Respiratory complications of children with Prader-Willi syndrome

Prader- Willi Syndrome (PWS) is a multisystem genetic disorder. In infancy, patients are typically hypotonic and may fail to thrive, but as they grow older, many become obese and develop obstructive sleep apnoea (OSA). We aimed to determine the prevalence of the respiratory complications seen in our cohort of patients referred from a regional PWS clinic in London.We performed a retrospective casenote review of the 49 children with PWS seen from 2010 to 2016, highlighting conditions that could influence their respiratory status.The median age was 5.2[0.4-15.8][range]yrs, 27/49(55%)male, BMI z score 0.7[2.1] mean[SD]. Only 7/49(14%) were overweight/ obese. 75% are on growth hormone (GH). 4/49(8%) had a history of recurrent chest infections: 1 child isolated P. aeruginosa from airway secretions and received oral ciprofloxacin and nebulised colomycin in an attempt at eradication. 11/49(22%) children were at risk of aspiration on videofluoroscopy: 1 was treated with thickened fluids, 7 required nasogastric (ng) tube feeds, 3 have had gastrostomies. Of the 7/11 who needed ng tube feeds, 3 improved and resumed a normal diet within 1 year. 5/49(10%) have scoliosis for which 2 have required spinal surgery. 1 is using CPAP for OSA, 3 NIV for nocturnal hypoventilation and 1 is on nocturnal oxygen for central apnoeas.With early initiation of GH and better clinical management, obesity is not as common in PWS as previously described. Younger children can have problems with recurrent chest ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: 7.1 Paediatric Respiratory Physiology and Sleep Source Type: research

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Conclusions: C. indologenes and E. meningosepticum are rare, nosocomial- or healthcare-associated pediatric bacteremia pathogens. Bacteremia was associated with young age, but in contrast to the literature, the majority of our cases were older than the neonatal age period. In addition, they were associated with central line-associated bloodstream infection and malignancy. The most adequate antibiotics according to resistance patterns were ciprofloxacin, trimethoprim-sulfamethoxazole and piperacillin-tazobactam.
Source: The Pediatric Infectious Disease Journal - Category: Infectious Diseases Tags: Original Studies Source Type: research
Our world is amidst a crucial overweight and obesity pandemic that continues to grow, with 2 billion of the world ’s population overweight and approximately 700 million adults with obesity.1 Once thought to be a disease of the rich and affluent, weight-related and metabolic diseases are on the rise across all socioeconomic classes and residential settings. The enduring physiologic and hormonal changes that oc cur with weight gain make sustainable weight loss with just diet and exercise, commonly known as lifestyle modification, not feasible for most people.
Source: Gastrointestinal Endoscopy - Category: Gastroenterology Authors: Tags: Original article Source Type: research
In this study, we explored the effect of β -Lapachone ( β L), a compound obtained from the bark of the lapacho tree, against obesity. In vivo administration of β L into either high fat diet (HFD)-induced obese C57BL6 mice and genetically obese Lepr -∕- mice prevented body weight gain, which was associated with tissue weight loss of white adipose tissue (WAT). In addition, β L elevated thermogenic proteins including uncoupling protein 1 (UCP1) and mitochondrial count in BAT and human adipose tissue-derived mesenchymal stem cells (hAMSCs). β L also induced AMP-activated protein kinase (AMPK) phosph...
Source: The American Journal of Chinese Medicine - Category: Complementary Medicine Authors: Tags: Am J Chin Med Source Type: research
In conclusion, yacon syrup has a postprandial decreasing effect glucose and insulin concentrations in adult women. This effect was not evident for triglyceride concentration.Clinical trial registry: RBR-33wf46. Available in: http://www.ensaiosclinicos.gov.br/rg/RBR-33wf46/Graphical abstract
Source: Food Research International - Category: Food Science Source Type: research
Authors: Gan X, Xu W, Yu K Abstract Background: With the rapid economy development of China, the overweight and obesity rates of school-age children and adolescents in China have been on the rise. The purpose of this study is to explore how powerful an influence economic growth has on the weight of children and adolescents and examine the relationship between China's macroeconomic development and weight of children and adolescents in the past 30 years using provincial panel data collected from more than 1 million children and adolescents. Methods: Panel data from seven longitudinal surveys (1985, 1991, 1995, 2000, ...
Source: Childhood Obesity - Category: Eating Disorders & Weight Management Tags: Child Obes Source Type: research
Authors: McMaster CM, Gow ML, Neal R, Alexander S, Baur LA, Cohen J Abstract High rates of attrition from pediatric weight management services are well documented in the literature; however, studies reporting on acceptability of "real-world" services have not previously been reviewed. This narrative synthesis aimed to summarize quantitative and qualitative research reporting on satisfaction of families who attended a secondary or tertiary pediatric weight management service. Electronic databases were searched for studies of family and/or patient satisfaction with hospital-based pediatric weight management...
Source: Childhood Obesity - Category: Eating Disorders & Weight Management Tags: Child Obes Source Type: research
Publication date: Available online 17 October 2019Source: Physiology &BehaviorAuthor(s): Janelle A Skinner, Manohar L Garg, Christopher V Dayas, Tracy L BurrowsABSTRACTThe neuropeptide oxytocin is best known for its role during parturition and the milk-let down reflex. Recent evidence identifies a role for oxytocin in eating behaviour. After oxytocin administration, caloric intake is reduced with stronger inhibitory effects in individuals with obesity. Whether the experience of visual food cues affects secretion or circulating levels of oxytocin is unknown. This pilot study had three aims: 1) to measure fasting appetit...
Source: Physiology and Behavior - Category: Physiology Source Type: research
In this study, we purified and identified anti-obesity peptides from hazelnut protein hydrolysates by chromatography and liquid chromatography-tandem mass spectrometry (LC-MS/MS). Subsequently, we evaluated the inhibitory effect of the synthetic peptide on adipogenesis in 3T3-L1 adipocytes by Oil Red O staining, reverse transcription polymerase chain reaction (RT-PCR) and western blot. The results showed that a novel synthetic pentapeptide, Arg-Leu-Leu-Pro-His (RLLPH), derived from the C3 fraction, attenuated adipogenesis by downregulating the expression of several mRNAs related to adipogenesis, including peroxisome prolif...
Source: Journal of Bioscience and Bioengineering - Category: Biomedical Science Source Type: research
Authors: Corfitsen HT, Krantz B, Larsen A, Drago A Abstract OBJECTIVE: Antipsychotics often induce excessive weight gain. We hypothesized that individuals with genetic variations related to known obesity-risk genes have an increased risk of excessive antipsychotic-induced weight gain. This hypothesis was tested in a subset of the CATIE trial data-set. METHODS: The CATIE trial compared effects and side effects of five different antipsychotics through an 18 month period. Based on the maximum weight gain recorded, excessive weight gain was defined as>7% weight-gain. Cytoscape and GeneMania were instrumental in ...
Source: Acta Neuropsychiatrica - Category: Psychiatry Tags: Acta Neuropsychiatr Source Type: research
Publication date: Available online 17 October 2019Source: Respiratory Physiology &NeurobiologyAuthor(s): Yu-Chi Kuo, Hui-Lin Chang, Ching-Feng Cheng, Toby Mündel, Yi-Hung LiaoAbstractThis investigation examined the effects of a six-week inspiratory resistance training (IRT) on metabolic health biomarkers, pulmonary function, and endurance in obese individuals. Twenty-eight obese adults (BMI > 27 kg/m2 Taiwan obesity criteria) were randomly assigned to either IRT (IRT; N = 16) or sham control (PLA; N = 12). The training parameters (twice/day; 3 days/week; 30 breat...
Source: Respiratory Physiology and Neurobiology - Category: Respiratory Medicine Source Type: research
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