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1.
Rev. Hosp. Ital. B. Aires (2004) ; 41(4): 171-175, dic. 2021. tab
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1366760

ABSTRACT

Introducción: la amiloidosis AA puede ser una complicación de ciertos trastornos inflamatorios crónicos, aunque entre el 21% y 50% puede ser idiopática. No existe un tratamiento específico. El tocilizumab, dirigido contra el receptor de IL-6 y orientado a disminuir la producción de SAA, podría ser eficaz. Métodos: en este estudio informamos datos de 6 pacientes con amiloidosis AA tratados con tocilizumab monoterapia subcutáneo en el período 2011-2018. Los criterios de valoración principales fueron la mejora clínica y bioquímica de los órganos afectados y los parámetros bioquímicos marcadores de inflamación. Resultados: el riñón estaba afectado en todos los pacientes, manifestándose con caída del filtrado glomerular y síndrome nefrótico. La hemorragia digestiva se presentó en un paciente y otro tenía afectación pulmonar en la biopsia. Luego del posterior al tratamiento, todos mejoraron el hematocrito, la albúmina sérica y el índice de masa corporal. El SAA disminuyó en 5 pacientes. Un paciente mejoró su función renal, mientras 4 se mantuvieron estables. Tres pacientes disminuyeron los valores de proteinuria. Conclusión: el tratamiento con tocilizumab podría ser eficaz en el tratamiento de los pacientes con amiloidosis AA. (AU)


Introduction: AA amyloidosis can be a complication of certain chronic inflammatory disorders, although between 21% and 50% can be idiopathic. There is no specific treatment. Tocilizumab, directed against the IL-6 receptor and aimed at decreasing SAA production, could be effective. Methods: in this study, we report data from 6 patients with AA amyloidosis treated with subcutaneous tocilizumab monotherapy between the period 2011-2018. The main endpoints were the clinical and biochemical improvement of the affected organs and the biochemical parameters markers of inflammation. Results: the kidney was affected in all patients, manifesting with a fall in glomerular filtration rate and nephrotic syndrome. Gastrointestinal bleeding occurred in one patient and another had lung involvement on biopsy. After treatment, all improved hematocrit, serum albumin, and body mass index. SAA decreased in 5 patients. One patient improved his kidney function, while 4 remained stable. Three patients decreased proteinuria values. Conclusion: treatment with tocilizumab could be effective in the treatment of patients with AA amyloidosis. (AU)


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Serum Amyloid A Protein/drug effects , Antibodies, Monoclonal, Humanized/therapeutic use , Amyloidosis/drug therapy , Body Mass Index , Receptors, Interleukin-6/drug effects , Antibodies, Monoclonal, Humanized/administration & dosage , Glomerular Filtration Rate/drug effects , Gastrointestinal Hemorrhage/complications , Amyloidosis/blood , Inflammation/complications , Lung Diseases/complications , Nephrotic Syndrome/complications
2.
J. bras. nefrol ; 43(3): 440-444, July-Sept. 2021. graf
Article in English, Portuguese | LILACS | ID: biblio-1340119

ABSTRACT

Abstract Primary atypical hemolytic-uremic syndrome is a rare disease characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia, and renal dysfunction; it is related to alterations in the regulation of the alternative pathway of complement due to genetic mutations. The association with nephrotic syndrome is unusual. We present here a pediatric patient diagnosed with primary atypical hemolytic-uremic syndrome associated with nephrotic syndrome who responded to eculizumab treatment.


Resumo A síndrome hemolítico-urêmica atípica primária é uma doença rara, caracterizada por anemia hemolítica microangiopática não-imune, trombocitopenia e disfunção renal; está relacionado a alterações na regulação da via alternativa do complemento devido a mutações genéticas. A associação com a síndrome nefrótica é incomum. Apresentamos aqui um paciente pediátrico com diagnóstico de síndrome hemolítico-urêmica atípica primária associada à síndrome nefrótica que respondeu ao tratamento com eculizumab.


Subject(s)
Humans , Child , Purpura, Thrombotic Thrombocytopenic , Atypical Hemolytic Uremic Syndrome/complications , Anemia, Hemolytic , Nephrotic Syndrome/complications , Complement System Proteins
3.
J. bras. nefrol ; 43(1): 20-27, Jan.-Mar. 2021. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1154663

ABSTRACT

ABSTRACT Introduction: Emergence of acute kidney injury (AKI) in patients with nephrotic syndrome (NS) requires prompt diagnosis and differentiation between acute tubular necrosis (ATN) and proliferative glomerulonephritis. We studied the potential use of commercial urinary biomarkers' tests in the diagnosis of AKI in patients with NS. Methods: A cross sectional estimate of urinary concentrations of KIM-1 and NGAL was performed in 40 patients with NS: 9 with proliferative glomerulopathy, being 4 with AKI and 31 without proliferative glomerulopathy, being 15 with AKI. AKI was defined using the KDIGO criteria. Results: The mean age was 35 ± 16 years. The main diagnoses were focal and segmental glomerulosclerosis (10, 25%), membranous glomerulopathy (10, 25%), minimal change disease (7, 18%), lupus nephritis (6, 15%), and proliferative glomerulonephritis (3, 8%). Patients with ATN had higher levels of urinary KIM-1 (P = 0.0157) and NGAL (P = 0.023) than patients without ATN. The urinary concentrations of KIM-1 (P= 0.009) and NGAL (P= 0.002) were higher in patients with AKI than in patients without AKI. Urinary NGAL and KIM-1 levels were significantly higher in patients with ATN without proliferative glomerulonephritis than in patients with proliferative glomerulonephritis (P = 0.003 and P=0.024, respectively). Conclusions: Neutrophil gelatinase associated lipocalin (NGAL) and kidney injury molecule 1 (KIM-1) estimates correlated with histological signs of ATN and were able to discriminate patients with AKI even in conditions of NS. Furthermore, urinary levels of NGAL and KIM-1 may be useful in the differential diagnosis of acute tubular necrosis and exudative glomerulonephritis in patients with nephrotic syndrome.


RESUMO Introdução: O surgimento de lesão renal aguda (LRA) em pacientes com síndrome nefrótica (SN) requer diagnóstico imediato e diferenciação entre necrose tubular aguda (NTA) e glomerulonefrite proliferativa. Avaliamos o uso potencial de testes de biomarcadores urinários comerciais no diagnóstico de LRA em pacientes com SN. Métodos: Uma estimativa transversal das concentrações urinárias de KIM-1 e NGAL foi realizada em 40 pacientes com SN: 9 com glomerulopatia proliferativa, sendo 4 com LRA e 31 sem glomerulopatia proliferativa, sendo 15 com LRA. A LRA foi definida usando os critérios da KDIGO. Resultados: A média de idade foi de 35 ± 16 anos. Os principais diagnósticos foram glomeruloesclerose segmentar e focal (10, 25%), glomerulopatia membranosa (10, 25%), doença por lesão mínima (7, 18%), nefrite lúpica (6, 15%) e glomerulonefrite proliferativa (3, 8 %). Os pacientes com NTA apresentaram níveis mais elevados de KIM-1 urinário (P = 0,0157) e NGAL (P = 0,023) do que pacientes sem NTA. As concentrações urinárias de KIM-1 (P = 0,009) e NGAL (P = 0,002) foram maiores em pacientes com LRA do que em pacientes sem LRA. Os níveis urinários de NGAL e KIM-1 foram significativamente maiores em pacientes com NTA sem glomerulonefrite proliferativa do que em pacientes com glomerulonefrite proliferativa (P = 0,003 e P = 0,024, respectivamente). Conclusões: As estimativas de lipocalina associada a gelatinase de neutrófilos (NGAL) e molécula de lesão renal 1 (KIM-1) se correlacionaram com sinais histológicos de NTA, e foram capazes de discriminar pacientes com LRA mesmo em condições de SN. Além disso, os níveis urinários de NGAL e KIM-1 podem ser úteis no diagnóstico diferencial de necrose tubular aguda e glomerulonefrite exsudativa em pacientes com síndrome nefrótica.


Subject(s)
Humans , Adult , Acute Kidney Injury/diagnosis , Acute Kidney Injury/etiology , Nephrotic Syndrome/complications , Biomarkers , Cross-Sectional Studies , Lipocalin-2 , Kidney Function Tests
4.
Rev. bras. oftalmol ; 80(5): e0045, 2021. graf
Article in English | LILACS | ID: biblio-1347259

ABSTRACT

ABSTRACT Exudative retinal detachment occurs when fluid accumulates between the neurosensory retina and the retinal pigment epithelium. Ocular diseases or multisystem conditions such as nephrotic syndrome may lead to exudative retinal detachment. This report describes a case of nephrotic syndrome secondary to minimal change disease, anasarca and bilateral serous macular detachment in an adult patient. A 75-year-old male patient presented to the emergency department with generalized edema, asthenia, and visual impairment. Medical history included a recent diagnosis of nephrotic syndrome secondary to minimal change disease, which had been controlled with corticosteroid therapy. At presentation, best corrected visual acuity was 20/100. Slit-lamp examination revealed xanthelasmas and mild bilateral eyelid edema and chemosis. Dilated fundus examination confirmed bilateral macular detachment. The patient did not respond to diuretic therapy. Ttherefore, hemodialysis was started. Two months later, visual acuity improved to 20/25 and near normal restoration of retinal anatomy was achieved, with concurrent remission of proteinuria. Exudative retinal detachment is a multifactorial condition. However, in diseases associated with severe hypoalbuminemia, such as nephrotic syndrome, low oncotic pressure in choroidal vessels and high interstitial pressure in the choroid may explain retinal detachment. Patients with chronic kidney disease carry a high risk of ophthalmic disease development. Several mechanisms that affect ocular vessels, the retina and the choroid are thought to be involved. A multidisciplinary approach is crucial to resolve the ophthalmic condition and improve overall health.


RESUMO O descolamento de retina exsudativo ocorre quando o fluido se acumula entre a retina neurossensorial e o epitélio pigmentado da retina. Patologias oculares isoladas ou doenças multissistêmicas, como a síndrome nefrótica, podem levar ao descolamento de retina exsudativo. Apresenta-se aqui o caso de um adulto com síndrome nefrótica por doença de lesões mínimas, anasarca e descolamento de retina exsudativo macular bilateral. Trata-se de um homem de 75 anos de idade, que recorreu ao serviço de urgência com edema generalizado, astenia e diminuição da acuidade visual. Os antecedentes pessoais incluíam diagnóstico recente de síndrome nefrótica secundária à doença de lesões mínimas, em uso de corticoterapia. Na apresentação, a melhor acuidade visual corrigida era 20/100. A biomicroscopia revelou xantelasmas, edema palpebral leve e quemose nos dois olhos. Fundoscopia mostrou descolamento macular bilateral. O doente iniciou diuréticos com pouca resposta clínica, tendo sido adicionada hemodiálise. Verificou-se melhora da acuidade visual para 20/25 e restauração quase total da anatomia da retina 2 meses após o início do tratamento, coincidindo com a remissão da proteinúria. A fisiopatologia dos descolamentos de retina exsudativos é multifatorial, mas, em doenças com hipoalbuminemia grave, como a síndrome nefrótica, a baixa pressão oncótica e a alta pressão intersticial na coroide podem explicar o descolamento macular exsudativo. Doentes com doença renal crônica constituem um grupo de risco para o desenvolvimento de doença ocular, envolvendo vários mecanismos que afetam vasos, retina e coroide. Uma abordagem multidisciplinar é crucial para a melhoria da doença oftalmológica e do estado geral do doente.


Subject(s)
Humans , Male , Aged , Retinal Detachment/etiology , Renal Insufficiency, Chronic/complications , Nephrotic Syndrome/complications , Ophthalmoscopy , Retinal Detachment/diagnosis , Visual Acuity , Tomography, Optical Coherence , Diagnostic Techniques, Ophthalmological , Renal Insufficiency, Chronic/diagnosis , Exudates and Transudates , Slit Lamp Microscopy , Fundus Oculi , Macula Lutea
5.
J. bras. nefrol ; 42(1): 59-66, Jan.-Mar. 2020. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1098349

ABSTRACT

ABSTRACT Introduction: Mast cells may be involved in inflammation and contribute to the onset of fibrosis in lupus nephritis (LN). Objective: This study aimed to correlate the presence of mast cells in kidney biopsy specimens of pediatric patients with LN with activity (AI) and chronicity (CI) indices and assess how effectively mast cells may be used as a prognostic factor. Method: The study included 40 patients aged 6-18 years diagnosed with LN at the Renal Disease Service of the Federal University of Triângulo Mineiro between 1996 and 2015. Workup and epidemiological data were evaluated vis-à-vis AI, CI, and mast cell counts (MCC). Results: Significant positive correlations were found between mast cell counts (MCC) and AI (p = 0.003; r: 0.66) and MCC and CI (p = 0.048; r: 0.48). The ROC curve showed that mast cells were highly sensitive and specific in the differentiation of patients with an AI > 12 from individuals with an AI ≤ 12. Serum creatinine levels were higher in individuals with class IV LN than in patients with class V disease [1.50 (0.40-20.90) vs. 0.70 (0.62-0.90), p = 0.04]. Blood urea nitrogen had a positive significant correlation with MCC (p = 0.002; r: 0.75). A trend toward a negative correlation was observed between MCC and serum albumin (p = 0.06; r: -0.5459). Kidney biopsies of patients with nephrotic syndrome had higher MCC [2.12 (0.41-5.140) vs. 0.53 (0.0-3.94), p = 0.07]. Conclusion: Inflammatory cell infiltration and morphological differences between cell types in the inflammatory infiltrate are relevant factors in the assessment of the LN. Mast cell analysis and AI/CI assessment may be relevant prognostic indicators for pediatric patients with LN.


RESUMO Introdução: Mastócitos podem participar da inflamação e contribuir para fibrose na nefrite lúpica (NL). Objetivo: Correlacionar mastócitos em biópsias renais (BR) de pacientes pediátricos com NL com índices de atividade (IA) e cronicidade (IC), avaliando sua efetividade como fator prognóstico. Metodologia: Foram estudados 40 pacientes, entre 6 e 18 anos, diagnosticados com NL pelo Serviço de Nefropatologia da UFTM entre 1996 e 2015. Dados laboratoriais e epidemiológicos foram correlacionados com IA, IC e contagem de mastócitos (CM). Resultados: Encontramos correlação positiva e significativa entre contagem de mastócitos (CM) e IA (p = 0,003; r: 0,66) e entre CM e IC (p = 0,048; r: 0,48). Conforme a curva Roc, os mastócitos têm alta sensibilidade e especificidade na diferenciação de pacientes com IA menor ou maior do que 12. A creatinina sérica foi mais elevada na classe IV em relação à classe V [1,50 (0,40 - 20,90) versus 0,70 (0,62 - 0,90), p = 0,04]. Ureia sérica apresentou correlação positiva e significativa com CM (p = 0,002; r: 0,75). Observou-se tendência à correlação negativa entre CM e albumina sérica (p = 0,06; r: -0,5459). BR de pacientes com síndrome nefrótica apresentaram maior CM [2,12 (0,41 - 5,140) versus 0,53 (0,0 - 3,94), p = 0,07]. Conclusão: Não apenas o infiltrado inflamatório como também a diferenciação morfológica dos tipos celulares que o constituem são importantes para a avaliação da NL. Isso indica que a análise dos mastócitos, juntamente com a dos IA e IC, pode ajudar os nefrologistas a definirem o prognóstico de pacientes pediátricos.


Subject(s)
Humans , Male , Female , Child , Adolescent , Severity of Illness Index , Lupus Nephritis/diagnosis , Kidney/pathology , Mast Cells/pathology , Prognosis , Biopsy , Blood Urea Nitrogen , Lupus Nephritis/complications , Lupus Nephritis/blood , Serum Albumin/analysis , Cell Count , Creatinine/blood , Nephrotic Syndrome/complications , Nephrotic Syndrome/pathology , Nephrotic Syndrome/blood
6.
J. bras. nefrol ; 42(1): 113-117, Jan.-Mar. 2020. graf
Article in English, Portuguese | LILACS | ID: biblio-1098333

ABSTRACT

Abstract Introduction: Some cases of membranous nephropathy (MGN) present focal segmental glomerulosclerosis (FSGS) typically associated with disease progression. However, we report a case of a patient who seemed to have MGN and FSGS, both primary. Case presentation: A 17-year-old female, Caucasian, presenting lower extremity edema associated with episodes of foamy urine and high blood pressure, had physical and laboratorial exams indicating nephrotic syndrome. A renal biopsy was performed and focal and segmental glomerulosclerosis were observed under light microscopy in some glomeruli presented as tip lesion, and in others it was accompanied by podocyte hypertrophy and podocyte detachment in urinary space, compatible with podocytopathy FSGS. Besides, there were thickened capillary loops with basement membrane irregularities due to "spikes" compatible with MGN stage II. Immunofluorescence showed finely granular IgG, IgG4, and PLA2R deposits in capillary loops and, in electron microscopy, subepithelial deposits and foot process effacement. These morphological findings are compatible with FSGS and MGN stage II. Conclusions: In the present case, clinical and morphological characteristics showed a possible overlap of primary FSGS and MGN as focal and segmental glomerulosclerosis does not seem to be related with MGN progression but with the podocytopathy FSGS.


Resumo Introdução: Alguns casos de nefropatia membranosa (NM) apresentam glomeruloesclerose segmentar e focal (GESF) tipicamente associada a progressão da doença. Contudo, relatamos o caso de uma paciente que parece ter NM e GESF, ambas primárias. Apresentação do caso: Uma jovem branca de 17 anos de idade com edema de membros inferiores associado a episódios de urina espumosa e hipertensão apresentou-se com achados físicos e laboratoriais sugestivos de síndrome nefrótica. Foi realizada biópsia renal. GESF foi observada por microscopia de luz em alguns glomérulos que apresentavam lesões de ponta, enquanto em outros o achado era acompanhado por hipertrofia podocitária e descolamento de podócitos no espaço urinário, compatíveis com podocitopatia GESF. Além disso, as alças capilares estavam espessadas com irregularidades na membrana basal devido a "espículas" compatíveis com NM estágio II. Imunofluorescência revelou depósitos finamente granulares de IgG, IgG4 e PLA2R nas alças capilares. Microscopia eletrônica exibiu depósitos subepiteliais e apagamento de pedicelos. Tais achados morfológicos são compatíveis com GESF e NM estágio II. Conclusões: No presente caso, as características clínicas e morfológicas revelaram uma possível sobreposição de GESF primária e NM, uma vez que a glomeruloesclerose segmentar e focal não parece estar relacionada com a progressão da NM, mas com a podocitopatia GESF.


Subject(s)
Humans , Female , Adolescent , Glomerulosclerosis, Focal Segmental/complications , Glomerulosclerosis, Focal Segmental/diagnosis , Glomerulonephritis, Membranous/complications , Glomerulonephritis, Membranous/diagnosis , Nephrotic Syndrome/complications , Nephrotic Syndrome/diagnosis , Biopsy , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Glomerulosclerosis, Focal Segmental/pathology , Glomerulosclerosis, Focal Segmental/drug therapy , Glomerulonephritis, Membranous/pathology , Glomerulonephritis, Membranous/drug therapy , Treatment Outcome , Kidney/pathology , Nephrotic Syndrome/drug therapy
7.
Chinese Journal of Contemporary Pediatrics ; (12): 1326-1330, 2020.
Article in Chinese | WPRIM | ID: wpr-879797

ABSTRACT

A boy, aged 6 years and 11 months, was admitted due to nephrotic syndrome for 2 years, cough for 1 month, and shortness of breath for 15 days. The boy had a history of treatment with hormone and immunosuppressant. Chest CT after the onset of cough and shortness of breath showed diffuse ground-glass opacities in both lungs. Serum (1, 3)-beta-D glucan was tested positive, and the nucleic acid of cytomegalovirus was detected in respiratory secretions. After the anti-fungal and anti-viral treatment, the child improved temporarily but worsened again within a short period of time.


Subject(s)
Child , Humans , Male , Cough/etiology , Cytomegalovirus Infections/therapy , Dyspnea/etiology , Extracorporeal Membrane Oxygenation , Nephrotic Syndrome/complications , Pneumonia, Pneumocystis/therapy , Respiratory Distress Syndrome, Newborn/therapy
8.
Chinese Medical Journal ; (24): 206-211, 2020.
Article in English | WPRIM | ID: wpr-878028

ABSTRACT

BACKGROUND@#Minimal change nephropathy (MCD) is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury (AKI). This study aimed to investigate the clinical characteristics and related factors of AKI in patients with MCD and nephrotic syndrome.@*METHODS@#Patients from Chinese People's Liberation Army General Hospital who were diagnosed with pathological renal MCD with clinical manifestations of nephrotic syndrome were included from January 1, 2013 to December 31, 2017. Patients diagnosed with membranous nephropathy (MN) by renal biopsy from January 1, 2013 to December 31, 2017 are included as a control population. We retrospectively analyzed the clinical and pathological characteristics of patients as well as the percentages and clinical characteristics of AKI in different age groups. We assessed the correlation of pathological characteristics with serum creatinine using multivariate linear regression analysis.@*RESULTS@#A total of 367 patients with MCD were included in the analysis, with a sex ratio of 1.46: 1 (male: female) and an age range of 6 to 77 years. Among all the patients, 109 developed AKI (29.7%), and of these patients, 85 were male (78.0%). In the 586 patients with MN, 27 (4.6%) patients developed AKI. The percentage of AKI in MCD patients was significantly higher than that in MN patients (χ2 = 41.063, P < 0.001). The percentage of AKI increased with age in the MCD patients. The percentage of AKI in patients aged 50 years or older was 52.9% (46/87), which was significantly higher than that [22.5% (63/280)] in patients under 50 years (χ2 = 6.347, P = 0.013). We observed statistically significant differences in age (43 [27, 59] years vs. 28 [20, 44] years, Z = 5.487, P < 0.001), male (78.0% vs. 51.4%, χ2 = 22.470, P < 0.001), serum albumin (19.9 ± 6.1 g/L vs. 21.5 ± 5.7 g/L, t = 2.376, P = 0.018), serum creatinine (129.5 [105.7, 171.1] μmol/L vs. 69.7 [57.7, 81.9] μmol/L, Z = 14.190, P < 0.001), serum urea (10.1 [6.2, 15.8] mmol/L vs. 4.7 [3.6, 6.4] mmol/L, Z = 10.545, P < 0.001), IgE (266.0 [86.7, 963.0] IU/ml vs. 142.0 [35.3, 516.5] IU/ml, Z = 2.742, P = 0.007), history of diabetes (6.4% vs. 1.2%, P = 0.009), and history of hypertension (23.9% vs. 5.1%, χ2 = 28.238, P < 0.001) between the AKI group and the non-AKI group. According to multivariate linear regression analysis, among the renal pathological features analyzed, renal tubular epithelial cell damage (β = 178.010, 95% CI: 147.888-208.132, P < 0.001) and renal interstitial edema (β = 28.833, 95% CI: 11.966-45.700, P = 0.001) correlated with serum creatinine values.@*CONCLUSIONS@#The percentage of AKI in MCD patients is significantly higher than that in MN patients. Patients over 50 years old are more likely to develop AKI. Renal tubular epithelial cell injury and renal interstitial edema may be the main pathological lesions that are associated with elevated serum creatinine in patients with MCD.


Subject(s)
Adolescent , Adult , Aged , Child , Female , Humans , Male , Middle Aged , Young Adult , Acute Kidney Injury/etiology , Cross-Sectional Studies , Kidney , Nephrosis, Lipoid/complications , Nephrotic Syndrome/complications , Retrospective Studies
9.
J. bras. nefrol ; 41(4): 526-533, Out.-Dec. 2019. tab, graf
Article in English | LILACS | ID: biblio-1056617

ABSTRACT

ABSTRACT Introduction: Children with nephrotic syndrome are at increased risk of infections because of disease status itself and use of various immunosuppressive agents. In majority, infections trigger relapses requiring hospitalization with increased risk of morbidity and mortality. This study aimed to determine the incidence, spectrum, and risk factors for major infections in hospitalized children with nephrotic syndrome. Methods: All consecutive hospitalized children between 1-12 years of age with nephrotic syndrome were enrolled in the study. Children with acute nephritis, secondary nephrotic syndrome as well as those admitted for diagnostic renal biopsy and intravenous cyclophosphamide or rituximab infusion were excluded. Results: A total of 148 children with 162 admissions were enrolled. Incidence of major infections in hospitalized children with nephrotic syndrome was 43.8%. Peritonitis was the commonest infection (24%), followed by pneumonia (18%), urinary tract infection (15%), and cellulitis (14%), contributing with two thirds of major infections. Streptococcus pneumoniae (n = 9) was the predominant organism isolated in children with peritonitis and pneumonia. On logistic regression analysis, serum albumin < 1.5gm/dL was the only independent risk factor for all infections (OR 2.6; 95% CI, 1.2-6; p = 0.01), especially for peritonitis (OR 29; 95% CI, 3-270; p = 0.003). There were four deaths (2.5%) in our study, all due to sepsis and multiorgan failure. Conclusions: Infection remains an important cause of morbidity and mortality in children with nephrotic syndrome. As Pneumococcus was the most prevalent cause of infection in those children, attention should be paid to the pneumococcal immunization in children with nephrotic syndrome.


RESUMO Introdução: Crianças com síndrome nefrótica apresentam maior risco de infecções devido ao próprio status da doença e ao uso de vários agentes imunossupressores. Em grande parte, as infecções desencadeiam recidivas que exigem hospitalização, com risco aumentado de morbidade e mortalidade. Este estudo teve como objetivo determinar a incidência, o espectro e os fatores de risco para infecções graves em crianças hospitalizadas com síndrome nefrótica. Métodos: Todas as crianças hospitalizadas consecutivamente entre 1 e 12 anos de idade com síndrome nefrótica foram incluídas no estudo. Crianças com nefrite aguda, síndrome nefrótica secundária, bem como aquelas admitidas para biópsia renal diagnóstica e infusão intravenosa de ciclofosfamida ou rituximabe foram excluídas. Resultados: Foram cadastradas 148 crianças com 162 internações. A incidência de infecções graves em crianças hospitalizadas com síndrome nefrótica foi de 43,8%. A peritonite foi a infecção mais comum (24%), seguida por pneumonia (18%), infecção do trato urinário (15%) e celulite (14%), contribuindo com dois terços das principais infecções. Streptococcus pneumoniae (n = 9) foi o organismo predominantemente isolado em crianças com peritonite e pneumonia. Na análise de regressão logística, a albumina sérica < 1,5gm / dL foi o único fator de risco independente para todas as infecções (OR 2,6; 95% CI, 1,2-6; p = 0,01), especialmente para peritonite (OR 29; IC95% 3 -270, p = 0,003). Houve quatro mortes (2,5%) em nosso estudo, todas devido a sepse e falência de múltiplos órgãos. Conclusões: A infecção continua sendo uma importante causa de morbimortalidade em crianças com síndrome nefrótica. Como o Pneumococo foi a causa mais prevalente de infecção nessas crianças, deve-se atentar para a imunização pneumocócica em crianças com síndrome nefrótica.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Hospitalization/statistics & numerical data , Infections/mortality , Infections/epidemiology , Nephrotic Syndrome/complications , Peritonitis/blood , Cellulitis/complications , Cellulitis/microbiology , Cellulitis/epidemiology , Incidence , Albumins/analysis , Hospitalization/trends , Immunosuppressive Agents/adverse effects , India/epidemiology , Infections/etiology , Multiple Organ Failure/mortality , Multiple Organ Failure/epidemiology , Nephrotic Syndrome/diagnosis
10.
J. bras. nefrol ; 41(3): 436-439, July-Sept. 2019. graf
Article in English | LILACS | ID: biblio-1040244

ABSTRACT

Abstract Introduction: The posterior reversible encephalopathy (PRES) syndrome encompasses a set of clinical-radiological findings associated with severe systemic arterial hypertension. This case report proposes to discuss the identification, diagnosis, and management of PRES in the pediatric population. Case presentation: Female patient, 10 years old, admitted to the emergency room with complaint of oliguria and generalized edema. At the initial physical exam, the only alteration present was anasarca. The diagnostic investigation revealed nephrotic syndrome, and clinical treatment was started. She evolved on the 8th day of hospitalization with peak hypertension, sudden visual loss, reduced level of consciousness, nystagmus, and focal seizures requiring intubation. She was transferred to the Intensive Care Unit, with neurological improvement, after the established therapy. CT scan revealed a discrete hypodense area in the white matter of the occipital lobe and anteroposterior groove asymmetry, compatible with PRES. Discussion: PRES is due to vasogenic cerebral edema of acute or subacute installation. Symptoms include headache and altered consciousness, stupor, coma, neurological deficits, seizures and cortical blindness. Nephropathies are the main cause of PRES in pediatrics. Magnetic resonance imaging with diffusion of molecules is the gold standard for diagnosis. The initial treatment objectives are the reduction of blood pressure, antiepileptic therapy, correction of hydroelectrolytic and acid-base disorders and management of intracranial hypertension. Conclusion: PRES is associated with acute hypertension. Early diagnosis and proper management may determine a better prognosis and minimize the severity of the clinical course.


Resumo Introdução: A Síndrome de Encefalopatia Posterior Reversível (SEPR) engloba um conjunto de achados clínico-radiológicos, associados a hipertensão arterial sistêmica grave. Este relato de caso propõe discutir a identificação, o diagnóstico e o manejo de SEPR na população pediátrica. Apresentação do caso: Paciente do sexo feminino, 10 anos, admitida em pronto-atendimento com queixa de oligúria e edema generalizado. Ao exame físico inicial, a única alteração presente era anasarca. A investigação diagnóstica revelou síndrome nefrótica, iniciando-se tratamento clínico. Evoluiu no 8º dia de internação com pico hipertensivo, perda visual súbita, redução do nível de consciência, nistagmo e crises convulsivas focais, demandando intubação. Foi transferida para Unidade de Terapia Intensiva, com melhora neurológica, após a terapêutica instituída. Tomografia de crânio evidenciou área de hipodensidade discreta em substância branca do lobo occipital e assimetria anteroposterior de sulcos, compatível com SEPR. Discussão: A SEPR decorre de edema cerebral vasogênico de instalação aguda ou subaguda. Sintomas descritos incluem cefaleia e alteração de consciência, estupor, coma, déficits neurológicos, convulsões e cegueira cortical. As nefropatias constituem as principais causas de SEPR em pediatria. A ressonância magnética com difusão de moléculas é o padrão-ouro para o diagnóstico. Os objetivos iniciais são a redução dos níveis pressóricos, terapia antiepiléptica, correção de distúrbios hidroeletrolíticos e do equilíbrio ácido-básico, e manejo da hipertensão intracraniana. Conclusão: A SEPR ocorre associada à hipertensão aguda. O diagnóstico precoce e manejo adequado podem determinar melhor prognóstico e minimizar a gravidade do curso clínico.


Subject(s)
Humans , Female , Child , Intracranial Hypertension/complications , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Hypertension/complications , Nephrotic Syndrome/complications , Tomography, X-Ray Computed , Treatment Outcome , Intracranial Hypertension/therapy , Diffusion Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome/drug therapy , Hypertension/drug therapy , Anticonvulsants/therapeutic use , Antihypertensive Agents/therapeutic use
11.
Rev. Assoc. Med. Bras. (1992, Impr.) ; 65(7): 988-992, July 2019. tab, graf
Article in English | LILACS | ID: biblio-1013006

ABSTRACT

SUMMARY OBJECTIVE The objective of this study was to investigate the effects of low triiodothyronine syndrome (LT3S) on platelet function and clotting factors in patients with nephrotic syndrome(NS). METHODS Patients with primary nephrotic syndrome were divided into two groups, normal thyroid function (group A) and LT3S (group B), based on whether they had LT3S or not. Healthy subjects were selected as the control group (group C). Blood coagulation function was detected in each group. The platelet activation function (CD62P, CD63) was determined by flow cytometry. The platelet aggregation rate was detected by an optical method using adenosine diphosphate and arachidonic acid as inducers. RESULTS The proportion of primary nephrotic syndrome with LT3S was 23.2% (69/298). Compared with group C, group A had higher CD62P and PAgTADP, and group B had higher CD62P, CD63, PAgTAA, and PAgTADP; the difference was statistically significant (all P < 0.05). There was no significant difference in renal pathology between group A and group B (X2 = 4.957, P = 0.421). Compared with group A, the 24-hour urine protein, CD63, PAgTAA, and PAgTADP were higher in group B, and APTT and Alb were lower. The difference was statistically significant (P < 0.05). Logistic regression analysis showed that LT3S was associated with CD36 (OR: 3.516; 95% CI: 1.742~8.186; P = 0.004) and PAgTAA (OR: 0.442; 95% CI: 1.001~1.251; P = 0.037). CONCLUSION NS patients are prone to LT3S. Patients with LT3S may have abnormal platelet activation and increase of platelet aggregation.


RESUMO OBJETIVO O objetivo deste estudo foi investigar os efeitos da síndrome do baixo triiodotironina (LT3S) na função plaquetária e nos fatores de coagulação em pacientes com síndrome nefrótica (SN). MÉTODOS Pacientes com síndrome nefrótica primária foram divididos em dois grupos, função tireoidiana normal (grupo A) e LT3S (grupo B), com base na presença ou não de LT3S. Indivíduos saudáveis foram selecionados como grupo de controle (grupo C). A função de coagulação do sangue foi analisada em cada grupo. A função de ativação plaquetária (CD62P, CD63) foi determinada por citometria de fluxo. A taxa de agregação plaquetária foi detectada por um método óptico usando adenosina difosfato e ácido araquidônico como indutores. RESULTADOS A proporção de síndrome nefrótica primária com LT3S foi de 23,2% (69/298). Em comparação com o grupo C, o grupo A apresentou níveis mais altos de CD62P e PAgTADP, e o grupo B apresentou maiores CD62P, CD63, PAgTAA e PAgTADP; a diferença teve significância estatística (P < 0,05). Não houve diferença significativa na patologia renal entre o grupo A e o grupo B (X2 = 4,957, P = 0,421). Em comparação com o grupo A, a proteína em urina de 24 horas, CD63, PAgTAA e PAgTADP foram maiores no grupo B, já APTT e Alb foram mais baixos. A diferença apresentou significância estatística (P < 0,05). A análise de regressão logística mostrou uma associação entre LT3S e CD36 (OR: 3,516; 95% IC: 1,742~8,186; P = 0,004) e PAgTAA (OR: 0,442; 95% IC: 1,001~1,251; P = 0,037). CONCLUSÃO Pacientes com síndrome nefrótica estão propensos à síndrome do baixo triiodotironina (LT3S). Pacientes com LT3S podem ter ativação plaquetária anormal e aumento da agregação plaquetária.


Subject(s)
Humans , Male , Female , Adult , Triiodothyronine/blood , Blood Platelets/physiology , Euthyroid Sick Syndromes/physiopathology , Euthyroid Sick Syndromes/blood , Nephrotic Syndrome/physiopathology , Nephrotic Syndrome/blood , Platelet Count , Platelet Function Tests , Reference Values , Triiodothyronine/deficiency , Platelet Activation/drug effects , Platelet Activation/physiology , Platelet Aggregation/drug effects , Platelet Aggregation/physiology , Regression Analysis , Flow Cytometry , Middle Aged , Nephrotic Syndrome/complications
12.
Rev. medica electron ; 41(3): 681-697, mayo.-jun. 2019. graf
Article in Spanish | LILACS | ID: biblio-1094076

ABSTRACT

RESUMEN Las glomerulopatías agrupan varias nefropatías con lesiones fundamentalmente del corpúsculo renal y que se expresan principalmente por proteinuria, hematuria, edemas e hipertensión arterial. La presentación clínica varía en dependencia del tipo de enfermedad de que se trate. Constituye la causa más frecuente de enfermedad renal crónica en adultos jóvenes, por lo que su estudio resulta imprescindible sobre todo para el nivel primario de salud. El propósito fue actualizar consideraciones pertinentes sobre la conducta diagnóstica y terapéutica integral ante una glomerulopatía y valorar emisión de recomendaciones al respecto. Se realizó una búsqueda, análisis y síntesis de información a través de Bases de datos ScieLO Cuba, ScieLO regional, Pubmed, Cumed, Clinical Key en el período 2012-2017 con las palabras clave: síndrome nefrótico, glomerulonefritis, diagnóstico, terapéutica, atención integral. El abordaje en las glomerulopatías es integral, multidisciplinario e individualizado. En Cuba constituyen la cuarta causa de enfermedad renal crónica y predomina el síndrome nefrítico agudo postinfeccioso. El método clínico juega en ello un papel trascendental a la hora de reconocer y registrar sus aspectos clínicos, su etiología, su fisiopatología, y los exámenes complementarios que confirman su presencia o sus complicaciones, así como un tratamiento oportuno que garanticen el perfeccionamiento asistencial. El arma más poderosa ante el reto de los trastornos glomerulares es la visión integradora y con enfoque individual y social protagonizado por el médico ante este grupo de nefropatías en adultos.


ABSTRACT Glomerulopathies encompass a group of several renal disorders with lesions, mainly in the renal corpuscle, expressed in proteinuria, hematuria, edemas and arterial hypertension. Their clinical manifestations change in dependence of the kind of disease. They are the most frequent cause of chronic renal disease in young adults; therefore their study is very important above all in the health care primary level. The aim was updating pertinent considerations on the diagnostic behavior and comprehensive therapy in the case of glomerulopathy, and evaluating the emission of recommendations regarding to them. A search, analysis and synthesis of information was carried out in the databases ScieLO Cuba, ScieLO regional, Pubmed, Cumed, and Clinical Key in the period 2012-2017, using the key words nephrotic syndrome, glomerulonephritis, diagnosis, therapeutics, comprehensive care. The approach to glomerulopathies is comprehensive, multidisciplinary and individualized. They are the fourth cause of chronic renal disease; the acute post-infectious nephritic syndrome predominates. The clinical method plays a transcendental role at the moment of recognizing and registering their clinical characteristics, etiology and physiopathology, while complementary tests confirm their presence or complications, and therefore an opportune treatment guarantying the healthcare improvement. The most powerful weapon against the challenge of the glomerular disorders is the integrated vision with an individual and social approach led by the physician in the case of these nephropathies in adults.


Subject(s)
Humans , Young Adult , Urination Disorders , Diabetes Mellitus/etiology , Renal Insufficiency, Chronic/etiology , Glomerulonephritis/complications , Glomerulonephritis/diagnosis , Glomerulonephritis/etiology , Glomerulonephritis/pathology , Glomerulonephritis/blood , Glomerulonephritis/therapy , Glomerulonephritis/epidemiology , Hypertension/etiology , Kidney/physiology , Kidney/physiopathology , Kidney/pathology , Kidney/diagnostic imaging , Kidney Glomerulus/physiopathology , Nephrotic Syndrome/complications , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/etiology , Nephrotic Syndrome/pathology , Nephrotic Syndrome/blood , Nephrotic Syndrome/therapy , Nephrotic Syndrome/epidemiology , Primary Health Care , Nephrosis, Lipoid
13.
Rev. argent. endocrinol. metab ; 56(1): 70-79, mar. 2019.
Article in Spanish | LILACS | ID: biblio-1041761

ABSTRACT

Las hormonas tiroideas tienen acciones renales directas en conjunto con efectos dinámicos y cardiovasculares que influyen en la función renal. Cuadros de hipotiroidismo con enfermedad renal son una combinación peculiar y poco descrita, es por ello que el propósito de este trabajo es exponer el caso de dos pacientes con síndrome nefrótico asociado a hipotiroidismo severo, los cuales presentaron disminución de la proteinuria y casi normalización de función renal luego de iniciar tratamiento de reemplazo con levotiroxina.


Thyroid hormones have direct renal actions in conjunction with dynamic and cardiovascular effects that influence renal function. Cases of primary hypothyroidism with renal disease are a peculiar and poorly described combination, is for it that the objective of this work is to expose the cases of two patients with nephrotic syndrome associated with severe hypothyroidism, whom presented decreased proteinuria and almost normalized renal function after starting replacement therapy with levothyroxine.


Subject(s)
Humans , Male , Middle Aged , Thyroxine/pharmacology , Hypothyroidism/complications , Nephrotic Syndrome/complications , Thyroid Hormones/physiology , Thyroid Hormones/metabolism
14.
J. bras. nefrol ; 40(4): 418-422, Out.-Dec. 2018. tab, graf
Article in English | LILACS | ID: biblio-984577

ABSTRACT

ABSTRACT Complications are rare in pediatric cases of idiopathic nephrotic syndrome (NS). Thromboembolism ranks among the most uncommon and difficult complications to diagnose, particularly in the first episode of NS, since clinical signs might be unspecific. This report describes the case of a 5-year-old girl with NS for the first time presenting with severe hypoalbuminemia (< 2g/dL). The patient responded poorly to therapy with corticosteroids. On day 8 of hospitalization she started having headaches and vomiting; she did not present hemodynamic alterations, fever or exanthems, and her neurological parameters were normal. The patient was suspected for intracranial hypertension, and computed tomography scans revealed she had cerebral venous sinus thrombosis (CVST). She was started on anticoagulants and showed clinical signs of improvement. The patient had no evident prothrombotic risk factors. She had three other episodes since she was diagnosed, one in which her plasma antithrombin level was low. Although antithrombin levels were normal in her first episode, she was tested after the resolution of proteinuria. The low levels of antithrombin seen in the first recurrence might have mirrored the initial drop in plasma antithrombin levels, an idea supported by the severe hypoalbuminemia she had when diagnosed. This severe manifestation of acquired thrombophilia might be in the origin of CVST. This report presents a rare case of thromboembolic complication in a pediatric patient with NS. The patient progressed well since she was started on anticoagulants. Although she did not present any evident risk factors at first, the development of her case indicated that severe acquired thrombophilia might have worked as the pathophysiological mechanism leading to CVST.


RESUMO A Síndrome Nefrótica (SN) idiopática em crianças pode, raramente, complicar-se. O tromboembolismo é uma das complicações mais raras, principalmente no primeiro episódio, e de diagnóstico mais difícil, uma vez que a clínica pode ser inespecífica. Descrevemos o caso de uma criança de 5 anos com episódio inaugural de SN, destacando-se hipoalbuminemia inicial grave (< 2g/dL). Apresentou fraca resposta inicial à corticoterapia e, após 8 dias de internamento, iniciou quadro de cefaleias e vômitos, sem alterações hemodinâmicas, sem febre, sem exantema e com exame neurológico normal. Perante a suspeita de hipertensão intracraniana, foi realizada TC-CE, que mostrou trombose venosa cerebral (TVC). Foi então iniciada terapêutica anticoagulante com posterior boa evolução clínica. Trata-se de uma criança sem fatores de risco pró-trombóticos evidentes. Desde o diagnóstico, teve 3 recaídas, uma das quais com níveis baixos de antitrombina, que no episódio inaugural eram normais, apesar de avaliados já numa fase não proteinúrica. Suspeita-se, assim, que esse déficit plasmático em antitrombina na recaída poderá mimetizar a queda plasmática inicial, hipótese também apoiada pela hipoalbuminemia grave ao diagnóstico. Esta trombofília grave adquirida poderá ter sido mecanismo etiológico para a trombose venosa cerebral. O interesse deste caso prende-se com a raridade de complicações tromboembólicas na SN Pediátrica, ainda mais raras no episódio inaugural. Nesse caso, a boa evolução foi possível após a associação da terapêutica anticoagulante. Embora sem fatores de risco iniciais evidentes, a evolução do caso permitiu a suspeita de uma trombofília adquirida grave como mecanismo fisiopatológico do tromboembolismo cerebral.


Subject(s)
Humans , Female , Child, Preschool , Sinus Thrombosis, Intracranial/etiology , Nephrotic Syndrome/complications
15.
J. pediatr. (Rio J.) ; 94(5): 546-553, Sept.-Oct. 2018. tab, graf
Article in English | LILACS | ID: biblio-975984

ABSTRACT

Abstract Objective: There is evidence of an important role of immune system changes in the triggering and maintenance of idiopathic nephrotic syndrome (INS). The aim of this study was to investigate the expression of cytokines in lymphocyte populations of patients with INS in comparison to healthy individuals, according to proteinuria. Methods: This cross-sectional study included 44 patients with INS and eight healthy children, matched for age and sex (controls). Patients were subdivided according to proteinuria: persistent proteinuria or partial remission (PP ≥ 300 mg/24 h, n = 17) and low proteinuria or complete remission (LP < 300 mg/24 h, n = 27). Ex vivo analysis of peripheral blood leukocytes by flow cytometry was performed using surface markers for T-lymphocytes, TCD4, TCD8, natural killer (NK) cells, NKT, and B-lymphocytes. Frequencies of intracellular cytokines were analyzed in these cells. Results: The frequencies of B-lymphocytes, NK cells, and NKT cells were lower in INS than in controls, whereas INS patients had a higher frequency of CD4+tumor necrosis factor (TNF)-α+ cells than controls. Cytotoxic-T-lymphocytes expressing IFN-γ were lower in INS than in controls. Patients with PP showed higher frequencies of CD4-T-lymphocytes expressing IFN-γ and TNF-α than controls. CD8-lymphocytes expressing TNF-α were increased in PP group when compared with LP and controls, while CD8+interferon (IFN)-γ+ cells were lower than in LP and in controls. Conclusion: Regardless the level of proteinuria, INS patients had increased expression of TNF-α in CD4-lymphocytes and reduced expression of IFN-γ in CD8-lymphocytes. Persistence of proteinuria was associated with higher levels of inflammatory markers.


Resumo Objetivo Há comprovação do importante papel das alterações no sistema imunológico no desencadeamento e manutenção da síndrome nefrótica idiopática (SNI). O objetivo deste estudo foi investigar a expressão das citocinas em populações de linfócitos de pacientes com SNI em comparação a indivíduos saudáveis e de acordo com a proteinúria. Métodos Este estudo transversal incluiu 44 pacientes com SNI e oito crianças saudáveis, pareados por idade e sexo (controles). Os pacientes foram subdivididos de acordo com a proteinúria: proteinúria persistente ou remissão parcial (PP ≥ 300 mg/24 h, n = 17) e proteinúria baixa ou remissão completa (PB < 300 mg/24 h, n = 27). A análise ex vivo de leucócitos no sangue periférico por citometria de fluxo foi feita utilizando marcadores de superfície para linfócitos T, TCD4, TCD8, células natural killer (NK), linfócitos NKT e B. As frequências das citocinas intracelulares foram analisadas nessas células. Resultados A frequência dos linfócitos B, células NK e células NKT foi menor em pacientes com SNI do que nos controles, ao passo que os pacientes com SNI apresentaram maior frequência de células CD4+fator de necrose tumoral (TNF)-α+ do que nos controles. Os linfócitos T citotóxicos que expressam interferon (IFN)-γ foram menores nos pacientes com SNI do que nos controles. Os pacientes com PP mostraram maiores frequências de linfócitos T CD4 que expressam IFN-γ e TNF-α que os controles. Os linfócitos CD8 que expressam TNF-α apresentaram aumento no grupo com PP, em comparação aos com PB e os controles, apesar de as células CD8+IFN-γ+ serem mais baixas nos pacientes com PB e nos controles. Conclusão Com relação ao nível de proteinúria, os pacientes com SNI apresentaram aumento na expressão de TNF-α nos linfócitos CD4 e expressão reduzida de IFN-γ nos linfócitos CD8. A persistência da proteinúria foi associada a maiores níveis de marcadores inflamatórios.


Subject(s)
Humans , Male , Female , Child , Adolescent , Proteinuria/etiology , Killer Cells, Natural/immunology , T-Lymphocytes/immunology , Cytokines/immunology , Nephrotic Syndrome/immunology , Proteinuria/immunology , Proteinuria/blood , Biomarkers , Case-Control Studies , Cross-Sectional Studies , Cytokines/blood , Disease Progression , Flow Cytometry , Leukocyte Count , Nephrotic Syndrome/complications , Nephrotic Syndrome/blood
16.
J. pediatr. (Rio J.) ; 93(2): 142-147, Mar.-Apr. 2017. tab
Article in English | LILACS | ID: biblio-841342

ABSTRACT

Abstract Objectives: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). Methods: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex. All of the participants were subjected to complete history taking, thorough clinical examination, laboratory investigations (serum creatinine, blood urea nitrogen [BUN], phosphorus [P], total and ionized calcium [Ca], parathyroid hormone [PTH], and alkaline phosphatase [ALP]). Bone mineral density was measured at the lumbar spinal region (L2-L4) in patients group using dual-energy X-ray absorptiometry (DXA). Results: Total and ionized Ca were significantly lower while, serum P, ALP, and PTH were higher in SSNS and SRNS cases than the controls. Osteopenia was documented by DXA scan in 11 patients (44%) and osteoporosis in two patients (8%). Fracture risk was mild in six (24%), moderate in two (8%), and marked in three (12%) of patients. Conclusion: Bone mineralization was negatively affected by steroid treatment in children with NS.


Resumo Objetivos: Avaliar a densidade mineral óssea (DMO) em crianças com síndrome nefrótica idiopática (SNI) e com taxa de filtração glomerular (TFG) normal. Métodos: O estudo transversal de caso-controle foi feito com 50 crianças: 25 casos de SNI [16 sensíveis a esteroides (SNSE) e nove resistentes a esteroides (SNRE) com acompanhamento na unidade de nefrologia pediátrica do hospital da Menoufia University, centro de cuidados terciário] foram comparados com 25 controles saudáveis do grupo de controle com idade e sexo equivalentes. Todos os participantes foram submetidos a anamnese completa, exame clínico completo, exames laboratoriais [creatinina sérica, nitrogênio ureico no sangue (BUN), fósforo (P), cálcio (Ca) total e ionizado, paratormônio (PTH) e fosfatase alcalina (ALP)]. A densidade mineral óssea foi mensurada na região da coluna lombar (L2-L4) no grupo de pacientes com a absorciometria por raios X de dupla energia (DXA). Resultados: Os níveis de cálcio total e ionizado eram significativamente menores, ao passo que o fósforo sérico, a FA e o PTH eram maiores em casos de SNSE e SNRE do que nos controles. A osteopenia foi documentada pelo exame DXA em 11 pacientes (44%) e a osteoporose em dois (8%). O risco de fratura era leve em seis (24%), moderado em dois (8%) e acentuado em três (12%). Conclusão: A mineralização dos ossos foi afetada negativamente pelo tratamento com esteroides em crianças com SNI.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Osteoporosis/etiology , Bone Density/physiology , Nephrotic Syndrome/complications , Osteoporosis/diagnosis , Osteoporosis/physiopathology , Osteoporosis/blood , Case-Control Studies , Cross-Sectional Studies , Risk Factors , Glomerular Basement Membrane , Nephrotic Syndrome/physiopathology , Nephrotic Syndrome/blood
17.
Rev. chil. pediatr ; 86(5): 366-372, oct. 2015. ilus, tab
Article in Spanish | LILACS | ID: lil-771652

ABSTRACT

El síndrome nefrótico idiopático es la glomerulopatía más frecuente en la infancia, afecta a 1-3/100 mil niños menores de 16 años y se presenta con más frecuencia entre los 2 y 10 años. Su causa es desconocida, y la mayoría de las veces responde a corticoides, con buen pronóstico a largo plazo. El síndrome nefrótico corticorresistente representa un 10-20% de los síndromes nefróticos idiopáticos en pediatría. Tiene mal pronóstico, y su manejo constituye un desafío terapéutico significativo. La mitad de los pacientes evoluciona a insuficiencia renal crónica terminal en un plazo de 5 años, estando expuestos además a las complicaciones secundarias a un síndrome nefrótico persistente y a efectos adversos de la terapia inmunosupresora. El objetivo fundamental del tratamiento es conseguir una remisión completa, pero una remisión parcial se asocia a una mejor sobrevida renal que la falta de respuesta. Este documento surgió de un esfuerzo colaborativo de la Rama de Nefrología de la Sociedad Chilena de Pediatría con el objetivo de ayudar a los pediatras y nefrólogos infantiles en el tratamiento del síndrome nefrótico idiopático en pediatría. En esta segunda parte, se discute el manejo del síndrome nefrótico corticorresistente, así como de las terapias no específicas.


Idiopathic nephrotic syndrome is the most common glomerular disease in childhood, affecting 1 to 3 per 100,000 children under the age of 16. It most commonly occurs in ages between 2 and 10. Its cause is unknown, and its histology corresponds to minimal change disease in 90% of cases, or focal segmental glomerulosclerosis. Steroid-resistant nephrotic syndrome represents 10-20% of idiopathic nephrotic syndrome in pediatrics. It has a poor prognosis, and its management is a significant therapeutic challenge. Half of patients evolve to end-stage renal disease within 5 years, and are additionally exposed to complications secondary to persistent NS and to the adverse effects of immunosuppressive therapy. The primary goal of treatment is to achieve complete remission, but even a partial remission is associated with a better renal survival than the lack of response. This paper is the result of the collaborative effort of the Nephrology Branch of the Chilean Society of Pediatrics with aims at helping pediatricians and pediatric nephrologists to treat pediatric idiopathic nephrotic syndrome. In this second part, handling of steroid-resistant nephrotic syndrome as well as nonspecific therapies are discussed.


Subject(s)
Humans , Child , Glomerulosclerosis, Focal Segmental/therapy , Nephrosis, Lipoid/therapy , Nephrotic Syndrome/therapy , Pediatrics , Prognosis , Remission Induction , Glomerulosclerosis, Focal Segmental/physiopathology , Chile , Kidney Failure, Chronic/prevention & control , Nephrosis, Lipoid/physiopathology , Nephrotic Syndrome/complications , Nephrotic Syndrome/physiopathology
18.
Rev. bras. parasitol. vet ; 24(1): 95-100, Jan-Mar/2015. tab
Article in English | LILACS | ID: lil-744664

ABSTRACT

Neosporosis is an emergent disease responsible for considerable economic impact due to reproductive losses. Its zoonotic potential remains unknown. This study involved a survey of antibodies to Neospora caninum in slaughtered sheep and their association with epidemiological variables. Serum samples from 596 sheep from the states of São Paulo and Rio Grande do Sul, Brazil, were collected in two slaughterhouses located in São Paulo and evaluated by indirect fluorescence antibody test (IFAT), using cut-off titers of 25. Among these samples, 353/596 (59.23%; 95%CI 55.23-63.10) were positive and 263/353 (74.50%; 95%CI 69.71-78.77%) were from Rio Grande do Sul. Statistical associations were determined in the univariate analysis between the serological results and sex, breed and municipality of origin. Sheep that came from extensive breeding system showed higher chance (OR=2.09) of presenting antibodies to N. caninum in relation to those from semi-intensive system. Higher chance was also observed for the different studied breeds, except Bergamácia, in relation to Hampshire Down. The results revealed the presence of infection by N. caninum in sheep from slaughterhouses.


A neosporose é uma doença emergente responsável por considerável impacto econômico devido a perdas reprodutivas e seu potencial zoonótico permanece desconhecido. Este estudo envolveu a pesquisa de anticorpos para Neospora caninum em ovinos abatidos e sua associação com variáveis epidemiológicas. Amostras de soro sanguíneo de 596 ovinos, procedentes dos estados de São Paulo e Rio Grande do Sul, Brasil, foram colhidas em dois abatedouros localizados em São Paulo, e avaliadas pelo teste de reação de imunofluorescência indireta (RIFI), utilizando-se como ponto de corte o título de 25. Dentre essas amostras, 353/596 (59,23%; IC95% 55,23-63,10) foram positivas e dentre os ovinos positivos 263/353 (74,50%; IC95% 69,71-78,77%) eram procedentes do Rio Grande do Sul. Associações estatísticas foram determinadas na análise univariada entre os resultados sorológicos e o sexo, raça e município de origem. Ovinos oriundos de sistema de criação extensivo demonstraram maior chance (OR=2.09) de apresentarem anticorpos para N. caninum em relação aos de sistema semi-intensivo. Uma maior chance também foi observada para as diferentes raças estudadas, exceto Bergamácia, em relação à raça Hampshire Down. Os resultados revelaram a presença de infecção por N. caninum em ovinos de abatedouros.


Subject(s)
Adolescent , Humans , Male , Syphilis/diagnosis , Exanthema/etiology , Nephrotic Syndrome/complications , Syphilis/complications
19.
Rev. bras. ter. intensiva ; 26(4): 430-434, Oct-Dec/2014. graf
Article in Portuguese | LILACS | ID: lil-732914

ABSTRACT

A síndrome nefrótica associa-se a um estado de hipercoagulabilidade, apresentando risco aumentado de complicações tromboembólicas. A trombose dos seios venosos cerebrais é uma complicação rara da síndrome nefrótica, com poucos casos descritos na literatura, mas com diagnósticos cada vez mais frequentes. A verdadeira incidência pode estar subestimada, uma vez que muitos eventos são assintomáticos ou não são diagnosticados a tempo. Descrevemos aqui o caso de uma criança do sexo masculino, de 2 anos e 10 meses, com síndrome nefrótica, que apresentou, na evolução, cefaleia, crises epilépticas e rebaixamento sensorial, com o diagnóstico de trombose do seio sagital superior e transverso. Foi realizada revisão da literatura internacional por meio de estratégia de busca definida, nas bases de dados PubMed, SciELO e Lilacs, utilizando os termos “nephrotic syndrome” e “cerebral sinovenous thrombosis”. O diagnóstico de trombose venosa deve ser considerado em qualquer paciente com síndrome nefrótica que manifeste sinais e sintomas neurológicos, destacando que a suspeita clínica precoce tem relação com um desfecho favorável.


Nephrotic syndrome is associated with a hypercoagulable state and an increased risk of thromboembolic complications. Cerebral venous sinus thrombosis is a rare complication of nephrotic syndrome, with few cases described in the literature, although the disease may be under-diagnosis. The true incidence of cerebral venous sinus thrombosis may be underestimated because many events are asymptomatic or are not diagnosed in time. Here, we describe the case of a male child, 2 years and 10 months old, with nephrotic syndrome presenting with headache, epileptic seizures and sensory inhibition who was diagnosed with superior sagittal and transverse sinuses thrombosis. An international literature review was performed with a defined search strategy in the PubMed, SciELO and Lilacs databases using the terms ‘nephrotic syndrome’ and ‘cerebral sinovenous thrombosis’. The diagnosis of venous thrombosis should be considered in any patient with nephrotic syndrome who presents with neurological signs and symptoms, as early clinical diagnosis promotes favorable outcomes.


Subject(s)
Child, Preschool , Humans , Male , Nephrotic Syndrome/complications , Sinus Thrombosis, Intracranial/etiology , Headache/etiology , Seizures/etiology , Sinus Thrombosis, Intracranial/diagnosis
20.
J. bras. nefrol ; 35(3): 191-199, jul.-set. 2013. ilus, tab
Article in English | LILACS | ID: lil-687820

ABSTRACT

INTRODUTION: Steroid resistant idiopathic nephrotic syndrome (SRINS) in children is one of the leading causes of progression to chronic kidney disease stage V (CKD V)/end stage renal disease (ESRD). OBJECTIVE: The aim of this retrospective study is to evaluate the efficacy of immunosuppressive drugs (IS) and to identify risk factors for progression to ESRD in this population. METHODS: Clinical and biochemical variables at presentation, early or late steroid resistance, histological pattern and response to cyclosporine A (CsA) and cyclophosfamide (CP) were reviewed in 136 children with SRINS. The analyzed outcome was the progression to ESRD. Univariate as well as multivariate Cox-regression analysis were performed. RESULTS: Median age at onset was 5.54 years (0.67-17.22) and median follow up time was 6.1 years (0.25-30.83). Early steroid-resistance was observed in 114 patients and late resistance in 22. Resistance to CP and CsA was 62.9% and 35% respectively. At last follow-up 57 patients reached ESRD. The renal survival rate was 71.5%, 58.4%, 55.3%, 35.6% and 28.5% at 5, 10, 15, 20 and 25 years respectively. Univariate analysis demonstrated that older age at onset, early steroid-resistance, hematuria, hypertension, focal segmental glomerulosclerosis (FSGS), and resistance to IS were risk factors for ESRD. The Cox proportional-hazards regression identified CsAresistance and FSGS as the only predictors for ESRD. CONCLUSION: Our findings showed that CsA-resistance and FSGS were risk factors for ESRD.


INTRODUÇÃO: A síndrome nefrótica idiopática córtico-resistente (SNICR) é uma das principais causas de falência renal crônica (FRC)/doença renal crônica estadio V (DRC V) em crianças. Objetivo: Avaliar a resposta aos imunossupressores e identificar fatores de risco para a FRC. MÉTODOS: Variáveis clínicas e bioquímicas na apresentação, resistência inicial ou tardia aos esteroides, lesão histológica e resposta à ciclosporina A (CsA) e à ciclofosfamida (CF) foram analisados retrospectivamente em 136 crianças com SNICR. O desfecho analisado foi a progressão para FRC e os métodos utilizados foram a análise univariada e a regressão multivariada de Cox. RESULTADOS: A idade mediana do início da doença foi de 5,54 anos (0,67-17,22) e o tempo mediano de seguimento foi de 6,1 anos (0,25-30,83). Resistência inicial aos esteroides ocorreu em 114 pacientes e tardia em 22. Resistência à CF e à CsA ocorreu em 62,9% e 35% dos pacientes, respectivamente. FRC ocorreu em 57 pacientes. A sobrevida renal foi de 71,5%, 58,4%, 55,3%, 35,6% e 28,5% aos 5, 10, 15, 20 e 25 anos, respectivamente. A análise univariada demonstrou que a idade maior ao início da doença, resistência inicial aos esteroides, hematúria, hipertensão, glomeruloesclerose segmentar e focal (GESF) e resistência aos imunossupressores foram fatores de risco para FRC. A regressão de Cox identificou a resistência à CsA e a GESF como os únicos fatores preditores para FRC. CONCLUSÃO: Nossos achados mostraram que a resistência à ciclosporina e a presença de GESF foram fatores de risco para a progressão para DRCV.


Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Cyclophosphamide/therapeutic use , Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Kidney Failure, Chronic/etiology , Nephrotic Syndrome/congenital , Cohort Studies , Disease Progression , Follow-Up Studies , Nephrotic Syndrome/complications , Nephrotic Syndrome/drug therapy , Retrospective Studies , Risk Factors , Time Factors
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