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Titolo:
CRESCENTIC NODULAR GLOMERULOSCLEROSIS SECONDARY TO TRUNCATED IMMUNOGLOBULIN-A HEAVY-CHAIN DEPOSITION
Autore:
CHENG IKP; HO SKN; CHAN DTM; NG WK; CHAN KW;
Indirizzi:
UNIV HONG KONG,QUEEN MARY HOSP,DEPT MED HONG KONG HONG KONG UNIV HONG KONG,QUEEN MARY HOSP,DEPT PATHOL HONG KONG HONG KONG
Titolo Testata:
American journal of kidney diseases
fascicolo: 2, volume: 28, anno: 1996,
pagine: 283 - 288
SICI:
0272-6386(1996)28:2<283:CNGSTT>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
DISEASE; AMYLOIDOSIS; GENE;
Keywords:
IMMUNOGLOBULIN; A HEAVY CHAIN; IMMUNOGLOBULIN A; GLOMERULONEPHROPATHY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
13
Recensione:
Indirizzi per estratti:
Citazione:
I.K.P. Cheng et al., "CRESCENTIC NODULAR GLOMERULOSCLEROSIS SECONDARY TO TRUNCATED IMMUNOGLOBULIN-A HEAVY-CHAIN DEPOSITION", American journal of kidney diseases, 28(2), 1996, pp. 283-288

Abstract

Nodular glomerulosclerosis secondary to deposition of monoclonal immunoglobulin (Ig) light chains with or without heavy chains is a recognized clinicopathological entity, Recent reports have demonstrated that an identical glomerular lesion may also occur as a result of truncatedtau Ig heavy chain deposition, We investigated the nature of Ig deposits in a patient who presented with rapidly progressive renal failure secondary to crescentic nodular glomerulosclerosis. This patient had arelapsing clinical course responsive to treatment with steroid and cyclophosphamide therapy, In this case, both the glomeruli and tubular basement membrane contained granular immune deposits that were reactiveto polyclonal antibodies against alpha but not tau or mu Ig heavy chains and nonreactive to anti-kappa and anti-lambda light chain reagents, A monoclonal population of plasma cells secreting alpha and kappa chains was present in the patient's marrow despite the finding of a normal percentage of plasma cells. Serum immunoelectrophoresis was normal,but immunofixation demonstrated the presence of a monoclonal alpha/kappa band. Immunoblot under dissociating and nondissociating conditionsshowed that both the patient's urine and serum contained Ig fragmentsthat comprised dimer or monomer of an abnormally short alpha Ig heavychain (approximately 26 kd) with or without associated re light chain, The identity of the abnormal serum alpha Ig heavy chain with that ofthe glomerular Ig deposits was supported by the finding that both were nonreactive against alpha 1 and alpha 2 subclass-specific monoclonalantibodies despite their reactivity to polyclonal antibodies, Becausethese monoclonal antibodies would react with structural determinants,which differ between alpha 1 and alpha 2 Ig heavy chains but not those common between them, and because the differences in amino acid sequence between the two largely lie in the CH1 and CH2 domains of the alpha Ig heavy chain, it is hypothesized that the abnormally short alpha Ig heavy chain produced by plasma cells in this patient contains deleted CH1 and CH2 domains similar to the findings in patients with tau Ig heavy chain deposition disease. (C) 1996 by the National Kidney Foundation, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 13/08/20 alle ore 12:48:08