Ataxia-telangiectasia in Latin America: clinical features, immunodeficiency, and mortality in a multicenter study.

Ataxia-telangiectasia (AT) is a rare genetic disorder leading to neurological defects, telangiectasias, and immunodeficiency. We aimed to study the clinical and immunological features of Latin American patients with AT and analyze factors associated with mortality. Referral centers from 9 Latin American countries participated in this retrospective cohort study, and 218 patients were included. Median (IQR) ages at symptom onset and diagnosis were 1.0 (1.0-2.0)  and 5.0 (3.0-8.0) years, respectively. Most patients presented recurrent airway infections, which was significantly associated with IgA deficiency. IgA deficiency was observed in 60.8% of patients and IgG deficiency in 28.6%. T- and B-lymphopenias were also present in most cases. Mean survival was 24.2 years, and Kaplan-Meier 20-year-survival rate was 52.6%, with higher mortality associated with female gender and low IgG levels. These findings suggest that immunologic status should be investigated in all patients with AT.

Descarte de antimicrobianos vencidos e/ou em desuso: estudo de caso / Disposal of expired and/or discontinued antimicrobials - case study / Descarto de antimicrobiales muertos y/o desaparecidos: estudio de casos

A temática do descarte inadequado de medicamentos e sua relevância no desenvolvimento dos mecanismos de resistência aos antibióticos são os menos debatidos na literatura. Portanto, este trabalho tem como objetivo apresentar e discutir os dados obtidos sobre o recebimento de antimicrobianos vencidos e/ou sem uso descartados no ponto de coleta de medicamentos localizado no Departamento de Ciências Farmacêuticas, da Universidade Federal de Pernambuco, Campus Recife. Ao todo foram recolhidos 50,03 kg de medicamentos, dos quais 4,12 kg eram de antimicrobianos, sendo a terceira classe farmacológica mais frequente; destes, 93,07% (n=1.948 unidades) estavam vencidos e a forma farmacêutica mais encontrada foram os comprimidos (36,32%). Entre as classes de antibióticos predominaram, em relação ao mecanismo de ação, os que inibem a síntese proteica (29,33%), e pela estrutura química, as quinolonas (17,45%). A realização do descarte adequado desses medicamentos possibilita uma redução dos impactos que esse quantitativo recolhido causaria no meio ambiente. Portanto, deve-se investir em campanhas de conscientização sobre o uso correto dos medicamentos e seguimento da prescrição médica para evitar consumo indiscriminado destes fármacos. Com base o que estabelece o Decreto n° 10.388/2020, o descarte correto de medicamentos precisa ser divulgado, para que os estabelecimentos realizem a logística reversa dessas substâncias.

The issue of inappropriate disposal of medicines and its relevance in development of mechanisms of resistance to antibiotics is the least discussed in the literature. Therefore, this work aims to present and discuss the data obtained on the receipt of expired and/or unused antimicrobials discarded at the medication collection point located in the Department of Pharmaceutical Sciences, Federal University of Pernambuco, Campus Recife. In all, 50.03 kg of drugs were collected, of which 4.12 kg were antimicrobials, being the third most frequent pharmacological class; of these, 93.07% (n=1,948 units) were expired and the most common pharmaceutical form was pills (36.32%). Among the classes of antibiotics, in terms of mechanism of action, those that inhibit protein synthesis (29.33%) and chemical structure, quinolones (17.45%) predominated. Carrying out the proper disposal of these medicines makes it possible to reduce the impacts that this collected quantity would cause on the environment. Therefore, one should invest in awareness campaigns about the correct use of medicines and follow-up of medical prescriptions to avoid indiscriminate consumption of these drugs. Based on the provisions of Decree No. 10.388/2020, the correct disposal of medicines needs to be disclosed, so that establishments carry out the reverse logistics of these substances.

La cuestión de la eliminación inadecuada de los medicamentos y su relevancia en el desarrollo de mecanismos de resistencia a los antibióticos son los menos discutidos en la literatura. Por lo tanto, este trabajo tiene el objetivo de presentar y discutir los datos obtenidos sobre la recepción de antimicrobianos perdidos y/o no utilizados desechados en el punto de recolección de medicamentos ubicado en el Departamento de Ciencias Farmacéuticas de la Universidad Federal de Pernambuco, Campus Recife. En total, se tomaron 50,03 kg de medicamentos, de los cuales 4,12 kg fueron antimicrobianos, siendo la tercera clase farmacológica más común el 93,07% (n=1.948 unidades) retrasada y la forma farmacéutica más encontrada fueron los comprimidos (36,32%). Entre las clases de antibióticos predominaron, en relación con el mecanismo de acción, las que inhiben la síntesis de proteínas (29,33%), y por la estructura química, las quinolonas (17,45%). La eliminación adecuada de estos medicamentos permite reducir el impacto que esta cantidad recolectada podría causar en el medio ambiente. Por lo tanto, se deberían invertir en campañas de sensibilización sobre el uso correcto de medicamentos y el seguimiento de las recetas médicas para evitar el consumo indiscriminado de estos medicamentos. Sobre la base del Decreto no 10.388/2020, es necesario hacer pública la eliminación correcta de los medicamentos, para que los establecimientos realicen la logística inversa de estas sustancias.

The Emotional Impact of Patient Loss on Brazilian Veterinarians.

Veterinarians, unlike human physicians, could potentially care for the patient for several years, from conception to end-of-life care. Because of their close relationship with the animal, healthcare providers (for example, veterinarians and staff) are more likely to be affected by bad events and end-of-life care. The purpose of this study was to assess the emotional impact of patients' deaths on Brazilian veterinarians; 549 Brazilian veterinarians (78.3% females) completed a 20-item online questionnaire. Females were more emotionally affected than males by having to talk to the owner about their animal's death and more emotionally affected by the animal's death itself. Furthermore, the emotional impact of an animal's death was heavily influenced by the number of animals euthanized and varied greatly across veterinarians based on their age, with vets over 50 years old being less affected than vets between the ages of 31 and 40. The majority of responders (91.0%) were not trained to deal with grief during their degree. Those who had some training reported being less affected by bereavement. These findings indicate that patient death is a significant emotional concern for veterinarians. Specific education during the degree course, aimed at preparing future veterinarians to deal with death and death communication, is lacking but necessary.

Clearance of Persistent SARS-CoV-2 RNA Detection in a NFκB-Deficient Patient in Association with the Ingestion of Human Breast Milk: A Case Report.

Currently, there are no evidence-based treatment options for long COVID-19, and it is known that SARS-CoV-2 can persist in part of the infected patients, especially those with immunosuppression. Since there is a robust secretion of SARS-CoV-2-specific highly-neutralizing IgA antibodies in breast milk, and because this immunoglobulin plays an essential role against respiratory virus infection in mucosa cells, being, in addition, more potent in neutralizing SARS-CoV-2 than IgG, here we report the clinical course of an NFκB-deficient patient chronically infected with the SARS-CoV-2 Gamma variant, who, after a non-full effective treatment with plasma infusion, received breast milk from a vaccinated mother by oral route as treatment for COVID-19. After such treatment, the symptoms improved, and the patient was systematically tested negative for SARS-CoV-2. Thus, we hypothesize that IgA and IgG secreted antibodies present in breast milk could be useful to treat persistent SARS-CoV-2 infection in immunodeficient patients.

Effective combined antiretroviral therapy provides partial immune recovery to mycobacterial antigens in vertically infected, BCG-vaccinated youth living with HIV.

BACKGROUND: We assessed the cytokine response by PBMC of youth living with HIV (YLHIV) under combined antiretroviral therapy (cART) to Mycobacterium tuberculosis (Mtb) and Mycobacterium bovis (BCG) antigens. METHODS: PBMC from 20 Brazilian YLHIV under cART with long-term (≥1 year) virological control, and 20 healthy controls were cultured for 24-96 h under stimulation with BCG, Mtb lysates, ESAT-6 and SEB. We measured TNF-α, IFN-γ, IL-2, IL-4, IL-5, IL-10 and IL-17 in culture supernatants using a cytometric bead array. RESULTS: Controls had higher IFN-γ production at 24, 48, 72 and 96 h upon stimulation with BCG lysate, plateauing at 48 h (Median = 1991 vs. 733 pg/mL; p = 0.01), and after 48-72 h of stimulation with Mtb lysate, plateauing at 48 h (3838 vs. 2069 pg/mL; p = 0.049). YLHIV had higher TNF-α production at all time points upon stimulation with ESAT-6, with highest concentration at 36 h (388 vs. 145 pg/mL; p = 0.02). Within the YLHIV group, total CD4 T cell count and CD4/CD8 ratio were associated with IFN-γ response to Mtb lysate and ESAT-6, respectively. CONCLUSIONS: Even under long-term cART, YLHIV seem to have a suboptimal T-helper-1 response to mycobacterial antigens. This can be explained by early immunodeficiency in vertical infection, with lasting damage.

Ambulatório de Saúde Integral para Travestis e Transexuais de Diadema (Didlrans): o SUS não tem preconceito / Comprehensive Health Outpatient Clinic for Transvestites and Transsexuals in Diadema (DiaTrans): the SUS has no prejudice

Este artigo narra a formação do Ambulatório de Saúde Integral para População de Travestis e Transexuais no Município de Diadema (DiaTrans), primeiro serviço de atenção a este publico na regido do ABC, na Grande São Paulo. A experiência nas atividades de prevenção, promoção e proteção e as ações em campo que nos permitiram a atenção recorrente a esta população, considerando suas especificidades, possibilitaram a reflexão e a verificação das condutas, fluxos que vão além do processo transexualizador realizado pelo SUS e do acompanhamento hormonal, o que colaborou para a confecção de uma política de atenção a sua saúde integral e regulamentações acerca do uso do nome social desta população no município de Diadema.

Elevated IgA and IL-10 levels in very-early-onset inflammatory bowel disease secondary to IL-10 receptor deficiency / Níveis elevados de IgA e IL-10 na doença inflamatória intestinal de início muito precoce secundária à deficiência do receptor de IL-10

ABSTRACT Objective: To report two patients with very-early-onset inflammatory bowel disease (VEOIBD) secondary to interleukin-10 receptor (IL-10R) mutations, explore immunophenotyping data and plasma cytokine profile on these cases compared to healthy controls, and describe the phenotype of IL-10/IL-10R mutations based on a literature review. Case description: We report on two female infants referred to our tertiary center at the age of ten months, with severe colonic and perianal disease, as well as significant malnutrition, who had shown limited response to usual inflammatory bowel disease (IBD) therapy agents. In the first case, whole-exome sequencing (WES) revealed a homozygous (c.537G>A/p.T179T) mutation in exon 4 of the IL-10RA gene, while in the second patient, compound heterozygosity was identified, also in the IL-10RA gene (chr11:117.859.199 variant A>G/p.Tyr57Cys and chr11: 117.860.335 variant G>T/p.Val123Leu). Both patients underwent hematopoietic cell transplantation (HCT). Immunological work-up of these patients revealed increased IL-10 plasma levels and increased IgA. Comments: Our case reports disclose novel findings on plasma cytokine profile in IL-10R deficiency, and we describe the severe phenotype of IL-10/IL-10R deficiency that should be recognized by physicians.

RESUMO Objetivo: Relatar os casos de duas pacientes com doença inflamatória intestinal de início muito precoce (em inglês VEOIBD) secundária a mutações do receptor de interleucina 10 (IL-10R), explorar dados de imunofenotipagem e perfil de citocinas plasmáticas nesses casos em comparação com indivíduos saudáveis e descrever o fenótipo de mutações IL-10/IL-10R com base em uma revisão da literatura. Descrição do caso: Duas lactentes do sexo feminino foram encaminhadas ao nosso centro terciário, ambas com dez meses no momento do encaminhamento, com doença colônica e perianal grave, bem como desnutrição significativa, tendo uma resposta limitada aos agentes de terapia usuais de doença inflamatória intestinal (DII). No primeiro caso, o sequenciamento completo do exoma revelou mutação homozigótica (c. 537G>A/p.T179T) no exon 4 do gene IL-10RA, enquanto no segundo caso heterozigosidade composta foi identificada também no gene IL-10RA [chr11: 117.859.199 - variante A>G/p.Tyr57Cys e chr11: 117.860.335 - variante G>T/ p.Val123Leu]. Ambas as pacientes foram submetidas a Transplante de Células-Tronco Hematopoiéticas. A investigação imunológica das pacientes revelou aumento dos níveis plasmáticos de IL-10 e aumento da IgA. Comentários: Nossos relatos de casos descrevem novos achados no perfil de citocinas plasmáticas na deficiência de IL-10R, e relatamos o fenótipo grave da deficiência de IL-10/IL-10R que deve ser reconhecido pelos médicos.

Elevated IgA and IL-10 levels in very-early-onset inflammatory bowel disease secondary to IL-10 receptor deficiency.

OBJECTIVE: To report two patients with very-early-onset inflammatory bowel disease (VEOIBD) secondary to interleukin-10 receptor (IL-10R) mutations, explore immunophenotyping data and plasma cytokine profile on these cases compared to healthy controls, and describe the phenotype of IL-10/IL-10R mutations based on a literature review. CASE DESCRIPTION: We report on two female infants referred to our tertiary center at the age of ten months, with severe colonic and perianal disease, as well as significant malnutrition, who had shown limited response to usual inflammatory bowel disease (IBD) therapy agents. In the first case, whole-exome sequencing (WES) revealed a homozygous (c.537G>A/p.T179T) mutation in exon 4 of the IL-10RA gene, while in the second patient, compound heterozygosity was identified, also in the IL-10RA gene (chr11:117.859.199 variant A>G/p.Tyr57Cys and chr11: 117.860.335 variant G>T/p.Val123Leu). Both patients underwent hematopoietic cell transplantation (HCT). Immunological work-up of these patients revealed increased IL-10 plasma levels and increased IgA. COMMENTS: Our case reports disclose novel findings on plasma cytokine profile in IL-10R deficiency, and we describe the severe phenotype of IL-10/IL-10R deficiency that should be recognized by physicians.

Phenotypes of STAT3 gain-of-function variant related to disruptive regulation of CXCL8/STAT3, KIT/STAT3, and IL-2/CD25/Treg axes.

STAT3 is a cytokine-signaling transcription factor critical for gene regulation. Gain-of-function (GOF) mutations in STAT3 are associated with lymphoproliferation, autoimmune cytopenias, increased susceptibility to infection, early-onset solid-organ autoimmunity, short stature, and eczema. We studied the JAK/STAT signaling pathway gene expression and the cytokine profile in two families carrying STAT3-GOF variants to shed light on the STAT3-GOF-associated variable expressivity, including the identification of disease markers. Considering 92 target genes, KIT and IL2RA were downregulated only in patients with high clinical penetrance, while CXCL8 was markedly downregulated for all of them. Unlike previous studies, SOCS3-a STAT3 inhibitor-was not upregulated in patients. In addition, low levels of IL-2 and a reduced numbers of Tregs cells were strikingly prevalent in patients. This study shows a disruptive role of STAT3-GOF variants in the regulatory axis activities CXCL8/STAT3, KIT/STAT3, IL2/CD25/Treg, which, by slightly different mechanisms, underlie the broad clinical spectrum seen in the studied patients. In addition, we suggest the investigation of CXCL8 as a biomarker for identifying STAT3-GOF mutation.

Historical and social construction of the practices of auxiliary nurses and nurse technicians in Primary Care.

OBJECTIVES: to analyze the practices of nursing auxiliaries and technicians in the context of Primary Health Care in a city in the interior of the state of São Paulo. METHODS: transversal, quantitative study, realized with 104 nursing auxiliaries and 46 nursing technicians acting in the Primary Attention. The data, collected using structured questionnaires, are presented by descriptive statistics, and analyzed based on the essential elements of First Contact and Longitudinality. RESULTS: the professionals were, majority, women, married, with children, low political and collective participation. We identified thirteen activities related to the First Contact: and ten on Longitudinality. CONCLUSIONS: the insertion and capillarization of auxiliary and nursing technicians' practices show these professionals as the main ones responsible for the first contact in this context, highlighting their relevance in the longitudinality of care and the work composition of health teams, contributing to the consolidation of the Unified Health System (SUS).

Assessment of delivery and childbirth care in the maternity units of Rede Cegonha: the methodological paths. / Avaliação da atenção ao parto e nascimento nas maternidades da Rede Cegonha: os caminhos metodológicos.

This article describes the methodology used to evaluate delivery and childbirth care practices in maternity hospitals that belong to the Rede Cegonha, according to scientific evidence and rights guarantee. It shows the maternity selection criteria, the evaluated guidelines, their devices and check items, the method used to collect information and the treatment of data to obtain the results. It discusses the chosen guidelines and the strategy of returning results to managers and services and discusses their potential to foster management qualification processes and obstetric and neonatal care. This is a study of delivery and childbirth care practices of 606 maternity hospitals selected for the second evaluation cycle of the Rede Cegonha. The methodological paths stood out for the construction of tripartite co-responsibility for the process and the evaluation results, with an emphasis on its usefulness for the decision-makers and the hospital institutions involved.

Este artigo descreve o método utilizado na avaliação de práticas de cuidado ao parto e nascimento em maternidades da Rede Cegonha. Apresenta os critérios de seleção das maternidades, as diretrizes avaliadas, seus dispositivos e itens de verificação, o método utilizado para coleta das informações e o tratamento dos dados para obtenção dos resultados. Dialoga a respeito das diretrizes escolhidas e da estratégia de devolutiva dos resultados aos gestores e serviços, e discute seu potencial para fomentar processos de qualificação da gestão e atenção obstétrica e neonatal. Trata-se de estudo das práticas de atenção ao parto e nascimento de 606 maternidades selecionadas para o segundo ciclo avaliativo da Rede Cegonha. Os caminhos metodológicos primaram pela construção de corresponsabilidade tripartite para com o processo e os resultados da avaliação, com ênfase na sua utilidade para os tomadores de decisão e instituições hospitalares envolvidas.

Labor and childbirth care in maternities participating in the "Rede Cegonha/Brazil": an evaluation of the degree of implementation of the activities. / Atenção ao parto e nascimento em Maternidades da Rede Cegonha/Brasil: avaliação do grau de implantação das ações.

Using a judgment framework, this article analyzes the degree of implementation of the best practices in labor and childbirth care contained in the guidelines of the Rede Cegonha (RC) across Brazil. The study eligibility criteria were public and mixed hospitals located in a health region with a RC action plan in place in 2015, resulting in a total of 606 facilities distributed across the country. Three different data collection methods were used: face-to-face interviews with managers, health professionals and puerperal women; document analysis; and on-site observation. The framework was built around the five guidelines of the Labor and Childbirth component of the RC. Degree of implantation was rated as follows: adequate; partially adequate and inadequate. The performance of maternity facilities was rated as partially adequate for all guidelines except for hospital environment, which was rated as inadequate. A huge variation in degree of implementation was observed across regions, with the South and Southeast being the best-performing regions in most items. The results reinforce the need for an ongoing evaluation of the actions developed by the RC to inform policy-making and the regulation of labor and childbirth care.

O artigo analisa o grau de implantação das Boas Práticas de Atenção ao Parto e Nascimento conforme preconizado pela Rede Cegonha (RC) a partir da configuração de uma matriz de julgamento para o Brasil e grandes regiões. Foram elegíveis para a avaliação todos os 606 hospitais públicos e mistos das regiões de saúde que dispunham de plano de ação da RC em 2015. Foram utilizados três diferentes métodos de coleta de dados: entrevista pessoal com gestores, profissionais de saúde e puérperas; análise documental; e observação in loco. A matriz foi composta com as cinco diretrizes da RC. Para julgamento da adequação da implantação, foi utilizado como parâmetro: adequado; parcialmente adequado e não adequado. Todas as diretrizes foram avaliadas como parcialmente adequada, exceto a ambiência que foi não adequado. A atenção ao parto e nascimento encontra-se em estágios diferenciados de implantação com variações entre as grandes regiões. As regiões Sul e Sudeste, apresentaram situação privilegiada quanto ao grau de implantação da maioria dos itens analisados. Os resultados evidenciam que a avaliação das ações da RC deve fazer parte do alicerce de informações empregadas no direcionamento de políticas e regulamentação na atenção hospitalar ao parto e nascimento.

Reduction of inequities of access to appropriate childbirth care in Rede Cegonha. / Redução das iniquidades sociais no acesso às tecnologias apropriadas ao parto na Rede Cegonha.

This article compares the findings of "Avaliação da Rede Cegonha" (ARC - Stork Network Assessment), an evaluative study on the Rede Cegonha (RC - Stork Network) program, with Nascer no Brasil (NB - Born in Brazil), a national survey on labor and birth, conducted in 2011-12, before the start implementation of RC. ARC was conducted in 2017, in 606 maternity hospitals involved in RC and NB included a sample with national representation of 266 hospitals. In the current analysis, we included the 136 SUS hospitals that participated in both studies, totaling 3,790 and 12,227 puerperal women. We perform comparisons of best practices and interventions in the management of labor and delivery using Pearson's chi-square test for independent samples. The prevalence of best practices was, on average, 150% higher in ARC than in NB, with a greater relative increase in less developed regions, for older, brown and black women and less educated. Regarding interventions, there was an average reduction of 30% between NB and ARC, with a greater relative reduction in less developed regions and less educated women. There was a significant improvement in the scenario of care for labor and childbirth, with a reduction in regional, educational and racial inequalities in access to appropriate technologies, suggesting that the RC intervention was effective.

Este artigo compara os achados da Avaliação da Rede Cegonha (ARC), estudo avaliativo sobre o programa Rede Cegonha (RC), com o Nascer no Brasil (NB), inquérito nacional sobre parto e nascimento, realizado em 2011-12, antes do início da implementação da RC. A ARC foi conduzida em 2017, em 606 maternidades envolvidas na RC e o NB e empregou uma amostra com representatividade nacional de 266 hospitais. Na análise atual, incluímos os 136 hospitais do SUS que participaram de ambos os estudos, totalizando 3.790 e 12.227 puérperas, respectivamente. Realizamos as comparações de boas práticas e intervenções no manejo do trabalho de parto e de parto utilizando o teste qui-quadrado para amostras independentes. A prevalência das boas práticas foi, em média, 150% maior na ARC que no NB, com maior aumento relativo nas regiões menos desenvolvidas, para mulheres mais velhas, pardas e pretas e menos escolarizadas. Com relação às intervenções, houve redução média de 30% entre o NB e a ARC, com maior redução relativa nas regiões menos desenvolvidas e nas mulheres menos escolarizadas. Houve melhoria significativa no cenário da atenção ao trabalho de parto e parto, com diminuição de iniquidades regionais, de nível de instrução e raciais no acesso às tecnologias apropriadas, sugerindo que a intervenção da RC foi efetiva.

Avaliação da atenção ao parto e nascimento nas maternidades da Rede Cegonha: os caminhos metodológicos / Assessment of delivery and childbirth care in the maternity units of Rede Cegonha: the methodological paths

Resumo Este artigo descreve o método utilizado na avaliação de práticas de cuidado ao parto e nascimento em maternidades da Rede Cegonha. Apresenta os critérios de seleção das maternidades, as diretrizes avaliadas, seus dispositivos e itens de verificação, o método utilizado para coleta das informações e o tratamento dos dados para obtenção dos resultados. Dialoga a respeito das diretrizes escolhidas e da estratégia de devolutiva dos resultados aos gestores e serviços, e discute seu potencial para fomentar processos de qualificação da gestão e atenção obstétrica e neonatal. Trata-se de estudo das práticas de atenção ao parto e nascimento de 606 maternidades selecionadas para o segundo ciclo avaliativo da Rede Cegonha. Os caminhos metodológicos primaram pela construção de corresponsabilidade tripartite para com o processo e os resultados da avaliação, com ênfase na sua utilidade para os tomadores de decisão e instituições hospitalares envolvidas.

Abstract This article describes the methodology used to evaluate delivery and childbirth care practices in maternity hospitals that belong to the Rede Cegonha, according to scientific evidence and rights guarantee. It shows the maternity selection criteria, the evaluated guidelines, their devices and check items, the method used to collect information and the treatment of data to obtain the results. It discusses the chosen guidelines and the strategy of returning results to managers and services and discusses their potential to foster management qualification processes and obstetric and neonatal care. This is a study of delivery and childbirth care practices of 606 maternity hospitals selected for the second evaluation cycle of the Rede Cegonha. The methodological paths stood out for the construction of tripartite co-responsibility for the process and the evaluation results, with an emphasis on its usefulness for the decision-makers and the hospital institutions involved.

Redução das iniquidades sociais no acesso às tecnologias apropriadas ao parto na Rede Cegonha / Reduction of inequities of access to appropriate childbirth care in Rede Cegonha

Resumo Este artigo compara os achados da Avaliação da Rede Cegonha (ARC), estudo avaliativo sobre o programa Rede Cegonha (RC), com o Nascer no Brasil (NB), inquérito nacional sobre parto e nascimento, realizado em 2011-12, antes do início da implementação da RC. A ARC foi conduzida em 2017, em 606 maternidades envolvidas na RC e o NB e empregou uma amostra com representatividade nacional de 266 hospitais. Na análise atual, incluímos os 136 hospitais do SUS que participaram de ambos os estudos, totalizando 3.790 e 12.227 puérperas, respectivamente. Realizamos as comparações de boas práticas e intervenções no manejo do trabalho de parto e de parto utilizando o teste qui-quadrado para amostras independentes. A prevalência das boas práticas foi, em média, 150% maior na ARC que no NB, com maior aumento relativo nas regiões menos desenvolvidas, para mulheres mais velhas, pardas e pretas e menos escolarizadas. Com relação às intervenções, houve redução média de 30% entre o NB e a ARC, com maior redução relativa nas regiões menos desenvolvidas e nas mulheres menos escolarizadas. Houve melhoria significativa no cenário da atenção ao trabalho de parto e parto, com diminuição de iniquidades regionais, de nível de instrução e raciais no acesso às tecnologias apropriadas, sugerindo que a intervenção da RC foi efetiva.

Abstract This article compares the findings of "Avaliação da Rede Cegonha" (ARC - Stork Network Assessment), an evaluative study on the Rede Cegonha (RC - Stork Network) program, with Nascer no Brasil (NB - Born in Brazil), a national survey on labor and birth, conducted in 2011-12, before the start implementation of RC. ARC was conducted in 2017, in 606 maternity hospitals involved in RC and NB included a sample with national representation of 266 hospitals. In the current analysis, we included the 136 SUS hospitals that participated in both studies, totaling 3,790 and 12,227 puerperal women. We perform comparisons of best practices and interventions in the management of labor and delivery using Pearson's chi-square test for independent samples. The prevalence of best practices was, on average, 150% higher in ARC than in NB, with a greater relative increase in less developed regions, for older, brown and black women and less educated. Regarding interventions, there was an average reduction of 30% between NB and ARC, with a greater relative reduction in less developed regions and less educated women. There was a significant improvement in the scenario of care for labor and childbirth, with a reduction in regional, educational and racial inequalities in access to appropriate technologies, suggesting that the RC intervention was effective.

Atenção ao parto e nascimento em Maternidades da Rede Cegonha/Brasil: avaliação do grau de implantação das ações / Labor and childbirth care in maternities participating in the "Rede Cegonha/Brazil": an evaluation of the degree of implementation of the activities

Resumo O artigo analisa o grau de implantação das Boas Práticas de Atenção ao Parto e Nascimento conforme preconizado pela Rede Cegonha (RC) a partir da configuração de uma matriz de julgamento para o Brasil e grandes regiões. Foram elegíveis para a avaliação todos os 606 hospitais públicos e mistos das regiões de saúde que dispunham de plano de ação da RC em 2015. Foram utilizados três diferentes métodos de coleta de dados: entrevista pessoal com gestores, profissionais de saúde e puérperas; análise documental; e observação in loco. A matriz foi composta com as cinco diretrizes da RC. Para julgamento da adequação da implantação, foi utilizado como parâmetro: adequado; parcialmente adequado e não adequado. Todas as diretrizes foram avaliadas como parcialmente adequada, exceto a ambiência que foi não adequado. A atenção ao parto e nascimento encontra-se em estágios diferenciados de implantação com variações entre as grandes regiões. As regiões Sul e Sudeste, apresentaram situação privilegiada quanto ao grau de implantação da maioria dos itens analisados. Os resultados evidenciam que a avaliação das ações da RC deve fazer parte do alicerce de informações empregadas no direcionamento de políticas e regulamentação na atenção hospitalar ao parto e nascimento.

Abstract Using a judgment framework, this article analyzes the degree of implementation of the best practices in labor and childbirth care contained in the guidelines of the Rede Cegonha (RC) across Brazil. The study eligibility criteria were public and mixed hospitals located in a health region with a RC action plan in place in 2015, resulting in a total of 606 facilities distributed across the country. Three different data collection methods were used: face-to-face interviews with managers, health professionals and puerperal women; document analysis; and on-site observation. The framework was built around the five guidelines of the Labor and Childbirth component of the RC. Degree of implantation was rated as follows: adequate; partially adequate and inadequate. The performance of maternity facilities was rated as partially adequate for all guidelines except for hospital environment, which was rated as inadequate. A huge variation in degree of implementation was observed across regions, with the South and Southeast being the best-performing regions in most items. The results reinforce the need for an ongoing evaluation of the actions developed by the RC to inform policy-making and the regulation of labor and childbirth care.

Human Inborn Errors of Immunity (HIEI): predominantly antibody deficiencies (PADs): if you suspect it, you can detect it.

OBJECTIVE: This minireview gathers the scientific foundations of the literature on genetic errors in the development of the humoral immune system to help pediatricians suspect these defects. SOURCES: A systemic search using the PubMed MEDLINE database was performed for all Predominantly Antibody Deficiencies (PADs) described in the 2020 IUIS Expert Committee for PID classification system, combined with terms for hypogammaglobulinemia. Search terms for PADs were based on the listed names and affected genes as classified by the IUIS 2020. Abstracts of the results were reviewed to find relevant case series, review articles of PADs associated with infection, opportunistic infection, autoimmunity, cytopenias, malignancies, inflammatory diseases, neurological and respiratory diseases. References from relevant articles were further reviewed for additional references. Relevant findings were grouped in accordance with the IUIS 2020 classification system. Clinical and genetic features, if known, were described. DATA SYNTHESIS: PADs refer to impaired antibody production due to molecular defects intrinsic to B cells or a failure of interaction between B and T cells. The patients develop recurrent or chronic infection or respond to the antigens with dysregulation of the immune function, causing severe allergy, autoimmunity, inflammation, lymphoproliferation and malignancy. The diagnosis is a combined exercise of clinical and laboratory investigation similar to that performed by Bruton (1952). In the context of SARS-CoV-2 infection, the experience of XLA and CVID patients has been surprising. Variants in 39 genes were reported as causing PADs, but the clinical heterogeneity within each variant is not clear. CONCLUSION: Bruton (1952) used clinical expertise and protein electrophoresis to identify XLA. The IUIS (2020) committee used immunoglobulins and B lymphocyte to characterize PADs. Pediatricians should suspect it to detect it and prevent morbidities that can have an astonishing and irreversible impact on the child's life.

Longitudinalidade na atenção primária à saúde: revisão integrativa da literatura / Longitudinality in primary health care: an integrative literature review / Longitudinalidad en la atención primaria a la salud: revisión integrativa de la literatura

Objetivo: analisar a produção científica que descreve a longitudinalidade na Atenção Primária à Saúde e verificar como ocorre nos serviços. Método: revisão integrativa da literatura de estudos que tratam sobre a longitudinalidade do cuidado nos serviços. Foram utilizadas as bases de dados Literatura Latino-America e Caribe em Ciências da Saúde, Public MEDLINE, SciVerseScopus e no Portal Regional da Biblioteca Virtual em Saúde utilizando os termos: "Primary Health Care", "Longitudinality" e "Public Health". Resultados: 18 artigos, os quais foram categorizados pela sua similaridade nos temas: estudos sobre os atributos da APS, participação dos usuários na efetivação da longitudinalidade e longitudinalidade no cuidado à criança. Conclusão: a confiança mútua entre profissionais e usuários, a continuidade da atenção, a escuta, a credibilidade e a vinculação do usuário com os profissionais configuram a longitudinalidade nos serviços de saúde

Objective:To analyze the scientific production that describes longitudinality in Primary Health Care and to verify how it occurs in the services. Method: integrative review of the literature of studies that deal with the longitudinality of care in services. The Latin American and Caribbean Literature in Health Sciences, Public Medline, SciVerseScopus and the Regional Portal of the Virtual Health Library were used using the terms "Primary Health Care", "Longitudinality" and "Public Health". Results: 18 articles, which were categorized by their similarity in the themes: studies on the attributes of APS, participation of users in the effectiveness of longitudinality and longitudinality in child care. Conclusion: mutual trust between professionals and users, continuity of attention, listening, credibility and the link between the user and the professionals configures longitudinality in health services

Objetivo: Analizar la producción científica que describe la longitudinalidad en la Atención Primaria a la Salud y verificar cómo ocurre en los servicios. Método: revisión integrativa de la literatura de estudios que tratan sobre la longitudinalidad del cuidado en los servicios. Se utilizaron las bases de datos Literatura Latinoamérica y Caribe en Ciencias de la Salud, Public Medline, SciVerseScopus y en el Portal Regional de la Biblioteca Virtual en Salud utilizando los términos: "Primary Health Care", "Longitudinality" y "Public Health". Resultados: 18 artículos, los cuales fueron categorizados por su similitud en los temas: estudios sobre los atributos de la APS, par-ticipación de los usuarios en la efectividad de la longitudinalidad y longitudinalidad en el cuidado al niño. Conclusión: la confianza mutua entre profesionales y usuarios, la continuidad de la atención, la escucha, la credibilidad y la vinculación del usuario con los profesionales configuran la longitudinalidad en los servicios de salud