ESPE2023 Poster Category 2 Adrenals and HPA Axis (37 abstracts)
Should we routinely assess hypothalamo-pituitary-adrenal axis in pediatric patients with Prader-Willi Syndrome?
Wedrychowicz Anna 1,2 , Dolezal-Oltarzewska Katarzyna 2 , Zygmunt-Gorska Agata 2 , Kalicka-Kasperczyk Anna 1,2 , Wojcik Malgorzata 1,2 , Janus Dominika 1,2 , Kot Adrianna 2 , Lecka-Ambroziak Agnieszka 3,4 , Petriczko Elzbieta 5 , Wielopolska Joanna 5 & Starzyk Jerzy B 1,2
1Department of Pediatric and Adolescent Endocrinology, Pediatric Institute, Jagiellonian University - Medical College, Krakow, Poland. 2Department of Pediatric and Adolescent Endocrinology, University Children’s Hospital, Krakow, Poland. 3Department of Endocrinology and Diabetology, The Children’s Memorial Health Institute, Warsaw, Poland. 4Endocrinology Outpatient Clinic, Institute of Mother and Child, Warsaw, Poland. 5Department of Pediatrics, Endocrinology, Diabetology, Metabolic Disorders and Cardiology of Developmental Age, Pomeranian Medical University, Szczecin, Poland
Background: It was reported recently that central adrenal insufficiency (CAI) in pediatric patients (pts) with Prader-Willi Syndrome (PWS), presented in up to 60% of them, could be a potential cause of sudden death. Moreover it has been suggested that CAI could be aggravated by rhGH recombinant human growth hormone (rhGH) treatment.
Objective: To prevent both over- and undertreatment with hydrocortisone, we assessed the prevalence of CAI in a large multicenter cohort of pediatric patients with PWS analysing adrenal response in Low-Dose ACTH Test (LDAT) or/and in Glugacon Stimulation Test (GST).
Methods: Forty-six pts with PWS were included into the study. Thirty-three of them were treated with rhGH, with median dose 0.21 mg/kg/week. LDAT with 1 μg tetracosactrin i.v. was performed in 33 pts. Serum cortisol and plasma ACTH at baseline and cortisol response after stimulation were measured. Serum cortisol at baseline and after stimulation was measured in GST (0.1 mg/kg i.m.) in 2 pts. Both tests were performed in 11 pts. Tests started at 8.00 a.m. All hormones were measured using radioimmunoassays. A serum cortisol response > 181.2 ng/ml (500 nmol/l) in LDAT and > 199.3 ng/ml (550 nmol/l) in GST was considered a normal response. Additionally, either delta of cortisol response (the difference between the baseline and its highest value) > 90 ng/ml or doubling/triplication of baseline cortisol indicates normal adrenal reserve.
Results: Three GSTs were not diagnostic (no hypoglycemia). The LDAT results suggested CAI in 4 pts, but in 2/4 CAI was excluded in GST. The GST suggested CAI only in one patient, but it was excluded in LDAT. Therefore CAI was diagnosed in 2/46 pts (4.3%), one treated and one untreated with rhGH, with the highest cortisol values 162 and 175 ng/dl, but only in one test. However, in one of them delta cortisol response was > 90 ng/ml and more than tripled from baseline. So finally CAI was diagnosed in one pt with PWS treated with rhGH (2.2%).
Conclusion: Our data do not confirm a necessity of the routine diagnostics towards CAI in PWS pts. We present low occurrence of CAI in pediatric pts with PWS. Moreover, either LDAT or GST are not sensitive enough in diagnosing CAI in PWS and a suspicion of CAI in one of them should be confirmed in another. Treatment with rhGH in PWS pts rather does not increase CAI incidence.
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