(11-58 months), the estimated probability of relapse free survival was 41% (7%) for the whole group. After adjustment in the Cox's multivariate model, malnutrition was the most significant adverse factor affecting duration of complete remission. Age above 8 years and high peripheral white celi count were also significant adverse factors. Among the nutritional indices, the height for age and weight for age z scores were both significant, whether the cut off points of z-2 or z=-1*28 were chosen to define malnutrition. A strong statistical association between the two indices was found; the contribution of height for age z score to the prediction of relapse free survival was more significant. Children with height for age z score <-2 had a relapse risk of 8*2 (95Gb confidence interval 3*1 to 21-9) relative to children with z score > -2. The results of this study suggest that socioeconomic and nutritional factors should be considered in the prognostic evaluation of children with leukaemia in developing countries. (Arch Dis Child 1994; 71: 304-3 10)

either PFS (P ¼ 0.63) or OS (P ¼ 0.52), nor did IgV H mutational status. However, patients with mutated IgV H had a longer treatment-free survival compared with those with unmutated IgV H (Figure 3).The overall response rate of 64% in this cohort of 126 patients with previously untreated B-CLL is comparable to results reported with intravenous fludarabine. Using the same response criteria, Rossi et al. 2 reported an overall response rate of 80% with the same schedule of oral fludarabine in a smaller group of previously untreated B-CLL patients but the proportion of patients in Rai stage III or IV in our study was higher (31% versus 22%) than in the Rossi trial.This study again confirms that the efficacy and toxicity of oral fludarabine are comparable to that of IV fludarabine given on a similar schedule. Although several randomized trials have confirmed a better overall and complete response rate for the combination of fludarabine and cyclophosphamide compared with fludarabine alone, no differences in the OS were observed. 7,8 In the British study, the oral formulation of fludarabine was introduced in both fludarabine arms when the drug became available, and differences in response rates between the two routes of administration did not appear significant. Oral fludarabine is an effective treatment for patients with previously untreated B-CLL, with less health resource utilization compared with IV administration. In an older population, the hematologic toxicity of the combination of fludarabine with cyclophosphamide and the absence of a demonstrable survival advantage, may favor initial therapy with single agent fludarabine and deferring combination therapy. In fludarabine-based combination therapies, the oral formulation should be considered an acceptable alternative. Conflict of interestThe authors declare no conflict of interest.

PURPOSE To describe event-free survival (EFS) and toxicities in children with low-risk acute lymphoblastic leukemia (ALL) assigned to receive either continuous 6-mercaptopurine (6-MP) and weekly methotrexate (MTX) or intermittent 6-MP with intermediate-dose MTX, as maintenance treatment. PATIENTS AND METHODS Between October 1, 2000, and December 31, 2007, 635 patients with low-risk ALL were enrolled onto Brazilian Childhood Cooperative Group for ALL Treatment (GBTLI) ALL-99 protocol. Eligible children (n = 544) were randomly allocated to receive either continuous 6-MP/MTX (group 1, n = 272) or intermittent 6-MP (100 mg/m(2)/d for 10 days, with 11 days resting) and MTX (200 mg/m(2) every 3 weeks; group 2, n = 272). RESULTS The 5-year overall survival (OS) and EFS were 92.5% +/- 1.5% SE and 83.6% +/- 2.1% SE, respectively. According to maintenance regimen, the OS was 91.4% +/- 2.2% SE (group 1) and 93.6% +/- 2.1% SE (group 2; P = .28) and EFS 80.9% +/- 3.2% SE (group 1) and 86.5% +/- 2.8% SE (group 2; P = .089). Remarkably, the intermittent regimen led to significantly higher EFS among boys (85.7% v 74.9% SE; P = .027), while no difference was seen for girls (87.0% v 88.8% SE; P = .78). Toxic episodes were recorded in 226 and 237 children, respectively. Grade 3 to 4 toxic events for groups 1 and 2 were, respectively, 273 and 166 for hepatic dysfunction (P = .002), and 772 and 636 for hematologic episodes (P = .005). Deaths on maintenance were: seven (group 1) and one (group 2). CONCLUSION The intermittent use of 6-MP and MTX in maintenance is a less toxic regimen, with a trend toward better long-term EFS. Boys treated with the intermittent schedule had significantly better EFS.

Objective: To describe the deaths of children with sickle cell disease (SCD) in Minas Gerais, Brazil, and followed up at the Fundação Hemominas. (March/1998 -February/2005). Deaths were identified by searching for children who did not attend scheduled consultations at hemocenters. Clinical and epidemiological data were abstracted from death certificates, the newborn screening database, individual medical records, and from interviews with families. Methods: Cohort of children diagnosed by the Neonatal Screening Program in Minas GeraisResults: During the period, 1,833,030 newborns were screened; 1,396 had SCD (1:1,300). There were 78 deaths: 63 with SS genotype, 12 with SC genotype, and three with Sß + thalassemia genotype. Fifty-six children (71.8%) died before 2 years of age; 59 died in hospitals and 18 at home or during transportation. Causes of death according to certificates (n = 78): infections, 38.5%; acute splenic sequestration, 16.6%; other causes, 9%; did not receive medical care, 15.4%; and not identified on certificates, 20.5%. According to interviews (n = 52), acute splenic sequestration was responsible for one third of deaths, in contrast with 14% recorded on death certificates. Survival probabilities at 5y (SEM) for children with SS, SC, and Sß + thalassemia were 89.4 (1.4), 97.7 (0.7), and 94.7% (3.0), respectively (SS vs. SC, p < 0.0001). Conclusions:Even with a carefully controlled newborn screening program, the probability of SS children dying was still found to be high. Causes not identified on death certificates may indicate difficulties recognizing SCD and its complications. Educational campaigns directed at health professionals and SCD patients' families should be boosted in order to decrease SCD mortality. Resultados: Foram triadas 1.833.030 crianças no período, sendo 1.396 com DF (1:1.300). Ocorreram 78 óbitos: 63 em crianças com genótipo SS, 12 em crianças com genótipo SC e três em crianças com genótipo S/ß + talassemia. Cinquenta e seis crianças (71,8%) morreram antes dos 2 anos de idade; 59 morreram em hospitais e 18 no domicílio ou trânsito. Causas de óbito pelo atestado (n = 78): 38,5% infecção; 16,6% sequestro esplênico agudo; 9% outras causas; 15,4% sem assistência médica; e 20,5% indeterminada. Segundo as entrevistas (n = 52), o sequestro esplênico foi responsável por quase 1/3 dos óbitos, contrastando com a porcentagem de apenas 14% registrada nos atestados de óbito. As probabilidades de sobrevida aos 5 anos (erro padrão da média) para crianças SS, SC e Sß + talassemia foram: 89,4 (1,4), 97,7 (0,7) e 94,7% (3,0), respectivamente (SS versus SC, p < 0,0001). J Pediatr (Rio J) Conclusões:Mesmo em um programa de triagem neonatal com rigoroso controle do tratamento, a probabilidade de óbito em crianças com genótipo SS ainda é elevada. Os óbitos com causa indeterminada indicam dificuldades no reconhecimento da DF e das suas complicações. Esforços educativos dirigidos a profissionais da saúde e familiares devem ser incrementados para diminuir a mortalidade pela DF. J Pediatr (Ri...