GAMIFANT 5mg/ml Price

Overview

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This medicine contains an important and useful components, as it consists of
Emapalumabis available in the market in concentration

Name

Bosentan

Precaution

Bosentan is a medication used primarily to treat pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). It should be used with caution in patients with liver impairment, as bosentan can cause hepatotoxicity, leading to liver injury. Regular liver function tests are essential during treatment to monitor for any signs of hepatic damage. Additionally, bosentan can cause low blood pressure (hypotension), so it should be used carefully in patients who are prone to low blood pressure or are already on antihypertensive therapy. Patients with a history of heart failure or those taking other medications that can affect heart function should be monitored closely. Women of childbearing age must be aware that bosentan is teratogenic, meaning it can cause birth defects and should not be used during pregnancy. Female patients must use effective contraception during treatment and for at least one month after discontinuation of the drug. As bosentan is primarily metabolized by the liver, drug interactions with other medications that affect liver enzymes, particularly those metabolized by CYP450, need to be considered to avoid potential interactions.

Indication

Bosentan is indicated for the treatment of pulmonary arterial hypertension (PAH), a condition characterized by high blood pressure in the arteries of the lungs, which can lead to right heart failure. It is also approved for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH), a form of PAH caused by chronic blood clots in the pulmonary arteries. Bosentan works as an endothelin receptor antagonist, helping to relax and dilate the blood vessels in the lungs, thus reducing pulmonary vascular resistance and improving blood flow. This results in decreased symptoms such as shortness of breath and fatigue, and may improve exercise capacity and quality of life in individuals with PAH. Off-label uses may include treatment of conditions with similar pathophysiology, but its primary indication is for PAH and CTEPH management. Clinical guidelines and studies support its use as a long-term treatment option for these conditions, particularly when other therapies, such as prostacyclin analogs or phosphodiesterase inhibitors, are not sufficient.

Contra indication

Bosentan is contraindicated in patients with moderate to severe liver dysfunction, as it can exacerbate liver injury, potentially leading to liver failure. Patients with known hypersensitivity to bosentan or any of its components should avoid using the drug. It is also contraindicated during pregnancy due to its teratogenic effects, as it can cause serious birth defects. Bosentan should not be used in patients who are concurrently taking cyclosporine, a medication that can significantly increase bosentan levels in the blood, increasing the risk of toxicity. Women of childbearing potential must use reliable contraception during treatment and for one month after discontinuing bosentan. In addition, it should not be administered to patients with severe renal impairment without close monitoring, as there is limited information on the safety and efficacy in this population.

Side Effect

The side effects of bosentan can range from mild to severe, with the most common being headaches, flushing, and nasal congestion. These effects are often related to the vasodilatory actions of the drug, as it works to widen blood vessels. Liver enzyme elevations (hepatotoxicity) are a more serious potential side effect, and routine liver function tests are required during treatment to monitor for any liver damage. Other serious side effects include anemia, which can occur due to the drug’s effect on red blood cells, and peripheral edema (swelling of the legs or ankles), which can be a result of fluid retention. Rare but serious side effects include liver failure, severe hypotension, and teratogenicity (birth defects in pregnant women). If any signs of liver injury (e.g., dark urine, jaundice, or persistent fatigue) occur, treatment should be discontinued, and liver function should be closely monitored. Some individuals may also experience dizziness, nausea, or gastrointestinal upset. If any serious side effects arise, such as unexplained swelling, severe fatigue, or signs of an allergic reaction (e.g., rash or difficulty breathing), medical attention should be sought immediately.

Pregnancy Category ID

5

Mode of Action

Bosentan works by blocking the effects of endothelin-1, a peptide that plays a critical role in regulating blood vessel constriction and blood pressure. Endothelin-1 binds to endothelin receptors (ETA and ETB) on the smooth muscle cells lining the blood vessels, leading to vasoconstriction (narrowing of blood vessels) and an increase in blood pressure. By antagonizing these receptors, bosentan inhibits endothelin-1’s ability to cause constriction, thus relaxing the blood vessels and reducing pulmonary vascular resistance. This results in improved blood flow to the lungs, reduced right heart strain, and alleviation of the symptoms of pulmonary arterial hypertension (PAH), such as shortness of breath and fatigue. Additionally, by lowering pulmonary pressure, bosentan helps improve exercise capacity and quality of life in patients with PAH. Its dual action on both endothelin receptors (ETA and ETB) makes it effective in reducing the vasoconstrictive effects of endothelin while also improving endothelial function.

Interaction

Bosentan has the potential for significant drug-drug interactions, primarily because it is metabolized by the liver enzyme CYP450, specifically CYP3A4 and CYP2C9. Medications that induce these enzymes, such as rifampin or phenytoin, may reduce the effectiveness of bosentan by decreasing its blood concentration. Conversely, medications that inhibit these enzymes, such as ketoconazole or clarithromycin, can increase bosentan levels, raising the risk of side effects and liver toxicity. Cyclosporine, an immunosuppressant, can significantly increase bosentan levels and should be avoided when taking bosentan, as it may lead to serious adverse effects, including liver toxicity. Patients taking bosentan should also be cautious when using other antihypertensive medications, as bosentan can potentiate the effects of these drugs, potentially leading to hypotension. Additionally, bosentan may reduce the effectiveness of oral contraceptives, and women should be advised to use an alternative non-hormonal form of contraception during treatment. Always consult a healthcare provider to assess potential interactions with other medications before starting bosentan.

Pregnancy Category Note

Information not available

Adult Dose

The usual starting dose of bosentan for adults with pulmonary arterial hypertension (PAH) is 62.5 mg twice daily for the first four weeks. After this initial period, the dose is typically increased to 125 mg twice daily, based on patient tolerance and response. In cases of more severe PAH or patients who do not tolerate the starting dose, the dosage may be adjusted accordingly. It is important to titrate the dosage gradually and monitor for any adverse effects, particularly liver function. Bosentan should be taken orally, with or without food, and should be taken at evenly spaced intervals to maintain steady blood levels. Regular liver function tests, as well as hematological and blood pressure monitoring, are essential during treatment. If liver enzyme elevations are detected, dosage adjustments or discontinuation of bosentan may be necessary. Patients should be monitored for signs of anemia, peripheral edema, and other side effects that could require further dose modification or discontinuation.

Child Dose

Bosentan is not approved for use in children under 12 years of age, and its safety and efficacy in pediatric populations have not been well-established. In children aged 12 years and older, the dosing regimen typically mirrors that of adults, starting with 62.5 mg twice daily for the first four weeks, followed by an increase to 125 mg twice daily. As with adults, close monitoring of liver function, hemoglobin levels, and blood pressure is essential. Pediatric patients should be closely monitored for signs of adverse effects such as liver toxicity or anemia. For any use in children, a healthcare provider should be consulted to ensure the appropriate dosage and monitoring plan based on the child’s overall health condition and response to the medication.

Renal Dose

Renal impairment does not significantly affect the pharmacokinetics of bosentan, as it is primarily metabolized by the liver. Therefore, no specific dose adjustments are required for patients with mild to moderate renal impairment. However, in patients with severe renal impairment (e.g., creatinine clearance < 30 mL/min), caution should be exercised due to the potential for accumulation of the drug’s metabolites. Regular monitoring of renal function is advised in these patients, and dosage adjustments should be considered on an individual basis. Bosentan is not recommended for use in patients with end-stage renal disease or those requiring dialysis unless deemed necessary by a healthcare provider.

Administration

Information not available