Objective To study the clinical pathological features of Wegener's granulomatosis (WG) in middle-aged and elderly patients,and enhance understanding of this disease.Methods Totally 21 patients with WG (11 males,10 females,aged 45 to 76 years,mean age 58.1 years) in our hospial from February 1999 to July 2012 were selected.The clinical and pathological data of WG patients were retrospectively analyzed.34 biopsies including 2 autopsies from different organs were paraffin embedded and stained by hematoxylin and eosin and histochemistry.13 renal biopsies were all examined by immunofluorescence and electron microscope.Results The average time from the onset of clinical symptoms to the diagnosis was 5.3 months (from 24 days to 11.0 months).Eyes,nose and salivary glands were the most commonly involved parts at the beginning of Wegener's granulomatosis (52.4％,11 cases).The percentages of the skin,lung and renal involvement were 14.3％ (3 cases),81.0％ (17 cases) and 71.4％ (15 cases),respectively.Among 21 patients,18 patients were examined with anti-neutrophil cytoplasmic antibody (ANCA).c-ANCA was positive in 72.2 ％ patients (13 cases,13/18),p-ANCA was positive in 16.7％ patients (3 cases,3/18),and ANCA was negative in 11.1％ patients (2 cases,2/18).3 major pathological manifestations were observed:7 kinds of vasculitis including capillaritis,acute vasculitis,chronic vasculitis,fibrinoid necrosis in vasculitis,necrotizing granulomatous vasculitis,non-necrotizing granulomatous vasculitis and cicatricial vascular changes; 4 kinds of granulomatous inflammation including scattered giant cells,palisading histiocytes,poorly formed granulomas and microabscess surrounded by granulomatousinflammation;2 kinds of parenchymal necrosis including geographic necrosis and microabscess.13 kinds of histopathologic features in 3 major manifestations were found from 2 autopsies,but various kinds histopathologic features presented in small biopsy samples.Minor manifestations such as diffuse pulmonary hemorrhage were found at the periphery of WG.Conclusions The wide variation and broad spectrum of pathologic features can occur in WG.Vasculitis,granulomatous inflammation and parenchymal necrosis are the most important histopathological features.The correct diagnosis of WG requires careful correlation of pathology with complicated clinical features.

?-arbutin is biosynthesized by whole cell method with Xanthomona maltophilia BT-112.The conditions for cell biosynthesized ?-arbutin are investigated as follows:temperature,25℃;concentration of hydroquinone,30mmol/L;mol ratio of sucrose and hydroquinone,20∶1;time course of ?-arbutin biosynthesis,45 hours;rotational speed,160r/min;concentration of Xanthomona maltophilia BT-112,85g/L;concentration of K-2HPO-4-KH-2PO-4 buffer solution,25mmol/L;pH of K-2HPO-4-KH-2PO-4 buffer solution,8.0.Under the above optimal conditions,the maximum of molar conversion yield based on the amount of hydroquinone supplied reaches 86.7%.

β-Amyrin synthase (β-AS) genes of Glycyrrhiza uralensis from 6 different regions were analyzed by PCR-SSCP and sequenced, then the correlationship between β-AS SNP and regions of Glycyrrhiza uralensis were determined. According to the 1 coding single nucleotide polymorphism on the first exon of β-AS gene at 94 bp site, Glycyrrhiza uralensis could be divided into 3 genotypes. In these genotypes, the percentage of 94A type in genuine regions was much higher, and it had significant differences with the percentage in non-genuine regions (P < 0.001). The results of the experiment proved that different β-AS genotypes at 94 bp site from different regions may be one of the important reasons to result in the genuineness of Glycyrrhiza uralensis.
Exons ; Genotype ; Glycyrrhiza uralensis ; classification ; enzymology ; genetics ; Intramolecular Transferases ; genetics ; Plant Proteins ; genetics ; Polymorphism, Single Nucleotide ; Polymorphism, Single-Stranded Conformational

A 42-year-old woman was admitted to the hospital for an 11-month history of pustular lesions of the mouth and lips as well as a 5-month history of pustules,ulcer,proliferative plaques on the head,neck and trunk.She had suffered from 8 years of ulcerative colitis.Dermatological examination revealed mild erosion of eyelids,swelling of buccal mucosa and oral lips,as well as typical snail track lesions formed by pustules,exudates and mucilage.There was a large pitchy proliferative plaque sized 20 cm × 10 cm with an elevated margin in the neck as well as palm-to persian walnut-sized proliferative plaques in the prothorax,armpit,periumbilical area and left inguinal area.Histopathology revealed epitheliomatous hyperplasia of the epidermis with the formation of eosinophilic microabscesses,and superficial dermal infiltrate with numerous neutrophils and eosinophils.Direct immunofluorescence tests for IgA,IgG,and C3 were negative.A diagnosis of pyodermatitis-pyostomatitis vegetans was made.The patient was given intravenous methylprednisolone 40 mg/day and symptomatic treatment,which leaded to a gradual subsidence of skin lesions.

Objective To detect the characteristics of benign infantile seizures.Methods Fifty seven cases of benign infantile seizures were analyzed.Results All patients had a normal development before and after the onset of the seizures.The age of onset was from 1.5-30 months.The main manifestations included tonic clonic seizure,staring and motion arrest,64.9 % occurred in clusters.A family history of epilepsy or febrile seizures was present in 6 cases.Interictel electroen cephalograms were normal.The seizures were easily ceased after taking phenobarbital,carbamazepine or valproate.Antiepileptic drugs(AEDs)were discontinued in 51 patients.The mean ages of seizure stopping were 12.8 months and mean seizure′s durations were 4.1 months.Conclusion The benign infantile seizures can be easily controlled with a single AEDs for short time with favorable prognosis.

Methylacetoacetyl-CoA thiolase deficiency (T2 deficiency) is a rare congenital and metabolic disease affecting the ketone body and isoleucine metabolism. The typical symptoms are refractory metabolic acidosis, in which large amounts of 2-methyl-3-hydroxybutyry1 carnitine, 2-methyl-3-hydroxybutyrate and tiglylglycine are often detected in the blood and urine. We herein describe an atypical case of T2 deficiency with a high level of 3-hydroxybutyrate and a low level of 2-methyl-3-hydroxybutyrate in the urine. Such a case was diagnosed by urinary organic analysis in combination with gene mutation evaluation. Organic acids in the urine were measured using a gas chromatography mass spectrometer and all exons were sequenced via deep sequencing. Molecular biology analysis confirmed the presence of a homozygous mutation in the acetyl-CoA acetyltransferase 1 (ACAT1) gene. The patient received a special diet of deeply hydrolyzed protein milk powder and raw corn starch. She was followed about 6 months. There were no ketoacidotic episodes and hypoglycemia even when she had fever. In conclusion, patients with atypical features of T2 deficiency should also be investigated early. Gas chromatography mass spectrometry and next-generation full exome sequencing may be helpful in diagnosis.

Adult ; Air Pollutants, Occupational ; adverse effects ; Chemical Industry ; Female ; Humans ; Insecticides ; adverse effects ; Male ; Maximum Allowable Concentration ; Middle Aged ; Occupational Diseases ; chemically induced ; diagnosis ; Organothiophosphorus Compounds ; adverse effects ; Phosphorus Compounds ; adverse effects ; Sulfides ; adverse effects

This study was purposed to explore the feasibility of BIOMED-2 protocols for detection of immunoglobin (IG) and T-cell receptor (TCR) gene clonal rearrangement in bone marrow of Non-Hodgkin's lymphoma(NHL) patients, and to evaluate its clinical value. Gene clonal rearrangment (IGH, IGK, IGL, TCRβ, TCRγ, TCRδ) was detected by using BIOMED-2 protocols in 73 bone marrow examples of NHL patients. The PCR results were compared with the cytomorphologic examination of bone marrow. The correlation between PCR detection results and clinical stage, pathological factors were also evaluated. The results showed that clonal IG or TCR gene rearrangements were found in 31 of 73 cases (42.5%), higher than the positive rate of cytological analysis (24.7%, 18/73, p < 0.05). IG/TCR clonality rates were 40.0% (22/55) for B-NHL and 50% (9/18) for T-NHL. IG/TCR clonality rates detected in patients with III/IV stage were higher than those with I/II stage (p < 0.05). It is concluded that BIOMED-2 protocols are effective methods for detection of abnormalities in bone marrow in patients with lymphoma, and are superior to cytomorphologic examination. The positive rate of PCR detection is correlated with Ann Arbor stage, but is not related with malignant degree, age, treatment status, B symptoms or the involvement of spleen.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Bone Marrow ; pathology ; Female ; Gene Rearrangement, T-Lymphocyte ; Humans ; Immunoglobulins ; genetics ; Lymphoma, Non-Hodgkin ; genetics ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; Polymerase Chain Reaction ; methods ; Young Adult

OBJECTIVETo discuss the epidemiological and clinical characteristics of the hospitalized children with hand foot and mouth disease (HFMD) in Yantai area.

METHODSEpidemiological and clinical data of HFMD children from 2009 to 2010 were summarized and analyzed retrospectively.

RESULTSMost of the infected (94.6%) were under 5 years old and the ratio between male and female was 1.5: 1. Oral mucosal pox or ulcer as well as hand and foot rashes were observed in all 931 patients. Fever and neurological disorders occurred in 840 (90.2%) and 121 (13.0%) patients respectively. The incidence was positively correlated with air temperature (r = 0.887, P < 0.001), with a peak in April to September (88.9%). The ratio of children from countryside, total duration of fever, serum concentration of c-reacting protein (CRP) and fasting blood glucose (FBG) were significantly higher in severe cases than in those mild ones. Multivariate analysis showed longer mean duration of fever( Odds ratio [OR], 1.491; 95% confidence interval [ CI] 1.170-1.901; P = 0.001) and hyperglycemia (OR, 1.124; 95% CI 1.016-1.245; P = 0.024) were independent risk factors of severity.

CONCLUSIONChildren younger than 5 years old are susceptible to HFMD and most cases occur in April to September. The monthly incidence is positively correlated with temperature of that month. Longer duration of fever and hyperglycemia are independent risk factors for severity. Most cases could have a favorable prognosis after timely diagnosis and proper intervention.

Adolescent ; Child ; Child, Preschool ; China ; epidemiology ; Epidemiologic Studies ; Female ; Hand, Foot and Mouth Disease ; epidemiology ; Humans ; Incidence ; Infant ; Male ; Retrospective Studies ; Seasons ; Temperature

OBJECTIVETo develop a RP-HPLC method for determination of three glycosides in Swertia punicea.

METHODChromatographic column: Alltimal C18 (4.6 mm x 250 mm, 5 microm). Mobile phase: methanol-water (including 0.05% H3PO4), and gradient elution. Flow rate: 1 mL x min(-1). Wavelength: 254 nm. Column temperture: 30 degrees C.

RESULTThe calibration curves of gentiopicroside, mangiferin and swertrianolin were in good linearity over the range of 31.3-281.7, 0.31-2.78, 0.55-4.91 microg, (r = 0.9996, 0.9993, 0.9995). The average recoveries were 103.36%, 101.42% and 97.39%, with RSD less then 3% (n = 5).

CONCLUSIONIt is a simple and sensitive meathod in controlling the quality of S. punicea.

Calibration ; Chromatography, High Pressure Liquid ; methods ; Drugs, Chinese Herbal ; chemistry ; standards ; Glucosides ; analysis ; Glycosides ; analysis ; Iridoid Glucosides ; Iridoids ; analysis ; Plants, Medicinal ; chemistry ; Quality Control ; Reproducibility of Results ; Swertia ; chemistry ; Xanthones ; analysis