2026 ICD-10-CM Diagnosis Code E77.1Defects in glycoprotein degradation

AHRQ’s CCSR groups this code into broader clinical categories.

• Aspartylglucosaminuria

  a recessively inherited, progressive lysosomal storage disease caused by a deficiency of glycosylasparaginase activity. the lack of this enzyme activity results in the accumulation of n-acetylglucosaminylasparagine (the linkage unit of asparagine-linked glycoproteins) in lysosomes.

• Fucosidosis

  an autosomal recessive lysosomal storage disease caused by a deficiency of alpha-l-fucosidase activity resulting in an accumulation of fucose containing sphingolipids; glycoproteins, and mucopolysaccharides (glycosaminoglycans) in lysosomes. the infantile form (type i) features psychomotor deterioration, muscle spasticity, coarse facial features, growth retardation, skeletal abnormalities, visceromegaly, seizures, recurrent infections, and macroglossia, with death occurring in the first decade of life. juvenile fucosidosis (type ii) is the more common variant and features a slowly progressive decline in neurologic function and angiokeratoma corporis diffusum. type ii survival may be through the fourth decade of life. (from menkes, textbook of child neurology, 5th ed, p87; am j med genet 1991 jan;38(1):111-31)

• Fucosidosis

  an autosomal recessive lysosomal storage disease characterized by a defective alpha-l-fucosidase. it results in accumulation of fucose in the tissues. signs and symptoms include psychomotor retardation, dysostosis multiplex, and neural disturbances.

• Alpha-Mannosidosis

  an autosomal recessive lysosomal storage disease characterized by deficient activity of the enzyme alpha-d-mannosidase. there is a wide range of signs and symptoms including hepatomegaly, splenomegaly, hearing loss, respiratory infections, mental retardation, skeletal abnormalities, leveled nasal bridge and protruding forehead.

• Beta-Mannosidosis

  an autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzyme beta-mannosidase. it is caused by mutations in the manba gene. common features of this disorder are mental retardation, developmental delays and recurrent respiratory infections.

• Mannosidosis

  a rare autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzymes alpha-d-mannosidase or beta-mannosidase. clinical signs and symptoms include hepatomegaly, splenomegaly, hearing loss, mental retardation, skeletal abnormalities, and recurrent respiratory infections.

• I-Cell Disease|Inclusion-cell Disease|Mucolipidosis Type II

  an inherited lysosomal storage disease characterized by the presence of dense intracytoplasmic inclusions in mesenchymal cells, especially fibroblasts. signs and symptoms include developmental delay, psychomotor deterioration, and growth failure.

• Mucolipidosis

  a group of inherited lysosomal storage diseases characterized by accumulation of lipids and carbohydrates in the tissues, resulting in mental disabilities and skeletal malformations.

• Mucolipidosis Type III Gamma|Mucolipidosis III Gamma

  an autosomal recessive condition caused by mutation(s) in the gnptag gene, encoding n-acetylglucosamine-1-phosphotransferase subunit gamma. it is characterized by a slowing of the growth rate in childhood, joint stiffness, mild cognitive impairment, and cardiorespiratory insufficiency.

• Mucolipidosis Type IIIA

  a lysosomal storage disease characterized by multiple bone formation abnormalities, progressive joint stiffness, developmental abnormalities, hearing loss, hepatosplenomegaly, increased acne, enlarged tongue, and cornea clouding due to accumulation of lipid substances.

• Mucolipidosis Type IV|Mucolipidosis IV

  an autosomal recessive lysosomal storage disease caused by mutations in the mcoln1 gene. it is characterized by psychomotor developmental delays and ophthalmologic abnormalities.

• Neuraminidase Deficiency|Mucolipidosis I|Sialidosis Type II

  an autosomal recessive inherited lysosomal storage disease characterized by excessive intracellular accumulation and urinary excretion of sialic acid associated with neuraminidase deficiency.

• MCOLN1 wt Allele|MG-2|ML1|ML4|MLIV|MST080|MSTP080|Mucolipidosis Type IV Gene|Mucolipin 1 Gene|Mucolipin TRP Cation Channel 1 wt Allele|TRP-ML1|TRPM-L1|TRPML1|Transient Receptor Potential Cation Channel Mucolipin Subfamily Member 1 Gene|Transient Receptor Potential Cation Channel, Mucolipin Subfamily, Member 1 Gene|Transient Receptor Potential Mucolipin 1 Gene

  human mcoln1 wild-type allele is located in the vicinity of 19p13.2 and is approximately 11 kb in length. this allele, which encodes mucolipin-1 protein, plays a role in the regulation of membrane trafficking events and of cation homeostasis. mutation of the gene is associated with mucolipidosis iv.

Carbohydrate Metabolism Disorders

Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.

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