MAGDA MARIA SALES CARNEIRO SAMPAIO

(Fonte: Lattes)
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Departamento de Pediatria, Faculdade de Medicina - Docente
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder

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  • conferenceObject
    IPEX syndrome with Dent's disease manifestations - Case Report
    (2013) KOSTIC, Dusan; BRASIL, Saulo Couto; JACOB, Cristina Miuki Abe; SAMPAIO, Magda Carneiro; KOCH, Vera Hermina Kalika
    Objective: IPEX syndrome, a hereditary (X-linked) immune dysregulation with autoimmune polyendocrinopathy and enteropathy, as the basic manifestations, presents a rare and severe disease. The objective of this case report is to highlight the pleomorphism of the syndrome. Methods: The authors report the case of a male infant, with a family history of three male siblings affected by IPEX syndrome. The patients’ medical records were reviewed in order to describe the case of the youngest one. Results: During the follow-up of the youngest of three siblings, who presented eczema and intestinal manifestation, without compromised pancreatic and thyroid function, different from other two siblings, it was noticed the pattern of Dent’s disease. We registered hypophosphatemia, hypercalciuria, glycosuria, low molecular weight proteinuria and ultrasound revealed second stage bilateral nephrocalcinosis. In this child there was no apparent glomerular involvement, as it was seen in the eldest sibling. Conclusion: Dent’s disease is an X-linked renal proximal tubulopathy associated with mutations in the chloride channel gene CLCN5 (Xp11.22), which is next to FOXP3 gene on the X chromosome (Xp11.23-q13.3). It seems that in this sibling mutations occurred inexons of both of these genes. This case is to remind on pleomorphic potential of mutations that occur near the coding regions of the FOXP3 gene.
  • article 6 Citação(ões) na Scopus
    Latent and Overt Polyautoimmunity in Children and Adolescents With Immune Thrombocytopenia
    (2020) KAMIOKA, Priscila E.; LIPHAUS, Bernadete L.; BEATRICE, Julia M.; MATSUMOTO, Lucy C.; REIS, Joyce M. A.; LIMA, Laila; CARNEIRO-SAMPAIO, Magda M. S.; CARNEIRO, Jorge D. A.
    Autoantibodies are biomarkers for autoimmune disease diagnosis, monitoring, and prediction. Therefore, this study established the frequency of latent and overt polyautoimmunity in children and adolescents with >6 months of diagnosis of immune thrombocytopenia (ITP). Forty-seven patients with chronic or persistent disease had non-organ-specific and organ-specific autoantibodies assessed. Frequency of latent polyautoimmunity was 36.2%, and, of overt polyautoimmunity, it was 4.3%. Of ITP patients with latent polyautoimmunity, 52.9% were positive for antinuclear antibody (ANA), 47.1% for autoantibodies other than ANA, and 64.7% for multiple autoantibodies. In addition, patients with latent polyautoimmunity and those positive for ANA were significantly older at disease onset. Both ITP patients positive and negative for autoantibodies reported family members with autoimmune diseases. The autoantibodies observed were as follows: ANA, anti-dsDNA, anti-SSA/Ro, IgM aCL, anti-GAD, anti-IA2, anti-IAA, anti-TG, anti-TPO, anti-LKM1, and SMA. Of ITP patients with overt polyautoimmunity, 1 was diagnosed with type 1 diabetes mellitus and the other with thyroiditis. In conclusion, children and adolescents with ITP present high frequency of latent and overt polyautoimmunity even for autoantibodies other than ANA. Therefore, ANA and other non-organ-specific and organ-specific autoantibodies should be considered for assessment during ITP patients' follow-up.
  • article 2 Citação(ões) na Scopus
    PEDIATRICIANS AFTER RESIDENCY: A SURVEY OF PERSONAL/PROFESSIONAL DATA AND ISSUES
    (2021) SILVA, Clovis Artur; TRINDADE, Vitor Cavalcanti; ABEL, Roberta Capretz D’Oliveira; SILVA, Marcelo Oliveira; SANTOS, João Fernando Vecchi; KOCH, Vera Hermina Kalika; FERRER, Ana Paula Scoleze; BRENTANI, Alexandra; ODONE-FILHO, Vicente; TANNURI, Uenis; CARVALHO, Werther Brunow; CARNEIRO-SAMPAIO, Magda; GRISI, Sandra Josefina Ferraz Ellero
    ABSTRACT Objective: To assess personal, professional, medical, and scientific educational characteristics and issues reported by pediatricians. Methods: Cross-sectional study based on an online survey including 614 pediatricians who graduated in the last 15 years at a University Pediatric Department in Brazil. Results: The response rate was 331/614(54%). The majority were females (82%), the median age was 33 years (27-40) and median years of pediatric practice was 5 (1-13). High workload (>60 hours/week) occurred in 25% and 47% earned ≥15 minimum wages/month. The most work-related issues reported were long working hours, poor social life and a sedentary lifestyle (>50%). Pediatricians were further divided into two groups, according to years of pediatric clinical practice: group 1 (≤5 years) and group 2 (>5 years). The median of overall satisfaction with pediatric residency [8(0-10) vs. 9 (4-10); p=0.002] was significantly reduced in group 1. The frequencies of workload >60 hours, work on pediatric ward and pediatric intensive care were significantly higher in the first group (p<0.05). Regarding main issues related to clinical practice in the last year, long working hours (73 vs. 53%; p<0.001), poor social life (75 vs. 62%; p=0.018) and harassment (23 vs. 4%; p=0.003) were significantly higher in the first group. Conclusions: Very early career pediatricians (≤5 years) reported higher workload, lower income, work-related issues and different location of pediatric practice compared to early career pediatricians (>5 years). The overall satisfaction with pediatric residency was good, however, reduced in very early career pediatricians.
  • article 28 Citação(ões) na Scopus
    A proposal of warning signs for primary immunodeficiencies in the first year of life
    (2011) CARNEIRO-SAMPAIO, Magda; JACOB, Cristina Miuki Abe; LEONE, Clea Rodrigues
  • article 18 Citação(ões) na Scopus
    Differences among Severe Cases of Sars-CoV-2, Influenza, and Other Respiratory Viral Infections in Pediatric Patients: Symptoms, Outcomes and Preexisting Comorbidities
    (2020) SOUSA, Braian L. A.; SAMPAIO-CARNEIRO, Magda; CARVALHO, Werther B. de; SILVA, Clovis A.; FERRARO, Alexandre A.
    OBJECTIVES: Previous studies focusing on pediatric patients hospitalized with severe coronavirus disease 2019 (COVID-19) have been limited to small case series. We aimed to evaluate the characteristics of a large population of pediatric patients with severe COVID-19 and compare them with patients with severe cases of influenza and other respiratory viruses (ORV). METHODS: We performed a cross-sectional study of Brazilian data from the National Epidemiological Surveillance Information System, gathered from January 1st to July 14th, 2020. The sample included 4,784 patients (2,570 with confirmed COVID-19, 659 with influenza, 1,555 with ORV). Outcome measures included clinical features, preexisting comorbidities, pediatric intensive care unit admissions, need for ventilatory support, and death. RESULTS: Compared with the influenza and ORV groups, the COVID-19 group had a higher proportion of newborns and adolescents, as well as lower frequencies of fever, cough, dyspnea, respiratory distress, and desaturation. Although use of invasive ventilatory support was similar among groups, death rate was highest for COVID-19 (15.2% vs. 4.5% vs. 3.2%, p <0.001), with death risk more than three times the other groups (adjusted OR=3.7 [95% CI 2.5-5.6]). The presence of two or more comorbidities further increased this risk (OR=4.8 [95% CI 3.5-6.6]). Preexisting comorbidities were reported in 986 patients with severe COVID-19 (38%). Mortality rate among COVID-19 patients was significantly higher for almost all comorbidities reported. CONCLUSION: Severe COVID-19 had a higher mortality rate than other viral respiratory illnesses, despite the lower frequency of fever, cough, dyspnea, respiratory distress, and desaturation. Death risk was strongly associated with preexisting comorbidities.
  • article 49 Citação(ões) na Scopus
    An Update on the Management of Childhood-Onset Systemic Lupus Erythematosus
    (2021) TRINDADE, Vitor Cavalcanti; CARNEIRO-SAMPAIO, Magda; BONFA, Eloisa; SILVA, Clovis Artur
    Childhood-onset systemic lupus erythematosus (cSLE) is a prototype of a multisystemic, inflammatory, heterogeneous autoimmune condition. This disease is characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality. Racial/ethnic background, socioeconomic status, cost of medications, difficulty accessing health care, and poor adherence seem to impact lupus outcomes and treatment response. In this article, the management of cSLE patients is updated. Regarding pathogenesis, a number of potential targets for drugs have been studied. However, most treatments in pediatric patients are off-label drugs with recommendations based on inadequately powered studies, therapeutic consensus guidelines, or case series. Management practices for cSLE patients include evaluations of disease activity and cumulative damage scores, routine non-live vaccinations, physical activity, and addressing mental health issues. Antimalarials and glucocorticoids are still the most common drugs used to treat cSLE, and hydroxychloroquine is recommended for nearly all cSLE patients. Disease-modifying antirheumatic drugs (DMARDs) should be standardized for each patient, based on disease flare and cSLE severity. Mycophenolate mofetil or intravenous cyclophosphamide is suggested as induction therapy for lupus nephritis classes III and IV. Calcineurin inhibitors (cyclosporine, tacrolimus, voclosporin) appear to be another good option for cSLE patients with lupus nephritis. Regarding B-cell-targeting biologic agents, rituximab may be used for refractory lupus nephritis patients in combination with another DMARD, and belimumab was recently approved by the US Food and Drug Administration for cSLE treatment in children aged > 5 years. New therapies targeting CD20, such as atacicept and telitacicept, seem to be promising drugs for SLE patients. Anti-interferon therapies (sifalimumab and anifrolumab) have shown beneficial results in phase II randomized control trials in adult SLE patients, as have some Janus kinase inhibitors, and these could be alternative treatments for pediatric patients with severe interferon-mediated inflammatory disease in the future. In addition, strict control of proteinuria and blood pressure is required in cSLE, especially with angiotensin-converting enzyme inhibitor and angiotensin receptor blocker use.
  • article 187 Citação(ões) na Scopus
    SARS-CoV-2 in cardiac tissue of a child with COVID-19-related multisystem inflammatory syndrome
    (2020) DOLHNIKOFF, Marisa; FERRANTI, Juliana Ferreira; MONTEIRO, Renata Aparecida de Almeida; DUARTE-NETO, Amaro Nunes; GOMES-GOUVEA, Michele Soares; DEGASPARE, Natalia Viu; DELGADO, Artur Figueiredo; FIORITA, Carolina Montanari; LEAL, Gabriela Nunes; RODRIGUES, Regina Maria; CHAIM, Khallil Taverna; PINHO, Joao Renato Rebello; CARNEIRO-SAMPAIO, Magda; MAUAD, Thais; SILVA, Luiz Fernando Ferraz da; CARVALHO, Werther Brunow de; SALDIVA, Paulo Hilario Nascimento; CALDINI, Elia Garcia
  • article 1 Citação(ões) na Scopus
    Let's reduce the blood volume collected for laboratorial exams?
    (2014) CARNEIRO-SAMPAIO, Magda; SLHESSARENKO, Natasha
  • article 1 Citação(ões) na Scopus
    Expanding the Phenotype of 8p23.1 Deletion Syndrome: Eight New Cases Resembling the Clinical Spectrum of 22q11.2 Microdeletion
    (2023) MONTENEGRO, Marilia Moreira; CAMILOTTI, Debora; QUAIO, Caio Robledo D'Anglioli Costa; GASPARINI, Yanca; ZANARDO, Evelin Aline; RANGEL-SANTOS, Andreia; NOVO-FILHO, Gil Monteiro; FRANCISCO, Gleyson; LIRO, Lucas; NASCIMENTO, Amom; CHEHIMI, Samar Nasser; SOARES, Diogo Cordeiro Queiroz; KREPISCHI, Ana C. V.; GRASSI, Marcilia Sierro; HONJO, Rachel Sayuri; PALMEIRA, Patricia; KIM, Chong Ae; CARNEIRO-SAMPAIO, Magda Maria Sales; ROSENBERG, Carla; KULIKOWSKI, Leslie Domenici
    Objective To report the effectiveness of early molecular diagnosis in the clinical management of rare diseases, presenting 8 patients with 8p23.1DS who have clinical features that overlap the phenotypic spectrum of 22q11.2DS. Study design This report is part of a previous study that aims to provide a precocious molecular diagnosis of the 22q11.2 deletion syndrome in 118 infants with congenital heart disease. To confirm the clinical diagnosis, patients underwent comparative genomic screening by the multiplex ligation-dependent probe amplification (MLPA) assay with the SALSA MLPA probemix kits P064-B2, P036-E1, P070-B2, P356-A1, and P250-B1. Subsequently, the patients performed the genomic microarray using the Infinium CytoSNP-850K BeadChip to confirm the deletion, determine the breakpoints of the deletion, and search for genomic copy number variations. Results MLPA performed with 3 different kits revealed the 8p23.1 typical deletion involving the PPP1R3B, MSRA, and GATA4 genes in the 5 patients. The array analysis was performed on these 5 patients and 3 other patients (8 patients) who also had clinical suspicion of 22q11 deletion (8 patients) allowed a precise definition of the breakpoints and excluded other genomic abnormalities. Conclusions Cytogenomic screening was efficient in establishing a differential diagnosis and ruling out the presence of other concomitant syndromes. The clinical picture of the 8p23.1 deletion syndrome is challenging; however, cytogenomic tools can provide an exact diagnosis and help to clarify the genotype-phenotype complexity of these patients. Our reports underline the importance of early diagnosis and clinical follow-up of microdeletion syndromes.
  • article 16 Citação(ões) na Scopus
    COMPLEXITY OF PEDIATRIC CHRONIC DISEASE: CROSS-SECTIONAL STUDY WITH 16,237 PATIENTS FOLLOWED BY MULTIPLE MEDICAL SPECIALTIES
    (2020) PASSONE, Caroline Gouveia Buff; GRISI, Sandra Josefina; FARHAT, Sylvia Costa; MANNA, Thais Della; PASTORINO, Antonio Carlos; ALVENO, Renata Antunes; MIRANDA, Caroline Vasconcelos Sá; WAETGE, Aurora Rosaria; CORDON, Mariana Nutti; ODONE-FILHO, Vicente; TANNURI, Uenis; CARVALHO, Werther Brunow; CARNEIRO-SAMPAIO, Magda; SILVA, Clovis Artur
    ABSTRACT Objective: To assess demographic data and characteristics of children and adolescents with pediatric chronic diseases (PCD), according to the number of specialties/patient. Methods: We performed a cross-sectional study with 16,237 PCD patients at outpatient clinics in one year. Data were analyzed by an electronic data system, according to the number of physician appointments for PCD. This study assessed: demographic data, follow-up characteristics, types of medical specialty, diagnosis (International Statistical Classification of Diseases and Related Health Problems - ICD-10), number of day hospital clinic visits, and acute complications. Results: Patients followed by ≥3 specialties simultaneously showed a significantly higher duration of follow-up compared to those followed by ≤2 specialties [2.1 (0.4-16.4) vs. 1.4 (0.1-16.2) years; p<0.001] and a higher number of appointments in all specialties. The most prevalent medical areas in patients followed by ≥3 specialties were: Psychiatry (Odds Ratio - OR=8.0; confidence interval of 95% - 95%CI 6-10.7; p<0.001), Palliative/Pain Care (OR=7.4; 95%CI 5.7-9.7; p<0.001), Infectious Disease (OR=7.0; 95%CI 6.4-7.8; p<0.001) and Nutrology (OR=6.9; 95%CI 5.6-8.4; p<0.001). Logistic regressions demonstrated that PCD patients followed by ≥3 specialties were associated with high risk for: number of appointments/patient (OR=9.2; 95%CI 8.0-10.5; p<0.001), day hospital clinic visits (OR=4.8; 95%CI 3.8-5.9; p<0.001), emergency department visits (OR=3.2; 95%CI 2.9-3.5; p<0.001), hospitalizations (OR=3.0; 95%CI 2.7-3.3; p<0.001), intensive care admissions (OR=2.5; 95%CI 2.1-3.0; p<0.001), and deaths (OR=2.8; 95%CI 1.9-4.0; p<0.001). The diagnosis of asthma, obesity, chronic pain, and transplant was significantly higher in patients followed by ≥3 specialties. Conclusions: The present study showed that PCD patients who required simultaneous care from multiple medical specialties had complex and severe diseases, with specific diagnoses.