| Physical health problems in adults with Prader-Willi syndrome. | |
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MedLine Citation:
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PMID: 21834028 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Prader-Willi syndrome (PWS) is a genetic disorder which is characterized by severe hypotonia and feeding problems in early infancy. In later childhood and adolescence, this is followed by hyperphagia and extreme obesity if the diet is not strictly controlled. Data on physical health problems in adults with PWS are scarce. We report on the prevalence of physical health problems in a Dutch cohort of adults with PWS in relation to age, BMI, and genetic subtype. Participants (n = 102) were retrieved via the Dutch Prader-Willi Parent Association and through physicians specializing in persons with intellectual disabilities (ID). Details regarding physical health problem spanning the participants' lifespan were collected from caretakers through semi-structured interviews. Cardiovascular problems included diabetes mellitus, hypertension, and cerebrovascular accidents. Respiratory infections were frequent in adulthood. In males, cryptorchidism was almost universal, for which 28/48 males had a history of surgery, mostly orchidopexy. None of the women had a regular menstrual cycle. Sixteen individuals had a diagnosis of osteoporosis. Spinal deformation, hip dysplasia, and foot abnormalities were common. Skinpicking, leg edema, and erysipelas were frequent dermatological problems. The findings in our group support the notion that the prevalence of physical health problems is underestimated. This underscores the importance of developing monitoring programs which would help to recognize physical health problems at an early stage. © 2011 Wiley-Liss, Inc. |
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Authors:
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Margje Sinnema; Marian A Maaskant; Henny M J van Schrojenstein Lantman-de Valk; I Caroline van Nieuwpoort; Madeleine L Drent; Leopold M G Curfs; Constance T R M Schrander-Stumpel |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-8-10 |
Journal Detail:
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Title: American journal of medical genetics. Part A Volume: - ISSN: 1552-4833 ISO Abbreviation: - Publication Date: 2011 Aug |
Date Detail:
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Created Date: 2011-8-11 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101235741 Medline TA: Am J Med Genet A Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Copyright Information:
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Copyright © 2011 Wiley-Liss, Inc. |
Affiliation:
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Department of Clinical Genetics, Maastricht UMC, Maastricht University, Maastricht, The Netherlands; GROW School for Oncology and Developmental Biology, Maastricht University, Maastricht, The Netherlands; Governor Kremers Centre, Maastricht University, Maastricht, The Netherlands; CAPHRI School for Public Health and Primary Care, Maastricht University, Maastricht, The Netherlands. margje.sinnema@mumc.nl. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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