[Adult-onset cyclic neutropenia: a case report].
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Biomedical subjects
Publications and source records attributed to H Kobashi.
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Three patients with clinically different severities of vitamin D-dependent rickets, type II, with alopecia, which is 1,25-dihydroxyvitamin D-receptor-defect rickets and is particularly resistant to treatment with calciferol analogues, were treated with large doses of 1 alpha-hydroxyvitamin D3 (1 alpha-(OH)D3) and 2 g of calcium lactate. Except for the alopecia, all of the abnormalities of patients 1 and 2 were reversed by treatment with 3 micrograms/kg/d of 1 alpha-(OH)D3, and those of patient 3, who had the severest manifestations, were reversed by treatment with 6 micrograms/kg/d. The serum 24,25-dihydroxyvitamin D concentrations of the three patients were low before treatment and those of patients 1 and 2 increased during treatment. These findings suggest that in patients 1 and 2, 25-hydroxyvitamin D-24-hydroxylase was stimulated via a 1,25-dihydroxyvitamin D-receptor-mediated system by treatment with 1 alpha-(OH)D3.
The interactions of 1,25-dihydroxyvitamin D3 [1,25-(OH)2D3] with phytohemagglutinin (PHA)-stimulated lymphocytes from normal subjects and three patients with vitamin D-dependent rickets (DDR) type II were investigated. Impaired nuclear uptake and normal cytosol binding of [3H]1,25-(OH)2D3 were observed with PHA-stimulated lymphocytes of these patients as with their cultured skin fibroblasts. Furthermore, the incorporation of [14C]thymidine into PHA-stimulated lymphocytes of the patients was not reduced by 1,25-(OH)2D3, which is known to inhibit proliferation of various cells. These findings suggest that 1,25-(OH)2D3 receptors are reduced or absent in patients with DDR type II. Thus, the capacities of cytosol binding and nuclear uptake of 1,25-(OH)2D3 in PHA-stimulated lymphocytes seem to reflect those of endo-organs such as the intestine and bone. These findings show that a test of the effect of 1,25-(OH)2D3 on thymidine incorporation into PHA-stimulated lymphocytes is useful for rapid diagnosis of DDR type II.
Cefixime (CFIX) was given orally to 25 children with acute bacterial infections including 13 with acute tonsillitis, 1 with acute tonsillitis and cervical lymphadenitis, 1 with acute bronchitis, 5 with bronchopneumonia and 5 with urinary tract infection. Good to excellent clinical response was obtained in 23, and bacteriological response was obtained in 14 of the 16 children who underwent bacteriological tests. Side effects with soft stool or eosinophilia were observed in 1 child each. The flavor and odor of CFIX appeared to be well accepted by children. Our clinical experience has suggested the usefulness of this antibiotic for the treatment of various bacterial pediatric infections.
Aspoxicillin (TA-058, ASPC) was given intravenously to 20 children with the following acute bacterial infections; 11 cases of bronchopneumonia, 5 cases of urinary tract infection, 2 cases of acute bronchitis, 1 case of staphylococcal scalded skin syndrome and 1 case of subcutaneous abscess. Clinical effectiveness was obtained in 14 cases out of 20 cases (70%) and bacteriological eradication in 8 cases out of 10 cases (80%). With ASPC the following side effects developed; transient diarrhea in 1 case, eruption in 1 case and slight elevation of GOT in 1 case. From the above clinical results, it is apparent that ASPC is an useful antibiotic for treating pediatric patients with various kinds of bacterial infections.
Ceftizoxime suppositories (CZX-S), containing 250 mg or 125 mg of CZX, were given to 6 children, 4 with acute bronchopneumonia and 2 with acute pharyngobronchitis, who were not suited to treatment with injectable or oral form of the drug. The clinical response was "good" in all the children and the causative organisms were eradicated in 2 children (H. influenzae or S. aureus). Adverse reactions consisted of 1 case each of diarrhea and transiently increased GPT. In conclusion, CZX-S proved to be highly effective in the treatment of bacterial infections in children.
Cefminox (CMNX, MT-141) was given intravenously to 20 children with the following acute bacterial infections; 10 cases of bronchopneumonia, 4 cases of urinary tract infection, 2 cases of staphylococcal scalded skin syndrome, each 1 case of acute pharyngitis, acute tonsillitis, purulent cervical lymphadenitis and acute tonsillitis, and pleuritis. Good clinical responses were obtained in 18 patients out of 20 patients and bacteriological effectiveness in 13 strains out of 14 strains. No side effect was observed except for 1 case with diarrhea and 1 case with slight elevation of GOT and GPT. From the above clinical results, it is apparent that CMNX is a useful antibiotic for treating pediatric patients with various kinds of bacterial infections.
Aztreonam (AZT) was given intravenously to 20 children with the following acute bacterial infections: 13 cases of bronchopneumonia, 5 cases of urinary tract infection, 1 case of cervical lymphadenitis with acute tonsillitis and 1 case of acute enteritis. Clinical effectiveness was obtained in 17 cases of 20 cases and bacteriological effectiveness in 10 strains out of 11 strains. No side effect was observed except for 2 cases with eruption, 1 case with slight elevation of GPT, 1 case with slight elevation of GOT and 1 case with slight elevation of GOT and GPT. From the above clinical results, it is apparent that AZT is a useful antibiotic for treating pediatric patients with various kinds of bacterial infections.
The fructose 1,6-diphosphatase activities in peripheral lymphocytes from the parents of a patient with fructose 1,6-diphosphatase deficiency were lower than the mean value of normal controls, but the value of the mother overlapped lower values for normal controls. The fructose 1,6-diphosphatase activities in lymphocytes of normal adults and the parents increased progressively during in vitro culture, but no enzyme activity could be detected in the lymphocytes of the patient even after culture. None of the values for the parents overlapped those of normal controls on either day 5 or 10 of culture. Thus, it seems probable that heterozygotes for fructose 1,6-diphosphatase deficiency can be distinguished from normal individuals by measuring the fructose 1,6-diphosphatase activity in their cultured lymphocytes.
The effects of dichloroacetate (DCA) on the activity of the pyruvate dehydrogenase (PDH) complex and associated changes in the lactate and glucose levels in rat brain were investigated in vivo. The average activities of the active form of the PDH complex in the brain, liver and muscle of starved rats were respectively 0.40 +/- 0.04, 0.07 +/- 0.04, and 0.17 +/- 0.11 mumol/min/g tissue, and amounted to 21, 11, and 16% of the total activity of the complex. Intraperitoneal injection of DCA (125 mg/kg) increased the percentage of the active form of the PDH complex in the brain, liver, and muscle to 107, 40, and 84%, respectively. DCA significantly lowered the lactate and glucose concentrations of the brain and blood. A lower dose of DCA (12.5 mg/kg) also caused significant increase in activity of the PDH complex in the brain, but did not significantly change the lactate or glucose concentration of the brain. These results suggest that DCA crosses the blood-brain barrier reasonably well.
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