ANDRE SILVA FRANCO
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
16 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 16
- Improvement of bone microarchitecture parameters after 12 months of treatment with asfotase alfa in adult patient with hypophosphatasia Case report(2018) FREITAS, Thiago Quadrante; FRANCO, Andre Silva; PEREIRA, Rosa Maria RodriguesRationale: Hypophosphatasia is an inborn error of metabolism that can appear any time in life, mainly with bone manifestations due to low alkaline phosphatase activity. Asfotase alfa is a specific enzyme reposition treatment that has shown promising results in children; however, there are few reports about the outcomes in adult patients. Patient concerns: A 36-year-old male presented with an early history of craniosynostosis, short stature, and multiple fractures since the age of 13 years-which needed numerous surgical corrections. He was admitted with a previous diagnosis of osteogenesis imperfecta, taking alendronate, calcium carbonate, cholecalciferol, and calcitriol. Bone mineral density was low (lumbar spine Z-score = -3.0 SD), with impairment of all parameters of high-resolution peripheral quantitative computed tomography (HR-pQCT). Kidney impairment was also observed with reduced creatinine clearance, nephrolithiasis, and nephrocalcinosis. Diagnosis: Alkaline phosphatase was unexpectedly low (6 U/L, reference value: 30-120U/L), with high serum vitamin B6 (260 mcg/L, reference value: 5.2-34.1). Genetic testing showed a homozygous missense mutation in ALPL gene c.443 C>T: p.Thr148Ile. Intervention: Asfotase alfa was requested due to important bone deterioration, ambulatory disability, and kidney impairment. It was given subcutaneously 2 mg/kg per dose, 3 times a week, for 12 months before reassessment. Outcomes: Bone mineral densities of the lumbar spine and whole body, besides almost all HR-pQCT microstructural parameters of the distal tibia, showed improvements and the patient was able to walk without assistant device. Kidney function did not further deteriorate. Lessons: Hypophosphatasia should be considered as a differential diagnosis in young patients with multiple fractures and kidney impairment, since the use of antiresorptive drugs, calcium and vitamin D, commonly used to treat fractures, worsen its symptoms and prognosis. A 12-month asfotase alfa treatment improved bone density and structural parameters even in an adult patient with late diagnosis.
- Visceral adipose tissue in granulomatosis with polyangiitis: association with disease activity parameters(2021) FURLAM, Pedro L.; PEREZ, Mariana O.; FRANCO, Andre S.; CAPARBO, Valeria F.; SHINJO, Samuel K.; PEREIRA, Rosa M. R.Objective To assess the body composition (BC) of patients with granulomatosis with polyangiitis (GPA) compared to healthy controls, emphasizing visceral adipose tissue (VAT) and associated BC parameters with disease activity, the damage index, and inflammatory parameters in patients with GPA. Methods This study was conducted in 43 patients with GPA and 43 healthy controls matched by sex, age, and body mass index (BMI). BC was analyzed using dual-energy X-ray absorptiometry (DXA). The fat mass parameters evaluated were total fat mass (FM), adiposity (%), the fat mass index (FMI: fat mass/ht(2)), and VAT (g, cm(2), cm(3)). Disease activity was assessed by the Birmingham Vasculitis Activity Score (BVAS). Damage was assessed by the Vasculitis Damage Index (VDI). C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) were measured. Results Comparing patients with GPA with healthy controls, patients had a significantly greater VAT (VAT in g: 685.81 +/- 306.10 vs. 581.21 +/- 235.57, p = 0.04; VAT in cm(2): 142.23 +/- 63.48 vs. 119.84 +/- 49.54, p = 0.03; VAT in cm(3): 741.33 +/- 330.97 vs. 628.44 +/- 254.66, p = 0.04). Patients with higher VAT (>= 768 g) had an increased value of ESR (22.77 +/- 26.79 vs. 11.57 +/- 11.30 mm/1st hour, p = 0.04) and an increased value of BVAS (3.18 +/- 4.15 vs. 0.90 +/- 1.70, p = 0.01) when compared to patients with less VAT (< 768 g). Conclusion Patients with GPA have altered BC compared to healthy controls. Moreover, higher VAT was associated with disease activity and higher inflammatory markers, suggesting a relationship between GPA activity and adiposity parameters.
- Social impact of disease parameters and damage accrual in adult Brazilian patients with childhood-onset Systemic Lupus Erythematosus(2022) TANIGAVA, Nicolas Y.; SAKAMOTO, Ana P.; FRANCO, Andre S.; BALBI, Gabriela G. M.; SALES, Lucas P.; AIKAWA, Nadia E.; TERRERI, Maria T.; PEREIRA, Rosa M. R.Objectives To describe the frequency and investigate potential associations of unemployment, need of financial assistance and health-related quality of life in adult patients with childhood-onset Systemic Lupus Erythematosus (cSLE). Methods In this multicenter cross-sectional retrospective cohort study including cSLE adult patients, questionnaires were applied evaluating demographic characteristics, medical history, treatment, receipt of government financial assistance, work status, quality of life, economic classification, disease activity, and damage accrual. Disease activity and disease damage were measured at the study visit. Results Sixty-nine cSLE patients with a median age of 21 years from two Brazilian tertiary centers were included (median disease duration 9 years). Twenty-eight (40.6%) patients were unemployed and 16 (23.2%) were receiving financial assistance or retirement pension. Work unemployment was associated with higher damage scores (OR 1.83, 95% CI 1.08 to 3.09, p = 0.024), and the need of financial assistance was associated with longer disease duration (OR 1.15, 95% CI 1.00 to 1.31, p = 0.045) and worse economic score (OR 0.87, 95% CI 0.77 to 0.99, p = 0.038). Emotional health and body image perception were the most compromised domains of quality of life but showed no association with disease parameters. Disease activity, on the other hand, was inversely associated with symptoms scores (beta = -1.377, p = 0.014) and scores of adverse effects of medications (beta = -1.286, p = 0.020). Conclusion cSLE is a disease with severe outcomes and high social burden that profoundly impacts patients. Damage accrual is a major contributor to unemployment during adulthood and its prevention must be central in the management of cSLE.
- Critical digital ischaemia in systemic sclerosis exacerbated by multiple myeloma: A case report(2023) FRANCO, Andre Silva; POLHO, Gabriel Berlingieri; ASSAD, Ana Paula Luppino; MIOSSI, Renata; SAMPAIO-BARROS, Percival DegravaIntroduction: The overlapping of systemic sclerosis with hematologic malignancy has been described previously in the literature. This case report presents a patient with systemic sclerosis and multiple myeloma who had severe digital ischaemia that culminated in the amputation of several fingers. Case report: A 65-year-old White female patient was diagnosed with limited systemic sclerosis in 2002, smouldering multiple myeloma IgG/kappa in 2017 and liver cirrhosis in 2018 due to autoimmune hepatitis. In 2021, she was admitted to the emergency room with dry ischaemia of all fingers and toes despite optimized therapy, associated with visual blurring. The diagnostic hypothesis was hyperviscosity syndrome associated with multiple myeloma reactivation. The patient underwent chemotherapy and despite initial laboratory improvement, 19 digits required amputation. Conclusion: Although the association between systemic sclerosis and multiple myeloma is rare, it should be remembered in cases of significant worsening of Raynaud's phenomenon. Causes unrelated to systemic sclerosis should also be considered in the presence of severe exacerbations in patients with other comorbidities.
- Assessment of Bone Microarchitecture in Patients with Systemic Mastocytosis and its Association with Clinical and Biochemical Parameters of the Disease(2023) FRANCO, Andre S.; MURAI, Igor H.; TAKAYAMA, Liliam; CAPARBO, Valeria F.; MARCHI, Luan L.; VELLOSO, Elvira D. R. P.; PEREIRA, Rosa M. R.Patients with systemic mastocytosis (SM) are at high risk of bone deterioration. However, the evaluation of bone microarchitecture in this disease remains unclear. We aimed to assess bone microarchitecture in patients with SM. This was a cross-sectional study of 21 adult patients with SM conducted in a quaternary referral hospital in Sao Paulo, Brazil. A healthy, age-, weight-, and sex-matched cohort of 63 participants was used to provide reference values for bone microarchitecture, assessed by high resolution peripheral quantitative computed tomography (HR-pQCT). Total volumetric bone mineral density (vBMD), cortical vBMD, and cortical thickness at the radius were significantly lower in the control group compared with the SM group (all P < 0.001). Patients with aggressive SM had significantly lower trabecular number (Tb.N) (P = 0.035) and estimated failure load (F.load) (P = 0.032) at the tibia compared with those with indolent SM. Handgrip strength was significantly higher in patients who had more Tb.N at the radius (rho, 0.46; P = 0.036) and tibia (rho, 0.49; P = 0.002), and lower who had more trabecular separation at the radius (rho, -0.46; P = 0.035) and tibia (rho, -0.52; P = 0.016). Strong and positive associations between F.load (rho, 0.75; P < 0.001) and stiffness (rho, 0.70; P < 0.001) at the radius, and between F.load at the tibia (rho, 0.45; P = 0.038) were observed with handgrip strength. In this cross-sectional study, aggressive SM was more susceptible to bone deterioration compared with indolent SM. In addition, the findings demonstrated that handgrip strength was associated with bone microarchitecture and bone strength.
- LONGITUDINAL ASSESSMENT OF HAND AND WRIST BONE DESTRUCTION BY ULTRASOUND, AND ITS ASSOCIATION WITH DISEASE ACTIVITY IN PRIMARY SJOGREN'S SYNDROME(2023) FRANCO, A. Silva; MURAI, I.; YANG, T.; SALES, L. Peixoto; GUEDES, L.; PASOTO, S.; FIGUEIREDO, C.; PEREIRA, R. M.
- Vitamin D supplementation and disease activity in patients with immune-mediated rheumatic diseases: A systematic review and meta-analysis(2017) FRANCO, Andre Silva; FREITAS, Thiago Quadrante; BERNARDO, Wanderley M.; PEREIRA, Rosa Maria R.Background:Vitamin D serum levels and the presence and activity of rheumatic conditions have been associated. However, many studies are merely observational, and the existent randomized clinical trials were never systematically analyzed. Therefore, this study aims to provide a systematic review and meta-analysis of such a topic.Methods:MEDLINE, EMBASE, LILACS, COCHRANE, and CINAHL were explored to identify randomized trials that investigated clinical repercussions of vitamin D (or analogs) supplementation for at least 3 months in rheumatic diseases. Standardized clinical and/or laboratorial outcomes related to disease activity were analyzed according to each disease before and after supplementation.Results:Database searches rendered 668 results; 9 were included5 on rheumatoid arthritis, 3 on systemic lupus erythematosus, and 1 on systemic sclerosis. Seven of the studies were meta-analyzed. After vitamin D supplementation, rheumatoid arthritis recurrence decreased; however, not significantly (risk difference=-0.10, 95% CI=-0.21, 0.00, P=.05). No statistical significance was observed regarding visual analog scale (mean difference=2.79, 95% CI=-1.87, 7.44, P=.24) and disease activity score28 (mean difference=-0.31, 95% CI=-0.86, 0.25, P=.28). Regarding systemic lupus erythematosus, anti-dsDNA positivity was significantly reduced (risk difference=-0.10, 95% CI=-0.18, -0.03; P=.005).Conclusion:Vitamin D supplementation reduced anti-dsDNA positivity on systemic lupus erythematosus and could possibly reduce rheumatoid arthritis recurrence, although novel randomized clinical trials are needed to confirm and extend the benefits of this hormone in immune-mediated rheumatic diseases.
- KLOTHO polymorphisms and age-related outcomes in community-dwelling older subjects: The SAo Paulo Ageing & Health (SPAH) Study(2020) PEREIRA, Rosa Maria R.; FREITAS, Thiago Quadrante; FRANCO, Andre Silva; TAKAYAMA, Liliam; CAPARBO, Valeria F.; DOMICIANO, Diogo S.; MACHADO, Luana G.; FIGUEIREDO, Camille P.; MENEZES, Paulo R.; ONUCHIC, Luiz Fernando; CASTRO, Isac deDefective KLOTHO gene expression in mice led to a syndrome resembling human ageing. This study evaluated three KLOTHO polymorphisms, namely G395A, C1818T, and C370S, in an elderly population (mean age of 73 years) and their associations with ageing-related outcomes (cardiovascular events, kidney function, osteoporosis, sarcopenia) and mortality. Estimated glomerular filtration rates (eGFR) was lower in subjects with 1818TT (P=0.047) and 370SS (P=0.046) genotypes. The 1818TT genotype (P=0.006) and 1818T allele were associated with higher frequency of myocardial infarction (MI) (CC:1.7% vs. CT+TT:7.0%; P=0.002). The 370SS genotype was associated with lower stroke frequency (P=0.001). MI (OR 3.35 [95% CI: 1.29-8.74]) and stroke (OR 3.64 [95% CI: 1.48-8.97]) were associated with mortality. Regarding MI, logistic regression showed 1818T allele was a risk factor for death-related MI (OR 4.29 [95% CI: 1.60-11.52]; P=0.003), while 370C was protective (OR 0.03 [95% CI: 0.01-0.08]; P<0.001). Regarding stroke, the 395A and 370C alleles were protective factors (respectively: OR 0.28 [95% CI: 0.20-0.80]; P=0.018; OR 0.10 [95% CI: 0.05-0.18]; P<0.001). This is the first study to determine potential associations between common ageing-related outcomes/mortality and KLOTHO polymorphisms. The 1818T allele was a risk factor for MI-related death. The 395A and 370C alleles were protective factors for stroke-related death in elderly from community.
- Human islet xenotransplantation in rodents: A literature review of experimental model trends(2017) IUAMOTO, Leandro Ryuchi; FRANCO, Andre Silva; SUGUITA, Fabio Yuji; ESSU, Felipe Futema; OLIVEIRA, Lucas Torres; KATO, Juliana Mika; TORSANI, Matheus Belloni; MEYER, Alberto; ANDRAUS, Wellington; CHAIB, Eleazar; D'ALBUQUERQUE, Luiz Augusto CarneiroAmong the innovations for the treatment of type 1 diabetes, islet transplantation is a less invasive method of treatment, although it is still in development. One of the greatest barriers to this technique is the low number of pancreas donors and the low number of pancreases that are available for transplantation. Rodent models have been chosen in most studies of islet rejection and type 1 diabetes prevention to evaluate the quality and function of isolated human islets and to identify alternative solutions to the problem of islet scarcity. The purpose of this study is to conduct a review of islet xenotransplantation experiments from humans to rodents, to organize and analyze the parameters of these experiments, to describe trends in experimental modeling and to assess the viability of this procedure. In this study, we reviewed recently published research regarding islet xenotransplantation from humans to rodents, and we summarized the findings and organized the relevant data. The included studies were recent reports that involved xenotransplantation using human islets in a rodent model. We excluded the studies that related to isotransplantation, autotransplantation and allotransplantation. A total of 34 studies that related to xenotransplantation were selected for review based on their relevance and current data. Advances in the use of different graft sites may overcome autoimmunity and rejection after transplantation, which may solve the problem of the scarcity of islet donors in patients with type 1 diabetes.
- Associations between OPG and RANKL polymorphisms, vertebral fractures, and abdominal aortic calcification in community-dwelling older subjects: the Sao Paulo Ageing & Health Study (SPAH)(2016) PEREIRA, R. M. R.; FIGUEIREDO, C. P.; CHA, C. C.; CAPARBO, V. F.; OLIVEIRA, R. M.; FRANCO, A. S.; MENEZES, P. R.; CASTRO, I. de; ONUCHIC, L. F.This is the first study analyzing concomitantly osteoprotegerin (OPG)/receptor activator of nuclear factor kappa B ligand (RANKL) polymorphisms and OPG/RANKL serum levels and their association with bone mineral density (BMD), vertebral fractures, and vascular aortic calcification in a cohort of 800 subjects in community-dwelling older individuals. Introduction Osteoprotegerin (OPG) and RANKL play an important role in osteoclast activation and differentiation as well as in vascular calcification. At present, there are no studies of OPG or RANKL gene polymorphisms in Brazilian older populations. The aim of this study was to evaluate OPG/RANKL polymorphism and their association with vertebral fractures (VFs) and aortic calcification. Methods Eight hundred subjects (497 women/303 men) were genotyped for the OPG 1181G>C (rs2073618), 163C>T (rs3102735), 245T>G (rs3134069), and 209G>A (rs3134070) and RANKL A>G (rs2277438) single-nucleotide polymorphisms (SNPs). VFs were evaluated by spine radiography (Genant's method). Aortic calcification was quantified using Kauppila's method. Results The isolated genotype analyses and single-allele frequency data showed association of OPG 163C, 245G, and 209A alleles with presence of VFs (P < 0.05). Multiple logistic regression of subjects with absence of VFs vs. those with VFs (grades II/III) revealed only OPG 209A homozygosity as a risk factor for higher-grade VFs (odds ratio (OR) = 4.17, 95 % CI 1.03-16.93, P = 0.046). Regarding aortic calcification, the isolated genotype analysis frequency data revealed a significant association of OPG 1181G, 163C, 245G, and 209A alleles with absent aortic calcification (P < 0.05). Multiple logistic regression data confirmed that the OPG 209A allele was protective for aortic calcification (OR = 0.63, 95 % CI 0.45-0.88, P = 0.007) and the OPG 1181C allele was a risk factor for aortic calcification (OR = 1.26, 95 % CI 1.00-1.58, P = 0.046). Conclusion This study showed that the OPG 209AA genotype was a risk factor for higher-grade VFs, the OPG 209A allele was protective for aortic calcification, and the OPG 1181C was a risk factor for aortic calcification, supporting the involvement of OPG polymorphisms in the analyzed phenotypes and the concept that the related pathogenesis is multifactorial.