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.

Detrusor Pressures Change with Repeat Filling Cystometry in Myelodysplastic Children with Neurogenic Lower Urinary Tract Dysfunction.

PURPOSE: The International Children's Continence Society recommends urodynamics repeated at least twice for the optimal result. We aimed to search if 3 times repeat filling urodynamics in the same session would change the urodynamics parameters in children with neurogenic lower urinary tract dysfunction due to myelodysplasia. MATERIALS AND METHODS: We investigated urodynamic reports and charts of 80 consecutive children with neurogenic lower urinary tract dysfunction due to myelodysplasia who underwent 3 repeat, same session filling cystometry studies between June 2017 and December 2018. Maximum detrusor pressure, maximum cystometric capacity, detrusor leak point pressure, compliance, residual urine volume and maximum cystometric capacity/estimated bladder capacity for age ratio were compared among all 3 filling cystometries. RESULTS: The median age was 4.3 years (IQR 5.8). Of the patients 39 (48.75%) were girls and 41 (51.25%) were boys. Primary pathological finding was myelomeningocele in 69 patients (86.3%). Maximum cystometric capacity, compliance, residual urine and maximum cystometric capacity/estimated bladder capacity for age were found comparable in 3 repeat cystometries. However, maximum detrusor pressure at first filling was higher compared to others (second, p=0.015, and third, p=0.002). Detrusor leak point pressure at the first filling was also higher compared to the third (p <0.001). Detrusor overactivity was persistent in all 3 fillings in 85% of patients (68 of 80). Of the patients 68 (85%) remained in the same risk group according to detrusor leak point pressure (cutoff 40 cmH2O). CONCLUSIONS: Maximum cystometric capacity and detrusor overactivity are comparable in 3 repeat cystometries but detrusor pressures significantly decrease in the repeat fillings. In our practice we plan our management according to the most worrisome urodynamics parameters for a safer proactive approach.

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.

Eosinophilic tubulointerstitial nephritis on treatment with isotretinoin.

Drug-related acute tubulointerstitial nephritis is one of the most common causes of childhood acute renal failures which originate from kidneys. Sixteen-year old male patient with the history of isotretinoin use for the last 3 months was admitted with acute renal failure. Renal function parameters were measured as follows: blood urea nitrogen 21 mg/dL, serum creatinine 1.68 mg/dL, cystatin C 1.15 mg/L, and estimated glomerular filtration rate based on cystatin C 56.5 mL/min/1.73 m2. The patient whom pathological signs of renal biopsy sections revealed interstitial mononuclear cell and eosinophilic infiltration was diagnosed with acute tubulointerstitial nephritis. CONCLUSION: Isotretinoin is a vitamin A-derived agent which is commonly used in the treatment of acne and may cause drug-related acute tubulointerstitial nephritis. What is Known: •Drug-related acute tubulointerstitial nephritis (ATIN) is one of the most common causes of childhood acute renal failures. What is New: •Isotretinoin may cause drug-related acute tubulointerstitial nephritis.

Use of Eculizumab in Pediatric Patients with Late Antibody-Mediated Rejection After Kidney Transplantation.

Late antibody-mediated rejection triggered by donor-specific antibodies is a leading cause of kidney allograft failure. Effective treatment options for late antibody-mediated rejection are limited in renal transplant recipients. Here, we report 2 pediatric cases of severe late antibody-mediated rejection resistant to conventional immunosuppressive therapy who were successfully treated with eculizumab. Two patients who fulfilled the late antibody-mediated rejection diagnostic criteria (positive donor-specific antibodies, elevated mean fluorescence index, acute and/or chronic morphological lesions in the microvasculature, and abnormal kidney function test) were included in this study. Both patients were previously unsensitized with negative panel-reactive antibody. Case 1 was a 12-year-old male patient with kidney failure secondary to vesicoureteral reflux who underwent related-living donor kidney transplantation 2 years ago. Eleven months later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After the patient was treated with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 3.8 to 1.5 mg/dL. Case 2 was an unsensitized 16-year-old male patient with kidney failure secondary posterior urethral valve who underwent related-living donor kidney transplantation 4 years ago. Two years later, he was diagnosed with late antibody-mediated rejection. Despite an aggressive conventional immunosuppressive regimen, signs of rejection persisted. After treatment with 2 doses of eculizumab, his mean fluorescence index dropped and serum creatinine decreased from 2.1 to 1.01 mg/dL. In both patients, eculizumab therapy effectively reduced the markers of late antibody-mediated rejection and improved the kidney function.

Bilateral Native Kidney Papillary Renal Cell Carcinomas in a 11-Year-Old Renal Transplant Patient.

Renal cell carcinomas (RCCs) are the most common renal tumors in adults and are usually sporadic and unilateral. Renal transplant recipients have an increased risk of developing RCC. RCC development after kidney transplantation is very rarely reported in children. We present a 11-year-old boy who had cadaveric kidney transplantation for kidney failure 2 years ago. He was under immunosuppressive therapy and presented with microscopic hematuria. An ultrasound (US) revealed bilateral solid renal masses. Further cross-sectional imaging showed a 60 × 70 × 60-mm right renal mass with claw sign and a 5 × 6 × 6-mm mass in the left renal lower pole. A bilateral radical nephroureterectomy of native kidneys was performed. The pathology revealed bilateral papillary RCC without TFE3 upregulation. The patient was kept on low-dose immunosuppressive therapy in the perioperative period. He received no chemotherapy but a close radiological surveillance was undertaken. He is tumor-free 2 years after the operation. RCC is a rare tumor for children and bilateralism is even rarer. The child had a history of chronic kidney disease, peritoneal dialysis, and immunosuppressive therapy. As there are no standardized protocols regarding imaging in transplanted kidneys routine surveillance, US follow-up should also focus on detecting malignancy.

Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35 months (0.4-231 months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3 mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12 months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year's follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF ( - ) stones than in MRF ( +) stones. However, remission rate with medical treatment was higher in cases with MRF ( +) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.

Graft Survival in Patients With Lower Urinary Tract Dysfunction.

OBJECTIVES: We aimed to the determine urinary tract infection and graft survival rates in pediatric renal transplant recipients with lower urinary tract dysfunction with particular focus on neurogenic bladder, posterior urethral valve, and vesicoureteral reflux nephropathy. MATERIALS AND METHODS: Patients were grouped according to primary diseases as those with and without lower urinary tract dysfunction. Urinary tract infections during year 1 posttransplant were investigated. Estimated glomerular filtration rate was calculated using Schwartz formula. RESULTS: Our study investigated 133 kidney transplant recipients. Lower urinary tract dysfunction was found in 58 patients (43.6%): 25 with posterior urethral valve, 24 with vesicoureteral reflux nephropathy, and 9 with neurogenic bladder. Rates of posttransplant urinary tract infection were higher in patients with lower urinary tract dysfunction than in those without during both the first 6 months posttransplant (24.6% vs 10.8%; P = .037) and between posttransplant months 6 and 12 (24.6% vs 8.2%; P = .01). Patients with neurogenic bladder had the highest rate of urinary tract infections, and their estimated glomerular filtrations rates were lower compared with patients with posterior urethral valve and vesicoureteral reflux nephropathy at month 6 and years 1, 2, and 5 posttransplant (P < .001). The 5-year graft survival rates of patients without lower urinary tract dysfunction and those with vesicoureteral reflux nephropathy were similar (51.3% vs 51.6%; P = .891). CONCLUSIONS: Graft survival rates of patients with posterior urethral valve and vesicoureteral reflux nephropathy were similar to those shown in patients without lower urinary system dysfunction; however, patients with neurogenic bladder had worse graft survival and urinary tract infection rates.