Vogt-Koyanagi-Halada syndrome is an autoimmune disease attacking melanocytes, which involves eye, ear, meninge and skin. Patient offen has uveitis and extraocular symptoms and signs and the misdiagnosis of missed diagnosis of VKHS is very common. Patients have good prognosis after hormone therapy.
Adult ; Diagnostic Errors ; Hearing Loss, Sudden ; diagnosis ; Humans ; Male ; Uveomeningoencephalitic Syndrome ; diagnosis

Hereditary angioneurotic laryngeal edema (HALE) is an autosomal dominant hereditary disease in which there is a decrease or defect in the C1 inhibitor (C1-INH). The pathophysiology of HALE is characterized by recurrent spontaneous episodes of transient edema of the laryngeal mucose and submucosal tissue with remission at irregular. Patients may die because of a life-threatening acute upper airway obstruction caused by laryngeal edema. HALE was diagnosed on the clinical symptoms, family history, and markedly decreased serum C1-INH activity and C1-INH protein.
Angioedemas, Hereditary ; diagnosis ; Complement C1 Inactivator Proteins ; analysis ; metabolism ; Complement C1 Inhibitor Protein ; Humans ; Laryngeal Edema ; diagnosis ; Recurrence

Objective To observe the rates of local complications (bleeding/swelling) after trans-radial coronary intervention (TRI),to investigate possible impact factors,in order to provide guidance for the prevention of these adverse events.Methods A total of 198 cases who performed TRI at the department of interventional cardiology of the First Affiliated Hospital of Sun Yat-sen University from August to December,2011 were included in the present study.Data with regards to local complications were collected peri-operatively,by means of direct inquiry,point-of-care observation and measurements,and laboratory testing.Patients who developed complications and patients who did not were compared in terms of compressor position,loosening protocol and so on.Variables with statistical significance in univariate analysis were further included in Cox regression.SPSS 18.0 software was applied for all statistical analysis.Results The relative risks (RR) of the following two variables,namely whether or not the midpoint of compressor was on the midline of forearm,and the vertical distance from the horizontal plate of the compressor to the puncture site,were 2.602 and 1.403,respectively.Conclusions The midpoint of compressor on the midline of forearm,and the vertical distance from the horizontal plate of the compressor to the puncture site were major risk factors of local complications after TRI.

Objective To summarize the clinical features of histiocytic necrotizing lymphadenitis (HNL) and the lesion of central neural system in children.Methods Data of the 4 cases in Beijing Children's Hospital from January 2009 to December 2014 were reviewed and analyzed,as well as other files of 12 patients from PubMed,CNKI,etc.Results All 4 patients mentioned above started from fever and lymphadenopathy,3 cases had headache,2 cases of them experienced altered mental status,and 1 case had cclampsia.The findings of cerebrospinal fluid (CSF) test was similar to viral encephalitis;2 cases had normal magnetic resonance imaging (MRI) result;1 case with MRI test indicated brain white matter diseases with extensive intracerebral hemorrhage,and 1 case had small range of encephalomalacia in left cerebral hemisphere and cerebral dura mater.Through CSF examination,2 cases were positive to Epstein-Barr viral capsid antigen (EBV-VCA)-IgM,while the 2 other cases had no positive trace of EBV-VCA-IgM.Lymph node biopsy was in accordance with HNL typical changes,1 case was positive for Epstein-Barr virus encoded RNA (EBER).Twelve cases in previous literatures were diagnosed as HNL by lymph node biopsy,and their clinical features of central nervous system were headache and altered mental status.There was no change in pathogen.It is also found that the cases in which CSF was performed had shown being negative.CT/MRI image descriptions involved in temporosphenoid lobe,hippocampus,caudate lobe,leptomeninges,etc.Conclusions HNL can be complicated with lesions of the central nervous system,characterized by aseptic meningitis,encephalitis,etc.The prognosis of most cases is good,a few can have neurological sequelae.HNL may relapse,so long-term follow-up is ne-cessary.Epstein-Barr virus in HNL and lesion of central nervous system mechanism still needs further research.

Objective To establish the expression profiles of microRNA (miRNA) in SMMC-7721 and CCC-HEL-1 cell lines in order to provide new clues to study the mechanism of miRNA function in hepatocellular carcinoma (HCC) and its treatment. Methods SMMC-7721 and CCC-HEL-1 cell lines were cultured in vitro. Total RNAs were extracted by TRIzol, followed by RNA quantification and quality control. The RNAs were used to detect the expression of miRNA in these two cell lines by miRNA array. The miRNA of interest was then verified by real-time PCR. Results In total,238 differentially expressed miRNAs were found, including 154 overexpressed and 84 underexpressed miRNAs. 64 miRNAs were upregulated more than 4 times and26 miRNAs were upregulated more than 10 times. 22 miRNAs were downregulated more than 4 times 10 miRNAs weredownregulated more than 5times, and 1 miRNA was downregulated more than 10 times. Real-time PCR validation suggested that has-miR-205 and has-let-7f in liver cancer increased by 2. 7 and 2. 3 times, respectively.Conclusion There are differences in the expression of miRNA between the SMMC-7721 and CCC-HEL-1 cell lines and the profiles of miRNA expression were established for both cell lines. It was found that has-miR-205 and has-let-7f were upregulated in liver cancer.

Objective: To summarize the clinical characteristics, virological and histopathological features, clinical outcome of Epstein-Barr virus-positive lymphoproliferative disease (EBV⁺LPD) in children. Methods: The clinical and follow-up data of 13 children histopathologically diagnosed as EBV⁺LPD in the Department of Infectious Disease of Beijing Children's Hospital between January 2011 and December 2016 were summarized. Results: Of the 13 patients, 5 were males and 8 females. The median age of disease onset was 6.0 years (range 1.3 to 15.0 years). The median duration between disease onset and diagnosis was 3 months (range 1 to 24 months). All the 13 patients had fever, 9 cases had hepatosplenomegaly and lymphoadenopathy, 4 cases had only lymphoadenopathy, 7 cases had reduced peripheral blood cells, 7 cases had lung involvement, 3 cases had central nervous system involvement, 3 cases had cardiac involvement, 3 cases had intestinal involvement, 2 cases had skin involvement and 1 case had abdominal mass. All the 13 patients underwent whole blood EBV-DNA PCR examination and the copies ranged from 1×10⁸/L to 1×10¹¹/L. Pathology of lymph node confirmed 6 cases, skin pathology confirmed 2 cases, lung pathology, ileum mucosa pathology, liver pathology, abdominal mass pathology and bone marrow pathology confirmed 1 case each. Among 13 patients, 9 cases presented with EBV-positive T cell lymphoproliferative disease(EBV⁺ T-LPD), 2 cases with hydroa vacciniforme (HV) and 2 cases with EBV-positive diffuse large B-cell lymphoma (EBV⁺ DLBCL) . All the patients were followed up for 2 days to 65 months after discharge. Among 9 cases of EBV⁺T-LPD, 1 case died in a short time, 1 case died after evolved to T-cell lymphoma, 2 cases recovered after hematopoietic stem cell transplantation, 1 case recovered after the chemotherapy of hemophagocytic lymphohistiocytosis(HLH) 2004 protocol and 4 cases were stable now. Of 2 cases of HV patients, 1 case died after evolved to HV like lymphoma and the other still have symptoms. Among 2 cases of EBV⁺ DLBCL, 1 case died shortly after discharge and the other was still stable after chemotherapy. Conclusions Chronic recurrent fever, lymphadenopathy and hepatosplenomegaly are the most common clinical manifestations in children with EBV⁺LPD. Involvement of lung, central nervous system, intestinal tract, skin and other organs are also involved frequently. For children with chronic fever of unknown cause and accompanied by lymphadenopathy and (or) hepatosplenomegaly, EBV ⁺ LPD should be considered highly when the whole blood EBV-DNA load continues to increase significantly, early biopsy of the proliferative lesion should be performed to make a definite diagnosis. The prognosis of EBV ⁺ LPD is poor, and some evolve to lymphoma, hematopoietic stem cell transplantation is an effective way to treat this disease.

Objective To investigate the relationship between serum miR-505,miR-15 and endoscopic activity of ulcerative colitis(UC).Methods 98 patients with UC from May 2020 to May 2022 were selected as the observation group,and 80 patients with irritable bowel syndrome(IBS)were selected as the control group during the same period.The modified Mayo score was used to evaluate the endoscopic activity in patients with UC,and the relationship among the changes of serum miR-505 and miR-15 levels and the endoscopic activity in patients with UC was analyzed.Results The level of serum miR-505 in observation group was lower than that in control group,while the level of miR-15 in observation group was higher than that in control group(P<0.05).The level of serum miR-505 in patients with active UC was lower than that in patients with the healing stage,while the level of serum miR-15 in patients with active UC was higher than that in patients with healing stage(P<0.05).The serum miR-505 level of E3 type was lower than that of E2 type and E1 type,and the serum miR-15 level of E3 type was higher than that of E2 type and E1 type(P<0.05).The Mayo score of type E3 was higher than that of type E2 and E1(P<0.05).Pearson correlation analysis showed that serum miR-505 was negatively correlated with Mayo score(r =-0.51,P<0.05),while serum miR-15 was positively correlated with Mayo score(r = 0.54,P<0.05).Receiver operator characteristic curve(ROC curve)results showed that the cut-off value of miR-505 was 0.93,and the area under the curve(AUC)for evaluating the endoscopic activity of patients with UC was 0.67(95%CI:0.659～0.785),and the sensitivity and specificity were 73.24%and 68.55%,respectively.The cut-off value of miR-15 was 0.85,and the AUC for evaluating the endoscopic activity of patients with UC was 0.69(95%CI:0.672～0.814),and the sensitivity and specificity were 76.38%and 69.72%,respectively.The AUC for the joint evaluation of UC by both was 0.82(95%CI:0.809～0.912),and the sensitivity and specificity were 84.56%and 68.49%,respectively.Conclusion Serum miR-505 levels decrease and serum miR-15 levels increase in UC patients than IBS,and the level is closely related to patients'pathological conditions and endoscopic activity.Combined detection of serum miR-505 and miR-15 has a good value in evaluating endoscopic activity in UC patients.

Objective To compare different nursing methods impacting on the wound healing effects of circumcision for patients with Chinese Shang ring circumcision. Methods A total of 144 male patients with Chinese Shang ring circumcision were randomly divided into A and B group by coin tossing. The patients of A group received circumcised dressing to wrap wound with dressing change 3 times/week;the patients of B group had no dressing for wound to exposure wound and keep wound dry. Followed interviewed 2 months after operation, we compared the incision edema, the infection, rate of incision rupture, postoperative pain, wound healing time, satisfaction of incision appearance and so other indexes between two groups. Results The patients in the group B suffered less pain when erected in 2 weeks after operation (P<0. 05). The length of healing time in B group was (25.9 ±4.7) d, shorter than (27.8 ±5.0) d in the group A (P<0.05). The postoperative incision edema, incision rupture, infection and satisfaction of incision appearance were no statistical significance between two groups (P>0. 05). Conclusions To exposure the incision and keep it dry after Chinese Shang ring circumcision can minimize the level of pain and length of wound healing, and is worthy to promote.

Objective:To analyze the characteristics and prognosis of hearing loss in children with bacterial meningitis.Methods:This was a single-center retrospective cohort study. Patients diagnosed with bacterial meningitis who were hospitalized in Beijing Children′s Hospital between 2010 and 2016 and older than 28 days and younger than 18 years at symptom onset were included in this study ( n=573). All clinical information including hearing assessment results during hospitalization were reviewed. All patients with hearing loss were followed up to repeat their hearing test and assess their hearing condition with parents′ evaluation of aural and (or) oral performance of children (PEACH). Patients were grouped according to their hearing assessment results, and Logistic regression analysis was used to analyze the risk factors for hearing loss in patients with bacterial meningitis. Results:Five hundred and seventy-three patients were enrolled in this study, including 347 males and 226 females. The onset age ranged from 29 days to 15.8 years. Two hundred and forty-six patients had identified causative pathogens, among whom 92 cases (37.4%) were pneumococcal meningitis cases. Hearing loss was found in 160 cases (27.9%) during hospitalization, involving 240 ears. Permanent hearing loss was found in 20 cases (16.9%), involving 32 ears. In the patients with permanent hearing loss, 87.5% (28/32) of ears were identified as severe or profound hearing loss during hospitalization. Logistic regression analysis showed that dystonia, the protein concentration level in cerebrospinal fluid>1 g/L, glucose concentration level lower than 1 mmol/L and subdural effusion were independent risk factors for hearing loss ( OR=2.426 (1.450-4.059), 1.865 (1.186-2.932), 1.544 (1.002-2.381) and 1.904 (1.291-2.809)). Conclusions:Hearing loss is a common sequela of bacterial meningitis in children. Most patients have transient hearing loss, but patients with severe or profound hearing impairment have a higher risk of developing permanent hearing loss.

OBJECTIVE: To investigate the correlation of polysomnography parameters and CT measurements in upper airway of mild and severe obstructive sleep apnea hypopnea syndrome (OSAHS) patients before and after uvulopalatopharyngoplasty (UPPP). METHOD: Having PSG detection and spiral computed tomograph scan for 30 mild and severe OSAHS patients both before and after UPPP operation, compare the morphology change of upper airway on CT measurements, use pearson correlation analysis to analysis the correlation between the minimum cross-sectional area, left and right diameter, anteroposterior diameter in upper airway and apnea hypopnea index (AHI). RESULT: The difference of the minimum cross-sectional area, left and right diameter, anteroposterior diameter in upper airway before and after UPPP operation was significant. The minimum cross-sectional area, left and right diameter was negatively correlated with AHI; Left and right diameter was not correlated with AHI. CONCLUSION The minimum cross-sectional area, left and right diameter, anteroposterior diameter after operation is bigger than before operation. The minimum cross-sectional area, left and right diameter is negatively correlated with AHI.
Adult ; Female ; Humans ; Intraoperative Period ; Male ; Middle Aged ; Palate ; surgery ; Pharynx ; surgery ; Polysomnography ; Sleep Apnea, Obstructive ; diagnostic imaging ; surgery ; Tomography, X-Ray Computed ; Uvula ; surgery