01 Certain infectious or parasitic diseases
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02 Neoplasms
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03 Diseases of the blood or blood-forming organs
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04 Diseases of the immune system
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05 Endocrine, nutritional or metabolic diseases
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Endocrine diseases
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Nutritional disorders
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Metabolic disorders
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Inborn errors of metabolism
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5C50 Inborn errors of amino acid or other organic acid metabolism
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5C51 Inborn errors of carbohydrate metabolism
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5C52 Inborn errors of lipid metabolism
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H01589 Systemic primary carnitine deficiency
H01981 Carnitine palmitoyltransferase I deficiency
H01982 Carnitine palmitoyltransferase II deficiency
H01983 Carnitine-acylcarnitine translocase deficiency
H02596 Disorders of carnitine transport and the carnitine cycle
H00392 VLCAD deficiency
H00488 MCAD deficiency
H00489 LCHAD deficiency
H00525 Disorders of mitochondrial fatty-acid oxidation
H00925 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency
H01352 Mitochondrial trifunctional protein deficiency
H01364 3-Hydroxyacyl-CoA dehydrogenase deficiency
H01929 2,4-Dienoyl-CoA reductase deficiency
H01980 SCAD deficiency
H02085 Acyl-CoA dehydrogenase 9 deficiency
H00179 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H01121 Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
H01123 HMG-CoA synthase deficiency
H00162 Sjogren-Larsson syndrome
H02284 Leukotriene C4 synthase deficiency
H02689 Retinal dystrophy with leukodystrophy
H00161 Smith-Lemli-Opitz syndrome
H00206 Mevalonate kinase deficiency
H00617 Desmosterolosis
H01281 Lathosterolosis
H00151 Cerebrotendinous xanthomatosis
H00624 Progressive familial intrahepatic cholestasis
H00628 Congenital bile acid synthesis defect
H02099 Alpha-methylacyl-CoA racemase deficiency
H00152 Sitosterolemia
H00736 Dorfman-Chanarin syndrome
H01297 Neutral lipid storage disease with myopathy
H00654 Barth syndrome
H02527 Lipid storage myopathy due to FLAD1 deficiency
H02711 Acetyl-CoA carboxylase-alpha deficiency
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5C53 Inborn errors of energy metabolism
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5C54 Inborn errors of glycosylation or other specified protein modification
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5C55 Inborn errors of purine, pyrimidine or nucleotide metabolism
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5C56 Lysosomal diseases
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5C57 Peroxisomal diseases
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5C58 Inborn errors of porphyrin or heme metabolism
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5C59 Inborn errors of neurotransmitter metabolism
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5C5A Alpha-1-antitrypsin deficiency
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5C5Y Other specified inborn errors of metabolism
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5C5Z Inborn errors of metabolism, unspecified
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Disorders of metabolite absorption or transport
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Disorders of fluid, electrolyte or acid-base balance
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Disorders of lipoprotein metabolism or certain specified lipidaemias
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5C90 Metabolic or transporter liver disease
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Other metabolic disorders
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5D2Z Metabolic disorders, unspecified
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Postprocedural endocrine or metabolic disorders
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06 Mental, behavioural or neurodevelopmental disorders
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07 Sleep-wake disorders
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08 Diseases of the nervous system
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09 Diseases of the visual system
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10 Diseases of the ear or mastoid process
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11 Diseases of the circulatory system
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12 Diseases of the respiratory system
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13 Diseases of the digestive system
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14 Diseases of the skin
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15 Diseases of the musculoskeletal system or connective tissue
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16 Diseases of the genitourinary system
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17 Conditions related to sexual health
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18 Pregnancy, childbirth or the puerperium
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19 Certain conditions originating in the perinatal period
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20 Developmental anomalies
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21 Symptoms, signs or clinical findings, not elsewhere classified
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22 Injury, poisoning or certain other consequences of external causes
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25 Codes for special purposes
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