Bullous lesions following phototherapy in a newborn.

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.

Bullous lesions following phototherapy in a newborn

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.

Clinical characteristics and evolution of 71 neonates born to mothers with COVID-19 at a tertiary center in Brazil

Abstract Introduction Limited data are available on pregnant women with COVID-19 and their neonates. Objective This study aimed to describe clinical characteristics and evolution from birth to discharge of a retrospective cohort of 71 neonates, with one set of twins, born to women with COVID-19 diagnosed at the end of pregnancy. The authors included all newborns admitted into a neonatal unit of a tertiary hospital in Brazil, between March 2020 and March 2021, whose unvaccinated mothers had COVID-19 symptoms and RT-PCR (Real-Time Polymerase Chain Reaction) for SARS-CoV-2 positive within fourteen days prior to delivery. Newborns to mothers with COVID-19 symptoms and negative tests for SARS-CoV-2 were excluded. Results The main route of birth delivery was cesarean, corresponding to 60 pregnant women (84.5%). The foremost indications for cesarean were pregnant with critical disease (24.6%) and acute fetal distress (20.3%). The mean birth weight was 2452 g (865‒3870 g) and the mean gestational age was 345/7weeks (25‒40 weeks). There were 45 premature newborns (63.3%), of which 21 newborns (29.5%) were less than 32 weeks of gestational age. RT-PCR for SARS-CoV-2 on oropharyngeal swabs was positive in 2 newborns (2.8%) and negative in the other 69 newborns (97.2%). Most newborns (51.4%) needed respiratory support. Therapeutic interventions during hospitalization were inotropic drugs (9.9%), antibiotics (22.8%), parenteral nutrition (26.8%), and phototherapy (46.5%). Conclusion Maternal COVID-19 diagnosticated close to delivery has an impact on the first days of neonatal life.

Vertical transmission of SARS-CoV2 during pregnancy: A high-risk cohort.

OBJECTIVE: Identify the potential for and risk factors of SARS-CoV-2 vertical transmission. METHODS: Symptomatic pregnant women with COVID-19 diagnosis in whom PCR for SARS-CoV-2 was performed at delivery using maternal serum and at least one of the biological samples: cord blood (CB), amniotic fluid (AF), colostrum and/or oropharyngeal swab (OPS) of the neonate. The association of parameters with maternal, AF and/or CB positivity and the influence of SARS-CoV-2 positivity in AF and/or CB on neonatal outcomes were investigated. RESULTS: Overall 73.4% (80/109) were admitted in hospital due to COVID-19, 22.9% needed intensive care and there were four maternal deaths. Positive RT-PCR for SARS-CoV-2 was observed in 14.7% of maternal blood, 13.9% of AF, 6.7% of CB, 2.1% of colostrum and 3.7% of OPS samples. The interval between COVID-19 symptoms and delivery was inversely associated with SARS-CoV-2 positivity in the maternal blood (p = 0.002) and in the AF and/or CB (p = 0.049). Maternal viremia was associated with positivity for SARS-CoV-2 in AF and/or CB (p = 0.001). SARS-CoV-2 positivity in the compartments was not associated with neonatal outcomes. CONCLUSION: Vertical transmission is possible in pregnant women with COVID-19 and a shorter interval between maternal symptoms and delivery is an influencing factor.

Gastrointestinal manifestations and nutritional therapy during COVID-19 pandemic: a practical guide for pediatricians.

Coronavirus disease 2019 (COVID-19) is a disease caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which has spread globally in pandemic proportions. Accumulative evidence suggests SARS-CoV-2 can be transmitted through the digestive system, the so-called fecal-oral route of transmission, and may induce several gastrointestinal manifestations. MEDLINE® and Embase databases were extensively searched for major clinical manifestations of gastrointestinal involvement in children and adolescents with COVID-19 reported in medical literature, and for nutritional therapy-related data. Findings and recommendations were pragmatically described to facilitate overall pediatric approach. A total of 196 studies addressing gastrointestinal or nutritional aspects associated with the global COVID-19 pandemic were found. Of these, only 17 focused specifically on pediatric patients with regard to aforementioned gastrointestinal or nutritional aspects. Most articles were descriptive and six addressed guidelines, established protocols, or expert opinions. Children and adolescents with gastrointestinal symptoms, such as nausea, vomiting and diarrhea, should be seriously suspected of COVID-19. Gastrointestinal signs and symptoms may occur in 3% to 79% of children, adolescents and adults with COVID-19, and are more common in severe cases. These include diarrhea (2% to 50%), anorexia (40% to 50%), vomiting (4% to 67%), nausea (1% to 30%), abdominal pain (2% to 6%) and gastrointestinal bleeding (4% to 14%). Patients with inflammatory bowel disease or chronic liver disease are not at greater risk of infection by SARS-CoV-2 relative to the general population. Nutritional support plays an important role in treatment of pediatric patients, particularly those with severe or critical forms of the disease. The digestive system may be a potential route of COVID-19 transmission. Further research is needed to determine whether the fecal-oral route may be involved in viral spread. Nutritional therapy is vital to prevent malnutrition and sarcopenia in severe cases.

Beyond ventilatory support: challenges in general practice and in the treatment of critically Ill children and adolescents with SARS-CoV-2 infection.

Severe acute respiratory syndrome coronavirus 2 (Sars-CoV-2 infection) is a new challenge for all countries, and children are predisposed to acquire this disease. Some studies have demonstrated more severe diseases in adults, but critically ill pediatric patients have been described in all ages. Pulmonary involvement is the major feature, and ventilatory support is common in critical cases. Nevertheless, other very important therapeutic approaches must be considered. In this article, we reviewed extensively all recent medical literature to point out the main clinical attitudes to support these pediatric patients during their period in respiratory support. Radiologic findings, fluid therapy, hemodynamic support, use of inotropic/vasopressors, nutritional therapy, antiviral therapy, corticosteroids, antithrombotic therapy, and immunoglobulins are analyzed to guide all professionals during hospitalization. We emphasize the importance of a multi-professional approach for adequate recovery.

Beyond ventilatory support: challenges in general practice and in the treatment of critically Ill children and adolescents with SARS-CoV-2 infection

SUMMARY Severe acute respiratory syndrome coronavirus 2 (Sars-CoV-2 infection) is a new challenge for all countries, and children are predisposed to acquire this disease. Some studies have demonstrated more severe diseases in adults, but critically ill pediatric patients have been described in all ages. Pulmonary involvement is the major feature, and ventilatory support is common in critical cases. Nevertheless, other very important therapeutic approaches must be considered. In this article, we reviewed extensively all recent medical literature to point out the main clinical attitudes to support these pediatric patients during their period in respiratory support. Radiologic findings, fluid therapy, hemodynamic support, use of inotropic/vasopressors, nutritional therapy, antiviral therapy, corticosteroids, antithrombotic therapy, and immunoglobulins are analyzed to guide all professionals during hospitalization. We emphasize the importance of a multi-professional approach for adequate recovery.

RESUMO A síndrome respiratória aguda grave (SRAG) pelo novo coronavirus (SARS-CoV-2) é um novo desafio para todos os países e crianças estão predispostas a adquirir a doença. Alguns estudos demonstraram quadros mais graves em adultos, mas crianças criticamente doentes foram descritas em todas as idades. O envolvimento pulmonar é a principal característica e a necessidade de suporte ventilatório é comum nos casos mais graves. Entretanto, outras abordagens terapêuticas importantes devem ser consideradas. Nesse artigo revisamos extensamente a literature médica até o momento a fim de citar os principais recursos terapêuticos para o manejo dos pacientes pediátricos durante o período de suporte ventilatório. Achados radiológicos, terapia fluídica, terapia antiviral, o uso de corticosteroides, terapia antitrombótica e o uso de imunoglobulinas foram analisados a fim de guiar os profissionais durante o período de hospitalização desses pacientes. Nós reforçamos a importância de uma abordagem multiprofissional para recuperação adequada.

Caracterização da evolução clínica dos recém-nascidos com gastrosquise em uma unidade de terapia intensiva neonatal de referência da américa latina / Characteristics of the clinical development of a newborn with gastroschisis in an intensive care unit in latin america

INTRODUCTION: Congenital malformations are major diseases observed at birth. They are the second most common cause of death in the neonatal population, the first one being prematurity. OBJECTIVE: To characterise the clinical outcome of newborns with gastroschisis (GS) in a neonatal intensive care unit. METHODS: A retrospective observational clinical study in 50 infants with GS using the association of intestinal abnormalities, impossibility of primary closure of the abdominal defect and reoperation necessity as classification criteria for the disease. The significance level was p < 0.05. RESULTS: The hospitalisation to primary surgery occurred with a median age of 2 hours. Fourteen percent of children were subjected to a primary silo interposition and 24% had associated intestinal malformation. Nineteen newborns (NB) required more than one surgery. The median length of stay was 33 days, higher in patients with complex GS (56 days). All NB recovered from urine output 48 hours after surgery and 40% had hyponatraemia and oligoanuria in this period. There was no difference between the natraemia and fasting time (p = 0.79). Weight gain was similar in both groups with total parenteral nutrition and became significantly higher in patients with simple GS after enteral feeding (p = 0.0046). These NB evolved 2.4 times less cholestasis. Late-onset sepsis occurred in 58% of patients and was related to the infection of the central venous catheter in 37.9% of cases. Mortality was higher in infants infected with complex GS and the overall mortality rate was 14%. CONCLUSION: Clinical characterisation of newborns with gastroschisis depends on the complexity and the knowledge and conduct of morbidities to reduce mortality.

INTRODUÇÃO: As malformações congênitas fazem parte das principais doenças observadas ao nascimento. Entre as causas de óbito no período neonatal as malformações foram a segunda causa, sendo ainda a primeira, a prematuridade. OBJETIVOS: Caracterizar a evolução clínica dos recém-nascidos (RN) com gastrosquise (GTQ) em uma unidade de terapia intensiva neonatal e descrever as morbidades renal, nutricional e infecciosa relacionados ao manejo clínico pós-natal na unidade de terapia intensiva neonatal MÉTODO: Foi realizado estudo observacional retrospectivo em 50 RN com GTQ, utilizando a associação de anormalidades intestinais, impossibilidade de fechamento primário do defeito abdominal e necesidade de reoperação como critérios de classificação para a doença. O nível de significância foi p < 0,05. RESULTADOS: A admissão hospitalar para cirurgia primária ocorreu com mediana de idade de 2 horas. O total de 14% das crianças foram submetidas a uma interposição de silo primária e 24% apresentaram malformação intestinal associada. Dezenove RN necessitaram mais de uma intervenção cirúrgica. A mediana do tempo de estadia foi de 33 dias, sendo maior nos pacientes com GTQ complexa (56 dias). Todos os RN recuperaram o débito urinário a partir de 48 horas do pós-operatório e 40% apresentaram hiponatremia e oligoanúria nesse período. Não houve diferença entre a natremia e o tempo de jejum (p = 0,79). O ganho ponderal foi similar em ambos os grupos com nutrição parenteral total e tornou-se significativamente maior nos pacientes com GTQ simples após a alimentação enteral (p = 0,0046). Esses RN evoluíram 2,4 vezes com menos colestase. Sepse tardia ocorreu em 58% dos pacientes e foi relacionada à infecção do CVC em 37,9% dos casos. A mortalidade foi maior nos RN infectados com GTQ complexa e a taxa global de mortalidade foi de 14%. CONCLUSÃO: A caracterização clínica dos RN com GTG depende da complexidade e do conhecimento e condução das morbidades para diminuir a mortalidade.

O impacto da introdução precoce de terapia nutricional enteral na redução da morbimortalidade na terapia intensiva pediátrica: uma revisão sistemática / Early enteral nutrition therapy and mortality in a pediatric intensive care unit

OBJETIVO: Avaliar o impacto da introdução precoce de terapia nutricional enteral na redução da morbimortalidade em unidades de terapia intensiva pediátrica. MÉTODOS: Pesquisa bibliográfica nas bases de dados PubMed, Lilacs e Embase dos últimos 10 anos, em língua inglesa e população-alvo de indivíduos de 1 mês de idade a 18 anos, internados em unidades de terapia intensiva pediátrica, usando as palavras-chave: Critical Care, Nutritional Support e Nutrition Disorders or Malnutrition. RESULTADOS: Apesar dos avanços na qualidade dos cuidados clínicos, a prevalência de desnutrição em crianças hospitalizadas permanece imutável nos últimos 20 anos (15-30%) e tem implicações no tempo de internação, curso da doença e morbidade. A desnutrição é comum e é com frequência pouco reconhecida e então, não tratada. A terapia nutricional é parte essencial no tratamento dos pacientes pediátricos gravemente doentes que apresentam estado de hipercatabolismo proteico, que pode ser minimizado com um plano terapêutico nutricional efetivo. Neste estudo, foram revisadas publicações que mostraram que ainda há uma escassez de pesquisas controladas e randomizadas com bom tratamento estatístico em relação à terapia nutricional enteral com desfecho relacionado à morbimortalidade. As diretrizes atuais para terapia nutricional desses pacientes são amplamente baseadas na opinião de experts e em dados extrapolados de estudos em adultos, bem como de estudos realizados em crianças saudáveis. CONCLUSÃO: A evidência científica na utilização de terapia nutricional enteral na melhora da evolução dos pacientes pediátricos gravemente doentes ainda é escassa e são necessários novos estudos focados nisso, além de diretrizes mais bem-formuladas.

OBJECTIVE: To assess the impact of early introduction of enteral nutrition therapy in reducing morbidity and mortality in pediatric intensive care unit. METHODS: Search in the literature of the last 10 years, in English and the target population of individuals aged 1 month to 18 years admitted to pediatric intensive care units in the databases PubMed, Lilacs and Embase using the keywords: Critical Care, Nutritional Support and Nutrition Disorders or Malnutrition. RESULTS: Despite advances in the quality of clinical care, the prevalence of malnutrition in hospitalized children remains unchanged in the last 20 years (15-30%) and has implications for the time of admission, course of illness and morbidity. Malnutrition is common and is often poorly recognized and therefore, untreated. Nutritional therapy is an essential part in the treatment of pediatric patients who have severely ill hypercatabolic state protein, which can be minimized with an effective nutritional treatment plan. In this study, we reviewed publications which have shown that there is still a paucity of randomized and controlled studies with good statistical treatment in relation to enteral nutritional therapy with outcomes related to morbidity and mortality. The current guidelines for nutritional therapy in these patients are largely based on expert opinion and data extrapolated from adult studies and studies in healthy children. CONCLUSION: The scientific evidence on the use of enteral nutrition therapy in improving the development of critically ill pediatric patients is still scarce and further studies are needed focusing on it, and better guidelines must be formulated.

Impact of early enteral nutrition therapy on morbimortality reduction in a pediatric intensive care unit: a systematic review.

OBJECTIVE: To assess the impact of early introduction of enteral nutrition therapy in reducing morbidity and mortality in pediatric intensive care unit. METHODS: Search in the literature of the last 10 years, in English and the target population of individuals aged 1 month to 18 years admitted to pediatric intensive care units in the databases PubMed, Lilacs and Embase using the keywords: Critical Care, Nutritional Support and Nutrition Disorders or Malnutrition. RESULTS: Despite advances in the quality of clinical care, the prevalence of malnutrition in hospitalized children remains unchanged in the last 20 years (15-30%) and has implications for the time of admission, course of illness and morbidity. Malnutrition is common and is often poorly recognized and therefore, untreated. Nutritional therapy is an essential part in the treatment of pediatric patients who have severely ill hypercatabolic state protein, which can be minimized with an effective nutritional treatment plan. In this study, we reviewed publications which have shown that there is still a paucity of randomized and controlled studies with good statistical treatment in relation to enteral nutritional therapy with outcomes related to morbidity and mortality. The current guidelines for nutritional therapy in these patients are largely based on expert opinion and data extrapolated from adult studies and studies in healthy children. CONCLUSION: The scientific evidence on the use of enteral nutrition therapy in improving the development of critically ill pediatric patients is still scarce and further studies are needed focusing on it, and better guidelines must be formulated.

Atuação da equipe multidisciplinar na terapia nutricional de pacientes sob cuidados intensivos / The action of a multidisciplinary team in the nutritional care of critically ill patients

Pacientes hospitalizados podem ter necessidades nutricionais especiais em função da desnutrição e dos desequilíbrios metabólicos impostos pelas doenças. A terapia nutricional, principalmente nos estágios críticos das enfermidades, deve ser administrada de modo seguro e eficaz. Vários estudos têm avaliado o papel da equipe multidisciplinar na administração da terapia nutricional. Com o objetivo de identificar os problemas inerentes à administração da terapia nutricional em pacientes hospitalizados e verificar o impacto da atuação de uma equipe multidisciplinar na qualidade dos procedimentos, foi realizada uma revisão que analisou artigos publicados entre 1980 e 2004 sobre o papel da atuação de equipes multidisciplinares no cuidado e na evolução nutricional de pacientes hospitalizados, principalmente os que se encontravam sob cuidados intensivos. Os termos utilizados na pesquisa foram: "multidisciplinary team", "nutritional support", "parente-ral nutrition", "enteral feeding", "critically ill", "intensive care unit","critically ill child". Dos 130 estudos inicialmente identificados, foram selecionados 24, dos quais 14 compararam o padrão de terapia nutricional com e sem a presença da equipe multidisciplinar. Os principais problemas detectados na administração de terapia nutricional em pacientes hospitalizados foram a oferta inadequada de nutrientes, as complicações infecciosas e metabólicas e o uso excessivo de nutrição parenteral. Nos estudos comparativos, a presença da equipe multidisciplinar melhorou o padrão de oferta nutricional, reduziu a incidência de complicações e os custos.

A suplementacão de glutamina é benéfica em criancas com doencas graves? / Glutamine supplementation: is it beneficial to critically ill children?

Embora haja vários estudos clínicos randômicos avaliando os benefícios da suplementacão de glutamina em pacientes adultos, há pouca informacão em pacientes pediátricos, particularmente naqueles com doencas graves. O objetivo deste estudo foi avaliar criticamente os estudos sobre suplementacão de glutamina, para verificar os possíveis benefícios clínicos desta suplementacão em criancas gravemente doentes. Levou-se a cabo uma pesquisa dos estudos publicados entre 1992 e 2003, do tipo prospectivo, randômico e controlado, conduzidos em pacientes pediátricos gravemente doentes, divididos em dois grupos: o dos que receberam suplementos de glutamina por via enteral ou parenteral, e o dos que receberam placebo. As variáveis de desfecho foram: tempo para atingir oferta plena de dieta enteral, tempo de hospitalizacão, complicacões e mortalidade. Foram utilizadas as bases de dados Medline, LILACS e Cochrane, com as palavras-chave: glutamine, critically ill, trauma, sepsis, burned, injured, bone marrow transplantation, intensive care, mechanical ventilation. Dos 33 estudos inicialmente identificados, 7 preencheram os critérios de inclusão. A análise dos resultados mostrou que nos pacientes suplementados com glutamina, houve melhor aceitacão por via enteral e reducão do tempo de nutricão parenteral em recém-nascidos, não havendo efeito sobre o tempo de internacão, custos hospitalares e mortalidade. Os estudos controlados na faixa etária pediátrica, não permitem que se adote o uso rotineiro da glutamina em criancas gravemente enfermas. O número reduzido de pacientes e a heterogeneidade quanto ao grupo etário, doencas e via de administracão são fatores que limitam a interpretacão dos resultados.