Objective This study aimed to analyze the clinical characteristics and prognosis of pediatric idiopathic pulmonary hemosiderosis (IPH). Methods Pediatric IPH cases that were diagnosed at West China Second University Hospital, Sichuan University between 1996 and 2017 were reviewed. Follow-up data from 34 patients were collected. Results A total of 107 patients were included (42 boys and 65 girls). The median age was 6 years at diagnosis. The main manifestations of the patients were as follows: anemia (n = 100, 93.45%), cough (n = 68, 63.55%), hemoptysis (n = 61, 57%), fever (n = 23, 21.5%), and dyspnea (n = 23, 21.5%). There were relatively few pulmonary signs. The positive rates of hemosiderin-laden macrophages in sputum, gastric lavage fluid, and bronchoalveolar lavage fluid were 91.66%, 98.21%, and 100%, respectively. Seventy-nine patients were misdiagnosed. A total of 105 patients were initially treated with glucocorticoids, among whom 102 survived and three died. Among the followed up patients, two died and 32 survived, among whom 10 presented with recurrent episodes. Conclusions The classic triad of pediatric IPH is not always present. The rates of misdiagnosis and recurrence of IPH are high. Early recognition and adequate immunosuppressive therapy are imperative for improving prognosis of IPH.

Nephronophthisis is an autosomal recessive cystic kidney disease and one of the most common genetic disorders causing end‐stage renal disease in children. Nephronophthisis is a genetically heterogenous disorder with more than 25 identified genes. In 10%–20% of cases, there are additional features of a ciliopathy syndrome, such as retinal defects, liver fibrosis, skeletal abnormalities, and brain developmental disorders. This review provides an update of the recent advances in the clinical features and related gene mutations of nephronophthisis, and novel approaches for therapy in nephronophthisis patients may be needed.

Background Renal hypoxia is considered a final pathway in the progression of chronic kidney disease (CKD). Blood-oxygenlevel-dependent magnetic resonance imaging (BOLD-MRI) has shown merit for evaluating renal oxygenation in adults.Objective To investigate renal cortical and medullary R2* values by CKD stage and by renal function index in children with chronic kidney disease. Materials and methods Twenty-one children with CKD Stage 1-3, 16 children with CKD Stage 4-5, and 6 healthy volunteers underwent a renal MRI using multigradient recalled-echo sequence with 16 echoes. We measured the R2* values of the renal cortex and medulla on BOLD-MRI. Results The cortical R2* value was ranked as CKD Stage 4-5 > CKD Stage 1-3 > healthy controls, and the medullary R2* value was ranked as CKD Stage 4-5 > CKD Stage 1-3. There was no significant difference in the medullary R2* value between CKD Stage 1-3 patients and the healthy controls. There was a positive correlation between the R2* values in the renal cortex (r=0.73) and medulla (r=0.89), and the serum creatinine level (P<0.001), and the renal cortical and medullary R2* values were negatively correlated with the estimated glomerular filtration rate (r=-0.71 and r=-0.89, respectively; P<0.001). Conclusion BOLD-MRI might contribute to noninvasive assessment of renal oxygenation in children with CKD in vivo but it did not reflect renal function in our sample.

Objective This study aimed to investigate the clinical features and risk factors of acute kidney injury (AKI) in children with acute paraquat intoxication. Methods A retrospective study of 110 hospitalized children with acute paraquat intoxication in West China Second University Hospital, Sichuan University was conducted from January 2010 to May 2017. The Kaplan–Meier method was used to compare the survival rates of children with different AKI stages. Multivariate logistic regression was applied to analyse the risk factors for paraquat-induced AKI. Results AKI occurred in 42 of 110 (38.2%) children. We observed AKI stage 1 in two (4.8%) children, AKI stage 2 in 11 (26.2%), and AKI stage 3 in 29 (69.0%). The survival rate of children in AKI stage 3 (34.5%) was significantly lower than that in children in AKI stage 2 (63.6%) and AKI stage 1 (100%). Multivariate analysis showed that oral ulcers and elevated blood glucose levels were significant independent risk factors for paraquat-induced AKI in children (odds ratio = 4.223 and 5.545, respectively). Conclusions The incidence and mortality rates of paraquat-induced AKI in children are high. Oral ulcers and elevated blood glucose levels are independent risk factors affecting the occurrence of paraquat-induced AKI in children.

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