Background: TRACK syndrome, first described by Canpolat et al., is a rare disorder caused by splice-site mutations in TSEN2, characterized by craniofacial malformations, central nervous system (CNS) anomalies, progressive kidney disease. Case Presentation: We present a 2.5-year-old boy, third child of consanguineous parents, admitted with fever and cough, diagnosed with atypical hemolytic uremic syndrome (aHUS). Whole exome sequencing revealed a homozygous intronic TSEN2 variant (c.1100-5T>A), consistent with TRACK syndrome. Two deceased siblings had similar dysmorphic features and early-onset renal failure. The proband exhibited microcephaly, craniofacial dysmorphism, left atrial dilation, and laboratory findings consistent with thrombotic microangiopathy (TMA). Despite pathogenic TSEN2 variant, he’d normal intellectual and motor development. He’s currently 6 years old, undergoing peritoneal dialysis, receiving biweekly eculizumab, and antiepileptic therapy. Conclusion: This report highlights a rare cause of aHUS and end-stage kidney disease (ESKD) due to a TSEN2 splice-site mutation, further expanding the phenotypic spectrum of TRACK syndrome.

Steroid-resistant nephrotic syndrome (SRNS) requires prolonged immunosuppressive therapy and is associated with an increased risk of severe infections. Panophthalmitis is an extremely rare but life-threatening ocular complication in immunosuppressed pediatric patients. Case Presentation We report a 7-year-old child with SRNS receiving long-term immunosuppressive therapy who presented with rapid unilateral vision loss, conjunctival hyperemia, severe ocular pain, and progressive periorbital edema. Orbital magnetic resonance imaging revealed findings consistent with panophthalmitis. Despite prompt and aggressive treatment—including systemic broad-spectrum antibiotics, intravitreal antimicrobial injections, antifungal therapy, and intravenous immunoglobulin—the infection progressed rapidly. The patient developed sepsis with multiorgan failure and ultimately died from cardiopulmonary arrest. Microbiological analysis of both blood and vitreous samples yielded Staphylococcus aureus. Conclusion This case underscores panophthalmitis as a rare yet fatal complication in children undergoing immunosuppressive treatment for SRNS. Early recognition of ocular symptoms and prompt multidisciplinary management are essential to prevent irreversible morbidity and mortality among immunosuppressed pediatric patients.

PURPOSE:This study evaluated the impact of needle size and the number of passes on complication rates and glomerular yield in pediatric native kidney biopsies performed under real-time ultrasound (US) guidance, aiming to determine the optimal biopsy approach. MATERIALS AND METHODS:Pediatric native kidney biopsies performed between March 2019 and February 2022 at a single tertiary-care children's hospital were included. Biopsies were done with a semi-automated 16 G needle until December 2020, then with an 18 G needle, with varying numbers of passes. US examinations were conducted 24 h post-biopsy to assess complications. Patients were categorized into six subgroups using needle size and the number of passes and compared for complication rates, glomerular yield, and diagnostic adequacy. RESULTS:A total of 145 pediatric patients (59.3% female, 40.7% male) with a mean age of 10.74 (±4.69) years were included. An 18 G needle was used in 76 (52.4%) cases and a 16 G needle in 69 (47.6%) cases. Minor complications occurred in 56 (38.6%) cases, with no major complications. Complication rates were not significantly different between 18 G and 16 G (p=0.088) but increased with the number of passes (p<0.001). Mean glomerular yield was higher with 16 G than 18 G (34.6 vs. 22.6, p<0.001), though not significantly affected by the number passes (p=0.123). The 18 G needle was associated with inadequate sampling (p=0.024). The optimal scenario was a 16 G needle with one or two passes. CONCLUSION:Real-time US-guided pediatric kidney biopsy is safe. A 16 G needle with one or two passes optimizes glomerular yield without increasing complications. Three or more passes should be avoided.

Background Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney stones and risk factors for their development in epileptic children receiving KD is unclear. The aim of this study was to determine the frequency and risk factors for the development of renal stones in children receiving KD therapy. Methods A total of 95 patients receiving KD were identified. Of these, seven patients were excluded from the study due to the duration of KD being less than 12 months. The remaining 88 children were enrolled in the study. Results Renal stones were detected in 15 patients (17%), of which 12 (73.3%) received potassium citrate treatment. Two (13.3%) patients needed lithotripsy despite receiving potassium citrate treatment, and one of these, who received potassium citrate treatment for 5 months, developed acute vesicourethral reflux and underwent surgery. No patient discontinued KD due to renal stone development. The serum uric acid concentrations and urine calcium/creatinine ratio did not change significantly over the 24-month follow-up period. Age, gender, etiology, age at seizure onset, duration of KD, mobility status, use of topiramate or zonisamide, and the number of antiepileptic drugs used were not significantly different between patients with and without kidney stones. Conclusion Renal stone appears to be a common adverse effect of KD therapy. Although adequate hydration and potassium citrate treatment are effective in most patients, lithotripsy and surgery may be required in a minority of patients.

Objective: The COVID-19 pandemic has caused a routine non-clinical course due to primary COVID-19 infection and affected non-COVID patients' access to healthcare services. This study aims to evaluate the changes in hospitalization diagnoses of patients admitted to a nephrology unit before and during the COVID-19 pandemic. Methods: The study evaluates the inpatients admitted between March 2018- 2020 and March 2020-2022 in the Nephrology Unit of Izmir Beh & ccedil;et Uz Pediatrics and Surgery Training and Research Hospital, University of Health Sciences. Results: This study includes a total of 1,453 patients. Of these patients, 882 were hospitalized in the pre-COVID period, and 571 were hospitalized during the pandemic. Although a significant difference occurs between genders, no significant differences were found in terms of age and length of stay. The most common diagnoses pre-COVID were urinary tract infections (UTI; 40.1%), nephrotic syndrome (NS; 12.9%), chronic kidney diseases (CKDs; 10.3%), and hypertension (HT; 10%). This order after the pandemic was UTI (35.7%), CKDs (20.8%), NS (10.7%), and HT (10.2%). A significant increase occurred regarding the frequency of CKDs post-pandemic (p = 0.000) and the frequency of hematuria pre-pandemic (p = 0.025). Conclusions: The study is important for being the only study conducted in the field of pediatric nephrology regarding the changes in pandemic hospitalizations. During the pandemic, hospitalizations of chronic patients increased, and hospitalizations of examination patients decreased due to the postponement of elective conditions. What is noteworthy is the decreased incidence of upper respiratory tract infections (URTI) and Henoch-Sch & ouml;nlein purpura (HSP), which are known to trigger nephrotic syndrome and glomerulonephritis. Another remarkable result is the decreased number of patients with recurrent UTIs. Although a decrease did occur in this group, the frequency of UTIs actually increased in general pediatric applications, which led to the emergence of missed diagnoses pre-pandemic

AMAÇ: Renal parankimal hastalıkların değerlendirilmesinde önemli bir tanı yöntemi olan böbrek biyopsilerinin değerlendirilmesi amaçlanmıştır. GEREÇ VE YÖNTEM: Bu çalışmada 333 çocuk olguya yapılan nativ böbrek biyopsisi değerlendirildi. Biyopsiler yarı otomatik ve ince (16-18 G) iğneler kullanılarak yapıldı. BULGULAR: : En sık biyopsi endikasyonu proteinüri ve hematüri birlikteliği, en sık saptanan patolojik tanı ise minimal lezyon hastalığınında dahil olduğu normal biyopsi sonucu olarak saptandı. Biyopsi sırasında ya da sonrasında komplikasyon izlenmedi. Ortalama glomerül sayısı 27.1±17.8, yeterli sayıda glomerül saptanma oranı % 81.4, sınır yeterlilikte glomerül saptanma oranı % 8.4 ve yetersiz sayıda glomerül saptanma oranı % 10 olarak hesaplandı. Ultrasonografi olmaksızın yapılan biyopsiler ile karşılaştırıldığında, ultrasonografi eşliğinde biyopsi yapılan % 28.2 olguda ortalama glomerül sayısı daha yüksek ve yetersiz glomerül oranı daha düşük saptandı (31.5±18.1 vs 25.4±17.5 ; % 2.1 vs % 13.3). SONUÇ: Böbrek biyopsisi çocuklarda USG eşliğinde veya USG olmaksızın, otomatik ve ince iğneler kullanılarak kolay uygulanabilen ve komplikasyon olasılığı düşük bir tanı yöntemidir. Ultrasonografi ile yapılan biyopsilerde yeterli glomerül elde edilme oranı daha yüksek olup olanaklı olduğu sürece ulatrasonografi eşliğinde biyopsi yapılmalıdır.

There are two major pandemics in the new millennium, including the pandemic of swine influenza and the COVID-19 pandemic. These two pandemics affected children as well as the adult population. In this case-control study, we compared children with COVID-19 infection and those with H1N1pdm09 virus infection. We also compared the demographic factors, underlying disease, and the requirement for intensive care admission between the hospitalized children with COVID-19 infection and children with H1N1pdm09 virus infection who were hospitalized during the 2009 H1N1 pandemic. In this study, we evaluated 103 patients with H1N1pdm09 virus infection and 392 patients with COVID-19 infection. The age was significantly higher in the COVID-19 patients' group compared to the pandemic influenza group (p < 0.001). The ratio of the children >= 12 years was 10.7% (n = 11) in the H1N1pdm09 virus infection and 36.2% (n = 142) in the COVID-19 group. The rate of underlying disease was significantly higher in the patients with H1N1pdm09 virus infections (p = 0.02). The prevalence of underlying disease in patients requiring PICU hospitalization was 69.2% (n = 9/13) compared to 25.7% (n = 124/482) in patients who did not require PICU hospitalization. The rate of underlying disease was significantly higher in the PICU group regardless of COVID-19 or H1N1pdm09 virus (p = 0.002). Our results suggest that older children were more hospitalized for COVID-19 infections compared to pandemic influenza. In addition, regardless of the type of pandemic infection, the underlying disease is an important factor for pediatric intensive care unit admission. This finding is important for developing strategies for the protection of children with the underlying disease in the upcoming pandemics.

Objective: In this study, we sought to assess pediatric residents’ level of knowledge about the diagnosis, treatment, and follow-up of urinary tract infection in children. Methods: This survey was a descriptive study applied to pediatric residents. In the study, a questionnaire form prepared by researchers consisting of questions about socio-demographic features, about the diagnosis, treatment, and follow-up of urinary tract infection in children was used. Results: Eighty-eight physicians participated in this research. The percentage of participants who correctly indicated urine culture based on the results of routine urinalysis in the diagnosis of urinary tract infection ranged from 95.5% to 96.6. 54.5% of participants (n=48) correctly identified the indication for ultrasonography in children with acute urinary tract infection. 67.0% (n=59) of the participants answered that Mercaptuacetyltriglycin was not appropriate for initial evaluation of recurrent urinary tract infection under the age of one year, while 33.0% (n=29) answered the question incorrectly. In clinical scenarios, 48.9% (n=43.0) participants made the proper decision for treatment of extended spectrum beta-lactamase-positive E. coli treatment. Conclusion: In conclusion, pediatric residents had appropriate training and experience in the diagnosis of urinary tract infection in children. However, over half of the residents lacked sufficient training in the management of resistant bacteria and additional radiological imaging techniques. Considering this, we believe it will be good to keep the knowledge updated concerning the treatment and follow-up of children with urinary tract infection through in-service training and post-graduate education.

Background There are limited numbers of studies focusing on renal effects of coronavirus disease 2019 (COVID-19) infection and proximal tubular dysfunction in children with COVID-19 infections. The purpose of this study was to evaluate the functions of the proximal tubule in hospitalized children with confirmed acute COVID-19. Methods The children who were hospitalized for confirmed COVID-19 were included in this prospective descriptive analysis. The presence of at least two of the following four abnormalities was used to diagnose proximal tubule injury: abnormal tubular reabsorption of phosphate, normoglycemic glycosuria, hyperuricosuria, and proteinuria. Results A total of 115 patients were included in the study. About a third of the individuals had elevated blood creatinine levels or proteinuria. In addition, abnormal renal tubular phosphate loss measured by renal tubular phosphate loss was found in 10 (8.7%) patients, as was hyperuricosuria in 28.6%. As a result, total proximal tubular dysfunction was found in 24 (20.9%) patients. Conclusions One in every five children with acute COVID-19 infections had proximal tubular dysfunction, according to our data. Although, the rate of proximal tubular dysfunction was lower than in adults, it should be noted. The recovery of proximal tubular function in children with COVID-19 should be followed.

IntroductionPeritoneal dialysis is the treatment of choice for end-stage renal disease. Peritoneal dialysis related peritonitis is of great importance for patient and technical survival. The aim of our study was to evaluate the accuracy and the correlation between the three methods (complete blood count, urinalysis device, urine dipstick test) and with the reference manual method (Thoma Cell Counter Chamber).Materials and methodsWe retrospectively analyzed 167 peritoneal fluid samples taken from 25 patients receiving peritoneal dialysis treatment. Leukocyte counts were evaluated with Thoma Cell Counter Chamber, complete blood count, urinalysis device and urine dipstick test.ResultsThere was a significant positive correlation between Thoma Cell Counter Chamber and complete blood count results (Spearman's rho=0.70), between Thoma Cell Counter Chamber and urinalysis device (Spearman's rho=0.73), and between Thoma Cell Counter Chamber and urine dipstick test (Spearman's rho=0.71). Area under curve for complete blood count, urinalysis device and urine dipstick test were 0.93, 0.94 and 0.89 respectively, indicating good accuracy. Sensitivity and specificity were 89.7% and 86.7% in the complete blood count analysis (associated criterion: 130 cells/mm3). Sensitivity and specificity were 89.7% and 86.7% in the urinalysis device (associated criterion: 10 cells/HPF). Sensitivity and specificity were 79.6% and 91.4% when in the urine dipstick test analysis (associated criterion: +1 positivity). The Bland-Altman plot showed good agreement.ConclusionAutomatic complete blood count and urinalysis devices have good correlation and agreement with manual method in the diagnosis of peritonitis in the pediatric age group. Urine dipstick test in the home setting can be useful for screening patients with suspected peritonitis.

BACKGROUND: We aimed to determine overall incidence of severe and mild isoniazid (INH) hepatotoxicity and outcome of hepatotoxicity in children who were receiving INH for latent tuberculosis. METHODS: Patients who had received isoniazid for treatment of latent tuberculosis were included in the study. Hepatotoxicity was classified according to the World Health Organization Toxicity Classification Standards. RESULTS: Among 1038 patients, overall hepatotoxicity was observed in 22 patients (2.2%), while 5 patients (0.48%) had moderate-severe hepatotoxicity; while other 17 patients had grade I-II hepatotoxicity (1.63%). Age and gender did not appear to be risk factors for hepatotoxicity. The median time for therapy rechallenge in patients with grade III-IV hepatotoxicity was 21 days (ranging from 14 to 25 days). CONCLUSIONS: Isoniazid hepatotoxicity is lower and generally reversible after cessation of INH in children. The grade of hepatotoxicity affects the duration for recovery of hepatotoxicity and restarting of INH therapy.

Pheochromocytoma is a rare tumor that develops from the chromaffin cells of the sympathetic nervous system. Although hypertension is the most common manifestation of pheochromocytoma, it is less common in children than in adults. This condition may be overlooked like in our patient who had an undiagnosed headache for several years. Our patient was admitted to our emergency department due to the increase in headache, which lasted for about 6 years, and a neurological evaluation was performed. Due to the high blood pressure in her follow-up, she was examined further. Abdominal USG revealed a 36x26x27 mm solid lesion in the right adrenal gland with a mild hyperechogenic appearance with a cystic component. With supporting findings in magnetic resonance imaging and high catecholamine levels in blood, she was diagnosed with pheochromocytoma. Our aim in sharing this case is to emphasize the importance of measuring blood pressure in patients accurately and at each examination, evaluating them with percentile charts, and monitoring them closely.

BACKGROUND:Amphotericin B is a broad-spectrum antifungal agent and is the backbone of the treatment for medically important opportunistic fungal pathogens in children. This study aimed to compare the nephrotoxicity associated with L-AmB in children with acute lymphoblastic leukemia and acute myeloid leukemia.MATERIALS AND METHODS:A total of 112 pediatric acute lymphoblastic leukemia or acute myeloid leukemia patients who received treatment with L-AmB (Ambisome®) at the University of Health Sciences Dr Behcet Uz Children's Hospital over 7 years were included. The incidence of hypokalemia, decreased estimated glomerular filtration rate and presence of acute kidney injury was recorded.RESULTS:The average L-AmB treatment duration was 17.1±15.0 days. Five patients (4.4%) of the patients had grade I acute renal injury according to KDIGO criteria and 16 patients (14.2%) had increased risk for kidney injury according to RIFLE criteria. There were no patients with eGFR decrease above 50% and no renal injury and failure were observed during L-AmB treatment. The rate of patients with hypokalemia in the pre-treatment was 17.9% and the post-L-AmB group was 50.0%. The rate of hypokalemia was higher in the post-treatment group (P=0.0015). Among the 112 patients, only two patients (1.7%) required cessation of L-AmB treatment due to resistant hypokalemia despite supplementation.CONCLUSIONS:Hypokalemia was more common compared to glomerulotoxicity and acute renal injury (according to KDIGO and RIFLE criteria) in pediatric leukemia patients treated with L-AmB. Hypokalemia developed in nearly half of the patients and the study shows the need for randomized controlled trials and strategies for hypokalemia associated with L-AmB treatment.

Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia and acute renal failure. HUS is generally classified into two types. While typical HUS follows a gastrointestinal infection with shiga-toxin producing E. coli (STEC), aHUS is associated with autoantibodies or mutations that lead to irregular complement activation. However, according to some recent studies, it was observed that diarrhea and multisystem involvement were seen in both HUS types. STEC positivity was also detected in a little percent of aHUS cases. Histopathological findings compatible with myocarditis were found in postmortem examinations in some cases. Although our case was diagnosed with atypical HUS, typical HUS symptoms such as bloody diarrhea and Shiga toxin 2 positivity were reported and myocardial involvement was observed as a rare complication.

Wilms tumor (nephroblastoma) is the most common kidney tumor in childhood. Two-thirds of cases are diagnosed before the age of 5 and more than 95% before the age of 10. Patients may present with abdominal mass, abdominal pain, bloody urination, fever, and weight loss [1,2]. In this article, we present a 6-year-old male patient who was examined for macroscopic hematuria. Laboratory tests for common causes of hematuria in childhood were found to be normal. Renal malignancy, a rare cause of hematuria, was considered due to a mass found in the left kidney on renal USG and abdomen tomography, and a left total nephroureterectomy was performed. After histopathological examination, Wilms tumor was diagnosed. If renal malignancies, especially Wilms tumor, are kept on mind as rare causes of macroscopic hematuria, the diagnosis can easily be made with radiological findings and pathological examination.

Objective: Uroflowmetry is on essential noninvasive test with important diagnostic method in patients with initial diagnosis of lower urinary tract dysfunction. We aimed to compare the results of conventional uroflowmetry with those of the new smart "self-directed outpatient" uroflowmetry in children with suspected lower urinary tract dysfunction. Methods: This cross-sectional study included children who had performed two sequential urinations in the same day recorded by conventional and smart "self-directed outpatient" uroflowmetry. Results of the measurements of maximum, and overage urinary flow rates were recorded and compared. Results: The mean difference between average urinary flow rates detected by both diagnostic methods was -1.7. The Bland-Altman plot showed that most of the data points were tightly clustered around the zero line of the difference between the measurements, with only 4% of the readings falling outside the 95% level of confidence. The mean difference between average urinary flow rates measured by both conventional and "self-directed outpatient" uroflowmetry was -4.5. The Bland-Altman plot showed that most of the data points were tightly clustered around the zero line of the difference between the measurements, with only 2% of the readings falling outside the 95% level of confidence. Discussion: The maximum, and average urinary flow rates measured with "self-directed outpatient" uroflowmetry were statistically significantly higher compared to conventional uroflowmetry. These results could be due to the children being much shyer and being affected by the presence of someone in the room while urinating. Conclusion: The maximum urinary flow rate and average urinary flow rate measured with "self-directed outpatient" uroflowmetry are higher compared to conventional uroflowmetry, which might ensure patient privacy.

Objective Smartphones and associated messaging applications have become the most common means of communication among health care workers and the general population. The aim of this study was to evaluate the reliability and accuracy of smartphones for the diagnosis of rash in children admitted to emergency departments during the night shift. Methods The images of the children who were admitted to the paediatric emergency department with rash were included in this study, and at least two images taken with smartphones by residents or paediatric infectious disease fellows were re-directed to the chief consultant of the Paediatric-Infectious Department via smartphone. Initial diagnosis by the consultant was recorded, and the patient’s physical examination was performed by another clinician on the first working day; diagnostic tests were planned by this clinician. The definitive diagnosis was recorded and compared with the initial diagnosis. Results Among the 194 patients, the most common final diagnoses were chickenpox (varicella-zoster infections) in 33 patients (17.0%) and skin infections (including impetigo, ecthyma, erysipelas and cellulitis) in 33 patients (17.0%). The initial diagnosis, which was performed via WhatsApp on a smartphone, was identical to the final diagnosis in 96.3% of the cases. Incompatible initial diagnoses included 4 measles cases, 1 staphylococcal scalded skin syndrome case, 1 cutaneous leishmaniasis case and 1 petechial rash case. Conclusions Our study has shown that the use of a smartphone-based instant messaging application for transmitting images of paediatric rash is accurate and useful for diagnosis. However, physical examination and medical history are still the primary methods. Consultation via smartphones in emergency departments for paediatric rashes during nightshifts would help both clinicians and patients.