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.

The purpose of this study was to identify risk factors associated with the presence of pressure ulcer development in adult patients at an intensive care unit hospital in Indonesia. The prospective cohort design was conducted in this study. A total of 105 patients participated and a pressure ulcer developed in 35 patients. The initial analysis identified several variables as significant risk factors for pressure ulcer development (interface pressure, fecal incontinence, skin moisture, diastolic blood pressure, smoking and body temperature). However, when entered into a final multivariate analysis, four factors, interface pressure [odds ratio (OR) 17.6, 95% confidence interval (CI) 4.1, 74.3], skin moisture (OR 8.2, 95% CI 2.2, 30.9), smoking (12.7, 95% CI 2.8, 56.7) and body temperature (OR 102.0, 95% CI 7.7, 98.8) were found to be significant. The results suggest that interface pressure measured using a multipad pressure evaluator, skin moisture measured by a moisture checker, thermometer for body temperature and smoking status are adequate instruments for the prediction of pressure ulcer development.

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A basic fibroblast growth factor (bFGF) case and a control case whose total scores of Pressure Ulcer Healing Process-Ohura (PUHP-Ohura) and risk factors for pressure ulcers, and level of care for pressure ulcers were equivalent were paired. Twenty-three such eligible pairs were enrolled in this study. Both cases in each pair were treated under conditions in which extrinsic factors such as the use of a pressure-relief mattress and the frequency of postural change were equivalent. The efficacy of bFGF was assessed by analyzing the data obtained over time as the scores of PUHP-Ohura for nine observation items using the SAS MIXED procedure. Treatment of pressure ulcers with bFGF accelerated wound healing over time more significantly than the control in six observation items (exudate volume, ulcer depth, granulation formation, wound edge, epithelialization, total score of the PUHP-Ohura). These data suggest that it may be possible to evaluate drugs for the treatment of pressure ulcers using the PUHP-Ohura wound-assessment tool.

Hemoglobin Hammersmith, a rare, unstable hemoglobin variant, was diagnosed in a 9-year-old Japanese girl. She presented with the typical manifestations of this disorder, including neonatal hyperbilirubinemia, followed by progressive hepatosplenomegaly, jaundice, and bilirubinuria. Because of severe hemolytic anemia, she received transfusions of red blood cells every 3 to 4 weeks. However, she underwent splenectomy at the age of 4 years and has continued to be in partial remission without requiring further transfusions. DNA sequence analysis of the polymerase chain reaction-amplified beta-globin gene revealed a point mutation (T --> C) in the second nucleotide of the 42nd codon of the beta-globin chain (beta 42(CD1) Phe --> Ser).