Sideroblastic anemia medical therapy: Difference between revisions
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=== Non pharmacologic and general measures === | === Non pharmacologic and general measures === | ||
The following measures are used to treat sideoblastic anemia.<ref name="pmid24003969">{{cite journal |vauthors=Fujiwara T, Harigae H |title=Pathophysiology and genetic mutations in congenital sideroblastic anemia |journal=Pediatr Int |volume=55 |issue=6 |pages=675–9 |date=December 2013 |pmid=24003969 |doi=10.1111/ped.12217 |url=}}</ref> | The following measures are used to treat sideoblastic anemia.<ref name="pmid24003969">{{cite journal |vauthors=Fujiwara T, Harigae H |title=Pathophysiology and genetic mutations in congenital sideroblastic anemia |journal=Pediatr Int |volume=55 |issue=6 |pages=675–9 |date=December 2013 |pmid=24003969 |doi=10.1111/ped.12217 |url=}}</ref> | ||
* Avoid alcohol | * Avoid [[alcohol]] | ||
* Avoid zinc supplements | * Avoid [[zinc]] supplements | ||
* Remove toxic agents | * Remove toxic agents | ||
** Lead | ** [[Lead]] | ||
** Drugs | ** Drugs | ||
*** With isoniazid, pyridoxine is given to reverse its adverse effect on heme synthesis. | *** With [[isoniazid]], [[pyridoxine]] is given to reverse its adverse effect on [[heme]] synthesis. | ||
* Blood transfusion | * Blood transfusion | ||
* | * | ||
| Line 23: | Line 23: | ||
=== Pharmacologic measures === | === Pharmacologic measures === | ||
* Medications are used to control, anemia, morbidity and complications. | * Medications are used to control, [[anemia]], [[morbidity]] and complications. | ||
* The two groups are | * The two groups are | ||
** Vitamins | ** [[Vitamins]] | ||
** Iron chelators | ** [[Iron]] chelators | ||
==== Vitamins ==== | ==== Vitamins ==== | ||
* Folic acid. | * [[Folic acid]]. | ||
** Preffered regimen: Adults 0.4mg, PO,once daily | ** Preffered regimen: Adults 0.4mg, PO,once daily | ||
** preffered regimen peadiatric: 1 mg/day IV/PO/IM/SC initially, then 0.1-0.4 mg/day | ** preffered regimen peadiatric: 1 mg/day IV/PO/IM/SC initially, then 0.1-0.4 mg/day | ||
* Pyridoxine | * Pyridoxine | ||
** Preffered regimen:Aminoxin, adults,2.5-10 mg, PO,once daily | ** Preffered regimen:[[Aminoxin]], adults,2.5-10 mg, PO,once daily | ||
** Preffered regimen:Aminoxin, peadiatrics0.1-1.2 mg, I/M,once daily | ** Preffered regimen:[[Aminoxin]], peadiatrics0.1-1.2 mg, I/M,once daily | ||
* Thiamine | * [[Thiamine]] | ||
** Preffered regimen:adults 50mg,PO,once daily | ** Preffered regimen:adults 50mg,PO,once daily | ||
** Preffered regimen(1) peadiatric 10-25 mg IV/IM qDay | ** Preffered regimen(1) peadiatric 10-25 mg IV/IM qDay | ||
| Line 44: | Line 44: | ||
==== Iron chelators ==== | ==== Iron chelators ==== | ||
* Used in patietns having iron overload to remove toxic iron from tissues. | * Used in patietns having iron overload to remove toxic iron from tissues. | ||
** Preffered rgimen(1) adults Desferal 0.5-1g QD ,I/M | ** Preffered rgimen(1) adults [[Desferal]] 0.5-1g QD ,I/M | ||
** Preffered rgimen(2) adults | ** Preffered rgimen(2) adults [[Desferal]] 20-40mg/kg/day SC over 8-24 hours with portable pump providing continuous mini-infusion | ||
** Preffered rgimen(1) peadiatric DesferalSC 1-2g (20-40mg/kg/day) SC over 8-24 hours by small portable pump | ** Preffered rgimen(1) peadiatric DesferalSC 1-2g (20-40mg/kg/day) SC over 8-24 hours by small portable pump | ||
** Preffered rgimen(2) peadiatric Desferal IV, 40-50mg/kg/day over 8-12 hours for 5-7 days/ week,maximum, < 60mg/kg/day and an IV infusion rate of <15mg/kg/hr) | ** Preffered rgimen(2) peadiatric Desferal IV, 40-50mg/kg/day over 8-12 hours for 5-7 days/ week,maximum, < 60mg/kg/day and an IV infusion rate of <15mg/kg/hr) | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Nazia Fuad M.D.
Overview
The medical therapy for sideroblastic anemia include pyridoxine, thiamine and follic acid. For iron overload iron chelators are used.
Medical Therapy
Non pharmacologic and general measures
The following measures are used to treat sideoblastic anemia.[1]
- Avoid alcohol
- Avoid zinc supplements
- Remove toxic agents
- Lead
- Drugs
- With isoniazid, pyridoxine is given to reverse its adverse effect on heme synthesis.
- Blood transfusion
Pharmacologic measures
Vitamins
- Preffered regimen: Adults 0.4mg, PO,once daily
- preffered regimen peadiatric: 1 mg/day IV/PO/IM/SC initially, then 0.1-0.4 mg/day
- Pyridoxine
- Thiamine
- Preffered regimen:adults 50mg,PO,once daily
- Preffered regimen(1) peadiatric 10-25 mg IV/IM qDay
- Preffered regimen(2) peadiatric10-50 mg PO qDay for 2 weeks,
- Then 5-10 mg/day PO for 1 month
Iron chelators
- Used in patietns having iron overload to remove toxic iron from tissues.
- Preffered rgimen(1) adults Desferal 0.5-1g QD ,I/M
- Preffered rgimen(2) adults Desferal 20-40mg/kg/day SC over 8-24 hours with portable pump providing continuous mini-infusion
- Preffered rgimen(1) peadiatric DesferalSC 1-2g (20-40mg/kg/day) SC over 8-24 hours by small portable pump
- Preffered rgimen(2) peadiatric Desferal IV, 40-50mg/kg/day over 8-12 hours for 5-7 days/ week,maximum, < 60mg/kg/day and an IV infusion rate of <15mg/kg/hr)
References
- ↑ Fujiwara T, Harigae H (December 2013). "Pathophysiology and genetic mutations in congenital sideroblastic anemia". Pediatr Int. 55 (6): 675–9. doi:10.1111/ped.12217. PMID 24003969.