237 results match your criteria Pediatrics Reye Syndrome


A Case of Reye Syndrome Caused by Influenza A Virus.

Ochsner J 2018 ;18(4):425-427

The University of Queensland School of Medicine, Ochsner Clinical School, New Orleans, LA.

Background: Reye syndrome is a rare and potentially life-threatening disease characterized by liver failure and hepatic encephalopathy. Multiple possible etiologies have been suggested, but only aspirin (acetylsalicylic acid) has been statistically proven to be a causative factor. We describe a case of Reye syndrome secondary to influenza A virus. Read More

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http://www.ochsnerjournal.org/lookup/doi/10.31486/toj.18.009
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http://dx.doi.org/10.31486/toj.18.0098DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6292460PMC
January 2018
11 Reads

Management and long-term evolution of a patient with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency.

Ital J Pediatr 2017 Jan 19;43(1):12. Epub 2017 Jan 19.

Department of Radiology, Hospital Clínico Universitario, Avda. Blasco Ibáñez 17, 46010, Valencia, Spain.

Background: 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency is a rare inborn error of metabolism characterized by recurrent metabolic crises caused by fasting, intercurrent illness and excessive physical exercise. Non ketotic hypoglycemia is normally the cause of primary symptoms but without an immediate treatment the illness can evolve into a worsening metabolic state resembling the Reye's syndrome that may cause the patient's death. We report a case with some clinical and therapeutic features not previously described. Read More

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http://ijponline.biomedcentral.com/articles/10.1186/s13052-0
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http://dx.doi.org/10.1186/s13052-017-0333-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5347826PMC
January 2017
8 Reads

Aspirin increases mitochondrial fatty acid oxidation.

Biochem Biophys Res Commun 2017 Jan 14;482(2):346-351. Epub 2016 Nov 14.

Department of Pediatrics, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA 15224, United States. Electronic address:

The metabolic effects of salicylates are poorly understood. This study investigated the effects of aspirin on fatty acid oxidation. Aspirin increased mitochondrial long-chain fatty acid oxidation, but inhibited peroxisomal fatty acid oxidation, in two different cell lines. Read More

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http://dx.doi.org/10.1016/j.bbrc.2016.11.066DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5195905PMC
January 2017
30 Reads

"Symptomatic" infection-associated acute encephalopathy in children with underlying neurological disorders.

Brain Dev 2017 Mar 22;39(3):243-247. Epub 2016 Oct 22.

Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan. Electronic address:

Background: Development of infection-associated acute encephalopathy (AE) is precipitated by several factors, including viral agents, age, and genetic polymorphisms. In addition, children with prior underlying neurological disorders can also present with AE.

Method: We reviewed 55 children with AE who were referred to hospitals participating in the Status Epilepticus Study Group from 1988 to 2013. Read More

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http://linkinghub.elsevier.com/retrieve/pii/S038776041630168
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http://dx.doi.org/10.1016/j.braindev.2016.09.014DOI Listing
March 2017
10 Reads

Acetylsalicylic acid as a potential pediatric health hazard: legislative aspects concerning accidental intoxications in the European Union.

J Occup Med Toxicol 2016 13;11:32. Epub 2016 Jul 13.

Institute of Occupational Medicine, Social Medicine and Environmental Medicine, Departments of Female Health and Preventive Medicine, Goethe University, Frankfurt am Main, Theodor-Stern-Kai 7, Frankfurt, 60590 Germany.

Acetylsalicylic acid is a frequently used medication worldwide. It is not used in pediatrics due its association with Reye syndrome. However, in case of pediatric intoxication, children are more fragile to salicylate poisoning because of their reduced ability of buffer the acid stress. Read More

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http://dx.doi.org/10.1186/s12995-016-0118-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4944482PMC
July 2016
6 Reads

An 8-year-old girl with abdominal pain and mental status changes.

Pediatr Emerg Care 2015 Jun;31(6):459-62

Section of Metabolic Disease The Children's Hospital of Philadelphia and Department of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA.

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http://dx.doi.org/10.1097/PEC.0000000000000504DOI Listing
June 2015
3 Reads

Impact of fatty acid oxidation disorders in child neurology: from Reye syndrome to Pandora's box.

Authors:
Ingrid Tein

Dev Med Child Neurol 2015 Apr;57(4):304-6

Division of Neurology, Department of Pediatrics, Laboratory Medicine and Pathobiology, The Hospital for Sick Children, The University of Toronto, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1111/dmcn.12717DOI Listing
April 2015
11 Reads

Update on pediatric neurocritical care.

Authors:
Robert C Tasker

Paediatr Anaesth 2014 Jul 2;24(7):717-23. Epub 2014 Apr 2.

Departments of Neurology and Anesthesiology, Perioperative and Pain Medicine, Division of Critical Care Medicine, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.

Paralytic poliomyelitis, Reye syndrome, Hemophilus Influenzae type B epiglottitis, bacterial meningitis, and meningococcal septic shock are catastrophic illnesses that in the last 60 years have shaped the development of pediatric intensive care. Neurocritical care has been at the forefront of our thinking and, more latterly, as a specialty we have had the technology and means to develop this focus, educate the next generation and show that outcomes can be improved-first in adult critical care and now the task is to translate these benefits to critically ill children. In our future we will need to advance interventions in patient care with clinical trials. Read More

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http://dx.doi.org/10.1111/pan.12398DOI Listing
July 2014
4 Reads

Pediatric parechovirus infections.

J Clin Virol 2014 Jun 13;60(2):84-9. Epub 2014 Mar 13.

Department of Medical Microbiology, Division of Clinical Virology, The University of Groningen, University Medical Center Groningen, The Netherlands.

Human parechoviruses (HPeVs) are members of the large and growing family of Picornaviridae. Although 16 types have been described on the basis of the phylogenetic analyses of the VP1 encoding region, the majority of published reports relate to the HPeV types 1-8. In pediatrics, HPeV1, HPeV2 and HPeV4-8 mainly cause mild gastrointestinal or respiratory illness; only occasionally more serious diseases have been reported, including myocarditis, encephalitis, pneumonia, meningitis, flaccid paralysis, Reye syndrome and fatal neonatal infection. Read More

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http://dx.doi.org/10.1016/j.jcv.2014.03.003DOI Listing
June 2014
12 Reads

Bismuth salicylate for diarrhea in children.

Authors:
Ran D Goldman

Can Fam Physician 2013 Aug;59(8):843-4

BC Children's Hospital, Department of Pediatrics, Room K4-226, Ambulatory Care Bldg, 4480 Oak St, Vancouver, BC V6H 3V4, Canada.

Question: Recently, I had a visit from a 5-year-old patient who had been given bismuth subsalicylate for a diarrheal illness by a local family physician during a trip to South America. Is this a practice we should encourage?

Answer: Research from developing countries has found the use of bismuth subsalicylate to be effective in shortening the duration of diarrheal illness. Despite these findings, its limited effectiveness and concerns about it potentially causing Reye syndrome, compliance, and cost are the key reasons it is not routinely recommended for children. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3743694PMC
August 2013
3 Reads

Cerebellar mutism caused by primary varicella infection in an immunocompetent child.

J Child Neurol 2014 Jun 26;29(6):830-2. Epub 2013 Feb 26.

Division of Child Neurology, Baskent University Faculty of Medicine, Adana, Turkey.

Varicella (chickenpox) is a common childhood infection caused by the varicella-zoster virus, which is often self-limiting and usually benign. Although uncommon, neurologic complications of varicella have been documented that include postinfectious cerebellar ataxia, meningoencephalitis, Reye syndrome, myelitis, optic neuritis, stroke, Guillain-Barré syndrome, seventh cranial nerve palsy, and Ramsay-Hunt syndrome. In this case study, the authors describe a 7-year-old girl who presented with varicella skin rash with unsteady gait and anarthria on day 2, and her condition was attributed to cerebellar mutism. Read More

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http://dx.doi.org/10.1177/0883073813477202DOI Listing
June 2014
5 Reads

Sudden unexpected infant death (SUDI) in a newborn due to medium chain acyl CoA dehydrogenase (MCAD) deficiency with an unusual severe genotype.

Ital J Pediatr 2012 Oct 24;38:59. Epub 2012 Oct 24.

Department of Pediatrics, University of Torino, Regina Margherita Children's Hospital, Torino, Italy.

Medium chain acyl CoA dehydrogenase deficiency (MCAD) is the most common inborn error of fatty acid oxidation. This condition may lead to cellular energy shortage and cause severe clinical events such as hypoketotic hypoglycemia, Reye syndrome and sudden death. MCAD deficiency usually presents around three to six months of life, following catabolic stress as intercurrent infections or prolonged fasting, whilst neonatal-onset of the disease is quite rare. Read More

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http://ijponline.biomedcentral.com/articles/10.1186/1824-728
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http://dx.doi.org/10.1186/1824-7288-38-59DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3502270PMC
October 2012
10 Reads

Antiplatelet therapy in children: why so different from adults'?

Curr Pharm Des 2012 ;18(21):3019-33

Department of Cardiovascular and Neurological Sciences, University of Cagliari, Italy.

Antiplatelet agents are administered in the treatment of a large number of adult diseases: coronary heart disease, ischemic stroke, peripheral arterial disease, arrhythmias with their thromboembolic complications, primary and secondary prevention. In childhood however, the situation is substantially different. The lack of large interventional trials on the use of antiplatelet drugs in children, has led to greater uncertainty, and a less extensive use of these drugs, limited to fewer indications. Read More

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December 2012
3 Reads

Transaminase in rotavirus gastroenteritis.

Pediatr Int 2012 Feb;54(1):86-8

Department of Pediatrics, Tokyo Medical University, Tokyo, Japan.

Background: Children infected with rotavirus often show increased levels of transaminase, and symptoms are characterized by white stool, similar to biliary atresia. Rotavirus infections are also sporadically accompanied with convulsions, encephalopathy and Reye syndrome. The aim of the present study was therefore to investigate transaminase and interleukin (IL)-6 levels in rotavirus infection, in order to better understand their clinical significance. Read More

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http://dx.doi.org/10.1111/j.1442-200X.2011.03532.xDOI Listing
February 2012
6 Reads

[Reye syndrome and Reye-like syndrome].

Authors:
Akihiko Kimura

Nihon Rinsho 2011 Mar;69(3):455-9

Department of Pediatrics Child Health, Kurume University School of Medicine.

Reye syndrome (RS) is an acute metabolic encephalopathy and hepatopathy affecting children and adolescents. Outbreaks of RS were common in United States until the early 1980s. However, after the abolition of salicylate (aspirin) therapy for infectious diseases such as influenza or varicella in patients under 18 years of age the incidence decreased. Read More

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March 2011
3 Reads

Carnitine palmitoyltransferase 2 deficiency: the time-course of blood and urinary acylcarnitine levels during initial L-carnitine supplementation.

Tohoku J Exp Med 2010 Jul;221(3):191-5

Department of Pediatrics, Gifu University Graduate School of Medicine.

Carnitine palmitoyltransferase 2 (CPT2) deficiency is one of the most common mitochondrial beta-oxidation defects. A female patient with an infantile form of CPT2 deficiency first presented as having a Reye-like syndrome with hypoglycemic convulsions. Oral L-carnitine supplementation was administered since serum free carnitine level was very low (less than 10 micromol/L), indicating secondary carnitine deficiency. Read More

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July 2010
5 Reads

Lack of genotype-phenotype correlations and outcome in MCAD deficiency diagnosed by newborn screening in New York State.

Mol Genet Metab 2010 Mar 1;99(3):263-8. Epub 2009 Nov 1.

Department of Pediatrics, University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, Box 777, Rochester, NY 14642, USA.

Introduction: Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is one of the most common inborn errors of metabolism. Affected patients have impaired ability to break down medium chain fatty acids during fasting, and typically present in the early years of life with hypoketotic hypoglycemia, Reye syndrome-like symptoms, brain damage or death. The development of newborn screening (NBS) for MCAD deficiency has greatly improved outcome, but some patients still appear at risk for severe complications. Read More

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http://dx.doi.org/10.1016/j.ymgme.2009.10.188DOI Listing
March 2010
9 Reads

Acute encephalopathy of Bacillus cereus mimicking Reye syndrome.

Brain Dev 2010 Sep 30;32(8):688-90. Epub 2009 Sep 30.

Department of Pediatrics, Yokohama City University Medical Center, Minami-ku, Yokohama, Japan.

We present an 11-year-old boy diagnosed as having acute encephalopathy and liver failure with the underlying condition of a metabolic dysfunction. He developed convulsions and severe consciousness disturbance following gastroenteritis after the ingestion of some fried rice. He showed excessive elevation of transaminases, non-ketotic hypoglycemia and hyperammonemia, which were presumed to reflect a metabolic dysfunction of the mitochondrial beta-oxidation, and he exhibited severe brain edema throughout the 5th hospital day. Read More

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http://dx.doi.org/10.1016/j.braindev.2009.09.004DOI Listing
September 2010
2 Reads

Kids versus trees: Reye's syndrome and spraying for spruce budworm in New Brunswick.

Authors:
Michael S Kramer

J Clin Epidemiol 2009 Jun 5;62(6):578-81. Epub 2009 Apr 5.

Department of Pediatrics, McGill University Faculty of Medicine, Montreal, Quebec, Canada.

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http://dx.doi.org/10.1016/j.jclinepi.2009.01.002DOI Listing
June 2009
4 Reads

Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism.

Biochim Biophys Acta 2008 Oct 3;1777(10):1276-82. Epub 2008 Jun 3.

Department of General Pediatrics, Division of Inborn Metabolic Diseases, University Children's Hospital, Heidelberg, Germany.

Accumulation of organic acids as well as their CoA and carnitine esters in tissues and body fluids is a common finding in organic acidurias, beta-oxidation defects, Reye syndrome, and Jamaican vomiting sickness. Pathomechanistic approaches for these disorders have been often focused on the effect of accumulating organic acids on mitochondrial energy metabolism, whereas little is known about the pathophysiologic role of short- and medium-chain acyl-CoAs and acylcarnitines. Therefore, we investigated the impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on central components of mitochondrial energy metabolism, namely alpha-ketoglutarate dehydrogenase complex, pyruvate dehydrogenase complex, and single enzyme complexes I-V of respiratory chain. Read More

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http://dx.doi.org/10.1016/j.bbabio.2008.05.447DOI Listing
October 2008
9 Reads

Wolcott-Rallison syndrome with 3-hydroxydicarboxylic aciduria and lethal outcome.

J Inherit Metab Dis 2008 Dec 20;31 Suppl 2:S293-7. Epub 2008 May 20.

Section for Pediatrics, Department of Clinical Medicine, University of Bergen, Bergen, Norway.

Wolcott-Rallison syndrome (WRS) (OMIM 226980) is a rare, autosomal recessive disorder with infancy-onset diabetes mellitus, multiple epiphyseal dysplasia, osteopenia, mental retardation or developmental delay, and hepatic and renal dysfunction as main clinical findings. Patients with WRS have mutations in the EIF2AK3 gene, which encodes the pancreatic eukaryotic translation initiation factor 2-alpha kinase 3. We report a female patient who developed insulin-requiring diabetes at 2. Read More

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http://dx.doi.org/10.1007/s10545-008-0866-1DOI Listing
December 2008
2 Reads

Glycosphingolipid expression in cerebrospinal fluid of infants with neurological abnormalities: report of three cases.

Coll Antropol 2008 Jan;32 Suppl 1:189-93

Department of Pediatrics, University Hospital Split, Split, Croatia.

The aim of this study was to analyse glycosphingolipid expression in cerebrospinal fluid (CSF) from one idiopathic West syndrome (IWS) infant, one with Reye like syndrome, and one with congenital hydrocephalus, in comparison to control group (n=7) using highly sensitive thin-layer chromatography-immunostaining methods. Gangliotetraose-series gangliosides (acidic glycosphingolipids) were not detected in CSF of infant with idiopathic West syndrome and infant with congenital hydrocephalus. CSF of infant with IWS showed traces of neolacto-tetraose ganglioside fractions, which were absent in all other CSF examined. Read More

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January 2008
8 Reads

Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA carboxylase deficiency.

J Hum Genet 2007 30;52(12):1040-3. Epub 2007 Oct 30.

Department of Pediatrics, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan.

Isolated 3-methylcrotonyl-CoA carboxylase (MCC) deficiency appears to be the most frequent organic aciduria detected in tandem mass spectrometry (MS/MS) screening programs in the United States, Australia, and Europe. A pilot study of newborn screening using MS/MS has recently been commenced in Japan. Our group detected two asymptomatic MCC deficiency patients by the pilot screening and collected data on another three MCC deficiency patients to study the molecular bases of the MCC deficiency in Japan. Read More

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http://dx.doi.org/10.1007/s10038-007-0211-9DOI Listing
February 2008
12 Reads

Acute encephalopathy associated with influenza and other viral infections.

Acta Neurol Scand Suppl 2007 ;186:45-56

Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.

Acute encephalopathy is the most serious complication of pediatric viral infections, such as influenza and exanthem subitum. It occurs worldwide, but is most prevalent in East Asia, and every year several hundreds of Japanese children are affected by influenza-associated encephalopathy. Mortality has recently declined, but is still high. Read More

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October 2007
2 Reads

Acute encephalopathy associated with influenza and other viral infections.

Acta Neurol Scand 2007 Apr;115(4 Suppl):45-56

Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.

Acute encephalopathy is the most serious complication of pediatric viral infections, such as influenza and exanthem subitum. It occurs worldwide, but is most prevalent in East Asia, and every year several hundreds of Japanese children are affected by influenza-associated encephalopathy. Mortality has recently declined, but is still high. Read More

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http://dx.doi.org/10.1111/j.1600-0404.2007.00809.xDOI Listing
April 2007
3 Reads

Identification of novel mutations of the HADHA and HADHB genes in patients with mitochondrial trifunctional protein deficiency.

Int J Mol Med 2007 Jan;19(1):81-7

Department of Pediatrics, Chungnam National University Hospital, College of Medicine, Chungnam National University, Daejeon 301-721, Korea.

Patients with long-chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) deficiency manifest hypoketotic hypoglycemia, hepatomegaly, hypotonia, lactic acidemia, acute renal failure, cardiomyopathy, and sudden death. We describe four novel mutations of the alpha- and beta-subunits of the mitochondrial trifunctional protein in four patients from three unrelated families. Their plasma acylcarnitine profiles suggested the presence of LCHAD deficiency by demonstrating highly elevated 3-hydroxyacyl carnitines by tandem mass spectrometry (MS/MS). Read More

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January 2007
3 Reads

Dengue virus infection: a major cause of acute hepatic failure in Thai children.

Ann Trop Paediatr 2006 Mar;26(1):17-23

Center of Excellence in Viral Hepatitis Research, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and Hospital, Bangkok, Thailand.

Background: Acute hepatic failure (AHF) can be caused by a variety of viruses, drugs, toxins and metabolic disorders.

Aims: A prospective study was conducted to determine the aetiology and outcome of AHF in Thai children aged 1-15 years.

Methods: All serum samples were tested for anti-HAV IgM, HBsAg, anti-HBc IgM, anti-HCV, anti-HEV IgM and anti-dengue IgG and IgM. Read More

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http://dx.doi.org/10.1179/146532806X90565DOI Listing
March 2006
4 Reads

Reye's syndrome.

Kathmandu Univ Med J (KUMJ) 2003 Apr-Jun;1(2):138-40

Dept. of Paediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal.

Five and half years male child with one day history of pain abdomen and vomiting who was on aspirin for suspected rheumatoid arthritis presented initially with acute gastritis. Next day, however he developed the signs of encephalopathy with altered liver function. Read More

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January 2006
5 Reads

Mitochondrial tRNA gene mutations in patients having mitochondrial disease with lactic acidosis.

Mitochondrion 2006 Feb 5;6(1):29-36. Epub 2005 Dec 5.

Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-Machi, Kurume 830-0011, Japan.

Lactic acidosis has been associated with a variety of clinical conditions and can be due to mutation in nuclear or mitochondrial genes. We performed mutations screening of all mitochondrial tRNA genes in 44 patients who referred as hyperlactic acidosis. Patients showed heterogeneous phenotypes including Leigh disease in four, MELAS in six, unclassified mitochondrial myopathy in 10, cardiomyopathy in five, MERRF in one, pure lactic acidosis in six, and others in 12 including facio-scaplo-femoral muscular dystrophy (FSFD), familial cerebellar ataxia, recurrent Reye syndrome, cerebral palsy with mental retardation. Read More

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http://dx.doi.org/10.1016/j.mito.2005.10.003DOI Listing
February 2006
2 Reads

A case of Reye syndrome with rotavirus infection accompanied with high cytokines.

J Infect 2006 Apr 14;52(4):e124-8. Epub 2005 Oct 14.

Department of Paediatrics, Tokyo Medical University, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo 160-0023, Japan.

We report on a 23-month-old boy with a rare complication of rotavirus gastroenteritis. He was diagnosed as acute encephalopathy with DIC accompanied with high levels of cytokines. The liver pathology also revealed mild infiltration and fatty changes. Read More

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http://dx.doi.org/10.1016/j.jinf.2005.07.028DOI Listing
April 2006
2 Reads

Reye's syndrome developing in an infant on treatment of Kawasaki syndrome.

J Paediatr Child Health 2005 May-Jun;41(5-6):303-4

Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.

Aspirin is commonly used as an anti-inflammatory therapy for Kawasaki syndrome. Early initiation with high dose aspirin (80 to > 100 mg/kg per day), followed by low-dose therapy at the afebrile stage, has been often used to reduce morbidity and mortality in coronary complications. We report a 10-month-old infant who was diagnosed with Kawasaki syndrome. Read More

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http://dx.doi.org/10.1111/j.1440-1754.2005.00617.xDOI Listing
October 2005
3 Reads

Influenza-associated acute encephalopathy in Japanese children in 1994-2002.

Virus Res 2004 Jul;103(1-2):75-8

Department of Pediatrics, Sapporo City General Hospital, N 11 W 13, Chuo-ku, Sapporo 060-8604, Japan.

We addressed the incidence of influenza-associated acute encephalopathy, which is distinct from Reye syndrome, in children in Japan. Eighty-nine children with a mean age of 3.8 years were reported to have developed this disease during eight influenza seasons (December 1994-April 2002) in Hokkaido, Japan. Read More

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http://dx.doi.org/10.1016/j.virusres.2004.02.016DOI Listing
July 2004
2 Reads

Serum free carnitine in medium chain acyl-CoA dehydrogenase deficiency.

Bratisl Lek Listy 2003 ;104(12):405-7

1st Department of Pediatrics, Comenius University Children's Hospital, Bratislava, Slovakia.

Medium chain acyl-CoA dehydrogenase (MCAD) deficiency is the most common disorder of fatty acid beta-oxidation and presents acutely with hypoglycemia, or a Reye-like illness with low free carnitine, often provoked by an infection or an excessive period of fasting. After acute attack these children are for the most time asymptomatic and may have normal plasma free carnitine concentrations. We observed a regularity in time course of serum free carnitine concentration during two attacks of Reye-like illness in patient with MCAD deficiency. Read More

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April 2004
3 Reads

Reassessing Reye syndrome.

Arch Pediatr Adolesc Med 2003 Dec;157(12):1241-2

Department of Pediatrics, Mattel Children Hospital, 10833 Le Conte Ave, Box 951752, Los Angeles, CA 90095-1752, USA.

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http://dx.doi.org/10.1001/archpedi.157.12.1241DOI Listing
December 2003
3 Reads

Silent and symptomatic primary carnitine deficiency within the same family due to identical mutations in the organic cation/carnitine transporter OCTN2.

J Inherit Metab Dis 2003 ;26(6):613-5

Clinic of General Pediatrics, University Children's Hospital, Düsseldorf, Germany.

A family of Turkish origin with primary systemic carnitine deficiency in the father and his two sons is described. In all three individuals, the same homozygous mutation in the OCTN2 gene (R471H) was present and carnitine uptake in fibroblasts was deficient. Whereas one boy became symptomatic with a Reye-syndrome-like picture of hepatopathy and encephalopathy in infancy, the other affected family members remained asymptomatic up to their current ages of 28 and 5 years, respectively. Read More

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June 2004
2 Reads

Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to beta-subunit mutations.

Hum Mutat 2003 Jun;21(6):598-607

Department of Pediatrics and Vanderbilt Children's Hospital, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA.

The mitochondrial trifunctional protein (TFP) is a multienzyme complex of the fatty acid beta-oxidation cycle. It is composed of four alpha-subunits (HADHA) harboring long-chain enoyl-CoA hydratase and long-chain L-3-hydroxyacyl-CoA dehydrogenase (LCHAD) and four beta-subunits (HADHB) harboring long-chain 3-ketoacyl-CoA thiolase (LKAT). Mutations in either subunit can result in TFP deficiency with reduced activity of all three TFP enzymes. Read More

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http://doi.wiley.com/10.1002/humu.10211
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http://dx.doi.org/10.1002/humu.10211DOI Listing
June 2003
5 Reads

Primary systemic carnitine deficiency presenting as recurrent Reye-like syndrome and dilated cardiomyopathy.

Authors:
Jia-Woei Hou

Chang Gung Med J 2002 Dec;25(12):832-7

Division of Medical Genetics, Department of Pediatrics, Chang Gung Children's Hospital, Taoyuan, Taiwan, ROC.

Carnitine deficiency syndrome is a rare and potentially fatal but treatable metabolic disorder. I present a 6-year-old girl with primary systemic carnitine deficiency (SCD) proved by very low plasma carnitine level. Her major clinical features included neonatal metabolic acidosis, epilepsy, recurrent infections, acute encephalopathy, and dilated cardiomyopathy with heart failure before 4 years of age. Read More

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December 2002
4 Reads

Reye's syndrome: down but not out.

South Med J 2003 Jan;96(1):43-5

Department of Pediatrics, University of Arkansas for Medical Sciences and Arkansas Children's Hospital, Little Rock, AR 72202-3591, USA.

Reye's syndrome presents as acute central nervous system and liver dysfunction in children. Its incidence has seen a sharp decline in parallel with the decline in the use of aspirin in the pediatric age group. This report describes a patient with Reye's syndrome and serves as a reminder for health professionals to continue to discourage the use of aspirin for the treatment of viral infections. Read More

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http://dx.doi.org/10.1097/01.SMJ.0000047764.97329.1CDOI Listing
January 2003
11 Reads

A survey of Japanese patients with mitochondrial fatty acid beta-oxidation and related disorders as detected from 1985 to 2000.

Brain Dev 2002 Oct;24(7):675-80

Department of Pediatrics, Shimane Medical University, 89-1 En-ya-cho, Izumo, Shimane 693-8501, Japan.

A clinical survey of Japanese patients with mitochondrial fatty acid beta-oxidation and related disorders (FAODs) was performed with questionnaires sent to 187 institutions, where inborn errors of metabolism could be managed in Japan, including a search of related literature published between 1985 and 2000. Sixty-four patients with ten types of FAODs were found. Carnitine palmitoyltransferase 2 deficiency and glutaric aciduria type 2 were most common (17 and 14 patients, respectively). Read More

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October 2002
4 Reads

Influenza-associated encephalopathy in Japan.

Authors:
Norio Sugaya

Semin Pediatr Infect Dis 2002 Apr;13(2):79-84

Department of Pediatrics, Nippon Kokan Hospital, 1-2-1 Kokandori, Kawasaki-ku, Kawasaki, Kanagawa 210-0852, Japan.

Although the clinical entity of influenza-associated encephalopathy (influenza encephalopathy) has not gained universal recognition, it has been reported frequently as a complication of influenza in Japanese children. The influenza type A (H3N2) virus was detected in most cases. Most of the patients have been young children. Read More

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April 2002
4 Reads

Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency.

J Hum Genet 2002 ;47(7):342-7

Department of Pediatrics, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan.

Carnitine palmitoyltransferase I (CPT I) is one of the carnitine cycle enzymes that plays a role in the transportation of long-fatty acids into the mitochondria for beta-oxidation. Hepatic carnitine palmitoyltransferase I (CPT IA) is one of the isozymes of CPT I, and its deficiency results in an autosomal recessive mitochondrial fatty acid oxidation disorder. To date, 19 patients with CPT IA deficiency and 9 CPT IA mutations have been reported. Read More

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http://link.springer.com/content/pdf/10.1007/s100380200047.p
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June 2003
3 Reads

[Mitochondrial diseases due to drug toxicity].

Nihon Rinsho 2002 Apr;60 Suppl 4:473-7

Department of Pediatrics, Dokkyo University School of Medicine.

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April 2002
3 Reads

Important role of prodromal viral infections responsible for inhibition of xenobiotic metabolizing enzymes in the pathomechanism of idiopathic Reye's syndrome, Stevens-Johnson syndrome, autoimmune hepatitis, and hepatotoxicity of the therapeutic doses of acetaminophen used in genetically predisposed persons.

Authors:
Joseph Prandota

Am J Ther 2002 Mar-Apr;9(2):149-56

Department of Pediatrics, Korczak Memorial Children's Hospital, Wroclaw, Poland.

Upper respiratory tract febrile illnesses caused by various viruses, mycoplasma, chlamydia infections, and/or inflammatory diseases are usually observed a few days to a few (several) weeks before the onset of Reye's syndrome, Stevens-Johnson syndrome, autoimmune hepatitis (hepatotropic virus infections), or hepatotoxicity associated with therapeutic administration of acetaminophen in persons with varying degrees of deficits of important enzymatic activity. Activation of systemic host defense mechanisms by inflammatory component(s) results in depression of various induced and constitutive isoforms of cytochrome P-450 mixed-function oxidase system superfamily enzymes in the liver and most other tissues of the body. Because several cytochrome P-450 enzymes activities important for biotransformation of many endogenous and egzogenous substances show considerable variability between individuals, in some genetically predisposed persons, even the administration of therapeutic doses of a drug may result in serious clinical mishaps, if an important concomitant risk factor (eg, acute viral infection) is involved. Read More

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June 2002
1 Read

Carnitine palmityl transferase I deficiency.

Saudi Med J 2001 Nov;22(11):1025-9

Department of Pediatrics, Riyadh Armed Forces Hospital, Kingdom of Saudi Arabia.

Carnitine palmityl transferase I is the key enzyme in the carnitine dependent transport of long chain fatty acids across the mitochondrial inner membrane and its deficiency results in a decrease rate of fatty acids beta-oxidation with decreased energy production. We reported a family of 3 affected siblings who are the product of a first degree cousin marriage. The first 2 presented with typical Reye-like syndrome with unconsciousness, hepatomegaly, hypoglycemia, hyperammonemia and very high liver enzymes. Read More

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November 2001
3 Reads

Severe complications of varicella in previously healthy children in Germany: a 1-year survey.

Pediatrics 2001 Nov;108(5):E79

Pediatric Infectious Diseases, Zentrum für Präventive Pädiatrie, Johannes Gutenberg University Mainz, Mainz, Germany.

Objective: Varicella is a common infectious disease, usually benign and self-limited, and complications are believed to be rare. The purpose of this study was to describe the epidemiology of severe varicella complications in immunologically healthy children in Germany.

Methods: Information on any admission of children with a severe complication associated with chickenpox was solicited throughout 1997 from all 485 pediatric hospitals in Germany using an established surveillance system. Read More

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http://pediatrics.aappublications.org/content/pediatrics/108
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November 2001
13 Reads

[Short-chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) deficiency].

Ryoikibetsu Shokogun Shirizu 2001 (36):74-6

Department of Pediatrics, Shimane Medical University.

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January 2002
3 Reads

[Medium-chain acyl-CoA dehydrogenase deficiency].

Ryoikibetsu Shokogun Shirizu 2001 (36):68-70

Department of Pediatrics, Shimane Medical University.

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January 2002
2 Reads

[Very-long-chain acyl-CoA dehydrogenase deficiency].

Ryoikibetsu Shokogun Shirizu 2001 (36):65-7

Department of Pediatrics, Shimane Medical University.

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January 2002
3 Reads

[Mitochondrial beta-oxidation defects: overview].

Authors:
S Yamaguchi

Ryoikibetsu Shokogun Shirizu 2001 (36):60-4

Department of Pediatrics, Shimane Medical University.

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January 2002
3 Reads

Incidence of Reye's syndrome in France: a hospital-based survey.

J Clin Epidemiol 2001 Aug;54(8):857-62

Department of Clinical Pharmacology, Hôpital Bretonneau-University François Rabelais, Tours, France.

At the time of the study no information was available in France about the incidence of Reye's Syndrome (RS) and no warnings about RS and aspirin. The objective was to evaluate the incidence of RS in France by a hospital-based study. For a period of 1 year from November 1995 to November 1996, all French paediatric departments were required to report any child under 15 years with unexplained noninflammatory encephalopathy (i. Read More

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August 2001
2 Reads