Bilateral renal dysplasia, pancreatic fibrosis, intrahepatic biliary dysgenesis, and situs inversus totalis in a boy

Hiraoka, Katsumi; Haratake, Joji; Horie, Akio; Miyagawa, Takayuki

doi:10.1016/s0046-8177(88)80273-9

Cited by 22 publications

(12 citation statements)

References 9 publications

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“…[32][33][34][35] The fact that there were no intrahepatic cysts, Meyenburg complexes, or fibrosis in the livers of the inv mice argues against the possibility that the inv mice represent an animal model of a variant of Caroli' s disease or congenital hepatic fibrosis. However, it is possible that these histopathological changes would only become apparent over longer time intervals postnatally.…”

Section: Discussionmentioning

confidence: 99%

Anomalous development of the hepatobiliary system in the inv mouse

et al. 1999

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Extrahepatic biliary atresia (BA) is a devastating disease of the neonate in which the hepatic and/or common bile duct is obliterated or interrupted. Infants and children with this diagnosis constitute 50% to 60% of the pediatric population that undergoes orthotopic liver transplantation. However, there is still very little known about the etiology and pathogenesis of BA. Several recent studies have demonstrated that anomalies of situs determination are more commonly associated with BA than previously recognized. In this study, we examined the pathogenesis of jaundice in the inv mouse, a transgenic mouse in which a recessive deletion of the inversin gene results in situs inversus and jaundice. The results show that these mice have cholestasis with conjugated hyperbilirubinemia, failure to excrete technetium-labeled mebrofenin from the liver into the small intestine, lack of continuity between the extrahepatic biliary tree and the small intestine as demonstrated by Trypan blue cholangiography, and a liver histological picture indicative of extrahepatic biliary obstruction with negligible inflammation/necrosis within the hepatic parenchyma. Lectin histochemical staining of biliary epithelial cells in serial sections suggests the presence of several different anomalies in the architecture of the extrahepatic biliary system. These results suggest that the inversin gene plays an essential role in the morphogenesis of the hepatobiliary system and raise the possibility that alterations in the human orthologue of inversin account for some of the cases of BA in which there are also anomalies of situs determination. (HEPATOLOGY 1999;30:372-378.)

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Section: Discussionmentioning

confidence: 99%

Anomalous development of the hepatobiliary system in the inv mouse

et al. 1999

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“…1,3,4 Five cases of bilateral renal dysplasia with situs inversus totalis and hepatic and pancreatic fibrosis have also been noted. 2,[5][6][7][8] Pinar and Rogers called these spectrum of anomalies a new syndrome. 6 Association of situs inversus totalis and renal hypoplasia has not been reported.…”

Section: Introductionmentioning

confidence: 99%

Renal hypoplasia and situs inversus totalis

Kaynar¹,

Ulusoy²,

Gul³

et al. 2005

Nephrology

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“…1,2,6-8 Split cord malformation, congenital aglossia, single coronary ostium, renal dysplasia, and pancreatic fibrosis have also been reported associated with situs inversus in humans. [15][16][17][18][19][20] Primary ciliary dyskinesia, hydrocephalus, and cardiac malformations have been reported in one dog with situs inversus. 8 The cat in this report had no clinical evidence of ciliary immotility, however, the mild cardiac abnormalities identified may have been related to the situs inversus.…”

Section: Discussionmentioning

confidence: 98%

“…The dysmotility of the ciliary apparatus leads to mucus accumulation and subsequent paranasal sinus, bronchial, and pulmonary disease 1,2,6–8 . Split cord malformation, congenital aglossia, single coronary ostium, renal dysplasia, and pancreatic fibrosis have also been reported associated with situs inversus in humans 15–20 . Primary ciliary dyskinesia, hydrocephalus, and cardiac malformations have been reported in one dog with situs inversus 8 …”

Section: Discussionmentioning

confidence: 99%