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.

Objective To know the prevalence of type 2 diabetes and its risk factors in the elderly in Gushan Town, Fuzhou City. Methods From July to November of 2007, 4653 elderly permanent residents from 14 administrative units were selected using random cluster sampling. Results The prevalence of elderly type 2 diabetes was 11.2%. Non-conditional logistic regression analysis showed that age, occupation, monthly income (OR=1.42, 95%CI: 1.13～1.77), family history of diabetes (OR=6.49, 95%CI: 4.62～9.10), concen- tric obesity (OR=1.47, 95%CI: 1.15～1.88) were independent risk factors for diabetes. Conclusion The type 2 diabetes prevalence rate of the elderly was higher, and the health education and interventions should be strengthened to the people who have family history of diabetes and concentric obesity.

Objective To investigate the possibility of affecting transgenic rescue for Wilson disease using the human ATP7B transgenic LEC rat.Methods The 7.1kb transgene constructed with human ATP7B cDNA and chicken ?-actin promoter was introduced into the fertilized oocytes of LEC rats, an animal model of Wilson disease, by microinjection. The expressions of human ATP7B protein in the transgenic rats were detected by Western blot. The plasma AST and ALT activities, and the total bilirubin levels in transgenic rats were measured continuously from 6 to 16 weeks using non-transgenic rats and LEA rat as controls. The pathological and histochemistry changes in the liver of the transgenic rats at 13 weeks were analyzed. Results The intact and correct product derived from human ATP7B was confirmed in the liver of transgenic rats. At the age around 12 weeks, the plasma AST and ALT activities, and the total bilirubin levels in transgenic rats were significantly decreased, while the inflammatory reation in the liver of transgenic rats was much mild as compared with that of non-transgenic rats, and the granules of stained copper were less in the hepatocytes of transgenic rats. By the age of 16 weeks, the transgenic rats were phenotypically normal, and the survival rate was 100%. These data showed that the LEC rats were successfully rescued from fulminant hepatitis after introducing of human ATP7B gene. Conclusion The hepatitis in Wilson disease is directly related to the toxicity of excessive accumulated copper, which attributed to the functional deficiency of the ATP7B. Gene transfer probably is the effective method for the therapy of Wilson disease.

Objective To investigate the possibility of affecting transgenic therapy for the hepatic cirrhosis and hepatoma in Wilson disease by human ATP7B cDNA. Methods The 7.1*!kb transgene consisting of human ATP7B cDNA and chiken ?-actin promoter was introduced into the LEC rats which is an animal model of Wilson disease by microinjection.The plasma AST and ALT activities in transgenic rats were measured continuously from weeks 17 to 30 using non-transgenic and LEA rats as controls.The histological and histochemistry changes of liver in the transgenic rats at 30 and 60 weeks of age were examined. Results The plasma AST and ALT activities in transgenic rats were kept at the relatvie lower levels from 17 to 60 weeks of age, as compared to the age-matched non-transgenic rats.By the age of 60 weeks,none of the transgenic males developed cholangiofibrosis or hepatoma,whereas all of the non-transgenic rasts had severe cholangiofibrosis at the age of 30 weeks and one male rat had hepatoma at 60 weeks.The transgenic rats were phenotypically normal,and the survival rate was 95.7%.In addition,the distribution and the numbers of the granules of stained copper in the hepatocytes of the transgenic rats did not show any significant difference between 30 and 60 weeks.Conclusion The human ATP7B successfully delayed the onset of hepatic cirrhosis,and suppressed the development of hepatoma in the LEC rats by gene transfer.The hepatic cirrhosis and hepatoma in Wilson disease may be not directly related to the copper accumulation.

Objective To study and explore Glucose-reducing effect of induced human bone mes-enchymal stem cells on diabetic mice.Methods The diabetic model of rats was duplicated by injection of STZ intraperitoneally.The induced cells were implanted into diabetic mice.Blood glucose levels were moni-tored every 3 days after implantation for 14 days.Results The mice receiving the treated Cells began to decrease their blood glucose levels after 3days.But control Animals that did not receive induced cells exhib-ited persistent hyperglycemia.Conclusions Induced cells by hBMSCs can decrease blood glucose levels on diabetic mice.

Diabetes mellitus has become one of the diseases which threaten the heath of human being in the 21st century.A goal of research in diabetes is to find a way to increase the number of functional insulin-producing cells. Islet transplantation has been considered to be the most effective approach to cure type Ⅰ and part of type Ⅱ diabetes mellitus.This approach, however, is severely limited by an inadequate supply of donor islets available for transplantation.Moreover, recent progress of stem cells research has shown that stem cells may act as a new source of islet transplantation in diabetes mellitus treatment. Recent evidence indicates that Glucagon-Like PeptideⅠ(GLP-1) plays a very important role in targeted differentiation of stem cells into Insulin-Producing Cells and pancreatic development. GLP-1 is an intestine-derived insulinotropic hormone that stimulates glucose dependent insulin production and secretion. GLP-1 can induce differentiation of stem cells into insulin-producing cells, which is achieved by up regulation of PDX-1 expression.PDX-1 is a transcription factor critical for pancreatic development and endocrine cell neogenesis and a marker for pancreatic stem cells. These new findings suggest an approach to create Insulin-Producing cells in vitro by expanding stem/progenitor cells and then to convert them into Insulin-Producing cells by treatment with GLP-1. Thus GLP-1 may be a means by which to create Insulin-Producing cells ex vivo for transplantation into patients with insulinopenic type Ⅰ diabetes and severe forms of type Ⅱ diabetes. This article reviews recent progress about GLP-1 and targeted differentiation of stem cells induced by GLP-1.

Word frequency and co-word analysis of key words and comparative analysis of hot spots and topics in core journals-published papers on health policy in China from 2004 to 2008 and from 2009 to 2013 showed that health policy research and health system reform promoted each other and developed with each other, the research tipics on health policy were scattered and expanded rapidly , the focus of researches was more clear with stress laid on their macro-aspects, and the research methods were variable.

After the information construction in grass-root medical and health institutions was considered according to the thinking of Internet+, the action plan for Internet+ in grass-root medical and health institutions was elaborated, and the target of Internet+ and information construction in grass-root medical and health institutions was defined, the information communication-oriented, use-oriented, and users-oriented strategies were put forward for the Internet+ and information construction in grass-root medical and health institutions.

Objective To present a method for breast reconstruction with the superficial inferior epigastric artery (S1EA) flap and to summarize the operative experiences. Methods The diameter and distribution were evaluated with multipledetector-row computed tomography (MDCT) angiography and doppler perfusion flowmeter. Bipedicle superficial inferior epigastric artery flap was designed below umbilicus. Superficial inferior epigastric artery and vein were anastomosed to the internal mammary artery and vein. Results Since 2007, we have used the superficial inferior epigastric artery flap in 4 cases of breast reconstruction. Four flaps survived completely. With the follow-up of 6-12 months, the reconstructed breasts were well-shaped and there were no complications such as abdominal hernia, bulge and weakness in donor sites. Conclusions Breast reconstruction using the superficial inferior epigastric artery flaps can not only preserve the advantages of the traditional method using the deep inferior epigastric perforator flaps, but also retain the maximal function of the fascia and the rectus abdominal muscle and prevent the occurrence of abdominal weakness and hernia. It is an ideal alternative method of breast reconstruction on condition that definitive preoperative assessment of vessels and skilled surgical technique are provided.

Body Height ; Child ; Female ; Ghrelin ; pharmacology ; Growth Disorders ; physiopathology ; Growth Hormone-Releasing Hormone ; pharmacology ; Human Growth Hormone ; blood ; physiology ; urine ; Humans ; Insulin-Like Growth Factor Binding Protein 3 ; blood ; Insulin-Like Growth Factor I ; physiology ; Male