2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Other And Unspecified Disorders Of Metabolism 277.* > 2006 ICD-9-CM Diagnosis 277.1 Disorders of porphyrin metabolism View the most recent version of ICD-9-CM 277.1 A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues. An autosomal dominant porphyria that is due to a deficiency of FERROCHELATASE (heme synthetase) in both the LIVER and the BONE MARROW, the last enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include mainly neurological symptoms, rarely cutaneous lesions, and elevated levels of protoporphyrin and COPROPORPHYRINS in the feces. An autosomal dominant porphyria that is due to a deficiency of protoporphyrinogen oxidase (EC 1.3.3.4) in the LIVER, the seventh enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, COPROPORPHYRINS and protoporphyrinogen. An autosomal dominant porphyria that is due to a deficiency of COPROPORPHYRINOGEN OXIDASE in the LIVER, the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINIC ACID and COPROPORPHYRINS. An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine. 277.1 is a specific code that can be used to specify a diagnosis 277.1 contains 22 index entries View the ICD-9-CM Volume 1 277.* hierarchy Alternate Terminology Hematoporphyria Hematoporphyrinuria Hereditary coproporphyria Porphyria Porphyrinuria Protocoproporphyria Protoporphyria Pyrroloporphyria  Index entries containing 277.1: Coproporphyria, hereditary 277.1 Disease, diseased - see also Syndrome Günther's (congenital erythropoietic porphyria) 277.1 Disorder - see also Disease metabolism NEC 277.9 porphyrin 277.1 porphyrin metabolism 277.1 Günther's disease or syndrome (congenital erythropoietic porphyria) 277.1 Hematoporphyria (acquired) (congenital) 277.1 Hematoporphyrinuria (acquired) (congenital) 277.1 Photocoproporphyria 277.1 Porphyria (acute) (congenital) (constitutional) (erythropoietic) (familial) (hepatica) (idiopathic) (idiosyncratic) (intermittent) (latent) (mixed hepatic) (photosensitive) (South African genetic) (Swedish) 277.1 acquired 277.1 cutaneatarda hereditaria 277.1 symptomatica 277.1 due to drugs correct substance properly administered 277.1 overdose or wrong substance given or taken 977.9 specified drug - see Table of Drugs and Chemicals secondary 277.1 toxic NEC 277.1 variegata 277.1 Porphyrinuria (acquired) (congenital) (secondary) 277.1 Porphyruria (acquired) (congenital) 277.1 Protocoproporphyria 277.1 Protoporphyria (erythrohepatic) (erythropoietic) 277.1 Pyrroloporphyria 277.1 Syndrome - see also Disease Günther's (congenital erythropoietic porphyria) 277.1