Since the discovery of the KATP channel in 1983, numerous studies have revealed its physiological functions. The KATP channel is expressed in various organs, including the pancreas, brain and skeletal muscles. It functions as a "metabolic sensor" that converts the metabolic status to electrical activity. In pancreatic beta-cells, the KATP channel regulates the secretion of insulin by sensing a change in the blood glucose level and thus maintains glucose homeostasis. In 2004, heterozygous gain-of-function mutations in the KCNJ11 gene, which encodes the Kir6.2 subunit of the KATP channel, were found to cause neonatal diabetes. In some mutations, diabetes is accompanied by severe neurological symptoms [developmental delay, epilepsy, neonatal diabetes (DEND) syndrome]. This review focuses on mutations of Kir6.2, the pore-forming subunit and sulfonylurea receptor (SUR) 1, the regulatory subunit of the KATP channel, which cause neonatal diabetes/DEND syndrome and also discusses the findings of the pathological mechanisms that are associated with neonatal diabetes, and its neurological features.
Objective Iron deficiency anemia (IDA) has become important with regard to mortality in hemodialysis (HD) patients. Therefore, it is necessary to optimize the treatment of these patients.
Methods IDA in end-stage renal disease patients on HD was observed in 42 (33.6%) of 125 patients. We examined the influence of daily orally iron [sodium ferrous citrate (SFC) iron/tablet 50 mg, 1-2 tablets] on the renal function markers, anemia and iron data for about 6 months.
Results The hematocrit and hemoglobin levels were significantly increased in the patients treated with SFC [hematocrit: before 28.5%±2.1% (mean ± standard deviation), 1st month 30.0%±2.3%, p<0.05; 3rd month 32.4%±2.9%, p<0.05; 6th month 31.3%±3.4%, p<0.05; and hemoglobin: before 9.25±0.70, 1st month 9.72±0.71, p<0.05; 3rd month 10.54±0.96, p<0.05; 6th month 10.25±1.21 g/dL, p<0.05]. The transferrin saturation (TSAT) and serum ferritin levels were significantly increased in the patients treated with SFC (TSAT: before 21.5%±10.0%, 1st-3rd month, 34.1%±15.1%, p<0.05; 6-8th month 34.7%±11.9%, p<0.05; and ferritin: before 38.2±37.1, 6-8th month 67.5±44.0 ng/mL, p<0.05). The present findings clearly indicate that oral iron is an effective route of iron supplementation in HD patients, and no adverse effects associated with SFC occurred during the treatment and follow-up period.
Conclusion Our results clearly indicate that oral iron delivered via SFC is a well-tolerated and effective form of iron supplementation in long-term HD and IDA patients in Japan.
Objective Among elderly patients with chronic obstructive pulmonary disease (COPD), there are some patients who cannot inhale tiotropium via Respimat® due to poor hand-lung coordination. This study aimed to examine whether or not tiotropium inhalation therapy using Respimat® with a spacer increased the forced expiratory volume in 1 s (FEV1) in patients with COPD.
Methods A randomized, crossover, single-center study was conducted in 18 patients with stable COPD. Tiotropium (5 μg) via Respimat® with or without a spacer (AeroChamber®) was administered for 2 weeks. Following a 2-week washout period using a transdermal tulobuterol patch (2 mg per day), participants were then crossed over to the other inhalation therapy with respect to spacer use. The trough FEV1 was measured at every visit using a spirometer. A questionnaire regarding inhalation therapy was administered to patients at the final visit.
Results The administration of tiotropium via Respimat® both with and without a spacer significantly increased the trough FEV1 from baseline during each treatment period, with mean differences of 115.0±169.6 mL and 92.8±128.1 mL, respectively. There was no significant difference in the change in the trough FEV1 between the 2 procedures (p=0.66). A total of 86% of patients reported that inhalation using a spacer was not difficult, and more than half also rated both the usage and maintenance of the AeroChamber® as easy.
Conclusion Tiotropium inhalation therapy administered via Respimat® using a spacer exerted a bronchodilatory effect similar to that observed with tiotropium Respimat® alone.
Objective We retrospectively compared the clinical efficacy and toxicity of rituximab (R)-THP-COP (pirarubicin, cyclophosphamide, vincristine, and prednisolone) with that of R-CHOP (rituximab, adriamicin, cyclophosphamide, vincristine, and prednisolone) in previously untreated old patients with diffuse large B-cell lymphoma (DLBCL).
Patients and Methods Patients admitted to our institution between 2004 and 2013 were examined. The patients received either R (375 mg/m2, day 1)-THP-COP (pirarubicin 50 mg/m2 day 1, cyclophosphamide 750 mg/m2 day 1, vincristine 1.4 mg/m2 day 1, and prednisolone 100 mg day 1-5) or R-CHOP (adriamicin 50 mg/m2 day 1, cyclophosphamide 750 mg/m2 day 1, vincristine 1.4 mg/m2 day 1, and prednisolone 100 mg day 1-5). The doses of chemotherapeutic agents were adjusted depending on the patient's age and associated complications. The treatment was performed for 6 to 8 cycles.
Results Among 74 patients with DLBCL (median 76, range 65-90 years; male 39, female 35), 29 received R-THP-COP, while 45 received R-CHOP. The overall response rates were 94.6% (complete response 86.4%, partial response 8.1%). The 2-year overall and progression-free survival rates were 77.6% and 68.5% for the R-THP-COP regimen and 79.2% and 78.9% for R-CHOP, respectively. No significant differences were found between these two regimens regarding the clinical efficacies. The most frequent adverse event was neutropenia (72.4% for the R-THP-COP regimen, 88.9% for the R-CHOP regimen). The cardiac function as evaluated by ejection fraction values was not impaired in either regimen.
Conclusion R-THP-COP was effective and safe as an alternative to R-CHOP.