, Eun-Kyoung Pang , Jieun Jang, Nayoung Kim Citations
, Ji Eun Moon, Yeo Jin Jung, Young Ae Yu Prader-Willi syndrome (PWS), which is considered the most common genetic form of obesity, results from the absence of imprinted genes in the paternally derived PWS critical region located on chromosome 15q11.2−13. Infants with PWS exhibit poor sucking, neonatal hypotonia, and delayed motor milestones. These patients begin to experience hyperphagia and obesity from 2 to 3 years of age. PWS is a multisystemic disorder, and its clinical manifestations include developmental delay/intellectual disability, behavioral problems, dysmorphic facial features, short stature, scoliosis, and endocrine abnormalities such as hypogonadism, growth hormone deficiency, hypothyroidism, and central adrenal insufficiency. Although the underlying mechanism of hyperphagia is not completely understood, hypothalamic and endocrine dysregulation is believed to be responsible for the lack of satiety and abnormal food-seeking behaviors that lead to severe obesity. The management of PWS requires a multidisciplinary team approach. Early diagnosis and comprehensive early intervention are essential to prevent the development of obesity-related morbidities, including metabolic syndrome, diabetes mellitus, obstructive sleep apnea, respiratory failure, pulmonary hypertension, and cardiovascular complications. Although several clinical trials have been conducted on the pharmacologic treatment of obesity in PWS, no drugs have demonstrated a consistently beneficial effect to date. Nevertheless, ongoing research efforts should be directed toward understanding the mechanism of the unique obesity phenotype of PWS and developing pharmacological therapies.
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, Sung Yoon Cho Prader-Willi syndrome (PWS) is a neurodevelopmental disorder caused by the absence of paternally expressed imprinted genes on chromosome 15q11–13. Individuals with PWS typically experience feeding difficulties and a lack of appetite in infancy, followed by weight gain, uncontrolled appetite, and a lack of satiety. Hyperphagia in PWS is exacerbated by impaired satiety, low energy expenditure, and intellectual difficulties, including obsessive-compulsive disorder and/or autistic behaviors. Without rigorous external management of their eating behaviors, patients with PWS become severely obese and are at a higher risk of obesity-related morbidities, such as type 2 diabetes, obstructive sleep apnea, and hypertension. Moreover, the main causes of death for PWS are obesity-related comorbidities, such as renal failure, pulmonary embolism, and respiratory and heart failure. Clinical experiences with different supplements, diets, and other methods have not been encouraging. However, therapeutic options for patients with PWS may be improving, based on recent clinical trials for a number of medications. This report reviews the causes and management of hyperphagia, as well as previous and recent clinical trials aimed at treating hyperplasia in PWS. We are optimistic that the novel treatments currently in development will help alleviate the complex metabolic issues associated with PWS.
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Metabolic syndrome (MetS) is a cluster of metabolic abnormalities that include hypertension, altered glucose metabolism, dyslipidemia, and abdominal obesity and is strongly associated with an increased risk for diabetes and cardiovascular disease onset in obese adults and children. A progressively greater number of children and adolescents are being affected by this syndrome due to the constant increase in the prevalence of obesity. Like obesity, childhood MetS highly tracks to adulthood. The pathogenesis of MetS includes the interaction between obesity, insulin resistance, and inflammation. Early diagnosis and intervention are important in order to conduct lifestyle modification. In this article, we review the definition and pathophysiology of MetS, the importance of screening, and prevention and treatment options for MetS in childhood.
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Bone marrow adipose tissue (BMAT) increases with aging and once disregarded as a passive marrow space filler. However, accumulating evidence suggests that BMAT is an active modulator of bone, hematopoiesis, and metabolism. Characterization of BMAT in molecular and cellular levels identified that it is distinct from white or brown adipose tissue. This review summarizes current knowledge on changes of BMAT under physiological and pathophysiological conditions of bone and marrow. Expansion of BMAT is closely linked with increased fracture risk, therefore regulation of BMAT can be considered as a novel therapeutic approach to enhance bone strength. Regarding hematopoiesis, increase in BMAT is negatively associated with the marrow function, but it is indispensable for maintaining myelopoiesis in acute myeloid leukemia. In addition, BMAT expansion is paradoxically identified in obesity as well as anorexia nervosa. It is considered that BMAT performs a different function in different nutritional states. Future studies would involve more detailed research about regulatory factors of BMAT and its functions in health and diseases. Enhancing our understanding about BMAT would open a new avenue for combating BMAT-related diseases.
, Ki-Nam Shim, Won Young Cho, Chan Young Kim, Hyeon-Ju Kang, Mi Yeon Kim, So Young Ahn, Yoon Pyo Lee, Hyoung Won Cho, Sung Ae Jung, Joo-Ho Lee Laparoscopic sleeve gastrectomy can reduce morbidity and mortality in patients with morbid obesity, but it can cause complications such as a gastrointestinal leak. A 30-year-old morbidly obese female who had type 2 diabetes mellitus and hypertension with estimated body mass index of 40.2 kg/m2 was admitted. Laparoscopic sleeve gastrectomy was performed. On postoperative day 19, a leak was suspicious on physical examination and radiologic findings. Conservative management was performed, but the patient was hemodynamically unstable and imminently septic. After laparoscopic drainage procedure, esophagogastroduodenoscopy was performed and revealed the fistula opening at staple line just below gastroesophageal junction. Fibrin tissue adhesive was injected around the fistula and the esophageal covered stent was inserted to cover the leak. At 14th days after stent insertion, the barium study confirmed no more leak. In this case, we experienced that the esophageal stent insertion with fibrin tissue adhesive injection may reduce recovery time of the fistula developed after laparoscopic sleeve gastrectomy.
Obesity, the most common health problem facing children, is known to have been ascribed to multifactors. Our research is aimed at finding out if there exists any relation of children's obesity with their family and also with their daily habits.
The study included 145 obese children and 44 non-obese children, who visited our pediatric clinic from January 2006 to December 2008. The children were divided into three groups according to body mass index(BMI)(group I:non-obese control children, group II:children with BMI between 85 and 94 percentile, group III:children with BMI above 95 percentile). Research was performed in three groups by measuring of body weight, height and questionnaires.
There was no significant difference in sex and age. The parental BMI of the obese children were higher than those of non-obese children. Obesity of children was more highly related to maternal BMI than partenal BMI. Birth weight and birth order in the obese children showed no remarkable difference from those of non-obese children. The sleeping hours of the obese children were much longer than those of non-obese children. Television viewing hours of the obese children showed no remarkable difference from those of non-obese children. Family members with group III children had many complications which developed from obesity.
The development of obesity in children is attributed to the hereditary factors of their parents and daily habits such as sleeping hours. Therefore, family-based weight control is important to treat childhood obesity.
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