Gene polymorphism of complement factor H in a Turkish patient with membranoproliferative glomerulonephritis type II

Membranoproliferative glomerulonephritis (MPGN) is characterized by proliferation of mesangial and endothelial cells and by thickening of the peripheral capillary walls. Type II of the MPGN is associated with complement abnormalities which are factor H deficiencies due to mutations in the complement factor H (CFH) gene. We report a 15-year-old boy diagnosed with MPGN II in whom genetic analyses of the CFH gene revealed that the patient was heterozygote for a polymorphism in exon 2 of the CFH (c.184G>A), heterozygote for a polymorphism in exon 9 of the CFH (c.1204C>T), and heterozygote for a polymorphism in exon 10 of the CFH (c.1419G>A). These data recapitulate a prototypical complement genetic profile, the presence of major risk factors for MPGN II, which support the hypothesis that these dense deposit diseases have a common pathogenic mechanism involving dysregulation of the alternative pathway of complement activation.     

Endobronchial findings of hydatid cyst disease: a report of five pediatric cases

Hydatid disease is still an important public health problem throughout the world. Diagnosis of the disease is generally based on clinical and radiological findings. Evaluation of pulmonary disorders by flexible bronchoscopy (FOB) is a rapidly developing facility, but diagnostic and therapeutic FOB for pulmonary hydatid cysts is still controversial. This study examines the findings of endobronchial hydatid cyst disease in five pediatric patients from Turkey, and clinical experience about this subject is reviewed. All our patients presented with unusual symptoms of the disease, and for all of them, diagnosis had been delayed using current diagnostic methods. As a result of our experience, it can be reported that the endobronchial appearance of the hydatid cyst membrane is whitish-yellow, and it is difficult to differentiate it radiologically from some other common causes of endobronchial lesions in childhood, such as endobronchial tuberculosis, foreign body aspirations, mucous plaques, and granulation scars. The findings of these cases show that, hydatid cyst should also be kept in mind in differential diagnosis of endobronchial lesions. In the diagnosis of pulmonary hydatid cyst in children without typical clinical and radiological findings of the disease, FOB examination is a valuable diagnostic procedure.     

Increased P wave dispersion: a new finding in patients with syndrome X

The present clinical study was undertaken in patients with syndrome X, namely angina with normal coronary arteries, to investigate the presence of increased P wave dispersion by comparing patients with coronary artery disease (CAD) and healthy control subjects. Three groups were studied - group A, 21 patients (48 6 years) with syndrome X; group B, 16 patients (56 9 years) with CAD; and group C, 16 healthy subjects (49 8 years). Patients with CAD were older than those in groups A and C (P=0.005 and P=0.035, respectively). All groups demonstrated similar PQ, QRS and RR intervals. Group B had a lower minimum P wave duration than group C (P=0.05). P wave dispersion in group A was found to be higher than that in groups B and C (P=0.018 and P=0.0001, respectively). Patients with syndrome X demonstrated increased P wave dispersion compared to patients with CAD and healthy subjects. High sympathetic tone or autonomic imbalance observed in patients with syndrome X may affect intra-atrial and interatrial conduction times, and leave them prone to develop atrial arrhythmias.     

The in vivo antioxidant effectiveness of alpha-tocopherol in oxidative stress induced by sodium nitroprusside in rat red blood cells

Reactive oxygen species avidly reacts with nitric oxide (NO) producing cytotoxic reactive nitrogen species capable of nitrating proteins and damaging other molecules which leads to the reduction of erythrocyte deformability. The aim of this investigation was to assess the importance of alpha-tocopherol (Vit-E) in the total antioxidant status of the erythrocytes in sodium nitroprusside (SNP), a nitric oxide donor, induced oxidative stress and its relation to erythrocyte deformability. Male Swiss Albino rats were used in 4 groups, comprising of 10 animals in each group. The first group was the control, and the other groups were administered SNP (10 mg/kg, i.p.), Vit-E (10 mg/kg, i.p.) + SNP, and SNP + L-NAME (10 mg/kg, i.p.), respectively. Relative filtration rate (RFR), relative filtration time (RFT) and relative resistance (Rrel) were determined as the indexes of erythrocyte deformability. In addition, malondialdehyde (MDA, as an index of lipid peroxidation) and nitric oxide levels and the antioxidant activities of glutathione peroxidase (GSH-Px), superoxide dismutase (SOD) and catalase (CAT) were also determined in the red blood cells of all groups revealing the oxidant-antioxidant activity. RFT and the Rrel of the erythrocytes of the SNP-treated rats increased significantly (p<0.05) whereas the RFR of the erythrocytes decreased (p<0.05) in comparison to all groups reflecting the impaired deformability. This reduction in RFR was prevented with both L-NAME or Vit-E incubation. Vit-E has also reduced the Rrel of the erythrocyte which reveals that it has improved the erythrocyte deformability. Lipid peroxidation was suppressed by Vit-E and L-NAME significantly, where the red blood cell deformability was improved. Furthermore, SOD and CAT activities were significantly stimulated with SNP treatment (p<0.05), where as GSH-Px remained unchanged. In the contrary, GSH-Px activity was triggered significantly by Vit-E administration, whereas the SOD and CAT activities were reduced (p<0.05). As a result, these data reveal that Vit-E improves the erythrocyte deformability in SNP-induced oxidative stress by its antioxidant effects on the lipid peroxidation and antioxidant enzyme activities.     

The rate of hepatitis B and C virus infections and the importance of HBV vaccination in children with acute lymphoblastic leukemia

Aim: The aim of the study was to evaluate the rate of hepatitis B and C virus infection and emphasize the importance of hepatitis B virus (HBV) vaccination in leukemic children. Methods: One hundred and sixty children who were treated for acute lymphoblastic leukemia (ALL) at Hacettepe University Faculty of Medicine, Pediatric Hematology Unit were included in the study. They were 71 (44.4%) girls and 89 (55.6%) boys with a mean age of 6.45 +/- 3.87 years. Results: Of these 160 children, 22 (13.8%) were anti-HBs-positive and 138 (86.2%) were anti-HBs-negative at the diagnosis of ALL. Among the 138 anti-HBs-negative children, 67 (41.9%) were vaccinated for HBV during maintenance chemotherapy, and 71 (44.3%) could not be vaccinated. Two (2.9%) vaccinated and 22 (30.9%) unvaccinated children developed HBV infection during the follow-up period (P < 0.001). Among 160 children treated for ALL, 24 (15.0%) had HBV, three (1.9%) had hepatitis C virus (HCV) infections, and 29 (18.1%) had toxic hepatitis. The majority of patients with HBV or HCV infections had high risk (HR) protocol, whereas most of the patients with toxic hepatitis had low risk (LR) protocol, especially St Jude Total XIII LR protocol. Conclusion: Viral hepatitis and toxic hepatitis were observed more commonly in the HR and LR group, respectively, of ALL patients. This could be explained by intensive chemotherapy and more heavy blood product administration in the HR group and the chemotherapeutic agents of methotrexate and 6-mercaptopurine, basic drugs used in the LR group. In respect to protection from these complications, periodical liver function tests, serological tests for HBV and HCV, and vaccination for HBV should be performed for all children with ALL.     

The validation of left atrial strain imaging for the assessment of diastolic functions in patients with ST-segment elevation myocardial infarction

Assessment of left ventricular filling pressure (LVFP) is crucial in patients with ST-segment elevation myocardial infarction (STEMI). Since current guideline recommended echocardiographic parameters have limited value, more comprehensive assessment methods are required in this patient subset.In this study, we aimed to investigate the clinical utility of left atrial reservoir strain (LARS) imaging in patients treated with primary percutaneous coronary intervention (pPCI). Patients who underwent successful pPCI were included. Left ventricular end-diastolic pressure (LVEDP) was measured invasively following pPCI. Left atrial strain imaging was performed following pPCI within 24 h of pPCI. Normal LARS value was accepted as above 23%. We prospectively enrolled 69 patients; there were 18 patients with LARS below 23% who were included into group 1 and rest of the study population included into group 2. There was no significant difference between groups in terms of comorbidities.Troponin and pro-BNP levels were significantly higher in group 1 (p: 0.036 and 0.047 respectively). Left atrial volume and tricuspid regurgitation velocity were similar between groups (p: 0.416 and p: 0.351 respectively). Septal tissue velocity was higher (p: 0.001) and Septal E/e’ ratio was lower (p: 0.004) in group 2. Left ventricular (LV) global longitudinal strain value was higher in group 1 which is consistent with observed lower ejection (LVEF) fraction in group 1 (p: 0.001 for LV strain and p: 0.001 for LVEF). Estimated mean LVFP was also higher in group 1 (p: 0.003).Correlation analyses revealed moderate correlation between LARS and LVEDP (r: ? 0.300). Our results indicate that left atrial strain imaging is a promising tool for the assessment of left atrial pressure in patients with STEMI.

     

Is chronic nipple piercing associated with hyperprolactinemia?

Literature on hyperprolactinemia in the setting of a nipple piercing is limited to individuals with concomitant breast/chest wall infection. It is unclear if chronic nipple stimulation from a piercing alone can cause sustained elevations of serum prolactin. Nipple piercing is emerging as a more mainstream societal form of body art, and the answer to this clinical question would potentially alter patient management. Our aim was to assess serum prolactin levels in subjects with nipple piercing. Inclusion criteria were as follows: men and women ≥18 years old with nipple piercing(s) present >6 months. Exclusion criteria included: women who are pregnant, lactating or <6 months postpartum; subjects on medications known to increase prolactin levels; chest wall/breast infection at the time of phlebotomy or conditions known to be associated with hyperprolactinemia. Three men and eight women were enrolled. Median (range) ages for men and women were 33 (24–42) and 27 years (23–42), respectively. All except one subject had bilateral piercings. The median interval from nipple piercing to blood draw was 4.0 (2.0–12.0) years. None of the subjects had hyperprolactinemia. Median (range) prolactin levels for men and women were 5.6 ng/mL (3.8–7.4) and 8.0 ng/mL (2.8–10.9), respectively. Our results suggest that in the absence of any concomitant infection, chronic nipple piercing is not associated with hyperprolactinemia.     

Allogeneic hematopoietic stem cell transplantation versus hypomethylating agents in patients with myelodysplastic syndrome: A retrospective case–control study

Allogeneic stem cell transplantation (allo‐SCT) is the only potentially curative treatment for myelodysplastic syndrome (MDS). Recently, hypomethylating agents (HMAs) have been shown to improve survival in patients with high‐risk MDS. We conducted a retrospective case–control study to compare survival with these treatment modalities in patients with untreated MDS. Controls were identified using a departmental database and transplant patients were matched in at least three of the following five criteria: year of diagnosis, age, blast percentage, International Prognostic Scoring System cytogenetic risk, and time from diagnosis to treatment. Median overall survival (OS) was 26 and 25 months for, respectively, allo‐SCT [(n = 53); range, 2–210 months] and HMA [(n = 40); range, 2–98 months] (P = 0.89). Four‐year survival rates were 24 and 23% for allo‐SCT patients and the nontransplant cohort, respectively. Patients undergoing allo‐SCT after 2000 had longer median OS compared with those transplanted before 2000 (41 versus 7 months, P=0.001). These results would suggest that prospective studies are needed to delineate the timing and efficacy of allo‐SCT in the HMA era. Am. J. Hematol. 88:198–200, 2013. © 2012 Wiley Periodicals, Inc.     

CD34/CD117 positivity in assessment of prognosis in children with myelodysplastic syndrome

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders that are characterized by morphology identifying dysplastic changes in one or more cell lineages, peripheral blood cytopenias and a propensity to evolve into secondary acute myeloid leukemia (AML). CD34 is commonly expressed in all types of childhood leukemias, whereas CD117 is a reliable and specific marker to detect leukemia cells committed to myeloid lineage. Co-expression of CD34/CD117 may strongly suggest the diagnosis of AML (Rytting ME. Pediatric myelodysplastic syndromes. Curr hematol Rep 2004;3(3):173-7. May; U?kan D, Hi?s?nmez G, Yetgin S, Gürgey A, Cetin M, Karaa?ao?lu E, et al. CD34/CD117 co-expression in childhood acute leukemia. Leukemia Res 2000;24:201-6.). We describe the case of a 22 month-old-girl with MDS and Down syndrome who was presented with severe anemia and thrombocytosis at diagnosis, transformed into AML-M7. In our patient, CD34 and CD117 markers were positive on the blast cells of the BM 6 months before the chemotherapy decision. As the disease progressed, CD34/117 co-existence was increased and MDS transformed into AML. As a result, an increase in CD34 and CD117 positivity of the BM blast cells may be associated with a higher risk of leukemic transformation.