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Inflammaging

Id Subject Object Predicate Lexical cue
T1 0-116 Sentence denotes [Progressive familial intrahepatic cholestasis and hereditary anomalies lf hepatocellular metabolism of bile acids].
T2 117-243 Sentence denotes Cholestases intrahépatiques fibrogènes familiales et anomalies héréditaires du métabolisme hépatocytaire des acides biliaires.
T3 244-540 Sentence denotes Progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, is an inherited cholestasis of hepatocellular origin which is characterized by cholestasis presenting often in the neonatal period leading to death due to liver failure at ages ranging from infancy to adolescence.
T4 541-655 Sentence denotes The pattern of appearance of affected children within families is consistent with autosomal recessive inheritance.
T5 656-823 Sentence denotes The etiology is poorly understood but several studies have recently provided support for an heterogeneity with at least three subcategories among the spectrum of PFIC.
T6 824-1238 Sentence denotes The first subtype is characterized by an early onset, often during the neonatal period, a severe pruritus, normal serum gamma-glutamyltransferase (GGT) activity and cholesterol level, high concentration of serum primary bile acids, absence or very low levels of primary bile acids, absence or very low levels of primary bile acids in bile, and absence of ductular proliferation on standard optical liver histology.
T7 1239-1324 Sentence denotes Its leads to death due to liver failure within a few years, rarely after adolescence.
T8 1325-1547 Sentence denotes It is possibly due to an inborn error in primary bile acid secretion and recently, a locus for this subtype has been mapped in the original Byler pedigree to 18q21-q22, the benign recurrent intrahepatic cholestasis region.
T9 1548-1757 Sentence denotes In the second subtype, affected children exhibit also normal serum GGT activity and cholesterol level and absence of ductular proliferation, but have no pruritus and only traces of primary bile acids in serum.
T10 1758-1843 Sentence denotes An inborn error in primary bile acid synthesis has been demonstrated in this subtype.
T11 1844-2002 Sentence denotes The third subtype presents later in life, carries a higher risk of portal hypertension and gastrointestinal bleeding and ends in liver failure at a later age.
T12 2003-2302 Sentence denotes It is characterized by a mild and unconstant pruritus, high GGT serum activity, moderately raised concentrations of serum primary bile acids, normal concentration of biliary primary bile acids, and ductular proliferation and inflammatory infiltrate with patency of intra and extrahepatic bile ducts.
T13 2303-2355 Sentence denotes An abnormal expression of the MDR3 gene is involved.
T14 2356-2461 Sentence denotes A fair proportion of children affected with all subtypes of PFIC may benefit from oral bile acid therapy.
T15 2462-2555 Sentence denotes In some cases partial external biliary diversion or liver transplantation should be proposed.
T1 0-116 Sentence denotes [Progressive familial intrahepatic cholestasis and hereditary anomalies lf hepatocellular metabolism of bile acids].
T2 117-243 Sentence denotes Cholestases intrahépatiques fibrogènes familiales et anomalies héréditaires du métabolisme hépatocytaire des acides biliaires.
T3 244-540 Sentence denotes Progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, is an inherited cholestasis of hepatocellular origin which is characterized by cholestasis presenting often in the neonatal period leading to death due to liver failure at ages ranging from infancy to adolescence.
T4 541-655 Sentence denotes The pattern of appearance of affected children within families is consistent with autosomal recessive inheritance.
T5 656-823 Sentence denotes The etiology is poorly understood but several studies have recently provided support for an heterogeneity with at least three subcategories among the spectrum of PFIC.
T6 824-1238 Sentence denotes The first subtype is characterized by an early onset, often during the neonatal period, a severe pruritus, normal serum gamma-glutamyltransferase (GGT) activity and cholesterol level, high concentration of serum primary bile acids, absence or very low levels of primary bile acids, absence or very low levels of primary bile acids in bile, and absence of ductular proliferation on standard optical liver histology.
T7 1239-1324 Sentence denotes Its leads to death due to liver failure within a few years, rarely after adolescence.
T8 1325-1547 Sentence denotes It is possibly due to an inborn error in primary bile acid secretion and recently, a locus for this subtype has been mapped in the original Byler pedigree to 18q21-q22, the benign recurrent intrahepatic cholestasis region.
T9 1548-1757 Sentence denotes In the second subtype, affected children exhibit also normal serum GGT activity and cholesterol level and absence of ductular proliferation, but have no pruritus and only traces of primary bile acids in serum.
T10 1758-1843 Sentence denotes An inborn error in primary bile acid synthesis has been demonstrated in this subtype.
T11 1844-2002 Sentence denotes The third subtype presents later in life, carries a higher risk of portal hypertension and gastrointestinal bleeding and ends in liver failure at a later age.
T12 2003-2302 Sentence denotes It is characterized by a mild and unconstant pruritus, high GGT serum activity, moderately raised concentrations of serum primary bile acids, normal concentration of biliary primary bile acids, and ductular proliferation and inflammatory infiltrate with patency of intra and extrahepatic bile ducts.
T13 2303-2355 Sentence denotes An abnormal expression of the MDR3 gene is involved.
T14 2356-2461 Sentence denotes A fair proportion of children affected with all subtypes of PFIC may benefit from oral bile acid therapy.
T15 2462-2555 Sentence denotes In some cases partial external biliary diversion or liver transplantation should be proposed.

QFMC_MEDLINE

Id Subject Object Predicate Lexical cue
T1 117-144 DISO denotes Cholestases intrahépatiques
T1c 117-144 UMLS:C0008372 denotes Cholestases intrahépatiques
T2 226-242 CHEM denotes acides biliaires
T2c 226-242 UMLS:C0005390 denotes acides biliaires
#1 T1 T1c Normalization Cholestases intrahépatiques,Cholestases intrahépatiques
#2 T2 T2c Normalization acides biliaires,acides biliaires

PubmedHPO

Id Subject Object Predicate Lexical cue
T1 265-289 HP_0001406 denotes intrahepatic cholestasis
T2 278-289 HP_0001396 denotes cholestasis
T3 343-354 HP_0001396 denotes cholestasis
T4 406-417 HP_0001396 denotes cholestasis
T5 482-495 HP_0001399 denotes liver failure
T6 623-654 HP_0000007 denotes autosomal recessive inheritance
T7 623-642 HP_0000007 denotes autosomal recessive
T8 921-929 HP_0000989 denotes pruritus
T9 1265-1278 HP_0001399 denotes liver failure
T10 1515-1539 HP_0001406 denotes intrahepatic cholestasis
T11 1528-1539 HP_0001396 denotes cholestasis
T12 1701-1709 HP_0000989 denotes pruritus
T13 1911-1930 HP_0001409 denotes portal hypertension
T14 1918-1930 HP_0000822 denotes hypertension
T15 1935-1960 HP_0002239 denotes gastrointestinal bleeding
T16 1973-1986 HP_0001399 denotes liver failure
T17 2048-2056 HP_0000989 denotes pruritus