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Mitochondrial DNA depletion syndrome 13 encephalomyopathic type

disorder
SNOMED 765403009CUI C3809592

Overview

Mitochondrial DNA depletion syndrome 13 encephalomyopathic type is a disorder.

Auto-generated from clinical reference data. Not a substitute for medical advice.

Signs & Symptoms

Based on Human Phenotype Ontology (HPO) disease-phenotype annotations.

3-Methylglutaconic aciduria
Always present (100%)HP:0003535
Absolute lymphocyte count decrease
Always present (100%)HP:0001888
CNS hypomyelination
Always present (100%)HP:0003429
Decreased activity of cytochrome C oxidase in muscle tissue
Always present (100%)HP:0003688
Decreased serum immunoglobulin
Always present (100%)HP:0004313
Delay in head control
Always present (100%)HP:0002421
Elevated urinary 3-hydroxybutyric acid
Always present (100%)HP:0040155
Elevated urine fumaric acid level
Always present (100%)HP:0034648
Encephalopathy
Always present (100%)HP:0001298
Enlarged fetal cisterna magna
Always present (100%)HP:0011427
Extensor plantar responses
Always present (100%)HP:0003487
Frontal protuberance
Always present (100%)HP:0002007
Generalised brain degeneration
Always present (100%)HP:0002283
Higher than normal levels of lactate in blood
Always present (100%)HP:0002151
Hydronephrosis
Always present (100%)HP:0000126
Hyperalaninemia
Always present (100%)HP:0003348
Hyperkinetic movements
Always present (100%)HP:0002487
Increased reflexes
Always present (100%)HP:0001347
Increased urine lactate
Always present (100%)HP:0003648
Inguinal hernia
Always present (100%)HP:0000023
Lacticacidemia
Always present (100%)HP:0003128
Leukodystrophy
Always present (100%)HP:0002415
Low number of red blood cells or haemoglobin
Always present (100%)HP:0001903
Metabolic acidosis
Always present (100%)HP:0001942
More grooves in brain
Always present (100%)HP:0002126
Nonsyndromal hydrocephalus
Always present (100%)HP:0000238
Prominent frontal suture
Always present (100%)HP:0005487
Prominent supraorbital margins
Always present (100%)HP:0000336
Short chin
Always present (100%)HP:0000331
Small cerebellum
Always present (100%)HP:0001321

Quick Facts

SNOMED CT
765403009
UMLS CUI
C3809592
Fully Specified Name
F-box and leucine rich repeat protein 4 related mitochondrial deoxyribonucleic acid depletion syndrome encephalomyopathic form (disorder)
Specialists
0
Diagnostic Biomarkers
0
HPO Phenotypes
30
Medical Disclaimer

This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider regarding any medical condition or treatment plan.

Clinical content is derived from the SNOMED CT clinical ontology and curated medical knowledge graphs.

Mitochondrial DNA depletion syndrome 13 encephalomyopathic type — Symptoms, Testing & Specialists | Ltrl | Healos