(Q40966808)

25 September 2017

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title

Pediatric gastroenterology. Update on metabolic liver disease (English)

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25 September 2017

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Sarah J Schwarzenberg

series ordinal

1

object named as

Schwarzenberg SJ

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25 September 2017

2

Sharp HL

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25 September 2017

publication date

1 February 1996

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Pediatric Clinics of North America

25 September 2017

published in

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25 September 2017

volume

43

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25 September 2017

issue

1

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25 September 2017

page(s)

27-56

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The interaction of copper (Cu++) with the erythrocyte membrane and 2,3-dimercaptopropanesulphonate in vitro: a source of activated oxygen species

25 September 2017

cites work

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Evaluation of a transgenic mouse model for alpha-1-antitrypsin (AAT) related liver disease

7 January 2021

1 reference

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A case of congenital cirrhosis of the liver with renal tubular defects akin to those in the Fanconi syndrome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Effect of hematin administration to patients with protoporphyria and liver disease

7 January 2021

1 reference

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Iron therapy for hepatic dysfunction in erythropoietic protoporphyria

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Liver transplantation in a patient with protoporphyria.

7 January 2021

1 reference

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Heme synthetase deficiency in human protoporphyria. Demonstration of the defect in liver and cultured skin fibroblasts

7 January 2021

1 reference

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Initial therapy of patients with Wilson's disease with tetrathiomolybdate

7 January 2021

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https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Use of zinc-copper metabolic interactions in the treatment of Wilson's disease

7 January 2021

1 reference

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Treatment of Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Cloning the Wilson disease gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Inborn errors of bile acid metabolism

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

3-Oxo-delta 4 bile acids in liver disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Familial neonatal hemochromatosis with survival

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Absorption, transport, and hepatic metabolism of copper and zinc: special reference to metallothionein and ceruloplasmin

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Molecular studies of ceruloplasmin deficiency in Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Cardiac Abnormalities in Primary Hemochromatosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Hepatic regenerating nodules in hereditary tyrosinemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Primary liver cancer in genetic hemochromatosis: a clinical, pathological, and pathogenetic study of 54 cases.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Faetal and neonatal hepatitis and its sequelae

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Neonatal hepatitis induced by alpha 1-antitrypsin: a transgenic mouse model

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Tyrosinaemia type I and hypertrophic obstructive cardiomyopathy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Genetic analysis using the polymerase chain reaction

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Coagulation defect of congenital tyrosinaemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Prognostic factors for hepatocellular carcinoma in genetic hemochromatosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Mechanisms of hemolysis induced by copper

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Idiopathic hemochromatosis, an iron storage disease. A. Iron metabolism in hemochromatosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Early liver transplantation is indicated for tyrosinemia type I

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Histopathology of α1-antitrypsin liver disease in a transgenic mouse model

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Recessive inheritance of erythropoietic protoporphyria with liver failure

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Instability of hematin used in the treatment of acute hepatic porphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Acute hereditary tyrosinaemia type I: clinical, biochemical and haematological studies in twins

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

A single mutation of the fumarylacetoacetate hydrolase gene in French Canadians with hereditary tyrosinemia type I

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Pregnancies from biopsied human preimplantation embryos sexed by Y-specific DNA amplification

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Reversible pancytopenia secondary to treatment with tetrathiomolybdate.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Neonatal hemochromatosis: diagnosis with MR imaging

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria: unusual skin and neurological problems after liver transplantation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Treatment of Wilson's disease with zinc. I. Oral zinc therapy regimens

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Bone-marrow transplantation for congenital erythropoietic porphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Hemolytic anemia in protoporphyria: possible precipitating role of liver failure and photic stress

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

An analysis of liver transplant experience from 37 transplant centers as reported to Medicare.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Persistent iron overload 4 years after inadvertent transplantation of a haemochromatotic liver in a patient with primary biliary cirrhosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

7 January 2021

Cardiac Wilson's disease

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Human Erythropoietic Protoporphyria: Two point mutations in the ferrochelatase gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Loop-sheet polymerization: the structural basis of Z alpha 1-antitrypsin accumulation in the liver

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The mechanism of Z alpha 1-antitrypsin accumulation in the liver

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Intractable neurological Wilson's disease treated with orthotopic liver transplantation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria and iron therapy

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Haemochromatosis presenting as severe cardiac failure in a young adolescent

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Liver transplantation for tyrosinemia. A review of 10 cases from the University of Pittsburgh

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Neurologic crises in hereditary tyrosinemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Coagulopathy Associated with Hematin Treatment for Acute Intermittent Porphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Survival and causes of death in cirrhotic and in noncirrhotic patients with primary hemochromatosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Neurological crisis in hereditary tyrosinaemia and complete reversal after liver transplantation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria and hepatic complications

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Copper disposition of the fetus and placenta in a patient with untreated Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Plasma copper and antioxidant status in Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Genetic haemochromatosis--preventable rust

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Determination of hepatic iron concentration in fresh and paraffin-embedded tissue: diagnostic implications

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Mapping, cloning and genetic characterization of the region containing the Wilson disease gene.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Prenatal diagnosis of type I hereditary tyrosinaemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Protoporphyrin metabolic balance in human protoporphyria.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The effect of liver transplantation in a 13-year-old boy with erythropoietic protoporphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Primary and secondary amenorrhea associated with spironolactone therapy in chronic liver disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

High prevalence of viral infection in adults with homozygous and heterozygous alpha 1-antitrypsin deficiency and chronic liver disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Early involvement of hepatic parenchymal cells in erythrohepatic protoporphyria? An ultrastructural study of patients with and without overt liver disease and the effect of chenodeoxycholic acid treatment

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Accumulation of iron in erythroblasts of patients with erythropoietic protoporphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Hematin therapy for the neurologic crisis of tyrosinemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Liver in disorders of porphyrin metabolism

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Orthotopic liver transplantation for hepatic complications of Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Initial and follow-up brain MRI findings and correlation with the clinical course in Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Tyrosinemia type 1--complex splicing defects and a missense mutation in the fumarylacetoacetase gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Fulminant hepatic failure resulting from coexistent Wilson's disease and hepatitis E

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Recessive inheritance of erythropoietic protoporphyria with liver failure

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Screening for mutations by RNA single-strand conformation polymorphism (rSSCP): comparison with DNA-SSCP

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The use of trientine in preventing the effects of interrupting penicillamine therapy in Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Liver transplantation for Wilson's disease: indications and outcome

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Delta 4-3-oxosteroid 5 beta-reductase deficiency causing neonatal liver failure and hemochromatosis

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Molecular defects in erythropoietic protoporphyria with terminal liver failure

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Molecular and cytogenetic analysis of an interstitial 20p deletion associated with syndromic intrahepatic ductular hypoplasia (Alagille syndrome)

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria: evidence for multiple sites of excess protoporphyrin formation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Z and the insoluble answer

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

At long last: an animal model of Wilson's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

A sequential study of the skeletal abnormalities which develop in rats given a small dietary supplement of ammonium tetrathiomolybdate.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Chimerism after liver transplantation for type IV glycogen storage disease and type 1 Gaucher's disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Tyrosinemia with acute intermittent porphyria: aminolevulinic acid dehydratase deficiency related to elevated urinary aminolevulinic acid levels

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria.

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Identification of a single base pair deletion (40 del G) in exon 1 of the ferrochelatase gene in patients with erythropoietic protoporphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria, transfusion therapy and liver disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Erythropoietic protoporphyria in the house mouse. A recessive inherited ferrochelatase deficiency with anemia, photosensitivity, and liver disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Chenodeoxycholic acid therapy in erythrohepatic protoporphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Blood exchange and transfusion therapy for acute cholestasis in protoporphyria

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

The occurrence of hepatoma in the chronic form of hereditary tyrosinemia

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Progressive hemochromatotic cardiomyopathy despite reversal of iron deposition after liver transplantation

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

PROGRESSIVE LENTICULAR DEGENERATION: A FAMILIAL NERVOUS DISEASE ASSOCIATED WITH CIRRHOSIS OF THE LIVER

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Isolation and characterization of a human liver cDNA as a candidate gene for Wilson disease

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Exclusion of ferritins and iron-responsive element (IRE)-binding proteins as candidates for the hemochromatosis gene

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

Wilson's disease presenting as symptomatic urolithiasis: a case report and review of the literature

7 January 2021

1 reference

https://api.crossref.org/works/10.1016%2FS0031-3955%2805%2970396-5

inferred from DOI database lookup

7 January 2021

based on heuristic

Identifiers

DOI

10.1016/S0031-3955(05)70396-5

1 reference

25 September 2017

1 reference