5C56.00

ICD-11 MMS 2026-01

Gangliosidosis

Index terms

  • Gangliosidosis
  • GM1 gangliosidosis
  • Landing disease
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  • GM1 gangliosidosis type 1
  • Generalised gangliosidosis
  • Infantile GM1 gangliosidosis
  • GM1 gangliosidosis type 2
  • Juvenile GM1 gangliosidosis
  • GM1 gangliosidosis type 3
  • Adult GM1 gangliosidosis
  • GM2 gangliosidosis
  • GM 2 gangliosidosis NOS
  • GM<sub>2</sub> gangliosidosis
  • deficiency of beta-n-acetylhexosaminidase isoenzymes
  • Retinal dystrophy in GM2 gangliosidosis
  • Sandhoff disease
  • GM2 gangliosidosis 0 variant
  • GM2 gangliosidosis type 2
  • GM<sub>2</sub> gangliosidosis, type 2
  • GM>2< gangliosidosis, type 2
  • Hexosaminidase A and B deficiency
  • Total hexosaminidase deficiency
  • Infantile Sandhoff disease
  • Total hexosaminidase deficiency, infantile form
  • infantile GM2 gangliosidosis
  • infantile GM2 gangliosidosis Type 2
  • infantile GM2 gangliosidosis, type II
  • Juvenile Sandhoff disease
  • Total hexosaminidase deficiency, juvenile form
  • juvenile GM2 gangliosidosis
  • juvenile GM<sub>2</sub> gangliosidosis
  • juvenile GM>2< gangliosidosis
  • Adult Sandhoff disease
  • Total hexosaminidase deficiency, adult form
  • adult GM2 gangliosidosis
  • Tay-Sachs disease
  • GM2-gangliosidosis, B, B1, AB variant
  • Hexosaminidase A deficiency
  • severe hexosaminidase a deficiency
  • B variant of hexosaminidase A deficiency
  • B variant of hexosaminidase A deficiency, infantile form
  • Classic infantile acute Tay-Sachs disease
  • GM2 gangliosidosis type 1
  • GM<sub>2</sub> gangliosidosis, type 1
  • GM>2< gangliosidosis, type 1
  • B variant of hexosaminidase A deficiency, juvenile form
  • GM2 gangliosidosis type 3
  • GM<sub>2</sub> gangliosidosis, type 3
  • GM>2< gangliosidosis, type 3
  • Juvenile subacute Tay-Sachs disease
  • B variant of hexosaminidase A deficiency, adult form
  • Adult Tay-Sachs disease
  • Chronic GM2 gangliosidosis
  • B1 variant of hexosaminidase A deficiency
  • Late infantile Tay-Sachs disease
  • Pseudo-AB variant of hexosaminidase deficiency
  • Hexosaminidase activator deficiency
  • AB variant of hexosaminidase deficiency
  • GM2 activator deficiency

Exclusions from above levels

  • 5C51.3 – Glycogen storage disease due to LAMP-2 deficiency
  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
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  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C56.01 – Fabry disease
  • 5C56.02 – Metachromatic leukodystrophy
  • 5C56.0Y – Other specified sphingolipidosis
  • 5C56.0Z – Sphingolipidosis, unspecified

Foundation entity

Gangliosidosis – http://id.who.int/icd/entity/797306953

Part of: 5C56.0: Sphingolipidosis › 5C56: Lysosomal diseases › 05: Endocrine, nutritional or metabolic diseases