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277 Other and unspecified disorders of metabolism

277.0 Cystic fibrosis

          Fibrocystic disease of the pancreas
          Mucoviscidosis

277.1 Disorders of porphyrin metabolism

          Porphyria Porphyrinuria

277.2 Other disorders of purine and pyrimidine metabolism

          Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
          Lesch-Nyhan syndrome
          Excludes: gout (274.-)
           orotaciduria anemia (281.4)

277.3 Amyloidosis

          Amyloidosis:
           NOS
           inherited systemic
           nephropathic
           neuropathic (Portuguese) (Swiss)
           secondary
           Familial Mediterranean fever
           Hereditary cardiac amyloid

277.4 Disorders of bilirubin excretion

          Syndrome:
           Crigler-Najjar
           Dubin-Johnson
           Gilbert's
           Rotor's
          Excludes: hyperbilirubinemias specific to the perinatal period (774.-)

277.5 Mucopolysaccharidosis

          Gargoylism Hurler's syndrome
          Hunter's syndrome

277.6 Other deficiencies of circulating enzymes

          Alpha 1-antitrypsin deficiency Hereditary angioedema

277.8 Other

277.9 Unspecified

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