Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia
Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenou...
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| Veröffentlicht in: | Pediatric nephrology (Berlin, West) West), 2006-01, Vol.21 (1), p.36-38 |
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| creator | Yoshino, Atsunori Honda, Masataka Kanegane, Hirokazu Obata, Kazuo Matsukura, Hiroyoshi Sakazume, Satoru Katada, Yasuki Miyawaki, Toshio Ueda, Yoshihiko Nagai, Toshiro |
| description | Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0-4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN. |
| doi_str_mv | 10.1007/s00467-005-2029-z |
| format | Article |
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X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0-4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN.</description><identifier>ISSN: 0931-041X</identifier><identifier>EISSN: 1432-198X</identifier><identifier>DOI: 10.1007/s00467-005-2029-z</identifier><identifier>PMID: 16550363</identifier><language>eng</language><publisher>Germany: Springer</publisher><subject>Agammaglobulinemia ; Agammaglobulinemia - complications ; Agammaglobulinemia - drug therapy ; Agammaglobulinemia - genetics ; Case studies ; Complications and side effects ; Diagnosis ; Drug therapy ; Genes, X-Linked ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative - etiology ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous - therapeutic use ; Infant ; Male ; Risk factors</subject><ispartof>Pediatric nephrology (Berlin, West), 2006-01, Vol.21 (1), p.36-38</ispartof><rights>COPYRIGHT 2006 Springer</rights><rights>IPNA 2005</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c357t-4acd1d890b3af530ca98b59c010e69b5097ea08e8563801193198dcad77ec1103</citedby><cites>FETCH-LOGICAL-c357t-4acd1d890b3af530ca98b59c010e69b5097ea08e8563801193198dcad77ec1103</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16550363$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Yoshino, Atsunori</creatorcontrib><creatorcontrib>Honda, Masataka</creatorcontrib><creatorcontrib>Kanegane, Hirokazu</creatorcontrib><creatorcontrib>Obata, Kazuo</creatorcontrib><creatorcontrib>Matsukura, Hiroyoshi</creatorcontrib><creatorcontrib>Sakazume, Satoru</creatorcontrib><creatorcontrib>Katada, Yasuki</creatorcontrib><creatorcontrib>Miyawaki, Toshio</creatorcontrib><creatorcontrib>Ueda, Yoshihiko</creatorcontrib><creatorcontrib>Nagai, Toshiro</creatorcontrib><title>Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia</title><title>Pediatric nephrology (Berlin, West)</title><addtitle>Pediatr Nephrol</addtitle><description>Immune complex and complement systems play an important role in membranoproliferative glomerulonephritis (MPGN). X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0-4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN.</description><subject>Agammaglobulinemia</subject><subject>Agammaglobulinemia - complications</subject><subject>Agammaglobulinemia - drug therapy</subject><subject>Agammaglobulinemia - genetics</subject><subject>Case studies</subject><subject>Complications and side effects</subject><subject>Diagnosis</subject><subject>Drug therapy</subject><subject>Genes, X-Linked</subject><subject>Glomerulonephritis</subject><subject>Glomerulonephritis, Membranoproliferative - etiology</subject><subject>Humans</subject><subject>Immunoglobulins</subject><subject>Immunoglobulins, Intravenous - therapeutic use</subject><subject>Infant</subject><subject>Male</subject><subject>Risk factors</subject><issn>0931-041X</issn><issn>1432-198X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNpdkU2LFDEQhoMo7rj6A7xI48FbtNLpfB2XxS9Y8aIyt1DdXTOTNd0Zk27F_fVmnQHBXALJ8xZv8TD2XMBrAWDeFIBOGw6geAut43cP2EZ0suXC2e1DtgEnBYdObC_Yk1JuAcAqqx-zC6GVAqnlhn37RFOfcU7HnGLYUcYl_KRmH9NEeY1ppuMhhyWUJswNNsf6TfPS_ArLodnyGObvNDa4x2nCmunX-kJTwKfs0Q5joWfn-5J9fff2y_UHfvP5_cfrqxs-SGUW3uEwitE66CXulIQBne2VG0AAadcrcIYQLFmlpQUh6j7OjgOOxtAgBMhL9uo0t9b_sVJZ_BTKQDHiTGktXhujrTSygi__A2_Tmufazbf1OLB_ofO0PUbyB8K4HEqK6xLSXPyVUK01re10BcUJHHIqJdPOH3OYMP_2Avy9Gn9S46saf6_G39XMi3ODtZ9o_Jc4u5B_ADwHigQ</recordid><startdate>200601</startdate><enddate>200601</enddate><creator>Yoshino, Atsunori</creator><creator>Honda, Masataka</creator><creator>Kanegane, Hirokazu</creator><creator>Obata, Kazuo</creator><creator>Matsukura, Hiroyoshi</creator><creator>Sakazume, Satoru</creator><creator>Katada, Yasuki</creator><creator>Miyawaki, Toshio</creator><creator>Ueda, Yoshihiko</creator><creator>Nagai, Toshiro</creator><general>Springer</general><general>Springer Nature B.V</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>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>M0R</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope></search><sort><creationdate>200601</creationdate><title>Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia</title><author>Yoshino, Atsunori ; 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X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by severe hypogammaglobulinemia. We report the case of an XLA patient who developed MPGN during an intravenous immunoglobulin (IVIG) treatment. In this patient, the serum IgG level was maintained at more than 400 mg/dl of regular IVIG administration (2.5 g/dose/month). The patient presented with microscopic hematuria, proteinuria (U-pro/Cr: 4.0-4.2) and low serum complement levels (C3: 57.8 mg/dl) 3 years after IVIG treatment and was diagnosed histopathologically as having MPGN type III. Both hematuria and proteinuria significantly improved, and the serum complement level returned to a normal level following methylprednisolone pulse therapy. To our knowledge, this is the first case report of MPGN associated with XLA. Although it is unclear how MPGN occurred in this XLA patient, we suggest that residual humoral immunity in the patient could be associated with the development of MPGN.</abstract><cop>Germany</cop><pub>Springer</pub><pmid>16550363</pmid><doi>10.1007/s00467-005-2029-z</doi><tpages>3</tpages></addata></record> |
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| subjects | Agammaglobulinemia Agammaglobulinemia - complications Agammaglobulinemia - drug therapy Agammaglobulinemia - genetics Case studies Complications and side effects Diagnosis Drug therapy Genes, X-Linked Glomerulonephritis Glomerulonephritis, Membranoproliferative - etiology Humans Immunoglobulins Immunoglobulins, Intravenous - therapeutic use Infant Male Risk factors |
| title | Membranoproliferative glomerulonephritis in a patient with X-linked agammaglobulinemia |
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