Objective To report the development of clinical symptoms in a Chinese family with autosomal dominant progressive external ophthalmoplegia(adPEO).Methods Electromyologram and muscle biopsy were performed in the proband and 4 family members with the disease.Results The proband was a 57 year-old woman,who developed bilateral ptosis after the age of 30,external ophthalmoplegia after the age of 35 years old,weakness of extremities at the age of 37 years old and bulb palsy with palmus at the age of 47 years old.In the family there were 20 male and female members from five generations.All of them complained about bilateral ptosis between 26—33 years old,external ophthalmoplegia(12/15)and weakness of all extremities(14/15)between 35—45,facial and masticatory weakness(9/9)as well as dysphagia(8/9)between 44—60,accompanied with heart lesions(4/7)after 50 years old.Some patients died due to cardiac impairment.Electromyologram showed myopathic abnormalities in the examined patients. The main myopathological changes were ragged red fibers,cytochrome c oxidase negative fibers and ragged blue fibers in succinate dehydrogenase staining.Conclusions The adPEO started from extra-ocular muscles to limbs,finally facial and bulbar muscles.Heart lesions were presented in late stage and lead to death in some members.The developing process of symptoms suggested that we should pay more attention to cardiac manifestations in this disease.

Objective To investigate the axonal lesion in chronic inflammatory demyelinating polyneuropathy(CIDP).Methods Eighteen patients had undergone sural nerve biopsy.The clinical and electrophysiological distinction based on the different pathological changes were analyzed.Results Five patients with demyelination predominance which presented myelinated fiber with thin myelin.Three of them showed also mild axonal degeneration.Eight patients with axonal lesion predominance which presented Wallerian degeneration and regeneration of myelinated fibers.Three patients with mixed myelin and axon lesion of myelinated fibers and two with mild lesion.There was no significant difference between CIDP predominantly with axonal lesion and demyelination.Electrophysiological examination shows both axonal lesion and demyelination feature in some of the 2 types patients at the same time.Conclusions Axonal lesion is a common pathological change in CIDP and should not be considered as an exclusive criterion in diagnosis of the disease.Infiltration of macrophages is a common change.

OBJECTIVETo study the hepatotoxicity effects in rats with different extract of Fructus Gardeniae.

METHODObserve the change of appearance, behavior and weight of rats through oral gavage daily for 3 d. Weigh the liver and calculate the liver index. Detect the ALT, AST and TBIL. Observe the liver tissue by optical microscope.

RESULTThe weight and index of liver were increased by 3.08 g x kg(-1) aqueous extract, 1.62 g x kg(-1) alcoholic extract and 0.28 g x kg(-1) geniposide, compared to those of the blank group (P < 0.005, P < 0.001) and the activities of ALT, AST and the content of TBIL were also increased, compared to those of the blank group (P < 0.05, P < 0.001). The liver cells were obviously swell, necrotic and changed with inflammatory infiltrate.

CONCLUSIONAqueous extract, alcoholic extract and geniposide displayed hepatotoxicity, and the geniposide which was the main substance of the Fructus Gardeniae might be mainly responsible for the hepatotoxicity.

Alanine Transaminase ; blood ; Animals ; Aspartate Aminotransferases ; blood ; Bilirubin ; blood ; Drugs, Chinese Herbal ; isolation & purification ; toxicity ; Female ; Fruit ; chemistry ; Gardenia ; chemistry ; Iridoids ; isolation & purification ; toxicity ; Liver ; pathology ; Male ; Organ Size ; drug effects ; Plants, Medicinal ; chemistry ; Pyrans ; isolation & purification ; toxicity ; Random Allocation ; Rats ; Rats, Sprague-Dawley

BACKGROUNDRadiofrequency (RF) ablation has become a widely accepted treatment for atrial fibrillation (AF). This study aimed to identify the efficacy and safety of pulmonary vein (PV) ablation with ethanol and to explore an alternative energy source for catheter ablation of AF.

METHODSTwelve open-chest mongrel dogs were randomized into ethanol ablation group and control group. Both the injections and electrophysiological mapping procedures were performed epicardialy. In ethanol ablation group (n = 6), injections were performed to circumferentially ablate the root of each PV (0.2 ml each site, 3 mm apart) with 95% ethanol using an 1 ml injector. In control group (n = 6), saline was injected other than ethanol. PV isolation was confirmed with a circular catheter immediately after the procedure and at follow up of 30 days. PV isolation was defined as the absence of PV potentials at each electrode of the circular catheter positioned at the PV side of the lesions, as well as complete conduction block into left atrium (LA) during PV pacing.

RESULTSPV electrical isolation with complete bidirectional conduction block was achieved with ethanol immediately and at 30 days in 95% of PVs, while saline injection caused only transient conduction changes between LA and PVs. In ethanol group, histologic analysis showed transmural lesions at 30 days. And there was no evidence of PV stenosis or thrombus formation. Mean LA diameter was not significantly different between baseline and 30 days.

CONCLUSIONEthanol is a safe energy source to effectively isolate PV in canine model and may be promising in endocardial ablation procedure of AF patients in the future.

Animals ; Catheter Ablation ; methods ; Dogs ; Electrophysiology ; Ethanol ; Pulmonary Veins ; physiology ; surgery ; Random Allocation

Aim To analyze the molecular mechanism of rhubarb in the treatment of aeute pancreatitis ( AP) by network pharmacology and molecular docking.Methods TCMSP,TCMID and Swiss target predic¬tion databases were used to screen the active compo¬nents and targets of rhubarb,and genecards and OMIM databases were used to screen the targets of AP.Then the active ingredient drug target network of rhubarb and theactive ingredient disease target network of rhubarb for AP were constructed by using Cytoscape software.PPI network was constructed in string database, and go and KEGG enrichment analysis was performed in metascape database and R language.Finally,molecular docking was used to verify the possibility of binding the core active components to the core target.Results A total of 192 active components and 1 882 AP targets were obtained.The first three active components of rhubarb in the treatment of AP were beta sitosterol, aloe emodin and eupatin.The core target of rhubarb in the treatment of AP was hsp90aal.Go enrichment analysis focused on reaction to toxic substances, while KEGG enrichment analysis was significantly enriched in p53 signaling pathway closely related to AP.Molecular docking showed good binding and stable conformation.Conclusions Rhubarb can affect the expression of AP related genes and proteins through p53 signaling pathway, thereby inhibiting cell apoptosis and allevia¬ting the inflammatory injury of AP.

OBJECTIVE: To provide reference of the ideal entry point for antegrade femoral nailing according to analysis correlation between highest point of greater trochanter and the middle line of the medullary cavity in adults by three-dimensional images. METHODS: From January 2016 to January 2017, 107 adults who underwent continuous computed tomography (CT) scans were ultimately enrolled, including 64 males and 43 females with an average age of (51.7±16.4) years old;54 patients on the left side and 53 patients on the right side. Three-dimensional images were built by using image-processing software (Volume Viewer) to reconstruct geometry of cortex and medullary canal. All people weregrouped according to different femoral greater trochanter morphology, such as anterior apex (AA), posterior apex (PA), middle apex (MA) and none apex (NA). Forwards inclination was adjusted to apparent neck shaft angle (ANSA) and true neck shaft angle (TNSA) on the coronal and saggittal view, recorded as C-ANSA, C-TNSA, S-ANSA, S-TNSA respectively, vertical distance from the middle line of femur medullary cavity to the highest point of greater trochanter of femur on the 4 positions were measured and performed statistical analysis, multiple linear regression was applied to analysis relationship between clinical data and VD value. RESULTS: (1)Comparison of VD value among 4 groups on the 4 positions, there were no difference in VD value between AA and MA groups on the S-ANSA position;and no differences in VD value among AA, MA and NA groups on the C-ANSA and C-TNSA position. (2)There were differences in VD value among AA, MA and NA groups on the sagittal plane;while had difference in VD value between PA and NA group on the coronal plane. (3)Prediction equation of VD value on S-ANSA and S-TNSA position by multiple linear regression showed R=0.343, F=3.409, =0.012 on the S-ANSA position;R=0.317, F=2.846, =0.028 on the S-TNSA position; neck shaft angle and sex were risk factors of VD value on the sagittal plane, while no difference in VD value on the coronal position. CONCLUSION (1)When indentify insertion point in adult femoral nail according to the highest point of greater trochanter as anatomic landmark, the morphology of greater trochanter of femur should be distinguished to certain observation position, then evaluate migration before and after on the sagttial plane and lateral offset on the coronal plane. (2)Migration before and after on the sagttial plane is increase with increase of neck shaft angle, and the degree of migration of female before and after is less than that of male.
Adult ; Aged ; Female ; Femur ; Fracture Fixation, Intramedullary ; Humans ; Image Processing, Computer-Assisted ; Imaging, Three-Dimensional ; Male ; Middle Aged ; Tomography, X-Ray Computed

OBJECTIVETo compare the efficacy of Kirschner wire tension band with hole and common Kirschner wire tension band in the treatment of olecranon fractures in adults, so as to guide the selection of clinical surgical procedures.

METHODSFrom July 2011 to October 2015, a total of 49 patients with olecranon fractures underwent open reduction and fixation with Kirschner wire tension band, which were retrospectively analyzed. Of the 49 patients, 21 patients(group A) were treated with Kirschner wire tension band with hole, including 12 males and 9 females, with an average age of(37.6 ±8.2) years old;28 patients(group B) were treated with common Kirschner wire, including 18 males and 10 females, with an average age of(38.9±7.3) years old. There were no statistically significant differences between the two groups in general data. The differences of operative duration, frequency of radiation, fracture healing time, complications and postoperative elbow function scores between the two groups were observed and analyzed by parallel statistical analysis.

RESULTSIn group A, the operative duration was(60.4±10.7) min, the average number of radiation times was (12.5±2.9); in group B, the operative duration was (62.3±11.8) min, and the average radiation times was(13.7±3.8); there was no significant difference in operative time and radiation times between the two groups (P >0.05). In group A, the fracture healing time was (13.2±2.6) weeks without K-wire migration, skin irritation and other complications; and fracture healing time in group B was(14.6±1.8) weeks with complications(K-wire migration in 6 cases, skin irritation in 7 cases, internal fixation failure in 2 cases);the fracture healing time of group A was shorter than that in group B. Evaluation of elbow joint function in two groups of patients after operation showed that in group A, pain score was 41.0±3.5, movement function score was 18.0±2.1, stability score was 9.0±0.8, daily activities score was 18.0±4.3, the total average score was 87.0±7.8; and in group B, the pain score was 39.0±5.6, movement function score was 17.0±3.2, the stability score was 8.0±2.4, daily activities score was 16.0±5.2, the total average score was 83.0±10.7. There were no statistically significant in the scores between the two groups.

CONCLUSIONSCompared with ordinary Kirschner wire, the treatment of olecranon fracture with Kirschner tension band with hole can shorten the time of fracture healing, significantly reduce the occurrence of complications, and do not affect the recovery of postoperative function, which is suitable for clinical use.