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Childhood Obesity (Print) Apr 2023Probiotics have been proposed as a prevention or treatment for pediatric overweight and obesity. Conduct a scoping review on probiotic use in children and adolescents... (Review)
Review
Probiotics have been proposed as a prevention or treatment for pediatric overweight and obesity. Conduct a scoping review on probiotic use in children and adolescents with overweight or obesity and those with weight-related conditions and to identify knowledge gaps and research priorities. Seven databases using keywords and medical subject heading terms for articles reporting probiotic use in children or adolescents with overweight or obesity published from database conception until initiation of the study. Articles reporting primary data on probiotics use in children or adolescents with overweight or obesity. We utilized the Arksey and O'Malley framework, PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines, followed a predetermined study protocol for level-one abstract and level-two full-text screenings, synthesized information into subject-area domains, and identified research gaps. Heterogeneity of probiotic interventions, host factors, and genomics. Database search yielded 1356 unique articles with 19 randomized placebo-controlled studies, 945 participants, duration of interventions from 8 weeks to 9 months. Disease indications included Nonalcoholic Fatty Liver Disease, insulin resistance, hypercholesterolemia, Prader-Willi Syndrome, metabolic syndrome, and obesity. Limited and heterogeneous evidence for probiotic use in children and adolescents with weight-related conditions noted. Heterogeneity among published articles in probiotic strains, doses, design, biomarkers, confirmation, and outcomes observed. Despite complex existing and limited data, studies to date of children and adolescents with overweight and obesity demonstrate potential beneficial treatment effects of probiotics on BMI, adiposity, metabolic parameters, inflammatory markers, fatty liver, transaminase levels, and glucose metabolism. Clinical trials to address heterogeneous results are needed.
Topics: Child; Humans; Adolescent; Overweight; Pediatric Obesity; Probiotics; Adiposity; Metabolic Syndrome
PubMed: 35723657
DOI: 10.1089/chi.2022.0059 -
Tijdschrift Voor Psychiatrie 2021Prader-Willi syndrome (PWS) is a genetic syndrome characterized by dysmorphic features and endocrine, cognitive and psychiatric problems. Psychiatric problems interfere...
BACKGROUND
Prader-Willi syndrome (PWS) is a genetic syndrome characterized by dysmorphic features and endocrine, cognitive and psychiatric problems. Psychiatric problems interfere with the transition from pediatric to adult care. Psychiatric expertise is needed to facilitate this transition.
AIM
To provide a literature review on the prevalence and clinical presentation of psychiatric disorders in adults with PWS.
METHOD
A systematic literature review following the PRISMA-guidelines.
RESULTS
Thirty-three articles were included. Most adults with PWS had a specific behavioral profile with disruptive, autistic and compulsive characteristics. Psychotic symptoms occured in one third of adults with PWS, mostly in patients with maternal uniparental disomy. Mood disorders were present in 10 to 20% of adults with PWS and often accompanied by psychotic features. Studies were limited and heterogeneous in samples and methods.
CONCLUSION
There is a broad spectrum of psychiatric symptoms in adults with PWS. The clinical presentation does not fully fit within the DSM categories and shows differences between genetic subgroups. Longitudinal studies assessing the psychiatric symptoms with standardized methods are needed to improve practices on diagnosing, prevention, and treatment.
Topics: Adult; Child; Humans; Longitudinal Studies; Prader-Willi Syndrome; Psychotic Disorders; Transition to Adult Care
PubMed: 34231862
DOI: No ID Found -
The Laryngoscope Apr 2021Prader-Willi syndrome (PWS) increases the risk of obstructive sleep apnea (OSA) due to obesity, hypotonia, and abnormal ventilatory responses. We evaluated... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
Prader-Willi syndrome (PWS) increases the risk of obstructive sleep apnea (OSA) due to obesity, hypotonia, and abnormal ventilatory responses. We evaluated post-adenotonsillectomy complications, polysomnography changes, and quality of life in children with OSA and PWS.
STUDY DESIGN
Systematic review and meta-analysis.
METHODS
We conducted a systematic review and meta-analysis by searching PubMed, Embase, Cochrane, Web of Science, and Scopus. Two researchers independently reviewed studies about adenotonsillectomy for OSA in patients <21 years with PWS. We extracted study design, patient numbers, age, complications, polysomnography, and quality of life. We pooled postoperative changes in apnea hypopnea index (AHI) for meta-analysis. We applied Methodological Index for Nonrandomized Studies (MINORS) criteria to assess study quality.
RESULTS
The initial search yielded 169 studies. We included 68 patients from eight studies with moderate to high risk of bias. Six studies reported on complications and 12 of 51 patients (24%) had at least one. Velopharyngeal insufficiency was the most commonly reported complication (7/51, 14%). We included seven studies in meta-analysis. Mean postoperative improvement in AHI was 7.7 (95% CI: 4.9-10.5). Postoperatively 20% (95% CI: 3%-43%) had resolution of OSA with AHI < 1.5 while 67% (95% CI: 50%-82%) had improvement from severe/moderate OSA to mild/resolved (AHI < 5). Two studies evaluated quality of life and demonstrated improvement.
CONCLUSIONS
Children with PWS undergoing adenotonsillectomy for OSA have a substantial risk of postoperative complications that may require additional interventions, especially velopharyngeal insufficiency. Despite improvements in polysomnography and quality of life, many patients had residual OSA. This information can be used to counsel families when considering OSA treatment options. Laryngoscope, 131:898-906, 2021.
Topics: Adenoidectomy; Child; Humans; Polysomnography; Postoperative Complications; Prader-Willi Syndrome; Quality of Life; Sleep Apnea, Obstructive; Tonsillectomy
PubMed: 33026674
DOI: 10.1002/lary.28922 -
European Journal of Medical Genetics Jan 2022Prader-Willi Syndrome (PWS) is a multi-system genetically determined neurodevelopmental disorder and the commonest cause of syndromal obesity. The development of... (Meta-Analysis)
Meta-Analysis
Prader-Willi Syndrome (PWS) is a multi-system genetically determined neurodevelopmental disorder and the commonest cause of syndromal obesity. The development of hyperphagia in early childhood is part of the phenotype arising as a result of an impaired neural response to food intake and the inability to regulate food intake in line with energy needs. Severe obesity develops if access to food is not controlled. In this review we evaluate the evidence for increased morbidity and mortality in PWS in order to establish the extent to which it is directly related to the obesity; a consequence of the eating behaviour itself independent of obesity; or associated with other characteristics of the syndrome. Medline, Cochrane, PsychINFO, CINAHL, Web of Science and Scopus databases were used to systematically identify published material on PWS and hyperphagia and syndrome-related morbidity and mortality. One hundred and ten key papers were selected. Data on 500 people with PWS indicated that the average age of death was 21 years and obesity was, as expected, a significant factor. However, the behaviour of hyperphagia itself, independent of obesity, was also important, associated with choking, gastric rupture, and/or respiratory illness. Other syndrome-related factors increased the risk for, and seriousness of, co-morbid illness or accidents. We conclude that improving life-expectancy largely depends on managing the immediate non-obesity and obesity-related consequences of the hyperphagia, through improved support. The development of new treatments that significantly reduce the drive to eat are likely to decrease morbidity and mortality improving quality of life and life expectancy.
Topics: Humans; Hyperphagia; Morbidity; Prader-Willi Syndrome
PubMed: 34748997
DOI: 10.1016/j.ejmg.2021.104379 -
Journal of the American Academy of... Sep 2020The behavioral phenotype of neurogenetic disorders associated with intellectual disability often includes psychiatric comorbidity. The objectives of this systematic... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The behavioral phenotype of neurogenetic disorders associated with intellectual disability often includes psychiatric comorbidity. The objectives of this systematic review and meta-analysis were to systematically review the prevalence of psychiatric disorders and symptoms in children and adolescents with these disorders and compare phenotypic signatures between syndromes.
METHOD
MEDLINE and PsycINFO databases were searched for articles from study inception to December 2018. Eligible articles were peer reviewed, were published in English, and reported prevalence data for psychiatric disorders and symptoms in children and adolescents aged 4 to 21 years using a formal psychiatric assessment or a standardized assessment of mental health symptoms. Pooled prevalence was determined using a random-effects meta-analysis in studies with sufficient data. Prevalence estimates were compared with general population data using a test of binomial proportions.
RESULTS
Of 2,301 studies identified for review, 39 articles were included in the final pool, which provided data on 4,039 children and adolescents. Ten syndromes were represented, and five were predominant: Down syndrome, 22q11.2 deletion syndrome, fragile X syndrome, Williams syndrome, and Prader-Willi syndrome. The Child Behavior Checklist was the most commonly used assessment tool for psychiatric symptoms. The pooled prevalence with total scores above the clinical threshold was lowest for Down syndrome (32% [95% confidence interval, 19%-44%]) and highest for Prader-Willi syndrome (74% [95% CI, 65%-82%]) with each syndrome associated with significantly higher prevalence than in the general population. Parallel trends were observed for the internalizing and externalizing domains and social subscale scores.
CONCLUSION
Differential vulnerability for psychiatric phenotype expression across the disorders was observed. Syndromes with higher levels of social ability or competence appear to offer relative protection against developing psychopathology. This preliminary finding merits further exploration.
Topics: Adolescent; Adult; Child; Child, Preschool; Comorbidity; Humans; Intellectual Disability; Mental Disorders; Mental Health; Prader-Willi Syndrome; Prevalence; Young Adult
PubMed: 31945412
DOI: 10.1016/j.jaac.2020.01.006 -
Epilepsy & Behavior : E&B Jun 2024To estimate the prevalence of epilepsy and febrile seizures and their association with genotype, i.e., 15q11-q13 deletions, uniparental chromosome 15 disomy (UPD) and... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To estimate the prevalence of epilepsy and febrile seizures and their association with genotype, i.e., 15q11-q13 deletions, uniparental chromosome 15 disomy (UPD) and other mutations, in the population with Prader-Willi syndrome (PWS).
METHODS
A systematic search of Medline, Scopus, Web of Science and the Cochrane Library was conducted. Studies estimating the prevalence of seizures, epilepsy and febrile seizures in the PWS population were included. Meta-analyses of the prevalence of epilepsy and febrile seizures and their association with genotype using the prevalence ratio (PR) were performed.
RESULTS
Fifteen studies were included. The prevalence of epilepsy was 0.11 (0.07, 0.15), similar to the prevalence of febrile seizures, with a prevalence of 0.09 (0.05, 0.13). The comparison "deletion vs. UPD" had a PR of 2.03 (0.90, 4.57) and 3.76 (1.54, 9.18) for epilepsy and febrile seizures.
CONCLUSIONS
The prevalence of seizure disorders in PWS is higher than in the general population. In addition, deletions in 15q11-q13 may be associated with a higher risk of seizure disorders. Therefore, active screening for seizure disorders in PWS should improve the lives of these people. In addition, genotype could be used to stratify risk, even for epilepsy, although more studies or larger sample sizes are needed.
Topics: Humans; Prader-Willi Syndrome; Epilepsy; Prevalence; Genotype; Chromosomes, Human, Pair 15
PubMed: 38663143
DOI: 10.1016/j.yebeh.2024.109803 -
Journal of Pediatric Endocrinology &... Jun 2022Registries are considered valuable data sources for identification of pediatric conditions treated with growth hormone (GH), and their follow-up. Currently, there is no... (Review)
Review
BACKGROUND
Registries are considered valuable data sources for identification of pediatric conditions treated with growth hormone (GH), and their follow-up. Currently, there is no systematic literature review on the scope and characteristics of pediatric GH registries. Therefore, the purpose of this systematic review is to identify worldwide registries reported on pediatric GH treatment and to provide a summary of their main characteristics.
CONTENT
Pediatric GH registries were identified through a systematic literature review. The search was performed on all related literature published up to January 30th, 2021. Basic information on pediatric GH registries, their type and scope, purpose, sources of data, target conditions, reported outcomes, and important variables were analyzed and presented.
SUMMARY
Twenty two articles, reporting on 20 pediatric GH registries, were included in this review. Industrial funding was the most common funding source. The main target conditions included in the pediatric GH registries were: growth hormone deficiency, Turner syndrome, Prader Willi syndrome, small for gestational age, idiopathic short stature, and chronic renal insufficiency. The main objectives in establishing and running pediatric GH registries were assessing the safety and effectiveness of the treatment, describing the epidemiological aspects of target growth conditions and populations, serving public health surveillance, predicting and measuring treatment outcomes, exploring new and useful aspects of GH treatment, and improving the quality of patient care.
OUTLOOK
This systematic review provides a global perspective on pediatric GH registries which can be used as a basis for the design and development of new GH registry systems at both national and international levels.
Topics: Child; Dwarfism, Pituitary; Growth Disorders; Growth Hormone; Human Growth Hormone; Humans; Registries
PubMed: 35567286
DOI: 10.1515/jpem-2022-0045 -
Clinical Endocrinology Feb 2022The mainstay management of hyperphagia and obesity in Prader-Willi syndrome (PWS) relies on dietary restrictions, strict supervision and behavioural modifications, which...
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/m , 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.
Topics: Adolescent; Adult; Diabetes Mellitus, Type 2; Glucagon-Like Peptide 1; Glucagon-Like Peptide-1 Receptor; Glycemic Control; Humans; Liraglutide; Prader-Willi Syndrome; Young Adult
PubMed: 34448208
DOI: 10.1111/cen.14583 -
Clinical Endocrinology Apr 2021Despite clear benefits in the management of children with Prader-Willi syndrome (PWS), the role of growth hormone (GH) in adults is unclear. The aim of this study was to...
OBJECTIVE
Despite clear benefits in the management of children with Prader-Willi syndrome (PWS), the role of growth hormone (GH) in adults is unclear. The aim of this study was to conduct a systematic review to evaluate the effects of GH on body composition, bone health and cardiovascular health in adults with PWS.
DESIGN
A systematic computerized literature search of the PubMed database was conducted by two independent reviewers. Inclusion criteria were individuals over the age of 16 years with a genetic diagnosis of PWS who had received GH therapy, together with assessment of body composition, bone health or cardiovascular health.
RESULTS
Twenty full-text papers met the inclusion criteria, encompassing 364 unique patients. No differences in body mass index (BMI) were noted, although 2 studies reported increased BMI after GH cessation. Data demonstrated statistically significant increases in lean body mass and reductions in percentage fat mass. Studies reported inconsistent effects of GH on cholesterol and echocardiography parameters. No studies reported differences in bone mineral density, although one reported improved bone geometry. Minor adverse events including pretibial oedema, headache and transient impaired glucose tolerance were reported in 7 studies.
CONCLUSIONS
These data suggest that GH is safe and well tolerated in adults with PWS, with evidence of improvement in body composition. Further longitudinal studies are still required to investigate the effects of GH on bone and cardiovascular health. Where GH is used in adults with PWS, this should be managed by a specialist multidisciplinary team with regular monitoring initiated.
Topics: Adolescent; Adult; Body Composition; Bone Density; Child; Growth Hormone; Human Growth Hormone; Humans; Prader-Willi Syndrome
PubMed: 33296095
DOI: 10.1111/cen.14372 -
Pediatric Obesity May 2023A systematic review of value and preference studies conducted in children and their caregivers related to the estimated benefits and harms of interventions for managing... (Review)
Review
OBJECTIVE
A systematic review of value and preference studies conducted in children and their caregivers related to the estimated benefits and harms of interventions for managing paediatric obesity.
METHODS
We searched Ovid Medline (1946-2022), Ovid Embase (1974-2022), EBSCO CINAHL (inception to 2022), Elsevier Scopus (inception to 2022), and ProQuest Dissertations & Theses (inception to 2022). Reports were eligible if they included: behavioural and psychological, pharmacological, or surgical interventions; participants between (or had a mean age within) 0-18 years old with overweight or obesity; systematic reviews, primary quantitative, qualitative, or mixed/multiple methods studies; and values and preferences as main study outcomes. At least two team members independently screened studies, abstracted data, and appraised study quality.
RESULTS
Our search yielded 11 010 reports; eight met the inclusion criteria. One study directly assessed values and preferences based on hypothetical pharmacological treatment for hyperphagia in individuals with Prader-Willi Syndrome. Although not having reported on values and preferences using our a priori definitions, the remaining seven qualitative studies (n = 6 surgical; n = 1 pharmacological) explored general beliefs, attitudes, and perceptions about surgical and pharmacological interventions. No studies pertained to behavioural and psychological interventions.
CONCLUSION
Future research is needed to elicit the values and preferences of children and caregivers using the best available estimates of the benefits and harms for pharmacological, surgical, and behavioural and psychological interventions.
Topics: Child; Humans; Infant, Newborn; Infant; Child, Preschool; Adolescent; Pediatric Obesity; Overweight; Hyperphagia
PubMed: 36810978
DOI: 10.1111/ijpo.13006