Abnormalities, Multiple ; diagnosis ; pathology ; physiopathology ; Branchio-Oto-Renal Syndrome ; diagnosis ; pathology ; physiopathology ; therapy ; Child ; Deafness ; etiology ; physiopathology ; Diagnosis, Differential ; Ear ; abnormalities ; Female ; Humans ; Kidney ; abnormalities ; Renal Insufficiency ; etiology ; physiopathology ; therapy

Calculi ; chemically induced ; Ceftriaxone ; adverse effects ; Child ; Humans ; Lithiasis ; chemically induced

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.

Liver ischemia reperfusion injury is a common pathophysiological process in hepatic surgery,which is the consequence of multiple mechanisms such as oxidative stress,inflammatory reaction,apoptosis,and autophagy and so on.However,the molecular mechanisms behind these processes have not been completely elucidated,the current treatment of liver I/R injury is merely supportive care,and thus new therapeutic strategies are needed.The recent studies indicate that the ischemic precondition and pharmacological precondition may protect the liver against hepatic ischemiareperfusion injury.It will be an important clinical significance of further studying its molecular mechanisms and protective strategies.

Child ; Humans ; Tuberculosis, Renal

OBJECTIVE: To evaluate the curative effect and clinical significance of bone cement on coagulation functions during percutaneous vertebroplasty in patients with osteoporotic spinal compression fractures.METHODS: A total of 24 patients, comprising 18 females and 6 males, aged 69 years averagely (range 48-83 years), with 44 osteoporotic vertebral compression fractures underwent percutaneous vertebroplasty in Department of Spinal Surgery, Third Hospital of Hebei Medical University between December 2006 and December 2007. The fracture segment was within T_5-L_3 (20 thoracic vertebrae and 24 lumbar vertebrae). Under the guidance of C-arm fluoroscopy, bone marrow biopsy needle was inserted percutaneously via transpedicular way into the fractured vertebrae. Polymethylmethacrylate (PMMA, bone cement) was injected into the fractured vertebrae. The relative parameters were observed in all patients 10 minutes before, 10 minutes, 30 minutes, 1 hour, 2 hours and 3 hours after bone cement implantation, including prothrombin time (PT), activated partial thromboplastin time (APTT), thrombin time (TT), fibrinogen (FIB), plasma protamine paracoagulation test (3P test), and D-dipolymer (D-D). RESULTS: PT was decreased, and FIB, 3P test, D-D were increased 10 minutes after bone cement implantation in percutaneous vertebroplasty peaked at 1 hour and gradually decreased afterward; moreover, there were significant difference between bone cement preimplantation and 10 minutes, 30 minutes, 1 hour, 2 hours and 3 hours after bone cement implantation (P < 0.05), but no difference was observed in APTT and TT (P > 0.05). The influence of bone cement on the parameters was vanished in 3 hours after bone cement implantation, and all indexes were similar to pre-implantation (P > 0.05).CONCLUSION: Bone cement implantation causes temporal hypercoagulabale state in percutaneous vertebroplasty. It is important to monitor blood clotting state in 3 hours after bone cement implantation in order to avoid thrombus disease.

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.

AIM: To investigate the effect of mfn2 on mitochondrial function in steatosis hepatocytes. METHODS: Plasmid pEGFP-mfn2 was transfected into hepatocyte strain L02 by Lipofectamine 2000 in vitro, then the steatosis model of hepatocytes was establish by oleic acid induction. RT-PCR was used to evaluate mRNA expression and Western blotting was use to detect the protein expression. ATP level was determined by firefly luciferase bioluminescent. ROS production was measured by fluorescence probe DCFH-DA. Chondrosome transmembrane potential of L02 was observed by labeling of JC-1 and FCM. RESULTS: The stable expression of ectogenesis mitofusin2 in L02 cells was confirmed by RT-PCR and Western blotting. In the model of oleic acids induced lipid formation, Mfn2 obviously inhibited the descent of chondrosome transmembrane potential and ATP level, and increased ROS production in L02 cells. CONCLUSION: Up-regulated expression of mfn2 attenuates mitochondria dysfunction caused by oleic acids induced lipid formation.

Protein is the executor of physiological function, and direct embodiment of the life phenomena. Proteomics aims to systematically clarify all or parts of proteins' role and function in life movement. In post genome era, proteomics began to play more important role in life science field. Actinobacteria are closely linked to human production and life, which have produced many clinically important secondary metabolites, including antibiotics, antitumorals and enzymes. Actinobacterial systematics and its model organism Streptomyces coelicolor in 2001 genome sequence laid the foundation for further functional genomic studies. Actinobacterial proteomics was more directly and exactly to interpret the activity of life than genomics and transcriptomics, which grew much faster and received so much attention from scientists in the near years. Complex morphological differention, stronge environment adaptiveness, nitrogen-fixing capacity, metabolic mechanism, pathogenicity and natural produces' discovery were systematically reviewed in this study, which was expected to be the basis for promoting Actinobacterial proteomics study in the near future.
Actinobacteria ; genetics ; metabolism ; Genomics ; Proteomics ; Streptomyces coelicolor ; genetics ; metabolism