Lometapai has been approved for adjuvant therapy of HoFH in multiple countries and has significant clinical value.

1、 Drug name and main ingredients

1. Common name: Lomitapide

2. Product Name: JUXTAPID ™

3. Dosage form: Oral hard capsules

4. Main ingredients: Each capsule contains Lometapine Methanesulfonate (equivalent to 5mg, 10mg, or 20mg of base)

5. Accessories: pre gelatinized starch, microcrystalline cellulose, lactose monohydrate, silica, magnesium stearate; The capsule shell contains gelatin, titanium dioxide, iron oxide red (5mg/10mg specification), and black printing ink.

2、 Indications

1. Homozygous familial hypercholesterolemia (HoFH): Used as an adjuvant medication for low-fat diet and other lipid-lowering treatments (including LDL plasma exchange) to reduce low-density lipoprotein cholesterol (LDL-C), total cholesterol (TC), apolipoprotein B (apoB), and non high-density lipoprotein cholesterol (non-HDL-C).

2. Limitations: The safety and efficacy of non HoFH patients have not been established, and the impact on cardiovascular outcomes is not yet clear.

3、 Specifications and characteristics

1. Specifications: 5mg (orange/orange capsules), 10mg (orange/white capsules), 20mg (white/white capsules), 28 capsules per bottle.

2. Appearance: Opaque hard capsule with black label "A733" and corresponding dosage printed on it.

4、 Usage and dosage

1. Initial treatment: Take 5mg once a day on an empty stomach (at least 2 hours after meals) and swallow the whole pill.

2. Dose titration: Gradually increase to 10mg (≥ 2 weeks), 20mg (≥ 4 weeks), 40mg (≥ 4 weeks) according to tolerance, with a maximum of 60mg/day.

3. Omission treatment: skip the missed dose and take it according to the original plan the next day; If the interruption exceeds one week, re evaluation is required.

4. Nutritional supplementation: Daily supplementation of vitamin E (400IU) and essential fatty acids (such as linoleic acid, EPA/DHA) is required.

5、 Dose adjustment

1. Abnormal liver function: When ALT/AST is ≥ 3 times the upper limit of normal (ULN), it needs to be reduced or paused; Stop medication when the ULN is ≥ 5 times.

2. Special populations:

The dosage for end-stage renal disease dialysis patients or those with mild liver injury (Child Pugh A) should not exceed 40mg/day.

Moderate to severe liver injury (Child Pugh B/C) is contraindicated.

6、 Medication precautions

1. Monitoring requirements: Baseline and regular testing of ALT/AST, alkaline phosphatase, and total bilirubin; Pregnant women need contraception.

2. Dietary restrictions: Strictly follow a low-fat diet (fat supply<20%) and avoid alcohol (≤ 1 cup per day).

3. Drug interactions:

Do not use strong/medium acting CYP3A4 inhibitors in combination (such as clarithromycin and itraconazole).

The dosage of weak CYP3A4 inhibitors (such as atorvastatin) should not exceed 30mg/day when used in combination.

Warfarin requires close monitoring of INR.

7、 Medication for special populations

1. Pregnant women: prohibited (animal display teratogenicity).

2. Breastfeeding period: Stop taking medication or breastfeeding.

3. Children: Safety has not been established.

4. Elderly people: need to be cautious and consider liver and kidney function decline.

8、 Adverse reactions

1. Common (≥ 28%): Diarrhea (79%), nausea (65%), vomiting (34%), indigestion (38%), abdominal pain (34%).

2. Serious but rare: hepatotoxicity (elevated ALT/AST, hepatic steatosis), gastrointestinal bleeding, myopathy (in combination with statins).

9、 Contraindications

1. Pregnant or planned pregnant women.

2. Combination use of strong/medium acting CYP3A4 inhibitors.

3. Moderate to severe liver injury or active liver disease.

10、 Drug interactions

1. CYP3A4 inhibitor: significantly increases the blood concentration of Lometapine (strong inhibitors can increase by 27 times).

2. Statins: Simvastatin dosage needs to be halved (Lometapine doubles its exposure).

3. P-gp substrate: may increase the absorption of drugs such as digoxin, and reduction should be considered.

11、 Storage method

Store at room temperature (20-25 ° C), avoid moisture and light, and keep the container sealed.

12、 Manufacturer

Manufacturer: Aegerion Pharmaceuticals, Inc. (USA)

Note: If there is persistent diarrhea, jaundice, or severe abdominal pain during medication, seek medical attention immediately. Specific medication needs to comply with the JUXTAPIDREAMS plan requirements.

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