RESUMO
Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disorder that displays an important genetic background. Vitamin D3 (VD3 ) through its receptor (VDR) plays an important immunomodulatory role in autoimmune misbalance, being capable of modulating immune responses. Genetic alterations in VDR gene may contribute to an altered risk in SLE development and clinical manifestations. We investigated VDR SNPs (single nucleotide polymorphisms) frequencies in 128 SLE patients and 138 healthy controls (HC) and mRNA differential expression in 29 patients and 17 HC regarding SLE susceptibility as well as clinical features. We observed that rs11168268 G allele (OR = 1.55, p = .01) and G/G genotype (OR = 2.69, p = .008) were associated with increased SLE susceptibility. The rs2248098 G allele and A/G and G/G genotypes were associated to lower SLE susceptibility (OR = 0.66, p = .01; OR = 0.46, p = .01; OR = 0.44, p = .02, respectively). Regarding clinical features, we observed lower risk for: rs11168268 A/G genotype and nephritis (OR = 0.31, p = .01); rs4760648 T/T genotype and photosensitivity (OR = 0.24, p = .02); rs1540339 T/T genotype and antibody anti-dsDNA (OR = 0.19, p = .015); rs3890733 T/T genotype and serositis (OR = 0.10, p = .01). We identified a significant downregulation in VDR expression levels when compared patients and controls overall (p = 1.04e-7 ), in Cdx-2 A/G and G/G (p = .008 and p = .014, respectively) and in patients with nephritis (p = .016) Our results suggested that VDR SNPs influence upon SLE susceptibility and in particular clinical features, acting on mRNA expression in SLE patients overall and the ones with nephritis.
Assuntos
Lúpus Eritematoso Sistêmico , Nefrite , Estudos de Casos e Controles , Frequência do Gene , Predisposição Genética para Doença , Genótipo , Humanos , Lúpus Eritematoso Sistêmico/genética , Nefrite/complicações , Polimorfismo de Nucleotídeo Único , RNA Mensageiro/genética , Receptores de Calcitriol/genéticaAssuntos
Vasculite por IgA/sangue , Vasculite por IgA/microbiologia , Infecções Estreptocócicas/imunologia , Streptococcus , Biópsia , Estudos de Casos e Controles , Criança , Epitopos/imunologia , Globulinas , História do Século XX , História do Século XXI , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/história , Imunoglobulina A/imunologia , Imunoglobulina G/imunologia , Testes Imunológicos/métodos , Células Mesangiais/imunologia , Nefrite/complicações , Nefrite/microbiologia , Pediatria/história , Infecções Estreptocócicas/complicações , Infecções Estreptocócicas/históriaRESUMO
Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.
Assuntos
Vasculite por IgA/complicações , Nefrite/complicações , Paraproteinemias/etiologia , Glucocorticoides/administração & dosagem , Humanos , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/patologia , Imunossupressores/administração & dosagem , Masculino , Pessoa de Meia-Idade , Nefrite/tratamento farmacológico , Nefrite/patologia , Paraproteinemias/tratamento farmacológico , Paraproteinemias/patologiaRESUMO
Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Paraproteinemias/etiologia , Vasculite por IgA/complicações , Nefrite/complicações , Paraproteinemias/patologia , Paraproteinemias/tratamento farmacológico , Vasculite por IgA/patologia , Vasculite por IgA/tratamento farmacológico , Glucocorticoides/administração & dosagem , Imunossupressores/administração & dosagem , Nefrite/patologia , Nefrite/tratamento farmacológicoRESUMO
BACKGROUND: Systemic lupus erythematosus (SLE) and acquired immunodeficiency syndrome (AIDS) share many clinical manifestations and laboratory findings, therefore, concomitant diagnosis of SLE and human immunodeficiency virus (HIV) can be challenging. METHODS: Prospective cohort with 602 patients with SLE who attended the Rheumatology Clinic of the Hospital de Clínicas de Porto Alegre since 2000. All patients were followed until 01 May 2015 or until death, if earlier. Demographic, clinical and laboratory data were prospectively collected. RESULTS: Out of the 602 patients, 11 presented with the diagnosis of AIDS (1.59%). The following variables were significantly more prevalent in patients with concomitant HIV and SLE: neuropsychiatric lupus (10.9% vs. 36.4%; p = 0.028) and smoking (37.6% vs. 80%; p = 0.0009) while malar rash was significantly less prevalent in this population (56% vs. 18.2%; p = 0.015). Nephritis (40.5% vs. 63.6%; p = 0.134) and hemolytic anemia (28.6% vs. 54.5%; p = 0.089) were more prevalent in SLE patients with HIV, but with no statistical significance compared with SLE patients without HIV. The SLICC damage index median in the last medical consultation was significantly higher in SLE patients with HIV (1 vs. 2; p = 0,047). CONCLUSIONS: Our patients with concomitant HIV and SLE have clinically more neuropsychiatric manifestations. For the first time, according to our knowledge, higher cumulative damage was described in lupus patients with concomitant HIV infection. Further studies are needed to elucidate this complex association, its outcomes, prognosis and which therapeutic approach it's best for each case.
Assuntos
Infecções por HIV/complicações , Lúpus Eritematoso Sistêmico/complicações , Síndrome da Imunodeficiência Adquirida/complicações , Adulto , Anemia Hemolítica/complicações , Exantema/complicações , Feminino , Infecções por HIV/diagnóstico , Infecções por HIV/mortalidade , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/mortalidade , Vasculite Associada ao Lúpus do Sistema Nervoso Central/complicações , Masculino , Nefrite/complicações , Estudos ProspectivosRESUMO
Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.
Assuntos
Anemia Hemolítica Autoimune/complicações , Lúpus Eritematoso Sistêmico/complicações , Nefrite/complicações , Trombocitopenia/complicações , Adolescente , Idade de Início , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/patologia , Brasil/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/mortalidade , Mortalidade , Nefrite/diagnóstico , Nefrite/epidemiologia , Nefrite/mortalidade , Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Trombocitopenia/diagnóstico , Trombocitopenia/patologia , Resultado do TratamentoRESUMO
OBJECTIVES: To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment. METHODS: We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits. RESULTS: The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63). CONCLUSION: IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.
Assuntos
Interleucina-17/sangue , Lúpus Eritematoso Sistêmico/sangue , Adolescente , Adulto , Idade de Início , Ansiedade/psicologia , Brasil , Estudos de Casos e Controles , Criança , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Família , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/psicologia , Masculino , Nefrite/sangue , Nefrite/complicações , Nefrite/imunologia , Índice de Gravidade de Doença , Adulto JovemRESUMO
Chronic renal allograft injury is reflected by interstitial fibrosis and tubular atrophy (IF/TA) and by the accumulation of extracellular matrix (ECM). Metalloproteinases (MMPs) are renal physiologic regulators of ECM degradation. Changes in MMPs expression or activity may disturb ECM turnover leading to glomerular scarring and worsening renal function. Our goal was to investigate intragraft MMP2 and MMP9 activities and their correlation with renal dysfunction. Plasma MMP2 and MMP9 activities were analyzed as noninvasive markers of renal allograft deterioration. Transplanted patients were biopsied and histopathologically characterized as IF/TA+ or IF/TA-. Renal function was evaluated by serum creatinine, glomerular filtration rate (GFR) estimated by Modification of Diet in Renal Disease equation and urinary protein/creatinine ratio. Kidney and plasma MMP2 and MMP9 activities were analyzed by zymography. A significant renal dysfunction was observed in IF/TA+ patients. Intragraft proMMP9 showed a significant higher activity in IF/TA+ than in IF/TA- samples and was inversely correlated with the GFR. Intragraft proMMP2 activity tended to increase in IF/TA+ samples, although no statistic significance was reached. Circulating proMMP2 and proMMP9 activities did not show significant differences between groups. Our data provide evidence that correlates intragraft proMMP9 activity with the fibrotic changes and renal dysfunction observed in IF/TA.
Assuntos
Fibrose/fisiopatologia , Transplante de Rim , Túbulos Renais/patologia , Rim/fisiopatologia , Adulto , Atrofia/cirurgia , Biópsia , Estudos de Coortes , Nefropatias Diabéticas/complicações , Nefropatias Diabéticas/terapia , Dieta , Feminino , Fibrose/cirurgia , Taxa de Filtração Glomerular , Rejeição de Enxerto , Sobrevivência de Enxerto , Humanos , Hipertensão Renal/complicações , Hipertensão Renal/terapia , Rim/metabolismo , Rim/cirurgia , Testes de Função Renal , Masculino , Metaloproteinase 2 da Matriz/sangue , Metaloproteinase 2 da Matriz/metabolismo , Metaloproteinase 9 da Matriz/sangue , Metaloproteinase 9 da Matriz/metabolismo , Pessoa de Meia-Idade , Nefrite/complicações , Nefrite/terapiaRESUMO
La hipertensión sensible a sal es el aumento de la presión arterial luego de una sobrecarga salina, como consecuencia esencialmente de una disminución en la excreción renal de sodio. En los últimos años, ha sido desarrollada una teoría para explicar su origen que tiene como base la inflamación del tejido renal. El proceso inicia con la producción en los riñones de radicales libres derivados del metabolismo oxidativo. Luego se desarrolla un mecanismo de inflamación del intersticio renal por infiltración de linfocitos T, y otras células inmunológicas. Fundamentalmente los linfocitos T incrementan la producción de angiotensina II que estimula la retención de sodio y agua a este nivel, favoreciendo el desarrollo de hipertensión sensible a sal. La relación entre infiltración renal por células del sistema inmune e hipertensión sensible a sal permite, en parte, explicar la asociación entre enfermedades autoinmunes y la hipertensión arterial. El uso de antioxidantes y el diseño de nuevos fármacos pueden ser una alternativa adicional al tratamiento de los pacientes afectados.
Salt-sensitive hypertension is produced by a decrease in salt renal excretion after a salt overload. Over the last few years, a new theory has been developed to explain this condition based on renal tissue inflammation. This process begins with free radicals production in renal tissue due to oxidative metabolism. Then they favor a renal inflammation mechanism with T-lymphocytes infiltration and other immune cells. Essentially, T-lymphocytes determine an increase in angiotensin II production which raises sodium and water retention. Association among autoimmune diseases and hypertension may be explained, in part, by the relationship between salt-sensitive hypertension and renal inflammation. The use of antioxidant drugs and the development of new medicaments may be a choice for treating patients affected with this condition.
Assuntos
Humanos , Hipertensão/etiologia , Hipertensão/fisiopatologia , Nefrite/fisiopatologia , Cloreto de Sódio na Dieta/metabolismo , Doenças Autoimunes/complicações , Hipertensão/complicações , Nefrite/complicações , Estresse OxidativoRESUMO
Salt-sensitive hypertension is produced by a decrease in salt renal excretion after a salt overload. Over the last few years, a new theory has been developed to explain this condition based on renal tissue inflammation. This process begins with free radicals production in renal tissue due to oxidative metabolism. Then they favor a renal inflammation mechanism with T-lymphocytes infiltration and other immune cells. Essentially, T-lymphocytes determine an increase in angiotensin ii production which raises sodium and water retention. Association among autoimmune diseases and hypertension may be explained, in part, by the relationship between salt-sensitive hypertension and renal inflammation. The use of antioxidant drugs and the development of new medicaments may be a choice for treating patients affected with this condition.
Assuntos
Hipertensão/etiologia , Hipertensão/fisiopatologia , Nefrite/fisiopatologia , Cloreto de Sódio na Dieta/metabolismo , Doenças Autoimunes/complicações , Humanos , Hipertensão/complicações , Nefrite/complicações , Estresse OxidativoRESUMO
Nephritis characterized by IgA mesangial depositions has been described both in Henoch-Schoenlein purpura (HSP) and in Berger's disease (BD), but common genetic traits are still uncertain. We report here the case of two brothers, the first affected by HSP with persistent nephritis and the second by BD, accidentally discovered as silent microhematuria 1 year after HSP onset in the first brother. HLA genotyping demonstrated the presence of HLA-B35 in both patients. Our findings reinforce the need to screen for urinary abnormalities in family members of patients affected by HSP nephritis to identify a silent IgA nephropathy.
Assuntos
Glomerulonefrite por IGA/genética , Antígeno HLA-B35/genética , Nefrite/genética , Adolescente , Criança , Feminino , Genótipo , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/genética , Vasculite por IgA/patologia , Masculino , Pessoa de Meia-Idade , Nefrite/complicações , Nefrite/patologia , FenótipoRESUMO
Immune cell infiltration of the kidney is a constant feature in salt-sensitive hypertension (SSHTN). We evaluated the relationship between the renal inflammation and pressure natriuresis in the model of SSHTN that results from transient oral administration of N(ω)-nitro-L-arginine methyl ester (L-NAME). Pressure natriuresis was determined in Wistar rats that received 4 wk of a high-salt (4% NaCl) diet, starting 1 wk after stopping L-NAME, which was administered alone (SSHTN group, n = 17) or in association with mycophenolate mofetil (MMF; MMF group, n = 15). The administration of MMF in association with L-NAME is known to prevent the subsequent development of SSHTN. Control groups received a high (n = 12)- and normal (0.4%)-salt diet (n = 20). Rats with SSHTN had increased expression of inflammatory cytokines and oxidative stress. The severity of hypertension correlated directly (P < 0.0001) with the number of tubulointerstitial immune cells and angiotensin II-expressing cells. Pressure natriuresis was studied at renal arterial pressures (RAPs) of 90, 110, 130, and 150 mmHg. Glomerular filtration rate was similar and stable in all groups, and renal blood flow was decreased in the SSHTN group. Significantly decreased natriuresis (P < 0.05) was found in the SSHTN group at RAPs of 130 and 150 mmHg, and there was an inverse correlation (P < 0.01) between the urinary sodium excretion and the number of tubulointerstitial inflammatory cells (lymphocytes and macrophages) and cells expressing angiotensin II. We conclude that tubulointerstitial inflammation plays a key role in the impairment of pressure natriuresis that results in salt-dependent hypertension in this experimental model.
Assuntos
Pressão Sanguínea/efeitos dos fármacos , Hipertensão/induzido quimicamente , Túbulos Renais/patologia , Natriurese/fisiologia , Cloreto de Sódio na Dieta/administração & dosagem , Animais , Hipertensão/fisiopatologia , Túbulos Renais/citologia , Túbulos Renais/imunologia , Masculino , NG-Nitroarginina Metil Éster , Natriurese/efeitos dos fármacos , Nefrite/complicações , Nefrite/patologia , Ratos , Ratos Wistar , Cloreto de Sódio na Dieta/efeitos adversosRESUMO
Focal acute nephritis (FAN) or acute lobar nephronia is a rare clinical picture characterized by an infection localized in the kidney, with an inflammatory mass without liquefaction. Of variable clinical manifestations, diagnosis is achieved through CT scanning. Histologically, unlike acute pyelonephritis, it does not present a diffuse infíltrate. Objective: Case report of FAN in a pediatric patient. Case Report: Ten year old male complaining of abdominal pain, presents painful percussion in the right lumbar fossa. Urinary analysis and culture were negative, renal sonogram was negative. Abdominal CT sean showed múltiple hypodense renal foci. He responded well to cephotaxim, being discharged in the third day of hospitalization with completion of treatment as outpatient. Differential diagnosis with Acute Pyelonephritis is very important, as it requires controlled and opportune treatment to prevent renal absceses. Diagnosis of this pathology must be pursued despite a normal UA.
La nefritis aguda focal o nefronia lobar aguda constituye un cuadro poco común caracterizado por una infección localizada en el riñon, la que corresponde a una masa inflamatoria sin licuefacción. Posee una clínica variable, siendo la tomograña computada (TAC) la prueba más sensible y específica para el diagnóstico de esta enfermedad. Esta patología se diferencia histológicamente de la pielonefritis aguda por no presentar un infiltrado inflamatorio difuso. Objetivo: presentar un caso de nefronia aguda multifocal en un paciente pediátrico. Caso clínico: Escolar de 10 años que consultó por dolor abdominal, al examen destacaba la presencia de percusión dolorosa en fosa lumbar. Los exámenes de orina y urocultivo fueron negativos. Al ingreso no se detectó cambios renales ecográficamente evidenciables. Se realizó un TAC de abdomen que mostraba múltiples focos renales hipodensos. Respondió favorablemente a terapia antibiótica con cefotaxima siendo dado de alta al tercer día, completando terapia en forma ambulatoria. La diferenciación de este cuadro de otros procesos renales como la pielonefritis aguda (PNA) es muy importante, ya que precisa un tratamiento oportuno y controlado por el riesgo de evolucionar a absceso renal. El diagnóstico de esta patología debe ser buscado a pesar de contar con exámenes de orina negativos.
Assuntos
Humanos , Masculino , Criança , Infecções Urinárias/etiologia , Nefrite/complicações , Nefrite/diagnóstico , Doença Aguda , Antibacterianos/uso terapêutico , Cefadroxila/uso terapêutico , Cefotaxima/uso terapêutico , Infecções Urinárias/tratamento farmacológico , Nefrite/tratamento farmacológicoRESUMO
Some clinical reports suggest that allergic and reno-urinary diseases could be associated. Etiopathogenic aspects are described, as well as some clues of anamnesis that will help us to suspect allergic etiology.
Assuntos
Hipersensibilidade/complicações , Nefrite/complicações , Síndrome Nefrótica/complicações , Infecções Urinárias/complicações , HumanosRESUMO
Hematuria es una manifestación frecuentemente encontrada en la práctica clínica pediátrica. El objetivo central del presente trabajo es reportar las características clínico-epidemiológicas de 362 niños con hematuria atendidos durante el periodo junio 1998 a mayo 1999 en nuestra institución; esta cifra correspondió al 1,1 por ciento de todas las consultas y admisiones pediátricas y al 8.4 por ciento de las correspondientes a nefrología pediátrica para el período estudiado. El promedio de edad fue de 7,7 ñ 6,1 años, rango 0-17 años, 56 por ciento varones y 44 por ciento hembras. 62 por ciento presentaba hematuria microscópica y 38 por ciento macro y microscópica. La etiología de la hematuria para el grupo total fue: hipercalciuria idiopática y otras alteraciones metabólicas 23,5 por ciento, nefritis aguda 19,3 por ciento. infección documentada de vía urinarias 19 por ciento, urolitiasis 16 por ciento, malformaciones congénitas del tracto urinario 8,3 por ciento "hematuria primaria" 4,4 por ciento síndrome nefrótico 2,2 por ciento, hipoxia neonatal 1,6 por ciento, traumatismos del tracto urinario 1,4 por ciento, nefropatía por IgA 1,4 por ciento y otras 2,9 por ciento. El grupo etario más afectado fue el de los preescolares (34,3 por ciento), seguido por los escolares (27,3 por ciento), luego lactantes, preadolescentes y adolescentes y recién nacidos. Se especifican las causas de hematuria para cada grupo etario y las probables causas que expliquen la frecuencia de determinadas patologías en nuestra área geográfica. La presente casuística, eminentemente descriptiva, muestra la frecuencia, etiología y otras características de la hematuria en clínica nefrológica pediátrica en un centro de referencia y abre la posibilidad de estudios comparativos
Assuntos
Humanos , Feminino , Masculino , Lactente , Pré-Escolar , Adolescente , Hematúria/epidemiologia , Hospitais Pediátricos/estatística & dados numéricos , Distribuição por Idade , Cálcio/urina , Cálculos Urinários/complicações , Hematúria/diagnóstico , Hematúria/etiologia , Nefrite/complicações , Venezuela/epidemiologiaRESUMO
OBJECTIVES: To validate a scoring system to assess the severity of renal lesions and to correlate histology with clinical findings. We also examined the efficacy of treatment with prednisone (1 to 2 mg/kg/d) and azathioprine (1 to 2 mg/kg/d) for severe Henoch-Schonlein purpura (HSP) nephritis. METHODS: Twenty patients were evaluated retrospectively. All underwent biopsy before treatment, and 13 underwent biopsy after therapy. We developed a scale based on glomerular, tubulointerstitial (TI), and vascular changes and assigned all specimens acuity, chronicity, and TI scores. The outcomes of 17 patients were compared with those of a historical control group. RESULTS: Chronicity score at initial biopsy increased with increasing delay between onset of renal involvement and first biopsy (rho = 0.55, P =.016) but did not progress after treatment was initiated. Both acuity (rho = 0.57,P =. 016) and TI (rho = 0.69, P =.003) scores correlated with clinical severity at first biopsy. The TI score correlated negatively with serum albumin (rho = -.60, P <.01). Significantly more patients in the study group than in the control group had a favorable outcome (15 [88%] of 17 vs 32 [54%] of 59, P =.011). CONCLUSIONS: Our scale reflects disease activity and highlights the importance of TI changes in severe HSP nephritis. Outcome comparisons indicate that early treatment with prednisone and azathioprine prevents progression of chronic changes and improves outcome.
Assuntos
Anti-Inflamatórios/uso terapêutico , Azatioprina/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/patologia , Imunossupressores/uso terapêutico , Nefrite/tratamento farmacológico , Nefrite/patologia , Prednisona/uso terapêutico , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Vasculite por IgA/complicações , Masculino , Nefrite/complicações , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
La enfermedad de Kyrle es una dermatosis excepcional, de etiología desconocida y difícil diagnóstico clínico, precisando estudios histopatológicos para llegar a él. Presentamos dos pacientes con enfermedad de Kyrle secundaria a enfermedades sistémicas
Assuntos
Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Ceratose/diagnóstico , Diagnóstico Diferencial , Ceratose/tratamento farmacológico , Ceratose/patologia , Nefrite/complicações , Manifestações CutâneasRESUMO
La enfermedad de Kyrle es una dermatosis excepcional, de etiología desconocida y difícil diagnóstico clínico, precisando estudios histopatológicos para llegar a él. Presentamos dos pacientes con enfermedad de Kyrle secundaria a enfermedades sistémicas (AU)
Assuntos
Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Ceratose/diagnóstico , Ceratose/tratamento farmacológico , Ceratose/patologia , Nefrite/complicações , Manifestações Cutâneas , Diagnóstico DiferencialRESUMO
La amenaza de una guerra o un accidente nuclear nos llena de curiosidad e indiferencia. En general esto se refleja a nivel mundial con muy poca bibliografía disponible que nos dé información sobre estos eventos y las conductas más aconsejadas para su control y tratamiento. Veremos en este trabajo las características de las distintas radiaciones, nociones de lo significativamente importante para el especialista que son los efectos sobre el individuo y los criterios para diagnóstico, pronóstico y tratamiento.
Assuntos
Humanos , Liberação Nociva de Radioativos/mortalidade , Liberação Nociva de Radioativos/prevenção & controle , Partículas beta/efeitos adversos , Raios gama/efeitos adversos , Lesões por Radiação/diagnóstico , Lesões por Radiação/terapia , Nêutrons/efeitos adversos , Partículas alfa/efeitos adversos , Radioterapia/efeitos adversos , Anestesia , Cegueira , Queimaduras , Hepatite/complicações , Assistência a Feridos em Massa , Nefrite/complicações , Nefrite/mortalidade , Pneumonite por Radiação/complicações , Pneumonite por Radiação , Pneumonite por Radiação/terapia , Pericardite/complicações , Cuidados Pós-OperatóriosRESUMO
La amenaza de una guerra o un accidente nuclear nos llena de curiosidad e indiferencia. En general esto se refleja a nivel mundial con muy poca bibliografía disponible que nos dé información sobre estos eventos y las conductas más aconsejadas para su control y tratamiento. Veremos en este trabajo las características de las distintas radiaciones, nociones de lo significativamente importante para el especialista que son los efectos sobre el individuo y los criterios para diagnóstico, pronóstico y tratamiento. (AU)