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PubMed | Red Cross, Tokyo Health Service Association, Tokyo Women's Medical University, Yokohama Dai ichi Hospital and 7 more.
Type: Journal Article | Journal: Clinical and experimental nephrology | Year: 2016

In Fabry disease, progressive glycolipid accumulation leads to damage in kidney and other organs. This study was designed to determine the prevalence rate of Fabry disease in Japanese dialysis patients.All dialysis patients agreeing to Japan Fabry disease screening study (J-FAST) with informed consent were selected except for Fabry disease. The screening was performed by a method of measuring plasma and/or leukocytes lysosomal -galactosidase A protein level and -galactosidase A activity. If positive, genetic analysis was carried out upon patients agreement.J-FAST dealt with 8547 patients (male 5408, female 3139). At the tertiary examination, 26 out of 8547 patients were found to be positive. Six out of 26 patients could not accept genetic analysis because of death. Remaining 20 patients agreed with genetic analysis; then 2 patients (male 2, female 0) had a variation of the -Gal gene and 11 patients showed E66Q variations. Therefore, the frequency of Fabry disease in J-FAST was 0.04 % (2/5408) in males and 0 % (0/3139) in females, and then 0.02 % (2/8547) in all patients. The presumptive clinical diagnoses of end-stage kidney disease (ESKD) were 10 chronic glomerulonephritis, 7 diabetic nephropathy, 3 unknown etiology, 3 nephrosclerosis, 1 gouty nephropathy, 1 autosomal dominant polycystic kidney disease and 1 renal tuberculosis among 26 tertiary positive patients. Two male Fabry patients were initially diagnosed as nephrosclerosis and chronic glomerulonephritis.The prevalence rate of Fabry disease in J-FAST was 0.02 %. Moreover, Fabry disease could not be ruled out as the clinical diagnosis of ESKD.

Momotani N.,Tokyo Health Service Association | Iwama S.,Tokyo Health Service Association | Momotani K.,University of Virginia
Journal of Clinical Endocrinology and Metabolism | Year: 2012

Context: The importance of maternal T 4 for brain development prior to the onset of fetal thyroid function has been suggested in basic studies, and a correlation between mild maternal T 4 deficiency in early gestation and disturbance of neurodevelopment in progenies has been shown in large case-control studies. These findings suggest that maternal T 4 deficiency in early pregnancy potentially affects neurointellectual development. On the other hand, no apparent adverse effect in children born to mothers with overt hypothyroidism in Japan has been reported where maternal T 4 had been restored to normal by late pregnancy. Objective: We report five cases in Japan showing no apparent effect of maternal T 4 deficiency on neurodevelopment in progenies where low T4 levels had been corrected by late pregnancy. Methods: Five women with overt hypothyroidism detected at 6-16 wk gestation initiated T4 treatment. Four women restored euthyroidism by the 20th week. One remained in a subclinical hypothyroid state. Developmental scores of their children were evaluated between 25 months and 11 yr of age by either the Tsumori-Inage Infant's Developmental Test or the Wechsler Intelligence Scale for Children-Third Edition and compared to those of corresponding siblings with no exposure to maternal hypothyroidism. Results: The development scores of all the children turned out to be either normal or advanced. Conclusions: In iodine-sufficient areas, maternal T 4 deficiency in early pregnancy does not necessarily affect neurodevelopment. Therefore, other potential factors altering neurodevelopment, such as iodine deficiency, must be investigated. Copyright © 2012 by The Endocrine Society.

PubMed | Tokyo Health Service Association, The University of Shimane and Okayama Medical Center
Type: Case Reports | Journal: Molecular genetics and metabolism | Year: 2015

Sivelestat sodium (sivelestat), a neutrophil elastase inhibitor, is used to treat acute respiratory distress syndrome (ARDS). We report two cases that developed elevated C5-acylcarnitine (C5-AC) levels following treatment with sivelestat. Case 1 was a 14-day-old female infant born at 25 weeks and 1 day of gestation who was treated with sivelestat for the prophylaxis of Wilson-Mikity syndrome soon after birth. Isovaleric acidemia (IVA) was suspected based on a newborn screening using tandem mass spectrometry (MS/MS). Her C5-AC level was elevated to 4.49 M (cut-off, <1.0) after treatment with sivelestat. Case 2 was a 4-year-old female with pneumocystis pneumonia that developed during chemotherapy for disseminated medulloblastoma. Sivelestat was given for the complication of ARDS. Her C5-AC level increased (1.09 M) after eight days of treatment with sivelestat.In both cases, IVA was ruled out because isovalerylglycine was not observed in the urinary organic acid analysis. Case 1 was associated with carnitine deficiency (C0 9.16 M; reference value, 10-60). Liquid chromatography-MS/MS confirmed elevated pivaloylcarnitine (PVC) in both cases.Similar to antibiotics containing pivalic acid (PVA), sivelestat contains PVA, which has the potential to cause secondary carnitine deficiency. In addition, elevated PVC can lead to false positive findings of IVA in newborns screened using MS/MS.

Kobayashi M.,Jikei University School of Medicine | Ohashi T.,Jikei University School of Medicine | Fukuda T.,Jikei University School of Medicine | Yanagisawa T.,Kitasato University | And 6 more authors.
Molecular Genetics and Metabolism | Year: 2012

Background: Fabry disease is an X-linked lysosomal disorder resulting from mutations in the α-galactosidase A (GLA) gene. Recent reports described that the E66Q mutation of GLA is not a disease-causing mutation. However, no pathological study was reported. We carried out pathological studies using a cardiac biopsy specimen from a patient with the E66Q mutation. Materials and methods: The case was a 34. year old male patient with end-stage renal failure and cardiomegaly. He was diagnosed with gout at 15. years of age and hemodialysis was started for gouty nephropathy from 31. years of age. He was suspected of having Fabry disease as the result of a screening study for Fabry disease in patients with end-stage renal failure and was referred to our hospital for mutation analysis of the GLA gene. We carried out enzymatic and genetic analysis for GLA and pathological studies of a cardiac biopsy specimen. Results: The patient had the E66Q mutation in the GLA gene. GLA activity in leukocytes was 36.2% of the average of normal controls. The pathological study of the cardiac biopsy sample showed no characteristic findings of Fabry disease. The immunohistochemistry for GL3 of the cardiac biopsy sample showed no positive cells. Conclusion: Although the E66Q mutation reduced enzyme activity, the characteristic pathological findings of Fabry disease and the abnormal accumulation of GL3 were not detected in cardiac tissues. The E66Q mutation of the GLA gene is thought to be a functional polymorphism based on enzymatic and pathological studies. © 2012 Elsevier Inc.

Kawata Y.,Tokushima University | Niki N.,Tokushima University | Ohmatsu H.,Tokushima University | Kusumoto M.,National Cancer Center Hospital East | And 4 more authors.
Medical Physics | Year: 2012

Purpose: Quantification of the CT appearance of non-small cell lung cancer (NSCLC) is of interest in a number of clinical and investigational applications. The purpose of this work is to present a quantitative five-category (, , and ) classification method based on CT histogram analysis of NSCLC and to determine the prognostic value of this quantitative classification. Methods: Institutional review board approval and informed consent were obtained at the National Cancer Center Hospital. A total of 454 patients with NSCLC (maximum lesion size of 3 cm) were enrolled. Each lesion was measured using multidetector CT at the same tube voltage, reconstruction interval, beam collimation, and reconstructed slice thickness. Two observers segmented NSCLC nodules from the CT images by using a semi-automated three-dimensional technique. The two observers classified NSCLCs into one of five categories from the visual assessment of CT histograms obtained from each nodule segmentation result. Interobserver variability in the classification was computed with Cohen's statistic. Any disagreements were resolved by consensus between the two observers to define the gold standard of the classification. Using a classification and regression tree (CART), the authors obtained a decision tree for a quantitative five-category classification. To assess the impact of the nodule segmentation on the classification, the variability in classifications obtained by two decision trees for the nodule segmentation results was also calculated with the Cohen's statistic. The authors calculated the association of recurrence with prognostic factors including classification, sex, age, tumor diameter, smoking status, disease stage, histological type, lymphatic permeation, and vascular invasion using both univariate and multivariate Cox regression analyses. Results: The values for interobserver agreement of the classification using two nodule segmentation results were 0.921 (P 0.001) and 0.903 (P 0.001), respectively. The values for the variability in the classification task using two decision trees were 0.981 (P 0.001) and 0.981 (P 0.001), respectively. All the NSCLCs were classified into one of five categories (type , n 8; type , n 38; type , n 103; type , n 112; type , n 193) by using a decision tree. Using a multivariate Cox regression analysis, the classification (hazard ratio 5.64; P 0.008) and disease stage (hazard ratio 8.33; P 0.001) were identified as being associated with an increased recurrence risk. Conclusions: The quantitative five-category classifier presented here has the potential to provide an objective classification of NSCLC nodules that is strongly correlated with prognostic factors. © 2012 American Association of Physicists in Medicine.

Suka M.,Jikei University School of Medicine | Miwa Y.,Tokyo Health Service Association | Ono Y.,Tokyo Health Service Association | Yanagisawa H.,Jikei University School of Medicine
Journal of Occupational and Environmental Medicine | Year: 2012

OBJECTIVE:: To evaluate the impact of weight gain on cardiovascular risk factors among younger (25 to 44 years) and older (45 to 64 years) Japanese male workers in terms of population attributable risk percentage (PAR%). METHODS:: Using the 2008 and 2009 health examination data, 49,587 eligible male workers aged 25 to 64 years were examined for their 1-year changes in body weight and cardiovascular risk factors. RESULTS:: Mean weight change was significantly greater than zero in the younger group (+0.27 kg) but not in the older group (-0.08 kg). The PAR% due to weight gain for the development and maintenance of cardiovascular risk factors was estimated at 21.8% and 5.4%, respectively, in the younger and older groups. CONCLUSIONS:: The age-stratified PAR% estimates suggest that weight gain prevention programs will make greater contributions to cardiovascular health in younger than in older male workers. Copyright © 2012 by American College of Occupational and Environmental Medicine.

Togawa T.,Meiji Pharmaceutical University | Kodama T.,Meiji Pharmaceutical University | Suzuki T.,Meiji Pharmaceutical University | Sugawara K.,Meiji Pharmaceutical University | And 6 more authors.
Molecular Genetics and Metabolism | Year: 2010

Fabry disease is an X-linked genetic disorder caused by a deficiency of α-galactosidase A (GLA) activity. As enzyme replacement therapy (ERT) involving recombinant GLAs has been introduced for this disease, a useful biomarker for diagnosis and monitoring of therapy has been strongly required. We measured globotriaosylsphingosine (lyso-Gb3) and globotriaosylceramide (Gb3) in plasma samples from ten hemizygous males (six classic and four variant cases) and eight heterozygous females with Fabry disease, and investigated the responses of plasma lyso-Gb3 and Gb3 in a male Fabry patient who had undergone ERT for 4 years to determine whether plasma lyso-Gb3 and Gb3 could be biomarkers of Fabry disease. The results revealed that plasma lyso-Gb3 was apparently increased in male patients and was higher in cases of the classic form than those of the variant one. In Fabry females, plasma lyso-Gb3 was moderately increased in both symptomatic and asymptomatic cases, and there was a correlation between the increase in lyso-Gb3 and the decrease in GLA activity. As to plasma Gb3, the levels in the variant Fabry hemizygotes and Fabry heterozygotes could not be distinguished from those in the controls, although those in the classic Fabry hemizygotes were increased. The plasma lyso-Gb3 level in the Fabry patient who had received ERT was elevated at the baseline and fell more dramatically on ERT than that of Gb3. Plasma lyso-Gb3 could thus be a potential biomarker of Fabry disease. © 2010 Elsevier Inc. All rights reserved.

Suka M.,Jikei University School of Medicine | Miwa Y.,Tokyo Health Service Association | Ono Y.,Tokyo Health Service Association | Yanagisawa H.,Jikei University School of Medicine
Environmental Health and Preventive Medicine | Year: 2011

Objective: To examine whether the association between waist circumference (WC) and clustering of cardiovascular risk factors varies with obesity (BMI) status. Methods: Using the 2008 health examination data of a Japanese health service association, eligible 57,141 adults aged 20-65 years without coronary heart disease or stroke, whose blood sample had been taken in the fasting state, were enrolled in the study. The participants were classified as being underweight (BMI <18.5), normal weight (BMI 18.5-24.9), and overweight (BMI ≥25.0). Multiple logistic regression analysis was performed to calculate adjusted odds ratios (OR) for clustering of cardiovascular risk factors. Receiver operating characteristic analysis was performed to assess the ability of WC to discriminate subjects with and without a clustering of cardiovascular risk factors. Results: Clustering of cardiovascular risk factors was found in 16.0% of men and 3.4% of women. The adjusted OR [95% confidence intervals (CI)] per 5-cm increase in WC of the underweight, normal weight, and overweight groups was 1.57 (1.12-2.20), 1.55 (1.49-1.62), and 1.34 (1.30-1.38), respectively, for men and 1.50 (0.84-2.69), 1.53 (1.40-1.68), and 1.32 (1.23-1.41), respectively, for women. The area under curve (95% CI) of the underweight, normal weight, and overweight groups was 0.68 (0.59-0.77), 0.70 (0.69-0.71), and 0.62 (0.61-0.63), respectively, for men and 0.70 (0.53-0.86), 0.75 (0.73-0.78), and 0.64 (0.61-0.68), respectively, for women. Conclusion: High WC was associated with increased risk of clustering of cardiovascular risk factors independent of BMI. As well as the magnitude of the association, the ability of WC to discriminate subjects with and without a clustering of cardiovascular risk factors varied with obesity (BMI) status. © 2010 The Japanese Society for Hygiene.

Momotani N.,Tokyo Health Service Association
Nihon rinsho. Japanese journal of clinical medicine | Year: 2012

In the treatment of pregnant patients with Graves' disease, propylthiouracil is preferred over methimazole in early pregnancy because of a possible teratogenicity of methimazole. Methimazole is preferable to propylthiouracil in other time of pregnancy on the basis of severe liver dysfunction occasionally caused by propylthiouracil. Fetal hypothyroidism can be avoided when maternal free T4 levels are maintained at or above the upper normal limit for non-pregnant subjects. However, maternal free T4 should be kept normal for pregnant reference range when pregnancy complications develop. Fetal hypothyroidism in this setting will not affect the infant's development as long as mothers are euthyroid and the infants recover from hypothyroid state within a short time after birth. In hypothyroid women, 1-T4 dose often needs to be increased in pregnancy. Maternal T4 deficiency in early pregnancy has been suggested to affect normal brain development in the offspring. However, it has recently been shown in iodine rich area that no adverse effect on neuropsychological development was seen irrespective of the severity of maternal T4 deficiency. Insufficient iodine intake in the mother can cause low T4 in pregnancy and also inadequate production of T4 in breast-fed infants when sufficient T4 is essential for normal brain development.

Kitagawa T.,Tokyo Health Service Association
Pediatric Endocrinology Reviews | Year: 2012

In 1977, the Ministry of Health and Welfare (MHW) directed prefectural officials in charge of maternal and child health to start publicly funded newborn mass-screening (NBS) for phenylketonuria (PKU), maple syrup urine disease (MSUD), histidinemia, homocystinuria and galactosemia and a study group of MHW formulated the treatment guideline for the target diseases. In 1980, MHW launched the Japan Cooperative Project on Special Formula (JCPSF) to ensure a stable supply of special formula and also organized the committee for JCPSF. From 1977 to 2003, a study group of MHW conducted a follow-up study of the patients detected by the screening. From the follow-up it was concluded that dietary therapy was unnecessary for histidinemia and the screening for the disease was discontinued. In 1995, the guideline for the treatment of PKU was revised to keep lower blood phenylalanine levels. The guideline committee for the treatment of BH4 deficiency was establish in 1996 to obtain better prognosis. In 2012, the MHW decided to initiate publicly funded NBS using MSIMS for inborn errors of amino acid, organic acid, and fatty acid metabolism. The Japanese nationwide NBS has been performed for 35 years. This paper reviews the Japanese history of the development of NBS which has enabled more IEM patients to lead active and productive lives today.

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