Does altitude affect blood gases in hemodialysis patients?

INTRODUCTION: This study aimed to determine whether predialysis blood gases is affected by altitude differences in hemodialysis patients with arteriovenous fistulas living in Turkey at three different altitudes. METHODS: Patients' predialysis blood gases were compared by standardizing both arterial blood gases collections and working methods for patients undergoing hemodialysis using a dialysate with the same properties at altitudes of 30 m (sea level), 1020 m (moderate altitude), and 1951 m (high altitude). FINDINGS: Blood gases disorders were detected in 32 (82.1%) high altitude group patients, whereas 49 (74.2%) sea level group patients had no blood gases disorders (P < 0.001). pH values in the high altitude group were significantly lower than those in the other groups, and the pH increased as altitude decreased (P < 0.001). The partial pressure of carbon dioxide (PaCO2 ) values was higher in the sea level group than in the other groups and increased at lower sea levels (P < 0.001). Bicarbonate values were significantly higher in the sea level group than in the other groups and increased at lower sea levels, similar to PaCO2 values (P < 0.001). The partial pressure of oxygen (PaO2 ) values in the high altitude and sea level groups were significantly higher and increased at lower sea levels (P < 0.001). The oxygen saturation (SaO2 ) values were significantly lower in the high altitude group than in the other groups and increased gradually at lower sea levels (P < 0.001). DISCUSSION: Predialysis metabolic acidosis was more pronounced in patients undergoing hemodialysis at high altitudes, whereas PaCO2 , PaO2 , and SaO2 values were lower.

Irritable bowel syndrome frequency and related factors in hemodialysis patients.

INTRODUCTION: Irritable bowel syndrome (IBS) is a functional bowel disease that is common in society, does not threaten life, impairs quality of life, and causes serious economic losses. Gastrointestinal system complaints and especially IBS are common in patients with chronic kidney disease. It has also been shown that psychiatric diseases are more common in patients with IBS. In this study, we aimed to determine the frequency of IBS in hemodialysis patients and to investigate the factors associated with IBS. METHODS: In this cross-sectional study, the questionnaire prepared to evaluate depression, anxiety, and abdominal pain was administered face-to-face to 686 patients by the same researcher in seven dialysis centers; 404 patients without exclusion criteria were included in the study. The diagnosis of IBS was made according to Rome IV criteria. A multivariate logistic regression model was used to identify factors that are significantly related to IBS. FINDINGS: In 69 (17.1%) of the patients included in the study, symptoms were consistent with IBS. Binominal logistic regression analysis was performed to evaluate the effect of age, dialysis duration, diabetes, proton pump inhibitor, non-steroidal anti-inflammatory drugs, calcium acetate use, Hamilton depression and anxiety scores associated with IBS in the presence of IBS of the participants. The logistic regression model was statistically significant, χ2 (3) = 69.748, P < 0.001. Independent risk factors for IBS in hemodialysis patients were determined as anxiety, long-term dialysis treatment, and using calcium acetate as a phosphorus binder. DISCUSSION: In hemodialysis patients, IBS occurs approximately twice as often as in a healthy population. Independent risk factors for IBS in hemodialysis patients are anxiety, long-term dialysis treatment, and using calcium acetate as a phosphorus binder.

Investigation of the Levels of Serum Amyloid A, YKL-40, and Pentraxin-3 in Patients with Familial Mediterranean Fever.

BACKGROUND: Familial Mediterranean Fever (FMF) is an autosomal recessive form of recurrent episodes of fever and an autoinflammatory disease characterized by inflammation of the serous membranes. The clinical diagnosis is supported by the laboratory findings. This study investigated the relationship of Serum Amyloid A (SAA), YKL-40, and Pentraxin-3 (PTX-3) with the FMF disease. METHODS: About 50 patients with FMF were enrolled in this study. Patients were divided into three groups according to disease severity score (mild, moderate, and severe). Thirty-seven healthy individuals were included as the control group. Serum SAA, YKL-40, and PTX-3 concentrations were measured using an ELISA kit. RESULTS: Serum SAA and YKL-40 levels of FMF patients were significantly higher than in the control (P < 0.001). PTX-3 levels were found to be higher in patients even though there was no significant difference (P = 0.113). Whereas the positive predictive value was 71.9% for cut-off point of SAA, the positive predictive value was 83.3% for cut-off point of YKL-40. Whereas a significant correlation was detected in SAA and PTX-3 with YKL-40 (respectively; P = 0.036, P < 0.001), there was no correlation between the PTX-3 with SAA (P = 0.219). CONCLUSIONS: YKL-40 can be used together with SAA to support the diagnosis of FMF and to monitor the severity of the disease. In this study, YKL-40 levels were examined for the first time in FMF patients and further studies are necessary using larger patient samples.

Genetic associations of the response to inhaled corticosteroids in children during an asthma exacerbation.

BACKGROUND: Genetic associations of the response to inhaled corticosteroids (ICSs) during an asthma exacerbation are unknown. OBJECTIVE: To evaluate the role of genetic variants in the therapeutic response to high-dose ICS in children with moderate-to-severe asthma exacerbations. METHODS: Eighty-two children (56 boys/26 girls, mean age 9.6 ± 3.2 years) with moderate-severe asthma exacerbation were genotyped for eight single-nucleotide polymorphisms that were a priori associated with ICS response in chronic asthma treatment: glucocorticosteroid receptor (NR3C1) rs41423247; corticotrophin-releasing hormone receptor1 (CRHR1) rs242939, rs242941, and rs1876828; T-box 21 (TBX21) rs2240017; glucocorticoid-induced transcript 1 (GLCCl1); and T gene rs3099266 and rs2305089. Children were treated with a single high-dose (4000 µg) fluticasone propionate given by a nebulizer followed by 1000 µg/day of inhaled fluticasone propionate for 6 days. Primary outcome measure was the improvement in FEV1 at 4 h. RESULTS: Mean FEV1 was 71.7 ± 14.2% at presentation. Overall, fluticasone treatment resulted in a significant improvement in asthma score and FEV1 (p < 0.0001 for both). Children with the GG genotype at NR3C1 rs41423247 (n = 26) had a higher improvement in FEV1 [24.2% (interquartile range 11.5-36.3)] compared to those with CG+CC (n = 19), [7.9% (interquartile range 6.1-24.6) (p = 0.006)]. CONCLUSION: Homozygosity for the G allele at rs41423247 of the glucocorticosteroid receptor (NR3C1) gene is associated with a higher improvement in FEV1 at 4 h in children with moderate-to-severe asthma exacerbation treated with high-dose ICS. This observation may have important clinical implications especially for children who use systemic steroids frequently for recurrent asthma exacerbations.

The Role of Apelin 13 in Progression of Chronic Kidney Disease.

INTRODUCTION: Apelin is an adipokine secreted by the adipose tissue and by the endothelial cells in various parts of the body. Apelin is also expressed by the glomerular arteriolar rectus and glomerular capillary cells. We evaluated the relationship between the initial serum levels of apelin 13 with the trend of glomerular filtration rate (GFR) during a 1-year follow-up of patients with chronic kidney disease (CKD). MATERIALS AND METHODS: Ninety-nine patients with CKD in the predialysis stages were included and completed the study. The demographic data, medications, and comorbidities of the patients were recorded. The relationship between the baseline apelin 13 levels and the 1-year GFR loss was evaluated.   Results. The mean 1-year GFR loss 1.6 mL/min for those with CKD stage 3, 5.1 mL/min for those with CKD stage 4, and 2.6 mL/min for those with CKD stage 5. Fifty-eight patients (58.6%) had a GFR loss less than 5 mL/min and 41 (41.4%) had a GFR loss of 5 mL/min and greater, for whom the mean apelin 13 levels were 2169 ± 1807 mL/min and 2513 ± 1920 mL/min, respectively (P = .36).  There was no significant correlation between the apelin 13 levels and GFR loss (P = .35). CONCLUSIONS: To our knowledge, this study was the first that clinically examined the relationship between apelin 13 and CKD progression. Apart from the diabetic nephropathy, several factors causing comorbidity and progression may have probably masked this potential relationship.

An Appeal to the Global Health Community for a Tripartite Innovation: An "Essential Diagnostics List," "Health in All Policies," and "See-Through 21(st) Century Science and Ethics".

Diagnostics spanning a wide range of new biotechnologies, including proteomics, metabolomics, and nanotechnology, are emerging as companion tests to innovative medicines. In this Opinion, we present the rationale for promulgating an "Essential Diagnostics List." Additionally, we explain the ways in which adopting a vision for "Health in All Policies" could link essential diagnostics with robust and timely societal outcomes such as sustainable development, human rights, gender parity, and alleviation of poverty. We do so in three ways. First, we propose the need for a new, "see through" taxonomy for knowledge-based innovation as we transition from the material industries (e.g., textiles, plastic, cement, glass) dominant in the 20(th) century to the anticipated knowledge industry of the 21st century. If knowledge is the currency of the present century, then it is sensible to adopt an approach that thoroughly examines scientific knowledge, starting with the production aims, methods, quality, distribution, access, and the ends it purports to serve. Second, we explain that this knowledge trajectory focus on innovation is crucial and applicable across all sectors, including public, private, or public-private partnerships, as it underscores the fact that scientific knowledge is a co-product of technology, human values, and social systems. By making the value systems embedded in scientific design and knowledge co-production transparent, we all stand to benefit from sustainable and transparent science. Third, we appeal to the global health community to consider the necessary qualities of good governance for 21st century organizations that will embark on developing essential diagnostics. These have importance not only for science and knowledge-based innovation, but also for the ways in which we can build open, healthy, and peaceful civil societies today and for future generations.

Meningitis caused by Neisseria Meningitidis, Hemophilus Influenzae Type B and Streptococcus Pneumoniae during 2005-2012 in Turkey. A multicenter prospective surveillance study.

Successful vaccination policies for protection from bacterial meningitis are dependent on determination of the etiology of bacterial meningitis. Cerebrospinal fluid (CSF) samples were obtained prospectively from children from 1 month to ≤18 years of age hospitalized with suspected meningitis, in order to determine the etiology of meningitis in Turkey. DNA evidence of Neisseria meningitidis (N. meningitidis), Streptococcus pneumoniae (S. pneumoniae), and Hemophilus influenzae type b (Hib) was detected using multiplex polymerase chain reaction (PCR). In total, 1452 CSF samples were evaluated and bacterial etiology was determined in 645 (44.4%) cases between 2005 and 2012; N. meningitidis was detected in 333 (51.6%), S. pneumoniae in 195 (30.2%), and Hib in 117 (18.1%) of the PCR positive samples. Of the 333 N. meningitidis positive samples 127 (38.1%) were identified as serogroup W-135, 87 (26.1%) serogroup B, 28 (8.4%) serogroup A and 3 (0.9%) serogroup Y; 88 (26.4%) were non-groupable. As vaccines against the most frequent bacterial isolates in this study are available and licensed, these results highlight the need for broad based protection against meningococcal disease in Turkey.

Translating biotechnology to knowledge-based innovation, peace, and development? Deploy a Science Peace Corps--an open letter to world leaders.

Scholarship knows no geographical boundaries. This science diplomacy and biotechnology journalism article introduces an original concept and policy petition to innovate the global translational science, a Science Peace Corps. Service at the new Corps could entail volunteer work for a minimum of 6 weeks, and up to a maximum of 2 years, for translational research in any region of the world to build capacity manifestly for development and peace, instead of the narrow bench-to-bedside model of life science translation. Topics for translational research are envisioned to include all fields of life sciences and medicine, as long as they are linked to potential or concrete endpoints in development, foreign policy, conflict management, post-crisis capacity building, and/or peace scholarship domains. As a new instrument in the global science and technology governance toolbox, a Science Peace Corps could work effectively, for example, towards elucidating the emerging concept of "one health"--encompassing human, environmental, plant, microbial, ecosystem, and planet health--thus serving as an innovative crosscutting pillar of 21(st) century integrative biology. An interdisciplinary program of this caliber for development would link 21(st) century life sciences to foreign policy and peace, in ways that can benefit many nations despite their ideological differences. We note that a Science Peace Corps is timely. The Intergovernmental Panel on Climate Change (IPCC) of the United Nations released the Fifth Assessment Report on March 31, 2014. Worrisomely, the report underscores that no person or nation will remain untouched by the climate change, highlighting the shared pressing life sciences challenges for global society. To this end, we recall that President John F. Kennedy advocated for volunteer work that has enduring, transgenerational, and global impacts. This culminated in establishment of the Peace Corps in 1961. Earlier, President Abraham Lincoln aptly observed, "nearly all men can stand adversity, but if you want to test a man's character, give him power." We therefore petition President Barack Obama, other world leaders, and international development agencies in positions of power around the globe, to consider deploying a Science Peace Corps to cultivate the essential (and presently missing) ties among life sciences, foreign policy, development, and peace agendas. A Science Peace Corps requires support by a credible and independent intergovernmental organization or development agency for funding, and arbitration in the course of volunteer work when the global versus local (glocal) value-based priorities and human rights intersect in synergy or conflict. In all, Science Peace Corps is an invitation to a new pathway for competence in 21(st) century science that is locally productive and globally competitive. It can open up scientific institutions to broader considerations and broader inputs, and thus cultivate vital translational science in a world sorely in need of solidarity and sustainable responses to the challenges of 21(st) century science and society.

Ready to put metadata on the post-2015 development agenda? Linking data publications to responsible innovation and science diplomacy.

Metadata refer to descriptions about data or as some put it, "data about data." Metadata capture what happens on the backstage of science, on the trajectory from study conception, design, funding, implementation, and analysis to reporting. Definitions of metadata vary, but they can include the context information surrounding the practice of science, or data generated as one uses a technology, including transactional information about the user. As the pursuit of knowledge broadens in the 21(st) century from traditional "science of whats" (data) to include "science of hows" (metadata), we analyze the ways in which metadata serve as a catalyst for responsible and open innovation, and by extension, science diplomacy. In 2015, the United Nations Millennium Development Goals (MDGs) will formally come to an end. Therefore, we propose that metadata, as an ingredient of responsible innovation, can help achieve the Sustainable Development Goals (SDGs) on the post-2015 agenda. Such responsible innovation, as a collective learning process, has become a key component, for example, of the European Union's 80 billion Euro Horizon 2020 R&D Program from 2014-2020. Looking ahead, OMICS: A Journal of Integrative Biology, is launching an initiative for a multi-omics metadata checklist that is flexible yet comprehensive, and will enable more complete utilization of single and multi-omics data sets through data harmonization and greater visibility and accessibility. The generation of metadata that shed light on how omics research is carried out, by whom and under what circumstances, will create an "intervention space" for integration of science with its socio-technical context. This will go a long way to addressing responsible innovation for a fairer and more transparent society. If we believe in science, then such reflexive qualities and commitments attained by availability of omics metadata are preconditions for a robust and socially attuned science, which can then remain broadly respected, independent, and responsibly innovative. "In Sierra Leone, we have not too much electricity. The lights will come on once in a week, and the rest of the month, dark[ness]. So I made my own battery to power light in people's houses." Kelvin Doe (Global Minimum, 2012) MIT Visiting Young Innovator Cambridge, USA, and Sierra Leone "An important function of the (Global) R&D Observatory will be to provide support and training to build capacity in the collection and analysis of R&D flows, and how to link them to the product pipeline." World Health Organization (2013) Draft Working Paper on a Global Health R&D Observatory.

Prevalence and risk factors of valvular calcification in hemodialysis patients.

INTRODUCTION: Valvular abnormalities frequently occur in patients with chronic kidney failure. This study evaluated the prevalence of heart valve calcification (HVC) in hemodialysis patients and factors associated with it. MATERIALS AND METHODS: Medical charts of 129 hemodialysis patients were reviewed retrospectively. Demographic features and laboratory analysis of the patients were systematically recorded. Echocardiographic findings were collected, including ejection fraction, aortic valve calcification (AVC), mitral valve calcification (MVC), left ventricle mass, left ventricle mass index, and pulmonary artery pressure. RESULTS: Valvular abnormalities were found in 43 patients (33.3%); 30 patients (23.3%) had MVC, 28 (21.7%) had AVC, and 15 (11.6%) had both MVC and AVC. Patients with HVC were older than other patients (P < .001). On echocardiography, higher left ventricle mass, left ventricle mass index, and pulmonary artery pressure levels were found in patients with HVC. Regarding the lipid profile, serum calcium, serum phosphorus, calcium-phosphorus product, and parathyroid hormone concentrations, there were no significant differences between patients with and without HVC. Ejection fraction levels were significantly lower in patients with HVC (P = .002) and serum albumin level of patients with HVC was significantly diminished. CONCLUSIONS: This study failed to show an association between HVC in hemodialysis patients and calcium-phosphorus product and parathyroid hormone levels; however, age and diabetes mellitus could be regarded as risk factors. In addition, HVC may lead to increased left ventricle mass index and pulmonary artery pressure and decreased ejection fraction, and low albumin levels may be attributable to inflammation.

Trends in the frequency of HLA DR-DQ haplotypes among children and adolescents with type 1 diabetes mellitus in the Southeast Region of Turkey.

OBJECTIVE: The aim of this study was to determine the frequency of HLA DR-DQ haplotypes in children with type 1 diabetes mellitus (T1DM) in the Southeast Region of Turkey. METHODS: Eighty children and adolescents with T1DM and eighty control subjects participated in the study. HLA-DR, DQ was typed using polymerase chain reaction and sequence-specific priming technique. RESULTS: HLA DRB1*03 allele was significantly more common in patients than in control subjects. HLA DRB1*11, HLA DRB1*13 and HLA DRB1*14 allele frequencies were significantly lower in patients than in controls. DQB1*02 allele was more common in patients, whereas DQB1*03 allele was more frequent in control subjects. HLA DRB1*03-DQB1*02 haplotype was more frequently observed among patients. CONCLUSION: These results confirm the similar potential trends in the frequency distribution of HLA susceptibility genes with T1DM previously observed in Turkey and in other Caucasian populations.

Exhaled RANTES and interleukin 4 levels after exercise challenge in children with asthma.

BACKGROUND: Despite the universality and clinical significance of exercise-induced bronchospasm (EIB), the mechanisms responsible for it are incompletely understood. OBJECTIVE: To investigate the role of exhaled RANTES (regulated on activation, normal T-cell expressed and secreted) and interleukin (IL) 4 in EIB in children with asthma. METHODS: Fifty-six children with asthma were evaluated with exercise challenge and exhaled RANTES and IL-4 levels. Exhaled breath condensate was collected before and 30 minutes after exercise challenge. RANTES and IL-4 concentrations were determined using a specific immunoassay kit. RESULTS: A significant increase was found in RANTES levels after exercise challenge in the asthmatic children (P<.001). A statistically significant increase in RANTES levels was noted after exercise challenge in both the asthmatic children with EIB (n=25, P=.007) and in the non-EIB asthmatic group (n=31, P=.005). Our study revealed that exhaled RANTES level correlates significantly well with percentage of forced expiratory volume in 1 second (FEV1), exacerbation frequency, serum IgE, and body mass index. No statistically significant increase was found in IL-4 levels after exercise challenge. The maximal postexercise decrease in FEV1 strongly correlated with total eosinophil count (P<.001, r = -0.61) and baseline ratio of FEV1 to forced vital capacity (FVC) (P=.002, r=0.40). Results from multivariate regression analysis adjusted for age, sex, and atopy as covariates showed that eosinophil count and FEV1/FVC ratio were significantly associated with EIB. CONCLUSION: We found that exercise challenge, leading to hyperosmolar stimulus, may increase exhaled RANTES levels in children with asthma. In addition, exhaled RANTES levels correlate well with serum IgE, severity of asthma, FEV1/FVC ratio, and body mass index. RANTES and IL-4 may not be independent predictors for EIB. Furthermore, eosinophil count and FEV1/FVC ratio may predict the presence and severity of EIB in asthmatic children.

Human kidney histopathology in acute obstructive jaundice: a prospective study.

INTRODUCTION: Cholemia and bacterial translocation with portal endotoxemia are integral in the pathogenesis of obstructive jaundice (OJ). There is sufficient experimental data about hemodynamic and histopathological consequences of OJ. In contrast, pathological information of renal changes in patients with OJ is still lacking. Therefore; the primary objective of this prospective study is to show the specific histopathological changes in kidneys of patients with short-term biliary tract obstruction receiving a standard perioperative medical treatment protocol. MATERIALS AND METHODS: Twenty consecutive patients with biliary obstruction were included in the study. Fluid replacement, prevention of biliary sepsis, and portal endotoxemia were mainstays of the perioperative treatment protocol. Fluid and electrolyte balance was maintained by twice daily body weight calculations, central venous pressure, and mean arterial pressure monitoring. Renal function was assessed by glomerular filtration rate estimation by modification of diet in renal disease-7 formula. Kidney biopsy evaluation was focused on tubular changes, thrombotic microangiopathy, endothelial damage, and peritubular capillary (PTC) dilatation with or without C4d staining. Fresh frozen sections were evaluated with immunofluorescence microscopy for glomerular IgG, IgA, IgM, C3, and C1q staining. RESULTS: The mean duration of OJ was 15.5 ± 1.4 days. Body weight increased before surgery through volume expansion (P = 0.001). All patients have shown mean arterial pressure ≥ 70 and ≤ 120 mmHg and renal function was very well preserved in all but one subject during the perioperative period. Despite those favorable figures, dilatation of peritubular venules and acute tubular necrosis were shown synchronously in all cases. C4d staining in PTC and arterioles and thrombotic microangiopathy were entirely absent in the study group. Immune complex deposits in PTCs and in glomeruli were not detected. Three patients had isolated glomerular C4d deposition without accompanying thrombotic microangiopathy and IgG, IgA, IgM, C3, and C1q staining of glomerular capillaries in I immunofluorescence microscopy. DISCUSSION: This study is the first in the literature to address the histopathological changes that occur in humans with short-term biliary obstruction. Acute tubular necrosis and venous dilatation was observed in all biopsies, without exception, despite the maintenance of strict volume control in all patients. The adequacy of volume control may not be implicated in those results; rather a possible mechanism related to untrapped endotoxin in the gut lumen or systemic circulation might lead to prolonged PTC dilatation and hypoperfusion with synchronous acute tubular necrosis. Absolute recovery of renal function in all patients and the demonstration of solitary acute tubular necrosis with no microvascular-glomerular-interstitial inflammation or injury, suggests that the perioperative treatment regime in this study is fairly efficacious in short-term OJ.

A prospective study of etiology of childhood acute bacterial meningitis, Turkey.

Determination of the etiology of bacterial meningitis and estimating cost of disease are important in guiding vaccination policies. To determine the incidence and etiology of meningitis in Turkey, cerebrospinal fluid (CSF) samples were obtained prospectively from children (1 month-17 years of age) with a clinical diagnosis of acute bacterial meningitis. Multiplex PCR was used to detect DNA evidence of Streptococcus pneumoniae, Haemophilus influenzae type b (Hib), and Neisseria meningitidis. In total, 408 CSF samples were collected, and bacterial etiology was determined in 243 cases; N. meningitidis was detected in 56.5%, S. pneumoniae in 22.5%, and Hib in 20.5% of the PCR-positive samples. Among N. meningitidis-positive CSF samples, 42.7%, 31.1%, 2.2%, and 0.7% belonged to serogroups W-135, B, Y, and A, respectively. This study highlights the emergence of serogroup W-135 disease in Turkey and concludes that vaccines to prevent meningococcal disease in this region must provide reliable protection against this serogroup.

Immunomodulation therapy in children with chronic hepatitis B.

PURPOSE: The aim of this study is to investigate the effects of HBsAg vaccine and levamisole on virological indicators in naive patients suffering from chronic hepatitis B (CHB) and in healthy carriers of hepatitis B. METHOD: Vaccination and treatment with levamisole were applied to 93 minor patients in total, 43 of them inactive CHB carriers and 50 patients suffering from CHB. RESULTS: 15 (30%) of 50 patients who had high ALT values in the beginning of the study had normal values after treatment. In nine (12%) patients, posttreatment ALT values were higher than pretreatment values, and six (10%) patients showed HBV-DNA loss. In spite of the presence of 50 (54%) HBeAg-positive patients before treatment, 17 (34%) patients proved to be HBeAg-negative after treatment. HBeAg sero-conversion was seen in 10 (20%) cases. In two (2%) patients, HBsAg sero-conversion occurred. CONCLUSION: It was found that treatment with levamisole and vaccine had positive effects on CHB patients and healthy carriers with respect to HBV DNA loss, HBeAg sero-conversion and ALT normalization. The viral load increases and ALT increases that occurred in certain cases were thought to be related to the early immune response. It was determined that combined levamisole and vaccine therapy had no additional positive effect.

Prevalence and hematological characteristics of beta-thalassemia trait in Gaziantep urban area, Turkey.

Thalassemia is one of the most common hereditary disorders in the Mediterranean region and studies have shown that the prevalence of beta-thalassemia trait is high in the southern part of Turkey. Gaziantep is a city located near this region and, therefore, the authors investigated the prevalence and hematological characteristics of the beta-thalassemia traits in primary school students in Gaziantep. Sixty primary schools were selected from a list of all primary schools using a systematic sampling method. Data were collected by a face-to-face questionnaire. Osmotic fragility testing (OFT) using single-tube 0.36% NaCl solution was used for the screening of beta-thalassemia. Students who were positive in regard to OFT went through a series of testing, including a complete blood count, serum ferritin levels, serum iron, and hemoglobin electroforesis. Chi-square test was used in statistical analysis. Of the 2439 students enrolled to the study from the selected 60 classrooms, 1353 (55.5%) were male and 1086 (44.5%) were female. The OFT was positive in 115 (4.7%) of the participants. CEA and confirmatory HPLC results of the students who were positive OFT indicated that 70 (60.8%) had normal results, 33(28.7%) showed high HbA2 levels, 7 (6.1%) showed high HbA2 and HbF levels, 5(5.2%) showed high HbA2 and Fe-deficiency anemia, and none showed increased HbF levels. The overall prevalence of beta-thalassemia trait was 1.84%. No gender differentials and highest rates among the Kahramanmaras (3.5%) and Sanliurfa (1.7%) born students were the other significant findings of this study. Implementation of a routine carrier-screening program offering genetic counseling, prenatal diagnosis, and selective termination of affected fetuses would be a wise approach to eliminate this disease from the region.

Alendronate treatment in osteogenesis imperfecta.

AIMS: Osteogenesis imperfecta (OI) is a chronic, disabling condition characterized by bone fragility resulting from defective production of type I collagen. Pamidronate therapy is the most extensively studied treatment and has proved beneficial. Our objective was to evaluate the effect of alendronate, a more potent bisphosphonate than pamidronate, in OI. MATERIALS AND METHODS: Three patients (age, 3-7 years; mean, 5 years) (one case, type III; 2 cases, type IV) have been given alendronate (0.3-0.56 mg/kg per day orally) for 2 years. Number of fractures, ambulation, height growth, and bone mineral density by dual-energy x-ray absorptiometry (DXA) were followed up. RESULTS: Bone mineral density improved significantly after the 2-year alendronate treatment, which increased by 47.8% to 106.6% in the lumbar spine and by 24% to 51.4% in forearm bones. The z-score of lumbar spine DXA values increased from -5.26 +/- 0.84 to -3.1 +/- 0.59. The mean of fracture rates did not change significantly. Only one of the patients was highly limited in ambulation. She had curved legs and could not sit without support before the treatment. She improved to walk with help by the treatment. Serum parathormone and alkaline phosphatase concentrations did not change significantly. No side effect was detected in clinical and laboratory evaluations. CONCLUSION: The study suggests that alendronate is a safe and well-tolerated drug and that it could increase bone density in children with OI, all of which encourage further studies with the bisphosphonates that are more potent than pamidronate and can be used orally. In addition, this study is the first report using the forearm bone mineral density measurement in OI.