Ann Pediatr Endocrinol Metab.  2022 Jun;27(2):148-152. 10.6065/apem.2142022.011.

Tailored management of life-threatening complications related to severe obesity in a young adult with Prader-Willi syndrome

Affiliations
  • 1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 2Department of Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

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.

Keyword

Prader-Willi syndrome; Hyperphagia; Obesity; Pulmonary heart disease; Heart failure; Obstructive sleep apnea

Figure

  • Fig. 1. (A) A skin ulcer on the left thigh. The inguinal area was folded, and a 1×3-cm-sized ulcer demonstrated a yellowish discharge. (B) Chest contrast computed tomography (CT) at admission. The CT revealed bilateral pleural effusion and peribronchial opacity. The subcutaneous fat (arrow) under the skin on CT was 82.7mm.

  • Fig. 2. (A) Changes in body weight and input/output balance. A continuous intravenous (IV) infusion of furosemide was used to achieve a negative balance of input/output (I/O) (negative 1,000–2,000 mL/day). The patient lost 54.3 kg (a reduction from 185.7 to 131.4 kg) over 7 weeks of hospitalization (HD). Serial chest x-rays showed cardiomegaly with combined pulmonary congestion and pleural effusion on HD 4, subsegmental atelectasis in the right middle lung field on HD 28, and improved pulmonary congestion and pleural effusion on HD 42. (B) Caloric restriction and glucose control. Total parenteral nutrition (TPN) was begun on HD 4. On HD 14, enteral nutrition was started via a nasogastric tube at 100 mL/day and was increased to 1,500 mL/day over 4 weeks. To restrict calories (1,500 kcal/day), TPN was decreased according to the increasing enteral feeding volume. As the amount of enteral feeding increased, the patient's blood glucose level rose, and insulin injections were required beginning on HD 28. On HD 35, we switched to full enteral feeding via a nasogastric (NG) tube. On HD 42, we began oral feeding (1,000 kcal/day). At discharge, an insulin dosage of 0.83 IU/kg/day (degludec at 50 IU/day and aspart at 59 IU/day) was required.


Reference

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