Delayed renal dysfunction after total body irradiation in pediatric malignancies

The purpose of this study was to retrospectively evaluate the incidence of delayed renal dysfunction after total body irradiation (TBI) in long-term survivors of TBI/hematopoietic stem cell transplantation (HSCT). Between 1989 and 2006, 24 pediatric patients underwent TBI as part of the conditioning regimen for HSCT at Chiba University Hospital. Nine patients who survived for more than 5 years were enrolled in this study. No patient had any evidence of renal dysfunction prior to the transplant according to their baseline creatinine levels. The median age at the time of diagnosis was 6 years old (range: 1–17 years old). The follow-up period ranged from 79–170 months (median: 140 months). Renal dysfunction was assessed using the estimated glomerular filtration rate (eGFR). The TBI dose ranged from 8–12 Gy delivered in 3–6 fractions over 2–3 d. The patients were treated with linear accelerators in the supine position, and the radiation was delivered to isocentric right–left and left–right fields via the extended distance technique. The kidneys and the liver were not shielded except in one patient with a left adrenal neuroblastoma. No patient required hemodialysis. The eGFR of four patients (44.4%) progressively decreased. The remaining patients did not demonstrate any eGFR deterioration. Only one patient developed hypertension. By evaluating the changes in eGFR, renal dysfunction among long-term survivors of TBI/HSCT could be detected. Our results suggested that the TBI schedule of 12 Gy in 6 fractions over three consecutive days affects renal function.     

Multiple calcifying lesions in the maxilla and mandible of a 4-year-old girl: report of a rare case and literature review

Calcifying lesions in the jaws are usually solitary and can be diagnosed by intraoral, panoramic, and conventional extraoral radiographs. It is extremely rare for these calcifying lesions to be multiple, unless they are associated with certain systematic diseases. Herein, we report a rare case of multiple odontomas in a 4-year-old girl. The lesions were scattered throughout both jaws on the panoramic image. The reconstructed CT image showed lesions in the left maxilla that occupied the sinus and affected the floor of the left orbit. The lesions in the mandible were widespread and extended to the tooth-bearing area. Enucleations of the maxillary parts were performed, and the pathological diagnosis was a mixture of complex and compound odontomas. The 3-year postoperative follow-up by panoramic imaging was uneventful. Preoperative CT was useful for the differential diagnosis by depicting the internal calcifying lesions and revealing the extent and complex relationships among the lesions.     

T cell receptor vβ repertoire of double-negative α/β t cells in patients with systemic sclerosis

Objective. To analyze the T cell receptor V_β gene on double-negative (DN) α/β T cells, which are increased in number, on peripheral blood lymphocytes (PBL) from patients with systemic sclerosis (SSc). Methods. The DN α/β T cells were sorted by flow cytometry from PBL obtained from 3 patients with SSc. The V_β repertoire was analyzed by polymerase chain reaction. Results. Only 1 or 2 V_β genes (V_β5/7, 5, or 17) were predominantly expressed on DN α/β T cells from these 3 patients. Conclusion. The V_β repertoire on DN α/β T cells in PBL from patients with SSc is rather restricted.     

An Earlier First Meal Timing Associates with Weight Loss Effectiveness in A 12-Week Weight Loss Support Program

Recent studies have reported that meal timing may play an important role in weight regulation, however it is unknown whether the timing of meals is related to the amount of weight loss. This study aimed to examine the relationship between indices of meal timing and weight loss during weight loss intervention in adults. A 12-week weight loss support program was conducted for 97 adults (age: 47.6 ± 8.3 years, BMI: 25.4 ± 3.7 kg/m²). After the program, body weight decreased by −3.0 ± 2.7%. Only the start of the eating window was positively correlated with the weight change rate in both sexes (men: r = 0.321, p = 0.022; women: r = 0.360, p = 0.014). The participants were divided into two groups based on the start of the eating window as follows: the early group (6:48 ± 0:21 AM) and the late group (8:11 ± 1:05 AM). The weight loss rate in the early group was significantly higher (−3.8 ± 2.7%) than that in the late group (−2.2 ± 2.5%). The present results showed that the start of the early eating window was associated with weight loss and suggested paying attention to meal timing when doing weight loss.     

Mutation analysis of a Japanese patient with fucosidosis

Fucosidosis is a rare autosomal recessive disorder resulting from a deficiency of α-L-fucosidase. Recently, various mutations have been reported in this disease, but it is difficult to elucidate the phenotype from the genetic mutations. We report a patient with chronic infantile type fucosidosis, with a compound heterozygote of a nonsense mutation (W148X, Trp at codon 148 to stop codon) and a large deletion, including all exons. This is the first report of a large deletion demonstrated in fucosidosis. It is interesting that this patient has a relatively mild clinical course despite the absence of the mRNA. This case also indicates the difficulty in determining the phenotype from the genotype in fucosidosis. Received: February 19, 1999 / Accepted: April 16, 1999     

New endoscopic treatment for intramucosal gastric tumors using an insulated-tip diathermic knife

BACKGROUND AND STUDY AIMS: For one-piece resection the conventional technique of endoscopic mucosal resection (EMR) is limited to gastric mucosal tumors of 10 mm or less in size. In this retrospective study, we investigated the efficacy and complications associated with a new EMR method, using an insulated-tip diathermic knife (IT-EMR). PATIENTS AND METHODS: In a total of 41 patients gastric mucosal tumors were resected using IT-EMR. Results: One-piece resection rates were 82% (14/17) for lesions of 10 mm or less, 75% (12/16) for those between 11 and 20 mm, and 14% (1/7) for those of over 20 mm. Complication rates for severe bleeding and perforation were 22% and 5%, respectively. With a median follow-up period of 32 months, no recurrence was observed after these procedures. CONCLUSIONS: Compared with conventional EMR, this new method may have significant benefits, particularly regarding one-piece resection of lesions between 11 and 20 mm in size, and may also have a lower recurrence rate.