[Reversible nephrotic syndrome in Crohn's disease complicated with renal amyloidosis]. / Syndrome néphrotique réversible au cours d'une maladie de Crohn compliquée d'amylose rénale.

A 24-year-old woman suffered from ano-rectal Crohn's disease and nephrotic syndrome due to glomerular amyloidosis AA. She received azathioprine and colchicine for two years. Both Crohn's disease and nephrotic syndrome resolved. However amyloid renal lesions were still present. This course is exceptional, and leads to a discussion of the treatment of amyloidosis associated with Crohn's disease.

[Arteriovenous fistula for chronic hemodialysis. 370 patients]. / Les fistules artério-veineuses pour hémodialyse chronique. 370 malades.

In cases of chronic renal failure, the usual technique for vascular approach is an arteriovenous fistula (AVF). This is the vascular approach with the longest lifetime. Microsurgery has improved the realization technique. This technique was used between 1978 and 1987 in 370 adult patients. Distal AVFs are made as a priority to preserve the patient's vascular capital. Radial AVF is the basic technique. Complications, including thrombosis, aneurysms, stenosis, low flow rate, require second surgery that must often be repeated. Arteriovenous bypass with a saphenous or cubital venous graft is used in difficult cases. Bovine carotid artery grafts are still used as bypass grafts in occasional cases; their lifetime is usually short. Catheterization through a jugular (never subclavian) site is a temporary alternative.

[Chronological study of signs of myoclonic encephalopathy in hemodialysis patients (author's transl)]. / Etude chronologique des signes de l'encéphalopathie myoclonique des hémodialysés. Signes précurseurs et évolution chez douze patients.

Twelve patients with chronic renal failure, on maintenance hemodialysis have a stereotyped illness. The dialysate was made up from a high level aluminium tap water. After having been on haemodialysis for 10 to 12 months, they suffer from more and more acute osteodystrophic, osteomalacic symptoms. Then, distinctive EEG abnormalities appear. These first symptoms allow an early diagnosis of the progressive dialytic encephalopathy. The neurological symptoms appear some months later. Eight patients died from this encephalopathy. Four patients are still alive and are, from at least two years, on maintenance haemodialysis with a free aluminium water. In these 4 cases, the evolution of the disease is good: osteomalacic symptoms disappear in a year; the neurological symptoms are still present though transient. EEG abnormalities remain and blood aluminium level is about 100 micrograms/L. These cases show, once more, the significant role of aluminium as an aetiological factor in "progressive dialysis encephalopathy".

Nodular glomerulosclerosis with deposition of monoclonal immunoglobulin heavy chains lacking C(H)1.

The objective of this study was to further characterize the clinical and immunopathologic features of heavy chain deposition disease (HCDD), a recently described entity. Four patients were diagnosed as having HCDD on a kidney biopsy. All presented with nodular glomerulosclerosis with deposition of gamma1 heavy chains lacking CH1 epitopes, but without light chains. Two different patterns were observed in the serum. First, patients 1 and 2 had a circulating monoclonal IgGlambda containing a short gamma1 heavy chain lacking CH1 epitopes, with an apparent molecular weight of 40 kD consistent with a complete CH1 deletion. Biosynthetic experiments also showed that the deleted heavy chain was produced in excess compared with light chains, and was secreted in vitro together with half Ig molecules, although these abnormal components were not detected by Western blot analysis of whole serum. Second, patients 3 and 4 had a circulating monoclonal IgG1lambda with an apparently normal, nondeleted heavy chain subunit, but serum fractionation followed by immunoblotting revealed an isolated monoclonal gamma1 chain lacking CH1 epitopes. These data strongly suggest that renal deposition of a CH1-deleted heavy chain circulating in low amounts in the serum as a free unassembled subunit is a major feature of HCDD. The CH1 deletion is most likely responsible for the premature secretion in blood of the heavy chain by a clone of plasma cells.