Disorders Of Metabolite Absorption Or Transport – ICD 11 Codes

Disorders of amino acid absorption or transport

Definition of Disorders of amino acid absorption or transport: Any condition caused by deficiencies in amino acid absorption and transport.

ICD 11 Code For Disorders of amino acid absorption or transport

5C60  Disorders of amino acid absorption or transport

Exclusions:

Coded Elsewhere:

  • Fanconi syndrome (GB90.42)

5C60.0 Oculocerebrorenal syndrome

Definition of Oculocerebrorenal syndrome: Oculocerebrorenal syndrome of Lowe (OCRL) is a multisystem disorder characterised by congenital cataracts, glaucoma, intellectual disabilities, postnatal growth retardation and renal tubular dysfunction with chronic renal failure.

5C60.1 Cystinosis

Definition of Cystinosis: Cystinosis is a metabolic disease characterised by an accumulation of cystine inside the lysosomes of different tissues due to a defect in cystine transport out of lysosomes. There are three clinical forms : infantile, juvenile and ocular. The infantile form is severe, multisystem disease, with impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, cystine deposits in various organs and progression towards renal failure after 6 years of age. Juvenile cystinosis appear around 8 years of age and has an intermediate clinical picture with end-stage renal disease occurring after the age of 15.The ocular, adult form presents with photophobia.

5C60.2 Cystinuria

Definition of Cystinuria: Cystinuria is a renal tubular aminoacid transport disorder characterised by recurrent formation of kidneys cystine stones.

5C60.Y Other specified disorders of amino acid absorption or transport

5C60.Z Disorders of amino acid absorption or transport unspecified

Disorders of carbohydrate absorption or transport

ICD 11 Code For Disorders of carbohydrate absorption or transport

  5C61  Disorders of carbohydrate absorption or transport

5C61.0 Glucose-galactose malabsorption

Definition of Glucose-galactose malabsorption: Glucose-galactose malabsorption is characterised by diarrhoea and severe neonatal dehydration. Around 300 cases have been described to date. Moderate glucosuria has also been reported, but fructose absorption is normal. Glucose-galactose malabsorption is caused by a mutation in the SLC5A1 gene, encoding the glucose-sodium cotransporter, SGTL1. The mode of transmission is autosomal recessive. The fatal consequences of this syndrome can be avoided by following a glucose and galactose restricted diet.

Exclusions:

5C61.1 Maltase-glucoamylase deficiency

Definition of Maltase-glucoamylase deficiency: Chronic diarrhea due to glucoamylase deficiency is characterised by chronic diarrhoea in infancy or childhood in association with intestinal glucoamylase deficiency.

5C61.2 Congenital sucrase-isomaltase deficiency

Definition of Congenital sucrase-isomaltase deficiency: Congenital sucrase-isomaltase deficiency (CSID) is a carbohydrate intolerance disorder characterised by malabsorption of oligosaccharides and disaccharides. CSID is transmitted as an autosomal recessive trait and is caused by mutations in the brush-border membrane complex sucrase-isomaltase (SI), which is required for the breakdown of sucrose and starch into monosaccharides. The SI deficiency results in an accumulation of disaccharides in the lumen, causing osmotic diarrhoea. The prognosis for patients is good as the starch intolerance usually resolves during the first few years of life and sucrose intolerance usually improves with age.

5C61.3 Alpha alpha trehalase deficiency

Definition of Alpha alpha trehalase deficiency: Alpha, alpha trehalase deficiency is characterised by diarrhoea and vomiting after ingestion of trehalose, a disaccharide found mainly in mushrooms. The disease is very rare in most populations but the incidence has been estimated at least 1 in 13 in Greenland. Isolated trehalose intolerance is due to a deficiency of trehalase (TREH; 11q23.3), a brush-border membrane glycoprotein.

5C61.4 Acquired monosaccharide malabsorption

Definition of Acquired monosaccharide malabsorption: This is an acquired condition in which the cells lining the intestine cannot take in one or all of the sugars glucose, galactose or fructose, which prevents proper digestion of these molecules and larger molecules made from them. 

It may cause osmotic diarrhoea.

5C61.40 Fructose malabsorption

5C61.4Y Other specified acquired monosaccharide malabsorption

5C61.4Z Acquired monosaccharide malabsorption unspecified

5C61.5 Disorders of facilitated glucose transport

Coded Elsewhere:

5C61.6 Lactose intolerance

Definition of Lactose intolerance: Lactose intolerance is the inability to digest lactose, a sugar found in milk and some dairy products, due to a deficiency of lactase, the enzyme that metabolizes lactose. Lactose intolerance occurs when lactose is not completely broken down and consequently the sugar cannot be absorbed into the blood.

5C61.60 Primary lactase deficiency

5C61.61 Congenital lactase deficiency

Definition of Congenital lactase deficiency: This is a congenital deficiency of lactase (EC 3.2.1.108), inherited as an autosomal recessive trait, presenting in infancy and manifested by profuse watery diarrhoea in response to dietary milk, due to inability to digest lactose, a sugar found in milk and to a lesser extent milk-derived dairy products. The condition may lead to marasmus and death if lactose is not eliminated from the diet.

5C61.62 Secondary lactase deficiency

Definition of Secondary lactase deficiency: This form of lactase deficiency results from some sort of damage to the intestines either due to a disease or surgery.

Coding Note:

  • Code also the causing condition

5C61.6Z Lactose intolerance unspecified

5C61.Y Other specified disorders of carbohydrate absorption or transport

5C61.Z Disorders of carbohydrate absorption or transport unspecified

Disorders of lipid absorption or transport

ICD 11 Code For Disorders of lipid absorption or transport

  5C62  Disorders of lipid absorption or transport

Disorders of vitamin or non-protein cofactor absorption or transport

ICD 11 Code For Disorders of vitamin or non-protein cofactor absorption or transport

  5C63  Disorders of vitamin or non-protein cofactor absorption or transport

Coded Elsewhere:

5C63.0 Disorders of cobalamin metabolism or transport

Coded Elsewhere:

5C63.1 Disorders of folate metabolism or transport

Coded Elsewhere:

  • Formiminoglutamic aciduria (3A02.Y)

5C63.2 Disorders of vitamin D metabolism or transport

5C63.20 Hypocalcaemic vitamin D dependent rickets

Definition of Hypocalcaemic vitamin D dependent rickets: Hypocalcaemic vitamin D-dependent rickets (VDDR-I) is an early-onset hereditary vitamin D metabolism disorder characterised by severe hypocalcaemia leading to osteomalacia and rachitic bone deformations, and moderate hypophosphatemia.

5C63.21 Hypocalcaemic vitamin D resistant rickets

Definition of Hypocalcaemic vitamin D resistant rickets: Hypocalcaemic vitamin D-resistant rickets is a hereditary disorder of vitamin D action characterised by hypocalcaemia, severe rickets and in many cases alopecia.

5C63.22 Hypophosphataemic rickets

Definition of Hypophosphatemic rickets: Hypophosphatemic rickets is a group of genetic diseases characterised by hypophosphatemia, rickets, and normal serum levels of calcium.

5C63.2Y Other specified disorders of vitamin D metabolism or transport

5C63.2Z Disorders of vitamin D metabolism or transport unspecified

5C63.Y Other specified disorders of vitamin or non-protein cofactor absorption or transport

5C63.Z Disorders of vitamin or non-protein cofactor absorption or transport unspecified

Disorders of mineral absorption or transport

Definition of Disorders of mineral absorption or transport: A condition in which there is a deviation or interruption in the processing of a specific mineral in the body: its absorption, transport, storage, and utilization

ICD 11 Code For Disorders of mineral absorption or transport

  5C64  Disorders of mineral absorption or transport

Exclusions:

5C64.0 Disorders of copper metabolism

Definition of Disorders of copper metabolism: Any condition caused by failure to correctly metabolize copper.

Coded Elsewhere:

  • X-linked cutis laxa (LD28.2)

5C64.00 Wilson disease

Definition of Wilson disease: Wilson disease is an autosomal recessive disorder of copper metabolism characterised by the toxic accumulation of copper, mainly in the liver and central nervous system that may present as hepatic, neurologic or psychiatric forms.

5C64.0Y Other specified disorders of copper metabolism

5C64.0Z Disorders of copper metabolism unspecified

5C64.1 Disorders of iron metabolism

Definition of Disorders of iron metabolism: This refers to any disorders of the set of chemical reactions maintaining human homeostasis of iron. The control of this necessary but potentially toxic substance is an important part of many aspects of human health and disease.

Exclusions:

5C64.10 Iron overload diseases

Definition of Iron overload diseases: Iron overload is the accumulation of excess iron in body tissues. Iron overload usually occurs as a result of a genetic predisposition to absorb and store iron in excess amounts, the most common form of which is hereditary hemochromatosis. Iron overload can also occur as a complication of other hematologic disorders that require chronic transfusion therapy, repeated injections of parenteral iron, or excessive iron ingestion. Excessive iron stores usually accumulate in the reticuloendothelial tissues and cause little damage (“hemosiderosis”). If overload continues, iron eventually begins to accumulate in tissues such as hepatic parenchyma, pancreas, heart and synovium, causing hemochromatosis.

Coded Elsewhere:

  • Friedreich ataxia (8A03.10)
  • Atransferrinaemia (3A00.Y)
  • Microcytic anaemia with liver iron overload (3A00.Y)

5C64.1Y Other specified disorders of iron metabolism

5C64.1Z Disorders of iron metabolism unspecified

5C64.2 Disorders of zinc metabolism

Definition of Disorders of zinc metabolism: Any condition caused by failure to correctly metabolize zinc. These conditions may present with dermatitis, diarrhoea, alopecia, loss of appetite, growth impairment, neuropsychological changes, or immune deficiency syndromes.

5C64.20 Acrodermatitis enteropathica

Definition of Acrodermatitis enteropathica: Acrodermatitis enteropathica is an uncommon autosomal recessive disorder of intestinal zinc absorption. Signs usually appear within the first months of life with an exudative and crusted erythema located predominantly around body orifices (mouth, anogenital) and on the scalp and distal extremities. The signs are often misdiagnosed as being due to infection. The condition responds rapidly to zinc supplementation which must be continued throughout life.

5C64.21 Zinc deficiency syndromes

Coded Elsewhere:

  • Acrodermatitis enteropathica (5C64.20)

5C64.2Y Other specified disorders of zinc metabolism

5C64.2Z Disorders of zinc metabolism unspecified

5C64.3 Disorders of phosphorus metabolism or phosphatases

Definition of Disorders of phosphorus metabolism or phosphatases: Any condition caused by errors in phosphorus metabolism, or in phosphatase activity.

Exclusions:

  • Adult osteomalacia (FB83.2)
  • Osteoporosis (FB83.1)

Coded Elsewhere:

  • Hypophosphataemic rickets (5C63.22)
  • Phosphate losing hypophosphataemia (GB90.48)

5C64.4 Disorders of magnesium metabolism

Definition of Disorders of magnesium metabolism: Any condition caused by failure to correctly metabolize magnesium.

5C64.40 Hypermagnesaemia

Definition of Hypermagnesaemia: This is an electrolyte disturbance in which there is an abnormally elevated level of magnesium in the blood. Usually this results in excess of magnesium in the body.

5C64.41 Hypomagnesaemia

Definition of Hypomagnesaemia: This is an electrolyte disturbance in which there is an abnormally low level of magnesium in the blood. Normal magnesium levels in humans fall between 1.5 – 2.5 mg/dL. Usually a serum level less than 0.7 mmol/L is used as reference for hypomagnesemia (not hypomagnesia which refers to low magnesium content in food/supplement sources).

Coded Elsewhere:

  • Neonatal hypomagnesaemia (KB61.0)

5C64.4Z Disorders of magnesium metabolism unspecified

5C64.5 Disorders of calcium metabolism

Definition of Disorders of calcium metabolism: This refers to and disorders in the mechanism by which the body maintains adequate calcium levels. Derangements of this mechanism lead to hypercalcaemia or hypocalcaemia, both of which can have important consequences for health.

Exclusions:

Coded Elsewhere:

5C64.6 Disorders of sodium metabolism

Coded Elsewhere:

  • Tubular disorders of sodium or potassium transport (GB90.46)
  • Congenital sodium diarrhoea (DA90.1)

5C64.7 Disorders of chloride metabolism

Coded Elsewhere:

  • Congenital chloride diarrhoea (DA90.1)

5C64.Y Disorders of other specified mineral absorption and transport

5C64.Z Disorders of mineral absorption or transport unspecified

Other specified disorders of metabolite absorption or transport

ICD 11 Code For Other specified disorders of metabolite absorption or transport

  5C6Y  Other specified disorders of metabolite absorption or transport

Disorders of metabolite absorption or transport unspecified

ICD 11 Code For Disorders of metabolite absorption or transport unspecified

  5C6Z  Disorders of metabolite absorption or transport unspecified

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