Congenital adrenal hyperplasia (CAH) owing to steroid 21-hydroxylase deficiency (21-OHD) was a relatively frequent of autosomal recessive disorders characterized by the inactivation of the steroid-synthesizing enzyme in the adrenocortex.Corticosteroids (glucocorticoids and mineralocorticoid) replacement therapy was the primary treatment of 21-OHD.The main objective of 21-OHD treatment in children was to maintain normal growth.Inadequate or excessive treatment was commonly observed.A number of studies reported that 21-OHD adult were at increasing risk of developing metabolic syndrome and cardiovascular events.However,there was few researches on 21-OHD children with metabolic disorders,and no domestic reports.The article summarized recent clinical research progresses in research on the alterations of lipid and carbohydrate metabolism in children with classic 21-OHD.

Objective To compare the prognosis efficacy between total laparoscopic gastrectomy and or-dinary gastric cancer for familial gastric cancer.Methods The selected 60 patients with ordinary gastric cancer from August 2012 to 2014 in our hospital as the control group,the same period in familial gastric cancer of 40 pa-tients were chosen as control group.All patients were performed by total laparoscopic gastrectomy.Results The operative time,blood loss,postoperative discharge time compared in the two groups were showed no significant differences.However,the postoperative hospital stayed in the observation group and the number of lymph nodes were significantly longer more than the control group( P<0.001) .The postoperative pulmonary infection,intesti-nal obstruction,abdominal infection,anastomotic leakage,anastomotic bleeding and other complications in the ob-servation group were significantly lower than in the control group( P<0.001) .After the investigation,the dyspha-gia,pain,anxiety,physical appearance,dry mouth scores in the observation group were significantly higher(P<0. 001).Conclusion Familial gastric cancer has unique clinical and pathological features,Compared to ordinary stomach,the total laparoscopic gastrectomy treatment of familial gastric cancer need more lymph nodes dissection, it can cause more complications,it has some impacts on the quality of life for patients.

Objective To investigate the adverse reaction of gastric cancer patients after inquiry application of bevacizumab combined with chemotherapy,and put forward some corresponding preventive measures.Methods Sixty-five cases patients with gastric cancer who were treated with advanced gastric cancer radical operation application of bevacizumab in combination with chemotherapy in the Central Hospital of Jiangmen from January 2012 to January 2014,including 41 male,24 female,with the average age of 48.7 years old.The adverse reactions were observed,and give the corresponding prevention and control measures on the basis of the adverse reaction conditions,the experience in the prevention and treatment were summarized according to the therapeutic effect with clinical and related literature.Results The adverse reactions caused by bevacizumab combined with chemotherapy in patients with gastric cancer including proteinuria (a total of 24 cases(37％),including 18 cases(27.8％) of (+),6 cases(9.2％) of (+ +)),gastrointestinal reaction(a total of 10 cases (15.4％),including 8 cases (12.3 ％) of grade Ⅰ,2 cases (3.1％) of grade Ⅱ),thrombosis (a total of 20 cases(30.7％),including 13 cases of grade Ⅰ (20％),7 cases(10.7％) of grade Ⅱ) and hypertension(a total of 11 cases(16.9％),including 9 cases(13.8％) of grade Ⅰ,2 cases(3.1％) of grade Ⅱ).Bevacizumab combined with chemotherapy in the treatment of gastric cancer patients with partial remission in 41 cases (63.1％),10 cases (15.4％) of stable disease progression,14 cases (21.5％) of disease progression,the complete remission rate was 63.1％.For adverse reactions of proteinuria,patients with asymptomatic proteinuria,if patients with urinary protein detection results for (+),without special treatment,when patients with urinary protein detection for (+ +),given the observation,if patients with urinary protein was more than or equal to 2 g/24 h,paused bevacizumab until proteinuria＜2 g.When the patients with digestive system damage,such as ALT and AST elevation,given the treatment of liver until the two hepatic enzymes returned to normal;patients with gastrointestinal reactions were nausea and vomiting,asked patient to light diet and regular esophagoscopy,if the patients appeared visceral fistula immediately.stopped bevacizumab.For patients with thrombosis,should be closely monitored in patients with signs,once found regardless of any level of embolism events,all immediately stopped the chemotherapy drugs.For the main adverse reactions of hypertension,given patients with proper medication explain before the treatment to reduce the fear,tension,and avoiding mood disorders which lead to an increase in blood pressure,blood pressure should be regularly detected in patients who use drugs,if patients occurred a sharp rise in blood pressure during the treatment,immediately stopped bevacizumab and given antihypertensive treatment.Conclusion Clinicians should take the possible corresponding prediction in patients underwent application of bevacizumab in combination with chemotherapy on the possible different adverse reactions according to the actual situation of patients,and take corresponding preventive measures,can to some extent avoid serious adverse reaction occurred,and improve the quality of life of the patients.

Objective To understand the current situation of smoking and quitting smoking in patients with cardiovascular disease. Methods 184 cases of inpatients or outpatients with cardiovascular disease treated in the First People's Hospital of Kunming were surveyed about quitting smoking, and the smoking and smoking cessation-related clinical features of patients with cardiovascular disease were analyzed. Results ( 1) Cardiovascular diseases in smokers was male-dominated (100%), mainly hypertension and/or coronary heart disease (179, 97.3%) . (2) Average age of starting smoking was 21.5, 97.8%of patients had smoked more than 10 years, with an average of 35.1 ±13.4 years, 69.6% of patients smoked ≥10 cigarettes/day, with an average of 16.3 ± 10.1 cigarettes/day, 65.8%of patients were in the low level of nicotine dependence, 81.5%of the patients thought that quitting smoking was important, 76.1% had quitting smoking ideas, 67.4% had confidence in the success of quitting smoking, 50.5%thought that it was difficult to quit smoking, 127 (69%) patients had seriously quit smoking. (3) 65 cases (51.2%) who seriously quit smoking had succeeded, 54 patients (83.1%) received varying degrees of medical staff advised to quit smoking, 62 cases (48.8%) in relapse, only 34 patients (54.8%) accepted medical personnel advised to quit. Two sets of chi-square test, p=0.000, prompted difference was statistically significant. Relapse reasons were pressure, craving and around other smokers. Conclusions Smoking patients with cardiovascular disease are mainly constituted by male patients with hypertension and/or coronary heart disease. Most of the patients recognize that quitting smoking is important, and have idea and seriously quit smoking. However, lack of confidence and smoking which has also become a lifestyle significantly affect the success of smoking cessation. The medical staffs' recommending smoking cessation persuasion can largely improve the success of smoking cessation for patients.

Radiotherapy is the main treatment for head and neck cancer,but it will result in adjacent tissue damage,including radioactive xerostomia,the most common complication.The mechanism of radiation-induced parotid damage injury is unclear.Parotid function test include detection of parotid salivary flow and salivary flow rate,parotid SPECT,CT and MRI,etc.How to protect the parotid glands becomes a hot research in recent years.At present,the main methods of protecting the parotid glands includes selecting patterns of radiotherapy,radiotherapy planning optimization,drugs,stem cell transplantation and gene transduction.

Along with the rapid development of global medical technology, great progress has been made in clinical diagnosis and treatment of childhood cancer,hence childhood cancer survival rate is increasing markedly. The clinicians have become concerned about life quality of childhood cancer survivors. A number of studies reported that long-term childhood cancer survivors are at increased risk of developing metabolic syn-drome,especially after cranial irradiation,abdomal irradiation,or total body irradiation. Metabolic syndrome is a variety of metabolic abnormalities commonly clustered together in a condition of the same individual,which sig-nificantly increases risk of cardiovascular diseases. Though the etiology of the metabolic syndrome in cohorts of childhood cancer survivors has not been elucidated,the predisposing factors have been identified as the lack of hormones after cancer treatment,damage from medicine or radiation therapy,endothelial dysfunction and so on. Accordingly,early diagnosis of metabolic syndrome is of great importance with medical interventions,such as encouraging cancer survivors to improve dietary habit and enhance exercise to achieve ideal weight,and to subse-quently decrease the risk of metabolic syndrome and cardiovascular events.

Objective To evaluate the difference in efficacy and safety between ~(131)Ⅰand antithyroid drugs (ATD)in the treatment of Graves'disease in children and adolescents.Methods MEDLINE(1966-2005), Cochrane Central Register of Controlled Trials(Cochrane Library Issue 2,2006),EMBASE(1984-2004), CBMDISK(1978-2005)and CNKI(1994-2006)were searched by computer.Isotopes(1989-2004),Radiologia Pratica(1986-2005),Chinese Journal of Endocrinology and Metabolism(1985-2004),and Endocrinology and Metabolism Clinics of North America(1988-2001)were manually searched.Trials comparatively analyzed ~(131)Ⅰand antithyroid drugs on the treatment of Graves'disease in children and adolescents were included.The quality of the study methodologies such as randomization,blinding and allocation concealment was evaluated and meta-analysis was performed by Revman 4.2 software.Results Five non-randomized controlled trials involving 538 patients were included.Among these trials one was prospective and the rest were all retrospective.~(131)Ⅰwas more effective in increasing the complete remission and decreasing the rate of recurrence as compared with ATD,but the rate of hypothyroidism was significantly increased after ~(131)Ⅰtreatment.Conclusion Based on the five studies,the evidence suggests that ~(131)Ⅰtherapy is effective and safe for children and adolescents,and the total curative effects in Graves'disease are superior to ATD.However great shortage of randomized controlled trial(RCT),and problems concerning randomization,blind method,follow-up and statistic analysis still exist in clinical controlled trials, hence more RCT with high quality should be conducted.

Objective: Clonidine, by activating peripheral α-adrenoceptors, produces transient pressor response after i. v. injection in anesthetized animals. Moxonidine, with at least 40-fold higher affinity to I1-imidazoline receptors than to α2-adrenoceptors, produces also a transient pressor response. This work was designed to investigate whether I1-imidazoline receptors are involved in this pressor effect of moxonidine. Methods: Female spontaneously hypertensive rats (SHRs, aged 14-16 weeks) were anesthetized with urethane. To observe the transient pressor responses, moxonidine 0. 1, 0. 3, 1. 0 mg/kg (intravenous, i. v.), 2.0 μg (intracerebroventricular, i. c. v.) and 1.0, 10.0 mg/kg (intragastric, i. g.) were administrated in different groups of rats. To evaluate the roles of α1-adrenoceptors, α2-adrenoceptors and I1-imidazoline receptors in the transient pressor responses to moxonidine, prazosin (10.0 μg/kg), yohimbine (2.0 mg/kg), phentolamine (0.2 mg/kg), idazoxan (1.0 mg/kg) or yohimbine + idazoxan (2.0 mg/kg + 1.0 mg/kg) were intravenously given to the animals before moxonidine 0.3 mg/kg i. v.). Results: It was found that i. v. moxonidine produced a greater pressor response than clonidine when producing a similar reduction of blood pressure. This effect of moxonidine was not influenced by prazosin, but was partly inhibited by yohimbine, phentolamine or idazoxan, and completely blocked by the combination of yohimbine and idzaxon. Neither i. c. v. injection nor i. g. administration of moxonidine induced transient pressor responses. Conclusion: The transient pressor response of i. v. moxonidine is mediated by both peripheral I1-imidazoline receptors and α2-adrenoceptors.

Background Congenital cataract is the major cause of blindness in children with plentiful clinical manifestations and closely linked with genetics. Objective Present study was to investigate the classification of congenital cataract in Yunnan province.Methods The manifestation characteristics of 184 eyes of 116 patients with congenital cataract in Yunnan province were analyzed.The relationship of performance of various types of congenital cataract between phenotype and hereditary feature were explored. Results All of the 116 congenital cataract eyes from 116 patients were divided into ten types based on the clinical appearance,including the complete cataract,nuclear cataract,posterior polar cataract,anterior polar cataract,coralliform cataract,coronary cataract,pulverulent cataracts,lamellar cataract and blue cataract.Bilateral nuclear cataract and unilateral entire cataract is most prevalent in these patients.Blue cataract and coralliform cataract belong to the less types.Forty-four in 116 cases were found to have the hereditary history and determined as autosomal dominant inheritance.Sporadic cases were determined in 72 cases.Conclusions The analysis of phenotype of the hereditary congenital cataract offers some clues to the classification of congenital cataract.

Objective To explore the clinical significance of the changes of renal artery blood stream parameters in patients with essential hypertensive by color Doppler ultrasonography. Methods Ninety patients with essential hypertension were enrolled and divided into three groups (grade 1,2,3 ) according to their clinical blood pressure from May 2006 to July 2010 and each group included 30 cases. Thirty normal individuals were assigned to the control group. Color Doppler ultrasonography was used to determine the blood flow spectrum of bilateral main renal arteries, segmental arteries and interlobar arteries of kidneys. The parameters included Vmax, Vmin, Vmin/Vmax, resistance index (RI) were recorded and analyzed. Results In the control group, the Vmax,Vmin,Vmin/Vmax and RI in main renal arteries were (63.99 ± 7.68)cm/s, (23.86 ± 3.07) cm/s,0. 38 ± 0. 05 and 0. 62 ± 0. 05 ;in segmental arteries were ( 52. 93 ± 4. 24) cm/s, ( 20. 89 ± 2. 47 ) cm/s,0. 40 ±0. 04 and 0. 60 ±0. 04;in interlobar arteries were (35. 32 ±4. 22)cm/s, ( 15.07 ±2. 54) cm/s,0. 43 ±0. 04 and 0. 57 ± 0. 04. Compared to the control, only Vmin, Vmin/Vmax and RI in interlobar arteries were statistically changed in grade 1 hypertension group ( P ＜ 0. 05 ). While in grade 2 hypertension group, all the blood flow parameters except Vmax in main renal arteries statistically changed compared to the control group(P ＜ 0.05 ). In grade 3 hypertension group, all the blood flow parameters showed statistically differences when compared to control group (P ＜ 0. 05 ). The Vmax, Vmin, Vmin/Vmax decreased along with the blood pressure increased,however, the RI increased along with BP. Conclusion The renal artery blood stream parameters determined by color Doppler ultrasonography can provide important information to evaluate abnormal renal function in patients with hypertension.