Systemic triglyceride storage disease with normal carnitine: a putative defect in long-chain fatty acid metabolism

1988 ◽  
Vol 85 (2) ◽  
pp. 149-159 ◽  
Author(s):  
Hiroshi Ibayashi ◽  
Hiroshi Ideguchi ◽  
Naohiko Harada ◽  
Shinji Ishimoto ◽  
Ikuo Goto
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Metabolism ◽  
Acid Metabolism ◽  
Storage Disease ◽  
Chain Fatty Acid ◽  
Long Chain Fatty Acid ◽  
Long Chain

ACBD5 deficiency causes a defect in peroxisomal very long-chain fatty acid metabolism

2016 ◽  
Vol 54 (5) ◽  
pp. 330-337 ◽  
Author(s):  
Sacha Ferdinandusse ◽  
Kim D Falkenberg ◽  
Janet Koster ◽  
Petra A Mooyer ◽  
Richard Jones ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Metabolism ◽  
Acid Metabolism ◽  
Chain Fatty Acid ◽  
Long Chain Fatty Acid ◽  
Long Chain

scholarly journals Efficacy and tolerability of a specialized dietary therapeutic product in long-chain and very long-chain fatty acid metabolism disorders in children

2021 ◽  
Vol 4 (1) ◽  
pp. 98-102
Author(s):  
N.A. Semenova ◽  
◽  
E.A. Shestopalova ◽  
S.I. Kutsev ◽  
◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Metabolism ◽  
Acid Metabolism ◽  
Chain Fatty Acid ◽  
Allergic Reactions ◽  
Long Chain Fatty Acid ◽  
Therapeutic Product ◽  
Normal Ranges ◽  
Vital Functions ◽  
Long Chain

Aim: to assess the efficacy and tolerability of a specialized dietary therapeutic product in long-chain and very long-chain fatty acid metabolism disorders in children. Patients and Methods: this open-label prospective uncontrolled study enrolled 5 children aged 35 ± 14 months who were diagnosed with longchain and very long-chain fatty acid metab-olism disorders. Diet therapy using the tested product (“Monogen”) was prescribed and corrected after the analysis of actual diet. Physical development and neurological status were evaluated, blood levels of specific acyl-carnitines (С16-ОН, С18-ОН, С18:1-ОН, С14:1, С14) were measured. Tolerability was assessed by the rate and severity of adverse events (including allergic reactions) and the changes in vital functions compared to baseline. Patient and doctor satisfaction was evaluated using a questionnaire. Results: children received the tested product throughout the study (30±2 days). The lev-els of acyl-carnitines were within normal ranges in all children. No dyspepsia, allergic reactions, somatic dysfunction, or neurological deviations were reported. Physical exam consistently fell in the range of normal for age. Parents and doctors were fully satisfied with the tested product (“Monogen”). Conclusions: “Monogen” is highly effective for therapeutic feeding of children with long-chain and very long-chain fatty acid metabolism disorders. KEYWORDS: long-chain fatty acid oxidation disorders, β-oxidation of fatty acids, long-chain acyl-coA dehydrogenase deficiency, biallelic mutation, therapeutic feeding. FOR CITATION: Semenova N.A., Shestopalova E.A., Kutsev S.I. Efficacy and tolerability of a specialized dietary therapeutic product in longchain and very long-chain fatty acid metabolism disorders in children. Russian Journal of Woman and Child Health. 2021;4(1):98–102. DOI: 10.32364/2618-8430-2021-4-1-98-102.

Age-related changes of long-chain fatty acid metabolism in rat liver

1988 ◽  
Vol 44 (2) ◽  
pp. 175-183 ◽  
Author(s):  
Seijiro Mori ◽  
Nobuhiro Morisaki ◽  
Yasushi Saito ◽  
Sho Yoshida
Keyword(s):  
Fatty Acid ◽  
Rat Liver ◽  
Fatty Acid Metabolism ◽  
Acid Metabolism ◽  
Chain Fatty Acid ◽  
Long Chain Fatty Acid ◽  
Age Related ◽  
Long Chain ◽  
Age Related Changes

Inhibition of long-chain fatty acid metabolism does not affect platelet aggregation responses

1998 ◽  
Vol 356 (2-3) ◽  
pp. 207-213 ◽  
Author(s):  
Scott R. Willoughby ◽  
Yuliy Y. Chirkov ◽  
Jennifer A. Kennedy ◽  
Geraldine A. Murphy ◽  
Larissa P. Chirkova ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Platelet Aggregation ◽  
Fatty Acid Metabolism ◽  
Acid Metabolism ◽  
Chain Fatty Acid ◽  
Long Chain Fatty Acid ◽  
Long Chain
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