PMID- 9696001
OWN - NLM
STAT- MEDLINE
DCOM- 19980831
LR  - 20031114
IS  - 0270-9139 (Print)
IS  - 0270-9139 (Linking)
VI  - 28
IP  - 2
DP  - 1998 Aug
TI  - Noncirrhotic portal hypertension and nodular regenerative hyperplasia of the 
      liver in dogs with mucopolysaccharidosis type I.
PG  - 385-90
AB  - Nodular regenerative hyperplasia (NRH) is an uncommon hepatic lesion often 
      associated with noncirrhotic portal hypertension (PHT). We have noted that NRH 
      and PHT are frequent occurrences in a colony of dogs with the genetic storage 
      disease, mucopolysaccharidosis I (MPS-I). This observation provides the 
      opportunity to study the histology and pathogenesis of NRH and noncirrhotic PHT 
      in a new animal model. Thirteen of 32 dogs (41%) with MPS-I developed multiple 
      portocaval shunts between 4 and 48 months of age that were grossly visible at 
      necropsy. Seven of the 13 developed marked ascites, whereas all those without 
      shunts and littermates (n = 24) heterozygous for the mutated alpha-L-iduronidase 
      allele (carriers unaffected by the storage disease) did not. The large and 
      medium-sized portal veins were widely patent without thrombosis or vascular 
      malformations. Hepatic parenchymal fibrosis was absent or mild and did not 
      correlate with shunt formation. All 32 livers had varying degrees of diffuse 
      periportal hepatocellular hyperplasia with multifocal atrophy and compression of 
      centrolobular cords (NRH) most prominent in dogs with shunts. Many small portal 
      veins were reduced in diameter or absent, especially in animals with shunts. 
      Noncirrhotic PHT and NRH appear to be related to the obliteration of small portal 
      veins in these dogs, but the pathogenesis of this vascular change remains 
      unknown.
FAU - McEntee, M F
AU  - McEntee MF
AD  - Department of Pathology, College of Veterinary Medicine, University of Tennessee, 
      Knoxville 37901-1071, USA.
FAU - Wright, K N
AU  - Wright KN
FAU - Wanless, I
AU  - Wanless I
FAU - DeVovo, R
AU  - DeVovo R
FAU - Schneider, J F
AU  - Schneider JF
FAU - Shull, R
AU  - Shull R
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Hepatology
JT  - Hepatology (Baltimore, Md.)
JID - 8302946
SB  - IM
MH  - Animals
MH  - Atrophy
MH  - *Disease Models, Animal
MH  - Dog Diseases/*pathology
MH  - Dogs
MH  - Female
MH  - Hyperplasia
MH  - Hypertension, Portal/*veterinary
MH  - Liver/pathology
MH  - Liver Diseases/*veterinary
MH  - Mucopolysaccharidosis I/*veterinary
EDAT- 1998/08/08 00:00
MHDA- 1998/08/08 00:01
CRDT- 1998/08/08 00:00
PHST- 1998/08/08 00:00 [pubmed]
PHST- 1998/08/08 00:01 [medline]
PHST- 1998/08/08 00:00 [entrez]
AID - S0270913998003152 [pii]
AID - 10.1002/hep.510280214 [doi]
PST - ppublish
SO  - Hepatology. 1998 Aug;28(2):385-90. doi: 10.1002/hep.510280214.