Abstract |
Five out of eight patients with congenital pure red cell aplasia (PRCA), who became refractory or did not respond to conventional prednisone treatment (2 mg/kg), responded to high-dose intravenous methylprednisolone administration. The drug was given daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, then subsequently 10, 5, and 2 mg/kg for a week, followed by 1 mg/kg until the hemoglobin level reached 12 g/dl. In the other three patients, although normoblasts appeared in the bone marrow, hemoglobin elevation was not observed. These three patients finally responded fully to a dose of 100 mg/kg intravenously. Therefore, we believe that resistance to corticosteroids could be overcome by increasing the dose in patients with Diamond-Blackfan syndrome. Four of these patients have been off the treatment for 2-13 months. Seven recurrences occurred in three patients 6-9 months following discontinuation of the treatment. However, all again responded to the same regimen.
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Authors | S Ozsoylu |
Journal | The American journal of pediatric hematology/oncology
(Am J Pediatr Hematol Oncol)
Vol. 10
Issue 3
Pg. 217-23
( 1988)
ISSN: 0192-8562 [Print] United States |
PMID | 3140684
(Publication Type: Journal Article)
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Chemical References |
- Prednisone
- Methylprednisolone
|
Topics |
- Body Height
(drug effects)
- Child, Preschool
- Drug Resistance
- Erythropoiesis
(drug effects)
- Female
- Humans
- Infant
- Injections, Intravenous
- Male
- Methylprednisolone
(administration & dosage, adverse effects)
- Prednisone
(therapeutic use)
- Red-Cell Aplasia, Pure
(blood, congenital, drug therapy)
- Syndrome
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