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Energy Expenditure in 21-Hydroxylase Congenital Adrenal Hyperplasia Patients and Comparison with Predictive Equations
Institution:1. From the Laboratory of Investigation on Metabolism and Diabetes, (LIMED/Gastrocentro), University of Campinas (UNICAMP), Campinas, Sao Paulo, Brazil;2. School of Applied Sciences, University of Campinas, Limeira, Sao Paulo, Brazil;3. Laboratory of Growth and Development (LabCreD), Center for Investigation in Pediatrics (CIPED), School of Medical Sciences, University of Campinas, Campinas, Sao Paulo, Brazil;4. Department of Paediatrics, School of Medical Sciences, University of Campinas, Campinas, Sao Paulo, Brazil;5. OCRC - Obesity and Comorbities Research Center, Institute of Biology, Cidade Universitária, University of Campinas, Sao Paulo, Brazil.;1. First Department of Cardiology, Hippokration Hospital, Athens Medical School, Athens, Greece;2. Department of Cardiology, Athens General Hospital “G. Gennimatas”, Athens, Greece;3. Section of Cardiovascular Medicine, Greece;4. Cardiology Department, Patras University Hospital, Patras, Greece;5. Department of Pathology, Athens Medical School, Athens, Greece;1. From Eli Lilly and Company, Indianapolis, Indiana;2. Albany Medical College, Albany, New York;3. HaaPACS GmbH, Schriesheim, Germany;4. Eli Lilly and Company, Windlesham, United Kingdom.
Abstract:Objective: To characterize resting energy expenditure (REE) in patients with classic 21-hydroxylase congenital adrenal hyperplasia (21-OH CAH) using indirect calorimetry and compare it to the most commonly used REE predictive equations.Methods: This case-control study comprised 29 post-pubertal 21-OH CAH patients regularly followed at the University of Campinas. Elevated serum 17-hydroxyprogesterone and CYP21 gene molecular analysis confirmed the diagnosis. A healthy control group paired by age, gender, and body mass index was examined. Dual-energy X-ray absorptiometry (DEXA) measured body compositions. A bioimpedance analyzer determined fat-free mass, and indirect calorimetry using a metabolic cart measured REE.Results: Unlike our initial hypothesis, REE was similar between the groups (18.7 ± 3.1 kcal/kg/day in CAH vs. 20.3 ± 3.5 kcal/kg/day in controls; P = .728). No predictive equations reached the stipulated accuracy criteria, thus lacking validity in REE assessment in adults with the characteristics of the group studied. DEXA analysis revealed higher body fat and diminished nonbone lean mass in 21-OH CAH. Anthropometric and bioelectrical impedance parameters were not significantly different.Conclusion: Classic 21-OH CAH is generally followed in reference centers, which may facilitate indirect calorimetry use for REE measurement. Alternatively, considering our REE findings in adult 21-OH CAH patients, nutrition management based on 25 kcal/body weight/day (measured REE × activity factor 1.2 to 1.3) may be reasonable for current body weight maintenance in these patients.Abbreviations: 17-OHP = 17-hydroxyprogesterone; 21-OH CAH = classic 21-hydroxylase deficiency congenital adrenal hyperplasia; BMI = body mass index; REE = resting energy expenditure; VO2 = volume of oxygen; VCO2 = volume of carbon dioxide
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