[Two cases of oral-facial-digital (O-F-D) syndrome (author's transl)].
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Biomedical subjects
Publications and source records attributed to T Wada.
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Data on maxillary growth for 62 normal and 87 complete unilateral cleft lip and palate subjects presented in 1975 is supplemented and extended. The children ranged in age from six months to four years and were evaluated at four developmental stages. In order to clarify the character of the maxillary arch deformity, measurements of depth, width, height, and length were made and analyzed statistically. Prior to lip repair, cleft and normal subjects showed similar patterns of maxillary growth. This similarity was not apparent by age two. Anterior-posterior growth inhibition of the maxilla appeared to occur after lip repair and to remain a problem by age four. The authors discuss possible ways of minimizing these differences by management techniques.
Single cell suspensions, obtained from rat kidneys, were cultured with various concentrations of HgCl2 for different times to examine the effects on cellular synthetic activity. Unexpectedly, the RNA synthesis of kidney cells was stimulated with low doses of HgCl2, whereas it was inhibited with higher doses. Even at a higher dose, RNA synthesis was stimulated during early incubation but was inhibited during longer incubations. Thus, the effect of mercury showed a biphasic pattern both in dose-response and time-response relationships. Moreover, once the cells were exposed to HgCl2 for a certain period, RNA syntehsis was irreversibly inhibited. Since RNA synthesis was inhibited earlier than DNA or a protein synthesis, the RNA synthesizing system appeared most susceptible. Without HgCl2, by the time DNA synthesis reached its maximum, protein and RNA synthesis had already declined. In contrast, RNA synthesis of HgCl2-treated cells remained at its maximal level and showed no decline when DNA synthesis reached its peak.
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Incidences of alpha2H were investigated in sera from patients with various malignant and nonmalignant diseases. With counterimmunoelectrophoresis, alpha2H was detected in a wide variety of neoplastic diseases, and also in nonmalignant hematological disorders. Diagnostic significance was described regarding primary hepatoma and acute myelogenous leukemia. Factors affecting alpha2H levels in sera were also discussed in relation to the neoplastic and nonneoplastic disease process.
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In order to investigate the relationship if any between the circadian rhythm of the hypothalamic-pituitary-adrenal system and of feedback sensitivity in the hypothalamic-pituitary axis, temporal changes of serum corticoid level and of ACTH responsiveness to metyrapone were studied in 5 controls, 4 patients with loss of consciousness due to central nervous system diseases (Group A), 2 blind subjects (Group B) and 2 patients with psychiatric disorders accompanied with sleep-wake cycle disturbance (Group C). Two patients in Group A with normal circadian rhythm of serum corticoid levels exhibited abnormal rhythm in ACTH responsiveness to metyrapone. In contrast, 2 other patients in Group A with normal rhythm in ACTH responsiveness to metyrapone exhibited abnormal circadian rhythms of serum corticoid levels. In spite of the disturbed circadian rhythm of serum corticoid levels, 2 patients in Group B showed normal rhythm in ACTH responsiveness to metyrapone. Two patients in Group C exhibited abnormalities in both circadian changes. It was concluded that there might be different control mechanisms between the circadian rhythmicity of the hypothalamic-pituitary-adrenal system and of the feedback sensitivity.
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Intra-arterial infusion chemotherapy was made through the internal mammary artery and subclavian artery in 12 cases of locally advanced breast cancer. Continuous infusion of 5-fluorouracil and intermittent injections of Mitomycin C were jointly made for 1 to 4 weeks in each artery. Marked response in not only the primary tumor but also lymph node metastasis was clinically observed, permitting extended radical mastectomy successfully in all cases. Remarkable regressive changes were histologically observed in cancer foci, especially in small ones, of resected specimens including axillary, parasternal, and supraclavicular lymph nodes. All cases except 1 have been healthy without any recurrence for 22 to 78 months after treatment. Intra-arterial infusion chemotherapy will this method is a useful preliminary procedure for surgical treatment of locally advanced breast cancer.
This study aims at the comparison of growth changes of the maxillary arch of 62 normals and 87 complete unilateral cleft lip and cleft palate subjects. This is achieved by measuring their maxillo-facial models, which will aid in clarifying such questions as: when and where the maxillary growth inhibition will occur, and the several aspects of growth-change. Patients with complete unilateral cleft lip and cleft palate were classified into four stages: 1) six month old infants before lip and palatal closure; 2) two year old children, lip repair at six months of age; 3) three year old children, lip repair at six months and palatal closure at two years; 4) four year old children, with repairs as in stage 3. Normal subjects were also classified into four stages, to match the cleft group by age and body weight. Impressions were taken for each patient's upper jaw and upper face simultaneously under general anesthesia, and the maxillo-facial model was made. Results obtained were as follows: (1) In the normal group, the forward and downward growth of the anterior alveolar region increased considerably between stages 1 and 2 and stages 3 and 4. The growth of the anterior alveolar arch was slight during all four stages. The depth and width of the retromolar point increased gradually through all four stages, and measured the growth of the posterior alveolar region. (2) In the cleft group: at stage 1 the anterior end of the larger segment was protruded and both the larger and smaller segments, especially in the anterior region, were laterally dislocated. The height of the anterior alveolar region was found to be noticeably less than that of the normal group. At stage 2 the growth inhibition in depth and height was noted in all the alveolar points of the cleft group, especially in the anterior alveolar region, in comparison to the normal group. At stage 3 the depths in all alveolar points decreased, but no significant difference was found when compared to stage 2. However, the increase in heights was marked. At stage 4 the depths and heights of alveolar points in the cleft group were found to be smaller than those of the normal group.