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Biomedical subjects

S Ozen

Publications and source records attributed to S Ozen.

At least 91 records · Page 5Linked to original sources

Membranoproliferative glomerulonephritis in childhood: factors affecting prognosis.

Membranoproliferative glomerulonephritis (MPGN) is a distinctive form of chronic glomerulonephritis. We present the results of our 96 paediatric patients with MPGN, reporting the survival and factors affecting prognosis in these patients. There were 64 boys and 32 girls with an age range of 2-17 (mean 10.6 +/- 3.7) years. All patients initially received oral corticosteroid therapy; remission was achieved in 22.9%. The unresponsive 77.1% either received cyclophosphamide and/or pulse methylprednisolone; 25.4% and 50.0% of these patients entered complete remission, respectively. The overall 1-year renal survivals of the MPGN patients were 90.1%, 5-year and 10-year survival rates were 81.9% and 61%, respectively. At multivariate analysis the factors affecting renal prognosis were haematuria at presentation (p < 0.05, risk factor 3.52), urinary protein/creatinine ratio (p < 0.05, risk factor 1.06 per 1 unit) and low haemoglobin values (p < 0.05, risk factor 1.43 for each 1 g/dl decrement). We suggest that more aggressive immunosuppression therapy should be instituted in patients unresponsive to steroids and that the aforementioned risk factors are higher for the development of renal failure.

Adolescent↗

Familial Mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis.

UNLABELLED: Familial Mediterranean fever (FMF) is a genetically transmitted disease characterized by recurrent attacks of fever and serositis. The most important complication of this disease is the development of amyloidosis. We present our analysis of 425 FMF patients without and 180 with amyloidosis (123 FMF having amyloidosis type 1 and 57 FMF having amyloidosis type II). The male female ratio was higher in the amyloidosis population (111/69) when compared to the FMF population (225 200) (P = 0.048). Consanguinity rate was the same among FMF and amyloidosis groups. However, a family history of amyloidosis was significantly more frequent in the amyloidosis group (P = 0.00001). Multivariate analysis has revealed that in FMF patients, the presence of a family history of amyloidosis plus consanguinity has a 6.04 fold increased risk of amyloidosis (P < 0.0001). The 5-year chronic renal failure free survival was 43.1% and 18.7% in type I and type II amyloidosis, respectively. The time interval to develop chronic renal failure after the development of amyloidosis was 4.8 in type I and 3.0 years in type II. respectively. We found ten cases of Henoch-Schönlein Purpura and nine of polyarteritis nodosa among our patients. The significance of the association between FMF and vasculitis awaits to be clarified. Among the FMF patients put on colchicine therapy (435), only 10 (2.3%) have developed amyloidosis confirming that this drug protects from amyloidosis. CONCLUSION: Since the presence of a familial history of amyloidosis has been defined as the most important risk factor in the development of amyloidosis, we suggest that additional genetic factors may be operative in the development of amyloidosis.

Amyloidosis↗

Idiopathic myelofibrosis (agnogenic myeloid metaplasia): clinicopathological analysis of 32 patients.

The prognostic value of clinicopathological parameters, recorded at diagnosis, in idiopathic myelofibrosis (IMF) was retrospectively analyzed in a consecutive series of 32 patients followed for a minimum of 5 and maximum of 134 months in the period 1983-1994. Of the 32 patients, 18 were males and 14 were females. The mean age was 59.8 years (S.D., 10.4; range, 44-78 years). At the time of closure of the study (May 1995), 10 patients were dead, 18 were alive, and four were lost to follow up. The mean survival was 41 months (range 2-130 months). Univariate analysis showed that the following features were associated with a significantly shorter survival: (1) anemia (hematocrit <30%), (2) platelet count < 100,000/mm3. Age, sex, size of spleen and liver, WBC count, increased reticulin and collagen fibrosis were not of prognostic significance. Clinicopathological correlation was found between the sinusal hemopoiesis and the number of WBC precursors in peripheral blood, and between WBC precursors and spleen size. Medullary fibrosis was found to be associated with striking predominance of large and very mature megakaryocytes. Erythroid hyperplasia in the bone marrow correlated negatively with WBC precursors and spleen and liver size, and positively with reticulocyte count.

Adult↗

Caffeic acid phenethyl ester to inhibit posterior capsule opacification in rabbits.

PURPOSE: To assess whether caffeic acid phenethyl ester (CAPE) prevents posterior capsule opacification (PCO) by suppressing the transformation of the lens epithelial cells. SETTING: Departments of Ophthalmology, Chemistry, and Pathology, Turgut Ozal Medical Center, University of Inonu, Malatya, Turkey. METHODS: Twenty pigmented island rabbits having phacoemulsification in their right eyes were randomized into two groups. In group 1, 10 micrograms/ml of CAPE was added to the anterior chamber irrigating solution and a 1% solution of CAPE was injected subconjunctivally for 3 weeks postoperatively. The irrigating solution in Group 2 (control) did not include CAPE. The development of PCO was assessed weekly and its density was graded by slitlamp biomicroscopy. Histologic analysis was performed 3 months after surgery. RESULTS: Group 1 had clear capsules or minor PCO. Group 2 developed more severe PCO or complete opacification. The difference between the two groups was statistically significant (P = .04). CONCLUSION: These preliminary results indicate that CAPE is effective in suppressing PCO in pigmented rabbits and may be beneficial in clinical use in humans because it has no documented harmful effects on normal cells.

Animals↗

Genetic linkage study of familial Mediterranean fever (FMF) to 16p13.3 and evidence for genetic heterogeneity in the Turkish population.

Familial Mediterranean fever (FMF) is an autosomal recessive condition that is almost entirely restricted to the non-Askhenazi Jews, Arabs, Armenians, and Turks. Genetic linkage study of a large group of non-Turkish families has previously mapped the FMF locus to the 16p13.3 region and shown that this locus resides 0.305 cM distal to D16S246. Furthermore, allelic association has also been shown with D16S3070 (75%) and D16S3275 (66%). However, no genetic heterogeneity has been described for any of the three major reported groups of FMF families. Here, we describe the genetic linkage relationship of the fourth major group of Turkish families and report the first evidence for genetic heterogeneity of this condition. Two point linkage analysis and haplotype inspection of 15 DNA markers from the reported region of the FMF locus identified tight linkage in a group of six Turkish FMF families. A maximum lod score of 9.115 at theta = 0.00 was observed for D16S3024. Nine other DNA markers provided similar evidence of linkage with lod score values of above 5.21. However, two other FMF families were completely unlinked to this region of chromosome 16. Haplotype construction of DNA markers in five consanguineous linked families showed that a segment of homozygosity has been conserved for D16S3070 and D16S2617. No other DNA markers showed any such conservation. Therefore, we suggested that these two markers reside in close proximity to the FMF locus. Furthermore, we observed 80% allelic association with D16S2617 but no association with D16S3070 or any other DNA markers from the FMF critical region. In summary, we conclude that our Turkish families are also linked to the reported FMF locus at 16p13.3, there is a genetic heterogeneity for this condition at least in our group of Turkish families, and D16S2617 is in linkage disequilibrium in the Turkish FMF families. Combination of this study with previously published observations suggests that the FMF locus resides between D16S246 and D16S3070/D16S2617 and within a region of about 250-300 kb.

Adolescent↗

Long-term treatment of nocturnal enuresis with desmopressin intranasal spray.

The purpose of this study was to determine the efficacy and safety of long-term treatment of nocturnal enuresis with desmopressin intranasal spray. Sixty-five children with primary nocturnal enuresis with a mean age of 11.3 years (range 7-17) underwent a 2-week observation period followed by dose titration period of 1 week. Those children completely dry with desmopressin entered a randomized, placebo-controlled, double-blind phase lasting 2 weeks, followed by a 6-month open treatment. The enuretic status of the children was documented for 2 weeks after the treatment was stopped. Eleven children had no change from baseline wetting with desmopressin. Thirty-two children receiving 20 mg and 9 children with 40 mg desmopressin were completely dry. Thirteen children were wet 1-2 nights per week, which was better than in the pretreatment period. During the 6-month open-treatment period, the effect of desmopressin was found to be stable. No side effects or adverse reactions were encountered. Two weeks after the treatment was stopped, 25 children were still completely dry (38% of the initial study population, 50% of the responders). The cure rate appeared to continue beyond 18 months after discontinuation of the treatment.

Administration, Intranasal↗

The role of cytokines in Henoch Schonlein purpura.

Serum levels of tumor necrosis factor (TNF) and interleukin(IL-1) were studied in 20 HSP patients, in the acute phase and after remission, by ELISA technique. Skin biopsies obtained during the acute phase both from a lesion and from unaffected skin, as well as during remission, were immunostained for TNF, IL-1, and IL-6. The mean age of the patients was 9.8 (5-13). Mean serum TNF levels during the acute phase and remission were 14.0 +/- 8.9 pg/ml, and 6.8 +/- 2.4 pg/ml, respectively (p < 0.05). Serum TNF levels in patients with renal involvement (18.8 +/- 10.2 pg/ml) were significantly higher than in those without (10.8 +/- 6.5 pg/ml) (p < 0.05). Serum levels of IL-1 in the acute phase and remission were undetectable. All specimens showed leukocytoclastic vasculitis. Immunohistochemical studies revealed TNF, and a less intense IL-1 and IL-6 staining in the nucleated epidermal layer, with a granular, intracellular pattern. Staining was significantly increased in the affected skin during the acute phase. These results suggest that TNF, IL-1, and IL-6 may play a role as a mediator of inflammation in HSP.

Adolescent↗

Enalapril-induced anemia in a renal transplant patient.

Side-effects of angiotensin converting enzyme (ACE) inhibitors, such as a slight decrease in hematocrit, are increasingly being reported. A 14-year-old renal transplant patient on enalapril therapy developed anemia with reticulocytosis. She was investigated for other causes of anemia and enalapril therapy was ceased. Her hemoglobin level increased and reticulocyte count decreased after cessation of therapy. No other cause of anemia was found. Although anemia in patients receiving ACE inhibitors such as enalapril has previously been reported, this is the first reported patient who developed anemia associated with mild reticulocytosis and macrocytosis.

Adolescent↗

Anaemia in juvenile chronic arthritis.

Anaemia is a common manifestation of juvenile rheumatoid arthritis (JCA). We have evaluated 26 JCA patients with anaemia and compared their laboratory parameters to those without anaemia. In the patients with anaemia, activation criteria such as erythrocyte sedimentation rate (ESR) and CRP were significantly higher than in those without anaemia. Anaemia was present in all systemic JCA patients and was present in 42% and 78% of the oligoarticular and polyarticular types, respectively. Serum iron levels and transferrin saturations were low in all, whereas serum iron-binding capacities of the patients were normal. Mean ferritin level was 249pg/l (range 8.46-1000pg/l). There was a significant correlation between ferritin levels and CRP and ESR (r = 0.48 and r = 0.55 respectively) (both p < 0.05). Epo levels were normal. Twelve (60%) of the bone marrow aspiration specimens stained positive for iron whereas 40% stained negative; there were also changes suggestive of myelodysplasia. Sideroblasts were also decreased in number. Thus, in these patients iron is not sufficiently transferred to the erythroid series and/or cannot be used by erythroblasts, accompanied by a possible absolute iron deficiency. Thus we suggest that the iron in JCA tends to be stored in the form of ferritin, not in an accessible form and impaired metabolism along with other factors are effective in the anaemia of JCA.

Adolescent↗

Plasma lipids and lipoproteins in juvenile chronic arthritis.

Altered levels of high density lipoprotein (HDL), low density lipoprotein (LDL), and very-low density lipoprotein (VLDL), as well as apolipoproteins have been previously described in rheumatoid arthritis patients. We have attempted to evaluate the serum triglyceride, total cholesterol, cholesterol in DHL, LDL, apolipoprotein A1 (apo-A1) and apolipoprotein B (apo-B) levels in juvenile chronic arthritis (JCA) and to correlate them with CRP and ESR in the active and non-active stages of JCA. A total of 37 children no fulfilled ARA criteria for the diagnosis of JCA were studied. There were 18 girls and 19 boys. Age range was 2.5-16 years with a mean of 9.5. The mean duration of disease was 1.8 years. Nineteen patients were accepted to have active disease. Eighteen age and sex matched healthy children served as controls. Apo-A1 was significantly lower in the active JCA group when compared to inactive patients and healthy controls (both p < 0.05). There were significant inverse correlations between apo-A1 and both ESR and CRP levels in these patients (r = 0.67, p < 0.05 and r = -0.61, p < 0.-05, respectively). Although mean LDL levels were numerically lower in the JCA patients (67.2 mg/dl in the active and 68.6 mg/dl in the inactive patients) the difference with healthy controls (91.7 mg/dl) was not statistically significant. There was no significant differences in regard to triglyceride, total cholesterol, cholesterol in HDL, and apo-B levels between neither of the groups. We conclude that JCA patients have a dyslipoproteinaemic state with already altered metabolism of lipids at different stages of the chronic inflammation from active to inactive disease.

Adolescent↗

Diffuse mesangial sclerosis: a unique type of congenital and infantile nephrotic syndrome.

Clinical and pathological findings in four Turkish infants with isolated diffuse mesangial sclerosis (DMS) are presented. All the patients were offsprings of consanguineous marriages and two had similarly affected sibs indicating an autosomal recessive inheritance. The onset of the nephrotic syndrome was at 7, 17, 11 and 3 months of age. They all died in a state of renal failure complicated by infections at the ages of 11, 33, 13 and 5 months. DMS was diagnosed at postmortem examination in all. Fluorescence-microscopical studies in all and an electron-microscopical study in one revealed nonspecific findings. The shorter survival in three of the cases was thought to be due to intervening infections. The variation of the clinical features along with the fluorescence and electron-microscopical findings are consistent with the previously mentioned heterogeneous aspect of DMS.

Consanguinity↗

Comparison of ceftriaxone versus cefotaxime for childhood upper urinary tract infections.

It is very important to treat patients with upper urinary tract infections (UTIs) promptly and effectively because of the potential sequelae. In the present study we compare the efficacy of the two cephalosporins, ceftriaxone and cefotaxime, in childhood pyelonephritis. The study protocal included 10 days of drug therapy. Both in patients receiving ceftriaxone and cefotaxime, successful eradication was achieved at the second day of therapy. The overall cure rate was significantly better in the ceftriaxone group than the cefotaxime group in terms of recurrence and reinfections (p < 0.05). Furthermore, in the complicated group, ceftriaxone was slightly superior to cefotaxime, approaching significance in terms of preventing recurrent infections. No serious adverse effects were observed in either of the groups. The present study has shown that ceftriaxone exhibits favorable clinical and bacteriologic efficacy in patients with complicated and uncomplicated upper UTI. Once-daily injection of ceftriaxone in children is also an attractive advantage of the drug when compared to twice-daily cefotaxime.

Adolescent↗

Systemic oxalosis: pathognomonic renal and specific extrarenal findings on US and CT.

Hyperoxaluria is characterized by nephrocalcinosis and nephrolithiasis on radiological examination and may also result in diffuse deposition of calcium oxalate crystals in multiple extrarenal organs (oxalosis). In two cases, the renal findings of primary hyperoxaluria were diagnosed by ultrasound and computed tomography scans. In addition to renal involvement, both patients had liver involvement, and one patient had cardiac involvement.

Child↗