Beneficial Outcomes of Sleeve Gastrectomy in a Morbidly Obese Patient With Bardet-Biedl Syndrome

Boscolo, Marina; Féry, Françoise; Cnop, Miriam

doi:10.1210/js.2017-00071

Cited by 16 publications

(26 citation statements)

References 13 publications

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“…Thus, circulatory system disease rates decreased with respect to preoperative values. Moreover, the life expectancy of patients increases after LSG [17]. In our study, the blood pressure levels of patients, who underwent LSG operation, were significantly reduced after losing weight, doing exercise, and following a proper diet program.…”

Section: Discussionmentioning

confidence: 51%

The Body Weight, Blood Pressure, and Biochemical Parameters of the Patients After Obesity Surgery

Kocaöz

Parlak

2019

SN Compr. Clin. Med.

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The objective of this study is to observe whether the patients' loss weight is long term after bariatric surgery, as well as evaluating the blood pressure and certain chemical parameters. This retrospective study was carried out based on the files of 70 patients, who underwent laparoscopic sleeve gastrectomy (LSG) operation between January 2013 and December 2017. Paired sample t test and Wilcoxon tests were used for comparison of patients' weight, blood pressure, and other parameters related to obesity within at least 6 months after LSG. While the body mass index (BMI) average of patients before bariatric surgery was 45.82 ± 4.73 kg/m 2 , it was reduced to 27.33 ± 2.98 kg/m 2 within approximately 3 years after the operation (p < 0.01). It was confirmed that there was a significant remission in hypertension, diabetes mellitus, hyperlipidemia, serum insulin, HbA1C, and HOMA-IR parameters of patients after bariatric surgery (p < 0.05). Additionally, it was also detected that the antihypertensive and antidiabetic drug usage dosage of patients was either minimized or discontinued after bariatric surgery. In our study, it was detected that the weight loss of patients after LSG was satisfactory and that blood pressure, including certain biochemical parameters related to obesity, was on remission. Therefore, it is thought that LSG in the early period is a reliable operation, which ensures the weight loss, provided that the patients accommodate themselves to the changes in their lifestyles.

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Section: Discussionmentioning

confidence: 51%

The Body Weight, Blood Pressure, and Biochemical Parameters of the Patients After Obesity Surgery

Kocaöz

Parlak

2019

SN Compr. Clin. Med.

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“…[26][27][28] Because obesity management can control these co-morbidities, weight reduction through hyperphagia management with setmelanotide could potentially effectively manage or alleviate obesity-related co-morbidities in individuals with BBS. 29,30 The current study has some limitations. The study design is openlabel, uncontrolled and non-comparative.…”

Section: Discussionmentioning

confidence: 91%

“…Uncontrolled obesity can lead to co‐morbidities, including cardiovascular issues, metabolic syndrome, diabetes and reduced quality of life 26–28 . Because obesity management can control these co‐morbidities, weight reduction through hyperphagia management with setmelanotide could potentially effectively manage or alleviate obesity‐related co‐morbidities in individuals with BBS 29,30 …”

Section: Discussionmentioning

confidence: 99%

Effect of setmelanotide, a melanocortin‐4 receptor agonist, on obesity in Bardet‐Biedl syndrome

Haws

Brady

Davis

et al. 2020

Diabetes Obesity Metabolism

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Aim: To report an analysis of 1 year of setmelanotide treatment for obesity and hunger, as well as metabolic and cardiac outcomes, in individuals with Bardet-Biedl syndrome (BBS). Materials and methods: Individuals aged 12 years and older with BBS received oncedaily setmelanotide. The dose was titrated every 2 weeks to establish the individual therapeutic dose (≤3 mg); treatment continued for an additional 10 weeks. Participants who lost 5 kg or more (or ≥5% of body weight if <100 kg at baseline) continued into the 52-week extension phase. The primary outcome was mean percent change from baseline in body weight at 3 months. Hunger scores and safety were secondary outcomes. Results: From February 2017 and February 2018, 10 individuals were screened; eight completed the 3-month treatment phase and seven completed the extension phase. Mean percent change in body weight from baseline to 3 months was −5.5% (90% CI, −9.3% to −1.6%; n = 8); change from baseline was −11.3% (90% CI, −15.5% to −7.0%; n = 8) at 6 months and −16.3% (90% CI, −19.9% to −12.8%; n = 7) at 12 months. All participants reported at least one treatmentemergent adverse event (AE), most commonly injection-site reaction. No AEs led to study withdrawal or death. Most, morning, and average hunger scores were reduced across time points. Conclusions: Setmelanotide reduced body weight and hunger in individuals with BBS and had a safety profile consistent with previous reports. Setmelanotide may be a treatment option in individuals with BBS-associated obesity and hyperphagia.

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“…A 1,000 kcal/day diet did not reduce the liver enzyme levels. However, bariatric surgery is reportedly effective against morbid obesity and NAFLD in BBS ( 36 ). In addition, in our case, the liver enzyme levels were markedly elevated after drug administration and remained elevated for a prolonged period.…”

Section: Discussionmentioning

confidence: 99%

Bardet-Biedl Syndrome Caused by Skipping of <i>SCLT1</i> Complicated by Microvesicular Steatohepatitis

et al. 2020

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We treated the case of a 22-year-old male patient with liver dysfunction. At 1 year of age, hepatic fibrosis was suspected. In addition, due to the presence of retinitis pigmentosa, renal failure, obesity, mental retardation, and hypogonadism, he was diagnosed with Bardet-Biedl syndrome (BBS). Skipping of exons 14 and 17 in the sodium channel and clathrin linker 1 ( SCLT1 ) gene was observed. At 22 years of age, the liver enzyme levels were further elevated and a diagnosis of microvesicular steatohepatitis was made. Insulin resistance, a reduction of muscle mass, an impairment of the fatty acid metabolism, and hyperleptinemia in this syndrome may cause steatohepatitis.

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Beneficial Outcomes of Sleeve Gastrectomy in a Morbidly Obese Patient With Bardet-Biedl Syndrome

Cited by 16 publications

References 13 publications

The Body Weight, Blood Pressure, and Biochemical Parameters of the Patients After Obesity Surgery

The Body Weight, Blood Pressure, and Biochemical Parameters of the Patients After Obesity Surgery

Effect of setmelanotide, a melanocortin‐4 receptor agonist, on obesity in Bardet‐Biedl syndrome

Bardet-Biedl Syndrome Caused by Skipping of <i>SCLT1</i> Complicated by Microvesicular Steatohepatitis

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