Idiopathic Hepatic Copper Toxicosis in a Child

Maggiore, Giuseppe; Giacomo, C De; Sessa, Fausto; Gr, Burgio

doi:10.1097/00005176-198711000-00028

Cited by 34 publications

(14 citation statements)

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“…poorly incorporated into ceruloplasmin at the Golgi when the translocase is defective 11 , excretion is diminished leading to the metal sequestration in lysosomes 12 , and typically a copper pan-toxicosis ensues severely damaging the liver and central nervous system. While the Wilson disease is Mendelianlinked to ATP7B, the ethiopathology of three hepatic copper toxicosis described in humans that are not Mendelian-linked to ATP7B remains unknown [13][14][15] , pointing to our limited knowledge of the mechanisms that function in copper disposal in the liver.…”

Section: Dysfunction Of Atp7b Disrupts Copper Homeostasis and Is Respmentioning

confidence: 99%

ATP7B Copper-Regulated Traffic and Association With the Tight Junctions: Copper Excretion Into the Bile

et al. 2008

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Whereas the ATP7B retained in the trans-Golgi-network is massively translocated to the bile canalicular membrane in response to increased copper levels, a pool of ATP7B associated with the tight junctions remains stable. In situ studies indicate that copper is excreted into the bile by 2 separate pathways. The results are discussed in the frame of the normal and impeded excretion of copper into the bile.

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Section: Dysfunction Of Atp7b Disrupts Copper Homeostasis and Is Respmentioning

confidence: 99%

ATP7B Copper-Regulated Traffic and Association With the Tight Junctions: Copper Excretion Into the Bile

et al. 2008

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“…Apparently the imposition of a high copper intake upon a neonatal liver with its physiological inefficiency in bitiary excretion leads to rapid accumulation of copper, liver damage and a vicious cycle of continued accumulation and continued inefficiency of biliary excretion of copper. This explanation has become even more plausible since a connection has been made between the Indian cases and a small number of babies with a similar liver disease described from Australia [40], Federal Republic of Germany [29], Italy [26] and the United States of America [25]. All these babies received artificial feeds containing copper-contaminated water.…”

Section: Indian Childhood Cirrhosismentioning

confidence: 99%

Copper and liver disease

Danks

1991

Eur J Pediatr

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There has been some limited progress in the understanding of the basic defect in Wilson disease and the gene concerned has been located to the chromosome region 13q14. Treatment with zinc has emerged as a definite alternative to penicillamine administration and some shortcomings and/or hazards of both forms of therapy have emerged as their modes of action have been studied more carefully. Tetrathimolybdate may have a place in treatment, especially when rapid complexing of copper is important. Hepatic copper accumulation occurs in a number of cholestatic diseases and they play an important part in pathogenesis and can occasionally lead to neurologic toxic effects. Copper overload in the newborn period when biliary excretion of copper is inefficient may establish a vicious cycle of copper accumulation and liver damage in Indian childhood cirrhosis and less frequently in babies in other countries.

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“…Drinking water levels were reported at between 2 and 3 mg/L. In Italy, liver cirrhosis due to copper accumulation in the liver has been reported in a 10year-old boy [40]. So far, very few cases of copperstorage disease of the liver have been reported outside of India and it is reasonable to assume that this disease develops only in individuals with special sensitivity.…”

Section: Adverse Health Effects Of Excessive Copper Intakementioning

confidence: 99%

Human health effects of metals in drinking water: Relationship to cultural acidification

Nordberg

1990

Enviro Toxic and Chemistry

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Under certain conditions high concentrations of lead or cadmium in drinking water have occurred as a result of contamination from piping and distribution systems Contamination has led to acute and chronic toxicity in humans Metal concentration is increased when the acidity and corrosivity of water increase due to cultural acidification Adverse health effects have occurred in sen sitive subsections of human populations exposed to lead contaminated acidic water Neutralization of the acidity can reduce these problems Acidic water may also dissolve copper from piping systems Cirrhosis of the liver has been reported in children in India (Indian Childhood Cirrhosis – ICC), commonly with fatal outcome High liver copper levels in ICC cases have been related to copper exposure from cooking utensils Some rare cases reported from Germany and Italy have been partly attributed to high concentrations of copper in drinking water and partly to a special sensitivity to copper toxicity Elevated aluminum concentrations may occur in acidified natural waters and as a result of the aluminum chemicals used in drinking water purification Use of water con taming high aluminum levels for dialysis may give rise to dialysis dementia and disorders of bone mineral metabolism A possible relationship between aluminum in drinking water and Alzheimer's disease requires further investigation

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Idiopathic Hepatic Copper Toxicosis in a Child

Cited by 34 publications

References 0 publications

ATP7B Copper-Regulated Traffic and Association With the Tight Junctions: Copper Excretion Into the Bile

ATP7B Copper-Regulated Traffic and Association With the Tight Junctions: Copper Excretion Into the Bile

Copper and liver disease

Human health effects of metals in drinking water: Relationship to cultural acidification

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