Statistical study of aca-talasemia, a review of thirty-eight cases appearing in the literatures

1. With a view to grasp more simply and clearly the characteristics of this disease and in order to find a clue for prompt discovery of cases when encountered in future, the authors undertook a statistical study of the cases already reported by various authors. 2. The cases reported so far amount to 17 familial groups which consisted of 38 acatalasemic cases. These groups were distributed widely throughout Japan. The disease seemed to be prevalent in the rural communities where adherence to the custom of consanguineous marriage occurs. As yet, we have not heard of the occurrence of this disease in other countries. 3. The disease has equal distribution in both sexes. About one half of patients showed a peculiar oral gangrene (Takahara's disease). The great majority of these were noted in those less than 10 years of age. 4. The great majority of them were children whose parents were united in consanguineous marriage and have siblings with acatalasemia. 5. As for the treatment of oral lesions in this disease, extraction of tooth at the site of the lesions, removal of the diseased tissues en masse by resection, and penicillin treatment given concomitantly are effective. The course and the length of time required in healing of the wound due to the operation are about the same as in the case of normal persons. 6. The authors wish to call special attention to the phenomenon peculiar to the acatalasemic blood. The blood of acatalasemic individuals changes to brownish-black color in the absence of foaming or bubble formation upon the application of hydrogen peroxide to blood.