Idiopathic nephrotic syndrome (INS) is characterized by marked urinary excretion of albumin and other intermediate-sized plasma proteins, such as transferrin and vitamin D-binding protein. Some cases even develop anemia. The aim of this study was to investigate the changes in serum iron, transferrin, and erythropoietin, and the relationships between serum and urine transferrin and erythropoietin. Thirty-seven children with INS and 35 age-and sex-matched healthy children were investigated. The indexes related to iron metabolism, including serum iron, ferritin, transferrin, total iron-binding capacity (TIBC), transferrin saturation, and hematological parameters [hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH)], and urinary transferrin and erythropoietin were measured in 37 children with INS before treatment and at the remission stage. Thirty-five age-and sex-matched healthy children served as controls. Serum iron levels (18.8 ¡ 3.8) mmol/L in INS patients before treatment were significantly lower than those of the healthy controls (22.2 ¡ 3.8) mmol/L and those measured at the remission stage (21.0 ¡ 3.5) mmol/L (all P , 001). Serum transferrin levels in INS patients before therapy (1.9 ¡ 0.3) g/L also decreased compared with the healthy controls (3.1 ¡ 0.5) g/L and the measures at the remission stage (2.9 ¡ 0.6) g/L (all P , 0.01). In contrast, serum TIBC and transferrin saturation were significantly higher in INS patients before treatment than in the healthy controls [TIBC (56.4 ¡ 9.2) mmol/L vs (50.7 ¡ 6.8) mmol/L, P , 0.01; transferrin saturation (55.7¡9.2)% vs (46.4 ¡ 8.2)%, P , 0.01] and they were also higher than the measures at remission stage [(51.9 ¡ 7.7) mmol/L and (47.4 ¡ 13.3) mmol/L] (all P , 0.01). Serum transferrin was positively correlated with serum albumin (r 5 0.609, P , 0.01) and negatively correlated with urinary transferrin (r 5 20.550, P , 0.01) in INS patients before treatment. We conclude that serum iron, transferrin and erythropoietin levels are markedly decreased in INS patients, which may be partially related to the urinary loss of these indexes.
BackgroundThis study aimed to gather evidence from clinical trials on the efficacy and safety of the available treatments for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) in children.MethodsThis work adopted the Newcastle–Ottawa scale to analyse the quality of the enrolled articles. A network meta-analysis was performed using clinical trials that compared drugs used to treat IVIG-resistant KD. Aggregate Data Drug Information System software v.1.16.5 was employed to analyse whether infliximab, second IVIG infusions, and intravenous pulse methylprednisolone (IVMP) were safe and effective.ResultsTen studies, involving 704 patients with IVIG-resistant KD, were identified and analysed. Overall, infliximab exhibited remarkable antipyretic activity compared with the second IVIG infusions (2.46, 1.00–6.94). According to the drug rank, infliximab was the best option against IVIG-resistant KD. Regarding adverse effects, the infliximab group was more prone to hepatomegaly. A second IVIG infusion was more likely to result in haemolytic anaemia. IVMP treatment was more susceptible to bradycardia, hyperglycaemia, hypertension, and hypothermia. In addition, infliximab, IVMP, and the second IVIG infusions showed no significant differences in the risk of developing a coronary artery aneurysm (CAA).ConclusionInfliximab was the best option against IVIG-resistant KD, and IVMP, infliximab, and second IVIG infusions have not significant differences of prevent CAA in patients with IVIG-resistant KD.Systematic Review RegistrationIdentifier: https://osf.io/3894y.
Objective This case–control study focused on the establishment and internal validation of a risk nomogram for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) using the Kawasaki Disease Database. Methods The Kawasaki Disease Database is the first public database for KD researchers. A prediction nomogram for IVIG-resistant KD was constructed using multivariable logistic regression. Then, the C-index was used to assess the discriminating ability of the proposed prediction model, a calibration plot was drawn to evaluate its calibration, and a decision curve analysis was adopted to assess its clinical usefulness. Bootstrapping validation was performed for interval validation. Results The median ages of IVIG-resistant and -sensitive KD groups were 3.3 and 2.9 years, respectively. Predicting factors incorporated into the nomogram were coronary artery lesions, C-reactive protein, percentage of neutrophils, platelets, aspartate aminotransferase, and alanine transaminase. Our constructed nomogram exhibited favorable discriminating ability (C-index: 0.742; 95% confidence interval: 0.673–0.812) and excellent calibration. Moreover, interval validation achieved a high C-index of 0.722. Conclusions The as-constructed new IVIG-resistant KD nomogram that incorporated C-reactive protein, coronary artery lesions, platelets, percentage of neutrophils, alanine transaminase, and aspartate aminotransferase may be adopted for predicting the risk of IVIG-resistant KD.
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