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 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 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: The connectivity alterations in the putamen were found in revealing the neural correlates of attention-deficit/hyperactivity disorder (ADHD), but whether the effective connectivity of the putamen is atypical in ADHD remains unclear. Investigating this abnormality contributes to describing the neural circuit of ADHD at the level of macrostructural organization. Methods: Data were acquired from thirty-two boys with ADHD and fifty-two matched typically developing controls (TDC) from Peking University (Peking) dataset deposited at the Neuroimaging Informatics Tools and Resources Clearinghouse (NITRC) platform. We examined the effective connectivity of the putamen using Granger causality analysis (GCA) and then determined whether these connections could differentiate ADHD from TDC. Results: Compared with TDC, the ADHD group showed decreased effective connectivity from the left ventral rostral putamen (VRP) to left calcarine (CAL), right medial part of the superior frontal gyrus, left orbital part of superior frontal gyrus and left middle occipital gyrus (MOG). Increased effective connectivity from the left inferior occipital gyrus and right lingual gyrus to left VRP was also found in ADHD. The result of the classification accuracy showed that 72.3% of participants were correctly classified using support vector machine. Moreover, GCA values from the left VRP to left CAL and left MOG were significantly correlated with hyper/impulsive scores of patients with ADHD. Conclusion The findings may help extend our understanding of the ADHD-related neural loops.

Objective: The connectivity alterations in the putamen were found in revealing the neural correlates of attention-deficit/hyperactivity disorder (ADHD), but whether the effective connectivity of the putamen is atypical in ADHD remains unclear. Investigating this abnormality contributes to describing the neural circuit of ADHD at the level of macrostructural organization. Methods: Data were acquired from thirty-two boys with ADHD and fifty-two matched typically developing controls (TDC) from Peking University (Peking) dataset deposited at the Neuroimaging Informatics Tools and Resources Clearinghouse (NITRC) platform. We examined the effective connectivity of the putamen using Granger causality analysis (GCA) and then determined whether these connections could differentiate ADHD from TDC. Results: Compared with TDC, the ADHD group showed decreased effective connectivity from the left ventral rostral putamen (VRP) to left calcarine (CAL), right medial part of the superior frontal gyrus, left orbital part of superior frontal gyrus and left middle occipital gyrus (MOG). Increased effective connectivity from the left inferior occipital gyrus and right lingual gyrus to left VRP was also found in ADHD. The result of the classification accuracy showed that 72.3% of participants were correctly classified using support vector machine. Moreover, GCA values from the left VRP to left CAL and left MOG were significantly correlated with hyper/impulsive scores of patients with ADHD. Conclusion The findings may help extend our understanding of the ADHD-related neural loops.

Objective:To explore the safety and efficacy of renal arterial hypothermia perfusion in robot-assisted laparoscopic partial nephrectomy.Methods:The data of 11 patients with complex renal tumors admitted to our hospital from March 2020 to December 2021 were retrospectively analyzed. There were 7 males and 4 females. The patients’age was (64.64±13.56) years old.The median R. E.N.A.L. score was 8 (7, 9) points. Preoperative glomerular filtration rate (GFR) was (64.40±25.52) ml/min. All patients had a renal artery cold solution perfusion robot-assisted laparoscopic partial nephrectomy. 4℃ sodium lactate Ringer's solution was injected into the affected kidney by the catheter, which could provide the kidney a hypothermic state during the operation and protected the renal function during the long period of warm ischemia. The intraoperative data and postoperative complications were recorded. The data of postoperative renal function, routine urine test, urinary CT, preoperative and postoperative glomerular filtration rate (GFR) were analyzed.Results:The renal artery blocking time was (34.09±2.84) min during the surgery. The patients’ body temperature was (36.10±0.44) ℃. The surgical duration was (126.73±47.08) min. The intraoperative bleeding was (81.82±53.07) ml. There were no complications, such as urinary leakage, low body temperature, fever, etc. There was no significant difference between the 3-months postoperative GFR (59.06±25.67) ml/min and preoperative GFR ( P=0.636). Conclusions:For patients with complex renal tumors, renal arterial cold solution perfusion in robot-assisted laparoscopic partial nephrectomy can help obtain longer operative duration during hot ischemia, preserve renal function, and it provide a safe and feasible surgical method for patients.

PURPOSE: The aim of this research was to assess the value of the transitional zone index (TZI) and intravesical prostatic protrusion (IPP) from transrectal ultrasonography in evaluating the severity and progression of disease by analyzing the relationship between the 2 parameters and symptoms, clinical history, and urodynamics in benign prostatic hyperplasia (BPH) patients undergoing different treatment. MATERIALS AND METHODS: A total of 203 patients receiving medication and 162 patients who underwent transurethral resection of the prostate because of BPH were enrolled in this retrospective analysis. The clinical history and subjective and objective examination results of all patients were recorded and compared after being classified by TZI and IPP level. Linear regression was used to find correlations between IPP, TZI, and urodynamics. RESULTS: The 2 parameters were found to differ significantly between patients receiving medication and patients undergoing surgical therapy (p<0.05). PSA, maximum flow rate (Qmax), detrusor pressure at Qmax (PdetQmax), and the bladder outlet obstruction index (BOOI) differed according to various TZI levels (p<0.05). In addition, the voiding symptom score, Qmax, and BOOI of subgroups with various IPP levels were also significantly different (p<0.05). Both TZI and IPP had significant effects on Qmax, BOOI, and PdetQmax (p<0.05) and the incidence of acute urinary retention (p=0.000). CONCLUSIONS: The results demonstrated that both TZI and IPP had favorable value for assessing severity and progression in patients with BPH. Further studies are needed to confirm whether the two parameters have predictive value in the efficacy of BPH treatment and could be considered as factors in the selection of therapy.
Humans ; Incidence ; Indoles ; Linear Models ; Prostate ; Prostatic Hyperplasia ; Retrospective Studies ; Urinary Bladder Neck Obstruction ; Urinary Retention ; Urodynamics

Streptococcus suis is regarded as one of the major pathogens of pigs, and Streptococcus suis type 2 (SS2) is considered a zoonotic bacterium based on its ability to cause meningitis and streptococcal toxic shock-like syndrome in humans. Many bacterial species contain genes encoding serine/threonine protein phosphatases (STPs) responsible for dephosphorylation of their substrates in a single reaction step. This study investigated the role of stp1 in the pathogenesis of SS2. An isogenic stp1 mutant (Δstp1) was constructed from SS2 strain ZJ081101. The Δstp1 mutant exhibited a significant increase in adhesion to HEp-2 and bEnd.3 cells as well as increased survival in RAW264.7 cells, as compared to the parent strain. Increased survival in macrophage cells might be related to resistance to reactive oxygen species since the Δstp1 mutant was more resistant than its parent strain to paraquat-induced oxidative stress. However, compared to parent strain virulence, deletion of stp1 significantly attenuated virulence of SS2 in mice, as shown by the nearly double lethal dose 50 value and the lower bacterial load in organs and blood in the murine model. We conclude that Stp1 has an essential role in SS2 virulence.
Animals ; Bacterial Load ; Humans ; Lethal Dose 50 ; Macrophages ; Meningitis ; Mice ; Oxidative Stress ; Parents ; Phosphoprotein Phosphatases ; Reactive Oxygen Species ; Streptococcus suis* ; Streptococcus* ; Swine ; Virulence*

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

In this study, we attempted to establish a culture system for in vitro spermatogenesis from spermatogonial stem cells (SSCs) of Bama mini-pig. Dissociated testicular cells from 1-month-old pigs were co-cultured to mimic in vivo spermatogenesis. The testicular cells were seeded in minimum essential medium alpha (α-MEM) supplemented with Knockout serum replacement (KSR). Three-dimensional colonies formed after 10 days of culture. The colonies showed positive staining for SSC-associated markers such as UCHL1, PLZF, THY1, OCT4, Dolichos biflorus agglutinin, and alkaline phosphatase. Induction of SSCs was performed in α-MEM + KSR supplemented with retinoic acid, bone morphogenetic protein 4, activin A, follicle-stimulating hormone, or testosterone. The results showed that STRA8, DMC1, PRM1, and TNP1 were upregulated significantly in the colonies after induction compared to that in testis from 1-month-old pigs, while expression levels of those genes were significantly low compared to those in 2-month-old testis. However, upregulation of ACROSIN was not significant. Replacement of α-MEM and KSR with Iscove's modified Dulbecco's medium and fetal bovine serum did not upregulate expression of these genes significantly. These results indicate that SSCs of Bama mini-pig could undergo differentiation and develop to a post-meiotic stage in α-MEM supplemented with KSR and induction factors.
Acrosin ; Activins ; Alkaline Phosphatase ; Bone Morphogenetic Protein 4 ; Dolichos ; Follicle Stimulating Hormone ; Humans ; In Vitro Techniques ; Infant ; Infant, Newborn ; Spermatogenesis ; Stem Cells ; Swine ; Testis ; Testosterone ; Tretinoin ; Up-Regulation