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Mucopolysaccharidosis, MPS-I-H

disorder
SNOMED 65327002CUI C0086795

Overview

Mucopolysaccharidosis, MPS-I-H 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.

Abdominal protuberance
Always present (100%)HP:0001538
Big calvaria
Always present (100%)HP:0000256
Bilateral ptosis
Always present (100%)HP:0001488
Dermatan sulphate excretion in urine
Always present (100%)HP:0008301
Diminished tissue alpha-L-iduronidase activity
Always present (100%)HP:6000910
Dysostosis multiplex
Always present (100%)HP:0000943
Heparan sulphate excretion in urine
Always present (100%)HP:0002159
Hepatosplenomegaly
Always present (100%)HP:0001433
Increased distance between eyes
Always present (100%)HP:0000316
Increased ossification of calvarial bones
Always present (100%)HP:0004490
Recurrent middle ear infection
Always present (100%)HP:0000403
Stiff joint
Always present (100%)HP:0001387
Thick skull bones
Always present (100%)HP:0004437
Umbilical hernia
Always present (100%)HP:0001537
Widened metaphyses
Always present (100%)HP:0003016
Abnormality of the tonsils
Very frequent (80-99%)HP:0100765
Broad flat nasal bridge
Very frequent (80-99%)HP:0000431
Central hypotonia
Very frequent (80-99%)HP:0001252
CP
Very frequent (80-99%)HP:0100021
Enlarged liver
Very frequent (80-99%)HP:0002240
Frontal protuberance
Very frequent (80-99%)HP:0002007
Generalized hirsutism
Very frequent (80-99%)HP:0002230
Hernia
Very frequent (80-99%)HP:0100790
Large facies
Very frequent (80-99%)HP:0100729
Limitation of joint mobility
Very frequent (80-99%)HP:0001376
Mitral regurgitation
Very frequent (80-99%)HP:0001653
Mucopolysacchariduria
Very frequent (80-99%)HP:0008155
Psychomotor development deficiency
Very frequent (80-99%)HP:0001263
Rhinitis
Very frequent (80-99%)HP:0012384
Short neck
Very frequent (80-99%)HP:0000470

Quick Facts

SNOMED CT
65327002
UMLS CUI
C0086795
Fully Specified Name
Mucopolysaccharidosis type I-H (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.