Objective To improve the diagnosis and treatment of von H ippel-Lindau (VHL) disease.Methods The clinic data of one kindred with VHL disease type Ⅱ B was reviewed including clinical manifestation,imaging,pathology and therapy.A 40-year-old male complained of the left upper extremity numbness for 6 months,and a variety of visceral tumors were found 3 months ago.Contrast-enhanced MR imaging showed multiple brain tumors.CT showed left kidney tumor,pancreatic tumor,pars-aortic tumor and left adrenal tumor.Fundoscopy showed multiple retinal hemangioblastoma.PET-CT discovered abdominal multiple tumors.And pedigree analysis was to determine the family medical history,and 6 members got genetic screening.Results In the kindred with VHL disease type Ⅱ B,9 members (30％) out of 40members in 4 generations got the disease.Six members got genetic screening,and the result showed 5 (5/6) members had mutation.Three (3/5) members with 3 sites genetic mutation showed clinical manifestation,1 (1/5) members with 3 sites genetic mutation without clinical manifestation found brain tumors by MRI,and 1 ( 1/5 ) member with 1 site genetic mutation did not find disease by comprehensive checkup.The main mutation located at exon 1 in chromosome 3p25 of VHL gene.All mutation was hetcrozygous mutation.The 295,337 and 337 nucleotide thymine of the VHL gene were substituted by cytosine,cytosine and adenine,which made the 98th,112th and 112th tyrosine substituted by histidine and asparagines.One member with 1 site mutation had 98th tyrosine substituted by cytosine.The first operation was to remove brain tumor,and the second operation was to remove adrenal tumor,para-aortic tumor and renal tumor.Pathology of the brain tumors showed hemangioblastoma,and the retroperitoneal tumors were clear cell carcinoma,paraganglioma and pheochromocytoma.Followed up for 6 months after operation,no relapse occurred.Conclusions VHL disease is a relatively rare autosomal dominant disorder.Comprehensive management of patients should also include genetic counseling and screening for other manifestations of the disease process.Genetic testing might be helpful in early detection of asymptomatic VHL patients.Members having gene mutation should be followed up strictly.Surgical management of VHL disease should be decided on the base of comprehensive assess.Multiple lesions could be cut off in one operation.For patients with pheochromocytoma,pheochromocytoma shuld be handled first.

Objective To assess the factors affecting survival of malignant pheochromocytoma/malignant paraganglioma.Methods The clinical data of 85 malignant pheochromocytoma/malignant paraganglioma patients were analyzed retrospectively.All the patients were followed up,the duration from 2 months to 283 months,the median time was 48 months.The overall survivals of 5 years and 10 years were calculated.The possible affecting factors,such as gender,age,tumor function,metastatic sites,metastatic fields,incipience or recurrence,the time to recur,were analyzed.Survival differences between groups were compared with the log rank test.Multivariate analysis was performed by using Cox regression analysis to detect variables independently associated with survival.All P values were 2 tailed with P ＜ 0.05 considered statistically significant.All statistical analysis was done by SPSS 17.0 software package.Results The overall median survival time was 62 months,5-year survival rate was 44.7％,10-year survival rate was 11.8％.Gender (P =0.649),age (P =0.228),incipience or recurrence (P =0.217) had no significant effect on the survival time.Significance was found in tumor function (P =0.034),metastatic sites,metastatic fields (P =0.009),the time to recur (P =0.003).The median survival time of patients with nonfunctioning tumor and functioning tumor was 90 months and 37 months.The median survival time of patients with multiple system metastases and single system metastasis was 37 months and 117 months.For the patients with single system invaded,there were 15 cases (26.8％) of bone metastasis,10 cases (17.9％) of lymph node metastasis,8 (14.3％) of liver metastasis,7 (12.5％) of lung metastasis,6 (10.7％) of renal metastasis,6 (10.7％) of intestine metastasis and 4 (7.1％) of brain metastasis.The median survival time was 110,77,28,14,26,8 and 19 months.The median survival time of patients with lung,liver,intestine metastasis were shorter than the other patients (P ＜ 0.05).Fifty-five patients were found recurrence after operation,recurrent time was from 4 to 65 months,and the median time was 22 months.The survival time of patients with recurrence within 2 years was shorter than patients above 2 years (P =0.003).Conclusions The prognosis of malignant pheochromocytoma/malignant paraganglioma is poor.Gender,age,incipience or recurrence could not affect the overall survival time.The survival time has relationship with the function of tumor,metastatic field,metastatic sites and time to recurrence.The patients with nonfunctioning tumor have longer survival time than those with functioning tumors.The survival time of patients with single system invaded is longer than ones with multiple systems invaded.The common metastatic sites are bone,lymph node,liver,lung,kidney,intestine and brain.Patients with intestine metastasis have worst prognosis.The patients with only bone and lymph node metastasis have best prognosis.Patients with recurrence above 2 years have a better prognosis than those within 2 years.

Objective To analyse the CT findings of Bochdalek hernia in adult.Methods The CT scan of 42 cases were reviewed showing a mass abutting the upper surface of the diaphragm, the defect was located in the posteromedial aspect of hemidiaphragm with continuity of subdiaphragmatic and supradiaphramatic densities through the diaphramatic defect. Among these, 25 cases were male and 17 cases female. The average age was 64 years, 71.4%≥60 old.Results 53 Bochdalek hernias were identified in 42 cases, 11 cases were bilateral, 21 cases were on the left and 10 cases were on the right side. The diaphragmatic defects diameter ranged 0.5～6.7 cm, the median was 2.8 cm. The range of Bochdalek hernias diameter was 1.5～9.0 cm, with median of 3.7 cm. There was a significant association between diaphragmatic defects and size of hernias (r s=0.72,P

Objective To evaluate the surgical effect for congenital nystagmus (CN) with an abnormal head position in all three axes of a predominant head turn. Design Retrospective case series. Participants 11 patients with horizontal nystagmus and abnormal head position treated in Xingtai Eye Hospital during 2003-2008. All had an abnormal head position in all three axes with a predomi-nant head turn. Methods Recession of the yoke muscles responsible for the slow phase of nystagmus (one medial rectus of 6mm, and one lateral rectus 8 mm), and simultaneously resection of the yoke muscles responsible for the fast phase of nystagmus (the other lateral rectus 9 mm, and medial rectus 7 mm) were performed. The follow-up was from 6 to 20 months. Main Outcome Measures The visual acuity of both eyes in primary position, the degree of head turn, chin elevation or depression, head tilt. Results At the last follow-up, the all 11 patients undergoing the operation for CN gained two or more lines of Snellen acuity with both eyes in primary position for be-ing corrected best. The postoperative mixed head position of 9 cases got disappear or ≤ 10°. Of 1 case developed overcorrection of the head turn. 1 case has the head turn of ≤15°, and 1 case remained the head turn of 25°. Conclusion Recessions and resections of the horizontal yoke rectus muscles in nystagmus with blockage position, when the head turn predominates over the vertical and torsional components, are effective in diminishing the abnormal head position on all three axes, and improve the visual acuity with both eyes in primary position. (Ophthalmol CHN, 2009, 18: 275-278)

MicroRNA (miRNA) are a class of endogenous single -stranded non-coding RNA, which regulate gene expression post-transcriptionally by combining with the target mRNA and play a vital role in biological and pathological processes including the growth of organism , metabolic regulation, disease prediction and intervention.Thus miRNA detection is of considerable significance in disease diagnosis and the research of miRNA function.Because of the restriction factors about miRNA itself such as short sequence, low abundance and highly homologous , traditional methods for miRNA detection cannot meet the current demands due to the limitations like unsatisfactory sensitivity and complicated operation .This review summarizes the newly development about signal amplification -based methods for miRNA, including the advantages and limitations of all kinds of novel methods , and highlights the future trends as well.

Objective Lymphedema is a common complication interrupting a large percentage of breast cancer survivors .How to release the breast cancer survivors from the pain of lymphedema remains a question to breast surgeons .The rapid development of the super-microsurgical measurement allows lymphaticovenular anasto-mosis to become a better way to treat breast cancer related lymphedema .Methods We followed a breast cancer related lymphedema case amd offered super-microsurgical lymphaticovenular anastomosis to her .Results The surgery lasted 4.5 h, and contained 11 anastomosis of lymph ducts .Improvement of edema could be seen during the surgery and the measurement of the upper limb became smaller just one day after surgery .Conclusion Su-per-microsurgical lymphaticovenular anastomosis is a safe improvement for extremity lymphedema after mastectomy and it can provide a better prognosis and appearance .

Objective To understand the current status of infections of intestinal parasites of population in Taixing City,Ji-angsu Province. Methods The infection rates and densities of human intestinal parasites were investigated according to the methods of the National Investigation Scheme on Human Principal Parasites,and the data of society,economy and disease con-trol were collected and analyzed. Results Among 2 556 people investigated in five villages,16 persons were found with intesti-nal parasites, with an infection rate of 0.63%. The infection rate was higher in residents with a low education level than in others and it was higher in the age group over 50 years than in the group under 50 years. The infection density was mild and the most was the single parasite infection. Conclusions The current status of intestinal parasite infections of population in Taixing City has reached the county-level control standard of Jiangsu Province. Therefore,the preventive strategy and measures should be ad-justed and the monitoring work should be strengthened.

Four natures are cold, hot, warm and cool natures of drug, it is the one of main theoretical bases for explaining the effects of traditional Chinese drugs. According to the natures of traditional Chinese drugs, the compatible modality of traditional Chinese drugs can be classified into two types: the compatibility of drugs with same natures and the compatibility of drugs with different natures. Both of them can play the actions of strengthening potency of drugs, enlarging the treated scope, preventing adverse reactions and bringing about new functions.

Objective To study the preparation of nifedipine liposomes (NP) and evaluate their quality. Methods The liposomes were prepared by a pH steps degree-freeze thawing method, with the entrapment and stability as indices. The entrapment was studied by Sephadex G-50 minicolumn centrifugation UV-Vis spectroscopy method. Entrapment, and degree distribution before and after freezing were compared, the effect of freezing times on entrapment was studied. Results The entrapment and the average size of NP liposomes were 96.65% and 2.0 ?m, respectively. After freezing, both the entrapment and stability were increased. Conclusion The selected formulation and preparation of nifedipine liposomes are practicable.

Objective To study the feasibility and security about misoprostol combined with fentanyl and propofol applied in visualization of artificial abortion.Methods 1270 cases of pregnant women who requested termination of pregnancy and voluntarily accepted abortion were selected.In the observe group,634 cases of pregnant women received oral misoprostol about 600 mg 90 minutes before surgery.After anesthetized by fentanyl and propofol,painless artificial abortion using visual abortion instrument was carried out.In the control group,636 cases of pregnant women anesthetized by fentanyl and propofol via the venous were carried out artificial abortion.The degree of cervical relaxation,loss of blood,total dose of propofol,surgical time,recovery time,the incidence of abortion complications and so on were observed.Results In the observation group and the control group,the average dosage of propofol are( 14.15 ± 2.37) ml and (16.90 ± 4.52) ml respectively;The average time required for surgery are ( 146.1 ± 38.2) s and ( 199.7 ± 37.3 ) s respectively;the average time required for awakening are ( 6.01 ± 1.90 ) min and ( 8.81 ± 2.02 )in;In the observation group,there are 405 ( 63.9% ) cases of cervical laxity markedly,in the control group,including 145 ( 22.8% ) cases of cervical laxity markedly,there is statistical significance ( P ＜ 0.05 ) between the observation group and the control group.There are no abortion syndrome in both groups.Conclusion Misoprostol combined with fentanyl and propofol was the first choice for painless artificial abortion using ultrasound visualization.