In this paper, we examined the details of severe infections, treatment efficacies, and the prognoses of 23 Japanese patients with chronic granulomatous disease (CGD). We described the mean ages at diagnosis and follow-up, which were 2.8 years (range, 0.7-10 years) and 14.9 years (range, 0.2-28.4 years), respectively. There were three deaths, two from Aspergillus pneumonia and one from liver abscess. Eighteen of the 23 patients (78%) had a complete loss of gp91phox, and three had p22-phox and one had p67phox deficiencies. Aspergillus species were found in 45% of 174 severe infections. The mean height and weight of the 20 surviving patients were -0.8 +/- 1.3SD and -1.9 +/- 1.9SD below the means for age, respectively. Short stature and underweight (below the 10th percentile of the means) for age were seen in 22% and 17% of the patients, respectively. This growth retardation reflects the severity of the disease. At 20 years of age, there was 87% survival. Ongoing prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX) or antifungal drugs was given in 16 and 11 patients, respectively. Interferon-gamma (IFN-gamma) was given once a week to 14 patients. Four patients underwent hematopoietic stem cell transplantation (HSCT) and are currently well. There were infections observed in three of 21 identified related carriers of X-linked CGD. A carrier with a liver abscess had 5% normal neutrophils during the acute phase of infection, which returned to 40% normal neutrophils after recovery. The high survival rate in this hospital results from regular follow-up and prophylaxis with TMP-SMX and anti-fungal drugs beginning at the time of diagnosis, along with treatment with weekly IFN-gamma.

Defect concentrations in AlxGa1−xSb which is in equilibrium with a liquid phase are calculated. When the liquid phase is Ga rich, a Ga antisite (Ga2−Sb) or an Al antisite (Al2−Sb) is dominant, and the concentrations of vacancies are much smaller than the antisite concentrations. Ga2−Sb is dominant in GaSb equilibrated with a Sb-rich solution, but the concentration of Sb antisites comes close to that of Ga2−Sb as temperature is lowered. For x larger than 0.6, a group-III vacancy is the predominant defect in the case of Sb-rich solutions. Calculated net acceptor concentrations agree well with those determined experimentally. A complex defect composed of GaSb and a Ga vacancy, which have been taken as the dominant residual acceptor, is expected to be negligible.

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM, Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase ( > 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease seventy. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.

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Objects: 1) To determine the transfer function of a roller card by means of a sampling card. 2) To investigate the optimum condition for controlling the roller card by a closed loop control system. Methods: 1) The outline of this system is assumed from transient response in step input. Its time constant is obtained from the variance of output web produced by creating rectangular wave input with periodic revolutions of the feed roller. 2) The optimum value of the controllers is obtained by taking the unevenness of output card web as a stationary Gaussian noise. Findings : 1) The transfer function of the roller card is a linear first-order control system with a dead time. 2) PJD control is best suited to a system with a long dead time, such as a roller card. 3) PD control works better than P or Pl.

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Dynamic properties represented by curved load-elongation