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Biomedical subjects

D Chauveau

Publications and source records attributed to D Chauveau.

At least 73 records · Page 4Linked to original sources

Autosomal dominant polycystic kidney disease: from molecular genetics to the patients.

One of the gene loci (PKD1) responsible for autosomal dominant polycystic kidney disease was located in 1985 to the short arm of chromosome 16. The clinical consequences of this finding are analyzed. Genetic heterogeneity has been demonstrated since 5%-15% of the families inherit a non-PKD1 mutation. Progress in molecular genetics allows better classification of patients with some atypical manifestations, e.g., those with early renal failure or those with congenital hepatic fibrosis. Identification of the gene(s) and of their defects will provide further progress.

Chromosome Mapping↗

Investigation of the biochemical effects of renin inhibition in normal volunteers treated by an ACE inhibitor.

1. In order to investigate accurately the biochemical effects of renin inhibition in man, we have developed a sensitive assay to measure angiotensin I (1-10) decapeptide. 2. Angiotensins were extracted from plasma by adsorption to phenylsilylsilica, and angiotensin I (Ang I) was quantified by radioimmunoassay. The detection limit was 0.77 fmol ml-1, and the extraction recovery of [125I]-Ang I added to albumin buffer was 83% at the inflection point (10 fmol ml-1) of the standard curve. The overall recovery was 98.5 +/- 3.5%. The intra- and inter-assay reproducibility was 10.4% and 9.7% respectively. Cross-reactivity of the antiserum used was low (less than 0.3%) with all angiotensin peptides tested except Ang (2-10) nonapeptide. 3. A human pharmacological model was subsequently used to assess in vivo the biochemical effects of the renin inhibitor CGP 38560A. Six healthy volunteers received 20 mg lisinopril, a long-acting ACE-inhibitor. During the following 24 h, the renin-angiotensin system was reset with typically elevated active plasma renin and Ang I, at respectively 275 and 429% of basal values. 4. In a randomized three-way cross-over protocol, the six volunteers received a 30 min infusion of the renin inhibitor CGP 38560A (125 or 250 micrograms kg-1) or 5% glucose. The fall in plasma Ang I was 92% and 97.5% after the lowest and highest dose of the renin inhibitor, respectively. A concomitant increase in active plasma renin was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

The effect of uninephrectomy on progression of renal failure in autosomal dominant polycystic kidney disease.

The evolution of renal failure was compared in 47 patients (21 male, 26 female) with autosomal dominant polycystic kidney disease (ADPKD) in Germany, France, Spain, and Portugal who had undergone uninephrectomy (UNX) (median age at uninephrectomy, 41 yr; range, 22 to 54) and 47 non-UNX matched controls. UNX was usually performed because of uncontrolled urinary tract infection (N = 30), stones (N = 8), trauma (N = 2), or hemorrhage (N = 7). Median serum creatinine at UNX was 2.1 mg/dL (0.9 to 4.3). Twenty-eight of the 47 uninephrectomized patients progressed to end-stage renal failure. When the age at renal death was evaluated by survival analysis, only minor and nonsignificant acceleration was seen in the uninephrectomized patients (median, 50 yr; p25 = 43.6 yr; p75 = 58.3 yr, where p is the percentile) compared with non-UNX patients matched for age, sex, and serum creatinine at the time of UNX in the propositus (51.2 yr; p25 = 48.6 yr; p75 = 56.1 yr). In addition, the median interval for serum creatinine to rise from 4 to 8 mg/dL was similar in UNX (21.3 months) versus nonuninephrectomized ADPKD patients (21.9 months). Renal survival differed in the two genders. In females, no significant difference of age at renal death was found between UNX (median age, 51.6 yr) and non-UNX ADPKD patients (53.7 yr). In male UNX patients, age at renal death was slightly (but not significantly) less than in non-UNX patients (median age, 47.3 versus 52.7 yr). All male patients reaching end-stage renal failure before age 44 were severely hypertensive.

Adult↗

Antigen specificities and clinical distribution of ANCA in kidney diseases.

The antigenic specificity and clinical distribution of the antineutrophil cytoplasmic antibodies (ANCA) in kidney diseases have recently been extensively studied. In patients with systemic vasculitis, the great predominance of two major ANCA antigens, proteinase 3 (PR3) and myeloperoxidase (MPO), is now established. PR3 and MPO are colocalized in the azurophilic granules of neutrophils and translocated to the cell surface during activation, and thus are able to interact with autoantibodies after neutrophil preactivation. Furthermore, by comparison of amino acid and DNA sequences, it has been shown that PR3 is identical to myeloblastin, which has been described independently and is involved in the control of growth and differentiation of leukemic cells. Aside from the two major ANCA antigens, a number of neutrophil cytoplasmic antigens recognized by ANCA have been identified, including human leukocyte elastase, lactoferrin, CAP57, and cathepsin G. These rare ANCA specificities occur in a limited number of patients. The variety of ANCA antigen specificities contrasts, however, with the fact that the vast majority of ANCA-positive sera are monospecific for one single ANCA antigen. With regard to clinical distribution, ANCA have major diagnostic significance in the four conditions in which they are frequently detected: Wegener's granulomatosis (WG), Churg and Strauss Syndrome (CSS), microscopic periarteritis (MPA), and necrotic and crescentic glomerulonephritis (NCGN). However, the initial dichotomy between MPO-associated vasculitis (NCGN, MPA) and that associated with anti-PR3 antibodies (WG) appears far from absolute.

Autoantibodies↗

[Use of intravenous nicardipine for the diagnosis of renovascular hypertension].

UNLABELLED: The diagnosis of remediable renovascular hypertension (RVH) requires demonstration of lateralization of renal vein renin (RVR). In order to increase the accuracy of RVR ratio, we investigated the acute effects of a bolus of Nicardipine (Nic.: 4 mg i.v.) on hemodynamics and RVR in 19 patients. 13 patients had an unilateral renal artery stenosis greater than 75% (RVH: 10 atheroma and 3 fibrodysplasia) and 6 patients had essential hypertension (EH). In 6 patients (5 RVH and 1 EH) treatment could not be discontinued and only a monotherapy by central alpha-agonists was prescribed. RVR samples were obtained 15 minutes after renal vein catheterization (baseline values: T0) and 10 minutes after Nic. injection (T10). During the whole procedure, mean blood pressure (MBP) and heart rate (HR) were monitored every 2 minutes by an automatic device. Active renin was measured by a new immunoradiometric assay. A RVR ratio (stenotic/contralateral side) greater than 1.5 was considered as a positive ischemic index. RESULTS: The relative changes in MBP and HR between T0 and T10 were of same magnitude in both groups. No patient suffered any untoward effect from the fall in MBP. Nic. increased RVR release from both sides in RVH group as well as in the EH group. At baseline, 6/13 of the RVH patients and none of the EH patients had a RVR greater than 1.5. After Nic. injection, all the RVH patients had a RVR greater than 1.5 and none among EH patients. We conclude that single i.v. Nic. bolus is a safe and a reliable procedure which increases diagnostic accuracy by enhancing RVR when there is an unilateral RVH disease.

Adult↗

Epithelioid sarcoma of soft tissues: a case of extrarenal renin-secreting tumour.

The case of a young woman presenting with a renin-secreting soft tissue sarcoma is described. The primary extrarenal tumour as well as metastatic disease were associated with severe hypertension and both required surgical treatment. The location of these rare malignant tumours and their association with renin-dependent hypertension is discussed. In cases of this type, reappearance of hypertension suggests tumour recurrence.

Adolescent↗

[Post-neurosurgical purulent meningitis. 31 cases].

Thirty-one cases of post neurosurgical bacterial meningitis from one center were reviewed to determine their promoting factors and responsible organisms. Most cases occurred after a protracted operation (6 hours on average). 26% cases were related to a primary cutaneous infection. 58% organisms were Gram negative bacilli. The course of meningitis was often complicated or prolonged due to a scalp decubitus ulcer or cerebrospinal fluid leakage. Vancomycin is often requested during staphylococcal meningitis. Third generation cephalosporins have low MIC against many Gram negative bacilli and should be used. Pefloxacin, a new quinolone, may be valuable. Treatment of scalp wound infection is often requested.

Adolescent↗

Piridoxilate-associated nephrocalcinosis: a new form of chronic oxalate nephropathy.

Piridoxilate is an association of glyoxylic acid and pyridoxine in which pyridoxine is supposed to facilitate in vivo transformation of glyoxylic acid to glycine rather than to oxalic acid. However, it has recently been shown that long-term treatment with piridoxilate may result in overproduction of oxalic acid and in calcium oxalate nephrolithiasis. We report a patient in whom piridoxilate induced both oxalate nephrolithiasis and chronic oxalate nephropathy with renal insufficiency, an association that has not been previously described. Therefore, piridoxilate should be added to the list of chemicals responsible for chronic oxalate nephropathy.

Aged↗

Cholangiocellular carcinoma in polycystic kidney and liver disease.

Cholangiocellular carcinoma developed in two uremic patients with polycystic kidney and liver disease, who had been treated with intermittent hemodialysis for one and nine years. In one case, in situ transformation of the liver cyst epithelium into cholangiocellular carcinoma could be demonstrated. The incidence of cholangiocellular carcinoma in patients undergoing long-term dialysis for polycystic kidney and liver disease, however, has yet to be determined.

Adenoma, Bile Duct↗