Adult ; Glomerulonephritis, IGA ; etiology ; Hepatitis C ; complications ; Humans ; Male

Crohn's disease is a condition of chronic inflammation potentially involving any location of the alimentary tract from mouth to anus. Numerous extraintestinal manifestations can also be present. Urologic complications of inflammatory bowel disease are seen in up to 25% of patients, but renal parenchymal disease has been rarely reported. IgA nephropathy is recognized worldwide as a most common form of primary glomerulonephritis. Clinical manifestations vary, ranging from microscopic hematuria to nephrotic syndrome. Recently, IgA nephropathy associated with systemic diseases has been reported. We describe a case of a 22 year-old man with Crohn's disease associated with IgA nephropathy. At the age of 8 years, microscopic hematuria appeared. After fourteen years, he presented with melena, mild fever, recurrent oral ulcer, microscopic hematuria and proteinuria. Colonoscopic examination revealed characteristic features of Crohn's disease such as multiple ulcers. Microscopic findings showed superficial ulceration with small noncaseating granulomas. Renal biopsy revealed IgA nephropathy. The patient was treated with oral prednisolone, olsalazine, and metronidazole followed by maintenance therapy with sulfasalazine and azathioprine resulting in clinical improvement of Crohn's disease and IgA nephropathy.
Adult ; Crohn Disease/*complications/pathology ; Glomerulonephritis, IGA/*complications/pathology ; Humans ; Male

Adult ; Atypical Hemolytic Uremic Syndrome ; etiology ; Female ; Glomerulonephritis, IGA ; complications ; Humans ; Puerperal Disorders ; etiology

The combination of idiopathic thrombocytopenic purpura (ITP) and chronic renal failure (CRF) is uncommon. This report highlights a case of renal transplantation in a patient with ITP. A 35-year-old man with ITP was admitted with uremic symptoms. A renal transplant and splenectomy was simultaneously performed. A prophylactic pneumococcous vaccination was performed and intravenous immunoglobulin (1 g/kg) was administered before and after the operation. The patient's platelet count increased gradually after the splenectomy. During a two-year follow up period, the graft function was well maintained. Renal transplantation in a patient with ITP is recommended with a well-designed strategy to prevent potential complications.
Adult ; Glomerulonephritis, IGA/complications ; Humans ; Kidney Failure, Chronic/*complications/etiology/*surgery ; *Kidney Transplantation ; Male ; Purpura, Thrombocytopenic, Idiopathic/*complications

OBJECTIVESeveral studies have suggested that non-diabetic renal disease (NDRD) was common among non-insulin dependent diabetes mellitus (NIDDM) patients with renal involvement.

METHODSThe prevalence of NDRD among Chinese NIDDM population in PUMC hospital center was retrospectively studied. Renal biopsy specimens were evaluated with light-, immunofluorescence- and electron-microscopy. The cohort consisted of 33 NIDDM patients who received renal biopsy.

RESULTSPatients with both isolated diabetic nephropathy (DN, n = 7) and NDRD (n = 22) had comparable duration of DM, creatinine clearance, serum creatinine, albumin and 24 hours proteinuria, as well as incidences of retinopathy, neuropathy and hypertension. Significantly more patients with NDRD had haematuria (P = 0.030) or non-nephrotic proteinuria (P = 0.016). IgA nephropathy accounted for 40.9% of the NDRD identified.

CONCLUSIONSIn this study, haematuria and non-nephrotic proteinuria predicted the presence of NDRD among NIDDM patients complicated with renal disease. IgA nephropathy is the most frequent type of NDRD in Chinese.

Adult ; Aged ; Biopsy, Needle ; Cohort Studies ; Diabetes Mellitus, Type 2 ; complications ; pathology ; Female ; Glomerulonephritis ; complications ; pathology ; Glomerulonephritis, IGA ; complications ; pathology ; Humans ; Kidney ; pathology ; Male ; Middle Aged ; Retrospective Studies

A retrospective study of 223 patients with IgA nephropathy (IgAN) was performed to clarify the prognostic factors and the renal survival rates of the disease. One hundred twenty-two patients were followed-up for more than 6 months after their renal biopsy (mean follow-up duration: 43.0 months), and 20 of them (16.4%) had progressed to end-stage renal disease (ESRD). Using univariate analysis, 8 risk factors (2 clinical and 6 histopathological findings) for developing ESRD were identified: renal insufficiency at initial presentation (serum creatinine > or = 1.5 mg/dl); heavy proteinuria(> or = 3.5 gm/day); moderate to severe histopathologic findings such as class IV/V lesions by W.H.O. classification, mesangial hypercellularity, glomerular sclerosis, interstitial infiltration, interstitial fibrosis, and tubular atrophy. In multivariate regression analysis, class IV/V lesions and renal insufficiency at initial presentation were the independent prognostic factors of IgAN. The renal survival rates were 100% at 1 year, 97.0% at 3 years, and 78.9% at 5 years. In conclusion, it seems that about 20% of IgAN patients have a risk to progress to ESRD within 5 years, and a careful follow-up is recommended especially in patients who have either renal insufficiency at the time of presentation or severe renal pathology (class IV/V lesions).
Adolescent ; Adult ; Female ; Glomerulonephritis, IGA/*complications/pathology ; Human ; Kidney Failure, Chronic/*epidemiology/*etiology/pathology ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

Immunoglobulin A nephropathy (IgAN), which can develop into end-stage renal disease, is the most common primary glomerulonephritis. The pathogenesis of IgAN is not clear. Many studies have confirmed that genetic susceptibility is associated with IgAN, and it belongs to polygenic disease. Some studies have found that IgAN is associated with chromosome 6q22-23, 2q36 by linkage analysis, and several candidate genes have been confirmed to be associated with IgAN, such as angiotensin converting enzyme, Fc fragment of IgA receptor, human leukocyte antigen. In recent years, as the progression of molecular genetics and the Human Genome Project, more attention has been paid to the role of genetic factors in the pathogenesis of IgAN.
Animals ; Chromosomes, Human, Pair 2 ; genetics ; Chromosomes, Human, Pair 6 ; genetics ; Genetic Association Studies ; Genetic Linkage ; Genetic Predisposition to Disease ; genetics ; Glomerulonephritis ; complications ; Glomerulonephritis, IGA ; etiology ; genetics ; Humans

The occurrence of immunoglobulin A nephropathy (IgAN) in patients with noninsulin dependent diabetes mellitus (NIDDM) is a rare event and of pathogenetic interest. It is not clear whether this is merely coincidence. We report here five patients with IgAN in NIDDM associated with or without diabetic glomerulosclerosis. All of the patients were Korean males. In three patients, diabetes mellitus was diagnosed at the same time with diagnosis of IgAN, and the known duration of the diabetes in the other two patients were three and seven years, respectively. There was no evidence of diabetic retinopathy in four patients, but it was found in one patient. In all cases, the diagnosis of IgAN was made by immunohistology.
Adult ; Biopsy ; Case Report ; Complement 3/analysis ; Diabetes Mellitus, Non-Insulin-Dependent/complications* ; Diabetic Nephropathies/pathology* ; Glomerular Mesangium/pathology ; Glomerulonephritis, IGA/pathology* ; Glomerulonephritis, IGA/etiology ; Human ; IgG/analysis ; Kidney Glomerulus/pathology ; Male ; Microscopy, Fluorescence ; Middle Age

PURPOSE: We conducted a multi-center randomized double-blind study to determine the effects of 6-month therapy with sulodexide on urinary protein excretion in patients with idiopathic Immunoglobulin A (IgA) nephropathy. MATERIALS AND METHODS: A total of seventy-seven patients participated in the study. They were randomly allocated to one of three groups: sulodexide 75 mg or 150 mg daily or the placebo for 6 months. The primary end point was the achievement, at 6 months, of at least 50% reduction in urine protein/creatinine ratio (UPCR) from the baseline value. RESULTS: At 6 months, the primary end point was achieved by 12.5% of the patients assigned to the placebo, 4.0% of the patients assigned to sulodexide 75 mg daily and 21.4% of those assigned to 150 mg (p=0.308). Treatment with sulodexide 150 mg daily for 6 months significantly reduced log UPCR from 6.38+/-0.77 at baseline to 5.98+/-0.94 at 6 months (p=0.045), while treatment with sulodexide 75 mg daily and placebo did not. CONCLUSION: A 6-month treatment with sulodexide did not achieve 50% reduction of urinary protein excretion in IgA nephropathy patients, but showed a tendency to increase the time-dependent anti-proteinuric effect. Therefore, long-term clinical trials on a larger scale are warranted to elucidate the hypothesis that sulodexide affords renal protection in IgA nephropathy patients.
Adult ; Anticoagulants/*therapeutic use ; Double-Blind Method ; Female ; Glomerulonephritis, IGA/complications/*drug therapy ; Glycosaminoglycans/*therapeutic use ; Humans ; Male ; Middle Aged ; Proteinuria/complications/*drug therapy

OBJECTIVETo study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases.

METHODSFifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups.

RESULTSThe IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (P<0.05). There were significant differences in serum creatinine, serum uric acid, serum total cholesterol, serum high-density lipoprotein cholesterol, serum IgE, serum complement C4, and hemoglobin levels between the IgA nephropathy and the control groups (P<0.05). The thresholds of serum IgE (<131.2 IU/mL) and high-density lipoprotein cholesterol (<1.35 mmol/L) were reference parameters in the differential diagnosis of IgA nephropathy with nephrotic syndrome and primary nephrotic syndrome.

CONCLUSIONSChildren with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

Adolescent ; Child ; Child, Preschool ; Cholesterol, HDL ; blood ; Complement C4 ; analysis ; Female ; Glomerulonephritis, IGA ; blood ; complications ; Hematuria ; etiology ; Humans ; Immunoglobulin E ; blood ; Male ; Nephrotic Syndrome ; blood ; complications