OTHER METABOLIC AND IMMUNITY DISORDERS (270-279)
Use additional code to identify any associated intellectual disabilities
277 Other and unspecified disorders of metabolism
277.0: Cystic fibrosis
Fibrocystic disease of the pancreas
Mucoviscidosis
277.1: Disorders of porphyrin metabolism
Hematoporphyria
Hematoporphyrinuria
Hereditary coproporphyria
Porphyria
Porphyrinuria
Protocoproporphyria
Protoporphyria
Pyrroloporphyria
277.2: Other disorders of purine and pyrimidine metabolism
Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
Lesch-Nyhan syndrome
Xanthinuria
Excludes:
gout (274.00-274.9)
orotic aciduric anemia (281.4)
277.3: Amyloidosis
277.4: Disorders of bilirubin excretion
Hyperbilirubinemia:
congenital
constitutional
Syndrome:
Crigler-Najjar
Dubin-Johnson
Gilbert's
Rotor's
Excludes:
hyperbilirubinemias specific to the perinatal period (774.0-774.7)
277.5: Mucopolysaccharidosis
Gargoylism
Hunter's syndrome
Hurler's syndrome
Lipochondrodystrophy
Maroteaux-Lamy syndrome
Morquio-Brailsford disease
Osteochondrodystrophy
Sanfilippo's syndrome
Scheie's syndrome
277.6: Other deficiencies of circulating enzymes
Hereditary angioedema
277.7: Dysmetabolic syndrome X
Use additional code for associated manifestation, such as:
cardiovascular disease (414.00-414.07)
obesity (278.00-278.03)
277.8: Other specified disorders of metabolism
277.9: Unspecified disorder of metabolism
Enzymopathy NOS
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