Episodes Of Macroscopic Hematuria Research Articles

Response of circulating immune complexes to food challenge in relapsing IgA nephropathy.

The response of circulating immune complexes (CIC) to food challenge was assessed in 15 subjects with IgA nephropathy (IgAN) and recurrent macroscopic haematuria. CIC were measured by solid-phase C1q binding assay (SP-C1q), immunoglobulin class-specific polyethylene glycol (PEG) precipitation assays (PEG-G, PEG-A, PEG-M) and by an antigen (ovalbumin)-specific radioimmunoassay after acid dissociation (OA-IC). CIC were measured when the subjects were fasting and hourly for 6 h after a test meal containing eggs. All 15 subjects were tested while clinically quiescent (remission) and 6 were tested again during episodes of macroscopic haematuria (relapse). The PEG-A CIC response to food challenge was significantly exaggerated in IgAN remission compared with controls at 3-6 h after food. There were also non-significant increases in PEG-G, though not in PEG-M. Paired data showed further exaggeration of PEG-G, PEG-A and PEG-M responses to food during IgAN relapse, but significance was not attained if the findings in 1 subject were separated. In this individual a florid clinical relapse with transient decline in renal function was associated with very high levels of PEG-IC, and only in this patient in relapse was OA-IC detectable, confirming that some PEG-precipitated material represented antigen-antibody complexes containing food antigen.

Clinicopathological associations in mesangial IgA nephropathy.

On hundred and fifteen renal biopsies performed in 112 patients with mesangial IgA nephropathy were reviewed and the histological disease patterns correlated with the clinical features at the time of initial biopsy. To determine the significance of macroscopic haematuria in this disease, specific comparisons were made between patients with a history of episodes of macroscopic haematuria and those with only microscopic haematuria. The mean age at initial biopsy was 38.3 years (90 males, mean 40.3 years; 22 females, mean 30.2 years). Histological examination showed 9 patients (8%) with class I disease (mesangial matrix expansion alone); 43 patients (38%) with class II disease (diffuse mesangial proliferation); 60 patients (54%) with class III disease (focal and segmental proliferation), including subsets of 20 patients (16%) with segmental sclerosis and/or synechiae and 23 patients (21%) with crescent formation. Class III disease and crescent formation correlated with an increased frequency of capillary loop IgA and glomerular fibrin deposition and with the presence of subendothelial and subepithelial deposits. The degree of renal impairment and the incidence of hypertension were increased in class III disease. Macroscopic haematuria patients were younger (mean 31.1 vs. 43.0 years; p less than 0.001), had less severe renal impairment (mean creatinine 116.2 vs. 213.3 mumol/l; p less than 0.001) and less class III disease (48 vs. 58%; p less than 0.05). The incidence of crescentic disease was equal in macroscopic (17%) and microscopic (23%) haematuria. Eventual progression to end-stage renal failure occurred in 12 patients (11%) and correlated with crescentic disease, renal impairment, hypertension and heavy proteinuria at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute worsening of renal function during episodes of macroscopic hematuria in IgA nephropathy

The appearance of renal failure during episodes of macroscopic hematuria (EMH) in IgA nephropathy (IgAN) has been described as very unusual. The results of a prospective investigation on the effect of EMH on renal function in IgAN are presented. During a 3-year period, 29 episodes of EMH occurring in 21 patients with IgAN have been studied. A derangement of renal function (increase of serum creatinine by more than 0.5 mg/dl) was observed in 11 episodes (37.9%) with peak creatinine values ranging from 1.2 to 6.7 mg/dl. The worsening of renal function was accompanied by a longer duration of EMH (4.8 +/- 1.3 vs. 3.5 +/- 1.5 days; P less than 0.05) but not by arterial hypertension or edema. A complete recovery of renal function was observed in every patient 1 to 2 months after the start of EMH. The histological survey disclosed that the decrease of renal function correlated closely with the presence of red blood cell casts in as much as 50% of the tubular lumen and with findings of tubular necrosis. We conclude that a worsening of renal function can be observed frequently during the EMH. Tubular damage and obstruction by red blood cell casts may play a significant role in the pathogenesis of this complication.

Bergerʼs Disease in Children

The clinical course and outcome of 91 children less than 15 years of age at onset and followed for at least 1 year have been retrospectively analyzed. The course has been characterized by recurrent macroscopic hematuria in 74 patients, by proteinuria-microscopic hematuria and a single episode of macroscopic hematuria occurring either at onset or a few months later in 8, by proteinuria-microscopic hematuria in 7, and by proteinuria only in 1. Lastly, one patient showed rapidly progressive renal failure. Four groups were identified by light microscopy: minimal glomerular changes (26), focal and segmental glomerulonephritis (41), pure mesangial proliferation (3) and proliferative glomerulonephritis with crescents (21). A good correlation was found between the glomerular lesions observed by light microscopy and the outcome. In this series we have not observed a dramatic clinical deterioration suggesting a transformation from one histologic type to another, as reported by others. None of the 70 patients belonging to the first three groups has impaired renal function but two with focal and segmental glomerulonephritis have developed hypertension. Although the clinical course is benign, many patients have, at the last observation, an abnormal urinalysis characterized by microscopic hematuria and/or mild proteinuria; the proteinuria is over 1 g/24 h in six patients with focal and segmental glomerulonephritis. Ten patients remained in clinical remission for several years, but mesangial IgA deposits were still present in the only patient who had a repeat biopsy while in remission. In contrast, none of the patients with proliferative glomerulonephritis with crescents has had a prolonged remission. Six patients developed terminal renal failure 0.7, 0.11, 2, 4, 8 and 10 years after onset. Two additional patients are in moderate chronic renal failure with hypertension 10 and 12 years after onset. Most children show a persistent nephropathy, (in five proteinuria is over 1 g/24 h), and two of them have developed hypertension. Therapeutic trials using drugs with side-effects should, therefore, be used only in this group of patients.

Significance of microhaematuria in young adults.

The medical records of 1000 asymptomatic male air force personnel were examined retrospectively for the results of 15 yearly examinations of urinary sediment. The study covered the period 1968-82, beginning with the subjects aged 18-33 years. The cumulative incidence of two to four or more red blood cells per high power field found at one or more examinations was 38.7% after an average of 12.2 yearly examinations per person. In 161 subjects two to four or more red blood cells per high power field were found at two or more yearly examinations within a five year period. Intravenous pyelography in 58 cases disclosed asymptomatic nephrolithiasis in six. Cystoscopy performed in 11 cases identified one patient with urethritis, one with a vesical calculus, and one with transitional cell carcinoma of the bladder. Two years before diagnosis the patient with carcinoma had had a single transient finding of 10-12 red blood cells per high power field which was not investigated further. Cystoscopy was performed after an episode of macroscopic haematuria. Renal biopsy in one subject with recurrent microhaematuria and trace proteinuria disclosed focal glomerulonephritis. None of the remaining subjects with microhaematuria developed hypertension or proteinuria, and at the end of the study period all were active and free of urinary symptoms. The observed cumulative incidence of urological neoplasms at 15 years (0.1%) was consistent with that expected in Israeli men aged 18-40 (0.09%). Hence microhaematuria detected during a screening examination probably should not be regarded as a specific sign of a significant lesion and does not of itself warrant urological investigation in adults aged 40 or less.

Macroscopic hematuria in mesangial IgA nephropathy: Correlation with glomerular crescents and renal dysfunction

One hundred and eighty-six renal biopsy specimens from 79 adult patients with mesangial IgA nephropathy were examined and correlated with clinical data at the time of biopsy. Forty patients (group 1) with a history of macroscopic hematuria were compared with 39 patients (group 2) without such a history. Group 1 patients had a higher serum creatinine, 240 +/- 20 mumoles/liter vs. 140 +/- 10 mumoles/liter (P less than 0.01), lower creatinine clearance 69 +/- 36 ml/min vs. 87 +/- 30 ml/min (P less than 0.05), and a higher percentage of patients presenting with serum creatinine greater than 300 mumoles/liter, 22.5% vs. 5.1% (P less than 0.05). Fourteen biopsies were performed in 11 patients during an episode of macroscopic hematuria (group 1A). One hundred percent of these biopsy specimens showed crescents. Ninety-one percent of 11 biopsy specimens from ten patients (group 1B), taken 3 to 27 days following an episode but at a time when urinary red cells were less than 1,000,000/ml, also showed crescent formation. Of 14 biopsy specimens from 13 patients without macroscopic hematuria, but with greater than 1,000,000 red cells/ml in the urine just prior to biopsy (group 2A), 79% had crescents. In conclusion, macroscopic hematuria in adult patients with mesangial IgA nephropathy is associated with a high likelihood of crescents on renal biopsy specimens and worse renal function. Careful quantitative assessment of the urine for renal bleeding may help to better define the activity of disease in these patients.

Immunologic studies in IgA nephropathy

Circulating immune complexes (CIC) were detected in 43.6% of 78 patients with primary IgA nephropathy by the solid-phase Clq radioimmunoassay. The IC were intermediate (9 to 17S) in size and contained IgA, IgG, and less commonly IgM. CIC were often present intermittently, correlating with episodes of macroscopic hematuria. Elevated serum IgA concentrations (38.7%) did not correlate with the detection of CIC. Similar findings were observed in sera samples from patients with Henoch Schonlein purpura and in IgA glomerulonephritis associated with alcoholic cirrhosis and/or portal systemic shunts. The factors responsible for the mesangial localization of the IC are not clear, but elevations in serum antibody titers to respiratory pathogens (mycoplasma pneumoniae, herpes virus, influenza), gut flora (E. coli 07), and bovine serum albumin suggest that common exogenous antigens may be involved in the pathogenesis. Primary defects in either mucosal antigen exclusion or reticuloendothelial IC sequestration are proposed to account for these findings.

The Clinical Course of Mesangial Proliferative Glomerulonephritis

Patients with a pure mesangial proliferative glomerulonephritis may present with either hematuria, asymptomatic proteinuria, or nephrotic syndrome. For patients with hematuria, the clinical course is self-limited, and spontaneous resolution of recurrent episodes of macroscopic hematuria occurred in many of these patients. For patients with asymptomatic proteinuria, a spontaneous decrease in proteinuria occurred and none of these patients developed a progressive course. The clinical course of patients with nephrotic syndrome was similar to that usually observed in children and adults with pure minimal change lesions. A complete remission of the nephrotic syndrome occurred in most of these patients during treatment with prednisone and cyclophosphamide. Overall, the prognosis for the patients reported in this series was quite good.

RENAL FUNCTIONAL CHANGES IN ACUTE GLOMERULONEPHRITIS IN CHILDREN. A ONE‐YEAR FOLLOW‐UP

Renal function was studied in three patients with post-streptococcal, four patients with IgA and one patient with non-streptococcal proliferative glomerulonephritis (GN) at the onset of the disease and two, six and 12 months later. Renal biopsies were performed at the onset of the disease and 12 months later. Standard clearance techniques were used for the functional studies. The latter were performed during hydropenia and continuous isotonic saline infusion. During hydropenia, the GFR was uniformly depressed shortly after the onset of the disease, but it normalized during the following two months. The filtration fraction was depressed in poststreptococcal GN at the onset and it normalized with the GFR. In IgA GN, the filtration fraction remained within normal limits during the entire course of the illness. The natriuretic response to isotonic saline volume expansion was low in all patients at the onset of the disease, but normalized in post-streptococcal and IgA GN during the one-year follow-up. In spite of normalized renal function, biopsy findings in IgA GN were unchanged 12 months later. An episode of macroscopic hematuria in one patient with IgA GN at the six-month investigation had no apparent effect on renal function.

The prognostic value of some clinical and histological parameters in membranoproliferative glomerulonephritis (MPGN): report of 112 cases.

112 cases of MPGN, whose diagnosis was made on light microscopy, were reviewed. Histological examination showed 66 cases of 'classical' MPGN, 33 of MPGN with a lobular pattern and 13 of MPGN with epithelial crescents. In 11 patients dense intramembranous deposits were observed. On immunofluorescence (95 cases) 62 patients showed deposits of C3 together with immunoglobulins, 20 had a predominant deposition of C3 and in 13 C3 alone was present. At the moment of biopsy 57 patients had nephrotic syndrome, 43 hypertension, 43 impaired renal function and 65 hypocomplementaemia. In 23 cases, one or more episodes of macroscopic haematuria occurred. The actuarial survival was 70% after 10 years and 50% after 20 years from onset. At last observation 25 patients were dead or on haemodialysis, 22 had impaired renal function, 62 had normal renal function and 2 were in complete remission. The histological variety with epithelial crescents had a significantly worse outcome. The presence of dense deposits or of any specific immunofluorescence pattern had no prognostic significance. Nephrotic syndrome, renal function impairment and hypertension indicated a poor prognosis: however, macroscopic hamaturia or hypocomplementaemia did not influence the outcome.