结节性硬化服用喜保宁的副作用？

Patients with tuberous sclerosis often experience side effects such as fatigue, drowsiness, blurred vision, weight gain, upper respiratory tract infection, joint pain, abnormal coordination and confusion, nystagmus, weight gain, memory impairment, aggression, and diplopia. If a patient develops any of the above or other suspected side effects during treatment, they can be dealt with by adjusting the drug treatment plan, discontinuing the drug, and other measures.

tuberous sclerosis use

Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome that can present at any age and affect multiple organ systems. The disease is typically recognized in infants and children by characteristic skin lesions, seizures, and cell overgrowth or hamartomas of the heart, brain, and kidneys. Tuberous sclerosis is a genetic disease caused by mutations in the TSC1 or TSC2 genes, leading to hamartin or tuberin dysfunction, respectively. Accurate diagnosis is essential for appropriate monitoring and treatment of patients with this disease. The International Tuberous Sclerosis Consensus Group has proposed specific guidelines for diagnosis, monitoring, and management.

The treatment of tuberous sclerosis is partly symptomatic, and vigabatrin is a structural analog of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA). It is one of the few anti-epileptic drugs (AEDs) with a specific targeting mechanism. It can be produced by irreversibly inhibiting the degrading enzyme GABA-aminase, so patients with tuberous sclerosis epilepsy can be treated with vigabatrin.

Patients with tuberous sclerosis taking Vigabatrin

1. Ear diseases: tinnitus, dizziness.

2. Eye diseases: blurred vision, double vision, eye fatigue, and eye pain.

3. Gastrointestinal diseases: diarrhea, nausea, vomiting, constipation, upper abdominal pain, indigestion, stomach discomfort, and abdominal pain.

4. Systemic diseases: fatigue, gait disorder, weakness, peripheral edema, fever, chest pain.

5. Infection: nasopharyngitis, upper respiratory tract infection, influenza, urinary tract infection, bronchitis.

6. Damage: bruises, joint sprains, muscle strains, wound secretions.

7. Metabolism and nutritional disorders: increased appetite and weight gain.

8. Musculoskeletal diseases: joint pain, back pain, limb pain, myalgia, muscle twitching, and muscle spasm.

9. Nervous system diseases: headache, drowsiness, dizziness, vertigo, nystagmus, memory impairment, abnormal coordination, attention disorder, perceptual impairment, hyporeflexia, lethargy, hyperreflexia, hypoesthesia, sedation, persistent epileptic state, dysarthria, retrorectal state, and sensory loss.

10. Mental illness: depression, confusion, anxiety, depressed mood, abnormal thinking, abnormal behavior, expressive language disorder, neurosis, abnormal dreams.

serious adverse reactions

1. Permanent Vision Loss: Vigabatrin can cause permanent blindness. Because of this risk, and because when it is effective, Vigabatrin provides observable symptomatic benefits, patients should be regularly evaluated for response and ongoing need for treatment.

2. Neurotoxicity: After administration of iridosin, vacuolation, characterized by fluid accumulation and separation of the outer layer of myelin, was observed in the brain white matter tracts of adult and juvenile rats, as well as adult mice, dogs, and possibly monkeys. This damage is known as intramuscular edema (IME) and is observed in animals within the human therapeutic dose range.

3. Suicidal behavior and ideation: Antiepileptic drugs (AEDs), including Vigabatrin, increase the risk of suicidal thoughts or behaviors in patients taking these drugs for any indication. Patients receiving any AED for any indication should be monitored for the emergence or worsening of depression, suicidal thoughts or behaviors, or any unusual changes in mood or behavior.

4. Anemia: In controlled trials in North American adults, 6% of patients treated with Vigabatrin and 2% of patients treated with placebo experienced the adverse event of anemia or met criteria for potentially clinically important hematological changes, including hemoglobin, hematocrit, or RBC index. In controlled trials in the United States, hemoglobin decreased by an average of approximately 3% and 0% in patients treated with Vigabatrin and placebo, respectively, and hematocrit decreased by an average of approximately 1% in patients treated with Vigabatrin and increased by an average of approximately 1% in patients treated with placebo.

5. Drowsiness and fatigue: Vigabatrin can cause drowsiness and fatigue. Patients should be advised not to drive a car or operate other complex machinery until they are familiar with the effects of Vigabatrin on their ability to perform these activities.

How to reduce side effects using Vigabatrin

It is recommended that patients undergo cognitive, age-appropriate visual field testing at baseline and then repeated at intervals as patients continue treatment. Infants were tested at baseline, every 3 months during the first 18 months of treatment, and every 6 months thereafter.

To select patients suitable for treatment with Vigabatrin, physicians must consider the benefits of seizure reduction and improved quality of life, as well as the potential risk of developing peripheral ventricular fibrillation. The efficacy of Vigabatrin can be detected within 12 weeks of starting treatment. Before there is a significant risk of vigabatrin-induced peripheral ventricular tachycardia, clinical trials of vigabatrin for 2-3 months to assess its effectiveness appear to be minimal.

If the patient does not obtain clinical benefit from Vigabatrin within 12 weeks of starting treatment, Vigabatrin should be discontinued. If a patient experiences a meaningful reduction in seizures or becomes seizure free, then the physician and the patient or caregiver must determine whether the benefits outweigh the potential risk of developing peripheral VFD. Patients must be closely monitored for changes in visual field when initiating Vigabatrin. If spasms or seizures do not improve within 12 weeks of starting treatment, Vigabatrin should be discontinued. If spasms completely cease and seizures are controlled or meaningfully improved within 12 weeks, treatment with Vigabatrin needs to be continued.

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