[Anatomo-clinical conference. Hôpital Cochin. Case No. 3-1989. Cerebral meningioma and adrenal mass in a 70-year-old woman].
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Biomedical subjects
Publications and source records attributed to G Grateau.
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The noninvasive diagnosis of amyloid arthropathy in dialysis patients is still uncertain. Therefore, we investigated the potential diagnostic value of the 99mTc-methylene diphosphonate scan in seven long-term hemodialysis patients suffering from chronic joint pain who had biopsy-proven osteoarticular amyloidosis of the recently discovered beta 2-microglobulin (beta 2-M) type. In six, but in none of five control patients on short-term hemodialysis, increased tracer uptake was found at the site of one or several articular and/or periarticular regions. Increased uptake at a given joint was often, but not always, associated with joint pain. It appeared to precede radiologically visible changes. In conclusion, the 99mTc-methylene diphosphonate scan may be of help in the early diagnosis of dialysis amyloidosis.
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Fever of undetermined origin in a young white heroin addict with a positive HIV (human immunodeficiency virus), serology was secondary to a staphylococcal perinephric abscess. Ultrasound examination and tomodensitometry allowed the diagnostic. Percutaneous drainage led to complete recovery. Interventional radiology associated with antibiotherapy appears to be an alternative to surgery in the treatment of perinephric abscesses.
Variants of Alport's syndrome include mainly those associated with hereditary macrothrombocytopenia (and occasionally leukocyte inclusions) or with esophageal, tracheobronchial and genital leiomyomatosis. Within Alport's syndrome there appears to be no justification for differentiating those with nephritis and deafness from those with nephritis alone. However, in indirect immunofluorescence studies using the mouse monoclonal antibody, MCA-P1, which recognizes the glomerular basement membrane (GBM), reduced or absent binding was found in 20 of 42 cases of hereditary nephritis. Most of these showed typical ultrastructural GMB changes. These results suggest that there is probably a subset of patients characterized by typical GBM lesions and an absence, inaccessibility or abnormality of the GBM antigen recognized by MCA-P1.
Isolated submandibular gland calcifications were found in a young woman with systemic lupus erythematosus (SLE) undergoing intermittent hemodialysis for 2 years. The calcifications appeared to be due to multiple factors including high calcium-phosphorus product and possibly local inflammation due to SLE.
Primary hyperoxaluria leading to calcium oxalate urinary stones and renal deposits occurs rarely in adults. We report three cases in whom end-stage renal failure was precipitated by urological surgery. In contrast, in one case renal stones were destroyed by extracorporeal shock-wave lithotripsy and renal function was not significantly altered. This emphasises the need for early diagnosis. This may not be easy in adult patients: urolithiasis may not be severe, radiological nephrocalcinosis may be lacking, and renal failure may develop late in life.
Six families including 27 patients with adult nephronophthisis were studied. The diagnosis was based on the evidence of heredofamilial chronic tubulointerstitial renal disease, not related to urologic abnormality, and progressing to renal failure in at least 2 members of each kindred. In two families, the mode of inheritance is compatible with recessive autosomal transmission; retinal heredodegeneration was found in both kindreds; mean age at end-stage renal failure (ESRF) was 22 years. In one additional case, kidney involvement is apparently sporadic, and associated with retinal changes and blindness. In one kindred, the mode of inheritance could not be determined. In the last 3 families, pedigrees are compatible with dominant autosomal transmission; retinal involvement was found in none; ESRF developed at a mean age of 47 years. Renal cysts were detected by ultrasonography in 9 of 12 patients; they were located at the cortico-medullary junction or in the medulla in 5 cases. Renal biopsy showed rather similar changes in the various kindreds. These results are compared with those already reported from 29 families with adult nephronophthisis.
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An adenopathy perforated into the esophagus in a thirty-two-year old woman from black Africa with mediastinal and abdominal tuberculous adenopathies. The fistula was disclosed upon endoscopic examination in the absence of esophageal symptoms. The characteristics of such fistulas are recalled: location in the middle third of the esophagus, inconspicuousness of symptoms, scarcity of complications among which digestive hemorrhage is the most significant, usually favorable course under medical management alone. The latency of fistulas between tuberculous adenopathies and the esophagus warrants routine fiberoptic endoscopy in patients with mediastinal tuberculous adenopathies.
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OBJECTIVE: To study the prevalence and characteristics of arterial thromboembolic events (ATEE) in the course of AL amyloidosis. METHODS: We report the case of a non-anticoagulated patient with AL amyloidosis restrictive cardiomyopathy who developed acute lower limb ischaemia. We then prospectively determined the prevalence of ATEE in all patients with AL amyloidosis who were evaluated in our institution for autologous peripheral stem cell transplantation. RESULTS: Nine out of 15 non-anticoagulated patients (60%) developed ATEE: ischaemic stroke (3), transient cerebral ischaemic attack (2), multiple peripheral arterial emboli (1), bilateral iliac artery thrombosis (1), bilateral optic nerve ischaemia (1), and mesenteric ischaemia (1). Haemodynamic stasis seemed to play a leading role in the pathophysiology of ATEE, in that all patients were on sinus rhythm and only one had a thrombus on echocardiography. We identified possible contributing factors to ATEE occurrence: concomitant treatments with oestroprogestogen regimen, thalidomide, granulocyte-macrophage colony-stimulating factor (GM-CSF) and extracellular volume disturbances related to the cytapheresis procedure. CONCLUSION: We report on an unusual frequency of ATEE among patients with AL cardiac amyloidosis. Despite its theoretical risks, anticoagulation should be discussed for patients with amyloid cardiomyopathy.
In patients with interstitial granulomatous sarcoid nephritis, renal failure often suggests the diagnosis, being rapidly progressive and isolated, i.e. without proteinuria and with few abnormalities of the urinary sediment. The extra-renal signs are usually discreet or absent, and the finding of a paradoxically high or normal calcaemia concomitant with renal failure should alert the clinician. When granulomatous lesions of the kidney are not too old, corticosteroids are remarkably effective in improving renal function.
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