Atypical Hemolytic Uremic Syndrome with Severe Extrarenal Manifestations: a Case Report

Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease that may lead to end-stage renal failure (ESRF) or death, and may be accompanied by a variety of extrarenal manifestations. We presented a child with aHUS accompanied by severe extrarenal manifestations. A 12-year-old girl was visited in the emergency departments with acute renal failure, symptoms of fluid overload, vomiting, and somnolence. Laboratory tests revealed microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure with severe electrolyte imbalance.  Diagnosis of HUS was made and emergency hemodialysis was performed to decrease the circulating volume, restore the electrolyte disturbance, and support the treatment of HUS, but conventional medical therapies were ineffective. The patient experienced frequent seizures and multiple cardiac arrests and became comatose. Thereafter, although she was diagnosed with aHUS, plasma infusions and plasmapheresis were performed. Upper gastrointestinal endoscopy and colonoscopy revealed erosive pangastritis, widespread gastric hemorrhagic ulcers, bulbitis, and hemorrhagic colitis. Since there was no improvement, the patient was transferred to a central university hospital where eculizumab was started. She responded to eculizumab. In conclusion, as aHUS can progress rapidly and is frequently fatal if untreated, it is important to be aware of unusual presentations and diagnose the condition promptly, particularly if supportive treatment is of little or no help

The prevalence of celiac disease in children with iron-deficiency anemia

Background/aims: Celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. Iron-deficiency anemia is the most commonly encountered anemia in humans. Iron-deficiency anemia also is a common extraintestinal manifestation of celiac disease. To determine the celiac disease prevalence in children with iron-deficiency anemia and to compare the hematologic parameters in iron-deficiency anemia patients with and without celiac disease. Materials and Methods: A total of 61 patients aged 2-16 years who presented with iron-deficiency anemia were included in this study. Hemoglobin, red cell indices (mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, red cell distribution width), serum iron, and serum ferritin were determined. Venous blood samples for anti-tissue transglutaminase antibody immunoglobuline A were obtained from these patients. Upper gastrointestinal endoscopy was recommended to patients who had positive serology. Results: Of 61 patients with iron-deficiency anemia, 13 (21,3%) had positive serology for celiac disease. The small intestine biopsy of all patients with positive serology showed villous atrophy (Marsh 3). The mean hemoglobin level was significantly lower in iron-deficiency anemia patients with celiac disease when compared to those without celiac disease (7,8 +/- 2,6 vs. 11,3 +/- 0,9 g/dL, p<0,05). There was a statistically significant negative correlation of tissue transglutaminase titers with hemoglobin, red cell indices, serum iron, and serum ferritin levels. Conclusions: Screening of celiac disease by anti-tissue transglutaminase antibody should be done as a routine investigation in children with iron-deficiency anemia. Biopsy should be recommended in patients with iron-deficiency anemia who have positive celiac disease serology

Effects of hydroxyethyl starch 130/0.4 on the kidney tissue of rats with ureteral obstruction

Objective: This study was conducted since the effects of colloid solutions on the renal system remain controversial and need to be adequately studied in animals. We aimed to evaluate the effects of hydroxyethyl starch (Voluven) on the kidney tissue of rats with late renal failure due to ureteral obstruction. Materials and methods: Rats were divided into four groups: Group C, control; Group HES, hydroxyethyl starch solution (HES) 130/0.4 (Voluven (R)); Group UUO, unilateral ureteral obstruction (UUO); and Group UUO-HES, UUO-HES 130/0.4 (Voluven (R)). In the groups with ureteral obstruction, the distal part of the right ureter was accessed and sutured through a lower abdominal incision under ketamine anesthesia. Any signs of late-stage renal failure were evaluated after three weeks. Rats in the HES group and the renal failure-HES group were administered with HES 130/0.4 as a single intravenous dose of 20 mL/kg. After a follow-up of 24 hours, intra-abdominal blood sample was collected, and the rats were sacrificed. Biochemical and histopathological parameters were then evaluated. Results: Ureteral obstruction significantly increased urea and creatinine levels. In addition, when the UUO-HES and HES groups were compared, the administration of HES increased urea and creatinine levels in the UUO-HES group. Nitric oxide enzyme activity and malondialdehyde levels have significantly increased in the UUO groups. In addition, HES significantly increased nitric oxide activity and malondialdehyde levels in the UUO-HES group, in comparison with the LIES group. The activity of caspases 3 and 8 was significantly increased in the UUO groups. In addition, HES significantly increased the activity of caspases 3 and 8 in the UUO-HES group, in comparison with the HES group. Light microscopy revealed significant changes in the UUO groups, especially in the obstructed kidneys. Conclusion: If indicated, HES should be used with caution in cases of UUO, but not in the cases of bilateral ureteral obstruction. Other aspects of these findings, including the clinical significance and practical applications, merit further experimental and clinical investigation

Exenatide reduces oxidative stress and cell death in testis in iron overload rat model

Glucagon-like peptide-1 (GLP-1) has been demonstrated to affect the oxidative stress status in several in vitro, in vivo and clinical studies. The aim of the present study was to evaluate the effect of a GLP-1 analogue, exenatide, on oxidative stress parameters and apoptotic markers in testicular cells in an iron overload rat model. To obtain this model, the animals were randomly divided into three groups (n=6/group). Rats in the control group received intraperitoneal injections of saline. Intraperitoneal iron dextran (60 mg/kg/day) was given to Group FE for 5 days a week for 4 weeks. The third group (Group Fe +E) was given subcutaneous injections of 10 mu g/kg exenatide in two divided doses for 4 weeks in addition to iron dextran. Testes of all rats were immediately removed for immunohistochemical staining and to measure the malondialdehyde level and superoxide dismutase enzyme activity. A significant reduction was observed in caspase-8 and -3 enzyme staining in testicular stromal and endothelial cells in exenatide injected iron overloaded rats when compared with controls. Oxidative stress markers malondialdehyde levels and superoxide dismutase enzyme activities were also significantly lower in exenatide-injected rats when compared with controls. These findings indicate that exenatide may be protective against the harmful effects of iron accumulation in testis. Further studies are required to evaluate how exenatide reduces oxidative stress and cell death in iron overloaded testis tissue

PROGRESS STUDY: Progression of chronic kidney disease in children and heat shock proteins

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

CLINICAL, EPIDEMIOLOGICAL AND PROGNOSTIC FEATURES OF CHILDREN WITH HEMOLYTIC UREMIC SYNDROME: MULTICENTER EXPERIENCE OF TURKEY

WOS: 00044399840014

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. Graphical abstract: [Figure not available: see fulltext.