Specific code 2014 ICD-9-CM Diagnosis Code 277.89
Other specified disorders of metabolism
  • Billable Code
  • Short description: Metabolism disorder NEC.
  • ICD-9-CM 277.89 will be replaced by an equivalent ICD-10-CM code (or codes) when the United States transitions from ICD-9-CM to ICD-10-CM on October 1, 2015.
  • ICD-9-CM 277.89 is a billable medical code that can be used to specify a diagnosis on a reimbursement claim.
Disease Synonyms
  • Acatalasemia
  • Acatalasia
  • Acatalasia (Takahara's disease)
  • Acute histiocytosis
  • Adenosine deaminase overproduction
  • Alpha-N-acetylgalactosaminidase deficiency
  • Anemia due to disorders of nucleotide metabolism
  • Autosomal variant form of transthyretin
  • Chronic histiocytosis
  • Combined complex deficiencies
  • Combined deficiency of long chain 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase
  • Defect in post-translational modification of lysosomal enzymes
  • Defective biosynthesis
  • Defective osmoregulation
  • Deficiency in enzyme complexes of mitochondrial respiratory chain
  • Deficiency of amylopectin 6-glucanohydrolase
  • Deficiency of glycosidase
  • Deficiency of halogenase
  • Deficiency of isobutyryl-CoA dehydrogenase
  • Deficiency of limit dextrinase
  • Deficiency of protease
  • Deletion and duplication of mitochondrial DNA
  • Depletion of mitochondrial DNA
  • Disorder of beta alanine, carnosine AND/OR homocarnosine metabolism
  • Disorder of chromium metabolism
  • Disorder of glucose regulation
  • Disorder of glycoprotein metabolism
  • Disorder of glycosaminoglycan metabolism
  • Disorder of iodine metabolism
  • Disorder of lysosomal enzyme
  • Disorder of mitochondrial respiratory chain complexes
  • Disorder of organic acid metabolism
  • Disorder of osmoregulation
  • Disorder of oxygen transport
  • Disorder of peroxisomal function
  • Disorder of protein metabolism
  • Disorder of pyruvate metabolism and mitochondrial respiratory chain
  • Disorder of sialic acid metabolism
  • Disorder of steroid metabolism
  • Disorder of strontium metabolism
  • Disorder with defective osteoid mineralization
  • Disorders of pyruvate metabolism and gluconeogenesis
  • Drug resistance to insulin
  • Enterokinase deficiency
  • Eosinophilic granuloma of bone
  • Extensive metabolizer due to cytochrome p450 CYP2D6 variant
  • Familial sea-blue histiocytosis
  • Fast acetylator due to N-acetyltransferase enzyme variant
  • Fat overload syndrome
  • Glutaric aciduria, type 1
  • Glutathione S-transferase deficiency
  • Hand-Sch�ller-Christian disease
  • Hand-Schüller-Christian disease
  • Histiocytic syndrome
  • Histiocytosis
  • Histiocytosis X syndrome
  • Histiocytosis, acute
  • Histiocytosis, chronic
  • Histiocytosis, undetermined cell
  • Histiocytosis, unifocal langerhans-cell
  • Homocarnosinase deficiency
  • Hyper-beta-carnosinemia
  • Hyperimidodipeptiduria due to proline dipeptidase deficiency
  • Impaired oxygen extraction
  • Inborn error of pyruvate metabolism
  • Increased auto-oxidation
  • Increased fluid pressure
  • Increased oxygen demand
  • Inherited metabolic disorder of nervous system
  • Intermediate metabolizer due to cytochrome p450 CYP2D6 variant
  • Langerhans cell histiocytosis
  • Langerhans cell histiocytosis - Hashimoto-Pritzker type
  • Langerhans cell histiocytosis of lung
  • Langerhans cell histiocytosis, disseminated (clinical)
  • Langerhans cell histiocytosis, disseminated (disorder)
  • Langerhans cell histiocytosis, multifoc unisystem
  • Langerhans cell histiocytosis, multifocal and unisystemic
  • Langerhans cell histiocytosis, unifocal
  • Langerhans cell histiocytosis, unifocal (clinical)
  • Lysosomal enzyme defect
  • Lysosomal enzyme disorder
  • Macroamylasemia
  • Methylene THF reductase deficiency AND homocystinuria
  • Mucinous histiocytosis of the colon
  • Organic acid metabolism disorder
  • Pancreatic colipase deficiency
  • Pancreatic triacylglycerol lipase deficiency
  • Phosphoenolpyruvate carboxykinase deficiency
  • Poor metabolizer due to cytochrome p450 CYP2C19 variant
  • Poor metabolizer due to cytochrome p450 CYP2C9 variant
  • Poor metabolizer due to cytochrome p450 CYP2D6 variant
  • Pseudoinfantile Refsum's disease
  • Pyruvate carboxylase deficiency
  • Pyruvate dehydrogenase complex deficiency
  • Refeeding syndrome
  • Sepiapterin reductase deficiency
  • Sinus histiocytosis with massive lymphadenopathy
  • Slow acetylator due to N-acetyltransferase enzyme variant
  • Solitary reticulohistiocytoma
  • Tetrahydrobiopterin synthesis defect
  • Trypsinogen deficiency
  • Tumor lysis syndrome
  • UGT1A1*28 polymorphism
  • Ultrarapid metabolizer due to cytochrome p450 CYP2D6 variant
  • Undetermined cell histiocytosis
  • Unifocal langerhans-cell histiocytosis
Clinical Information
  • A multifocal, unisystem form of langerhans-cell histiocytosis. There is involvement of multiple sites in one organ system, most frequently the bone. Patients are usually young children presenting with multiple destructive bone lesions.
  • A neoplastic proliferation of langerhans cells which contain birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, letterer-siwe disease, and hand-schuller-christian disease. The clinical course is generally related to the number of organs affected at presentation. (who, 2001)
  • Group of disorders of histiocyte proliferation which includes letterer-siwe disease; hand-schueller-christian syndrome; and eosinophilic granuloma; langerhans cells are components of the lesions.
277.89 Excludes
  • histiocytosis:
    • acute differentiated progressive (202.5)
    • adult pulmonary Langerhans cell (516.5)
    • X, acute (progressive) (202.5)
Applies To
  • Hand-Schüller-Christian disease
  • Histiocytosis (acute) (chronic)
  • Histiocytosis X (chronic)
Convert to ICD-10-CM: 277.89 converts approximately to:
  • 2014 ICD-10-CM C96.5 Multifocal and unisystemic Langerhans-cell histiocytosis
    Or:
  • 2014 ICD-10-CM C96.6 Unifocal Langerhans-cell histiocytosis
    Or:
  • 2014 ICD-10-CM E71.39 Other disorders of fatty-acid metabolism
    Or:
  • 2014 ICD-10-CM E80.3 Defects of catalase and peroxidase
    Or:
  • 2014 ICD-10-CM E88.89 Other specified metabolic disorders
    Or:
  • 2014 ICD-10-CM E88.9 Metabolic disorder, unspecified
ICD-9-CM Volume 2 Index entries containing back-references to 277.89:
  • Acatalasemia 277.89
  • Acatalasia 277.89
  • Christian's syndrome (chronic histiocytosis X) 277.89
  • Disease, diseased - see also Syndrome
    • Christian's (chronic histiocytosis X) 277.89
    • Erdheim-Chester (ECD) 277.89
    • Hand-Schüller-Christian (chronic histiocytosis X) 277.89
    • Schüller-Christian (chronic histiocytosis X) 277.89
  • Disorder - see also Disease
  • ECD (Erdheim-Chester disease) 277.89
  • Eosinophilic - see also condition
    • granuloma (bone) 277.89
  • Erdheim-Chester disease (ECD) 277.89
  • Granuloma NEC 686.1
    • bone (see also Osteomyelitis) 730.1
      • eosinophilic 277.89
      • from residual foreign body 733.99
    • eosinophilic 277.89
      • bone 277.89
      • lung 277.89
      • oral mucosa 528.9
    • lipid 277.89
    • lipoid 277.89
    • lung (infectious) (see also Fibrosis, lung) 515
      • coccidioidal 114.4
      • eosinophilic 277.89
    • reticulohistiocytic 277.89
  • Granulomatosis NEC 686.1
    • lipoid 277.89
  • Hand-Schüller-Christian disease or syndrome (chronic histiocytosis x) 277.89
  • Histiocytosis (acute) (chronic) (subacute) 277.89
    • acute differentiated progressive (M9722/3) 202.5
    • adult pulmonary Langerhans cell (PLCH) 516.5
    • cholesterol 277.89
    • essential 277.89
    • lipid, lipoid (essential) 272.7
    • lipochrome (familial) 288.1
    • malignant (M9720/3) 202.3
    • non-Langerhans cell 277.89
    • polyostotic sclerosing 277.89
    • X (chronic) 277.89
      • acute (progressive) (M9722/3) 202.5
  • Metabolism disorder 277.9
    • specified type NEC 277.89
  • Reticulohistiocytoma (giant cell) 277.89
  • Schüller-Christian disease or syndrome (chronic histiocytosis X) 277.89
  • Syndrome - see also Disease
    • Christian's (chronic histiocytosis X) 277.89
    • Erdheim-Chester 277.89
    • Hand-Schüller-Christian (chronic histiocytosis X) 277.89
    • Schüller-Christian (chronic histiocytosis X) 277.89
  • Xanthoma(s), xanthomatosis 272.2
    • craniohypophyseal 277.89
ICD-9-CM codes are used in medical billing and coding to describe diseases, injuries, symptoms and conditions. ICD-9-CM 277.89 is one of thousands of ICD-9-CM codes used in healthcare. Although ICD-9-CM and CPT codes are largely numeric, they differ in that CPT codes describe medical procedures and services. Can't find a code? Start at the root of ICD-9-CM, check the 2014 ICD-9-CM Index or use the search engine at the top of this page to lookup any code.