Prader-Willi Syndrome: Role of Bariatric Surgery in Two Adolescents with Obesity
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LETTER TO THE EDITOR
Prader-Willi Syndrome: Role of Bariatric Surgery in Two Adolescents with Obesity Marina Tripodi 1 & Alberto Casertano 1 & Martina Peluso 1 & Mario Musella 2 & Giovanna Berardi 2 & Enza Mozzillo 3 Adriana Franzese 1
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# Springer Science+Business Media, LLC, part of Springer Nature 2020
Bariatric surgery (BS) is an effective treatment for adolescents with severe obesity; limited research evidence suggests that BS can be safely performed in youth with developmental delay (DD) to improve obesity-related comorbidities [1]. We report two adolescents with Prader-Willi Syndrome (PWS) who underwent BS. PWS is a multisystemic genetic disorder caused by the lack of expression of genes on the paternally inherited chromosome 15q11.2-q13 region. The three main genetic subtypes are paternal 15q11-q13 deletion (65–75%), maternal uniparental disomy 15 (20–30%), and imprinting defect (1–3%). DNA methylation is the diagnostic test detecting PWS in all three molecular genetic classes. Clinical manifestations of PWS are different according to age, ranging from hypotonia with poor suck in the first years of life to excessive weight gain, DD, and behavioral problems becoming evident in youth. The phenotype is likely due to hypothalamic dysfunction, which is responsible for hyperphagia, temperature instability, high pain threshold, hypersomnia, and multiple endocrine abnormalities including growth hormone and thyroid-stimulating hormone deficiencies, hypogonadism, and central adrenal insufficiency. The estimated prevalence is 1 in 10,000 to 30,000, equally divided in males and females [2]. Obesity is a significant contributor to mortality in PWS due to both cardiorespiratory failure caused
by sleep hypoventilation disorders, obstructive sleep apnea syndrome (OSAS), and upper airway and respiratory muscle hypotonia [3], and to diabetes. Hyperphagia-related behaviors also increase the risk for gastrointestinal (GI) perforation and necrosis, aspiration, choking, and swallowing difficulties for a combination of factors from central hypotonia, reduced GI motility, to rapid food consumption [4]. Treatment with recombinant growth hormone (rGH) therapy is effective and improves body composition, promoting motor functions in youth [5], but if patients become severely obese and if OSAS occurs, rGH should be interrupted [6]. Obesity management involves environmental control with early institution of a low-calorie and well-balanced diet, regular exercise, restriction of access to food, and counseling of the patient and family. Nonetheless, even though discussion with parents about attempts to control hyperphagia starts even during infancy, it is often insufficient to prevent morbid obesity. Treatment with BS, as gastric banding or bypass, has not been shown to reduce hyperphagia or achieve long-term weight reduction and is associated with morbidity and mortality [7]. Literature debates about BS as an option to treat morbid obesity in PWS; we point out what we got in our two cases.
Marina Tripodi and Alberto Casertano contri
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