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Various molecular and cellular processes are involved in renal fibrosis, such as oxidative stress, inflammation, endothelial cell injury, and apoptosis. Heat shock proteins (HSPs) are implicated in the progression of chronic kidney disease (CKD). Our aim was to evaluate changes in urine and serum HSP levels over time and their relationships with the clinical parameters of CKD in children. In total, 117 children with CKD and 56 healthy children were examined. The CKD group was followed up prospectively for 24 months. Serum and urine HSP27, HSP40, HSP47, HSP60, HSP70, HSP72, and HSP90 levels and serum anti-HSP60 and anti-HSP70 levels were measured by ELISA at baseline, 12 months, and 24 months. The urine levels of all HSPs and the serum levels of HSP40, HSP47, HSP60, HSP70, anti-HSP60, and anti-HSP70 were higher at baseline in the CKD group than in the control group. Over the months, serum HSP47 and HSP60 levels steadily decreased, whereas HSP90 and anti-HSP60 levels steadily increased. Urine HSP levels were elevated in children with CKD; however, with the exception of HSP90, they decreased over time. In conclusion, our study demonstrates that CKD progression is a complicated process that involves HSPs, but they do not predict CKD progression. The protective role of HSPs against CKD may weaken over time, and HSP90 may have a detrimental effect on the disease course. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12192-021-01239-9.

Background/aim: this study aimed to evaluate the efficacy of rituximab in children with difficult-to-treat nephrotic syndrome, considering the type of disease (steroid-sensitive or -resistant) and the dosing regimen. Materials and methods: this multicenter retrospective study enrolled children with difficult-to-treat nephrotic syndrome on rituximab treatment from 13 centers. The patients were classified based on low (single dose of 375 mg/m(2)) or high (2-4 doses of 375 mg/m(2)) initial dose of rituximab and the steroid response. Clinical outcomes were compared. Results: data from 42 children [20 steroid-sensitive (frequent relapsing / steroid-dependent) and 22 steroid-resistant nephrotic syndrome, aged 1.9-17.3 years] were analyzed. Eleven patients with steroid-sensitive nephrotic syndrome (55%) had a relapse following initial rituximab therapy, with the mean time to first relapse of 8.4 +/- 5.2 months. Complete remission was achieved in 41% and 36% of steroid-resistant patients, with the median remission time of 3.65 months. At Year 2, eight patients in steroid-sensitive group (40%) and four in steroid-resistant group (18%) were drug-free. Total cumulative doses of rituximab were higher in steroid-resistant group (p = 001). Relapse rates and time to first relapse in steroid-sensitive group or remission rates in steroid-resistant group did not differ between the low and high initial dose groups. Conclusion: the current study reveals that rituximab therapy may provide a lower relapse rate and prolonged relapse-free survival in the steroid-sensitive group, increased remission rates in the steroid-resistant group, and a significant number of drug-free patients in both groups. The optimal regimen for initial treatment and maintenance needs to be determined., NA

BACKGROUND Acute kidney injury (AKI) is a common condition in the neonatal intensive care unit (NICU), particularly in preterm infants. Management of AKI in neonates is challenging. Peritoneal dialysis (PD) has been preferred as the most applicable modality in neonates when medical therapy fails. CASE A female infant was born at 24 and 4/7 weeks with a birth weight of 460 grams after an emergency cesarian section from a preeclamptic pregnacy. She developed AKI secondary to sepsis. A neonatal, straight single-cuff Tenckhoff catheter was inserted and PD was started on day 12. PD was discontinued after 6 days, on day 18 with adequate urine output and normalization of kidney function tests. However, the patient died on day 152 secondary to a nosocomial infection. CONCLUSION To the best of our knowlegde, our case is the smallest infant in whom PD was performed succesfully with a PD catheter. PD is a relatively safe, effective and a feasible therapy in the neonatal population even in the smallest infants. PD may be a live-saving procedure in extremely low birth weight infants with severe AKI.

Objective: To assess the morphological and clinical features of children with renal ectopia (RE) and assess whether the lateralization of ectopia determines the prognosis. Methods:The clinical, laboratory and radiological data of 61 patients diagnosed as RE in our center between the years of 2004 and 2014 were retrospectively reviewed. Results: There were 32 girls (52.5%) and 48 boys (47.5%). The median age was 48 (2-204) months and follow-up time was 60 (2-120) months. RE was right-sided in 33 (54%) and left-sided in 26 (46%) patients. 52 patients (85.2%) had simple RE whereas 9 (14.8%) had crossed RE. 27 children (44.3%) had additional urinary abnormality, 15 (24.6%) had extra-renal disease. The most common urinary tract malformation was vesicoureteral reflux (VUR) (21%). Renal scarring (RS) was detected in 17 patients (27.9%) on DMSA scan. 32 patients (52.5%) experienced urinary tract infection (UTI), 5 (8.2%) had hypertension, 11 (11%) had proteinuria, 5 (8%) had chronic renal disease (CKD). No significant difference was found between ectopia sides for evelopment of additional urinary or systemic disease, UTI, proteinuria, hypertension and CKD (p> 0.05). Conclusion: The children with RE should be carefully examined for additional urinary or systemic abnormalities. Voiding cystourethrogram might be recommended in those patients for the increased risk of VUR. The patients with RE should be closely followed-up for avoiding long term complications including proteinuria, hypertension and CKD because of susceptibility to UTI and RS. The lateralization of ectopia might not affect the incidence of additional abnormality or late complications.

PubMedID: 25019950 Background: We aimed to evaluate whether serum apelin could reflect the nutritional status of children on dialysis. Methods: Twelve patients on peritoneal dialysis (PD) and 20 patients on hemodialysis (HD) were enrolled. Patients received individualized diet for six months. Anthropometric and laboratory indices were measured at onset and the end of the study. Results: The anthropometric indices were all significantly lower in patients than in controls whereas similar in PD and HD patients. The protein catabolic rate (nPCR), height, mid-arm circumference (MAC), triceps skinfold thickness (TSF), arm muscle area (AMA) and arm fat area (AFA) z scores were significantly increased in dialysis patients after nutritional intervention. Weight z scores statistically increased in HD group whereas did not statistically change in PD group. Serum albumin levels were significantly improved in PD and HD patients. Apelin levels were similar in PD, HD and control groups. Post nutritional apelin values did not differ in each dialysis groups. On multivariate analysis, apelin was independently associated with age, weight, ESR and TG. Conclusions: Apelin seems to be not a useful indicator for monitoring the nutritional status in children on dialysis. However, the close link of apelin with inflammatory and lipid parameters suggested that apelin might be a novel target for slowing the atherogenic process in pediatric dialysis patients. © 2014 Informa Healthcare USA, Inc. All rights reserved. 2009BAP36 The study was funded by the unit of Scientific Research Project in Cukurova University: TF 2009BAP36. The authors thank to Ruksan Anarat and her staff for their strenuous efforts on biochemical analysis, and also to Safak Oznergiz, Gulcan Delidag and Hanifi Saban for allocation and preservation of the serum samples.

Congenital insensitivity to pain and anhidrosis (CIPA) is a rare form of hereditary sensory and autonomic neuropathy. It is characterized by impaired perception of pain and temperature, anhidrosis and intellectual disability. Self mutilating behaviors lead to accidental injuries. The limb lesions are often infected and frequently progress to chronic osteomyelitis. In pediatrics, amyloidosis usually occurs secondary to chronic inflammatory diseases. The coexistence of amyloidosis and CIPA has not previously been reported in literature. A CIPA case complicated with nephrotic syndrome and renal amyloidosis following chronic osteomyelitis is presented here. This report emphasizes the importance of close follow-up of patients by urine analysis for the risk of developing amyloidosis particularly in the presence of chronic infections.

Background: Drug-ethanol interaction can result in hepatotoxicity. The liver is capable of metabolizing both acetaminophen and ethanol, however, severe acute or moderate chronic simultaneous exposure can cause cell and tissue damage. Therapeutic doses can become harmful if gene activity is altered via competition for metabolic pathways. Simultaneous intake of ethanol and acetaminophen results in overactive CYP2E1 and depletion of glutathione, leaving NAPQI to build up in the liver. NAPQI is a hepatotoxic substance typically neutralized by glutathione. Methods: Bioinformatics tools including PharmGKB, Chemical Annotation Retrieval Toolkit, Transcriptome Analysis Console 4.0 (TAC), wikipathways, STRING, and Ingenuity Pathway Analysis (IPA) were used to explore interactive metabolic pathways of ethanol-acetaminophen exposure as a proof of concept for assessing drug-drug or drug-alcohol interactions. Results: As the ethanol-acetaminophen comparison indicates, bioinformatics tools may be used to understand interactive pathways following exposure to ethanol and acetaminophen, with potential extrapolation to other drug-drug/drug-ethanol interactions. Conclusions: Direct interactive effects were not able to be confirmed through this bioinformatics study due to the lack of existing ethanol-acetaminophen simultaneous exposure data. This work suggests that a battery of software applications should be used to assess interactive effects.

A congenital prepubic sinus is a tract that originates in the skin and overlays the base of the penis; however, its embryologic basis is still debated. We herein present a case involving a 3-year-old boy who was admitted for examination of overlying tissue located a few centimeters distal to the dorsal base of the penis. Examination revealed a tiny sinus in the prepubic area, and 3 cm of tissue was attached to the sinus. Pathologic examination showed that the tissue was lined with squamous epithelium and continued along the sinus tract, which was lined with urothelial epithelium. According to Stephens' classification, the sinus appeared to be a variant of type 2 dorsal urethral duplication, and the remnant tissue mimicked the corpus spongiosum of the penis.

Henoch-Schonlein purpurasi (HSP) cesitli sistemleri tu­tan ve farkli klinik seyirler gosteren bir vaskulittir. Purpura tarzinda dokuntu, karin agrisi, artrit, bobrek tutulumu ile ortaya cikabilmektedir. Nadiren, deri, gastrointestinal sis­tem (GIS) ve bobrek tutulumu agir seyretmekte ve erken veya gec donemde cesitli komplikasyonlara yol acabil­mektedir. Bu yazida yuksek doz intravenoz steroid teda­visine yanit vermeyen tekrarlayan GIS kanamasi intrave­noz siklofosfamid ile basarili bir sekilde tedavi edilen agir GIS tutulumlu iki HSP\'li cocuk olgunun sunulmasi amac­landi. Hastalarimizdan birinde GIS tutulumuna ilaveten deri grefti gerektirecek kadar agir nekrotik cilt tutulumu, digerinde ise nefrotik proteinuri ile seyreden bobrek tu­tulumu mevcuttu. HSP\'li cocuklarda yuksek doz steroide yanit alinamayan agir GIS tutulumunda intravenoz siklo­fosfamid yararli olabilir.

The nutcracker syndrome refers to compression of left renal vein between the superior mesenteric artery and aorta. Renal abscess consists of purulent and necrotic material localised to the renal parenchyma. These two entities are extremely rare and their coincidence has not previously been described in literature. Here, we report a case of a 10-year-old girl who developed left renal abscess probably due to nutcracker syndrome.

önenli-Mungan N, KörD, Karabay-Bayazıt A, Cengiz N, YavuzS, NoyanA, CeylanerG, Şeker-Yılmaz B, TopaloğluAK, YükselB, AnaratA. Genotypic and phenotypic features of the cystinosis patients from the South Eastern part of Turkey. Turk J Pediatr 2016; 58: 362-370.We have conducted this study for the purposes of demonstrating the spectrum of mutations and of identifying their effects on the phenotype, with a particular focus on the clinical course, prognosis and response to treatment. A total of 25 patients from 20 families, who have been treated and followed up after being diagnosed with cystinosis. Nine patients were identified with mutations of homozygous c.451A>G, 7 patients with homozygous c.681G>A, 6 patients with homozygous c.834_842del, 2 patients with homozygous c.18_21delGACT and 1 patient with compound heterozygous for c.451A>G/ c.1015G>A. The c.834_842del mutation identified in six patients from four families has not been previously identified.Progression to renal failure occurred earlier in the patients identified with the new mutation, despite treatment. Larger patient series are required to demonstrate the genotypic properties of the patients with cystinosis and their relationship with the clinical course. önenli-Mungan N, KörD, Karabay-Bayazıt A, Cengiz N, YavuzS, NoyanA, CeylanerG, Şeker-Yılmaz B, TopaloğluAK, YükselB, AnaratA. Genotypic and phenotypic features of the cystinosis patients from the South Eastern part of Turkey. Turk J Pediatr 2016; 58: 362-370.We have conducted this study for the purposes of demonstrating the spectrum of mutations and of identifying their effects on the phenotype, with a particular focus on the clinical course, prognosis and response to treatment. A total of 25 patients from 20 families, who have been treated and followed up after being diagnosed with cystinosis. Nine patients were identified with mutations of homozygous c.451A>G, 7 patients with homozygous c.681G>A, 6 patients with homozygous c.834_842del, 2 patients with homozygous c.18_21delGACT and 1 patient with compound heterozygous for c.451A>G/ c.1015G>A. The c.834_842del mutation identified in six patients from four families has not been previously identified.Progression to renal failure occurred earlier in the patients identified with the new mutation, despite treatment. Larger patient series are required to demonstrate the genotypic properties of the patients with cystinosis and their relationship with the clinical course.

Heiner syndrome is a food-induced pulmonary hypersensitivity disease that predominantly affects infants. Chronic respiratory symptoms with pulmonary infiltrates on radiography, positive milk precipitins and resolution of findings upon removal of cow's milk constitute the main features. Severe cases may present with pulmonary hemosiderosis. Few renal manifestations associated with this syndrome have been reported so far. Here we report the first case of Heiner syndrome complicated by crescentic glomerulonephritis after 5 years of follow-up.

Aim: To evaluate the association between urinary tract infection (UTI) and urinary metabolites. Material and Method: Eighty children aged below 14 years old who were following for recurrent UTI were enrolled into the study. Urinary calcium (Uca), oxalate (Uox), citrate (Ucit) and cysteine (Ucis) levels were studied in 24 hours urine samples. Hypercalciuria, hyperoxaluria and hypocitraturia were identified according to the reference values. The positivity of sodium nitroprussid test was accepted as cystinuria. The results were compared between patients and control groups involving thirty children. The patients were divided into two subgroups according to the presence of renal scarring (RS) on radionuclide scan. The similar comparisons were made between the subgroups. Results: There was no significant difference between the ratios of hypercalciuria, hypocitraturia and cystinuria in patient and control groups (p> 0.05). Uox/Ucr levels were significantly increased in patients compared to controls (p= 0.001) whereas Uca/Ucr and Ucit/Ucr levels were similar among study groups (p= 0. 082 and p= 0.466). There was no significant difference between RS (-) and RS ( ) groups for hypercalciuria, hyperoxaluria, hypocitraturia and cystinuria (p> 0.05). Discussion: The increase in urinary excretion of oxalate might be a risk factor for UTI. There was no evidence regarding that urinary metabolic abnormalities such as hypercalciuria, hyperoxaluria, hypocitraturia and cystinuria have affected the development of RS in the setting of UTI.

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PubMedID: 23957657 Background: Some patients with vesicoureteral reflux (VUR) develop reflux nephropathy (RN) and a number of them progress to chronic kidney disease (CKD). However, it is unclear to predict which patient will develop RN and/or CKD. The aim of this study is to evaluate the role of Interleukin-18 (IL-18), C-reactive protein (CRP) and procalcitonin (PCT) as an indicator of RN in VUR. Methods: Ninety-three children aged 3.5-16 years with primary VUR were enrolled. Patients were divided into two groups according to the presence of renal scarring (RS). CRP, PCT, blood urea nitrogen (BUN), serum creatinine (Scr), urinary protein (Up), creatinine (Ucr) and microalbumin (Umalb), serum and urine IL-18 levels were determined during urinary tract infection (UTI) free episode. Results: BUN, Scr, Up/Ucr and Umalb/Ucr concentrations were higher whereas calculated creatinine clearance (Ccr) values were lower in RS (+) group compared to RS (-) group. CRP, PCT, serum and urine IL-18 levels and mean urine IL-18/Cr concentrations were similar in both groups. Serum and urine IL-18 levels did not differ according to the grade of VUR. No significant correlation was found between CRP, PCT and IL-18. Conclusions: Proteinuria and microalbuminuria are valuable hallmarks of RN. CRP and PCT seem not to be reliable indicators of RN in VUR patients. Moreover, serum and urine IL-18 might not predict RN. © 2013 Informa Healthcare USA, Inc.

We conducted a multicenter pediatric study of ureteral stents unintentionally left in situ.A total of 22 patients with encrusted Double-J® ureteral stents unintentionally left in situ were treated at 4 centers between January 2007 and March 2012. Stone burdens were estimated using plain radiography and computerized tomography. Treatment decision was made based on clinical and radiological findings or stone burden.Nine girls and 13 boys with a mean age of 9.5 years (range 2 to 16) were analyzed. Mean indwelling time of ureteral stent was 21.7 months (range 6 to 60). Stents were inserted for the indication of urolithiasis (17 patients) and reconstructive urological intervention (5). In 2 patients stents had been placed bilaterally. Mean stent stone burden was 184 mm(2) on plain radiography and 247 mm(2) on computerized tomography, a difference that was statistically significant (p = 0.002). Shock wave lithotripsy was done in 6 cases. Endoscopic procedures were performed in all patients, including ureteroscopy in 8, simple stent removal in 7, endoscopic cystolithotripsy in 6, percutaneous nephrolithotomy in 5, retrograde intrarenal surgery in 3 and percutaneous cystolithotripsy in 2. Surgical removal of each stent required a mean of 1.5 interventions and a mean hospital stay of 4.4 days.At experienced centers combined endourological techniques can achieve successful and safe management of forgotten stents even in the pediatric age group. Thus, routine preprocedural tomography is a must in children with forgotten ureteral stents.

Takayasu arteritis is a potentially life threatening vasculitis that characterized by granulomatous inflammation of large vessels including aorta, its major branches and pulmonary arteries. True incidence of TA is not known in children, since TA is very rare; however, incidence of TA has been estimated as 0.8-2.6 per million children. Making a diagnosis is more difficult in childhood compared with adults due to non-specific clinical findings. The diagnosis of TA is done based on clinical criteria, laboratory investigations support and radiologic images confirm the diagnosis. Corticosteroids with other immunosuppressive drugs are used for treatment and biological agents are given to resistant cases. In this report, it was aimed to draw attention to a potentially fatal disease that frequently diagnostic delays and therapeutic difficulties occur. We, herein reported signs, symptoms, imaging methods and treatment modalities of two children, a 12 year-old male and a 13 year-old female patients, that presented with non-specific initial symptoms and diagnosed as TA with clinical findings and radiological images

WOS: 000306389200002, We report our experience in 41 patients with chronic phase (CF)-chronic myeloid leukemia (CML) who had discontinued imatinib switched to dasatinib, retrospectively. The CF-CML patients received dasatinib at starting dose of 100 once daily. Dose adjustment were observed in 11 patients, respectively. In case of other circumstances, treatment has been continued with a lower dose if needed. The median dose of dasatinib was 100 mg daily (range: 50 to 140 mg). We conclude that even low-dose dasatinib therapy is an effective and safe in second line treatment of CML patients patients.

Nutcracker syndrome (NCS) refers to the compression of the left renal vein (LRV) between aorta and superior mesenteric artery. It is very rare and usually manifests with hematuria, pain or proteinuria. Hypertension has been defined in two adult NCS cases. However ipsilateral renal scarring (RS) has not previously been demonstrated in any of NCS patients. Here we report a 14-year-old boy presented with hypertension and found to have NCS and left RS.

Post transplant lymphoproliferative disease: detection of tumor by Tc99m sestaMIBI and treatment with rituximab

PubMedID: 12853345 Background: Antiapoptotic signals are important in the development, progression and prognosis of malignant tumors. The aim of this study was to determine the two distinct antiapoptotic signals - survivin and aven - in acute leukemias and compare them with clinical and hematological findings and response to therapy. Real-time quantitative PCR was used and survivin and aven were detected at the messenger (m)RNA level. Patients and methods: Sixty-five patients with acute leukemia [37 with acute myeloblastic leukemia (AML) and 28 with acute lymphoblastic leukemia (ALL)] were used as the study group and 10 healthy subjects were used as the control group. Results: Survivin was between 0.0 and 0.829 copy number/cell (median 0.0721, mean 0.5424301909 ± 0.139799488589) and aven was between 0.0 and 0.853 copy number/cell (median 0.0124, mean 0.070335542 ± 0.1524685709). We found an important association between survivin and aven (P = 0.000). Both survivin and aven were higher in the study group than in the controls (P = 0.001 and 0.035, respectively). When we compared survivin and aven with other clinical and hematological parameters, there was an important association between survivin and extramedullary involvement (P = 0.033), survivin and alkaline phosphatase (P = 0.06), white blood cell (WBC) count and lactate dehydrogenase (LDH) (P = 0.000), WBC count and uric acid (P = 0.074), hemoglobin level and LDH (P = 0.072), LDH and uric acid (P = 0.057), CD7 expression and survivin (P = 0.097), and CD34 expression and aven (P = 0.058). Response to therapy was evaluated according to the survivin and aven levels. Survivin level was lower in refractory patients as compared with complete responders (P = 0.085). Aven level was higher in patients with relapse as compared with non-relapse patients (P = 0.04). There was no important association between survivin or aven and performance status, lymphadenopathy or organomegaly. Conclusions: Both survivin and aven are important antiapoptotic signals in acute leukemias, and the association between extramedullary involvement, CD7 expression and CD34 expression, which are important poor prognostic indicators in acute leukemias, suggests that survivin and/or aven may be novel prognostic indicators in acute leukemias. Further studies with a higher number of patients will be more informative.