Objective To explore the clinical features, diagnosis, and treatment of childhood fibrillary glomerulonephritis (FGN). Methods The clinical data of a child with FGN in April 2016 were analyzed retrospectively. Results A 12-year-old boy, who presented significant proteinuria (mainly albumin), hypoalbuminemia, hypercholesterolemia, and persistent microscopic hematuria in May 2010, met the criteria of nephrotic syndrome. Renal biopsy in May 2010 showed mesangial proliferative glomerulonephritis combined with glomerulosclerosis. It was not effective by treatment with intravenous infusion of methylprednisolone and prednisolone, and there were no responses by the combination with mycophenolate mofetil and traditional Chinese medicine. After admission, the second renal biopsy was performed. Under the light microscope, the moderate mesangial proliferative glomerulonephritis combined with membranoproliferative changes was observed. Under the electron microscope, the FGN was confirmed. Conclusion The first case of childhood FNG was diagnosed in China.

Objective To explore the rare cause of renal failure in childhood IgA nephropathy.MethodsA six year-old boy presented with recurrent gross hematuria for 3 months and increased serum creatinine for 5 days, blood and urine routine test, renal function, urinary protein concentration and renal biopsy were performed for diagnosis.Results The boy had three episodes of recurrent gross hematuria with a predisposed respiratory tract infection, he recovered within a week after antibiotic therapy from previous two episodes, but oliguria and renal failure were occurred in the third episode. Renal biopsy showed IgA nephropathy with presence of red blood cell casts in as much as 50% of the tubular lumen and acute tubular lesion. His renal function recovered gradually to normal within 4 weeks after treatment with anti-infection, diuresis and alkalization of urine. Conclusions This article reported the renal failure case induced by tubular damage and obstruction by red blood cell casts in childhood IgA nephropathy.

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 etiology and prognosis of Dent disease combined with renal failure in children. Methods The clinical data of 2 children with Dent disease combined with renal failure from January 2014 to December 2016 were analyzed and the related literature was reviewed. Results Both of them were male, with the age of 8 and 10 years old respectively. Their renal functions were normal, and no renal calcification. Both of them had the history of upper respiratory tract virus infections within 1 week before the onset of renal failure. In case 1, acute phase (10 days) renal biopsy showed combined with acute tubulointerstitial nephritis, and his renal function recovered completely after glucocorticoids treatment. In case 2, renal biopsy at 6 months in course of disease showed the combined with subacute tubulointerstitial nephritis, and his renal function was improved partly after glucocorticoids treatment. Conclusions For children with Dent disease combined with acute renal failure, especially with upper respiratory tract virus infections and other inducement, renal biopsy should be early performed to exclude the possibility of acute tubulointerstitial nephritis, so that the treatment can be timely conducted and the prognosis can be improved.

Objective: Hepatocyte nuclear factor 1 homeobox b (HNF1B) -associated disease is an autosomal dominant inherited disorder with a variable, multi-systemic phenotype. In China, five adult probands and one child proband with HNF1B-associated disease had been reported, whereas few fetuses are described. The aims of this retrospective study were to understand about the clinical manifestations of HNF1B-associated disease and to further improve the recognition of this disorder. Method: Four patients (3 males, 1 female) and three fetuses with HNF1B mutations were included in this study. They were admitted to our hospital from January 2013 to March 2017. HNF1B mutations were detected using targeted next generation sequencing and quantitative real-time PCR or Sanger sequencing. HNF1B heterozygous deletion of exons 1-9 was found in 4 patients and 2 fetuses, and HNF1B heterozygous missense mutation in 1 fetus. These two mutations had been reported. Two patients and 1 fetus had de novo mutations. Results of renal ultrasonography with or without magnetic resonance imaging, biochemical investigations, urine routine examination and other necessary investigations in 7 cases were analyzed. Result: Three patients were Han Chinese ethnicity, and one patient was Mongolian. In patients 1 and 4, abnormal fetal kidneys were discovered by routine ultrasonography, and the age at first feature identified in Patients 2 and 3 were 13 years and 28 years. Patient 3 had normal renal function and the remainder had reduced glomerular filtration rate. In addition, patient 4 presented with nephrotic syndrome and glycosuria, patient 2 with early onset hyperparathyroidism and renal osteodystrophy, and patient 3 with diabetes mellitus. All the 4 patients had renal structural abnormalities including bilateral multiple renal cysts, dysplasia and hyperechogenic kidneys. Only patient 3 had a positive family history of renal diseases, the remainder had a negative family history of renal diseases. In 3 fetuses, prenatal ultrasound anomalies were detected during the second trimester. These 3 fetuses had hyperechogenic kidneys with or without renal cysts. Polyhydramnios was detected in only one of the 3 fetuses. Two of the 3 fetuses had a positive family history of renal diseases. Conclusion Clinical phenotypes of HNF1B-related disease are heterogeneous, renal malformations clearly appear to be the most common manifestation, multiple renal cysts are characteristic, and patients can progress to impaired kidney function during childhood; HNF1B mutation is a differential diagnosis of fetal hyperechogenic kidneys or multiple renal cysts.