PURPOSE: To observe the safety and efficacy of hydroxyurea (HU), a drug that stimulates fetal hemoglobin (Hb F) production, in previously severely ill children with sickle cell disease. PATIENTS AND METHODS: HU was given in an uncontrolled study to 35 children with sickle cell disease, aged from 3 to 20 years, suffering from frequent painful crises. Mean duration of treatment was 32 months (range: 12-59 months). RESULTS: HU induced an increase in Hb F levels in all children out one; this increase was maximal after 9 months of treatment, was largely sustained thereafter, and was related to HU dose and inversely to patients' age. We also noted an apparent reduction in crisis, which occurred principally after 3 months of therapy and did not seem strictly correlated with the rise in Hb F level. No serious hematopoietic complication was observed. Growth curves and sexual development were not modified. CONCLUSION: Our data support the efficacy of HU in reducing painful events in children with sickle cell disease. Short- and middle-term tolerances are good. Thus, we think that HU can be given to children affected by frequent and severe painful crises. We recommend, however, very cautious use of this drug, because its long-term effects in children are still unknown.

Adolescent↗

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Renal enlargement as presentation of isolated renal relapse in childhood leukemia.

Extramedullary relapses in children with acute lymphoblastic leukemia occur most frequently in the central nervous system and in the testis. In this report, the authors describe a 16-year-old girl with an isolated renal relapse of acute lymphoblastic leukemia after a disease-free interval of 2 years and 8 months. This clinically inconspicuous renal relapse was suggested by a routine follow-up renal sonography. No evidence of disease was found in bone marrow or peripheral blood. Renal biopsy was required to establish the diagnosis. Treatment consisted of intensive chemotherapy and autologous bone marrow transplantation. The patient has been in second complete continuous remission for 7 years. The authors recommend the use of an intensive multidrug salvage regimen.

Adolescent↗

PubMedDate not supplied

[A new tool for evaluation of medication errors applied to pediatric hematology].

This study was conducted in a haematological paediatric department and was aimed at evaluating drug medication errors. Their frequency was studied, but also and mainly their degree of severity and preventability. Only adverse drug events that were identified as possibly due to pharmacological properties of drugs or medication errors were collected. An original method was used, based on a multidimensional mathematical tool, called Factorial Analysis of Multiple Correspondences (FAMC), in order to assess the grade of severity and preventability for each adverse drug event. A total of 155 adverse drug events were detected for 34 out of 52 patients hospitalized during the study period. The prevalence rate was 65 per cent and among these adverse drug events, 16 per cent were serious and 53 per cent were avoidable. Apart from the fact that the FAMC helped to determine the grade of preventability, FAMC allowed one to demonstrate that allergy and medication errors were the most avoidable adverse drug events. In this way the method used was validated. This study permitted the assessment of drug medication errors in this department and helped to choose the priorities for the management of preventive actions.

Child↗

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[Alpha chain disease. Report of a case (author's transl)].

A young patient, with alpha chain disease, is in good clinic condition, a little more than 4 years after the diagnosis. She received, during 1 year, antibiotics of tetracycline type, then antimitotics during 2 years. 9 months after antimitotics treatment was achieved, histologic signs remained unmodified, without evidence of degenerescence. Immunoelectrophoresis which was normal at the end of chemotherapy is always normal after 15 months without treatment.

Adolescent↗

PubMedDate not supplied

[Treatment of childhood acute lymphoblastic leukemia. Study of 171 patients. Value of pronostic factors for the differentiation and treatment of good and bad types. Study of 64 patients].

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Adolescent↗

PubMedDate not supplied

[Generalized BCG infection in mixed and severe immunologic deficiency. Unfavorable outcome in spite of an attempted bone marrow transplant].

Generalized BCG infection can occur after vaccination in patients with a severe combined or T-cell immunodeficiency. In the reported case, generalized BCG infection developed in an infant with a severe combined immunodeficiency disease and presented mainly with hematological manifestations. This infection led to death of the patient in spite of an histocompatible bone marrow transplantation performed late in the course of the disease and followed by a minimal graft-versus-host reaction. Based on this case report and others from the literature, it is suggested that neonatal BCG vaccination should be performed after knowing at least the complete family history. Although the presented patient died, it should seem reasonable to treat such patients with an early immunological reconstitution and with anti-TB antibiotics.

BCG Vaccine↗

PubMedDate not supplied