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Abstract Objective: To assess factors associated with emotional changes and Hyperactivity/Inattention (HI) motivated by COVID-19 quarantine in adolescents with immunocompromising diseases. Methods: A cross-sectional study included 343 adolescents with immunocompromising diseases and 108 healthy adolescents. Online questionnaires were answered including socio-demographic data and self-rated healthcare routine during COVID-19 quarantine and validated surveys: Strengths and Difficulties Questionnaire (SDQ), Pittsburgh Sleep Quality Index (PSQI), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0). Results: The frequencies of abnormal emotional SDQ scores from adolescents with chronic diseases were similar to those of healthy subjects (110/343 [32%] vs. 38/108 [35%], p = 0.548), as well as abnormal hyperactivity/inattention SDQ scores (79/343 [23%] vs. 29/108 [27%], p = 0.417). Logistic regression analysis of independent variables associated with abnormal emotional scores from adolescents with chronic diseases showed: female sex (Odds Ratio [OR = 3.76]; 95% Confidence Interval (95% CI) 2.00–7.05; p < 0.001), poor sleep quality (OR = 2.05; 95% CI 1.08–3.88; p = 0.028) and intrafamilial violence during pandemic (OR = 2.17; 95% CI 1.12–4.19; p = 0.021) as independently associated with abnormal emotional scores, whereas total PedsQL score was inversely associated with abnormal emotional scores (OR = 0.95; 95% CI 0.93–0.96; p < 0.0001). Logistic regression analysis associated with abnormal HI scores from patients evidenced that total PedsQL score (OR = 0.97; 95% CI 0.95–0.99; p = 0.010], changes in medical appointments during the pandemic (OR = 0.39; 95% CI 0.19–0.79; p = 0.021), and reliable COVID-19 information (OR = 0.35; 95% CI 0.16–0.77; p = 0.026) remained inversely associated with abnormal HI scores. Conclusion: The present study showed emotional and HI disturbances in adolescents with chronic immunosuppressive diseases during the COVID-19 pandemic. It reinforces the need to promptly implement a longitudinal program to protect the mental health of adolescents with and without chronic illnesses during future pandemics.

https://doi.org/10.1016/j.clinsp.2023.100167

Ceramidas Plasmáticas na Estratificação de Risco das Doenças Cardiovasculares
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Junqueira, Débora L. M.

; Stach, Alline

; Caixeta, Adriano

; Sallum, Juliana

; Yasaki, Erika

; Tsutsui, Jeane

; Rizatti, Edgar

; Rochitte, Carlos E.

; Ching-Jianhong,

; Kovalik, Jean-Paul

; Krieger, José E.

; Richards, A. Mark

; Chan, Mark Y.

; Carvalho, Leonardo P. de

Arquivos Brasileiros de Cardiologia

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Abr 2022, Volume 118 Nº 4 Páginas 768 - 777

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Resumo A produção de ceramida ocorre em todo o corpo e desempenha um papel importante na manutenção da fisiologia normal. No entanto, os níveis de ceramidas são alterados em estados de doença, principalmente durante o desenvolvimento de diabetes e dislipidemia. A produção de ceramidas também está associada à instabilidade das placas ateroscleróticas. Estudos recentes revelam que pacientes com doença arterial coronariana instável apresentam níveis plasmáticos aumentados de ceramidas (principalmente C16, C18 e C24:1). Atualmente, são consideradas biomarcadores emergentes nas doenças cardiovasculares, sendo utilizadas na predição de instabilidade da placa aterosclerótica e eventos cardiovasculares adversos de forma independente aos fatores de risco tradicionais. Com o objetivo de descrever e discutir o papel das ceramidas na estratificação das doenças cardiovasculares, o desenvolvimento desta revisão narrativa contextualiza a importância desse biomarcador no cenário atual da cardiologia.

Abstract Ceramide production takes place throughout the body and plays a key role in the maintenance of normal physiology. However, ceramide levels are altered during disease states, particularly considering the development of diabetes and dyslipidemia. Ceramide production is also associated with atherosclerotic plaque instability. Recent studies revealed that patients with unstable coronary artery disease (CAD) presented increased plasma ceramide levels (especially C16, C18, and C24:1). These molecules are currently considered emerging biomarkers of cardiovascular diseases (CVD), being used for predicting atherosclerotic plaque instability and adverse cardiovascular events independently from traditional risk factors. With the aim of describing and discussing the role of ceramides in the stratification of cardiovascular diseases, this narrative review contextualizes the importance of this biomarker in the present cardiology scenario.

https://doi.org/10.36660/abc.20201165

Poor Sleep quality and health-related quality of life impact in adolescents with and without chronic immunosuppressive conditions during COVID-19 quarantine
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Helito, Alberto C.

; Lindoso, Livia

; Sieczkowska, Sofia M.

; Astley, Camilla

; Queiroz, Ligia B.

; Rose, Natalia

; Santos, Claudia Renata P.

; Bolzan, Thalis

; Peralta, Rita María I.A.

; Franco, Ruth R.

; Cominato, Louise

; Pereira, Rosa Maria R.

; Tannuri, Uenis

; Campos, Lucia Maria A.

; Lourenço, Benito

; Toma, Ricardo K.

; Medeiros, Karina

; Watanabe, Andréia

; Grangeiro, Patricia Moreno

; Farhat, Sylvia C.

; Casella, Caio B.

; Polanczyk, Guilherme V.

; Gualano, Bruno

; Silva, Clovis A.

; Sallum, Adriana M. E.

; Iraha, Amanda Y.

; Ihara, Bianca P.

; Mazzolani, Bruna C.

; Martinez, Claudia A.

; Strabelli, Claudia A. A.

; Fonseca, Claudia B.

; Lima, Dandara C. C.

; Setoue, Debora N. D.

; Roz, Deborah F. P.

; Smaira, Fabiana I.

; Roschel, Hamilton

; Miyatani, Helena T.

; Marques, Isabela G.

; Oba, Jane

; Ferreira, Juliana C. O.

; Simon, Juliana R.

; Kozu, Katia

; Saccani, Ligia P.

; Martiniano, Lorena V. M.

; Miranda, Luana C. A.

; Silva, Luiz E. V.

; Laurentino, Moisés F.

; Aikawa, Nadia E.

; Sakita, Neusa K.

; Tanigava, Nicolas Y.

; Pereira, Paulo R. A.

; Palmeira, Patrícia

; Angelo, Simone S.

; Lavorato, Sofia S. M.

; Bernardes, Tamires M.

; Franco, Tathiane C.

; Viana, Vivianne S. L.

; Barros, Vera P. M. F. R.

; Zheng, Yingying

Clinics

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2021, Volume 76 elocation e3501

Resumo: En | Texto: En | PDF: En

OBJECTIVE: To assess the possible factors that influence sleep quality in adolescents with and without chronic immunosuppressive conditions quarantined during the coronavirus disease 2019 (COVID-19) pandemic. METHODS: This cross-sectional study included 305 adolescents with chronic immunocompromised conditions and 82 healthy adolescents. Online surveys were completed, which included questions on socio-demographic data and self-rated healthcare routine during COVID-19 quarantine and the following validated questionnaires: the Pittsburgh Sleep Quality Index (PSQI), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), and Pediatric Outcome Data Collection Instrument (PODCI). RESULTS: The median current age [14 (10-18) vs. 15 (10-18) years, p=0.847] and frequency of female sex (62% vs. 58%, p=0.571) were similar in adolescents with chronic conditions compared with healthy adolescents. The frequency of poor sleep quality was similar in both groups (38% vs. 48%, p=0.118). Logistic regression analysis, including both healthy adolescents and adolescents with chronic conditions (n=387), demonstrated that self-reported increase in screen time (odds ratio [OR] 3.0; 95% confidence interval [CI] 1.3-6.8; p=0.008) and intrafamilial violence report (OR 2.1; 95% CI 1.2-3.5; p=0.008) were independently associated with poor sleep quality in these adolescents. However, the PODCI global function score was associated with a lower OR for poor sleep quality (OR 0.97; 95% CI 0.94-0.99; p=0.001). Further logistic regression, including only adolescents with chronic conditions (n=305), demonstrated that self-reported increase in screen time (OR 3.1; 95% CI 1.4-6.8; p=0.006) and intrafamilial violence report (OR 2.0; 95% CI 1.2-3.4; p=0.011) remained independently associated with poor quality of sleep, whereas a lower PODCI global function score was associated with a lower OR for sleep quality (OR 0.96; 95% CI 0.94-0.98; p<0.001). CONCLUSION: Self-reported increases in screen time and intrafamilial violence report impacted sleep quality in both healthy adolescents and those with chronic conditions. Decreased health-related quality of life was observed in adolescents with poor sleep quality.

https://doi.org/10.6061/clinics/2021/e3501

Persistent symptoms and decreased health-related quality of life after symptomatic pediatric COVID-19: A prospective study in a Latin American tertiary hospital
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Fink, Thais T.

; Marques, Heloisa H.S.

; Gualano, Bruno

; Lindoso, Livia

; Bain, Vera

; Astley, Camilla

; Martins, Fernanda

; Matheus, Denise

; Matsuo, Olivia M.

; Suguita, Priscila

; Trindade, Vitor

; Paula, Camila S.Y.

; Farhat, Sylvia C.L.

; Palmeira, Patricia

; Leal, Gabriela N.

; Suzuki, Lisa

; Odone Filho, Vicente

; Carneiro-Sampaio, Magda

; Duarte, Alberto José S.

; Antonangelo, Leila

; Batisttella, Linamara R.

; Polanczyk, Guilherme V.

; Pereira, Rosa Maria R.

; Carvalho, Carlos Roberto R.

; Buchpiguel, Carlos A.

; Latronico, Ana Claudia

; Seelaender, Marilia

; Silva, Clovis Artur

; Pereira, Maria Fernanda B.

; Sallum, Adriana M. E.

; Brentani, Alexandra V. M.

; Neto, Álvaro José S.

; Ihara, Amanda

; Santos, Andrea R.

; Canton, Ana Pinheiro M.

; Watanabe, Andreia

; Santos, Angélica C. dos

; Pastorino, Antonio C.

; Franco, Bernadette D. G. M.

; Caruzo, Bruna

; Ceneviva, Carina

; Martins, Carolina C. M. F.

; Prado, Danilo

; Abellan, Deipara M.

; Benatti, Fabiana B.

; Smaria, Fabiana

; Gonçalves, Fernanda T.

; Penteado, Fernando D.

; Castro, Gabriela S. F. de

; Gonçalves, Guilherme S.

; Roschel, Hamilton

; Disi, Ilana R.

; Marques, Isabela G.

; Castro, Inar A.

; Buscatti, Izabel M.

; Faiad, Jaline Z.

; Fiamoncini, Jarlei

; Rodrigues, Joaquim C.

; Carneiro, Jorge D. A.

; Paz, Jose A.

; Ferreira, Juliana C.

; Ferreira, Juliana C. O.

; Silva, Katia R.

; Bastos, Karina L. M.

; Kozu, Katia

; Cristofani, Lilian M.

; Souza, Lucas V. B.

; Campos, Lucia M. A.

; Silva Filho, Luiz Vicente R. F.

; Sapienza, Marcelo T.

; Lima, Marcos S.

; Garanito, Marlene P.

; Santos, Márcia F. A.

; Dorna, Mayra B.

; Aikawa, Nadia E.

; Litvinov, Nadia

; Sakita, Neusa K.

; Gaiolla, Paula V. V.

; Pasqualucci, Paula

; Toma, Ricardo K.

; Correa-Silva, Simone

; Sieczkowska, Sofia M.

; Imamura, Marta

; Forsait, Silvana

; Santos, Vera A.

; Zheng, Yingying

Clinics

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2021, Volume 76 elocation e3511

Resumo: En | Texto: En | PDF: En

OBJECTIVES: To prospectively evaluate demographic, anthropometric and health-related quality of life (HRQoL) in pediatric patients with laboratory-confirmed coronavirus disease 2019 (COVID-19) METHODS: This was a longitudinal observational study of surviving pediatric post-COVID-19 patients (n=53) and pediatric subjects without laboratory-confirmed COVID-19 included as controls (n=52) was performed. RESULTS: The median duration between COVID-19 diagnosis (n=53) and follow-up was 4.4 months (0.8-10.7). Twenty-three of 53 (43%) patients reported at least one persistent symptom at the longitudinal follow-up visit and 12/53 (23%) had long COVID-19, with at least one symptom lasting for >12 weeks. The most frequently reported symptoms at the longitudinal follow-up visit were headache (19%), severe recurrent headache (9%), tiredness (9%), dyspnea (8%), and concentration difficulty (4%). At the longitudinal follow-up visit, the frequencies of anemia (11% versus 0%, p=0.030), lymphopenia (42% versus 18%, p=0.020), C-reactive protein level of >30 mg/L (35% versus 0%, p=0.0001), and D-dimer level of >1000 ng/mL (43% versus 6%, p=0.0004) significantly reduced compared with baseline values. Chest X-ray abnormalities (11% versus 2%, p=0.178) and cardiac alterations on echocardiogram (33% versus 22%, p=0.462) were similar at both visits. Comparison of characteristic data between patients with COVID-19 at the longitudinal follow-up visit and controls showed similar age (p=0.962), proportion of male sex (p=0.907), ethnicity (p=0.566), family minimum monthly wage (p=0.664), body mass index (p=0.601), and pediatric pre-existing chronic conditions (p=1.000). The Pediatric Quality of Live Inventory 4.0 scores, median physical score (69 [0-100] versus 81 [34-100], p=0.012), and school score (60 [15-100] versus 70 [15-95], p=0.028) were significantly lower in pediatric patients with COVID-19 at the longitudinal follow-up visit than in controls. CONCLUSIONS: Pediatric patients with COVID-19 showed a longitudinal impact on HRQoL parameters, particularly in physical/school domains, reinforcing the need for a prospective multidisciplinary approach for these patients. These data highlight the importance of closer monitoring of children and adolescents by the clinical team after COVID-19.

https://doi.org/10.6061/clinics/2021/e3511

Lower genital tract infections in young female juvenile idiopathic arthritis patients
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Ferreira, Gabriela R. V.

; Tomioka, Renato B.

; Queiroz, Ligia B.

; Kozu, Katia

; Aikawa, Nadia E.

; Sallum, Adriana M. E.

; Serafini, Paulo

; Tacla, Maricy

; Baracat, Edmund C.

; Pereira, Rosa M. R.

; Bonfá, Eloisa

; Silva, Clovis A.

Advances in Rheumatology

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2019, Volume 59 elocation 50

Resumo: En | Texto: En | PDF: En

Abstract Background: To evaluate human papillomavirus (HPV), Chlamydia trachomatis (CT) and Neisseria gonorrhoeae (NG) infections in juvenile idiopathic arthritis (JIA) patients. Methods: After exclusion, 33 female adolescent and young JIA patients (ILAR criteria) and 28 healthy controls were selected for this study. Demographic data, gynecological, sexual function, cervical cytology and histological abnormalities were evaluated. JIA clinical/laboratorial parameters and treatment were also assessed. HPV-DNA, CT-DNA and NG-DNA testing in cervical specimens were performed by Hybrid Capture 2 assays. Results: The mean current age was similar in JIA patients and controls (23.3 ± 6.24 vs. 26.1 ± 6.03 years, p = 0.09). The frequencies of sexual intercourse (76% vs. 89%, p = 0.201) and abnormal cervical cytology (24% vs. 11%, p = 0.201) were similar in JIA compared to controls. The higher frequency of HPV infection in JIA patients than controls (30% vs. 11%, p = 0.155) did not reach statistical significance. CT (0% vs. 7%, p = 0.207) and NG infections (0% vs. 4%, p = 0.459) were also alike in both groups. Further evaluation of JIA patients with abnormal and normal cervical cytology showed that the former group had a higher frequency of HPV infection (87% vs. 12%, p = 0.0002) with a low frequency of HPV vaccination (0% vs. 8%, p = 1.0). No differences were evidenced between these two JIA groups regarding demographic data, sexual function and clinical/laboratorial parameters. The frequencies of methotrexate (p =0.206) and biological agent use (p =0.238) were similar in both JIA groups. Conclusions: To our knowledge, this was the first study to assess lower genital infections in JIA patients allowing the identification of HPV as main cause of cervical dysplasia. Methotrexate and biological agents do not seem to increase risk of lower genital tract infections in JIA patients.

https://doi.org/10.1186/s42358-019-0092-6 407 downloads

Autoimmune hepatitis in 847 childhoodonset systemic lupus erythematosus population: a multicentric cohort study
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Balbi, Verena A.

; Montenegro, Bárbara

; Pitta, Ana C.

; Schmidt, Ana R.

; Farhat, Sylvia C.

; Coelho, Laila P.

; Ferreira, Juliana C. O.

; Pereira, Rosa M. R.

; Terreri, Maria T.

; Saad-Magalhães, Claudia

; Aikawa, Nadia E.

; Sakamoto, Ana P.

; Kozu, Kátia

; Campos, Lucia M.

; Sallum, Adriana M.

; Ferriani, Virginia P.

; Piotto, Daniela P.

; Bonfá, Eloisa

; Silva, Clovis A.

Advances in Rheumatology

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2018, Volume 58 elocation 43

Resumo: En | Texto: En | PDF: En

Abstract Objective: To evaluate autoimmune hepatitis (AIH) in a multicenter cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This retrospective multicenter study included 847 patients with cSLE, performed in 10 Pediatric Rheumatology services of São Paulo state, Brazil. AIH was defined according to the International Autoimmune Hepatitis Group criteria (IAHGC). The statistical analysis was performed using the Bonferroni's correction (p < 0.0033). Results: AIH in cSLE patients confirmed by biopsy was observed in 7/847 (0.8%) and all were diagnosed during adolescence. The majority occurred before or at cSLE diagnosis [5/7 (71%)]. Antinuclear antibodies were a universal finding, 43% had concomitantly anti-smooth muscle antibodies and all were seronegative for anti-liver kidney microsomal antibodies. All patients with follow-up ≥18 months (4/7) had complete response to therapy according to IAHGC. None had severe hepatic manifestations such as hepatic failure, portal hypertension and cirrhosis at presentation or follow-up. Further comparison of 7 cSLE patients with AIH and 28 without this complication with same disease duration [0 (0–8.5) vs. 0.12 (0–8.5) years, p = 0.06] revealed that the frequency of hepatomegaly was significantly higher in cSLE patients in the former group (71% vs. 11%, p = 0.003) with a similar median SLEDAI-2 K score [6 (0–26) vs. 7 (0–41), p = 0.755]. No differences were evidenced regarding constitutional involvement, splenomegaly, serositis, musculoskeletal, neuropsychiatric and renal involvements, and treatments in cSLE patients with and without AIH (p > 0.0033). Conclusions: Overlap of AIH and cSLE was rarely observed in this large multicenter study and hepatomegaly was the distinctive clinical feature of these patients. AIH occurred during adolescence, mainly at the first years of lupus and it was associated with mild liver manifestations.

https://doi.org/10.1186/s42358-018-0043-7 473 downloads

Characterization of scrotal involvement in children and adolescents with IgA vasculitis
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Buscatti, Izabel M.

; Abrão, Henrique M.

; Kozu, Katia

; Marques, Victor L. S.

; Gomes, Roberta C.

; Sallum, Adriana M. E.

; Silva, Clovis A.

Advances in Rheumatology

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2018, Volume 58 elocation 38

Resumo: En | Texto: En | PDF: En

Abstract Objective: To characterize scrotal involvement in children and adolescents with IgA vasculitis. Methods: A cross-sectional retrospective study included 296 IgA vasculitis (EULAR/PRINTO/PRES criteria) patients, 150/296 (51%) were males and assessed by demographic/clinical/laboratory and treatments. Scrotal involvement was defined by the presence of scrotal edema and/or pain/tenderness in physical examination and/or testicular Doppler ultrasound abnormalities. Results: Scrotal involvement was observed in 28/150 (19%) IgA vasculitis patients. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%). Acute recurrent scrotal involvement was observed in 2/150 (1%) and none had chronic subtype. Further analysis of patients with scrotal involvement at first episode (n = 27) compared to those without this complication (n = 122) revealed that the median age at diagnosis [4.0 (2.0–12) vs. 6 (1.3–13) years, p = 0.249] was similar in both groups. The frequency of elevated serum IgA was significantly lower in IgA vasculitis patients with scrotal involvement versus without this manifestation (18% vs. 57%, p = 0.017), whereas glucocorticoid (93% vs. 49%, p < 0.0001) and ranitidine use (63% vs. 30%, p = 0.003) were significantly higher in the former group. Conclusions: The scrotal involvement occurred in almost one fifth of IgA vasculitis patients and was commonly evidenced as acute subtype at diagnosis. Scrotal signs/symptoms improved after a prompt use of glucocorticoid and was associated with low frequency of elevated IgA serum levels.

https://doi.org/10.1186/s42358-018-0039-3 274 downloads

Juvenile dermatomyositis: is periodontal disease associated with dyslipidemia?
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Kozu, Kátia T.

; Silva, Clovis A.

; Aikawa, Nadia E.

; Pereira, Rosa M. R.

; Sallum, Adriana M.

; Savioli, Cynthia

; Borba, Eduardo

; Campos, Lucia M.

Advances in Rheumatology

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2018, Volume 58 elocation 28

Resumo: En | Texto: En | PDF: En

Abstract Background: Association between periodontal disease and dyslipidemia was recently reported in healthy adults. However, a systematic evaluation of concomitant periodontal diseases and lipid profile was not carried out in juvenile dermatomyositis (JDM). A cross-section study was performed in 25 JDM patients and 25 healthy controls, assessing demographic data, periodontal evaluation, fasting lipoproteins and anti-lipoprotein lipase antibodies. Disease parameters, laboratorial tests and treatment were also evaluated in JDM patients. Results: The mean current age was similar in patients and controls (11.5 ± 3.75 vs. 11.2 ± 2.58 years, p = 0.703). Regarding lipid profile, the median triglycerides [80(31–340) vs. 61(19–182)mg/dL, p = 0.011] and VLDL[16(6–68) vs. 13(4–36)mg/dL,p = 0.020] were significantly higher in JDM patients versus controls. Gingival vasculopathy pattern was significantly higher in the former group (60% vs. 0%, p = 0.0001), as well as the median of gingival bleeding index (GBI) [24.1(4.2–69.4) vs. 11.1(0–66.6)%,p = 0.001] and probing pocket depth (PPD) [1.7(0.6–2.4) vs.1.4(0–2.12) mm,p = 0.006]. Comparison between JDM patients with and without dyslipidemia revealed that the median of dental plaque index (PI) [100(26.7–100) vs. 59(25–100)%,p = 0.022], PPD[1.9(0.6–2.4) vs. 1.4(1.2–1.8)mm,p = 0.024] and clinical attachment level (CAL) [1.31(0.7–1.7) vs. 0.8(0.6–1.7)mm,p = 0.005] were significantly higher in patients with dyslipidemia. Further analysis between JDM patients with and without gingivitis revealed that the median of current age [12.4 (8.3–18.4) vs. 9.2 (5.5–17.5) years, p = 0.034] and disease duration [7.09 ± 3.07 vs. 3.95 ± 2.1 years, p = 0.008] were significantly higher in the former group. Conclusion: Our study showed that gingival inflammation seems to be related to dyslipidemia in JDM patients, suggesting underlying mechanisms for both complications.

https://doi.org/10.1186/s42358-018-0024-x 289 downloads

What are the benefits of two-dimensional speckle tracking echocardiography for diagnosis and treatment follow-up of childhood-onset systemic lupus erythematosus myocarditis?
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Leal, Gabriela Nunes

; Diniz, Maria de Fátima

; Brunelli, Juliana

; Lianza, Alessandro C.

; Sallum, Adriana M. E.

; Silva, Clovis A.

Revista da Associação Médica Brasileira

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Set 2016, Volume 62 Nº 6 Páginas 490 - 493

Resumo: En | Texto: En | PDF: En

https://doi.org/10.1590/1806-9282.62.06.490 1152 downloads

10.

Perfil de especialistas e de serviços em reumatologia pediátrica no estado de São Paulo
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Terreri, Maria Teresa

; Campos, Lúcia M. A.

; Okuda, Eunice M.

; Silva, Clovis A.

; Sacchetti, Silvana B.

; Marini, Roberto

; Ferriani, Virginia P.

; Ventura, Maria Heloiza

; Fernandes, Taciana

; Sato, Juliana O.

; Fernandes, Elizabeth C.

; Len, Claudio

; Barbosa, Cássia

; Lotito, Ana Paola

; Santos, Maria Carolina dos

; Aikawa, Nádia E.

; Facó, Mércia

; Piotto, Daniela

; Bugni, Vanessa

; Kozu, Kátia T.

; Romanelli, Paulo R.

; Sallum, Adriana M. E.

; Febronio, Marília

; Fraga, Melissa

; Magalhães, Cláudia S.

Revista Brasileira de Reumatologia

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Ago 2013, Volume 53 Nº 4 Páginas 346 - 351

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

INTRODUÇÃO: A reumatologia pediátrica (RP) é uma especialidade emergente, com número restrito de especialistas, e ainda não conta com uma casuística brasileira sobre o perfil dos pacientes atendidos e as informações sobre a formação de profissionais capacitados. OBJETIVO: Estudar o perfil dos especialistas e dos serviços em RP e as características dos pacientes com doenças reumáticas nessa faixa etária a fim de estimar a situação atual no estado de São Paulo (ESP). PACIENTES E MÉTODOS: No ano de 2010 o departamento científico de RP da Sociedade de Pediatria de São Paulo encaminhou um questionário respondido por 24/31 especialistas com título de especialização em RP que atuam no ESP e por 8/12 instituições com atendimento nesta especialidade. RESULTADOS: A maioria (91%) dos profissionais exerce suas atividades em instituições públicas. Clínicas privadas (28,6%) e instituições (37,5%) relataram não ter acesso ao exame de capilaroscopia e 50% das clínicas privadas não tem acesso à acupuntura. A média de tempo de prática profissional na especialidade foi de 9,4 anos, sendo 67% deles pós-graduados. Sete (87,5%) instituições públicas atuam na área de ensino, formando novos reumatologistas pediátricos. Cinco (62,5%) delas têm pós-graduação. Dois terços dos especialistas utilizam imunossupressores e agentes biológicos de uso restrito pela Secretaria da Saúde. A doença mais atendida foi artrite idiopática juvenil (29,1%-34,5%), seguida de lúpus eritematoso sistêmico juvenil (LESJ) (11,6%-12,3%) e febre reumática (9,1%-15,9%). Vasculites (púrpura de Henoch Schönlein, Wegener, Takayasu) e síndromes autoinflamatórias foram mais incidentes nas instituições públicas (P = 0,03; P = 0,04; P = 0,002 e P < 0,0001, respectivamente). O LESJ foi a doença com maior mortalidade (68% dos óbitos), principalmente por infecção. CONCLUSÃO: A RP no ESP conta com um número expressivo de especialistas pós-graduados, que atuam especialmente em instituições de ensino, com infraestrutura adequada ao atendimento de pacientes de alta complexidade.

INTRODUCTION: Paediatric rheumatology (PR) is an emerging specialty, practised by a limited number of specialists. Currently, there is neither a record of the profile of rheumatology patients being treated in Brazil nor data on the training of qualified rheumatology professionals in the country. OBJECTIVE: To investigate the profile of PR specialists and services, as well as the characteristics of paediatric patients with rheumatic diseases, for estimating the current state of rheumatology in the state of São Paulo. PATIENTS AND METHODS: In 2010, the scientific department of PR of the Paediatric Society of São Paulo administered a questionnaire that was answered by 24/31 accredited specialists in PR practising in state of São Paulo and by 8/21 institutions that provide PR care. RESULTS: Most (91%) of the surveyed professionals practise in public institutions. Private clinics (28.6%) and public institutions (37.5%) reported not having access to nailfold capillaroscopy, and 50% of the private clinics reported not having access to acupuncture. The average duration of professional practise in PR was 9.4 years, and 67% of the physicians had attended postgraduate programmes. Seven (87.5%) public institutions perform teaching activities, in which new paediatric rheumatologists are trained, and five (62.5%) offer postgraduate programmes. Two-thirds of the surveyed specialists use immunosuppressants and biological agents classified as "restricted use" by the Health Secretariat. The disease most frequently reported was juvenile idiopathic arthritis (29.1-34.5%), followed by juvenile systemic lupus erythematosus (JSLE) (11.6-12.3%) and rheumatic fever (9.1-15.9%). The incidence of vasculitis (including Henoch-Schönlein purpura, Wegener's granulomatosis, and Takayasu's arteritis) and autoinflammatory syndromes was higher in public institutions compared to other institutions (P = 0.03, P = 0.04, P = 0.002, and P < 0.0001, respectively). Patients with JSLE had the highest mortality rate (68% of deaths), mainly due to infection. CONCLUSION: The field of PR in the state of São Paulo has a significant number of specialists with postgraduate degrees who mostly practise at teaching institutions with infrastructures appropriate for the care of high-complexity patients.

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11.

Vasculite mesentérica em paciente com lúpus eritematoso sistêmico juvenil
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Albuquerque-Netto, Adão F.

; Cavalcante, Erica G.

; Sallum, Adriana M. E.

; Aikawa, Nádia E.

; Tannuri, Uenis

; Silva, Clovis Artur Almeida da

Revista Brasileira de Reumatologia

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Abr 2013, Volume 53 Nº 2 Páginas 219 - 222

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

A vasculite mesentérica lúpica (VML) é uma rara causa de dor abdominal aguda. Há poucos relatos de caso demonstrando VML em adultos e, particularmente, em crianças e adolescentes. No entanto, para o nosso conhecimento, a prevalência dessa grave vasculite em uma população pediátrica com lúpus ainda não foi estudada. Portanto, dados de 28 anos consecutivos foram revisados e incluídos 5.508 pacientes em seguimento no Hospital da Faculdade de Medicina da Univesidade de São Paulo (FMUSP). Identificamos 279 (5,1%) casos que preencheram critérios de classificação diagnóstica do American College of Rheumatology para lúpus eritematoso sistêmico (LES) e um (0,4%) desses apresentou VML. Este paciente recebeu diagnóstico de LES aos 11 anos de idade. Aos 13 anos foi hospitalizado com dor abdominal difusa e aguda, náuseas, vômitos biliosos, distensão e rigidez abdominal, com descompressão brusca positiva. O paciente foi prontamente submetido à laparotomia exploradora, identificando isquemia intestinal segmentar, com edema de parede intestinal e aderências. Foi realizada ressecção parcial de intestino delgado, com lise das aderências e pulsoterapia com metilprednisolona. A análise histopatológica identificou arterite de vasos mesentéricos. Após 13 dias, apresentou recorrência de dor abdominal difusa intensa, sendo novamente submetido à laparotomia exploradora, identificando obstrução em intestino delgado por aderências, com gangrena intestinal. Nova ressecção intestinal foi realizada, além de pulsoterapia com metilprednisolona e infusão de imunoglobulina. Portanto, VML é uma rara e grave manifestação abdominal na população com lúpus pediátrico, e pode ser a única manifestação de atividade da doença. Além disso, este estudo reforça a importância do diagnóstico precoce e do tratamento imediato.

Lupus mesenteric vasculitis (LMV) is a rare cause of acute abdominal pain. Few cases of LMV have been reported in adults, children and adolescents. However, to our knowledge, the prevalence of that severe vasculitis in a pediatric population with lupus is yet to be studied. This study reviewed data from 28 consecutive years and included 5,508 patients being followed up at the hospital of the Faculdade de Medicina of the Univesidade de São Paulo (FMUSP). We identifi ed 279 (5.1%) patients meeting the American College of Rheumatology classifi cation criteria for the diagnosis of systemic lupus erythematosus (SLE), one of whom (0.4%) had LMV. That male patient was diagnosed with SLE at the age of 11 years. At the age of 13 years, he was hospitalized with diffuse and acute abdominal pain, nausea, bilious vomiting, abdominal distension, rebound tenderness, and abdominal muscle guarding. The patient underwent laparotomy immediately, and segmentary intestinal ischemia with intestinal wall edema and adhesions were identifi ed. Partial small bowel resection with lysis of the adhesions was performed, as were pulses of intravenous methylprednisolone. The histopathologic analysis evidenced mesenteric arteritis. After 13 days, the diffuse and intense abdominal pain recurred, and the patient underwent a new laparotomy, during which adhesive small bowel obstruction with intestinal gangrene was identifi ed. New intestinal resection was performed, and the patient received pulses of intravenous methylprednisolone and infusion of immunoglobulin. Thus, LMV is a rare and severe abdominal manifestation of the pediatric population with lupus, and can be the only manifestation of disease activity. In addition, this study stresses the importance of the early diagnosis and immediate treatment.

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12.

Fatores de risco associados à calcinose na dermatomiosite juvenil
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Sallum, Adriana M. E.

; Pivato, Francine C. M. M.

; Doria-Filho, Ulysses

; Aikawa, Nádia E.

; Liphaus, Bernadete L.

; Marie, Suely K. N.

; Silva, Clovis A. A.

Jornal de Pediatria

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Fev 2008, Volume 84 Nº 1 Páginas 68 - 74

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

OBJETIVO: Identificar fatores de risco associados à calcinose em crianças e adolescentes com dermatomiosite juvenil. MÉTODOS: Prontuários de 54 pacientes com dermatomiosite juvenil foram estudados. Foram avaliados dados demográficos; características clínicas: grau de força muscular (I a V do Medical Research Council), presença de comprometimentos pulmonar (distúrbio ventilatório restritivo com presença ou ausência do anticorpo anti-Jo-1), gastrointestinal (refluxo gastroesofágico) e cardíaco (pericardite e/ou miocardite); exames laboratoriais: elevação de enzimas musculares (creatinoquinase, aspartato aminotransferase, alanina aminotransferase e desidrogenase lática) e terapias utilizadas: corticoterapia isolada ou associada à cloroquina e/ou imunossupressor. Os pacientes foram divididos em dois grupos de acordo com a presença ou ausência de calcinose e foram avaliados através de análise univariada e multivariada. RESULTADOS: Calcinose foi evidenciada em 23 (43%) pacientes, sendo em seis (26%) antes do diagnóstico e em 17 (74%) após. A análise univariada revelou que comprometimentos cardíaco (p = 0,01) e pulmonar (p = 0,02) e necessidade da utilização de um ou mais imunossupressores (metotrexato, ciclosporina A e/ou pulsoterapia com ciclofosfamida endovenosa) no tratamento da dermatomiosite juvenil (p = 0,03) foram associados com uma maior incidência de calcinose. A análise multivariada mostrou que comprometimento cardíaco (OR = 15,56; IC95% 1,59-152,2) e uso de um ou mais imunossupressores (OR = 4,01; IC95% 1,08-14,87) foram as únicas variáveis independentes associadas à presença de calcinose. CONCLUSÕES: O aparecimento da calcinose foi freqüente na dermatomiosite juvenil, habitualmente na evolução da doença. A calcinose foi associada aos casos mais graves, que apresentaram envolvimento cardíaco e necessitaram da utilização de imunossupressores no seu tratamento.

OBJECTIVE: To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis. METHODS: A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses. RESULTS: Calcinosis was identified in 23 (43%) patients, and in six (26%) patients it had emerged prior to diagnosis while in 17 (74%) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87) were independently associated with the presence of calcinosis. CONCLUSIONS: Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.

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13.

Dermatomiosite juvenil e linfoma de Hodgkin: uma rara associação
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Cavalcanti, André

; Terreri, Maria Teresa R. A.

; Sallum, Adriana M. E.

; Marie, Suely K. N.

; Luisi, Flávio

; Hilário, Maria Odete E.

Revista Brasileira de Reumatologia

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Dez 2007, Volume 47 Nº 6 Páginas 458 - 462

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

A dermatomiosite (DM) em adultos está freqüentemente associada com câncer. Já na faixa etária pediátrica, a dermatomiosite juvenil (DMJ) é predominantemente idiopática, e sua associação com neoplasia é rara e com base apenas em relatos de casos. Embora rara, a presença de neoplasia em pacientes com DMJ deve ser sempre suspeitada quando houver manifestações clínicas atípicas e alterações laboratoriais incomuns. A seguir, descrevemos e discutimos um caso de DMJ e linfoma de Hodgkin em uma adolescente.

Dermatomyositis (DM) in adults is frequently associated with cancer. In contrast, during childhood juvenile dermatomyositis (JDM) is predominantly idiopathic and its association with neoplasia is rare and based only in case reports. Although rare, the presence of neoplasia in JDM patients must always be suspected in face of atypical clinical manifestations and uncommon laboratorial findings. We describe and discuss a case of JDM and Hodgkin disease in an adolescent.

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14.

Periodic dynamic systems for infected hosts and mosquitoes
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Oliva, W. M.

; Sallum, E. M.

Revista de Saúde Pública

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Jun 1996, Volume 30 Nº 3 Páginas 218 - 223

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

Desenvolveu-se um modelo matemático para analisar a dinâmica das populações de indivíduos e mosquitos infectados quando as populações de mosquitos são periódicas no tempo. Pela determinação de um parâmetro lambda (o raio espectral de uma matriz de monodromia) pode-se estabelecer que a infecção termina naturalmente (lambda <= 1) ou se lambda > 1 que a infecção torna-se endêmica. O modelo generaliza, para o caso de coeficientes periódicos, modelos anteriores para malária; como também é uma variação de modelo para a gonorréia. A principal motivação para a consideração do modelo proposto foram os recentes estudos sobre mosquitos numa estação experimental de arroz irrigado, na região Sudeste do Brasil.

A mathematical model for the purpose of analysing the dynamic of the populations of infected hosts anf infected mosquitoes when the populations of mosquitoes are periodic in time is here presented. By the computation of a parameter lambda (the spectral radius of a certain monodromy matrix) one can state that either the infection peters out naturally) (lambda <= 1) or if lambda > 1 the infection becomes endemic. The model generalizes previous models for malaria by considering the case of periodic coefficients; it is also a variation of that for gonorrhea. The main motivation for the consideration of this present model was the recent studies on mosquitoes at an experimental rice irrigation system, in the South-Eastern region of Brazil.

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15.

Periodic dynamic systems for infected hosts and mosquitoes
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Oliva, W. M.

; Sallum, E. M.

Revista de Saúde Pública

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Jun 1996, Volume 30 Nº 3 Páginas 218 - 223

Resumo: Pt En | Texto: Pt En | PDF: Pt | PDF: En

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