Hydroxyurea-induced augmentation of fetal hemoglobin production in patients with sickle cell anemia

Five patients with sickle cell anemia were treated with hydroxyurea (HU), in hopes of augmenting their production of fetal hemoglobin. Laboratory responses in two patients treated for more than 2 years were encouraging and there were suggestions of clinical improvement. Long- term HU therapy should be considered for severely affected adults with sickle cell anemia who are willing to accept what is probably a small risk of carcinogenesis. Preliminary chromosomal analysis and knowledge of the clastogenic properties of HU suggest that conception and pregnancy should be avoided. Pharmacokinetic studies will probably be necessary to adjust individual dosage schedules so that cytotoxicity is avoided. F cell responses can be seen in 2 to 3 weeks if the HU dose is optimal, but establishment of a large number of F cells in the circulation may take a month or longer.     

A rare case of Cryptococcus gattii pneumonia in a renal transplant patient

Cryptococcus neoformans and Cryptococcus gattii are environmental fungi that can cause fever, cough, pneumonia, meningoencephalitis, dissemination, and death. C. gattii causes cryptococcomas more frequently than does C. neoformans and may require prolonged antifungal treatment. We present a rare case of C. gattii pneumonia in a renal transplant patient. A 44‐year‐old man, living in a rural area endemic for C. gattii and who had received a kidney transplant, was admitted to the hospital with fever, vomiting, weight loss, and diarrhea. A chest computed tomography revealed 2 alveolar, nodular, subpleural infiltrates in the periphery of the lungs. Differential diagnoses included infectious infiltrates, granulomatosis, embolization, and hemorrhage. C. gattii, molecular type VGI, was confirmed on day 28. Treatment consisted of amphotericin B at 1 mg/kg/day or fluconazole at 800 mg/day for first 6 weeks, followed by fluconazole at 400 mg/day for the subsequent 12 months. Response to the therapy has been slow. Because of the occurrence of outbreaks and its high morbidity and mortality rates, physicians must be aware of this complication in transplant recipients to avoid delays in diagnosis and to provide prompt management.     

The 14q+ chromosome in pre-B-ALL

A child who had acute lymphoblastic leukemia (ALL) associated with an 8;14 chromosome translocation and with a pre-B phenotype is described. The leukemic cells were determined to be pre-B-cells on the basis of intracytoplasmic mu-chain immunoglobulin (cIgM+) and the common-ALL antigen, lack of receptors for sheep erythrocytes, and lack of surface immunoglobulin. The 8;14 translocation is frequently found in patients with Burkitt's lymphoma and in most patients with B-cell ALL and is known to carry a poor prognosis. Thus far, no karyotypes have been reported for patients with pre-B-ALL. The present case indicates that a 14q+ chromosome may provide a proliferative advantage not only to cells with a B-cell phenotype, but also to pre-B-cells. The short survival of our patient also suggests that the 14q+ abnormality and the pre-B phenotype may signal a poor prognosis.     

4种支架构建复合式口腔黏膜的组织学特点

目的利用4种不同支架材料构建复合式口腔黏膜,并比较其组织结构特点。方法体外培养人口腔黏膜的成纤维细胞和角质形成细胞,在4种支架材料中加入成纤维细胞,培养7d后,在支架表面加入角质形成细胞,培养4d后,移至气-液界面继续培养7d。苏木精-伊红染色镜下观察构建的复合式口腔黏膜的组织形态学特点。结果 4种支架均可构建形成复层上皮。其中,上皮层与脱细胞真皮基质材料(de-epidermised dermis,DED)结合紧密,形成的人工黏膜有明显的上皮钉突。不同于以往报道,上皮层与Alloderm结合并不十分紧密。以胶原凝胶为基质形成的人工口腔黏膜最厚,有明显分层。以胶原海绵-胶原凝胶为基质形成的复层上皮在部分区域长入至胶原海绵的空隙中。结论以DED和胶原凝胶为支架构建的口腔黏膜更接近于天然结构,而后者脆性较大,限制了其临床应用的可能。     

Cutoff values of the Inflammatory Bowel Disease Questionnaire to predict a normal health related quality of life

One of the objectives in the treatment of the inflammatory bowel disease (IBD) is improving the patient's quality of life. However, we do not dispose of validated criteria to determine the questionnaire's scoring threshold that has to be reached in order to be able to assert that the patients' quality of life has normalized.ObjectiveTo determine the normality punctuation cutoff in the IBD specific quality of life questionnaire IBDQ-36.MethodCross-sectional study in a random sample of IBD patients, who have completed the questionnaires IBDQ-36 and EuroQol-5D. The IBDQ-36 normality was calculated according to its equivalence with the EuroQol-5D tariff ≥ 0.90, which corresponds to the 95% CI of the average obtained in a Spanish general population.Results218 patients were included. According to the EuroQol-5D tariff, 70 patients were considered to have a normal quality of life and 148 a quality of life poorer than the general population. The IBDQ-36 scoring was significantly higher in the normal quality of life group (222.9 ± 22.8 vs. 171.4 ± 44.8 in the bad quality of life group, p < 0.001). According to the linear regression between IBDQ-36 and EuroQol-5D, the cutoff point is 209, with a sensitivity and specificity to predict normality of 0.74 and 0.71 respectively.ConclusionsScores of the IBDQ-36 equal or superior to 209 suggest quality of life comparable to that perceived by the general population. This study allowed to set a threshold of normality in the management of the inflammatory bowel diseases.     

Biodistribution studies of boronophenylalanine-fructose in melanoma and brain tumor patients in Argentina.

A study of the (10)B-enriched p-boronophenylalanine-fructose complex ((10)BPA-F) infusion procedure in potential BNCT patients, including four melanoma of extremities and two high-grade gliomas (glioblastoma and ganglioglioma) was performed. T/B and S/B ratios for (10)B concentrations in tumor (T), blood (B) and skin (S) were determined. The T/B ratio for the glioblastoma was in the 1.8-3.4 range. The ganglioglioma did not show any significant boron uptake. For the nodular metastasic melanoma T/B values were between 1.5 and 2.6 (average 2.1+/-0.4), corresponding to the lower limit of the mean values reported for different melanoma categories. This result might suggest a lower boron uptake for nodular metastasic melanomas. S/B was 1.5+/-0.4. An open two-compartment pharmacokinetic model was applied to predict the boron concentration during the course and at the end of a BNCT irradiation.