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.

Urine soluble TLR4 levels may contribute to predict urinary tract infection in children: the UTILISE Study.

BACKGROUND: One of the most common bacterial infections in childhood is urinary tract infection (UTI). Toll-like receptors (TLRs) contribute to immune response against UTI recognizing specific pathogenic agents. Our aim was to determine whether soluble TLR4 (sTLR4), soluble TLR5 (sTLR5) and interleukin 8 (IL-8) can be used as biomarkers to diagnose UTI. We also aimed to reveal the relationship between urine Heat Shock Protein 70 (uHSP70) and those biomarkers investigated in this study. METHODS: A total of 802 children from 37 centers participated in the study. The participants (n = 282) who did not meet the inclusion criteria were excluded from the study. The remaining 520 children, including 191 patients with UTI, 178 patients with non-UTI infections, 50 children with contaminated urine samples, 26 participants with asymptomatic bacteriuria and 75 healthy controls were included in the study. Urine and serum levels of sTLR4, sTLR5 and IL-8 were measured at presentation in all patients and after antibiotic treatment in patients with UTI. RESULTS: Urine sTLR4 was higher in the UTI group than in the other groups. UTI may be predicted using 1.28 ng/mL as cut-off for urine sTLR4 with 68% sensitivity and 65% specificity (AUC = 0.682). In the UTI group, urine sTLR4 levels were significantly higher in pyelonephritis than in cystitis (p < 0.0001). Post-treatment urine sTLR4 levels in the UTI group were significantly lower than pre-treatment values (p < 0.0001). CONCLUSIONS: Urine sTLR4 may be used as a useful biomarker in predicting UTI and subsequent pyelonephritis in children with UTI. A higher resolution version of the Graphical abstract is available as Supplementary information.

The nailfold dermoscopy findings of patients with atypical haemolytic uremic syndrome.

INTRODUCTION: Hemolytic uremic syndrome (HUS) is a severe disease characterized by microangiopathic anemia, thrombocytopenia, and acute renal failure. OBJECTIVES: Atypical HUS (aHUS) that results due to genetic disorders of the alternative complement pathway results in inflammation, endothelial damage, and kidney injury. Therefore, simple and non-invasive tests are needed to evaluate the activity of the disease by assessing the microvascular structure in aHUS. METHODS: A dermoscope (×10) is an inexpensive and easily portable device used to visualize nailfold capillaries and has high clinical performance and interobserver reliability. In this study, the nailfold capillaries of patients with aHUS who were in remission under eculizumab treatment were examined, and the findings were compared to those of a healthy control group to evaluate disease characteristics. RESULTS: All children with aHUS had decreased capillary densities even if they were in remission. This may be indicative of ongoing inflammation and microvascular damage in aHUS. CONCLUSION: A dermoscopy can be used as a screening tool for disease activity in patients with aHUS.

The ureteral diameter ratio as a predictive factor in renal scarring associated with primary vesicoureteral reflux.

INTRODUCTION: The ureteral diameter ratio (UDR) is reported to be effective in predicting the outcomes of vesicoureteral reflux (VUR) in several studies. OBJECTIVE: The objective of the current study was to compare the risk of scarring in patients with VUR relative to UDR and the VUR grade. We also aimed to demonstrate other associated risk factors in scarring and investigate the long-term complications of VUR and their relationship with UDR. STUDY DESIGN: Patients diagnosed with primary VUR were retrospectively enrolled in the study. UDR was calculated by dividing the largest ureteral diameter (UD) by the distance between L1-L3 vertebral bodies. Demographic and clinical data, laterality, VUR grade, UDR, delayed upper tract drainage on voiding cystourethrogram, recurrent urinary tract infections (UTI), and long-term complications of VUR were compared between the patients with and without renal scars. RESULTS: A total of 127 patients and 177 renal units were included in the study. There was a significant difference between the patients with and without renal scars according to age at diagnosis, bilaterality, reflux grade, UDR, recurrent UTI, bladder bowel dysfunction, hypertension, decreased estimated glomerular filtration rate, and proteinuria. The logistic regression analysis revealed that UDR had the highest odds ratio among the factors affecting scarring in VUR. DISCUSSION: VUR grading based on the evaluation of the upper urinary tract is one of the most important predictors for treatment options and prognosis. However, it is more likely to reflect ureterovesical junctional anatomy and function, which play a crucial role in the pathogenesis of VUR. CONCLUSION: UDR measurement seems to be an objective method that can help clinicians predict renal scarring in patients with primary VUR.

Follow up of renal outcomes in children with solitary kidney.

BACKGROUND: Solitary kidney (SK) affects 1/1000 people worldwide, and there are controversies concerning renal outcomes in these patients. This study aimed to investigate clinical findings and renal outcomes in children with SK and to compare the results for congenital (CSK) and acquired SK (ASK) groups. METHODS: The study included patients that presented to our pediatric nephrology department with SK between January 2010 and January 2021. Demographic and clinical data were recorded retrospectively. RESULTS: Of the 101 patients with SK, 71 had CSK (55 had unilateral renal agenesis and 16 had a multicystic dysplastic kidney) and 30 had ASK (17 had previously undergone unilateral nephrectomy due to a renal tumor and 13 had urological structural anomalies). There were nine patients (9%) with renal injury. The serum uric acid level was significantly higher and the estimated glomerular filtration rate was significantly lower in the patients with ASK compared with those with CSK (p = 0.005 and p < 0.001, respectively). There was a positive correlation between renal injury and the uric acid level (p < 0.001, r = 0.45). CONCLUSION: In addition to the management of blood pressure and proteinuria, it is important to control uric acid levels in patients with SK, especially those with ASK, to prevent renal injury. The ASK group has a greater risk of renal injury than the CSK group. There is a need for new markers to predict early stage renal damage in SK.

Urinary HSP70 improves diagnostic accuracy for urinary tract infection in children: UTILISE study.

BACKGROUND: The accuracy of conventional urinalysis in diagnosing urinary tract infection (UTI) in children is limited, leading to unnecessary antibiotic exposure in a large fraction of patients. Urinary heat shock protein 70 (uHSP70) is a novel marker of acute urinary tract inflammation. We explored the added value of uHSP70 in discriminating UTI from other infections and conditions confused with UTI. METHODS: A total of 802 children from 37 pediatric centers in seven countries participated in the study. Patients diagnosed with UTI (n = 191), non-UTI infections (n = 178), contaminated urine samples (n = 50), asymptomatic bacteriuria (n = 26), and healthy controls (n = 75) were enrolled. Urine and serum levels of HSP70 were measured at presentation in all patients and after resolution of the infection in patients with confirmed UTI. RESULTS: Urinary (u)HSP70 was selectively elevated in children with UTI as compared to all other conditions (p < 0.0001). uHSP70 predicted UTI with 89% sensitivity and 82% specificity (AUC = 0.934). Among the 265 patients with suspected UTI, the uHSP70 > 48 ng/mL criterion identified the 172 children with subsequently confirmed UTI with 90% sensitivity and 82% specificity (AUC = 0.862), exceeding the individual diagnostic accuracy of leukocyturia, nitrite, and leukocyte esterase positivity. uHSP70 had completely normalized by the end of antibiotic therapy in the UTI patients. Serum HSP70 was not predictive. CONCLUSIONS: Urine HSP70 is a novel non-invasive marker of UTI that improves the diagnostic accuracy of conventional urinalysis. We estimate that rapid urine HSP70 screening could spare empiric antibiotic administration in up to 80% of children with suspected UTI. A higher resolution version of the Graphical abstract is available as Supplementary information.

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.

YouTube as a source of information on COVID-19 and dialysis.

INTRODUCTION: The coronavirus 2019 pandemic has become a serious threat to the global health and spreading rapidly. Dialysis patients may have developed anxiety because of their disease characteristics during this pandemic. The aim of this study was to assess the reliability and quality of the most viewed English-language YouTube videos on COVID-19 and dialysis link. METHODS: A YouTube search was achieved using the key words "COVID-19 dialysis," "SARS CoV-2 dialysis," "coronavirus hemodialysis," and "COVID peritoneal dialysis." RESULTS: Among the 43 videos analyzed, 24 (55.8%) were high quality, 11 (25.6%) were moderate quality, and 8 (18.6%) were of low quality according to global quality score. Significant differences were found between the groups in modified DISCERN scores and the number of views, likes, comments. CONCLUSION: The most reliable sources such as universities, governments, professional societies should better use YouTube for dissemination of accurate, reliable, and useful health-related information to the general public.

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.

COVID-19 in pediatric nephrology centers in Turkey.

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.

Fibrinogen-to-Albumin Ratio in Familial Mediterranean Fever: Association with Subclinical Inflammation.

BACKGROUND: Familial Mediterranean fever (FMF) is the most seen monogenic periodic fever syndrome characterised by bouts of fever and serositis. It is known that subclinical inflammation (SI) can persist in the symptom-free period and lead to amyloidosis even under colchicine treatment. This study aimed to evaluate the role of the fibrinogen-to-albumin ratio (FAR) in FMF and its correlation with SI. MATERIAL AND METHODS: A total of 112 patients with FMF and 78 controls were enrolled in this retrospective study. Demographic, laboratory and genetic data were obtained from the hospital records. RESULTS: The FAR values of the FMF cases were significantly higher than the control group (p<0.001). In the FMF group, the patients with SI had higher FAR values than those without SI (p<0.001). FAR was positively correlated with SI (r=0.413, p<0.001). The receiver operating characteristic curve analysis showed that FAR had a higher area under the curve value than albumin and fibrinogen. CONCLUSION: Detecting SI in patients with FMF is crucial in preventing amyloidosis, the most devastating complication of FMF. FAR is a simple, inexpensive, easily obtained indicator which can be used for reflecting SI in FMF. HINTERGRUND: Familiäres Mittelmeerfieber (FMF) ist das am häufigsten auftretende monogene periodische Fiebersyndrom, das durch Fieberschübe und Serositis gekennzeichnet wird. Die subklinische Entzündung (SI) kann bekanntlich auch in der symptomfreien Phase fortbestehen und zu Amyloidose führen, selbst unter Behandlung mit Colchicin. Ziel dieser Studie ist es, die Beziehung zwischen Fibrinogen und Albumin (AFR) bei FMF und deren Korrelation mit SI zu untersuchen. MATERIAL UND METHODEN: Insgesamt wurden für diese retrospektive Studie 112 Patienten mit FMF und 78 Kontrollpatienten eingeschlossen. Die demographischen, labortechnischen und genetischen Daten wurden aus den Krankenhausunterlagen entnommen. ERGEBNISSE: Die Werte von AFR bei Patienten mit FMF waren signifikant höher als die der Kontrollgruppe (<0,001). Patienten mit FMF mit SI zeigten höhere AFR-Werte als FMF-Patienten ohne SI (<0,001). AFR korrelierte positiv mit SI (r=0,413, p<0,001). Die Analyse der ROC-Kurve (Receiver Operating Characteristic) zeigte, dass AFR einen höheren Wert der Fläche unter der Kurve (AUC) hatte als Albumin und Fibrinogen. DISKUSSION: Die Erkennung von SI bei FMF-Patienten ist entscheidend für die Vermeidung von Amyloidose, der schlimmsten Komplikation von FMF. AFR ist ein einfacher, kostengünstiger, leicht durchzuführender Indikator, der in FMF zur Spiegelung von SI verwendet werden kann.

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.

Urinary NGAL is a Potential Biomarker for Early Renal Injury in Insulin Resistant Obese Non-diabetic Children

Objective: Neutrophil gelatinase-associated lipocalin (NGAL) is one of the new biomarkers for detecting acute renal injury. There are studies showing the relationship between NGAL and renal injury in obese children. The aim of this study was to investigate whether urinary levels of NGAL, kidney injury molecule-1, and serum cystatin C are increased in insulin resistance (IR) patients before the development of diabetes. Methods: Cross-sectional, case-controlled study that included non-diabetic obese children and adolescent patients with IR and a non-diabetic obese control group with no IR, who attended a tertiary center pediatric endocrinology outpatient clinic between 2016-2018. Those with diabetes mellitus and/or known renal disease were excluded. NGAL and creatinine (Cr) levels were evaluated in the morning spot urine from all participants. Serum renal function was evaluated. Results: Thirty-six control and 63 IR patients were included in the study, of whom 68 (68.7%) were girls. The mean age of all participants was 13.12±2.64 years and no statistically significant difference was found between the two groups in terms of age or gender distribution. Median (range) spot urinary NGAL (u-NGAL) values in the IR group were significantly higher at 26.35 (7.01-108.7) ng/mL than in the control group at 19.5 (3.45-88.14) ng/mL (p=0.018). NGAL/Cr ratio was also significantly higher in the IR group compared to the control group (p=0.018). Conclusion: Obese pediatric patients with IR were shown to have elevated levels of u-NGAL, a marker of renal injury. u-NGAL examination may show early renal injury before development of diabetes.

Indoxyl sulfate associates with cardiovascular phenotype in children with chronic kidney disease.

BACKGROUND: Cardiovascular disease is the leading cause of death in children with chronic kidney disease (CKD). Serum levels of gut-derived uremic toxins increase with deterioration of kidney function and are associated with cardiac comorbidities in adult CKD patients. METHODS: Indoxyl sulfate (IS) and p-cresyl sulfate (pCS) were measured by high-performance liquid chromatography in serum of children participating in the Cardiovascular Comorbidity in Children with CKD (4C) Study. Results were correlated with measurements of the carotid intima-media thickness (cIMT), central pulse wave velocity (PWV), and left ventricular mass index (LVMI) in children aged 6-17 years with initial eGFR of 10-60 ml/min per 1.73 m2. RESULTS: The median serum levels of total IS and of pCS, measured in 609 patients, were 5.3 µmol/l (8.7) and 17.0 µmol/l (21.6), respectively. In a multivariable regression model, IS and pCS showed significant positive associations with urea and negative associations with eGFR and uric acid. Furthermore, positive associations of pCS with age, serum albumin, and non-Mediterranean residency and a negative association with glomerular disease were observed. By multivariable regression analysis, only IS was significantly associated with a higher cIMT SDS at baseline and progression of PWV SDS within 12 months, independent of other risk factors. CONCLUSIONS: Serum levels of gut-derived uremic toxins IS and pCS correlated inversely with eGFR in children. Only IS was significantly associated with surrogate markers of cardiovascular disease in this large pediatric CKD cohort.

Low levels of urinary epidermal growth factor predict chronic kidney disease progression in children.

Urinary epidermal growth factor (uEGF) has recently been identified as a promising biomarker of chronic kidney disease (CKD) progression in adults with glomerular disease. Low levels of uEGF predict CKD progression and appear to reflect the extent of tubulointerstitial damage. We investigated the relevance of uEGF in pediatric CKD. We performed a post hoc analysis of the Cardiovascular Comorbidity in Children with CKD (4C) study, which prospectively follows children aged 6-17 years with baseline estimated glomerular filtration rate (eGFR) of 10-60 ml/min/1.73 m2. uEGF levels were measured in archived urine collected within 6 months of enrollment. Congenital abnormalities of the kidney and urinary tract were the most common cause of CKD, with glomerular diseases accounting for <10% of cases. Median eGFR at baseline was 28 ml/min/1.73 m2, and 288 of 623 participants (46.3%) reached the composite endpoint of CKD progression (50% eGFR loss, eGFR < 10 ml/min/1.73 m2, or initiation of renal replacement therapy). In a Cox proportional hazards model, higher uEGF/Cr was associated with a decreased risk of CKD progression (HR 0.76; 95% CI 0.69-0.84) independent of age, sex, baseline eGFR, primary kidney disease, proteinuria, and systolic blood pressure. The addition of uEGF/Cr to a model containing these variables resulted in a significant improvement in C-statistics, indicating better prediction of the 1-, 2- and 3-year risk of CKD progression. External validation in a prospective cohort of 222 children with CKD demonstrated comparable results. Thus, uEGF may be a useful biomarker to predict CKD progression in children with CKD.

Henoch-Schonlein purpura, post-streptococcal glomerulonephritis and acute rheumatic carditis after Group A ß-haemolytic streptococcal infection.

Besides association with acute rheumatic fever (ARF) and acute glomerulonephritis (APSGN), in up to 40% of cases, Group A ß-haemolytic streptococcal (GABHS) infections are also implicated as a trigger for Henoch-Schonlein purpura (HSP). A 7-year-old girl with GABHS throat infection who developed HSP, APSGN and rheumatic carditis is reported. She presented with palpable purpura and arthritis in both ankles and later developed carditis characterised by mitral/aortic regurgitation and glomerulonephritis characterised by mixed nephritic/nephrotic syndrome. She had a raised anti-streptolysin titre (ASOT), blood urea nitrogen and creatinine and hypocomplementaemia (C3), and renal biopsy demonstrated endocapillary and extracapillary proliferative glomerulonephritis with crescents. Immunofluorescence microscopy demonstrated a 'full house' of immunoglobulin and complement, viz. IgA + 2, IgG + 3, IgM + 2, C3c + 1, Clq + 2 with predominantly IgG deposition. One week earlier, her 4-year-old sister had presented to another hospital with HSP complicated by microscopic haematuria, nephrotic-range proteinuria and gastro-intestinal involvement, and with raised ASOT and low C3 levels. Although HSP has been associated with either ARF or APSGN, this is the first case of a child with HSP, ARF and APSGN in combination.

Evaluation of abnormal radiological findings in children aged 2 to 36 months followed by recurrent urinary tract infection: a retrospective study.

Our aim is to determine the rational usage of imaging techniques in order to prevent or minimize permanent renal damage in recurrent urinary tract infections (UTIs). This study was enrolled children aged between 2 and 36 months, following-up with the diagnosis of recurrent UTI. All children had ultrasonography (USG) and dimercaptosuccinic acid scanning, 39 of them had underwent on voiding cystourethrography. There were 133 children (87 girls, 46 boys) with the mean age of 32.82 ± 38.10 months included into the study. Forty-three kidney units were normal in ultrasonogram of which seven units had reflux whereas among 35 units with hydronephrosis 22 units had reflux. Sensitivity and specificity presence of hydronephrosis in ultrasonogram for prediction of reflux was 75.9% and 73.5%, respectively. There were 19 dilated ureters in ultrasonogram, and among them 14 had reflux. Sensitivity and specificity of presence with ureteral dilatation in ultrasonogram for prediction of reflux was found as 48.3% and 89.8%, respectively. The sensitivity of parenchymal thinning seen in ultrasonogram for the evaluation of renal parenchyma was 15.9%, whereas specificity was 98.2% .Sensitivity and specificity of dimercaptosuccinic acid for prediction of reflux was 51.6% and 72.3%, respectively. The normal ultrasonogram findings cannot rule out neither possibility of reflux presence nor development of renal scarring. Therefore, DMSA scanning has major role both in determination of parenchymal damage and prevention of scarring. Also we get an important result as ureteral dilatation seen in USG, related to presence of reflux.

Genetic screening in adolescents with steroid-resistant nephrotic syndrome.

Genetic screening paradigms for congenital and infantile nephrotic syndrome are well established; however, screening in adolescents has received only minor attention. To help rectify this, we analyzed an unselected adolescent cohort of the international PodoNet registry to develop a rational screening approach based on 227 patients with nonsyndromic steroid-resistant nephrotic syndrome aged 10-20 years. Of these, 21% had a positive family history. Autosomal dominant cases were screened for WT1, TRPC6, ACTN4, and INF2 mutations. All other patients had the NPHS2 gene screened, and WT1 was tested in sporadic cases. In addition, 40 sporadic cases had the entire coding region of INF2 tested. Of the autosomal recessive and the sporadic cases, 13 and 6%, respectively, were found to have podocin-associated nephrotic syndrome, and 56% of them were compound heterozygous for the nonneutral p.R229Q polymorphism. Four percent of the sporadic and 10% of the autosomal dominant cases had a mutation in WT1. Pathogenic INF2 mutations were found in 20% of the dominant but none of the sporadic cases. In a large cohort of adolescents including both familial and sporadic disease, NPHS2 mutations explained about 7% and WT1 4% of cases, whereas INF2 proved relevant only in autosomal dominant familial disease. Thus, screening of the entire coding sequence of NPHS2 and exons 8-9 of WT1 appears to be the most rational and cost-effective screening approach in sporadic juvenile steroid-resistant nephrotic syndrome.