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 evaluate the effect of laparoscopic-assisted anorectoplasty in the treatment of children′s congenital anal atresia.Methods:In this study , 49 children undergoing laparoscopic-assisted anorectoplasty between Mar 2009 and Mar 2015 were compared in terms of outcomes with 42 children under going posterior sagittal anorectoplasty during this period.The post-operative complications, bowel functions and courses were evaluated.Results:The ratio of primary healing in laparoscopic-assisted anorectoplasty was higher than posterior sagittal anorectoplasty(22 % vs. 10%, χ 2=4.306, P=0.038), the age of sequential operation in the former was lower than control group [(9.9±6.5) d vs. (13.4±5.1) d, t=2.823, P=0.003]. The perioperative complications were lower than that in the control group(20% vs. 50%, χ 2=8.817, P=0.003), the bowel function was better than control group(χ 2=7.419, P=0.025). Conclusions:Perioperative complications in laparoscopic-assisted anorectoplasty is lower than posterior sagittal anorectoplasty, with better bowel function and higher primary healing rate.

Objective:To explore the risk factors for recurrence of intussusception in children after successful ultrasound-guided saline enema reduction.Methods:The clinical and follow up data of 355 hospitalized children with intussusception at the First Affiliated Hospital of Zhengzhou University from Feb 2018 to Feb 2023 were reviewed.Patients were divided into two groups by recurrence develped and the differences were compared, Data with significant differences were incorporated into multi-factor logistic analysis.Results:The overall recurrence rate was 15.8% (56/355). By univariate variable analysis model, there were statistically significant differences between the two groups in age, previous intussusception history, vomiting, maximum diameter of concentric circles shown by ultrasound, and concurrent bowel organic diseases (lead points) (all P<0.05). In multivariate Logistic regression model, age, previous intussusception history, maximum diameter of concentric circles, and lead points were independent risk factors for recurrent intussusception after saline enema.The optimal cut-off values for age and maximum diameter of concentric circles were 2 years and 33.5 mm, respectively, according to ROC curve analysis. Conclusion:Age older than 2 years, previous intussusception history, maximum diameter of concentric circles longer than 33.5 mm, and lead points are independent risk factors for recurrence after saline enema.

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 explore the long?term efficacy of laparoscopic?assisted anorectoplasty and conventional anorectoplasty in the treatment of children with high and middle anal atresia. Methods A retrospective cohort study was used. Inclusion criteria: (1) children with high and middle anal atresia; (2) complicated with rectourethral or rectovesical fistula; (3) complete follow?up data. Exclusion criteria: (1) complicated with 21?trisomy; (2) cerebral palsy and other mentaldisabilities; (3) Currarino syndrome; (4) FG syndrome. Clinical data of 88 patients with middle and high anal atresia, who complicated with rectourethral fistula or rectovesical fistula, and underwent anoplasty at Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from January 2009 to June 2014 were enrolled in the study and analyzed. There were 24 cases with middle atresia and 64 cases with high atresia. All the cases were divided into 2 groups based on the operative method: laparoscopic group (laparoscopic?assisted anorectoplasty, 49 cases), pena group (posterior sagittal anorectoplasty, 39 cases). The demographic features of two groups were comparable. There were no statistically significant differences in gender, age, body mass, classification of anomaly types and sacral ratio (all P>0.05). Student t test and Chi square tests were used to compare the surgical conditions (operative time, postoperative hospital stay and complications), anal function (Kelly score), constipation (Krickenbeck constipation score) and anorectal pressure. Results Children of both groups all completed operation ssuccessfully. There were no statistically significant differences between laparoscopic group and pena group in the operative time [(120±31) minutes vs. (112±23) minutes, t=1.343, P=0.091] and postoperative hospital stay [(7.1±2.3) days vs. (10.7±3.3) days, t=6.021, P=1.000]. Complications were more common in the pena group [16.3% (8/49) vs. 35.9% (14/39), χ2=4.436, P=0.035]. The main complications in laparoscopic group were anal prolapse (8.2%, 4/49) and anal stenosis (6.2%, 3/49), while in pena group were anal stenosis (12.8%, 5/39) and perioperative perianal skin erosion (10.3%, 4/39). As for the anal function, the degree of feces, defecation control and sphincter contractility, the single scoring differences of Kelly scoring system were not statistically significant between the two groups, but the proportion of good function in the laparoscopic group was higher than that in the pena group [67.3% (8/49) vs. 38.5% (15/39), χ2=7.308, P=0.007]. Constipation occurred in 6 (12.2%) patients in the laparoscopic group, of whom 5 were improved by diet regulation and 1 required laxatives, while 9 (23.1%) patients developed constipation in the pena group, of whom 4 were improved by diet regulation and 5 required long?term laxatives. The difference of constipation ratio was not statistically significant (χ2=1.802, P=0.180). There were no cases of Krickenbeck constipation grade 3. Compared to the pena group, the laparoscopic group had higher anal resting pressure [(33.35 ± 9.69) mmHg vs. (27.68 ± 10.74) mmHg, t=2.599, P=0.011], higher dilating pressure [(9.00±5.61) mmHg vs.(6.51±3.24) mmHg, t=2.462, P=0.016], higher maximal squeeze pressure [(65.80 ± 17.23) mmHg vs. (56.74 ± 18.93) mmHg, t=2.389, P=0.019] and longer maximal contraction time [(21.16±5.02) seconds vs. (18.44±7.24) seconds, t=2.079, P=0.041]. The rectal resting pressure [(5.36 ± 3.00) mmHg vs. (4.61 ± 3.93) mmHg, t=1.015, P=0.312] was not statistically significantly different. Conclusions Compared with posterior sagittal anorectoplasty, laparoscopic?assisted anorectoplasty in the treatment of high and middle anal atresia has better long?term efficacy with less perioperative complications.

Objective To explore the long?term efficacy of laparoscopic?assisted anorectoplasty and conventional anorectoplasty in the treatment of children with high and middle anal atresia. Methods A retrospective cohort study was used. Inclusion criteria: (1) children with high and middle anal atresia; (2) complicated with rectourethral or rectovesical fistula; (3) complete follow?up data. Exclusion criteria: (1) complicated with 21?trisomy; (2) cerebral palsy and other mentaldisabilities; (3) Currarino syndrome; (4) FG syndrome. Clinical data of 88 patients with middle and high anal atresia, who complicated with rectourethral fistula or rectovesical fistula, and underwent anoplasty at Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from January 2009 to June 2014 were enrolled in the study and analyzed. There were 24 cases with middle atresia and 64 cases with high atresia. All the cases were divided into 2 groups based on the operative method: laparoscopic group (laparoscopic?assisted anorectoplasty, 49 cases), pena group (posterior sagittal anorectoplasty, 39 cases). The demographic features of two groups were comparable. There were no statistically significant differences in gender, age, body mass, classification of anomaly types and sacral ratio (all P>0.05). Student t test and Chi square tests were used to compare the surgical conditions (operative time, postoperative hospital stay and complications), anal function (Kelly score), constipation (Krickenbeck constipation score) and anorectal pressure. Results Children of both groups all completed operation ssuccessfully. There were no statistically significant differences between laparoscopic group and pena group in the operative time [(120±31) minutes vs. (112±23) minutes, t=1.343, P=0.091] and postoperative hospital stay [(7.1±2.3) days vs. (10.7±3.3) days, t=6.021, P=1.000]. Complications were more common in the pena group [16.3% (8/49) vs. 35.9% (14/39), χ2=4.436, P=0.035]. The main complications in laparoscopic group were anal prolapse (8.2%, 4/49) and anal stenosis (6.2%, 3/49), while in pena group were anal stenosis (12.8%, 5/39) and perioperative perianal skin erosion (10.3%, 4/39). As for the anal function, the degree of feces, defecation control and sphincter contractility, the single scoring differences of Kelly scoring system were not statistically significant between the two groups, but the proportion of good function in the laparoscopic group was higher than that in the pena group [67.3% (8/49) vs. 38.5% (15/39), χ2=7.308, P=0.007]. Constipation occurred in 6 (12.2%) patients in the laparoscopic group, of whom 5 were improved by diet regulation and 1 required laxatives, while 9 (23.1%) patients developed constipation in the pena group, of whom 4 were improved by diet regulation and 5 required long?term laxatives. The difference of constipation ratio was not statistically significant (χ2=1.802, P=0.180). There were no cases of Krickenbeck constipation grade 3. Compared to the pena group, the laparoscopic group had higher anal resting pressure [(33.35 ± 9.69) mmHg vs. (27.68 ± 10.74) mmHg, t=2.599, P=0.011], higher dilating pressure [(9.00±5.61) mmHg vs.(6.51±3.24) mmHg, t=2.462, P=0.016], higher maximal squeeze pressure [(65.80 ± 17.23) mmHg vs. (56.74 ± 18.93) mmHg, t=2.389, P=0.019] and longer maximal contraction time [(21.16±5.02) seconds vs. (18.44±7.24) seconds, t=2.079, P=0.041]. The rectal resting pressure [(5.36 ± 3.00) mmHg vs. (4.61 ± 3.93) mmHg, t=1.015, P=0.312] was not statistically significantly different. Conclusions Compared with posterior sagittal anorectoplasty, laparoscopic?assisted anorectoplasty in the treatment of high and middle anal atresia has better long?term efficacy with less perioperative complications.

Objective:To evaluate endoscopic retrograde appendicitis therapy in treatment of children with acute uncomplicated appendicitis.Methods:Sixty children patients were admitted at the Affiliated Hospital of Zhengzhou University from Oct 2019 to Jun 2021 and were divided into ERAT group ( n=30) and LA group ( n=30). Results:All operations were successfully performed . ERAT children started oral feeding earlier [(6.8±2.0) h vs. (12.3±2.0) h, t=-10.636, P<0.001], postoperative hospital stay was shorter [(3.2±1.3) d vs. (5.0±1.3) d, t=-5.360, P<0.001]. After 14 months follow up, the recurrence rate in the ERAT group was 6%. The complication rate of LA was 10%. Conclusion:ERAT is a safe and effective therapy in treating children with acute uncomplicated appendicitis with low,acceptable recurrence rate.

Objective:To investigate the clinical efficacy of anal dimple anorectoplasty on female infants with congenital anal atresia combined rectal vestibular fistula.Methods:Clinical data of 69 female infants with congenital anal atresia combined rectal vestibular fistula admitted to Department of Pediatric Surgery, the First Affiliated Hospital of Zhengzhou University from July 2012 to July 2022 were retrospectively analyzed.They were divided into 2 groups according to the surgical methods: 34 cases of anal dimple anorectoplasty(group A) and 35 cases of anterior sagittal anorectoplasty(group B). The operation time, length of stay, short-term complications, long-term complications and bowel function (determined by the Rintala score at 6 months postoperatively) of the two groups were compared.The difference in the incidence of postoperative complications between groups was compared by Chi- square test, and the remaining differences between groups were compared by the paired t-test. Results:The operative time [(80.18±9.29) min vs.(103.85±8.26) min] and postoperative hospital stay[(6.10±1.52) d vs.(7.63±2.40) d] in group A were significantly shorter than those of group B ( t=11.40, 2.62; all P<0.05). The Rintala total score at 6 months postoperatively in group A was significantly higher than that of group B[(19.36±0.93) points vs.(18.76±0.44) points]( t=3.20, P<0.05). There were no significant differences in the incidences of short-term [(4/34, 11.8%) vs.(7/35, 20.0%)] and long-term complications [(2/34, 5.9%) vs.(4/35, 11.4%)]between group A and group B ( χ2=0.75, 0.75; all P>0.05). Conclusions:Anal dimple anorectoplasty for female infants with congenital anal atresia combined rectal vestibular fistula is safe and effective.

Objective:To evaluate the safety and efficacy of enhanced recovery after surgery(ERAS) in the perioperative period of congenital choledochal cysts in children.Methods:This is a multicenter prospective randomized controlled study. The clinical data of 273 pediatric congenital choledochal cysts(CCC) patients who underwent surgery at 14 medical centers with complete follow-up data were collected through the medical data analysis platform. Among them, 123 cases in ERAS group were managed perioperatively in strict accordance with ERAS mode, and 150 cases in conventional group were managed according to traditional mode. The length of hospital stay,time to first farting, time to complete feeding, the incidence of complications, cost and readmission rate within 30 days,stress indexes and liver function were compared between the two groups.Results:Compared with the conventional group, median time to start farting (2.0 d vs. 3.0 d, P<0.001), median time to complete feeding (5.0 d vs. 7.0 d, P<0.001), median postoperative hospitalization time (6.0 d vs. 9.0 d, P<0.001),the median total length of stay(13.0 d vs. 15.0 d, P<0.001) were shorter,the median hospitalization cost (37,000 yuan vs.43,000 yuan P<0.001) was lower, and stress indexes recovered quickly. The incidence of postoperative hospital stay and readimission rate within 30 d were not statistically different between the two groups. Conclusion:It is safe and feasible to implement ERAS for children with CCC in the perioperative period, which can reduce stress response, speed up recovery,and save medical costs.