Josefina Aparecida Pellegrini Braga

Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil.

This study focused on clinical, hematological, and molecular aspects of sickle cell anemia pediatric patients from two different cites in Brazil. Seventy-one patients from São Paulo and Salvador, aged 3 to 18 years, were evaluated. Hematological analyses, betaS globin gene haplotypes, and alpha2 3.7kb-thalassemia were performed. Numbers of hospitalizations due to vaso-occlusive crises, infections, stroke, and cholelithiasis were investigated. São Paulo had more hospitalizations from vaso-occlusion, cholelithiasis, and stroke than Salvador. The Ben/CAR genotype predominated in both cities. Alpha2 3.7kb-thalassemia had a frequency of 28.2% in Salvador, mostly with Ben/CAR genotype (45.0%), while São Paulo had 22.5% with similar frequencies of the Ben/ CAR and CAR/CAR genotypes. Sickle cell anemia patients from São Paulo also had more episodes of stroke, which was observed among CAR/CAR, atypical, and BEN/CAR haplotypes. In Salvador stroke was only observed in the Ben/CAR genotype. Cholelithiasis had similar frequencies in the two cities. These data suggest a milder phenotype among patients in Salvador, possibly due to genetic, environmental, and socioeconomic factors. Further studies are needed to elucidate modulating factors and phenotype association.

Medidas gerais no tratamento das doenças falciformes

This article presents the most important measures in the care of patients with sickle cell disease, which is characterized by a high morbimortality rate. Effective preventive measures including newborn screening, education of patients and caregivers, nutrition support, protective vaccinations and prophylaxis using penicillin to prevent pneumococcal, contribute to a decrease in the morbimortality as well as to improve the quality of life of these patients.

Anemia ferropriva e estado nutricional de crianças de creches de Guaxupé

OBJECTIVE: To evaluate the nutritional status and the prevalence of iron deficiency anemia in children attending public day care centers in the city of Guaxupe (MG), Brazil. METHODS: Children of both genders, from 6 to 72 months, registered in public day-care centers in the city of Guaxupe, were studied. Nutritional evaluation was conducted using the Z-score for height-to-age and weight-to-height relationships. Hemoglobin dosage, serum iron, total iron binding capacity and the transferrin saturation index were used for diagnosis of anemia. RESULTS: Z score < - 2 was observed in 3.3% of the children for the height-to-age relationship and of 0.4% for the weight-to-height relationship. Prevalence of total iron deficiency anemia was of 16.1%. In the age span from 6 to 36 months it was of 44.6%. The entire sample showed 45% with low serum iron, 37.9% with high total iron binding capacity and 43.1% with a low transferrin saturation index. CONCLUSION: Malnutrition was not considered to be a problem due to its low prevalence; however, an important prevalence of iron deficiency was observed. Iron deficiency anemia is more prevalent in children between 6 and 36 months confirming that this age span is more vulnerable. No association between iron deficiency anemia and the Z score of the studied anthropometry relationships was found.

Deficiência de ferro na criança

Iron deficiency anemia afflicts an estimated two billion people and iron deficiency approximately 4 billion people in developed countries and is even more common in developing countries. In Brazil, depending on the region and age, studies point to high prevalences of iron-deficiency anemia in children. The high growth speed, which requires a greater amount of iron, connected with an inadequate iron diet and early weaning contribute to the high prevalence, mainly within the first 2 years of life. Other risk factors, such as prematurity, low birth weight, early umbilical cord clamping and weaning from exclusive breastfeeding may contribute. The impact of iron deficiency on growth is controversial as several other variables contribute to improve or worsen the nutritional status. Alterations in the psychomotor and neural-cognitive development of infants with iron deficiency have been reported in various studies with the catch-up growth rate after treatment being controversial. Additionally, some studies have demonstrated a decrease in the intellectual development and cognitive acquisition in school age children and adolescents that is reverted after iron therapy. The best preventive measure is nutritional education, however due to the high prevalence of iron deficiency anemia, other measures should also be used as iron supplementation and food fortification with iron.

Anemia in low-income exclusively breastfed infants.

OBJECTIVE To verify the behavior of hemoglobin levels and anemia prevalence in full term infants, aged 3 to 6 months and on exclusive breastfeeding. METHODS A cross-sectional study of 242 infants aged 3 to 6 months with birth weights of more than 2,500 g, on exclusive breastfeeding and monitored by the Program for the Promotion of Infant Growth and Development, part of the Paraisópolis Einstein Community Program. Hemoglobin was assayed by finger prick between the third and sixth months of life. Anemia was defined as Hb < 10.3 g/dL (Saarinen) or Hb < 10 g/dL (Brault-Dubuc) for infants aged 3 to 5 months and as Hb < 11.0 g/dL (WHO) for infants aged 6 months. RESULTS Mean hemoglobin concentration was 11.3 and 11.4 g/dL at 3 and 4 months and 11.2 and 11.1 g/dL at 5 and 6 months, respectively. The percentage of anemic infants varied depending on age and the cutoff adopted, being 11.8, 10.2 and 8.3% at 3, 4 and 5 months, respectively, according to the Brault-Dubuc criteria, and 20.6, 14.8 and 10.4% by the Saarinen criteria. Anemia prevalence at 6 months was 37.5%. CONCLUSIONS Anemia prevalence rates observed among infants aged between 3 and 6 months varied from 8.3 to 37.5%, justifying increased attention on the part of pediatricians to the hemoglobin levels of infants who are on exclusive breastfeeding, come from low-income families and present risk factors for iron deficiency.

Results from transcranial Doppler examination on children and adolescents with sickle cell disease and correlation between the time-averaged maximum mean velocity and hematological characteristics: a cross-sectional analytical study

CONTEXT AND OBJECTIVE Transcranial Doppler (TCD) detects stroke risk among children with sickle cell anemia (SCA). Our aim was to evaluate TCD findings in patients with different sickle cell disease (SCD) genotypes and correlate the time-averaged maximum mean (TAMM) velocity with hematological characteristics. DESIGN AND SETTING Cross-sectional analytical study in the Pediatric Hematology sector, Universidade Federal de São Paulo. METHODS 85 SCD patients of both sexes, aged 2-18 years, were evaluated, divided into: group I (62 patients with SCA/Sß(0) thalassemia); and group II (23 patients with SC hemoglobinopathy/Sß(+) thalassemia). TCD was performed and reviewed by a single investigator using Doppler ultrasonography with a 2 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. The hematological parameters evaluated were: hematocrit, hemoglobin, reticulocytes, leukocytes, platelets and fetal hemoglobin. Univariate analysis was performed and Pearsons coefficient was calculated for hematological parameters and TAMM velocities (P < 0.05). RESULTS TAMM velocities were 137 ± 28 and 103 ± 19 cm/s in groups I and II, respectively, and correlated negatively with hematocrit and hemoglobin in group I. There was one abnormal result (1.6%) and five conditional results (8.1%) in group I. All results were normal in group II. Middle cerebral arteries were the only vessels affected. CONCLUSION There was a low prevalence of abnormal Doppler results in patients with sickle-cell disease. Time-average maximum mean velocity was significantly different between the genotypes and correlated with hematological characteristics.

Anemia em lactentes de baixa renda em aleitamento materno exclusivo

OBJETIVO:Verificar o comportamento dos valores da hemoglobina e a prevalencia de anemia entre lactentes de termo de 3 a 6 meses de idade em aleitamento materno exclusivo. METODOS: Estudo transversal em 242 lactentes de 3 a 6 meses de idade com peso de nascimento superior a 2.500 g, em aleitamento materno exclusivo e em acompanhamento no Programa de Promocao do Crescimento e Desenvolvimento do Lactente do Programa Einstein na Comunidade de Paraisopolis. A dosagem de hemoglobina foi realizada por meio de puncao digital entre o terceiro e o sexto meses de vida. Adotaram-se os valores de Hb < 10,3 g/dL (Saarinen) e Hb < 10 g/dL (Brault-Dubuc) como criterio de anemia nas idades de 3 a 5 meses e Hb < 11,0 g/dL (OMS) para os lactentes de 6 meses. RESULTADOS: A media da hemoglobina foi de 11,3 e 11,4 g/dL aos 3 e 4 meses, 11,2 e 11,1 g/dL aos 5 e 6 meses, respectivamente. A porcentagem de anemicos variou conforme a idade e o padrao adotado, sendo de 11,8, 10,2 e 8,3% aos 3, 4 e 5 meses, segundo o criterio adotado por Brault-Dubuc, e de 20,6, 14,8 e 10,4%, respectivamente, para o criterio de Saarinen. Aos 6 meses, a prevalencia de anemia foi de 37,5%. CONCLUSOES: Observaram-se prevalencias de anemia variando entre 8,3 e 37,5% nos lactentes do terceiro ao sexto mes de vida, que justificam maior atencao do pediatra em relacao aos niveis de hemoglobina em lactentes em aleitamento materno exclusivo, que pertencam a familias de baixa renda e que apresentem fatores de risco para deficiencia de ferro.

Helicobacter pylori infection & immune thrombocytopenic purpura in children and adolescents: A randomized controlled trial.

Abstract Helicobacter pylori and immune thrombocytopenic purpura (ITP) association is not well established in chronic ITP (cITP) in children, although the cure of thrombocytopenia in approximately half of H. pylori eradicated adult patients has been described. The aim of this study was to investigate the effect of H. pylori eradication on platelet (PLT) recovery in cITP children and adolescents through a randomized, controlled trial. A total of 85 children (mean age 11.4 years) with cITP were prospectively enrolled. Diagnosis of H. pylori was established by two locally validated tests, 13C-urea breath test and monoclonal stool antigen test. Twenty-two infected patients were identified, and randomly allocated into two groups: H. pylori treatment group (n = 11) and the non-intervention control group (n = 11). The control group was offered treatment if the thrombocytopenia persisted after the follow-up. At baseline, there were no differences regarding age, sex, duration of disease, and PLT count between groups. Sixty three of 85 patients were uninfected. PLT response was classified as complete response: PLT > 150 × 109 l−1; partial response: PLT 50–150 × 109 l−1, or an increase of 20–30 × 109 l−1; no response: PLT < 50 × 109 l−1 or an increase of <20 × 109 l−1 after at least 6 months of follow-up. Complete response was observed in 60.0% (6/10, one excluded) H. pylori eradicated patients vs. 18.2% (2/11) in non-eradicated patients (p = 0.08; OR = 6.75) after 6–9 months of follow-up. Among uninfected patients, only 13.8% (8/58) presented complete response. Two non-treated controls were treated after 6–12 months of follow-up, and PLT response was observed in 61.5% (8/13) of H. pylori eradicated patients, and in 19.0% (11/58) of uninfected patients (p = 0.004). Cytotoxin associated gene A and vacuolating cytotoxin gene A IgG antibodies were present in almost all infected patients. Therefore, the study suggests that H. pylori eradication plays a role in the management of H. pylori infected cITP children and adolescents.

Use of Iron-Fortified Rice Reduces Anemia in Infants

Food fortification is advocated to tackle iron deficiency in anemic populations. Our objective was to evaluate the impact of iron-fortified rice (Ultrarice(®)) weekly on hemoglobin and anemia levels compared with standard rice (control). This cluster-randomized study deals with infants (10-23 months) from two public child day care centers in Brazil, n = 216, in an 18 week intervention. The intervention group received individual portions of fortified rice (50 g) provided 56.4 mg elemental/Fe. For intervention center: baseline mean hemoglobin was 11.44 ± 1.07 g/dl, and after intervention 11.67 ± 0.96 g/dl, p < 0.029; for control: baseline mean hemoglobin value was 11.35 ± 4.01 g/dl, and after intervention 11.36 ± 2.10 g/dl, p = 0.986. Anemia prevalence for intervention center was 31.25% at baseline, and 18.75% at end of study, p = 0.045; for control 43.50% were anemic at baseline, and 37.1% at the end of study, p = 0.22. Number Needed to Treat was 7. Iron-fortified rice was effective in increasing hemoglobin levels and reducing anemia in infants.

Randomized controlled trial of iron-fortified drinking water in preschool children.

Objectives: To evaluate the effects of fortified drinking water, with different concentrations of iron added, on hemoglobin and hematocrit values in preschoolers. Methods: Double-blind, randomized cluster clinical trial, with children aged 2 to 5 years of age, from 4 state-run schools, forming 1 group for each school. For fortification, ferrous sulphate in concentrations of 5 mg of elemental iron per liter of water (group A), 7.5 mg (group B), and 10 mg (group C), was used during a period of 4 months. In group D, the control, a placebo (Bixa orellana) was added. Hemoglobin and hematocrit values were checked before and after intervention. Results: Before fortification, hemoglobin and hematocrit averages were below the reference values adopted in all groups. After fortification, the prevalence of anemia showed a reduction in the 4 groups, which was more pronounced in group B, at 48.3%. The hemoglobin values in groups B (11.5) and C (11.4) were statistically similar. However, the average consumption of water/day/student was lower in group C. Comparison of hemoglobin values between groups A (11.2) and D (11.0) did not show a significant difference, suggesting insignificant efficacy with 5 mg Fe/L fortification. Conclusions: The consumption of drinking water fortified with 7.5 mg of elemental iron/L water resulted in greater adhesion and an increase in hemoglobin values, with a reduction in the prevalence of anemia.