Chronic copper toxicosis presenting as liver failure in an australian child

Pyrrolizidine Alkaloids: Potential Role in the Etiology of Cancers, Pulmonary Hypertension, Congenital Anomalies, and Liver Disease

“…In considering and selecting the six indicators comprising the above list, several cases of liver disease and cancers with a possible dehydroPA etiology have been identified. [158][159][160]163,168,176,[222][223][224][225][226][231][232][233][234][236][237][238]245,[247][248][249]259,260 Of these, ten cases 159,160,176,223,237,238,245,247−249 display at least f o u r o f t h e s u g g e s t e d i n d i c a t o r s , n i n e others 158,163,222,224,225,232,233,258,260 have three indicators, and five other cases 168,226,231,234,236 have two of the suggested indicators. Some of these cases also showed other features, including hypertrophic hepatocytes (ballooned, swelling), 222,232,233,237,238 inconsistent veno-occlusive disease (HSOS), 238 and rare mitotic figures ...…”

“…Dietary exposure to dehydroPAs should also be considered for all cases of cirrhosis of unknown etiology, especially those showing evidence of HSOS. While clinicians dealing with cases of HSOS, especially in neonates and young children, often question the mothers of the patients involved in regard to their consumption of herbal medicines containing dehydroPAs, − the potential for dehydroPAs being present in foods they have consumed, such as milk, honey, flour, tea, and eggs, is not normally considered.…”

“…Environmental copper is known to exacerbate the hepatotoxicity of dehydroPAs in livestock and to result in accumulation of very high levels of copper in the cirrhotic liver. ,,,− The poisoning of livestock by dehydroPAs was at one time called chronic copper toxicity until it was shown that this particular condition also required exposure to plants containing dehydroPAs. , Numerous investigations have sought to explain individual examples and clusters of cirrhosis in young children that have occurred in Germany, Austria, India, the USA, Australia, and elsewhere. Such cases are sometimes associated with copper accumulation. − ,− Many investigations have concluded, however, that high levels of environmental copper are not the only cause. ,, Some have suggested that other environmental agents, including exposure to dehydroPAs , and perhaps a non-Wilsonian genetic predisposition to copper accumulation and toxicity, may be required. ,− ,− This situation is very reminiscent of the historic debate in the 1950s on the etiology of chronic copper toxicity in livestock. ,, Cirrhosis accompanied by copper accumulation is, therefore, another important indication for suspecting possible dietary dehydroPA involvement.…”

“…In considering and selecting the six indicators comprising the above list, several cases of liver disease and cancers with a possible dehydroPA etiology have been identified. − ,,,,− ,− ,− ,,− ,, Of these, ten cases ,,,,,,,− display at least four of the suggested indicators, nine others ,,,,,,,, have three indicators, and five other cases ,,,, have two of the suggested indicators. Some of these cases also showed other features, including hypertrophic hepatocytes (ballooned, swelling), ,,,, inconsistent veno-occlusive disease (HSOS), and rare mitotic figures that are indicative of a dehydroPA etiology.…”

Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl

“…ICC, endemic in India, has occurred sporadically in the USA [11], United Kingdom [1,8], Australia [18,25], Austria [13] and Germany [3,4,12,14,15,26]. Usually, ICC manifests in early infancy by a rapidly progressing necrosis of liver cells with high mortality.…”