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2009 ICD-9-CM

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277 Other and unspecified disorders of metabolism
277.0 Cystic fibrosis
Fibrocystic disease of the pancreas
Mucoviscidosis
 eicd10
277.1 Disorders of porphyrin metabolism
Hematoporphyria
Hematoporphyrinuria
Hereditary coproporphyria
Porphyria
Porphyrinuria
Protocoproporphyria
Protoporphyria
Pyrroloporphyria
 eicd10
277.2 Other disorders of purine and pyrimidine metabolism
Hypoxanthine-guanine-phosphoribosyltransferase deficiency [HG-PRT deficiency]
Lesch-Nyhan syndrome
Xanthinuria
Excludes:
gout (274.0-274.9)
orotic aciduric anemia (281.4)
 eicd10
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)
 eicd10
277.5 Mucopolysaccharidosis
Gargoylism
Hunter's syndrome
Hurler's syndrome
Lipochondrodystrophy
Maroteaux-Lamy syndrome
Morquio-Brailsford disease
Osteochondrodystrophy
Sanfilippo's syndrome
Scheie's syndrome
 eicd10
277.6 Other deficiencies of circulating enzymes
Hereditary angioedema
 eicd10
277.7 Dysmetabolic syndrome X
Use additional code for associated manifestation, such as:
cardiovascular disease (414.00-414.07)
obesity (278.00-278.01)
 eicd10
277.9 Unspecified disorder of metabolism
Enzymopathy NOS
 
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