1. Certain infectious and parasitic diseases (A00-B99)
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2. Neoplasms (C00-D48)
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3. Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
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4. Endocrine, nutritional and metabolic diseases (E00-E90)
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E00-E07 Disorders of thyroid gland
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E10-E14 Diabetes mellitus
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E15-E16 Other disorders of glucose regulation and pancreatic internal secretion
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E20-E35 Disorders of other endocrine glands
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E40-E46 Malnutrition
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E50-E64 Other nutritional deficiencies
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E65-E68 Obesity and other hyperalimentation
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E70-E90 Metabolic disorders
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E70 Disorders of aromatic amino-acid metabolism
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E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
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H00172 Maple syrup urine disease
H02000 Dihydrolipoamide dehydrogenase deficiency
H00173 Isovaleric acidemia (IVA)
H00181 3-Methylcrotonylglycinuria
H00175 Propionic acidemia
H00174 Methylmalonic aciduria (MMA)
H00179 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
H00162 Sjogren-Larsson syndrome
H00176 Adrenoleukodystrophy (ALD)
H00178 Glutaric acidemia
H00525 Disorders of mitochondrial fatty-acid oxidation
H01980 SCAD deficiency
H00488 MCAD deficiency
H00489 LCHAD deficiency
H00392 VLCAD deficiency
H01981 Carnitine palmitoyltransferase I deficiency
H01982 Carnitine palmitoyltransferase II deficiency
H01983 Carnitine-acylcarnitine translocase deficiency
H00375 SBCAD deficiency
H00654 Barth syndrome (BTHS)
H00754 3-Methylglutaconic aciduria (MGCA)
H00925 2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency
H01121 Succinyl CoA:3-oxoacid CoA transferase (SCOT) deficiency
H01123 HMG-CoA synthase (HMGCS) deficiency
H01279 Isobutyryl-CoA dehydrogenase (IBD) deficiency
H00177 Adrenoleukodystrophy, neonatal (NALD)
H01349 Methacrylic aciduria
H01352 Mitochondrial trifunctional protein deficiency
H01364 3-Hydroxyacyl-CoA dehydrogenase deficiency
H00407 Peroxisomal beta-oxidation enzyme deficiency
H02085 Acyl-CoA dehydrogenase 9 deficiency
H02096 Peroxisomal acyl-CoA oxidase deficiency
H02098 D-bifunctional protein deficiency
H01589 Systemic primary carnitine deficiency
H01929 2,4-Dienoyl-CoA reductase deficiency
H02109 Combined malonic and methylmalonic aciduria
H02136 Branched-chain ketoacid dehydrogenase kinase deficiency
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E72 Other disorders of amino-acid metabolism
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E73 Lactose intolerance
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E74 Other disorders of carbohydrate metabolism
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E75 Disorders of sphingolipid metabolism and other lipid storage disorders
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E76 Disorders of glycosaminoglycan metabolism
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E77 Disorders of glycoprotein metabolism
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E78 Disorders of lipoprotein metabolism and other lipidaemias
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E79 Disorders of purine and pyrimidine metabolism
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E80 Disorders of porphyrin and bilirubin metabolism
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E83 Disorders of mineral metabolism
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E84 Cystic fibrosis
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E85 Amyloidosis
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E88 Other metabolic disorders
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5. Mental and behavioural disorders (F00-F99)
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6. Diseases of the nervous system (G00-G99)
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7. Diseases of the eye and adnexa (H00-H59)
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8. Diseases of the ear and mastoid process (H60-H95)
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9. Diseases of the circulatory system (I00-I99)
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10. Diseases of the respiratory system (J00-J99)
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11. Diseases of the digestive system (K00-K93)
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12. Diseases of the skin and subcutaneous tissue (L00-L99)
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13. Diseases of the musculoskeletal system and connective tissue (M00-M99)
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14. Diseases of the genitourinary system (N00-N99)
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15. Pregnancy, childbirth and the puerperium (O00-O99)
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16. Certain conditions originating in the perinatal period (P00-P96)
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17. Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
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18. Symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R99)
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19. Injury, poisoning and certain other consequences of external causes (S00-T98)
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20. External causes of morbidity and mortality (V01-Y98)
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21. Factors influencing health status and contact with health services (Z00-Z99)
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22. Codes for special purposes (U00-U85)
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