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Chronic kidney disease (CKD) is defined based on structural or functional abnormalities of the kidneys, or a glomerular filtration rate (GFR) below the threshold of 60 ml/min per 1.73 m2 for more than 3 months. It is an important noncommunicable disease with a rising worldwide, becoming a global public health problem. There are few studies about this problem, especially in low- and middle-income countries (LMIC), including Brazil, an upper-middle-income country. The objective of the study was to determine the cause-specific mortality rates for pediatric CKD patients (CKDMR) from 0 to 19 years old, based on the 10th revision of the International Classification of Diseases (ICD-10) and the Global Burden of Diseases Injuries and Risk Factors Study's (GBD) list. We calculated the impact of the annual human development indexes (HDI) in CKDMR in Brazil and its regions at two different times and compared it with the literature results. We obtained data from the Department of Informatics of the Brazilian Unified Health System (DATASUS) from 1996 to 2017. The Joinpoint regression analyses estimated the average annual percentage changes (AAPCs). The correlation between the HDI values and the number of deaths from each age group in Brazil and its different regions were assessed using the time series autoregressive integrated moving average (ARIMA) models. There were 8838 deaths in a pediatric and adolescent population of about 1.485 x 109 person-years observed in Brazil from 1996 to 2017. Our results demonstrated a significant increase in the AAPC in Brazil's less than 1-year-old age group and a decrease in children from 5 to 19 years old. We observed a positive correlation between CKDMR and HDI among children under 1 year of age. Conversely, there is a negative association in the age groups ranging from 5 to 19 years, indicating an inverse relationship between CKDMR and HDI.

Abstract Background This study aims to describe immunophenotypic explorations at diagnosis and follow up of a pediatric patient with leukemic phase of ALK+ anaplastic large cell lymphoma (ALCL) by multiparametric flow cytometry (MFC). Case An 8‐color MFC combination of antibodies allowed to identify neoplastic cells in concentrations until 0.02% during minimal residual disease (MRD) monitoring. Immunophenotypic shifts occurred in key markers as CD30, CD7, CD2, and CD5, however neoplastic cells were clearly discriminated from normal populations. Conclusion MFC can be a useful tool for ALCL diagnosis and MRD monitoring and may support therapeutic decisions.

Abstract Background Acute lymphoblastic leukemia (ALL) is the most common neoplasm in childhood. The probability of current overall survival (OS) is around 90% in developed countries. There are few studies that demonstrate the results in Brazil. Aim This work aims to analyze the results of children with ALL treated at a single institution in Rio de Janeiro. Methods and results Retrospective analysis survival study of a cohort of childhood ALL patients treated in Hemorio. Kaplan–Meier and log‐rank methods were used for the analysis of OS and events‐free survival (EFS) and the Cox proportional hazards regression model for multivariate analysis. The probability of OS and EFS at 6 years was 52% and 45%. The probability of OS and EFS in 6 years for patients aged 10‐17 years was 31% and 28% and for the younger was 65% and 55%, respectively (p

After the exclusion of iron deficiency and β-thalassemia, molecular research for α-thalassemia is recommended to investigate microcytic anemia. Aiming to suggest more efficiently the molecular analysis for individuals with a greater chance of having a symptomatic form of the disease, we have developed and validated a new decision tool to predict the presence of two or more deletions of α-thalassemia, increasing considerably the pre-test probability. The model was created using the variables: the percentage of HbA2, serum ferritin and mean corpuscular volume standardized by age. The model was trained in 134 patients and validated in 160 randomly selected patients from the total sample. We used Youden’s index applied to the ROC curve methodology to establish the optimal odds ratio (OR) cut-off for the presence of two or more α-globin gene deletions. Using the OR cut-off of 0.4, the model’s negative predictive value (NPV) was 96.8%; the cut-off point accuracy was 85.4%; and the molecular analysis pre-test probability increased from 25.9% to 65.4% after the use of the proposed model. This tool aims to assist the physician in deciding when to perform molecular studies for the diagnosis of α-thalassemia. The model is useful in places with few financial health resources.

Pediatric cancer is a relatively rare and heterogeneous group of hematological and non-hematological malignancies which require multiple procedures for its diagnostic screening and classification. Until now, flow cytometry (FC) has not been systematically applied to the diagnostic work-up of such malignancies, particularly for solid tumors. Here we evaluated a FC panel of markers for the diagnostic screening of pediatric cancer and further classification of pediatric solid tumors. The proposed strategy aims at the differential diagnosis between tumoral vs. reactive samples, and hematological vs. non-hematological malignancies, and the subclassification of solid tumors. In total, 52 samples from 40 patients suspicious of containing tumor cells were analyzed by FC in parallel to conventional diagnostic procedures. The overall concordance rate between both approaches was of 96% (50/52 diagnostic samples), with 100% agreement for all reactive/inflammatory and non-infiltrated samples as well as for those corresponding to solid tumors (n = 35), with only two false negative cases diagnosed with Hodgkin lymphoma and anaplastic lymphoma, respectively. Moreover, clear discrimination between samples infiltrated by hematopoietic vs. non-hematopoietic tumor cells was systematically achieved. Distinct subtypes of solid tumors showed different protein expression profiles, allowing for the differential diagnosis of neuroblastoma (CD56(hi)/GD2(+)/CD81(hi)), primitive neuroectodermal tumors (CD271(hi)/CD99(+)), Wilms tumors (>1 cell population), rhabdomyosarcoma (nuMYOD1(+)/numyogenin(+)), carcinomas (CD45(-)/EpCAM(+)), germ cell tumors (CD56(+)/CD45(-)/NG2(+)/CD10(+)) and eventually also hemangiopericytomas (CD45(-)/CD34(+)). In summary, our results show that multiparameter FC provides fast and useful complementary data to routine histopathology for the diagnostic screening and classification of pediatric cancer.