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Text
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<a href="http://doi.org/10.1016/s0022-3476(05)80736-x" target="_blank" rel="noreferrer">http://doi.org/10.1016/s0022-3476(05)80736-x</a>
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Title
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Liver involvement in Alpers disease
Publisher
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The Journal Of Pediatrics
Date
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1991
Subject
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Child; Female; Humans; Male; Biopsy; Preschool; Syndrome; infant; Q3 Literature Search; Brain/pathology; Liver Function Tests; Diffuse Cerebral Sclerosis of Schilder/diagnosis/metabolism/pathology; Liver/metabolism/pathology; Status Epilepticus/diagnosis/metabolism/pathology
Creator
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Narkewicz MR; Sokol RJ; Beckwith B; Sondheimer J; Silverman A
Description
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Alpers disease consists of diffuse cerebral degeneration manifested as developmental delay, seizures, vomiting, and progressive neuromuscular deterioration, with liver disease and death. We report the clinical course of the liver disease, histologic progression of the hepatic lesions, and etiologic investigations in five patients (four girls, three kinships). All had grown and developed normally until seen at 6 to 36 months of age (mean 20 months), with vomiting (n = 5), progressive hypotonia (n = 3), or seizures (n = 2). All had been given anticonvulsants, including valproic acid in three. Liver disease was noted at a mean age of 35 months (range 9 to 67 months), with hepatomegaly (two patients), abnormal hepatic synthetic function (three) or transaminase values (three), and cirrhosis in one. Patients survived for a mean of 4.6 weeks (range 1 to 8 weeks) after the identification of liver disease; all died of hepatic failure. Results of evaluation for infectious and metabolic causes of liver disease and causes of degenerative neuromuscular disease were negative in all patients. Premortem liver biopsy specimens (n = 3) demonstrated an early lesion consisting of lobular disarray, microvesicular steatosis, periportal acute and chronic inflammation, and individual hepatocyte necrosis. Autopsy findings (n = 5) consisted of macrovesicular steatosis, massive hepatocyte dropout, and proliferation of bile ductular elements, with almost complete replacement of hepatocytes by proliferating bile ductular elements in two patients. Brain showed characteristic neuronal degeneration. We conclude that Alpers disease can be a cause of rapidly progressive liver failure in early childhood. Although the cause of this autosomal recessive disease is not known, it does not appear to be related to peroxisomal dysfunction.
1991
Identifier
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<a href="http://doi.org/10.1016/s0022-3476(05)80736-x" target="_blank" rel="noreferrer">10.1016/s0022-3476(05)80736-x</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Type
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Journal Article
1991
Backlog
Beckwith B
Biopsy
Brain/pathology
Child
Diffuse Cerebral Sclerosis of Schilder/diagnosis/metabolism/pathology
Female
Humans
Infant
Journal Article
Liver Function Tests
Liver/metabolism/pathology
Male
Narkewicz MR
Preschool
Q3 Scoping Review Results
Silverman A
Sokol RJ
Sondheimer J
Status Epilepticus/diagnosis/metabolism/pathology
Syndrome
The Journal Of Pediatrics