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Background & objectives: Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome manifested by features of nephritic syndrome and progressive loss of renal function over a short time. The objective of this study was to investigate the relationship between neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR) and prognostic factors and pathological findings of renal biopsy in RPGN. Methods: Consecutive newly diagnosed RPGN patients who had follow up for at least six months were retrospectively analyzed. The estimated glomerular filtration rate (eGFR) was calculated. Albumin, C-reactive protein (CRP) levels and CRP/albumin ratio were also calculated. Results: Fifty four patients were included in the study. The mean age was 48.92±20.12 years. Clinicopathological diagnosis was pauci-immune glomerulonephritis (GN) in 40 while two had postinfectious GN, six systemic lupus erythematosus, three IgA nephropathy, two Henoch-Schönlein purpura and one membranoproliferative GN. The mean NLR was 7.02±6.34 and mean PLR was 273.90±39.15. Positive correlations between NLR and CRP levels (P=0.009, r=0.511) and CRP/albumin ratios (P=0.005, r=0.542) were observed. PLR and CRP/albumin ratios (P=0.041, r=0.412) were correlated positively. The per cent of fibrocellular crescents was negatively correlated with NLR (P=0.019, r=?0.291), and positively correlated with the lymphocyte count (P=0.05, r=0.256). In secondary crescentic subgroup, the per cent of fibrinoid necrosis had a positive correlation with PLR (P=0.013, r=0.642). Both NLR (P=0.036) and PLR (P=0.051) detected at the first month of the treatment period, were observed to be significantly correlated with mortality. Interpretation & conclusions: This study showed that NLR could predict mortality in patients with RPGN; correlated with systemic inflammation; showed a negative correlation with the per cent of fibrocellular crescents and could be regarded as a measure of glomerular inflammatory state. Moreover, PLR may be considered to be an indicator of disease severity in acute phase of crescentic GN.
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PURPOSE: Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A beta-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. METHODS: Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A beta-hemolytic streptococcal infection were evaluated. RESULTS: Six(12.5%) patients showed elevation of serum creatinine level but there was no patient with persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9%). Hematuria disappeared within 6 months(79%) and proteinuria within 6 months(100%) from the disease onset. CONCLUSION: Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4%) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequela.
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Criança , Humanos , Biópsia , Pressão Sanguínea , Proteínas do Sistema Complemento , Creatinina , Glomerulonefrite , Hematúria , Hipertensão , Falência Renal Crônica , Prognóstico , Proteinúria , Insuficiência Renal , Infecções EstreptocócicasRESUMO
Immunosuppressive medications after renal allograft transplantation have impacted the course of acute and chronic rejection: however, they have no defined effects on the prevention of recurrent and Glomerulonephritis (GN) in an allograft kidney. Authors experienced a case of rapidly progressive glomerulonephritis (RPGN). The 35-year-old female patient developed a rapid deterioration of renal function 4 years after renal transplantation. The allograft biopsy showed crescentic glomerulonephritis evolving from membranoproliferative glomerulonephritis (MPGN) type I. She was given pulse steroid and oral cyclophosphamide therapy immediately after the renal biopsy. Graft function stabilized and proteinuria decreased even though graft function did not recover to pre-treatment level and low grade proteinuria persisted.
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Adulto , Feminino , Humanos , Aloenxertos , Biópsia , Ciclofosfamida , Glomerulonefrite , Glomerulonefrite Membranoproliferativa , Transplante de Rim , Rim , Proteinúria , TransplantesRESUMO
OBJECTIVES: Rapidly progres s ive glomerulonephritis (RPGN) is a clinico- pathologic entity characterized by extens ive crescent formation(usually involving 50% or more of glomeruli) as the principal his tologic finding and a rapid deterioration of kidney function, which can lead to end s tage renal disease within a few weeks. T he etiology and incidence of RPGN has been well defined in Europe and North America, however, there has been no report of a large series in Korea. T he aim of the present s tudy was to analyze the etiology and clinico- pathologic features of 26 patients with RPGN, seen during 1983-1997. METHODS: T wenty-six patients with RPGN(crescents in > 50% of glomeruli) were obs erved during a period of las t 14 years. Male to female ratio was 1:1.4, and the mean age was 30(6-75) years. Mean time from the initial symptoms to the ESRD was 3.1 months . RESULTS: The incidence of RPGN in our series was 2.1% of primary glomerulonephritis. Immunecomplex mediated disease was presented in 14 cases (54%), including 6 sys temic lupus erythematos us, 3 post- streptococcal glomerulonephritis, 3 Henoch- Schonlein purpura, and 2 IgA nephropathy. Pauci- immune disease was presented in 12 cases (46%), including 3 Wegener' s granulomatos is, one necrotizing crescentic glomerulonephritis, and 8 idiopathic crescentic glomerulonephritis. However, there was none of anti-GBM- mediated disease in our s tudy. ANCA were found in 6 patients. All 3 patients with WG were C- ANCA pos itive, whereas one patient with PSGN, necrotizing cres centic GN, and idiopathic crescentic GN were P- ANCA pos itive, respectively. Initial clinical and laboratory features included edema(80%), hypertens ion(72%), oliguria(68%), a decreased renal function(serum creatinine > 5mg/dL, 35%), and gros s hematuria(36%). Renal biopsy showed large crescents more than 80% of the glomeruli in 14 cases (54%) which were predominantly fibrocellular. Fifteen patients (58%) were treated with prednis olone alone, and 12 of them received puls e doses of corticosteroids. Five patients were treated with prednisolone and cyclophos phamide IV pulse. Two cases received plasma exchange. During the mean follow-up of 31+/-37 months, 18 patients (69%) developed inexorable progression of renal failure, three(12%) showed recovery of renal function, and two(8%) showed partial improvement, which is followed by varying degrees of renal insufficiency. During follow-up, three patients died : two from res piratory failure with severe pulmonary hemorrhage and one from opportunistic pulmonary infection during immunosuppressive therapy. Poor prognos is is as sociated with hypertension, increased serum creatinine level at the time of diagnosis, large crescents more than 85% of glomeruli, and glomerular scleros is . CONCLUSION: We conclude that an earlier diagnos is including kidney biopsy and the more aggressive treatment are essential in the management of RPGN.
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Feminino , Humanos , Masculino , Corticosteroides , Anticorpos Anticitoplasma de Neutrófilos , Biópsia , Creatinina , Diagnóstico , Europa (Continente) , Seguimentos , Glomerulonefrite , Glomerulonefrite por IGA , Hemorragia , Hipertensão , Doenças do Sistema Imunitário , Incidência , Rim , Falência Renal Crônica , Coreia (Geográfico) , América do Norte , Troca Plasmática , Prednisolona , Púrpura , Insuficiência Renal , Vasculite SistêmicaRESUMO
RPGN is a catastrophic form of acute glomerulonephritis characterized by an abrupt onset and rapid deterioration of renal function resulting in oliguria within weeks or months. RPGN is seen in a variety of systemic disorders, including systemic lupus erythematosus, poly arteritis nodosa, Wegener's granulomatosis and subacute bacterial endocarditis. In addition, RPGN is seen in association with a variety of primary renal diseases such as poststreptococcal glomerulonephritis, membranoproliferative glomerulonephritis, and IgA nephropathy, Goodpasture's syndrome. Toxic epidermal necrolysis(TEN) is a drug induced life threatening disease characterized by extensive epidermal detachment, necrosis, and mucosal erosion. TEN may involve liver, lung, intestine, and kidney. But renal involvement has seldom been reported. We report on a 63-year-old patient who developed a RPGN with a TEN. Renal biopsy showed pauci-immune crescentric glomerulonephritis and skin biopsy showed edematous change with extravasated erythrocytes in upper dermis and several individually necrotic keratinocytes. ANCA and FANA test was negative. Our patient recovered renal function with steroid pulse therapy. The pathophysiology of TEN is unresolved but abnormal cytokine release(e.g., tumor necrosis factor) has been implicated in pathogenesis of TEN. Because various cytokines have direct toxic effect on kidney structure, the tubular and glomerular damage may be related to the cytokines involved in TEN. To our knowledge, this is the first case documenting the presence of RPGN in patients with TEN. And there maybe some relations between PRGN and TEN which require further study.
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Humanos , Pessoa de Meia-Idade , Doença Antimembrana Basal Glomerular , Anticorpos Anticitoplasma de Neutrófilos , Arterite , Biópsia , Citocinas , Derme , Endocardite Bacteriana Subaguda , Eritrócitos , Glomerulonefrite , Glomerulonefrite por IGA , Glomerulonefrite Membranoproliferativa , Intestinos , Queratinócitos , Rim , Fígado , Pulmão , Lúpus Eritematoso Sistêmico , Necrose , Oligúria , Pele , Síndrome de Stevens-Johnson , Granulomatose com PoliangiiteRESUMO
Pregnancy complicated with systemic lupus erythematosus (SLE) is notoriously variable in its presentation, course, and outcome. Renal involvement is demonstrated in half of patients. Exaggerated by pregnancy on renal function is not concluded. But renal failure is one of the leading causes of death. We report a case of lupus complicating pregnancy that no prepregnancy remission. She developed clinically pre-eclampsia and lupus nephritis in 17 weeks gestation. Flares of disease with deterioration of renal function despite corticosteroid therapy lead to stop pregnancy. Although pregnancy has been terminated, she became worse and died before starting hemodialysis. Histologic study from renal necropsy and clinical manifestation consistented with rapidly progressive glomerulonephritis (RPGN).