Yazar "Pekcan, Sevgi" seçeneğine göre listele
Listeleniyor 1 - 20 / 25
Sayfa Başına Sonuç
Sıralama seçenekleri
Öğe 8-year old patient with giant hydatid cyst consulted with chest wall asymmetry(WILEY-BLACKWELL, 2015) Uygun, Saime Sundus; Aribas, Olgun Kadir; Pekcan, SevgiCystic echinococcosis, a zoonotic disease, is common in Turkey and is caused by the Echinococcus granulosus tapeworm. We describe the case of an 8-year-old girl who was admitted to hospital with chest wall asymmetry after a fall from height, without any other symptoms. Cystic lesion was identified on radiology, and serology supported the diagnosis of hydatid cyst. The patient underwent thorax surgery. Postoperatively, she was treated with benzimidazoles for 2 months. Rarely, hydatid cysts can reach a size sufficiently large to cause chest deformity.Öğe Acute Necrotizing Encephalopathy of Childhood Associated With A Novel Influenza Type A Virus: A Case Report(2014) Kartal, Ayşe; Şahin, Akkız; Pekcan, Sevgi; Aydın, KürşadAkut Nekrotizan Ensefalit, akut ateşli hastalıkların sıklıkla da viral enfeksiyonların tetiklediği nadir görülen bir ensefalopati formudur. Bu sendrom özellikle talamus, beyin sapı, beyincik ve beyaz maddeyi içeren multifokal simetrik beyin lezyonların varlığı ile karakterizedir. En yaygın anormallikler artmış serum aminotransferaz aktivitesi ve artmış beyin omurilik sıvısı proteini düzeyidir. Hastalığın etyolojisi ve patogenezisi tam olarak bilinememektedir ve spesifik bir tedavisi ve korunma yöntemi yoktur. Prognoz genellikle kötüdür ve hastaların %10'undan azı tamamen iyileşir. Tanı genellikle karakteristik nörögörüntüleme bulguları ile konulur. Biz İnfluenza A enfeksiyonun neden olduğu ve Akut Nekrotizan Ensefalitin tipik klinik ve radyolojik özelliklerini taşıyan 8 yaşındaki olguyu sunarak, influenza enfeksiyonlarının bu nadir ve ciddi formuna dikkat çekmek istedik.Öğe Alternaria and Cladosporium spores in the atmosphere of Konya and their relationship with meteorological factors(2014) Artaç, Hasibe; Kızılpınar, İlginç Temizer; Özdemir, Hülya; Pekcan, Sevgi; Doğan, Cahit; Reisli, İsmailGiriş: Bu çalışmanın amacı; Konya ili atmosferindeki Alternaria ve Cladosporium sporlarını etkileyen meteorolojik faktörleri (sıcaklık, nispi nem, rüzgar hızı, rüzgar yönü ve yağış miktarı) saptamaktır. Gereç ve Yöntem: Alternaria ve Cladosporium sporları 1 Ocak 2008-31 Aralık 2009 tarihleri arasında Burkard cihazıyla toplandı. Mikroskobik sayımlar atmos- ferik konsantrasyonlara çevrildi ve spor/m3 şeklinde ifade edildi. Bulgular: 2008-2009 yıllarında sırasıyla, 424 (%19.2) ve 3977 (%8.6) spor/m3 Alternaria sporu, 1784 (%80.8) ve 42.158 (%91.4) spor/m3 Cladosporium sporu tespit edildi. 2008 yılında Cladosporium konsantrasyonuyla günlük ortalama sıcaklık (r 0.181, p 0.045), maksimum sıcaklık (r 0.193, p 0.033) ve rüzgar hızı (r 0.242, p 0.007) arasında pozitif korelasyon; nispi nem (r -0.215, p 0.017) ile negatif korelasyon saptandı. 2009 yılında ise Alternaria ve Cladosporium spor konsantrasyonlarının, günlük ortalama sıcaklık (r 0.44, p 0.001; r 0.44, p 0.001), minimum sıcaklık (r 0.46, p 0.001; r 0.44, p 0.001) ve maksimum sıcaklık (r 0.40, p 0.001; r 0.43, p 0.001) ile pozitif ilişkili olduğu; nispi nem ile (r -0.29, p 0.001; r -0.37, p 0.001) negatif korelasyonu olduğu bulundu. Sonuç: Konya ili atmosferindeki Alternaria ve Cladosporium miktarı meteorolojik faktörlere göre değişiklik göstermektedir. Ayrıca, Cladosporiuma ait sporlar Alternaria sporlarından daha yoğun olarak tespit edilmiştir.Öğe B-Cell Maturation and Antibody Responses in Individuals Carrying a Mutated CD19 Allele(Nature Publishing Group, 2010) Artaç, Hasibe; Reisli, İsmail; Kara, R.; Pico-Knijnenburg, I.; Adın, Suzan Çınar; Pekcan, Sevgi; Jol-Van der Zijde, C. M.; Van Tol, M. J. D.; Bakker-Jonges, L. E.; Van Dongen, J. J. M.; Van der Burg, M.; Van Zelm, M. C.Homozygous CD19 mutations lead to an antibody deficiency due to disruption of the CD19 complex and consequent impaired signaling by the B-cell antigen receptor. We studied the effects of heterozygous CD19 mutations on peripheral B-cell development and antibody responses in a large family with multiple consanguineous marriages. Sequence analysis of 96 family members revealed 30 carriers of the CD19 mutation. Lymphocyte subset counts were not significantly different between carriers and noncarriers in three different age groups (0-10 years; 11-18 years; adults). B cells of carriers had reduced CD19 and CD21 median expression levels, and had reduced proportions of transitional (0-10 years) and CD5(+) B cells (adults). CD19 carriers did not show clinical signs of immunodeficiency; they were well capable to produce normal serum Ig levels and had normal responses to primary and booster vaccinations. The frequency of mutated V(kappa) alleles was not affected. Heterozygous loss of CD19 causes some changes in the naive B-cell compartment, but overall in vivo B-cell maturation or humoral immunity is not affected. Many antibody deficiencies are not monogenetic, but likely caused by a combination of multiple genetic variations. Therefore, functional analyses of immune cell function should be carried out to show whether heterozygous mutations contribute to disease.Öğe A case of congenital agenesis of the right pulmonary artery presenting with hemoptysis and mimicking pulmonary hemosiderosis(SPRINGER, 2009) Simsek, Pelin Ozlem; Ozcelik, Ugur; Celiker, Alpay; Yalcin, Ebru; Cobanoglu, Nazan; Pekcan, Sevgi; Alehan, DursunCongenital unilateral absence of a pulmonary artery is a rare anomaly most frequently accompanied by other cardiovascular anomalies. We report a 10-year-old girl presenting with fatigue and recurrent hemoptysis who was initially misdiagnosed with idiopathic pulmonary hemosiderosis. Her symptoms did not resolve despite treatment so she was referred to our center for further evaluation. We carried out an angiography which revealed the absence of the right pulmonary artery and multiple collaterals originating from the right subclavian and right internal mammary arteries supplying the right lung. During the follow-up the patient developed a severe episode of pulmonary infection and pulmonary hypertension which responded well to medical treatment. Physicians should be aware of the congenital absence of the right pulmonary artery especially in patients presenting with recurrent respiratory symptoms. Although this condition is generally considered to have a good prognosis, close observation is mandatory in order to prevent further complications and comorbidities.Öğe CD19 deficiency: A village screening study(AVES, 2009) Reisli, Ismail; Artac, Hasibe; Pekcan, Sevgi; Kara, Reyhan; Yumlu, Kadir; Karagol, Cuneyt; Cimen, OmerAim: We identified a new CD19 deficient case who was a relative of the first CD19 deficient patient and lived in the same village. These cases suggested that other patients with CD19 deficiency or carriers could be found in this village. The aim of this study was to assess the warning signs of primary immunodeficiency and the CD19 molecule deficiency in this population. Material and Method: The relatives of CD19 deficient patients and controls were examined for the warning signs of primary immunodeficiency using a questionnaire form Blood samples were taken from these subjects CD19 and CD21 expressions (median fluorescent intensity, MFI) were analyzed by flowcytometry in these samples. Results: A total of 208 subjects (59 families, 126 females, 82 males) including 129 children 9.5 +/- 3 7 years and 79 adults (37 13 years) were evaluated. A new case with CD19 deficiency was not detected There was at least one warning sign for immunodeficiency in 67 subjects (57 children and 10 adults) CD19 median expression value was lower than 60 MFI value in 20 subjects, which was known as in the carriers who were detected to have heterozygous mutation Conclusions: Our findings suggested that CD19 median expression might be a diagnostic tool for the subjects who have CD19 heterozygous mutations. Mutation analyses are planned for the confirmation of CD19 heterozygous mutation in these subjects who have low CD19 median expression. (Turk Arch Ped 2009; 44. 127-30)Öğe CD19 Molekül Eksikliği: Bir Köy Taraması(Kare Publishing, 2009) Reisli, İsmail; Artaç, Hasibe; Pekcan, Sevgi; Kara, Reyhan; Yümlü, Kadir; Karagöl, Cüneyt; Çimen, Ömer; Şen, Metin; Artaç, MehmetAim: We identified a new CD19 deficient case who was a relative of the first CD19 deficient patient and lived in the same village. These cases suggested that other patients with CD19 deficiency or carriers could be found in this village. The aim of this study was to assess the warning signs of primary immunodeficiency and the CD19 molecule deficiency in this population. Material and Method: The relatives of CD19 deficient patients and controls were examined for the warning signs of primary immunode- ficiency using a questionnaire form. Blood samples were taken from these subjects. CD19 and CD21 expressions (median fluorescent intensity, MFI) were analyzed by flowcytometry in these samples. Results: A total of 208 subjects (59 families, 126 females, 82 males) including 129 children 9.5±3.7 years and 79 adults (37±13 years) were evaluated. A new case with CD19 deficiency was not detected. There was at least one warning sign for immunodeficiency in 67 subjects (57 children and 10 adults). CD19 median expression value was lower than 60 MFI value in 20 subjects, which was known as in the carriers who were detected to have heterozygous mutation. Conclusions: Our findings suggested that CD19 median expression might be a diagnostic tool for the subjects who have CD19 heterozygous mutations. Mutation analyses are planned for the confirmation of CD19 heterozygous mutation in these subjects who have low CD19 median expression.Öğe CD19 molekül eksikliği: Bir köy taraması(2009) Reisli, İsmail; Artaç, Hasibe; Pekcan, Sevgi; Kara, Reyhan; Yümlü, Kadir; Karagöl, Cüneyt; Çimen, ÖmerAmaç: CD19 eksikliğinin ilk kez tanımlandığı bir olgunun köyünde yaşayan ve akrabası olan bir olguda daha CD19 eksikliği tanımlandı. Bu olgular, bu köyde yaşayan başka olguların ve taşıyıcıların da olabileceğini düşündürdü. Bu amaçla köy halkı immün yetersizlik düşündüren klinik bulgular ve CD19 molekül eksikliği yönünden değerlendirildi. Gereç ve Yöntem: CD19 eksikliği olgularının akrabaları ve kontrol grubu, anket formları ile birincil immün yetersizlik hastalıklarının klinik belirtileri için sorgulandı. Olgulardan alınan kan örneklerinde, CD19 ve CD21 molekül varlığı akış sitometri ile incelendi. Bulgular: Toplam 59 aileden 129 çocuk (9,53,7 yıl) ve 79 yetişkin (3713 yıl) olmak üzere 208 olgu (126 kadın, 82 erkek) değerlendirildi. Yeni bir CD19 eksikliği olgusu saptanmadı. Anket formları ile 67 olguda (57 çocuk, 10 yetişkin) en az bir uyarıcı immün yetersizlik bulgusu vardı. CD19 mediyan ekspresyon değeri (MFI) 20 olguda, CD19 yönünden heterozigot taşıyıcı olan bireylerden elde edilen 60 MFI değerinin altındaydı. Çıkarımlar: Bulgularımız CD19 eksikliği heterozigot “taşıyıcılığı” açısından, CD19 taşıyan B lenfosit oranının değil, CD19 mediyan ekspresyon düşüklüğünün yol gösterici olduğunu düşündürmüştür. CD19 mediyan ekspresyon değerine göre taşıyıcı oldukları belirlenen bu olguların, mutasyon tetkiklerinin yapılarak taşıyıcı olduklarının doğrulanması planlanmıştır.Öğe Çocukluk çağında tüberkülozu taklit eden kistik ekinokokkoz vakası(2011) Pekcan, Sevgi; Kiper, Nural; Köse, Mehmet; Çobanoğlu, Nazan; Yalçın, Ebru; Doğru, Deniz; Özçelik, UğurHem kistik ekinokokkoz (KE), hem de tüberküloz (Tbc), gelişmekte olan ülkelerde önemli sağlık bir problemidir. Akciğer KE’u ve Tbc’u, artmış morbidite ve mortaliteye sahip olmaları nedeniyle diğer hastalıklardan ayrılmalıdır. Aynı zamanda, bu iki hastalık da birbirini taklit edebilmektedir. Burada, ateş, öksürük şikayetleri ile başvuran ve yuvarlak pnömoni düşünülüp nonspesifi k antibiyotik tedavisine cevap vermeyen, tüberkülin deri testi (TDT) pozitifl iğinin ve radyolojik incelemede Tbc’la uyumlu görünümün tespit edilmesi nedeniyle Tbc tedavisi başlanan ancak izleminde karaciğerde kist ve ekinokok spesifi k IgE pozitifl iği saptanıp KE tanısı alan ve antiparaziter tedavi ile tedavi edilen 7 yaşında bir çocuk hasta sunuyoruz.Öğe Çocukluk Çağında Yabancı Cisim Aspirasyonları(2010) Pekcan, Sevgi; Aslan, Ayşe TanaYabancı cisim aspirasyonları çocukluk çağında sık görülür ve ani çocuk ölümleri arasında önemli yer tutar. Üç yaş altında ve erkek çocuklarda daha sık rastlanır. Ani başlayan öksürük, hışıltı ve tek taraflı azalmış solunum sesleri en sık görülen bulgulardır. Radyolojik görüntüler tanısal olmasa da tek taraflı amfizem ve atelektazi en önemli bulgulardır. Belirgin öykü vermeyen hastalarda tanı gecikebilmektedir. Trakeobronşiyal yabancı cisimlerde tedavi acildir. Şüphelenilen vakalarda bronkoskopi yapılmalıdır. Koruyucu önlemler yabancı cisim aspirasyon insidansını azaltmaktadır.Öğe Comparing the efficacy of nebulizer recombinant human DNase and hypertonic saline as monotherapy and combined treatment in the treatment of persistent atelectasis in mechanically ventilated newborns(WILEY, 2012) Altunhan, Hüseyin; Annagür, Ali; Pekcan, Sevgi; Örs, Rahmi; Koç, HasanBackground: The purpose of the present study was to compare the cost-effectiveness and efficacy of nebulizer recombinant human DNase (rhDNase) and hypertonic saline (HS) as monotherapy and combined treatment in neonatal atelectasis. Methods: Eighty-seven newborns with persistent atelectasis who did not respond to traditional treatment were studied retrospectively. Group 1 did not receive nebulizer drugs; Group 2 received 7% HS; Group 3 received rhDNase; and Group 4 received both 7% HS and rhDNase. Subjects' chest X-ray scores, partial pressure of CO2, respiratory rate, fraction of inspired oxygen (FiO2) peak inspiratory pressure, atelectasis healing rate, median duration of nebulizer treatment and costs were compared. Results: Percentages of improvement in atelectasis on Day 3 of treatment in Group 1, Group 2, Group 3 and Group 4 were 27, 70, 81 and 95%, respectively, while median duration of treatment was 8.1, 3.3, 2.9 and 2.4 days, respectively. Comparison of chest X-ray scores, partial pressure of CO2, respiratory rate, FiO(2) and peak inspiratory pressure values before and 48 h after treatment did not yield a significant difference for the control group (P > 0.05), while a marked improvement was observed in other groups for all parameters (P < 0.05). The most distinct improvement was in Group 4, followed by Group 3. Conclusions: Although both the combined treatment with HS and rhDNase and their monotherapies are effective in the treatment of persistent atelectasis in newborns receiving mechanical ventilation, their combined use produces higher efficacy. The efficacy of rhDNase is superior to monotherapy with HS. Use of these two treatments concomitantly reduces the cost. To the best of our knowledge, the present study is the first to use HS alone or in combination with rhDNase in newborn patients.Öğe Congenital Tuberculosis in Premature Twins After in Vitro Fertilisation(BMJ Publishing Group, 2009) Altunhan, Hüseyin; Keser, Melike; Pekcan, Sevgi; Ural, Onur; Örs, RahmiGenital tuberculosis is a major cause of infertility in countries where tuberculosis is endemic. With recent advances in assisted reproductive technology, women with tuberculous endometritis may become pregnant and ultimately have children with congenital tuberculosis. In vitro fertilisation represents a useful treatment for infertility. Congenital tuberculosis is a rare and severe, rapidly progressive, disease. Making an early diagnosis of congenital tuberculosis is difficult, because it can mimic many neonatal illnesses. To the best of our knowledge, we report the first cases of premature twins with congenital tuberculosis following in vitro fertilisation. After the diagnosis of genital tuberculosis in the mother, the twins were screened and diagnosed for tuberculosis. The twins dramatically improved after anti-tuberculosis therapy. We also wish to draw attention to the importance of searching for genital tuberculosis as the possible cause of congenital infection, even if the mother is asymptomatic.Öğe Efficacy of intravenous immunoglobulin treatment in immunocompromised children with H1N1 influenza: a clinical observation(WILEY-BLACKWELL, 2016) Gokturk, Bahar; Pekcan, Sevgi; Guner, Sukru Nail; Artac, Hasibe; Keles, Sevgi; Kirac, Mine; Reisli, IsmailBackground and AimsThe appropriate treatment of pandemic H1N1 influenza which was first identified in April 2009 in Mexico is insufficient especially for immunocompromised patients. We aimed to evaluate the features and prognostic factors of the children with H1N1, especially immunocompromised ones, and whether intravenous immunoglobulin G (IVIG) replacement could aid for a better outcome. MethodsTwenty-one hospitalized children with laboratory-confirmed H1N1 were evaluated retrospectively. Data were extracted from files and electronic medical records. ResultsThe median age was 37 (1-216) months; 62% of them were under 5years of age and 71.4% had one or more underlying disorders. Main symptoms were high fever, cough, fatigue and vomiting. Lower respiratory tract manifestations were seen in 66.6% of children. Mortality rate was 4.7%. The patient who died had the lowest lymphocyte (100/mm(3)), thrombocyte (21000/mm(3)) and highest blood urea nitrogen (87mg/dL) levels. Fifty-eight percent of evaluated patients had one of the primary immunodeficiency disorders. Surprisingly, none of the six patients with primary immunodeficiency who are on regular IVIG replacement needed intensive care unit and died. Although median durations of cough, fever and hospitalization were lower, they did not change statistically according to get IVIG replacement regularly (P=0.47, 0.97, 0.09, respectively). ConclusionOur study is important while it is the first one that shows the course of primary immunodeficient children with H1N1 infection who were on regular IVIG replacement. A trial of high-dose IVIG may be a useful adjunctive therapy in severe H1N1 influenza, particularly in the immunocompromised patients.Öğe Freeman-Sheldon (Whistling Face) Syndrome With Hyperpyrexia in the Newborn: Case Report(Medecine Et Hygiene, 2010) Altunhan, Hüseyin; Annagür, A.; Ertuğrul, S.; Pekcan, Sevgi; Örs, R.Freeman-Sheldon (whistling face) syndrome with hyperpyrexia in the newborn: case report: Freeman-Sheldon syndrome (FSS) is a rare, multiple congenital contracture syndrome that is relatively well-known, since affected children have a striking appearance. This entity was historically referred to as the "whistling-face syndrome". Malignant hyperthermia and hyperpyrexia have been documented in FSS after general anesthesia related to the neuropathy. We report a male neonate with FSS and hyperpyrexia without anesthesia. To our knowledge, our patient is the first in the literature with hyperpyrexia in the newborn period without anesthesia.Öğe The Incidence of Cystic Fibrosis in the Central Region of Anatolia in Turkey Between 2015 and 2016(2019) Hangül, Melih; Pekcan, Sevgi; Köse, Mehmet; Acıcan, Deniz; Şahlar, Tuba Esra; Erdoğan, Murat; Kendirci, Mustafa; Güney, Deniz; Öznavruz, Hasan; Demir, Osman; Ercan, Ömür; Göçlü, FatmaBackground: Cystic fibrosis is the most common metabolic chronic disease among European Caucasian children. Cystic fibrosis incidence in Northern Europeans countries is approximately 1 in 3000 births while the worldwide prevalence varies considerably.Aims: To determine the incidence of cystic fibrosis in the central region of Anatolia in Turkey using the newborn screening program data.Study Design: Cross-sectional study.Methods: We used the records of the newborn screening program which is implemented by the Konya and Kayseri Provincial Health Directories. Between January 2015 and December 2016, there were a total of 119006 live births in Konya and Kayseri. The newborn screening test was applied to all these babies.Results: During this period, there were 22 live born babies diagnosed with cystic fibrosis in Konya with an incidence of 2.9 per 10000 live births and 13 live born babies diagnosed with cystic fibrosis in Kayseri with an incidence of 2.8 per 10000 live births. In genetic of 30 patients, fifteen patients were homozygous, and 15 patients were a compound heterozygote. Twenty-one different gene variants were detected and the most common mutation was F508del (17/30)Conclusion: We found the incidence of cystic fibrosis in central Anatolia similar to northern European countries.Öğe Konjenital Lober Amfizemi Taklit Eden Yabancı Cisim Aspirasyonu(2012) Pekcan, Sevgi; Uygun, Hasibe; Göktürk, Bahar; Altınok, Tamer; Resili, İsmail; Artaç, HasibeÖZET Giriş: Konjenital lober amfizem (KLA), süt çocuğunda solunum yetmezliğine yol açan nadir akciğer gelişim malformasyonlarındandır. Hastalar, sıklıkla yaşamın ilk 6 ayında tekrarlayan solunum sıkıntısıyla başvururlar. Yabancı cisim aspirasyonu (YCA), çocukluk çağında morbidite ve mortalitenin önemli bir sebebidir. Tekrarlayan alt solunum yolu infeksiyonu hikayesi olan ve tedaviye cevap vermeyen hastalarda YCA akla gelmelidir. Olgu Sunumu: KLAi taklit eden uzun süreli solunum şikayeti olan ve radyolojik bulguları 11 aydır havalanma farkı şeklinde sebat eden 16 aylık YCA olgusu sunuldu. Tartışma: Yabancı cismin akut ve kronik dönemdeki radyolojik bulgusu her iki akciğer arasında havalanma farkı olabilir ve bu bulgu küçük çocuklarda bazen KLAi düşündürebilir. (Gün cel Pediatri 2012; 10: 70-3)Öğe Konjenital Lober Amfizemi Taklit Eden Yabancı Cisim Aspirasyonu(Galenos Yayincilik,, 2012) Pekcan, Sevgi; Uygun, Hasibe; Göktürk, Bahar; Altınok, Tamer; Artaç, Hasibe; Reisli, İsmailIntroduction: Congenital lobar emphysema (CLE) is a rare malformation of lung development which may be the cause of respiratory insufficiency of the suckling child. Patients often present within the first 6 months of life with recurrent respiratory distress. Foreign body aspiration (FBA) is an important cause of morbidity and mortality in childhood. Foreign body aspiration should be thought in patients who have a history of recurrent lower respiratory infections and resistance to treatment. Case Report: We report a case of 16 month old girl with FBA mimicking CLE. She was suffering for persistan respiratory symptoms and radiological finding was maintaned as the difference between two sides of the lungs aeration for 11 months. Conclusion: In the acute and chronic period of FBA, the radiological finding can be different between two sides of the lungs aeration and this finding sometimes might suggest CLE in infancy.Öğe Mekonyum İleusunun 12 Ay Altında Tanı Alan Kistik Fibrozlu Hastalarda Klinik Seyire Etkisi(2010) Pekcan, Sevgi; Kiper, Nural; Köse, Mehmet; Aslan, Ayşe Tana; Çobanoğlu, Nazan; Yalçın, Ebru; Doğru, Deniz; Özçelik, UğurAmaç: Mekonyum ileusu kistik fibrozda %10-20 oranında görülür. İlk tanı bulgularından biridir. On iki ay altında tanı alan kistik fibrozlularda mekonyum ileusu varlığının seyire etkisini mekonyum ileusu olmayan grup ile karşılaştırmak. Gereç ve Yöntem: Bu çalışmada Hacettepe Üniversitesi Tıp Fakültesi Çocuk Göğüs Hastalıkları Bölümünde 1987-2005 yılları arasında 12 ay altında mekonyum ileusu ile kistik fibroz tanısı alan 22 (11K/11E) hasta ile diğer nedenlerle tanı alan 192 (85K/107E) hastanın demografik, klinik özellikleri, kolonizasyonları, solunum işlevleri, hastaneye yatış sayıları, mutasyon tipleri ve takip süreleri karşılaştırıldı. Bulgular: Hastaların son vücut kitle indeksi, yaş, zorlu ekspirasyonun 1. saniyesinde atılan volüm (FEV1), vücut ağırlığı ve boy persantilleri arasında istatistiksel olarak fark saptanmadı. Staphylococcus aureus kolonizasyonu, Pseudomonas aeruginosa kolonizasyonu, mutasyon analizi arasında istatistiksel olarak fark saptanmazken, bronşektazi gelişiminin ve psödo-Bartter sendromu sıklığının mekonyum ileusu olmayan grupta istatistiksel olarak anlamlı olarak yüksek olduğu bulundu. Çıkarımlar: Bu hastalar çok merkezli olarak ve ortak tedavi yaklaşımıyla tedavi edilip, takip edildiğinde erken tanı alan mekonyum ileusu olan ve olmayan grup arasında önemli fark yaşanmamaktadır. Mekonyum ileusu kistik fibrozda kötü seyir işareti değildir.Öğe Mekonyum Ileusunun 12 Ay Altında Tanı Alan Kistik Fibrozlu Hastalarda Klinik Seyire Etkisi(GALENOS YAYINCILIK, 2010) Pekcan, Sevgi; Kiper, Nural; Köse, Mehmet; Aslan, Ayşe Tana; Çobanoğlu, Nazan; Yalçın, Ebru; Doğru, Deniz; Özçelik, UğurAim: Meconium ileus (MI) is an early clue to the diagnosis of cystic fibrosis (CF) and it occurs in about 15-20% of CF patients. To determine the possible prognostic factors in patients with cystic fibrosis with and without MI. Material and Method: The sample consisted of 214 patients with CF diagnosed at younger than 12 months admitted to the pediatric pulmonology unit over a period of 18 years between January 1987 to December 2005. 22 patients presented with MI (MI group), 192 patients had non MI (nonMI group). Demographic, clinical, nutritional and laboratory data were obtained. Results: There were 11 female, 11 male patients in MI group, 85 female and 107 male patients in nonMI group. A statistically significant difference was not observed between the groups studied regarding the following variables: BMI, Staphylococcus aureus and Pseudomonas aeruginosa colonisations, mutation types, respiratory functions assesed by FEV1, lung findings. Pseudo-Bartter’s syndrome and progression to bronchiectasis were detected higher in nonMI group statistically. Conclusions: There is no prognostic difference between two groups when these patients are diagnosed early and followed-up by a multidisciplinary action and a collective treatment approach. The results of the present study suggest that meconium ileus is not an indication of a more severe phenotype of cystic fibrosis.Öğe Pediatric pulmonology in a developing country: our focus(TURKISH J PEDIATRICS, 2011) Pekcan, Sevgi; Aslan, Ayse Tana; Kiper, Nural; Kose, Mehmet; Cinel, Guzin; Cobanoglu, Nazan; Yalcin, EbruThis study was designed to determine the profile of our pediatric pulmonology unit in Turkey, a developing country, by investigating the patients admitted to our unit for the first time. Our objectives were: to determine the profile of patients admitted for the first time, to compare their initial diagnoses before referral to our hospital with the diagnoses determined in our unit, to determine the definitive diagnoses for patients requiring advanced intervention with invasive diagnostic methods, and to follow the treatments, operations and invasive-noninvasive mechanical ventilation practices. With these objectives in mind, the records of 412 patients who visited the pediatric pulmonology clinic within a six-month period were reviewed. The referral diagnoses, consisting mostly of primary ciliary dyskinesia, recurrent lung infections caused by immune deficiency and bronchiectasis, as well as definitive diagnoses were recorded. Tuberculosis (14%), cystic fibrosis (7.8%), bronchiectasis (4.6%), immune deficiency (1.6%), hydatid cyst (2%), and primary ciliary dyskinesia (1%) were the most commonly diagnosed diseases. Final diagnosis in 145 of the 412 patients (35.2%) differed from the referral diagnosis. Consanguineous marriages are encountered more commonly in developing countries like Turkey, leading to an increased incidence of genetic diseases such as primary ciliary dysgenesis, cystic fibrosis and immune deficiencies. Infectious diseases such as hydatid cyst and tuberculosis are also common. In any country in which there is a unique distribution of diseases, in other words, a characteristic and unique disease spectrum, courses and instructional fellowship programs should be arranged accordingly.
