Retrospective analysis of percutaneous intervention of the renal artery in transplanted kidneys in children and adolescents at a tertiary public hospital.

BACKGROUND: This study evaluated the long-term effects of percutaneous intervention in children and adolescents with transplant renal artery stenosis (TRAS). METHODS: Twenty patients had significant stenosis (>50%) and underwent percutaneous transluminal angioplasty (PTA/stenting) (TRAS group-intervention); 14 TNS (non-significant group -control) patients did not have significant stenosis (≤50%) and were treated clinically. The combined primary endpoints were death from all causes and late graft failure. The secondary endpoints were serum creatinine (SCr), systolic blood pressure (SBP), and diastolic blood pressure (DBP). RESULTS: No statistically significant difference was found between TRAS-Intervention(N = 20) and TNS groups-Control (N = 14) for these clinical parameters: deaths, 1 (5.0%) vs. 0 (0.0%) (p = 1.000) and graft loss, 4 (20.0%) vs. 2 (14.3%) (p = 1.000). For the secondary endpoints, after 1 month and 1 year the values of SCr, SBP, and DBP were similar between the two groups but not statistically significant. DISCUSSION: In the TRAS group (intervention), the stent implantation was beneficial for treating refractory hypertension and reducing blood pressure (BP) in children and adolescents. Despite the outcomes being similar in the two groups, it can be inferred that the patients in the TRAS group (intervention) would have had a worse outcome without the percutaneous intervention. CONCLUSION: TRAS treatment with stenting can be considered for children and adolescents. Because the sample in the present study comprised of only a specific population, further studies are needed for generalization. TRIAL REGISTRATION: The trial was registered at clinictrials.gov with trial registration number NCT04225338.

Pharmacological Modulation of the Ca2+/cAMP/Adenosine Signaling in Cardiac Cells as a New Cardioprotective Strategy to Reduce Severe Arrhythmias in Myocardial Infarction.

Acute myocardial infarction (AMI) is the main cause of morbidity and mortality worldwide and is characterized by severe and fatal arrhythmias induced by cardiac ischemia/reperfusion (CIR). However, the molecular mechanisms involved in these arrhythmias are still little understood. To investigate the cardioprotective role of the cardiac Ca2+/cAMP/adenosine signaling pathway in AMI, L-type Ca2+ channels (LTCC) were blocked with either nifedipine (NIF) or verapamil (VER), with or without A1-adenosine (ADO), receptors (A1R), antagonist (DPCPX), or cAMP efflux blocker probenecid (PROB), and the incidence of ventricular arrhythmias (VA), atrioventricular block (AVB), and lethality (LET) induced by CIR in rats was evaluated. VA, AVB and LET incidences were evaluated by ECG analysis and compared between control (CIR group) and intravenously treated 5 min before CIR with NIF 1, 10, and 30 mg/kg and VER 1 mg/kg in the presence or absence of PROB 100 mg/kg or DPCPX 100 µg/kg. The serum levels of cardiac injury biomarkers total creatine kinase (CK) and CK-MB were quantified. Both NIF and VER treatment were able to attenuate cardiac arrhythmias caused by CIR; however, these antiarrhythmic effects were abolished by pretreatment with PROB and DPCPX. The total serum CK and CK-MB were similar in all groups. These results indicate that the pharmacological modulation of Ca2+/cAMP/ADO in cardiac cells by means of attenuation of Ca2+ influx via LTCC and the activation of A1R by endogenous ADO could be a promising therapeutic strategy to reduce the incidence of severe and fatal arrhythmias caused by AMI in humans.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Factors related to the readiness of Brazilian chronic pediatric patients to transition to care in adult clinics.

OBJECTIVE: Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. METHOD: In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. RESULTS: The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. CONCLUSION: TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores.

Follow-up of children with sickle cell anemia screened with transcranial Doppler and enrolled in a primary prevention program of ischemic stroke

ABSTRACT Background: Stroke is a serious complication of sickle cell anemia (SCA). The transcranial Doppler (TCD) is the risk-screening tool for ischemic strokes. The objective of the study was to describe the clinical progression of children with SCA who presented with high risk for stroke by TCD or relevant changes by magnetic resonance angiography (MRA) and underwent the regular transfusion program (RTP) and/or hydroxyurea (HU) treatment between 2007 and 2018. Method: This was a neonatal retrospective/prospective cohort study with children born between 1999 and 2014 with the homozygotic form (HbSS) or Sβ0-thalassemia who underwent TCD at least once. Results: Of the 718 children screened during this period, 675 had HbSS and 43 Sβ0-thalassemia. In 54 children (7.5%), all with HbSS, a high-risk TCD (n = 45) or, when the TCD was inconclusive, an MRA with cerebral vasculopathy (n = 9) was used for detection. Of these, 51 started the RTP and the families of three refused treatment. Of the 43 children with a highrisk TCD who initiated the RTP, 29 (67.4%) reverted to low risk. In 18 of them (62%), HU was started at the maximum tolerated dose (MTD) before transfusion discontinuation. None of these 29 patients had a stroke. Eight children (18.6%) maintained a high-risk TCD, even using the RTP/HU and two had a stroke. Conclusions: The TCD was confirmed as a viable tool for tracking patients with a risk for stroke. The RTP was effective in preventing the primary event. New strategies are necessary to prevent stroke using HU and new drugs, in addition to bone marrow transplantation.

Growth Hormone/Insulin-like Growth Factor 1 Axis Associated with Modifier Factors in Children with Sickle Cell Anemia.

BACKGROUND: Sickle cell anemia is a disease that develops episodes of acute pain and multiple organ dysfunction that can affect the growth hormone/insulin-like growth factor 1 (GH/IGF-1) axis. The severity of sickle cell anemia is influenced by modifying factors, such as levels of fetal hemoglobin (HbF), the co-inheritance of alphathalassemia, or treatment with hydroxyurea. METHODS: This cross-sectional study in children with sickle cell anemia evaluated bone age (BA), adult height prediction (AHP) using BA, a target height (TH) calculated as the mean SDS of the parents, and laboratory parameters. Children were grouped according to serum levels of HbF, co-inheritance of alpha-thalassemia, and hydroxyurea therapy.. RESULTS: The mean age of the 39 children was 8.2 ± 2.2 years old. The average height was -0.75 ± 0.30 SDS, and 10.3% (4/39) had short stature. Adjusted levels of IGF-1 or IGFBP- 3 were significantly higher in children with sickle cell anemia on hydroxyurea treatment, in children with HbF levels >10%, and in those without alpha-thalassemia. Using SDS, the growth potential of children with sickle cell anemia in relation to their parents calculated by the difference between AHP and TH as well as the difference between children's height and their TH, were lower in children with co-inheritance of alphathalassemia. CONCLUSION: The study showed an association between modifying factors and the GH/IGF-1 axis in children with sickle cell anemia. Additionally, the co-inheritance of alpha-thalassemia was associated with decreased height in these children when adjusted for their parents' height.

Follow-up of children with sickle cell anemia screened with transcranial Doppler and enrolled in a primary prevention program of ischemic stroke.

BACKGROUND: Stroke is a serious complication of sickle cell anemia (SCA). The transcranial Doppler (TCD) is the risk-screening tool for ischemic strokes. The objective of the study was to describe the clinical progression of children with SCA who presented with high risk for stroke by TCD or relevant changes by magnetic resonance angiography (MRA) and underwent the regular transfusion program (RTP) and/or hydroxyurea (HU) treatment between 2007 and 2018. METHOD: This was a neonatal retrospective/prospective cohort study with children born between 1999 and 2014 with the homozygotic form (HbSS) or Sß0-thalassemia who underwent TCD at least once. RESULTS: Of the 718 children screened during this period, 675 had HbSS and 43 Sß0-thalassemia. In 54 children (7.5%), all with HbSS, a high-risk TCD (n = 45) or, when the TCD was inconclusive, an MRA with cerebral vasculopathy (n = 9) was used for detection. Of these, 51 started the RTP and the families of three refused treatment. Of the 43 children with a high-risk TCD who initiated the RTP, 29 (67.4%) reverted to low risk. In 18 of them (62%), HU was started at the maximum tolerated dose (MTD) before transfusion discontinuation. None of these 29 patients had a stroke. Eight children (18.6%) maintained a high-risk TCD, even using the RTP/HU and two had a stroke. CONCLUSIONS: The TCD was confirmed as a viable tool for tracking patients with a risk for stroke. The RTP was effective in preventing the primary event. New strategies are necessary to prevent stroke using HU and new drugs, in addition to bone marrow transplantation.

The complexity of having a child with a congenital heart defect in a developing country: A qualitative study of parental needs.

PURPOSE: To explore parental needs related to their experiences of living with a child with congenital heart defect (CHD) since the diagnosis. DESIGN AND METHODS: An interpretative qualitative study developed with nine parents of children between the ages of five months and 11 years diagnosed with CHD. Interviews were conducted at an ambulatory pediatric cardiology centre. Data were analyzed using inductive thematic analysis. The consolidated criteria for reporting qualitative research (COREQ) was followed for quality reporting. This research was approved by a research committee. RESULTS: One central theme emerged, namely 'A desire to feel safe in dealing with the demands of CHD,' along with two main themes. The first is 'Looking for effective relations with healthcare professionals and health care systems' which encompasses three types of need: (1) need for continuous, clear and accurate information; (2) need for resolution and the support of services such as the public health care system and social services; (3) need for trust in health care professionals. The second theme is 'Looking for balance in daily life' with two main needs: (1) maintain family functioning and (2) learn to deal with the child and CHD. CONCLUSION: The main parental needs are related to their interactions with healthcare professionals and healthcare systems, highlighted by a need for information and trust relationships to feel safe in their daily lives. PRACTICE IMPLICATIONS: Our results imply rethinking the nurse presence in ambulatory care, implementation of a family-centered care approach and addressing the diverse and multifaceted experiences and needs of parents and children with CHD in different health care contexts.

Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease.

Cerebrovascular disease, particularly stroke, is one of the most severe clinical complications associated with sickle cell disease and is a significant cause of morbidity in both children and adults. Over the past two decades, considerable advances have been made in the understanding of its natural history and enabled early identification and treatment of children at the highest risk. Transcranial Doppler screening and regular blood transfusions have markedly reduced the risk of stroke in children. However, transcranial Doppler has a limited positive predictive value and the pathophysiology of cerebrovascular disease is not completely understood. In this review, we will focus on the current state of knowledge about risk factors associated with ischemic stroke in patients with sickle cell disease. A search of PubMed was performed to identify studies. Full texts of the included articles were reviewed and data were summarized in a table. The coinheritance of alpha-thalassemia plays a protective role against ischemic stroke. The influence of other genetic risk factors is controversial, still preliminary, and requires confirmatory studies. Recent advances have established the reticulocyte count as the most important laboratory risk factor. Clinical features associated with acute hypoxemia as well as silent infarcts seem to influence the development of strokes in children. However, transcranial Doppler remains the only available clinical prognostic tool to have been validated. If our understanding of the many risk factors associated with stroke advances further, it may be possible to develop useful tools to detect patients at the highest risk early, improving the selection of children requiring intensification therapy.

Genetic, laboratory and clinical risk factors in the development of overt ischemic stroke in children with sickle cell disease

ABSTRACT Cerebrovascular disease, particularly stroke, is one of the most severe clinical complications associated with sickle cell disease and is a significant cause of morbidity in both children and adults. Over the past two decades, considerable advances have been made in the understanding of its natural history and enabled early identification and treatment of children at the highest risk. Transcranial Doppler screening and regular blood transfusions have markedly reduced the risk of stroke in children. However, transcranial Doppler has a limited positive predictive value and the pathophysiology of cerebrovascular disease is not completely understood. In this review, we will focus on the current state of knowledge about risk factors associated with ischemic stroke in patients with sickle cell disease. A search of PubMed was performed to identify studies. Full texts of the included articles were reviewed and data were summarized in a table. The coinheritance of alpha-thalassemia plays a protective role against ischemic stroke. The influence of other genetic risk factors is controversial, still preliminary, and requires confirmatory studies. Recent advances have established the reticulocyte count as the most important laboratory risk factor. Clinical features associated with acute hypoxemia as well as silent infarcts seem to influence the development of strokes in children. However, transcranial Doppler remains the only available clinical prognostic tool to have been validated. If our understanding of the many risk factors associated with stroke advances further, it may be possible to develop useful tools to detect patients at the highest risk early, improving the selection of children requiring intensification therapy.

Interleukin-10 haplotypes are not associated with acute cerebral ischemia or high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia.

BACKGROUND: The etiology of stroke, a severe complication of sickle cell anemia, involves inflammatory processes. However, the pathogenetic mechanisms are unknown. The aim of this study was to evaluate the influence of interleukin-10 polymorphisms and haplotypes on the risk of acute cerebral ischemia and high-risk transcranial Doppler in 395 children with sickle cell anemia from the state of Minas Gerais, Brazil. METHODS: Interleukin-10 haplotypes were determined by polymerase chain reaction-restriction fragment length polymorphism and sequencing. The outcomes studied were acute cerebral ischemia and high-risk transcranial Doppler. Clinical data were retrieved from the children's records. RESULTS: There was no statistically significant difference in the frequencies of polymorphisms and haplotypes between children with and without acute cerebral ischemia or children with or without high-risk transcranial Doppler. These data are consistent with a previous report that showed an absence of association between interleukin-10 plasma levels and high-risk transcranial Doppler velocity in children with sickle cell anemia. CONCLUSION: Interleukin-10 haplotypes were not associated with the risk of acute cerebral ischemia or high-risk transcranial Doppler velocity in children with sickle cell anemia from the state of Minas Gerais, Brazil.

Interleukin-10 haplotypes are not associated with acute cerebral ischemia or high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia

ABSTRACT Background: The etiology of stroke, a severe complication of sickle cell anemia, involves inflammatory processes. However, the pathogenetic mechanisms are unknown. The aim of this study was to evaluate the influence of interleukin-10 polymorphisms and haplotypes on the risk of acute cerebral ischemia and high-risk transcranial Doppler in 395 children with sickle cell anemia from the state of Minas Gerais, Brazil. Methods: Interleukin-10 haplotypes were determined by polymerase chain reaction-restriction fragment length polymorphism and sequencing. The outcomes studied were acute cerebral ischemia and high-risk transcranial Doppler. Clinical data were retrieved from the children's records. Results: There was no statistically significant difference in the frequencies of polymorphisms and haplotypes between children with and without acute cerebral ischemia or children with or without high-risk transcranial Doppler. These data are consistent with a previous report that showed an absence of association between interleukin-10 plasma levels and high-risk transcranial Doppler velocity in children with sickle cell anemia. Conclusion: Interleukin-10 haplotypes were not associated with the risk of acute cerebral ischemia or high-risk transcranial Doppler velocity in children with sickle cell anemia from the state of Minas Gerais, Brazil.

Ecocardiograma: da Solicitação do Exame pelo Pediatra à Realização pelo Cardiologista Pediátrico / Echocardiography: The Examination Request by the Pediatriciant Achieving the Pediatric Cardiologist

Fundamento: O ecocardiograma é uma ferramenta diagnóstica utilizada para avaliar anomalias cardíacas e esclarecer dúvidas quanto ao caráter benigno de certos achados do exame clínico cardiovascular. Objetivos: Identificar os principais motivos da solicitação do ecocardiograma pelo pediatra e avaliar o grau de ansiedade dos familiares gerado por essa conduta. Métodos: Foram incluídos pacientes com idade inferior a 18 anos, sem diagnóstico prévio de cardiopatia e encaminhados para o primeiro exame de ecocardiograma. Os familiares responsáveis pelos pacientes responderam a um questionário para avaliar o grau de ansiedade deles desde o pedido até a realização do ecocardiograma, com pontuação entre 0 e 19. Os dados foram analisados através de porcentagens, desvio padrão e teste T de Student. Resultados: Estudados 30 pacientes com idade média de 4,45 anos. O motivo de encaminhamento mais prevalente foi sopro cardíaco (23 casos), dos quais 70% não tiveram a hipótese de cardiopatia congênita confirmada após o ecocardiograma. A pontuação média no questionário de ansiedade foi 11 ± 6, sendo relacionado significativamente à presença de cardiopatia, com média de 13,0 ± 5,3 vs. 9,3 ± 5,2 no grupo sem a hipótese da mesma (p = 0,007). Conclusão: O achado de sopro foi o principal motivo para solicitação de ecocardiograma pelo pediatra. A confirmação de cardiopatia foi maior nos menores de 1 ano e com achado de sopro. Apesar do grau de ansiedade ter sido maior no grupo dos pacientes que tiveram o diagnóstico de cardiopatia congênita, este não foi desprezível nos familiares no grupo dos pacientes sem cardiopatia

Background: Echocardiography is a diagnostic tool used to evaluate cardiac anomalies and clarify doubts about the benign nature of certain findings of cardiovascular clinical examination. Objectives: To identify the main reasons for the request of echocardiography by pediatrician and evaluate the degree of anxiety of the family generated by this conduct. Methods: We included patients younger than 18 years without previous diagnosis of heart disease and referred for the first echocardiogram. Family members responsible for the patients answered a questionnaire to assess the degree of their anxiety from order to the performance of the echocardiogram, with scores between 0 and 19. Data were analyzed using percentages, standard deviation and Student's t test. Results: Studied 30 patients with a mean age of 4.45 years. The most prevalent reason for referral was heart murmur (23 cases), of which 70% patients had no congenital heart disease hypothesis confirmed, after echocardiography. The average score on the anxiety questionnaire was 11 ± 6 significantly associated with the presence of heart disease, with a mean of 13.0 ± 5.3 vs. 9.3 ± 5.2 in the group without the possibility of it (p = 0.007). Conclusion: The murmurs findings was the main reason for echocardiography request by the pediatrician. Confirmation of heart disease was higher in children under 1 year and with murmurs findings. Despite the anxiety level was higher in the group of patients who were diagnosed with congenital heart disease, this was not negligible in the family in the group of patients without heart disease

Reticulocyte count is the most important predictor of acute cerebral ischemia and high-risk transcranial Doppler in a newborn cohort of 395 children with sickle cell anemia.

Stroke is a severe clinical manifestation of sickle cell anemia (SCA). Despite the prognostic relevance of transcranial Doppler (TCD), more accurate tools to assess stroke risk in children with SCA are required. Here, we describe the effect of clinical, laboratory, and molecular features on the risk of stroke and high-risk TCD in children from the newborn cohort of Minas Gerais, Brazil. Outcomes studied were acute cerebral ischemia and high-risk TCD. Clinical and hematological data were retrieved from children's records. Genetic markers, which were known for their association with stroke risk, were genotyped by polymerase chain reaction/restriction fragment length polymorphism and sequencing. The cumulative incidence of acute cerebral ischemia by the age of 8 years was 7.4 % and that of high-risk TCD by the age of 11.5 years was 14.2 %. The final multivariate model for acute cerebral ischemia risk included high white blood cell count and reticulocyte count, acute chest syndrome rate, and the single nucleotide polymorphisms (SNPs) TEK rs489347 and TNF-α rs1800629. The model for high-risk TCD included high reticulocyte count and the SNPs TEK rs489347 and TGFBR3 rs284875. Children with risk factors should be considered for intensive risk monitoring and for intervention therapy.

Clinical, hematological and genetic data of a cohort of children with hemoglobin SD.

INTRODUCTION: The hemoglobin FSD is very uncommon in newborn screening programs for sickle cell disease. In the program of Minas Gerais, Brazil, the clinical course of children with hemoglobin SD was observed to be heterogeneous. The objective of this study was to estimate the incidence (1999-2012) and to describe the natural history of a cohort of newborns with hemoglobin SD. METHODS: Isoelectric focusing was the primary method used in newborn screening. Polymerase chain reaction-restriction fragment length polymorphism and gene sequencing were used to identify mutant alleles and for haplotyping. Gap-polymerase chain reaction was used to detect alpha-thalassemia. RESULTS: Eleven cases of hemoglobin S/D-Punjab and eight of Hb S-Korle Bu were detected. Other variants with hemoglobin D mobility were not identified. All hemoglobin D-Punjab and hemoglobin Korle Bu alleles were associated with haplotype I. Among the children with hemoglobin S/D-Punjab, there were four with the ß(S) CAR haplotype, six with the Benin haplotype, and one atypical. Results of laboratory tests for hemoglobin S/D-Punjab and hemoglobin S-Korle Bu were: hemoglobin 8.0 and 12.3g/dL (p-value <0.001), leukocyte count 13.9×10(9)/L and 10.5×10(9)/L (p-value=0.003), reticulocytes 7.5% and 1.0% (p-value <0.001), hemoglobin F concentration 16.1% and 6.9% (p-value=0.001) and oxygen saturation 91.9% and 97% (p-value=0.002), respectively. Only hemoglobin S/D-Punjab children had acute pain crises and needed blood transfusions or hydroxyurea. Those with the Benin ß(S) haplotype had higher total hemoglobin and hemoglobin F concentrations compared to the CAR haplotype. Transcranial Doppler was normal in all children. CONCLUSION: The clinical course and blood cell counts of children with hemoglobin S/D-Punjab were very similar to those of hemoglobin SS children. In contrast, children with hemoglobin S-Korle Bu had clinical course and blood cell counts like children with the sickle cell trait.

Prevalence and incidence of erythrovirus B19 infection in children with sickle cell disease: The impact of viral infection in acute clinical events.

B19V infection is common during childhood. It is self-limited in healthy individuals, but is often associated with transient aplastic crisis in children with sickle cell disease. The aim of this study was to estimate the prevalence and incidence of B19V infection in children with sickle cell disease screened by the Newborn Screening Program of Minas Gerais, Brazil, and followed-up at Fundação Hemominas. Serum or plasma samples from 278 patients were tested for anti-B19V IgG and IgM using commercial ELISA and for viral DNA using in-house real-time PCR assays; 127 negative-children were retested about 1 year later. The median age of children at first testing was 5.9 years (0.8-12.3). The estimated prevalence of B19V was 29.5 % (95%CI 24.1-34.9 %). The incidence of B19V in those 127 negative-children was 18.2 cases/100 patient-years. All DNA-positive samples were identified as genotype 1, except one sample, in which both genotypes 1 and 3 were identified. It was observed that the higher the child's age, the higher the probability of B19V infection. The analysis of clinical and hematological data showed a significant association of B19V infection with transient aplastic crisis and acute splenic sequestration, higher frequency of transfusions, and higher rate of hospitalization, but not with acute chest syndrome or stroke. These results emphasize the impact of B19V infection on the course of sickle cell disease. Strategies to prevent and monitor B19V infection in children with sickle cell disease should be considered to diminish its morbidity in this susceptible population.

Clinical, hematological and genetic data of a cohort of children with hemoglobin SD

Introduction The hemoglobin FSD is very uncommon in newborn screening programs for sickle cell disease. In the program of Minas Gerais, Brazil, the clinical course of children with hemoglobin SD was observed to be heterogeneous. The objective of this study was to estimate the incidence (1999­2012) and to describe the natural history of a cohort of newborns with hemoglobin SD. Methods Isoelectric focusing was the primary method used in newborn screening. Polymerase chain reaction-restriction fragment length polymorphism and gene sequencing were used to identify mutant alleles and for haplotyping. Gap-polymerase chain reaction was used to detect alpha-thalassemia. Results Eleven cases of hemoglobin S/D-Punjab and eight of Hb S-Korle Bu were detected. Other variants with hemoglobin D mobility were not identified. All hemoglobin D-Punjab and hemoglobin Korle Bu alleles were associated with haplotype I. Among the children with hemoglobin S/D-Punjab, there were four with the ßS CAR haplotype, six with the Benin haplotype, and one atypical. Results of laboratory tests for hemoglobin S/D-Punjab and hemoglobin S-Korle Bu were: hemoglobin 8.0 and 12.3 g/dL (p-value <0.001), leukocyte count 13.9 × 109/L and 10.5 × 109/L (p-value = 0.003), reticulocytes 7.5% and 1.0% (p-value <0.001), hemoglobin F concentration 16.1% and 6.9% (p-value = 0.001) and oxygen saturation 91.9% and 97% (p-value = 0.002), respectively. Only hemoglobin S/D-Punjab children had acute pain crises and needed blood transfusions or hydroxyurea. Those with the Benin ßS haplotype had higher total hemoglobin and hemoglobin F concentrations compared to the CAR haplotype. Transcranial Doppler was normal in all children. Conclusion The clinical course and blood cell counts of children with hemoglobin S/D-Punjab were very similar to those of hemoglobin SS children. In contrast, children with hemoglobin S-Korle Bu had clinical course and blood cell counts like children with the sickle cell trait.

Indicações atuais para anticoagulacão em crianças com Cardiopatias Congênitas / Current indications for anticoagulation in children with Congenital Heart Disease

Os avanços nas técnicas cirúrgicas e manejo clínico aumentaram a sobrevida das crianças com cardiopatia congênita. Eventos tromboembólicos representam importante causa de morbimortalidade nestes pacientes, principalmente no pós-operatório da cirurgia de Fontan e implante de próteses, acrescido do risco adicional do seu tratamento. Particularidades próprias da idade, fatores hemodinâmicos e limitações na investigação de trombose contribuem para a dificuldadede definir diretrizes para anticoagulação em crianças. Os medicamentos mais usados na fase aguda são: heparina não fracionada e de baixo peso molecular, enquanto em longo prazo os antagonistas da vitamina K, principalmente a varfarina. O manejo seguro de anticoagulação nesta faixa etária é dificultado pela estreita faixa terapêutica, interação alimentar, uso simultâneo de vários medicamentos, além da dificuldade de punção venosa frequente e, com frequência os níveis desejáveis não são atingidos. Ainda faltam dados que deem suporte para recomendação de anticoagulação em crianças. Os novos anticoagulantes como os inibidores diretos da trombina e os inibidores seletivos do fator Xa, são empregados em adultos, principalmente com fibrilação atrial, o uso pediátrico ainda não está definido. Esta revisão sumariza as indicações atuais do uso de anticoagulantes e antiagregantes plaquetários em crianças com cardiopatia congênita.

Advances in surgical techniques and clinical management have led to increased survival ofchildren with congenital heart disease. Thromboembolic events represent a major cause of morbidity and mortality in these patients, particularly following Fontan surgery and the implantation of prosthesis, added to the risk of their treatment. Characteristics such as age, hemodynamic factors and limitations in the investigation of thrombosis have contributed to the difficulty of defining guidelines for anticoagulation in children. The most commonly used medications in the acute phase are and low molecular weight heparin, and in the long term, vitamin K antagonists, particularly warfarin. The safe management of anticoagulation in this age range is hindered by the narrow therapeutic range, food interactions, simultaneous use of other medications, and the difficulty of frequent venous puncture. As a result, the desired levels are often not reached. There is also a lack of data to support the recommendation of anticoagulation in children. The pediatric use of the new anticoagulants, such as direct thrombin inhibitors and selective factor Xa inhibitors used in adults, particularly with atrial fibrillation, is not yet defined. This review summarizes the current indications of the use of anticoagulants and platelet antiaggregants in children with congenital heart disease.