Treatment of sideroblastic anemia

Treatment of sidero-achestic anemia

Treatment of patients with hereditary sidero-achestic anemia

1. Vitamin B ₆ in large doses - 4-8 ml of 5% solution per day intramuscularly. In the absence of effect, the appointment of coenzyme vitamin B ₁₂ - pyridoxal phosphate is indicated . The daily dose of the drug is 80-120 mg with oral administration.
2. Desferal (for binding and excretion from the body of iron) - 10 mg / kg / day monthly courses of 3-6 times a year.

Treatment of patients with acquired sidero-achestic anemia caused by lead intoxication

1. Identify and eliminate the source of lead. Before the complete elimination of the source of lead, the child should not be in the house. The risk of toxic effects increases even if the child only sleeps at home. It is necessary to carry out wet cleaning and vacuuming to remove lead dust.
2. To fill iron deficiency and reduce absorption of lead, iron preparations (6 mg / kg / day by elemental iron) are administered orally. The duration of the course is 1 month or until the normal level of protoporphyrin erythrocytes.
3. Therapy with complexing agents - EDTA, dimercaprol, penicillamine and succimer.

The goal of the treatment is to reduce the lead content to safe values (blood level less than 15 μg%), and the level of protoporphyrin of erythrocytes is up to the norm (less than 35 μg%).

Indications for therapy with complexing agents.

Therapy with complexing agents is indicated in the presence of at least one of three conditions:

1. the level of lead in the venous blood of 50 μg% in 2 consecutive samples;
2. the level of lead in venous blood is 25-49 μg%, and the level of protoporphyrin erythrocytes is 125 μg%;
3. positive sample with EDTA.

Easy lead poisoning (blood lead level 20-35 μg%)

Assign penicillamine at a dose of 900 mg / m ² / day in 2 divided doses. Penicillamine should not be taken together with dairy products and iron preparations, the drug is contraindicated for allergies to penicillins.

Moderate lead poisoning (blood lead level 35-45 μg%)

Conduct a sample with EDTA, with positive results of the sample appoint a calcium-disodium EDTA at 1000 mg / m ² / day intramuscularly with procaine for 3-5 days. The break between treatment courses should be at least 48-72 hours. The drug is completely canceled when the daily excretion of lead with urine is less than 1 μg of lead per 1 mg of EDTA.

Severe lead poisoning without encephalopathy (lead level in blood more than 45 μg%)

• At a lead level of less than 80 μg%: Succimer: 30 mg / kg / day in 3 divided doses for 5 days, then 20 mg / kg / day in 2 divided doses for 14 days.
• At a lead level of more than 80 μg%: Infusion therapy in a volume exceeding by 1.5 times the physiological fluid requirement. Dimercaprol in a dose of 300 mg / m ² intramuscularly, the dose is divided into 3 injections and administered for 1-3 days. EDTA in a dose of 1500 mg / m ² / day intravenously in the form of a long infusion or intramuscularly (once or a dose is divided and administered twice a day).

Severe lead poisoning, accompanied by encephalopathy

1. Hospitalization in the intensive care unit.
2. Infusion therapy.

Dimercaprol 600 mg / m ² / day intramuscularly, the dose is divided and injected 6 times a day. EDTA 1500 mg / kg / day in the form of intravenous infusion, the dose is divided and injected 3 times a day.

3. Anticonvulsants.

After a 5-day course of treatment, take a break for 48 hours, after which the treatment is resumed.

Monitoring in the treatment of complexing agents

To assess the effectiveness of treatment, daily urinary excretion of lead is measured, since its concentration in the blood in the presence of complexing agents may be low. The concentration of lead in the blood is measured every 48-72 hours in hospitalized and every 2-4 weeks in outpatients.

When EDTA is used, monitoring of urea and calcium levels in blood, lead levels in blood and urine, and periodic urinalysis are necessary. If signs of hypocalcemia or renal dysfunction appear, a dose of EDTA is reduced or canceled, after which the kidney function is normalized.

Prior to and during therapy with a succimer, biochemical indices of liver function, levels of urea and creatinine in the blood are examined every 5-7 days.

On the 14th and 28th days after the completion of therapy, complexing agents measure the level of lead in the blood.

Consequences of lead poisoning

All children exposed to lead toxicity should undergo an examination at the age of 5-6 years, including assessment of auditory and visual perception, gross and subtle movements, the ability to understand speech and speak.

Prophylaxis of lead poisoning

To avoid lead poisoning during the reconstruction of old houses, precautions should be taken by temporarily relocating children. It is especially dangerous to burn and dig in the ground lead paints, they should be scraped off or removed by chemical methods. Monitoring the condition of living quarters, tightening sanitary and building norms reduces the frequency of poisoning.