Arteriohepatic dysplasia (Alagille's syndrome): unusual hepatic architecture and function

Halvorsen, Robert A.; Garrity, S.; Kuni, Christopher C.; duCret, Rene P.; Letourneau, J G; Bloomer, Joseph R.

doi:10.1007/bf00200391

Cited by 12 publications

(7 citation statements)

References 14 publications

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“…The reported radiologic appearances of AGS are diverse and previous studies demonstrated a much higher rate of hepatic parenchymal changes when compared with our data. Halvorsen et al [ 19 ] observed spherical hepatic deformity in 63% (5/8) of AGS patients. Berrocal et al [ 20 ] reported that 68% (19/28) of AGS patients showed hepatomegaly, fibrosis, or cirrhosis.…”

Section: Discussionmentioning

confidence: 99%

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Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

Hwang¹,

Jeon²,

Yoo³

et al. 2016

PLoS ONE

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PurposeTo compare preoperative CT findings before liver transplantation between patients with Alagille syndrome (AGS) and those with end-stage biliary atresia (BA).Materials and MethodsThe institutional review board approved this retrospective study. Eleven children with AGS (median age, 19.0 ± 13.0 months; male to female ratio, 3:8) and 109 children with end-stage BA (median age, 17.9 ± 25.8 months; male to female ratio, 37:72) who underwent abdomen CT as candidates for liver transplant were included. CT images were reviewed focusing on hepatic parenchymal changes, vascular changes, presence of focal lesions, and signs of portal hypertension.ResultsHepatic parenchymal changes were present in 27% (3/11) of AGS patients and 100% (109/109) of end-stage BA patients (P < .001). The hepatic artery diameter was significantly smaller (1.9 mm versus 3.6 mm, P = 008), whereas portal vein diameter was larger (6.8 mm versus 5.0 mm, P < .001) in patients with AGS compared with patients with end-stage BA. No focal lesion was seen in patients with AGS, whereas 44% (48/109) of patients with end-stage BA had intrahepatic biliary cysts (39%, 43/109) and hepatic tumors (8%, 9/109) (P = .008). Splenomegaly was commonly seen in both groups (P = .082), and ascites (9% [1/11] versus 50% [54/109], P = .010) and gastroesophageal varix (0% [0/11] versus 80% [87/109], P < .001) were less common in patients with AGS than in patients with end-stage BA.ConclusionFibrotic or cirrhotic changes of the liver, presence of focal lesions, and relevant portal hypertension were less common in patients with AGS than in patients with end-stage BA.

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Section: Discussionmentioning

confidence: 99%

“…AGS is progressive cholangiopathy, and the prevalence and severity of PIBD can increase with increasing age as a result of retained toxins, inflammation, or disuse atrophy. The age of patients in previous studies range from 3 months to 33 years [ 19 , 20 ], and therefore included an older age group than in our study.…”

Section: Discussionmentioning

confidence: 99%

Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

Hwang¹,

Jeon²,

Yoo³

et al. 2016

PLoS ONE

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“…Unlike in adults, where HCC is usually seen with underlying liver disease, only 20–35% children with HCC children have underlying liver disease (65). Some childhood disorders predisposing to HCC are biliary atresia (66), BSEP deficiency (32), GSD type I (67), tyrosinemia (68), α1-antitrypsin deficiency (69), and Alagille syndrome (70). Fibrolamellar HCC is a distinct pathologic variant usually affecting adolescents and young adults without an underlying liver disease; better survival in this entity is presumably due to the young age of the patients and the lack of cirrhosis making aggressive surgical resections possible (71).…”

Section: Liver Tumorsmentioning

confidence: 99%

Liver Biopsy in Modern Clinical Practice

Ovchinsky

Moreira

Lefkowitch

et al. 2012

Advances in Anatomic Pathology

109

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“…Liver morphology may be abnormal, and, when associated with delayed excretion of radionuclide tracer, suggests the diagnosis of Alagille's [66]. Liver morphology may be abnormal, and, when associated with delayed excretion of radionuclide tracer, suggests the diagnosis of Alagille's [66].…”

Section: Syndromic Paucity Of Interlobular Bile Ductsmentioning

confidence: 99%

Biliary Disease in Infants and Children

Superina¹

2006

Diseases of the Gallbladder and Bile Ducts

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In judicandus ye are a physician, in curando, a surgeon. The patient asks for cure -surgery -and not for theory -medicine -it is the doctor who needs the latter. That is: there can be no surgeon who is not also a physician; the latter begets the surgeon and the surgeon tests the physician by the result of his work. Paracelsus O B J EC T I V E S• Become familiar with the diagnostic approach to the child with jaundice • Understand the pathogenesis and treatment strategies used in children with biliary atresia • Classify and discuss the treatment strategies in children with choledochal cysts • List the varying types of progressive familial intrahepatic cholestasis (PFIC)

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Arteriohepatic dysplasia (Alagille's syndrome): unusual hepatic architecture and function

Abstract: A diagnosis of Alagile's syndrome is suggested when a large, deformed and somewhat spherical liver is encountered, especially when hepatobiliary imaging studies demonstrate delayed excretion of radiopharmaceutical.

Cited by 12 publications

References 14 publications

Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

Liver Biopsy in Modern Clinical Practice

Biliary Disease in Infants and Children

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