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Öğe Ceftriaxone-Associated Biliary Sludge and Pseudolithiasis in Children(Lippincott Williams & Wilkins, 2007) Onlen, Yusuf; Gali, Edip; Incecik, Faruk; Deviren, Mehmet; Savas, LutfuDetection of incidence and the risk factors of ceftriaxone (CTX)-associated pseudolithiasis (PL) in children. One hundred fourteen patients (75 boys and 39 girls) who used CTX for the treatment of various infections were admitted to the study. Pseudolithiasis was diagnosed by ultrasonography (USG). Ultrasonography was performed at the beginning, on the 5th and 10th days, and at the end of the treatment. Weekly USG was performed to patients who had PL until the findings improved. It was investigated whether the age, sex, weight, treatment duration, dosage, and the way of administration have effects on PL development. Ceftriaxone was administered intravenously. Fifty-seven patients received oncedaily and 57 patients received twice-daily dosage. Pseudolithiasis was observed in 37 (32.5%) of 114 patients. Age, sex, weight, treatment duration, and dosage had no role in PL development, whereas once-or twice-daily administration was found effective. Once-daily dosage of CTX treatment is recommended because of its lower incidence of PL development. Besides that USG should be performed regarding the risk of PL in children who are treated with CTX, further studies are necessary to determine the risk factors.Öğe Co-existince of sickle cell disease and hemidiaphragm paralysis(2006) Babayiğit, Cenk; Melek, İsmet Murat; Duman, Taşkın; Şenyiğit, Abdurrahman; Gali, EdipOrak hücreli anemi, hücrenin rijid deformasyonuna yol açan anormal hemoglobin üretiminin neden olduğu bir hastalıktır. İnfeksiyonlar, akut splenik sekestrasyon krizleri, aplastik krizler, akut göğüs sendromu, inme, kolelitiyazis, renal hastalıklar ve ağrı hastalığın majör komplikasyonlarıdır. Unilateral veya bilateral diyafragma paralizisi, frenik sinir zedelenmesini takiben veya miyopatiler, nöropatiler ve miyelopatiler gibi çeşitli motornöron hastalıklarıyla birlikte görülebilir. Hemidiyafragma paralizisi, bilateral paraliziden daha sık görülür ve genellikle göğüs radyografisinde diyafragmanın tek taraflı anormal yüksekliği ile teşhis edilir. Orak hücre hastalığı olan 14 yaşındaki kız hastanın rutin kontrolü sırasında çekilen göğüs radyografisinde belirgin sağ hemidiyafragma yüksekliği izlendi. Hastanın ne travma ne de torasik cerrahi öyküsü vardı. Nörolojik muayenesinde duyu kusuru veya motor defisiti de yoktu. Toraksın bilgisayarlı tomografisi (BT)’nde belirgin derecede sağ hemidiyafragma elevasyonu saptandı. BT’sinde hiçbir patolojik bulgu yoktu. Spirometrik değerleri (beklenenin yüzdesi şeklinde) FEV1= %53, FVC= %55, FEV1/FVC= %97, PEF= %43 ve FEF%25-75= %58 olarak orta-ağır derecede restriksiyon ile uyumluydu. Sağ hemidiyafragma paralizisi tanısı floroskopide Hitzenber Snif testin pozitif saptanmasıyla doğrulandı. Orak hücre hastalığında her ne kadar çeşitli patofizyolojik mekanizmaların santral nörolojik komplikasyonlara yol açtığı biliniyor olsa da, periferik sinir tutulumu bugüne kadar bildirilmemiştir. Burada, orak hücre hastalığı ve unilateral hemidiyafragma paralizisi olan 14 yaşındaki kız hastayı, aradaki ilişkinin kanıtlanması için daha çok sayıda olgu saptanması gerekmesine rağmen bugüne kadar bu birliktelik bildirilmediği için sunuyoruz.Öğe Decreased heart rate variability in scikle cell disease: Effect of pulmonary hypertension(2011) Akgül, Ferit; Batyraliev, Talantbek; Seyfeli, Ergün; Seydaliyeva, Tunzale; Gali, Edip; Yalçın, FatihKalp hızı değişkenliğinin (KHD) kompüturize analizi, kardiyak anatomik fonksiyonu tayin etmede non-invaziv bir yöntemdir. Azalmış KHD çeşitli hastalıklarda ve normal populasyonda, artmış mortalite hızı ile birliktedir. Bu çalışmanın amacı, orak hücreli hastalığa (OHH) sahip olan hastalarda KHD yi ve pulmoner hipertansiyonun KHD üzerine etkisini araştırmaktır. Biz doppler ekokardiyografiyi kullanarak OHH ya sahip 61 hastada (yaş ortalaması, 18.3±8.0 yıl) ile sağlıklı 22 bireyde (yaş ortalaması, 19.3+7.1 yıl) pulmoner arter sistolik basıncını ölçtük. Düşük frekanslı power (DFP) ile yüksek frekanslı power (YFP) orak hücreli hastalıklı hastalarda kontrol grubuna göre düşüktü. Buna karşın düşük frekanslı power ile, yüksek frekanslı po-werin oranı (DFP/YFP) orak hücreli hastalığı bulu-nanlarda artmış idi (p<0.0001). Orak hücreli hastalıklı hastalar arasında pulmoner hipertansiy-onlu hastalar, pulmoner hipertansiyonu bulunmayan hastalardan daha düşük YFP çok daha yüksek DFP/YFP oranına sahipti (her biri için p<0.001, p<0.05 ). Buna rağmen, Pulmoner hipertansiyonu ve pulmoner hipertansiyonsuz OHH lı hastalar arasında DFP yönünden fark yoktu. Kalp hastalığı preklinik devresinde olan, özellikle pulmoner hipertansi-yonu bulunan OHH hastalarda, KHD önemli ölçüde azalmıştır. Kalp hızı değişkenliği, pulmoner hipertansiyon-lu hastaların erken tanısı için bilhassa faydalı olabilir. Zira, bu hastanın kötü prognoz ve yüksek mortalite riski altında olduğuna işaret edebilir.Öğe Increased QT dispersion in sickle cell disease(Karger, 2007) Akgul, Ferit; Seyfeli, Ergun; Melek, Ismet; Duman, Taskin; Seydaliyeva, Tunzale; Gali, Edip; Yalcin, FatihBackground: QT dispersion has been proposed to be a predictor of adverse outcomes in a variety of cardiac disease states. The objective of this study was to examine QT dispersion in patients with sickle cell disease (SCD) and to assess the effect of pulmonary hypertension (PHT) on QT dispersion. Methods: We performed Doppler echocardiographic assessments of pulmonary artery systolic pressure in 73 (mean age 18.5 +/- 8.0 years) steady-state SCD patients and 25 (mean age 19.6 +/- 7.2 years) healthy subjects. Resting 12-lead electrocardiogram was recorded and QT dispersion was calculated as the difference between maximum and minimum QT intervals. Bazett's formula was used to obtain a rate-corrected value of the QT interval (QTc). Results: Maximum QTc, minimum QTc and QTc dispersion were significantly increased in SCD patients compared to the control subjects (p < 0.0001, p < 0.05, p < 0.0001, respectively). Among SCD patients, patients with PHT had higher maximum QTc and QTc dispersion than patients without PHT (p < 0.0001). However, minimum QTc showed no significant differences between the two patient groups. Conclusion: QTc dispersion is significantly increased in SCD patients, especially those with PHT indicating regional inhomogeneity of ventricular repolarization. Copyright (c) 2007 S. Karger AG, Basel.Öğe Neurological soft signs as the stroke risk in sickle cell disease(Tohoku Univ Medical Press, 2006) Melek, Ismet; Akgul, Ferit; Duman, Taskin; Yalcin, Fatih; Gali, EdipSickle cell disease (SCD) is a common form of hemoglobinopathy and is highly prevalent worldwide. Silent cerebral infarction, which represents infarction without clinical signs, is a risk factor for clinical stroke in patients with SCD. It is well known that silent infarction predisposes patients with SCD to overt stroke. The aim of the present study is to investigate the effect of silent infarction on neurological soft signs (NSS), which demonstrate subtle impairments in sensory integration, motor coordination and the sequencing of complex motor acts and to evaluate whether NSS can be used in clinical practice to evaluate the patients at risk of stroke in SCD patients with silent infarction. Fifty-nine SCD patients without any documented history of cerebrovascular accident and 28 healthy controls were included in this study. All the patients with SCD were evaluated with cerebral magnetic resonance imaging. We found that the NSS scores were significantly higher in patients with silent cerebral infarction than those in patients without silent infarction and control subjects (p < 0.05). Importantly, there was no significant difference in the NSS scores between the patients without silent infarction and control subjects. These results indicate that high NSS scores represnt an important finding for diagnosis of silent infarction in SCD patients. As silent infarction increases the risk for stroke in patients with SCD, NSS can be used to provide additional information in diagnosis of the patients with possible stroke risk during the course of SCD.Öğe Orak hücreli anemili çocuklarda inme(2004) Melek, İsmet Murat; Duman, Taşkın; Gali, EdipSerebrovasküler hastalıklar açısından özel risk grubu niteliğinde olan hasta grupları, etiyolojik değerlendirme ve klinik özellikleri açısından farklı öneme sahiptir. Orak hücreli anemi 20 yaş altı inmelerin etiyolojisinde önemli yer tutar. Çocukluk çağı serebrovasküler hastalık risk faktörleri için konjenital kalp hastalıklarından sonra en sık ikinci nedenin orak hücreli anemi olduğu ve bu hastaların benzer yaş gruplarına göre 250 kat daha fazla serebral infarkt riski taşıdığı bildirilmiştir. Türkiye'deki tüm hemoglobinopatilerin %17.49'unun Hatay doğumlu olduğu saptanmıştır. Bu çalışmada orak hücreli anemi taşıyıcılığının %10.5 olduğu Hatay ilinde 1994 yılından itibaren hemoglobin elektroforezi ile orak hücreli anemi tanısı almış ve bugüne kadar izlenmiş olan 506 hastada görülen inme olguları bildirildi. 403 olgu HbSS (%79.6), 99 olgu HbSS+ Thalessemi (%19.6), 2 olgu HbSD (%0.4), 2 olgu HbSE idi. 506 hastanın izlendiği 10 yıllık izlem süresinde, inme gelişen 5 hastanın en küçüğü 3, en büyüğü 15 yaşındaydı. Olguların hematolojik parametreleri ile inmelere ait klinik ve etiyolojik özellikler incelendi. Otozomal resesif bir hastalık olan ve kromozom 1 İp.15.4 bölgesinde bulunan, hemoglobin beta genindeki nokta mutasyon oluşan orak hücreli anemi hematolojik nitelikleri yanında inme açısından yüksek risk oluşturma özelliğine sahiptir. Ülkemizde gen taşıyıcılarının yoğun olarak bulunduğu bölgelerde erken tanı ve izlemle komplikasyonlarm önlenmesinde yapılacak çalışmalar çocukluk çağı inmeleri açısından da önem taşımaktadır.Öğe Prevalence of abdominal ultrasonographic abnormalities in patients with sickle cell disease(Turkish Soc Radiology, 2008) Balcı, Ali; Karazincir, Sinem; Sanguen, Oezlem; Gali, Edip; Daplan, Turgay; Cingiz, Cihangir; Egilmez, ErtugrulPURPOSE The aim of this study was to evaluate the type and prevalence of abdominal ultrasonographic abnormalities in patients with sickle cell disease. MATERIALS AND METHODS A total of 102 patients with sickle cell disease at the Center of Hemoglobinopathy of the Antakya State Hospital were screened for intra-abdominal abnormalities by abdominal ultrasonography (US). Eighty-four patients were homozygous or sickle-cell disease (S/S), and 18 patients were compound heterozygotes for sickle cell-beta thalassemia (S/beta(thal)). At the time of examination, 15.7% (16/102) of patients had undergone gone splenectomy, and 18.6% (19/102) of patients had undergone cholecystectomy. RESULTS The most frequent US findings (expressed as percentages of all patients) were hepatomegaly (71.6%), renal enlargement (30.4%), autosplenectomy (33.3%), cholelithiasis (30.4%) and splenomegaly (17.4%). A bright liver was identified in 6 patients (5.9%), an echogenic pancreas in 4 patients (3.9%), and pancreatic punctate echogenic foci were identified in 5 patients (4.9%). Medullary or diffusely increased renal echogenicity was observed in 16 patients (15.7%). Sonographic findings typical of renal papillary necrosis were observed in one patient with S/S. Periportal lymphadenopathy was detected in 10 (11.9%) of 84 patients of the S/S group, and 2 (11.1%) of 18 patients of S/beta(thal) group. CONCLUSION Abdominal ultrasonographic imaging of patients with sickle cell disease revealed a high prevalence of abdominal abnormalities, especially in solid organs.Öğe The prevalence of silent cerebral infarct in patients with sickle cell disease(Turkish Society of Cerebrovascular Diseases, 2008) Balcı, Ali; Okuyucu, Esra; Duman, Taşkin; Gali, Edip; Karazincir, Sinem; Melek, Ismet; Yanmaz, RasimObjective: To determine the prevalence of silent cerebral infarct and abnormal brain magnetic resonance imaging (MRI) findings in patients with sickle cell disease (SCD). Materials and methods: Fifty-two brain MR images of the asymptomatic patients with SCD have been reviewed retrospectively. The abnormal MRI findings were recorded. Results: Forty-four of the 52 patients with SCD were homozygous (S/S) and remaining 8 heterozygous (S/ßthal) for SCD. Twelve (27.2%) of the 44 patients with S/S and 2 (25%) of the 8 patients with S/ßthal had silent infarcts. Ten (22.7%; 7 diffuse cerebral, 2 focal cerebral and 1 cerebellum) of the 44 S/S patients demonstrated atrophy. None of the 8 patients with S/ßthal had cerebral atrophy. Conclusion: Patients with SCD living in Hatay demonstrated a high rate of silent cerebral infarct and cerebral atrophy. These findings concur with the results of the literature.Öğe Pulmonary hypertension in sickle-cell disease(Karger, 2007) Akgul, Ferit; Yalcin, Fatih; Seyfeli, Ergun; Ucar, Edip; Karazincir, Sinem; Balcı, Ali; Gali, EdipBackground: Our aim is to determine comorbidities associated with pulmonary hypertension (PHT) in clinically stable sickle-cell disease (SCD) patients and to evaluate left ventricular (LV) and right ventricular (RV) function in those patients. Methods: Echocardiography was performed in 87 SCD patients that were divided into group I (without PHT) and group II (with PHT). Both groups were compared with healthy controls. Results: A history of retinopathy and leg ulcer was more frequent in group II than group I (p < 0.01). Haemoglobin levels were lower (p < 0.05), whereas blood urea nitrogen, lactate dehydrogenase and total bilirubin levels were higher in group II (p < 0.01). Although group II patients had larger LV end-diastolic, LV end-systolic and RV diastolic diameters compared with group I patients and controls (p < 0.05), LV ejection fraction was similar in the three groups. The mitral peak early diastolic inflow velocity to peak late diastolic inflow velocity (E/A) ratio was similar in group I, group II and the control group. The tricuspid E/A ratio was lower in group II than group I and controls (p < 0.05). Conclusion: End organ damage occurs more often and haemolysis is severer in SCD patients with PHT than SCD patients without PHT. Although LV systolic and diastolic function is well preserved, RV diastolic function is disturbed in those patients with PHT. Copyright (c) 2007 S. Karger AG, Basel.Öğe Quantitative Brain Diffusion-Tensor MRI Findings in Patients With Sickle Cell Disease(Amer Roentgen Ray Soc, 2012) Balcı, Ali; Karazincir, Sinem; Beyoglu, Yeliz; Cingiz, Cihangir; Davran, Ramazan; Gali, Edip; Okuyucu, EsraOBJECTIVE. The aim of this study was to evaluate the microstructure of various regions of the brain using diffusion-tensor imaging (DTI) in patients with sickle cell disease (SCD) and in age- and sex-matched healthy control subjects. We also investigated the fiber tractography findings of the corpus callosum (CC) and corticospinal tracts (CSTs). SUBJECTS AND METHODS. Sixteen right-handed patients with SCD and 14 age- and sex-matched right-handed healthy control subjects were scanned using conventional MR sequences and DTI. Fractional anisotropy (FA) and apparent diffusion coefficients (ADCs) were calculated and regions of interest were selected in various brain areas (superior and inferior frontal, parietal, occipital, and temporal white matter areas), anterior and posterior periventricular areas, centrum semiovale, basal ganglia (lentiform nucleus, head of caudate nucleus), thalamus, cerebral peduncles, pons, cerebellar white matter areas, and CC. Diffusion-tensor tractography of the CC and CSTs was also performed. RESULTS. For the patients with SCD, significantly reduced FA values, increased ADC values, or both were seen clustered in several brain areas, including the CC, frontal white matter, centrum semiovale, periventricular areas, head of the caudate nucleus, thalamus, brainstem, and pons (p < 0.05). Statistically significant reductions in fiber counts in the first and fifth segments of the CC and in CSTs bilaterally were also observed in patients with SCD (p < 0.05). CONCLUSION. DTI shows microstructural abnormalities of various brain areas in patients with SCD.Öğe Right ventricular and pulmonary function in sickle cell disease patients with pulmonary hypertension(Springer, 2006) Akgul, Ferit; Yalcin, Fatih; Babayigit, Cenk; Seyfeli, Ergun; Seydaliyeva, Tunzale; Gali, EdipThe effects of sickle cell disease (SCD) on right ventricular (RV) and pulmonary function in SCD patients with pulmonary hypertension is not well-known. The aim of this study was to investigate RV and pulmonary functions in patients suffering from SCD with or without pulmonary hypertension using color tissue Doppler imaging and spirometry. We evaluated 48 asymptomatic patients with SCD. All patients underwent echocardiography with tissue Doppler imaging and pulmonary function test. Patients were divided into two groups: Group 1 consisted of 27 patients (age, 18.1 +/- 7.1 years) with normal pulmonary artery pressure, and group 2 consisted of 21 patients (age, 21.4 +/- 7.4 years) with pulmonary hypertension. Both groups were compared with a sex- and age-matched control group including 24 normal healthy subjects (age, 19.8 +/- 9.2 years). Tricuspid lateral annular systolic (S-m) and early diastolic velocity (E-m) were higher in group 1 than group 2 and the control group (p < 0.05). Tricuspid lateral annular late diastolic velocities (A(m)), isovolumetric contraction time, and myocardial performance index (MPI) were higher and the E-m/A(m) ratio was lower in group 2 than group 1 and the control group (p < 0.05). However, no differences were found in the tricuspid lateral annular E-m deceleration time, ejection time, and isovolumetric relaxation time between group 1, group 2, and the control group. Tricuspid lateral annular S-m and E-m were similar in group 2 and the control group. Forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and the diffusion capacity of the lung for carbon monoxide were decreased in both groups of patients compared to the control group (p < 0.05). However, there was no difference in respiratory rate, FEV1/FVC ratio, peak expiratory flow, and total lung capacity between group 1, group 2, and the control group. There were no differences in any indices of lung function between the two groups of patients. MPI is useful index to evaluate RV function in patients with SCD. RV diastolic function was disturbed in only SCD patients with pulmonary hypertension. On the other hand, the restrictive pattern of pulmonary function abnormalities had developed in both groups of patients.Öğe Seroprevalence of hepatitis B and hepatitis C in patients with thalassemia and sickle cell anemia in a long-term follow-up(Elsevier Science Inc, 2006) Ocak, Sabahattin; Kaya, Hasan; Cetin, Meryem; Gali, Edip; Ozturk, MugeBackground. Transfusion-dependent patients are more prone to acquiring various transfusion-transmitted infections such as hepatitis B (HBV), hepatitis C (HCV) and human immunodeficiency virus (HIV). The aim of the study was to investigate the prevalence of these infections in patients with thalassemia and with sickle cell anemia (SCA) receiving multiple blood transfusions. Methods. The subjects of the present study were 399 multi-transfused patients with beta-thalassemia major or intermedia and SCA who have been registered at the two regional hemoglobinopathy centers in Turkey since 1996. Hepatitis B surface antigen (HBsAg), hepatitis C virus antibodies (anti-HCV) and human immunodeficiency virus antibodies (anti-HIV) tests were assayed by a second-generation enzyme-linked immunosorbent assay method. Results. Of the 399 patients, 3 were HBsAg positive (0.75%), 18 were anti-HCV positive (4.5%), and none was anti-HIV positive. All patients with HBsAg and 14 (77.7%) patients with HCV received initial blood transfusions before second-generation tests were performed. Patients who were anti-HCV positive had a significantly higher mean number of blood transfusions and peak serum alanine transaminase level than anti-HCV-negative patients. Conclusions. These results showed that after introduction of more sensitive screening tests and stringent donor selection procedures, incidence of HCV infection was significantly reduced, but there was still a serious risk for HCV infection, and there was a minor risk for HBV infection in patients with thalassemia and SCA. (C) 2006 IMSS. Published by Elsevier Inc.Öğe Stroke in children with sickle cell anemia(Turkish Society of Cerebrovascular Diseases, 2004) Melek, Ismet M.; Duman, Taşkin; Gali, EdipPatient groups at high risk with respect to cerebrovascular disease has a particular importance in terms of etiological and clinic evaluations. Sickle cell anemia has an important place in the etiology of patients who have had stroke before the age of 20. Among the risk factor of cerebrovascular diseases occurring during childhood, sickle cell anemia is the second important cause following the congenital heart diseases. The probability of a patient with sickle cell anemia to have cerebral infarction is 250 times higher when compared to those who don't have this disease at same age. Those who are born in Hatay forms the 17.49% of the all hemoglobinopathy cases reported in Turkey. In this study, the data on stroke obtained from 506 cases by hemoglobin electrophoresis since 1994 in Hatay, where 10.5% of the population is the carrier, was reported. Of the cases, 403 (79,6%), 99 (19,6%), 2 (0,4%) and 2 (0,4%) were HbSS, HbSS+Thalessemia, HbSD and HbSE, respectively. During a period of 10 year, 5 patients had stroke. Among them, the youngest one was 3 yr old while the oldest one was 15 yr old. The data on hemopatolgical parameters and on clinical and etiological were investigated. Being an autosomal recessive disease and arising from the mutation of hemoglobin beta gene, sickle cell anemia is considered to be a high risk factor for stroke besides the hematological features. In the regions where gene carriers are densely populated, studies on early diagnosis and monitoring appears to be highly important in the detection and prevention of childhood strokes.Öğe Thyroid Doppler indices in patients with sickle cell disease(Elsevier Science Inc, 2013) Karazincir, Sinem; Balcı, Ali; Yonden, Zafer; Gali, Edip; Daplan, Turgay; Beyoglu, Yeliz; Kaya, HasanObjective: To evaluate the intrathyroidal hemodynamic changes and thyroidal volume in sickle cell disease (SCD) patients. Methods: Thirty-two patients with homozygous SCD and 32 control subjects were examined with color Doppler ultrasonography. None of the patients and control subjects had clinical or laboratory evidence of thyroid disease. Results: SCD patients had significantly higher resistance index (RI) and pulsatility index (PI) values and lower thyroid volume compared with control group. Conclusion: Increased intrathyroidal RI and PI and decreased thyroid volume may be due to impaired thyroidal microcirculation. Further and follow-up studies are needed to explain the relationship between Doppler parameters and thyroid functions. (C) 2013 Elsevier Inc. All rights reserved.
