Outcome of Prader-Willi Syndrome at Queen Sirikit National Institute of Child Health: An Eight-year Retrospective Study
Keywords:
Comorbidity, Obesity, Outcome, Prader-Willi syndromeAbstract
Background: Prader-Willi syndrome is a genetic disorder caused by absent of expression of genes on chromosome 15. Delayed development, obesity and its complications are found in these patients during childhood. There is few information about Prader-Willi syndrome in Thailand.
Objective: To study clinical manifestations, growth, nutritional status, development, comorbidities, illness, complications and treatment in patient with Prader-Willi syndrome at Queen Sirikit National Institute of Child Health.
Method: A retrospective descriptive study were performed by gathering data from medical records of Prader-Willi syndrome patients at Queen Sirikit National Institute of Child Health between January 2013 and December 2020.
Results: Among 46 patients, mean age of diagnosis was 2 years (most in infantile, 58.7%). The most first clinical presentation was hypotonia (43.5%). Other common clinical manifestations such as global developmental delay and obesity were found in all patient
and 83.9% respectively. There were two patients who were malnutrition. Both of them had early diagnosis and dietary control.
Conclusions: Majority of Prader-Willi syndrome patients in this study had global developmental delay and obesity. Early diagnosis and dietary control might decrease obesity and its complications in Prader-Willi syndrome.
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