Diagnostic-Value Of Bone-Marrow Biopsy In Patients With Renal-Disease Secondary To Familial Mediterranean Fever

Systemic AA type amyloidosis with renal involvement is the major cause of morbidity and mortality in patients with familial Mediterranean fever (FMF). A histopathological examination is essential to achieve a definite diagnosis in systemic amyloidosis. The diagnostic yield of the procedure varies according to the biopsy site and renal biopsy has the highest yield. On the other hand this procedure has its own complications and requires hospitalization of the patient. Alternative biopsy sites have been proposed with varying degrees of sensitivity and morbidity to reduce the morbidity and mortality of solid organ biopsies. We performed bone marrow biopsies in 39 patients with FMF who had different stages of renal disease. Thirty-one (79.5%) of the 39 specimens showed significant perivascular amyloid infiltration when stained with crystal violet and Congo red. An immunoperoxidase stain with a monoclonal antibody proved that these deposits were AA type amyloid. We suggest that bone marrow biopsy can be utilized for a safe and quick diagnosis of systemic amyloidosis in patients with FMF and renal disease.Wo

Hypernatraemia And Polyuria Due To High-Dose Colchicine In A Suicidal Patient

WoSScopu

Serum Thrombopoietin Levels in Haemodialysis Patients: Involvement of Arteriovenous Fistula

Background. Thrombopoietin (Tpo) is a recently cloned growth factor which plays a critical role in the regulation of thrombopoiesis. Tpo has also been shown to stimulate in vitro and in vivo erythroid cell growth. Although Tps transcripts were detected in hepatocytes, proximal tubules and endothelium, mechanisms regulating the level of circulating Tpo have not been fully delineated. Changes in the vessel wall and blood flow in arteriovenous fistula (AVF)might alter Tpo activity. Methods. Serum thrombopoietin levels and serum erythropoietin levels in samples concurrently obtained from venous returns of AVF and contralateral peripheral veins in 31 haemodialysis patients were determined and compared with 12 healthy controls. Levels were also compared between 14 haemodialysis patients (group I) treated with recombinant human erythropoietin (rHu-Epo) and 17 haemodialysis patients (group II) not requiring rHu-Epo. Results. Serum Tpo levels (44.8 +/- 23.9 pg/ml, vs 129.9 +/- 113.6 pg/ml, P < 0.05) and platelet counts (194 +/- 55. 10(6)/ml vs 273 +/- 94. 10(6)/ml, P < 0.05) of haemodialysis patients were lower than healthy controls. Serum Tpo levels were inversely correlated with platelet counts in the control group (R=-0.61, P < 0.05), but not in haemodialysis patients. Tpo concentrations of AVF samples were lower than peripheral venous samples (31.6 +/- 17.7 pg/ml vs 44.8 +/- 23.9 pg/ml, P=0.001). No significant difference was present between the serum Tpo concentrations of haemodialysis patients in group I and group II. Serum Tpo levels were not correlated with haemoglobin levels or serum erythropoietin levels in haemodialysis patients. Conclusion. Decreased serum Tpo levels despite low platelet counts in haemodialysis patients suggest that the proposed feedback mechanism of platelet uptake of Tpo is not fully operative in these patients. Moreover, AVF might affect the local production and/or catabolism of this growth factor.WoSScopu

Effect of Enalapril on Exaggerated Erythropoietin Response to Phlebotomy in Erythrocytosic Renal Transplant Patients

Background. Exaggerated erythropoietin (EPO) response to phlebotomy regardless of the baseline EPO levels have been shown in patients with post-transplant erythrocytosis (PTE) and administration of angiotensin-converting enzyme inhibitors (ACE-1) seems to be effective in controlling PTE. However, the mechanism of this ACE-1 induced reduction in haematocrit (Hct) is not well known. Although some authors have suggested that ACE-1 may reduce EPO secretion, this is still controversial. The aim of the present study was to assess the effect of a single dose ACE-1 on exaggerated EPO response to phlebotomy. Methods. In this study. we compared serum EPO and renin (PRA levels of 10 PTE patients, 10 non-PTE patients and 10 healthy blood donors before and after phlebotomy. The effects of a single dose of ACE-1 (enalapril, 5 mg p.o.) in PTE patients were also evaluated in the second phlebotomy. Results. While the mean basal serum EPO level was significantly higher in the PTE group than the other two groups (P < 0.01), the mean basal PRA levels did not differ significantly between these groups. Serum EPO and PRA levels increased significantly after the phlebotomy (P < 0.001) and exaggerated EPO response to phlebotomy was suppressed by single dose enalapril (P < 0.001) in the PTE patients. Conclusion. The present study has shown that the renin-angiotensin system plays an important role in EPO formation and the Hct lowering effect of the ACE-1 is through reduction of EPO in PTE patients.WoSScopu

Bone Mineral Density in Haemodialysis Patients: A Comparative Study of Dual-Energy X-Ray Absorptiometry and Quantitative Ultrasound

Background. Quantitative ultrasound (QUS) of bone is a relatively new technique that appears to assess 'bone quality' in addition to bone mineral density. The purpose of this study was to evaluate the diagnostic potential of QUS of calcaneum and to correlate it with dual energy X-ray absorptiometry (DEXA) in chronic haemodialysis patients. Methods. Broad-band ultrasound attenuation (BUA; dB/MHz) and speed of sound (SOS; m/s) of calcaneum and DEXA (g/cm(2)) measurements of the lumbar spine and hip were made in 39 patients. The indices obtained by either method were compared with age-and sex-matched controls. Calcaneal measurements were correlated to DEXA and relevant clinical and biochemical data of patients. Results. BUA and SOS values were markedly reduced in dialysis patients compared to controls (59.1+/-13.8 vs 73.0+/-16.2 dB/MHz, P<0.001 and 1533+/-28 vs 1560+/-29 m/s, P=0.014 respectively). There was a moderate, but significant association between calcaneal parameters and DEXA (r = 0.32-0.53, P < 0.05). Both BUA and SOS scores were inversely correlated with age (r = -0.69, P < 0.001) and duration of menopause (r = -0.74, P < 0.01). Additionally, BUA values showed a moderate negative association with serum intact parathyroid values (r = -0.38, P = 0.018). Conclusion. Chronic haemodialysis patients have reduced calcaneal BUA and SOS scores. QUS of the calcaneum is an easy-to-apply and radiation-free technique. It could be a useful substitute for assessment of bone density in such patients. However, further studies in large patient groups and comparisons with plasma markers of bone turnover and bone biopsy findings are needed to assess its potential place in the management of renal osteodystrophy.WoSScopu

EFFECT OF DESMOPRESSIN (DDAVP) ON PROTEIN-C AND PROTEIN-C INHIBITORS IN UREMIA

The effect of desmopressin (DDAVP) on protein C (PC) and PC inhibitors was investigated in 7 uremic predialysis patients, 7 hemodialysis patients and 7 controls. Significant decrease in PC activity was observed in all groups after DDAVP administration. Desmopressin did not influence PC antigen and two well-known PC inhibitors, plasminogen activator inhibitor-3 and alpha1-antitrypsin. Thus, it is suggested that DDAVP-induced decrease in PC activity is not related to the changes in PC inhibitors; further studies are needed to clarify the precise mechanisms of the effect of DDAVP on PC in uremia