Position paper of diagnosis and treatment of post-extubation laryngitis in children: a multidisciplinary expert-based opinion.

OBJECTIVES: To make recommendations on the diagnosis and treatment of post-extubation laryngitis (PEL) in children with or without other comorbidities. METHODS: A three-iterative modified Delphi method was applied. Specialists were recruited representing pediatric otolaryngologists, pediatric and neonatal intensivists. Questions and statements approached topics encompassing definition, diagnosis, endoscopic airway evaluation, risk factors, comorbidities, management, and follow-up. A consensus was defined as a supermajority >70%. RESULTS: Stridor was considered the most frequent symptom and airway endoscopy was recommended for definitive diagnosis. Gastroesophageal reflux and previous history of intubation were considered risk factors. Specific length of intubation did not achieve a consensus as a risk factor. Systemic corticosteroids should be part of the medical treatment and dexamethasone was the drug of choice. No consensus was achieved regarding dosage of corticosteroids, although endoscopic findings help defining dosage and length of treatment. Non-invasive ventilation, laryngeal rest, and use of comfort sedation scales were recommended. Indications for microlaryngoscopy and bronchoscopy under anesthesia were symptoms progression or failure to improve after the first 72-h of medical treatment post-extubation, after two failed extubations, and/or suspicion of severe lesions on flexible fiberoptic laryngoscopy. CONCLUSIONS: Management of post-extubation laryngitis is challenging and can be facilitated by a multidisciplinary approach. Airway endoscopy is mandatory and impacts decision-making, although there is no consensus regarding dosage and length of treatment.

Adverse events in the postoperative period of cardiac surgery in a pediatric intensive care unit: the contribution of the VIS score and the RACHS-1 / Eventos adversos em pós-operatório cardíaco em uma unidade de terapia intensiva pediátrica: a contribuição do escore VIS e do RACHS-1

ABSTRACT Objective: To evaluate the occurrence of adverse events in the postoperative period of cardiac surgery in a pediatric intensive care unit and to find any patient characteristics that can predict such events. Methods: This was a historical cohort study of patients recovering in the pediatric intensive care unit for the first 7 days after cardiac surgery between April and December 2019, by reviewing the medical records. The following were reviewed: demographic, clinical, and laboratory characteristics; patient severity scores; and selected adverse events, grouped into device-related, surgical, and nonsurgical. Results: A total of 238 medical records were included. At least one adverse event occurred in 110 postoperative patients (46.2%). The total number of adverse events was 193 (81%). Vascular catheters were the most common cause, followed by cardiac arrest, bleeding, and surgical reexploration. In the univariate analysis, the vasoactive-inotropic score (VIS), Risk Adjustment in Congenital Heart Surgery (RACHS-1) score, age, Pediatric Index of Mortality (PIM-2), cardiopulmonary bypass and aortic clamping duration were significantly associated with adverse events. In the multivariate analysis, VIS ≥ 20 (OR 2.90; p = 0.004) and RACHS-1 ≥ 3 (OR 2.11; p = 0.019) were significant predictors, while age and delayed sternal closure showed only trends toward significance. To predict the occurrence of adverse events from VIS and RACHS-1, the area under the curve was 0.73 (95%CI 0.66 - 0.79). Conclusion: Adverse events were quite frequent in children after cardiac surgery, especially those related to devices. The VIS and RACHS-1, used together, predicted the occurrence of adverse events well in this pediatric sample.

RESUMO Objetivo: Avaliar a ocorrência de eventos adversos em pós-operatório cardíaco em uma unidade de terapia intensiva pediátrica e estabelecer eventuais associações das características dos pacientes e a possibilidade de predizer tais eventos. Métodos: Coorte histórica de 7 dias de pós-operatório cardíaco, de abril a dezembro de 2019, por revisão de prontuários de pacientes com recuperação em unidade de terapia intensiva pediátrica. Foram revisados: características demográficas e clínico-laboratoriais, escores de gravidade dos pacientes e eventos adversos selecionados agrupados em: relacionados a dispositivos, a aspectos cirúrgicos e a aspectos não cirúrgicos. Resultados: Foram incluídos 238 prontuários. Ocorreu pelo menos um evento adverso em 110 pós-operatórios (46,2 %). O número total de eventos adversos foi 193 (81%), sendo mais frequente a complicação com cateteres vasculares, seguida de parada cardíaca, sangramento e reexploração cirúrgica. Na análise univariada, escore vasoativo-inotrópico (VIS- vasoactive-inotropic score), Risk Adjustment in Congenital Heart Surgery (RACHS-1) score, idade, Pediatric Index of Mortality (PIM-2), tempo de circulação extracorpórea e de clampeamento aórtico foram estatisticamente significantes com eventos adversos. Na análise multivariável, VIS ≥ 20 (OR 2,90; p = 0,004) e RACHS-1 ≥ 3 (OR 2,11; p = 0,019) mostraram-se relevantes e com significância estatística, enquanto idade e fechamento tardio do esterno possuíam apenas tendência a essa associação. Considerando a previsão de ocorrência de eventos adversos a partir dos valores de escore vasoativo-inotrópico e de RACHS-1, a área sob a curva mostrou valor de 0,73 (IC95% 0,66 - 0,79). Conclusão: A frequência de eventos adversos foi expressiva e aqueles relacionados a dispositivos foram os mais frequentes. O VIS e o RACHS-1, utilizados em conjunto, foram capazes de predizer a ocorrência de eventos adversos nesta amostra pediátrica.

Adverse events in the postoperative period of cardiac surgery in a pediatric intensive care unit: the contribution of the VIS score and the RACHS-1.

OBJECTIVE: To evaluate the occurrence of adverse events in the postoperative period of cardiac surgery in a pediatric intensive care unit and to find any patient characteristics that can predict such events. METHODS: This was a historical cohort study of patients recovering in the pediatric intensive care unit for the first 7 days after cardiac surgery between April and December 2019, by reviewing the medical records. The following were reviewed: demographic, clinical, and laboratory characteristics; patient severity scores; and selected adverse events, grouped into device-related, surgical, and nonsurgical. RESULTS: A total of 238 medical records were included. At least one adverse event occurred in 110 postoperative patients (46.2%). The total number of adverse events was 193 (81%). Vascular catheters were the most common cause, followed by cardiac arrest, bleeding, and surgical reexploration. In the univariate analysis, the vasoactive-inotropic score (VIS), Risk Adjustment in Congenital Heart Surgery (RACHS-1) score, age, Pediatric Index of Mortality (PIM-2), cardiopulmonary bypass and aortic clamping duration were significantly associated with adverse events. In the multivariate analysis, VIS ≥ 20 (OR 2.90; p = 0.004) and RACHS-1 ≥ 3 (OR 2.11; p = 0.019) were significant predictors, while age and delayed sternal closure showed only trends toward significance. To predict the occurrence of adverse events from VIS and RACHS-1, the area under the curve was 0.73 (95%CI 0.66 - 0.79). CONCLUSION: Adverse events were quite frequent in children after cardiac surgery, especially those related to devices. The VIS and RACHS-1, used together, predicted the occurrence of adverse events well in this pediatric sample.

Impact of the Implementation of a Vancomycin Protocol on Trough Serum Vancomycin Concentrations in a Pediatric Intensive Care Unit.

BACKGROUND: Vancomycin is an antibiotic that is widely used in pediatric intensive care, but the safe and effective use of this drug is challenging. OBJECTIVE: This study aimed to assess the impact of a vancomycin protocol on trough serum concentrations. METHODS: We conducted a retrospective quasiexperimental study in patients aged ≤ 18 years in intensive care who received vancomycin for at least 5 days. Patients were divided into two groups: before and after a protocol implemented in 2017 that suggested an initial vancomycin dose of 60 mg/kg/day, target serum levels of 15-20 µg/mL, and dose adjustments. We compared patient characteristics, target serum level achievement, and vancomycin levels over time. RESULTS: Each group contained 65 patients; most were male infants with heart disease as the main reason for hospitalization. Only 29.2% of the patients had pretreatment cultures for bacteria identification recorded, with 1.5% identified as methicillin-resistant Staphylococcus aureus. For the first serum levels, 10.8% of patients in the pre-protocol group and 21.5% in the post-protocol group achieved the 15-20 µg/mL target (p = 0.153); during the first 5 days of treatment, this proportion significantly increased from 52.3 to 73.8% (p = 0.018). We observed a difference between the first and fifth levels: 8.9 µg/mL (95% confidence interval [CI] - 3.1 to 21) pre-protocol and 0.4 µg/mL (95% CI - 6.1 to 6.9) post-protocol (p = 0.175). CONCLUSIONS: Reaching adequate trough vancomycin concentrations in critically ill pediatric patients remains a challenge, and clinical practice protocols allow better dose adjustment and control even when monitoring technologies are unavailable.

Functional deficit in children with congenital heart disease undergoing surgical correction after intensive care unit discharge. / Déficit funcional em crianças com cardiopatias congênitas submetidas à correção cirúrgica após alta da unidade de terapia intensiva.

OBJECTIVE: To evaluate the functional status of pediatric patients undergoing congenital heart surgery after discharge from the intensive care unit, and to evaluate the correlations among clinical variables, functional status and surgical risk. METHODS: Cross-sectional study including patients aged 1 month to less than 18 years undergoing congenital heart surgery between October 2017 and May 2018. Functional outcome was assessed by the Functional Status Scale, surgical risk classification was determined using the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and clinical variables were collected from electronic medical records. RESULTS: The sample comprised 57 patients with a median age of 7 months (2 - 17); 54.4% were male, and 75.5% showed dysfunction, which was moderate in 45.6% of the cases. RACHS-1 category > 3 was observed in 47% of the sample, indicating higher surgical risk. There was a correlation between functional deficit and younger age, longer duration of invasive mechanical ventilation and longer intensive care unit stay. Moreover, greater functional deficit was observed among patients classified as RACHS-1 category > 3. CONCLUSION: The prevalence of functional deficit was high among children and adolescents with congenital heart disease after cardiac surgery. Higher surgical risk, longer duration of invasive mechanical ventilation, longer intensive care unit stay and younger age were correlated with worse functional status.

Tolerability and Effects of the Use of Energy-Enriched Infant Formula After Congenital Heart Surgery: A Randomized Controlled Trial.

BACKGROUND: Undernutrition is a common problem among children with congenital heart disease (CHD) and may lead to poorer surgical outcomes. A higher intake of energy during the postoperative period of CHD surgery seems to be associated with better outcomes. This study aimed to investigate the effect of the use of energy-enriched formula (EE-formula) compared with normocaloric formula during 30 days after CHD surgery. METHODS: A randomized controlled trial with patients undergoing heart surgery in a tertiary hospital in southern Brazil from March 2017 to December 2017 was performed. The intervention group received EE-formula (1 kcal/mL), and the control group received normocaloric formula (0.67 kcal/mL). The researcher in charge of anthropometric evaluation was blinded to the randomization. RESULTS: Fifty-nine patients were included; 30 in control group and 29 in intervention group. There were no statistically significant differences between groups regarding age, gender, anthropometry, and surgical risk classification after randomization. A statistically significant difference in z-score of weight for age and in weight gain variation rate between groups after intervention was observed. Antibiotic use was less frequent in the intervention group, and hospital length of stay was shorter. General gastrointestinal side effects were similar between groups, whereas diarrhea was more frequent in the intervention group. However, this side effect was limited and had spontaneous resolution in 4 out of 6 cases. CONCLUSION: This study demonstrates that EE-formula use after heart surgery of patients with CHD is well tolerated and may improve short-term nutrition outcome, decrease hospital stay, and reduce antibiotic use.

Functional Status Scale: Cross-Cultural Adaptation and Validation in Brazil.

OBJECTIVE: The goal of the present study was to perform a cross-cultural adaptation and clinical validation of the Functional Status Scale for use in the Brazilian population. DESIGN: Cross-cultural adaptation study followed by a cross-sectional validation study. SETTING: Single-center PICU at a hospital in Porto Alegre, Brazil. PATIENTS: Children and adolescents of both sexes, 1 month and under 18 years old, who had been treated at the PICU. INTERVENTIONS: The cross-cultural adaptation consisted of the following stages: translation, synthesis of the translated versions, back translations, synthesis of the back translations, committee review, and pretesting. For the clinical validation stage, the Brazilian Functional Status Scale was applied within 48 hours after discharge from the PICU. The Brazilian Functional Status Scale's reliability and validity properties were tested. MEASUREMENTS AND MAIN RESULTS: A total of 314 patients were evaluated. Median age was 24 months (7.0-105.0 mo), 54.1% were males, and their overall functional score was 9 ± 2.8. The Brazilian Functional Status Scale demonstrated excellent interobserver reliability, with an intraclass correlation coefficient of 0.98, and κ coefficients between 0.716 and 1.000 for the functional domains, which indicated good to excellent agreement. Using the Bland-Altman method, we confirmed low variability among the evaluator's responses (0.93 to -1.06 points). Regarding the Brazilian Functional Status Scale's content validity, there was a correlation between length of PICU stay (r = 0.378; p < 0.001) and time on invasive mechanical ventilation (r = 0.261; p < 0.05), and the test could discriminate between groups with different comorbidity levels (p < 0.001). CONCLUSIONS: The Functional Status Scale has been culturally adapted and validated for use in Brazil and is now available for use in the assessment of functionality in Brazilian children and adolescents.

Avaliação do desempenho do PIM-2 entre pacientes cardiopatas cirúrgicos e correlação dos resultados com RACHS-1 / Assessment of PIM-2 performance among surgical patients with heart disease and correlation of results with RACHS-1

RESUMO Objetivo: Avaliar o desempenho do Pediatric Index of Mortality (PIM) 2 e do Escore de Risco Ajustado para Cirurgia Cardíaca Congênita (RACHS) no pós-operatório de cardiopatas congênitos. Métodos: Estudo transversal retrospectivo. Foram coletados dados de prontuário para gerar os escores e predições com as técnicas preconizadas, os dados demográficos e os desfechos. Para estatística, utilizaram-se o teste de Mann-Whitney, o teste de Hosmer-Lemeshow, a taxa de mortalidade padronizada, a área sobre a curva COR, qui quadrado, regressão de Poisson com variância robusta e teste de Spearman. Resultados: Foram avaliados 263 pacientes, e 72 foram a óbito (27,4%). Estes apresentaram valores de PIM-2 significativamente maiores que os sobreviventes (p < 0,001). Na classificação RACHS-1, a mortalidade foi progressivamente maior, de acordo com a complexidade do procedimento, com aumento de 3,24 vezes na comparação entre os grupos 6 e 2. A área abaixo da curva COR para o PIM-2 foi 0,81 (IC95% 0,75 - 0,87) e, para RACHS-1, de 0,70 (IC95% 0,63 - 0,77). A RACHS apresentou melhor poder de calibração na amostra analisada. Foi encontrada correlação significativamente positiva entre os resultados de ambos os escores (rs = 0,532; p < 0,001). Conclusão: A RACHS apresentou bom poder de calibração, e RACHS-1 e PIM-2 demonstraram bom desempenho quanto à capacidade de discriminação entre sobreviventes e não sobreviventes. Ainda, foi encontrada correlação positiva entre os resultados dos dois escores de risco.

ABSTRACT Objective: To assess the performance of the Pediatric Index of Mortality (PIM) 2 and the Risk Adjustment for Congenital Heart Surgery (RACHS) in the postoperative period of congenital heart disease patients. Methods: Retrospective cross-sectional study. Data were collected from patient records to generate the scores and predictions using recommended techniques, demographic data and outcomes. The Mann-Whitney test, Hosmer-Lemeshow test, standardized mortality rate, area under the receiver operating characteristic (ROC) curve, chi square test, Poisson regression with robust variance and Spearman's test were used for statistical analysis. Results: A total of 263 patients were evaluated, and 72 died (27.4%). These patients presented significantly higher PIM-2 values than survivors (p < 0.001). In the RACHS-1 classification, mortality was progressively higher according to the complexity of the procedure, with a 3.24-fold increase in the comparison between groups 6 and 2. The area under the ROC curve for PIM-2 was 0.81 (95%CI 0.75 - 0.87), while for RACHS-1, it was 0.70 (95%CI 0.63 - 0.77). The RACHS presented better calibration power in the sample analyzed. A significantly positive correlation was found between the results of both scores (rs = 0.532; p < 0.001). Conclusion: RACHS presented good calibration power, and RACHS-1 and PIM-2 demonstrated good performance with regard to their discriminating capacities between survivors and non-survivors. Moreover, a positive correlation was found between the results of the two risk scores.

Assessment of PIM-2 performance among surgical patients with heart disease and correlation of results with RACHS-1. / Avaliação do desempenho do PIM-2 entre pacientes cardiopatas cirúrgicos e correlação dos resultados com RACHS-1.

OBJECTIVE: To assess the performance of the Pediatric Index of Mortality (PIM) 2 and the Risk Adjustment for Congenital Heart Surgery (RACHS) in the postoperative period of congenital heart disease patients. METHODS: Retrospective cross-sectional study. Data were collected from patient records to generate the scores and predictions using recommended techniques, demographic data and outcomes. The Mann-Whitney test, Hosmer-Lemeshow test, standardized mortality rate, area under the receiver operating characteristic (ROC) curve, chi square test, Poisson regression with robust variance and Spearman's test were used for statistical analysis. RESULTS: A total of 263 patients were evaluated, and 72 died (27.4%). These patients presented significantly higher PIM-2 values than survivors (p < 0.001). In the RACHS-1 classification, mortality was progressively higher according to the complexity of the procedure, with a 3.24-fold increase in the comparison between groups 6 and 2. The area under the ROC curve for PIM-2 was 0.81 (95%CI 0.75 - 0.87), while for RACHS-1, it was 0.70 (95%CI 0.63 - 0.77). The RACHS presented better calibration power in the sample analyzed. A significantly positive correlation was found between the results of both scores (rs = 0.532; p < 0.001). CONCLUSION: RACHS presented good calibration power, and RACHS-1 and PIM-2 demonstrated good performance with regard to their discriminating capacities between survivors and non-survivors. Moreover, a positive correlation was found between the results of the two risk scores.

OBJETIVO: Avaliar o desempenho do Pediatric Index of Mortality (PIM) 2 e do Escore de Risco Ajustado para Cirurgia Cardíaca Congênita (RACHS) no pós-operatório de cardiopatas congênitos. MÉTODOS: Estudo transversal retrospectivo. Foram coletados dados de prontuário para gerar os escores e predições com as técnicas preconizadas, os dados demográficos e os desfechos. Para estatística, utilizaram-se o teste de Mann-Whitney, o teste de Hosmer-Lemeshow, a taxa de mortalidade padronizada, a área sobre a curva COR, qui quadrado, regressão de Poisson com variância robusta e teste de Spearman. RESULTADOS: Foram avaliados 263 pacientes, e 72 foram a óbito (27,4%). Estes apresentaram valores de PIM-2 significativamente maiores que os sobreviventes (p < 0,001). Na classificação RACHS-1, a mortalidade foi progressivamente maior, de acordo com a complexidade do procedimento, com aumento de 3,24 vezes na comparação entre os grupos 6 e 2. A área abaixo da curva COR para o PIM-2 foi 0,81 (IC95% 0,75 - 0,87) e, para RACHS-1, de 0,70 (IC95% 0,63 - 0,77). A RACHS apresentou melhor poder de calibração na amostra analisada. Foi encontrada correlação significativamente positiva entre os resultados de ambos os escores (rs = 0,532; p < 0,001). CONCLUSÃO: A RACHS apresentou bom poder de calibração, e RACHS-1 e PIM-2 demonstraram bom desempenho quanto à capacidade de discriminação entre sobreviventes e não sobreviventes. Ainda, foi encontrada correlação positiva entre os resultados dos dois escores de risco.

Gestational and family risk factors for carriers of congenital heart defects in southern Brazil.

BACKGROUND: Congenital heart disease (CHD) is a serious threat to public health. Despite this, its etiology is poorly understood and few cardiac teratogens have been defined. The aim of the present study was to identify gestational and family risk factors for CHD in a sample of patients from a pediatric hospital in southern Brazil. METHODS: A prospective and consecutive sample from subjects with or without CHD, hospitalized at a pediatric intensive care unit, was enrolled. All patients with CHD underwent a GTG-banding karyotype. Chromosomal abnormalities were observed in 47 subjects (15.8%), and these were excluded from the study. The final sample consisted of 250 CHD subjects and 303 controls. RESULTS: After statistical analysis, using logistic regression, the variables age, rural location, gestational loss, use of anti-hypertensive medication, antibiotics and alcohol in the first trimester of pregnancy were all independently associated with CHD. These results were similar to those of some studies and different from others. It should be noted, however, that, for several variables, the data in the literature as well as the present study were insufficient to determine risk. CONCLUSIONS: Some differences found may be explained by genetic factors and sociocultural diversity. In contrast, because CHD consists of a heterogeneous group of lesions, the etiology may vary. The standardization of research data and classification of methods for future studies are essential.

Serum lactate as mortality and morbidity marker in infants after Jatene's operation.

OBJECTIVE: To assess the morbidity and mortality after Jatene's operation using lactate as the main marker. METHODS: We performed a historical cohort with infants admitted in a pediatric intensive care unit during 1995 to 2005 who underwent this surgery. We assessed the preoperative, immediate (IPD), third hour (3h), six hour (6h) and first day (POD1) serum lactate as well as other factors such as sepsis, increased bleeding, low cardiac output syndrome, renal insufficiency, pulmonary hypertension, cardiac arrythmias, chylothorax, myocardial ischemia, seizures, presence of other complication, and also information about length of PICU stay and death. RESULTS: The mean age of 76 patients was 14.59± 19.09 days, birth weight 3.128± 0.48 kg Forty-four patients had the diagnosis of simple transposition of great arteries. The circulatory bypass time was 143.78± 28.77 minutes and aortic clamping time of 87.68± 22.3 minutes and LOS of 20.28 ± 15.62 days. Twenty four (31.58%) died during hospital stay. Lactate increased in IPD, returning to baseline at 24 hours. Patients who died raised and maintained IPD lactate higher. The 3h lactate best discriminated mortality with area under the curve of 0.68 (CI 0.54 to 0.83) P = 0.035. However, considering a cutoff point for lactate greater or equal to 5.8 mmol/dl in the 3-h PO, we obtained only 67% sensitivity and specificity of 64% for mortality. There is positive correlation between number of complications and lactate. The low cardiac output syndrome with an odds ratio (OR) of 7.67 (2.38-24), increased bleeding with OR 2.91 (1.07-7.94) and respiratory complication with OR 1.67 (1.35-2.05) are risk factors when combined. CONCLUSION: After Jatene's operation, morbidity and mortality can be assessed with the serum lactate levels, suggesting increased values in the third hour is suggestive of a worse prognosis.

Lactato sérico como marcador de morbimortalidade no pós-operatório de operação de Jatene em lactentes / Serum lactate as mortality and morbidity marker in infants after Jatene's operation

OBJETIVO: Avaliar a morbidade e mortalidade após a operação de Jatene utilizando a dosagem de lactato sérico como principal marcador. MÉTODOS: Foi realizada uma coorte histórica com lactentes da UTI no período de 1995 a 2005 submetidos a essa cirurgia. Foram avaliados o lactato do pré-operatório, pós-operatório imediato (POI), da terceira hora de PO (3ªh), sexta hora (6ªh) de PO e do 1º dia de PO; bem como outros fatores como sepse, sangramento aumentado, síndrome de baixo débito, insuficiência renal, hipertensão pulmonar, arritmias cardíacas, quilotórax, isquemia miocárdica, convulsões e outras complicações. Também foram coletadas informações referentes ao tempo de internação na UTI e ao desfecho (se o paciente morreu ou teve alta da UTI). RESULTADOS: A média de idade dos 76 pacientes foi de 14,59 ± 19,09 dias, peso ao nascimento de 3,128 ± 0,48 kg. Quarenta e quatro pacientes tinham o diagnóstico anatômico exclusivo de transposição de grandes artérias. O tempo médio de CEC foi de 143,78 ± 28,77 minutos, de pinçamento de 87,68 ± 22,3 minutos e de internação na UTI de 20,28 ± 15,62 dias. Vinte quatro (31,58 por cento) pacientes foram a óbito. O lactato aumentou no POI, retornando aos níveis basais em 24h. Os pacientes que foram a óbito apresentaram e mantiveram a partir do POI níveis de lactato mais elevados. O lactato da 3ªh foi o que melhor discriminou mortalidade, área sob a curva 0,68 (IC 0,54-0,83) P=0,035. Entretanto, considerando um ponto de corte para o lactato maior ou igual a 5,8 mmol/dl na 3ªh de PO, obteve-se apenas sensibilidade de 67 por cento e especificidade de 64 por cento para mortalidade. Existe correlação positiva entre o número de complicações e os níveis de lactato. A síndrome de baixo débito com odds ratio (OR) de 7,67 (2,38-24), sangramento aumentado com OR de 2,91 (1,07-7,94) e complicações respiratórias com OR de 1,67 (1,35-2,05) são fatores de risco de óbito quando somados. CONCLUSÃO: Após a operação de Jatene, a morbidade e a mortalidade podem ser avaliadas com auxílio da dosagem de lactato sérico, sugerindo que valores aumentados na terceira hora são sugestivos de pior prognóstico.

OBJECTIVE: To assess the morbidity and mortality after Jatene's operation using lactate as the main marker. METHODS: We performed a historical cohort with infants admitted in a pediatric intensive care unit during 1995 to 2005 who underwent this surgery. We assessed the preoperative, immediate (IPD), third hour (3h), six hour (6h) and first day (POD1) serum lactate as well as other factors such as sepsis, increased bleeding, low cardiac output syndrome, renal insufficiency, pulmonary hypertension, cardiac arrythmias, chylothorax, myocardial ischemia, seizures, presence of other complication, and also information about length of PICU stay and death. RESULTS: The mean age of 76 patients was 14.59± 19.09 days, birth weight 3.128± 0.48 kg Forty-four patients had the diagnosis of simple transposition of great arteries. The circulatory bypass time was 143.78± 28.77 minutes and aortic clamping time of 87.68± 22.3 minutes and LOS of 20.28±15.62 days. Twenty four (31.58 percent) died during hospital stay. Lactate increased in IPD, returning to baseline at 24 hours. Patients who died raised and maintained IPD lactate higher. The 3h lactate best discriminated mortality with area under the curve of 0.68 (CI 0.54 to 0.83) P = 0.035. However, considering a cutoff point for lactate greater or equal to 5.8 mmol/dl in the 3-h PO, we obtained only 67 percent sensitivity and specificity of 64 percent for mortality. There is positive correlation between number of complications and lactate. The low cardiac output syndrome with an odds ratio (OR) of 7.67 (2.38-24), increased bleeding with OR 2.91 (1.07-7.94) and respiratory complication with OR 1.67 (1.35-2.05) are risk factors when combined. CONCLUSION: After Jatene's operation, morbidity and mortality can be assessed with the serum lactate levels, suggesting increased values in the third hour is suggestive of a worse prognosis.

[22q11.2 deletion syndrome: importance of clinical evaluation and FISH analysis]. / Síndrome de deleção 22q11.2: importância da avaliação clínica e técnica de FISH.

OBJECTIVE: The 22q11.2 deletion syndrome nowadays is considered one of the most often observed genetic diseases in humans. It is clinically characterized by a rather wide phenotypic spectrum, with more than 180 clinical features physical as well as behavioral, already described. However, none is pathognomonic or obligatory which makes diagnosis even more difficult. Thus, this study intended to determine the prevalence and clinical characteristics of patients with 22q11.2 microdeletion in a selected sample of subjects with clinical suspicion of 22q11.2 deletion syndrome and normal karyotype. METHODS: A selected sample of 30 patients with clinical suspicion of 22q11.2 deletion syndrome and normal karyotype was evaluated by application of a standard clinical protocol and cytogenetic analysis with fluorescent in situ hybridization. RESULTS: 22q11.2 microdeletion was identified in 3 patients (10%), a prevalence similar to the majority of published studies, which ranged from 4 to 21%. The 22q11.2 deletion syndrome patients in this study were characterized by a variable phenotype and shared few clinical features, in agreement with the literature description. CONCLUSIONS: These findings strengthen the idea that clinical diagnosis of 22q11.2 deletion syndrome is difficult due to the large phenotypic variability. Therefore a detailed clinical evaluation associated to a sensitive test such as fluorescent in situ hybridization analysis is crucial for the identification of these patients.

Síndrome de deleção 22q11.2: importância da avaliação clínica e técnica de FISH / 22q11.2 deletion syndrome: importance of clinical evaluation and FISH analysis

OBJETIVO: A síndrome de deleção 22q11.2 é considerada hoje uma das doenças genéticas mais frequentes em humanos. Caracteriza-se clinicamente por um espectro fenotípico bastante amplo, com mais de 180 achados já descritos, tanto físicos como comportamentais. Contudo, nenhum deles é patognomônico ou mesmo obrigatório, o que acaba dificultando o diagnóstico. Assim, o objetivo do presente estudo foi determinar a prevalência e as características clínicas de pacientes com microdeleção 22q11.2 em uma amostra selecionada de indivíduos com suspeita clínica de síndrome de deleção 22q11.2 e cariótipo normal. MÉTODOS: Uma amostra selecionada de 30 pacientes com suspeita clínica da síndrome de deleção 22q11.2 e cariótipo normal foi avaliada através da aplicação de um protocolo clínico padrão e análise citogenética por meio da técnica de hibridização in situ fluorescente. RESULTADOS: A microdeleção 22q11.2 foi identificada em três pacientes (10 por cento), sendo esta prevalência similar a da maioria dos estudos descritos na literatura que oscila de 4 por cento a 21 por cento. Os pacientes com síndrome de deleção 22q11.2 do nosso trabalho se caracterizaram por um fenótipo variável, com poucos achados clínicos similares, o que foi concordante com a descrição da literatura. CONCLUSÃO: Nossos achados reforçam a ideia de que o diagnóstico clínico da síndrome de deleção 22q11.2 é difícil devido à sua grande variabilidade fenotípica. Assim, uma avaliação clínica detalhada associada a um teste sensível como a hibridização in situ fluorescente, são fundamentais para a identificação destes pacientes.

OBJECTIVE: The 22q11.2 deletion syndrome nowadays is considered one of the most often observed genetic diseases in humans. It is clinically characterized by a rather wide phenotypic spectrum, with more than 180 clinical features physical as well as behavioral, already described. However, none is pathognomonic or obligatory which makes diagnosis even more difficult. Thus, this study intended to determine the prevalence and clinical characteristics of patients with 22q11.2 microdeletion in a selected sample of subjects with clinical suspicion of 22q11.2 deletion syndrome and normal karyotype. METHODS: A selected sample of 30 patients with clinical suspicion of 22q11.2 deletion syndrome and normal karyotype was evaluated by application of a standard clinical protocol and cytogenetic analysis with fluorescent in situ hybridization. RESULTS: 22q11.2 microdeletion was identified in 3 patients (10 percent), a prevalence similar to the majority of published studies, which ranged from 4 to 21 percent. The 22q11.2 deletion syndrome patients in this study were characterized by a variable phenotype and shared few clinical features, in agreement with the literature description. CONCLUSIONS: These findings strengthen the idea that clinical diagnosis of 22q11.2 deletion syndrome is difficult due to the large phenotypic variability. Therefore a detailed clinical evaluation associated to a sensitive test such as fluorescent in situ hybridization analysis is crucial for the identification of these patients.

Treatment of pulmonary arterial hypertension.

OBJECTIVE: To perform a review of the diagnostic and therapeutic management of pulmonary hypertension in the pediatric population, with emphasis on pharmacological factors. SOURCES: Electronic search of publications on the MEDLINE/PubMed, LILACS and Cochrane Collaboration databases. The search strategy adopted gave priority to the identification of clinical trials (controlled or uncontrolled), systematic reviews and directives published during the last 10 years. SUMMARY OF THE FINDINGS: Many advances have been incorporated into our understanding of pulmonary hypertension during recent years. Issues related to differences in the pathophysiological mechanism of the disease between different age groups have altered both the treatment and prognosis of patients. The combined effect of more selective vasodilatory properties and antiproliferative action and the employment of new drugs are the basic principles of new treatment proposals. In order to be able to gauge the benefits associated with the use of these new therapies, it is of fundamental importance that all patients have their disease correctly diagnosed, the degree of functional compromise classified and their vascular reactivity capacity established, which is more difficult with pediatric patients. CONCLUSIONS: To date there is no treatment that can be considered ideal for the management of pulmonary hypertension. With reference to the possibility of employing new drugs, the majority of studies that have been published were undertaken with adult populations. Few data are available on children, and the majority of studies are uncontrolled trials or case series. Taking into account differences that have already been established between different age groups in terms of disease mechanisms and prognostic aspects, it is difficult to claim that these drugs can be incorporated into the treatment of childhood pulmonary hypertension with the same indications and results.

Treatment of pulmonary arterial hypertension

OBJETIVO: Estabelecer uma revisão acerca do manejo diagnóstico e terapêutico da hipertensão pulmonar na população pediátrica, com ênfase nos aspectos farmacológicos. FONTES DOS DADOS: Busca eletrônica de publicações nas bases de dados MEDLINE/PubMed, LILACS e Cochrane Collaboration. Estabeleceu-se uma estratégia de busca priorizando a identificação de ensaios clínicos (controlados ou não controlados), revisões sistemáticas e diretrizes publicados nos últimos 10 anos. SíNTESE DOS DADOS: Muitos avanços têm sido incorporados ao conhecimento da hipertensão pulmonar nos últimos anos. Aspectos relativos a diferenças nos mecanismos fisiopatológicos da doença entre as diferentes faixas etárias têm modificado o tratamento e o prognóstico dos pacientes. Uma ação combinada de propriedades vasodilatadoras mais seletivas e ação antiproliferativa e o emprego de novas drogas representam princípios fundamentais das novas propostas terapêuticas. Para considerar benefícios associados à utilização dessas novas terapêuticas, é fundamental que cada paciente tenha a sua doença adequadamente diagnosticada, classificado o grau de comprometimento da doença e a sua capacidade de reatividade vascular estabelecida, o que é mais difícil na população pediátrica. CONCLUSÃO: Até o momento, não existe um tratamento que possa ser considerado ideal para o manejo da hipertensão pulmonar. Considerando a possibilidade do emprego de novas drogas, a maioria dos estudos existentes foi conduzida em populações adultas. Poucos dados são disponíveis para crianças, sendo a maioria ensaios clínicos não controlados e séries de casos. Considerando diferenças já estabelecidas entre os mecanismos da doença e aspectos prognósticos entre as diferentes faixas etárias, é difícil afirmar que tais drogas possam ser incorporadas, com as mesmas indicações e os mesmos resultados, ao tratamento da hipertensão pulmonar infantil.

OBJECTIVE: To perform a review of the diagnostic and therapeutic management of pulmonary hypertension in the pediatric population, with emphasis on pharmacological factors. SOURCES: Electronic search of publications on the MEDLINE/PubMed, LILACS and Cochrane Collaboration databases. The search strategy adopted gave priority to the identification of clinical trials (controlled or uncontrolled), systematic reviews and directives published during the last 10 years. SUMMARY OF THE FINDINGS: Many advances have been incorporated into our understanding of pulmonary hypertension during recent years. Issues related to differences in the pathophysiological mechanism of the disease between different age groups have altered both the treatment and prognosis of patients. The combined effect of more selective vasodilatory properties and antiproliferative action and the employment of new drugs are the basic principles of new treatment proposals. In order to be able to gauge the benefits associated with the use of these new therapies, it is of fundamental importance that all patients have their disease correctly diagnosed, the degree of functional compromise classified and their vascular reactivity capacity established, which is more difficult with pediatric patients. CONCLUSIONS: To date there is no treatment that can be considered ideal for the management of pulmonary hypertension. With reference to the possibility of employing new drugs, the majority of studies that have been published were undertaken with adult populations. Few data are available on children, and the majority of studies are uncontrolled trials or case series. Taking into account differences that have already been established between different age groups in terms of disease mechanisms and prognostic aspects, it is difficult to claim that these drugs can be incorporated into the treatment of childhood pulmonary hypertension with the same indications and results.