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Article: Fibrillary glomerulonephritis in siblings.

TitleFibrillary glomerulonephritis in siblings.
Authors
Issue Date1998
Citation
American Journal Of Kidney Diseases : The Official Journal Of The National Kidney Foundation, 1998, v. 31 n. 5, p. E4 How to Cite?
AbstractFibrillary/immunotactoid glomerulopathy is characterized by organized glomerular deposition of extracellular, nonbranching, immunoglobulin-derived microfibrils, which is not associated with systemic diseases such as amyloidosis, cryoglobulinemia, or monoclonal gammopathy. This is an uncommon condition with an obscure etiology and accounts for approximately 1% of primary glomerular diseases in white populations. We report the first case of familial fibrillary/immunotactoid glomerulopathy affecting a brother and a sister in a Chinese family. Both patients presented with heavy proteinuria, which improved transiently on treatment with prednisolone and cyclophosphamide. Human lymphocyte antigen typing for the siblings showed no haplotype association. Despite the generally poor renal prognosis reported in the literature, with 50% of patients reaching end-stage renal failure within 2 to 4 years, both patients had relative preservation of renal function (creatinine clearance from 79 to 76 mL/min/1.73 m2 after 2 years in one patient and from 111 to 99 mL/min/1.73 m2 after 3 years in the other). Our observations show that fibrillary/immunotactoid glomerulopathy can present as a familial condition. Compared with sporadic cases, patients with familial fibrillary/immunotactoid glomerulopathy may have a more favorable renal prognosis.
Persistent Identifierhttp://hdl.handle.net/10722/148128
ISSN
2015 SCImago Journal Rankings: 2.313

 

DC FieldValueLanguage
dc.contributor.authorChan, TMen_HK
dc.contributor.authorChan, KWen_HK
dc.date.accessioned2012-05-29T06:11:01Z-
dc.date.available2012-05-29T06:11:01Z-
dc.date.issued1998en_HK
dc.identifier.citationAmerican Journal Of Kidney Diseases : The Official Journal Of The National Kidney Foundation, 1998, v. 31 n. 5, p. E4en_HK
dc.identifier.issn1523-6838en_HK
dc.identifier.urihttp://hdl.handle.net/10722/148128-
dc.description.abstractFibrillary/immunotactoid glomerulopathy is characterized by organized glomerular deposition of extracellular, nonbranching, immunoglobulin-derived microfibrils, which is not associated with systemic diseases such as amyloidosis, cryoglobulinemia, or monoclonal gammopathy. This is an uncommon condition with an obscure etiology and accounts for approximately 1% of primary glomerular diseases in white populations. We report the first case of familial fibrillary/immunotactoid glomerulopathy affecting a brother and a sister in a Chinese family. Both patients presented with heavy proteinuria, which improved transiently on treatment with prednisolone and cyclophosphamide. Human lymphocyte antigen typing for the siblings showed no haplotype association. Despite the generally poor renal prognosis reported in the literature, with 50% of patients reaching end-stage renal failure within 2 to 4 years, both patients had relative preservation of renal function (creatinine clearance from 79 to 76 mL/min/1.73 m2 after 2 years in one patient and from 111 to 99 mL/min/1.73 m2 after 3 years in the other). Our observations show that fibrillary/immunotactoid glomerulopathy can present as a familial condition. Compared with sporadic cases, patients with familial fibrillary/immunotactoid glomerulopathy may have a more favorable renal prognosis.en_HK
dc.languageengen_US
dc.relation.ispartofAmerican journal of kidney diseases : the official journal of the National Kidney Foundationen_HK
dc.subject.meshActin Cytoskeleton - Ultrastructureen_US
dc.subject.meshAdulten_US
dc.subject.meshBiopsyen_US
dc.subject.meshFemaleen_US
dc.subject.meshGlomerular Mesangium - Pathologyen_US
dc.subject.meshGlomerulonephritis - Diagnosis - Geneticsen_US
dc.subject.meshHumansen_US
dc.subject.meshMaleen_US
dc.subject.meshNuclear Familyen_US
dc.titleFibrillary glomerulonephritis in siblings.en_HK
dc.typeArticleen_HK
dc.identifier.emailChan, TM:dtmchan@hku.hken_HK
dc.identifier.emailChan, KW:hrmtckw@hku.hken_HK
dc.identifier.authorityChan, TM=rp00394en_HK
dc.identifier.authorityChan, KW=rp00330en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1016/S0272-6386(98)70066-6-
dc.identifier.pmid10074581-
dc.identifier.scopuseid_2-s2.0-0032057599en_HK
dc.identifier.hkuros46047-
dc.identifier.volume31en_HK
dc.identifier.issue5en_HK
dc.identifier.spageE4en_HK
dc.identifier.epageE4en_HK
dc.identifier.scopusauthoridChan, TM=7402687700en_HK
dc.identifier.scopusauthoridChan, KW=16444133100en_HK

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