Alterações no estado nutricional em adolescentes sobreviventes de leucemias e linfomas

Objective To examine the changes in the nutritional status of adolescents aged 10-19 years after a minimum 12 months interval following oncological treatment for leukemias and lymphomas. Methods Longitudinal design quantitative study conducted at Hospital de Clínicas, Porto Alegre. Adolescents aged 10-19 years after a minimum 12 months interval following oncological treatment for leukemias and lymphomas were included. The measures of weight, height, brachial circumference, triceps skinfold thickness, arm muscle circumference and abdominal circumference were collected. Results The sample comprised 50 adolescents who had survived leukemias and lymphomas. In the follow up 38% of the patients were classified as overweight according to the body mass index for their age. There was a significant increase in body mass index for age between the beginning and the end of treatment and follow up (p=0.013) in female individuals, compared to males. The results indicate a reduction in the Z-score means of height for age, with significant differences between the beginning of treatment and follow up (p=0.016); and end of treatment and follow up (p=0.006) in patients of both genders Conclusion The anthropometric indicators show an important frequency of excess weight and increased tricipital skinfold, as well as a significant increase of the body mass index for age and also a growth deficit among the survivors.Objetivo Verificar as alterações no estado nutricional em adolescentes de 10-19 anos, observadas após 12 meses, no mínimo, da conclusão de tratamento oncológico de leucemias e linfomas. Métodos Trata-se de estudo quantitativo de delineamento longitudinal realizado no Hospital de Clínicas de Porto Alegre. Foram incluídos adolescentes de 10-19 anos, que haviam concluído tratamento oncológico para leucemias e linfomas há no mínimo doze meses. Foram coletadas as medidas de peso, estatura, circunferência braquial, dobra cutânea tricipital, circunferência muscular do braço e circunferência abdominal. Resultados A amostra foi constituída por cinquenta adolescentes sobreviventes de leucemias e linfomas. No follow up, 38% dos pacientes foram classificados como excesso de peso de acordo com o índice de massa corporal para a idade. Houve um aumento significativo no índice de massa corporal para a idade, ocorrida entre o início do tratamento, seu fim e follow up (p=0,013) nos indivíduos do sexo feminino, quando comparados a indivíduos do sexo masculino. Os resultados apontam uma diminuição nas médias de Escore-Z de estatura para a idade, com diferenças significativas entre o início do tratamento e follow up (p=0,016) e entre término do tratamento e follow up (p=0,006), em pacientes de ambos os sexos. Conclusão Os indicadores antropométricos demonstram uma frequência importante de excesso de peso e aumento da dobra cutânea tricipital, um aumento significativo do índice de massa corporal para a idade, bem como um deficit de crescimento entre os sobreviventes

Retinoblastoma in a pediatric oncology reference center in southern brazil

Background: Retinoblastoma (Rb) is the most common intraocular tumor diagnosed in children in Brazil. However, detailed information is lacking regarding patient clinical demographics. This study aimed to determine the clinical profile of patients with Rb who were treated in a public university hospital in southern Brazil from 1983 to 2012. Methods: Patients’ medical records were reviewed to retrospectively identify patients with a principal diagnosis of Rb. Rb was classified as hereditary or non-hereditary. Clinical staging was reviewed by an ophthalmologist. Statistical analysis was performed using SPSS. Results: Of 165 patients with a diagnosis of Rb during this period, 140 were included in the study. Disease was unilateral in 65.0 % of patients, bilateral in 32.9 %, and trilateral in 2.1 %. The mean age at onset of the first sign/ symptom was 18.1 month, and 35.7 % of patients were diagnosed during the first year of life. The most common presenting signs were leukocoria (73.6 %) and strabismus (20.7 %). The mean age at diagnosis was 23.5 months, and time to diagnosis was 5.4 months. In patients with clinical features of hereditary Rb, both onset of the first sign/symptom and diagnosis were at an earlier age than in patients without these features (12.3 vs 21.6 months [P = 0.001] and 15.9 vs 28.0 months [P < 0.001], respectively). However, there was no significant difference in overall survival between the two groups. Ocular stage at diagnosis was advanced in 76.5 % (Reese V) and 78.1 % (International Classification D or E). Of patients with unilateral and bilateral disease, 35.2 % and 34.8 %, respectively, had extraocular disease at diagnosis; 10.7 % had metastatic disease at diagnosis. Enucleation was observed in 88.1 % and exenteration in 11.9 % of patients; 93.6 % patients were followed until 2012, and 22.9 % relapsed. Overall survival was 86.4 %. Conclusions: Most Rb diagnoses are still diagnosed in advanced stages of the disease, considerably reducing overall survival time and the rate of eye and vision preservation

Pediatric cancer and Li-Fraumeni/Li-Fraumeni-like syndromes : a review for the pediatrician

Introduction: cancer is the second leading cause of death in children between the ages of 0 and 14 years, corresponding to approximately 3% of all cases diagnosed in Brazil. A significant percentage (5-10%) of pediatric cancers are associated with hereditary cancer syndromes, including Li-Fraumeni/Li-Fraumeni-like syndromes (LFS/LFL), both of which are caused by TP53 germline mutations. Recent studies have shown that a specific TP53 mutation, known as p.R337H, is present in 1 in 300 newborns in Southern and Southeast Brazil. In addition, a significant percentage of children with LFS/LFL spectrum tumors in the region have a family history compatible with LFS/LFL. Objective: to review clinical relevant aspects of LFS/LFL by our multidisciplinary team with focus on pediatric cancer. ( continues) Methods: the NCBI (PubMed) and SciELO databases were consulted using the keywords Li-Fraumeni syndrome, Li-Fraumeni-like syndrome and pediatric cancer; and all manuscripts published between 1990 and 2014 using these keywords were retrieved and reviewed. Conclusion: although LFS/LFL is considered a rare disease, it appears to be substantially more common in certain geographic regions. Recognition of population- specific risks for the syndrome is important for adequate management of hereditary cancer patients and families. In Southern and Southeastern Brazil, LFS/ LFL should be considered in the differential diagnosis of children with cancer, especially if within the spectrum of the syndrome. Due to the complexities of these syndromes, a multidisciplinary approach should be sought for the counseling, diagnosis and management of patients and families affected by these disorders. Pediatricians and pediatric oncologists in areas with high prevalence of hereditary cancer syndromes have a central role in the recognition and proper referral of patients and families to genetic cancer risk evaluation and management programs.Câncer pediátrico e síndrome de Li-Fraumeni/Li-Fraumeni- like: uma revisão para o pediatra. Introdução: o câncer é a segunda principal causa de morte em crianças com idades entre 0 e 14 anos, correspondendo a cerca de 3% de todos os casos diagnosticados no Brasil. Um percentual significativo (5-10%) dos cânceres pediátricos são associados a síndromes hereditárias para câncer, incluindo Li-Fraumeni/Li-Fraumeni-like síndromes (LFS/LFL), causadas por mutações germinativas no gene TP53. Estudos recentes têm demonstrado que uma mutação específica em TP53, conhecida como p.R337H, está presente em 1 em 300 recém-nascidos no Sul e Sudeste do Brasil. Além disso, um percentual significativo de crianças com tumores do espectro LFS/LFL na região têm uma história familiar compatível com a síndrome. Objetivos: revisão dos aspectos clínicos relevantes da LFS/LFL por equipe multidisciplinar, com foco no câncer pediátrico Métodos: o NCBI (PubMed) e SciELO foram consultados, usando as palavras-chave síndrome de Li-Fraumeni,síndrome de Li-Fraumeni-like e câncer pediátrico. Todos os artigos publicados entre 1990 e 2014 usando essas palavras- chave foram recuperados e revisados. Conclusão: apesar de LFS/LFL ser considerada uma doença rara, ela parece ser mais frequente em certas regiões. Reconhecer os critérios e condutas para identificação de pacientes em risco para LFS/LFL é fundamental para o manejo adequado dos pacientes com câncer hereditários e suas famílias. Devido à complexidade dessas síndromes, a abordagem multidisciplinar deve ser realizada. Pediatras e oncologistas pediátricos em áreas com alta prevalência de síndromes hereditárias de câncer têm um papel central no reconhecimento e encaminhamento adequado dos pacientes e famílias para programas de avaliação do risco de câncer genético e de gestão