Browsing by Publisher "Turkish National Pediatric Society"

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    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.
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    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.
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    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.