Resumo Fundamento: Seguimento de coorte retrospectiva de 30 anos que se aproxima da história natural dos tumores cardíacos diagnosticados no feto uma vez que nenhum caso foi submetido à interrupção da gestação. Objetivo: Avaliar a morbidade e mortalidade perinatal e em longo prazo em fetos com diagnóstico de tumor cardíaco. Como objetivo secundário avaliar os fatores que influenciaram os resultados perinatais e pós-natais. Método: Estudo de coorte retrospectiva envolvendo 74 gestantes com diagnóstico ecocardiográfico fetal de tumor cardíaco acompanhadas em dois serviços de referência no período de maio de 1991 a novembro de 2021. Foi realizada análise descritiva dos dados por meio de frequências absolutas (n) e relativas (%), mediana e intervalos interquartis. Para avaliar a associação entre as características ecocardiográficas e as manifestações clínicas com os resultados perinatais e pós-natais, foi aplicado o teste exato de Fisher. O cálculo da sobrevida global foi realizado pelo método de Kaplan-Meier e a comparação de curvas pelo teste de log-rank. O tempo de seguimento, calculado em meses, foi definido a partir da data de alta do hospital à data do status atual (vivo/censura ou óbito). O nível de significância considerado foi de 5% (p<0,05). Resultados: o rabdomioma é o tipo mais frequente (85%) de tumor cardíaco; apresenta alta morbidade (79,3%) e mortalidade geral de 17,4%; a presença de hidropisia fetal preditiva de óbito. Conclusão: A presença de hidropisia fetal teve impacto na mortalidade, sendo fator importante para aconselhamento e estabelecimento de prognóstico. A maioria dos óbitos ocorrem antes da alta hospitalar. Fundamento 3 gestação Objetivo pósnatais. pósnatais pós natais. natais pós-natais Método 7 199 2021 n (n %, % , (%) interquartis pósnatais, natais, Fisher KaplanMeier Kaplan Meier logrank. logrank log rank. rank log-rank seguimento meses vivo/censura vivocensura vivo censura óbito . óbito) 5 p<0,05. p005 p p<0,05 0 05 (p<0,05) Resultados 85% 85 (85% 79,3% 793 79 (79,3% 17,4% 174 17 4 Conclusão prognóstico hospitalar 19 202 (% p00 p<0,0 (p<0,05 8 (85 79,3 (79,3 17,4 1 20 ( p0 p<0, (p<0,0 (8 79, (79, 17, 2 p<0 (p<0, (79 p< (p<0 (7 (p< (p

Abstract Background: This was a 30-year retrospective cohort study that approximates closely to the natural history of cardiac tumors diagnosed in the fetus, since there was no case of pregnancy interruption Objective: To assess morbidity and mortality in the perinatal period and at long term in fetuses diagnosed with cardiac tumor. Our secondary objective was to assess the evaluating factors of perinatal and postnatal results. Methods: This was a retrospective cohort study with 74 pregnant women with an echocardiographic diagnosis of fetal cardiac tumor at two referral centers between May 1991 and November 2021. A descriptive analysis was performed, and data were expressed as absolute (n) and relative (%) frequencies, median and interquartile range. Fisher's exact test was used to evaluate the association of echocardiographic characteristics and clinical manifestations with perinatal and postnatal results. Global survival was calculated using the Kaplan-Meier method and the curves were compared by the log-rank test. The time of follow-up, calculated in months, corresponded to the time elapsed from hospital discharge to current status (survived/ censoring or death). The level of significance was set at 5% (p<0.05). Results: Rhabdomyoma is the most common type of cardiac tumor (85%), with a high morbidity (79.3%) and overall mortality of 17.4%. The presence of fetal hydrops was a predictor of death. Conclusion: The presence of fetal hydrops had an impact on mortality, and hence is an important factor in counselling and determining the prognosis. Most deaths occurred before hospital discharge. Background 30year year 30 fetus Objective results Methods 7 199 2021 performed n (n % (% frequencies range Fishers Fisher s KaplanMeier Kaplan Meier logrank log rank followup, followup follow up, up follow-up months survived/ survived (survived death . death) 5 p<0.05. p005 p p<0.05 0 05 (p<0.05) Results 85%, 85 85% , (85%) 79.3% 793 79 3 (79.3% 174 17 4 17.4% Conclusion prognosis 19 202 ( p00 p<0.0 (p<0.05 8 (85% 79.3 (79.3 1 17.4 20 p0 p<0. (p<0.0 (85 79. (79. 17. 2 p<0 (p<0. (8 (79 p< (p<0 (7 (p< (p

RESUMO Objetivo: Analisar a incidência e os fatores de risco associados à colestase em recém-nascidos com gastrosquise. Métodos: Estudo de coorte retrospectivo em um único centro terciário, que analisou 181 recém-nascidos com gastrosquise entre 2009 e 2020. Foram examinados os seguintes fatores de risco associados à colestase: idade gestacional, peso ao nascer, tipo de gastrosquise, fechamento com silo ou fechamento imediato, dias de uso nutrição parenteral, tipo de emulsão lipídica, dias de jejum, dias para atingir a dieta completa, dias com cateter venoso central, presença de infecções e desfechos. Resultados: Dos 176 pacientes avaliados, 41 (23,3%) evoluíram com colestase. Baixo peso ao nascer (p=0,023), prematuridade (p<0,001), emulsão lipídica com triglicerídeos de cadeia média e triglicerídeos de cadeia longa (p=0,001) e óbito (p<0,001) foram associados à colestase. Na análise multivariada, os pacientes que receberam emulsão lipídica com óleo de peixe em vez da emulsão diária de triglicérides de cadeia média/triglicérides de cadeia longa (MCT/LCT) apresentaram menor risco de colestase. Conclusões: Nosso estudo mostra que a emulsão lipídica com óleo de peixe está associada a menor risco de colestase em neonatos com gastrosquise, porém este é um estudo retrospectivo, e um estudo prospectivo deve ser realizado para confirmar os resultados. Objetivo recémnascidos recém nascidos Métodos terciário 18 200 2020 gestacional imediato parenteral jejum completa central desfechos Resultados 17 avaliados 4 23,3% 233 23 3 (23,3% p=0,023, p0023 p p=0,023 , 0 023 (p=0,023) p<0,001, p0001 p<0,001 001 p=0,001 (p=0,001 (p<0,001 multivariada médiatriglicérides MCT/LCT MCTLCT MCT LCT (MCT/LCT Conclusões resultados 1 20 202 23,3 2 (23,3 p002 p=0,02 02 (p=0,023 p000 p<0,00 00 p=0,00 (p=0,00 (p<0,00 23, (23, p00 p=0,0 (p=0,02 p<0,0 (p=0,0 (p<0,0 (23 p0 p=0, p<0, (p=0, (p<0, (2 p=0 p<0 (p=0 (p<0 ( p= p< (p= (p< (p

ABSTRACT Objective: To describe the incidence and to analyze risk factors associated with cholestasis in neonates with gastroschisis. Methods: This is a retrospective cohort study in a tertiary single center analyzing 181 newborns with gastroschisis between 2009 and 2020. The following risk factors associated with cholestasis were analyzed: gestational age, birth weight, type of gastroschisis, silo closure or immediate closure, days of parenteral nutrition, type of lipid emulsion, days of fasting, days to reach a full diet, days with central venous catheter, presence of infections, and outcomes. Results: Among the 176 patients evaluated, 41 (23.3%) evolved with cholestasis. In the univariate analysis, low birth weight (p=0.023), prematurity (p<0.001), lipid emulsion with medium-chain triglycerides and long-chain triglycerides (p=0.001) and death (p<0.001) were associated with cholestasis. In the multivariate analysis, patients who received lipid emulsion with fish oil instead of medium chain triglycerides/long chain triglycerides (MCT/LCT) emulsion had a lower risk of cholestasis. Conclusions: Our study shows that lipid emulsion with fish oil is associated with a lower risk of cholestasis in neonates with gastroschisis. However, this is a retrospective study and a prospective study should be performed to confirm the results. Objective Methods 18 200 2020 analyzed age nutrition fasting diet catheter infections outcomes Results 17 evaluated 4 23.3% 233 23 3 (23.3% analysis p=0.023, p0023 p p=0.023 , 0 023 (p=0.023) p<0.001, p0001 p<0.001 001 mediumchain longchain long p=0.001 (p=0.001 (p<0.001 triglycerideslong MCT/LCT MCTLCT MCT LCT (MCT/LCT Conclusions However results 1 20 202 23.3 2 (23.3 p002 p=0.02 02 (p=0.023 p000 p<0.00 00 p=0.00 (p=0.00 (p<0.00 23. (23. p00 p=0.0 (p=0.02 p<0.0 (p=0.0 (p<0.0 (23 p0 p=0. p<0. (p=0. (p<0. (2 p=0 p<0 (p=0 (p<0 ( p= p< (p= (p< (p

Abstract Children are at higher risk of atelectasis due to their anatomical and physiological particularities. Several physiotherapy techniques are used to treat atelectasis, but only four studies cite methods in pediatric patients undergoing Invasive Mechanical Ventilation (IMV). The objective of this study was to evaluate the Structured Respiratory Physiotherapy Protocol (SRPP) for airway clearance and lung reexpansion for infants on IMV with atelectasis. This is a prospective study including 30 infants (mean ± standard deviation age 8.9 ± 8.0 months; weight 7.5 ± 3.0 kg; BMI 15.8 ± 1.6 kg/cm2 and IMV duration 7.7 ± 4.3 days). The sample was randomized into a Control Group (CG), which received routine physiotherapy, and an Intervention Group (IG), submitted to SRPP (postural drainage, mechanical thoracic vibration, manual hyperinflation, stretching of the accessory respiratory muscles, and functional positioning). Both groups were evaluated before and after physiotherapy for respiratory effort using the Wood Downes Score (WD) and pulmonary aeration using lung ultrasonography (Lung Ultrasound Score ‒ LUS). The outcome of the intervention was evaluated by the magnitude of the effect by the Hedges' g test [(small (0.2 < Hedges' g < 0.5), moderate (0.5 < Hedges' g < 0.8) and large (Hedges' g > 0.8) effects]. There were large within-group effects on the reduction of WD in the CG after intervention in both the CG (Hedges' g = -1.53) and IG (Hedges' g = -2.2). There was a moderate effect on LUS reduction in the CG (Hedges' g = -0.64) and a large effect on IG (Hedges' g = -1.88). This study has shown that the SRPP appears to be safe and may be effective in improving airway clearance and lung reexpansion in children on IMV with atelectasis. particularities IMV. . (IMV) (SRPP 3 mean 89 8 9 8. 80 0 months 75 7 5 7. 3. kg 158 15 15. 16 1 6 1. kgcm2 kgcm cm2 cm kg/cm 77 43 4 4. days. days days) CG, , (CG) IG, (IG) postural drainage vibration hyperinflation muscles positioning. positioning positioning) (WD Lung LUS. LUS) Hedges small 0.2 02 2 (0. 0.5, 05 0.5 0.5) 0.8 08 (Hedges effects. effects] withingroup within group 1.53 153 53 -1.53 2.2. 22 2.2 -2.2) 0.64 064 64 -0.64 1.88. 188 1.88 88 -1.88) (IMV (CG (IG 0. (0 1.5 -1.5 2. -2.2 0.6 06 -0.6 18 1.8 -1.88 ( -1. -2. -0. -1.8 -1 -2 -0 -

Abstract Objective: This study aimed to evaluate the Neuropsychomotor Development (NPMD) of newborns exposed to SARS-CoV-2 in the perinatal period using the Bayley III scale at 6 months of age. Methods: Childcare appointments were scheduled for the included newborns in the study. During the 6-month consultation, the Screening Test for Bayley III Scale and, based on it, children were classified as “low risk”, “moderate risk” or “high risk” in the domains: of cognitive, receptive language, expressive language, fine motor, and gross motor. Those classified as “moderate risk”; or “high risk” received guidance about NPMD stimuli and were instructed to maintain follow-up. Results: Only 13 (37.1 %) of the newborns were classified as low risk in receptive language and 18 (51.4 %) in gross motor skills, with the domains most affected. Prematurity was a risk for cognitive incompetence (moderate risk/high-risk classification) (coefficient: 1.89, Odds Ratio = 6.7, 95 % CI 1.3‒35, p = 0.02). Lower birth weight that 2.500g had a similar effect on cognitive incompetence (coefficient: 1.9, Odds Ratio = 6.2, 95 % CI 1.2‒32.2, p = 0.02). Exclusive breastfeeding at hospital discharge (n = 8) was protective for incompetence (high risk/moderate risk) in the language domain (coefficient -2.14, OR = 0.12, 95 % CI 0.02‒0.71, p = 0.02). Conclusions: The children included in the study must be monitored and their development monitored in order to clarify whether there is a relationship between the delay in NPMD and perinatal exposure to COVID-19, as delays were observed in these preliminary results. Objective (NPMD SARSCoV2 SARSCoV SARS CoV 2 SARS-CoV- age Methods 6month month consultation it risk, , moderate high followup. followup follow up. up follow-up Results 1 37.1 371 37 (37. 51.4 514 51 4 (51. skills affected risk/highrisk riskhighrisk risk/high classification coefficient 189 89 1.89 67 7 6.7 9 1335 3 35 1.3‒35 0.02. 002 0.02 . 0 02 0.02) 2500g g 500g 19 1.9 62 6.2 12322 32 1.2‒32.2 n 8 riskmoderate 2.14, 214 14 -2.14 012 12 0.12 002071 71 0.02‒0.71 Conclusions COVID19, COVID19 COVID 19, COVID-19 results SARS-CoV 37. (37 51. 5 (51 highrisk riskhigh 1.8 6. 133 1.3‒3 00 0.0 1. 1232 1.2‒32. 2.14 21 -2.1 01 0.1 00207 0.02‒0.7 COVID1 COVID-1 (3 (5 1.3‒ 0. 123 1.2‒32 2.1 -2. 0020 0.02‒0. COVID- ( 1.3 1.2‒3 2. -2 0.02‒0 1.2‒ - 0.02‒ 1.2

Abstract Objective Acute kidney injury (AKI) in the neonatal period is associated with worst outcomes as increased mortality and increased length of hospital stay. Very low birth weight (VLBW) newborns are at higher risk for developing several other conditions that are associated with worst outcomes. Understanding the risk factors for AKI may help to prevent this condition and improve neonatal care for this population. Methods This retrospective cohort study included 155 very low birth weight newborns admitted between 2015 and 2017. The authors compared the newborns who developed neonatal AKI with the non-AKI group and analyzed the main risk factors for developing AKI in the population. The authors also performed an analysis of the main outcomes defined as the duration of mechanical ventilation, length of stay, and death. Results From the cohort, a total of 61 (39.4%) patients had AKI. The main risk factors associated with Neonatal AKI were necrotizing enterocolitis (aOR 7.61 [1.69 - 34.37]; p = 0.008), neonatal sepsis (aOR 2.91 [1.17 - 7.24], p = 0.021), and hemodynamic instability (aOR 2.99 [1.35 - 6.64]; p = 0.007). Neonatal AKI was also associated with an increase in the duration of mechanical ventilation in 9.4 days (p = 0.026) and in an increase in mortality 4 times (p = 0.009), after adjusting for the other variables. Conclusion The present results highlight the importance of minimizing sepsis and necrotizing enterocolitis, as well as the importance of identifying hemodynamic instability, to prevent AKI and diminish the burden of morbimortality in VLBW newborns. (AKI stay (VLBW population 15 201 2017 nonAKI non death 6 39.4% 394 39 (39.4% aOR 761 7 7.6 1.69 169 1 69 [1.6 34.37 3437 34 37 34.37] 0.008, 0008 0.008 , 0 008 0.008) 291 2 91 2.9 1.17 117 17 [1.1 7.24, 724 7.24 24 7.24] 0.021, 0021 0.021 021 0.021) 299 99 1.35 135 35 [1.3 6.64 664 64 6.64] 0.007. 0007 0.007 . 007 0.007) 94 9 9. 0.026 0026 026 0.009, 0009 0.009 009 0.009) variables 20 39.4 3 (39.4 76 7. 1.6 16 [1. 34.3 343 000 0.00 00 29 2. 1.1 11 72 7.2 002 0.02 02 1.3 13 6.6 66 39. (39. 1. [1 34. 0.0 6. (39 [ 0. (3 (

Abstract Objective: Among the mechanisms proposed for the development of bronchopulmonary dysplasia is the increase in the pulmonary inflammatory process and oxidative stress. Thus, the control of this process may result in improvements in bronchopulmonary dysplasia-related outcomes. This study aims to analyze the current scientific evidence regarding the use of budesonide, a potent anti-inflammatory drug, associated with a pulmonary surfactant to prevent bronchopulmonary dysplasia. Methods: A systematic review of the literature was performed on the Embase and MEDLINE platforms, and studies that compared budesonide with pulmonary surfactant versus pulmonary surfactant for treating respiratory distress syndrome were included. The primary outcome was a reduction in bronchopulmonary dysplasia or death. Results: Four randomized clinical trials and two observational studies were included in this systematic review. Three of the randomized clinical trials found a reduction in bronchopulmonary dysplasia or death in the use of budesonide with the surfactant, all the other studies (1 clinical trial and 2 observational studies) found no statistical differences between the groups for the primary outcomes. The three main studies showed a reduction in the primary outcome; however, all studies showed great heterogeneity regarding the type of surfactant (poractant or beractant) and the method of administration. Conclusion: Robust clinical studies, in a heterogeneous population, using porcine surfactant associated with budesonide, with administration by a minimally invasive technique are necessary for there to be a recommendation based on scientific evidence for its widespread use. Objective stress Thus dysplasiarelated related outcomes antiinflammatory anti drug Methods platforms Results 1 ( however poractant beractant Conclusion population

Abstract Purpose: The authors aimed to develop a Machine-Learning (ML) algorithm that can predict positive blood culture in the neonatal intensive care unit, using complete blood count and C-reactive protein values. Methods: The study was based on patients’ electronic health records at a tertiary neonatal intensive care unit in São Paulo, Brazil. All blood cultures that had paired complete blood count and C-reactive protein measurements taken at the same time were included. To evaluate the machine learning model’s performance, the authors used accuracy, Area Under the Receiver Operating Characteristics (AUROC), recall, precision, and F1-score. Results: The dataset included 1181 blood cultures with paired complete blood count plus c-reactive protein and 1911 blood cultures with paired complete blood count only. The f1-score ranged from 0.14 to 0.43, recall ranged from 0.08 to 0.59, precision ranged from 0.29 to 1.00, and accuracy ranged from 0.688 to 0.864. Conclusion: Complete blood count parameters and C-reactive protein levels cannot be used in ML models to predict bacteremia in newborns. Purpose MachineLearning Machine Learning (ML Creactive C reactive values Methods patients Paulo Brazil model s performance AUROC, AUROC , (AUROC) F1score. F1score Fscore F1 score. score F F1-score Results 118 creactive c 191 only f1score fscore f1 f 014 0 14 0.1 043 43 0.43 008 08 0.0 059 59 0.59 029 29 0.2 100 1 00 1.00 0688 688 0.68 0864 864 0.864 Conclusion newborns (AUROC 11 19 01 0. 04 4 0.4 05 5 0.5 02 2 10 1.0 068 68 0.6 086 86 0.86 1. 06 6 8 0.8

RESUMO Delirium é uma síndrome comum em unidades de terapia intensiva, associando-se a maiores morbidade e mortalidade. No entanto, nas unidades de terapia intensiva neonatal, ele raramente é diagnosticado em razão da baixa familiaridade do neonatologista com a suspeita diagnóstica e das dificuldades na aplicabilidade dos questionários diagnósticos. Este relato de caso tem como objetivos mostrar que delirium está presente nesse grupo de pacientes e apontar as dificuldades encontradas no seu diagnóstico e tratamento. Relatamos o caso de um recém-nascido prematuro com enterocolite necrosante, submetido a três abordagens cirúrgicas. O recém-nascido apresentou intensa irritabilidade, tendo recebido altas doses de fentanil, dexmedetomidina, clonidina, cetamina, fenitoína e metadona, sem controle dos sintomas. Em seguida, foi feita a hipótese diagnóstica de delirium e iniciado tratamento com quetiapina, com reversão completa dos sintomas. Este é o primeiro caso notificado no Brasil e o primeiro que descreve a suspensão da quetiapina. associandose associando se mortalidade entanto neonatal diagnósticos recémnascido recém nascido necrosante cirúrgicas irritabilidade fentanil dexmedetomidina clonidina cetamina metadona sintomas seguida quetiapina

Abstract Delirium is a common disorder in intensive care units, being associated with greater morbidity and mortality. However, in neonatal intensive care units, delirium is rarely diagnosed, due to the low familiarity of the neonatologist with the subject and the difficulties in the applicability of diagnostic questionnaires. This case report aimed to assess the presence of this disorder in this group of patients and identify the difficulties encountered in the diagnosis and treatment. We report the case of a premature newborn with necrotizing enterocolitis during hospitalization and underwent three surgical approaches. The newborn exhibited intense irritability, having received high doses of fentanyl, dexmedetomidine, clonidine, ketamine, phenytoin, and methadone, without the control of the symptoms. A diagnosis of delirium was then made and treatment with quetiapine was started, with a complete reversal of the symptoms. This is the first case reported in Brazil and the first describing the withdrawal of the quetiapine. units mortality However diagnosed questionnaires approaches irritability fentanyl dexmedetomidine clonidine ketamine phenytoin methadone symptoms started

Abstract Objective To describe post-natal risk factors associated with death in Newborns (NB) with Congenital Diaphragmatic Hernia (CDH) in a Brazilian reference center. Methods In this retrospective cohort study, post-natal clinical factors of all NB diagnosed with CDH were reviewed in an 11-year period (2007‒2018). The primary outcome was death. Secondary outcomes included clinical features, prognostic indexes, type of mechanical ventilation, complications during hospitalization and surgical repair. Results After applying the exclusion criteria, the authors analyzed 137 charts. Overall mortality was 59% (81/137), and the highest rates were observed for low-birth-weight NB (87%), syndromic phenotype (92%), and those with major malformations (100%). Prognostic indexes such as Apgar, SNAPPE-II and 24hOI (best oxygenation index in 24 hours) were all associated with poor evolution. In a multivariate analysis, only birth weight and 24hOI were statistically significant risk factors for mortality, with a reduction in mortality risk of 17.1% (OR = 0.829, 95% IC 0.72‒0.955, p = 0.009) for each additional 100g at birth and an increase by 26.5% (OR = 1.265, 95% IC 1.113‒1.436, p = 0.0003) for each unitary increase at the 24hOI. Conclusion Prognostic indexes are an important tool for predicting outcomes and improving resource allocation. Post-natal risk factors may be more suitable for settings where antenatal diagnosis is not universal. Classical risk factors, such as prematurity, low birth weight, higher need for supportive care, and poorer prognostic indexes were associated with mortality in our CDH population. postnatal post natal (NB (CDH center study 11year year 11 2007‒2018. 20072018 2007‒2018 . 2007 2018 (2007‒2018) features ventilation repair criteria 13 charts 59 81/137, 81137 81/137 , 81 (81/137) lowbirthweight 87%, 87 87% (87%) 92%, 92 92% (92%) 100%. 100 100% (100%) Apgar SNAPPEII SNAPPE II hOI best 2 hours evolution analysis 171 17 1 17.1 OR 0829 0 829 0.829 95 0720955 72 955 0.72‒0.955 0.009 0009 009 g 265 26 5 26.5 1265 1.265 11131436 113 436 1.113‒1.436 0.0003 00003 0003 allocation Postnatal Post universal prematurity care population 2007201 2007‒201 200 201 (2007‒2018 8113 81/13 8 (81/137 (87% 9 (92% 10 (100% 17. 082 82 0.82 072095 7 0.72‒0.95 0.00 000 00 26. 126 1.26 1113143 43 1.113‒1.43 0.000 0000 200720 2007‒20 20 (2007‒201 811 81/1 (81/13 (87 (92 (100 08 0.8 07209 0.72‒0.9 0.0 12 1.2 111314 4 1.113‒1.4 20072 2007‒2 (2007‒20 81/ (81/1 (8 (9 (10 0. 0720 0.72‒0. 1. 11131 1.113‒1. 2007‒ (2007‒2 (81/ ( (1 072 0.72‒0 1113 1.113‒1 (2007‒ (81 07 0.72‒ 111 1.113‒ (2007 0.72 1.113 (200 0.7 1.11 (20 1.1 (2

ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood’s lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy. trunk abdomen phototherapy hemochromatosis syndrome lupus 24hour hour 24 collected 1823.6µg 18236µg µg 1823 6µg 100mL. 100mL mL . 100mL) 5 life Woods Wood s urinestained stained blood testing then scars fingers observed treatment recommended damage Here 2 182 18 1

RESUMO Objetivo: Descrever o status da função tireoidiana em lactentes com disfunção intestinal grave. Descrição do caso: Estudo retrospectivo, realizado em um centro de terapia intensiva neonatal de nível terciário, que incluiu recém-nascidos e lactentes com disfunção intestinal grave, internados entre 2015 e 2020. Dos prontuários foram obtidos: idade gestacional; peso de nascimento; patologia de base que levou à disfunção intestinal; tempo de internação; presença de disfunção tireoidiana; idade no início da disfunção tireoidiana; dose inicial e máxima da reposição de levotiroxina; via de administração da levotiroxina e desfecho. Sete crianças (0,76% do total de 914 internações) evoluíram com insuficiência intestinal grave: vanishing gastrosquise (42,9%), síndrome de Berdon (28,5), apple peel (14,3%) e síndrome onfalocele, ânus imperfurado, extrofia de cloaca e espinha bífida — OIES (14,3%). A média de idade gestacional foi de 33,3±1,6 semanas e de peso ao nascimento de 2113,9±370,9 gramas; a mediana de internação foi de 420 dias e a mortalidade foi de 42,9%. Desses sete casos, quatro (57,1%) apresentaram disfunção tireoidiana, avaliada por dosagens hormonais séricas, e a dose de reposição de levotiroxina variou de 25 a 100 mcg/dia, administrada por via gástrica ou retal. Comentários: Esta série de casos chama a atenção para a disfunção tireoidiana (hipotireoidismo) em crianças com insuficiência intestinal severa que receberam nutrição parenteral exclusiva por tempo prolongado, cuja etiologia é a deficiência de iodo, pois no Brasil as soluções de micronutrientes adicionadas à nutrição parenteral não contêm iodo. Objetivo grave caso retrospectivo terciário recémnascidos recém nascidos 201 2020 obtidos desfecho 0,76% 076 0 76 (0,76 91 internações 42,9%, 429 42,9% , 42 9 (42,9%) 28,5, 285 28,5 28 5 (28,5) 14,3% 143 14 3 (14,3% onfalocele imperfurado 14,3%. . 33316 33 1 6 33,3±1, 211393709 2113 370 2113,9±370, gramas 57,1% 571 57 (57,1% séricas 2 10 mcgdia mcg dia mcg/dia retal Comentários hipotireoidismo (hipotireoidismo prolongado iodo 20 202 0,76 07 7 (0,7 42,9 4 (42,9% 28, (28,5 14,3 (14,3 3331 33,3±1 21139370 211 37 2113,9±370 57,1 (57,1 0,7 (0, 42, (42,9 (28, 14, (14, 333 33,3± 2113937 21 2113,9±37 57, (57, 0, (0 (42, (28 (14 33,3 211393 2113,9±3 (57 ( (42 (2 (1 33, 21139 2113,9± (5 (4 2113,9 2113,

Abstract Objective: The aim of this study was to describe the status of thyroid function in infants with severe intestinal dysfunction. Case description: A retrospective study was conducted in a tertiary neonatal intensive care center, including newborns and infants with severe intestinal dysfunction, hospitalized between 2015 and 2020. From the medical records, the following data were collected: gestational age, birth weight, underlying pathology that led to intestinal dysfunction, hospital stay, presence of thyroid dysfunction, age from the onset of thyroid dysfunction, initial and maximum dose of levothyroxine replacement, and levothyroxine administration route and outcome. Seven children (0.76% of 914 hospitalizations) developed severe intestinal insufficiency: vanishing gastroschisis (42.9%), Berdon syndrome (28.5%), apple peel (14.3%), and OIES syndrome (14.3%) – omphalocele, exstrophy of cloaca, imperforate anus, and spina bifida. The mean gestational age was 33.3±1.6 weeks, the mean birth weight was 2,113.9±370.9 g, the median hospitalization was 420 days, and mortality was 42.9%. Of these seven cases, four (57.1%) presented thyroid dysfunction, evaluated by blood hormone dosages and the dose of levothyroxine replacement ranged from 25 to 100 μg/day, administered by gastric or rectal route. Comments: This series of cases draws attention to thyroid dysfunction (hypothyroidism) in children with severe intestinal insufficiency receiving exclusive parenteral nutrition for a prolonged period, whose etiology is iodine deficiency, because, in Brazil, micronutrient solutions added to parenteral nutrition do not contain iodine. Objective description center 201 2020 records collected stay outcome 0.76% 076 0 76 (0.76 91 hospitalizations 42.9%, 429 42.9% , 42 9 (42.9%) 28.5%, 285 28.5% 28 5 (28.5%) 14.3%, 143 14.3% 14 3 (14.3% omphalocele cloaca anus bifida 33316 33 1 6 33.3±1. weeks 211393709 2 113 370 2,113.9±370. g days 57.1% 571 57 (57.1% 10 μgday μg day μg/day Comments hypothyroidism (hypothyroidism period deficiency because Brazil 20 202 0.76 07 7 (0.7 42.9 4 (42.9% 28.5 (28.5% 14.3 (14.3 3331 33.3±1 21139370 11 37 2,113.9±370 57.1 (57.1 0.7 (0. 42. (42.9 28. (28.5 14. (14. 333 33.3± 2113937 2,113.9±37 57. (57. 0. (0 (42. (28. (14 33.3 211393 2,113.9±3 (57 ( (42 (28 (1 33. 21139 2,113.9± (5 (4 (2 2113 2,113.9 211 2,113. 21 2,113 2,11 2,1 2,

Abstract Objective: Pediatric acute liver failure (PALF) is a heterogeneous, rare, and severe condition, which outcome is survival due to liver spontaneous recovery or death. The patients who do not recover may be allocated to liver transplantation, which is the standard treatment. This study aimed to build a prognostic model to support the clinical decision to indicate liver transplantation for patients with PALF in a Brazilian center. Methods: The authors retrospectively analyzed the clinical variables of 120 patients in the liver transplantation program of the 'Children's Institute of the University of São Paulo, Brazil. The authors conducted a univariate analysis of variables associated with survival in PALF. Logistic multivariate analysis was performed to find a prognostic model for the outcome of patients with pediatric acute liver failure. Results: Risk factors were analyzed using univariate analysis. Two prognostic models were built using multiple logistic regression, which resulted in 2 models: model 1(INR/ALT) and model 2 (INR/Total bilirubin). Both models showed a high sensitivity (97.9%/96.9%), good positive predictive value (89.5%/90.4%), and accuracy (88.4%/88.5%), respectively. The receiver operating characteristic was calculated for both models, and the area under the curve was 0.87 for model 1 and 0.88 for model 2. The Hosmer-Lemeshow test showed that model 1 was good. Conclusion: The authors built a prognostic model for PALF using INR and ALT that can contribute to the clinical decision to allocate patients to liver transplantation.

Resumo Fundamento O período neonatal é marcado por muitas alterações importantes no sistema cardiovascular, principalmente na primeira semana de vida. Diferentemente da população adulta, estudos sobre dados de eletrocardiograma (ECG) no período neonatal são escassos. Este é o primeiro estudo a descrever alterações eletrocardiográficas em uma coorte de recém-nascidos com ecocardiogramas normais. Objetivos Analisar padrões eletrocardiográficos de uma população de recém-nascidos a termo, sem anomalias morfológicas ou funcionais cardíacas, e comparar os resultados com a literatura. Métodos Neste estudo observacional, ecocardiogramas e resultados de ECG de 94 neonatos divididos em três grupos etários (até 24 horas, entre 25 e 72 horas, e entre 73 e 168 horas de vida) foram avaliados e comparados com aqueles descritos por Davignon et al. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados Diferenças significativas na direção da onda T foram detectadas nas derivações V1 (p= 0,04), V2 (p= 0,02), V3 (p= 0,008) e V4 (p= 0,005). Houve diferenças entre nossos resultados e a literatura atual na maioria dos parâmetros. Conclusão Recém-nascidos a termo com menos de 24 horas de vida apresentaram significativamente mais ondas T positivas que aqueles com mais horas de vida. Encontramos muitas diferenças nos parâmetros de ECG em comparação aos descritos por Davignon et al., particularmente nas amplitudes de P, Q, R, S, duração do QRS, R/S e R+S. Esses achados indicam a necessidade de mais estudos para uma interpretação definitiva do ECG em recém-nascidos.

Abstract Background The neonatal period is marked by major changes in the cardiovascular system, especially in the first week of life. Unlike the adult population, studies on electrocardiogram (ECG) data in the neonatal period are scarce. This is the first study to describe electrocardiographic changes in a cohort of newborns with normal echocardiograms. Objectives To analyze the electrocardiographic patterns of a population of full-term NB, without any cardiac morphological or functional anomalies, and compare the results with the literature. Methods In this observational study, echocardiograms and ECG results from 94 newborns divided in three age groups (up to 24 hours, between 25 and 72 hours, and between 73 and 168 hours of life) were evaluated and compared with those reported by Davignon et al. A p-value <0.05 was considered statistically significant. Results There were significant differences in T-wave direction in leads V1 (p= 0.04), V2 (p= 0.02), V3 (p= 0.008) and V4 (p= 0.005) between the three age groups. There were differences between our findings and the current literature in most of the parameters. Conclusion Term newborns within 24 hours of life showed significantly more positive T waves than older ones. Many differences from the Davignon’s ECG parameters were found, particularly in the P, Q, R, S amplitudes, QRS duration, R/S and R+S. These findings indicate that more studies are needed for a definitive interpretation of the ECG in newborns.

RESUMO O objetivo do estudo foi verificar a aplicabilidade do Test of Infant Motor Performance (TIMP) em recém-nascidos (RN) cirúrgicos como ferramenta para avaliação do desempenho motor; e avaliar o benefício da fisioterapia. RN cirúrgicos, divididos em grupo sem fisioterapia motora (n=38) e com fisioterapia motora (n=38), foram avaliados pelo TIMP e reavaliados duas semanas depois. O grupo com fisioterapia realizou exercícios para estimulação sensório-motora uma vez ao dia, enquanto o grupo sem fisioterapia recebeu os cuidados habituais da Unidade de Terapia Intensiva Neonatal (UTIN). O diagnóstico cirúrgico predominante nos dois grupos foi de gastrosquise, seguido de hérnia diafragmática congênita. Na avaliação, não houve diferença no desempenho motor entre os grupos, classificados como “abaixo da média” para a idade, com escore-z=−1,28 (p=0,992). Na reavaliação, o grupo com fisioterapia apresentou melhor pontuação (p<0,001) e maior diferença de escore (p<0,001), com uma porcentagem maior de RN classificados como “dentro da média” em comparação ao grupo sem fisioterapia (44% e 2,6%, respectivamente); além de maior porcentagem de ganho de peso (p=0,038). Na reavaliação, verificou-se piora do desempenho motor em 10,5% do grupo sem fisioterapia (p<0,001), enquanto a melhora do desempenho motor foi observada em 81,6% do grupo com fisioterapia e em apenas 13,2% do grupo sem fisioterapia (p<0,001). Concluiu-se que o TIMP foi aplicado de forma segura e eficaz e necessita ser realizado por profissionais experientes na abordagem dessa população. A fisioterapia motora melhorou significativamente o desempenho motor, e, se iniciada precocemente, pode ser capaz de adequar o desenvolvimento motor desses RN, antes mesmo da alta hospitalar.

ABSTRACT This study aimed to verify the applicability of the Test of Infant Motor Performance (TIMP) in surgical neonates as a tool for evaluating motor performance and to evaluate the benefit of physical therapy. Surgical neonates, divided into group without physical therapy (n=38) and group with motor physical therapy (n=38), were evaluated by TIMP and reasssment two weeks later. The physical therapy group performed standardized exercises for motor sensory stimulation once a day, whereas the other group received the usual care from the Neonatal Intensive Care Unit. The predominant surgical diagnosis in both groups was gastroschisis, followed by congenital diaphragmatic hernia. In the initial evaluation, motor performance was equal between the groups, classified as “below average” for age, with z-score=−1.28 (p=0.992). In the reassessment, the physical therapy group presented better scores (p<0.001) and a higher difference in z-score (p<0.001), higher percentage of neonates classified as “within average” (44% in the physical therapy group and 2.6% in the no physical therapy group), as well as a higher weight gain percentage (p=0.038). We found a worsening of motor performance in 10.5% of the no physical therapy group (p<0.001) in the reassment. Motor performance improved in 81.6% of the physical therapy group and in only 13.2% of the no physical therapy group individuals (p<0.001). Conclusion: TIMP was safely and effectively applied to newborns; notably, the test must be performed by professionals experienced in care of neonates. Motor intervention was beneficial, significantly improving motor performance, and if applied early, it may adequate the motor development of these neonates, even before hospital discharge.

RESUMEN El objetivo de este estudio fue verificar la aplicabilidad del Test of Infant Motor Performance (TIMP) en recién nacidos quirúrgicos (RN) como una herramienta de evaluación del desempeño motor, y analizar el beneficio de la fisioterapia. Los RN quirúrgicos, divididos en los grupos sin fisioterapia motora (n=38) y con fisioterapia (n=38), fueron evaluados por el TIMP y reevaluados dos semanas después. El grupo con fisioterapia realizó ejercicios de estimulación sensoriomotora una vez al día, mientras que el grupo sin fisioterapia recibió la atención habitual de la Unidad de Cuidados Intensivos Neonatal (UCIN). El diagnóstico quirúrgico predominante en ambos grupos fue el de la gastrosquisis, seguido de hernia diafragmática congénita. En la evaluación, no hubo diferencia en el desempeño motor entre los grupos, clasificados como “por debajo del promedio” para la edad, con puntaje -z=−1,28 (p=0,992). En la reevaluación, el grupo con fisioterapia tuvo mejor puntuación (p<0,001) y mayor diferencia de puntaje (p<0,001), con mayor porcentaje de RN clasificados como “dentro de la media” en comparación con el grupo sin fisioterapia (44% y 2,6%, respectivamente); además de un mayor porcentaje de ganancia de peso (p=0,038). En la reevaluación hubo un empeoramiento del desempeño motor en el 10,5% del grupo sin fisioterapia (p<0,001), mientras que se observó una mejora en el desempeño motor en el 81,6% del grupo con fisioterapia y solo en el 13,2% del grupo sin fisioterapia (p<0,001). Se concluyó que el TIMP se aplicó con seguridad y eficacia y que este debe ser realizado por profesionales con experiencia con esta población. La fisioterapia motora mejoró significativamente el rendimiento motor y, si se inicia precozmente, puede ser capaz de adaptar el desarrollo motor de estos RN, incluso antes del alta hospitalaria.