Renal disease in type I glycogen storage disease

Although kidney enlargement occurs in Type I glycogen storage disease, renal disease has not been considered a major problem. Death from renal failure in three patients known to us prompted a study of renal function in this disorder. Of the 38 patients with Type I glycogen storage disease under our care, the 18 children under 10 years old had normal renal function. Fourteen of the 20 older patients (13 to 47 years) had disturbed renal function, manifested by persistent proteinuria; many also had hypertension, hematuria, or altered creatinine clearance. Progressive renal insufficiency developed in 6 of these 14 patients, leading to three deaths from renal failure. At the onset of proteinuria, creatinine clearance was increased in seven patients (3.05 +/- 0.68 ml per second per 1.73 m2 of body-surface area; range, 2.47 to 4.13 [normal range, 1.33 to 2.33 ml per second per 1.73 m2]). Renal biopsies were performed in three patients after an average of 10 years of proteinuria. All three biopsies demonstrated focal segmental glomerulosclerosis in various stages of progression. Our data suggest that chronic renal disease is a frequent and potentially serious complication of Type I glycogen storage disease. In addition to treating hypoglycemia vigorously, physicians should monitor renal function carefully in patients with this disorder.     

Comparison of dysmorphic erythrocytes with other urinary sediment parameters of renal bleeding

Dysmorphic erythrocytes have been described in the urine sediment of patients with renal parenchymal bleeding. This study compares the prevalence of dysmorphic erythrocytes in 5,128 urine specimens with blood, erythrocytic, or fibrin casts, proteinuria, and significant numbers of exfoliated renal tubular cells (RTCs). Of the 510 samples containing pathologic casts, 15% had dysmorphic erythrocytes, 60% had proteinuria, 71% had RTCs, and 12% had no other urinalysis abnormality. Of the 186 samples containing dysmorphic erythrocytes, 55% had pathologic casts, 42% had proteinuria, 71% had RTCs, and 13% had no other abnormality. Renal hematuria can best be evaluated by examination of all four of these urinalysis abnormalities rather than using a single entity for diagnosis. Patients with urinalysis evidence of renal hematuria should be evaluated further for renal disease rather than lower urinary tract disorders.     

Differences in Clinical Manifestations and Outcomes between Adult and Child Patients with Henoch-Sch?nlein Purpura

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients.

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Prevalence and pathologic features of sickle cell nephropathy and response to inhibition of angiotensin-converting enzyme.

BACKGROUND. Nephropathy may develop in patients with sickle cell disease. We determined the prevalence of proteinuria and renal insufficiency in a group of patients with sickle cell disease and investigated the renal pathologic changes and the effects of an angiotensin-converting-enzyme inhibitor (enalapril) on protein excretion in patients found to have nephropathy. METHODS. We prospectively screened 381 patients with sickle cell disease for the presence of proteinuria and renal insufficiency. Renal biopsy and measurements of glomerular filtration rate, effective renal plasma flow, and urinary protein excretion were performed in 10 patients with mild nephropathy before and after the administration of enalapril, and again two to three weeks after its discontinuation. RESULTS. Of the 381 patients with sickle cell disease, 26 (7 percent) had serum creatinine concentrations above the normal range and 101 (26 percent) had proteinuria of at least 1+. The renal lesions in the 10 patients who had biopsies consisted of glomerular enlargement and perihilar focal segmental glomerulosclerosis. The mean (+/- SD) glomerular area in these patients was 28.7 +/- 4.1 x 10(3) micron 2, as compared with 15.8 +/- 4.3 x 10(3) micron 2 in 10 control patients without renal disease who had died of trauma (P less than 0.0001). During the administration of enalapril, the mean 24-hour urinary protein excretion decreased 57 percent (range, 23 to 79 percent) below the base-line value (P less than 0.001), and it increased to 25 percent below the base-line value after enalapril was discontinued. The glomerular filtration rate and effective renal plasma flow did not change significantly. CONCLUSIONS. Approximately 25 percent of patients with sickle cell disease have proteinuria. Treatment with enalapril reduces the degree of proteinuria in these patients, suggesting that glomerular capillary hypertension may be a pathogenic factor in sickle cell nephropathy.     

儿童原发性膜性肾病16例病理特点与远期预后

目的了解儿童原发性膜性肾病（IMN）病理特点及其远期预后。方法回顾性收集1979至2010年6月复旦大学附属儿科医院肾脏风湿科经病理诊断为IMN的连续病例为研究对象,分析一般情况、临床表现、病理特点、治疗和随访情况,探讨其远期预后。结果 16例IMN患儿进行分析,占同期肾穿刺活检病例（1710例）的0.94%。男10例,女6例,年龄2～12岁,平均（5.2±2.6）岁。①临床表现以肾病综合征为主（11例,68.8%）,无症状性蛋白尿5例（31.2%）,伴有高血压2例（12.5%）,起病时伴有肾功能不全2例（12.5%）。②14/16例行肾组织电镜检查,其中Ⅰ期6/14例（42.9%）,Ⅰ～Ⅱ期6/14例（42.9%）,Ⅱ期1/14例（7.1%）,Ⅱ～Ⅲ期1/14例（7.1%）。病理学检查均未见肾小管萎缩、间质纤维化等肾小管间质损伤。③未达到大量蛋白尿标准的7例患儿予随访观察,其中1例病情进展予激素和免疫抑制剂治疗;达到大量蛋白尿标准的9例患儿均予足量激素（2mg·kg-1·d^-1）治疗,其中5例激素依赖或耐药加用免疫抑制剂治疗。至2010年6月,2例失访,14例IMN患儿随访12～91个月,平均（34.0±18.7）个月,在起病后3～16个月均达完全缓解,无一例进展至慢性肾脏疾病;2例起病时伴肾功能不全的患儿肾功能均恢复。结论儿童IMN临床表现以肾病综合征为主,小年龄、病理分期较轻且不伴肾小管间质损伤患儿的预后相对较好。     

X-linked recessive nephrolithiasis with renal failure

BACKGROUND AND METHODS. Nephrolithiasis may occur as a consequence of a number of hereditary disorders. We describe a large kindred from northern New York with hereditary nephrolithiasis accompanied by urinary concentrating defects, nephrocalcinosis, renal insufficiency, and renal wasting of potassium, phosphate, calcium, and uric acid. The pattern of inheritance was established by examining the patients and their records and interviewing family members. Selected members of the family were evaluated in detail, with measurements of erythrocyte cation fluxes and carbonic anhydrase (carbonate dehydratase) activity. RESULTS. The kindred consisted of 162 family members from six generations. All nine affected persons were male and appeared to have inherited the disease from their mothers. No affected man transmitted the gene to a son, but the daughters of affected men were carriers. The patients presented in childhood with calcium nephrolithiasis and proteinuria, with progression to nephrocalcinosis, urinary concentrating defects, and renal insufficiency. Renal biopsies revealed tubular atrophy, interstitial fibrosis, and glomerulosclerosis; the characteristic features of other forms of hereditary nephritis were absent. Abnormalities in the renal excretion of calcium, phosphate, potassium, and uric acid were found only in the adult members of the kindred, although renal biopsies were abnormal even in younger members. In one patient who has had a renal transplant for seven years, the disease has not recurred. CONCLUSIONS. This kindred manifested an X-linked recessive nephrolithiasis with renal failure, a new form of hereditary renal disease. Most of the identifiable physiologic abnormalities occurred after the development of nephrolithiasis and renal insufficiency and may not be of pathogenetic importance.     

Clinical and pathological features in Venezuelan children with IgA nephropathy

IgA nephropathy (IgAN) is the most common glomerulonephritis in humans worldwide; its prevalence and prognosis vary according with geographical areas. The incidence is higher in adults under 30 years of age and in children, it occurs more frequently in patients between 3 and 10 years. Hematuria is the predominant manifestation at presentation of the disease and 20-40% of the cases progress to terminal chronic renal disease. Renal biopsies were performed in 426 children during the period 1980-2002, of them, 12 cases corresponded to IgAN. The clinico-pathological characteristics and evolution of patients were evaluated during an average of 3.85 years. Mean age of patients was 6.2 years, and it was more frequent in males. Hematuria and proteinuria were found in 100% of cases and proteinuria of nephrotic range in 75%. Hypertriglyceridemia and hypercholesterolemia in 91%, arterial hypertension in 50% and acute renal failure at presentation in 25%. The predominant histopathological patterns (WHO) were II and III, deposits of mesangial IgA, IgG and C3 were observed in all cases and C4 deposits in 25%. 41.7% of cases had complete remission, 41.7% maintained normal renal function with persistent proteinuria and 16% progressed to terminal chronic renal failure. The actuarial survival of patients was 100% at 3 years, 87% at 4 years and 76% at 8 years. Two patients died during the period of study, at 3.5 and 8.5 years. The variability of presentation of IgA nephropathy was confirmed in this study, which could be attributable to geographical differences, racial influences and clinicopathological features related to sanitary conditions. Despite of the frequency of bad prognosis characteristics at presentation of IgAN in our series, the evolution was similar to reports of other groups.     

Long-term follow-up after partial removal of a solitary kidney

BACKGROUND. The removal of more than one kidney in animals leads to proteinuria and progressive renal failure due to focal segmental glomerulosclerosis. This injury may be the result of chronic glomerular hyperfiltration. The purpose of this study was to determine the effect of a reduction in renal mass of more than 50 percent on residual renal function and morphology in humans. METHODS. We evaluated long-term renal function in 14 patients with a solitary kidney who had undergone partial nephrectomy for renal-cell or transitional-cell carcinoma. In 12, the first kidney had been removed 2 months to 21 years previously for the same type of cancer; in 2, the other kidney was congenitally atrophic. Before surgery, no patient had clinical or histopathological evidence of primary renal disease. All 14 patients underwent partial nephrectomy to remove a localized tumor, with 25 to 75 percent of the solitary kidney being excised. They were evaluated 5 to 17 years after surgery (mean, 7.7). RESULTS. Twelve patients had stable postoperative renal function, and end-stage renal failure developed in two. There were no changes in blood pressure in any patient during follow-up. Nine patients had proteinuria, which was mild (0.15 to 0.8 g of urinary protein per day) in five. The extent of proteinuria was inversely correlated with the amount of remaining renal tissue (P = 0.0065) and directly correlated with the duration of follow-up (P = 0.0005). Four patients with moderate-to-severe proteinuria had renal biopsies, which revealed focal segmental glomerulosclerosis in three patients and global glomerulosclerosis in one. CONCLUSIONS. Long-term renal function remains stable in most patients with a reduction in renal mass of more than 50 percent. These patients are, however, at increased risk for proteinuria, glomerulopathy, and progressive renal failure.     

Colchicine in the prevention and treatment of the amyloidosis of familial Mediterranean fever

To determine whether colchicine prevents or ameliorates amyloidosis in patients with familial Mediterranean fever, we followed 1070 patients with the latter disease for 4 to 11 years after they were advised to take colchicine to prevent febrile attacks. Overall, at the end of the study, the prevalence of nephropathy was one third of that in a study conducted before colchicine was used to treat familial Mediterranean fever. Among 960 patients who initially had no evidence of amyloidosis, proteinuria appeared in 4 who adhered to the prophylactic schedule and in 16 of 54 who admitted non-compliance. Life-table analysis showed that the cumulative rate of proteinuria was 1.7 percent (90 percent confidence limits, 0.0 and 11.3 percent) after 11 years in the compliant patients and 48.9 percent (18.8 and 79.0 percent) after 9 years in the noncompliant patients (P less than 0.0001). A total of 110 patients had overt nephropathy when they started to take colchicine. Among 86 patients who had proteinuria but not the nephrotic syndrome, proteinuria resolved in 5 and stabilized in 68 (for more than eight years in 40). Renal function deteriorated in 13 of the patients with proteinuria and in all of the 24 patients with the nephrotic syndrome or uremia. We conclude that colchicine prevented amyloidosis in our high-risk population and that it can prevent additional deterioration of renal function in patients with amyloidosis who have proteinuria but not the nephrotic syndrome.     

Idiopathic nodular glomerulosclerosis: a clinicopathologic study of 15 cases

Idiopathic nodular glomerulosclerosis is an enigmatic condition closely resembling diabetic nodular glomerulosclerosis without evidence of diabetic mellitus or other specific disease. Idiopathic nodular glomerulosclerosis remains a rare disease entity with an unclear pathogenesis. Clinicopathologic features of 15 patients with idiopathic nodular glomerulosclerosis were evaluated in a retrospective review of renal biopsies between 1998 and 2007. Our study cohort consisted predominantly of older (mean age, 64.2 years) white (73%) women (67%). Fourteen patients (93%) had a history of hypertension, and 10 (67%) were active smokers at the time of biopsy. Nine patients (60%) were obese (body mass index, >30 kg/m²) and 4 (27%) were overweight (body mass index, 25-29.9 kg/m²). Fourteen patients (93%) presented with renal insufficiency with mean serum creatinine level of 2.8 mg/dL. All 15 patients presented with proteinuria (mean urinary protein excretion, 5.6 g/24 h). Eleven patients (73%) presented with nephrotic-range proteinuria and 8 (53%) with nephrotic syndrome. Histopathologic findings showed nodular glomerulosclerosis (100%), moderate to severe arterio-arteriolosclerosis (100%), and glomerular basement membrane thickening (100%). Immunofluorescence and electron microscopy studies had no other specific findings. Our results confirm previous studies of a close association of hypertension and smoking with idiopathic nodular glomerulosclerosis. A significantly higher incidence of obesity and overweight in patients with idiopathic nodular glomerulosclerosis suggests that increased body mass index may also contribute to the development and progression of idiopathic nodular glomerulosclerosis.     

The types of renal disease in the acquired immunodeficiency syndrome

Between January 1982 and December 1986, among the 750 patients with the acquired immunodeficiency syndrome (AIDS) who were treated at two adjacent hospitals in New York City, 78 (10.4 percent) needed evaluation for renal disorders. Reversible acute renal failure due to nephrotoxic injury, ischemic injury, or both was present in 23 patients (30 percent) (Group I). The remaining 55 (70 percent) had massive proteinuria, azotemia, or both (AIDS-associated nephropathy; Group II), and irreversible uremia developed in 43. In an additional 18 patients, all of whom had a history of intravenous narcotic drug use, AIDS was diagnosed after the initiation of maintenance hemodialysis for chronic renal failure (Group III). Survival for more than six months after the onset of chronic uremia occurred in only two subjects in Group II; all patients in Group III died within three months of the diagnosis of AIDS. Death in the patients in Groups II and III followed a syndrome of "failure to thrive" characterized by inanition unresponsive to intensive nutritional support and hemodialysis. In contrast, 8 of 17 patients with acute renal failure (Group I) and a serum creatinine concentration above 6 mg per deciliter regained renal function (serum creatinine level, less than 2.0 mg per deciliter). Four of the seven lived for 10 to 24 months, whereas the other four died of sepsis within a month. Our observations suggest that maintenance hemodialysis is not effective in prolonging life either in patients with AIDS-associated nephropathy and uremia or in patients with end-stage renal failure in whom AIDS develops during the course of maintenance dialysis. Hemodialysis may be useful in the management of potentially reversible acute renal failure in patients with AIDS.     

Spectrum of Renal Abnormalities in Acquired Immune-Deficiency Syndrome

Data from 27 patients with acquired immunedeficiency syndrome (AIDS) and AIDS-related complex (ARC) managed at the University of California, Irvine Medical Center since 1982 were extracted from medical records. Functional renal insufficiency occurred with considerable frequency among the AIDS patients. In contrast renal function was stable in the ARC patients studied. The majority of the AIDS patients exhibited persistent or transient proteinuria. Hematuria, leukocyturia, bacteruria, and nonvenereal urinary tract infections were seen with considerable frequency among AIDS patients and much less frequently among ARC patients. Similarly various disorders of fluids, electrolytes, acid base and abnormal phosphorus and calcium levels were common among AIDS patients and uncommon among ARC patients. These observations point to the prevalence and significance of the renal and associated abnormalities in acquired immune-deficiency syndrome.     

Clinical and histopathological predictors of progressive disease in IgA nephropathy

The clinical course and biopsy findings of twenty-one patients with IgA nephropathy, followed up for a mean period of 37.4 mth (range 24-54 mth) after diagnosis, were reviewed retrospectively to determine whether the clinical presentations, the laboratory findings or histopathologic changes have prognostic implications. An age of 24 yr or above and a serum creatinine of 0.18 mmol/l or above at diagnosis correlated significantly with renal insufficiency at the end of the follow-up (P = 0.023 & 0.03). Proteinuria of 1.5 g/d or more and hypertension (systolic greater than 150 mmHg or diastolic greater than 100 mmHg) when well controlled, were not found to be significant. Asymptomatic proteinuria and gross hematuria, on the other hand, correlated negatively with renal insufficiency at the end of the follow up (P = 0.034). With respect to histopathological changes, greater than 30% global glomerular sclerosis and moderate or marked tubular atrophy correlated significantly with renal insufficiency (P = 0.005 and 0.005). However, less than 10% glomerular crescents, small amounts of mesangial electron dense deposits or absent ultrastructural peripheral glomerular capillary wall abnormalities correlated negatively with renal insufficiency (P = 0.017, 0.03 & 0.03).     

How well do general practitioners manage urinary problems in children? South Bedfordshire Practitioners' Group.

Thirteen general practitioners examined the notes of 1072 patients born in 1974 for evidence of enuresis, suspected urinary tract infection, and renal tract imaging. Of these children 63 (5.9%) had presented with enuresis -6.7% of the boys and 5.0% of the girls. Of the 63 children 65.1% had had midstream urinalysis. One hundred and ninety five children (18.2%, 64 boys and 131 girls) had experienced 303 episodes of possible urinary infections. Midstream urine samples were obtained in 80.2% of episodes and 17.7% of samples were positive. Ten boys (1.9% of the total) and 28 girls (5.2%) had proven infections. Only 14 of these 38 children (36.8%) had undergone renal tract imaging, 30.9% of the boys and 39.3% of the girls. All imaging was normal except in the case of one girl whose micturating cystourethrogram showed reflux. Fifteen other children were investigated; two further abnormalities were detected, one renal scar with reflux and one duplex system. This study demonstrates deficiencies in the investigation and follow up of children with urinary problems by general practitioners. Possible means of improvement are discussed.     

与食用受三聚氰胺污染配方奶粉相关儿童泌尿系统结石的现况调查

目的探讨食用受三聚氰胺污染配方奶粉与儿童泌尿系统结石发生的关系。方法对0～14岁食用受三聚氰胺污染配方奶粉的儿童,通过其家长填写调查问卷、临床问诊、体格检查、尿常规、泌尿系统B超、肾功能、肝功能及其他血、尿生化指标等检查,筛查是否存在泌尿系统结石及其他损害。将受三聚氰胺污染的配方奶粉分为三聚氰胺高含量组和三聚氰胺低含量组,将食用受三聚氰胺污染配方奶粉的时间分为≤30d和〉30d,依据年龄分为≤1岁、～2岁、～3岁、～6岁和～14岁组。分析性别、年龄、配方奶粉中不同三聚氰胺含量和用奶时间对泌尿系统结石发生的影响。分析泌尿系统结石患儿的临床表现、实验室检查结果及病情转归。结果接受筛查儿童22091名,患泌尿系统结石374名,其中男性223名,女性151名,男∶女约为1.5∶1。①泌尿系统结石发生率：≤3岁各年龄组,三聚氰胺高含量组均高于三聚氰胺低含量组（P〈0.001）;～6岁组,三聚氰胺高含量组高于三聚氰胺低含量组（P〈0.05）;～14岁组,三聚氰胺高含量组与三聚氰胺低含量组差异无统计学意义（P〉0.05）。三聚氰胺高含量组各相邻年龄组儿童泌尿系统结石发生率差异均无统计学意义（P〉0.05）;三聚氰胺低含量组除≤1岁组与～2岁组泌尿系统结石发生率差异有统计学意义外（P〈0.05）,其余各相邻年龄组差异均无统计学意义（P〉0.05）。②单侧肾结石309例,双侧肾结石60例,单侧输尿管结石6例,双侧输尿管结石2例,膀胱结石4例,尿道结石2例。③93例住院治疗的泌尿系统结石患儿中,血尿8例（8.6%）、脓尿7例（7.5%）、蛋白尿3例（3.2%）、尿痛或尿哭5例（5.4%）、少尿或无尿2例（2.2%）、水肿2例（2.2%）、血β2-微球蛋白（β2-MG）增高25例（26.9%）、尿β2-MG增高4例（4.3%）;BUN和SCr增高各2例（2.2%）,均无磷酸激酶同工酶和ALT升高。9例需外科治疗,其余内科保守治疗。治愈34例,好转55例,未愈2例,自动出院或转院2例。结论食用受三聚氰胺污染配方奶粉与儿童泌尿系统结石的发生有关,泌尿系统结石发生可能与年龄无关,与食用受三聚氰胺污染配方奶粉相关的泌尿系统结石可能对肾脏有一定损害。     

Nephropathy in type 2 diabetes mellitus in Third World countries--Chandigarh study

We studied the profile of nephropathy in 250 patients, 177 males and 73 females, with type 2 diabetes mellitus. The mean age was 55.9 +/- 8.8 years. Therapy for control of diabetes included diet alone in 1.6%, oral hypoglycaemic agents in 90.6% and insulin in 7.8%. Glycaemic control was satisfactory in 4.8%, fair in 41.2% and poor in 54.0%. Blood sugar values were normal without any therapy in 33 out of the 206 patients (16%) after the onset of renal insufficiency. The mean interval between the onset of diabetes and the appearance of proteinuria was 9.5 +/- 7.05 years. Proteinuria appeared within one year in 23 patients (9.2%), 1-5 years in 32 (12.8%), 6-10 years in 86 (34.4%) and more than 10 years in the remaining 109 patients (43.6%). Proteinuria was of nephrotic range in 17.6% of patients. Renal insufficiency was present in 206 (82.4%) patients and occurred 10.5 +/- 7.5 years after the detection of diabetes. Hypertension was present in 61.2% and was first detected 7.5 +/- 7.4 years after onset of diabetes. Endstage renal disease occurred 11.8 +/- 6.8 years after the onset of diabetes mellitus. Thus, clinical evidence of diabetic nephropathy is present in most patients with type 2 diabetes mellitus within a decade after the detection of diabetes. Subsequent progression to end-stage renal failure is rapid in the face of poorly controlled hypertension and hyperglycemia in the economically poor countries.     

A population study of renal function in sickle cell anemia

To define normal limits for serum creatinine levels, as well as to explore the relationship between age and the prevalence and severity of renal disease in patients with sickle cell anemia (SCA), we retrospectively analyzed renal function parameters in 368 patients followed in our SCA clinic. Dipstick proteinuria was present in 78 patients (20.6%). Chronic renal insufficiency (CRI) was present in 17 patients (4.6%) and showed a high degree of association with proteinuria and increased age. In patients with CRI, the severity of renal dysfunction was also age-related. In the 284 patients without proteinuria or CRI, mean serum creatinine levels were lower than predicted. We conclude that in patients with SCA, serum creatinine levels at the upper limit of normal should be regarded with suspicion, and that the prevalence and severity of proteinuria and CRI in SCA is high and increases with age.     

Thin glomerular basement membrane disease: light microscopic and electron microscopic studies.

Benign recurrent hematuria usually indicates a good prognosis. This condition is associated with abnormally thin glomerular basement membranes. Of 680 renal biopsy cases in which lower urinary tract disease had been excluded by careful study, 25 cases from seven children and eighteen adults met the criteria for thin glomerular basement membrane disease, placing the incidence of the disease at 3.7%. The mean patient age was 32.4 years and the male to female ratio was 1 to 5.3. The primary finding was microscopic hematuria in eighteen patients and gross hematuria in five patients. Among eighteen patients who had microscopic hematuria, one patient also exhibited proteinuria and one patient suffered from acute renal failure due to acute drug-induced interstitial nephritis. Proteinuria was only found in one patient. All of the patients had normal renal function, with the exception of one who suffered from acute renal failure. The duration of hematuria from the time of detection to the date of biopsy ranged from 3 months to 30 years with a mean interval of 56.6 months. No apparent evidence of familial hematuria in any patient was noted. Under light microscopy most glomeruli were normal. However, five cases showed focal global sclerosis. Under immunofluorescence microscopy seventeen cases were negative for all immunoglobulins, for complement, and for fibrinogen. Eight cases showed nonspecific mesangial deposition of fibrinogen and/or IgM. Ultrastructurally, extensive diffuse thinning of the GBM was a constant finding. The mean thickness of the GBM was 203.2 +/- 28.3 nm (n = 25); the thickness in adult (201.4 +/- 27.5 nm; n = 18) did not differ from that in children (208.1 +/- 32.0 nm; n = 7).     

前列腺增生并肾后性肾功能不全的治疗方法

目的：探讨前列腺增生并肾后性肾功能损害的处理方法及手术时机及其术后效果。方法：对30例前列腺增生并肾后性肾功能损害的患者,经留置尿管或耻骨上膀胱穿刺造瘘尿液引流,在肾功能恢复和尿动力检查膀胱逼尿肌功能恢复的前提下行经尿道前列腺电切。结果：经持续尿液引流后肾功均有明显改善,其中22例于1周后成功行前列腺电切,8例行膀胱造瘘后1～2月行前列腺电切术,手术中及术后顺利,均无严重并发症,术后顺访1～2年,排尿通畅满意,肾功能正常。结论：尿液引流是首要处理方法,肾功能恢复及膀胱逼尿肌功能恢复是手术的前提,经尿道前列腺电切是手术的主要方法。