We present eight cases of the homozygous MCPggaac haplotype, which is considered to increase the likelihood and severity of atypical hemolytic uremic syndrome (aHUS), especially in combination with additional risk aHUS mutations. Complement blockade (CBT) was applied at a median age of 92 months (IQR 36–252 months). The median number of relapses before CBT initiation (Eculizumab) was two. Relapses occurred within an average of 22.16 months (median 17.5, minimum 8 months, and maximum 48 months) from the first subsequent onset of the disease (6/8 patients). All cases were treated with PI/PEX, and rarely with renal replacement therapy (RRT). When complement blockade was applied, children had no further disease relapses. Children with MCPggaac haplotype with/without additional gene mutations can achieve remission through renal replacement therapy without an immediate need for complement blockade. If relapse of aHUS occurs soon after disease onset or relapses are repeated frequently, a permanent complement blockade is required. However, the duration of such a blockade remains uncertain. If complement inhibition is not applied within 4–5 relapses, proteinuria and chronic renal failure will eventually occur.

Aim To determine an outcome of acute kidney injury (AKI) in critically ill children (CIC) who needed renal replacement therapy (RRT) and were admitted to the Paediatric and Neonatal Intensive Care Unit (PICU and NICU) at the Paediatric Clinic, University Clinical Centre Sarajevo (UCCS). Methods The research included 81 children with AKI. The Kidney Disease: Improving Global Outcomes (KDIGO) criteria to define AKI was used. Other laboratory findings and imaging tests were made depending on children's primary disease that led to the AKI. Results Among 81 children with AKI, 38 were girls and 43 boys. A total of 39 (48.1%) patients died; the death was due to the nature of the primary disease and multiple organ failure syndromes. Out of the total of 81 patients the highest mortality rate was found in children in the first year of life, 22 (56.4%), while 17 (43.6%) patients died after the first year of life. Conclusion Without an accurate diagnosis at the right time, due to the lack of adequate biomarkers for AKI screening, the heterogeneity of AKI, comorbidities often lead to unfavourable outcomes of the disease, among CIC, especially in infants with low birth weight and extreme immaturity. Some causes of AKI are preventable and can be reduced by a better organization of primary and secondary health care.

Introduction:The diagnosis of acute kidney injury (AKI) in preterm newborns with perinatal asphyxia based on increased serum creatinine (sCr) value and oliguria/anuria is usually delayed. The Aim of this paper is to evaluate serum cystatin C as an early predictor of AKI. Materials and methods:The study included 42 preterm newborns (24-37 weeks) with perinatal asphyxia (Apgar score (AS) ≤ 3 at 5 minutes of life or blood pH on admission ≤ 7.00). The sCr and sCysC levels were measured on the 1st, 3rd, and 7th day of life. According to KDIGO criteria, the newborns were classified into groups, and sCr and sCys-C values were compared. Results:The mean gestational age was 29.9 ± 3.0 weeks. AKI was diagnosed in 62.8 % of patients. Of these patients, 81.5% belonged to AKI 1 group, and 18.5 % to AKI 2 group.No newborns had the criteria for AKI 3. On day 7 the mean sCr values were significantly higher in AKI (65.4± 21.8) compared with the non-AKI group (168.4±38.2) (p<0.001), but not on day 1 and 3 (p = 0.322, 0.012, respectively). The sCys-C values were significantly higher in the AKI group on day 3 ( AKI vs. non-AKI group, 0.69 ±0.22 vs. 1.22 ±0.20; p <0.001) and day 7 (AKI vs. non-AKI group, 0.62 ±0.41 vs. 1.68 ±0.20; p <0.001). The sCys-C was also an earlier marker of a more severe stage of AKI than sCr. Conclusion:The sCys-C was elevated earlier than sCr, making it a valuable diagnostic tool for AKI in preterm newborns.

Introduction: Brain MRI scans can predict neurodevelopmental outcomes in neonates treated with therapeutic hypothermia. It is a common clinical practice to perform brain MRI before discharge, but brain MRI scans performed at around four months of age have a better prognostic value for a long-term neurological outcome in asphyxiated neonates. Aim: To identify which of three selected clinical parameters (oral feeding ability, muscle tone, history of seizure) evaluated 10 days after therapeutic hypothermia could predict the primary outcome of an abnormal brain MRI. Methods: We reviewed the medical records of neonates ≥ 36 completed weeks of gestation consecutively treated with therapeutic hypothermia who underwent brain MRI. Clinical parameters on day 10 after therapeutic hypothermia were correlated with brain MRI findings in the first 7-14 days of life. Logic regression analysis was performed using all three covariates of the clinical status, with an abnormal MRI as the primary outcome. Results: Brain MRI was abnormal in 42 (51.85 %) neonates with the following distribution of brain injury patterns: abnormal signal in the basal nuclei in 6, an abnormal signal in the cortex in 16, an abnormal signal both in the cortex and basal nuclei in 20 neonates. Out of three analyzed clinical parameters, feeding difficulty (P < 0.001, OR 8.3, 95% CI 2.9 - 28.9) and a history of seizures (P < 0.001, OR 11.95, 95% CI 3 - 44.5) were significantly associated with an abnormal MRI.  Conclusion: Neonates who were capable of full oral feeding by day 10 after therapeutic hypothermia and had no history of seizures were unlikely to have an abnormal MRI. This may be used in selective planning of pre-discharge MRI in asphyxiated neonates.

Background: Acute kidney injury (AKI) is the result of various causes and is associated with significant morbidity and mortality as well as long-term renal sequelae in pediatric patients. Objectives: The aim of the study is to determine the causes of AKI in pediatric patients who needed renal replacement therapy (RRT) and were admitted to the Pediatric and Neonatal Intensive Care Unit (PICU and NICU) at the Pediatric Clinic, University Clinical Center Sarajevo (UCCS). Methods: Our research included 81 children with AKI who needed RRT. We used the Kidney Disease: Improving Global Outcomes (KDIGO) criteria to define AKI. Severe acute kidney injury was defined as stage 2 or 3 of AKI when plasma creatinine level ≥2 times the baseline level or urine output <0.5 ml per kilogram of body weight per hour for ≥12 hours. Other laboratory findings and imaging tests were made depending on their primary disease that led to the AKI and its complications. Results: Our research analyzed 81 children with AKI who needed RRT 38 girls and 43 boys ages from birth to 18 years. Mean age of presentation was 6.28 years. Male female ratio in this study was 1.1:1. Non-olyguric AKI was diagnosed in 12 (14.8%) of children with AKI, while the rest 69 (85.2%) had the olyguric type. Patients with AKI were analyzed after a rough division on prerenal in 57 (70.4%) children, intrarenal in 23 (28.4%) and post-renal in 1 (1.2%) patient. Conclusion: As the AKI plays a key role in the mortality and morbidity in pediatric patients, especially in infants, it is important to recognise and treatment on time different etiologies of this serious condition. Some causes of AKI in our country can be prevented by better organization of primary and secondary health care, which would also reduce mortality and morbidity from AKI.

Background: Respiratory distress syndrome (RDS) in premature neonates has high morbidity, and it is encountered as one of the most common pathological conditions in these patients. This prospective study included 51 patients with a mean gestational age of 31 gestational weeks, hospitalized in the neonatal intensive care unit (NICU) in a tertiary-level university hospital. Aims and Objectives: The aim of our study is a comparison between lung ultrasound (US) and chest radiography in diagnosing RDS in premature neonates. Materials and Methods: US findings were classified into three profile scales and X-ray findings into a four-grade radiographic scale. Results: The results show a good concordance between chest radiography and lung US with kappa, sensitivity, and negative predictive value results in favor of US. Most of the patients had US-profile 1, which corresponds to X-ray profiles 3 and 4. US finding included the presence of confluent B lines, “white lungs,” subpleural consolidations, and thick pleura, but also A-lines, mostly found in a resolution of the disease and mild cases. Conclusion: Lung US still is not a routine procedure despite its wide use in NICUs, and as non-harmful, repeatable method, it can be used as a complementary modality to a chest X-ray. By introducing lung US as a standard method of examination in day-to-day work, it would be possible to reduce the use of X-ray studies in premature neonates.

Introduction: Congenital hydronephrosis (CH) is a condition with dilated renal pelvis with or without dilation of renal calyces. Aim: To examine the role of magnetic resonance urography in the detection of congenital hydronephrosis in comparison to dynamic renal scintigraphy (DRS). Patients and methods: Resarch included 58 (n=58) patients with diagnosis of congenital unilateral or bilateral dilatation of kidney duct system. Each patient had a one-time or multiple hospitalization at the Nephrology Department of the Pediatric Clinic, with performed: ultrasound which confirmed CH, voiding cystourethrography / voiding urosonography was confirmed based on which the vesicoureteral reflux (VUR) was established / excluded, dynamic renal scintigraphy and magnetic urography (analysis was made by CHOP-fMRU software) on the basis of which the uterine anatomy and the relative renal function were evaluated. Results: Male patients were represented in 40 cases (69%). The average age was 4.4 ± 4.3 years with the youngest patient at the age of 2 months, and the oldest was 17 years old. According to diagnostic entities, the most common diagnosis was ureteropelvic junction (UPJ) obstruction in over half of cases (30 or 51.7%), followed by subjects with ureterovesical junction (UVJ) obstruction (11 or 19%), VUR was recorded in 9 (15.5%) cases, and pyelon fissus in 7 (12.1%), and one case (1.7%) was recorded with bilateral megaureter. Comparison of the value of the renal function obtained with DRS and CHOP-fMRU methods shows that there were no statistically significant differences between two methods. In the case of right kidney, the mean DRS value was 53.4 ± 18.4% (range 13-100%), while CHOP-fMRU was 51.8 ± 22.4 (range 0-96.7%). In the case of left kidney, the average value according to the DRS method was 46.9 ± 18.9% (range 0-87%) and according to CHOP-fMRU 47.6 ± 21.5% (range 8.3-100%). The correlation coefficients of both right and left kidneys show a highly statistically significant correlation between these two methods. Conclusion: Magnetic resonance urography in the pediatric population in CH based on results should be an integral part of the management of these patients, especially in congenital obstructive uropathy, in complex and associated congenital anomalies, as it provides morphological and functional data on the state of the kidneys and urinary tract.

Introduction: The objective of this research was to determine whether vesicoureteral reflux(VUR) was associated with evolution to renal scarring (RS) following a febrile urinary tract infection (UTI) in infants. Materials and methods: Our research included 100 infants, ages up to 1 year with a first febrile UTI. The diagnostic was based on results of: laboratory findings, ultrasonography (USG), voiding cystourethrography (VCUG) and initial and control renal scintigraphy (DMSA renal scan) withtechnetium99mTcsuccimer (dimercaptosuccinic acid), to assess the acute pyelonephritis (APN), VUR and RS. Results: APN was proven with DMSA renal scan in 66 (66%) infants. Twenty-two infants (33.3%) had VUR in-group of patients with APN. On the control DMSA scan, performed 6 months after the first DMSA, the presence of RS was found in 18 (27.27%) infants. In infants with renal scars VUR were discovered in 9 of them (50%). Conclusions: The pathogenesis of RS after febrile UTI in young children is multifactorial. Children with VUR have an increased risk for APN and RS. However, VUR is not the only precondition for RS. Creating a renal scarring cannot be imagined without the inflammatory process of the upper urinary system. Therefore, early detection and treatment of febrile UTIs in children and identify children at risk for RS are of primary importance.

Background: Nephrotic syndrome (NS) is one of the most frequent glomerular diseases among children. While most of the children with primary NS respond to steroid treatment, 10 to 20% of the patients are steroid-resistant, and the best therapy for such cases has never been defined. Objective: The present study aimed to evaluate steroid-resistant nephrotic syndrome (SRNS) patients. Materials and methods: Our research included 50 children (56% female and 44% male) with NS. NS was defined as the presence of edema, massive proteinuria, hypoalbuminemia and hyperlipidemia. Patients with NS were treated according to international protocol. SRNS was diagnosed in patients with idiopathic NS based on lack of complete remission despite treatment with steroids. Renal biopsy was performed in 22 patients with SRNS at the Pediatric Clinic II of the University Clinical Center in Sarajevo (UCCS). Histopathologic analyzes of renal biopsy were performed at the Department of Pathology, University Clinical Center in Tuzla (UCCT). Patients with SRNS, after kidney biopsy were treated with nonsteroidal immunosuppressant’s. Results: Eight (36.4%) of the 22 patients who had undergone renal biopsies had minimal change disease (MCNS) and seven (31.8%) had focal segmental glomerulosclerosis (FSGS). The immunosuppressive drugs used in SRNS were Cyclosporine (CsA), Cyclophosphamide (CYC), Mycophenolat mofetil (MMF) and Rituximab (RTX). Three patients (13.6%) did not respond to any treatment and had developed end - stage renal disease (ESRD). Conclusion: With current treatments, some children will ultimately achieve a sustained remission with one of the second line or third line of the proposed drugs. Patients with refractory NS will go to progression towards ESRD. The rapid development of molecular genetics will give a new contribution to the pathogenesis and treatment of this disease.