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C Orfila

Publications and source records attributed to C Orfila.

At least 55 records · Page 3Linked to original sources

Unsuitable value of abdominal fat tissue aspirate examination for the diagnosis of amyloidosis in long-term hemodialysis patients.

Abdominal fat tissue aspiration was used in 22 long-term hemodialysis patients (5-17 years). Fourteen of these patients had carpal tunnel syndrome and amyloid deposits of beta 2-microglobulin in the synovium. One patient had a spontaneous rupture of the spleen with amyloid deposits in spleen vessels. Seven other patients presented carpal tunnel syndrome and/or articular pains, and radiological lytic lesions in bone, strongly suggesting an amyloid origin. As a control group, in 22 patients with biopsy-proven amyloidosis, abdominal fat tissue aspirates were performed and were studied under the same conditions: by light microscopy these tissues were stained with Congo red and examined with a polarizing microscope; these specimens were also studied by electron microscopy. In all hemodialyzed patients, no amyloid deposit was present in fat tissue with Congo red staining and by electron microscopy. On the contrary, amyloid was observed in 17 of 22 cases in other types of amyloidosis. It seems that this method which has been proved to be simple and sensitive for the diagnosis of systemic amyloidosis is not a good marker for the presence of amyloid in long-term hemodialysis patients.

Abdominal Muscles↗

Localization of a renal kallikrein immunoreactive-like substance in rat ureter.

An antibody against rat kallikrein was produced in rabbits and its localization was studied in various organs of the rat to confirm its specificity. The distribution of immunoreactive kallikrein was studied in rat ureter by use of immunochemical techniques. Ureteral tissue was fixed in Zamboni's-glutaraldehyde fixative and immunostained with indirect immunofluorescence and the peroxidase-antiperoxidase (PAP) method for light and electron microscopy. Preabsorption of the primary polyclonal antiserum with purified rat urinary kallikrein and substitution with normal serum were used as controls. By light microscopy, kallikrein was localized in the lamina propria and in the adventitial connective tissue surrounding the entire ureter. Immunoelectron microscopy confirmed this immunolocalization. Immunoreactive kallikrein was concentrated in fibroblasts of connective tissue and was not present in collagen fibers. Immunoreactivity was associated with the Golgi complex, free polyribosomes, and rough endoplasmic reticulum. No immunostaining was observed in other subcellular components of fibroblasts.

Animals↗

[Segmental renal hypoplasia of an adult originating from pseudo-tumor hyperreninism].

A 38 year-old man was admitted for severe hypertension with hypokalemia. Blood pressure was 180-120 mmHg, funduscopic examination revealed grade II retinopathy and left ventricular hypertrophy was present. Laboratory data disclosed: natremia = 140 mmol/ml, kalemia = 2.8 mmol/l, chloride = 105 mmol/l, bicarbonate = 30 mmol/l, creatinine clearance = 100 ml/mn, natriuresis = 140 mmol/day, kaliuresis = 80 mmol/day. Intravenous pyelography was normal. Angiography revealed a defect in the mid third of the right kidney without arterial abnormalities. Study of renin angiotensin aldosterone system showed: plasma renin activity: peripheric blood = 36 ng/ml/h (normal range I to 2), right renal vein = 30 ng/ml/h. Left renal vein = 18 ng/ml/h, inferior cava vein = 19 ng/ml/h. Plasma aldosterone level = 86 ng/100 ml (normal range 10 to 15). Captopril acute administration was followed by a fall of BP to 70-50 mmHg at 2 hours. Right nephrectomy was performed and revealed an ischemic retracted cortical area without necrosis nor tumoral aspect. The day after BP was 140/80 mmHg. Eight days after, kaliemia was 4.2 mmol/l, PRA was 0.5 ng/ml/h. Light microscopy showed that affected area was sharply delimited from surrounding normal tissue. In this area, glomeruli were present and seemed more numerous as usual; interstitial fibrosis and infiltrates of inflammatory cells were also noted. The main fact was tortuosities of intralobular arteries, thickened, with intimal proliferation. There was a pelvic recess near this cortical tissue. Immunofluorescence findings: antirenin serum fixed on JGA but also on interlobular arterial walls and on peritubular interstitium.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Abdominal fat tissue aspirate in human amyloidosis: light, electron, and immunofluorescence microscopic studies.

Abdominal fat tissue aspirates from 12 patients with biopsy-proved amyloidosis were investigated by different morphologic techniques. By light microscopy, after staining of the fat tissue aspirates with Congo red and examination with a polarizing microscope, positive results were obtained in nine patients with amyloidosis, two of the three with primary (AL) amyloidosis and seven of the nine with secondary (AA) amyloidosis. By indirect immunofluorescence, using AA antiserum, positive results were obtained in five of the nine cases of AA amyloidosis (aspirates from these five patients were positive on Congo red staining). By electron microscopy, amyloid fibrils were observed in five cases of amyloidosis (two of the AL and three of the AA type, all positive on Congo red staining). Although amyloid was demonstrated less frequently by immunofluorescence and electron microscopy, perhaps because of the small numbers of fat particles examined, it seems that, with Congo red staining, abdominal fat tissue aspiration is a simple and sensitive method for the diagnosis of amyloidosis. Immunofluorescence studies allow discrimination between the different types of amyloidosis. The method could be used in patients in whom other types of tissue biopsy are not recommended because of risks of bleeding or other problems.

Adipose Tissue↗

[Renal biopsy and diagnosis of angiitis. 13 cases].

The renal biopsies of 13 patients presenting with a predominantly renal form of angiitis were reviewed. The principal lesions were glomerular with a segmental and focal extracapillary glomerular nephritis in all cases. Arteriolar lesions with necrosis or granuloma were inconstant (6/13) but necrosis of the tuft reflecting capillary involvement were common (11/13). Interstitial tubular disease and immunofluorescence were not specific. Renal biopsy is a good indication of diagnostic when performed early before the development of sclerosis: arteriolar necrosis and suggestive changes: extracapillary segmental and focal glomerulonephritis. In the latter case, a careful search for extrarenal involvement with guided biopsy studies usually allow a diagnosis to be confirmed. The renal and extrarenal clinical signs, the biochemical changes, treatment and outcome were analysed. Two of the 13 patients had Wegener's disease, one patient had angiitis and linear fixation of IgG along the glomerular and tubular basal membranes.

Adult↗

Immunofluorescence and ultrastructural localization of beta 2-microglobulin in human renal allografts.

The localization of beta 2-microglobulin (beta 2m) was studied in renal biopsies from 18 patients with pathological transplanted kidney using immunofluorescence and electron-immunohistochemical techniques; the renal biopsies of 4 cases with normal kidneys were used as controls. Using immunofluorescence, beta 2m was not observed in the normal kidneys, beta 2m was found in the glomeruli (7 cases) and the tubular epithelium (16 cases) of the transplanted kidneys. Using immunoelectron microscopy, some labelling of the normal kidneys was observed mainly along the cell coat of foot processes and in tubular-epithelial lysosomes. In the glomeruli of transplanted kidneys, particularly in cases of acute or chronic rejection, beta 2m was most frequently localized on the outer layer of the basement membrane and along the cell coat of foot processes. The brush border of the proximal tubules and the lysosomal structures were intensely labelled. Although immunoelectron-microscopy studies are unable to discriminate between the localization of beta 2m in normal and transplanted kidneys, these findings nevertheless suggest the glomerular filtration of beta 2m and its metabolism in the tubular epithelium.

Adolescent↗

Immunofluorescence study of "non-idiopathic" renal amyloidosis.

The authors report the results of immunofluorescence (IF) studies of 17 cases of "non-idiopathic" renal biopsy-proven amyloidosis and 18 cases of various nephropathies and normal kidneys (as controls), investigated by IF by simultaneous use of antisera against routine IgG, IgM, IgA, C3, C4, Clq, beta-lipoprotein, albumin, and fibrinogen. Antisera against kappa and lambda light chains and amyloid A and amyloid P components were also used. Six of the 17 cases of amyloidosis were associated with immunocyte dyscrasia, and 11 were cases of reactive systemic amyloidosis associated with chronic infections or inflammatory and neoplastic disorders. In amyloidosis, IF deposits appeared for all antisera as homogeneous staining of mesangial nodules, and, more rarely, there was staining along the glomerular basement membranes. Overall immunoglobulins and C3 were present in 11 cases (64 per cent). Kappa and lambda light chains were demonstrated in 14 (82 per cent) and 12 (70 per cent) cases, respectively. In immunocyte dyscrasia associated with amyloidosis, immunoglobulin and light-chain deposits corresponding to a paraprotein abnormality were demonstrated in glomeruli and in tubular casts. Amyloid P component was always present in glomeruli with a bright and characteristic fluorescence, and it was frequently observed in arterioles. Amyloid A component was observed in six cases of reactive systemic amyloidosis but also in one case of immunocyte dyscrasia with amyloidosis. In view of the diversity of amyloid fibril types and their chemical nature, IF studies confirm the presence of different constituents but do not warrant any conclusion concerning the pathogenesis of this disease.

Adult↗

Immunofluorescence study of amyloid P-component patterns in human nephropathies.

The localization of amyloid P-component (AP) staining was studied by immunofluorescence in renal biopsies from 106 patients with various nephropathies and from 3 patients with normal kidneys. Linear staining was observed along the glomerular basement membranes (GBM) of normal kidneys. In amyloidosis, AP was always present in the glomeruli. In arteriolar walls, AP was present in numerous cases with varied intensity. No fixation was observed along tubular basement membranes. The possible modification of the permeability of GBM, related to a possible modification of the electrical charge of the filtration barrier, can be supposed.

Amyloid↗

Mesangial isolated C3 deposition in patients with recurrent or persistent hematuria.

We reviewed 222 renal biopsies performed on patients with nonurologic hematuria alone or hematuria and proteinuria. This review revealed 31 patients who presented minor glomerular abnormalities with granular mesangial C3 deposition without any immunoglobulin or other complement component deposits. These patients presented with gross hematuria in five cases, microscopic hematuria alone in 12 cases, and proteinuria and microscopic hematuria in 14 cases. Light microscopy disclosed a segmentally increased mesangial matrix, sometimes associated with a segmental and focal mesangial hypercellularity. Focal tubulointerstitial and arteriolar lesions occurred in seven cases. In all cases, immunofluorescence showed diffuse granular mesangial deposition of C3. However, the deposits were not observed along the glomerular and tubular basement membranes. We should point out the high frequency of arteriolar C3 deposits (27 of 31). They were either isolated or associated with IgM or other complement components. Using electron microscopy, an increase of mesangial matrix and/or a proliferation of mesangial cells was observed. In two cases, electron-dense deposits were present in the mesangium. Glomerular basement membranes were normal in all but five cases, where they appeared segmentally thickened. After a 3-year follow-up study the clinical features remained unchanged.

Adolescent↗

A correlative study of immunofluorescence, electron, and light microscopy in immunologically mediated renal tubular disease in man.

Renal biopsy specimens obtained from patients with various chronic nephropathies were studied by immunofluorescence (IF), electron and light microscopy. Of 49 biopsies studied by IF, 22 had granular deposits of IgG and/or C3 along the basement membranes of proximal tubules while 14 had linear and 13 both linear and granular deposits. The possible importance of immunologically mediated renal tubular lesions in chronic nephropathies is discussed.

Basement Membrane↗

[Are surgical renal biopsies useful for clinical prognosis in renal artery stenosis?].

The most significant results show the predominance of arteriolar lesions in the controlateral kidney, The most significant results show the predotients with unilateral renal artery stenosis. and parenchymal lesions of the kidney with stenosis in patients with atherosclerosis. These results do not seem to provide information which would alter the clinical management in any particular case.

Adult↗

Postpartum hemolytic uremic syndrome: a study of three cases with a review of the literature.

Three cases of postpartum hemolytic uremic syndrome (HUS) are presented. Symptoms of acute renal failure, hypertension and microangiopathic hemolytic anemia with thrombocytopenia occurred 10, 17 and 24 days after delivery. Despite early heparin therapy in all cases, one patient went into terminal renal failure needing chronic hemodialysis, with persistent hypertension which became uncontrollable requiring bilateral nephrectomy 6 months later. The second patient had diuresis one month after starting hemodialysis, but 3 months later developed malignant hypertension. Slight improvement in renal function with persistent hypertension occurred after hemodialysis for 20 months. The third patient showed complete clinical recovery after 2 months. Pathological examination of renal tissue showed the typical lesions of thrombotic microangiopathy (TMA). However, striking differences were observed in the lesion seen in early and late specimens. Early lesions could be differenciated from infancy TMA because the medium-dize arteries were more severely involved. Late lesions were variable, ranging from minor changes in glomeruli and blood vessels, via ischemic and sclerotic lesions in glomeruli with arteriolosclerosis, to the vascular and glomerular lesions seen in malignant nephrosclerosis. There was a good correlation between the renal pathology and the clinical outcome of the patients. HUS with renal TMA as a cuase of postpartum renal failure has been reported in 49 patients with a fatal outcome in 61%. The pathogenesis of the syndrome probably involves a primary endothelial damage. This causes local renal intravascular coagulation in the presence of the usual postpartum hypercoagulable state. This is shown by the presence of fibrin-fibrinogen in glomeruli and vessels, increased plasma fibrin degradation products, thrombocytopenia and lowered levels of coagulation factors. There is little hematological or pathological evidence fo disseminated intravascular coagulation or an immune-complex disease. Hypocomplementemia seen frequently is probably due to local C3 activation via the alternative pathway.

Adult↗