277: Other and unspecified disorders of metabolism
277.8 Other specified disorders of metabolism
Carnitine deficiency due to:
Hemodialysis
Valproic acid therapy
Carnitine palmitoyltransferase deficiencies (CPT1, CPT2)
Glutaric aciduria type II (type IIA, IIB, IIC)
Long chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHAD)
Long chain/very long chain acyl CoA dehydrogenase deficiency (LCAD, VLCAD)
Medium chain acyl CoA dehydrogenase deficiency (MCAD)
Short chain acyl CoA dehydrogenase deficiency (SCAD)
Excludes:
primary carnitine deficiency (277.81)
Adrenomyeloneuropathy
Neonatal adrenoleukodystrophy
Rhizomelic chrondrodysplasia punctata
X-linked adrenoleukodystrophy
Zellweger syndrome
Excludes:
infantile Refsum disease (356.3)
Kearns-Sayre syndrome
Mitochondrial Encephalopathy, Lactic Acidosis and Stroke-like episodes (MELAS syndrome)
Mitochondrial Neurogastrointestinal Encephalopathy syndrome (MNGIE)
Myoclonus with Epilepsy and with Ragged Red Fibers (MERRF syndrome)
Neuropathy, Ataxia and Retinitis Pigmentosa (NARP syndrome)
Use additional code for associated conditions
Excludes:
disorders of pyruvate metabolism (271.8)
Leber's optic atrophy (377.16)
Leigh's subacute necrotizing encephalopathy (330.8)
Reye's syndrome (331.81)
Spontaneous tumor lysis syndrome
Tumor lysis syndrome following antineoplastic drug therapy
Use additional E code to identify cause, if drug-induced
Hand-Schüller-Christian disease
Histiocytosis (acute) (chronic)
Histiocytosis X (chronic)
Excludes:
histiocytosis:
acute differentiated progressive (202.5)
adult pulmonary Langerhans cell (516.5)
X, acute (progressive) (202.5)

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