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D Ferluga

Publications and source records attributed to D Ferluga.

At least 19 recordsLinked to original sources
PubMed2005

Follow-up of kidney graft recipients with cyclosporine-associated hemolytic-uremic syndrome and thrombotic microangiopathy.

The study was based on 462 patients who underwent kidney transplantation from 1986 through 2004. Cyclosporine (CsA)-related thrombotic microangiopathy (TMA) was observed in 15 (3.3%) patients. The donor ages ranged from 9 to 51 years and cold ischemia times from 12 to 31 hours. Hemolytic-uremic syndrome (HUS) developed 2 weeks after transplantation in 14 patients and later in 1 subject. Histopathologic examination demonstrated glomerular-type TMA in 3 patients, a mixed type (glomerular and vascular) in 11 patients, and a nonspecific mesangial widening with tubulointerstitial lesions in 1 patient. Follow-up biopsies revealed resolution of TMA in 4 patients and chronic vascular TMA in 1 patient. Six patients with mixed-type TMA needed transient hemodialysis. No patient with the glomerular-type TMA needed dialysis (P = .103), and 14 of 15 had good resolution of graft function after CsA dose reduction or temporary discontinuation or continuation of optimal dose. Only 1 graft with mixed-type TMA was lost due to irreversible HUS. The mean glomerular filtration rate (GFR), predicted by the Nankivell equation, was 76 +/- 13 mL/min and 80 +/- 27 mL/min at 1 month after discharge for glomerular- and mixed-type TMA, respectively (P > .05). GFRs 1 year after HUS were 82 +/- 12 and 87 +/- 21 mL/min for the glomerular and the mixed types, respectively (P > .05). We concluded that the mixed-type TMA was associated with a more severe early clinical course than the glomerular-type TMA. The 1-year prognosis was good in the majority of patients, with no significant differences between those with the glomerular- and mixed-type TMA.

Adolescent↗
PubMed2003

Xanthogranulomatous pyelonephritis in nonfunctioning transplanted kidney.

Xanthogranulomatous pyelonephritis in native and allografted kidneys is a rare variant of severe chronic infection of the renal parenchyma. In a native kidney the diagnosis may sometimes be established by ultrasonography and computed tomography. In the renal allograft, the diagnosis could only be established by histologic evaluation of the transplant biopsy or nephrectomy. The reported case presents a febrile patient with a failing renal graft, in whom xanthogranulomatous pyelonephritis was established by histologic evaluation of transplantectomy specimens. Xanthogranulomatous pyelonephritis should therefore be included in the list of possible etiologies of the fever in patient with nonfunctioning transplanted kidney.

Cadaver↗
PubMed2001

Real-time ultrasound-guided renal biopsy with a biopsy gun in children: safety and efficacy.

UNLABELLED: Real-time ultrasound-guided renal biopsy (RB) with a biopsy gun has become a standard procedure in the treatment of children. The purpose of the study was to establish the complication rate after real-time ultrasound-guided RB with a biopsy gun, the adequacy of renal tissue samples for pathohistological tests, the rate of concurrence between clinical and pathohistological diagnoses, and the benefits of the procedure. From January 1994 to October 1999, 88 renal biopsies were performed on 82 children, 81 of whom (35M, 46F, aged 3-20 y) were included in this retrospective study. The nephrotic syndrome (in infants, older children, those with evidence of nephritis or failing corticosteroid therapy) was the most frequent indication of RB. Other indications were non-nephrotic proteinuria, nephritic syndrome, glomerular haematuria, renal allograft dysfunction, unexplained acute or chronic renal failure, and kidney disease progression monitoring. No serious complications were noted. The adequacy rate of renal tissue samples ranged from 93.1 to 96.6%, depending on which definition of the adequacy of renal tissue samples was used. Clinical and pathohistological diagnoses matched in 81.4% of the cases. Data obtained by RB were very beneficial to patients in terms of establishing, confirming or altering the diagnosis and, consequently, the treatment. CONCLUSION: The results confirm that real-time ultrasound-guided RB with a biopsy gun is a safe procedure and provides information that is very beneficial to patients.

Adolescent↗
PubMed2000

Antineutrophil cytoplasmic autoantibodies-antigen specificity and associated diseases.

Antineutrophil cytoplasmic antibodies (ANCA) are widely used as a useful diagnostic marker for small vessel vasculitides, although the test may occasionally be positive in various other conditions. The aim of this study was to assess ANCA in various clinical-pathological settings. ANCA were tested by indirect immunofluorescence and enzyme-linked immunosorbent assay and were found to be positive in 423 patients in the period from 1989-1999. Patients were grouped in accordance with their clinical-pathological setting as follows: 1. pauci-immune vasculitis confirmed by biopsy (n = 151), 2. clinically suspected vasculitis (n = 59), 3. inflammatory bowel diseases and autoimmune hepato-biliary disorders (n = 83), and 4. miscellaneous diseases (n = 130). The association of proteinase 3 ANCA with Wegener's granulomatosis (45/56) and myeloperoxidase ANCA with microscopic polyangiltis (45/54) and pauci-immune necrotising glomerulonephritis (24/28) was established. However, ANCA with other specificities were also shown to be present in these forms of vasculitides. ANCA, specific mostly for myeloperoxidase but also for other or unknown ANCA antigens, frequently revealing atypical immunofluorescence patterns, were characteristically found in other diseases. The titres of ANCA were significantly higher (p < 0.05) in patients with pauci-immune vasculitis than in those with clinically suspected vasculitis and other diseases. In conclusion, well standardised techniques for ANCA testing in conjunction with the clinical picture and histopathologic findings, if available, may significantly contribute to the diagnosis of small vessel vasculitides.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2000

Correlation among WHO classes, histomorphologic patterns of glomerulonephritis and glomerular immune deposits in SLE.

In addition to the conventional World Health Organization (WHO) classification of lupus glomerulonephritis (GN), various concomitant approaches have been introduced in the evaluation of renal biopsies of patients with systemic lupus erythematosus (SLE) in order to increase the impact of biopsies on the decision concerning the most appropriate therapy as well as for establishing the prognosis. Three hundred and seventy kidney tissue samples from 267 SLE patients were analysed using standardised light, electron and immunofluorescence microscopic techniques. In 155 patients, a comparative clinical follow-up study and statistical analysis were performed. The study highlighted the heterogeneity of WHO classes IV and III, which include 5 and 6 different conventional histomorphologic types of GN, respectively. Mixed membranous and proliferative GN associated with "full-house" mesangial-transmembranous immune deposits, demonstrated in more than one third of our SLE cases, appears to be diagnostically most characteristic. Immune deposits distributed in the glomeruli in five different patterns, obviously play a major role in the pathogenesis of various WHO classes and histomorphologic types of lupus GN. Additional mechanisms related to the occurrence of antiphospholipid antibodies and antineutrophil cytoplasmic antibodies are suggested to contribute to the histomorphologic heterogeneity of WHO class III and IV lupus GN, particularly to the development of thrombotic, necrotising and crescentic glomerular lesions. In the present study, a statistically significant association was demonstrated between increasing mean values of the activity index and glomerular deposit distribution patterns labeled by subendothelial deposits. Furthermore, a significant correlation was established between an increasing risk of developing renal failure and increasing mean values of the chronicity index. Differences in the increasing risk of developing renal failure between groups with different histomorphologic types of GN and different immune deposit distribution patterns were not statistically significant. The surprisingly high renal survival rate of more than 80% noted in lupus patients with predominantly necrotising crescentic GN during the mean follow-up period of 40 months appears to be related to the more aggressive treatment of those patients. Our study confirmed a significant role of the WHO classification of lupus GN in the decision concerning the most appropriate treatment and prognostication. An increasing risk of irreversible renal failure in patients with WHO class IV lesions in contrast to those of WHO class III and in contrast to those of the category incorporating all other WHO classes was shown to be statistically significant.

Adolescent↗
PubMed2000

Tubulo-interstitial involvement in lupus nephritis with emphasis on pathogenesis.

Glomerular lesions in lupus nephritis have been extensively studied in recent decades, but much less attention has been paid to the tubulo-interstitial compartment. The aim of this study was to contribute to the understanding of the pathogenesis of tubulo-interstitial lesions in lupus nephritis by analysing their incidence, character, and their associations. One hundred and ninety kidney biopsies of 190 patients fulfilling American Rheumatology Association (ARA) criteria of systemic lupus erythematosus (SLE) were examined by traditional light, immunofluorescence and electron microscopy. Interstitial inflammatory infiltration and tubulo-interstitial immune deposits concurred in 72 cases (37.9%). Their frequency was the highest in WHO class IV lupus glomerulonephritis. By multivariate analysis, the intensity of interstitial inflammatory infiltration correlated best with the percentage of renal corpuscules with extracapillary crescents and the extent of interstitial fibrosis. On immunohistochemical assessment, the inflammatory infiltrate was found to be composed of CD45RO positive T lymphocytes (191.3/mm2), CD68 positive macrophages (101.7/mm2) and CD45RA positive B lymphocytes (17.2/mm2). For all cell types the median value was higher in cases with extracapillary crescents, and did not correlate with presence and intensity of tubulo-interstitial immune deposits. Infiltration showed the tendency of periglomerular distribution, especially around glomeruli showing extracapillary proliferation and destruction of the capsular basal membrane. Rare S100 positive cells were only found in the interstitium. Tubulo-interstitial lesions estimated semiquantitatively correlated with the degree of proteinuria. Our findings suggest that tubulo-interstitial deposits do not play a major role in the pathogenesis of tubulo-interstitial lesions. The formation of interstitial cell infiltrates appears to be greatly influenced by the development of extracapillary crescents, perhaps by direct transmission of the severe inflammatory process to the adjacent interstitium. The composition of the infiltrate, including antigen presenting cells may signalize an additional involvement of cell-mediated immune mechanisms acting against so far hypothetical tubular epithelial neoantigens.

Adult↗
PubMed2000

Small vessel thrombosis without major thrombotic events in systemic lupus erythematosus patients with antiphospholipid syndrome.

Antiphospholipid syndrome has been defined by the presence of antiphospholipid antibodies or lupus anticoagulant in association with certain clinical events, including recurrent arterial or venous thromboses and recurrent fetal loss. It comprises two separate clinical entities: simple, characterized by large vessel occlusions, and catastrophic, with multiple occlusive events predominantly affecting small vessels. Three patients with systemic lupus erythematosus and permanently increased IgG anticardiolipin antibody levels are being described. Only postmortem histopathological examination revealed microangiopathic thrombotic changes in different organs, which were clinically silent in early stages of the disease and misinterpreted later in its course because of a peculiar clinical picture. All patients presented features of catastrophic antiphospholipid syndrome in the final stage of the disease.

Adolescent↗
PubMed2000

Fingerprint and other organised deposits in lupus nephritis.

A prominent feature of lupus glomerulonephritis is extracellular, predominantly homogeneous electron dense deposits. Fingerprint-like deposits have been reported in 6 to 10% of cases. On electron microscopy, we studied the frequency and characteristics of organised deposits in 227 kidney tissue samples obtained by biopsy in 185 patients with systemic lupus erythematosus (SLE). Fingerprint forms of deposits were demonstrated in 34 biopsies of 32 patients (17.3%). In the control group of 626 kidney biopsies of patients with primary renal and systemic diseases other than SLE, no fingerprint deposits were found. In 227 kidney biopsy samples, fingerprint deposits were found to be associated with mesangial (8.8%), mesangial-subendothelial (3.8%), subepithelial (28.6%), mesangial-subepithelial (11.1%) and mesangial-transmembranous (19.4%) glomerular deposit distribution patterns. They were demonstrated more often at different locations along the peripheral capillary glomerular basal membrane (77.4%) than within the mesangial matrix (43.7%). In extraglomerular locations, fingerprint deposits were present in the interstitium in 8.8%, along the tubular in 23.5% and peritubular capillary basal membrane in 20.5%, in the wall of the arterioles in 64.7% and in the juxtaglomerular apparatus in 18.2% of biopsies. Organised fingerprint deposits consisted of semicircular dark and light lines, each with a diameter of about 10 to 15 nm. Unilaterally, spiky processes at a periodicity of 10 to 15 nm were seen. Among 14 of 185 SLE patients with cryoglobulinemia, fingerprint deposits were demonstrated in only 2 patients. We conclude that fingerprint deposits are characteristic, diagnostically relevant for SLE and represent morphologically a homogeneous group of organised deposits unrelated to cryoglobulins. In 3 SLE patients, 20 to 100 nm tubules and in 2 SLE patients, 10 and 18 nm Congo red negative fibrils were found. By their morphology and their structural characteristics, the tubules and fibrils resemble the tubules in primary immunotactoid glomerulopathy and fibrils in primary fibrillary glomerulonephritis.

Adolescent↗
PubMed2000

Role of lipids in the progression of renal disease in systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease marked by immune-complex mediated lesions in small blood vessels of various organs, especially the kidneys, although other factors may also be implicated in the pathogenesis of the disease. This article focuses on the role of lipids in the progression of glomerular, vascular and tubulo-interstitial lesions in two patients with lupus nephritis associated with pronounced hyper- and dyslipidemia. The pathogenesis of progressive glomerulosclerosis in both patients appears to be multifactorial. In addition to immune complex mediated lupus glomerulonephritis, progressively active in the first patient, severe nephrotic-range persistent proteinuria, arterial hypertension associated with hyperfiltration and hyperperfusion injuries and, to a minor extent, hyper- and dyslipidemia were observed. Immunological and non-immunological factors were shown to contribute to the development of tubulo-interstitial lesions. In both patients, in addition to local immune deposits, prominent tubulo-interstitial lipid deposits were probably causally related to both hyperlipidemia and the increased permeability of the glomerular filtration barrier. Tubular lesions were highlighted by intracytoplasmic lipid droplets as well as small cleft-like spaces found to be impacted in the tubular lumina. They were seen to penetrate tubular epithelial cells and eventually lodge in the interstitium, surrounded by mononuclear cell infiltrates and foam cells. In both patients, hypertensive angiopathy and extraglomerular vascular immune deposits were demonstrated. In addition, in the second patient, arteriolar and small arterial hyaline was found at the age of 28 years to be full of lipids and calcium precipitates, suggesting a peripheral atherosclerosis-like process which never occurs as a natural age-related condition. In conclusion, all parts of the nephron may be involved in the pathogenetic process causally related or influenced by hyper- or dyslipidemia. Associated either with endothelial cell injury and consequent insudation of lipids in the vascular walls, glomerular filtration barrier injury with hyperfiltration, or tubulo-interstitial lipid deposition, the mechanism of tissue damage by lipids in all parts of the nephron shares similarities with the pathogenesis of systemic atherosclerosis.

Adult↗
PubMed1998

Unfavorable course of minimal change nephrotic syndrome in children with intrauterine growth retardation.

BACKGROUND: Intrauterine growth retardation (IUGR) is associated with higher morbidity and mortality not only in perinatal life but also in later life. The purpose of our study was to determine whether IUGR has any effect on the course of minimal change nephrotic syndrome (MCNS) in children. METHODS: Forty children who were between 1 and 16 years old at the onset of MCNS, who have been followed for at least three years and for whom we were able to obtain birth weights and gestational ages, were included. The diagnosis of MCNS was predicted on the basis of clinical and laboratory features, and in 11 children (27.5%) the diagnosis was confirmed by renal biopsy. IUGR was defined as birth weight below the tenth percentile for gestational age. RESULTS: Five children (12.5%) had signs of IUGR at birth. In children with IUGR, we observed a higher mean number of relapses (10.4 vs. 3.3, P < 0.001) and a higher incidence of steroid dependency (80% vs. 21%, P < 0.02) than in children without IUGR. Other differences between children with and those without IUGR included more frequent treatment with cytotoxic agents and cyclosporine, and a higher incidence of renal biopsy in children with IUGR. CONCLUSION: Our study demonstrated an unfavorable course of MCNS in children with IUGR. IUGR could therefore enable early identification of those children who are at risk of becoming frequent relapsers and of developing steroid dependency. This, however, should be confirmed in a larger number of patients.

Adolescent↗
PubMed1998

Skeletal muscle immune deposits in systemic lupus erythematosus. Correlation with histologic changes, autoantibodies, and clinical involvement.

Skeletal muscle biopsy and autopsy samples of 132 SLE patients were studied by immunofluorescence and light microscopic techniques. Immune deposits were compared to histologic abnormalities and clinical and serologic findings. Immune deposits with a mainly granular pattern were observed at different locations in 49 patients (37%). They correlated significantly (p<0.001) with inflammatory myopathy, demonstrated in 33(25%), with noninflammatory myopathy which occurred in 38 (29%) vasculitis, observed in 13(10%), and with noninflammatory vasculopathy which was noted in 10 patients (8%). The correlation of muscle immune deposits with anti-DNA antibodies was significant at p= 0.016. Anti-Sm and anti-U1 RNP antibodies were associated significantly with the intensity of immune deposits. Furthermore, a correlation of immune deposits with increased creatine phosphokinase and myopathic electromyogram, but not with evidence of clinical involvement, was shown. A key pathogenetic role of immune deposits in the development of skeletal muscle histologic abnormalities in SLE was demonstrated.

Adolescent↗
PubMed1998

Effect of intrauterine growth retardation on the clinical course and prognosis of IgA glomerulonephritis in children.

Intrauterine growth retardation (IUGR) resulting in a reduced number of nephrons is one of the nonimmune mechanisms that have been recently proposed as contributing to the progression of renal diseases. The purpose of our study was to determine whether IUGR has any effect on the clinical course and prognosis of IgA glomerulonephritis (IgA GN) in children. Fifty children with biopsy-proven IgA GN, who were followed for at least 3 years, were included. Six of the 50 children (12%) had signs of IUGR at birth, defined as birth weight below the 10th percentile for gestational age. There were no significant differences in initial clinical presentation between children with IUGR and those without IUGR. However, in kidney biopsy specimens, we found a significantly higher mean percentage of sclerotic glomeruli in children with IUGR than in those without IUGR (33 vs. 13%, p < 0.015). At the end of the follow-up period, we observed a significantly higher incidence of arterial hypertension in children with IUGR than in those without IUGR (50 vs. 11 %, p < 0.05). Other differences between the two groups of children were not statistically significant. In conclusion, our study demonstrated an increased risk of the development of arterial hypertension and glomerulosclerosis in children with IgA GN who had suffered from IUGR with a birth weight below the 10th percentile for gestational age. IUGR may therefore help to identify early in the course of IgA GN those children who are at higher risk of an unfavorable course.

Adolescent↗