Pancreatic Insufficiency with Bone Marrow Dysfunction (Shwachman-Diamond-Oski-Khaw's Syndrome) Report of a Case
A 2-year-9-month-old Japanese boy was reported of the ‘pancreatic insuffi- ciency with bone marrow dysfunction syndrome’, which was first described by Shwachman, Diamond, Oski and Khaw in 1964. Main clinical features of our own patient consisted of failure to gain weight, steatorrhea, pancytopenia,...
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| Veröffentlicht in: | The Tohoku Journal of Experimental Medicine 1967, Vol.92(1), pp.1-12 |
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| container_title | The Tohoku Journal of Experimental Medicine |
| container_volume | 92 |
| creator | Higashi, Ototaka Hayashi, Tadashi Ohara, Kazuo Honda, Yoshiyuki Konno, Tasuke Sato, Yasushi Yoshida, Toshio Dohi, Senri Takezawa, Yoshiaki |
| description | A 2-year-9-month-old Japanese boy was reported of the ‘pancreatic insuffi- ciency with bone marrow dysfunction syndrome’, which was first described by Shwachman, Diamond, Oski and Khaw in 1964. Main clinical features of our own patient consisted of failure to gain weight, steatorrhea, pancytopenia, the absence of trypsin, lipase and amylase activities in the pancreatic juice, and no increase in the sweat chloride concentration. At autopsy there were massive hemorrhages into the brains and gastrointes-tinal tracts and petechial hemorrhages in the lungs, pleurae, pen- and endocar-dium, psoas muscles and renal pelvis. Histological examination of the pancreas revealed that the tissue was mostly composed of adipose tissue containing the islets of Langerhans which were preserved well and had almost normal appearance. Histological examination of the bone marrow revealed the absence of mega- karyocytes. |
| doi_str_mv | 10.1620/tjem.92.1 |
| format | Article |
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Histological examination of the bone marrow revealed the absence of mega- karyocytes.</description><identifier>ISSN: 0040-8727</identifier><identifier>EISSN: 1349-3329</identifier><identifier>DOI: 10.1620/tjem.92.1</identifier><identifier>PMID: 6059658</identifier><language>eng</language><publisher>Japan: Tohoku University Medical Press</publisher><subject>Bone Marrow - pathology ; Bone Marrow Diseases - complications ; Brain - pathology ; Child, Preschool ; Humans ; Male ; Pancreas - pathology ; Pancreatic Diseases - complications ; Pancreatic Juice - analysis</subject><ispartof>The Tohoku Journal of Experimental Medicine, 1967, Vol.92(1), pp.1-12</ispartof><rights>Tohoku University Medical Press</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c409t-472f5f6da0da41cbbd5a8a36e141ee5009461e7d2361039ba3a60dfe999101163</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,1883,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/6059658$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Higashi, Ototaka</creatorcontrib><creatorcontrib>Hayashi, Tadashi</creatorcontrib><creatorcontrib>Ohara, Kazuo</creatorcontrib><creatorcontrib>Honda, Yoshiyuki</creatorcontrib><creatorcontrib>Konno, Tasuke</creatorcontrib><creatorcontrib>Sato, Yasushi</creatorcontrib><creatorcontrib>Yoshida, Toshio</creatorcontrib><creatorcontrib>Dohi, Senri</creatorcontrib><creatorcontrib>Takezawa, Yoshiaki</creatorcontrib><title>Pancreatic Insufficiency with Bone Marrow Dysfunction (Shwachman-Diamond-Oski-Khaw's Syndrome) Report of a Case</title><title>The Tohoku Journal of Experimental Medicine</title><addtitle>Tohoku J. Exp. Med.</addtitle><description>A 2-year-9-month-old Japanese boy was reported of the ‘pancreatic insuffi- ciency with bone marrow dysfunction syndrome’, which was first described by Shwachman, Diamond, Oski and Khaw in 1964. Main clinical features of our own patient consisted of failure to gain weight, steatorrhea, pancytopenia, the absence of trypsin, lipase and amylase activities in the pancreatic juice, and no increase in the sweat chloride concentration. At autopsy there were massive hemorrhages into the brains and gastrointes-tinal tracts and petechial hemorrhages in the lungs, pleurae, pen- and endocar-dium, psoas muscles and renal pelvis. Histological examination of the pancreas revealed that the tissue was mostly composed of adipose tissue containing the islets of Langerhans which were preserved well and had almost normal appearance. Histological examination of the bone marrow revealed the absence of mega- karyocytes.</description><subject>Bone Marrow - pathology</subject><subject>Bone Marrow Diseases - complications</subject><subject>Brain - pathology</subject><subject>Child, Preschool</subject><subject>Humans</subject><subject>Male</subject><subject>Pancreas - pathology</subject><subject>Pancreatic Diseases - complications</subject><subject>Pancreatic Juice - analysis</subject><issn>0040-8727</issn><issn>1349-3329</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1967</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo9kMtOwzAQRS0EglJY8AFI3gGLlHGcOPUSyqsCBOKxjqbOmKQQu7JTVf17Woq6mVmcq7M4jJ0IGAiVwmU3pXag04HYYT0hM51Imepd1gPIIBkWaXHADmOcAsgMCrXP9hXkWuXDHvOv6Ewg7BrDxy7OrW1MQ84s-aLpan7tHfFnDMEv-M0y2rkzXeMdP3-vF2jqFl1y02DrXZW8xO8meaxxcRb5-9JVwbd0wd9o5kPHveXIRxjpiO1Z_Il0_P_77PPu9mP0kDy93I9HV0-JyUB3SVakNreqQqgwE2YyqXIcolQkMkGUA-hMCSqqVCoBUk9QooLKktZagBBK9tnFxmuCjzGQLWehaTEsSwHlulm5blbqtBSr7elmO5tPWqq2y_9IK3694dPY4RdtOYZVtR_6Mwm9Uq5tmyO20NQYSnLyFz83gAg</recordid><startdate>19670101</startdate><enddate>19670101</enddate><creator>Higashi, Ototaka</creator><creator>Hayashi, Tadashi</creator><creator>Ohara, Kazuo</creator><creator>Honda, Yoshiyuki</creator><creator>Konno, Tasuke</creator><creator>Sato, Yasushi</creator><creator>Yoshida, Toshio</creator><creator>Dohi, Senri</creator><creator>Takezawa, Yoshiaki</creator><general>Tohoku University Medical Press</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></search><sort><creationdate>19670101</creationdate><title>Pancreatic Insufficiency with Bone Marrow Dysfunction (Shwachman-Diamond-Oski-Khaw's Syndrome) Report of a Case</title><author>Higashi, Ototaka ; Hayashi, Tadashi ; Ohara, Kazuo ; Honda, Yoshiyuki ; Konno, Tasuke ; Sato, Yasushi ; Yoshida, Toshio ; Dohi, Senri ; Takezawa, Yoshiaki</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c409t-472f5f6da0da41cbbd5a8a36e141ee5009461e7d2361039ba3a60dfe999101163</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1967</creationdate><topic>Bone Marrow - pathology</topic><topic>Bone Marrow Diseases - complications</topic><topic>Brain - pathology</topic><topic>Child, Preschool</topic><topic>Humans</topic><topic>Male</topic><topic>Pancreas - pathology</topic><topic>Pancreatic Diseases - complications</topic><topic>Pancreatic Juice - analysis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Higashi, Ototaka</creatorcontrib><creatorcontrib>Hayashi, Tadashi</creatorcontrib><creatorcontrib>Ohara, Kazuo</creatorcontrib><creatorcontrib>Honda, Yoshiyuki</creatorcontrib><creatorcontrib>Konno, Tasuke</creatorcontrib><creatorcontrib>Sato, Yasushi</creatorcontrib><creatorcontrib>Yoshida, Toshio</creatorcontrib><creatorcontrib>Dohi, Senri</creatorcontrib><creatorcontrib>Takezawa, Yoshiaki</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><jtitle>The Tohoku Journal of Experimental Medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Higashi, Ototaka</au><au>Hayashi, Tadashi</au><au>Ohara, Kazuo</au><au>Honda, Yoshiyuki</au><au>Konno, Tasuke</au><au>Sato, Yasushi</au><au>Yoshida, Toshio</au><au>Dohi, Senri</au><au>Takezawa, Yoshiaki</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pancreatic Insufficiency with Bone Marrow Dysfunction (Shwachman-Diamond-Oski-Khaw's Syndrome) Report of a Case</atitle><jtitle>The Tohoku Journal of Experimental Medicine</jtitle><addtitle>Tohoku J. Exp. Med.</addtitle><date>1967-01-01</date><risdate>1967</risdate><volume>92</volume><issue>1</issue><spage>1</spage><epage>12</epage><pages>1-12</pages><issn>0040-8727</issn><eissn>1349-3329</eissn><abstract>A 2-year-9-month-old Japanese boy was reported of the ‘pancreatic insuffi- ciency with bone marrow dysfunction syndrome’, which was first described by Shwachman, Diamond, Oski and Khaw in 1964. Main clinical features of our own patient consisted of failure to gain weight, steatorrhea, pancytopenia, the absence of trypsin, lipase and amylase activities in the pancreatic juice, and no increase in the sweat chloride concentration. At autopsy there were massive hemorrhages into the brains and gastrointes-tinal tracts and petechial hemorrhages in the lungs, pleurae, pen- and endocar-dium, psoas muscles and renal pelvis. Histological examination of the pancreas revealed that the tissue was mostly composed of adipose tissue containing the islets of Langerhans which were preserved well and had almost normal appearance. Histological examination of the bone marrow revealed the absence of mega- karyocytes.</abstract><cop>Japan</cop><pub>Tohoku University Medical Press</pub><pmid>6059658</pmid><doi>10.1620/tjem.92.1</doi><tpages>12</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
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| ispartof | The Tohoku Journal of Experimental Medicine, 1967, Vol.92(1), pp.1-12 |
| issn | 0040-8727 1349-3329 |
| language | eng |
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| source | MEDLINE; J-STAGE (Japan Science & Technology Information Aggregator, Electronic) Freely Available Titles - Japanese; EZB-FREE-00999 freely available EZB journals |
| subjects | Bone Marrow - pathology Bone Marrow Diseases - complications Brain - pathology Child, Preschool Humans Male Pancreas - pathology Pancreatic Diseases - complications Pancreatic Juice - analysis |
| title | Pancreatic Insufficiency with Bone Marrow Dysfunction (Shwachman-Diamond-Oski-Khaw's Syndrome) Report of a Case |
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