Polymorphism C242T of the gene of the p22phox subunit for nicotinamide adenine dinucleotide phosphate oxidase, and erythrocytic antioxidant enzymes, in patients with tetralogy of Fallot.

BACKGROUND: Nicotinamide adenine dinucleotide phosphate oxidase of the vascular cell membrane is an important source of reactive oxygen species. The aim of our study was to evaluate the possible influence of the p22phox C242T gene polymorphism on blood pressure and some markers of oxidative stress in children with tetralogy of Fallot. METHODS: After surgical repair in early life, we recruited 38 children, aged 11.7 plus or minus 3.2 years, including 185 healthy individuals as controls for the purposes of establishing frequencies of alleles and genotypes. From this latter group, we matched a sub-sample of 53 healthy caucasian children, aged 11.0 plus or minus 1.0 years, in order to compare enzymic activities. RESULTS: The children with tetralogy of Fallot showed significantly lower values of low-molecular-weight protein tyrosine phosphatase, particularly in carriers of CC genotype for the p22phox gene, with values of 145.2 plus or minus 77.4 micromol/g Hb/h, compared to controls, at 344.4 plus or minus 100.4 micromol/g Hb/h (p less than 0.001). Methemoglobin reductase activity in the patients with tetralogy was also lower in those with the CC genotype, at 9.8 plus or minus 3.2 micromol/g Hb-1 min(-1) compared to 24.2 plus or minus 11.8 micromol/g Hb(-1) min(-1) as measured in the controls (p less than 0.01). Lower systolic (p less than 0.05) and diastolic (p less than 0.01) blood pressures were also observed in the patients with tetralogy of Fallot. CONCLUSIONS: Patients with tetralogy of Fallot having the CC genotype may be at a higher state of oxidative stress than T allele carriers, a finding which could have prognostic implications. Long term follow-up of these patients, however, may be necessary in order to draw definite conclusions.

Diagnosis of first case of Balamuthia amoebic encephalitis in Portugal by immunofluorescence and PCR.

We report here the first Portuguese case of acute fatal granulomatous encephalitis attributed to Balamuthia mandrillaris, initially thought to be a brain tumor, which had a progressive and fatal outcome. Balamuthia mandrillaris is a free-living amoeba recognized as an uncommon agent of granulomatous encephalitis. Infections have been identified in immunocompromised hosts and in immunocompetent pediatric patients. Balamuthia infections are very rare, with only two reported cases in Europe. The case presented here occurred in a previously healthy boy who died 5 weeks after the onset of the symptoms. No evidence of immunological deficiency was noted, and testing for human immunodeficiency virus antibodies was negative. The symptoms were initially thought to be the result of a tumor, but histopathologic examination showed evidence of amoebic infection. Immunofluorescence staining of brain tissue identified B. mandrillaris as the infectious agent. The diagnosis was confirmed with PCR by detecting Balamuthia DNA in formalin-fixed brain tissue sections. Despite initiation of empirical antimicrobial therapy for balamuthiasis, the patient died 3 weeks after being admitted to the hospital. No source of infection was readily apparent.

[Iron deficiency anemia in infants. Preliminary development results at five years]. / Anemia por deficiência de ferro no lactente. Resultados preliminares do desenvolvimento aos cinco anos.

INTRODUCTION: Research, mostly conducted in developing countries, have suggested short and long term developmental delay in children who had iron-deficiency anemia (IDA) in their early life. This study aims to evaluate the development of 5-years old children who were diagnosed IDA at 9 months of age. METHODS: 27 children with IDA diagnosed at 9 months of age and 28 non-anemic controls were followed-up until they were 5 years old. At 9 months of age and at 5 years of age, blood samples were collected for blood count, iron, transferrin and ferritin. Children with hemoglobin < 110 g/l and ferritin < 12 ng/ml were diagnosed with IDA and received iron treatment for IDA correction. C Reactive Protein and antibody to hepatitis A virus were also studied at 5 years of age. At this age, children's motor and cognitive development were accessed using Griffith's test. RESULTS: At 9 months of age, IDA children had [mean (SD)] 102.5 g/ l (5.9) g/l of hemoglobin and 5.6 ng/l (3.1) ng/ml of ferritin. At 5 years of age all children were healthy and without anemia. One child had positive antibody to hepatitis A virus. The results of Griffith's test at five years--anemic/non-anemic at 9 months of age. The mean (sd)-p: general coefficient--100.8 (6.6)/99.5 (7.1)-0.48; locomotor 110.2 (6.9)/109.3 (6.5)-0.62; personal-social--99.1 (11.4)/95.7(11.6)-0.28; hearing-speech--93.8 (9.1)/95.3 (13.7)-0.64; hand-eye--98.2 (13.2)/99.1 (14.3)-0.79; performance--97.5 (17.6)/96.9 (13.6)-0.88; practical reasoning--89.8 (9.1)/86.3(7.1)-0.21. CONCLUSIONS: As only one child had positive antibody to hepatitis A virus, the study population may be considered as belonging to a developed region. These results suggest that, at least in developed regions, infants who receive an early treatment for their mild IDA may not be affected in global development at the age of five.

Bony density in adolescents after surgical repair of tetralogy of fallot: a comparative study with healthy adolescents.

BACKGROUND: Adverse influences arising in fetal life or immediately after birth have a permanent effect on body structure, physiology and metabolism. Evidence is now accumulating that programming of bone growth might be an important contributor to the later risk of osteoporosis. Long-term morbidity and mortality associated with retralogy of Fallot is not completely known. The aim of the present study was to evaluate the state of the bones in adolescents after surgical repair of tetralogy of Fallot, so as to ascertain any possible repercussions of the disease on bone mineralization. MATERIAL AND METHODS: We studied 34 adolescents with repaired tetralogy of Fallot, between the ages of 11 and 18 years, to establish their nutritional status, in terms of height, weight, and skinfolds, their body composition using an anthropometric method, their sexual maturity according to Tanner, and their food-habits as based on 24-hour recall. Bone density was evaluated by lumbar dual-energy X-ray absorptiometry. We included 34 healthy eutrophic adolescents, matched for gender and age, as controls. RESULTS: No significant differences were observed between the patients and their controls concerning nutritional status, body composition, total energy intake and nutritional supply in macronutrients, calcium, phosphorus, magnesium and vitamin D. Bone mineral density, expressed in Z-score and g/cm2, was significantly higher in patients with tetralogy of Fallot (p < 0.01). The age at the time of the first surgical procedure, or at complete surgical repair, and the total number of surgical procedures, had no significant influence on nutritional status or bone mineralization. Gender, chronological age, sexual maturity and the index of body mass are the major determinants of bone density for both samples. Obese adolescents with repaired tetralogy of Fallot had a significantly higher bone density (p < 0.05) compared to undernourished or euthrophic patients. CONCLUSIONS: Being born with tetralogy of Fallot has no significant repercussion, by the stage of adolescence, on nutritional status, pubertal progression, and accretion of bone minerals subsequent to surgical repair. Nutritional status is the major influence on the accretion of bone mass.

Modificadores biológicos: técnica de obtención y manejo clínico del plasma rico en plaquetas / Biologic modifiers: technique of obtention and clinical manipulation of plasma-enriched platelets

Los modificadores biológicos, son sustancias de estructura proteica que participan en los procesos de diferenciación y proliferación celular. Las plaquetas contienen por lo menos dos de estas sustancias, el factor de crecimiento derivado de las plaquetas (PDGF-platelet derived growth factor) y el factor de crecimiento transformador beta (TGF b-transforming growth factor beta). El plasma rico en plaquetas es un preparado autólogo que se utiliza en técnicas de regeneración e injertos óseos. Una vez que las plaquetas liberan su contenido en la zona quirúrgica, los factores de crecimiento contenidos en ellas estimulan el proceso de cicatrización normal, logrando un hueso de mayor calidad en períodos más cortos. En este trabajo se hace una síntesis de los procesos de proliferación y diferenciación celular y de la influencia que en dichos procesos tienen los factores de crecimiento. Se describe una técnica simplificada para la obtención de un plasma rico en plaquetas (PRP) cuya activación promueve la liberación de los factores de crecimiento plaquetarios en el lecho quirúrgico. La textura y adherencia de este preparado facilitan la manipulación y esculpido del material a injertar

Modificadores biológicos: técnica de obtención y manejo clínico del plasma rico en plaquetas / Biologic modifiers: technique of obtention and clinical manipulation of plasma-enriched platelets

Los modificadores biológicos, son sustancias de estructura proteica que participan en los procesos de diferenciación y proliferación celular. Las plaquetas contienen por lo menos dos de estas sustancias, el factor de crecimiento derivado de las plaquetas (PDGF-platelet derived growth factor) y el factor de crecimiento transformador beta (TGF b-transforming growth factor beta). El plasma rico en plaquetas es un preparado autólogo que se utiliza en técnicas de regeneración e injertos óseos. Una vez que las plaquetas liberan su contenido en la zona quirúrgica, los factores de crecimiento contenidos en ellas estimulan el proceso de cicatrización normal, logrando un hueso de mayor calidad en períodos más cortos. En este trabajo se hace una síntesis de los procesos de proliferación y diferenciación celular y de la influencia que en dichos procesos tienen los factores de crecimiento. Se describe una técnica simplificada para la obtención de un plasma rico en plaquetas (PRP) cuya activación promueve la liberación de los factores de crecimiento plaquetarios en el lecho quirúrgico. La textura y adherencia de este preparado facilitan la manipulación y esculpido del material a injertar (AU)

História da Pediatria brasileira: coletânea de textos e depoimentos / The history of the Brazilian pediatrics: collection of texts and statements

Mostra a trajetória da construção da Pediatria brasileira e da Sociedade Brasileira de Pediatria, através de textos e depoimentos.

História da pediatria brasileira: coletânea de textos e depoimentos / History of the Brazilian pediatrics: collectanea of texts and statements

Narra a evoluçäo da pediatria no Brasil com base em coletânea de documentos e depoimentos. Apresenta os autores e textos clássicos sobre o assunto e, também, a produçäo recente, de forma descritiva. Faz uma análise reflexiva e crítica da situaçäo da criança e do adolescente no Brasil. Aborda, ainda, de acordo com esta análise, o exercício da pediatria dentro do contexto histórico social. (JGC)

História da Pediatria brasileira: coletânea de textos e depoimentos / The history of the Brazilian pediatrics: collection of texts and statements

Mostra a trajetória da construçäo da Pediatria brasileira e da Sociedade Brasileira de Pediatria, através de textos e depoimentos.(AU)