The morbidity of hyperuricemia(HUA) has showed an increasing tendency in recent years,along with the improved social living conditions.HUA has been proved to be closely related to kidney diseases,metabolic diseases and cardiovascular diseases.However,people pay little attention to the relationship between uric acid and renal diseases in pediatrics,and the relevant studies and progress are lacking.Now a review is made about the metabolism of uric acid,the etiology and pathogenesis of HUA,and the relationship between HUA and kidney diseases or drugs,in order to pay attention to the relationship between HUA and kidney diseases.

The complement system, the chief component of innate immunity, is not only required for host defense against pathogens and homeostasis, but also related to the pathogenesis and development of various kidney diseases. Recent study has shown that tissue-derived complement and immune cell-derived complement can each mediate local inlfammation. The comple-ment system acts as a bridge between innate and adaptive immunity. Furthermore it’s also a functional bridge between pathogenic humoral and cellular immune responses in an array of kidney diseases. Increasing evidence links inappropriate complement acti-vation and deifciencies of complement proteins to the pathogenesis of kidney autoimmune disease, ischemia-reperfusion injury, transplant rejection and complications in hemodialysis. The development of pharmacologic agents that target complement in pa-tients with this assortment of immune and/or inlfammatory kidney diseases has the potential to abrogate disease progression and improve patient health.

Humans ; Nephritis, Interstitial ; diagnosis ; therapy ; Uveitis ; diagnosis ; therapy

Objective To explore the causes of acute renal failure resulted from tacrolimus in the treatment of nephrotic syndrome. Method The clinical data of acute renal failure caused by tacrolimus in treatment of nephrotic syndrome in 3 children during January 2012 and December 2015 were retrospectively analyzed. Results There were 2 male and 1 female aged 3, 11,and 13 years respectively. Clinical manifestations were consistent with simple type of primary nephrotic syndrome. One child was frequently recurrent and another two were secondary steroid resistant. The renal pathology showed minimal changes. Acute renal failure occurred within 4 weeks after treatment with tacrolimus on the basis of hormone therapy in all patients who had infection within one week. Renal function recovered to normal within 2 weeks after discontinuation or reduction of tacrolimus combined with anti-infection and diuresis treatment. Two children continued with tacrolimus, but the other one was replaced with cyclosporin A. The renal function of all patients remained normal during the follow-up for 10-42 months. Conclusion In the first 4 weeks of tacrolimus therapy in children with nephrotic syndrome, infection may lead to reversible acute renal failure.

Homocysteine has been confirmed as the independent factor for cardiovascular disease.Hyperhomocysteinemia remains the prominent manifestation in the cases of chronic kidney disease, atypical hemolytic anemia,methylmalonic aciduria combined with kidney injuries.As the recognition of the kidney injuries caused by homocysteine remains obscure, this review tries to show the synthesis, metabolism of homocysteine and the possible mechanism of homocysteine-induced heart and kidney injuries.Besides, summarize the current treatment of lowering-hyperhomocysteinemia in order to arouse the pediatricians' attention of homocysteine which has endothelial cell toxicity and causes the wide lesions in great, middle and small vessels, and thus improve the outcomes of the patients.

Objective To explore the diagnosis of primary bladder telangiectasia. Methods The clinical data of a child with primary bladder telangiectasia were reviewed. Results A 9-year-old girl had gross hematuria without obvious cause at 3 years old. After that she presented intermittent gross hematuria and persistent microscopic hematuria with blood clots in the urine following repeatedly respiratory tract infections, and had hemorrhagic shock once. Urine routine examination showed albumin 1+~2+ and RBC full in entire field of view. 24 hours urine protein quantitation was 0.96 g. Ultrasound of abdomen and urinary tract and enhanced CT of urinary system had no abnormal findings. Renal artery angiography showed no arteriovenous malformation or fistula. Cystoscopy showed telangiectasia. There was neither family history nor telangiectasia in other parts. Both genetic telangiectasia and ataxia telangiectasia gene mutation analysis were normal. Conclusion It is rarely seen primary bladder telangiectasia in children. However, children with early onset, long-term, and intermittent gross hematuria with blood clots, especially suffered with hemorrhagic shock, vascular disease should firstly be considered. And routine urinary imaging should be performed, including angiography and ,if necessary, cystoscopy.

Objective To explore the diagnosis of pediatric clinical hematuria disease. Methods The clinical data of one pediatric patient with IgA nephropathy combined with multiple bladder hemangioma were summarized and analyzed. Results For more than 6 years, 9-year-old female presented with repeated intermittent gross hematuria and persistent microscopic hematuria with the blood clot in urine after several respiratory tract infections. Routine urine test showed protein+++, RBC in full ifeld of vision/HP, and 0 . 54-1 . 02 g of 24 h urine protein quantitation. Early damage index of kidney is mainly based on microalbumin. The ultrasound showed no abnormal abdomen and urinary tract. Also there was no abnormality in enhanced urinary tract CT scan. Renal arteriography showed no ifstula or arteriovenous malformation. Pathological diagnosis of renal biopsy was focal proliferative IgA nephropathy. Cystoscopy examination suggested multiple hemangioma of bladder. Conclusion Bladder hemangioma is a rare condition in childhood. For children presented gross hematuria with blood clots, when the imaging ifnds no abnormalities or other diseases and the treatment of IgA nephropathy is unsatisfatry after diagnosis, the cystoscopy should be performed to exclude the possibility of bladder hemangioma.

Objective To explore the diagnosis and treatment of Dent’s disease.MethodsThe clinical characteristics, treatment process and disease-causing gene mutation were retrospectively analyzed in 6 pediatric patients with Dent’s disease misdiagnosed of nephritic syndrome from January 2014 to August 2015.ResultsIn these 6 male patients aged 4.5-9.8 years old, the main clinical manifestations were nephropathy-level of proteinuria and transient low serum albumin (26-30 g/L) without obvious edema or high serum cholesterol. In 4 patients who had renal biopsy, 2 cases showed mesangial proliferative glomerulonephritis and other 2 cases showed focal segmental glomerulosclerosis. All of 6 patients were treated with at least one immunosuppressive agent after resistance to full dose of hormone and no changes in proteinuria were observed. After admission, the indexes of early renal damage and urinary protein electrophoresis pointed to low-molecular proteinuria. The ratio of alpha 1 micro albumin (α1-MG) / micro albumin (MA) (the early renal damage index) was?>?1, there was hypercalciuria, and renal function was normal. The B ultrasonography showed renal calciifcation in 2 patients. The ifndings in all the patients were in accord with the clinical diagnosis of Dent’s disease. Further genetic analysis conifrmed the presence ofCLCN5 gene mutation in these 6 patients.ConclusionAs a type of rare inherited renal tubular disorder, Dent’s disease is easily misdiagnosed, to which pediatricians need to pay attention. The early renal damage index, α1-MG/MA?>?1, can be regarded as one of the diagnostic criteria of renal tubular proteinuria.

Objective To investigate the detection rate and possible factors of hyperhomocysteinemia(HHcy) in children with chronic kidney disease(CKD).Methods The clinical data of children with CKD between July 2012 and September 2016 in the Department of Pediatrics,Peking University First Hospital were retrospectively collected.The homocysteine(Hcy) level of patients were measured.The other data included the general information,diagnosis and laboratory test results.Results Seventy-six pediatric patients with CKD were enrolled including 49 boys and 27 girls.The average age of the patients was (9.9±3.4) years old.The main cause of the patients in the study was primary glomerulopathy(48.7%,37/76 cases),and the rest were congenital and inherited glomerular diseases(36.8%,28/76 cases),secondary glomerular diseases(9.2%,7/76 cases)and renal tubular diseases(5.3%,4/76 cases).Fifty patients (65.8%,50/76 cases) had normal level of Hcy which was 10.40(7.30,11.62) μmol/L.Twenty-six patients(34.2%,26/76) were detected with HHcy whose Hcy level was 17.93(16.76,24.11) μmol/L.The detection rate of HHcy in CKD stage 1,stage 2,stage 3,stage 4 and stage 5 was 13.9%(5/36 cases),22.2%(2/9 cases),50.0%(4/8 cases),57.1%(4/7 cases) and 68.8%(11/16 cases) respectively,and the detection rate increased with CKD stages and the difference was statistically significant (χ2=17.574,P<0.001).The level of Hcy was 10.05(7.04,12.47) μmol/L,11.75(10.78,16.44) μmol/L,13.73(10.09,18.23) μmol/L,15.81(11.12,20.71) μmol/L and 17.39(11.86,24.76) μmol/L in CKD stage 1,stage 2,stage 3,stage 4 and stage 5,respectively.The Kruskal-Wallis test revealed that the distribution of homocysteine in CKD stages had statistically significant difference(P=0.001).Multiple linear regression model showed that creatinine clearance was an independent predicator of HHcy.Conclusions In this study of the CKD patients,the detection rate of HHcy was high and increased with the progression of CKD.HHcy is mainly influenced by creatinine clearance in CKD.The level of Hcy should be monitored regularly in children with CKD and HHcy should be treated with proper measures.

Objective To observe the glucocorticoid-induced osteoporosis (GIOP) in children with kidney diseases by quantitative ultrasound (QUS), and to analyze its influencing factors.Methods The tibia/radius bone mineral density(BMD) was checked obtained in 67 cases with childhood kidney diseases treated with glucocorticoid by QUS,BMD was measured in children over the age of 12 by dual-energy X-ray absorptiometry(DXEA) ,and BMD was measured with QUS consistency and different stages of osteoporsis were compared.The clinical data of gender, age,body mass index(BMI) ,administration duration and daily dosage of glucocorticoid were analyzed.The association between the duration of glucocorticoid use,and daily dosage of glucocorticoid and the different degrees of BMD was analyzed by Logistic regression analysis.Results Sixty-seven patients(male 45 ,female 22) were divided into 4 groups according to the reference standard of Asian children BMD data provided by Sunlight Company:the normal BMD group(41 cases), the mild osteoporosis group (18 cases), moderate osteoporosis group (5 cases), and severe osteoporosis group (3 cases).Both QUS and DEXA were highly correlated with BMD in patients measured (P ＞ 0.05).The duration of glucocorticoid treatment and daily dosage of glucocorticoid in 3 abnormal BMD groups were all significantly longer and larger than those of the normal BMD group (all P ＜ 0.05).Correlation analysis showed that BMI was positively correlated with the bone mass of the tibia(r =0.395 ,P ＜ 0.01).The duration of glucocorticoid treatment and daily dosage of glucocorticoid were negatively correlated with those of radius BMD(r =-0.474,-0.381 ,all P ＜ 0.01).Analysis showed that both the duration of glucocorticoid,and the daily dosage of glucocorticoid were the risk factors for GIOP.Conclusions QUS is a better method for evaluating of BMD and diagnosing of GIOP compared with DEXA in children.The daily dosage of glucocorticoid and the duration of glucocorticoid treatment are both the risk factors for GIOP.