+ Resolve Article
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter

+ Translate
+ Subscribe to Site Feed
GeoScience Most Shared ContentMost Shared Content

A new patient with dicarboxylic aciduria suggestive of medium-chain Acyl-CoA dehydrogenase deficiency presenting as Reye's syndrome

, : A new patient with dicarboxylic aciduria suggestive of medium-chain Acyl-CoA dehydrogenase deficiency presenting as Reye's syndrome. Journal of Inherited Metabolic Disease 7(2): 62-64

A new patient with medium-chain dicarboxylic aciduria and suberyl glycinuria during an attack of acute illness is reported. When, inadvertently he was given medium-chain triglycerides for 2 days, the excretion of abnormal metabolites of medium-chain fatty acids increased and hepatomegaly became more pronounced. During remission a low excretion of the metabolites were observed. After 16 h of fasting hypoglycaemia was accompanied by an increase of urinary dicarboxylic acids and psi-hydroxyacids similar to that found on admission. Interestingly this urinary organic acid pattern persisted 8 h after intravenous administration of glucose. In a blood sample obtained after 16 h of fasting there was hypoketonaemia and increased levels of total free fatty acids, octanoic, decanoic and cis-4-decenoic acids. These biochemical data suggest the existence of a deficiency at the level of medium-chain acyl-CoA dehydrogenase.


PMID: 6434827

Other references

Chase E.T.; Menard H.W., 1969: Four fracture zones off Mexico. Inst Geofs

Villa Caballero, L.; Nava Ocampo, A.; Frati Munari, A.; Hernandez Rodriguez D.L.S.fia; Ponce Monter, H.; Becerra Perez, A.; Ceja, R., 2003: Oxidative stress and hemodynamic profile in type 2 diabetic subjects under acute exercise. Diabetes 52(Supplement 1): A506

Ercoli, A.; Falconi, G.; Gardi, R.; Vitali, R., 1972: Synthesis and antiinflammatory activity of betamethasone 17-benzoate. Journal of Medicinal Chemistry 15(7): 783-784

Tekin, N.; Yurdaydin, N.; Klug, E.; Daskin, A.; Keskin, O.; Kucuk, H., 1993: Deepfreezing preservation trials with semen of Arab and Haflinger stallions in the Turkish National Studs Karacabey. Within a German-Turkish university partnership deep-freezing preservation of stallion semen was performed as a part project of the cooperation contract. In this study a modification of the introduced Makrotub method was used for semen freezing. Th...

Bauer Fridtjof, 1961: Die Entwicklung von Karstformen in Abhaengigkeit von den Umweltsfaktoren. Environmental conditions are reflected in the development of karst features, which therefore serve as a basis for interpreting late and postglacial climatic changes.

Sung, R.R.; Al-Wahabi, K.S., 2012: Model-centric 3-D petrophysical application technology. Middle East Geosciences Conference and Exhibition 10

Agard, D.A.; Hiraoka, Y.; Sedat, J.W., 1988: Three-dimensional light microscopy of diploid Drosophila chromosomes. Fluorescence microscopy, uniquely, provides the ability to examine specific components within intact, even living, cells. Unfortunately, high-resolution conventional fluorescence microscopy is intrinsically a two-dimensional technique and performs...

Israel E.; Lee T.H.; Austen K.F.; Corey E.J.; Robinson D.R.; Drazen J.M., 1984: Fish oil enriched diet modifies the guinea pig pulmonary parenchymal response to leukotriene e 4. American Review of Respiratory Disease 129(4 SUPPL): A232

Fan, R., 2000: Freedom, responsibility, and care: Hong Kong's health care reform. Journal of Medicine and Philosophy 24(6): 555-570

Tripathi, A.K.; Solanki, J.V.; Joshi, C.G., 2013: H19 gene methylation study in Indian buffalo (Bubalus bubalis). The methylation of DNA at cytosine residues within CpG dinucleotides is associated with transcriptional repression and is implicated in maintaining genomic stability and also the silencing of repetitive elements. These imprinted genes are unique a...