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Biomedical subjects

A K Bayazit

Publications and source records attributed to A K Bayazit.

6 recordsLinked to original sources

Intravenous pulse cyclophosphamide therapy in focal segmental glomerulosclerosis.

AIMS: We herein report the results of intravenous pulse cyclophosphamide (IVCP) therapy of 5 patients with steroid-resistant focal segmental glomerulosclerosis (FSGS). All patients had been treated with oral and intravenous pulse methylprednisolone and failed to respond to steroids from onset and were considered as primary steroid-resistant. Before starting IVCP, all patients were also treated with other immunosuppressive drugs with or without steroids, but none of them responded to such therapies and no patient had any NPSH2 gene mutations. METHODS: IVCP was given monthly at a dose of 500 mg/m2 for 6 months. At the end of 6 months, IVCP was discontinued in case there was no response. Otherwise, IVCP was continued for every 2 months. Oral prednisone was given concurrently at 60 mg/m2 daily for 6 weeks and then 40 mg/m2 on alternate days for 4 weeks. Prednisone was then tapered to 10 mg/m2 alternate days and continued during the therapy period. RESULTS: Only 1 of these patients achieved remission after IVCP while 4 patients showed no response to IVCP. 2 patients who did not achieve remission progressed to end-stage renal disease (ESRD) and 2 others who had not been treated with cyclosporine before underwent cyclosporine therapy. None of our patients has suffered from adverse effects of IVCP. CONCLUSION: We found that IVCP had a limited beneficial effect in treatment of steroid-resistant FSGS and it may be suggested that IVCP can be tried to treat steroid-resistant patients, also for patients with primary steroid resistance and those who do not respond to other immunosuppressive therapies.

Administration, Oral↗

A child with Beckwith-Wiedemann syndrome and posterior urethral valves.

Beckwith-Wiedemann syndrome is a somatic overgrowth syndrome characterized by a variable incidence of congenital anomalies, including hemihypertrophy, omphalocele, macroglossia and renal malformations. We report a child with Beckwith-Wiedemann syndrome and posterior urethral valves. Urethral valve resection was successfully performed under general anesthesia after voiding cystourethrography. This is the first report of Beckwith-Wiedemann syndrome associated with posterior urethral valves.

Beckwith-Wiedemann Syndrome↗

Mycophenolate mofetil in children with multidrug-resistant nephrotic syndrome.

AIM: The aim of the present study is to report our clinical experiences with MMF in problematic children with chronic glomerulonephritis resistant to corticosteroids and/or other immunosuppressive drugs. PATIENTS AND METHODS: Ten patients with chronic glomerulonephritis resistant to treatment with corticosteroids and other immunosuppressive drugs were treated with mycophenolate mofetil (MMF). Causes of chronic glomerulonephritis were mesangial proliferative glomerulonephritis (4), membranoproliferative glomerulonephritis (3), chronic sclerosing glomerulonephritis (1), focal segmental glomerulosclerosis (1), diffuse endo- and extracapillary proliferative glomerulonephritis (1). MMF 15 mg/kg was used in combination with low-dose corticosteroids and angiotensin-converting enzyme inhibitors. RESULTS: During 24 weeks of MMF therapy, no significant changes were detected in mean serum creatinine, albumin and proteinuria. Severe leukopenia was seen in 1 patient. Additional adverse effects, including nausea and diarrhea, were observed in another patient when the dosage was increased to 20 mg/kg per day. During MMF treatment proteinuria decreased slightly without remission in 6 of 10 patients. CONCLUSION: Further data and clinical trials are needed to evaluate the possible role of MMF in the treatment of chronic glomerulonephritis of similar etiologies in pediatric patients.

Adolescent↗

Urinary nephrocalcin excretion in children with urolithiasis.

The aim of this study was to investigate the role of nephrocalcin in childhood urolithiasis. Forty-one patients with urinary stones and 25 age- and sex-matched healthy controls were admitted to the study. Blood and timed urine samples were taken from both patient and control groups for biochemical analysis. Serum and urine creatinine (Cr) and urinary nephrocalcin (NC) were measured. NC excretion was expressed as a NC/Cr (mg/g) ratio. NC-PreA/Cr and NC-D/Cr ratios were found to be significantly higher in patients than in the control group. No statistically significant differences were found in NC-A/Cr, NC-B/Cr, NC-C/Cr ratios between the patient and control groups. The high NC-PreA/Cr ratio (p = 0.012) observed in stone-forming patients indicates that this ratio may also be an important stimulatory factor for urinary stone disease.

Calcium↗

Natural coagulation inhibitors (protein C, protein S, antithrombin) in patients with sickle cell anemia in a steady state.

BACKGROUND: Patients with sickle cell anemia (SCA) run the risk of having decreased levels of natural coagulation inhibitors. This may be due to either hemostatic abnormalities or hepatic dysfunction. This study is designed to evaluate coagulation profiles of patients with SCA in a steady state and to determine whether hypercoagulable state is present or not. METHODS: Seventeen children with SCA in a steady state were included in this study. The routine hematological evaluation was done with a coulter-counter. Reticulocyte percentage and blood coagulation tests were also determined. The coagulation inhibitors such as protein C (as activated partial thromboplastine time prolongation time), protein S (as Factor V inhibition) and antithrombin (colorimetric assay) were measured in all cases. RESULTS: In the coagulation profile, mean euglobuline lysis time and mean fibrin degradation product levels were both significantly higher in the patient group than in the control group (P<0.05), although other parameters were within normal limits. The values for aspartate aminotransferase (AST), alanine aminotransferase (ALT) and indirect reacting bilirubin were significantly higher in the patient group than in the control group (P<0.001, P<0.005, P<0.0001, respectively). The serum protein levels were normal. Mean factor V level was significantly lower and mean factor VIII level was found significantly higher in the patient group than in the control group (P<0.05 and P<0.005, respectively). Protein C and AT levels were lower in patients with SCA than in control subjects (P<0.001). Protein S levels were also lower in the patient group than in the control group, but the difference between the two groups was not significant (P>0.05). CONCLUSION: It is indicated that antithrombotic functions of patients with SCA are handicapped even in a steady state; and both hemostatic abnormalities and hepatic dysfunction contribute to low levels of natural coagulation inhibitors.

Adolescent↗