Congenital diseases of intrahepatic bile ducts: variations on the theme "ductal plate malformation".
about
Joubert Syndrome and related disordersThe microRNA-30 family is required for vertebrate hepatobiliary developmentMutations in 3 genes (MKS3, CC2D2A and RPGRIP1L) cause COACH syndrome (Joubert syndrome with congenital hepatic fibrosis)Liver and kidney disease in ciliopathiesPathogenesis of biliary atresia: defining biology to understand clinical phenotypesMaternal microchimerism in biliary atresia: are maternal cells effector cells, targets, or just bystanders?Spectrum of clinical diseases caused by disorders of primary ciliaActivation of the MEK5/ERK5 cascade is responsible for biliary dysgenesis in a rat model of Caroli's diseaseControl of liver cell fate decision by a gradient of TGF beta signaling modulated by Onecut transcription factorsSecondary and tertiary structure modeling reveals effects of novel mutations in polycystic liver disease genes PRKCSH and SEC63.Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis (ARPKD/CHF).Distribution of elastic system fibres in human fetal liverCorrelation of kidney function, volume and imaging findings, and PKHD1 mutations in 73 patients with autosomal recessive polycystic kidney disease.Biliary atresia: will blocking inflammation tame the disease?Novel TMEM67 mutations and genotype-phenotype correlates in meckelin-related ciliopathies.MKS3-related ciliopathy with features of autosomal recessive polycystic kidney disease, nephronophthisis, and Joubert SyndromeEarly detection of severe cholestatic hepatopathy in COACH syndrome.Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease.Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: etiological analysis and introduction of a new concept.Functional anatomy of normal bile ducts.Intrahepatic cholangiocarcinoma arising in Caroli's diseaseMKS3/TMEM67 mutations are a major cause of COACH Syndrome, a Joubert Syndrome related disorder with liver involvementMolecular pathology and genetics of congenital hepatorenal fibrocystic syndromesHepatorenal findings in obligate heterozygotes for autosomal recessive polycystic kidney disease.Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease.Contribution of apoptosis and apoptosis-related proteins to the malformation of the primitive intrahepatic biliary system in Meckel syndrome.Polycystic kidney rat is a novel animal model of Caroli's disease associated with congenital hepatic fibrosis.Analysis of liver repair mechanisms in Alagille syndrome and biliary atresia reveals a role for notch signaling.Cholangiocytes with mesenchymal features contribute to progressive hepatic fibrosis of the polycystic kidney ratMutation of sec63 in zebrafish causes defects in myelinated axons and liver pathologyA Family of Congenital Hepatic Fibrosis and Atypical Retinitis Pigmentosa.Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background miceMolecular and cellular pathogenesis of autosomal recessive polycystic kidney disease.Insight into congenital absence of the portal vein: is it rare?Clues to the etiology of bile duct injury in biliary atresiaRecurrent Cholangitis in a Patient with Autosomal Dominant Polycystic Kidney Disease (ADPKD) and Caroli's Disease.Monosegmental hepatobiliary fibropolycystic disease mimicking a mass: report of three cases.The amazing universe of hepatic microstructure.Cellular Mechanisms of Liver Regeneration and Cell-Based Therapies of Liver Diseases.Biliary atresia: a new immunological insight into etiopathogenesis.
P2860
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P2860
Congenital diseases of intrahepatic bile ducts: variations on the theme "ductal plate malformation".
description
1992 nî lūn-bûn
@nan
1992 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1992 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
name
Congenital diseases of intrahe ...... e "ductal plate malformation".
@ast
Congenital diseases of intrahe ...... e "ductal plate malformation".
@en
type
label
Congenital diseases of intrahe ...... e "ductal plate malformation".
@ast
Congenital diseases of intrahe ...... e "ductal plate malformation".
@en
prefLabel
Congenital diseases of intrahe ...... e "ductal plate malformation".
@ast
Congenital diseases of intrahe ...... e "ductal plate malformation".
@en
P356
P1433
P1476
Congenital diseases of intrahe ...... e "ductal plate malformation".
@en
P2093
P2860
P304
P356
10.1002/HEP.1840160434
P407
P577
1992-10-01T00:00:00Z