[A case of a Japanese family with Hb San Diego [beta 109(G11)Val----Met]-beta zero thalassemia].
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Biomedical subjects
Publications and source records attributed to K Nibu.
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Asymmetrical cross-linked FS (alpha 2 gamma beta s) hybrid hemoglobin (Hb FS-fumarate) was prepared by reacting mixtures of hemoglobins F and S with double-headed aspirin, bis(3,5-dibromosalicyl) fumarate. When the molar ratio of hemoglobin to the cross-linking agent was 1 to 2 in a 1:1 FS mixture, the relative ratio of the products, cross-linked hemoglobins F (Hb F-fumarate), FS (HB FS-fumarate), and S (Hb S-fumarate), was 1.0:2.6:2.0, in contrast to a 1:2:1 ratio of cross-linked hemoglobins A, AS, and S in a 1:1 AS mixture. These results suggest that the fumaryl group reacts differently with Hb F, Hb FS and Hb S, and that the difference could be attributed to the difference in the structure in the vicinity of the EF6 Lys of non alpha-chains. The oxygen-binding properties of Hb F-fumarate, Hb FS-fumarate, and Hb S-fumarate were similar, except that the n-value of Hb F-fumarate was slightly lower than n-values of Hb S-fumarate and Hb FS-fumarate. Kinetic studies on aggregation showed that the addition of Hb FS-fumarate to unmodified Hb S did not affect the delay time prior to aggregation, but did increase the total turbidity. Electrophoretic and densitometric scanning analysis of the aggregate phase of this mixture showed the fraction of Hb FS-fumarate to be 19%. Hb F-fumarate's effect on the delay time is concentration-dependent; the greater the concentration of Hb F-fumarate, the longer the delay time. The turbidity after aggregation of the mixture of Hb S and Hb F-fumarate was much less than that of Hb S and Hb FS-fumarate. However, the fraction of Hb F-fumarate in the aggregate phase was 19%, which is similar to that of Hb FS-fumarate. These data suggest that Hb F and FS hybrid hemoglobin cannot participate in nuclei formation, but can participate in aggregation after sufficient amounts of nuclei are formed from Hb S, and that increased levels of Hb F do not have an inhibitory effect on the formation of nuclei but on the growth of aggregates.
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PURPOSE: As locoregional control of head and neck cancer has improved, distant metastases have become increasingly common problems. PATIENTS AND METHODS: To determine the role of surgical treatment, we reviewed 32 patients with squamous cell carcinoma (SCC) of the head and neck who underwent thoracotomy for pulmonary metastases. RESULTS: The overall 5-year survival rate was 32%. The 5-year survival rate of the patients with SCC of the oral cavity was significantly poorer than that of the patients with other primary site (15.4% v 45.2%; P = .01). In the patients with single nodule, extent of the tumor was a significant prognostic factor (P = .007). Mediastinal lymph node involvement (P = .004) and pleural invasion (P = .04) also correlated with survival. CONCLUSION: TNM classification of the primary tumor did not have an impact on survival in this study. Further studies of a large series should be performed to determine the indications and modalities of the surgical treatment for pulmonary metastases of the SCC of head and neck.
A total of 62 patients with standard-risk acute lymphoblastic leukemia received three-drug induction consisting of vincristine, prednisolone, and L-asparaginase (l-Asp) followed by consolidation therapy with intermediate-dose methotrexate (MTX), intrathecal MTX, and 18 Gy of cranial irradiation. Maintenance therapy consisting of 6 drugs including daunorubicin (DNR, 450 mg/m2 in total) was continued for 3 years. Patients were randomized and half of them received weekly l-Asp during maintenance therapy as a late intensification. Complete remission (CR) was achieved in 61/62 (98.4%), and 11 of 61 patients relapsed. At 10 years, the event-free survival (EFS) was 80.6 +/- 5.0% and overall survival was 88.7 +/- 4.0%; median follow-up time was 9.3 years. The 10-year EFS of patients with additional l-Asp (84.8 +/- 6.2%) was superior to that without l-Asp (75.9 +/- 7.9%), although it was not statistically significant. No patients who received a full dose of DNR and maintained CR developed heart failure, although the shortening fraction decreased from 41.0% at diagnosis to 35.2% (median). The protocol AL841 provided good long-term disease control without severe late cardiac dysfunction.
A 63-year-old man had undergone hypopharyngectomy for hypopharyngeal carcinoma in 1985. A free radial forearm flap was used for reconstruction of the pharynx. Ten years after the surgery, a second primary squamous cell carcinoma occurred in the neopharynx. Persistent exposure to alcohol, saliva and foodstuffs was considered as a possible cause of second primary carcinoma. Long-term follow-up is indicated in patients undergoing reconstruction using cutaneous or musculocutaneous flaps.
We evaluated the efficacy and toxicity of aclarubicin for acute non-lymphocytic leukemia (ANLL) refractory to daunorubicin in childhood. Twenty-four patients were treated with aclarubicin and prednisolone with or without 6-mercaptopurine and behenoyl-cytosine arabinoside daily for 5 to 14 days. Of 21 evaluable patients, 14 (67%) responded: 12 obtained complete remission and 2 partial remission. The median time to reach complete remission was 37 days (range, 16 to 60 days), and the median duration of complete remission was 5.5 months (range, 2 to 41 months). The cumulative dose of anthracycline administered before the study was not considered significant for the response. The only major complication was severe bone marrow suppression; infectious episodes occurred in 14 patients (58%) and three died of sepsis and/or bleeding. The observed non-hematologic toxicities included hematuria, an elevation of serum amylase, nausea/vomiting, and angitis. In addition, one patient showed abnormal cardiac function. Aclarubicin is therefore considered a highly active drug for remission reinduction of previously treated children suffering from ANLL with an acceptable toxicity.
Sixty-three patients with squamous cell carcinoma of the hypopharynx were treated at the University of Tokyo between 1985 and 1993. Twelve patients were treated with surgery alone, 16 with preoperative irradiation, 26 with postoperative irradiation, and nine with irradiation alone. A tumor dose of 50 Gy (25 fractions/5 weeks) was used in postoperative irradiation, 40-60 Gy in preoperative irradiation, and 70 Gy in irradiation alone. When "number of indications," including, for example, partial pharyngectomy, positive or close (< or = 5 mm) margin, and extracapsular extension, was used for multivariate analysis in patients who underwent surgery, it proved to be the only significant prognostic factor for gross survival. Six of seven patients with a positive surgical margin or close margin who received postoperative irradiation had local recurrence. A tumor dose of 50 Gy (25 fractions/5 weeks) in postoperative irradiation is not enough. It is difficult to cure by irradiation metastases to the parapharyngeal lymph nodes large enough to be detected with CT. It is necessary to irradiate parapharyngeal lymph nodes prophylactically, but 50 Gy in postoperative irradiation may not be enough from our results. The same may be true for metastases to the paratracheal nodes, which lie close to the lower poles of the thyroid gland or within the superior mediastinum and are difficult to access through surgery. In the patients who were operated on, N stage and the number of lymph node metastases were significant for gross survival. When "number of indications" was used for analysis, "number of indications" proved to be the most significant prognostic factor for gross survival.