Browsing by Publisher "Turkish National Pediatric Society"
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Item Xanthogranulomatous pyelonephritis due to calculi in a 5-year-old girl(Turkish National Pediatric Society, 2020) Önder E.N.A.; Özkol M.; Neşe N.; Taneli C.; Özünan İ.Background. Xanthogranulomatous pyelonephritis is characterized by the inflammatory destruction of the renal parenchyma and intensive renal fibrosis. It is named because of its pathological appearance; that of its granulomatous inflammatory process with lipid-laden macrophages, which appear yellow, hence ‘xantho’ which is Greek for yellow. Xanthogranulomatous pyelonephritis is predominantly a disease of adults. In children it is diagnosed sporadically and is extremely rare in infants. The age of onset varies (21 days to 16 years), although 60-75% of cases have been diagnosed before 5 years of age. Recurrent urinary tract infections, obstructive nephropathy caused by renal calculus, malnutrition, abnormal lipid metabolism, altered immunologic response, lymphatic blockage, congenital urinary anomalies have been implicated in the etiology of xanthogranulomatous pyelonephritis in children. Case. We report an unusual case of xanthogranulomatous pyelonephritis in a 5-year-old girl and discuss its clinical features, histopathological findings and treatment. In this article, we also emphasized the importance of diagnostic imaging in urinary tract infections which enabled us not to miss the underlying kidney stone disease. Conclusion. Nephrolithiasis may lead to very serious conditions such as xanthogranulomatous pyelonephritis. This condition can be easily diagnosed by ultrasound, but if not detected, it can lead to complete loss of renal function as in the case. © 2020, Turkish Journal of Pediatrics. All rights reserved.Item Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data(Turkish National Pediatric Society, 2023) Onay Z.R.; Eyüboğlu T.Ş.; Aslan A.T.; Gürsoy T.R.; Yalçın E.; Kiper N.; Emiralioğlu N.; Şen H.S.; Şen V.; Ünal G.; Yılmaz A.İ.; Kılınç A.A.; Çokuğraş H.; Başkan A.K.; Yazan H.; Çollak A.; Uzuner S.; Şasihüseyinoğlu A.Ş.; Özcan D.; Altıntaş D.U.; Öztürk G.K.; Demir E.; Bingöl A.; Başaran E.; Çekiç Ş.; Sapan N.; Irmak İ.; Damadoğlu E.; Tuğcu G.D.; Polat S.E.; Özdemir A.; Harmancı K.; Kılıç G.; Hangül M.; Köse M.; Tamay Z.; Yüksel H.; Özcan G.; Topal E.; Can D.; Korkmaz P.; Çaltepe G.; Kılıç M.; Özdoğan Ş.; Çakır E.; Çobanoğlu N.; Pekcan S.; Cinel G.; Özçelik U.; Doğru D.Background. We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV₁) decline and LT candidates without rapid FEV₁ decline in the last year to identify a preventable cause in patients with such rapid FEV₁ decline. Methods. All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV₁ below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV₁ decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. Results. Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV₁ in 2017 in Group 1 and between FEV₁ values in 2017 and 2018 in Group 2. Conclusions. There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral. © 2023, Turkish National Pediatric Society. All rights reserved.Item Common viral respiratory infections in children with cancer during the COVID-19 pandemic: a multicenter study from Türkiye(Turkish National Pediatric Society, 2024) Kaçar D.; Kebudi R.; Özyörük D.; Tuğcu D.; Bahadır A.; Özdemir Z.C.; Özgüven A.A.; Orhan M.F.; Yıldırım A.T.; Albayrak C.; Kartal İ.; Sarı N.; Tokgöz H.; Albayrak M.; Ayhan A.C.; Eroğlu N.; Aydın S.; Üzel V.H.; Zülfikar B.; Yıldırım Ü.M.; Büyükavcı M.; Gülen H.; Töret E.; Bör Ö.; Özbek N.Y.; İlhan İ.E.; Yaralı N.Background. Microbiologic confirmation of respiratory tract infections gained importance during the coronavirus disease 2019 (COVID-19) pandemic. This study retrospectively evaluated seasonal distribution, clinical presentation, and complications of respiratory viral infections (RVIs) other than COVID-19 in children with cancer during and after the pandemic lockdown. Methods. Two hundred and sixty-five inpatient and outpatient RVI episodes in 219 pediatric cancer patients confirmed by multiplex reverse transcriptase polymerase chain reaction (RT-PCR) panels from 13 centers were enrolled. Results. Eighty-six (32.5%) of the total 265 episodes occurred in 16 months corresponding to the lockdowns in Türkiye, and the remaining 67.5% in 10 months thereafter. Human rhinovirus/enterovirus (hRE) (48.3%) was the most common agent detected during and after lockdown. Parainfluenza virus (PIV) (23.0%), influenza virus (9.8%), and respiratory syncytial virus (RSV) (9.1%) were the other common agents. The 28.7% of episodes were lower respiratory tract infections (LRTIs), and complications and mortality were higher than upper respiratory tract infections (URTIs) (25.0% vs 5.3%). Bacteremia was identified in 11.5% of culture-drawn episodes. Treatment delay in one-third and death within four weeks after RVI in 4.9% of episodes were observed. Conclusion. During the pandemic, fewer episodes of RVIs occurred during the lockdown period. Respiratory viruses may cause complications, delays in treatment, and even death in children with cancer. Therefore, increased awareness of RVIs and rapid detection of respiratory viruses will benefit the prevention and, in some cases, abrupt supportive and some antiviral treatment of RVI in children with cancer. © 2024, Turkish National Pediatric Society. All rights reserved.Item A child with intravascular fasciitis mimicking deep vein thrombosis: a case report(Turkish National Pediatric Society, 2024) Yiğit Y.; Erdoğan O.; Yıldırım A.T.; Özer E.; Gülen H.Background. Intravascular fasciitis (IF) is a benign, reactive, myofibroblastic proliferation that originates from the superficial or deep fascia of small / medium-sized arteries and veins. Case Report. An 8-year-old male patient was admitted to a health center with the complaint of swelling in the inguinal region. Lower extremity venous Doppler ultrasonography showed deep vein thrombosis (DVT) of the femoral vein and anticoagulation with low-molecular weight heparin (LMWH) was initiated. The patient was referred to our center for follow-up. The D-dimer level was detected within normal limits. Doppler ultrasonography was repeated and showed an intraluminal expanding mass lesion with increasing vascularity, without distinct borders and LMWH was discontinued. This lesion at the sapheno-femoral junction was excised surgically and the histopathological examination revealed intravascular fasciitis. Conclusion. Clinicians should be aware that the clinical findings of IF may mimic sarcoma and thrombosis. © 2024, Turkish National Pediatric Society. All rights reserved.Item Eligibility of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies: cohort of cystic fibrosis registry of Türkiye(Turkish National Pediatric Society, 2025) Erdal M.A.; Büyükşahin H.N.; Şen V.; Kılınç A.A.; Çokuğraş H.; Doğan G.; Yılmaz A.İ.; Ünal G.; Serbes M.; Altıntaş D.U.; Arık E.; Keskin Ö.; Özaslan M.M.; Karcıoğlu O.; Köse M.; Başaran A.E.; Çakır E.P.; Canıtez Y.; Özdemir A.; Harmancı K.; Uytun S.; Polat S.E.; Hangül M.; Yüksel H.; Özcan G.; Korkmaz P.; Kılıç M.; Aydın Z.G.G.; Çaltepe G.; Can D.; Doğru S.; Öztürk G.K.; Süleyman A.; Topal E.; Özsezen B.; Hızal M.; Demirdöğen E.; Ogun H.; Börekçi Ş.; Yazan H.; Şen H.S.; Demir A.D.; Çakır E.; Eyüboğlu T.Ş.; Emiralioğlu N.; Pekcan S.; Özçelik U.; Doğru D.Background. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variants are essential for determining eligibility for CFTR modulator drugs (CFTRms). In contrast to Europe and the USA, the treatment eligibility profile of cystic fibrosis (CF) patients in Türkiye is not known. In this study we aimed to determine the eligibility of CF patients in Türkiye for the CFTRms. Methods. The Cystic Fibrosis Registry of Türkiye (CFrT) data was used to determine the age of patients in the year 2021 and the genetic variants they were carrying. Age-and CFTR-variant appropriate modulator therapies were determined using the Vertex® algorithm. Results. Among a total of 1930 registered patients, CTFR gene analysis was performed on a total of 1841 (95.4%) patients. Mutations were detected in one allele in 10.7% (198 patients), and in both alleles in 79% (1455 patients) of patients. A total of 855 patients (51.7% for whom at least 1 mutation was detected) were eligible for the drugs. The most appropriate drug among genotyped patients was found to be elexacaftor/tezacaftor/ivacaftor for 486 patients (26.4%), followed by ivacaftor for 327 patients (17.7%) and lumacaftor/ivacaftor for 42 patients (2%). Conclusions. Only half of patients registered in CFrT were eligible for CFTRms, which is a significant difference from the CFTR variant profile seen in USA and Europe. However, access to treatment is hampered for some patients whose genes are not analysed. Further studies in CF populations, where rare mutations are relatively more common, will contribute to the field of CFTR modulator treatments for such rare mutations. © 2025 The Author(s).