OBJECTIVES: To study the clinical and bronchoscopic characteristics of tracheobronchial tuberculosis (TBTB) in children and to identify factors influencing residual airway obstruction or stenosis. METHODS: The clinical data of children with TBTB were retrospectively collected. The children were divided into two groups based on the last bronchoscopic result within one year of follow-up: a group with residual airway obstruction or stenosis (n=34) and a group without residual airway obstruction or stenosis (n=58). A multivariate logistic regression analysis was used to identify the factors influencing residual airway obstruction or stenosis in children with TBTB. Receiver operating characteristic (ROC) curves were used to analyze the predictive value of the factors influencing residual airway obstruction or stenosis in children with TBTB. RESULTS: A total of 92 children with TBTB were included, and the main symptoms were cough (90%) and fever (68%). In children under 1 year old, the incidence rates of dyspnea and wheezing were significantly higher than in other age groups (P<0.008). Chest CT findings included mediastinal or hilar lymph node enlargement (90%) and tracheobronchial stenosis or obstruction (61%). The lymphatic fistula type was the main type of TBTB observed bronchoscopically (77%). All children received interventional treatment, and the effective rate was 84%. During one year of follow-up, 34 children had residual airway obstruction or stenosis. The TBTB diagnostic time and the initiation of interventional treatment were significantly delayed in the group with residual airway obstruction or stenosis compared with the group without residual airway obstruction or stenosis (P<0.05). The multivariate logistic regression analysis showed that the TBTB diagnostic time was closely related to residual airway obstruction or stenosis in children (P<0.05). ROC curve analysis showed that at the cut-off value of 92 days of TBTB diagnostic time, the area under the curve for predicting residual airway obstruction or stenosis in children with TBTB was 0.707, with a sensitivity of 58.8% and a specificity of 75.9%. CONCLUSIONS The clinical manifestations of TBTB are nonspecific, and symptoms are more severe in children under 1 year old. TBTB should be suspected in children with tuberculosis and chest imaging indicating airway involvement. Delayed diagnosis of TBTB is associated with the development of residual airway obstruction or stenosis.
Infant ; Child ; Humans ; Bronchoscopy/methods* ; Constriction, Pathologic/complications* ; Bronchial Diseases/therapy* ; Retrospective Studies ; Tuberculosis/diagnosis* ; Airway Obstruction/therapy*

Objective: To investigate outcomes of laryngotracheal reconstruction (LTR)with anterior and posterior costal cartilage grafts in severe pediatric subglottic stenosis (SGS) or laryngeal web (LW). Methods: A review of patients with severe subglottic stenosis or laryngeal web between January 2020 and January 2022 was performed. Demographic features including gender, age at diagnosis, age at surgery, etiology, airway support, and other comorbidities were collected preoperatively. Patients were evaluated in surgical site, breathing, swallowing, phonation and complications postoperatively.Descriptive analysis was used in this research. Results: Eight patients were included: six with grade Ⅲ SGS following Cotton-Myer grading scale, and two with type Ⅲ LW following Cohen's classification. All patients underwent LTR with anterior and posterior costal cartilage grafts. Five patients underwent single-stage LTR (ssLTR), and three patients underwent double-stage LTR (dsLTR). Seven out of eight patients were able to successfully extubate or decannulate with normal swallowing function; four patients had mild hoarseness, and three had moderate hoarseness. One patient failed in extubation, and underwent tracheotomy. Conclusions: LTR with anterior and posterior costal cartilage grafts is an effective and safe treatment for severe SGS or LW. Careful preoperative assessment of disease severity and overall medical status will help selection between ssLTR and dsLTR, thereby maximizing patient outcomes for both modalities.
Child ; Humans ; Constriction, Pathologic/complications* ; Costal Cartilage ; Hoarseness ; Laryngeal Diseases/complications* ; Laryngostenosis/etiology* ; Retrospective Studies ; Treatment Outcome

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
Child ; Humans ; Cholangitis, Sclerosing/diagnosis* ; Constriction, Pathologic/complications* ; In Situ Hybridization, Fluorescence ; Cholangiocarcinoma/therapy* ; Liver Diseases/complications* ; Cholestasis ; Inflammatory Bowel Diseases/therapy* ; Bile Ducts, Intrahepatic/pathology* ; Bile Duct Neoplasms/therapy*

Central lung cancer is a common disease in clinic which usually occurs above the segmental bronchus. It is commonly accompanied by bronchial stenosis or obstruction, which can easily lead to atelectasis. Accurately distinguishing lung cancer from atelectasis is important for tumor staging, delineating the radiotherapy target area, and evaluating treatment efficacy. This article reviews domestic and foreign literatures on how to define the boundary between central lung cancer and atelectasis based on multimodal images, aiming to summarize the experiences and propose the prospects.
Humans ; Lung Neoplasms/diagnostic imaging* ; Pulmonary Atelectasis/complications* ; Bronchi ; Constriction, Pathologic/complications* ; Multimodal Imaging

Long-segment lichen sclerosus (LS) urethral stricture is a challenge for urologists. Limited data are available for surgeons to make a surgical decision between Kulkarni and Asopa urethroplasty. In this retrospective study, we investigated the outcomes of these two procedures in patients with LS urethral stricture. Between January 2015 and December 2020, 77 patients with LS urethral stricture underwent Kulkarni and Asopa procedures for urethroplasty in the Department of Urology, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine (Shanghai, China). Of the 77 patients, 42 (54.5%) underwent the Asopa procedure and 35 (45.5%) underwent the Kulkarni procedure. The overall complication rate was 34.2% in the Kulkarni group and 19.0% in the Asopa group, and no difference was observed ( P = 0.105). Among the complications, no statistical difference was observed in the incidence of urethral stricture recurrence ( P = 0.724) or glans dehiscence ( P = 0.246) except for postoperative meatus stenosis ( P = 0.020). However, the recurrence-free survival rate between the two procedures was significantly different ( P = 0.016). Cox survival analysis showed that antiplatelet/anticoagulant therapy use ( P = 0.020), diabetes ( P = 0.003), current/former smoking ( P = 0.019), coronary heart disease ( P < 0.001), and stricture length ( P = 0.028) may lead to a higher hazard ratio of complications. Even so, these two techniques can still provide acceptable results with their own advantages in the surgical treatment of LS urethral strictures. The surgical alternative should be considered comprehensively according to the patient characteristics and surgeon preferences. Moreover, our results showed that antiplatelet/anticoagulant therapy use, diabetes, coronary heart disease, current/former smoking, and stricture length may be contributing factors of complications. Therefore, patients with LS are advised to undergo early interventions for better therapeutic effects.
Male ; Humans ; Urethral Stricture/etiology* ; Retrospective Studies ; Constriction, Pathologic/surgery* ; Lichen Sclerosus et Atrophicus/surgery* ; Treatment Outcome ; Urologic Surgical Procedures, Male/methods* ; China ; Urethra/surgery* ; Postoperative Complications/etiology* ; Mouth Mucosa ; Diabetes Mellitus/etiology* ; Anticoagulants ; Coronary Disease

OBJECTIVES: To investigate the clinical application of endoscopic esophageal dilation in the treatment of corrosive esophageal strictures in children. METHODS: A retrospective analysis was performed on the clinical data of 15 children with corrosive esophageal strictures who underwent endoscopic esophageal dilation in Children's Hospital, Zhejiang University School of Medicine. The clinical features, treatment modality of endoscopic esophageal dilation, number of dilations, complications, and prognosis were reviewed. RESULTS: A total of 96 esophageal dilations were performed in the 15 children with corrosive esophageal strictures, with a median of 6 dilations per child. Among them, 9 children (60%) underwent 6 or more dilations. The children with a stricture length of >3 cm had a significantly higher number of dilations than those with a stricture length of ≤3 cm (P<0.05). The children with strictures in a single segment had a significantly better treatment outcome than those with strictures in multiple segments (P=0.005). No complication was observed during all sessions of dilation. The overall effective rate (including significant improvement and improvement) of endoscopic esophageal dilation treatment was 87%, with 2 cases of failure. CONCLUSIONS Endoscopic esophageal dilation is an effective and relatively safe treatment method for corrosive esophageal strictures in children, and children with strictures in a single segment tend to have a better treatment outcome than those with strictures in multiple segments.
Child ; Humans ; Esophageal Stenosis/therapy* ; Constriction, Pathologic/complications* ; Dilatation/methods* ; Caustics ; Retrospective Studies ; Treatment Outcome

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is the endoscopic modality of choice for the treatment of biliary and pancreatic diseases. However, patients with cirrhosis, particularly those with decompensated cirrhosis, are believed to be at increased risk for complications associated with ERCP. There is a paucity of literature describing the outcomes of ERCP for patients with cirrhosis. This study aimed to investigate the outcomes of ERCP for cirrhosis patients, especially adverse events, and evaluated its safety and efficacy. METHODS: We performed a multicenter, retrospective study of all patients at Beijing Friendship Hospital affiliated to Capital Medical University, Xijing Hospital affiliated to Air Force Military Medical University, Beijing Youan Hospital affiliated to Capital Medical University, and the Fifth Medical Center of the People's Liberation Army General Hospital from June 2003 to August 2019. The adverse events of inpatient ERCP for patients with ( n  = 182) and without liver cirrhosis (controls; n  = 385) were compared. RESULTS: A total of 567 patients underwent ERCP between January 2003 and December 2019 were enrolled in this study. Compared to patients without cirrhosis, patients with cirrhosis were at higher risk for postoperative complications (odds ratio [OR], 4.172; 95% confidence interval [CI], 1.232-7.031; P  < 0.001) such as postoperative pancreatitis (OR, 2.026; 95% CI, 1.002-4.378; P  = 0.001) and cholangitis (OR, 3.903; 95% CI, 1.001-10.038; P  = 0.036). The main indications for ERCP for patients with cirrhosis in this study included choledocholithiasis (101 cases; 55.5%), benign bile duct strictures (46 cases; 25.3%), and malignant bile duct strictures (28 cases; 15.4%). Among them, 23 patients (12.6%) underwent balloon dilation and 79 patients (43.4%) underwent sphincterotomy. Of the patients with cirrhosis, delayed bleeding occurred in ten patients (5.5%), postoperative pancreatitis occurred in 80 patients (44.0%), and postoperative cholangitis occurred in 25 patients (13.7%). An additional multivariate analysis showed that the total bilirubin (TBIL) level (OR, 4.58; 95% CI, 2.37-6.70) and Child-Pugh score of C (OR, 3.11; 95% CI, 1.04-5.37) were risk factors for postoperative complications in patients with cirrhosis. CONCLUSIONS Compared with the general population of patients undergoing ERCP, patients with cirrhosis were more prone to postoperative pancreatitis and cholangitis. TBIL levels and Child-Pugh scores were risk factors for postoperative complications in patients with cirrhosis.
Humans ; Cholangiopancreatography, Endoscopic Retrograde/adverse effects* ; Retrospective Studies ; Constriction, Pathologic ; Risk Factors ; Liver Cirrhosis/complications* ; Pancreatitis/etiology* ; Postoperative Complications/epidemiology* ; Cholangitis

Objective To investigate the clinical characteristics and genetic mutations in Kindler syndrome(KS)and provide a theoretical basis for the diagnosis and treatment of KS. Methods The clinical data of one case of KS from Peking Union Medical College Hospital and 185 cases reported in literature were collected. The gene mutation types,patient clinical data,and tumor characteristics were statistically analyzed. Results A total of 186 cases were enrolled,including 110 males and 76 females,with the mean age of(28±16)years. The data of gene mutation and specific clinical manifestations were available in 151 and 94 patients,respectively. The main clinical manifestations of KS included poikiloderma,occurrence of blister in childhood,and photosensitivity,and the secondary clinical manifestations included oral inflammation,palmoplantar keratoderma,webbing/pseudoainhum,dysphagia,urethral stricture and so on.Oral inflammation(r=0.234,P=0.023),palmoplantar keratoderma(r=0.325,P=0.001),webbing/pseudoainhum(r=0.247,P=0.016),dysphagia(r=0.333,P=0.001),urethral stricture(r=0.280,P=0.006)were significantly correlated with age,showing significantly higher incidence in the patients over 32 years old.Urethral stricture(χ²=11.292,P=0.001)and anal stenosis(χ²=4.014,P=0.045)were significantly correlated with sex,with higher incidence in males.Eighty different mutations were found in 151 patients,and the most common gene mutation was c.676C>T.Forty-one tumors occurred in 27 patients,among which squamous cell carcinoma accounted for 92.7%. The gene mutation site had no significant correlation with squamous cell carcinoma or patient country. Conclusions The c.676C>T in FERMT1 gene is the most common mutation in KS.The patients are prone to squamous cell carcinoma and mainly attacked at the exposure sites(hand and mouth).
Adolescent ; Adult ; Ainhum ; Blister ; Carcinoma, Squamous Cell ; Child ; Constriction, Pathologic ; Deglutition Disorders/complications* ; Epidermolysis Bullosa ; Female ; Humans ; Inflammation ; Keratoderma, Palmoplantar/complications* ; Male ; Membrane Proteins ; Mutation ; Neoplasm Proteins/genetics* ; Periodontal Diseases ; Photosensitivity Disorders ; Urethral Stricture/complications* ; Young Adult

Objective: To study the clinical diagnosis and treatment strategies for vein-related pulsatile tinnitus patients with transverse sinus stenosis. Methods: The clinical data of patients with vein-related pulsatile tinnitus, from January 2015 to August 2019, were collected,whose digital subtraction angiography showing transverse sinus stenosis. Taking December 2019 as the last follow-up time, we analyzed the clinical characteristics, CT angiography and digital subtraction angiography results, lumbar puncture pressure and cerebrospinal fluid composition, and other auxiliary examination results (pure tone audiometry, fundus examination of papilledema, carotid ultrasonography, bone density screening, endocrinous test), as well as tinnitus handicap inventory, treatment options and follow-up results. Results: 83 patients were enrolled with female of 89.2% (74/83) and male of 10.8%(9/83); 65.1% (54/83) with right tinnitus, 31.3% (26/83) with left tinnitus, and 3.6% (3/83) with bilateral tinnitus; 67.5% (56/83) with right dominant sinus, 19.3% (16/83) with left dominant sinus, 13.3% (11/83) with bilateral equalization; Bilateral and ipsilateral stenosis accounted for 55.4% and 44.6% respectively; BMI was overweight or obese in 41 cases (49.4%, 41/83). Patients with tinnitus handicap inventory level three or above accounted for 79.5% (66/83). Eventually, 33 patients chose conservative observation (39.8%, 33/83), 40 patients (48.2%), 8 patients (9.6%) and 2 patients (2.4%) received sigmoid sinus-related surgery, interventional surgery, or emissary vein occlusion respectively. The mean follow-up time of 74 patients was 26.2 months. The data of 48 surgery patients showed that the pressure differences of venous sinus among the recurrent patients were more obvious; Interventional surgery with simultaneous stenting placement was effective. Tinnitus did not decrease in two patients with emissary vein occlusion. Analysis of 26 patients with lumbar puncture revealed eight cases of normal cranial pressure and 18 cases of high cranial pressure. The sinus pressure difference between the two groups was different (P=0.025), but the difference of age of onset, concomitant symptoms, BMI, proportion of empty sella or papilledema was not statistically significant (P>0.05). Conclusions: The evaluation of patients with vein-related pulsatile tinnitus requires a standardized procedure. Papilledema cannot be used as a sensitive indicator in patients with early intracranial hypertension. Venous sinus pressure difference may be one of the indicators of intracranial hypertension, and the lumbar puncture is the gold standard for the diagnosis. Weight loss can be used as a conservative treatment during the observation period. Significant sinus stenosis is a risk factor for recurrence in patients undergoing sigmoid sinus surgery. Interventional stenting is an effective treatment for tinnitus secondary to transverse sinus stenosis.
Constriction, Pathologic/complications* ; Cranial Sinuses ; Female ; Humans ; Male ; Neoplasm Recurrence, Local ; Stents ; Tinnitus/etiology*

PURPOSE: The laparoscopic rectopexy has become increasingly popular with verified stability, surgical route selection should be tailored to individual patient characteristics rather than operative risk. The perineal approach is useful in young male patients who need to preserve fertility. This study aimed to compare the characteristics of men and women who underwent Delorme-Thiersch procedures and analyze the postoperative outcomes of the perineal approach by sex. METHODS: We retrospectively reviewed the medical records of 293 patients who underwent Delorme-Thiersch operations in Seoul Song Do Colorectal Hospital between January 2011 and September 2017. Patient clinical characteristics and postoperative complications were analyzed by sex. We analyzed surgical outcomes with preoperative and 3-month postoperative incontinence questionnaires, constipation levels, and anal manometry. RESULTS: In this study, men with rectal prolapse were younger than women with the same condition. American Society of Anesthesiologists physical status classifications were higher in women and women had more L-spine X-ray and pudendal nerve terminal motor latency test abnormalities. Anorectal manometry pressures were higher in men. Men also had longer operation times and hospital stays and more postoperative complications (8 T ring infections, 6 patients with bleeding, 3 with strictures, 2 with severe pain, and 2 with rectal perforations). The recurrence rate was higher among women. CONCLUSION: Men with rectal prolapse were younger, healthier, and had relatively better anorectal function than women. The Delorme-Thiersch operation in men promoted lower recurrence rates and was advantageous in preserving the fertility of young patients, but the incidence of complications was also higher in men. Adequate counseling and preparation for the possibility of complications are needed.
Classification ; Constipation ; Constriction, Pathologic ; Counseling ; Female ; Fertility ; Hemorrhage ; Humans ; Incidence ; Length of Stay ; Male ; Manometry ; Medical Records ; Music ; Postoperative Complications ; Pudendal Nerve ; Rectal Prolapse ; Recurrence ; Retrospective Studies ; Seoul