Prader–Willi Syndrome, Management of Impulsivity, and Hyperphagia in an Adolescent

Puri, Muhammad; Sahl, Robert; Ogden, Shawwna; Malik, Salma

doi:10.1089/cap.2015.0240

Cited by 14 publications

(8 citation statements)

References 2 publications

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“…PWS patients display learning difficulties especially in observational learning 46 , 47 . A deficit in executive function has been observed with impaired mental switching 30 , 48 , and impaired inhibition capacities have also been noted 49 . Concerning visual perception, some authors have argued that patient with PWS display higher skills to detect similarities which possibly explain their high performance in puzzle processing 45 .…”

Section: Rdoc Approach and Pwsmentioning

confidence: 99%

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

et al. 2020

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The Research Domain Criteria project (RDoc) proposes a new classification system based on information from several fields in order to encourage translational perspectives. Nevertheless, integrating genetic markers into this classification has remained difficult because of the lack of powerful associations between targeted genes and RDoC domains. We hypothesized that genetic diseases with psychiatric manifestations would be good models for RDoC gene investigations and would thereby extend the translational approach to involve targeted gene pathways. To explore this possibility, we reviewed the current knowledge on Prader–Willi syndrome, a genetic disorder caused by the absence of expression of some of the genes of the chromosome 15q11–13 region inherited from the father. Indeed, we found that the associations between genes of the PW locus and the modification identified in the relevant behavioral, physiological, and brain imaging studies followed the structure of the RDoC matrix and its six domains (positive valence, negative valence, social processing, cognitive systems, arousal/regulatory systems, and sensorimotor systems).

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Section: Rdoc Approach and Pwsmentioning

confidence: 99%

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

et al. 2020

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“…Naltrexone is an opioid receptor antagonist used as monotherapy for alcohol and opioid dependence and bupropion is a dopamine/norepinephrine reuptake inhibitor used as monotherapy to treat depression and smoking [ 39 ]. The combination medication works through the anorexigenic effect of a-melanocyte-stimulating hormone from pro-opiomelanocortin neurons of the hypothalamus [ 39 , 40 ]. Side effects include increased blood pressure, headache, insomnia, dry mouth, and gastrointestinal symptoms [ 39 ].…”

Section: Resultsmentioning

confidence: 99%

“…There were three results when searching for PWS and naltrexone–bupropion with one meeting inclusion criteria. This included a 13-year-old with PWS and impulse control on naltrexone–bupropion who had decreased BMI with improved aggression [ 40 ].…”

Section: Resultsmentioning

confidence: 99%

Anti-Obesity Medication Use in Children and Adolescents with Prader–Willi Syndrome: Case Review and Literature Search

Goldman

Naguib

Vidmar

2021

JCM

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(1) Background: children with Prader-Willi syndrome (PWS) have high obesity rates due to hyperphagia and decreased metabolic rates. Although anti-obesity medications (AOMs) are prescribed to this population, there are no consensus guidelines on acceptability, safety, and efficacy. We present literature review and case series on AOMs in youth with PWS. (2) Methods: we performed PubMed review from January 2000 to April 2021 utilizing keywords: “Prader-Willi syndrome” or “PWS” and “medication” including: topiramate, metformin, phentermine, liraglutide, orlistat, oxytocin, semaglutide, naltrexone-bupropion. For our case series, patients were identified through retrospective chart reviews from a multi-disciplinary PWS clinic. Eligibility criteria: age ≤ 18 years, genetically confirmed PWS, AOM use for at least 16 weeks, and recent anthropometric data. (3) Results: a literature search yielded 14 articles (3 topiramate, 1 metformin, 4 liraglutide, 5 oxytocin, 1 naltrexone–bupropion). All studies reported improved hyperphagia with variable BMI effects. Ten adolescents met case series eligibility (mean age 13.2 ± 2.6 years, 40% female; AOMs: 6 metformin, 5 topiramate, 2 semaglutide, 3 liraglutide). After AOM course, 60% had decreased or stable BMI z-score. No significant side effects. (4) Conclusions: results suggest AOMs may be useful for weight management in youth with PWS. Additional studies are required to validate findings and support AOM treatment guidelines.

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“…Naltrexone monotherapy has been studied for opioid drug use in adolescents (age 13 and older) and PWS for appetite reduction since the 1980s, although long‐term safety has not been established in the pediatric population. There is one case report in the literature of using NB combination therapy for PWS in a 13‐year‐old girl, with a small reduction in BMI and improvements in hyperphagia . Off‐label drug‐use documentation, along with consent for treatment from the patient’s parent or guardian, is recommended. The effects of naltrexone and bupropion on pubertal development in humans are not known. In an animal study, bupropion did appear to alter pubertal onset .…”

Section: Recommendations (Table )mentioning

confidence: 99%

“…Naltrexone monotherapy has been studied for opioid drug use in adolescents (ages 13 years and older)(111) and PWS for appetite reduction (112) since the 1980s, although long-term safety has not been established in the pediatric population. There is one case-report in the literature of using NB combination therapy for PWS in a 13-year old girl with small reduction in BMI and improvements in hyperphagia (113).…”

Section: Available Pharmacotherapy (Fda-approved and Off-label Drug Umentioning

confidence: 99%

Clinical Considerations Regarding the Use of Obesity Pharmacotherapy in Adolescents with Obesity

Srivastava

Fox

Kelly

et al. 2019

Obesity

100

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A growing number of youth suffer from obesity and in particular severe obesity for which intensive lifestyle intervention does not adequately reduce excess adiposity. A treatment gap exists wherein effective treatment options for an adolescent with severe obesity include intensive lifestyle modification or metabolic and bariatric surgery while the application of obesity pharmacotherapy remains largely underutilized. These youth often present with numerous obesity‐related comorbid diseases, including hypertension, dyslipidemia, prediabetes/type 2 diabetes, obstructive sleep apnea, nonalcoholic fatty liver disease, musculoskeletal problems, and psychosocial issues such as depression, anxiety, and social stigmatization. Current pediatric obesity treatment algorithms for pediatric primary care providers focus primarily on intensive lifestyle intervention with escalation of treatment intensity through four stages of intervention. Although a recent surge in the number of Food and Drug Administration‐approved medications for obesity treatment has emerged in adults, pharmacotherapy options for youth remain limited. Recognizing treatment and knowledge gaps related to pharmacological agents and the urgent need for more effective treatment strategies in this population, discussed here are the efficacy, safety, and clinical application of obesity pharmacotherapy in youth with obesity based on current literature. Legal ramifications, informed consent regulations, and appropriate off‐label use of these medications in pediatrics are included, focusing on prescribing practices and prescriber limits.

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Prader–Willi Syndrome, Management of Impulsivity, and Hyperphagia in an Adolescent

Cited by 14 publications

References 2 publications

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

The RDoC approach for translational psychiatry: Could a genetic disorder with psychiatric symptoms help fill the matrix? the example of Prader–Willi syndrome

Anti-Obesity Medication Use in Children and Adolescents with Prader–Willi Syndrome: Case Review and Literature Search

Clinical Considerations Regarding the Use of Obesity Pharmacotherapy in Adolescents with Obesity

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