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Deficiency of branching enzyme

disorder
SNOMED 124267007CUI C0017923

Overview

Source: MedlinePlus.gov, National Library of Medicine. Not a substitute for medical advice.

Signs & Symptoms

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

Less than 10 fetal movements in 12 hours
Always present (100%)HP:0001558
Abnormal cardiomyocyte morphology
Very frequent (80-99%)HP:0031331
Abnormal muscle glycogen content
Very frequent (80-99%)HP:0012269
Abnormal neuronal branching
Very frequent (80-99%)HP:0500032
Decreased liver function
Very frequent (80-99%)HP:0001410
Enlarged liver
Very frequent (80-99%)HP:0002240
Generalized abnormality of skin
Very frequent (80-99%)HP:0011354
Bradycardia
Frequent (30-79%)HP:0001662
Fetal foot inversion
Frequent (30-79%)HP:0001762
Generalised decreased muscle tone
Frequent (30-79%)HP:0001290
Hyporeflexia
Frequent (30-79%)HP:0001265
Low albumin
Frequent (30-79%)HP:0003073
Myopathy
Frequent (30-79%)HP:0003198
No development of motor milestones
Frequent (30-79%)HP:0001270
Peripheral hypotonia
Frequent (30-79%)HP:0001252
Stretched and thinned heart muscle
Frequent (30-79%)HP:0001644
Subclinical abnormal liver function tests
Frequent (30-79%)HP:0002910
Undergrowth
Frequent (30-79%)HP:0001508
Ascites
Occasional (5-29%)HP:0001541
Cardiac insufficiency
Occasional (5-29%)HP:0001635
Cirrhosis
Occasional (5-29%)HP:0001394
Esophageal varix
Occasional (5-29%)HP:0002040
Fetal akinesia sequence
Occasional (5-29%)HP:0001989
Flexion contractures
Occasional (5-29%)HP:0001371
Hepatosplenomegaly
Occasional (5-29%)HP:0001433
Increased amniotic fluid index
Occasional (5-29%)HP:0001561
Laboured breathing
Occasional (5-29%)HP:0002098
Low factor II activity
Occasional (5-29%)HP:0008151
Muscle wasting
Occasional (5-29%)HP:0003202
Nonimmune hydrops fetalis
Occasional (5-29%)HP:0001790

Quick Facts

SNOMED CT
124267007
UMLS CUI
C0017923
Fully Specified Name
Deficiency of 1,4-alpha-glucan branching enzyme (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.