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Epidemiological surveys in the foci of ALS of the Kii Peninsula of Japan started in the early 1960s. Continuous surveys conducted for decades revealed that there have been two foci in the Kii Peninsula: one in Kozagawa in the southern part, and the other in Hobara in the south-east. Clinically, ALS patients of the Kii foci occasionally showed parkinsonian features or dementia that have not been reported in the sporadic form of ALS. Neuropathologically, numerous NFT that are identical to those of Alzheimer's disease were observed in the cerebral cortex and in the brainstem nuclei. To elucidate the etiopathogenesis of this unique form of ALS, an analysis was conducted of the environment in the focus areas and of the specimens from the patients with ALS. It was hypothesized that the long exposure of these environments to low calcium and magnesium, and an excess of aluminum and manganese in the drinking water and the soil, might lead to the deposition of some trace elements in the CNS, eventually causing neuronal degeneration and death. [ABSTRACT FROM AUTHOR]

The cause of biliary atresia (BA) remains an enigma. However, an ethnic diversity in the incidence of BA is so unique that anthropological approach may provide some etiopathogenetic implications in the disease mechanism. We previously reported that an association of maternal microchimerism (MMc) in BA and a significant compatibility of HLA-A between the patient with BA and their mother. Across the 10 countries (Japan, South Korea, Taiwan, Philippines, New Zealand (Maori population), UK, France, Germany, Norway, and Sweden), we determined the frequency of the most prevalent HLA haplotypes of each country from Allele Frequency Database and found that it was significantly correlated with the incidence of BA of the respective country (p = 0.0126). This observation better fits the MMc theory as an etiopathogenesis, that is, maternal effector cells are likely to migrate into the fetus in a relatively homogenous population and may damage the developing bile duct structure and portal vein endothelium, depending on materno-fetal tolerance and immunity., (Copyright © 2018. Published by Elsevier Inc.)