ef695de87d56f9fb3f8ef481dc0fcf01 281743.pdf 5320cae91228806856b4f4b29ccff58e28df7a0a 281743.pdf 72535fa7c5d4c30e257f5108e282b127c8467956e47181418310fd3dff5a24c9 281743.pdf Title: Bone Health in Adults With Prader–Willi Syndrome: Clinical Recommendations Based on a Multicenter Cohort Study Subject: DOI: 10.1210/clinem/dgac556; The Journal of Clinical Endocrinology & Metabolism, 108, 1, 2022-9-23.; Abstract: Context: Prader–Willi syndrome (PWS) is a rare complex genetic syndrome, characterized by delayed psychomotor development, hypotonia, and hyperphagia. Hormone deficiencies such as hypogonadism, hypothyroidism, and growth hormone deficiency are common. The combination of hypotonia, low physical activity, and hypogonadism might lead to a decrease in bone mass and increase in fracture risk. Moreover, one would expect an increased risk of scoliosis due to hypotonia and low physical activity. Objective: To study the prevalence and risk factors for skeletal problems (reduced bone mineral density, fractures, and scoliosis) in adults with PWS. Methods: We retrospectively collected patient characteristics, medical history, medication, biochemical measurements, dual-energy X-ray absorptiometry scans, and spinal X-rays and reviewed the current literature. Results: We included 354 adul Keywords: Prader–Willi syndrome; osteoporosis; bone density; human growth hormone; hormone replacement therapy; scoliosis Author: Denise H. van Abswoude Creator: Servigistics Arbortext Advanced Print Publisher 11.1.4546/W-x64 Producer: PDFlib+PDI 9.0.7p3 (C++/Win64); modified using iTextSharp 4.1.6 by 1T3XT CreationDate: Tue Dec 13 06:25:31 2022 CET ModDate: Mon Nov 6 15:16:37 2023 CET Tagged: yes UserProperties: no Suspects: no Form: AcroForm JavaScript: no Pages: 26 Encrypted: no Page size: 593.972 x 782.986 pts Page rot: 0 File size: 1240842 bytes Optimized: no PDF version: 1.5 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- CMXDWT+SabonLTStd-Roman Type 1C Custom yes yes yes 71 0 XQZHTP+SabonLTStd-Bold Type 1C Custom yes yes yes 77 0 RVTTGH+UniversLTStd-Light Type 1C Custom yes yes yes 66 0 YYZKPT+UniversLTStd-Bold Type 1C Custom yes yes yes 69 0 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edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@569cfc36 Item: Title: Results Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@43bd930a Children: Item: Title: Baseline Characteristics Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@33723e30 Item: Title: Osteoporosis Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@64f6106c Item: Title: Risk Factors for Osteoporosis Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@553a3d88 Item: Title: Fractures Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@7a30d1e6 Item: Title: Scoliosis Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@5891e32e Item: Title: Results of the Literature Review Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@cb0ed20 Children: Item: Title: Osteoporosis and Bone Mineral Density Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@8e24743 Item: Title: Growth Hormone Treatment in Relation to Bone Health Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@74a10858 Item: Title: Vitamin D Levels and Bone Markers and Bone-related Factors Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@23fe1d71 Item: Title: Scoliosis Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@28ac3dc3 Item: Title: Discussion Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@32eebfca Children: Item: Title: Clinical Recommendations Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@4e718207 Item: Title: Recommendation 1: Optimizing Calcium and Vitamin D Intake Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@1d371b2d Item: Title: Recommendation 2: Optimizing Physical Activity Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@543c6f6d Item: Title: Recommendation 3: Avoidance of Unnecessary Corticosteroid use in Central Adrenal Insufficiency Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@13eb8acf Item: Title: Recommendation 4: Yearly Screening for (and Treatment of) Hypogonadism Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@51c8530f Item: Title: Recommendation 5: Yearly Screening for Hyperthyroidism and Primary Hyperparathyroidism Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@7403c468 Item: Title: Recommendation 6: Extra Caution in Patients Using Psychotropic Medication Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@43738a82 Item: Title: Recommendation 7: Cessation of Smoking and Alcohol use Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@c81cdd1 Item: Title: Awareness of High Pain Threshold and Intellectual Disability Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@1fc2b765 Item: Title: Yearly Screening for Scoliosis and Other Orthopedic Conditions Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@75881071 Item: Title: Strengths and Limitations Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@2a70a3d8 Item: Title: Conclusion Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@289d1c02 Item: Title: Acknowledgments Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@22eeefeb Item: Title: Author Contributions Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@17d0685f Item: Title: Conflict of Interest Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@3891771e Item: Title: Data Availability Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@78ac1102 Item: Title: References Destination: edu.harvard.hul.ois.jhove.module.pdf.PdfSimpleObject@2de8284b Info: Title: Bone Health in Adults With Prader Willi Syndrome: Clinical Recommendations Based on a Multicenter Cohort Study Author: Denise H. van Abswoude Subject: DOI: 10.1210/clinem/dgac556; The Journal of Clinical Endocrinology & Metabolism, 108, 1, 2022-9-23.; Abstract: Context: Prader Willi syndrome (PWS) is a rare complex genetic syndrome, characterized by delayed psychomotor development, hypotonia, and hyperphagia. Hormone deficiencies such as hypogonadism, hypothyroidism, and growth hormone deficiency are common. The combination of hypotonia, low physical activity, and hypogonadism might lead to a decrease in bone mass and increase in fracture risk. Moreover, one would expect an increased risk of scoliosis due to hypotonia and low physical activity. Objective: To study the prevalence and risk factors for skeletal problems (reduced bone mineral density, fractures, and scoliosis) in adults with PWS. Methods: We retrospectively collected patient characteristics, medical history, medication, biochemical measurements, dual-energy X-ray absorptiometry scans, and spinal X-rays and reviewed the current literature. Results: We included 354 adul Keywords: Prader Willi syndrome; osteoporosis; bone density; human growth hormone; hormone replacement therapy; scoliosis Creator: Servigistics Arbortext Advanced Print Publisher 11.1.4546/W-x64 Producer: PDFlib+PDI 9.0.7p3 (C++/Win64); modified using iTextSharp 4.1.6 by 1T3XT CreationDate: Tue Dec 13 06:55:31 CET 2022 ModDate: Mon Nov 06 15:16:37 CET 2023 ID: 0x635230c2c64215e78b4c4c09f7b27f3e, 0xcad5e44649d8799132e0e2b68831adba Filters: FilterPipeline: FlateDecode FilterPipeline: DCTDecode Images: Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1328 ImageHeight: 1351 BitsPerSample: 8 BitsPerSampleUnit: integer Image: NisoImageMetadata: FormatName: image/jpg CompressionScheme: JPEG ImageWidth: 1333 ImageHeight: 1489 BitsPerSample: 8 BitsPerSampleUnit: integer Fonts: Type0: Font: BaseFont: QXNZLY+TimesNewRomanPSMT Encoding: Identity-H ToUnicode: true Type1: Font: BaseFont: HXLNTV+UniversLTStd-LightObl FontSubset: true 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application/pdf 10.1210/clinem/dgac556 en Oxford University Press Bone Health in Adults With Prader–Willi Syndrome: Clinical Recommendations Based on a Multicenter Cohort Study Denise H. van Abswoude Karlijn Pellikaan Anna G. W. Rosenberg Kirsten Davidse Muriel Coupaye Charlotte Høybye Tania P. Markovic Graziano Grugni Antonino Crinò Assumpta Caixàs Christine Poitou Helena Mosbah Tessa Weir Leo A. van Vlimmeren Joost P. H. J. Rutges Luuk W. L. De Klerk M. Carola Zillikens Aart J. van der Lely Laura C. G. de Graaff DOI: 10.1210/clinem/dgac556; The Journal of Clinical Endocrinology & Metabolism, 108, 1, 2022-9-23.; Abstract: Context: Prader–Willi syndrome (PWS) is a rare complex genetic syndrome, characterized by delayed psychomotor development, hypotonia, and hyperphagia. Hormone deficiencies such as hypogonadism, hypothyroidism, and growth hormone deficiency are common. The combination of hypotonia, low physical activity, and hypogonadism might lead to a decrease in bone mass and increase in fracture risk. Moreover, one would expect an increased risk of scoliosis due to hypotonia and low physical activity. Objective: To study the prevalence and risk factors for skeletal problems (reduced bone mineral density, fractures, and scoliosis) in adults with PWS. Methods: We retrospectively collected patient characteristics, medical history, medication, biochemical measurements, dual-energy X-ray absorptiometry scans, and spinal X-rays and reviewed the current literature. Results: We included 354 adul © The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. Prader–Willi syndrome osteoporosis bone density human growth hormone hormone replacement therapy scoliosis Journal The Journal of Clinical Endocrinology & Metabolism © The Author(s) 2022. 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Destination: 141 Annotation: Subtype: Link Rect: 302, 586, 315, 594 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 555, 315, 563 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 525, 315, 533 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 494, 315, 502 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 463, 315, 471 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 432, 315, 440 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 391, 315, 399 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 360, 315, 368 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 329, 315, 337 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 298, 315, 306 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 267, 315, 275 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 236, 315, 244 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 216, 315, 224 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 174, 315, 182 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 144, 315, 151 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 102, 315, 110 Flags: Print Destination: 141 Annotation: Subtype: Link Rect: 302, 71, 315, 79 Flags: Print Destination: 141 Checksum: 1c6a0f2e Type: CRC32 Checksum: ef695de87d56f9fb3f8ef481dc0fcf01 Type: MD5 Checksum: 5320cae91228806856b4f4b29ccff58e28df7a0a Type: SHA-1