Objective Galactosialidosis(GS) is an autosomal recessive lysosomal storage disease caused by a combined deficiency of lysosomal ?-galactosidase and neuraminidase as a result of a primary defect in the protective protein/cathepsin A(PPCA).Mouse model of GS has been generated by targeted deletion of PPCA gene and closely resembled the phenotypes in human conditions.However,it remains to be determined whether hearing loss observed in human also occurs in the mouse model.In this study,we observed their alterations of the auditory function and morphology of the ear,and explored pathophysiological mechanisms of hearing impairment.Methods PPCA homozygous(PPCA-/-) mice at 1 and 2 months of age,and their wildtype littermates(PPCA+/+) were examined for auditory thresholds through auditory brainstem responses(ABR) to click,tone pips 8,16,and 32 kHz stimuli.Morphological analyses in ears were performed by series temporal bone section and light microscopy.Results PPCA-/-mice at 1 month of age showed a normal threshold and the morphology of ears.Up to 2months of age,their thresholds were elevated 40～45 dB SPL above those of PPCA+/+ mice.There were distinct pathological changes of middle and inner ear in PPCA-/-mice of 2 months old.The severe otitis media and the vacuolation associated with lysosomal storage were observed within ossicles and cochlear bone cells,stria vascularis cells,spiral ganglion neurons,spiral limbus,Reissner's membrane cells,and the mesothelial cells of the perilymphatic scala and basilar membrane,but not within the organ of Corti.Vestibular organ did not show vacuolation.Conclusion The deficiency of lysosomal protective protein/cathepsin A may result in hearing loss and morphological alterations of ear.The otitis media and ossicle changes,and the defects in lysosomal storage of neurons,stria vascularis,spiral limbus,Reissner's membrane and basilar membrane cells may contribute to the conductive and sensorineural hearing loss respectively.

Objective To prepare an influenza A(H1N1) split-virus vaccine and observe its safe-ty and effectiveness. Methods According to the process for preparing seasonal flu split-virus vaccine two batches of vaccine were prepared with the flu A (H1N1) vaccine virus strain recommended by WHO. The pilot products were tested against the requirements of flu split-virus vaccine. Results The quality of the pi-lot vaccine has been tested by National Control Laboratory and conformed to the requirements. Nine hundred and sixty volunteers received one dose of vaccine containing either 15 μg or 30 μg of hemagglutinin. The re-suits indicated the both seroconversion rate and protection rate were higher the 70%. The GMT of HIAb of the volunteers who received 1 dose of 15 μg increased significantly by 15, 39, 37 and 25 times compared to those before vaccination in the age groups of 3-11, 12-17, 18-59 and ≥60, respectively. And 26, 72, 68 and 36 times rise were found in the postvaccinated volunteers of 30 μg group. The total adverse reaction rates of 15 μg and 30 μg dose group were 29.38% and 43.75%, respectively. The grade 2 adverse reaction rates of 15 μg and 30 μg dose group were 6.25% and 15.42%, and the grade 3 adverse reaction rates of 15 μg and 30 μg dose group were 0.83% and 1.46%, respectively. No serious adverse reactions were found. Conclusion The influenza A (H1N1) split-virus vaccine prepared according to the requirements of season-al flu vaccine is safe and effective.

OBJECTIVE: To investigate the expressions of LL-37 and IL-8 in chronic sinusitis with nasal polyps. METHOD: Semi-quantitative reverse transcription polymerase chain reaction (RT-PCR) and immunohistochemical staining were used to detect the expressions of LL-37 and IL-8 in nasal polyp tissues of 31 patients with chronic sinusitis and inferior turbinate tissues of 11 patients with chronic rhinitis. RESULT: LL-37 and IL-8 mRNA were all positively expressed in all nasal polyps and inferior turbinate tissues. There were significant increases of LL-37 and IL-8 mRNA expressions in nasal polyps compared with the inferior turbinate tissues (P < 0.01). There were also significant increases of positive expression rates of LL-37 and IL-8 protein in nasal polyps, compared with the inferior turbinate tissues (P < 0.01). There was a positive relationship between the mRNA and protein expressions of LL37 and IL-8 (P < 0.01). CONCLUSION The expressions of LL-37 and IL-8 in nasal polyps suggest that they may play a role in the pathogenesis of chronic sinusitis. Besides its innate immune, LL-37 could enhance human body's anti-infected function by increasing acquired immune.
Adult ; Antimicrobial Cationic Peptides ; Cathelicidins ; metabolism ; Chronic Disease ; Female ; Humans ; Interleukin-8 ; metabolism ; Male ; Middle Aged ; Nasal Mucosa ; metabolism ; Nasal Polyps ; metabolism ; Sinusitis ; metabolism ; Young Adult

OBJECTIVE: To explore the causes and the management of serious eye complications occurring in the endoscopic sinus surgery. METHOD: Three patients of chronic sinusitis and nasal polyps suffered with blindness in endoscopic sinus surgery and in nasal packing with iodoform and petrolatum gauze were treated. RESULT: Orbital wall and structure were injured in 2 cases during endoscopic sinus surgery, among which, 1 case blinded with deformation of the eyeball during operation underwent optic nerve exploration and orbital muscle reparation immediately. One case developed periocular swelling, eyelid hematoma, conjunctiva edema and blinded 2 days later, and was treated with hematoma clearance and optic nerve decompression. Another 1 case blinded immediately after ethmoid packing, and vision recovered after nasal pack removed. Antibiotics, corticosteroid and nerve growth factor were administered for 4 weeks in all patients. After 6-month follow-up, 1 case was blinded with eyeball atrophy, 1 case was only photonasty, another regained normal vision. CONCLUSION The causes of blindness in endoscopic sinus surgery are directly related to orbital structure trauma and orbital hematoma. The optic nerve during operation should be protected carefully, if ethmoid sinus over development is demonstrated by CT scan. The application of gauze should be avoided when the medial orbital wall is injured. Decompression of optic nerve should be performed as early as possible, if vision damaged.
Adult ; Blindness ; etiology ; prevention & control ; Endoscopy ; adverse effects ; Female ; Humans ; Male ; Middle Aged ; Nose ; surgery ; Otorhinolaryngologic Surgical Procedures ; adverse effects ; Postoperative Complications ; prevention & control

OBJECTIVE: To enhance the safety of nasal endoscopic surgery and decrease its complications of eyes. METHOD: Three patients of chronic rhinosinusitis and nasal polyposis with lipogranulomas of the eyelids after nasal endoscopic surgery and nasal packing of petrolatum gauze were reported and analyzed, and their treatment results were presented during the last 2 years. RESULT: The medial orbital wall injury occurred in all three patients during endoscopic sinus surgery. The patients developed an ipsilateral periocular swelling, eyelid hematoma and palpebral conjunctival edema during 2 to 3 hours after surgery. Nasal packs petrolatum gauze were removed 10-24 hours after surgery. The patients were discharged from hospital when periorbital swelling and eyelid ecchymoma disappeared, and nasal cavity obstruction was improved 6 to 8 days after surgery. The swelling and nodular mass of ipsilateral eyelids (one in left upper eyelid and two in right lower eyelid) were found 12-15 days after surgery, and their eye movement and eyesight were normal. Antibiotic and corticosteroid were administered for 3 4 weeks with only improvement in eyelid swelling. These masses of eyelids were completely excised through palpebral margin 1-6 months after surgery. The histopathological examination of the surgical specimens showed lipogranuloma. No recurrence and symptom of the eyes had been observed during 4-18 months follow up. CONCLUSION The lipogranuloma of the eyelid is a rare and late complication after nasal endoscopic surgery and nasal packing with vaspetrolatum gauze. The medial orbital wall injury and bleeding during surgery, and vaseline of nasal packing permeated into the eyelid are the direct causes of this complication. The application of petrolatum gauze should be avoided when the medial orbital wall trauma is identified. The complete excision of granulomas is a best effective therapy.
Adult ; Endoscopy ; adverse effects ; Eyelid Diseases ; diagnosis ; etiology ; therapy ; Female ; Granuloma ; diagnosis ; etiology ; therapy ; Humans ; Male ; Middle Aged ; Postoperative Complications ; diagnosis ; therapy

Objective To know the anxiety status of preschool children in rural areas and to explore its influential factors. Methods Subjects of 1363 rural preschool children aged 3-6 years were selected from Anhui Province( Changfeng and Feixi county of Hefei city; Qianshan county of Anqing city; Funan county of Fuyang city) . The primary caregivers of preschool children were investigated by Chinese version of Spence Preschool Anxiety Scale (SPAS),Devereux Early Childhood Assessment for Preschoolers (DECA-P2) ,Neglect Norms for Children Aged 3 to 6 Years in Rural Areas of China,Adaptation Partnership Growth Affection Resolve (APGAR),Self-rating anxiety scale (SAS) and Self-rating depression (SDS). Result-s Among the 1363 preschool children,the positive rate of anxiety was 14. 7％. Physical injury fears score was highest (1. 24±0. 84),followed by social phobia anxiety (0. 86±0. 75) and separation anxiety (0. 85± 0. 74) . Except for obsessive compulsive disorder,the other four types of anxiety and total anxiety scores were negatively correlated with the total protective factors of mental resilience (r=-0. 054- -0. 070,P<0. 05). Besides,all anxiety scores were positively correlated with behavioral problems(r=0. 121-0. 237,P<0. 05)and neglect degree (r=0. 157-0. 269,P<0. 05). The results of multiple linear regression analysis of different anxiety types showed that gender,family type,family income status,parental rearing pattern was consistent or inconsistent,children' s neglect degree,behavior problem and anxiety/depression status of primary caregivers were the main factors affecting the anxiety level of preschool children in rural areas. Conclusions In order to reduce or avoid anxiety of preschool children,it is necessary to establish a good family environment,im-prove parents' upbringing level,and take effective education intervention.

Objective: To investigate the etiologic and epidemiologic features of an infectious diarrhea outbreak in a boarding school in Fuyang city, Anhui province. Methods: Traceability hypothesis of this study was tested according to the epidemiological characteristics of the cases. Feces, anal swabs, water samples and food residues related to the patients and chefs were collected for pathogen isolation and detection. Biochemical identification, virulence gene detection, drug susceptibility test, PFGE and multilocus sequence typing were performed. Results: The incidence rate (3.41%) of different dormitory buildings within the water supply area by shallow wells was higher than that (0.98%) of the deep wells, with statistical significance (χ²=17.215, P<0.001). Sixteen strains belonged to the Shigella Sonneri family were isolated from the patient’s samples, and all carrying the ipaH gene. Seven strains belonged to sen and ial genes. Set1 gene that did not appear in all the 16 strains were highly resistant to ampicillin, tetracycline, compound xinnomine, cefazoline, cefotaxime, gentamicin, naphthidinic acid and streptomycin, including 9 strains to doxycycline. The pulse field pattern of the 16 strains of Shigella sonneri appeared the same, with the ST type as ST152. Conclusion When combined data from the etiological and epidemiological investigation, it was confirmed that Shigella sonneri was the pathogen of this outbreak, and water from the shallow wells might be responsible for the source of infection.

Dendritic cell-based cancer vaccines (DC vaccines) have been proved efficient and safe in immunotherapy of various cancers, including melanoma, ovarian and prostate cancer. However, the clinical responses were not always satisfied. Here we proposed a novel strategy to prepare DC vaccines. In the present study, a fusion protein SNU containing a secretin-penetratin (SecPen) peptide, NY-ESO-1 and ubiquitin was designed and expressed. To establish the DC vaccine (DC-SNU), the mouse bone marrow-derived DCs (BMDCs) were isolated, pulsed with SNU and maturated with cytokine cocktail. Then peripheral blood mononuclear cells (PBMCs) from C57BL/6 mice inoculated intraperitoneally with DC-SNU were separated and cocultured with MC38/MC38