Several insulin resistance syndromes, such as severe insulin resistance syndrome caused by mutations of insulin receptor gene and lipodystrophy caused by mutations of lamin gene, have been characterized clinically. The gene mutations, pathogenesis and clinical features of these monogenie diseases were reviewed in this article.

The extensor tendon rupture is a common disease.Traditional management includes "8" suture and cross suture.But second operation is commonly needed for the reason of postoperative Nodules or adhesion.So a new technique is invented by the author:micro-weave suture combined with sodium hyaluronate.The new technique has improved dynamic intensity of the tendon and avoided postoperative adhesion.In this paper,this technique is reviewed.

Children avascular necrosis of femoral head is a self-healing,self-limiting and non-systematic disease occouring locally in femoral head of children.The purpose of traditional conservative and surgical treatments is to improve blood circulation,decrease intraosseous pressue,decrease arthrosis pressure,reduce capital mechanical pressure to the femoral head and increse its containment.Anyway,no treatment could stop the progress of coxa plana and degeneration of the bone,which could lead to disorder of the hip.As a new technique,autologous bone marrow stem cellular transplantation has obtained preliminary curative effect.It has been proved to be a new effective treatment of children avscular necrosis of the femoral head with combined use of tissue enginering bone.

Objective To investigate and compare the clinical efficacy of mifepristone alone and combined with Gongliuxiao capsule in the treatment of uterine fibroids.Methods 106 patients with uterine fibroids in Harrison International Peace Hospital from December 2012 to December 2013 were selected.The patients were randomly divided into control group and experimental group(n=53).The former were treated with mifepristone alone while the later were treated with the combination of mifepristone and Gongliuxiao capsule.All the patients were given 2 courses of treatment with 12 weeks for a course.The menstrual phase,volume of menstruation,uterus and the volorne of uterine fibroids before and after the treatment,and the clinical efficacy of both groups were evaluated.All the results were recorded and compared. Results The menstrual phase,volume of menstruation,uterus and uterine fibroids of both groups after the treatment were significantly less than those before treatment (P <0.05 ).And the total effective rate of experimental group was 92.45%,which was significantly higher than 73.58% of control group.Both groups had some mild adverse reactions,which didn’t affect the treatment.Conclusion The combination of mifepristone and Gongliuxiao capsule has a great efficacy in the treatment of uterine fibroids.Compared with the use of mifepristone alone,the combination treatment is much more effective.

The dissolution rates of the cephalexin tablets made by five t actories in China were determined with rotating basket method. The dissolution constants Ki were calculated using single exponential method. The analysis of variance was carried out for Ki. The results showed that constants Ki of products from different factories were significantly different (p

Objective:To explore the types and characteristics of the grief reactions of adolescents to friends'death repeatedly,so as to provide reference for effective psychological intervention. Methods:Three college students aged nineteen and twenty years who experienced the death of friends at least twice in adolescence participa-ted in the study by means of an open recruitment in a college. They had friends died at the age of 9 and 12. And then it happened again when they were 15 and 17. Data was collected by semi-structured interviews with the three selected,and analyzed by thematic analysis to classify the grief reactions after the loss of their friends. Results:The common grief reactions of a friend's death in the interview are:shocked,regret and angry,denying,disbelieving, fear of the death,feeling the cruelty of death,crying,searching for support,shirking and recognizing the cruelty of death. The special grief reactions in the interview,especially when they experienced the second death of a friend are:caring for the dead loved ones,having a mental shadow over friend's role,reducing social activities,seeking stimulating and venting,revolting against the school,violence,and working harder. Conclusion:The grief reactions of adolescents to friends'death repeatedly are diversity. It presents the characteristics of the psychological develop-ment of adolescence. And the adolescents need to be given special attention and appropriate psychological interven-tion.

Isolated superior mesenteric artery dissection (ISMAD) is a clinically common disease,of which multi-slice spiral computed tomography (MSCT) is effective method for screening and follow-up.The diagnosis and treatment should be made based on pathological changes of true or and false lumen.Most patients can relieve by medical treatment,and those with treatment failure can adapt endovascular treatment.Patients with aneurysm rupture or intestinal necrosis should undergo emergent surgical treatment.

Objective To evaluate the effiicacy of nasotracheal intubation using blind tracheal intubation device and end-tidal carbon dioxide monitoring technique in patients with difficult airway.Methods Sixty ASA Ⅰ or Ⅱ patients of both sexes,aged 35-60 yr,weighing 55-75 kg,requiring nasotracheal intubation,undergoing selective oral and maxillofacial surgery,were involoved in this study.All of them were expected to have difficuh airway such as mouth opening ＜ 3 cm,neck upward degree ＜ 30° and Mallampati classification Ⅲ or Ⅳ,The patients were randomly divided into 2 groups(n =30):group Ⅰ received tracheal intubation with the blind tracheal intubation device and group H received tracheal intubation with the blind intubation device combined with end-tidal carbon dioxide monitoring technique.The number of successful intubation,the inlubation time and adverse effects during intubation(epistaxis,tachycardia,hypertension and hypoxemia)and after operation(sore throat and hoarseness)were recorded.Results The rate of successful intubation in the two groups was 100％.Tachycardia,hypertension,hypoxemia and hoarseness were not found in the two groups.Compared with group I,the rate of successful intubation at first attempt was significantly increased,the intubation time was significantly shortened,and the incidence of epistaxis and sore throat was decreased in group Ⅱ(P ＜ 0.05).Conclusion The blind intubation device combined with end-tidal carbon dioxide monitoring technique used in diffficult airway can shorten the intubation time,increase the success rate of tracheal intubation,and decrease the occurrence of adverse effects.

Clinical practice guidelines have been widely applied worldwide.Compared with the developed countries,however,the development of guidelines still has many limitations in China,which is mainly due to the lack of a guideline for guiding this process.Such a guideline,defined as “a structured and coordinated programme designed with the specific aim of producing several clinical practice guidelines should be able to specify the development,dissemination,implementation,evaluation,and update of each clinical practice guideline.Furthermore,the lack of high-quality randomized controlled trials and cohort studies also restricts the development of high-level guidelines.After the development of such a guideline for developing guidelines,we can expect the faster development of Chinese clinical practice guidelines that are more carefully designed,more reliable,and more practical.

Clinical practice guidelines are systematically developed clinical multi-discipline advisory statements which assist providers, recipients and other stakeholders to make informed decisions about appropriate health interventions. Chinese government and some clinical academic communities began to develop the domestic guidelines after 2000. Nevertheless, there were still some shortcomings presented in the domestic guidelines if assessed by the advanced appraisal instrument of the developed countries. There were four aspects as follow. The first, misunderstood the guidelines are the rule or text book. The second, ignored presentation of the procedure about development of guidelines. The third, there were a lack of high level evidences. The fourth, not declared editorial independence. However, it is necessary to develop the domestic guideline of China. With rising conflicts between the increasing needs for health care and shortage of our environment resource, development of the guidelines become one of the key steps in the process of clinical practice.