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- Impact of COVID-19 Infection on Lung Function and Nutritional Status Amongst Individuals with Cystic Fibrosis: A Global Cohort Study.Publication . Semenchuk, Julie; Naito, Yumi; Charman, Susan C; Carr, Siobhán B; Cheng, Stephanie Y; Marshall, Bruce C; Faro, Albert; Elbert, Alexander; Gutierrez, Hector H; Goss, Christopher H; Karadag, Bulent; Burgel, Pierre-Régis; Colombo, Carla; Salvatore, Marco; Padoan, Rita; Daneau, Géraldine; Harutyunyan, Satenik; Kashirskaya, Nataliya; Kirwan, Laura; Middleton, Peter G; Ruseckaite, Rasa; de Monestrol, Isabelle; Naehrlich, Lutz; Mondejar-Lopez, Pedro; Jung, Andreas; van Rens, Jacqui; Bakkeheim, Egil; Orenti, Annalisa; Zomer-van Ommen, Domenique; da Silva-Filho, Luiz Vicente Rf; Fernandes, Flavia Fonseca; Zampoli, Marco; Stephenson, Anne LBackground: Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF). Methods: This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021. Forced expiratory volume in one second percent predicted (ppFEV1) and body mass index (BMI) twelve months prior to and following a diagnosis of COVID-19 were recorded. Change in mean ppFEV1 and BMI were compared using a t-test. A linear mixed-effects model was used to estimate change over time and to compare the rate of change before and after infection. Results: A total of 6,500 cases of COVID-19 in pwCF from 33 countries were included for analysis. The mean difference in ppFEV1 pre- and post-infection was 1.4 %, (95 % CI 1.1, 1.7). In those not on modulators, the difference in rate of change pre- and post-infection was 1.34 %, (95 % CI -0.88, 3.56) per year (p = 0.24) and -0.74 % (-1.89, 0.41) per year (p = 0.21) for those on elexacaftor/tezacaftor/ivacaftor. No clinically significant change was noted in BMI or BMI percentile before and after COVID-19 infection. Conclusions: No clinically meaningful impact on lung function and BMI trajectory in the year following infection with COVID-19 was identified. This work highlights the ability of the global CF community to unify and address critical issues facing pwCF.
- Cold Agglutinin Syndrome and Hemophagocytic Lymphohistiocytosis: An Unusual Combination Caused by Epstein-Barr Virus Infection.Publication . Sousa Nunes, Beatriz; Gouveia, Catarina; Kjollerstrom, Paula; Farela Neves, JoãoAutoimmune hemolytic anemia (AIHA) and hemophagocytic lymphohistiocytosis (HLH) are rare complications of infectious mononucleosis. The authors describe a 12-year-old male with acute infectious mononucleosis, hepatitis, cholestasis, and an autoimmune hemolytic disorder caused by cold agglutinins IgM (anti-i specificity). Clinical deterioration with persistent fever, anemia, and hepatosplenomegaly was consistent with cold AIHA plus concomitant HLH. The patient was treated with corticosteroids and acyclovir, with an uneventful recovery. Although rare, cold agglutinin syndrome and HLH can complicate infectious mononucleosis and should be considered in a patient with clinical deterioration. Corticosteroids are the mainstay treatment of HLH and may be beneficial in infection-associated cold agglutinin syndrome.
- Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases: Five-year Experience of a Pediatric Tertiary Hospital in Portugal.Publication . Rebelo, Mafalda; Francisco, Telma; Perry da Câmara, Rosário; Pereira, Andreia; Iraneta, Amets; Amorim, Marta; Paiva Lopes, Maria João; Lopes da Silva, Rita; Cordeiro, Ana IsabelIntroduction: Neurocutaneous syndromes (NCS) are a heterogeneous group of conditions with multiorgan involvement and diverse manifestations, evolving throughout life with significant morbidity. A multidisciplinary approach to NCS patients has been advocated, although a specific model is not yet established. The aim of this study was 1) to describe the organization of the recently created Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases (MOCND) at a Portuguese pediatric tertiary hospital; 2) to share our institutional experience focusing on the most common conditions, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC); 3) to analyze the advantages of a multidisciplinary center and approach in NCS. Methods: Retrospective analysis of 281 patients enrolled in the MOCND over the first five years of activity (October 2016 to December 2021), reviewing genetics, family history, clinical features, complications, and therapeutic strategies for NF1 and TSC. Results: The clinic works weekly with a core team of pediatricians and pediatric neurologists supported by other specialties as needed. Of the 281 patients enrolled, 224 (79.7%) had identifiable syndromes such as NF1 (n = 105), TSC (n = 35), hypomelanosis of Ito (n = 11), Sturge-Weber syndrome (n = 5), and others. In NF1 patients, 41.0% had a positive family history, all manifested café-au-lait macules, 38.1% neurofibromas with 45.0% being large plexiform neurofibromas. Sixteen were under treatment with selumetinib. Genetic testing was performed in 82.9% of TSC patients with pathogenic variants found in TSC2 gene in 72.4% patients (82.7% if considered contiguous gene syndrome). Family history was positive in 31.4%. All TSC patients presented hypomelanotic macules and fulfilled diagnostic criteria. Fourteen patients were being treated with mTOR inhibitors. Conclusion: Offering a systematic and multidisciplinary approach to NCS patients enables timely diagnosis, promotes a structured follow-up, and encourages discussion to outline management plans for optimal care to every patient, with significant impact on the quality of life of patients and families.
- Under-Five Mortality and Associated Risk Factors in Children Hospitalized at David Bernardino Pediatric Hospital (DBPH), Angola: A Hierarchical Approach.Publication . Avelino, Israel C; Van-Dúnem, Joaquim; Varandas, LuísReducing under-five mortality is a crucial indicator of overall development in a country. However, in Angola, understanding the factors contributing to hospital deaths in this vulnerable demographic remains incomplete despite improvements in healthcare infrastructure and public health policies. With one of the highest under-five mortality rates in sub-Saharan Africa, Angola faces significant challenges such as malaria, malnutrition, pneumonia, neonatal conditions, and intestinal infectious diseases, which are the leading causes of death among children. This study aimed to identify factors associated with hospital deaths among children aged 28 days to five years admitted to DBPH in Luanda between May 2022 and June 2023. Using a hospital-based case-control design, the study included 1020 children, among whom 340 experienced hospital deaths. Distal and intermediate determinants emerged as primary predictors of hospital mortality, showing significant associations with: mother without schooling (OR [95%CI] 4.3 [1.2-15.7], < 0.027); frequent alcohol consumption during pregnancy (OR [95%CI] 3.8 [2.5-5.9], < 0.001); hospital stay ≤24 h (OR [95%CI] 13.8 [6.2-30.8], < 0.001); poor nutritional status (OR [95%CI] 2.1 [1.4-3.2], < 0.001); short interbirth interval (OR [95%CI] 1.7 [1.1-2.5], < 0.014); maternal age ≤19 years (OR [95%CI] 5.6 [3.0-10.8], < 0.001); and maternal age ≥35 years (OR [95%CI] 2.1 [1.2-3.7], < 0.006). These findings highlight the preventable nature of most under-five hospital deaths and underscore the urgent need to address social inequities and improve the quality of primary healthcare services to effectively reduce child mortality in Angola.
- The Role of Nurses Caring for Children Diagnosed with Sickle Cell Anemia and Their Families in a Hospital Setting: A Rapid Review of the Recent Literature.Publication . Freitas, Eduarda; Loura, David; Inês, Mariana; Martins, Carla; Duarte, Inês: Sickle cell anemia (SCA) affects a significant number of children worldwide, for whom the progression of the disease can lead to functional disability-impaired development. Nurses are pivotal in providing holistic care to these children and their families. This review aims to identify recent evidence on the role of nurses in intervening with children with SCA and their families in a hospital setting. : A rapid review reported under the PRISMA methodology was carried out in the CINAHL, MEDLINE, and Scopus databases with the expression (sickle cell anemia OR sickle cell disease) AND (child* OR family OR pediatric*) AND (nurs* OR "nursing interventions" OR "pediatric nursing") AND (hospital*), considering studies between 2019 and 2024, written in English, identifying articles with insights about the role of nurses in this context. Articles other than primary or secondary studies were excluded. Data were analyzed through a rapid qualitative approach. : Fifty-two studies were identified and seventeen articles were included. The nurse's role is key and multidisciplinary, focusing on the child and family (care management and therapeutic education), the team (training, and the promotion of safety and quality of care), and the health system (optimizing access to care and promoting adequate resources for its implementation). Such a role is important for short-term clinical problems and to prevent long-term complications. : Nurses play a central role in empowering families and coordinating multidisciplinary care. Greater investment is needed at a clinical level, through a more effective response to the needs of these patients, and in research, through experimental studies and other designs focused on multidisciplinary interventions.
- Under-Five Mortality and Social Determinants in Africa: a Systematic ReviewPublication . Avelino, Israel C; Van-Dúnem, Joaquim; Varandas, LuísPurpose: Under-five mortality is a key public health indicator, highly responsive to preventive interventions. While global efforts have made strides in reducing mortality rates in this age group, significant disparities persist, particularly in Sub-Saharan Africa. This study aimed to systematically review the factors influencing under-five mortality in Africa, focusing on sociodemographic factors and health-related determinants. Methods: A systematic review was conducted adhering to PRISMA guidelines. Studies were identified from a range of well-established indexed academic databases. Keywords and Boolean operators facilitated relevant study retrieval. Only articles published in English, Portuguese, or Spanish between January 2013 and November 2024, in peer-reviewed journals, were included. Methodological quality assessment utilised the Joanna Briggs Institute tool. Results: Of the 602 studies identified, 39 met the inclusion criteria. Key determinants of under-five mortality included socioeconomic factors such as poverty and maternal education, along with maternal age extremes, multiparity, inadequate prenatal care, and low birth weight. Conclusion: Addressing social disparities, particularly through enhanced maternal education and improved access to primary healthcare, is critical in reducing under-five mortality in Africa. The findings underscore the importance of targeted interventions that address both social and healthcare-related factors to mitigate child mortality in the region. What is known: •Under-five mortality in Sub-Saharan Africa is primarily driven by preventable infectious diseases, such as diarrhoea, pneumonia, malaria, and HIV/AIDS, compounded by malnutrition and inadequate healthcare infrastructure. •Socio-economic factors, including poverty, maternal education, and limited access to quality healthcare, are consistently identified as key determinants of high child mortality rates in the region. What is new: •This review applies the Mosley and Chen framework to categorise the determinants of under-five mortality into distal, intermediate, and proximal factors, providing a structured understanding of their interconnections. •The findings underscore how socio-economic conditions, maternal education, and healthcare access interact to influence child survival outcomes in Sub-Saharan Africa, offering valuable insights for region-specific public health interventions.
- Severe Neonatal Hyperbilirubinaemia in European and Indian Subcontinent Descendent Newborns: a Retrospective Cohort StudyPublication . Simões, João Ferreira; Simão, Margarida; Rocha, Paula; Ferreira, Sara; da Câmara, Rosário Perry; Amaral, Diana; Costa, Beatriz; Coelho, MárioNeonatal hyperbilirubinaemia is more common in Asian-descendent populations, but differences in disease severity are poorly reported. Our study aimed to compare neonatal hyperbilirubinaemia severity between European and Indian subcontinent descendent newborns. We conducted a single-centre retrospective cohort study including newborns admitted with unconjugated hyperbilirubinaemia (January 2016 to December 2021). Patients were followed during admission, comparing those with European ancestry (control group) and Indian subcontinent ancestry (India, Pakistan, Bangladesh and Nepal) (study group). The primary outcome was severe hyperbilirubinemia (TSB > 25 mg/dL, phototherapy > 6 h or need for exchange transfusion [ET]), and the secondary was TSB levels. Adjusted analysis for potential confounding factors was performed using binary logistic regression models. Of 110 newborns included, 27 (24.5%) had Indian subcontinent ancestry. Occurrence of TSB > 25 mg/dL was significantly higher in the study group (22.2% vs. 4.8%, p = 0.006), while no differences were noted in exposure to phototherapy > 6 h and ET therapy. Logistic regression models for confounding factors adjustment showed Indian subcontinent ancestry as an independent risk factor for TSB > 25 mg/dL (OR 7.49, CI 95% [1.23-45.50]). The study group revealed also higher absolute values of TSB both at admission (22.0 mg/dL vs. 19.6 mg/dL, p = 0.013) and at discharge (13.6 mg/dL vs. 11.4 mg/dL, p = 0.005). Conclusion: Our findings suggest that newborns with Indian subcontinent ancestry might show a higher risk for the development of severe hyperbilirubinemia compared to European ancestry newborns. Implementing earlier treatment thresholds in this subset of patients may help prevent severe hyperbilirubinemia. What is Known: • Indian subcontinent descendent populations have high incidence of neonatal hyperbilirubinaemia but data regarding its severity are scarce. What is New: • This article shows that, compared to European descendent newborns, Indian subcontinent descendent newborns might be at higher risk for severe hyperbilirubinaemia.
- Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases: Five-year Experience of a Pediatric Tertiary Hospital in Portugal.Publication . Rebelo, Mafalda; Francisco, Telma; Perry da Câmara, Rosário; Pereira, Andreia; Iraneta, Amets; Amorim, Marta; Paiva Lopes, Maria João; Lopes da Silva, Rita; Cordeiro, Ana IsabelIntroduction: Neurocutaneous syndromes (NCS) are a heterogeneous group of conditions with multiorgan involvement and diverse manifestations, evolving throughout life with significant morbidity. A multidisciplinary approach to NCS patients has been advocated, although a specific model is not yet established. The aim of this study was 1) to describe the organization of the recently created Multidisciplinary Outpatient Clinic of Neurocutaneous Diseases (MOCND) at a Portuguese pediatric tertiary hospital; 2) to share our institutional experience focusing on the most common conditions, neurofibromatosis type 1 (NF1) and tuberous sclerosis complex (TSC); 3) to analyze the advantages of a multidisciplinary center and approach in NCS. Methods: Retrospective analysis of 281 patients enrolled in the MOCND over the first five years of activity (October 2016 to December 2021), reviewing genetics, family history, clinical features, complications, and therapeutic strategies for NF1 and TSC. Results: The clinic works weekly with a core team of pediatricians and pediatric neurologists supported by other specialties as needed. Of the 281 patients enrolled, 224 (79.7%) had identifiable syndromes such as NF1 (n = 105), TSC (n = 35), hypomelanosis of Ito (n = 11), Sturge-Weber syndrome (n = 5), and others. In NF1 patients, 41.0% had a positive family history, all manifested café-au-lait macules, 38.1% neurofibromas with 45.0% being large plexiform neurofibromas. Sixteen were under treatment with selumetinib. Genetic testing was performed in 82.9% of TSC patients with pathogenic variants found in TSC2 gene in 72.4% patients (82.7% if considered contiguous gene syndrome). Family history was positive in 31.4%. All TSC patients presented hypomelanotic macules and fulfilled diagnostic criteria. Fourteen patients were being treated with mTOR inhibitors. Conclusion: Offering a systematic and multidisciplinary approach to NCS patients enables timely diagnosis, promotes a structured follow-up, and encourages discussion to outline management plans for optimal care to every patient, with significant impact on the quality of life of patients and families.
- Health-Illness Transition Processes in Children with Complex Chronic Conditions and their Parents: a Scoping Review.Publication . Loura, David; Ferreira, Ana Margarida; Romeiro, Joana; Charepe, ZaidaBackground: The prevalence of complex chronic conditions (CCC), which cause serious limitations and require specialized care, is increasing. The diagnosis of a CCC is a health-illness transition for children and their parents, representing a long-term change leading to greater vulnerability. Knowing the characteristics of these transitional processes is important for promoting safe transitions in this population. This scoping review aimed to map the available evidence on health-illness transition processes in children with complex chronic conditions and their parents in the context of healthcare. Methods: Six databases were searched for studies focusing on children aged 0-21 years with CCC and their parents experiencing health-illness transition processes, particularly concerning adaptation to illness and continuity of care, in the context of healthcare. Studies within this scope carried out between 2013 and 2023 and written in Portuguese or English were identified. The articles were selected using the PRISMA methodology. The data were extracted to an instrument and then presented with a synthesizing approach supporting the interpretation of the results. Results: Ninety-eight methodologically broad but predominantly qualitative articles were included in this review. Children with CCC have specific needs associated with complex and dynamic health-illness transitions with a multiple influence in their daily lives. Several facilitating factors (p.e. positive communication and a supportive therapeutic relationship with parents and professionals, as well as involvement in a collaborative approach to care), inhibiting factors (p.e. the complexity of the disease and therapeutic regime, as well as the inefficient organization and coordination of teams) and both positive (p.e. well-being and better quality of life) and negative response patterns (p.e. negative feelings about the chronic illness) were identified. Some interventions to support the transitional process also emerged from the literature. Pediatric palliative care is seen as a good practice and an integrative approach for these children and families. Conclusion: Health professionals play a fundamental role in supporting the transitional process and promoting positive response patterns. More significant investment is needed at the clinical and academic levels regarding production and dissemination of knowledge in this area to ensure the awareness of children with CCC and that their needs are fully enhanced.
- An Insight into the Burden of Drug-Resistant Tuberculosis in ChildrenPublication . Dias, Joana Valente; Varandas, Luís; Gonçalves, Luzia; Kagina, Benjamin M