Tailored management of life-threatening complications related to severe obesity in a young adult with Prader-Willi syndrome
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Prader-Willi syndrome (PWS) is characterized by hypotonia, distinctive facial features, hyperphagia, obesity, short stature, hypogonadism, intellectual disability, and behavior problems. Uncontrolled hyperphagia can lead to dangerous food-seeking behavior and with life-threatening obesity. Severe obesity is prone to obstructive sleep apnea (OSA) and can lead to cor pulmonale. This study reports on a case involving a 21-year-old man with PWS who developed OSA due to severe obesity, which led to cor pulmonale, a life-threatening complication. Multidisciplinary care provided in the intensive care unit included weight reduction, ventilation support, antipsychotics, sedative drugs, rehabilitation, and meticulous skin care. The patient did recover. To prevent severe obesity in adults with PWS, hyperphagia must be controlled, and the patient must also be managed by an endocrinologist throughout childhood.
Abstract
Prader-Willi syndrome (PWS) is characterized by hypotonia, distinctive facial features, hyperphagia, obesity, short stature, hypogonadism, intellectual disability, and behavior problems. Uncontrolled hyperphagia can lead to dangerous food-seeking behavior and with life-threatening obesity. Severe obesity is prone to obstructive sleep apnea (OSA) and can lead to cor pulmonale. This study reports on a case involving a 21-year-old man with PWS who developed OSA due to severe obesity, which led to cor pulmonale, a life-threatening complication. Multidisciplinary care provided in the intensive care unit included weight reduction, ventilation support, antipsychotics, sedative drugs, rehabilitation, and meticulous skin care. The patient did recover. To prevent severe obesity in adults with PWS, hyperphagia must be controlled, and the patient must also be managed by an endocrinologist throughout childhood.
참고문헌 (15)
- Butler MG, "Causes of death in Prader-Willi syndrome : Prader-Willi Syndrome Association(USA)40-year mortality survey", Genet Med, 19, pp.635~642 (2017)
- Heymsfield SB, "Hyperphagia : current concepts and future directions proceedings of the 2nd international conference on hyperphagia", Obesity (Silver Spring), 22(Suppl 1), pp.S1~S17 (2014)
- Eiholzer U, "Prader-Willi Syndrome as a Model for Obesity" (2003), Karger
- Wollmann HA, "Reference values for height and weight in Prader-Willi syndrome based on 315 patients", Eur J Pediatr, 157, pp.634~642 (1998)
- Angulo MA, "Prader-Willi syndrome : a review of clinical, genetic, and endocrine findings", J Endocrinol Invest, 38, pp.1249~1263 (2015)
- Butler MG, "Management of Prader-Willi syndrome" (2006), Springer
- Vieira JL, "Chagas cardiomyopathy in Latin America review", Curr Cardiol Rep, 21, pp.8~ (2019)
- Stoohs R, "MESAM 4 : an ambulatory device for the detection of patients at risk for obstructive sleep apnea syndrome(OSAS)", Chest, 101, pp.1221~1227 (1992)
- Jones JB, "Oxyhemoglobin saturation during sleep in subjects with and without the obesity-hypoventilation syndrome", Chest, 88, pp.9~15 (1985)
- Brent BN, "Physiologic correlates of right ventricular ejection fraction in chronic obstructive pulmonary disease : a combined radionuclide and hemodynamic study", Am J Cardiol, 50, pp.255~262 (1982)
- MacNee W, "The effects of controlled oxygen therapy on ventricular function in patients with stable and decompensated cor pulmonale", Am Rev Respir Dis, 137, pp.1289~1295 (1988)
- Roza AM, "The Harris Benedict equation reevaluated : resting energy requirements and the body cell mass", Am J Clin Nutr, 40, pp.168~182 (1984)
- Stroud M, "Guidelines for enteral feeding in adult hospital patients", Gut, 52(Suppl 7(Suppl 7)), pp.vii1~vii12 (2003)
- Acute Respiratory Distress Syndrome Network, "Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome", N Engl J Med, 342, pp.1301~1308 (2000)
- Crinò A, "Obesity management in Prader-Willi syndrome : current perspectives", Diabetes Metab Syndr Obes, 11, pp.579~593 (2018)