Objective We reported 16 eosinophilic fasciitis (EF) patients with eosinophilic fasciitis and performed a systematic review of the literature to improve the disease awareness.Methods The clinical course of 16 patients with eosinophilic fasciitis at the Peking Union Medical College Hospital were described,inclu-ding demographic data,clinical manifest-ations,laboratory tests,pathology and treatment.Results The mean age at diagnosis was (47±8) years,with 13 female and 3 male patients.Three cases had exertion or strenuous sports before the onset of EF.Positive ANA was noted in 6 of 12,positive RF was noted in 3 of 10,hyper-gammaglobulinemia was noted in 6 of 7,elevated IgG was noted in 8 of 13,peripheral blood eosinophilia was noted in 10 of 16,while thrombocytopenia was found in one patient.Conclusion Based on this and other reported cases in the literature,EF may be a kind of autoimmune disease.Genetic influence and environ-mental factors are involved in the development of this disease.Systemic involvement is rare.In general,corticosteroids and immunosuppressive are effective in EF.

Objective To analyze the clinical data of diagnosis and treatment of Wegener' s granulomatosis (WG) in order to understand the nature of this disease. Methods The clinical data including clinical manifestations,laboratory findings imaging features,pathological changes and efficacy of treatment of 34 patients with WG admitted from January 2002 to March 2012 were analyzed.Results Of the 34 patients,male to female ratio was 18 to 16,and the average age subjected to WG onset was (45 ± 15)years old ranged from 18 to 81 years old.The average duration before the diagnosis confirmed was ( 140 ±72) mouths,while 29.4％ was diagnosed within 3 months. The presenting symptoms included initial involvements in lung (41.1％),nose (38.2％ ),eyes ( 11.7％ ),ear (8.8％ ) and constitutional symptoms such as fever (26.4％ ). Throughout the whole disease course,the incidence of systemic impairments were as follows:lung (76.4％),nose (73.5％),kidney (67.6),eyes (58.5％),ear (47.0％),nervous system (41.1％),oral ulcer (20.5％ ).Of laboratory findings,C - ANCA/PR3 -ANCA was positive in 61.7％ patients,P - ANCA/MPO - ANCA positive in 20.5％ patients,ANCA negative in 11.7％ patients,P- ANCA positive with MPO- ANCA negative in one patient (2.9 ％ ) and C -ANCA positive with PR3 and MPO positive in one patient (2.9％ ).Of imaging findings,nodules or masses were most commonly observed (34％), followed by fibrotic lesions (20.5％ ), cavitations ( 17.6％ )、opacities ( 17.6％ ).Typically pathological triad ( vasculitis,necross and granuloma formation)was found in one patient ( 4.3％ ),while two pathological changes of the triad occurred in 10 patients (43.4％). Pulmonary infection was highly prevalent (50％), and of them,20.5％ patients were misdiagnosed as tuberculosis,malignancy or abscess.After admission,high dose of corticosteroids and cyclophosphamide were administered as bolus and maintenance therapy. Plasma exchange,intravenous immunoglobulin,and rituximab were added for refractory cases and optimal response were obtained in most cases.Conclusions Wegeners granulomatosis is a clinically complicated entity and sometimes can have progression,leading to misdiagnosis. Early and prompt use of steroids and cyclophosphamide may confer good therapeutic efficacy and avoid life -threatening complications.

ObjectiveTo evaluate the clinical safety and efficacy of SilverHawk directional atherectomy for femoropopliteal occlusive lesions. MethodsEighteen ischemia occlusive lesions in 11 patients of the lower extremity were treated with SilverHawk directional atherectomy.The mean lesion number was 1.6 ± 1. 1 per patient. The mean lesion length was ( 3.4 ± 2. 2 ) cm. The average degree of diameter stenosis was 96％ ± 14％. 9 lesions were totally occlusive. Clinical symptoms included claudication in 4 cases ( Rutherford classes: 3) and critical limb ischemia ( Rutherford classes: 4) in 7 cases. Lesions characteristics were divided by TASC classification: TASC B in 7 cases; TASC C in 1 case (in-stent occlusion); TASC D in 3 cases.Mean ABI was 0. 5± 0.4. Patency was evaluated with color duplex sonography or CTA besides clinical examination during follow-up.ResultsNine totally occlusive lesions were recanalizated successfully via intraluminal approach. 18 lesions achieved technical success (residual stenosis ＜50％ ) leaving 15％ ±7％ mean residual stenosis in mean (8 ±3)min, predilation was needed in one lesion ( in-stent occlusion) prior to atherectomy. Clinical symptoms improved or disappeared with mean ABI 1.07 ±0. 12 and Rutherford grades: 0 (n =9) and 1 (n =2). Patency rate was 100％ with mean 0. 93 ± 0. 14 ABI and Rutherford grades remain unchanged after follow-up of mean ( 9 ± 4 ) monthes.ConclusionsSilverHawk directional atherectomy is safe and effective for the treatment of lower extremity ischemia.

ObjectiveTo evaluate the clinical application of endovascular treatment for severe Takayasu's arteritis (TA).MethodsIn this study,35 target lesions in 32 patients [28 women,mean age (30 ±8) years] with severe Takayasu's arteritis were treated with endovascular merthod.The average length of lesion was 3.1 cm( range 2.7 -5.3).The overall average degree of diameter stenosis was 90％ ± 11％ (range 70- 100)in which 15 lesions were completely occlusive.There were 10 patients whose ESR were higher than 20 mm/h( range 25 -37).Follow-up included physical examination and patency evaluated by color duplex souography/computed tomography angiography/angiography at 6 months and then annually.ResultsRecanalization was unsuccessful in 3 completely occlusive lesions,with a successful rate of 80％(12/15).There was one case in which embolization leading to acute thrombogenesis developed during interventional procedure and resulting in severe stroke.The technical successful rate ( residual stenosis ＜ 50％ ) was 88.6％ ( 31/35 ).The transient cerebral ischemia attack ( TIA ) symptoms disappeared in 31 cases.26 cases were followed up for an average of (19 ± 10) months (range 13 -40).Occipital infarction following severe in-stent restenosis developed 13 months later in one case.Symptomatic in-stent restenosis18monthslaterwasfoundin2cases. Patencyratewas88.5％( 23/26 ).ConclusionsEndovascular treatment is safe and effective for severe TA.Strict indication and accurate targeting the lesions help ensure the success of management.

AIMTo study the chemical constituents of Glechoma longituba (Nakai) Kupr.

METHODSManifold chromatography methods were used to separated the chemical constituents, and the chemical structures were determined by spectral analyses.

RESULTSNine compounds were isolated from Glechoma longituba and identified, as: glecholone (1), 6R,9R-3-oxo-alpha-ionol (2), S(+)-dehydrovomifoliol (3), vomifoliol (4), corosolic acid (5), quercetin (6), stigmastenol (7), myristic acid (8) and triacontanol (9).

CONCLUSIONCompound 1 is a new compound. Compounds 2 - 9 were isolated from this plant for the first time.

Cyclohexanones ; chemistry ; isolation & purification ; Lamiaceae ; chemistry ; Molecular Conformation ; Molecular Structure ; Plant Components, Aerial ; chemistry ; Plants, Medicinal ; chemistry ; Triterpenes ; chemistry ; isolation & purification

OBJECTIVETo study the chemical constituents of Prunella vulgaris.

METHODTo separate the constituents of P. vulgaris by using various kinds of chromatography and identify their structures on the basis of spectral analysis.

RESULTSeven compounds were isolated from the spikes of P. vulgaris. Their structures were established as autantiamide acetate (1), rhein (2), tanshinone I (3), danshensu (4), stigmast-7, 22-dien-3-one (5), 3, 4, alpha-trihydroxy-methyl phenylpropionate (6), butyl rosmarinate (7).

CONCLUSIONCompounds 1-4 were isolated from this genus for the first time.

Amides ; chemistry ; isolation & purification ; Anthraquinones ; chemistry ; isolation & purification ; Chromatography, High Pressure Liquid ; Diterpenes, Abietane ; Flowers ; chemistry ; Lactates ; chemistry ; isolation & purification ; Magnetic Resonance Spectroscopy ; Phenanthrenes ; chemistry ; isolation & purification ; Plants, Medicinal ; chemistry ; Prunella ; chemistry

Modern drying technology was used to explore suitable drying process to provide scientific basis for improving drying processing methods of Scrophulariae Radix. Controlled temperature and humidity drying, vacuum drying apparatus, microwave vacuum drying apparatus, short infrared drying device were used to gain samples for analyzing. The character appearance, concentration of main components and power consumption indicators were chosen for preliminary judging. Six major components, including iridoids and phenylpropanoids were analyzed by UPLC-MS/MS method. The contents of polysaccharides were determined by UV-visible spectrophotometry. The character appearance with controlled temperature and humidity drying and short infrared drying meet the pharmacopoeia standard (Ch. p, edition 2015), while samples with vacuum and microwave vacuum drying apparatus didn't. Compared to fresh sample, concentrations of harpagide, harpagoside, aucubin and catalpol were lower in the dried samples. Angoroside-C showed no significant change before and after drying. Concentration of acteoside increased after drying. Samples with controlled temperature (70 degrees C) and humidity (15% - 10%) drying had high content and short drying time. The better drying process of Scrophulariae Radix was controlled temperature and humidity drying. The method will provide the reference for the drying technology standard of roots medicine.
Desiccation ; methods ; Drugs, Chinese Herbal ; chemistry ; Plant Roots ; chemistry ; Quality Control ; Scrophularia ; chemistry

To improve the quality standard of Angelica sinensis, solve the problem of lacking relevant reference substance, a new method-based on the standard reference extract (SRE) was applied to achieve the quality control of Angelica sinensis. SRE of Angelica sinensis was obtained by chromatographic separation technology. After calibration of three makers of the SRE, an UPLC analytical method was developed to determinate the contents of the makers. T-test was used for comparison of the determination results of two methods (reference substances and SRE as reference, respectively), and the results demonstrated that there is no significant difference between the two methods. The presented method is very convenient and practical, which can be used for the quality control of Angelica sinensis.
Angelica sinensis ; chemistry ; Calibration ; Chromatography, High Pressure Liquid ; standards ; Drugs, Chinese Herbal ; chemistry ; standards ; Quality Control ; Reference Standards

OBJECTIVETo compare results of the growth and development of the upper dental arch and the velopharyngeal closure of the cleft patients treated by two methods.

METHODSThe dental cast of patient and X-ray films were measured and the statistical medical records were analyzed.

RESULTSThe transverse distance of upper dental arch was found to be wider in group A than in group B. The anterior-posterior distance of the dental arch in bilateral cleft group was shorter in group A than in group B. The difference of the two groups were gradually lessened as age increases. Bony bridge in alveolar gap was 63% and 83.3% in unilateral and bilateral cleft group respectively. 15% of cases in group A and 35.2% in group B needed pharyngeal flap.

CONCLUSIONSThe stable upper dental arch in group A can opposes the pressure from the lip muscles, this maintains the width of the arch. But A-P distance of upper dental arch in BCLP in group A should be followed up after the age of 9 years. Pharyngeal flap is needed less in group A than in group B.

Alveolar Process ; growth & development ; Child ; Child, Preschool ; Cleft Lip ; surgery ; Cleft Palate ; surgery ; Humans ; Infant ; Orthodontics, Corrective ; Palate, Soft ; surgery