Brazilian pediatric reference data for quantitative ultrasound of phalanges according to gender, age, height and weight

To establish normative data for phalangeal quantitative ultrasound (QUS) measures in Brazilian students. The sample was composed of 6870 students (3688 females and 3182 males), aged 6 to 17 years. The bone status parameter, Amplitude Dependent Speed of Sound (AD-SoS) was assessed by QUS of the phalanges using DBM Sonic BP (IGEA, Carpi, Italy) equipment. Skin color was obtained by self-evaluation. The LMS method was used to derive smoothed percentiles reference charts for AD-SoS according to sex, age, height and weight and to generate the L, M, and S parameters. Girls showed higher AD-SoS values than boys in the age groups 7-16 (p<0.001). There were no differences on AD-SoS Z-scores according to skin color. In both sexes, the obese group showed lower values of AD-SoS Z-scores compared with subjects classified as thin or normal weight. Age (r(2) = 0.48) and height (r(2) = 0.35) were independent predictors of AD-SoS in females and males, respectively. AD-SoS values in Brazilian children and adolescents were influenced by sex, age and weight status, but not by skin color. Our normative data could be used for monitoring AD-SoS in children or adolescents aged 6-17 years106CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQCOORDENAÇÃO DE APERFEIÇOAMENTO DE PESSOAL DE NÍVEL SUPERIOR - CAPESFUNDAÇÃO DE AMPARO À PESQUISA DO ESTADO DE SÃO PAULO - FAPESPsem informaçãosem informação2006/01978-0; 2011/23460-1; 2002/13021-1; 2012/16778-

Validation Of The Ebmt Risk Score In Chronic Myeloid Leukemia In Brazil And Allogeneic Transplant Outcome.

The management of chronic myeloid leukemia (CML) has changed radically since the introduction of imatinib therapy. The decision of whether to offer a patient a hematopoietic stem cell transplant (HSCT) must be based on the probability of success of the procedure. The aim of this retrospective analysis of 1,084 CML patients who received an allogeneic HSCT in 10 Brazilian Centers between February 1983 and March 2003 was to validate the EBMT risk score. The study population comprised 647 (60%) males and 437 (40%) females, with a median age of 32 years old (range 1 - 59); 898 (83%) were in chronic phase, 146 (13%) were in accelerated phase and 40 (4%) were in blast crisis; 151 (14%) were younger than 20 years old, 620 (57%) were between 20 and 40 and 313 (29%) were older than 40; 1,025 (94%) received an HLA fully matched sibling transplant and only 59 (6%) received an unrelated transplant. In 283 cases (26%) a male recipient received a graft from a female donor. The interval from diagnosis to transplantation was less than 12 months in 223 (21%) cases and greater in 861 (79%). The overall survival, disease-free survival, transplant-related mortality and relapse incidence were 49%, 50%, 45% and 25%, respectively. Of the 1084 patients, 179 (17%) had a risk score of 0 or 1, 397 (37%) had a score of 2, 345 (32%) had a score of 3, 135 (12%) had a score of 4 and 28 (2%) a score of 5 or 6. The overall survival (OS) rate in patients with risk scores 0-1 and 2 was similar (58% and 55%, respectively) but significantly better than that in patients with scores 3 or more (score 3 - 44%, 4 - 36 % and 5-6 - 27%, respectively) pp<0.001). Disease-free survival (DFS) and transplant related mortality (TRM) in a patients with a score of 3 or more were 46% and 49%, respectively and the relapse rate beyond score 5-6 was 77%. Disease status had a negative impact on all outcomes (OS, DFS, TRM, and relapse). The OS rate for male recipients of a graft from a female donor was 40% compared to 52% among the other donor-recipient pairs (p=0.004). DFS and TRM were significant for disease phase and female donor-male recipient (p<0.001 and p<0.003, respectively). In our experience, age and interval between diagnosis and transplant did influence OS, DFS, TRM, and relapse rate. Our results validate the EBMT risk score in the context of a developing country and confirm its usefulness for making point decisions in the imatinib era.90232-

RETINOBLASTOMA BILATERAL DE APARECIMENTO TARDIO: REVISÃO DE LITERATURA

This article aims to scan the current medical literature on the issues surrounding retinoblastoma, as although it is the most common ocular tumor in childhood, retinoblastoma can be diagnosed late, given its less specific signs and symptoms. In situations where the ocular examination is not performed correctly and other diagnostic work-up strategies are questionable, patients may be subjected to incorrect treatments. The most suggestive signs of the disease may not be present during medical evaluation. Delay in diagnosis of more than 6 months is associated with mortality of approximately 70%. Among the propaedeutic exams is fundoscopy, which allows a more accurate diagnosis in the face of suspicion and helps in monitoring the patient. This examination must be carried out monthly in the first year after the end of treatment, and quarterly thereafter. Additional imaging techniques are also important in the search for an assertive diagnosis and for the correct staging of the disease.Este artigo tem por objetivo realizar uma varredura da literatura médica vigente sobre as questões que envolvem o retinoblastoma, pois embora&nbsp; seja&nbsp; o&nbsp; tumor&nbsp; ocular&nbsp; mais&nbsp; comum&nbsp; na&nbsp; infância,&nbsp; o retinoblastoma&nbsp; pode&nbsp; ser&nbsp; diagnosticado&nbsp; de&nbsp; forma&nbsp; tardia,&nbsp; tendo&nbsp; em&nbsp; vista&nbsp; que&nbsp; sinais&nbsp; e&nbsp; sintomas menos específicos. Em situações em que o exame ocular não é realizado corretamente e as demais estratégias de propedêutica diagnóstica são questionáveis, os pacientes podem ser submetidos a tratamentos incorretos. Os sinais mais sugestivos da doença podem não estar presentes durante a avaliação médica. O&nbsp; atraso no diagnóstico superior a 6 meses está associado à mortalidade de aproximadamente 70%. Dentre os exames propedêuticos tem-se a fundoscopia, a qual permite um diagnóstico mais preciso diante da suspeita e auxilia no acompanhamento do paciente. Esse exame&nbsp; deve&nbsp; ser&nbsp; efetuado&nbsp; de&nbsp; forma&nbsp; mensal&nbsp; no&nbsp; primeiro&nbsp; ano&nbsp; após&nbsp; o&nbsp; término&nbsp; do&nbsp; tratamento,&nbsp; e posteriormente, trimestralmente. As técnicas de imagem adicionais também são importantes na busca de um diagnóstico assertivo e para o estadiamento correto da doença

Brazilian Pediatric Reference Data for Quantitative Ultrasound of Phalanges According to Gender, Age, Height and Weight

<div><p>Aims</p><p>To establish normative data for phalangeal quantitative ultrasound (QUS) measures in Brazilian students.</p><p>Methods</p><p>The sample was composed of 6870 students (3688 females and 3182 males), aged 6 to 17 years. The bone status parameter, Amplitude Dependent Speed of Sound (AD-SoS) was assessed by QUS of the phalanges using DBM Sonic BP (IGEA, Carpi, Italy) equipment. Skin color was obtained by self-evaluation. The LMS method was used to derive smoothed percentiles reference charts for AD-SoS according to sex, age, height and weight and to generate the L, M, and S parameters.</p><p>Results</p><p>Girls showed higher AD-SoS values than boys in the age groups 7–16 (p<0.001). There were no differences on AD-SoS Z-scores according to skin color. In both sexes, the obese group showed lower values of AD-SoS Z-scores compared with subjects classified as thin or normal weight. Age (r² = 0.48) and height (r² = 0.35) were independent predictors of AD-SoS in females and males, respectively.</p><p>Conclusion</p><p>AD-SoS values in Brazilian children and adolescents were influenced by sex, age and weight status, but not by skin color. Our normative data could be used for monitoring AD-SoS in children or adolescents aged 6–17 years.</p></div

AD-SoS Z-scores according to skin color and weight status.

<p>^a Significant differences from obese, p<0,001</p><p>^b Significant differences from overweight, p<0,001</p><p>^c Significant differences from obese, p<0,05</p><p>^d Significant differences from overweight, p<0,01.</p><p>AD-SoS Z-scores according to skin color and weight status.</p

General characteristics of the sample.

<p>^aDifferences between sexes, p<0.001, Mann-Whitney Test.</p><p>^bWeight status established according to BMI Z-score cut-offs defined by IOTF: Thinness: <-1.01 and <0.98; Normal: between -1.01 to 1.30 and -0.98 to 1.23; Overweigh: between 1.31 to 2.28 and 1.24 to 2.18 and Obese: >2.28 and >2.18 for boys and girls, respectively.</p><p>General characteristics of the sample.</p

Relationship of AD-SoS (left panel) and AD-SoS Z-score (right panel) versus independents variables for total sample and divided by weight categories in boys.

<p>Relationship of AD-SoS (left panel) and AD-SoS Z-score (right panel) versus independents variables for total sample and divided by weight categories in boys.</p

Linear (solid line) and cubic (dotted line) relationship between AD-SoS and BMI for girls and boys.

<p>Linear (solid line) and cubic (dotted line) relationship between AD-SoS and BMI for girls and boys.</p

Smoothed percentile charts of AD-SoS for girls according to age (year), height (cm) and weight (kg).

<p>Smoothed percentile charts of AD-SoS for girls according to age (year), height (cm) and weight (kg).</p

Relationship of AD-SoS (left panel) and AD-SoS Z-score (right panel) versus independents variables for total sample and divided by weight categories in girls.

<p>Relationship of AD-SoS (left panel) and AD-SoS Z-score (right panel) versus independents variables for total sample and divided by weight categories in girls.</p