AIM:To observe the structural basis of ocular motility abnormalities in patients with congenital fibrosis of the extraocular muscles type Ⅰ ( CFEOM Ⅰ) due to missense mutations in the developmental kinesin KIF21A using high - resolution magnetic resonance imaging ( MRI) .
METHODS: Totally 11 affected individuals reported KIF21A mutations were correlated with MRI studies demonstrating extraocular muscles ( EOMs ) size, location, contractility, and innervation. EOMs and the motor nerve in the orbits were imaged with T1 weighted in a triplanar scan using a dual-phased coils with 2. 0mm thick. Motor nerves were imaged at the brainstem using head coils and 3D-FIESTA with 0. 6-mm thick.
RESULTS: Patients with CFEOM Ⅰ exhibited different degrees of hypoplasia of oculomotor nerve, the abducens nerve and the trochlear nerve were also affected, of which 8 cases of orbital section could see the signal of abnormal nerve dominated by oculomotor nerve to lateral rectus. The both sides of six EOMS in all patients exhibited variable atrophy and abnormal bright internal signal on T1 imaging, particularly severe for the superior rectus and levator muscles.
CONCLUSION: High - resolution MRI can directly demonstrate pathology of motor nerves,affected EOMs, and ‘Pulley' hypoplasia caused by CFEOM Ⅰ due to mutations in KIF21A,and these findings suggest that the neuronal hypoplasia is the etiological factor of CFEOM.

OBJECTIVETo explore the surgical method and clinical curative effect of Ilizarov technique combined with limited surgical treatment for neurotrophic malnutrition equinovarus accompanied with weight-bearing area ulcer.

METHODSFrom July 2004 to December 2011, 21 cases of neurological disorders equinovarus with skin ulcer in weight-bearing area were treated including 14 males and 7 females with an average age of 21.3 years old ranging from 8 to 32 years. Among them,19 cases with talipes equinovarus were on account of spina bifid and 2 cases on account of spinal cord injury of lumbar sacral segment. Nine cases were on the left foot and 12 cases were right foot. The ulcer area in out-below side of the fifth metatarsal bone was in 6 cases, in basement of the fifth metatarsal bone in 5 cases,in lateral of calcaneocuboid joint 2 cases, and in lateral of subtalar joint in 8 cases. Surgical procedure included plantar medial release technique, tendo-chilles lengthenning, and three-joint partial osteotomy. During operation,the skin and soft tissue of ulcer were resected and the incision was sutured when the ulcer was in the lateral of subtalar joint, and ankle Ilizarov external fixation for extension was installed after correcting talipes equinovarus partially. For the legacy skin ulcer in weight-bearing area,the Shenrg-ji cream (Chinese characters) was used after operation for one time per day until the wound healed. Adjusting Ilizarov external fixation for correcting residual deformity until it is satisfaction. During this process weight-bearing walking was keeping.

RESULTSAll cases were followed up from 6 to 52 months with an average of 28 months. All ulcers were healing and no recurrence. The ulcer healing time was 14 to 36 days postoperative (26 days in average). Eighteen deformity feet were corrected satisfactorily, and recovered fully plantar foot, 3 feet appeared mild deformity after 1 year. Ankle joint activity appeared limited in 15 cases and got completely rigidity in 6 cases. The AOFAS increased from preoperative 34.0±7.2 to 86.0±8.5 postoperatively; 8 feet got excellent, 10 good,3 fair.

CONCLUSIONThe clinical effect of Ilizarov technique combined with limited operation and the Sheng-ji cream in correction of the talipes equinovarus with skin ulcer in weigh-bearing area on account of neurotrophic malnutrition is satisfactory, the surgical method is simple and the treating course in security, and serious complications can be avoided.

Adolescent ; Adult ; Child ; Clubfoot ; surgery ; External Fixators ; Female ; Foot Ulcer ; surgery ; Humans ; Ilizarov Technique ; Male

Objective To investigate influence of combined use of allopurinol and warfarin on the INR of chronic permanent atrial fibrillation hyperuricemia patients. Methods 80 cases of patients with chronic atrial fibrillation complicated with hyperuricemia, in Sanmen County People's Hospital from February 2016 to February 2017 were randomly divided into the control group (n=40) and the observation group (n=40), the control group was given warfarin plus low purine diet treatment, the observation group was given additional Allopurinol treatment. Changes of INR were compared. Results There was no statistical significance in INR level before treatment between the two groups of patients with permanent atrial fibrillation complicated with chronic hyperuricemia. The INR level of the control group was not changed after treatment, the observation group increased significantly after treatment, and there was statistical significance between the two groups (P<0.05). There were no bleeding events in the control group, there were 6 cases of the observation group with minor bleeding and subcutaneous ecchymosis, including 2 cases with fecal occult blood, 1 cases with hematuria, nasal hemorrhage . The change of liver and kidney function was not obvious. Conclusion In application of allopurinol treatment plus warfarin for patients with chronic persistent atrial fibrillation and hyperuricemia patients, , INR need to be regularly monitored, in order to provide reference for Warfarin dosage adjustment, and ensure clinical safety.

OBJECTIVETo test the dissolution rate of silymarin dropping pill as well as to be compared with other three commercial products of the silymarin.

METHODBy UV spectrophotometry, we studied the dissolution conditions of silymarin dropping pill and compared its dissolution rate with Yiganling tablets (film-coating, sugar-coating) and Legalon capsule which are available in the market.

RESULTThe dissolution parameters T50 and Td of silymarin dropping pill, Yiganling tablet (film-coating), Yiganling tablet (sugar-coating) and Legalon capsule are 6.78, 9.85 min, 51.01, 73.78 min, 74.35, 86.97 min and 53.10, 72.65 min.

CONCLUSIONThe dissolution rate of silymarin dropping pill is superior to that of two kinds of Yiganling tablets and Legalon capsule.

Capsules ; Drugs, Chinese Herbal ; administration & dosage ; chemistry ; Silymarin ; administration & dosage ; chemistry ; Solubility ; Spectrophotometry, Ultraviolet ; Tablets

The epididymis is a single and highly convoluted tubule system in mammals. The epithelium is the major compartment for epididymal function. Proteins synthesized and secreted by epididymal epithelium provide a special and ever-changing luminal fluid environment for sperm as they progress through the epididymis, which makes sperm achieve motility and ultimately results in sperm functional maturation. Specialized genes expressed in the epididymis have regional-specific characteristics. They are regulated by androgen and/or testicular factors and present spatial and tempel-specialized expression pattern in postnatal development, all these hint that they play important and unique roles in epididymis.
Animals ; Epididymal Secretory Proteins ; genetics ; Epididymis ; physiology ; Gene Expression Regulation, Developmental ; Male ; Mammals ; Sperm Maturation ; genetics

An electrolytically-detachable microcoil is introduced here in the paper. The testing results indicate that, the microcoils have stable mechanical properties, clear radiographic images and fine insulation performance. Their detaching time varies from 30s to 200s when voltage changes from 2V to 5V.
Embolization, Therapeutic ; instrumentation ; Equipment Design ; Intracranial Aneurysm ; therapy

BACKGROUNDEndothelial and smooth muscle cells were used as seeding cells and heterogeneous acellularized matrix was used as scaffold to construct the tissue-engineered graft.

METHODSA 2 weeks piglet was selected as a donor of seeding cells. Two-centimetre length of common carotid artery was dissected. Endothelial cells and smooth muscle cells were harvested by trypsin and collagenase digestion respectively. The isolated cells were cultured and expanded using routine cell culture technique. An adult sheep was used as a donor of acellularized matrix. The thoracic aorta was harvested and processed by a multi-step decellularizing technique to remove the original cells and preserve the elastic and collagen fibers. The cultured smooth muscle cells and endothelial cells were then seeded to the acellularized matrix and incubated in vitro for another 2 weeks. The cell seeded graft was then transplanted to the cell-donated piglet to substitute part of the native pulmonary artery.

RESULTSThe cultured cells from piglet were characterized as endothelial cells by the presence of specific antigens vWF and CD31, and smooth muscle cells by the presence of specific antigen alpha-actin on the cell surface respectively with immunohistochemical technique. After decellularizing processing for the thoracic aorta from sheep, all the cellular components were extracted and elastic and collagen fibers kept their original morphology and structure. The maximal load of acellular matrix was decreased and 20% lower than that of untreated thoracic aorta, but the maximal tensions between them were not different statistically and they had similar load-tension curves. Three months after transplantation, the animal was sacrificed and the graft was removed for observation. The results showed that the inner surfaces of the graft were smooth, without thrombosis and calcification. Under microscopy, a great number of growing cells could be seen and elastic and collagen fibers were abundant.

CONCLUSIONCultured self-derived endothelial and smooth muscle cells could be used as seeding cells and heterogeneous acellularized matrix could be used as scaffold in constructing tissue-engineered graft.

Animals ; Cell Differentiation ; Cell Transplantation ; Cells, Cultured ; Graft Survival ; Sheep ; Swine ; Tissue Engineering ; instrumentation ; methods ; Transplantation, Heterologous

AIMTo immunolocalize the c-mos gene product and to investigate its spatial and temporal expression in mouse testis during postnatal development.

METHODSSemi-quantitative reverse transcription-polymerase chain reaction (RT-PCR) and in situ hybridization techniques were used to examine c-mos mRNA and indirect immunofluorescence was used to localize c-Mos protein in mouse testis on postnatal days 14, 21, 25, 28, 30, 35, 49 and 70.

RESULTSc-mos mRNA remained low on postnatal days 14-21, increased abruptly from day 25 and peaked on day 30. Its levels decreased a little on day 35 and became almost stable thereafter until day 70. c-mos mRNA was localized in the nucleus and cytoplasm of the spermatocytes and round spermatids. The nuclear staining was much stronger than the cytoplasmic staining. Using a polyclonal anti-c-Mos antibody, Western blotting detected a single band at 43 kDa in testis lysate. c-Mos protein was exclusively localized to the elongating spermatids and was first detected on postnatal day 30. The number of c-Mos-positive spermatids increased progressively till day 49 and stabilized thereafter.

CONCLUSIONThe c-mos gene displays a spatial and temporal expression pattern in the mouse testis during postnatal development at both the mRNA and protein level. This suggests that c-mos might play important roles in spermatogenesis.

Animals ; Fluorescent Antibody Technique, Indirect ; Gene Expression ; Genes, mos ; genetics ; Male ; Mice ; Reverse Transcriptase Polymerase Chain Reaction ; Spermatocytes ; growth & development ; metabolism ; Spermatogenesis ; genetics ; Testis ; metabolism

OBJECTIVETo study therapeutic effects of limited internal fixation combined with external fixation in the treatment of deformity of distal end of femur, as well as to explore its effect on the function of knee joint.

METHODSFrom June 2004 to June 2009, 38 patients with deformity of distal end of femur were treated with osteotomy at femoral supracondylar and internalfixation combined with hybrid external fixator. Among the patients, 19 patients were male and 19 patients were female, ranging in age from 14 to 58 years, with an average of 26 years. Six patients had genu valgum, 4 patients had varus of the distal end of femur, 2 patients had varus combined with rotation deformity,3 patients had genu recurvation and 23 patients had deformity of genuflex. The function of knee joint was evaluated according to Dimeglio clinical assessment after the treatment.

RESULTSAll the patients were followed up and the during ranged from 6 to 48 months, with a mean period of 23 months. All the deformity obtained satisfactory correction, without complications such as limitation of motion of the knee joint, under correction or over correction,displacement of the section of the osteotomy,internal fixation loosening and fracture. Nineteen patients got an excellent result, 12 good, 5 poor and 2 bad.

CONCLUSIONSupracondylar femoral osteotomy and internal fixation combined with hybrid external fixator is a safe and effective surgery for the treatment of deformity of distal end of femur. This method combines the advantages of internal fixation and external fixation.

Adolescent ; Adult ; External Fixators ; Female ; Femur ; abnormalities ; surgery ; Fracture Fixation, Internal ; methods ; Humans ; Male ; Middle Aged

OBJECTIVETo study the methods and effects of Ilizarov distraction technique in treating the flexion deformity of the knee of arthrogryposis multiplex congenita.

METHODSBetween August 1998 and February 2003, 6 patients (10 knees) with the arthrogryposis multiplex congenita were treated, 4 patients in double knees, 5 males, 1 female, mean age 8 years and 2 months, ranged from 3 years and 7 months to 13 years. The preoperative flexion degree was averagely 51 degrees. The patients accompanied 13 other parts malformation of limbs. The modified Ilizarov distraction apparatus of the knee was used. While installing the apparatus in the operation, the knees should be kept in the of location of maximum extension, the center of joint hinges on the apparatus should be placed towards the rotatory center of the knee, two groups of 2 mm K-wires were passed through the femur and tibia around the knee, who were fixed on the proximal and distal rings. Distraction was started after the surgical procedure 5 days via rotating the threaded rods at the posterior of the knee, at an average of 2 to 3 mm per day, at the first week, after 2 weeks with the rate modified to 2 mm per day, up to the knee extended to 0 degrees. The accompanied deformities of the hip and/or the foot might be corrected at the same time or next time. The average duration of the distraction was 37 days (23-48 days). During the correction all limbs might undergo weight. After 2 weeks at the end of distraction the fixator was removed and the patients could walk by a long-leg brace.

RESULTSTen knees with the flexion contracture were sufficiently corrected without severe complications. Nine knees of all were followed up at an average time of 1 years and 3 mouths, no recurrence of the deformity was seen in all patients, their function of walk was significantly improved.

CONCLUSIONSIlizarov technique is a simply, safe and effective method for managing the flexion deformity of the knee of the arthrogryposis multiplex congentia. The procedure is conformable to the biological theories and microsurgical principles.

Adolescent ; Arthrogryposis ; surgery ; Child ; Child, Preschool ; Female ; Humans ; Ilizarov Technique ; Knee Joint ; surgery ; Male ; Retrospective Studies ; Treatment Outcome