ESPE2024 Poster Category 1 Adrenals and HPA Axis 3 (8 abstracts)
Non tumoral ACTH-Independent Cushing Syndrome in Pediatric Patients: A Retrospective Observational Cohort Study
Myriam Hassan 1 , Dinane Samara-Boustani 1 , Alix Besançon 1 , Anya Rothenbuhler 2 , Caroline Storey 3 , Graziella Pinto 1 , Athanasia Stoupa 1 , Marc Nicolino 4 , Laura Gonzalez-Briceno 1 , Adrien Nguyen Quoc 1 , Gaelle Vermillac 1 , Sibylle Rovani 1,5 , Isabelle Flechtner 1 , Caroline Thalassinos 1 , Yamina Dassa 1 , Maria Beatriz Arrom Branas 1 , Magali Viaud 1 , Jacques Beltrand 1,5 , Virginie Ribault 6 , Laetitia Martinerie 3,5 , Agnès Linglart 2,7 , Jerôme Bertherat 8,5 , Thomas Blanc 9,5 , Michel Polak 1,5 & Dulanjalee Kariyawasam 1,5
1Pediatric Endocrinology, Diabetology and Gynecology Department, Necker-Enfants Malades University Hospital, AP-HP, Paris, France. 2Pediatric Endocrinology and Diabetology Department, Kremlin-Bicêtre University Hospital, AP-HP, Paris, France. 3Pediatric Endocrinology and Diabetology Department, Robert Debré University Hospital, AP-HP, Paris, France. 4Pediatric Endocrinology, Diabetology and Inherited Metabolic Diseases Department, Lyon Civil Hospices, Lyon, France. 5Paris Cité University, Paris, France. 6Pediatric Endocrinology and Diabetology Department, Caen University Hospital, Caen, France. 7Bicetre Paris - Sud, Paris-Sud - Paris Saclay University, Paris, France. 8Endocrinology Department, Cochin University Hospital, AP-HP, Paris, France. 9Pediatric Surgery Department, Necker-Enfants Malades University Hospital, AP-HP, Paris, France
Purpose: ACTH-independent bilateral adrenal hyperplasia is a rare cause of pediatric Cushing's syndrome (CS). Our objective was to describe the features of pediatric ACTH-independent CS and to compare groups defined by etiology.
Methods: We conducted a retrospective observational study of patients aged 0–18 years at management in 1992–2022 for ACTH-independent CS in any of three Paris university pediatric hospitals. Etiological groups were compared. Among patients with McCune-Albright syndrome (MCAS) or Carney complex (CNC), we analyzed the groups with vs. without CS.
Results: Of the 15 patients with CS, seven had MCAS, seven primary pigmented nodular adrenocortical disease (PPNAD) as part of CNC, and one no identified cause. CS was often the sign that led to the diagnosis of MCAS or PPNAD. Age at CS diagnosis was infancy in MCAS and late childhood in PPNAD. The MCAS group had worse growth impairments (height SDS, P =0.006) and higher prevalences of intrauterine growth retardation (P =0.01) and liver dysfunction (P =0.04) compared to the PPNAD group. Learning disabilities were present in all seven MCAS patients vs. only two Carney-complex patients. Bilateral adrenalectomy was performed in 12 patients overall. One patient responded to metyrapone and two did not require CS therapy. Growth hormone therapy was not used in the MCAS patients, despite the substantial growth retardation. After CS therapy, growth recovered in both groups, albeit less completely in the MCAS group, in which liver dysfunction often persisted.
Conclusion: ACTH-independent CS is rare but can lead to serious complications in children. Early diagnosis and management is essential.
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