Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis

 Between 1980 and 1994, 38 children with severe forms of Sch?nlein-Henoch purpura glomerulonephritis were entered into a prospective study to evaluate methylprednisolone pulse therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological changes. The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome and/or had 50% or more crescentic glomeruli. Initial renal biopsies were obtained from all patients and revealed diffuse proliferative endocapillary glomerulonephritis in 2, focal and segmental glomerulonephritis in 4, and endo- and extracapillary glomerulonephritis in 32, 21 of whom had 50% or more glomeruli with crescents. Patients were treated with intravenous pulse methylprednisolone (3 days) followed by oral prednisone (3.5 months). At the latest follow-up, 1–16 years after initiation of therapy, 27 children had clinically recovered, 3 showed minimal urinary abnormalities, 4 persistent nephropathy, and 4 had progressed to end-stage renal failure. Sequential renal biopsies were obtained from 30 patients, 7–25 months after initiation of therapy. The clinical outcome correlated well with of the activity (hypercellularity, cellular and fibrocellular crescents, and interstitial edema with mononuclear cell infiltrates) and the chronicity (fibrous crescents, glomerular sclerosis, tubular atrophy, and interstitial fibrosis) indexes of post-therapy biopsies. Of particular interest were the post-therapy biopsies of the 18 patients who clinically recovered. They showed a significant decrease of the activity index from 5.1±1.1 to 0.4±0.8 with a decrease or even a disappearance of IgA deposits, while the chronicity index remained low (0.4±0.8 compared with 1.4±1). Although uncontrolled, our study suggests that methylprednisolone pulse therapy is effective in those patients at risk of progression of their nephropathy, especially if started early during the course of the disease before the crescents become fibrous. Received February 26, 1997; received in revised form and accepted June 15, 1997     

Outcome of pediatric renal transplants in a developing country

This study was conducted to evaluate the outcome of pediatric renal transplants at our center. A retrospective analysis was done on 39 pediatric transplants (age at transplant <18 years) done at our center over the last 10 years. The mean age at transplant was 15.6±1 years (10–17 years). They comprised 4.2% of all renal transplants done at our center (39/921) over the period. Girls comprised 17.5% of total recipients (n=7). Two patients had a preemptive transplant. The underlying causes of end stage renal disease were chronic glomerulonephritis (n=21), chronic interstitial nephritis (n=17) and Alport syndrome [1]. All the 39 children were initiated on triple drug immunosuppression (cyclosporin A (CsA) azathioprine, prednisolone). All patients received grafts from living related donors. In the first month, three patients had graft loss (serum creatinine, SCr, >5 mg/dl). Of these, two patients died because of septicemia and one had acute cortical necrosis. There was evidence of infection in 16 patients (40%). Acute rejection was seen in 17 patients (45.8%). The 1-year patient and graft survival was 89% and at 3 years 70%. The actuarial graft survival at 5 years was 50%. Twelve children discontinued CsA after 1 year post-transplant and five of these had graft loss. Graft losses were significantly greater in patients who discontinued CsA as compared to those who continued CsA (5/12 vs 2/22). After a mean follow-up of 31.5±3.5 months, of the 37 patients, 10 had graft loss and chronic graft dysfunction was observed in another 9 patients. The rest of the 17 (48%) patients had a mean SCr of 1.2 mg/dl. The long-term outcome of pediatric renal transplants in our country remains suboptimal. CsA discontinuation due to financial constraints and/or non-compliance remain the most important reasons for this.     

Etiology and outcome of chronic renal failure in Indian children

A prospective analysis of all new pediatric cases of chronic renal failure (CRF) was performed at our hospital over a 1-year period. The diagnosis of CRF was based on serum creatinine >2 mg/dl with supportive clinical, laboratory, and radiological findings. There were a total of 48 patients with CRF with a median age of 13 years (range 10 days to 16 years). The causes of CRF included glomerulonephritis (37.5%), obstruction and interstitial (52%), hereditary (6.3%), and undetermined (4.2%). Patients were symptomatic for a mean of 33.2 months (range 10 days to 11 years) at presentation. Eight patients (16.7%) had acute reversible deterioration of renal function at presentation. This was due to accelerated hypertension in 2, infection in 3, volume depletion in 2, and nonsteroidal antiinflammatory drugs in 1 patient. At presentation, 22 (46%) children had mild to moderate renal failure and 26 (54%) had end-stage renal disease. Twenty-one children (43.7%) had associated illness at presentation. Mean follow-up was 22.9 weeks (range 2–126 weeks). At the end of the study period, 10 (21%) patients were on conservative treatment, 7 (14.6%) on maintenance dialysis, 8 (16.7%) patients had functioning allografts, 4 (8.3%) patients had died, and 19 (39.6%) opted against further therapy. We conclude that CRF in Indian children carries a poor prognosis due to late referral and the limited availability and high cost of renal replacement therapy. Received: 31 July 1998 / Revised: 7 December 1998 / Accepted: 13 December 1998     

Crescentic glomerulonephritis in children

Data on patients with crescentic glomerulonephritis (>50% glomeruli with crescents), referred to the Hospital for Sick Children during the past 13 years, were reviewed. Thirty patients (13 male, 17 female) aged 3.7–15.7 years (mean 9.5) were evaluated. Initial clinical features included: oedema (24/30), hypertension (19/30), gross haematuria (15/30), oliguria (15/30) and a decreased glomerular filtration rate (GFR<30 ml/min per 1.73 m²) (22/30). Henoch-Schönlein purpura was present in 9 patients, microscopic polyarteritis in 3, polyarteritis nodosa in 1, Wegener's granulomatosis in 1, systemic lupus erythematosus in 1, post-streptococcal glomerulonephritis in 2, mesangiocapillary glomerulonephritis in 7, anti-glomerular basement membrane glomerulonephritis in 2, and 4 were idiopathic. In 10 patients 50%–79% of glomeruli were affected by crescentic changes (group 1) and in the remaining 20, 80% or more (group 2). The crescents were cellular, fibrocellular or fibrous, and the degree of sclerosis was assessed. Patients in both groups were treated with plasma exchange, corticosteroids, anticoagulants, cyclophosphamide and azathioprine in different combinations. On follow-up, 3 patients were dead, 1 was lost to follow-up, 12 were on dialysis/transplant programmes, 4 had a GFR of less than 30 and 10 a GFR of more than 30 ml/min per 1.73 m². In our experience, 50% progressed to end-stage renal failure. The interval between disease onset and start of treatment was a prognostic factor for outcome. Fibrous crescents were associated with a worse outcome than fibrocellular crescents (P<0.05). Outcome was not, however, related to the percentage of glomeruli affected (P>0.05). Although the effectiveness of the individual components of the treatment regimens used was difficult to assess, one-third of patients at the latest follow-up had a GFR of more than 30 ml/min per 1.73 m².     

Acute Postinfectious Crescentic Glomerulonephritis: Clinicopathologic Presentation and Risk Factors

Background: Glomerular crescent formation is a feature of the most severe forms of human glomerulonephritis. The postinfectious form of rapidly progressive glomerulonephritis with crescents is a form of immune complex glomerulonephritis which seem to have a better prognosis. A relatively poorer prognosis for crescentic postinfectious glomerulonephritis in South Africa has been reported. In the present study, we have tried to determine the mode of presentation, and the prognostic factors for renal and patient outcome for cases with postinfectious crescentic glomerulonephritis (CGN). Methods:Between 1990 and 2000 a total number of 128 patients with CGN were managed at our center, among them 23 cases were diagnosed as postinfectious CGN. They were followed-up for a mean period of 40.1 ± 28.9 months. Among them 12 were males and 11 were females. The median age was 12.35 years (range 4–55 years). The median serum creatinine at presentation was 7.24 mg/dl (range 1.3–14.5 mg/dl). We studied the clinical, laboratory and histopathological data ^.of our cases and their impact on the renal and patient outcome. Results:By univariate study the risk factors for renal dysfunction were the age, hypertension, and nephrotic range proteinuria during the follow-up period. By multivariate analysis only the, hypertension, and presence of nephrotic range proteinuria during the follow-up period were the significant risk factors. The risk factors that significantly affected patient mortality were hypertension and serum creatinine at last follow-up. Conclusion: postinfectious CGN is a severe form of glomerulonephritis that usually presents with rapidly progressive renal failure. The persistence of hypertension and nephrotic range proteinuria during the follow-up are major bad prognostic predictors for renal dysfunction.     

Progression of renal failure in patients with renal disease of diverse etiology on protein-restricted diet

The rate of progression of early renal failure was evaluated in three groups of adult patients with renal disease of diverse etiology on dietary protein and phosphorus restriction (about 0.6 g/kg of protein, 700 mg of phosphorus) and in a control group of 22 patients with the same renal disease, retrospectively studied, on a free diet. Group 1 had 33 patients with chronic glomerulonephritis (CG), initial serum creatinine (Scr) of 1.4 to 4.3 mg/dl (mean, 2.20), followed for 5 to 94 months (mean, 44). Group 2 had 17 patients with polycystic kidney disease (PKD), Scr 1.3 to 4.7 mg/dl (mean, 2.40), followed for 8 to 81 months (mean, 42). Group 3 had 28 patients with primary chronic pyelonephritis (CP), Scr of 1.5 to 4.5 mg/dl (mean, 2.57), followed for 9 to 92 months (mean, 41). The control group had 22 patients (11 with CG, five with PKD, and six with CP), with Scr 1.7 to 4.1 mg/dl, followed for 6 to 72 months (mean, 24). In the regression analysis between reciprocal creatinine and time, the slopes were -0.0017, -0.0025, and -0.00016 dl/mg/month in the three patient groups on a protein-restricted diet, respectively. The difference between both groups 1 and 2 and group 3 was statistically significant (P less than 0.05). The slopes in patients on a free diet were significantly greater than those found in patients on a protein-restricted diet. The actuarial survival probability at 72 months, assuming as "renal death" a Scr of 10 mg/dl, was 45% in patients with CG, 44% in those with PKD, and 67% in those with CP on a protein-restricted diet.(ABSTRACT TRUNCATED AT 250 WORDS)     

Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis in children

Two cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing and crescentic glomerulonephritis are reported. A 12-year-old girl and a 10-year-old boy presented with polyarthritis, anaemia, haematuria, proteinuria, impaired renal function, anorexia, nausea, marked loss of weight and lethargy. The boy also had a vasculitic rash and anterior uveitis. Both children had diffuse cytoplasmic ANCA identified by indirect immunofluorescence and confirmed by specific enzyme-linked immunosorbent assay. Renal biopsies showed severe focal and segmental necrotizing glomerulonephritis with 100% crescents. They were treated with plasma exchange, prednisolone, cyclophosphamide and heparin. Within 1 month of commencing treatment, both had normal serum creatinine concentrations and ANCA was not detectable. Renal biopsies 6 weeks following commencement of treatment revealed quiescent disease, although up to 40% of glomeruli were sclerosed or had fibrous crescents. Following cessation of cyclophosphamide and heparin after 7 months and reduction in steroid dose, a biopsy at 10 months in the boy revealed quiescent disease, but the girl had recurrent disease associated with reappearance of a low titre of ANCA and small cellular crescents in 20% of the glomeruli. These cases reflect the potential usefulnes of ANCA determination for categorizing paediatric patients, helping in the selection of therapy and as a possible marker of disease activity, similar to the experience in adults.     

Postinfectious glomerulonephritis in the elderly

Postinfectious glomerulonephritis (PIGN) is primarily a childhood disease that occurs after an upper respiratory tract infection or impetigo; its occurrence in older patients is not well characterized. Here, we report 109 cases of PIGN in patients ≥65 years old diagnosed by renal biopsy. The male to female ratio was 2.8:1. An immunocompromised background was present in 61%, most commonly diabetes or malignancy. The most common site of infection was skin, followed by pneumonia and urinary tract infection. The most common causative agent was staphylococcus (46%) followed by streptococcus (16%) and unusual gram-negative organisms. Hypocomplementemia was present in 72%. The mean peak serum creatinine was 5.1 mg/dl, and 46% of patients required acute dialysis. The most common light microscopic patterns were diffuse (53%), focal (28%), and mesangial (13%) proliferative glomerulonephritis. IgA-dominant PIGN occurred in 17%. Of the 72 patients with ≥3 months of follow-up (mean, 29 months), 22% achieved complete recovery, 44% had persistent renal dysfunction, and 33% progressed to ESRD. The presence of diabetes, higher creatinine at biopsy, dialysis at presentation, the presence of diabetic glomerulosclerosis, and greater tubular atrophy and interstitial fibrosis predicted ESRD. In summary, the epidemiology of PIGN is shifting as the population ages. Older men and patients with diabetes or malignancy are particularly at risk, and the sites of infection and causative organisms differ from the typical childhood disease. Prognosis for these older patients is poor, with fewer than 25% recovering full renal function.     

Crescentic glomerulonephritis in children: a review of 43 cases.

Forty-three children with crescentic glomerulonephritis (GN), having large crescents in more than 50% of the glomeruli, were observed during a period of 22 years. There were 17 boys and 26 girls between the ages of 3.5 and 14 years (mean 8.7 +/- 2.6). Thirty-one patients (72%) presented with acute nephritic features and increasing renal insufficiency (rapidly progressive GN) whereas 12 had an insidious onset with nephrotic syndrome, or rarely with nonspecific symptoms. Eleven patients had evidence of poststreptococcal GN and 6 an underlying systemic disorder. Renal biopsy showed large crescents in greater than 80% of the glomeruli in 38 cases (100% in 28) which were predominantly fibrocellular or fibrous in 80% of the patients. Nineteen patients (44%) were treated with prednisolone, cyclophosphamide and dipyridamole; in addition, 8 were also given anticoagulants. Six patients received pulse doses of corticosteroids. In 23 patients, there was inexorable progression of renal failure, 14 showed partial improvement but subsequently had varying degrees of renal insufficiency and in 6, there was recovery of renal function with normal levels of serum creatinine. Of the latter, 4 had received immunosuppressive anticoagulant therapy and 2 only supportive care. Of 11 patients with poststreptococcal crescentic GN, 7 progressed to end-stage renal disease and 2 developed chronic renal insufficiency. Our findings confirm the poor outcome of crescentic GN in children, irrespective of the underlying etiology. In a small proportion of cases, the disorder may have an insidious onset and a slowly progressive course, but an equally grave prognosis.     

Henoch-Schoenlein nephritis in adults: a clinical and morphological study

Renal prognosis is not clear in adults with Henoch-Schoenlein nephritis (HSN). Renal biopsy material from seventeen adult patients with HSN was studied by light-, electron-, and immunofluorescent microscopy, and a clinicopathologic correlation was made. The outstanding glomerular lesion was a mesangial IgA deposition, apart from the proliferative glomerulonephritis associated with segmental lesions or crescents. At the time of biopsy five patients (29%) presented with renal insufficiency complicated by nephrotic syndrome and/or hypertension. After a mean follow-up period of 3.2 years, ten patients showed complete recovery, two had minor urinary abnormalities, and five exhibited moderate proteinuria with or without hematuria. No patients had died nor developed chronic renal failure. Our data indicate that the outcome of HSN in adults is favorable similar to that in children. No initial clinical nor pathological features could be associated with a poor prognosis in this study. Further follow-up is needed in view of the unpredictable nature of this disease.     

Renal histopathology and clinical course in 94 patients with Wegener's granulomatosis.

BACKGROUND: The main purpose of this study was to examine histopathological changes seen in renal biopsies from patients with Wegener's granulomatosis (WG) with varying degrees of renal involvement and to study possible correlations between the morphological variables and the severity of the disease. METHODS: Ninety-four patients with WG and active renal disease were included in this retrospective study. All patients had a percutaneous renal biopsy taken on their first admission to the hospital and 14 patients had a second biopsy. The patients were followed for a median of 42.5 months (range 0.5-184). RESULTS: Segmental necrotizing glomerulonephritis and extracapillary proliferation were present in 85.1 and 91.5% respectively. Of seven patients (7.4%) with normal serum creatinine and urinary protein excretion <0.5 g/day, all had crescents and six had segmental glomerular necrosis. Serum creatinine at biopsy correlated significantly with the percentage of glomeruli with crescents (rho=0.52, P=0.0004), with necrosis (rho=0.36, P=0.002) and with the percentage of normal glomeruli (rho=-0.55, P=0.0003). On a multivariate analysis, only the percentage of normal glomeruli was significantly associated with renal function and development of end-stage renal disease. In 14 second biopsies after a mean of 41.2 (+/-26) months, chronicity scores had increased significantly in 13 biopsies in spite of full immunosuppressive treatment. CONCLUSION: Although renal biopsy is of value in defining renal involvement in WG, it is of limited help in the early stage of the disease in predicting renal outcome for the individual patient. A follow-up biopsy can be useful in revealing the degree of activity and chronicity and hence be of importance for the choice of further therapy.     

Long-term outcome of adult patients with minimal urinary abnormalities and normal renal function.

AIM: To define the long-term outcome of patients with minimal urinary abnormalities (defined by the presence of microscopic hematuria with no or less than 1 gm/day proteinuria), and normal renal function (defined by a serum creatinine < 1.3 mg/dl), we retrospectively studied patients who fulfilled the above criteria and had a kidney biopsy done before the year of 1992 (i.e. at least followed up for 5 years), with a definite pathological diagnosis. METHODS: A total of 41 cases among 719 cases of primary glomerulonephritis (5.7%) were enrolled into the study. There were 19 males and 22 females with a mean age of 35.4+/-14.7 years at biopsy. The duration of renal disease was 116.0+/-60.5 months and the duration of follow-up post biopsy was 100.2+/-38.1 months. The pathological diagnosis was: IgA nephropathy (21 cases), focal glomerulosclerosis (9 cases), mesangial proliferative glomerulonephritis (8 cases), membranous glomerulonephritis (2 cases) and acute glomerulonephritis (1 case). RESULTS: At the end of follow-up, 8 cases (19.5%) had a certain degree of renal insufficiency including 2 (4.9%) in end-stage renal disease. The other cases were either in complete remission (6 cases) or stable condition (27 cases) with persistent microscopic hematuria and normal renal function. The long-term outcome was not correlated with any of the following parameters: age, sex, disease duration, serum creatinine at presentation, daily protein loss at presentation, degree of glomerular change and degree of interstitial inflammatory cell infiltration. However, a poor long-term outcome was correlated with tubular atrophy (p < 0.05) and interstitial fibrosis (p < 0.05). CONCLUSION: We conclude that a minimal urinary abnormality with normal renal function at presentation does not necessarily imply a favorable long-term outcome in certain patients. Tubular atrophy and interstitial fibrosis but not glomerular change correlates with a worse prognosis. This further emphasizes the importance of renal biopsy in such cases.     

Rapidly progressive glomerulonephritis in a patient with Chlamydia pneumoniae infection: a possibility of superantigenic mechanism of its pathogenesis

Herein we describe a case of a patient with rapidly progressive glomerulonephritis after Chlamydia pneumoniae infection. An 88-year-old woman who had had C. pneumoniae infection two months previously was admitted to our hospital with complaints of dyspnea and generalized edema. Laboratory tests revealed acute renal failure, polyclonal hypergammaglobulinemia, highly increased level of C-reactive protein, and hematoproteinuria. A renal biopsy revealed mesangial and endocapillary proliferative glomerulonephritis with crescents. She responded to high-dose steroids, cyclophosphamide, minocycline, and plasma exchange treatment with the remission of oliguric renal failure. The percentage of the subset of CD3+ TCR+ Vbeta11+ cells markedly increased to 9.6% (normal range: < 1.04%) at the onset of the disease and decreased to 0.1% after the treatment. These clinicopathological features were similar to those of superantigen-associated glomerulonephritis after methicillin-resistant Staphylococcus aureus infection. We suggest that the superantigenic mechanism is one of the possible pathomechanisms of this glomerulonephritis.     

Incidence and prognostic importance of glomerular crescents in renal diseases of childhood

The renal biopsies of 372 children with various glomerular disorders were reviewed and crescent formation was seen in 56 cases (15%). Four disorders, i.e. systemic lupus erythematosus, membranoproliferative glomerulonephritis (MPGN) types I and II and Henoch-Sch?nlein disease accounted for 74% of 10 diagnostic categories. Idiopathic rapidly progressive glomerulonephritis (RPGN) was seen in only 2 cases. Crescents associated with MPGN types I or II or idiopathic RPGN had a bad renal prognosis, whereas the presence of crescents in other disorders did not necessarily affect the renal outcome. Immunofluorescent and electron microscopic findings are essential to distinguish many conditions which may be associated with crescent formation in childhood renal disease.     

Patient with antibody-negative relapse of Goodpasture syndrome

Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of hemodialysis treatment. It is important to consider that in patients with anti-GBM disease antibody-negative relapses are possible.     

Etiology,clinical profile and short-term outcome of acute kidney injury in children at a tertiary care pediatric nephrology center in Pakistan

Background: The reported prevalence rates and etiologies of acute kidney injury (AKI) are quite variable in different regions of the world. The current study was planned to determine the etiology, clinical profile, and short-term outcome of pediatric AKI at our hospital.

Methods: A prospective, observational study was carried out from April 2014 to March 2015. All pediatric patients (1 month to?≤15 years) diagnosed as AKI using modified pRIFLE criteria were studied and followed for 3 months to document short-term outcome.

Results: AKI was diagnosed in 116 children. The mean age was 7.5?±?4.4 years and males were predominant (60.3%). At presentation, 83.6% had oliguria/anuria, 37.1% hypertension and 17.2% severe anemia. Etiology included primary renal (74/116; 63.8%), postrenal (28/116; 24.1%) and prerenal (11/116; 9.5%) causes. Postinfectious glomerulonephritis (PIGN) and crescentic glomerulonephritis in primary renal, obstructive urolithiasis in postrenal and sepsis in prerenal, were the most common etiologies. At presentation, 89/116 (76.7%) patients were in pRIFLE Failure category. Regarding outcome, 68 (58.6%) patients recovered, six (5.2%) died, 18 (15.5%) developed chronic kidney disease (CKD) and 22 (19%) end-stage renal disease (ESRD). Comparison of recovered and unrecovered AKI showed that characteristics such as hypertension, severe anemia, edema, volume overload, requirement of mechanical ventilation, initiation of dialysis and need of >5 sessions of dialysis had statistically significant (p?<0.05) association with nonrecovery.

Conclusion: Glomerulonephritides (PIGN and crescentic) and obstructive urolithiasis are major causes of pediatric AKI at our center. A fairly high percentage of cases recovered and these mainly comprised of PIGN and obstructive urolithiasis.     

Clinical and pathologic features of fibrillary glomerulonephritis.

A diagnosis of fibrillary glomerulonephritis was made in 31 renal biopsies from 28 patients on the basis of the electron microscopic identification of glomerular deposits of randomly arranged fibrils that resembled amyloidosis but were larger. This accounted for approximately 1% of all nontransplant renal biopsy diagnoses. Renal biopsy specimens with parallel arrays of 30 nm to 50 nm microtubules (that is, immunotactoid glomerulopathy) were not included in the study. The patients had a mean age of 49 years with a range of 21 to 75. The male to female ratio was 1:1.8 and the ratio of Whites to Blacks was 8.3:1, which differs from the 3:1 ratio in our overall biopsy population. All patients had proteinuria (mean 6.0 g/day), and most had hematuria and renal insufficiency. After a mean follow-up of 24 months, there was 48% renal survival. The light microscopic appearance of the fibrillary glomerulonephritis was quite varied. Capillary wall thickening and matrix expansion were the most frequent alterations. Nineteen percent of specimens had crescents. Morphometric ultrastructural analysis demonstrated a mean fibril diameter of 22.4 +/- 7.4 nm. Immunofluorescence microscopy revealed that IgG was the dominant and often the only immunoglobulin class in immune deposits, and subclass analysis revealed that IgG4 was the dominant or exclusive subclass in all specimens tested. We hypothesize that the relatively homogeneous nature of the immunoglobulin in the immune deposits is the basis for the fibril formation.     

中晚期慢性肾脏病患者肾功能进展危险因素——单中心慢性肾脏病专业门诊队列研究

目的 探讨在慢性肾脏病（CKD）专业门诊管理下CKD 3~5期未透析患者肾功能进展相关危险因素。 方法 采取前瞻性队列研究设计,收集北京大学第一医院CKD专业门诊规律随访的CKD 3~5期未透析患者的血压、血红蛋白、钙磷代谢及蛋白尿等指标控制及肾功能的变化情况,进行肾功能进展的多因素分析。肾功能进展定义为每年估计的肾小球滤过率（eGFR）下降大于4 ml&#8226;min-1&#8226;(1.73 m2)-1、开始肾脏替代治疗和（或）肾脏病相关的死亡。 结果 共纳入138例患者,其中CKD 3期84例,4期36例,5期18例。进入队列时基线年龄为（56.5±16.7）岁,基线eGFR为（32.3±13.4） ml&#8226;min-1&#8226;(1.73 m2)-1,平均随访（27.1±12.1）个月。随访过程中患者平均血压（126.5±12.4）/（76.4±7.9） mm Hg;平均血红蛋白（123.4±17.6）g/L;平均钙磷乘积（45.2±7.7） mg2/dl2。分别有70例（50.7%）血压控制达标;102例（73.9%）血红蛋白控制达标;123例（89.1%）患者钙磷乘积控制达标;62例（44.9%）患者肾功能进展。多因素分析显示,随访过程中蛋白尿和血红蛋白水平与肾功能进展独立相关。 结论 通过CKD专业门诊的一体化治疗,能够有效控制中晚期CKD患者的各种并发症。控制蛋白尿和（或）改善贫血有利于延缓中晚期CKD患者肾功能进展。     

Steroid pulse therapy in rapidly progressive glomerulonephritis]

In an attempt to clarify the indication and efficacy of the methylprednisolone pulse therapy (1000 mg x 3 times) for rapidly progressive glomerulonephritis (RPGN), 3 patients with the disease were carefully followed and the clinical course during and after the treatment were precisely analysed. According to the declination rate of reciprocals of serum creatinine (1/Cr), one patient were divided into the acute type (-1.00 x 10(-2) dl/mg/day or less) and the others into the subacute type (more than -1.00 x 10(-2) dl/mg/day). In the patient of acute type, renal biopsy revealed cellular crescent formation in 93.8% of glomeruli observed. One course of the pulse therapy resulted in a decrease in Cr from 3.0 mg/dl to 1.3 mg/dl and transformation of cellular crescents to fibrocellular or fibrous crescents. In the other two patients of subacute type, crescents were observed in 72.7% and 72.0% of glomeruli observed, and 87.5% and 38.9% of them were composed of cellular crescents respectively. Initial courses of the pulse therapy resulted in decreases of Cr from 3.5 mg/dl to 2.4 mg/dl and from 3.0 mg/dl to 1.4 mg/dl respectively. Additional courses, given because of insufficient reduction of Cr in the former, induced a further lowering to 1.3 mg/dl, and because of re-elevation of Cr to 2.2 mg/dl and remaining of cellular crescents in 20% in the latter, induced a decrease of Cr to 1.5 mg/dl and disappearance of cellular crescents.(ABSTRACT TRUNCATED AT 250 WORDS)     

Glomerular diseases in children

A total of 411 children, aged from 0.3 to 18 years, suffering from glomerular diseases, were studied by renal biopsy between 1976 and 1985. The clinical presentation included nephrotic syndrome (79% of cases), renal failure (43%), and arterial hypertension (38%). In all, 177 cases presented with primary nephrotic syndrome; all had complicated courses and most were either corticosteroid-dependent or-resistant. Only 26.6% had minimal change disease on renal biopsy; 56.5% had focal-segmental sclerosis; and immunofluorescent deposits were observed in half of the group. Acute poststreptococcal (36 cases), mesangiocapillary (80 cases), and lupus (34 cases) glomerulonephritis occurred frequently; IgA glomerulopathy (10 cases) and haemolytic uraemic syndrome (6 cases) were uncommon. Glomerular crescents were observed in 71 cases. These observations illustrate the types of glomerular diseases seen in Iranian children.