Orthotopic Liver Transplantation for Autoimmune Hepatitis and Cryptogenic Chronic Hepatitis in Children1

Bahar, Ron; Yanni, George; Martin, Mart n G.; McDiarmid, Sue V.; Vargas, Jorge; Gershman, George; Heyman, Melvin B.; Rosenthal, Philip; Tipton, James R.; Nanjundiah, Prathiba; Starr, Amy J.; Ament, Marvin E.

doi:10.1097/00007890-200109150-00015

Cited by 32 publications

(22 citation statements)

References 34 publications

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“…These patients often appear to be steroid dependent and have variable degrees of portal and lobular inflammation along with bridging fibrosis [43,44].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Patients who undergo liver transplantation for AIH-associated liver failure or end-stage liver disease are at risk to develop recurrence of their AIH in the transplanted organ. In addition, development of AIH de novo in pediatric transplant patients has been reported [43,44,50]. Patients with unexplained elevation in aminotransferase levels who do not respond to increased cyclosporine or tacrolimus should be evaluated for evidence of AIH.…”

Section: Treatmentmentioning

confidence: 99%

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Autoimmune hepatitis in children

Squires

2004

Curr Gastroenterol Rep

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Autoimmune hepatitis (AIH) remains an enigmatic condition that affects children of all ages. Although much knowledge has emerged over the past four decades regarding autoimmune diseases, the lack of a spontaneous animal model has hindered a more complete understanding of the pathophysiology of AIH. Serum autoimmune markers, combined with biochemical and histologic evidence of hepatocellular injury and absent other diagnostic considerations, are necessary to diagnose AIH. The clinical spectrum ranges from asymptomatic elevation of aminotransferase levels to acute liver failure. Patients with AIH type 1 are more likely to be older and have cirrhosis at the time of diagnosis, whereas those with AIH type 2 may have a more fulminant course and require life-long immunosuppressant medications. Overlap syndromes occur in children but are rare. Treatment with prednisone and azathioprine, alone or in combination, reverses the clinical course and improves biochemical abnormalities. Should liver transplantation be necessary, autoimmune injury may recur in the allograft.

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“…These patients often appear to be steroid dependent and have variable degrees of portal and lobular inflammation along with bridging fibrosis [43,44].…”

Section: Clinical Featuresmentioning

confidence: 99%

Section: Treatmentmentioning

confidence: 99%

Autoimmune hepatitis in children

Squires

2004

Curr Gastroenterol Rep

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“…A late consequence of the disease is cryptogenic cirrhosis, but the frequency of progression to cirrhosis is unclear. Children with cryptogenic chronic hepatitis appear to have more aggressive disease than children with type 1 autoimmune hepatitis, and the frequency of liver transplantation (86% vs. 51%) has been higher in these children [76]. Patients with cryptogenic chronic hepatitis and autoimmune features respond well to corticosteroids [61][62][63], and the frequencies of remission (83% vs. 78%) and treatment failure (9% vs. 11%) have been similar between these patients and those with classical autoimmune hepatitis [2].…”

Section: Outcomementioning

confidence: 95%

“…Further refinement of the diagnostic category by excluding features of NAFLD reduced the number of patients previously considered to have cryptogenic chronic liver disease by 28-73% [31,51,59,73,74]. The frequency of cryptogenic cirrhosis in patients with cirrhosis ranged from 5 to 30% in studies through 2005, and this diagnosis in liver transplantation centers has ranged from 3-14% in adults [60,70,75] to 22% in children [76] over the past decade.…”

Section: Frequency Of Cryptogenic Chronic Hepatitismentioning

confidence: 99%

“…In this regard, it has the same natural history as patients with classical forms of chronic liver disease, and it cannot be discounted as an inactive, nonaggressive disorder. Studies that indicate that it may be more aggressive in children than autoimmune hepatitis [76], have modifier gene mutations that distinguish it from other chronic liver diseases [75,197], recur after liver transplantation in different forms than patients with other original diagnoses [53,60], and have poorer 1-and 5-year survivals after liver transplantation [70] support the possibility that within the designation of cryptogenic chronic hepatitis there may exist an independent, greatly modified, or atypical form of chronic liver disease whose natural history and management are unclear.…”

Section: Outcomementioning

confidence: 99%

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Cryptogenic Chronic Hepatitis and Its Changing Guise in Adults

Czaja

2011

Dig Dis Sci

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Cryptogenic chronic hepatitis is a disease that is unexplained by conventional clinical, laboratory and histological findings, and it can progress to cirrhosis, develop hepatocellular carcinoma, and require liver transplantation. The goals of this review are to describe the changing phenotype of cryptogenic chronic hepatitis in adults, develop a diagnostic algorithm appropriate to current practice, and suggest treatment options. The frequency of cryptogenic hepatitis is estimated at 5.4%. Cryptogenic cirrhosis is diagnosed in 5-30% of patients with cirrhosis, and it is present in 3-14% of adults awaiting liver transplantation. Nonalcoholic fatty liver disease has been implicated in 21-63% of patients, and autoimmune hepatitis is a likely diagnosis in 10-54% of individuals. Viral infections, hereditary liver diseases, celiac disease, and unsuspected alcohol or drug-induced liver injury are recognized infrequently in the current cryptogenic population. Manifestations of the metabolic syndrome heighten the suspicion of nonalcoholic fatty liver disease, and the absence of hepatic steatosis does not discount this possibility. The diagnostic scoring system of the International Autoimmune Hepatitis Group can support the diagnosis of autoimmune hepatitis in some patients. Certain genetic mutations may have disease-specificity, and they suggest that some patients may have an independent and uncharacterized disease. Corticosteroid therapy is effective in patients with autoimmune features, and life-style changes and specific therapies for manifestations of the metabolic syndrome are appropriate for all obese patients. The 1- and 5-year survivals after liver transplantation have ranged from 72-85% to 58-73%, respectively.

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