Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome)
<p>Abstract</p> <p>Background</p> <p>Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.</p> <p>Case presentation</p> <p>We describe two unrelat...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2007-07-01
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| Series: | Diagnostic Pathology |
| Online Access: | http://www.diagnosticpathology.org/content/2/1/24 |
| _version_ | 1818142929172037632 |
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| author | Kakkar Nandita Gupta Kirti Vankalakunti Mahesha Das Ashim |
| author_facet | Kakkar Nandita Gupta Kirti Vankalakunti Mahesha Das Ashim |
| author_sort | Kakkar Nandita |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.</p> <p>Case presentation</p> <p>We describe two unrelated cases of Renal-Hepatic-Pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-Hepatic-Pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.</p> <p>Conclusion</p> <p>These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.</p> |
| first_indexed | 2024-12-11T11:23:35Z |
| format | Article |
| id | doaj.art-cd1335bde28a4f728f8dca8e01521128 |
| institution | Directory Open Access Journal |
| issn | 1746-1596 |
| language | English |
| last_indexed | 2024-12-11T11:23:35Z |
| publishDate | 2007-07-01 |
| publisher | BMC |
| record_format | Article |
| series | Diagnostic Pathology |
| spelling | doaj.art-cd1335bde28a4f728f8dca8e015211282022-12-22T01:09:05ZengBMCDiagnostic Pathology1746-15962007-07-01212410.1186/1746-1596-2-24Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome)Kakkar NanditaGupta KirtiVankalakunti MaheshaDas Ashim<p>Abstract</p> <p>Background</p> <p>Renal-Hepatic-Pancreatic dysplasia syndrome described by Ivemark in 1959 constitutes a triad pancreatic fibrosis, renal dysplasia and hepatic dysgenesis.</p> <p>Case presentation</p> <p>We describe two unrelated cases of Renal-Hepatic-Pancreatic dysplasia syndrome in stillborn babies. The characteristic microscopic features were present in both the cases. The second case illustrates the unique association lymphangiectasia with Renal-Hepatic-Pancreatic dysplasia syndrome. Both cases are unrelated and there is no history of any consanguineous marriage.</p> <p>Conclusion</p> <p>These two cases are unrelated and are rare. In the developmental research, the perinatal autopsy needs to be utilized as a major tool and an Ad hoc committee formation is required to formulate the approach towards syndromic diseases.</p>http://www.diagnosticpathology.org/content/2/1/24 |
| spellingShingle | Kakkar Nandita Gupta Kirti Vankalakunti Mahesha Das Ashim Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) Diagnostic Pathology |
| title | Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) |
| title_full | Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) |
| title_fullStr | Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) |
| title_full_unstemmed | Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) |
| title_short | Renal-hepatic-pancreatic dysplasia syndrome (ivemark's syndrome) |
| title_sort | renal hepatic pancreatic dysplasia syndrome ivemark s syndrome |
| url | http://www.diagnosticpathology.org/content/2/1/24 |
| work_keys_str_mv | AT kakkarnandita renalhepaticpancreaticdysplasiasyndromeivemarkssyndrome AT guptakirti renalhepaticpancreaticdysplasiasyndromeivemarkssyndrome AT vankalakuntimahesha renalhepaticpancreaticdysplasiasyndromeivemarkssyndrome AT dasashim renalhepaticpancreaticdysplasiasyndromeivemarkssyndrome |