A patient with cyclic neutropenia complicated by severe persistent neutropenia successfully delivered a healthy baby.

We describe a 24-year-old pregnant woman complicated by cyclic neutropenia (CN), who was successfully treated with granulocyte-colony stimulating factor (G-CSF). Her white blood cell (WBC) and neutrophil count fluctuated from 2,600 to 4,600/microl, and 26 to 2,530/microl, respectively. The peak neutrophil count gradually decreased as pregnancy advanced, resulting in the disappearance of its cyclicity. At 39 weeks of pregnancy when the neutrophil count became 84/microl, the patient was started on G-CSF and her neutrophil count increased to 1,550/microl on the fourth day after delivery. She delivered a healthy baby without any complications at 39 weeks of pregnancy.

Predictors of improvement in FEV1 (forced expiratory volume in 1s) after lung volume reduction surgery.

We evaluated various preoperative and operative factors to identify the predictors of improvement in forced expiratory volume in 1s (FEV1) after lung volume reduction surgery (LVRS). Fifty-eight emphysema patients received bilateral LVRS either via a sternotomy (n = 53) or by thoracoscopy (n = 5). The patients were divided into the following two groups: group I (n = 17), patients whose FEV1 improved by less than 20%; group II (n = 41), patients whose FEV1 improved by more than or equal to 20%. The preoperative factors (age, degree of dyspnea, oxygen use, steroid use, pulmonary function test, arterial blood gas, pulmonary hemodynamics, 6-min walking distance) and operative factors (removed lung weight, number of staplers) were both similar between the two groups. Upper lobe type emphysema was more frequently seen in group II and the average improvement in FEV1 was significantly better in the patients with upper lobe type emphysema (62.8% +/- 8.5%) than lower lobe type (36.9% +/- 7.6%) and mixed type (35.5% +/- 6.5%), P < 0.01. Severe pleural adhesion was more frequently found in group I and the average improvement in FEV1 was also significantly lower in patients with severe pleural adhesion (19.8% +/- 6.4%) than mild-to-moderate adhesion (46.8% +/- 5.3%) and no-to-minimal adhesion (62.3% +/- 10.4%), P < 0.01. These results lead us to conclude that upper lobe type emphysema may thus be a predictor of better FEV1 improvement while severe pleural adhesion is considered to be a predictor of a poorer FEV1 improvement after bilateral LVRS.

A case of thoracoscopic bilateral lung volume reduction surgery in a supine position.

We present a case of thoracoscopic bilateral lung volume reduction surgery performed with the patient in a supine position. By rotating the operative table, bilateral apical resection could be performed without difficulty. The duration of the operation was 160 minutes and the patient's forced expiratory volume in 1 second improved from 0.81 l to 2.49 l.

Bilateral lung volume reduction surgery via median sternotomy for severe pulmonary emphysema.

BACKGROUND: Lung volume reduction surgery either via sternotomy or by thoracoscopy has been demonstrated to be effective for selected emphysema patients in North America and Europe. The present study summarizes short-term results of bilateral lung volume reduction performed via median sternotomy for the first consecutive 39 patients with severe diffuse emphysema in Okayama, Japan, from July 1995 to February 1997. METHODS: There were 35 men and 4 women, and the age range was 54 to 74 years with a mean age of 65 years. All were former heavy smokers and none of them had alpha1-antitrypsin deficiency. Only 9 patients (23%) showed a bilateral upper lobe pattern of emphysema. The operation was done through a median sternotomy, and the most emphysematous portions were excised bilaterally with a linear stapling device fitted with strips of bovine pericardium to prevent air leakage. RESULTS: No operative death was encountered. The first 33 patients completed 3-month follow-up assessment, and their mean forced expiratory volume in 1 second had improved by 41% from 735 mL to 1,037 mL. Other parameters of pulmonary function tests, arterial blood gas analysis, 6-minute walking distance, and dyspnea scale also had improved significantly. These improvements lasted for at least a year. CONCLUSIONS: Bilateral lung volume reduction surgery via median sternotomy is a safe and effective procedure for selected severe emphysema patients. Although the pattern of emphysema might be different between countries, the results in Japanese patients were similar to those previously reported in North American and European patients.

Radiation induction of p53 in cells from Nijmegen breakage syndrome is defective but not similar to ataxia-telangiectasia.

p53-mediated signal transduction after exposure to ionizing radiation was examined in cells from patients with Nijmegen breakage syndrome (NBS), an autosomal recessive disease characterized by microcephaly, immunodeficiency, predisposition to malignancy, and a high sensitivity to ionizing radiation. NBS cells accumulated p53 protein in a dose-dependent fashion, with a peak level 2 hrs after irradiation with 5 Gy. However, the maximal level of p53 protein in NBS cells was constantly lower than in normal cells. Moreover, this attenuation of p53 induction was confirmed by decreased levels of p21WAF1 protein, which is transcriptionally regulated by p53 protein. This defective induction of p53 protein in NBS is similar to that in ataxia-telangiectasia (AT), although the induced levels of p53 protein in NBS appeared to be the intermediate between normal cells and AT cells. This moderate p53 induction in NBS cells is consistent with the relatively mild radiation sensitivity and the abnormal cell cycle regulation post-irradiation, as present in NBS. Furthermore, all NBS cell lines used here exhibited time courses of p53 induction similar to normal cells, which is in contrast with p53 induction in AT cells, where the maximum induction shows a delay of approximately 2 hrs compared with normal cells. These evidences suggest a different function of each gene product in an upstream p53 response to radiation-induced DNA damage.

Auto-positive end-expiratory pressure during one-lung ventilation using a double-lumen endobronchial tube.

The present study was undertaken to investigate the possible relationships between the magnitude of autopositive end-expiratory pressure (auto-PEEP) and measured PaO2 during one-lung ventilation (OLV). Forty-one adults received OLV anesthesia using a tidal volume of 8 mL/kg and a respiratory rate of 12 breaths/min. Auto-PEEP was quantified using an end-expiratory port occlusion method. During two-lung ventilation (2LV), auto-PEEP was observed in 18 of 41 patients and ranged from 0.5 to 2.5 cm H2O. During OLV, auto-PEEP was observed in 34 of 41 patients and ranged from 0.5 to 10 cm H2O. The mean (+/- SD) value of auto-PEEP was significantly higher during OLV than during 2LV (3.2 +/- 3.3 cm H2O versus 0.5 +/- 0.7 cm H2O, P < 0.0001). Auto-PEEP during OLV correlated inversely with preoperative forced expiratory volume in 1 s/forced vital capacity (y = 12.5 - 0.13x, r = -.05, P < 0.005). During OLV, there was no significant correlation between auto-PEEP and measured PaO2. These findings confirm that many patients do not exhale completely to functional residual capacity during OLV.

[Hemostatic mechanism associated with aprotinin during and after the extracorporeal circulation for cardiac and great vessel surgery].

It has been reported that aprotinin (apr) is effective for hemostasis during and after the extracorporeal circulation (ECC) for cardiac or great vessel surgery. However its mechanism remains still obscure. In this study, we observed relationships between changes in interleukin-8 (IL-8), endothelial factors, or D-dimer and aprotinin administered before and after the ECC. Fourteen patients in whom cardiac or great vessel surgery was done using ECC were divided randomly into two groups. Seven subjects in Group I (G-I) were infused 10(6) KIU of apr immediately after anesthesia, immediately before and after the ECC, respectively, and 10(6) KIU of apr were also added during the ECC. Seven other subjects (G-II) were infused 10(6) KIU of apr immediately before and at three hours after the ECC, and also 2 x 10(6) KIU of apr immediately after the ECC. No marked hemorrhagic tendency was noted in all subjects and increase in plasma D-dimer was not marked. Immediately after the ECC, granulocyte elastase in plasma increased markedly in both groups but subsequently decreased in the G-II alone while it remained unchanged in the G-I. Similar changes were observed in plasma IL-8 values. During the ECC, intercellular adhesion molecule-1 in plasma decreased in both groups but von Willebrand factor activity decreased in the G-I alone. From the above data, the hemostatic effect of apr may contribute to protect the dysfunction of endothelial cells or to enhance recovering from the dysfunction during the ECC.

[bcl-2 gene rearrangement and its expression in tumor cells].

Both the rearrangement and the expression of the bcl-2 gene in Japanese hematopoietic tumor cells were studied by Southern and Northern blot hybridizations. The expression of the bcl-2 gene was studied in seven cultured cell lines and twenty clinical samples, including eleven non-lymphoid tumors and nine lymphoid tumors. All lymphoid tumors except one sample from a patient with ALL expressed the bcl-2 gene. The bcl-2 gene was strongly expressed in B cell tumors. This gene was also expressed in T-ALL samples studied, although the expression was not as marked as in the B cell tumors. Non-lymphoid tumors did not express bcl-2 gene. bcl-2 gene rearrangement was studied in five cultured cell lines and six clinical samples including four follicular lymphomas and two T-ALLs. No abnormal bcl-2 gene configurations were found in any of the clinical samples. Among the cultured cell lines, the BALL-1 line showed two-fold amplification. The frequency of bcl-2 rearrangement in Japanese follicular lymphomas is reported to be lower than that seen in the American follicular lymphomas. Nevertheless, the increased expression of the bcl-2 gene seen in the Japanese B cell tumors studied supports the contention that the bcl-2 gene plays an important role in the pathogenesis of B cell tumors.

[The changes of mRNAs of both c-myc and MDR1 in CML-bc tumor cells during the clinical course: a case report].

We examined the expressions of both c-myc and MDR1 in four samples isolated from a CML-bc patient in series during the clinical course. A 46-year-old man was diagnosed as chronic phase of CML in june 1985. In February 1987, the diagnosis of blastic transformation was made because of marked increase of blastic cells. He was initially treated with vincristine (V) and prednisolone (P) successfully. However, the effect of VP therapy was gradually attenuated, so that combined chemotherapies including anthracyclines were started. After the treatments of several courses, tumor cells acquired the refractory to both vincristine and adriamycin . He died in January, 1988. Northern blot hybridization studies revealed no expression of MDR1 mRNAs. However, the expression of c-myc was increased in the latest sample. These findings suggest that the expression of c-myc mRNA in tumor cells of this case reflects one characteristic of clinically refractory states to chemotherapies.

[Hormone secreting ability in patients with parathyroid autograft].

In order to elucidate the function of parathyroid autograft, we determined the plasma parathyroid hormone (PTH) levels in the blood from cubital veins in two patients who had parathyroid autotransplantation after total parathyroidectomy. The first patient with chronic renal failure had been treated by hemodialysis for the past nine years and showed marked symptoms due to secondary hyperparathyroidism for five years. The second patient showed an evidence of recurrence of parathyroid cancer three years after the initial operation carried out elsewhere. The parathyroid tissue of 80 mg was sliced into 25 pieces and transplanted into separate pockets in one of the brachioradial muscles of the forearm. These patients showed an increase in plasma PTH levels two weeks after surgery. Plasma calcium level returned to the normal range three months after operation. Artificial hypocalcemia was induced by an injection of porcine calcitonin at ten months after surgery and a reserve of PTH secretion was tested. An increase of plasma C-PTH and N-PTH levels were recognized in the two patients as well as in the normal healthy volunteers. It was shown that parathyroid autograft has sufficient function to maintain normocalcemia and a reserve of function to respond against an artificially induced hypocalcemia at the tenth month after autografting.