Urinary albumin to creatinine (ACR) and beta2 microglobulin to creatinine ratios (BCR) are the surrogate and robust markers of renal glomerulopathy and tubulopathy, respectively. These markers predict short-term renal deterioration and mortality in various conditions. We aimed to assess the frequency and predictors of glomerular and tubular defects, renal impairment, and hyperfiltration in 96 adult patients with beta thalassemia intermedia and major. ACR > 300 mg/g creatinine and BCR > 300 μg/g creatinine were used to define the renal glomerular and tubular damages, respectively. Glomerular filtration rate (eGFRcreat) was estimated according to 2009 the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. Decreased eGFRcreat was defined as less than 60 mL/min per 1.73 m(2). Renal glomerular and/or tubular defects were observed in about 68.8 % of all patients. Forty percent of patients had glomerular hyperfiltration. None of the patients had a decreased eGFRcreat. T2* value ≤20 msec on cardiac magnetic resonance (cMR) was the only independent predictor of glomerular damage (p = 0.013). Use of alendronate was associated with less renal tubular damage (p = 0.007). Female gender and previous history of splenectomy were the independent predictors of glomerular hyperfiltration in multivariate analysis (p < 0.001 and p = 0.040, respectively). Renal tubular and glomerular damage is frequent in adult patients with thalassemia intermedia and major. T2* value on cMR was the only independent predictor of glomerular damage. However, since we did not explore all the parameters of iron, it is not possible to draw a definite conclusion about the association of cMR and glomerular damage. There is no association with cardiac iron overload/accumulation and tubular damage or hyperfiltration.

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Iron overload is one of the major prognostic factor in thallassemia patients. We aimed to evaluate iron accumulation in the heart and liver by MRI in thalassemia major, thalassemia intermedia, and S-ß thalassemia patients and to examine its association with ferritin and hepcidin levels. Serum ferritin and hepcidin levels were recorded. Iron overload (IOL) in the heart and liver parenchyma was determined based on the standardized T2* and R2 values measured by MRI. The results were evaluated considering the tissue iron overload, serum ferritin and hepcidin levels. Comparing the 109 patients with the 30 healthy controls revealed the mean age: 24.4 ± 11 versus 31.2 ± 5 years, median levels of serum ferritin: 1693 versus 40 ng/mL, and hepcidin: 1.94 versus 0.355 ng/mL; < 0.001, respectively. Comparison of age, serum ferritin and hepcidin levels and MRI findings of the patients with or without IOL revealed that, ferritin and T2* were significantly different in the patients with IOL in cardiac tissue ( = 0.004 and < 0.001), and, age, ferritin and R2 were significantly different in the patients with IOL in liver tissue ( = 0.036, < 0.001 and < 0.001). The MRI-based T2* and R2 values were moderately and inversely correlated with serum ferritin (r = -0.37; < 0.001 and r = -0.46; < 0.001). No correlations were found between the MRI-based T2*, R2 values and serum hepcidin. A moderate and positive correlation existed between serum ferritin and hepcidin (r = 0.45; < 0.001). We considered that, enhanced intestinal iron absorption characterized by decreased serum hepcidin levels in the intervals between successive transfusions were resulted in iron accumulation in our patients.

SummaryGlycated hemoglobin (HbA1c) is used for the assessment of glycemic control in patients with diabetes. The presence of genetic variants of hemoglobin can profoundly affect the accuracy of HbA1c measurement. Here, we report two cases of Hemoglobin G-Coushatta (HBB:c.68A>C) variant that interferes in the measurement of HbA1c by a cationexchange HPLC (CE-HPLC) method. HbA1c was measured by a CE-HPLC method in a Tosoh HLC-723 G7 instrument. The HbA1c levels were 2.9% and 4%. These results alerted us to a possible presence of hemoglobinopathy. In the hemoglobin variant analysis, HbA2 levels were detected as 78.3% and 40.7% by HPLC using the short program for the Biorad Variant II. HbA1c levels were measured by an immunoturbidimetric assay in a Siemens Dimen sion instrument. HbA1c levels were reported as 5.5% and 5.3%. DNA mutation analysis was performed to detect the abnormal hemoglobin variant. Presence of Hemoglobin G-Coushatta variant was detected in the patients. The Hb G-Coushatta variants have an impact on the determination of glycated hemoglobin levels using CE-HPLC resulting in a false low value. Therefore, it is necessary to use another measurement method.Keywords: Hb G-Coushatta (HBB:c.68A>C), HbA1c, cation-exchange HPLC Kratak sadr`ajGlikolizirani hemoglobin (HbA1c) koristi se za procenu glikemijske kontrole kod obolelih od dijabetesa. Prisustvo genetskih varijanti hemoglobina mo`e sna`no uticati na ta~nost merenja HbA1c. Ovde je prikazan izve{taj o dva slu ~aja gde je varijanta hemoglobina G-Coushatta (HBB:c.68A>C) uticala na merenje HbA1c metodom HPLC sa katjonskom izmenom (CE-HPLC). HbA1c meren je metodom CE-HPLC na instrumentu Tosoh HLC-723 G7. Nivoi HbA1c bili su 2,9% i 4%. Ovi rezultati ukazali su nam na potencijalno prisustvo hemoglobinopatije. Prilikom analiziranja varijante hemoglobina, otkriveni su nivoi HbA2 od 78,3% i 40,7% metodom HPLC uz kori{}enje kratkog programa za Biorad Variant II. Nivoi HbA1c mereni su pomo}u imunoturbidimetrijskog eseja na instrumentu Siemens Dimension. Dobijeni su nivoi HbA1c od 5,5% i 5,3%. Analiza mutacije DNK izvedena je kako bi se otkrile abnormalne varijante hemoglobina. Kod pacijenata je otkriveno prisustvo varijante hemoglobina G-Coushatta. Va rijante Hb G-Coushatta uti~u na odre|ivanje nivoa glikoliziranog hemoglobina pomo}u metode CE-HPLC, {to ima za posledicu la`no niske vrednosti. Dakle, neophodno je primeniti druge metode merenja.

β-Thalassemia (β-thal) is a common autosomal recessive disorder resulting from over 300 different mutations of the β-globin genes. Our aim was to create a mutation map of β-thal in the province of Antalya, Turkey. In this study, mutation analysis of a total 146 of β-thal patients followed at the Thalassemia Center of the Antalya Education and Research Hospital, Antalya, Turkey, were included. Direct DNA sequence analysis was performed for mutation scanning of the β-globin gene. One hundred and forty-six patients with β-thal including all types were analyzed, and 14 different β-thal mutations were detected. The most frequently seen mutation was HBB: c.93 - 21G > A [IVS-I-110 (G > A)] (52.7%), followed by HBB: .c.92 + 6T > C [IVS-I-6 (T > C)] (14.4%), HBB: c.-80T > A [-30 (T > A)] (8.2%), HBB: c.315 + 1G > A [IVS-II-1 (G > A)] (8.2%), which made up 83.1% of the observed mutations. Our results indicate the importance of micromapping and epidemiology studies of thalassemia, which will assist in establishing the national prevention and control program in Turkey.

Studies on HIF2α, hepcidin, and ferroportin molecules in beta thalassemia majör (β-TM) patients show that there are disorders in these pathways. In recent years, studies conducted in patients with iron deficiency anemia have investigated the relationship between ghrelin hormone and iron metabolism. In this study, we aimed to contribute to the etiopathogenesis of this disease by examining the changes in ghrelin hormone levels in patients with β-TM. 52 β-TM and 23 controls were included in our study. Cell blood counts, biochemical parameters, HIF2α, hepcidin and ghrelin levels were studied from blood samples taken from the volunteers. Serum HIF2α, hepcidin and ghrelin levels were measured by ELISA method. Erythrocyte indexs, serum total bilirubin, direct bilirubin, iron, unsaturated iron binding capacity, total iron binding capacity and ferritin levels showed significant differences between the two groups (p < 0.05). There was no significant difference between the two groups in serum HIF2α and hepcidin levels. When the patient group and healthy controls were compared, serum ghrelin levels were found to be significantly higher in the patient group (p < 0.05). There was a significant positive correlation between serum ghrelin and ferritin levels in the patient group (r = 0.401) (p < 0.05). It suggested that high ghrelin levels may have an important role in the regulation of impaired iron metabolism in patients with β-TM. The positive correlation between serum ghrelin levels and ferritin suggested that serum iron may have an important role in ghrelin synthesis, and that increased ghrelin levels may be a factor that increases ferritin synthesis.

Bedel, et al.: Pharmacology of Punicic AcidPlant extracts and nutraceuticals are the most ancient and widespread form of medication employed by the general population. The pomegranate is a prehistoric, mystical and a highly differentiated fruit. Moreover, pomegranate is found in some medicinal systems as a cure for a variety of illnesses. Pomegranate has been used for a long time for nutraceutical purposes. Current research indicates that the most medicinally useful pomegranate components include ellagitannins, anthocyanins, anthocyanidins, flavonoids, estrogenic flavonols and flavones. Also pomegranate seed oil contains 64-83% punicic acid. Therefore, this review focussed on the effects of punicic acid, particularly those that have been reported such as the anticarcinogenic, antioxidant, antiinflammatory and antidiabetic effects. As nutraceuticals appear to play a major role in the prophylaxis of various diseases, punicic acid could be an important and phytoconstituent among these agents. Nutraceuticals are generally regarded as safe to use with lower incidence of side effects. In spite of all these reports it is obvious that there is a clear need for more clinical studies.

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