Search PubMed⌕ Search

Biomedical subjects

F Mignon

Publications and source records attributed to F Mignon.

At least 145 records · Page 8Linked to original sources
PubMed1983

[Articular manifestations of Whipple's disease. A case of sacroiliitis and destructive coxopathy].

Whipple's disease is a systemic illness whose pathogenesis, which is still poorly understood, implies an infectious agent accessible to antibiotic therapy. The authors report a case which is made unusual by the manifold systemic manifestations, important eosinophilia and destructive nature of the joint lesions of the hips. The physiopathological mechanisms of ocular and cutaneous manifestations are considered. A dramatic improvement is obtained with tetracyclin.

Adult↗
PubMed1983

Clinical significance of anti-Sm antibodies in systemic lupus erythematosus.

Case records of 34 patients with systemic lupus erythematosus (SLE) were analyzed. Twelve patients had both anti-DNA and anti-Sm antibodies (Group I) and 22 had anti-DNA antibodies only (Group II). The disease patterns were comparable, except for (1) cutaneous vasculitis, which was observed in six of 12 patients in Group I and one of 22 in Group II (p less than 0.01); (2) pulmonary manifestations, nine of 12 in Group I and two of 22 in Group II (p less than 0.001); (3) cardiac manifestations, eight of 12 in Group I and four of 22 in Group II (p less than 0.01); and (4) renal biopsy, which showed milder lesions in Group I than in Group II (p less than 0.05). Evolution was fatal in four patients in Group I and in none in Group II. It is suggested that in SLE, the presence of anti-Sm antibody is associated with a much higher incidence of vasculitis, resulting in peculiar visceral manifestations, which can be poorly responsive to therapy. Whether there is a direct association between anti-Sm antibody and vasculitis or whether the common denominator is a genetic selection remains to be determined.

Adolescent↗
PubMed1983

Renal lesions in multiple myeloma: their relationship to associated protein abnormalities.

Renal biopsy and autopsy specimens were studied in 43 patients with renal complications of multiple myeloma and correlated with immunoelectrophoresis (IEP) and other clinical data at the time of biopsy. Lesions specifically related to multiple myeloma fell into two categories, with different patterns of protein excretion. (1) Myeloma Cast Formation: When other lesions which might contribute to renal insufficiency (RI) were excluded, there was a good correlation between the extent of myeloma cast formation and severity of RI. Sixteen of 19 patients excreted free light chains (LCs) in the urine, in seven as the predominant or sole urinary protein. (2) Tissue Deposition of Paraproteins: Nine cases had generalized glomerular, tubular basement membrane and vascular deposits of presumed kappa-chains (one with associated alpha-heavy chains). Four patients, all with myelomas secreting lambda LCs, had diffuse amyloid deposits in similar distribution. All patients (save two who were anuric) had diffuse, nonselective proteinuria by IEP, most within the nephrotic range. Four patients had free LCs in the urine, but in none was this the predominant component. Cast nephropathy and LC tissue deposition tended to occur in mutually exclusive fashion. Cases with diffuse tissue deposits of LCs showed few or no myeloma casts. Cases with cast nephropathy had only occasional mild mesangial lesions and focal interstitial and vascular deposits of amyloid. Evidence indicates that these lesions represent incidental LC deposition in cases whose basic lesion is longstanding and/or severe cast nephropathy, and that their contribution to RI is minor in comparison to that of the myeloma casts.

Adult↗
PubMed1983

Immunopathological studies of polyarteritis nodosa and Wegener's granulomatosis: a report of 43 patients with 51 renal biopsies.

Although it is generally considered that vasculitis of the polyarteritis nodosa (PAN) group and Wegener's granulomatosis (WG) is immune complex (IC) mediated, there are no simultaneous data on circulating IC, complement levels and deposits of Ig and complement in the kidney. Therefore we have performed a retrospective study of 43 patients suffering from PAN and WG. Ig glomerular deposits were uncommon and scanty, except in two patients with WG; C3 deposits were detected in 12 patients, whereas fibrinogen was constantly found when lesions were recent and active. Similar data were obtained for the renal vessel walls. Contrasting with these results, rheumatoid factors and cryoglobulins, suggestive of the presence of circulating IC, were detected respectively in nine of 39 and seven of 37 patients, and IC 'activity' assessed by the Raji cell assay and the Clq binding assay was found respectively in six of 17 and nine of 10 patients before treatment, and in none of 10 and five of seven patients in remission. Haemolytic complement activity and complement components were never decreased, but the C3d breakdown product of C3 was elevated in all the eight patients studied before treatment. Signs of persistent hepatitis B virus (HBV) infection were detected in five of 25 patients of the PAN group, whereas three of eight patients with WG had only anti-HBV antibodies. Furthermore, cytomegalovirus (CMV) could be isolated from the blood in a case of WG before the treatment was started. Persistent interferonaemia was detected in one of five patients. These results suggest either that renal deposition of CIC is transient, the paucity of Ig deposits being due to rapid clearance of IC by phagocytic cells; or alternatively that vascular and glomerular lesions are not caused by CIC, as in some cases of experimental vasculitis induced by infectious agents.

Adolescent↗
PubMed1983

[Acute hematogenic interstitial nephritis of urinary origin: an unrecognized factor in the exacerbation of chronic kidney failure].

Hypertension, phosphate retention, hyperfiltration hyalinosis and the natural course of the underlying are well known factors leading to progression of chronic renal failure. Acute bacterial interstitial nephritis occurring in a previously diseased kidney, although well documented in experimental animals, has not been shown to aggravate chronic renal failure in man. We report on 3 cases of acute suppurative interstitial nephritis, due to E. coli urinary infection complicated by septicemia. All had rapid aggravation of previously mild renal failure secondary to chronic interstitial nephritis. Sepsis originated from the urinary tract which in 2 instances had been temporarily obstructed. Renal biopsy disclosed a diffuse interstitial infiltrate containing numerous polymorphonuclear leukocytes. This was superimposed on chronic tubular and interstitial lesions. In 1 case there were glomerular lesions with crescents and mesangial C3 deposits. A 2nd biopsy performed in 2 cases was of prognostic interest. In one case it showed active lesions and the necessity of continuing the treatment and in the other a satisfactory healing allowing cessation of therapy. Treatment was guided by antibiograms, the clinical and urinary signs of activity, renal biopsy findings and antibiotics known to be concentrated in renal tissue. The duration of treatment seemed important for the regression of acute renal lesions. Hematogenous bacterial interstitial nephritis should be considered as a possible cause of aggravation in chronic renal failure.

Acute Disease↗
PubMed1983

[Myxoma of the left atrium and advanced renal failure].

A 34 years old woman with a history of childhood rheumatic fever presented with a number of episodes of systemic embolism over a 10 years period attributed to mitral stenosis despite long-term anticoagulant therapy. All preoperative electrocardiograms showed sinus rhythm. After one episode of recurrent embolism (brain, limbs) requiring femoral embolectomy, she developed oligo-anuric renal failure and needed 21 days of hemodialysis. She made a full recovery. Several years later, she presented with chronic progressive renal failure. The diagnosis of left atrial myxoma was eventually made at echocardiography: surgical excision was successful. Periodic hemodialysis was started six months later. The authors use this case to illustrate unusual presentations of myxoma and above all their vascular complications; the possibility of embolic renal disease leading to advanced renal failure is discussed, a complication of left atrial myxoma which seems to be very rare.

Adult↗
PubMed1981

[Familial paroxysmal polyserositis: modern therapeutic possibilities (author's transl)].

Prognosis of familial paroxysmal polyserositis is primarily related to the presence of renal insufficiency due to the amyloidosis. Current treatment of terminal renal failure--haemodialysis and renal transplantation--has increased survival in these patients, but prognosis remains relatively poor because of the extrarenal, mainly cardiac deposits which have had time to develop. Preventive treatment is therefore all that can be hoped for, and initial results of the use of colchicine in this amyloid affection, by Israelian authors, appear encouraging.

Adolescent↗
PubMed1980

Synthesis of abnormal immunoglobulins in lymphoplasmacytic disorders with visceral light chain deposition.

Three patients presented with renal or more diffuse tissue deposits of a nonamyloid material reactive with anti-kappa antibody by immunofluorescence. All patients had progressive renal failure with the nephrotic syndrome and extensive tubular basement membrane deposits. Glomerular lesions were conspicuous but heterogeneous. One patient also had hepatic deposits with peliosis at histopathologic examination. An underlying lymphoplasmacytic disorder was found in all patients: multiple myeloma in one, pleomorphic lymphoplasmacytic malignancy analogous to Waldenström's macroglobulinemia in one and bone marrow monoclonal plasmacytosis without overt myeloma in one. Biosynthesis experiments in two cases showed production of abnormal kappa chains which were not detected in appreciable amounts in serum and urine. These light chains had an aberrant size (abnormally short or large), their apparent molecular weight was larger in secretion than in cytoplasmic extracts (suggesting their glycosylation) and they were secreted as polymers. These results suggest a causal relationship between production of abnormal light chains and tissue deposition.

Adult↗
PubMed1980

[Hepatic and renal deposits of kappa light chains revealing a dysglobulinemia (author's transl)].

A 38 year old woman presented portal hypertension and proteinuria. Liver biopsy showed peliosis hepatis with non amyloid deposits, reactive with anti-kappa antibody by immunofluorescence, along the sinusoids and in the Disse spaces. There are identical deposits along glomerular and tubular basement membranes. An underlying pleomorphic lymphoplasmocytic malignancy, analogous to Waldenström's macroglobulinemia was found, but without serum or urine monoclonal Ig. Biosynthesis experiments showed that the bone marrow cells synthesized abnormally short light chains with a larger apparent molecular weight when secreted than in the cytoplasmic extracts, presumably because of glycosylation. These results suggest a causal relationship between production of abnormal Ig chains and tissue deposition.

Female↗
PubMed1980

[Nephropathy and nodular regenerative hyperplasia of the liver (author's transl)].

A renal biopsy performed on a young woman with proteinuria showed a nephropathy consisting of major interstitial infiltration by lipophages and thickening of the glomerular capillary walls with a large amount of "spikes". Anti-IgG, IgM, C3 and C1q sera bound themselves to the extramembranous deposits and the anti-IgM serum to the endomembranous deposits. This striking finding was associated with a regenerative nodular hyperplasia of the liver, an entity that has been observed in only about 20 cases. An underlying immunological defect was suggested by the continuous presence of a mixed cryoglobulinemia.

Adult↗
PubMed1979

[Non-amyloid nephrotic syndrome: first isolated manifestation of a kappa light chain myeloma (author's transl)].

The authors describe a case which demonstrates that nephrotic syndromes occuring during the course of a myeloma are not always of the amyloid type. They emphasize the possibility of a dysglobulinemia being involved in a glomerular nephropathy syndrome, especially when there are histological lesions of a glomerulosclerosis. The published literature is reviewed and the possible pathogenesis of these nephropathies briefly discussed: either protein deposits, the nature of which has not been fully determined, or mesangial modifications induced by the filtered light chains.

Diagnosis, Differential↗
PubMed1978

[Extramembranous glomerulitis associated with visceral tumours. Clinical and histological study based upon 10 cases and a review of the literature (author's transl)].

The frequency of the association between extramembranous glomerulopathy and a visceral tumour suggests the responsibility of various antigen-antibody systems related to malignancy, at the origin of the nephropathy. In a number of special cases, the role of these systems has been more precisely demonstrated. The cases reported here emphasise the need to seek a visceral tumour when the onset of extramembranous glomerulitis occurs after the age of 40 years, in particular since the onset of proteinuria may precede the first clinical signs of the tumour by several months. The presence of polynuclear cells in the glomerular capillary loops, associated with extramembranous deposits, is an additional element of orientation in the direction of neoplastic aetiology.

Adenocarcinoma↗
PubMed1977

[Extramembranous glomerulitis. Apropos of 92 cases].

92 cases of extramembranous glomerulitis (EMG) documented by histology and immunofluorescence have been studied. At the time of renal biopsy the clinical and biologic picture was as follows: no proteinuria in 2%, isolated proteinuria in 18%, nephrotic syndrome without hypertension or azotemia in 41%, and hypertension and/or azotemia associated with proteinuria or nephrotic syndrome in 39%. A possible cause of the EMG was found in 27 cases: it was drug-induced or toxic in 10 instances, paraneoplastic in 7, lupus in 5 and parasitic (loasis) in 5.65 cases are regarded as idiopathic. The evolution is known in 66 cases and varies with the etiology: it is usually benign in secondary forms except the paraneoplastic cases. In the idiopathic forms it results in hypertension or more or less severe chronic renal failure in 62% of cases. The prognosis cannot be established on the basis of the initial histology. No treatment has proved effective.

Adolescent↗