Similar content being viewed by others In brief PWS is a common and complex disorder affecting multiple systems Early diagnosis is important to effective long-term management Hypotonia, beginning prenatally, causes poor feeding and development in the perinatal period and infancy If untreated obesity typically begins after 12 years of age and is later exacerbated by hyperphagia with lack of satiety The major cause of morbidity and mortality is morbid obesity Obesity can be controlled externally by diet restrictions and behavior modification Parental education about appropriate parenting techniques (structure and consistency) for the behavioral and eating issues of PWS correlates with prognosis Growth hormone treatment improves growth, physical phenotype and body composition Clinical diagnostic criteria have been developed, but must be confirmed with genetic testing because of clinically overlapping disorders, particularly as an infant Over 99% can be diagnosed with a simple molecular test, DNA methylation analysis Three major genetic causes exist: 57 Mb deletion of the paternally inherited chromosomal 15q11.2q13 region, maternal uniparental disomy 15, and a defect in the imprinting process in the 15q11.2q13 region on the paternally inherited chromosome
Stability of GSH under large input fluctuations Hepatocytes are seldom at steady state [21] because they receive large protein inputs during and shortly after meals and relatively little protein input between meals
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doi: 10.1002/(SICI)1097-4547(19980901)53:5 3.0.CO;2-8 71
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