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Chapters ENES DE

05Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C56Lysosomal diseases

5C56.3 category

Mucopolysaccharidosis

Inclusions

Disorders of glycosaminoglycan metabolism

6 subordinate codes

5C56.30Mucopolysaccharidosis type 1
5C56.31Mucopolysaccharidosis type 2
5C56.32Mucopolysaccharidosis type 4
5C56.33Mucopolysaccharidosis type 6
4B4YOther specified diseases of the immune system
4B4ZDiseases of the immune system, unspecified

Nearby in Lysosomal diseases

5C56.0Sphingolipidosis
5C56.1Neuronal ceroid lipofuscinosis
5C56.2Glycoproteinosis
5C56.4Disorders of sialic acid metabolism
4B4YOther specified diseases of the immune system
4B4ZDiseases of the immune system, unspecified

Previous5C56.2 — Glycoproteinosis Next5C56.4 — Disorders of sialic acid metabolism

Browse ICD-11

01Certain infectious or parasitic diseases
02Neoplasms
03Diseases of the blood or blood-forming organs
04Diseases of the immune system
05Endocrine, nutritional or metabolic diseases
06Mental, behavioural or neurodevelopmental disorders
07Sleep-wake disorders
08Diseases of the nervous system
09Diseases of the visual system
10Diseases of the ear or mastoid process
11Diseases of the circulatory system
12Diseases of the respiratory system
13Diseases of the digestive system
14Diseases of the skin
15Diseases of the musculoskeletal system or connective tissue
16Diseases of the genitourinary system
17Conditions related to sexual health
18Pregnancy, childbirth or the puerperium
19Certain conditions originating in the perinatal period
20Developmental anomalies
21Symptoms, signs or clinical findings, not elsewhere classified
22Injury, poisoning or certain other consequences of external causes
23External causes of morbidity or mortality
24Factors influencing health status or contact with health services
25Codes for special purposes
26Supplementary Chapter Traditional Medicine Conditions
VSupplementary section for functioning assessment
XExtension Codes
5A00-5B3ZEndocrine diseases
5B50-5C3ZNutritional disorders
5C50-5D2ZMetabolic disorders
5D40-5D46Postprocedural endocrine or metabolic disorders
5C50-5C5ZInborn errors of metabolism
5C60-5C6ZDisorders of metabolite absorption or transport
5C70-5C7ZDisorders of fluid, electrolyte or acid-base balance
5C80-5C8ZDisorders of lipoprotein metabolism or certain specified lipidaemias
5C90Metabolic or transporter liver disease
5D00-5D0YOther metabolic disorders
4B4ZDiseases of the immune system, unspecified
5C52Inborn errors of lipid metabolism
5C53Inborn errors of energy metabolism
5C54Inborn errors of glycosylation or other specified protein modification
5C55Inborn errors of purine, pyrimidine or nucleotide metabolism
5C56Lysosomal diseases
5C57Peroxisomal diseases
5C58Inborn errors of porphyrin or heme metabolism
5C59Inborn errors of neurotransmitter metabolism
5C5AAlpha-1-antitrypsin deficiency
4 more
5C56.0Sphingolipidosis
5C56.1Neuronal ceroid lipofuscinosis
5C56.2Glycoproteinosis
5C56.3Mucopolysaccharidosis
5C56.4Disorders of sialic acid metabolism
4B4YOther specified diseases of the immune system
4B4ZDiseases of the immune system, unspecified
5C56.30Mucopolysaccharidosis type 1
5C56.31Mucopolysaccharidosis type 2
5C56.32Mucopolysaccharidosis type 4
5C56.33Mucopolysaccharidosis type 6
4B4YOther specified diseases of the immune system
4B4ZDiseases of the immune system, unspecified

International Classification of Diseases, Eleventh Revision (ICD-11), World Health Organization (WHO) 2019/2021, https://icd.who.int/browse11. Licensed under Creative Commons Attribution-NoDerivatives 3.0 IGO (CC BY-ND 3.0 IGO).

Release 2026-01. View this entity in the official WHO ICD-11 browser.

This page reproduces WHO classification content for reference. It is not medical advice and must not be used to diagnose or treat. For coding decisions, consult the official release and your national coding guidelines.

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