icd 9 code for porphyria

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What is unspecified porphyria?

A disease characterized by the presence of large amounts of porphyrins in the blood and urine.

What is the ICD 10 code for porphyria?

Acute intermittent (hepatic) porphyria E80. 21 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2022 edition of ICD-10-CM E80. 21 became effective on October 1, 2021.

What is the most common porphyria?

The most common type of cutaneous porphyria—and the most common type of porphyria overall—is porphyria cutanea tarda, which affects about 5 to 10 out of every 100,000 people. The most common type of porphyria in children is a cutaneous porphyria called erythropoietic protoporphyria.

What kind of genetic disorder is porphyria?

Porphyria (por-FEAR-e-uh) refers to a group of disorders that result from a buildup of natural chemicals that produce porphyrin in your body. Porphyrins are essential for the function of hemoglobin — a protein in your red blood cells that links to porphyrin, binds iron, and carries oxygen to your organs and tissues.

What is the ICD-10 code for porphyria cutanea tarda?

ICD-10 code: E80. 1 Porphyria cutanea tarda | gesund.bund.de.

What causes acute intermittent porphyria?

Acute intermittent porphyria (AIP) is one of the liver (hepatic) porphyrias. AIP is caused by low levels of porphobilinogen deaminase (PBGD), an enzyme also often called hydroxymethylbilane synthase.

What are the 8 types of porphyria?

The specific names of the eight types of porphyrias are:Delta-aminolevulinate-dehydratase deficiency porphyria.Acute intermittent porphyria.Hereditary coproporphyria.Variegate porphyria.Congenital erythropoietic porphyria.Porphyria cutanea tarda.Hepatoerythropoitic porphyria.Erythropoietic protoporphyria.

Is porphyria an autoimmune disease?

The aetiology of porphyria cutanea tarda (PCT) has not been elucidated, but the possibility of an autoimmune mechanism has been proposed. We report a case of an unknown clinical combination of PCT with autoimmune hypothyroidism, alopecia universalis and vitiligo with thyroid and parietal cell circulating antibodies.

What part of the body does porphyria affect?

Porphyria occurs when the body cannot convert compounds called 'porphyrins' into heme. While all tissues have heme, those that use it the most are the red blood cells, liver and bone marrow. Porphyria can affect the skin, nervous system and gastrointestinal system. More women than men are affected by porphyria.

What is trigger of porphyria?

Porphyria can be triggered by drugs (barbiturates, tranquilizers, birth control pills, sedatives), chemicals, fasting, smoking, drinking alcohol, infections, emotional and physical stress, menstrual hormones, and exposure to the sun.

Is porphyria a metabolic disorder?

General Discussion. Acute intermittent porphyria (AIP) is a rare metabolic disorder that is characterized by partial deficiency of the enzyme hydroxymethylbilane synthase (also known as porphobilinogen deaminase). This enzyme deficiency can result in the accumulation of porphyrin precursors in the body.

Why is porphyria called the vampire disease?

Porphyria cutanea tarda (PCT) is a type of porphyria or blood disorder that affects the skin. PCT is one of the most common types of porphyria. It's sometimes referred to colloquially as vampire disease. That's because people with this condition often experience symptoms following exposure to sunlight.

What is porphyria cutanea tarda?

Summary. Porphyria cutanea tarda (PCT) is a rare disorder characterized by painful, blistering skin lesions that develop on sun-exposed skin (photosensitivity). Affected skin is fragile and may peel or blister after minor trauma. Liver abnormalities may also occur.

What is AIP disease?

Acute intermittent porphyria (AIP) is a rare metabolic disorder that is characterized by partial deficiency of the enzyme hydroxymethylbilane synthase (also known as porphobilinogen deaminase). This enzyme deficiency can result in the accumulation of porphyrin precursors in the body.

What is the ICD 10 code for dehydration?

ICD-10 code E86. 0 for Dehydration is a medical classification as listed by WHO under the range - Endocrine, nutritional and metabolic diseases .

What is the ICd 10 code for porphyria?

It requires blood, urine and stool tests. Each type has a different treatment. It usually involves medicine, treatment with heme or drawing blood. ICD-10-CM E80.20 is grouped within Diagnostic Related Group (s) (MS-DRG v38.0): 642 Inborn and other disorders of metabolism.

Is a porphyria hereditary?

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.

How to treat cutaneous porphyrias?

Treatment of cutaneous porphyrias focuses on reducing exposure to triggers such as sunlight and reducing the amount of porphyrins in your body to help eliminate your symptoms. This may include: Periodically drawing blood (phlebotomy) to reduce the iron in your body, which decreases porphyrins.

What tests are needed for porphyria?

Tests include a combination of blood, urine or stool testing. More tests may be needed to confirm the type of porphyria you have. Genetic testing and counseling may be recommended in the family of a person with porphyria.

What kind of doctor would you see for porphyria?

However, because porphyria can be difficult to diagnose, you may be referred to a doctor who specializes in blood disorders (hematologist). Here's some information to help you get ready, and what to expect from your doctor.

Is porphyria a rare disease?

Many signs and symptoms of porphyria are similar to those of other more common diseases. Also, because porphyria is rare, it can be more difficult to diagnose. Lab tests are required to make a definitive diagnosis of porphyria and to determine which form of the disease you have. Different tests are performed depending on the type ...

Can porphyria be cured?

Coping and support. Porphyria is considered a chronic illness, as the underlying cause can't be cured. However, porphyria usually can be managed by treatment and lifestyle changes so that you can live a full and healthy life.

Is givlaari safe for hepatic porphyria?

In 2019, the FDA approved givosiran (Givlaari) as a monthly injection for adults with acute hepatic porphyria to reduce the number of porphyria attacks. But it’s important to discuss safety information and potential serious side effects with your doctor.

What is the first line of screening for acute porphyria?

The first-line screening test is measurement of urinary porphobilinogen (PBG). PBG is markedly increased in all patients during acute porphyria attacks and not markedly elevated in other medical conditions that can present with similar symptoms. Therefore, this test is both sensitive and specific for diagnosing an acute porphyria. An exception is ADP, in which ALA is elevated but not PBG. However, the condition is not usually under consideration because of its extreme rarity.

Is ADP elevated in porphyria?

Therefore, this test is both sensitive and specific for diagnosing an acute porphyria. An exception is ADP, in which ALA is elevated but not PBG. However, the condition is not usually under consideration because of its extreme rarity. Measurement of PBG is often combined with ALA and total urine porphyrins.

Can urinary porphyrins increase with ALA?

However, mild increases in urinary porphyrins can occur in other medical conditions and is therefore much less specific than increases in ALA and PBG.

What is the ICD code for porphyria?

E80.29 is a billable ICD code used to specify a diagnosis of other porphyria. A 'billable code' is detailed enough to be used to specify a medical diagnosis.

What is the approximate match between ICd9 and ICd10?

This is the official approximate match mapping between ICD9 and ICD10, as provided by the General Equivalency mapping crosswalk. This means that while there is no exact mapping between this ICD10 code E80.29 and a single ICD9 code, 277.1 is an approximate match for comparison and conversion purposes.