Evaluation of pediatric trauma score and pediatric age-adjusted shock index in pediatric patients admitted to the hospital after an earthquake.

BACKGROUND: In our earthquake-prone country, it is crucial to gather data from regional hospitals following earthquakes. This information is essential for preparing for future disasters and enhancing healthcare services for those affected by earthquakes. This study aimed to evaluate the Pediatric Trauma Score (PTS) and the Shock Index, Pediatric Age-Adjusted (SIPA), in children affected by earthquakes, to provide clinicians with insights into the severity of trauma and hemodynamic stability. METHODS: The study included patients admitted to our hospital's pediatric emergency service within the three weeks following the earthquake. We evaluated their age, sex, admission vital signs, mechanical ventilation requirements, development of crush syndrome, length of hospital stay, PTS, and SIPA. RESULTS: Our study included 176 children (89 females and 87 males) with trauma. Fifty-eight (32.95%) children had crush syndrome, and 87 (49.43%) were hospitalized. The median PTS was 10 (ranging from -3 to 12), and the median SIPA was 1.00 (ranging from 0.57 to 2.10). We observed a negative correlation between the time spent under debris and PTS (r=-0.228, p=0.002) and a positive correlation with the SIPA score (r=0.268, p<0.001). The time spent under debris (p<0.001) and SIPA score (p<0.001) were significantly higher in hospitalized children. PTS was significantly lower in hospitalized children than in others. A PTS cutoff point of 7.5, and a SIPA cutoff point of 1.05, predicted hospitalization in all children. Time spent under debris and SIPA were significantly higher in children with crush syndrome than in others (p<0.001). PTS at a cutoff point of 8.5 and SIPA at a cutoff point of 1.05 predicted crush syndrome in all children. CONCLUSION: PTS and SIPA are important practical scoring systems that can be used to predict the severity of trauma, hospitalization, crush syndrome, and the clinical course in pediatric patients admitted to the hospital due to earthquake trauma.

The 2023 Türkiye-Syria earthquakes: analysis of pediatric victims with crush syndrome and acute kidney Injury.

BACKGROUND: On February 6th, 2023, two consecutive earthquakes struck southeastern Türkiye with magnitudes of 7.7 and 7.6, respectively. This study aimed to analyze the clinical and laboratory findings, as well as management of pediatric victims with Crush Syndrome (CS) and Acute Kidney Injury (AKI). METHODS: The study included pediatric earthquake victims who were presented to Mersin University Hospital. Clinical and laboratory characteristics of the patients were collected retrospectively. RESULTS: Among 649 patients, Crush injury (CI), CS and AKI was observed in 157, 59, and 17 patients, respectively. White blood cell count (12,870 [IQR: 9910-18700] vs. 10,545 [IQR: 8355-14057] /µL, P < 0.001), C-reactive protein (51.27 [IQR: 14.80-88.78] vs. 4.59 [1.04-18.25] mg/L, P < 0.001) and myoglobin levels (443.00 [IQR: 198.5-1759.35] vs. 17 [11.8-30.43] ng/ml) were higher in patients with CS, while their sodium (IQR: 134 [131-137] vs. 136 [134-138] mEq/L, P < 0.001) levels were lower compared to non-CS patients. An increase in myoglobin levels was identified as an independent risk factor for developing CS (OR = 1.017 [1.006-1.027]). Intravenous fluid replacement was administered to the patients with CS at a dose of 4000 cc/m2/day. Hypokalemia was observed in 51.9% of the CS patients on the third day. All patients with AKI showed improvement and no deaths were reported. CONCLUSIONS: Hyponatremia and increase in inflammation markers associated with CS may be observed. An increase in myoglobin levels was identified as a risk factor for CS. Hypokalemia may be seen as a complication of vigorous fluid therapy during hospitalization.

Pediatric kidney care experience after the 2023 Turkey/Syria earthquake.

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

Autonomic activity and cardiovascular system risk assessment in pediatric patients with hemolytic uremic syndrome.

In pediatric patients with hemolytic uremic syndrome (HUS), cardiac involvement and autonomic nervous system function can be evaluated by a non-invasive method called heart rate variability (HRV). This study aims to evaluate heart rate variability and electrocardiography findings in patients with HUS by comparing a healthy group. Patients who are diagnosed with HUS at a university hospital from December 2020 to June 2022 are screened by electrocardiography (ECG), echocardiography, and 24-h Holter ECG. A healthy control group, compatible in age and gender with the patient group, was selected from healthy subjects. HRV parameters, laboratory values, and ECG findings were analyzed and compared with the healthy group and each other. There were 25 patients with HUS and 51 participants in the healthy control group. Statistically significant differences were found in some HRV parameters: standard deviation of normal to normal intervals, the mean of the 5-min RR interval standard deviations, the standard deviation of 5-min RR interval means, the triangular interpolation of normal to normal interval, and very-low-frequency power. HUS patients had impaired and declined HRV values compared to the healthy group. There was a significant decrease in the PR distance, while a significant increase in the corrected QT and QT dispersion values was detected in the electrocardiographic findings of the patient group. HRV values impaired as renal failure parameters increased.  Conclusion: Patients with HUS may have autonomic nervous system dysfunction. HRV measurement is a non-invasive method that can evaluate this. It can be thought that there may be an increased risk of cardiovascular events and arrhythmias in some patients with HUS. ECG should be also considered to detect arrhythmia. What is Known: • Hemolytic uremic syndrome (HUS) primarily effects the hematologic parameters and kidney. • Secondary cardiomyopathy with hypertension and renal failure could be observed in these patients. • Rhythm problems are not expected primarily in these patients. • There is very limited data in evaluating autonomic function and arrhythmia risk for these patients. What is New: • Patients with HUS may have autonomic nervous system dysfunction. • HRV measurement is a non-invasive method that can evaluate this. • Cardiovascular events and arrhythmias due to the deterioration of the balance between the sympathetic and parasympathetic systems could manifest in patients with HUS. • An ECG and screening patients for cardiac events, and monitoring them closely should be considered.

Comprehensive evaluation of patients with primary hyperoxaluria type 1: A nationwide study.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is characterized by increased endogenous oxalate production and deposition as calcium oxalate crystals. The main manifestations are nephrocalcinosis/nephrolithiasis, causing impaired kidney function. We aimed to evaluate the clinical characteristics and overall outcomes of paediatric PH1 patients in Turkey. METHODS: This is a nationwide, multicentre, retrospective study evaluating all available paediatric PH1 patients from 15 different paediatric nephrology centres in Turkey. Detailed patient data was collected which included demographic, clinical and laboratory features. Patients were classified according to their age and characteristics at presentation: patients presenting in the first year of life with nephrocalcinosis/nephrolithiasis (infantile oxalosis, Group 1), cases with recurrent nephrolithiasis diagnosed during childhood (childhood-onset PH1, Group 2), and asymptomatic children diagnosed with family screening (Group 3). RESULTS: Forty-eight patients had a mutation consistent with PH1. The most common mutation was c.971_972delTG (25%). Infantile oxalosis patients had more advanced chronic kidney disease (CKD) or kidney failure necessitating dialysis (76.9% vs. 45.5%). These patients had much worse clinical course and mortality rates seemed to be higher (23.1% vs. 13.6%). Patients with fatal outcomes were the ones with significant comorbidities, especially with cardiovascular involvement. Patients in Group 3 were followed with better outcomes, with no kidney failure or mortality. CONCLUSION: PH1 is not an isolated kidney disease but a systemic disease. Family screening helps to preserve kidney function and prevent systemic complications. Despite all efforts made with traditional treatment methods including transplantation, our results show devastating outcomes or mortality.

Evaluation of the Claria sharesource system from the perspectives of patient/caregiver, physician, and nurse in children undergoing automated peritoneal dialysis.

BACKGROUND: Automated peritoneal dialysis (APD) is increasingly preferred worldwide. By using a software application (Homechoice with Claria sharesource system (CSS)) with a mod-M added to the APD device, details of the home dialysis treatment become visible for PD nurses and physicians, allowing for close supervision. We aimed to evaluate the perceptions of patients/caregivers, PD nurses, and physicians about the advantages and disadvantages of CSS. METHODS: Three different web-based questionnaires for patients/caregivers, nurses, and physicians were sent to 15 pediatric nephrology centers with more than 1 year of experience with CSS. RESULTS: Respective questionnaires were answered by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses. Most of the nurses and physicians (87% and 73%) reported that CSS improved patient monitoring. A total of 73% of nurses suggested that CCS is not well known by physicians, while half of them reported reviewing CSS data for all patients every morning. Sixty-eight percent of physicians thought that CSS helps save time for both patients/caregivers and healthcare providers by reducing visits. However, only 20% of patients/caregivers reported reduced hospital visits. A total of 90% of patients/caregivers reported that being under constant monitoring made them feel safe, and 83% stated that the patient's sleep quality improved. CONCLUSIONS: A remote monitoring APD system, CSS, can be successfully applied with children for increased adherence to dialysis prescription by giving shared responsibility and may help increase the patient's quality of life. This platform is more commonly used by nurses than physicians. Its potential benefits should be evaluated in further well-designed clinical studies with larger patient groups. A higher resolution version of the Graphical abstract is available as Supplementary information.

Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study.

BACKGROUND: Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. METHODS: Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. RESULTS: Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 ± 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. CONCLUSIONS: CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures.

A nationwide retrospective study in Turkish children with nephrocalcinosis.

BACKGROUND: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. METHODS: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. RESULTS: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5-208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. DISCUSSION: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease.

Could plasma based therapies still be considered in selected cases with atypical hemolytic uremic syndrome?

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone. METHODS: The outcomes of seven genetically confirmed aHUS patients (2 girls, 5 males) were evaluated by means of clinical presentation, response to plasma therapy, course of the disease during the follow-up period and last status. RESULTS: The median age of the patients at admission was 6.7 years (IQR 0.7-7.8). Three patients received plasma exchange therapy and the other four patients were treated with plasma infusions. One patient was lost to follow-up after one year; the median duration of follow-up for other patients was 3.7 years (IQR 2.7-6.5). During the follow up, two patients from our historical records when complement blocking therapies had not been in clinical use yet in Turkey, underwent kidney transplantation. One transplant patient experienced an acute rejection episode without graft loss. The remaining five patients had a glomerular filtration rate of more than 90 ml/min./1.73 m < sup > 2 < /sup > at the last visit. CONCLUSION: Although we had a relatively small patient population, our findings indicate that PT might still be considered in selected patients particularly in countries where complement blocking therapies are difficult to reach due to their unavailability or costs that are not covered by the health care systems.

Posterior reversible encephalopathy syndrome (PRES) due to acute hypertension in children: 12 years single-center experience.

The objective of the study is to evaluate the clinical and neuroradiological findings, the risk factors for recurrence and the prognosis in patients with posterior reversible encephalopathy syndrome developed secondary to acute hypertension in children. The study was conducted between 2008 and 2019 at Mersin University Faculty of Medicine. A total of 49 episodes were evaluated retrospectively in 38 patients with PRES secondary to acute hypertension. The demographic data, etiology, and clinical and neuroradiological findings were recorded. Twenty-one (55.3%) patients were female; the mean age was 11.8 years. The etiology of acute hypertension in 29 (76.3%) patients was end-stage renal disease (ESRD). The most common clinical findings were seizure (81.6%) and altered consciousness (79.6%). Status epilepticus developed in eight (16.3%) episodes. MRI lesions were atypical in 33 episodes (67.3%). In eight (21%) patients, PRES recurred. Irreversible brain damage was detected after PRES in three (7.8%) patients. C-reactive protein and erythrocyte sedimentation rate were elevated in 82.2% and 71.4% of the episodes, respectively. A statistically significant relationship was found between the recurrence, the duration of hospitalization at the PICU, SE and the occurrence of irreversible lesion (p = 0.013, p = 0.015, p = 0.001 respectively). Also, there were statistically significant relationships between recurrence and ESRD; epilepsy and recurrences; SE and irreversible brain damage (p = 0.02, p = 0.012, p = 0.025 respectively). Although PRES is usually known to have a good prognosis, the mortality and morbidity rates may increase in the long-term follow-up as in our study. In this study, the etiology, the presence of status epilepticus, PICU history, atypical MRI lesions and increased inflammatory markers were found to be important for the prognosis in PRES.

Evaluation of non-infectious complications of peritoneal dialysis in children: a multicenter study.

BACKGROUND: Peritoneal dialysis (PD) is the most common kidney replacement therapy in children. Complications associated with PD affect treatment success and sustainability. The aim of this study was to investigate the frequency of PD-related non-infectious complications and the predisposing factors. METHODS: Retrospective data from 11 centers in Turkey between 1998 and 2018 was collected. Non-infectious complications of peritoneal dialysis (NICPD), except metabolic ones, in pediatric patients with regular follow-up of at least 3 months were evaluated. RESULTS: A total of 275 patients were included. The median age at onset of PD and median duration of PD were 9.1 (IQR, 2.5-13.2) and 7.6 (IQR, 2.8-11.9) years, respectively. A total of 159 (57.8%) patients encountered 302 NICPD within the observation period of 862 patient-years. The most common NIPCD was catheter dysfunction (n = 71, 23.5%). At least one catheter revision was performed in 77 patients (28.0%). Longer PD duration and presence of swan neck tunnel were associated with the development of NICPD (OR 1.191; 95% CI 1.079-1.315, p = 0.001 and OR 1.580; 95% CI 0.660-0.883, p = 0.048, respectively). Peritoneal dialysis was discontinued in 145 patients; 46 of whom (16.7%) switched to hemodialysis. The frequency of patients who were transferred to hemodialysis due to NICPD was 15.2%. CONCLUSIONS: Peritoneal dialysis-related non-infectious complications may lead to discontinuation of therapy. Presence of swan neck tunnel and long duration of PD increased the rate of NICPD. Careful monitoring of patients is necessary to ensure that PD treatment can be maintained safely.

Multicystic Dysplastic Kidney and Incontinentia Pigmenti: Coexistence of 2 Rare Diseases.

Multicystic dysplastic kidney is a congenital kidney malformation consisting of multiple cysts of various sizes without a normal kidney morphology. Incontinentia pigmenti is a rare X-linked dominant genodermatosis, which is usually lethal in males, that presents clinically in 4 stages. Here, we report a case of multicystic dysplastic kidney with ureterovesical junction obstruction and incontinentia pigmenti. Coexistence of these two rare diseases may be a coincidental phenomenon or an association between the two may exist.

2D-Speckle tracking echocardiography contributes to early identification of impaired left ventricular myocardial function in children with chronic kidney disease.

IntroductionThe aim of this study is to determine early changes in cardiac function of children with chronic kidney disease by using 2D-speckle tracking echocardiography. METHOD: The study included 38 children - 16 girls and 22 boys - diagnosed as having chronic kidney disease in the nephrology department with a glomerular filtration rate of <90 ml/minute/1.73 m2 for at least 3 months. A total of 37 - 15 girls and 22 boys - age- and sex-matched healthy children were included as the control group. 2D-Speckle tracking echocardiography was performed in all subjects. RESULTS: The mean age was 13.45±2.8 years in patients and 12.89±3.07 years in controls. Systolic and diastolic blood pressures and left ventricular mass index were significantly higher in patients (p<0.05). The values of mitral e, mitral a, mitral e/a ratio, and mitral deceleration time were not different between the groups. Tricuspid annular plane systolic excursion values were lower in patients (p<0.01). Global strain values in apical long-axis 3-chamber and 2-chamber views were significantly lower in patients (p<0.05). Longitudinal, radial, and circumferential peak systolic strain values were lower in patients, but the difference was statistically significant in all segments of longitudinal view and basal segment of circumferential view (p<0.05). Radial and circumferential systolic strain rates were significantly lower in patients in all three segments (p<0.05). Moreover, early diastolic strain rate was significantly lower in longitudinal and radial apical segments and in all segments of circumferential measurements in patients. Besides, strain rate e/a ratio was significantly lower in all longitudinal segments of patients (p=0.01). CONCLUSION: The study concluded that 2D-speckle tracking echocardiography method can determine cardiac involvement earlier than conventional echocardiography in children with chronic kidney disease having preserved ejection fraction.

Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not?

Sürmeli-Döven S, Delibas A, Gürses I, Kayacan UR, Coskun-Yilmaz B, Esen K, Korkmaz E, Özaltin F. Hemolytic uremic syndrome and IgA nephropathy in a child: Coincidence or not? Turk J Pediatr 2018; 60: 81-85. A previously healthy 18-month old boy, presenting with diarrhea, anemia, thrombocytopenia and acute renal failure was admitted to our hospital. Hemolytic uremic syndrome (HUS) was diagnosed with his clinical and laboratory findings. His stool was negative for Shiga toxin producing E. coli (STEC). During follow-up he developed respiratory distress, hypertrophic cardiomyopathy and seizure. His genetic tests for atypical HUS (aHUS) were negative. His clinical and histological findings indicated hemolytic uremic syndrome and immunglobulin A nephropathy (IgAN). The patient responded to steroid treatment and plasma exchange therapy with peritoneal dialysis. We discuss the probable connection between HUS and IgAN.

Evaluation of T-wave alternans in pediatric patients with chronic renal failure.

INTRODUCTION: Microvolt T-wave alternans (TWA) is known to be useful in prediction of ischemia and sudden death in high-risk populations and there are no studies in children with chronic renal failure (CRF). Cardiac problems seem to be responsible for an important part of death in children and young adults with CRF. The aim of this study is to evaluate Holter microvolts TWA measurements in children with CRF comparing to the control group. METHODS: This prospective study included 40 patients with CRF and 48 healthy controls. The history, echocardiography and microvolt TWA values based on 24-hour ECG recordings of the patients were evaluated. Analysis of microvolt TWA was considered on the basis of three leads (V5, V1 and AVF). RESULTS: Compared with the controls, the mean systolic and diastolic blood pressure values and average heart rates were significantly higher in the children with CRF (p = 0.001 and p = 0.026, respectively). Also, the values of left ventricular internal dimensions at end diastole and end-diastolic volume were significantly higher in CRF group (p = 0.01 and p = 0.049, respectively) and couplet ventricular extrasystole was detected in 2 patients with CRF. Consequently, all TWA values in three leads were increased in CRF group than the control group but the only increase in V5 lead was statistically significant (p = 0.028). CONCLUSIONS: This study has demonstrated that microvolt TWA values increased in pediatric patients with CRF. TWA might be used for early risk assessment in pediatric patients with CRF in the future.

The impact of surgical intervention on renal function in cystinuria.

INTRODUCTION: Cystinuria is an autosomal recessive disorder due to intestinal and renal transport defects in cystine and dibasic amino acids, which result in recurrent urolithiasis and surgical interventions. This study aimed to assess the impact of surgical interventions on renal function by analyzing estimated glomerular filtration rates. METHODS: Thirteen pediatric patients with cystinuria, who were followed-up in a single tertiary institution between 2004 and 2016, were included in the study. Medical records were reviewed to collect data on clinical presentation of patients, urine parameters, stone formation, medical treatment, surgical intervention, stone recurrence after surgical procedure, stone analysis, ultrasonography, 99m-technetium dimercaptosuccinic acid (99mTc-DMSA) radionuclide imaging results, and follow-up time. Creatinine clearances estimated by modified Schwartz (eGFR) formula before and after surgery were used to assess renal function and compared statistically. RESULTS: Nine patients (69.2%) had renal scarring which were detected with 99mTc-DMSA radionuclide imaging. In ten patients (76.9%), open surgical intervention for stones were needed during follow-up. Significant difference was not detected between eGFR before and after surgical intervention (mean 92 versus 106, p = 0.36). Nine of the patients (69.2%) were stone free in the last ultrasonographic examination. Relapses of stone after surgery were seen in 66.6% of patients who underwent surgical intervention. CONCLUSIONS: Surgical interventions for urinary stones are commonly required in patients with cystinuria. Renal scarring is a prevalent finding in cystinuric patients. Surgical interventions have no negative impact on eGFR in patients with cystinuria according to the present study.

Diagnosis of Nephropathic Cystinosis in a Child During Routine Eye Exam.

We present a 7-year-old patient who was diagnosed with asymptomatic nephropathic cystinosis following the detection of the pathognomonic corneal white crystalline opacities during a routine eye examination.

Effective predictors for surgical decision in antenatal hydronephrosis: a prospective multiparameter analysis.

OBJECTIVE: The majority of antenatal hydronephrosis disappears spontaneously. In our study, we have investigated the effective predictors for surgical decision in antenatal hydronephrosis. MATERIAL AND METHODS: Forty-four patients found to have renal pelvic anteroposterior (AP) diameter more than 10-mm on antenatal ultrasonograpy were followed after birth. The study groups were divided into two groups: follow-up and surgery group. On follow-up, longitudinal diameter of the kidney, parenchymal thickness of the kidney, AP diameter of renal pelvis, AP diameter of middle calyces on ultrasonograpy, and differential renal function, 20th minute clearance, half-life of radionuclide tracer (T1/2), Tmax, Tmax-T1/2, normalised residual activity (NORA) on diuretic renography were evaluated. Ultrasonograpy and diuretic renography measurements were compared in patients whose hydronephrosis resolve or proceeded to surgery. RESULTS: Forty-four patients were diagnosed as antenatal hydronephrosis, spontaneous resolution occurred in 23 (52%), and surgery was performed in 21 patients (48%). Mean age at operation was 8.5±6.5 months (3-24 months). Mean AP diameter of renal pelvis was 29.5±14.2 mm for surgery group, 13.6±4.2 mm for follow-up group (p<0.001). Univariate analyses showed significance for all ultrasonographic measurements and only the differential renal function by diuretic renography. Multivariate logistic regression analysis showed significance for AP diameter of renal pelvis (odds ratio 1.37; 95% Cl 1.13-1.66), and differential renal function (odds ratio 1.14; 95% Cl 1.01-1.29). CONCLUSION: AP diameter of renal pelvis and differential renal function were the most effective parameters for surgical decision. These parameters can be used for appropriate management of antenatal hydronephrosis.

Short-cut diagnostic tool in cystinosis: Bone marrow aspiration.

BACKGROUND: Cystinosis is a rare metabolic genetic disorder caused by a mutation in cystinosin lysosomal cystine transporter (CTNS). The diagnosis of nephropathic cystinosis (NC) is made by observing corneal cystine crystals and/or measuring the cystine content of leukocytes. CTNS mutation analysis confirms the diagnosis of cystinosis, but leukocyte cystine measurement and CTNS analysis have not been widely available, and cystine crystals in the cornea may not be apparent in the first months of life. Cystine crystal deposition can be seen in the bone marrow earlier than corneal deposition, in patients with NC. METHODS: Ten patients with cystinosis diagnosis were enrolled in the study. Medical records were reviewed retrospectively to collect demographic and clinical data such as age at diagnosis, disease presentation, parental consanguinity, family history, corneal cystine deposition, leukocyte cystine level, bone marrow cystine deposition, presence of renal failure, follow-up time and prognosis. RESULTS: Cystine crystals were seen in all of the patients' fresh bone marrow aspiration samples. Eight patients had corneal cystine deposition. Leukocyte cystine measurement could have been performed in four patients who had come from another center. Complications such as pulmonary hypertension and idiopathic intracranial hypertension (IIH) were observed in two patients. CONCLUSIONS: Bone marrow aspiration might be an easy and short-cut diagnostic tool for NC especially when it is not possible to measure fibroblast cystine content. Additionally some rare complications such as pulmonary hypertension and IIH can be encountered during the course of NC.

The outcomes of two different bulking agents (dextranomer hyaluronic acid copolymer and polyacrylate-polyalcohol copolymer) in the treatment of primary vesico-ureteral reflux.

PURPOSE: Subureteral injection of bulking agents in the endoscopic treatment of vesicoureteral reflux is widely accepted therapy with high success rates. Although the grade of vesicoureteric reflux and experience of surgeon is the mainstay of this success, the characteristics of augmenting substances may have an effect particularly in the long term. In this retrospective study, we aimed to evaluate the clinical outcomes of the endoscopic treatment of vesicoureteric reflux (VUR) with two different bulking agents: Dextranomer/hyaluronic acid copolymer (Dx/HA) and Polyacrylate polyalcohol copolymer (PPC). MATERIALS AND METHODS: A total 80 patients (49 girls and 31 boys) aged 1-12 years (mean age 5.3 years) underwent endoscopic subureteral injection for correction of VUR last six years. The patients were assigned to two groups: subureteral injections of Dx/HA (45 patients and 57 ureters) and PPC (35 patients and 45 ureters). VUR was grade II in 27 ureters, grade III in 35, grade IV in 22 and grade V in 18 ureters. RESULTS: VUR was resolved in 38 (66.6%) of 57 ureters and this equates to VUR correction in 33 (73.3%) of the 45 patients in Dx/HA group. In PPC group, overall success rate was 88.8% (of 40 in 45 ureters). Thus, Thus, this equates to VUR correction in 31 (88.5%) of the 35 patients. CONCLUSIONS: Our short term data show that two different bulking agent injections provide a high level of reflux resolution and this study revealed that success rate of PPC was significantly higher than Dx/HA with less material.