Paper Search Console

Home Search Page About Contact

Journal Title

Title of Journal: Eur J Pediatr

Search In Journal Title:

Abbravation: European Journal of Pediatrics

Search In Journal Abbravation:

Publisher

Springer-Verlag

Search In Publisher:

ISSN

1432-1076

Search In ISSN:
Search In Title Of Papers:

Radical trapping in glycogen storage disease 1a

Authors: Birgit Wittenstein Marcus Klein Barbara Finckh Kurt Ullrich Alfried Kohlschütter
Publish Date: 2014/05/02
Volume: 161, Issue: 1, Pages: S70-S74
PDF Link

Abstract

Oxidative mechanisms involving lipid peroxidation in the subendothelium of the arterial vessel wall play a key role in atherogenesis Despite severe hyperlipidaemia patients with glycogen storage disease type la GSD1a do not develop premature atherosclerosis Therefore we analysed parameters of antioxidative defence and oxidative stress in plasma and serum of patients with GSD1a n=17 and compared them with those of patients with type 1 diabetes mellitus n=17 familial hypercholesterolaemia n=18 and healthy controls n=20 We measured the total radical trapping ability parameter TRAP single plasma antioxidants sulfhydrylgroups uric acid vitamin C alphatocopherol coenzymeQ10 markers of lipid peroxidation lipoprotein a and homocysteine Patients with GSD1a showed an elevated TRAP P001 compared to the three other groups This can mainly be attributed to elevated uric acid levels P005 versus control Lipoprotein a was significantly lower in the GSD1a group compared to the three other groups P005Conclusion Patients with glycogen storage disease type 1a show an increased antioxidative defence in plasma which may protect them against lipid peroxidation and thus against premature atherosclerosis Our finding of low lipoprotein a levels in this small group of patients warrants further investigation in a greater number of patients before assessing its role in atherogenesis in glycogen storage disease type 1a

Keywords:

References

.
Search In Abstract Of Papers:
Other Papers In This Journal:
  1. Massive gastrointestinal haemorrhage in isolated intestinal Henoch-Schonlein purpura with response to intravenous immunoglobulin infusion
  2. Reversible posterior encephalopathy syndrome associated with micronodular adrenocortical disease and Cushing syndrome
  3. Auto-immune pancytopenia in a child with DiGeorge syndrome
  4. Effective parenteral clodronate treatment of a child with severe juvenile idiopathic osteoporosis
  5. Evaluation of the Omron MX3 Plus monitor for blood pressure measurement in adolescents
  6. Analysis of the interleukin-12/interferon-γ pathway in children with non-tuberculous mycobacterial cervical lymphadenitis
  7. The effectiveness of octreotide in the treatment of post-operative chylothorax
  8. Persistence of anti-HBs antibody in children whom vaccinated during infantile period and need to booster needs more discussion
  9. Is 99m Tc-HMPAO granulocyte scan an alternative to endoscopy in pediatric chronic inflammatory bowel disease (IBD)?
  10. Paroxetine during breast-feeding: infant weight gain and maternal adherence to counsel
  11. Characteristics of the menstrual cycle in 13-year-old Flemish girls and the impact of menstrual symptoms on social life
  12. Proportional assist versus assist control ventilation in premature infants
  13. Thrombus obstructing the right ventricle outflow tract in a neonate with methylenetetrahydrofolate reductase 677TT genotype
  14. Evidence of Bordetella pertussis infection in vaccinated 1-year-old Danish children
  15. BTNL2 gene polymorphisms may be associated with susceptibility to Kawasaki disease and formation of coronary artery lesions in Taiwanese children
  16. Eosinophilic tubulointerstitial nephritis on treatment with isotretinoin
  17. A European Society of Paediatric and Neonatal Intensive Care (ESPNIC) survey of European critical care management of young people
  18. Kawasaki disease after burns
  19. Clinical practice
  20. de Quervain thyroiditis in a young boy following hand–foot–mouth disease
  21. Über Wesen und Entstehung der interstitiellen Pneumonie
  22. What’s new in autism?
  23. Parameters affecting length of stay in a pediatric emergency department: a retrospective observational study
  24. Health-related quality of life and symptom reporting: similarities and differences between children and their parents
  25. Clustering of (auto)immune diseases with early-onset and complicated inflammatory bowel disease
  26. Risk factors for ventilator-associated pneumonia in the neonatal intensive care unit: a meta-analysis of observational studies
  27. Changes in the incidence and etiology of congenital hypothyroidism detected during 30 years of a screening program in central Serbia
  28. Immunisation status of children in Germany: temporal trends and regional differences
  29. Onset of acquired autoimmune hypothyroidism in infancy: a presentation of delayed gross-motor development and rhabdomyolysis
  30. Unusual clinical presentation of primary hypothyroidism in a very young infant caused by autoimmune thyroiditis: case report and update of the literature
  31. Burkholderia gladioli sepsis in newborns
  32. A quality improvement initiative to reduce central line infection in neonates using checklists
  33. Cognition and the risk of eating disorders in Spanish adolescents: the AVENA and AFINOS studies
  34. Transient antiphospholipid antibodies associated with acute infections in children: a report of three cases and a review of the literature
  35. Reply to the correspondence letter by Meyer et al. ‘Complementary and alternative medicine in children’
  36. Changes in the perfusion waveform of the internal cerebral vein and intraventricular hemorrhage in the acute management of extremely low-birth-weight infants
  37. Barth syndrome diagnosed in the subclinical stage of heart failure based on the presence of lipid storage myopathy and isolated noncompaction of the ventricular myocardium
  38. Asthma and atopic dermatitis in children born moderately and late preterm
  39. Acute renal failure after treatment with non-steroidal anti-inflammatory drugs
  40. Clinical application of wireless capsule endoscopy in pediatric patients for suspected small bowel diseases
  41. Relationship among the Manual Ability Classification System (MACS), the Gross Motor Function Classification System (GMFCS), and the functional status (WeeFIM) in children with spastic cerebral palsy
  42. Pain and musculoskeletal pain syndromes related to computer and video game use in adolescents
  43. Purpura fulminans in a newborn infant with galactosemia
  44. Hospital-based surveillance of congenital rubella syndrome in Indonesia
  45. Eponym
  46. Outcome of three cases of untreated maternal glutaric aciduria type I
  47. Early intravenous immunoglobin (two-dose regimen) in the management of severe Rh hemolytic disease of newborn—a prospective randomized controlled trial
  48. Genealogy of breastfeeding
  49. Magicplex TM Sepsis Real-Time test to improve bloodstream infection diagnostics in children
  50. Pressure-induced angioedema associated with endotracheal tube: successful treatment with epinephrine in two cases
  51. CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature—a rare case with a novel mutation
  52. Adolescent medicine in Europe: integration and cooperation are needed
  53. Expert consensus statement ‘Neonatologist-performed Echocardiography (NoPE)’—training and accreditation in UK
  54. Primary care-based surveillance to estimate the burden of rotavirus gastroenteritis among children aged less than 5 years in six European countries
  55. Simultaneous manifestation of fulminant infectious mononucleosis with haemophagocytic syndrome and B-cell lymphoma in X-linked lymphoproliferative disease
  56. Congenital diaphragmatic hernia: current status and review of the literature
  57. Intravenous paracetamol for PDA closure in the preterm: a single-center experience
  58. Volvulus as a complication of chronic intestinal pseudo-obstruction syndrome
  59. Disseminated nontuberculous mycobacterial infection in a child with interferon-γ receptor 1 deficiency
  60. Clinical manifestations and enzymatic activities of mitochondrial respiratory chain complexes in Pearson marrow-pancreas syndrome with 3-methylglutaconic aciduria: a case report and literature review
  61. Medullary sponge kidney associated with distal renal tubular acidosis in a 5-year-old girl
  62. Non-accidental chlorpyrifos poisoning—an unusual cause of profound unconsciousness
  63. Desferrioxamine treatment of iron overload secondary to RH isoimmunization and intrauterine transfusion in a newborn infant
  64. Clinical and diagnostic relevance of Meckel’s diverticulum in children
  65. Recommended clinical evaluation of infants with an apparent life-threatening event. Consensus document of the European Society for the Study and Prevention of Infant Death, 2003
  66. A late presenter and long-term survivor of alveolar capillary dysplasia with misalignment of the pulmonary veins
  67. A case of Mondini dysplasia with recurrent Streptococcus pneumoniae meningitis
  68. Familial X-linked cardiomyopathy (Danon disease): diagnostic confirmation by mutation analysis of the LAMP2 gene
  69. Comparison of the developmental tests Bayley-III and Bayley-II in 7-month-old infants born preterm
  70. A previously healthy 15-year-old girl with high fever and progressive dyspnoea
  71. Lack of CD4 + CD25 + FOXP3 + regulatory T cells is associated with resistance to intravenous immunoglobulin therapy in patients with Kawasaki disease
  72. Tendency toward atopy in Kawasaki disease
  73. Sweat test practice in pediatric pulmonology after introduction of cystic fibrosis newborn screening
  74. The effects of estradiol on mood and behavior in human female adolescents: a systematic review
  75. Your diagnosis? Congenital foot drop
  76. Exocrine pancreatic insufficiency and its consequences on physical development and metabolism in children and adolescents with type 1 diabetes mellitus
  77. Pediatric complicated pneumonia and pneumococcal serotype replacement: trends in hospitalized children pre and post introduction of routine vaccination with Pneumococcal Conjugate Vaccine (PCV7)
  78. Discordant expression of a new WT1 gene mutation in a family with monozygotic twins presenting with congenital nephrotic syndrome
  79. Acute hemorrhagic edema of young children: a concise narrative review
  80. Is procalcitonin a good marker of renal lesion in febrile urinary tract infection?
  81. An 8-year-old boy with autoimmune hepatitis and Candida onychosis as the first symptoms of autoimmune polyglandular syndrome (APS1): identification of a new homozygous mutation in the autoimmune regulator gene (aire)
  82. Aetiological heterogeneity of asymptomatic hyperglycaemia in children and adolescents
Search Result: