PMID- 3138174
OWN - NLM
STAT- MEDLINE
DCOM- 19881014
LR  - 20190722
IS  - 0046-8177 (Print)
IS  - 0046-8177 (Linking)
VI  - 19
IP  - 9
DP  - 1988 Sep
TI  - Light chain deposition disease with liver dysfunction.
PG  - 1008-14
AB  - Light and transmission electron microscopic study as well as immunohistochemical 
      investigation were performed on three cases of light chain deposition disease 
      (LCDD) with severe liver dysfunction. In two cases, the amount of light chain 
      deposits in the liver was moderate and did not correlate with the severity of 
      clinical and biological symptoms. Ultrastructural study demonstrated a 
      collagenization of the Disse's space, with basement membrane-like material in 
      association with light chain deposits. Immunohistochemical investigation showed a 
      marked increase of collagen types I, III, and IV, as well as fibronectin and 
      laminin in perisinusoidal space. This study suggests that collagenization of the 
      Disse's space has a minor role in liver dysfunction. The analogy between kidney 
      and liver lesions in diabetes and in LCDD is stressed, but the mechanism of this 
      abnormal accumulation of matrix proteins remains unknown.
FAU - Bedossa, P
AU  - Bedossa P
AD  - Service d'Anatomie Pathologique, Hopital Antoine Beclere, Clamart, France.
FAU - Fabre, M
AU  - Fabre M
FAU - Paraf, F
AU  - Paraf F
FAU - Martin, E
AU  - Martin E
FAU - Lemaigre, G
AU  - Lemaigre G
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Hum Pathol
JT  - Human pathology
JID - 9421547
RN  - 0 (Fibronectins)
RN  - 0 (Immunoglobulin Light Chains)
RN  - 9007-34-5 (Collagen)
SB  - IM
MH  - Aged
MH  - Collagen/analysis
MH  - Female
MH  - Fibronectins/analysis
MH  - Humans
MH  - Hypergammaglobulinemia/*complications
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Immunohistochemistry
MH  - Liver Diseases/*complications/metabolism
MH  - Male
MH  - Microscopy, Electron
MH  - Middle Aged
MH  - Paraproteinemias/*metabolism/physiopathology
EDAT- 1988/09/01 00:00
MHDA- 1988/09/01 00:01
CRDT- 1988/09/01 00:00
PHST- 1988/09/01 00:00 [pubmed]
PHST- 1988/09/01 00:01 [medline]
PHST- 1988/09/01 00:00 [entrez]
AID - S0046-8177(88)80078-9 [pii]
AID - 10.1016/s0046-8177(88)80078-9 [doi]
PST - ppublish
SO  - Hum Pathol. 1988 Sep;19(9):1008-14. doi: 10.1016/s0046-8177(88)80078-9.