabrina Kalbouza, Sara León Cariñena, Enrique Cuñat Navarro, Francisca Moreno Macián, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, University Hospital of Vinalopó, Tonico Sansano Mora 14, 03293, Elche (Alicante). Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain (+2 others)
2022 Journal of Pediatrics Research Reviews & Reports  

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Cushing's syndrome diagnosis in childhood is a challenge, due to its atypical presentation. Primary Pigmented Nodular Adrenocortical Disease (PPNAD) is a rare cause of ACTH- independent Cushing's Disease. We present the case of a four-year-old patient evaluated for Cushing's Syndrome due to a rapid onset of obesity, pubarche and hirsutism with a characteristic phenotypic appearance. Initial biochemical examinations were compatible with ACTH- independent Cushing's Syndrome, but imaging studies
more » ... re confusing (pituitary MRI detected an image compatible with adenoma). The study was completed with tests which confirmed independence from ACTH and finally the patient underwent a bilateral adrenalectomy. The anatomopathological findings of adrenal glands confirmed the diagnosis. Replacement hormonal treatment was applied, leading to the progressive recovery of a normal phenotype
doi:10.47363/jprrr/2022(4)136 fatcat:uziwdha6zjhk7nadiof3opcnvi
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Laura Buceta Cuéllar, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Raquel Nebot Martín, María del Carmen de Mingo Alemany, Sabrina Kalbouza, Sara León Cariñena, Enrique Cuñat Navarro, Francisca Moreno Macián, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, University Hospital of Vinalopó, Tonico Sansano Mora 14, 03293, Elche (Alicante). Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain, Pediatric Endocrinology Unit, University and Polytechnic Hospital La Fe, Fernando Abril Martorell 106, 46026, Valencia, Spain. "Primary Pigmented Nodular Adrenocortical Disease (PPNAD) Presenting as ACTH-Independent Cushing's Syndrome: A Case Report." Journal of Pediatrics Research Reviews & Reports (2022) 1-5