Disease Information for LCHAD/Long chain OH-CoA dehydrogenase def

Synonyms:

Acyl CoA Dehydrogenase Long Chain, Acyl CoA Dehydrogenase Very Long Chain, deficiency, Dehydrogenase Long Chain Acyl CoA, Dehydrogenase Long Chain Acyl Coenzyme A, Dehydrogenase Very Long Chain Acyl CoA, Long ch acyl CoA dehydrogenase, Long chain acyl CoA dehydrog, Long Chain Acyl CoA Dehydrogenase, Long chain acyl CoA dehydrogenase (substance), Long Chain Acyl Coenzyme A Dehydrogenase, Long chain acyl coenzyme A dehydrogenase (substance), Very Long Chain Acyl CoA Dehydrogenase, VLCAD

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Acyl CoA Dehydrogenase Long Chain, Acyl CoA Dehydrogenase Very Long Chain, ... (more)

Definition:

Long chain 3-Hydroxacyl-CoA dehydrogenase Deficiency metabolic enzyme defect. Hereditary extremely rare mitochondrial defect in all body cells. Severe fat dietary

restriction is indicated. Presentation often as failure to thrive infants. Family history positive.

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Long chain 3-Hydroxacyl-CoA dehydrogenase Deficiency metabolic enzyme defec... (more) (edit)

68 related Findings found. Compare "LCHAD/Long chain OH-CoA dehydrogenase def" to ...?

▼ Demographics & Risk Factors
▼ Clinical Manifestations
▼ Laboratory Tests
▼ Diagnostic Test Results
▼ Associated Diseases & Rule outs
▼ Disease Mechanism & Classification
▼ Synonyms
▼ Treatment
▼ External Links Related to LCHAD/Long chain OH-CoA dehydrogenase def

Demographics & Risk Factors[next]
- Population Group
  - Population/Pediatrics population
- Family History
  - Family history/Enzyme defect
  - Family history/Metabolic disease
- Sex & Age Groups
  - Population/Child
  - Population/Child-Infant Only
  - Population/Children/all
  - Population/Infant
Clinical Manifestations[previous][top][next]
- Typical Clinical Presentation
  - Presentation/Maternal difficulty/caused by fetus (metabolism)
  - Presentation/Non diabetic ketoacidosis kids/infants
- Disease Progression
  - Course/Chronic disorder
  - Course/Chronic only
- Signs & Symptoms
Laboratory Tests[previous][top][next]
- Abnormal Lab Findings (Non Measured)
- Abnormal Lab Findings - Increased
  - pH, arterial blood (Lab)
Diagnostic Test Results[previous][top][next]
- Other Tests & Procedures
  - Amniocentesis/Chorion villi enzyme assay/abnormal
Associated Diseases & Rule outs[previous][top][next]
- Associated Disease & Complications
- Disease Synergy - Causes
  - Synergy/Fasting/starvation
Disease Mechanism & Classification[previous][top][next]
- Class
  - CLASS/Pediatric disorders (ex)
- Pathophysiology
  - Pathophysiology/Beta oxidation fat defct variant
  - Pathophysiology/Fails to metabolize fat/mitochondrial level
  - Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
  - Pathophysiology/Intractable neonatal hypoglycemia
  - Pathophysiology/Long chain 3-Hydroxyacl-CoA dehydrogenase defic
  - Pathophysiology/Beta-oxidation cycle defect
- Process
  - PROCESS/Autosomal recessive disorder (ex)
  - PROCESS/Enzyme defect/Metabolic disorder (ex)
  - PROCESS/Fatty acid Oxidation Disorders (FOD)
  - PROCESS/Hereditary mitochondrial disorder (ex)
  - PROCESS/Hereditofamilial (category)
  - PROCESS/INCIDENCE/Esoteric disease (example)
  - PROCESS/INCIDENCE/Extremely rare disease
  - PROCESS/Metabolic/storage disorder (category)
  - PROCESS/Mitochondrial disorders (ex)
Synonyms[previous][top][next]
- Synonym
  - Synonym/Fat beta oxidation mitochodrial defct variant
Treatment[previous][top][next]
- Other Treatments
  - TX/Diet restriction treatment.
- Other Treatments
  - TX/Diet Limit long chain fatty acids
  - TX/Diet restriction treatment.
  - TX/Frequent feedings/Avoid fasting
  - TX/High carbohydrate diet
  - TX/Medium chain TGA/fatty acid diet
  - TX/Restricted long chain fatty acid diet
External Links Related to LCHAD/Long chain OH-CoA dehydrogenase def[previous][top]

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