Grade I Reye syndrome and profound dicarboxylic aciduria

1988 ◽  
Vol 113 (1) ◽  
pp. 161-162
Author(s):  
Akihiko Kimura ◽  
Ichiro Yoshida ◽  
Atsushi Nishiyori ◽  
Takuji Fujisawa ◽  
Ken Yuge ◽  
...  
Keyword(s):  
Reye Syndrome ◽  
Dicarboxylic Aciduria

Sudden Child Death and `Healthy' Affected Family Members With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

PEDIATRICS ◽  
1986 ◽  
Vol 78 (6) ◽  
pp. 1052-1057
Author(s):  
Marinus Duran ◽  
Marchinus Hofkamp ◽  
William J. Rhead ◽  
Jean-Marie Saudubray ◽  
Sybe K. Wadman
Keyword(s):  
Fatty Acids ◽  
Coenzyme A ◽  
Family Members ◽  
Reye Syndrome ◽  
Dicarboxylic Aciduria ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Chain Fatty Acids

A family is described in which the father and three (and probably all four) of his children had a decreased capacity for the oxidation of medium-chain fatty acids. One of the children suddenly died at the age of 16 months following an episode of a rapidly deteriorating Reye syndrome-like illness with hypoketotic hypoglycemia and dicarboxylic aciduria, but without any previous alarming symptoms. The eldest sibling had died at the age of 19 months under similar conditions. The other family members had always been healthy. On fasting, all affected family members accumulated in their plasma the medium-chain fatty acids octanoic, decanoic, and cis-4-decenoic acids. Their urinary organic acid excretion profile could be characterized as "dicarboxylic aciduria." A deficiency of medium-chain acyl-coenzyme A dehydrogenase was demonstrated in a postmortem liver sample of the index patient. Cultured fibroblasts from the father and the two healthy children had a decreased rate of [14C]octanoate oxidation. It is suggested that a deficiency of medium-chain acyl-coenzyme A dehydrogenase may lead to a life-threatening illness when other complicating factors such as diarrhea and vomiting result in an abnormal depletion of the body's glycogen stores. Careful monitoring of at-risk patients during a minor illness is necessary.

scholarly journals Development of encephalopathic features similar to Reye syndrome in rabbits.

1984 ◽  
Vol 81 (19) ◽  
pp. 6169-6173 ◽  
Author(s):  
E. S. Kang ◽  
G. Olson ◽  
J. T. Jabbour ◽  
S. S. Solomon ◽  
M. Heimberg ◽  
...  
Keyword(s):  
Reye Syndrome

Reye syndrome

2015 ◽  
pp. 524-528
Author(s):  
Debra L. Weiner
Keyword(s):  
Reye Syndrome

Reye syndrome or side-effects of anti-emetics?

1991 ◽  
Vol 150 (7) ◽  
pp. 456-459 ◽  
Author(s):  
M. Casteels-Van Daele
Keyword(s):  
Side Effects ◽  
Reye Syndrome

scholarly journals Reye Syndrome and Aspirin

1987 ◽  
Vol 1 (6) ◽  
pp. 43
Author(s):  
J Gordon Millichap
Keyword(s):  
Reye Syndrome

scholarly journals Reye Syndrome – A Case Report with Review of Literature .

2008 ◽  
Vol 47 (169) ◽  
Author(s):  
Arnab Ghosh ◽  
S Pradhan ◽  
R Swami ◽  
S R KC ◽  
O P Talwar
Keyword(s):  
Case Report ◽  
Liver Biopsy ◽  
Needle Biopsy ◽  
Differential Diagnoses ◽  
Low Grade ◽  
Loss Of Consciousness ◽  
Reye Syndrome ◽  
Supportive Treatment ◽  
Review Of Literature ◽  
Drug Ingestion

Here we describe a case of Reye syndrome diagnosed at postmortem liver biopsy of a three-year oldgirl who presented with vomiting, low grade fever for three days and loss of consciousness for 18hours. Clinically, the differential diagnoses were meningitis, encephalitis and septicemia. No historyof past illness or any drug ingestion including aspirin were present. Laboratory investigationsindicated a diagnosis of Reye syndrome. The child was given supportive treatment but died aftertwo days of admission and postmortem needle-biopsy of the liver showed microvesicular steatosisconsistent with Reye syndrome.Key words: Microvesicular steatosis, mitochondrial hepatopathy, Reye syndrome, Reye-like syndrome

Dicarboxylic aciduria: deficient [1-14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts

Science ◽  
1983 ◽  
Vol 221 (4605) ◽  
pp. 73-75 ◽  
Author(s):  
W. Rhead ◽  
B. Amendt ◽  
K. Fritchman ◽  
S. Felts
Keyword(s):  
Dicarboxylic Aciduria ◽  
Medium Chain ◽  
Chain Acyl

Kawasaki Syndrome, Reye Syndrome, and Aspirin

PEDIATRICS ◽  
1986 ◽  
Vol 77 (4) ◽  
pp. 617-617
Author(s):  
MARTHA BUSHORE
Keyword(s):  
Airway Resistance ◽  
Platelet Adhesion ◽  
Therapeutic Benefit ◽  
Kawasaki Syndrome ◽  
Reye Syndrome ◽  
Optimal Outcome ◽  
Appropriate Use ◽  
Excellent Review ◽  
Pediatric Diseases

In Reply.— Because the incidence of each of these serious pediatric diseases is low, but the need for prompt recognition and skillful management is essential to optimal outcome, Drs Takahashi and Mason's excellent review and knowledgeable guidance are invaluable to the practicing pediatrician who rarely manages either of these disorders. Our concern that aspirin may interfere with platelet adhesion (when not therapeutically desirable), may increase airway resistance, and may be associated with Reye syndrome should not lead us to discontinue recommending appropriate use of this excellent drug when therapeutic benefit outweighs risk.

Hepatic Monoamine Oxidase Deficiency in Reye Syndrome

PEDIATRICS ◽  
1980 ◽  
Vol 65 (3) ◽  
pp. 673-673
Author(s):  
Robert A. Mitchell ◽  
Edgardo L. Arcinue
Keyword(s):  
Monoamine Oxidase ◽  
Oxidase Activity ◽  
Reye Syndrome ◽  
Disruptive Effect ◽  
Monoamine Oxidase Activity ◽  
Reye's Syndrome ◽  
Oxidase Deficiency ◽  
Mitochondrial Injury ◽  
Subsequent Decrease

Faraj et al1 recently reported a positive correlation between plasma tyramine levels and the number of days in coma, for patients with biopsy-proven Reye's syndrome. They suggested that the mitochondrial injury might have a disruptive effect upon hepatic monoamine oxidase, with a subsequent decrease in clearance of tyramine. We have reached a similar conclusion on the possible role of tyramine in the development of the encephalopathy, based on the finding that the hepatic monoamine oxidase activity was reduced about 60% in stage V Reye's syndrome patients.

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