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Effectiveness of Deep Brain Stimulation in Reducing Body Mass Index and Weight: A Systematic Review.Stereotactic and Functional Neurosurgery 2022Obesity has become a major public health concern worldwide, with current behavioral, pharmacological, and surgical treatments offering varying rates of success and...
BACKGROUND
Obesity has become a major public health concern worldwide, with current behavioral, pharmacological, and surgical treatments offering varying rates of success and adverse effects. Neurosurgical approaches to treatment of refractory obesity include deep brain stimulation (DBS) on either specific hypothalamic or reward circuitry nuclei, which might contribute to weight reduction through different mechanisms. We aimed to determine the safety and clinical effect of DBS in medical refractory obesity.
SUMMARY
Adhering to PRISMA guidelines, we performed a systematic review to identify all original studies - observational and experimental - in which DBS was performed to treat refractory obesity. From database inception to April 2021, we conducted our search in PubMed, Scopus, and LILACS databases using the following MeSH terms: "Obesity" OR "Prader-Willi Syndrome" AND "Deep Brain Stimulation." The main outcomes were safety and weight loss measured with the body mass index (BMI). The Grading of Recommendations Assessment, Development, and Evaluation methods were applied to evaluate the quality of evidence. This study protocol was registered with PROSPERO ID: CRD42019132929. Seven studies involving 12 patients met the inclusion criteria; the DBS target was the nucleus accumbens in four (57.1%), the lateral hypothalamic area in two (29.6%), and the ventral hypothalamus in one (14.3%). Further, 33% of participants had obesity secondary to Prader-Willi syndrome (PWS) and 66.6% had primary obesity. The global BMI average at baseline was 46.7 (SD: 9.6, range: 32.2-59.1), and after DBS, 42.8 (SD: 8.8, range: 25-53.9), with a mean difference of 3.9; however, the delta in PWS patients was -2.3 and 10 in those with primary obesity. The incidence of moderate side effects was 33% and included manic symptoms (N = 2), electrode fracture (N = 1), and seizure (N = 1); mild complications (41.6%) included skin infection (N = 2), difficulties falling asleep (N = 1), nausea (N = 1), and anxiety (N = 1).
KEY MESSAGES
Despite available small case series and case reports reporting a benefit in the treatment of refractory obesity with DBS, this study emphasizes the need for prospective studies with longer follow-ups in order to further address the efficacy and indications.
Topics: Body Mass Index; Deep Brain Stimulation; Humans; Nucleus Accumbens; Prospective Studies; Weight Loss
PubMed: 34583359
DOI: 10.1159/000519158 -
Journal of Assisted Reproduction and... Jun 2018To determine whether a history of conception by assisted reproductive technology (ART) is associated with occurrence of one or more imprinting disorders of either... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
To determine whether a history of conception by assisted reproductive technology (ART) is associated with occurrence of one or more imprinting disorders of either maternal or paternal origin.
METHODS
We implemented a systematic review of scholarly literature followed by comprehensive meta-analysis to quantitatively synthesize data from reports relating to use of ART to occurrence of any imprinting disorder of humans, including Beckwith-Wiedemann (BWS), Angelman (AS), Prader-Willi (PWS), and Silver-Russell (SRS) syndromes, as well as transient neonatal diabetes mellitus (TNDB) and sporadic retinoblasoma (RB).
RESULTS
The systematic review identified 13 reports presenting unique data from 23 studies that related conception following ART to occurrence of imprinting disorders. Multiple studies of four disorder were identified, for which meta-analysis yielded the following summary estimates of associations with a history of ART: AS, summary odds ratio (sOR) = 4.7 (95% confidence interval (CI) 2.6-8.5, 4 studies); BWS, sOR = 5.8 (95% CI 3.1-11.1, 8 studies); PWS, sOR = 2.2 (95% CI 1.6-3.0, 6 studies); SRS, sOR = 11.3 (95% CI 4.5-28.5, 3 studies). Only one study reported on each of TNDB and RB.
CONCLUSION
Published data reveal positive associations between history of ART conception and each of four imprinting disorders. Reasons for these associations warrant further investigation.
Topics: Chromosome Disorders; Female; Fertilization; Genomic Imprinting; Humans; Reproductive Techniques, Assisted; Risk Factors
PubMed: 29696471
DOI: 10.1007/s10815-018-1173-x -
Research in Developmental Disabilities Aug 2014Over the past three decades, the potential effects of lifestyle interventions targeting changes in body weight and composition (weight, body mass index, fat mass, waist... (Review)
Review
Over the past three decades, the potential effects of lifestyle interventions targeting changes in body weight and composition (weight, body mass index, fat mass, waist circumference) among adults with an intellectual disability (ID) have been examined in various systematic reviews. Nevertheless, since the middle of the 1980s, the potential effects of these interventions for youth with an ID remain an open question. The purpose of this article is to review the effects of lifestyle interventions targeting changes in body weight and composition among youth with an ID. This review will focus on changes in body weight and composition, healthy lifestyle, and secondary health conditions. A systematic review of English- and French-language studies, published between 1981 and 2013, was performed on Academic Search Complete, PsycARTICLES, Medline and Scopus. The nine studies included in this review focused mainly on: a sample with a wide age range (e.g., 7-22 years); males; overweight-obese youth having a mild-to-moderate ID with Down or Prader-Willi syndrome; physical activity interventions; cohort pre- and post-test designs with/without a control group; and changes in body weight and composition. Taken together, results from these studies suggest successful changes in weight, body mass index and fat mass. However, intervention effects on healthy lifestyle and secondary health conditions are scarce and inconclusive. Given the weaknesses of the reviewed studies, the present findings should be considered preliminary and indicative of the need for future research.
Topics: Adiposity; Body Composition; Body Weight; Health Promotion; Humans; Intellectual Disability; Life Style
PubMed: 24830882
DOI: 10.1016/j.ridd.2014.04.014 -
Health Policy (Amsterdam, Netherlands) Jul 2015Cost-of-illness studies, the systematic quantification of the economic burden of diseases on the individual and on society, help illustrate direct budgetary consequences... (Review)
Review
Cost-of-illness studies, the systematic quantification of the economic burden of diseases on the individual and on society, help illustrate direct budgetary consequences of diseases in the health system and indirect costs associated with patient or carer productivity losses. In the context of the BURQOL-RD project ("Social Economic Burden and Health-Related Quality of Life in patients with Rare Diseases in Europe") we studied the evidence on direct and indirect costs for 10 rare diseases (Cystic Fibrosis [CF], Duchenne Muscular Dystrophy [DMD], Fragile X Syndrome [FXS], Haemophilia, Juvenile Idiopathic Arthritis [JIA], Mucopolysaccharidosis [MPS], Scleroderma [SCL], Prader-Willi Syndrome [PWS], Histiocytosis [HIS] and Epidermolysis Bullosa [EB]). A systematic literature review of cost of illness studies was conducted using a keyword strategy in combination with the names of the 10 selected rare diseases. Available disease prevalence in Europe was found to range between 1 and 2 per 100,000 population (PWS, a sub-type of Histiocytosis, and EB) up to 42 per 100,000 population (Scleroderma). Overall, cost evidence on rare diseases appears to be very scarce (a total of 77 studies were identified across all diseases), with CF (n=29) and Haemophilia (n=22) being relatively well studied, compared to the other conditions, where very limited cost of illness information was available. In terms of data availability, total lifetime cost figures were found only across four diseases, and total annual costs (including indirect costs) across five diseases. Overall, data availability was found to correlate with the existence of a pharmaceutical treatment and indirect costs tended to account for a significant proportion of total costs. Although methodological variations prevent any detailed comparison between conditions and based on the evidence available, most of the rare diseases examined are associated with significant economic burden, both direct and indirect.
Topics: Cost of Illness; Europe; Health Care Costs; Humans; Orphan Drug Production; Prevalence; Quality of Life; Rare Diseases
PubMed: 25661982
DOI: 10.1016/j.healthpol.2014.12.016 -
Health Technology Assessment... 2002
Review
Topics: Child; Cost-Benefit Analysis; Human Growth Hormone; Humans; Kidney Failure, Chronic; Practice Guidelines as Topic; Prader-Willi Syndrome; Quality of Life; Randomized Controlled Trials as Topic; State Medicine; Treatment Outcome; Turner Syndrome; United Kingdom
PubMed: 12433316
DOI: 10.3310/hta6180 -
Clinical Genetics Dec 2019Pathogenic MAGEL2 variants result in the phenotypes of Chitayat-Hall syndrome (CHS), Schaaf-Yang syndrome (SYS) and Prader-Willi syndrome (PWS). We present five patients...
Pathogenic MAGEL2 variants result in the phenotypes of Chitayat-Hall syndrome (CHS), Schaaf-Yang syndrome (SYS) and Prader-Willi syndrome (PWS). We present five patients with mutations in MAGEL2, including the first patient reported with a missense variant, adding to the limited literature. Further, we performed a systematic review of the CHS and SYS literature, assess the overlap between CHS, SYS and PWS, and analyze genotype-phenotype correlations among them. We conclude that there is neither a clinical nor etiological difference between CHS and SYS, and propose that the two syndromes simply be referred to as MAGEL2-related disorders.
Topics: Abnormalities, Multiple; Adult; Child, Preschool; Cluster Analysis; DNA Mutational Analysis; Female; Humans; Infant; Infant, Newborn; Male; Mutation; Proteins; Young Adult
PubMed: 31397880
DOI: 10.1111/cge.13620 -
PloS One 2022This systematic review aims to describe 1) the epidemiology of the diseases indicated for treatment with growth hormone (GH) in Italy; 2) the adherence to the GH...
OBJECTIVES
This systematic review aims to describe 1) the epidemiology of the diseases indicated for treatment with growth hormone (GH) in Italy; 2) the adherence to the GH treatment in Italy and factors associated with non-adherence; 3) the economic impact of GH treatment in Italy; 4) the quality of life of patients treated with GH and their caregivers in Italy.
METHODS
Systematic literature searches were performed in PubMed, Embase and Web of Science from January 2010 to March 2021. Literature selection process, data extraction and quality assessment were performed by two independent reviewers. Study protocol has been registered in PROSPERO (CRD42021240455).
RESULTS
We included 25 studies in the qualitative synthesis. The estimated prevalence of growth hormone deficiency (GHD) was 1/4,000-10,000 in the general population of children; the prevalence of Short Stature HOmeoboX Containing gene deficiency (SHOX-D) was 1/1,000-2,000 in the general population of children; the birth prevalence of Turner syndrome was 1/2,500; the birth prevalence of Prader-Willi syndrome (PWS) was 1/15,000. Treatment adherence was suboptimal, with a range of non-adherent patients of 10-30%. The main reasons for suboptimal adherence were forgetfulness, being away from home, pain/discomfort caused by the injection. Economic studies reported a total cost for a complete multi-year course of GH treatment of almost 100,000 euros. A study showed that drug wastage can amount up to 15% of consumption, and that in some Italian regions there could be a considerable over- or under-prescribing. In general, patients and caregivers considered the GH treatment acceptable. There was a general satisfaction among patients with regard to social and school life and GH treatment outcomes, while there was a certain level of intolerance to GH treatment among adolescents. Studies on PWS patients and their caregivers showed a lower quality of life compared to the general population, and that social stigma persists.
CONCLUSION
Growth failure conditions with approved GH treatment in Italy constitute a significant burden of disease in clinical, social, and economic terms. GH treatment is generally considered acceptable by patients and caregivers. The total cost of the GH treatment is considerable; there are margins for improving efficiency, by increasing adherence, reducing drug wastage and promoting prescriptive appropriateness.
Topics: Adolescent; Child; Child, Preschool; Female; Human Growth Hormone; Humans; Italy; Male; Prader-Willi Syndrome; Prevalence; Quality of Life; Treatment Adherence and Compliance; Turner Syndrome
PubMed: 35213607
DOI: 10.1371/journal.pone.0264403 -
Systematic review of ghrelin response to food intake in pediatric age, from neonates to adolescents.The Journal of Clinical Endocrinology... May 2014Food intake and energy balance are regulated during the lifespan with critical changes in each specific period (infancy, adulthood, aging). Some of ghrelin's changes may... (Review)
Review
OBJECTIVE
Food intake and energy balance are regulated during the lifespan with critical changes in each specific period (infancy, adulthood, aging). Some of ghrelin's changes may contribute to the regulation of food intake and weight in children. We aimed to analyze the ghrelin response to feeding in lean or obese subjects from birth to adolescence.
METHODS
We searched PubMed, Scopus, Google Scholar, Cochrane, and EMBASE (December 1999 to February 2013) and identified 62 relevant articles, of which 29 were suitable to be included.
RESULTS AND CONCLUSIONS
Total ghrelin response to meals is particular, with refractoriness in neonates and lean children and an inhibition that starts from puberty. Total ghrelin levels are decreased after meals, irrespective of pubertal stages in obese children and adolescents. Conversely, total ghrelin is decreased after an oral glucose tolerance test in all ages, with the exception of neonates. Data on unacylated ghrelin response are scant but resemble those of total ghrelin. The acylated ghrelin response to meals or oral glucose tolerance test is discordant, although a precocious inhibition followed by a rise back is present in both lean and obese children. The post-feeding profile in children with Prader-Willi syndrome is also peculiar, with a conserved and deeper inhibition of all ghrelin forms.
Topics: Adolescent; Body Weight; Child; Child, Preschool; Eating; Ghrelin; Humans; Obesity; Postprandial Period
PubMed: 24601727
DOI: 10.1210/jc.2013-4010 -
Journal of Intellectual Disabilities :... Mar 2019In Florida, the Agency for Persons with Disabilities provides waivers for adults with the following types of disabilities: intellectual disability, autism spectrum...
In Florida, the Agency for Persons with Disabilities provides waivers for adults with the following types of disabilities: intellectual disability, autism spectrum disorder, cerebral palsy, spina bifida, Down syndrome, and Prader-Willi syndrome. This review examined the peer-reviewed literature to indicate and assess the common needs for individuals with intellectual and developmental disabilities. Current models of service delivery, the efficacy of these services, and remaining gaps in the need fulfillment of individuals within the six diagnostic categorizations of interest were examined. Severity level within each diagnostic category was plotted on a matrix according to whether the needs of individuals were minimal, moderate, severe, or universal. The study found that sexual health education, socialization, and adult-focused medical care are universal needs among the six conditions. The study indicates that health-care professionals must work toward addressing the many unmet needs in comprehensive life span care services for adult individuals with neurodevelopmental disorders.
Topics: Adult; Autism Spectrum Disorder; Cerebral Palsy; Delivery of Health Care; Developmental Disabilities; Humans; Intellectual Disability; Needs Assessment; Spinal Dysraphism
PubMed: 28847208
DOI: 10.1177/1744629517726209 -
Health Technology Assessment... Sep 2010Recombinant human growth hormone (rhGH) is licensed for short stature associated with growth hormone deficiency (GHD), Turner syndrome (TS), Prader-Willi syndrome (PWS),... (Review)
Review
BACKGROUND
Recombinant human growth hormone (rhGH) is licensed for short stature associated with growth hormone deficiency (GHD), Turner syndrome (TS), Prader-Willi syndrome (PWS), chronic renal insufficiency (CRI), short stature homeobox-containing gene deficiency (SHOX-D) and being born small for gestational age (SGA).
OBJECTIVES
To assess the clinical effectiveness and cost-effectiveness of rhGH compared with treatment strategies without rhGH for children with GHD, TS, PWS, CRI, SHOX-D and those born SGA.
DATA SOURCES
The systematic review used a priori methods. Key databases were searched (e.g. MEDLINE, EMBASE, NHS Economic Evaluation Database and eight others) for relevant studies from their inception to June 2009. A decision-analytical model was developed to determine cost-effectiveness in the UK.
STUDY SELECTION
Two reviewers assessed titles and abstracts of studies identified by the search strategy, obtained the full text of relevant papers, and screened them against inclusion criteria.
STUDY APPRAISAL
Data from included studies were extracted by one reviewer and checked by a second. Quality of included studies was assessed using standard criteria, applied by one reviewer and checked by a second. Clinical effectiveness studies were synthesised through a narrative review.
RESULTS
Twenty-eight randomised controlled trials (RCTs) in 34 publications were included in the systematic review. GHD: Children in the rhGH group grew 2.7 cm/year faster than untreated children and had a statistically significantly higher height standard deviation score (HtSDS) after 1 year: -2.3 ± 0.45 versus -2.8 ± 0.45. TS: In one study, treated girls grew 9.3 cm more than untreated girls. In a study of younger children, the difference was 7.6 cm after 2 years. HtSDS values were statistically significantly higher in treated girls. PWS: Infants receiving rhGH for 1 year grew significantly taller (6.2 cm more) than those untreated. Two studies reported a statistically significant difference in HtSDS in favour of rhGH. CRI: rhGH-treated children in a 1-year study grew an average of 3.6 cm more than untreated children. HtSDS was statistically significantly higher in treated children in two studies. SGA: Criteria were amended to include children of 3+ years with no catch-up growth, with no reference to mid-parental height. Only one of the RCTs used the licensed dose; the others used higher doses. Adult height (AH) was approximately 4 cm higher in rhGH-treated patients in the one study to report this outcome, and AH-gain SDS was also statistically significantly higher in this group. Mean HtSDS was higher in treated than untreated patients in four other studies (significant in two). SHOX-D: After 2 years' treatment, children were approximately 6 cm taller than the control group and HtSDS was statistically significantly higher in treated children. The incremental cost per quality adjusted life-year (QALY) estimates of rhGH compared with no treatment were: 23,196 pounds for GHD, 39,460 pounds for TS, 135,311 pounds for PWS, 39,273 pounds for CRI, 33,079 pounds for SGA and 40,531 pounds for SHOX-D. The probability of treatment of each of the conditions being cost-effective at 30,000 pounds was: 95% for GHD, 19% for TS, 1% for PWS, 16% for CRI, 38% for SGA and 15% for SHOX-D.
LIMITATIONS
Generally poorly reported studies, some of short duration.
CONCLUSIONS
Statistically significantly larger HtSDS values were reported for rhGH-treated children with GHD, TS, PWS, CRI, SGA and SHOX-D. rhGH-treated children with PWS also showed statistically significant improvements in body composition measures. Only treatment of GHD would be considered cost-effective at a willingness-to-pay threshold of 20,000 to 30,000 pounds per QALY gained. This analysis suggests future research should include studies of longer than 2 years reporting near-final height or final adult height.
Topics: Biomarkers; Body Composition; Cost-Benefit Analysis; Dwarfism, Pituitary; Growth Disorders; Human Growth Hormone; Humans; Incidence; Linear Models; Models, Economic; Prevalence; Prognosis; Quality Assurance, Health Care; Quality of Life; Quality-Adjusted Life Years; United States
PubMed: 20849734
DOI: 10.3310/hta14420