Objective To report a case of multiple endocrine neoplasia type 2B (MEN 2B) combined with analogous Marfan's syndrome with the related literature review,in order to improve the knowledge of this disease.Methods A case of MEN 2B combined with analogous Marfan's syndrome was admitted in Peking Union Medical College Hospital in Nov 2011.The patient was a 21-year-old male with the chief complaint of tongue thick for 13 years,found a tumor in right adrenal gland for 3 months.The patient underwent radical thyroidectomy and lymph node dissection in April 2011 because of thyroid tumor,and postoperative pathology confirmed the diagnosis of medullary thyroid carcinoma(T2N1bM0).The patient had normal blood pressure without fluctuation.Physical examination indicated that the patient had thin limbs,long fingers and long toes.Carpal syndrome and finger syndrome were positive.There were multiple tumor like nodules in the tip of the tongue,lips,inner canthus of eyelids,and laryngoscopy showed multiple nodulars in bilateral vocal cord and bilateral tip splitting.Enhanced CT showed a tumor of 2.9 cm×3.4 cm×3.8 cm in the right adrenal gland.Endocrine examination revealed high catecholamines:norepinephrine 159.3 nmol,epinephrine 13.3 nmol,and DA 918.2 nmol.131I-MIBG was positive for pheochromocytoma.The clinical manifestation was in stationary state.Preoperative diagnosis was MEN 2B,right adrenal pheochromocytoma,medullary thyroid carcinoma (T2N1bM0)after operation,multilple mucosa neurofibroma and analogous Marfan's syndrome.Results The pheochromocytoma in right adrenal gland was removed by laparoscopy under general anesthesia successfully on Dec 12,2011.The postoperative pathology confirmed the diagnosis of pheochromocytoma.And gene mutation was found in exon 16 of RET gene.MEN 2B with analogous Marfan's syndrome was diagnosed.During the follow-up period for 28 months,the patient had normal blood pressure and heart rate without tumor recurrence or metastasis.Conclusions MEN 2B combined with analogous Marfan's syndrome is extremely rare.For patients with medullary thyroid carcinoma,pheochromocytoma should be considered before operation.For patients with analogous Marfan's appearance,Marfan's syndrome should be differentially diagnosed.

Since Zika virus (ZIKV) has firstly been isolated in 1947, Uganda, outbreaks of Zika fever have been reported in many areas such as in Africa, Southeast Asia and America. Imported cases in China also have been reported. Zika virus belongs to the family Flaviviridae, genus Flavivirus, and include Africa subtype and Asia subtype. It is a mosquito-borne virus primarily transmitted by Aedes aegypti mosquitoes. Sexual transmission, Blood transmission and mother-to-fetus transmission were also reported. Zika virus can go though blood-brain barrier and infect central nervous system. Symptoms are generally mild and self-limited, but recent evidence suggests a possible association between maternal Zika virus infection and adverse fetal outcomes, such as congenital microcephaly, as well as a possible association with Guillain-Barré syndrome. Laboratorial Diagnosis includes nucleic acid detection, Serological test, and isolation of virus. Currently, no vaccine or medication exists to prevent or treat Zika virus infection. Preventive measures against Zika virus infection should be taken through prevention of mosquito bites and surveillance in epidemic area.
Aedes ; physiology ; virology ; Animals ; Humans ; Insect Vectors ; physiology ; virology ; Zika Virus ; genetics ; physiology ; Zika Virus Infection ; transmission ; virology

Objective To study the clinical factors affecting the prognosis of adrenocortical car-cinoma (ACC) in order to guide the diagnosis and treatment of ACC. Methods The clinical data of 40 cases of ACC were retrospectively analyzed. The patients were diagnosed as ACC by histopatholo-gical examination after operation or biopsy, including 19 cases of nonfunctional ACC and 21 cases of functional ACC. The functional ACC included 14 cases of Cushing's syndrome, 4 cases of sexual ab-normality (3 cases of androphany and one case of male sexual precosity) and 3 cases of primary aldo-steronism. The clinical stage included 3 cases in stage Ⅰ , 10 cases in stage Ⅱ , 7 cases in stage Ⅲ, and 21 cases in stage Ⅳ. Eighteen cases underwent complete excision, 10 cases underwent palliative excision, 12 cases underwent expectant treatment (2 cases underwent arterial embolism). All patients were followed up for 2 to 121 months. The total life span and survival rate were calculated, and the dependability between life span and certain indices (tumor size, function, clinical stage and surgical treatment, etc) was compared. The results were statistically analyzed by utilizing the SPSS 11.5 soft-ware. Rank-sum test was used for the two independent samples, one-factor analysis of variance was used for multiple samples and Pearson correlation analysis was used for the correlation of two vari-ances. Results There were no statistical correlations between gender, age, sides and survival time (P>0.05). The survival time of nonfunctional ACC patients was 37.0 months, of which 11 dead ca-ses survived 11.0 months and 8 survival cases survived 73.5 months. The survival time of functional ACC patients was 11.5 months, of which 19 dead cases survived 12.2 months and 2 survival cases survived 4.8 months. The survival time of nonfunctional ACC was significantly longer than that of functional ACC (P<0. 05). The actual survival time was 66 months and 120 months in stage Ⅰ , of which both survived. The actual survival time was (44.9±40. 2) months in stage Ⅱ, of which 4 sur-vival cases survived 59.0 months. The actual survival time was(34. 5±29.8) months in stage Ⅲ, ofwhich 2 survival cases survived 42.0 months. The actual survival time was (7.1±6.7) months instage Ⅳ, of which 2 survival cases survived 8. 0 months. There was significant difference of the sur-vival time among different clinical stages(F=11. 078, P<0. 05). There was no significant difference of survival time between treatment methods[(10.04±0.0) vs (4.5±1.5) months, P>0. 05]. Con-clusions The most important influencing prognostic factors of ACC are function and clinical stage. For ACC in stage Ⅰ and Ⅱ , tumor resection is the most effective treatment, and second surgical oper-ation is recommended for local recurrence. For ACC in stage Ⅲ, radical or extensive surgical operation is recommended, and for ACC in stage Ⅳ, surgical operation has no effect on the prognosis.

Objective:To detect the expression level of microRNA-634 (miR-634) in hepatocellular carcinoma (HCC) and its regulatory effect on the common biological behavior of hepatocellular carcinoma cells .Methods: Real-time fluorescence quantitative PCR (RT qPCR) method was used to detect HCC cell lines (HepG2, SMMC7721, BEL7402, bel7404, SNU739), 69 cases of HCC tissues and matching relative quantification of miR-634 paracancerous tissues and analysis of relationship between miR-634 expression and HCC patients gender, age, tumor size, degree of differentiation, child Pugh classification, BCLC staging, portal vein tumor thrombus and liver metastasis , while building a miR-634 eukaryotic expression vector and transfected into hepatocellular carcinoma cell lines, using live cell counting kit-8 CCK-8, flow cytometric annexin V/PI double staining and Transwell experiment to detect the transfection miR-634 on cell proliferation , apoptosis and invasion effects .Results:Compared with the normal human liver cell line L-02 and hepatocellular carcinoma cells miR-634 were decreased ( P<0.05 ) , the expression followed by HepG 2>SNU739>Bel7402>Bel7404>SMMC7721;69 cases of hepatocellular carcinoma ( HCC ) of miR-634 level ( 0.253 ±0.019 ) and lower than that of the matched paracancerous tissues ( P<0.05 ) , and related with the tumor size , degree of differentiation , BCLC stage , portal vein tumor thrombus and liver metastasis ( P<0.05 ) .Over expression in the transfected group 24-96 h after miR-634 level continues to rise , control group and blank vector transfected group differences were statistically significant ( P<0.05 );and the control transfection group and blank group compared to transfection proliferation inhibition rate , apoptosis rate was increased , but wear the number of cell membrane decreased, the difference was statistically significant (P<0.05).Conclusion:miR-634 in hepatocellular carcinoma tissues and cells were low expression and related to clinical pathological parameters , raised its level can inhibit the proliferation and invasion of hepatocellular carcinoma cells and induce apoptosis , for the prevention and treatment of liver cancer has an important reference value .

Objective To evaluate the safety and efficacy of adrenal autotransplantation for the treatment of persistent Cushing's disease after transsphenoidal pituitary tumor resection. Methods Four patients were treated by adrenal autotransplantation with attached blood vessels after bilateral adrenalectomy for persistent Cushing's disease from April 1991 to March 2008 in our institute. The four patients were 3 females and 1 male. Their ages ranged from 14 to 36 years, with an average of 30 years. Right adrenalectomy was performed 1 to 3 months before the left adrenalectomy. 30 % ?50 % of the left hyperplastic adrenal was placed in the left inguinal region with the anastomosis of the inferior epigastric artery and the central adrenal vein, and between the left saphenous vein and the incised adrenal envelope with the adrenal middle artery inside. The hormone replacement dosage decreased gradually after operation. They were followed up for 1.0, 1. 5, 8. 0 and 10. 0 years. The patient's symptoms, adrenal hormone (serum cortisol, urinary free cortisol and adrenocorticotroph) levels and the steroid replacement dosages were recorded and analyzed. Results The symptom of the Cushing's disease disappeared completely after operation and there was no Nelson's syndrome except one patient with slight darken skin. Through 4 ways of imaging examination, operation, clinical presentation and endocrine examination, the 4 transplanted adrenals functioned well with less steroid replacement dosage needed after operation. The 4 transplanted adrenal glands functioned steadily 1 year after the auto-transplantation, and no hyperplasia was detected in the transplanted adrenals with the stimulus of high dosage adrenocorticotroph. Conclusions The long-term effects of adrenal autotransplantation with attached blood vessels after bilateral adrenalectomy is effective and safe. Adrenal autotransplantation can be a feasible option for the treatment of persistent Cushing's disease.

The effects of SHCDCT (selective head coolingdehydration combined therapy) on hypermetabolism, and its relation with thyroigenous hormones.The changes of glucose,phosphlipids,FT3 and TSH at 30, 180, 360 min of reperfusion following 30 min complete cerebral ischemia (four-vessel model ) in rabbits were observed.The effect of SHC (selective head cooling), DH(dehydration),SHCDCT on these changes were compared.SHC (28'C ) was induced by surface cooling methods.Compared with non-ischemia group,glouse, phospholipids decreased during reperfusoin,FT3 and TSH increased (P

Objective: To assess whether selective head cooling (SHC)could ameliorate the post-ischemie reperfusion injury. Method: Complete cerebral ischemia (CCI)was induced with the four vessel model for 30 min. 196 New Zealand rebbits were randomly allocated into three groups:group Ⅰ served as nonischemie control; animals in three subgroups of other groups had reperfused lasting 30,180 and 360 min respectively following CCI. Group Ⅱ served as normothermic reperfusion; group Ⅲ treated with selective head cooling(28℃,). Twenty-one endogenous parameters in brain were determined, and histomorphological assessment of neuronal chanes was observed. Neurons were classified respectively into type A(normal)and B(mild damage)etc. Result: As compared with group Ⅰ, the percentage of type A neurons was progressively decreased, while that of type B neurons increased sigmficantly in group Ⅰ (P

The neurogenesis after cerebral ischemia is one of the research hotspots in the field of neuroscience.This article mainly expounds the advances in research on neurogenesis from the main processes,regulatory factors,signal pathways and microenvironment as well as how to promote neurogenesis following cerebral ischemia.Our purpose is to provide new treatment ideas for the recovery of neurological function following stoke.

Objective To study the classification and treatment of juxtaglomerular cell tumor of the kidney. Methods Five cases of juxtaglomerular cell tumors of the kidney were diagnosed and treated surgically in our hospital during the last 4 years. Seven large series of cases report of juxtaglomerular cell tumors have been published in the Pubmed. These series of cases report plus 1 case report of static juxtaglomerular cell tumor were incorporated into a review of 71 cases previously published in English literature. The clinical presentation, laboratory examination and imaging study were summarized. The clinical presentation including blood pressure, the laboratory examinations including kalium, plasma renin activity and aldosterone, and renal venous sampling for renin assay, and the imaging study including ultrasonography, CT, excretory urography, and selective renal angiography were studied retrospectively. Results The 71 cases juxtaglomerular cell tumors could be classified into 3 types, that is, typical type, atypical type and static type. The 57 typical cases had the typical characteristics of hypertension, hyperaldosteronism, and hypokalemia secondary to tumor renin secretion.The 12 atypical cases had hypertension with normal kalium, and the 2 static cases had normal blood pressure and kalium. Typical juxtaglomerular cell tumor of the kidney should be considered in hypertensive patients with secondary aldosteronism. Atypical juxtaglomerular cell tumor of the kidney should be considered in patients with hypertension and renal tumor. Static juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor. Conclusions The classification of typical, atypical and static juxtaglomerular cell tumors depends on blood pressure and serum kalium. As a surgically corrected disease, juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor, and nephron-sparing surgery is the first choice.

Objective To study a modified method of laparoscopic partial nephrectomy with hypothermic renal artery perfusion in situ.Methods Laparoscopic partial nephrectomy with hypothermic renal artery perfusion in situ was performed in 5 patients from March 2009 to May 2009.There were 3 males and 2 females,with an average age of 49 years (39 to 63 years).There were 3 tumors in the left kidney and 2 in the right kidney,with an average diameter of 5.6 cm (range,3.8 to 7.0 cm).There were 2 cases of chronic renal insufficiency,1 case of solitary kidney,1 case of contralateral renal atrophy and 1 case of larger benign tumor.The procedure of laparoscopic partial nephrectomy with hypothermic renal artery perfusion in situ was as follows:A catheter with balloon was placed in the renal artery with percutaneous approach through the femoral artery preoperatively.During the operation the balloon of the catheter was inflated with water in order to block the renal artery,and renal artery perfusion of about 200ml saline of 4 ℃ was conducted through the catheter under high pressure in order to achieve low temperature in situ kidney.The water of the balloon was extracted after the accomplishment of the laparoscopic partial nephrectomy.Results Laparoscopic partial nephrectomies with hypothermic renal artery perfusion in situ were carried out successfully in 5 cases.The average operative time was 102 min (80 to 120 min),the average renal artery occlusion time was 35 min (range,29 to 39 min),and the average amount of bleeding was 190 ml(50 to 300 ml).The temperature of skin,kidneys,and tumor after hypothermic perfusion dropped by an average of 0.6℃,10.0 ℃,and 9.8℃,respectively.The endogenous creatinine clearance rate was(84.7±16.9),(48.9±14.5),(52.1±12.4),(54.5±13.8),and(54.6±11.7)ml/min before and 1 day,3 days,5 days and 10 days after operation.There was significant difference among the 5 groups in endogenous creatinine clearance rate(P=0.001).There was significant difference between each 2 groups except that between the group at 5 days and 10 days after operaton,The endogenous creatinine clearance rate kept steady in 5 days after operation.Conclusions Laparoscopic partial nephrectomy with hypothermic renal artery perfusion in situ is feasible and safe in favor of the extension of renal ischemic time and the protection of renal function.And it has resolved 2 problems in laparoscopic partial nephrectomy,that is,the laparoscopic artery occlusion and the hypothermic perfusion.