Efficacy of sodium-glucose cotransporter 2 inhibitor with glucagon-like peptide-1 receptor agonist for the glycemic control of a patient with Prader-Willi syndrome: a case report

Sano, Hitomi; Kudo, Eriko; Yamazaki, Takeshi; Ito, Tsubasa; Hatakeyama, Kinya; Kawamura, Nobuaki

doi:10.1297/cpe.29.81

Cited by 9 publications

(15 citation statements)

References 15 publications

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“…Currently, the evidence regarding the combined use of GLP-1 agonists and SGLT-2 inhibitors in the treatment of type 2 diabetes mellitus in PWS is limited to some case reports. In a 20-year-old woman with PWS, the addition of SGLT2 inhibitor, empagliflozin (10 mg/day) to existing liraglutide therapy resulted in significant improvements in both weight and glycemic control: a weight loss of approximately 5.5 kg (7.4%) was achieved during the subsequent 5 months without altering dietary intake and HbA1c level notably improved from 9.2 to 7.2% [ 101 ]. In an adolescent with T2DM and PWS the add-on of Metformin to different insulin regimens or Liraglutide did not improve glucose levels.…”

Section: Obesity-related Comorbiditiesmentioning

confidence: 99%

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Muscogiuri

Barrea

Faggiano

et al. 2021

J Endocrinol Invest

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Prader–Willi syndrome (PWS) is a 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 represented by paternal 15q11-q13 deletion, maternal uniparental disomy 15, and imprinting defect. Clinical picture of PWS changes across life stages. The main clinical characteristics are represented by short stature, developmental delay, cognitive disability and behavioral diseases. Hypotonia and poor suck resulting in failure to thrive are typical of infancy. As the subjects with PWS age, clinical manifestations such as hyperphagia, temperature instability, high pain threshold, hypersomnia and multiple endocrine abnormalities including growth hormone and thyroid-stimulating hormone deficiencies, hypogonadism and central adrenal insufficiency due to hypothalamic dysfunction occur. Obesity and its complications are the most common causes of morbidity and mortality in PWS. Several mechanisms for the aetiology of obesity in PWS have been hypothesized, which include aberration in hypothalamic pathways of satiety control resulting in hyperphagia, disruption in hormones regulating appetite and satiety and reduced energy expenditure. However, despite the advancement in the research field of the genetic basis of obesity in PWS, there are contradictory data on the management. Although it is mandatory to adopt obesity strategy prevention from infancy, there is promising evidence regarding the management of obesity in adulthood with current obesity drugs along with lifestyle interventions, although the data are limited. Therefore, the current manuscript provides a review of the current evidence on obesity and PWS, covering physiopathological aspects, obesity-related complications and conservative management.

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Section: Obesity-related Comorbiditiesmentioning

confidence: 99%

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Muscogiuri

Barrea

Faggiano

et al. 2021

J Endocrinol Invest

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“…One hundred and seventeen articles were identified after the search. After removing duplicates and screening for articles that fulfilled the inclusion criteria, a total of 10 articles 17–26 were included in the systematic review. Figure 1 shows the PRISMA flow chart detailing the study selection process.…”

Section: Resultsmentioning

confidence: 99%

The effects of glucagon‐like peptide (GLP)‐1 receptor agonists on weight and glycaemic control in Prader–Willi syndrome: A systematic review

Low

Rajgor

et al. 2021

Clinical Endocrinology

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Objective The mainstay management of hyperphagia and obesity in Prader–Willi syndrome (PWS) relies on dietary restrictions, strict supervision and behavioural modifications, which can be stressful for the patient and caregiver. There is no established pharmacological strategy to manage this aspect of PWS. Theoretically, glucagon‐like peptide‐1 (GLP‐1) receptor agonists (GLP1‐RA) used in patients with obesity and type 2 diabetes mellitus (T2DM) may be efficacious in weight and glycaemic control of PWS patients. We conducted a systematic review of the literature to summarize the evidence on the use of GLP1‐RA in PWS patients. Design Primary studies were searched in major databases using key concepts ‘Prader–Willi syndrome’ and ‘GLP1 receptor agonist’ and outcomes, ‘weight control OR glycaemic control OR appetite regulation’. Results Ten studies included, summarizing GLP1‐RA use in 23 PWS patients (age, 13–37 years), who had used either exenatide (n = 14) or liraglutide (n = 9) over a duration of 14 weeks to 4 years. Sixteen (70%) of these patients had T2DM. Ten patients experienced improvement in body mass index, ranging from 1.5 to 16.0 kg/m2, while improvement in HbA1c was seen in 19 of 23 cases, ranging between 0.3% and 7.5%. All five studies reporting appetite or satiety showed improvement in satiety levels. There were no reported serious side effects. Conclusions GLP1‐RA appears safe in PWS patients and may have potential benefits for weight, glycaemic and appetite control. Nonetheless, we also highlight a significant gap in the literature on the lack of well‐designed studies in this area, which limits the recommendation of GLP1‐RA use in PWS patients at present.

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“…There were six results for PWS and liraglutide, with four case reports meeting inclusion criteria. These included: a 17-year-old with PWS on liraglutide and metformin who had weight loss, increased satiety, as well as lower HbA1c and fasting glucose; an 18-year-old with PWS and dyspnea requiring mechanical ventilation due to severe obesity who maintained weight reduction on liraglutide; a 13-year-old with PWS and diabetes, with a decrease in glucose and HbA1c on liraglutide and empagliflozin; and a 19-year-old with PWS and diabetes who had improved glycemic control, HbA1c, insulin resistance, and weight loss on liraglutide and empagliflozin [ 21 , 24 , 25 , 26 , 27 ]. The remaining cases were focused on populations over the age limit or in a different language.…”

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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Efficacy of sodium-glucose cotransporter 2 inhibitor with glucagon-like peptide-1 receptor agonist for the glycemic control of a patient with Prader-Willi syndrome: a case report

Cited by 9 publications

References 15 publications

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

Obesity in Prader–Willi syndrome: physiopathological mechanisms, nutritional and pharmacological approaches

The effects of glucagon‐like peptide (GLP)‐1 receptor agonists on weight and glycaemic control in Prader–Willi syndrome: A systematic review

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

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