Ali Tekin

In recent years, the emergence of newly characterized entities with eosinophilic/oncocytic cytoplasm has posed significant diagnostic challenges, particularly due to the overlapping morphologic features of chromophobe renal cell carcinoma (ChRCC) and renal oncocytoma (RO). In this study, we analyzed the clinicopathological and immunohistochemical (IHC) characteristics of 49 eosinophilic renal tumors to determine whether GATA3 expression could aid in the differential diagnosis of these neoplasms. On the basis of integrated morphologic and IHC evaluation, the tumors were reclassified into the following diagnostic categories: ChRCC (n=15), RO (n=13), unclassified eosinophilic/oncocytic renal cell carcinoma (U-EO-RCC; n=9), low-grade oncocytic tumor (LOT; n=5), and eosinophilic clear cell renal cell carcinoma (E-CCRCC; n=3). In addition, the cohort included single cases of papillary renal neoplasm with reverse polarity (PRNRP), unclassified RCC with MiT family translocation-like features (U-MiTFL-RCC), SDHB-deficient RCC, and FH-deficient RCC. Notably, one of the U-EO-RCC cases was further characterized by the presence of a TSC mutation. All LOT cases, one SDHB-deficient RCC, one FH-deficient RCC, one PRNRP, and one of fifteen chromophobe RCC’s had diffuse/strong staining with GATA3. In contrast, all E-CCRCC (n=3), renal oncocytoma (n=13), and U-MiTFL-RCC (n=1) cases demonstrated complete GATA3 negativity. In conclusion, GATA3 serves as a valuable diagnostic adjunct in the differential diagnosis of emerging eosinophilic renal tumor entities. However, due to its expression across diverse subtypes, GATA3 results must be interpreted within the morphologic context and as part of a comprehensive immunohistochemical panel—including K7, K20, CD117, vimentin, FH, SDHB, and cathepsin K—to avoid potential diagnostic pitfalls and ensure the accurate identification of clinically significant syndromes.

Background. Cystinuria is a rare autosomal recessive disorder leading to recurrent cystine stone formation, often necessitating repeated surgical interventions. In Türkiye, tiopronin—a proven medical therapy—has become inaccessible since 2019, raising concerns about its economic and clinical consequences. This study aimed to compare the annual hospital costs of surgical management with the theoretical cost of tiopronin therapy in pediatric cystinuria patients. Methods. This single-center, retrospective study included 10 consecutive pediatric patients (median age: 12 years; range: 3-17 years) with genetically or biochemically confirmed cystinuria who underwent surgery for cystine stones in 2023. The annual cost of surgical management was calculated by summing all direct medical costs (operating room, anesthesia, devices, hospitalization, and related diagnostics) obtained from the hospital billing database. The theoretical annual cost of tiopronin therapy was calculated based on the last accessible market price of the drug in Türkiye, converted to Turkish Lira at the 2023 average exchange rate, and adjusted per patient using a standard dosing regimen of 15 mg/kg/day. Costs were compared using the Mann-Whitney U test. Results. The median annual surgical cost per patient was 49,936 TL (range: 14,791–84,576 TL), compared to a theoretical tiopronin cost of 27,923 TL (range: 9,307–27,925 TL). Surgical management was significantly more expensive than tiopronin therapy (p=0.001) in 9 of 10 cases. Six patients had a history of tiopronin use, and five of them experienced a surgery-free interval during medication (median duration: 3 years; range: 2-6 years). Preliminary follow-up data for 2024 revealed that 70% of patients required further surgical interventions (median: 2 surgeries per patient), demonstrating the persistent and recurrent nature of the disease when managed solely with surgery. Conclusion. Surgical treatment of cystinuria poses a substantially higher economic burden compared to tiopronin therapy. Our findings support the reintroduction of tiopronin into the Turkish healthcare system, particularly for pediatric patients with frequent stone recurrence.

The use of the vermiform appendix as a replacement for ureteral segments has been rarely reported, particularly in children, and even more rarely in the left ureteral stenosis. Herein, a case where the appendix was successfully used to bridge a left mid-ureteral iatrogenic stricture in a child was presented. A two-year-old male with a history of ureteric stone and laser lithotripsy at six months of age presented with ongoing hydroureteronephrosis and recurrent urinary tract infections despite prophylactic antibiotics. Hydroureteronephrosis arose two months after the surgery and progressed slowly. Retrograde pyelogram revealed a stenosis in the left mid-ureter 4 cm above the iliac cross. The mesoappendix allowed the appendix to be used for interposition on the left ureter. The patient was followed with a JJ stent for two months. Ureterorenoscopy during JJ removal showed no stricture. Three years after the surgery, imaging showed improved hydronephrosis, stable function, and no obstructive findings on the nuclear renal scan. Children undergoing stone surgery should be monitored for potential complications, including strictures. Appendiceal interposition should be considered a viable treatment option for ureteric injuries, even for the left ureter.

Objectives: In this study, we aimed to assess the perspective of the patients and the parents regarding their satisfaction with the televisits in our center. Patients and methods: Between November 2019 and December 2021, a total of 247 participants (155 males, 92 girls, median age 76.9 months; range, 4.9 to 518.6 months) who met the inclusion criteria were analyzed. A questionnaire which was prepared to evaluate parents' satisfaction with telemedicine, was sent to parents of the children who were evaluated with televisits. Results: There was no statistically significant difference between the groups in terms of the satisfaction and simplicity scores according to the diagnosis (p=0.099 and p=0.243, respectively). Also, 77% of parents in our study found televisits similar to face-to-face (in-person) visits. Conclusion: Our study results show that patient satisfaction with televisit is high in terms of ease of use, shortened travel time and reduced cost. In addition, televisit provides parents with a positive opportunity to take time off from work. The majority of our participants reported that they desired to continue televisit after the end of the pandemic.

Objectives: Ureteroscopic lithotripsy and extracorporeal shock wave lithotripsy methods are commonly used in the treatment of proximal ureteral stones. There are no adequate studies showing which method is more effective in children. In our study, we aimed to evaluate and compare the efficacy of two treatment methods, commonly used for proximal ureteral stones in children.Methods: A total of 78 patients who underwent ureteroscopic lithotripsy (n = 38) and extracorporeal shock wave lithotripsy (n = 40) due to stones located in the proximal ureter between 2010 and 2021 were included in the study. Demographic data, clinical characteristics, and treatment outcomes were retrospectively analyzed. Kolmogorov–Smirnov, Chi‐square, and Mann–Whitney U tests were used for statistical analysis.Results: There was no statistical difference between the demographic characteristics of the groups, except for the mean age values (p = 0.008). A statistically significant difference was found in favor of the extracorporeal shock wave lithotripsy group in terms of stone‐free rates after the first intervention, complication rates requiring intervention, re‐intervention rates, and the average number of anesthesia sessions per patient until stone‐free status (p = 0.043, p = 0.009, p = 0.017, and p < 0.001, respectively).Conclusions: The results of this retrospective study suggest that extracorporeal shock wave lithotripsy is the primary treatment option for single, non‐complicated proximal ureteral stones.

Bu olgu sunumunda, 22. gestasyonel haftada rutin fetal ultrasonografide anomali ön tanısı alan ve 39. haftada sezaryenle doğan bir erkek yenidoğanda saptanan klasik mesane ekstrofisi vakası ele alınmaktadır. Fizik muayenesinde karın ön duvarı ile mesane ön duvarının tamamen açık olduğu, penisin kısa ve üretranın açık olduğu saptanmıştır. 20.000-33.000 canlı doğumda bir görülen bu kompleks anomalide cerrahi öncesi mesane mukozasının nemli tutularak korunması büyük önem taşır. Olguya postnatal 3. saatte bilateral üreteroneosistostomi, mesane boynu onarımı ve primer kapatma uygulanmış; 18 aylıkken ise epispadias onarımı başarıyla tamamlanmıştır. Tedavi sürecinde ve sonrasında fistül, reflü, enfeksiyon ve fıtık gibi komplikasyonlar gelişebilmektedir. This case report presents a male newborn diagnosed with classical bladder exstrophy, suspected during routine fetal ultrasonography at the 22nd gestational week and delivered via cesarean section at the 39th week. Physical examination revealed a completely open anterior abdominal and bladder wall, a short penis, and an open urethra. In this complex anomaly, occurring in 1 in 20,000-33,000 live births, keeping the bladder mucosa moist to protect it prior to surgery is crucial. The patient underwent bilateral ureteroneocystostomy, bladder neck reconstruction, and primary closure at the 3rd postnatal hour, followed by successful epispadias repair at 18 months of age. Complications such as fistulas, reflux, infections, and hernias may develop during and after treatment.

Konjenital böbrek ve üriner sistem anomalileri, sık görülen, günümüzde görüntüleme yöntemlerindeki gelişmeler neticesinde daha sık tanı alan malformasyonlardır. Çocukluk çağında görülen son dönem böbrek hastalığının %30-60'ını, yetişkin son dönem böbrek hastalığının %7'sini oluştururlar ve bu durum hastaları yaşam boyu hipertansiyon ve kardiyovasküler hastalığa duyarlı hâle getirir. Bu anomalilere sahip olan çocukların bir kısmı asemptomatik olabilir, ancak diğerleri çok ağır bulgularla başvurabilir. Doğum öncesi ultrasonografi ile saptanan en sık anomali, antenatal hidronefrozdur. Altta yatan çok farklı konjenital ve üriner sistem anomalisinin bulgusu şeklinde karşımıza çıkabilir. İşte tam da bu nedenden ötürü, bu farklı kliniklerin geniş yelpazesini siz okuyucularımıza sunabilmek için bu kitap fikri oluştu. Bu amaçla hastanemiz çocuk nefroloji ve çocuk üroloji kliniklerinde tanı almış, takip edilmiş, tedavisi yapılmış ve izlemlerine devam edilen konjenital böbrek ve üriner sistem anomalilerine sahip vakalarımızı bir araya getirerek bu farklı yelpazeyi siz okuyucularımızla paylaşmak istedik. Vakaları çözümlerken, bir taraftan da okuyucunun konu ile ilgili bilgilenmesine yardımcı olacak küçük bilgiler vakalara eklendi.