A comparative observational study of carbohydrate intake and continuous blood glucose levels in relation to performance in ultramarathon.

Ultra-endurance events have gained global participation, whereas the critical factors of competition results remain to be well elucidated. This study used a nutritional approach to evaluate the association of competition results with carbohydrate intake and blood glucose control during a 100-mile ultramarathon. This observational study was conducted in the 2021 LAKE BIWA 100, which covered 100 miles (169 km) and 10,500 m elevation. The course was divided into 9 segments by aid station. According to the competition results, 22 participants (18 men and 4 women) were divided into higher finishers (n = 7), lower finishers (n = 9), and non-finishers (n = 6). The participants self-recorded their overall dietary intake throughout the race. Glucose levels were monitored every 15 min by a flash glucose monitoring system. Running speed in each segment was standardized to the average of the top five finishers for each gender. Among finishers, the carbohydrate intakes were significantly higher in the higher finishers than in the lower finishers during overall segments, especially in the first half of the race (p < 0.05). There was a significant positive correlation between running speed and carbohydrate intake in the lower finishers (rho = 0.700, p = 0.036). Two-way ANOVA analysis revealed that lowering glucose levels in each segment were more frequently observed in the lower finishers compared to the higher finishers (p = 0.012). Compared to the higher finishers, the lower finishers exhibited significantly greater fluctuations (⊿highest-lowest) in glucose levels (p < 0.001). The fluctuations in glucose levels were significantly and negatively correlated with the running speed of the finishers (rho = - 0.612, p = 0.012). Faster runners consume high amounts of carbohydrates and maintain glucose levels during the 100-mile ultramarathon on the trail, especially at the beginning. Lowering and fluctuating glucose levels during the race are associated with lower running speed in endurance athletes.

A novel SLC5A2 heterozygous variant in a family with familial renal glucosuria.

Familial renal glucosuria (FRG) is characterized by persistent glucosuria despite normal blood glucose levels in the absence of overt tubular dysfunction. SGLT2 is a sodium-glucose cotransporter expressed in the proximal tubule; loss-of-function variants in SLC5A2 are the primary cause of FRG. Heterozygous variants have rarely been reported in Japanese individuals. Here, we identified a novel SLC5A2 heterozygous variant, c.1348G>T: p.Gly450Trp, in a Japanese family comprising two children and their father.

Colonoscopic diagnosis and reduction of recurrent intussusception owing to Henoch-Schönlein purpura without purpura.

About 50-75% of patients with Henoch-Schönlein purpura (HSP) develop gastro-intestinal symptoms with surgical complications such as intussusception occurring in 0.7-13.6%. In 10-40% of patients, however, gastro-intestinal manifestations may precede the onset of purpura. In patients with gastro-intestinal tract involvement without purpura, confirming the diagnosis of HSP and determining the appropriate treatment remains difficult. A seven-year-old boy presented with recurrent intussusception owing to HSP without purpura. It was confirmed pathologically and treated via colonoscopy. Early colonoscopic intervention can contribute to the early diagnosis of HSP and its subsequent management by avoiding unnecessary surgical invasion.

Risk of coronary artery lesions in young infants with Kawasaki disease: need for a new diagnostic method.

AIM: To examine clinical characteristics of Kawasaki disease (KD) in infants younger than 3 months of age and to develop a method for detecting KD in febrile infants. METHOD: In a case-control study, we retrospectively collected clinical and laboratory data from 24 KD infants younger than 3 months of age out of 410 KD patients. We then compared younger infants with both older patients and febrile infants with respiratory syncytial virus (RSV) infection and urinary tract infections (UTI). RESULTS: The frequency of incomplete KD was higher in the younger group than in the control group (79% vs. 36%, P < 0.0001). Furthermore, before treatment, the incidence of coronary artery lesions (CAL) was significantly higher in the younger group (29% vs. 3.9%, P = 0.0001), resulting in a higher incidence of coronary artery sequelae (21% vs. 3.4%, P = 0.0023). Our results revealed that the serum N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level of KD patients was higher than that of RSV and UTI patients (3110 ± 2076 vs. 698 ± 436, P = 0.0001; and 971 ± 589 pg/mL, P = 0.0002, respectively). Thus, NT-proBNP might be suitable as a diagnostic marker of KD in young infants (P = 0.0005, criterion values: 1555 pg/mL [sensitivity: 80%, specificity: 85%]). CONCLUSION: Kawasaki disease infants younger than 3 months of age appear to be at higher risk for incomplete KD and early-onset CAL prior to the appearance of coronary artery sequelae. We suggest performing an echocardiogram and evaluating NT-proBNP in young infants with fever that has lasted longer than 2 days, regardless of the presence or absence of manifestations associated with KD.

Venous thromboembolism in two adolescents with Down syndrome.

Kurokami T, Takasawa R, Takeda S, Kurobe M, Takasawa K, Nishioka M, Shimohira M. Venous thromboembolism in two adolescents with Down syndrome. Turk J Pediatr 2018; 60: 429-432. Although venous thromboembolic events are relatively rare in children, they are an increasingly recognized clinical entity in pediatric tertiary care hospitals. Although vascular disorders are prevalent with Down syndrome, it remains unclear whether Down syndrome patients are at higher risk for venous thromboembolic events. We report two adolescent cases with Down syndrome who unexpectedly developed venous thromboembolism in a general care unit. Our cases had a few risk factors; laparoscopic radical surgery for Hirschsprung's disease with central venous catheterisation in Case 1, and bacterial hepatic abscess in Case 2. Despite preventive heparinization with catheterisation and minor surgery in Case 1 and non-sepsis in Case 2, bed rest for only a few days triggered sudden onset of deep vein thrombosis in lower limbs with pulmonary thromboembolism in both cases. We speculate that the characteristics of Down syndrome, including physical and behavioural problems, might cause venous thromboembolic events. Thus, we should pay more attention to the relationship specifically between venous thromboembolism and Down syndrome, especially in adolescents, and increase prevention, early detection and treatment efforts.

Abdominal paraganglioma in a young woman with 1p36 deletion syndrome.

1p36 deletion syndrome is the most common terminal deletion syndrome, and the genomic regions that contribute to specific 1p36 deletion syndrome-related phenotypes were recently identified. Deletions in the 1p36 region have been documented in various tumor tissues, which indicates correlation between loss of heterozygosity of 1p36 and tumor development, and the existence of tumor suppressors in this region. Therefore, it was suspected that patients with 1p36 deletion syndrome have a higher risk of tumor development; however, only a few child cases of neuroblastoma with 1p36 deletion syndrome have been reported. We report the first case of 1p36 deletion syndrome with paraganglioma (PGL) and include genetic investigation. The 24-year-old woman with 1p36 deletion syndrome had severe intellectual disability, dilated cardiomyopathy, and distinct dysmorphic features, and presented with persistent vomiting accompanied by hypertension (178/115 mmHg). Abdominal CT revealed a 40 × 50 mm retroperitoneal mass and substantial elevations of plasma and urine norepinephrine (15.4 nmol/L and 1022 µmol/mol creatinine, respectively); abnormal uptake of 123 I-MIBG in the tumor led to PGL diagnosis. The patient was not able to have surgery because of substantial surgical risks; however, a combination of α- and ß-blockade was effective for blood pressure control. Array CGH revealed a deletion over 4.5 Mb, from the 1p telomere but excluding the SDHB region. Comprehensive mutational analysis of PGL-associated genes (RET, VHL, TMEM127, MAX, and SDHA/B/C/D) was negative. These results indicate that the germline 1p36 deletion might be "1st hit" of tumor development, and PGL might be a novel complication of 1p36 deletion syndrome. © 2016 Wiley Periodicals, Inc.

Improved growth velocity of a patient with Noonan-like syndrome with loose anagen hair (NS/LAH) without growth hormone deficiency by low-dose growth hormone therapy.

Noonan-like syndrome with loose anagen hair (NS/LAH; OMIM 607721) is caused by a heterozygous c.4A>G mutation in SHOC2. Most cases exhibit both growth hormone deficiency (GHD) and growth hormone insensitivity (GHI) and thus require a high dose of growth hormone (GH) therapy (e.g., 35-40 µg/kg/day). We report on a genetically diagnosed NS/LAH patient manifesting severe short stature (-3.85 SDs) with low serum level of IGF1, 30 ng/ml. The peak levels of GH stimulation tests were within the normal range, and GHI was not observed in the IGF1 generation test. However, with low-dose GH therapy (25 µg/kg/day) for two years, IGF1 level and height were remarkably improved (IGF1: 117 ng/ml, height SDs: -2.20 SDs). Further, catch-up of motor development and improvement of the proportion of extending limbs to trunk were observed (the Developmental Quotient score increased from 68 to 98 points, and the relative sitting height ratio decreased from 0.62 to 0.57). Our results suggest that endocrinological causes for short stature are variable in NS/LAH and that GH therapy should be considered as a possible treatment for delayed development in NS/LAH.

Effects of sliding velocity on friction: an in vitro study at extremely low sliding velocity approximating orthodontic tooth movement.

OBJECTIVE: To evaluate the effects of sliding velocity on friction, particularly at extremely low sliding velocity approximating orthodontic tooth movement. MATERIALS AND METHODS: Stainless-steel (SS) 0.022-inch preadjusted brackets and 0.016- and 0.016×0.022-inch SS wires and superelastic nickel-titanium 0.016×0.022-inch wires were used for this test. The wire was secured in a SS preadjusted bracket with an elastomeric module. One end of the wire was pulled upward 1.5 mm at a speed of 5.0×10(-7), 1.0×10(-5), 1.0×10(-4), 1.0×10(-3), 1.0×10(-2), and 1.0×10(-1) mm/s by the micrometer. The measurements were conducted 10 times and averaged. Tukey-Kramer tests were used to compare the mean differences of each testing measurement among the different sliding velocities. RESULTS: The frictional forces tended to increase as the sliding velocity decreased. The mean frictional force for 5.0×10(-7) mm/s sliding velocity (approximating orthodontic tooth movement) was 106.8 cN in 0.016×0.022-inch SS wires, almost double the 1.0×10(-1) mm/s sliding velocity. CONCLUSION: The effects of sliding velocity cannot be ignored when we estimate frictional forces in clinical orthodontics.

Exercise-induced myocardial ischemia in a case of anomalous origin of the left main coronary artery from the noncoronary sinus of valsalva.

We report a case of anomalous origin of the left main coronary artery (LCA) from the noncoronary sinus of valsalva (LCANCS) in a young healthy patient who presented with syncope and cardiopulmonary arrest during exercise. The enhanced computed tomography showed acute angle take-off (AAT) of LCA, and the exercise stress thallium-201 myocardial scintigraphy demonstrated a large defect at the LCA perfusion region. We propose that the coexistence of AAT and resulting ischemia causes sudden cardiac death during exercise in the patients with LCANCS.

Clinical efficacy and cost-effectiveness of lanthanum carbonate as second-line therapy in hemodialysis patients in Japan.

BACKGROUND AND OBJECTIVES: Lanthanum carbonate (LC) is a nonaluminum, noncalcium phosphate binder that is effective for hyperphosphatemia in dialysis patients. However, its efficacy and cost-effectiveness as second-line therapy have not been fully examined. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We first conducted a multicenter, open-label, 16-week clinical trial to examine the effect of additive LC in 116 hemodialysis patients who had uncontrolled hyperphosphatemia with conventional phosphorus-lowering therapy alone. Based on these clinical data, a state transition model was developed to evaluate the benefits and costs associated with LC as second-line therapy. Reduced risks for cardiovascular morbidity and mortality among patients treated with LC arise through more of the population achieving the target phosphorus levels. Uncertainty was explored through sensitivity analysis. RESULTS: After 16 weeks of additive LC treatment, mean serum phosphorus levels decreased from 7.30 ± 0.90 to 5.71 ± 1.32 mg/dl, without significant changes in serum calcium or intact parathyroid hormone levels. A subsequent cost-effectiveness analysis showed that compared with conventional treatment, additive LC incurred an average additional lifetime cost of $22,054 per person and conferred an additional 0.632 quality-adjusted life years (QALYs). This resulted in an incremental cost-effectiveness ratio of $34,896 per QALY gained. Applying a cost-effectiveness threshold of $50,000 per QALY, a probabilistic sensitivity analysis showed that additive LC had a 97.4% probability of being cost-effective compared with conventional treatment. CONCLUSIONS: Our results indicate that the use of LC as second-line therapy would be cost-effective among hemodialysis patients with uncontrolled hyperphosphatemia in Japan.

Effect of intravenous saccharated ferric oxide on serum FGF23 and mineral metabolism in hemodialysis patients.

BACKGROUND/AIMS: Fibroblast growth factor-23 (FGF23) plays a central role in the development of hypophosphatemia and inappropriately low 1,25-dihydroxyvitamin D induced by iron therapy for iron-deficiency anemia. The aim of this study was to examine the effect of intravenous saccharated ferric oxide on serum FGF23 levels and mineral metabolism in hemodialysis patients. METHODS: This prospective study enrolled 27 hemodialysis patients who had iron-deficiency anemia defined by a hemoglobin concentration < 10.5 g/dl and serum ferritin < 100 ng/ml. Intravenous saccharated ferric oxide at a dose of 40 mg was administered three times weekly over 3 weeks. The dose of active vitamin D and phosphate binders was kept unchanged. Serum FGF23, intact parathyroid hormone (PTH) and other parameters were prospectively monitored for 5 weeks. RESULTS: Serum FGF23 levels were markedly elevated [3,453 (338-6,383) pg/ml] at baseline. After 3 weeks of intravenous saccharated ferric oxide treatment, serum FGF23 further increased to 4,701 (1,251-14,396) pg/ml, and returned to the baseline values after 2 weeks of observation. There was also a significant decrease in intact PTH but no changes in serum calcium and phosphorus. CONCLUSIONS: Intravenous saccharated ferric oxide induces further increase in elevated FGF23 levels in hemodialysis patients. This increase does not induce hypophosphatemia and inappropriately low 1,25-dihydroxyvitamin D in the absence of functioning kidney, but may result in transient PTH suppression - possibly by directly acting on the parathyroid.

Cinacalcet effectively reduces parathyroid hormone secretion and gland volume regardless of pretreatment gland size in patients with secondary hyperparathyroidism.

BACKGROUND AND OBJECTIVES: Cinacalcet is effective in reducing serum parathyroid hormone (PTH) in patients with secondary hyperparathyroidism. However, it has not been proven whether parathyroid gland size predicts response to therapy and whether cinacalcet is capable of inducing a reduction in parathyroid volume. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This 52-week, multicenter, open-label study enrolled hemodialysis patients with moderate to severe secondary hyperparathyroidism (intact PTH >300 pg/ml). Doses of cinacalcet were adjusted between 25 and 100 mg to achieve intact PTH <180 pg/ml. Ultrasonography was performed to measure the parathyroid gland size at baseline, week 26, and week 52. Findings were also compared with those of historical controls. RESULTS: Of the 81 subjects enrolled, 56 had parathyroid glands smaller than 500 mm(3) (group S) and 25 had at least one enlarged gland larger than 500 mm(3) (group L). Treatment with cinacalcet effectively decreased intact PTH by 55% from baseline in group S and by 58% in group L. A slightly greater proportion of patients in group S versus group L achieved an intact PTH <180 pg/ml (46 versus 32%) and a >30% reduction from baseline (88 versus 78%), but this was not statistically significant. Cinacalcet therapy also resulted in a significant reduction in parathyroid gland volume regardless of pretreatment size, which was in sharp contrast to historical controls (n = 87) where parathyroid gland volume progressively increased with traditional therapy alone. CONCLUSIONS: Cinacalcet effectively decreases serum PTH levels and concomitantly reduces parathyroid gland volume, even in patients with marked parathyroid hyperplasia.

Effect of water impurity in CsLiB6O10 crystals on bulk laser-induced damage threshold and transmittance in the ultraviolet region.

We investigate the effect of water impurity in a CsLiB(6)O(10) (CLBO) crystal on the ultraviolet properties of the bulk laser-induced damage threshold (LIDT) and transmittance. The water impurity was eliminated by heating the CLBO sample with dimensions of 5 mm x 5 mm x 10 mm at 150 degrees C in an ambient atmosphere and subsequently in a dry atmosphere. The bulk LIDT of the sample after heat treatment improved by about 1.6-fold compared with that before heat treatment.

Continuous-wave all-solid-state 244 nm deep-ultraviolet laser source by fourth-harmonic generation of an optically pumped semiconductor laser using CsLiB6O10 in an external resonator.

We report an all-solid-state laser system that generates over 200 mW cw at 244 nm. An optically pumped semiconductor laser is internally frequency doubled to 488 nm. The 488 nm output is coupled to an external resonator, where it is converted to 244 nm using a CsLiB(6)O(10) (CLBO) crystal. The output power is limited by the available power at 488 nm, and no noticeable degradation in output power was observed over a period of several hours.

Carpal tunnel syndrome and plasma beta2-microglobulin concentration in hemodialysis patients.

As of the end of June 2005, 27 of 96 dialysis outpatients at our clinic had developed carpal tunnel syndrome (CTS). Of 19 patients who had undergone dialysis for 30 years or longer, 15 had CTS, whereas none of the 38 patients who had received dialysis for less than 10 years had CTS. These data reflect trends in CTS development: from 1983 the incidence of CTS increased for many years, but more recently there has been a decline in new cases of CTS. Comparison of the 27 CTS and 69 non-CTS dialysis patients at our clinic showed that those in the CTS group were older and had a longer duration of dialysis. Patients in the CTS group were found to have had a high plasma beta2-microglobulin (BMG) level in the distant past (15-21 years ago), but conversely had a much lower BMG level in recent years. Simple correlation analysis and multiple logistic regression analysis showed that the presence of CTS was correlated with high BMG levels in the distant past, in addition to age and duration of dialysis. These findings suggest that reduction of the plasma BMG level due to advances in dialysis therapy in recent years has contributed to the decreased incidence of CTS.

TMJ osteoarthritis/osteoarthrosis and immune system factors in a Japanese sample.

OBJECTIVE: To determine whether there is an association between temporomandibular joint (TMJ) osteoarthritis/osteoarthrosis (OA) and immune system factors in a Japanese sample. MATERIALS AND METHODS: The records of 41 subjects (7 men, aged 22.0 +/- 3.8 years; 34 women, aged 24.8 +/- 6.3 years) and 41 pair-matched controls (7 men, aged 22.1 +/- 2.3 years; 34 women, aged 24.8 +/- 6.4 years) based on age and gender were reviewed. Information on medical history included local or systemic diseases, details on medication type and use, and the presence of allergies and asthma. Dental history questions referred to details regarding past oral injuries. The validity of the hypothesis, defining allergies and asthma as risk factors in OA, was tested by using a logistic regression analysis. RESULTS: The incidence of allergy was significantly higher in the TMJ OA (P = .008), with a mean odds ratio of 4.125 and a 95% confidence interval of 1.446-11.769. CONCLUSION: These results suggest that allergy may be a risk factor in association with TMJ OA in this Japanese sample.