Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus
We report the case of a 52-year-old woman with Prader-Willi syndrome (PWS) and diabetes. Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested p...
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| Veröffentlicht in: | Internal Medicine 2021/05/01, Vol.60(9), pp.1423-1426 |
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| creator | Tomoda, Yuki Okauchi, Yukiyoshi Deguchi, Arichika Takenoshita, Yu Iwahashi, Hiromi Mineo, Ikuo |
| description | We report the case of a 52-year-old woman with Prader-Willi syndrome (PWS) and diabetes. Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested positive for anti-GAD antibodies, and had a high-risk genotype - DRB1*09:01-DQB1*03:03 - for slowly progressive insulin-dependent diabetes mellitus (SPIDDM) in the HLA-DR/DQ region, confirming the diagnosis of SPIDDM. Dysglycemia in PWS is thought to be attributable to hyperphagia and obesity. However, the possibility of SPIDDM might be considered if the insulin secretory capacity is almost lost in patients with PWS. |
| doi_str_mv | 10.2169/internalmedicine.5267-20 |
| format | Article |
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Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested positive for anti-GAD antibodies, and had a high-risk genotype - DRB1*09:01-DQB1*03:03 - for slowly progressive insulin-dependent diabetes mellitus (SPIDDM) in the HLA-DR/DQ region, confirming the diagnosis of SPIDDM. Dysglycemia in PWS is thought to be attributable to hyperphagia and obesity. However, the possibility of SPIDDM might be considered if the insulin secretory capacity is almost lost in patients with PWS.</description><identifier>ISSN: 0918-2918</identifier><identifier>EISSN: 1349-7235</identifier><identifier>DOI: 10.2169/internalmedicine.5267-20</identifier><identifier>PMID: 33250457</identifier><language>eng</language><publisher>Japan: The Japanese Society of Internal Medicine</publisher><subject>Case Report ; Diabetes ; Diabetes mellitus (insulin dependent) ; Diabetes Mellitus, Type 1 - complications ; Diabetes Mellitus, Type 1 - diagnosis ; Drb1 protein ; Female ; GAD antibody ; Genotypes ; Glutamate decarboxylase ; Histocompatibility antigen HLA ; Humans ; Hyperphagia ; Insulin ; insulin secretion capacity ; Internal medicine ; Middle Aged ; Obesity - complications ; Prader-Willi syndrome ; Prader-Willi Syndrome - complications ; Prader-Willi Syndrome - diagnosis ; Prader-Willi Syndrome - drug therapy ; slowly progressive insulin-dependent diabetes mellitus ; Sulfonylurea</subject><ispartof>Internal Medicine, 2021/05/01, Vol.60(9), pp.1423-1426</ispartof><rights>2021 by The Japanese Society of Internal Medicine</rights><rights>Copyright Japan Science and Technology Agency 2021</rights><rights>Copyright © 2021 by The Japanese Society of Internal Medicine</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c609t-58643735f15a357536c7928330139b0edc93d47fb8f9563fc56f71331404ae163</citedby><cites>FETCH-LOGICAL-c609t-58643735f15a357536c7928330139b0edc93d47fb8f9563fc56f71331404ae163</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8170260/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8170260/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,885,1883,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33250457$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Tomoda, Yuki</creatorcontrib><creatorcontrib>Okauchi, Yukiyoshi</creatorcontrib><creatorcontrib>Deguchi, Arichika</creatorcontrib><creatorcontrib>Takenoshita, Yu</creatorcontrib><creatorcontrib>Iwahashi, Hiromi</creatorcontrib><creatorcontrib>Mineo, Ikuo</creatorcontrib><title>Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus</title><title>Internal Medicine</title><addtitle>Intern. Med.</addtitle><description>We report the case of a 52-year-old woman with Prader-Willi syndrome (PWS) and diabetes. Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested positive for anti-GAD antibodies, and had a high-risk genotype - DRB1*09:01-DQB1*03:03 - for slowly progressive insulin-dependent diabetes mellitus (SPIDDM) in the HLA-DR/DQ region, confirming the diagnosis of SPIDDM. Dysglycemia in PWS is thought to be attributable to hyperphagia and obesity. However, the possibility of SPIDDM might be considered if the insulin secretory capacity is almost lost in patients with PWS.</description><subject>Case Report</subject><subject>Diabetes</subject><subject>Diabetes mellitus (insulin dependent)</subject><subject>Diabetes Mellitus, Type 1 - complications</subject><subject>Diabetes Mellitus, Type 1 - diagnosis</subject><subject>Drb1 protein</subject><subject>Female</subject><subject>GAD antibody</subject><subject>Genotypes</subject><subject>Glutamate decarboxylase</subject><subject>Histocompatibility antigen HLA</subject><subject>Humans</subject><subject>Hyperphagia</subject><subject>Insulin</subject><subject>insulin secretion capacity</subject><subject>Internal medicine</subject><subject>Middle Aged</subject><subject>Obesity - complications</subject><subject>Prader-Willi syndrome</subject><subject>Prader-Willi Syndrome - complications</subject><subject>Prader-Willi Syndrome - diagnosis</subject><subject>Prader-Willi Syndrome - drug therapy</subject><subject>slowly progressive insulin-dependent diabetes mellitus</subject><subject>Sulfonylurea</subject><issn>0918-2918</issn><issn>1349-7235</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNplkVtvEzEQhS0EoiHwF9BKvPCyxZf17QUJlVullhYVxKPleGcTR4432Lut8u_xkjSC9mX8MN-cOeODUEXwKSVCv_NxgBRt2EDrnY9wyqmQNcVP0IywRteSMv4UzbAmqqalnKAXOa8xZkpq-hydMEY5bricoe_XybaQ6l8-BF_d7GKb-g1Ud35YVTehvwu76jr1ywQ5-1uozmMeg491C1uILcSh-ujtAgbI1SUUhWHML9GzzoYMrw7vHP38_OnH2df64urL-dmHi9oJrIeaK9EwyXhHuGVcciZc8aYYw4TpBYbWadY2sluoTnPBOsdFJwljpMGNBSLYHL3f627HRfkHV8wkG8w2-Y1NO9Nbb_7vRL8yy_7WKCIxFbgIvD0IpP73CHkwG59ducJG6MdsaCOKL9UUT3P05gG67scpgEJxijmV6q-g2lMu9Tkn6I5mCDZTbuZhbmbKzdBp9PW_xxwH74MqwLc9sM6DXcIRsGnwLsBjZYGNnsphwxF0K5sMRPYHdoG2jA</recordid><startdate>20210501</startdate><enddate>20210501</enddate><creator>Tomoda, Yuki</creator><creator>Okauchi, Yukiyoshi</creator><creator>Deguchi, Arichika</creator><creator>Takenoshita, Yu</creator><creator>Iwahashi, Hiromi</creator><creator>Mineo, Ikuo</creator><general>The Japanese Society of Internal Medicine</general><general>Japan Science and Technology Agency</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>7U9</scope><scope>H94</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20210501</creationdate><title>Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus</title><author>Tomoda, Yuki ; Okauchi, Yukiyoshi ; Deguchi, Arichika ; Takenoshita, Yu ; Iwahashi, Hiromi ; Mineo, Ikuo</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c609t-58643735f15a357536c7928330139b0edc93d47fb8f9563fc56f71331404ae163</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Case Report</topic><topic>Diabetes</topic><topic>Diabetes mellitus (insulin dependent)</topic><topic>Diabetes Mellitus, Type 1 - complications</topic><topic>Diabetes Mellitus, Type 1 - diagnosis</topic><topic>Drb1 protein</topic><topic>Female</topic><topic>GAD antibody</topic><topic>Genotypes</topic><topic>Glutamate decarboxylase</topic><topic>Histocompatibility antigen HLA</topic><topic>Humans</topic><topic>Hyperphagia</topic><topic>Insulin</topic><topic>insulin secretion capacity</topic><topic>Internal medicine</topic><topic>Middle Aged</topic><topic>Obesity - complications</topic><topic>Prader-Willi syndrome</topic><topic>Prader-Willi Syndrome - complications</topic><topic>Prader-Willi Syndrome - diagnosis</topic><topic>Prader-Willi Syndrome - drug therapy</topic><topic>slowly progressive insulin-dependent diabetes mellitus</topic><topic>Sulfonylurea</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Tomoda, Yuki</creatorcontrib><creatorcontrib>Okauchi, Yukiyoshi</creatorcontrib><creatorcontrib>Deguchi, Arichika</creatorcontrib><creatorcontrib>Takenoshita, Yu</creatorcontrib><creatorcontrib>Iwahashi, Hiromi</creatorcontrib><creatorcontrib>Mineo, Ikuo</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Internal Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Tomoda, Yuki</au><au>Okauchi, Yukiyoshi</au><au>Deguchi, Arichika</au><au>Takenoshita, Yu</au><au>Iwahashi, Hiromi</au><au>Mineo, Ikuo</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus</atitle><jtitle>Internal Medicine</jtitle><addtitle>Intern. Med.</addtitle><date>2021-05-01</date><risdate>2021</risdate><volume>60</volume><issue>9</issue><spage>1423</spage><epage>1426</epage><pages>1423-1426</pages><issn>0918-2918</issn><eissn>1349-7235</eissn><abstract>We report the case of a 52-year-old woman with Prader-Willi syndrome (PWS) and diabetes. Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested positive for anti-GAD antibodies, and had a high-risk genotype - DRB1*09:01-DQB1*03:03 - for slowly progressive insulin-dependent diabetes mellitus (SPIDDM) in the HLA-DR/DQ region, confirming the diagnosis of SPIDDM. Dysglycemia in PWS is thought to be attributable to hyperphagia and obesity. However, the possibility of SPIDDM might be considered if the insulin secretory capacity is almost lost in patients with PWS.</abstract><cop>Japan</cop><pub>The Japanese Society of Internal Medicine</pub><pmid>33250457</pmid><doi>10.2169/internalmedicine.5267-20</doi><tpages>4</tpages><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; PubMed Central Open Access; J-STAGE (Japan Science & Technology Information Aggregator, Electronic) Freely Available Titles - Japanese; PubMed Central |
| subjects | Case Report Diabetes Diabetes mellitus (insulin dependent) Diabetes Mellitus, Type 1 - complications Diabetes Mellitus, Type 1 - diagnosis Drb1 protein Female GAD antibody Genotypes Glutamate decarboxylase Histocompatibility antigen HLA Humans Hyperphagia Insulin insulin secretion capacity Internal medicine Middle Aged Obesity - complications Prader-Willi syndrome Prader-Willi Syndrome - complications Prader-Willi Syndrome - diagnosis Prader-Willi Syndrome - drug therapy slowly progressive insulin-dependent diabetes mellitus Sulfonylurea |
| title | Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus |
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