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2025 ICD-10-CM code E80.6

Other disorders of bilirubin metabolism, including Dubin-Johnson syndrome and Rotor syndrome.

This code should be used when the specific disorder of bilirubin metabolism is not represented by another, more specific code.It should not be used for transient neonatal jaundice or conditions classified elsewhere.

Medical necessity is established by the presence of signs, symptoms, and laboratory findings consistent with a disorder of bilirubin metabolism.The documentation should support the need for diagnostic testing and any subsequent treatment or management of the condition.

Diagnosis involves evaluating patient history, physical examination, and symptoms such as jaundice, abdominal pain, lethargy, and muscle weakness. Laboratory tests include blood and urine bilirubin levels, liver function tests, genetic testing, and enzyme assays. Imaging studies like abdominal X-rays, ultrasound, CT, and MRI may be conducted.Liver biopsy may also be necessary.

In simple words: This code represents a group of inherited conditions that affect how the body processes bilirubin, a byproduct of red blood cell breakdown.These conditions can cause jaundice (yellowing of skin and eyes) due to a buildup of bilirubin in the body.Examples include Dubin-Johnson syndrome and Rotor syndrome.

This code encompasses inherited disorders affecting bilirubin metabolism, excluding those specifically classified elsewhere. These disorders arise from deficiencies or abnormalities in enzymes responsible for converting bilirubin to urobilin or stercobilin, leading to bilirubin accumulation in the blood. Examples include Dubin-Johnson syndrome and Rotor syndrome.

Example 1: A patient presents with mild jaundice, fatigue, and occasional abdominal pain.Blood tests reveal elevated bilirubin levels. After further investigation, including genetic testing and a liver biopsy, a diagnosis of Dubin-Johnson syndrome is confirmed., An asymptomatic patient undergoes routine blood work that shows slightly elevated bilirubin.Further evaluation, including imaging and a nicotinic acid provocation test, leads to a diagnosis of Rotor syndrome., A patient presents with jaundice and other symptoms that are initially thought to be indicative of a more common liver disorder. After extensive testing and ruling out other conditions, a diagnosis of a rare, inherited bilirubin metabolism disorder, not otherwise specified, is made.

Documentation should include detailed patient history, physical examination findings, results of laboratory tests (bilirubin levels, liver function tests, genetic tests, enzyme assays), imaging reports (if applicable), and liver biopsy results (if performed).The specific type of bilirubin metabolism disorder should be clearly documented.

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