Objective To explore the clinical characteristics and treatment of methylmalonic acidemia(MMA) in order to improve our understanding of it.Methods We analyzed the clinical manifestations,laboratory examinations and treatments of 26 cases of methylmalonic acidemia in children.Results Twenty-four cases were involved in nervous system.Nine patients were involved in renal system.Eight cases of hematological involved.Liver enzyme elevated in 2 cases as well as the cardiac system were involved in 3 cases.One case was with pneumonia onset.The laboratory findings showed metabolic acidosis in 12 cases,hyperhomocysteinemia in 8 cases and remarkable elevation of urinary methylmalonic acid concentration in all cases.Some abnormalities in globus pallidus and cerebral white matter as well as diffuse cerebral atrophy were noted by the brain CT and MRI in 15 cases.Sixteen children have received therapy of vitamin B12,and supplementation of L-carnitine with restricted-protein diet.The follow-up for a period ranging from 3 months to 1.5 years( mean 8.5 months) of 15 cases with medical therapy showed a favorable outcome of nervous system improvement in 12 cases,however,2 patients died from severe metabolic acidosis.8 patients with renal involvement were normal in urine routine and renal function.Conclusion Methylmalonic acidemia has different clinical features,so early urine organic acids analysis by GC/MS method is essential Long-term and reasonable treatment after diagnosis is an effective way to improve the prognosis.

Intrauterine infection is an important risk factor for neonatal brain damage and neurological dysfunction. Viruses, bacteria, and protozoa can cause intrauterine infection which results in neonatal brain damage. The inlfammatory response is an important pathogenic factor for neonatal brain damage caused by intrauterine infection. Intrauterine infection in different periods of pregnancy might cause different types of brain damage in neonates. Clinicians should pay attention to the prevention of intrauterine infection during pregnancy. It is necessary to further strengthen the clinical and basic research to explore effective interventions for neonatal brain damage caused by intrauterine infection.

Objective To investigate the clinical curative effect of titanium microplate to repair and fix obsolete cracky orbital fracture.Methods According to the diagnosis of CT scanning and three-dimensional imaging, 20 cases of obsolete orbital fractures were repaired and fixed by using titanium microplate along fracture lines. The microplates were placed according to the part nad shape of fractures. For the part of comminuted fractures, the two ends of fractures were fixed like a bridge. Results After the repair and fixation of titanium microplate, facial deformity became recuperative completely, eye-ball-movement and mastication function were recovered. During 6~12 months follow-up period, no reject reaction or cracking or dropping of microplate occured.Conclusions The titanium microplate can make orbital fractures rigid and internal fixed, and the procedure is simple and easy mastered. Therefore, it is one of the most effective materials in the repair and fixation of orbital and facial fractures.

Objective To observe the effect of angiotensin Ⅱ receptor blockers(ARBs)on peroxisome proliferator-activated receptor gamma(PPAR-γ)and to explore its mechanism for improving glucose and lipids metabolism.Methods Dual-luciferase gene reporting system was used to reflect PPAR-γ promoter activation by irbesartan and telmisartan.RT-PCR was used to reflect PPAR-γ mRNA by activation of irbesartan and telmisartan.Results Irbesartan and telmisartan may increase COS-7 cells PPAR-γ promoter expression and increase 3T3-L1 cells PPAR-γ mRNA expression in dose and time-dependent manners.Conclusions ARB may activate PPARs system to play a role in improving the glucose and lipids metabolism

OBJECTIVE: To observe the effect of Shenqi Recipe on the proliferation of glomerular mesangial cells (MC) and interstitial fibroblasts (FC) in rats. METHODS: The effect of serum containing prepared drugs from normal and chronic renal failure rats treated by Shenqi Recipe on glomerular mesangial cells and intersititial fibroblasts in vitro was observed by MTT colorimetric assay and serophrmacology. RESULTS: Compared with the blank sample normal rats, serum containing Shenqi Recipe inhibited the proliferation of MC and FC (P<0.001), and the serum containing Shenqi Recipe of CRF rats had significantly inhibited the proliferation of MC and FC compared with the model group(P<0.001). Conclusion Shenqi Recipe can postpone CRF progress by this mechanism.

Angiogenesis is a fundamental physiological and pathological process in human body.Angiogenesis is regulated by integrins,a family of cell surface receptors.After binding with their ligands such as extracellular matrix proteins and immunoglobulin superfamily molecules,integrins can promote the proliferation and migration of endothelial cells during angiogenesis.Several integrin-targeted therapeutic agents are currently used in clinical trials for the therapy of angiogenesis related diseases.Here,there will show the roles of integrins in angiogenesis and clinical application.

Objective To explorer the effect of angiotensin type 1 receptor blockers (ARB) on activating peroxisome proliferator-activated receptor (PPAR) ? and ?.MethodsLuciferase gene reporters of PPAR? and PPAR? were constructed in COS-7 cells.The cells were then cultured with various concentrations (0,0.01,0.1,1,10 and 100 ?mol/L) of valsartan,losartan,irbesartan or telmisartan for 12,24,36,48 and 60 hours;or co-cultured with PPAR? antagonist GW9662 (10,30?mol/L) for 1 hour,and then cultured with various concentrations (0,1 and 10?mol/L) of irbesartan or telmisartan for 12,24,36,48 and 60 hours.The peroxisome proliferator-response element (PPRE) luciferase activity was determined by Dual-Luciferase Reporter Gene Assay system.3T3-L1 cells were induced to differentiate into adipocytes,and then co-cultured with 10?mol/L of valsartan,losartan,irbesartan or telmisartan for 24 hours,and the expressions of PPAR? and PPAR? mRNA and protein were detected by RT-PCR and Western blotting respectively.ResultsStimulation of valsartan and losartan didn't increase the transcriptional activities of PPAR? and PPAR? in COS-7 cells,while of irbesartan and telmisartan significantly increased the transcriptional activity of PPAR? and PPAR? in COS-7 cells.After co-cultured with 100 ?mol/L of irbesartan or telmisartan for 48 hours,the PPAR? transcriptional activities reached their peak values (43.3?13.0 and 47.8?11.8 respectively),and were significantly higher than that of control group (4.3?0.5,P

Objective: To investigate whether antipsychotic agent-induced type 2 diabetes was associated with leptin gene -2548G/A functional polymorphism in schizophrenia patients who had treated by antipsychotics chronically. Methods: Fasting plasma glucose, 2-hour post meal plasma glucose, fasting plasma leptin and fasting plasma insulin were measured in 110 patients with chronic schizophrenia whose natural courses of the disorder were more than 5 years. According to the level of plasma glucose, these patients were divided into three groups, diabetes mellitus group, abnormal regulating glucose group and normal glucose group. The polymorphisms in the leptin promoter region (-2548 G/A) were determined with PCR-RFLP in the patients. Results: The patients in DM group were more likely to be homozygous for the -2548AA genotype. As compared with the patients with -2548AG/GG combined genotype, the patients with -2548AA genotype had significant increase in the level of plasma leptin(P=0.019). Conclusion: The -2548G/A polymorphism in promoter region of leptin gene may be associated with type 2 diabetes in schizophrenic patients treated by antipsychotics chronically. -2548A allele of leptin gene may be a genetic risk factor for the development of type 2 diabetes during antipsychotic treatment.

Objective To examine the role of synthetic cyclic citrullinated peptide (CCP) in T cell autoreactivity in rheumatoid arthritis (RA) by detecting T cell proliferation. Methods Twenty-nine RA, 8 primary Sjo gren syndrome (pSS) and 16 osteoarthritis (OA) patients were included in this study. Proliferation of T cells was measured by methyltetiazolium incorporation following incubation with CCP. Results T cell response to CCP was more common in RA patients than in controls. The response rate to CCP was higher in late RA than in early RA. However, the response wasn′t associated with disease activity. A strong association between T cell proliferation and RF, anti-CCP antibody was observed. Conclusions T cell proliferation in response to CCP was found in the majority of RA patients, even in early RA, suggesting that the citrullinated peptide play an important role in the pathogenesis of RA.