Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)
Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenom...
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| Veröffentlicht in: | Transplantation proceedings 2004-06, Vol.36 (5), p.1483-1484 |
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| creator | Panaro, F Andorno, E Basile, G Morelli, N Bottino, G Fontana, I Bertocchi, M DiDomenico, S Miggino, M Saltalamacchia, L Ghinolfi, D Bonifazio, L Jarzembowski, T.M Valente, U |
| description | Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease.
A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor.
The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts.
Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage. |
| doi_str_mv | 10.1016/j.transproceed.2004.05.070 |
| format | Article |
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A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor.
The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts.
Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage.</description><identifier>ISSN: 0041-1345</identifier><identifier>EISSN: 1873-2623</identifier><identifier>DOI: 10.1016/j.transproceed.2004.05.070</identifier><identifier>PMID: 15251364</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adult ; Female ; Glycogen Storage Disease Type I - pathology ; Glycogen Storage Disease Type I - surgery ; Hepatectomy ; Humans ; Kidney Transplantation ; Liver - pathology ; Liver Transplantation ; Renal Dialysis ; Treatment Outcome</subject><ispartof>Transplantation proceedings, 2004-06, Vol.36 (5), p.1483-1484</ispartof><rights>2004 Elsevier Inc.</rights><rights>Copyright 2004 Elsevier Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c376t-2ac75a9d7424b2be31e9b2091e0acb8a5f012c1637a0c85606f04b0cbb15c70b3</citedby><cites>FETCH-LOGICAL-c376t-2ac75a9d7424b2be31e9b2091e0acb8a5f012c1637a0c85606f04b0cbb15c70b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.transproceed.2004.05.070$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15251364$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Panaro, F</creatorcontrib><creatorcontrib>Andorno, E</creatorcontrib><creatorcontrib>Basile, G</creatorcontrib><creatorcontrib>Morelli, N</creatorcontrib><creatorcontrib>Bottino, G</creatorcontrib><creatorcontrib>Fontana, I</creatorcontrib><creatorcontrib>Bertocchi, M</creatorcontrib><creatorcontrib>DiDomenico, S</creatorcontrib><creatorcontrib>Miggino, M</creatorcontrib><creatorcontrib>Saltalamacchia, L</creatorcontrib><creatorcontrib>Ghinolfi, D</creatorcontrib><creatorcontrib>Bonifazio, L</creatorcontrib><creatorcontrib>Jarzembowski, T.M</creatorcontrib><creatorcontrib>Valente, U</creatorcontrib><title>Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)</title><title>Transplantation proceedings</title><addtitle>Transplant Proc</addtitle><description>Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease.
A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor.
The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts.
Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage.</description><subject>Adult</subject><subject>Female</subject><subject>Glycogen Storage Disease Type I - pathology</subject><subject>Glycogen Storage Disease Type I - surgery</subject><subject>Hepatectomy</subject><subject>Humans</subject><subject>Kidney Transplantation</subject><subject>Liver - pathology</subject><subject>Liver Transplantation</subject><subject>Renal Dialysis</subject><subject>Treatment Outcome</subject><issn>0041-1345</issn><issn>1873-2623</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkMFOGzEURS1EVVLaX0AWC9ouZvpsj-2EHaItRUJiQbu2bM-byGEyE2wnUv6-RhMQS1aWdc_z9TuEnDOoGTD1Y1XnaIe0iaNHbGsO0NQga9BwRGZsrkXFFRfHZFYCVjHRyBPyKaUVlDtvxEdywiSXTKhmRvAhrLd9tgOO20T7sMNYPYZ2wD2dSno7ZJvDONBujHTZ7_24xIGmPEa7RNqGhDYhzfsN0tsr-m1XyJuA8RG_ppf0-2fyobN9wi-H85T8-_3r7_Wf6u7-5vb66q7yQqtcceu1tItWl2867lAwXDgOC4ZgvZtb2QHjnimhLfi5VKA6aBx455j0Gpw4JRfTu8XN0xZTNuuQPPb9tJ9RSgPXnBfwcgJ9HFOK2JlNDGsb94aBeZZsVuatZPMs2YA0RXIZPju0bN26ZK-jB6sF-DkBWHbdFRkm-YCDxzZE9Nm0Y3hPz3_Nn5ZB</recordid><startdate>20040601</startdate><enddate>20040601</enddate><creator>Panaro, F</creator><creator>Andorno, E</creator><creator>Basile, G</creator><creator>Morelli, N</creator><creator>Bottino, G</creator><creator>Fontana, I</creator><creator>Bertocchi, M</creator><creator>DiDomenico, S</creator><creator>Miggino, M</creator><creator>Saltalamacchia, L</creator><creator>Ghinolfi, D</creator><creator>Bonifazio, L</creator><creator>Jarzembowski, T.M</creator><creator>Valente, U</creator><general>Elsevier Inc</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>7X8</scope></search><sort><creationdate>20040601</creationdate><title>Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)</title><author>Panaro, F ; Andorno, E ; Basile, G ; Morelli, N ; Bottino, G ; Fontana, I ; Bertocchi, M ; DiDomenico, S ; Miggino, M ; Saltalamacchia, L ; Ghinolfi, D ; Bonifazio, L ; Jarzembowski, T.M ; Valente, U</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c376t-2ac75a9d7424b2be31e9b2091e0acb8a5f012c1637a0c85606f04b0cbb15c70b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2004</creationdate><topic>Adult</topic><topic>Female</topic><topic>Glycogen Storage Disease Type I - pathology</topic><topic>Glycogen Storage Disease Type I - surgery</topic><topic>Hepatectomy</topic><topic>Humans</topic><topic>Kidney Transplantation</topic><topic>Liver - pathology</topic><topic>Liver Transplantation</topic><topic>Renal Dialysis</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Panaro, F</creatorcontrib><creatorcontrib>Andorno, E</creatorcontrib><creatorcontrib>Basile, G</creatorcontrib><creatorcontrib>Morelli, N</creatorcontrib><creatorcontrib>Bottino, G</creatorcontrib><creatorcontrib>Fontana, I</creatorcontrib><creatorcontrib>Bertocchi, M</creatorcontrib><creatorcontrib>DiDomenico, S</creatorcontrib><creatorcontrib>Miggino, M</creatorcontrib><creatorcontrib>Saltalamacchia, L</creatorcontrib><creatorcontrib>Ghinolfi, D</creatorcontrib><creatorcontrib>Bonifazio, L</creatorcontrib><creatorcontrib>Jarzembowski, T.M</creatorcontrib><creatorcontrib>Valente, U</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Transplantation proceedings</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Panaro, F</au><au>Andorno, E</au><au>Basile, G</au><au>Morelli, N</au><au>Bottino, G</au><au>Fontana, I</au><au>Bertocchi, M</au><au>DiDomenico, S</au><au>Miggino, M</au><au>Saltalamacchia, L</au><au>Ghinolfi, D</au><au>Bonifazio, L</au><au>Jarzembowski, T.M</au><au>Valente, U</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease)</atitle><jtitle>Transplantation proceedings</jtitle><addtitle>Transplant Proc</addtitle><date>2004-06-01</date><risdate>2004</risdate><volume>36</volume><issue>5</issue><spage>1483</spage><epage>1484</epage><pages>1483-1484</pages><issn>0041-1345</issn><eissn>1873-2623</eissn><abstract>Glycogen storage disease type Ia (GSDIa) is due to the deficiency of glucose-6-phosphatase activity in the liver, kidney, and intestine. Although significant progress has been achieved in the management of patients with GSDIa, complications still emerge. The potential for development of liver adenomatosis and kidney failure makes these patients candidates for simultaneous liver-kidney transplantation (SLKT). Herein, we describe such a transplantation in a patient affected by this rare storage disease.
A 25-year-old female patient with GSDIa developed hepatic adenoma and kidney failure despite dietary therapy. The patient underwent an SLKT from a cadaveric donor.
The operative time was 8 hours without hemotransfusion. Only a transitory lactic acidosis was observed. Laboratory results normalized on postoperative day 7. The patient was discharged on postoperative day 9. After 4 months, the patient is in good condition with well-functioning kidney and liver allografts.
Patients with end-stage renal disease secondary to GSDIa should be considered for SLKT, especially when the disease is in an early stage.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>15251364</pmid><doi>10.1016/j.transproceed.2004.05.070</doi><tpages>2</tpages></addata></record> |
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| subjects | Adult Female Glycogen Storage Disease Type I - pathology Glycogen Storage Disease Type I - surgery Hepatectomy Humans Kidney Transplantation Liver - pathology Liver Transplantation Renal Dialysis Treatment Outcome |
| title | Simultaneous liver-kidney transplantation for glycogen storage disease type IA (von Gierke's disease) |
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