Key Highlights:
- Deficiency of Interleukin-1 receptor antagonist or DIRA is an autosomal recessive disorder.
- This disorder is caused by mutations in the gene named IL1RN, which leads to loss of production and function of the IL-1Ra.
- A deficiency of Interleukin-1 receptor antagonists typically presents at or around birth and can escalate to critical inflammation.
- Clinical features of deficiency of interleukin-1 receptor antagonist include skin rash, bone pain and deformity, swelling of bone tissue, and swelling of the tissues around the bones.
- The disorder is often misdiagnosed as infectious osteomyelitis with pustulosis and systemic inflammation, leading to unsuccessful treatment with antibiotics.
- Deficiency of Interleukin-1 receptor antagonists has a 25 percent mortality rate, and at present, there are no known patients who, in absence of treatment, have reached adulthood.
Deficiency of Interleukin-1 receptor antagonist is an ultra-rare, potentially dangerous, or life-threatening autoinflammatory pediatric disease. As DIRA is an autoinflammatory disease, it mainly affects the body`s immune system. Instead of combating viruses, bacteria, and infections, the immune cells start attacking the child's own body by mistake. These attacks can be responsible for causing very serious and hazardous inflammation.
This autoinflammatory pediatric disease (DIRA) is developed due to mutations, or changes, that have occurred in a child's genes prior to birth. Signs and symptoms of the disease typically start within the initial 8 weeks after a child is born.
Symptoms can include swelling of bone tissue, bone pain, and deformity, a skin rash that can cover most of the body, and elevated C-reactive protein levels.
DIRA Symptoms:
- Rash
- Fever
- Bone Pain
- Deformity
- Joint Pain
- Joint Swelling
- Redness and Pain in Eyes
- Lymph Node Enlargement
Diagnosis: DIRA can be tough to diagnose. As it is so rare, health specialists are not used to seeing patients who have a deficiency of interleukin-1 receptor antagonists. It can take several visits to different health specialists before DIRA is diagnosed.
How is DIRA Treated:
Deficiency of Interleukin-1 Receptor Antagonist (DIRA) can be treated with an IL-1 blocking medicine, like Kineret (anakinra injection). This drug is designed to block the activity of interleukin-1 (IL-1), a protein in the body that typically causes inflammation. By supplementing the interleukin-1 blocking protein that deficiency of interleukin-1 receptor antagonist patients lack, Kineret injection 100 mg can help relieve painful and sometimes dangerous signs and symptoms.
In clinical trials, Kineret (anakinra 100 mg) improved or stabilized the below-mentioned DIRA symptoms:
- Skin rash
- Swelling of bone tissue
- Bone pain and deformity
Kineret 100 mg has been shown to be promising for the treatment of deficiency of interleukin-1 receptor antagonists (DIRA).
Kineret Dosage for DIRA:
A health specialist will determine how much Kineret to take each time (exact dosage). For children with a Deficiency of Interleukin-1 Receptor Antagonist (DIRA), once-daily injections are typically recommended.
Side Effects of Kineret:
Children treated with Kineret can experience side effects but, in clinical studies, most reported side effects were minimal and no patient had to terminate treatment with Kineret 100 mg due to side effects. The most commonly reported serious side effect was an infection.
The most commonly reported side effects in the Kineret trial were:
- Rash
- Fever
- Flu-like symptoms
- Stomach-flu-like symptoms
- Upper respiratory tract infections
Where Do I Get Anakinra (Kineret):
Anakinra can be made available from India. The medication comes under the brand name Kineret. It is supplied as 100 mg/0.67 mL solution for subcutaneous injection. Kindly contact us via TOLL-FREE: 1800-889-1064 to buy Kineret 100 mg injection in the approved quantity. We are a WHO GDP and ISO 9001 2015 Certified Pharmaceutical Wholesaler/ Supplier/ Exporter/ Importer from India of Kineret 100 mg injection.
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