Is neurodevelopment impaired in Brazilian children with intestinal failure on prolonged parenteral nutrition? A single center study.

PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.

Outcomes of the First 54 Pediatric Patients on Long-Term Home Parenteral Nutrition from a Single Brazilian Center.

OBJECTIVES: Data on multidisciplinary programs dedicated to home parenteral nutrition (HPN) in Latin America are limited. This study describes the results of the first multidisciplinary pediatric intestinal rehabilitation program for HPN at a public tertiary hospital in Brazil. METHODS: We retrospectively reviewed patients aged 0-18 years with intestinal failure (IF) who required parenteral nutrition (PN) for >60 days between January/2014 and December/2020. RESULTS: Fifty-four patients were discharged on HPN (15 achieved enteral autonomy, 34 continued on HPN at the end of the study, 1 underwent intestinal transplantation, and 4 died). The median (IQR) age at the study endpoint of patients who achieved enteral autonomy was 14.1 (9.7-19) versus 34.7 (20.4-53.9) months in those who did not achieve enteral autonomy. Overall prevalence of catheter-related thrombosis was 66.7% and catheter-related bloodstream infection rate was 0.39/1000 catheter-days. Intestinal failure-associated liver disease (IFALD) was present in 24% of all patients; none of the patients who achieved enteral autonomy had IFALD. All patients showed significant improvement in anthropometric parameters during the HPN period. The sociodemographic characteristics of the patients' family members were mothers less than 20 years old (7.5%), schooling time more than 10 years (55.5%), and household income between 1 and 3 times the minimum wage (64.8%). The 5-year survival rate for HPN is 90%, and 27.7% of patients achieve enteral autonomy. CONCLUSION: The treatment of pediatric patients with IF followed by a multidisciplinary pediatric intestinal rehabilitation program with HPN is feasible and safe in the Brazilian public health system.

Giant omphalocele: A novel approach for primary repair in the neonatal period using botulinum toxin.

INTRODUCTION: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience. METHODS: patients were followed from August 2020 to July 2022. BTA was applied to the lateral abdominal wall in the first days of life followed by surgical repair of the abdominal defect. RESULTS: while awaiting surgery, patients had minimal manipulation, without requiring mechanical ventilation, were on full enteral feeding, and in contact with their parents. The midline was approximated without tension and without the need for additional techniques or the use of a prosthesis. Patients were discharged with repaired defects. CONCLUSION: this approach represents a middle ground between staged and the nonoperative delayed repairs. It does not require aggressive interventions early in life, allowing maintenance of mother-child bonding and discharge of the patient with a repaired defect without the need for additional techniques or the use of a prosthesis. We believe that this technique should be considered as a new possible asset when managing this complex condition.

There is an association between disease location and gestational age at birth in newborns submitted to surgery due to necrotizing enterocolitis.

OBJECTIVES: To evaluate if there are differences regarding disease location and mortality of necrotizing enterocolitis, according to the gestational age at birth, in newborns submitted to surgery due to enterocolite. METHODS: A historical cohort study of 198 newborns submitted to surgery due to necrotizing enterecolitis in a tertiary hospital, from November 1991 to December 2012. The newborns were divided into different categories according to gestational age (<30 weeks, 30-33 weeks and 6 days, 34-36 weeks and 6 days, and ≥37 weeks), and were followed for 60 days after surgery. The inclusion criterion was the presence of histological findings of necrotizing enterocolitis in the pathology. Patients with single intestinal perforation were excluded. RESULTS: The jejunum was the most commonly affected site in extremely premature infants (p=0.01), whereas the ileum was the most commonly affected site in premature infants (p=0.002), and the colon in infants born at term (p<0.001). With the increasing gestational age, it was observed that intestinal involvement decreased for the ileum and the jejunum (decreasing from 45% to 0% and from 5% to 0%, respectively), with a progressive increase in colon involvement (0% to 84%). Total mortality rate was 45.5%, and no statistical difference was observed in the mortality at different gestational ages (p=0.287). CONCLUSIONS: In newborns submitted to surgery due to necrotizing enterocolitis, the disease in extremely preterm infants was more common in the jejunum, whereas in preterm infants, the most affected site was the ileum, and in newborns born close to term, it was the colon. No difference in mortality was observed according to the gestational age at birth.

Giant omphalocele: A novel approach for primary repair in the neonatal period using botulinum toxin / Onfalocele gigante: Uma nova abordagem para reparo primário no período neonatal utilizando toxina botulínica

ABSTRACT Introduction: Giant omphalocele (GO) is a complex condition for which many surgical treatments have been developed; however, no consensus on its treatment has been reached. The benefits and efficacy of botulinum toxin A (BTA) in the repair of large abdominal wall defects in adults has been proven, and its reported use in children has recently grown. The goal of this study is to describe a novel technique for primary repair of GO using BTA during the neonatal period and report our initial experience. Methods: patients were followed from August 2020 to July 2022. BTA was applied to the lateral abdominal wall in the first days of life followed by surgical repair of the abdominal defect. Results: while awaiting surgery, patients had minimal manipulation, without requiring mechanical ventilation, were on full enteral feeding, and in contact with their parents. The midline was approximated without tension and without the need for additional techniques or the use of a prosthesis. Patients were discharged with repaired defects. Conclusion: this approach represents a middle ground between staged and the nonoperative delayed repairs. It does not require aggressive interventions early in life, allowing maintenance of mother-child bonding and discharge of the patient with a repaired defect without the need for additional techniques or the use of a prosthesis. We believe that this technique should be considered as a new possible asset when managing this complex condition.

RESUMO Introdução: onfalocele gigante (OG) é uma condição complexa para a qual muitas alternativas terapêuticas foram desenvolvidas; no entanto, não há consenso sobre qual o melhor tratamento. Os benefícios e eficácia da toxina botulínica A (TBA) no reparo de grandes defeitos da parede abdominal em adultos foram comprovados, e o relato de uso em crianças cresceu recentemente. O objetivo deste estudo é descrever uma nova técnica para reparo primário de OG utilizando TBA durante o período neonatal e relatar nossa experiência inicial. Métodos: os pacientes foram acompanhados de agosto de 2020 a julho de 2022. A TBA foi aplicada na parede abdominal lateral nos primeiros dias de vida, seguida de correção cirúrgica do defeito abdominal. Resultados: enquanto aguardavam a cirurgia, os pacientes tiveram mínima manipulação, sem ser exigida ventilação mecânica, permaneceram em alimentação enteral plena e mantiveram contato com os pais. A linha média foi aproximada sem tensão e sem necessidade de técnicas adicionais ou uso de prótese. Os pacientes receberam alta com o defeito reparado. Conclusão: essa abordagem representa um meio-termo entre o reparo estagiado e o tratamento não-operatório. Não requer intervenções agressivas no início da vida, permitindo a manutenção do vínculo materno-infantil e a alta do paciente com defeito reparado sem a necessidade de uso de técnicas adicionais ou prótese. Acreditamos que esta técnica deva ser considerada como um novo possível recurso no manejo desta complexa condição.

[Prevalence of systemic inflammatory syndromes at a tertiary pediatric intensive care unit]. / Prevalência das síndromes inflamatórias sistêmicas em uma unidade de tratamento intensivo pediátrica terciária.

OBJECTIVE: To assess the prevalence of systemic inflammatory syndromes on admission to a tertiary-care university pediatric intensive care unit (ICU), and relate this to length of hospital stay, risk of death and mortality rate. METHODS: Cross-sectional, prospective, observational study, including all patients admitted to the Hospital de Clinicas de Porto Alegre (HCPA) ICU between August 1st 1999 and July 31st 2000. Patient demographic variables were considered together with the risk of mortality on admission, co-morbidities, length of hospital stay and ICU outcome, in addition to variables that characterize the systemic inflammatory syndromes (systemic inflammatory response syndrome, sepsis, severe sepsis and septic shock). RESULTS: We studied 447 admissions of 388 patients; 54% were male, with a median age of 20 months. The prevalence of systemic inflammatory response syndrome (SIRS) was 68%: 2/3 infectious (sepsis, severe sepsis or septic shock) and 1/3 non-infectious. Risk of mortality scores for patients with infectious SIRS were higher than for those with non-infectious SIRS (6.75% [P25 = 2.25 - P75 = 21.3] vs. 2.35% [P25 = 1.1 - P75 = 6.7]; p = 0) and increased according to SIRS severity (2.9; 10.85, 43.9%; p < 0.05). The observed mortality was 12% for patients with SIRS and 5.8% for those without SIRS (p = 0.057); the observed mortality for infectious SIRS was 14.9% and for non-infectious 6.3% (p = 0.041). The period spent in ICU for infectious SIRS was longer than for non-infectious cases: 3 days (P25 = 2 - P75 = 7) vs. 2 days (P25 = 1.5 - P75 = 4); p = 0.006. CONCLUSIONS: The prevalence rate of patients with systemic inflammatory response syndrome upon admission to HCPA pediatric intensive care unit was elevated, with a predominance of infectious syndromes, responsible for longer stays, increased risk of mortality and increased mortality of patients during the period evaluated.

Voluntary HIV counseling and testing during prenatal care in Brazil.

OBJECTIVE: Voluntary HIV counseling and testing are provided to all Brazilian pregnant women with the purpose of reducing mother-to-child HIV transmission. The purpose of the study was to assess characteristics of HIV testing and identify factors associated with HIV counseling and testing. METHODS: A cross-sectional study was carried out comprising 1,658 mothers living in Porto Alegre, Brazil. Biological, reproductive and social variables were obtained from mothers by means of a standardized questionnaire. Being counseling about HIV testing was the dependent variable. Confidence intervals, chi-square test and hierarchical logistic model were used to determine the association between counseling and maternal variables. RESULTS: Of 1,658 mothers interviewed, 1,603 or 96.7% (95% CI: 95.7-97.5) underwent HIV testing, and 51 or 3.1% (95% CI: 2.3-4.0) were not tested. Four (0.2%) refused to undergo testing after counseling. Of 51 women not tested in this study, 30 had undergone the testing previously. Of 1,603 women tested, 630 or 39.3% (95% CI: 36.9-41.7) received counseling, 947 or 59.2% (95% CI: 56.6-61.5) did not, and 26 (1.6%) did not inform. Low income, lack of prenatal care, late beginning of prenatal care, use of rapid testing, and receiving prenatal in the public sector were variables independently associated with a lower probability of getting counseling about HIV testing. CONCLUSIONS: The study findings confirmed the high rate of prenatal HIV testing in Porto Alegre. However, women coming from less privileged social groups were less likely to receive information and benefit from counseling.

There is an association between disease location and gestational age at birth in newborns submitted to surgery due to necrotizing enterocolitis / Existe associação entre a localização da doença e a idade gestacional ao nascimento de recém-nascidos operados por enterocolite necrosante

Abstract Objectives To evaluate if there are differences regarding disease location and mortality of necrotizing enterocolitis, according to the gestational age at birth, in newborns submitted to surgery due to enterocolite. Methods A historical cohort study of 198 newborns submitted to surgery due to necrotizing enterecolitis in a tertiary hospital, from November 1991 to December 2012. The newborns were divided into different categories according to gestational age (<30 weeks, 30-33 weeks and 6 days, 34-36 weeks and 6 days, and ≥37 weeks), and were followed for 60 days after surgery. The inclusion criterion was the presence of histological findings of necrotizing enterocolitis in the pathology. Patients with single intestinal perforation were excluded. Results The jejunum was the most commonly affected site in extremely premature infants (p = 0.01), whereas the ileum was the most commonly affected site in premature infants (p = 0.002), and the colon in infants born at term (p < 0.001). With the increasing gestational age, it was observed that intestinal involvement decreased for the ileum and the jejunum (decreasing from 45% to 0% and from 5% to 0%, respectively), with a progressive increase in colon involvement (0% to 84%). Total mortality rate was 45.5%, and no statistical difference was observed in the mortality at different gestational ages (p = 0.287). Conclusions In newborns submitted to surgery due to necrotizing enterocolitis, the disease in extremely preterm infants was more common in the jejunum, whereas in preterm infants, the most affected site was the ileum, and in newborns born close to term, it was the colon. No difference in mortality was observed according to the gestational age at birth.

Resumo Objetivos Avaliar se há diferença de localização e de mortalidade da enterocolite necrosante de acordo com a idade gestacional ao nascimento, em neonatos operados por enterocolite. Métodos Coorte histórica de 198 neonatos operados por enterocolite necrosante em hospital terciário, de novembro de 1991 a dezembro de 2012. Os recém-nascidos operados foram divididos em diferentes categorias de acordo com a idade gestacional (< 30 semanas, 30 a 33 semanas e seis dias, 34 a 36 semanas e seis dias e ≥ 37 semanas) e foram seguidos por 60 dias depois da cirurgia. O critério de inclusão foi a presença de achados histológicos de enterocolite necrosante no anatomopatológico e o de exclusão foi a presença de perfuração intestinal única. Resultados O jejuno foi mais acometido pela ECN nos prematuros extremos (p = 0,01); o íleo mais afetado nos recém-nascidos prematuros (p = 0,002) e o cólon nos recém-nascidos a termo ou próximos ao termo (p < 0,001). Com o aumento da idade gestacional, observam-se redução do acometimento do jejuno e do íleo (regrediu de 45% para 0% e de 5% para 0%, respectivamente) e aumento progressivo do acometimento do cólon (0% para 84%). A mortalidade total das crianças operadas por ECN foi de 45,5%; não existiu diferença estatística na mortalidade nas diferentes idades gestacionais (p = 0,287). Conclusões Em recém-nascidos operados por enterocolite necrosante, a doença no jejuno foi mais comum no prematuro extremo, no íleo no prematuro, e a doença no cólon nos recém-nascidos próximos ao termo. Não foi observada diferença de mortalidade de acordo com a idade gestacional ao nascimento.

Prevalência das síndromes inflamatórias sistêmicas em uma unidade de tratamento intensivo pediátrica terciária / Prevalence of systemic inflammatory syndromes at a tertiary pediatric intensive care unit

OBJETIVO: Avaliar a prevalência das síndromes inflamatórias sistêmicas na admissão em uma unidade de terapia intensiva (UTI) pediátrica universitária terciária e os respectivos tempo de permanência, probabilidade de morte e taxa de mortalidade. MÉTODOS: Estudo transversal prospectivo observacional, com todos os pacientes admitidos na UTI do Hospital de Clínicas de Porto Alegre (HCPA) entre 1º de agosto de 1999 e 31 de julho de 2000. Foram estudadas as variáveis demográficas dos pacientes, o risco de morte na admissão, co-morbidades, tempo de permanência e desfecho na UTI, além das variáveis que caracterizam as síndromes inflamatórias sistêmicas (síndrome da resposta inflamatória sistêmica, sepse, sepse grave e choque séptico). RESULTADOS: Foram estudadas 447 admissões de 388 pacientes; 54 por cento deles eram do sexo masculino, com mediana de idade de 20 meses. A prevalência de síndrome da resposta inflamatória sistêmica (SRIS) foi 68 por cento: 2/3 infecciosas (sepse, sepse grave ou choque séptico) e 1/3 não-infecciosas. O risco de morte dos pacientes com SRIS infecciosa foi maior do que naqueles com SRIS não-infecciosa (6,75 por cento [P25 = 2,25 e P75 = 21,3] versus 2,35 por cento [P25 = 1,1 e P75 = 6,7]; p = 0) e crescente de acordo com a sua gravidade (2,9; 10,85 e 43,9 por cento; p < 0,05). A mortalidade observada foi 12 por cento nos pacientes com SRIS e 5,8 por cento sem SRIS (p = 0,057); na SRIS infecciosa, a mortalidade observada foi 14,9 por cento e, na não-infecciosa, foi de 6,3 por cento (p = 0,041). A permanência na UTI na SRIS infecciosa foi significativamente superior à não-infecciosa: 3,0 dias (P25 = 2 e P75 = 7) versus 2 dias (P25 = 1,5 e P75 = 4), com p = 0,006. CONCLUSÕES: A taxa de prevalência de pacientes com síndrome da resposta inflamatória sistêmica na admissão da unidade de terapia intensiva pediátrica do HCPA foi elevada, com predomínio das síndromes infecciosas, associadas à maior permanência, risco de morte e mortalidade dos pacientes no período avaliado.

Barreiras, para a notificação pelo pediatra, de maus-tratos infantis / Barriers for reporter of child abuse by pediatricians

OBJETIVOS: determinar os fatores que interferem na notificação de maus-tratos infantis, pelos pediatras, aos serviços de proteção à criança. MÉTODOS: estudo observacional transversal. Uma amostra aleatória de pediatras de Porto Alegre foi selecionada entre 990 inscritos na sociedade de pediatria local. Variáveis sócio-demográficas, formação profissional, conhecimento diante de casos de maus-tratos infantis foram obtidos através de questionário anônimo. Análises descritiva e multivariada foram utilizadas para determinar os fatores associados a não notificação. RESULTADOS: foram incluídos 97 pediatras dos quais 92 concordaram em participar do estudo. Oitenta identificaram casos de maus-tratos, e destes 63 notificaram ao menos um caso. A maioria revelou medo de envolver-se legalmente, apresentou nível suficiente de conhecimento e baixo grau de confiança nos órgãos de proteção à criança. Conhecimento insuficiente (OR = 3,94), trabalhar exclusivamente no setor privado (OR = 6,33) foram fatores associados a não notificação. Após ajustes, o conhecimento insuficiente foi significativamente associado com o resultado OR = 5,06 (IC95 por cento = 1,45 - 17,59). CONCLUSÕES: verificou-se uma alta taxa de identificação e notificação, pelo pediatra, de maus-tratos infantis. Programas de educação continuada, melhoria dos serviços de proteção, suporte técnico profissional para o setor privado podem aumentar a taxa de identificação e notificação de maus-tratos.

Voluntary HIV counseling and testing during prenatal care in Brazil

OBJECTIVE: Voluntary HIV counseling and testing are provided to all Brazilian pregnant women with the purpose of reducing mother-to-child HIV transmission. The purpose of the study was to assess characteristics of HIV testing and identify factors associated with HIV counseling and testing. METHODS: A cross-sectional study was carried out comprising 1,658 mothers living in Porto Alegre, Brazil. Biological, reproductive and social variables were obtained from mothers by means of a standardized questionnaire. Being counseling about HIV testing was the dependent variable. Confidence intervals, chi-square test and hierarchical logistic model were used to determine the association between counseling and maternal variables. RESULTS: Of 1,658 mothers interviewed, 1,603 or 96.7 percent (95 percent CI: 95.7-97.5) underwent HIV testing, and 51 or 3.1 percent (95 percent CI: 2.3-4.0) were not tested. Four (0.2 percent) refused to undergo testing after counseling. Of 51 women not tested in this study, 30 had undergone the testing previously. Of 1,603 women tested, 630 or 39.3 percent (95 percent CI: 36.9-41.7) received counseling, 947 or 59.2 percent (95 percent CI: 56.6-61.5) did not, and 26 (1.6 percent) did not inform. Low income, lack of prenatal care, late beginning of prenatal care, use of rapid testing, and receiving prenatal in the public sector were variables independently associated with a lower probability of getting counseling about HIV testing. CONCLUSIONS: The study findings confirmed the high rate of prenatal HIV testing in Porto Alegre. However, women coming from less privileged social groups were less likely to receive information and benefit from counseling.