Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form
Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage. Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However,...
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| Veröffentlicht in: | Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2005-03, Vol.12 (3), p.258-263 |
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| container_title | Archives de pédiatrie : organe officiel de la Société française de pédiatrie |
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| creator | Crétolle, C de Lonlay, P Sauvat, F Brunelle, F Rahier, J Saudubray, J M Nihoul-Fékété, C |
| description | Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage. Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus. The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years. Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus. One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985). |
| doi_str_mv | 10.1016/j.arcped.2004.09.017 |
| format | Article |
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Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus. The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years. Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus. One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).</description><identifier>ISSN: 0929-693X</identifier><identifier>DOI: 10.1016/j.arcped.2004.09.017</identifier><identifier>PMID: 15734120</identifier><language>fre</language><publisher>France</publisher><subject>Biopsy ; Child ; Child, Preschool ; Congenital Hyperinsulinism - classification ; Congenital Hyperinsulinism - diagnosis ; Congenital Hyperinsulinism - genetics ; Congenital Hyperinsulinism - pathology ; Congenital Hyperinsulinism - surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Mutation ; Pancreas - pathology ; Pancreatectomy ; Postoperative Complications ; Retrospective Studies ; Time Factors ; Treatment Outcome</subject><ispartof>Archives de pédiatrie : organe officiel de la Société française de pédiatrie, 2005-03, Vol.12 (3), p.258-263</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27922,27923</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15734120$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Crétolle, C</creatorcontrib><creatorcontrib>de Lonlay, P</creatorcontrib><creatorcontrib>Sauvat, F</creatorcontrib><creatorcontrib>Brunelle, F</creatorcontrib><creatorcontrib>Rahier, J</creatorcontrib><creatorcontrib>Saudubray, J M</creatorcontrib><creatorcontrib>Nihoul-Fékété, C</creatorcontrib><title>Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form</title><title>Archives de pédiatrie : organe officiel de la Société française de pédiatrie</title><addtitle>Arch Pediatr</addtitle><description>Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage. Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus. The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years. Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus. One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).</description><subject>Biopsy</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Congenital Hyperinsulinism - classification</subject><subject>Congenital Hyperinsulinism - diagnosis</subject><subject>Congenital Hyperinsulinism - genetics</subject><subject>Congenital Hyperinsulinism - pathology</subject><subject>Congenital Hyperinsulinism - surgery</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Male</subject><subject>Mutation</subject><subject>Pancreas - pathology</subject><subject>Pancreatectomy</subject><subject>Postoperative Complications</subject><subject>Retrospective Studies</subject><subject>Time Factors</subject><subject>Treatment Outcome</subject><issn>0929-693X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2005</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo10L1OwzAUBWAPIFoKb4BQJraE6_-aDVUUkCoxABJb5Dg3xVXiBDsZ-va0okxnOJ_OcAi5oVBQoOp-V9joBqwLBiAKMAVQfUbmYJjJleFfM3KZ0g4AlrDkF2RGpeaCMpiT91Ufthj8aNvsez9g9CFNrQ8-dVnfZD40Nrj9Q5amuPXugMaIduwwjIcuU5A5mzAdadMf66aP3RU5b2yb8PqUC_K5fvpYveSbt-fX1eMmHyg3Y14JgSiM1BoNd8CFcxSklKxmVjDpuOEga3Bcy8oxW3NZUa41raypuWoMX5C7v90h9j8TprHsfHLYtjZgP6VSaaGEAjjA2xOcqg7rcoi-s3Ff_t_AfwF9FF7a</recordid><startdate>200503</startdate><enddate>200503</enddate><creator>Crétolle, C</creator><creator>de Lonlay, P</creator><creator>Sauvat, F</creator><creator>Brunelle, F</creator><creator>Rahier, J</creator><creator>Saudubray, J M</creator><creator>Nihoul-Fékété, C</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>200503</creationdate><title>Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form</title><author>Crétolle, C ; de Lonlay, P ; Sauvat, F ; Brunelle, F ; Rahier, J ; Saudubray, J M ; Nihoul-Fékété, C</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p139t-b44ee49577e93c034cc105552d2a425c39305d0c375bc2ad35b13771ba9d36f93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>fre</language><creationdate>2005</creationdate><topic>Biopsy</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Congenital Hyperinsulinism - classification</topic><topic>Congenital Hyperinsulinism - diagnosis</topic><topic>Congenital Hyperinsulinism - genetics</topic><topic>Congenital Hyperinsulinism - pathology</topic><topic>Congenital Hyperinsulinism - surgery</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Male</topic><topic>Mutation</topic><topic>Pancreas - pathology</topic><topic>Pancreatectomy</topic><topic>Postoperative Complications</topic><topic>Retrospective Studies</topic><topic>Time Factors</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Crétolle, C</creatorcontrib><creatorcontrib>de Lonlay, P</creatorcontrib><creatorcontrib>Sauvat, F</creatorcontrib><creatorcontrib>Brunelle, F</creatorcontrib><creatorcontrib>Rahier, J</creatorcontrib><creatorcontrib>Saudubray, J M</creatorcontrib><creatorcontrib>Nihoul-Fékété, C</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Archives de pédiatrie : organe officiel de la Société française de pédiatrie</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Crétolle, C</au><au>de Lonlay, P</au><au>Sauvat, F</au><au>Brunelle, F</au><au>Rahier, J</au><au>Saudubray, J M</au><au>Nihoul-Fékété, C</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form</atitle><jtitle>Archives de pédiatrie : organe officiel de la Société française de pédiatrie</jtitle><addtitle>Arch Pediatr</addtitle><date>2005-03</date><risdate>2005</risdate><volume>12</volume><issue>3</issue><spage>258</spage><epage>263</epage><pages>258-263</pages><issn>0929-693X</issn><abstract>Congenital hyperinsulinism of infancy is a severe disease that leads to important brain damage. Two different forms of the disease have been identified by pathologists: a diffuse and a focal form. A specific genetic anomaly identified in focal forms has never been described in diffuse ones. However, for most of authors, failure of medical treatment results in near-total pancreatectomy in all cases, which ends in diabetus. The aim of this retrospective study was to assess the results of elective partial pancreatectomy performed in 60 cases of focal form of hyperinsulinism over the last 18 years. Fifty-eight patients were cured with euglycemia at both fasting and hyperglycaemic tests without insulin-dependent diabetes mellitus. One patient is still in hypoglycaemia from unrecognized lesion; insulin-dependent diabetes mellitus occurred in one case nine years after surgery (a near-total pancreatectomy has been performed because of unknown focal form, in 1985).</abstract><cop>France</cop><pmid>15734120</pmid><doi>10.1016/j.arcped.2004.09.017</doi><tpages>6</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0929-693X |
| ispartof | Archives de pédiatrie : organe officiel de la Société française de pédiatrie, 2005-03, Vol.12 (3), p.258-263 |
| issn | 0929-693X |
| language | fre |
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| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Biopsy Child Child, Preschool Congenital Hyperinsulinism - classification Congenital Hyperinsulinism - diagnosis Congenital Hyperinsulinism - genetics Congenital Hyperinsulinism - pathology Congenital Hyperinsulinism - surgery Female Follow-Up Studies Humans Infant Infant, Newborn Male Mutation Pancreas - pathology Pancreatectomy Postoperative Complications Retrospective Studies Time Factors Treatment Outcome |
| title | Congenital hyperinsulinism of infancy: surgical treatment in 60 cases of focal form |
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