The glomerular basement membrane defect in Alport-type hereditary nephritis: absence of cationic antigenic components.

Alport-type hereditary nephritis is a familial disorder which results in progressive renal insufficiency and sensorineural hearing loss. It is thought to result from a biochemical defect affecting basement membranes. To study this further, non-collagenous components of type IV collagen were prepared from the glomerular basement membrane (GBM) by collagenase digestion from three male patients with hereditary nephritis.

Influence of captopril on renal hemodynamics and segmental tubular reabsorption of sodium in humans.

Acute angiotensin-converting enzyme inhibitors (ACEIs) have been found to induce natriuresis in humans as well as in experimental animals. However, the tubular sites involved have not been precisely evaluated in humans. Using both free-water and lithium clearance, the latter as a marker of proximal tubular reabsorption, we measured segmental tubular movement of sodium before and after acute captopril administration in eight healthy normotensive volunteers on normal sodium diet.

Plasma 5-hydroxyindoleacetic acid as an endogenous index of renal plasma flow.

5-Hydroxyindoleacetic acid (5-HIAA), the major serotonin metabolite, was measured in human plasma and urine using liquid chromatography and electrochemical detection. The renal extraction of 5-HIAA was 78%, a value compatible with an almost complete extraction during a single passage of the blood through the kidney. In addition, plasma levels of 5-HIAA measured in patients with a wide range of renal function was inversely correlated (r = 0.

[Ankylosing spondylitis with type AA amyloidosis. 6 cases].

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.

Linkage studies in X-linked Alport's syndrome.

Four kindreds segregating for Alport's syndrome (ASLN) compatible with a X-linked inheritance were studied for linkage with polymorphic markers of the human X chromosome. No recombinant was observed between the ASLN locus and the DXS101 and DXS94 loci, the maximum lod scores were z = 3.93 and 3.

[Renal failure disclosing interstitial granulomatous nephropathy of sarcoidosis. 4 cases].

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.

Role of the urinary concentrating process in the renal effects of high protein intake.

High protein diet is known to increase glomerular filtration rate (GFR) and induce kidney hypertrophy. The mechanisms underlying these changes are not understood. Since the mammalian kidney comprises different nephron segments located in well-delineated zones, it is conceivable that the hypertrophy does not affect all kidney zones and all nephron segments uniformly.

[Effect of cyclosporin A on blood pressure and renal hemodynamics in insulin dependent diabetes].

In 13 patients with very recent onset insulin-dependent diabetes (IDD) (less than 3 months) we studied arterial pressure and renal hemodynamics before (M0) and after 3 months (M3) of treatment with cyclosporine A (5 mg/kg/d). Glomerular filtration rate (GFR) and effective renal plasma flow (RPF) were measured by inulin and PAH clearance respectively. Results, expressed as mean +/- SD were compared to those obtained in 8 healthy controls (C).

Glucocorticoids modulate vascular reactivity in the rat.

To clarify the role of endogenous glucocorticoids in the regulation of blood pressure, the cardiovascular effects of RU 486, a steroid derivative with antiglucocorticoid properties, were investigated in Wistar rats. Pressor responses to angiotensin II (Ang II), norepinephrine, and vasopressin were studied in normal conscious rats before and after administration of RU 486. At 20 mg/kg/day, RU 486 significantly blunted pressor responses to Ang II and norepinephrine, whereas those to vasopressin were not greatly affected.

Acute renal failure in pregnancy.

Acute renal failure has become a very rare complication of pregnancy. This results from the virtual disappearance of septic abortion ARF and from the improvement of prenatal care, including the prevention of volume contraction which is mainly due to uterine haemorrhage, early diagnosis, and treatment of other classic maternal complications such as pre-eclampsia and acute pyelonephritis. The incidence of BRCN has also been declining during the last decade.

[Role of glucocorticoids in the regulation of blood pressure].

Glucocorticoids contribute to the regulation of blood pressure: glucocorticoid excess produces hypertension, and glucocorticoid deficiency is accompanied by low blood pressure. The role of endogenous glucocorticoids has been assessed in animals by using an antiglucocorticoid derivative, RU 486. It has been shown that glucocorticoids enhance vascular reactivity to angiotensin II and norepinephrine.

Variants of Alport's syndrome.

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.

[The role of glucocorticoids in the cardiovascular adaptation to sodium restriction in the rat].

The role of glucocorticoids (Gs) in the regulation of blood pressure has been examined using an antiglucocorticoid, RU 486, at a dose of 20 mg.kg-1 day-1 X 5 days, in male Wistar rats on a low sodium diet. In unanesthetized and chronically catheterized rats, RU 486 administration decreases mean blood pressure (MBP) by approximately 14 mmHg, without altering growth rate or urinary sodium excretion.

Acute renal failure in pregnancy: 1987.

Acute renal failure has become a rare complication of pregnancy due to the virtual disappearance of septic abortion and to better prenatal care, including prevention of blood volume contraction. The incidence of bilateral renal cortical necrosis also decreased in recent years. Severe preeclampsia-eclampsia may be accompanied by acute tubular necrosis.

Pregnancy in IgA nephropathy, reflux nephropathy, and focal glomerular sclerosis.

Fetal outcome was retrospectively studied in 217 pregnancies observed during the past two decades in 93 patients, 34 suffering from IgA nephropathy (IgAGN, 69 pregnancies), 53 from reflux nephropathy (RN, 137 pregnancies), and six from focal glomerular sclerosis (FGS, 10 pregnancies). Overall incidence of live births was 175 in 217 (81%). Fetal loss, corrected for induced abortions, was 10 in 66 (15%) in IgAGN, 18 in 129 (14%) in RN, and 2 in 10 in FGS.

Pregnancy in lupus nephritis and related disorders.

We studied retrospectively the influence of lupus nephropathy on the outcome of pregnancy and of pregnancy on the course of lupus nephritis in 213 pregnancies observed from 1962 to 1985 in a series of 73 patients with systemic lupus erythematosus (SLE). Renal biopsy demonstrated diffuse or focal proliferative glomerular lesions in 48 of 66 patients. The overall incidence of live births was 162 in 213 (76%).

Post-surgical deterioration of renal function in primary hyperoxaluria.

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.