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  5. Disorders of porphyrin and bilirubin metabolism (E80)

Disorders of porphyrin and bilirubin metabolism (E80)

The ICD-10 code section E80 covers disorders related to the metabolism of porphyrins and bilirubin, essential compounds in the body’s processing of heme. These codes are used to classify various inherited and acquired metabolic conditions including specific porphyrias and bilirubin metabolism disorders.

This section includes specific codes such as E80.0 for hereditary erythropoietic porphyria, also known by synonyms like erythropoietic protoporphyria or ferrochelatase deficiency, which affect the red blood cell pathway. E80.1 represents porphyria cutanea tarda, a hepatic porphyria variant marked by skin sensitivity and photosensitivity. Other notable codes include E80.21 for acute intermittent porphyria, a condition stemming from porphobilinogen deaminase deficiency, and E80.4 for Gilbert syndrome, a common inherited disorder affecting bilirubin metabolism often causing mild jaundice. Additionally, E80.5 covers Crigler-Najjar syndrome, a more severe inherited bilirubin disorder. These codes help medical coders link clinical diagnoses such as porphyria, hyperbilirubinemia, or neonatal jaundice to the correct ICD-10 classification, ensuring precise documentation of these complex metabolic disorders.

Instructional Notations

Includes

This note appears immediately under a three character code title to further define, or give examples of, the content of the category.

  • defects of catalase and peroxidase

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Crigler-Najjar Syndrome

A familial form of congenital hyperbilirubinemia transmitted as an autosomal recessive trait. It is characterized by icterus and brain damage caused by a glucuronyl transferase deficiency in the liver and faulty bilirubin conjugation.

Gilbert Disease

A benign familial disorder, transmitted as an autosomal dominant trait. It is characterized by low-grade chronic hyperbilirubinemia with considerable daily fluctuations of the bilirubin level.

Hyperbilirubinemia

A condition characterized by an abnormal increase of BILIRUBIN in the blood, which may result in JAUNDICE. Bilirubin, a breakdown product of HEME, is normally excreted in the BILE or further catabolized before excretion in the urine.

Hyperbilirubinemia, Hereditary

Inborn errors of bilirubin metabolism resulting in excessive amounts of bilirubin in the circulating blood, either because of increased bilirubin production or because of delayed clearance of bilirubin from the blood.

Hyperbilirubinemia, Neonatal

Accumulation of BILIRUBIN, a breakdown product of HEME PROTEINS, in the BLOOD during the first weeks of life. This may lead to NEONATAL JAUNDICE. The excess bilirubin may exist in the unconjugated (indirect) or the conjugated (direct) form. The condition may be self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) or pathological with toxic levels of bilirubin.

Jaundice, Chronic Idiopathic

A benign, autosomally recessive inherited hyperbilirubinemia characterized by the presence of a dark pigment in the centrilobular region of the liver cells. There is a functional defect in biliary excretion of bilirubin, cholephilic dyes, and porphyrins. Affected persons may be asymptomatic or have vague constitutional or gastrointestinal symptoms. The liver may be slightly enlarged, and oral and intravenous cholangiography fails to visualize the biliary tract.

Porphyria Cutanea Tarda

An autosomal dominant or acquired porphyria due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in the LIVER. It is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. Type I is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. Type II is the familial form.