Characteristics and outcome of 49 patients with symptomatic cryoglobulinaemia.

OBJECTIVE: To describe a population of patients with symptomatic cryoglobulinaemia, comparing manifestations and outcome as a function of hepatitis C virus (HCV) status. PATIENTS AND METHODS: A retrospective study on 179 patients who tested positive for cryoglobulins, seen between 1978 and 1998 in an internal medicine department. RESULTS: Among 179 cryoglobulin-positive patients, only 49 (18 men, 31 women; mean age 59.96+/-12 yr) had clinical manifestations attributable to cryoglobulinaemia. Thirty-three had HCV infection, 20 had systemic autoimmune diseases, two had haematological diseases, one had human immunodeficiency virus and HCV co-infection, one had HCV and HBV co-infection and six had essential mixed cryoglobulinaemia. The clinical manifestations and cryoglobulin levels in HCV(+) and HCV(-) patients did not differ significantly. Only arthralgias and elevated transaminases were significantly more frequent in HCV(+) patients (P<0.02 and <0.05, respectively). Five-year survival rates were comparable for HCV(+) and HCV(-) patients. Eight patients died (six HCV(+), two HCV(-)), with a median time between diagnosis and death of 38.7 months. CONCLUSION: Clinical manifestations of cryoglobulinaemia, except arthralgias, were comparable for HCV(+) and HCV(-) patients. When systemic manifestations are present, the prognosis is poor despite intensive or prolonged therapy.

Evidence for spinodal decomposition in nuclear multifragmentation.

Multifragmentation of a "fused system" was observed for central collisions between 32 MeV/nucleon 129Xe and (nat)Sn. Most of the resulting charged products were well identified due to the high performances of the INDRA 4pi array. Experimental higher-order charge correlations for fragments show a weak but nonambiguous enhancement of events with nearly equal-sized fragments. Supported by dynamical calculations in which spinodal decomposition is simulated, this observed enhancement is interpreted as a "fossil" signal of spinodal instabilities in finite nuclear systems.

IgA glomerulonephritis associated with microscopic polyangiitis or Churg-Strauss syndrome.

When renal insufficiency occurs in classical antineutrophil cytoplasm antibody- (ANCA) associated vasculitides, histological examination usually finds pauci-immune focal segmental glomerulonephritis. We report on 2 cases of histologically proven necrotizing vasculitis associated with IgA nephropathy. Concomitant vasculitis and IgA nephropathy has only rarely been reported but this joint occurrence may not be coincidental as its pathophysiology is not known. Among vasculitides, IgA nephropathy has more frequently been associated with Henoch-Schoenlein purpura: one microscopic polyangiitis unusual because the patient simultaneously presented ANCA and microaneurysms, and the other Churg-Strauss syndrome associated with mild renal insufficiency. This uncommon association might represent a possible overlap syndrome between these ANCA-associated vasculitides and IgA nephropathy or simply a new type of glomerulonephritis that must be taken into account in these vasculitides.

Enalapril plus frusemide MAG3 scintigraphy in hypertensive patients with atherosclerosis and moderate renal insufficiency.

We performed a retrospective study on 26 patients with moderate renal failure (mean GFR = 51 +/- 21 ml min-1 1.73 m-2), hypertension and atherosclerosis. Apart from three patients who had completely normal renal Doppler ultrasonography, all patients underwent renal angiography. Three groups of kidneys with different atherosclerotic renal artery involvement were identified: Group 1, 24 kidneys with no renal artery stenosis (RAS); Group 2, 18 kidneys with mild (> 25% and < 50% diameter) RAS; and Group 3, 10 kidneys with moderate (> 50% diameter) RAS. We used a two-day protocol with frusemide plus enalapril 99Tcm-MAG3 scintigraphy. The mean parenchymal transit time (MPTT), time to the maximum activity (time to peak) of the renal curve (Tmax), residual activity and split renal uptake were evaluated. The measured parameters did not differ before and after enalapril in Group 1 or in Group 2. In Group 3, MPTT and residual activity differed significantly (P < 0.025) before and after enalapril. The Tmax before and after enalapril, MPTT before and after enalapril and residual activity after enalapril differed significantly (P < 0.05) between Groups 1 and 3 and between Groups 2 and 3. Threshold values were obtained to maximize diagnostic accuracy. The Tmax, MPTT and residual activity after enalapril gave satisfactory results, and MPTT performed best with a 75% positive predictive value and a 98% negative predictive value for the diagnosis of renal artery stenosis. We conclude that MPTT, measured after enalapril administration, is a useful parameter to detect renal artery stenosis in patients with hypertension, atherosclerosis and moderate renal insufficiency.

[Diagnostic value of pulsed Doppler in atheromatous renal insufficiency. A study of 32 patients]. / Intérêt diagnostique du Doppler pulsé dans l'insuffisance rénale athéromateuse. Etude de 32 patients.

RATIONALE: atheromatous stenosis of both renal arteries, or of the artery of a functionally solitary kidney is a frequent cause of renal failure in the elderly. Atheromatous Ischemic Renal Insufficiency (AIRI) can be ameliorated by surgery or angioplasty. However, such procedures can be hazardous in a patient with extensive aortic atheromatous plaques. This justified a study of the validity of Pulsed Doppler (PD), a diagnostic procedure less invasive than renal angiography in AIRI. OBJECTIVES: to evaluate the diagnostic interest of PD in AIRI, using renal angiography as a criterion of adequacy. METHODS: renal arteries PD (Ultramak-4, 3.5 and 5 Mhz probes) followed by renal angiography (Seldinger). "Significant" stenosis = 50% on angiography. "Positive" PD = turbulences and/or acceleration. PATIENTS: 32 patients were investigated for suspected AIRI on the grounds of 1) age greater than 50; 2) atheromatous background and 3) renal insufficiency with no other evident etiology, or rapidly declining GFR in such a patient treated with ACE inhibitors. RESULTS: 16/32 angiographies disclosed significant stenosis of at least 1 renal artery. 56 renal arteries were investigated with both angio. and PD. Prevalence of stenoses was 16/56. PD had 93.7% sensitivity and 55% specificity. Positive predictive value was 45.5% and negative predictive value was 95.7%. Specificity was 80% when PD disclosed acceleration. CONCLUSIONS: negative PD is sufficient argument to reconsider the indication of renal angiography in a high risk pt with suspected AIRI.

Atheromatous renal disease.

PURPOSE: Atheroma as a cause of renal failure has been largely overlooked. We wanted to report our experience with atheromatous renal disease over a 12-year period. PATIENTS AND METHODS: Observations on 32 cases of various forms of renal failure in patients with atheromatous renal disease are presented. These patients had been hypertensive for an average of 10.2 +/- 9.2 years. The length of deterioration was an average of 17 months, and at presentation renal insufficiency was severe, with serum creatinine levels of (mean +/- SD) 616 +/- 358 mumol/liter (6.8 +/- 4.0 mg/dl). At this stage, the clinical picture was indistinguishable from other common causes of chronic renal failure in the elderly. Thus, the precise diagnosis would have been overlooked without an aggressive diagnostic workup. All patients underwent angiography and six patients underwent renal biopsy. RESULTS: In 22 cases, renal insufficiency was mainly due to atheromatous stenosis of renal arteries. In six of six patients, the results of renal biopsy showed cholesterol crystal embolism. In four additional cases, there was clinical or histologic evidence of extrarenal cholesterol embolism. In eight, renal artery plaques coexisted with cholesterol embolism. In two patients, renal failure was due only to cholesterol embolism. Renal atheromatous stenoses were developing, as shown on serial angiographies performed in five cases. In seven cases, stenoses involved both the main trunks of renal arteries and several intrarenal branches of too small a diameter to allow reconstructive surgery or percutaneous transluminal angioplasty. In addition, the general condition of most patients was so poor as to preclude surgery. Dialysis was begun in 11 patients, four other patients died, and renal failure was managed conservatively in 11. When undertaken, reconstructive surgery was successful in five of six patients. CONCLUSIONS: Atheromatous renal disease is a frequent and easily overlooked cause of chronic renal insufficiency. It is not only due to renal artery stenosis but also to complex intrarenal lesions, with multiple stenoses of intrarenal vasculature and cholesterol embolism. It should be diagnosed by early angiography and renal biopsy, before the stage of multivisceral complications and at a time when surgery can still be undertaken.

Renal toxicity of the antitumor drug N2-methyl-9-hydroxyellipticinium acetate in the Wistar rat.

Celiptium (N2-methyl-9-hydroxy-ellipticinium) is an antitumoral agent used to treat bone metastases from breast carcinomas. This new drug appeared to be of great interest because of the absence of hepato- or myelotoxicity. Three different investigators recently mentioned cases of celiptium-induced renal failure. We therefore undertook a study of renal function and morphology in female Wistar rats. Two single i.v. doses (10 or 20 mg/kg) were administered and animals were sacrificed 4, 8, 15, 28 and 60 days after injection. One group of rats received multiple doses, 5 mg/kg/week for 8 weeks. No mortality was observed. With the 10 mg/kg single dose creatinine clearance (Ccr) and urinary enzymes did not change, and tubular lesions were rare. With the 20 mg/kg single dose CCr decreased on day 4 and returned to normal on day 28. Urinary enzyme excretion (AAP, NAG, gamma GT) increased. Renal lesions were diffuse with tubular necrosis, luminal dilation and later (day 28) interstitial cellular infiltration. These lesions persisted on day 60 and appeared to be irreversible. Ultrastructural studies showed numerous large fat droplets in proximal tubular cells. Glycerol concentrations in renal cortex homogenates were increased while phospholipids are slightly decreased. With 5 mg/kg every week (multiple doses) Ccr decreased and tubular lesions similar to the observed with the 20 mg/kg single dose were seen. Thus celiptium induced dose-dependent nephrotoxicity in rats with prolonged tubular alterations.(ABSTRACT TRUNCATED AT 250 WORDS)