Renal amyloidosis in a patient with homozygous sickle cell anemia and M694V/M694V mutation.
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Biomedical subjects
Publications and source records attributed to N Duman.
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BACKGROUND: Angiotensin-converting enzyme (ACE) inhibitors have the capability of decreasing left ventricular mass index (LVMI) in chronic haemodialysis (HD) patients. On the other hand, recent reports provide conflicting information regarding the impact of ACE inhibitors on responsiveness to recombinant human erythropoietin (rHuEpo), and there are no data about the effect of withdrawing ACE inhibitors both on rHuEpo response and LVMI in HD patients. METHODS: ACE inhibitors were switched to another antihypertensive medication in 23 out of 68 patients in our HD unit who were receiving both rHuEpo and an ACE inhibitor for more than 1 year. Blood pressure at the pre- and post-dialysis phases, haematocrit levels and rHuEpo doses were determined at the end of the first and of the third years, and the LVMI was determined at the end of the third year. Statistical analyses were done in 15 patients in whom the study could be completed. RESULTS: The mean (+/-SD) haematocrit level was increased from 26.3+6.4% to 29.8+/-6.3% at the first year (P<0.05), and to 29.4+/-6.5% at the third year (P<0.05 vs before), while the mean dose of rHuEpo was decreased from 208.3+/-99.0 UI/kg/week to 141.0+/-91.8 at the first year (P=0.01), and to 141.4+/-81.0 at the third year (P=0.01 vs before). Administration of rHuEpo had been stopped in two patients at the end of the first year. The mean blood pressure level and the mean LVMI were not changed (P>0.05 vs before). There were no significant changes in dialysis parameters, iron status, plasma renin activities, and levels of aldosterone, intact parathyroid hormone, aluminum and erythropoietin. CONCLUSION: The findings of this small uncontrolled study indicate that withdrawal of ACE inhibitors in hypertensive chronic HD patients receiving rHuEpo may result in an increase in haematocrit level, and a decrease in dose of rHuEpo without any significant changes in the blood pressure level and LVMI. Controlled prospective studies are needed to clarify this issue.
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In the present study we investigated the role of endothelin and AT II in radiocontrast nephropathy induced in rats with reduced renal mass (70-75%). Thirty-five male Wistar albino rats weighing between 280 and 400 g were anaesthetized with ketamine (130 mg/kg b.w.) and right total, left 50% nephrectomy were performed. After this operation, the rats were kept under observation for six to eight weeks and then they were randomly separated into three groups. Group I rats were infused with 8.9 ml/kg (or 2.9 g of iodine/kg body weight) Na diatrizoate (Urovision, 1,500 mosm/kg). Group II rats were infused with 0.9% NaCl in an equal volume with the radiocontrast material. Group III rats were given 4.5% NaCl that had the same volume and osmolality as the radiocontrast material. Two hours after the drug infusions, blood and accumulated urine samples were collected from all the rats and tested for endothelin, AT II, BUN, creatinine, uric acid, electrolytes, calcium and phosphorus. We found that the plasma endothelin levels in Group I (77.64 +/- 29.62 pg/ml) were significantly higher than in Group II (20.52 +/- 5.83 pg/ml) and Group III (15.04 +/- 5.15 pg/ml) (t = 8.34 and t = 9.14, respectively, p < 0.001). Therefore elevation in circulating endothelin might have been an additional factor leading to the radiocontrast-induced nephrotoxicity.
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The purpose of this study was to investigate if enalapril could be administered with cyclosporin A (CyA) to reduce its nephrotoxicity. Sixty rats were divided into five groups: group I, Control group; group II, rat treated with oral enalapril; group III, rats treated with CyA: group IV, rats treated with CyA and enalapril; group V, rats treated with enalapril before the CyA therapy. At the end of the therapy mean serum creatinine concentrations were not statistically different between the groups (P > 0.05), in groups treated with CyA there were no statistically significant differences between mean CyA levels (P > 0.05), and mean blood urea nitrogen levels of the groups treated with CyA were significantly elevated (P < 0.05) compared with groups not treated with CyA. Morphologically acute CyA nephrotoxicity was evaluated by the following features: (1) tubular vacuolization, (2) tubular necrosis, (3) tubular microcalcification, and (4) peritubular capillary congestion. These lesions were scored semiquantitatively on a scale from 0 to 4+. The most common tubular pathology was tubular vacuolization, which was more severe in groups III and IV. Tubular necrosis was most severe in group III. In conclusion, enalapril seems to suppress the severest form of CyA nephrotoxicity, namely tubular necrosis, if administered prior to CyA treatment.
BACKGROUND: The present study was performed to assess the value of ambulatory blood pressure monitoring (ABPM) in determining the adequacy of blood pressure (BP) control, and its relationship to echocardiographic findings in haemodialysis (HD) patients. METHODS: We studied 40 non-diabetic adult patients who had been on regular HD treatment for a median duration of 43 months. Twenty-four-hour ABPM was performed using a non-invasive ABP monitor (Pressurescan, ERKA). Casual BP (cBP) was defined as the average of two measurements obtained at two HD sessions, one preceding and one following the ABP recordings, and was calculated for both the predialysis and postdialysis phases. Two-dimensional and M-mode echocardiography were performed in each patient to determine interventricular septal thickness (IVS), left ventricular posterior wall thickness (LVPW), left ventricular fractional shortening (FS), and left ventricular mass index (LVMI). RESULTS: According to average 24-h BP levels, 50% of the patients had systolic hypertension (HT) (> 139 mmHg), and 72.5% had diastolic HT (> 87 mmHg), while only 25% had been diagnosed as HT by cBP measurements (P < 0.01 and P < 0.0001 respectively). Diurnal variation in BP was not present in about 80% of the patients. Echocardiography was normal in only four patients (10%). LVMI and LV wall thickness were correlated to ABPM data better than to cBP measurements. Using stepwise linear regression analysis, LVMI and IVS were positively correlated with systolic BP load (P < 0.0001 and P = 0.0001 respectively), and LVPW was positively correlated with night-time systolic BP level (P < 0.001). CONCLUSIONS: ABPM is necessary to assess the adequacy of BP control, and is well correlated to end-organ damage of HT in HD patients.
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This report describes a girl with phocomelia of the right upper limb, ectrodactyly, sacral hypoplasia and a large skull defect, but with normal growth and mental development. Ultrasonography and intravenous pyelography showed bilateral hydronephrosis and dilated ureters. We conclude that this patient may represent Schinzel-phocomelia syndrome with additional urinary tract anomalies.
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