Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome.

Pro-inflammatory cytokines have been suggested in the pathogenesis of idiopathic nephrotic syndrome (INS), with conflicting results. This study was performed to identify alteration of different serum interleukins (ILs) in children with INS, and their predictive value in response to steroid treatment. Three groups of children (27; steroid-sensitive INS, 21; steroid-resistant INS, and 19 healthy controls) with normal serum C3, negative serologic tests of hepatitis B virus (HBV), hepatitis C virus (HCV), human immune deficiency virus (HIV), and parasitic infections were included in this study. Serum concentrations of IL-1ß, IL-2, IL-6, IL-8, IL-13, and IL-18 were measured, using quantitative colorimetric sandwich ELISA kits. Children with secondary nephrotic syndrome, inflammations, systemic disorders, and chronic kidney disease were excluded. The serum concentration of all ILs; except IL-13 and IL-18; was significantly higher in children with INS, compared with the healthy controls. Serum IL-2 had the highest sensitivity of (95.24%) in patients with INS. All of the serum ILs had acceptable accuracy in children with INS, compared with the control group. The serum concentration of IL-1ß, IL-6, and IL-8 was significantly higher in children with steroid-sensitive nephrotic syndrome (SSNS), compared with steroid-resistant nephrotic syndrome (SRNS). All of these ILs had acceptable accuracy for the prediction of steroid response in patients with INS. Our findings suggested the pathogenic role of pro-inflammatory cytokines in children with INS, of which IL-1ß, IL-6, and IL-8 were accurate biomarkers for the prediction of steroid response in these patients.

Improvement Of Congenital Chloride Diarrhea With Corticosteroids: An Incidental Finding.

Congenital chloride diarrhea of infancy is a life threatening disease. We discuss two boys with congenital chloride diarrhea over a long time period before and after kidney transplantation. In the first case, prenatal sonography revealed polyhydramnios and generalized bowel loop distention. The genetic study confirmed congenital chloride diarrhea of infancy. Multiple episodes of severe dehydration, hyponatremia and acute tubular necrosis were seen during the follow up period. He underwent a year of hemodialysis before kidney transplantation. Three periods of improvement concerning diarrhea occurred with the use of corticosteroids, taken for other reasons. These improvements were seen after prednisolone administration for mastoiditis and following prednisolone administration for kidney transplantation. The second case was a 3.5 year old boy who is the cousin of the first case. He was referred to hospital with chronic watery diarrhea, metabolic alkalosis, hypokalemia, hyponatremia and failure to thrive in the first year of life. He was also treated with prednisolone and showed significant improvement.

High frequency of acquired ADAMTS13 deficiency after hemolysis in Hemiscorpius Lepturus (scorpion) stung children.

OBJECTIVE: To estimate the frequency of acquired ADAMTS13 deficiency in severe cases of Hemiscorpius lepturus stung patients and the frequency of acute kidney injury (AKI) in these patients. METHODS: Sixty scorpion stung children who were referred with severe hemolysis and hemoglobinuria were studied. None of them had received blood products and no one had a past medical history of renal failure. RESULTS: Plasma levels of ADAMTS13 and ADAMTS13 antibody (IgG) were measured using ELISA. ADAMTS13 was decreased in 91.7 % of patients and the anti-ADAMTS13 antibody (Ab) was increased in 98.3 %. ADAMTS13 decreased in all of the patients with acute kidney injury and none of those with normal levels of ADAMTS13 developed renal failure; all patients with AKI had also increased levels of ADAMTS13Ab. Acute kidney injury was found in 23.3 % and had significant association with severe anemia, thrombocytopenia, pyuria, hematuria and considerable proteinuria (p < 0.001). Disseminated intravascular coagulation (DIC) and Hemolytic uremic syndrome (HUS) developed in 6.7 % and 10 % respectively. CONCLUSIONS: The index findings demonstrate that Hemiscorpius lepturus sting is usually associated with ADAMTS13 deficiency, and increased ADAMTS13 autoantibody. These combined mechanisms may contribute to scorpion sting-induced coagulopathies and may predispose patients to develop DIC and HUS.