[Small cell lung cancer revealed by extramembranous glomerulonephritis]. / Carcinome à petites cellules révélé par une glomérulonéphrite extramembraneuse.

We describe a case of small-cell lung cancer limited to the thorax but with malignant pleural effusion in a 47-year-old man that was revealed by a nephrotic syndrome due to membranous glomerulonephritis (MGN). Chemotherapy led to a partial tumor response with total resolution of the nephrotic syndrome. Tumor relapse did not provoke proteinuria. Primary lung cancer is the cause of about 3% of all cases of MGN and 40% of tumor-related MGN. There are 49 cases of tumor-related MGN in the literature, including 9 cases of small-cell lung cancer.

[Homozygote deficiency of thiopurine methyltransferase. A contraindication to the use of azathioprine in kidney transplantation]. / Déficit homozygote en thiopurine méthyltransférase. Une contre-indication à l'utilisation de l'azathioprine en transplantation rénale.

Azathioprine-induced myelosuppression is the most important side effect observed in kidney transplantation. We report a case of severe neutropenia after kidney transplantation due to a thiopurine methyltransferase deficiency. This cause of azathioprine-induced myelotoxicity is rare, but its infectious consequences may be severe. Thiopurine methyltransferase deficiency must therefore be suspected when early and severe leukopenia occurs during azathioprine therapy. Erythrocyte thiopurine methyltransferase activity measurement confirms the diagnosis. Azathioprine and 6-mercaptopurine must afterwards be definitively avoided.

[Primary signet ring adenocarcinoma ofa diverted neurogenic bladder]. / Adénocarcinome primitif à cellules en bague à chaton sur vessie neurologique exclue. A propos d'un cas.

Primary signet-ring cell carcinoma of urinary bladder is an uncommon primitive bladder tumor. We report the first case occurring on a diverted neurogenic bladder. Except of adenocarcinoma of urachal origin, about 60 cases have been reported to date. The histogenesis of these tumors remains controversial.

[Long term course of idiopathic extramembranous glomerulonephritis. Study of predictive factors of terminal renal insufficiency in 82 untreated patients]. / Evolution à long terme des glomérulonéphrites extra-membraneuses idiopathiques: etude des facteurs prédictifs de l'insuffisance rénale terminale chez 82 malades non traités.

In a retrospective study of 82 adults with idiopathic membranous nephropathy, we have studied the predictive factors of renal death. Thirty one patients developed renal failure, sixteen of them progressed to end-stage renal failure (ESRF) (13 males and 3 females). Nine patients developed ESRF in the first 5 years following the renal biopsy. The males and the patients with a nephrotic syndrome have a high risk of ESRF. The age is not a predictive factor of poor outcome. Hypertension seems to be more frequent in patients who developed early but slowly progressive renal failure. Extensive renal interstitial lesions are indicative of poor renal prognosis.

[Ankylosing spondylitis with type AA amyloidosis. 6 cases]. / Spondylarthrite ankylosante avec amylose de type AA. Six observations.

The authors have studied 6 cases of systemic AA amyloidosis associated with ankylosing spondylitis. Renal failure occurred in all patients a mean of 19 years after the clinical onset of the rheumatic disease. Three patients progressed rapidly (between 3 months and 3 years) to end-stage renal failure. Such an outcome did not depend upon early onset of the renal impairment, degree of inflammation or treatment with colchicine. All patients were alive 2 to 10 years later, and this confirms a better prognosis than with AL amyloidosis. The utility of combining Wright's permaganate reaction with immunological methods to characterize the amyloid deposits was also confirmed. It is concluded that amyloidosis is a rare complication of ankylosing spondylitis and probably depends on a genetic predisposition. The possibility of amyloidosis should be kept in mind when proteinuria or renal failure appear in the course of ankylosing spondylitis.

[Long-term evolution of membranoproliferative glomerulonephritis in adults : spontaneous clinical remission in 13 cases with proven regression of glomerular lesions in 5 cases (author's transl)]. / Evolution à long terme des glomérulonéphrites membranoprolifératives de l'adulte : rémission spontanée durable chez 13 malades avec étude de biopsies rénales itératives dans 5 cas.

188 patients with type I MPGN were followed from 1957 to 1975. At last examination, 53% of patients with pure MPGN and 66% of those with lobular MPGN had renal insufficiency; 28 other patients showed clinical improvement and 13 of these had permanent complete remission. In all of these 13 cases, (10 pure MPGN and 3 lobular forms), the clinical remission was spontaneous and occurred 2 to 16 years after the apparent onset. At the time of the first renal biopsy, 7 of these patients had a nephrotic syndrome, 2 had hypertension and 1 had renal failure. A second biopsy was obtained in 5 patients during the clinical remission period and showed in all cases a clear regression of the glomerular lesions. The possibility of spontaneous permanent clinical remission in MPGN should be considered in the indications and methods of treatment and the interpretation of their results.

Hereditary nephritis associated with May-Hegglin anomaly.

Hereditary nephritis associated with hematologic abnormalities seems to be an exceptional occurrence. We have observed a family which nephritis was combined with May-Hegglin anomaly. A girl and her father suffered from proteinuria; a paternal uncle received kidney graft; a paternal grand aunt died on periodic hemodialysis. The girl, the father and the uncle presented macrothrombocytopenia (40-100 X 10(9)/l, size 4-8 mum) with prolonged bleeding time (which precluded renal biopsy) and cytoplasmic inclusions in neutrophils (Döhle bodies). These hematologic abnormalities characterize the May-Hegglin anomaly. This kind of association has not been reported so far.

Hepatitis B antigen-associated periarteritis nodosa in patients undergoing long-term hemodialysis.

Periarteritis nodosa was observed in three of 266 persistent hepatitis B antigen (HBsAg) carriers undergoing long-term hemodialysis; no cases of necrotizing vasculitis occurred among 384 other patients undergoing dialysis having either no or transient antigenemia. Circulating e antigen, but no e antibody, was found in two of these three patients. The serum level of the third component of complement (C3) was normal in two patients and low in the third. Circulating immune complexes were demonstrated in all three patients, using polyethylene-glycol (PEG) precipitation, PEG-C4, and solid phase C1q tests. HBsAg and anti-hepatitis B antibody (HBsAb) were identified in the PEG precipitates using radioimmunoassay and electron microscopy technics. Direct immunofluorescence performed on a muscle biopsy specimen from one patient was positive for HBsAg, but not for immunoglobulin G (IgG), immunoglobulin M (IgM), C3 or C1q. These data support the hypothesis that circulating immune complexes involving HBsAg may be involved in the pathogenesis of periarteritis nodosa.

Biologic, pathologic, and radiologic studies of cadaver kidneys preserved in Collins' solution.

Collins' solution is one of the world-wide methods used for kidney preservation. A long-term survey of transplanted kidneys thus preserved has never been reported. Clinical, biologic, radiologic, and pathologic data collected from 225 cadaver kidney transplantations were analyzed according to the total ischemic time (group 1: TIT less than or equal to 12 hr; group 2: TIT 12-30 hr). No differences could be demonstrated between the two groups, whether considering the percentage of immediate function, the serum creatinine level that was normal up to 2 years, the incidence of abnormal blood pressure, the pathologic changes on routine biopsies performed at 6 months and 2 years, or arteriographic features at 2 years. A better graft survival rate was consistently found at 6 months, 1 year, and 2 years when TIT was between 12 and 30 hr. This could be related to the higher incidence of well-matched kidneys in this group of patients, thereby emphasizing the efficiency of exchange programs for compatible kidneys.

[Long-term use of immunosuppressants in patients with kidney transplants]. / Utilisation au long cours des immunosupresseurs chez les malades porteurs d'un rein transplanté

The authors analyse the principal characteristics of immunosuppression needed after a kidney graft. They review various therapeutic methods and consider the main complication induced by long-term iummunosuppression. They associate published data with their own experience, based on more than 300 transplantations performed with corticosteroids and azathioprine at Necker Hospital.