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Page 1
Arteriohepatic dysplasia (Alagille syndrome; Watson-Alagille syndrome).
MacMillan JC, Shepherd R, Heritage M. MacMillan JC, et al. Baillieres Clin Gastroenterol. 1998 Jun;12(2):275-91. doi: 10.1016/s0950-3528(98)90135-x. Baillieres Clin Gastroenterol. 1998. PMID: 9890073 Review.
However, advances in medical and surgical therapy have improved the prognosis at the severe end of the spectrum. It is hoped that the enhanced understanding of the biology of AS resulting from the cloning of the JAG1 gene will enable us to develop additional strategies for …
However, advances in medical and surgical therapy have improved the prognosis at the severe end of the spectrum. It is hoped that the …
Long-term complications of arteriohepatic dysplasia.
Schwarzenberg SJ, Grothe RM, Sharp HL, Snover DC, Freese D. Schwarzenberg SJ, et al. Am J Med. 1992 Aug;93(2):171-6. doi: 10.1016/0002-9343(92)90047-f. Am J Med. 1992. PMID: 1497013
PURPOSE: It has been stated that arteriohepatic dysplasia is a form of biliary paucity with a good prognosis. We wished to determine the long-term morbidity and mortality associated with arteriohepatic dysplasia. ...
PURPOSE: It has been stated that arteriohepatic dysplasia is a form of biliary paucity with a good prognosis. We wished to determine …
Anterior segment and retinal pigmentary abnormalities in arteriohepatic dysplasia.
Puklin JE, Riely CA, Simon RM, Cotlier E. Puklin JE, et al. Ophthalmology. 1981 Apr;88(4):337-47. doi: 10.1016/s0161-6420(81)35026-x. Ophthalmology. 1981. PMID: 7254780
Accurate early diagnosis of the proper syndrome is important, as arteriohepatic dysplasia has a good prognosis, whereas the other syndromes usually lead to death in infancy or early adulthood. ...
Accurate early diagnosis of the proper syndrome is important, as arteriohepatic dysplasia has a good prognosis, whereas the other syn …
Nephropathy in arteriohepatic dysplasia (Alagille's syndrome).
Wolfish NM, Shanon A. Wolfish NM, et al. Child Nephrol Urol. 1988-1989;9(3):169-72. Child Nephrol Urol. 1988. PMID: 3252966
The recognition of kidney involvement in this syndrome and the characterization of the frequency and nature of the renal disorder are of great prognostic significance and could influence the morbidity and mortality of these patients....
The recognition of kidney involvement in this syndrome and the characterization of the frequency and nature of the renal disorder are of gre …
Arteriohepatic dysplasia (Alagille syndrome): extreme variability among affected family members.
Shulman SA, Hyams JS, Gunta R, Greenstein RM, Cassidy SB. Shulman SA, et al. Am J Med Genet. 1984 Oct;19(2):325-32. doi: 10.1002/ajmg.1320190215. Am J Med Genet. 1984. PMID: 6439040
Extreme intrafamilial variability has not been reported previously and most affected individuals described in the past have followed a benign course. The pattern of severity in this family suggests the possibility of a maternal factor augmenting the clinical expression in …
Extreme intrafamilial variability has not been reported previously and most affected individuals described in the past have followed a benig …
Renal abnormalities in paucity of interlobular bile ducts.
Tolia V, Dubois RS, Watts FB Jr, Perrin E. Tolia V, et al. J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):971-6. doi: 10.1097/00005176-198711000-00026. J Pediatr Gastroenterol Nutr. 1987. PMID: 3681584
The nonsyndromatic type of paucity of interlobular bile ducts occurs less frequently and is usually associated with more severe disease and a less favorable prognosis. We present two unusual renal anomalies in association with paucity of interlobular bile ducts. ...
The nonsyndromatic type of paucity of interlobular bile ducts occurs less frequently and is usually associated with more severe disease and …
17 results