Objective To analyze the diagnostic approach on hepatolenticular degeneration combined with thin basement membrane nephropathy.Methods A girl presented with microscopic hematuria, liver dysfunction and hypocomplementemia was diagnosed with hepatolenticular degeneration combined with thin basement membrane nephropathy, her clinical data were summarized and analyzed retrospectively.Results A ten years old girl presented with microscopic hematuria and liver dysfunction for a year, dysarthria for a month, and combined with hypocomplementemia but without proteinuria. Renal biopsy showed thin basement membrane nephropathy. Ceruloplasmin was 23.10 mg/L and urinary copper concentration was 120μg, respectively, ocular slit lamp examination showed Kayser-Fleischer ring, cranial MRI showed preternatural signal in both basal and putamen nucleus, mutation analysis showed homozygous mutations in ATP7B and heterozygous mutation in COL4A3 gene,respectively.Conclussion Hepatolenticular degeneration should be suspected in those cases with persistence microscopic hematuria, liver dysfunction and hypocomplementemia.

Objective To explore the clinical features of steroid resistant nephrotic syndrome caused by NPHS2 gene mutation. Methods The clinical data of two pediatric patients with steroid resistant nephrotic syndrome were retrospectively analyzed. The pertinent literatures were reviewed. Results Both patients were male with onset age at 2 and 3 years old. The clinical features were heavy proteinuria, hypoalbuminemia, and hypercholesterolemia, which met the diagnostic criteria of nephrotic syndrome. Renal pathology found one patient with focal segmental glomerulosclerosis, and other with minimal-change. Both of them suffered from recurrent inguinal hernioplasty and one was accompanied with hypoplasia of left testis. Gene detection verified a NPHS2 gene mutation. Both of them were hormone resistant at the beginning of onset and later hormone combined with different kinds of immunosuppressive therapy was still ineffective. Both of them entered the end-stage of renal disease 3 years after onset. Conclusions For male pediatric patients with steroid resistant nephrotic syndrome, combined with non-renal manifestations such as multiple hernia or testicular abnormalities, the possibility of the hereditary nephrotic syndrome caused by NPHS2 mutations should be considered.

Objective To explore the diagnosis and differential diagnosis of IgA nephropathy.Methods The clinical data of 2 children with IgA nephropathy were retrospectively analyzed.The pertinent literatures were reviewed.Results In 2 males aged 6 and 7 years,the clinical features were a large amount of proteinuria (mainly albumin),low serum albumin,high cholesterol,and persistent microscopic hematuria,which were in line with the diagnosis of nephrotic syndrome.The effects of hormone and immunosuppressive therapy were poor.Renal pathology immunofluorescence and light microscopy findings were in accord with mild to moderate mesangial proliferative IgA nephropathy (M1E0S0T0).Electron microscope showed glomerular basement membrane lesions (layering,breakage,and uneven thickness),which could not exclude Alport syndrome.Further gene detection confirmed a pathogenic mutation of COL4A5.Conclusions It is rare that IgA nephropathy is combined IgA nephropathy at the same time.Attention should by paid to those who had a poor effect of treatment or had a related family history in IgA patients because it is possible that IgA nephropathy and IgA nephropathy may occurred at the same time.

Objectives To explore the effects of gross hematuria on the results of several parmeters in laboratory urine examination. Methods Eighty (80) children with IgA nephropathy and 40 cases with acute post-streptococcal glomerulonephritis hospitalized in our hospital from January 2014 to December 2015 were recruited. The ratio of urinary calcium and protein to creatinine, quantitative test of 24 h urinary calcium and protein, quantitative test of 24 h urinary albumin,α1-microglobulinuria, microalbuminuria and urine protein electrophoresis were tested during and after the gross hematuria, respectively. Results The ratio of urinary calcium and protein to creatinine, quantitative test of 24 h urinary calcium and protein were much higher in the duration of gross hematuria as compared to those after the duration of gross hematuria, while α1-microglobulinuria, microalbuminuria and quantitative test of 24 h urinary albumin showed no difference between the two periods. Conclusions Gross hematuria could increase the level of urinary calcium and protein, while quantitative test of 24 h urinary albremin is not affected.

Neural crest stem cells originated from hair follicle (epidermal neural crest stem cell, EPI-NCSC) are easy to obtain and have potentials to differentiate into various tissues, which make them eminent seed cells for tissue engineering. EPI-NCSC is now used to repair nerve injury, especially, the spinal cord injury. To investigate their effects on repairing peripheral nerve injury, EPI-NCSC from a GFP-SD rat were primarily cultured on coated dishes and on a poly lactic acid coglycolic acid copolymer (PLGA) membrane. Methyl thiazolyl tetrazolium (MTT) assay showed that the initial adhesion rate of EPI-NCSC was 89.7% on PLGA membrane, and the relative growth rates were 89.3%, 87.6%, 85.6%, and 96.6% on the 1st, 3rd, 5th, 7th day respectively. Cell cycles and DNA ploidy analysis demonstrated that cell cycles and proliferation indexes of cultured EPI-NCSC had the same variation pattern on coated dishes and PLGA membrane. Then cultured EPI-NCSC were mixed with equal amount of extracellular matrix and injected into a PLGA conduit to connect a 10 mm surgery excision gap of rat sciatic nerve, Dulbecco's Modified Eagle's medium (DMEM) was used to substitute EPI-NCSC in the control group. After four weeks of transplantation, the defected sciatic nerve achieved a histological restoration, the sensory function of rat hind limb was partly recovered and the sciatic nerve index was also improved. The above results showed that a PLGA conduit filled with EPI-NCSC has a good repair effect on the peripheral nerve injury.
Animals ; Cells, Cultured ; Neural Crest ; cytology ; Neural Stem Cells ; cytology ; Rats ; Rats, Sprague-Dawley ; Sciatic Nerve ; pathology ; Spinal Cord Injuries ; Stem Cell Transplantation ; Tissue Engineering

ObjectiveTo investigate the influence of olsalazine on tumor necrosis factor-alpha (TNF-α) and interleukin-10 (IL-10) in patients with chronic ulcerative colitis.MethodsSixty patients with chronic ulcerative colitis(observation group),including 22 chronic recurrent cases and 38 chronic persistent cases,were enrolled and treated with olsalazine.Meanwhile,60 healthy volunteers without disease history of ulcerative colitis were selected as control group.The concentrations of TNF- α and IL-10 in serum of the two groups were detected by ELISA and compared.ResultsBefore treatment,the concentration of TNF- α in serum of the observation group was significantly higher than that of control group [ (57.2 ± 10.1 )ng/L vs.(27.2 ± 6.9) ng/L],while IL-10 was significantly lower than that of control group[ (9.2 ± 2.1 ) ng/L vs.(17.3 ±2.9) ng/L] (P ＜0.05).Before treatment,the concentration of TNF-α in serum in chronic persistent patients and chronic recurrent patients[ (56.9 ± 9.9),(57.3 ± 9.7) ng/L ] were significantly higher than that in control group,and serum IL-10 in chronic persistent patients and chronic recurrent patients [ (9.1 ± 2.3 ),(8.4 ± 2.5 ) ng/L ] was significantly lower than that in control group (P＜ 0.05 ).The concentration of TNF- α in serum in observation group after treatment was obviously lower than that before treatment [(28.1 ±8.9) ng/L vs.(57.2 ± 10.1 ) ng/L],and IL-10 was obviously higher than that before treatment [(13.4 ± 10.7) ng/L vs.(9.2 ±2.1 )ng/L] (P ＜ 0.05).The concentration of serum TNF-αand IL-10 in chronic persistent and chronic recurrent patients before and after treatment had statistical significance (P＜0.05 ).ConclusionsOlsalazine can significantly decrease the concentration of TNF- α and increase the concentration of IL-10 in serum in patients with chronic ulcerative colitis.It is worthy of application in clinic.

Objective To investigate the expression of 14-3-3 β in esophageal squamous cell carcinoma (ESSC) tissue,and to explore the relationship between 14-3-3 β and clinicopathological factors,prognosis.Methods The 14-3-3 β expressed in tumors and adjacent normal epithelia from 78 esophageal squamous cell carcinoma patients was detected by immunohistochemical staining.Results The mild and strong expression in the nucleus of esophageal squamous cell carcinoma and adjacent tissues was 62.8％ (49/78) and 17.9％ (14/78),there was significant difference (P ＜ 0.01).The mild and strong expression in the cytoplasm of esophageal squamous cell carcinoma and adjacent tissues was 69.2％ (54/78) and 29.5％ (23/78),there was significant difference (P ＜ 0.01).The 14-3-3 β was associated with the differentiation degree of esophageal squamous cell carcinoma(P ＜ 0.01).It had nothing to do with sex,age,TNM stage,size and survival rate (P ＞ 0.05).Conclusions The expression of 14-3-3 β is significantly up-regulated in esophageal squamous cell carcinoma patients.The expression of 14-3-3 β in the nucleus and cytoplasm is higher and has positively associated with tumor differentiation degree.

Objective To observe the expression of brain-derived neurotrophical factor (BDNF) in injury spinal cord after transplantation olfactory ensheathing cells (OECs), and to investigate the mechanism of OECs repairing spinal cord injury.Methods OECs from GFP transgenic rats were separated and cultured for transplantation. Spinal cord injury rats were separated two groups by random digits table. In experimental group, OECs suspension were transplanted into injured spinal cord following spinal cord injury. In control group, DMEM was transplanted into the injured spinal cord after spinal cord injury. Motor function was evaluated per week after transplantation. The expression levels of BDNF mRNA and protein were detected by using RT-PCR and immunohistochemistry respectively, and compared with those from normal SD rats.Results Motor function of two groups was improved gradually after transplantation. The motor function scores in experimental group was obviously higher than in control group at 21st day after transplantation (P<0.05). A lot of survival GFP OECs distributed around impaired myeloid tissue. At 21st day after transplantation, BDNF mRNA and protein expression in experimental group were strongest (P<0.05), and stronger in control group than in normal group (P<0.05).Conclusion The transplantation of OECs can repair the injured spinal cord by increasing the expression of BDNF mRNA and protein to improve local microenvironment.

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 observe the migration and distribution of OECs in injured spinal cord and discuss their relation with the recovery of spinal cord function. Methods The rats were contused by a force of 10 g · 25 mm with NYU-impactor at T10 level. The OECs acutely isolated from green fluorescence protein (GFP) rats were purified, identified and then transplanted into the injured site and the rostral and caudal parts of the spinal cord one week after injury, with total volume of the transplanted OECs for 90 000/μl. Within 13 weeks after transplantation, the migration and distribution of OECs were qualitatively observed on the cryo-sections under fluorescence light microscope. The area and the length of OECs distribution were semi-quantitatively determined. The locomotor function of the spinal cord was appraised by BBB score. Results OECs were located collectively in the transplanted site at early stage after transplantation and then spread gradually mainly along the long axis of the cord. OECs could be found in the cavity of the contused spinal cord. The area and the length of OECs distribution were increased from 1.33 mm2 and 4.23 mm respectively at one week to 3.30 mm2 and 7.68 mm respectively at 13 weeks after transplantation. In the meantime, the locomotor function was gradually improved. Conclusion OECs can migrate within the contused spinal cord, as may contribute to the recovery of locomotor function.