• Clinical and Experimental Pediatrics
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• 제54권2호
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• pp.55-63
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• 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused by genomic imprinting. It can occur via 3 main mechanisms that lead to the absence of expression of paternally inherited genes in the 15q11.2-q13 region: paternal microdeletion, maternal uniparental disomy, and an imprinting defect. Over 99% of PWS cases can be diagnosed using DNA methylation analysis. Early diagnosis of PWS is important for effective long-term management. Growth hormone (GH) treatment improves the growth, physical phenotype, and body composition of patients with PWS. In recent years, GH treatment in infants has been shown to have beneficial effects on the growth and neurological development of patients diagnosed during infancy. There is a clear need for an integrated multidisciplinary approach to facilitate early diagnosis and optimize management to improve quality of life, prevent complications, and prolong life expectancy in patients with PWS.

• Fisheries and Aquatic Sciences
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• 제15권4호
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• pp.305-311
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• 2012

We investigated compensatory growth of Nile Tilapia Oreochromis niloticus in structural size and live weight in response to different deprivation periods and refeeding. Four treatments were assigned randomly to fish in 12 glass tanks, with each treatment performed in triplicate. The control group was fed to satiation three times a day throughout the experiment. The other three treatment groups were starved for 1 week (S1), 2 weeks (S2), or 4 weeks (S4) and then fed until the end of the experiment. After the experiment, no significant differences were observed among S1, S2, and the control group in average weight or length, whereas the weight and length of S4 were significantly reduced. Relative condition factors of the three starved groups decreased significantly until the end of the restricted period but recovered rapidly after refeeding. The specific growth rate in weight ($SGR_W$) of the three restricted groups recovered quickly upon refeeding and were significantly higher than the control group, but these differences disappeared gradually until the end of the experiment. No significant difference in specific growth rate in length ($SGR_L$) was noted between the control group and the three restricted groups after refeeding. All three groups showed hyperphagia for a short period upon refeeding, and no statistical differences were observed in feeding efficiency among the four groups.

• 대한간호학회지
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• 제46권1호
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• pp.109-117
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• 2016

Purpose: The aim of this study was to develop a Korean version of Night Eating Questionnaire (KNEQ) and test its psychometric properties and evaluate items according to item response theory. Methods: The 14-item NEQ as a measure of severity of the night eating syndrome was translated into Korean, and then this KNEQ was evaluated. A total of 1171 participants aged 20 to 50 completed the KNEQ on the Internet. To test reliability and validity, Cronbach's alpha, correlation, simple regression, and factor analysis were used. Each item was analyzed according to Rasch-Andrich rating scale model and item difficulty, discrimination, infit/outfit, and point measure correlation were evaluated. Results: Construct validity was evident. Cronbach's alpha was .78. The items of evening hyperphagia and nocturnal ingestion showed high ability in discriminating people with night eating syndrome, while items of morning anorexia and mood/sleep provided relatively little information. The results of item analysis showed that item2 and item7 needed to be revised to improve the reliability of KNEQ. Conclusion: KNEQ is an appropriate instrument to measure severity of night eating syndrome with good validity and reliability. However, further studies are needed to find cut-off scores to screen persons with night eating syndrome.

• 대한장애인치과학회지
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• 제11권2호
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• pp.67-71
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• 2015

본 논문에서 소개된 환자는 전형적인 PWS 증상인 섭식 장애, 타액분비감소, 지적장애, 비만과 같은 특성이 관찰되었다. 전신마취 하에서 적극적인 치과 치료를 시행하였고 보호자에게 비만 및 치아 우식을 예방하기 위해 탄수화물과 당분 섭취를 제한하는 식습관에 대한 교육을 시행하였다. 환자의 내원 횟수를 늘려 치과 치료를 위한 전신마취까지 발전하지 않도록 정기적인 관리가 필요하다.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.42-44
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• 2018

Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

• 한국임상약학회지
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• 제32권4호
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• pp.336-351
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• 2022

Background: Prader-Willi Syndrome (PWS) is a rare genetic disorder. To improve the health deterioration of PWS, investigating optimal treatment options for PWS is required. Thus, we aimed to evaluate the efficacy of pharmacotherapies compared with supportive care or placebos in patients with PWS. Methods: PubMed and EMBASE databases were used to search for randomized controlled trials (RCTs) evaluating the efficacy of pharmacotherapy in PWS patients. Only RCTs that evaluating the efficacy of pharmacotherapy in PWS patients were retrieved. Results: A total of 26 studies were included to evaluate body composition, hormones, glucose levels and hyperphagia behavioral status. Pharmacological treatment group showed a significant decrease of body fat (mean difference (MD): -6.32, 95% confidence interval (CI): -10.58 to -2.06, p=0.004), a significant increase of lean body mass (LBM) (MD: 1.86, 95% CI: 1.43 to 2.30, p<0.00001) and insulin-like growth factor 1 (IGF-1) levels (MD: 241.62, 95% CI: 68.59 to 414.64, p=0.006) compared with the control group. Nevertheless, based on other outcomes evaluated by the current systematic review, pharmacological options showed different efficacy in treating PWS. Conclusion: Pharmacological therapies were effective to decrease significantly in body fat and increase significantly on LBM and IGF-1 levels in patients with PWS. However, still, individualized therapies should be considered in real-world practice in PWS treatment.

• 대한장애인치과학회지
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• 제10권1호
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• pp.26-30
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• 2014

프라더 윌리 증후군과 원인을 알 수 없는 심낭 삼출을 앓고 있는 3세 남환이 치아우식을 주소로 내원하여 임상 구강 검사 결과 다발성 우식증과 구강 전반에 걸친 치아 마모증이 관찰되었다. 약 10개월간 보존적인 수복치료와 구강 위생관리를 시도하였으나, 치아 우식의 빠른 진행과 환아의 협조도 부족으로 전신마취 하 우식 치료를 시행하였다.

• Asian-Australasian Journal of Animal Sciences
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• 제22권6호
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• pp.827-835
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• 2009

Insulin has crucial roles in energy metabolism in all mammals but has been less studied in ruminants. An experiment was conducted to investigate the effects of hypoinsulinemia induction on appetite, performance, carcass composition and blood metabolite levels in sheep. Treatments were intravenous injection of four doses of streptozotocin; 0, 25, 50 and 75 mg/kg BW named C, L, M and H, respectively. Twenty male lambs were divided into four treatment groups. Animals in group H could not continue the experiment because of abnormalities. The duration of the experiment was eight consecutive weeks, and injection was performed at the end of week 3. Feed and water intakes were measured weekly and weight changes of animals were recorded and used for calculation of other growth parameters. Blood samples were collected weekly via venipuncture at fasting and 2.5 h post-prandial and analyzed for hormones and blood metabolites. Results showed a marked hypoinsulinemia in group M with significant decrease in fasted and postprandial insulin concentrations and also fasted leptin concentrations vs. the control group C (p<0.05). Group M showed significant increases in blood glucose, triglycerides, cholesterol, total protein, blood urea nitrogen and ketone body levels vs. group C (p<0.05). After injection, animals in group M showed diabetic hyperphagia and enhanced water intake as compared to group C (p<0.05) but, despite increased feed intake, they did not gain more weight than controls (p<0.05), and consequently, their feed conversion ratio was greater. Protein and fat contents of meat and liver were not significantly different among groups (p>0.05). In conclusion, the results suggested a regulatory role of insulin in energy metabolism of ruminants by exerting two opposing effects; central catabolic and peripheral anabolic.

• 한국식품조리과학회지
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• 제18권3호
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• pp.300-308
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• 2002

The purpose of this study was to investigate the effect of chloroform(CHC1$_3$) fraction of Alisma canaliculatum All. Braun et Bouche(Ac) with selenium(Se) on the plasma glucose and lipid levels in streptozotocin(STZ)-induced diabetic rats. Male Sprague-Dawley rats were divided into five groups. The normal and diabetic rats were separated into four groups: the STZ-control group, the Ac group, the Ac-Se group and the Se group. Diabetes was induced in the male rats by an injection of STZ into the tail vein at a dose of 45 mg/kg. The CHCl$_3$fraction of Ac(250 mg/kg) was administered orally for 14 days. The supplementation was achieved with the AIN-93 recommended diet by adding 2 mg/kg diet of selenium as Na$_2$SeO$_3$. which was prepared freshly everyday. The body weight, hematocrit(Hct), glucose, insulin, cholesterol, HDL-cholesterol, triglyceride(TG) and free fatty acids(FFA) concentrations in plasma were measured. The aminotransferase activities were also analyzed. The changes of body weight in the experimental groups were not significantly different from that of the STZ-control group, but diabetes hyperphagia accompanied changes with body weight loss in Ac-Se group. The levels of Plasma cholesterol were not significantly different among the experimental groups. The concentrations of FFA in the Ac-Se group increased significantly compared with the STZ-control group. The effect of Se alone significantly increased aspartate aminotransferase activity and alanine aminotransferase activity. The results showed that the treatment of CHCl$_3$fraction of Ac in combination with Se has no synergistic effect. There was a tendency for the plasma glucose levels to decrease when Se was administered into diabetic rats. Supplementation of Se in diabetic rats did not elicit a significant increase in plasma insulin levels and exhibited hypotriglyceridemic effect.

• Tuberculosis and Respiratory Diseases
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• 제51권6호
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• pp.603-608
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• 2001

저자들은 속립성 폐결핵과 연관되어 급성 경과를 보인 결핵성 뇌수막뇌염 환자에서 뇌혈관 자기공명영상으로 혈관염 소견을 확인하고, 스테로이드의 투여와 중단, 재투여에 따라 뇌증의 증세가 뚜렷히 변하는 임상경과, 증상이 호전된 후 추적한 뇌혈관 자기공명영상에서 혈관 영상이 정상화된 것을 확인할 수 있었던 사례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.