ObjectiveTo investigate the clinical and pathological characteristics of dermatomyositis with muscular perifascicular atrophy (PFA).MethodsA series of 104 consecutive patients clinically and pathologically diagnosed as dermatomyositis by muscle biopsy in our laboratory from December,2003 to August,2011,were enrolled in this study. Muscle biopsy of all the enrolled patients had shown PFA of muscle fibers.ResultsAmong the 104 patients,34 were males and 70 were females with a mean age of 45 years old.Among them,8 cases had normal electromyogram;42 had normal serum creatine kinase level;11 were diagnosed as carcinoma;75 were found to be combined with interstitial lung disease (ILD).Based on morphologic changes of muscle biopsy,they were divided into pure PFA group with 54 cases and PFA plus focal damage group with 50 cases.Compared with the pure PFA group,there was prominent mononuclear cell infiltration into perimysial intermediate sized vessels and membrane attack complement (MAC) deposition in the intramuscular capillaries in the PFA plus group.Skin biopsy had been taken in 12 cases together with muscle biopsy and had shown the border effectof both PFA and interface dermatitis in muscle and skin.ConclusionsOur study suggests that chronic immune vascular damage and insufficiency in dermatomyositis may cause ischemia and focal myofiber damage in watershed regions. The incidence of ILD in our dermatomyositis patients with PFA is high.

Objective To explore the pathological changes of the livers from hepatic failure (HF)patients and its association with clinical disease stages.Methods Thirty-nine patients with liver failure caused by HBV infections were investigated,and none accompanied with hepatocellular carci- noma.The sections of tissue were taken from the liver after liver transplantation and stained with he- matoxylin eosin(H&E)or RT(reticular fiber)staining.The pathological features were analyzed and compared between the clinical and pathological diagnosis.Results 1.The range and the grade of the pathological changes were all well-proportioned in the whole liver but quite asymmetrical in the same spicemen.2.4 cases with clinical diagnosis of cirrhosis(active stage)were in accordance with the pathological diagnosis.Only 17 in 35cases can be pathologically diagnosed as chronic severe hepatitis (SH),while the other 18 cases were pathologically diagnosed as cirrhosis(active stage).Conclu- sion There were a great inconsistency between the clinical and pathological diagnosis.

OBJECTIVETo investigate the influence of total flavonoids of Elsholtzia splendens (TFES) on isolated ischemia/reperfusion rat hearts and its underlying mechanisms.

METHODSHearts isolated from male SD rats were perfused on the Langendorff apparatus and subjected to global ischemia for 30 min followed by 120 min of reperfusion. The cardiac infarct size was measured by TTC staining. Hemodynamic parameters and the level of lactate dehydrogenase (LDH) in the coronary effluent were measured. Absorbance at 520 nm was determined in isolated cardiac mitochondria exposed to 200 micromol/L CaCl2 to detect the opening of the mitochondrial permeability transition pore.

RESULTSPretreatment with TFES (1, 10, 100 microg/ml) for 5 min decreased infarct size and LDH release and improved the recovery of the left ventricular developed pressure. In mitochondria, the decrease of absorbance at 520 nm evoked by CaCl2 was greatly inhibited by TFES.

CONCLUSIONTFES prevents myocardial ischemia/reperfusion injury, and this cardioprotective effect is probably via inhibiting mitochondrial permeability transition pore opening.

Animals ; Cardiotonic Agents ; pharmacology ; Disease Models, Animal ; Flavones ; pharmacology ; In Vitro Techniques ; Lamiaceae ; chemistry ; Male ; Mitochondria, Heart ; drug effects ; Mitochondrial Membrane Transport Proteins ; drug effects ; Myocardial Reperfusion ; Myocardial Reperfusion Injury ; prevention & control ; Rats ; Rats, Sprague-Dawley

The biochemical mechanism of degrading keratins by S.fradiae var S-221 was primarily studied.The compounds (Na_ 2 SO_ 4 , Na_ 2 SO_ 3 and sulfdryl acohol), which respecitively enhance specific activity of keratinase, activate keratinase intensively and mainly act on the disulfide bonds reductase in the keratinase, Na_ 2 SO_ 3 activates intensively both disulfide bonds reductase and polypeptide hydrolytase at 0.01 mol/L, whereas Na_ 2 S_ 2 O_ 3 , which acts on the disulfide bonds reductase, inhibits keratinase.On the condition that substrate, keratins exists, S.fradiae var S-221 is induced to produce exo-keratinase, which is a multiproteinase, containing disulfide bonds reductase, which is a key enzyme degrading keratins, then, with polypeptidic, hydrolytase, graduately hydrolyzates denatured keratins into polypeptides, oligopeptides and free amino acids, so that keratins have been decomposed completely.Sulfur in the keratins was transferred into sulfhydryl compounds, H_ 2 S and sulfates in the course of keratinolysine.

ObjectiveIn an attempt to clarify the usefulness of combined nerve and muscle biopsy in the diagnosis of neuromuscular disease when compared with traditional sural nerve biopsy.Methods Fifteen biopsies of superficial peroneal nerve (SPN) and peroneus brevis muscle ( PBM ) by one incision performed within one neurological clinic were reviewed.All patients had peripheral neuropathy while 3 of them had myopathy clinically.The diagnostic significance of SPN and PBM biopsies were classified into 3 grade: essential,helpful,no value.ResultsOf 15 SPN and PBM biopsies,7 showed essential pathological findings whichreachedthe etiologicaldiagnosis, including 5definitevasculitis, 1inflammatory demyelinating polyneuropathy and 1 amyloid neuropathy.Five biopsies are helpful for etiological diagnosis,including demyelinating neuropathy,mild inflammation,and microvascular lesion,et al.Three biopsies are of no value for etiological diagnosis which only have nonspecific change such as type 2 fiber atrophy,neurogenic atrophy and axonal degeneration et al. Finally,SPN and PBM biopsies made the definite etiological diagnosis possible in 12 patients.ConclusionsSPN and PBM biopsy improved the yield of specific pathological and etiological diagnosis of neuropathy and myopathy such as vasculitis and amyloidosis with minor trauma and side effect.Further clinical and pathological studies will be necessary for a better practice of combined nerve and muscle biopsy.

OBJECTIVETo discuss the clinical effect of closed reduction and interlocking intramedullary nailing in the treatment of femoral shaft fracture.

METHODSFrom March 2006 to December 2011,103 patients with femoral shaft fracture were treated by closed reduction and interlocking intramedullary nailing including 76 males and 27 females with an average age of 36 years old ranging from 19 to 55 years old. According to AO classification,there were 64 cases with type A,27 with type B, 12 with type C. Thirteen cases were open fractures including 5 cases with Gustilo type I , 8 with Gustilo type II . The time of bone healing were observed after operation, the knee function recovery was evaluated by HSS scoring standard at 1 year after operation.

RESULTSThe intraoperative complications included femoral neck fracture in 1 case and proximal femoral fracture in 1 case,both of the patients were treated with reconstructive intramedullary interlocking nail and the fractures healed postoperatively. One patient was suffered from common peroneal nerve injury,which were fully recovered at 4 months later after medical treatment. All the patients were followed up from 12 to 28 months (averaged 22 months). All of the fractures were healed well and the average healing time was 3 to 9 months (averaged 5 months). All the hip joints were recovered to normal function. The average HSS was 90.89±5.06 at 1 year after operation.

CONCLUSIONInterlocking intramedullary nailing is the preferred treatment for patients with femoral shaft fracture. Carefully operating and closed reduction can reduce the complications.

Adult ; Bone Nails ; Female ; Femoral Fractures ; surgery ; Fracture Fixation, Intramedullary ; instrumentation ; Fractures, Open ; surgery ; Humans ; Male ; Middle Aged ; Young Adult

OBJECTIVETo analyze micromovement of distal tihiofihular joints in different motion range of ankle joint ,and define the micromovement characteristic and range of distal tibiofihular joints.

METHODSTwelve normal Chinese were chosen. There were 9 males and 3 females, aged from 19 to 37 years old with an average (26.5 +/- 0.5) years. Detection terminals of laser photographic scanner were installed near the highest point between medial malleolus and lateral malleolos, the change of detection terminals on the position of dorsiflex, extension, introversion and eversion of ankle joint were scanned by 3D-laser scanner. The displacement of two detection terminals on the X ,Y and Z-axis (X-axis stands for the vertical-axis between coronal plane and Z-axis Y-axis stands for the vertical-axis between sagittal plane and Z-axis Z-axis stands for macroaxis of tibia).

RESULTSAlong with increased range of motion on the position of dorsiflex ,extension, introversion and eversion of ankle joint, the range of micromovement of distal tibiofibolar joints increased too. The max-displacement of X, Y and Z were respectively (1.04 +/- 0.12) mm, (1.70 +/- 0.16) mm and--(0.87 +/- 0.10) mm.

CONCLUSION3D-laser scanner can be used to determine the detailed displacement of distal tibiofibolar joint on the X , Y and Z, and measure the motion of distal tibiofibular joint. The method can be used to study the pathologic change of distal tibiofibular joint ,and provide basic biomechnics data for internal fixtor fitting for the characteristic of distal tibiofibular joint.

Adult ; Ankle Joint ; physiology ; Asian Continental Ancestry Group ; Female ; Fibula ; Humans ; Male ; Movement ; Range of Motion, Articular ; Tibia ; Young Adult

OBJECTIVETo observe the effect of Tangke Decoction (TD) on the expression of TGF-beta1/Smad4 of rats with early diabetes and to explore the effect and mechanism of TD against the renal injury induced by diabetes.

METHODSSD rats were randomly divided into the normal control group (n = 12), the model group (n = 10), the Chinese herbs prevented group (n =10), the Chinese herbs treated group (n = 10), and the Western medicine control group (n = 10). TD (18 mg/kg) was given by gastrogavage to rats in the Chinese herbs prevented group immediately after successful modeling for 12 weeks, once daily. At the 4th week of successful modeling, rats in the rest 4 groups were administered by gastrogavage. Equal volume of normal saline was given to rats in the model group and the normal control group. Benazepril suspension (1 mg/kg) was administered by gastrogavage to rats in the Western medicine control group for 8 weeks, once daily. TD (18 mg/kg) was given by gastrogavage to rats in the Chinese herbs treated group for 8 weeks, once daily. The body weight, kidney weight, index of kidney weight, fasting blood sugar, 24 h urinary albumin excretion rate were examined after experiment. The pathological changes of the renal tissue were observed by HE staining, Masson staining, and electron microscope. The expression of renal transforming growth factor-beta1, (TGF-beta1) and Smad4 were detected using immunohistochemical assay.

RESULTSCompared with the normal control group, the body weight of rats decreased significantly; the kidney weight, index of kidney weight, blood sugar, 24 h urinary protein excretion, the urinary albumin excretion rate,TGF-beta1 and Smad4 expression increased significantly in the model group (all P < 0.01). Compared with the model group, the aforesaid indices were improved in each treatment group with statistical difference (P < 0.05, P < 0.01). Compared with the Western medicine control group, the kidney weight, index of kidney weight, blood sugar, 24 h urinary protein excretion, and the urinary albumin excretion rate were obviously improved in the Chinese herbs prevented group (P < 0.01). The renal pathological changes were most obvious in the model group significantly, but they were improved in all treatment groups.

CONCLUSIONTD could obviously improve the symptoms of diabetes and down-regulate the expression of renal TGF-beta1 and Smad4 of early diabetic nephropathy rats, which suggested that TD had certain preventive effect on early diabetic nephropathy.

Animals ; Diabetes Mellitus, Experimental ; drug therapy ; metabolism ; Drugs, Chinese Herbal ; therapeutic use ; Kidney ; metabolism ; Male ; Rats ; Rats, Wistar ; Smad4 Protein ; metabolism ; Transforming Growth Factor beta1 ; metabolism

Objective To summarize the clinical and pathological features of glycogen storage disease (GSD) type Ⅱ. Methods The clinical and pathological data of the 20 GSD type Ⅱ patients were reviewed. Results One patient with infantile-onset mainly presented hypotonia, muscle weakness, feeding difficulties, pulmonary infection and cardiomyopathy insufficiency and increase of serum creatine kinase (778 IU/L) and echographic evidence of hypertrophic cardiomyopathy were detected. Electromyography studies indicated a definite myopathy. Nineteen cases were late-onset, presenting a slowly progressive proximal myopathy with truncal involvement or with symptoms dominated by respiratory insufficiency. Not all muscles were equally affected. Increase of serum creatine kinase (208-2600 IU/L) was detected in 14 patients and normal level in 1 patient. Electromyography studies indicated a definite myopathy in 9 patients,with abnormal irritability in 1 patient and susceptible in 4 patients and myotonic discharge in 1 patient and no abnormalities in 2 patients. Echographic evidence of thickening of the interventricular septum and pulmonary hypertension were detected in 2 patients respectively. The common light microscopic feature of all case was a vacuolar myopathy with high glycogen content and acid phosphatase activity in the vacuoles. Conclusions GSD type Ⅱ often presents slowly progressive myopathy which often affect the toro and respiratory muscles.In most patients the serum creatine kinase level is elevated slightly. Muscle biopsy is of use to make the definite diagnosis of this disease.

Objective To explore the clinical efficacy and safety of using simple arthroscopic operation combined with post-operative radiotherapy in the treatment of knee joint diffuse pigmented villonodular synovitis (DPVNS).Methods A total of 30 cases with knee DPVNS in Wuhan Central Hospital and General Hospital of Beijing Military Region from January 2009 to January 2014 were enrolled,including 18 males and 12 females with average age of 42.5 years (28-64 years).The average duration was 35.6 months(4-121 months).According to the decision of the patients,16 cases treated with operation combined with postoperative radiotherapy and the other 14 cases with operation alone.The treatment group accepted arthroscopic synovial excision and received local radiotherapy,the total dose of radiotherapy was 40 Gy,2 Gy/fraction.While the control group received arthroscopic operation alone.Patients were followed up,which the average time of 38.7 months (12-72 months).The recurrence rate,clinical KSS score and function score were compared between the two groups.Results The efficiency of treatment group (14/16) was significantly higher than the control group (11/14) (x2 =9.87,P ＜ 0.05),and the recurrence rate of the former(1/16) was significantly lower than that latter (3/14) (x2 =1.83,P ＜ 0.05).Furthermore the KSS clinical score and function score were improved significantly in the treatment group(x2 =15.00,15.78,P ＜ 0.05).The main side effect of radiation therapy were leucopenia,limb edema,skin pigmentation and so on,meanwhile the treatment group was well tolerated.Conclusions Arthroscopic operation combined with postoperative radiotherapy is safe and effective in treatment of DPVNS,and it is worthy of clinical application.