H Nagasaka

Summary

Country: Japan

Publications

  1. ncbi request reprint Depletion of high-density lipoprotein and appearance of triglyceride-rich low-density lipoprotein in a Japanese patient with FIC1 deficiency manifesting benign recurrent intrahepatic cholestasis
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Heta Cho, Midori ku, Chiba, Japan
    J Pediatr Gastroenterol Nutr 45:96-105. 2007
  2. doi request reprint Intractable secretory diarrhea in a Japanese boy with mitochondrial respiratory chain complex I deficiency
    Kei Murayama
    Department of Metabolism, Chiba Children s Hospital, 579 1, Henda cho, Midori ku, Chiba, 266 0007, Japan
    Eur J Pediatr 168:297-302. 2009
  3. doi request reprint Characteristics of NO cycle coupling with urea cycle in non-hyperammonemic carriers of ornithine transcarbamylase deficiency
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, 4 5 1 Kohama Cho, Takarazuka 665 0827, Japan
    Mol Genet Metab 109:251-4. 2013
  4. doi request reprint Metabolic improvements in intrahepatic porto-systemic venous shunt presenting various metabolic abnormalities by 4-phenylacetate
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan
    Clin Chim Acta 419:52-6. 2013
  5. doi request reprint Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka 665 0827, Japan
    J Inherit Metab Dis 36:75-81. 2013
  6. doi request reprint Oxysterol changes along with cholesterol and vitamin D changes in adult phenylketonuric patients diagnosed by newborn mass-screening
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan
    Clin Chim Acta 416:54-9. 2013
  7. doi request reprint Fatty liver and anti-oxidant enzyme activities along with peroxisome proliferator-activated receptors γ and α expressions in the liver of Wilson's disease
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, 4 5 1 Kohama Cho, Takarazuka 665 0827, Japan
    Mol Genet Metab 107:542-7. 2012
  8. doi request reprint Lipoprotein profiles in children with two common cholesteryl ester transfer protein gene mutations, D442G and I14A, during the first year of life
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Clin Chim Acta 406:52-6. 2009
  9. doi request reprint Sustaining hypercitrullinemia, hypercholesterolemia and augmented oxidative stress in Japanese children with aspartate/glutamate carrier isoform 2-citrin-deficiency even during the silent period
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, 579 1 Heta Cho, Midori ku, Chiba 266 0007, Japan
    Mol Genet Metab 97:21-6. 2009
  10. doi request reprint Reduced apolipoprotein E-rich high-density lipoprotein level at birth is restored to the normal range in patients with familial hypercholesterolemia in the first year of life
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, 579 1 Heta Cho, Midori ku, Chiba, Japan
    J Clin Endocrinol Metab 93:779-83. 2008

Collaborators

Detail Information

Publications37

  1. ncbi request reprint Depletion of high-density lipoprotein and appearance of triglyceride-rich low-density lipoprotein in a Japanese patient with FIC1 deficiency manifesting benign recurrent intrahepatic cholestasis
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Heta Cho, Midori ku, Chiba, Japan
    J Pediatr Gastroenterol Nutr 45:96-105. 2007
    ..This study was performed to investigate the detailed lipoprotein metabolism in benign recurrent intrahepatic cholestasis (BRIC) caused by FIC1 deficiency...
  2. doi request reprint Intractable secretory diarrhea in a Japanese boy with mitochondrial respiratory chain complex I deficiency
    Kei Murayama
    Department of Metabolism, Chiba Children s Hospital, 579 1, Henda cho, Midori ku, Chiba, 266 0007, Japan
    Eur J Pediatr 168:297-302. 2009
    ..Complex I deficiency is a novel cause of secretory diarrhea and may act via disrupting the supply of adenosine triphosphate (ATP) needed for the maintenance of ion gradients across membranes...
  3. doi request reprint Characteristics of NO cycle coupling with urea cycle in non-hyperammonemic carriers of ornithine transcarbamylase deficiency
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, 4 5 1 Kohama Cho, Takarazuka 665 0827, Japan
    Mol Genet Metab 109:251-4. 2013
    ..In two of those three, the occurrence of headaches was decreased. These results suggest that NO cycle coupling with the urea cycle is altered substantially even in non-hyperammonemic OTCD carriers, predisposing them to headaches...
  4. doi request reprint Metabolic improvements in intrahepatic porto-systemic venous shunt presenting various metabolic abnormalities by 4-phenylacetate
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan
    Clin Chim Acta 419:52-6. 2013
    ..4-Phenylacetate (4-PA) has effects to eliminate blood ammonia, bile acids and bilirubin. 4-PA might be expected to improve the metabolic abnormalities in intrahepatic CPSVS...
  5. doi request reprint Sustained high plasma mannose less sensitive to fluctuating blood glucose in glycogen storage disease type Ia children
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka 665 0827, Japan
    J Inherit Metab Dis 36:75-81. 2013
    ..Plasma mannose is less sensitive to blood glucose and insulin in GSD Ia children. Its basal level and the fluctuation pattern differ by their metabolic activity...
  6. doi request reprint Oxysterol changes along with cholesterol and vitamin D changes in adult phenylketonuric patients diagnosed by newborn mass-screening
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, Takarazuka, Japan
    Clin Chim Acta 416:54-9. 2013
    ..Phenylketonuria (PKU) possibly leads to hypocholesterolemia and lowered vitamin D (VD) status. Metabolism of oxysterols linking with those of cholesterol and VD has never been examined in PKU...
  7. doi request reprint Fatty liver and anti-oxidant enzyme activities along with peroxisome proliferator-activated receptors γ and α expressions in the liver of Wilson's disease
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, 4 5 1 Kohama Cho, Takarazuka 665 0827, Japan
    Mol Genet Metab 107:542-7. 2012
    ..However, such PPARs have never been studied in WD...
  8. doi request reprint Lipoprotein profiles in children with two common cholesteryl ester transfer protein gene mutations, D442G and I14A, during the first year of life
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Clin Chim Acta 406:52-6. 2009
    ..Hyperalphalipoproteinemia is associated with cholesteryl ester transfer protein (CETP) deficiency in adults but has unclear associations in children...
  9. doi request reprint Sustaining hypercitrullinemia, hypercholesterolemia and augmented oxidative stress in Japanese children with aspartate/glutamate carrier isoform 2-citrin-deficiency even during the silent period
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, 579 1 Heta Cho, Midori ku, Chiba 266 0007, Japan
    Mol Genet Metab 97:21-6. 2009
    ..Results of this study showed, even during the silent period, sustained hypercitrullinemia, hypercholesterolemia, and augmented oxidative stress in children with citrin deficiency...
  10. doi request reprint Reduced apolipoprotein E-rich high-density lipoprotein level at birth is restored to the normal range in patients with familial hypercholesterolemia in the first year of life
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, 579 1 Heta Cho, Midori ku, Chiba, Japan
    J Clin Endocrinol Metab 93:779-83. 2008
    ..High-density lipoprotein (HDL) consists of apolipoprotein E (apoE)-rich and apoE-poor HDL particles. ApoE-rich HDL level is high at birth but decreases after birth with reciprocal elevation in low-density lipoprotein (LDL)-cholesterol...
  11. ncbi request reprint Children's toxicology from bench to bed--Liver Injury (3): Oxidative stress and anti-oxidant systems in liver of patients with Wilson disease
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Japan
    J Toxicol Sci 34:SP229-36. 2009
    ..Their CuZn-SOD, Mn-SOD and catalase activities were increased. These results suggest that excessive copper-derived oxidants contribute to development and progression of liver disease in WD...
  12. ncbi request reprint Improvements of hypertriglyceridemia and hyperlacticemia in Japanese children with glycogen storage disease type Ia by medium-chain triglyceride milk
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, 579 1 Heta Cho Midori Ku, Chiba, Japan
    Eur J Pediatr 166:1009-16. 2007
    ....
  13. ncbi request reprint Relationship between oxidative stress and antioxidant systems in the liver of patients with Wilson disease: hepatic manifestation in Wilson disease as a consequence of augmented oxidative stress
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Pediatr Res 60:472-7. 2006
    ..Their CuZn-SOD, Mn-SOD, and catalase activities were increased. These results suggest that excessive copper-derived oxidants contribute to development and progression of liver disease in WD...
  14. ncbi request reprint Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Eur J Pediatr 165:618-24. 2006
    ..In addition to height and body weight, blood levels of proteins, lipids, growth hormone (GH), insulin-like growth factor-I (IGF-I) and IGF-binding protein -3 (IGFBP-3) were monitored...
  15. doi request reprint Altered metabolisms of mediators controlling vascular function and enhanced oxidative stress in asymptomatic children with congenital portosystemic venous shunt
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 2666 0007, Japan
    Metabolism 59:107-13. 2010
    ..These results suggest the presence of altered metabolisms of vascular mediators and enhanced oxidative stress in asymptomatic preschool children with congenital PSVS...
  16. doi request reprint Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Midori ku, Chiba, Japan
    Mol Genet Metab 100:123-8. 2010
    ..These results suggest that 4-PA enhanced liver BSEP/ABCB11 function and thereby improved liver functions in OTC-deficient children. For treatment of liver disorders requiring enhancement of BSEP function, 4-PA might be a candidate...
  17. doi request reprint CD36 deficiency predisposing young children to fasting hypoglycemia
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Metabolism 60:881-7. 2011
    ..05). These results suggested that type I CD36 deficiency but not type II CD36 deficiency predisposes preschool children to hypoglycemia...
  18. doi request reprint Cross-sectional study of bone metabolism with nutrition in adult classical phenylketonuric patients diagnosed by neonatal screening
    Hironori Nagasaka
    Department of Pediatrics, Takarazuka City Hospital, 4 5 1 Kohama Cho, Takarazuka 665 0827, Japan
    J Bone Miner Metab 29:737-43. 2011
    ..001; males, P < 0.01). None of the bone parameters correlated significantly with serum phenylalanine or nutrient intake. PKU patients exhibited lower VD status and more rapid bone resorption despite normal calcium-VD intakes...
  19. ncbi request reprint Nitric oxide synthesis in ornithine transcarbamylase deficiency: possible involvement of low no synthesis in clinical manifestations of urea cycle defect
    Hironori Nagasaka
    Department of Pediatrics, Hokkaido University Graduate School of Medicine, Sapporo, Japan
    J Pediatr 145:259-62. 2004
    ..The patients were found to have low NO synthesis, suggesting that the low NO synthesis contributes to the clinical manifestations of urea cycle defect...
  20. ncbi request reprint Two cases with transient lipoprotein lipase (LPL) activity impairment: evidence for the possible involvement of an LPL inhibitor
    H Nagasaka
    Department of Pediatrics, Hokkaido University School of Medicine, Kita Ku N 15 W 7, 060 8638 Sapporo, Japan
    Eur J Pediatr 162:132-8. 2003
    ..The exact reasons why their post-heparin LPL activities were impaired for a certain period and why their HDL cholesterol levels have remained low are still unclear...
  21. doi request reprint Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defects
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Metabolism 58:278-82. 2009
    ..Although the molecular mechanisms remain poorly understood, we infer that the NO pathway might play a role in the pathophysiology related to congenital urea cycle disorders...
  22. ncbi request reprint Successful living-donor liver transplantation from an asymptomatic carrier mother in ornithine transcarbamylase deficiency
    H Nagasaka
    Department of Pediatrics, Hokkaido University School of Medicine, Sapporo, Japan
    J Pediatr 138:432-4. 2001
    ..One-year monitoring of plasma amino acid and urinary orotate/orotidine levels revealed no abnormality in the urea cycle in either subject...
  23. ncbi request reprint Effects of triamcinolone on brain and cerebrospinal fluid apolipoprotein E levels in rats
    H Chiba
    Department of Laboratory Medicine, Hokkaido University School of Medicine, Sapporo, Japan
    Life Sci 60:1757-61. 1997
    ..ApoA-I appears unlikely to play a significant role in the rat central nervous system...
  24. ncbi request reprint Resistance to parathyroid hormone in two patients with familial intrahepatic cholestasis: possible involvement of the ATP8B1 gene in calcium regulation via parathyroid hormone
    Hironori Nagasaka
    Department of Pediatrics, Hokkaido University Graduate School of Medicine, Japan
    J Pediatr Gastroenterol Nutr 39:404-9. 2004
  25. doi request reprint Effects of bezafibrate on dyslipidemia with cholestasis in children with familial intrahepatic cholestasis-1 deficiency manifesting progressive familial intrahepatic cholestasis
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Metabolism 58:48-54. 2009
    ..Liver expression of multidrug resistance protein-3, which regulates lipoprotein X synthesis, was enhanced by bezafibrate therapy. Bezafibrate treatment favorably affected pruritus, dyslipidemia, and cholestasis in PFIC-1...
  26. doi request reprint Fluctuation of lipoprotein metabolism linked with bile acid-activated liver nuclear receptors in Alagille syndrome
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Chiba 266 0007, Japan
    Atherosclerosis 198:434-40. 2008
    ..In the incomplete AGS parents, TBA and lipoprotein parameters showed little fluctuation. In summary, cholestasis is closely related to dyslipidemia and hepatic nuclear receptor expression in AGS patients...
  27. pmc Significant correlations between the flow volume of patent ductus venosus and early neonatal liver function: possible involvement of patent ductus venosus in postnatal liver function
    K Murayama
    Division of Metabolism, Chiba Children s Hospital, 579 1, Chiba, 266 0007 Japan
    Arch Dis Child Fetal Neonatal Ed 91:F175-9. 2006
    ..The ductus venosus remains open with shunt flow in most neonates for a certain period after birth. However, the effects of blood flow through the ductus venosus on neonatal liver function remain unclear...
  28. doi request reprint Fluctuating liver functions in siblings with MPV17 mutations and possible improvement associated with dietary and pharmaceutical treatments targeting respiratory chain complex II
    Shunsaku Kaji
    Department of Pediatrics, Tsuyama Central Hospital, Tsuyama shi, Okayama 708 0841, Japan
    Mol Genet Metab 97:292-6. 2009
    ..To describe the clinical and biological findings of two Japanese siblings with novel MPV17 gene mutations (c.451insC/c.509C > T) manifesting hepatic mitochondrial DNA depletion syndrome...
  29. ncbi request reprint Unique character and metabolism of high density lipoprotein (HDL) in fetus
    Hironori Nagasaka
    Department of Pediatrics, Hokkaido University School of Medicine, N15 W7, Sapporo 060 8638, Japan
    Atherosclerosis 161:215-23. 2002
    ..These results indicate that, in fetus, the character and metabolism of HDL, especially of apo E-rich HDL cholesterol, are distinct from those in adults...
  30. ncbi request reprint Molecular and clinical analyses of Japanese patients with carbamoylphosphate synthetase 1 (CPS1) deficiency
    Keiji Kurokawa
    Department of Pediatrics, Kyoto University Hospital, 54 Shogoin Sakyo, Kyoto, Japan
    J Hum Genet 52:349-54. 2007
    ..We also identified two common polymorphisms that might be useful for simple linkage analysis in prenatal diagnosis. The accumulated clinical data will also help to reveal the clinical presentation of this rare disorder in Japan...
  31. ncbi request reprint Evaluation of risk for atherosclerosis in Alagille syndrome and progressive familial intrahepatic cholestasis: two congenital cholestatic diseases with different lipoprotein metabolisms
    Hironori Nagasaka
    Division of Metabolism, Chiba Children s Hospital, Japan
    J Pediatr 146:329-35. 2005
    ..To evaluate the risk for atherosclerosis in Alagille syndrome (AGS) and progressive familial intrahepatic cholestasis (PFIC) on the basis of lipoprotein metabolism and by ultrasonography...
  32. ncbi request reprint Measles pneumonia: treatment of a near-fatal case with nitric oxide inhalation
    Akemi Kimura
    Department of Pediatrics, Saitama Medical School, Moroyama, Iruma gun, Saitama, Japan
    Pediatr Int 44:451-2. 2002
  33. doi request reprint Determination of asymmetric dimethylarginine, an endogenous nitric oxide synthase inhibitor, in umbilical blood
    Hirokazu Tsukahara
    Department of Pediatrics, Faculty of Medical Sciences, University of Fukui, Fukui 910 1193, Japan
    Metabolism 57:215-20. 2008
    ..We infer that the high ADMA levels play physiological roles in maintaining vascular tone and blood redistribution to vital organs during birth, thereby favoring the circulatory transition from fetal to neonatal life...
  34. doi request reprint Craniotabes in normal newborns: the earliest sign of subclinical vitamin D deficiency
    Junko Yorifuji
    Department of Pediatrics, Kyoto University Hospital, 54 Shogoin Sakyo, Kyoto 606 8507, Japan
    J Clin Endocrinol Metab 93:1784-8. 2008
    ..Craniotabes in otherwise normal neonates has been regarded as physiological and left untreated...
  35. ncbi request reprint Immunohistochemical analysis of Mallory bodies in Wilsonian and non-Wilsonian hepatic copper toxicosis
    Thomas Muller
    Department of Pediatrics, University of Innsbruck, Innsbruck, Austria
    Hepatology 39:963-9. 2004
    ..By sequestering potentially harmful misfolded ubiquitinated proteins as inert cytoplasmic inclusion bodies (e.g., as MBs), p62 may be a major player in an important cellular rescue mechanism in oxidative hepatocyte injury...
  36. ncbi request reprint Apolipoprotein E (ApoE) and ApoE-rich high-density cholesterol as potential early diagnostic markers of congenital biliary tract anomalies
    Hironori Nagasaka
    J Pediatr 143:411. 2003
  37. ncbi request reprint Improved HPLC assay for lipid peroxides in human plasma using the internal standard of hydroperoxide
    Shu Ping Hui
    Faculty of Pharmaceutical Sciences, Health Sciences University of Hokkaido, Hokkaido 061 0293, Japan
    Lipids 40:515-22. 2005
    ..Sensitive and specific monitoring of plasma lipid peroxides using the present chemiluminescence HPLC approach with the synthesized I.S. may help our understanding of chemical and pathophysiological aspects of lipid peroxidation...