Objective To explore the feasibility and application skills of transabdominal preperitoneal prosthesis (TAPP) with cross-encircling arms of mesh.Methods From Septemer 2009 to February 2012,46 cases were given TAPP with cross-encircling arms of mesh.The clinical data and surgery videos were retrospectively analyzed.Results All of the 46 cases with 53 inguinal hernias were successful in TAPP.The mean operative time of unilateral hernia was (57.74 ± 11.89) min,the mean operative time of 7 cases with biliateral hernia was (83.86 ±20.42) min and the longest time was 125 min.There were 6 cases with the contralateral hidden hernia,2 cases with hematoma,1 case with paresthesias of skin,no realpse.Conclusion The mesh with cross-encircling arms can be exactly fixed by spermatic cord,ductus deferens or round ligament of uterus,without stitching and stappling.

Objective To sum up the clinical manifestations and laboratory features for the diagnosis of pediatric tuberculous pleurisy,and to improve the recognition of this disease in early stage.Methods A retrospective study of 113 children diagnosed as tuberculous pleurisy from August 2006 to September 2014 in the Second Department of Respiratory Medicine,Beijing Children's Hospital Affiliated to Capital Medical University was conducted.Meanwhile,another 113 cases of children with mycoplasma pneumoniae pneumonia complicated with pleurisy were selected as control group.The general information,clinical symptoms,pleural effusion and imaging features between 2 groups were analyzed and compared by using SPSS 16.0 statistical software.Results The proportion of patients with cough in tuberculous pleurisy group and control group was 47.79％ (54/113 cases) and 99.12％ (112/113 cases) (x2 =76.33,P ＜ 0.01) respectively,and the proportion with severe cough was 3.70％ (2/54 cases) and 97.32％ (109/112 cases) (x2 =144.10,P ＜0.01),while the disease duration was 15.00 (10.00,30.00) days and 10.00 (8.00,14.50) days (W =8 668.00,P ＜ 0.01),respectively,and all the differences between 2 groups were significant.The proportion of patients with low fever,moderate fever,high fever and hyper fever was 8.65％ (9/104 cases),47.12％ (49/104 cases),44.23 ％ (46/104 cases) and 0,respectively in tuberculous pleurisy group,while the proportion was 0.90％ (1/111 cases),18.92％ (21/111 cases),79.28％ (88/111 cases) and0.90％ (1/111 cases) respectively in control group,and the difference between 2 groups was significant(W =9 064.00,P ＜ 0.01).The unilateral effusion ratio in tuberculous pleurisy group and the control group was 94.69％ (107/113 cases) and 71.68％ (81/113 cases),respectively (x2 =21.39,P ＜ 0.01).The monocyte ratio was higher in tuberculous pleurisy group [0.89 (0.76,0.93)] than that in the controlgroup [0.60 (0.30,0.78)] (W =888.50,P ＜ 0.01) and the level of protein in 2 groups was [51.00 (47.35,54.20) g/L] and [42.10 (37.85,46.15) g/L],respectively (W =842.50,P ＜ 0.01).The level of lactate dehydrogenase (LDH) in tuberculous pleurisy group[553.50 (358.00,749.25) U/L] was lower than that in the control group[1 189.10 (670.95,1 820.00) U/L] (W =2 186.00,P ＜ 0.01),and the differences were significant between 2 groups.In addition to pleural effusion,the high density was the main feature of imaging examination in 2 groups.The proportion of patients with atelectasis was 77.88％ (88/113 cases) and 4.42％ (5/113 cases) (x2 =125.90,P ＜ 0.01),while the proportion of patients with consolidation was 4.42％ (5/113 cases) and 72.57 ％ (82/113 cases),respectively (x2=110.80,P ＜ 0.01).All the differences between 2 groups were significant.The sputum culture-positive rate of mycobacterium tuberculosis was only 1.77％ (2/113 cases) and the other pathogen examinations were negative in tuberculous pleurisy group.Conclusions For patients with unilateral pleural effusion,when the onset only has fever (moderate-high fever),and respiratory symptoms are not clear or symptoms are not proportional to radiographic severity,or when high monocytes proportion (＞ 0.70) in pleural effusion and radiographic evidence of compression atelectasis are observed,tuberculous pleurisy should be considered and further questioning of the predisposing factors,purified protein derivative test should be taken so as to diagnose the tuberculous pleurisy at early stage.

We reported a women with omithine carbamoyltransferase deficiency who delivered a healthy boy after two pregnancies with adverse outcome with the help of a multidiscipline team.The woman was admitted to Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine with an acute prenatal hyperammonemic episode at 28 gestational weeks of her first pregnancy in 2013 and was diagnosed with ornithine transcarbamylase deficiency.Her hyperammonemic complications were controlled under a well-planned multidisciplinary management including a low-protein diet and appropriate medications assisting nitrogen removal.A boy was delivered by cesarean section at 32 weeks of gestation but died three days later.Mutation analysis revealed a hemizygous c.583G＞A (G195R) mutation in the neonatal omithine carbamyltransferase gene and his mother was a heterozygous carrier with the same mutation.Two years later in 2015,the patient was pregnant spontaneously.However,she received an induced abortion at 21 weeks of gestation because amniocentesis and DNA analysis showed that the male fetus had the same omithine transcarbamylase gene mutation.The index pregnancy was assisted by in vitro fertilization-embryo transfer and preimplantation genetic diagnosis in 2017 and the woman delivered a healthy boy with the management ofa multidisciplinary team.

Objective To assess the diagnostic value of sweat conductivity testing in Chinese children with cystic fibrosis (CF). Methods This is a retrospective study. Sweat conductivity tests were conducted in 45 CF children (CF group) and 200 non‐CF children (non‐CF group) diagnosed with other chronic pulmonary diseases at the No. 2 Department of Respiratory Medicine, Beijing Children′s Hospital from May 2014 to June 2018. Pearson′s chi‐square test was used to assess the differences between CF and non‐CF groups. A receiver operating characteristic curve was constructed to calculate the best cut‐off value to diagnose or rule out CF. The pulmonary function parameters (forced expiratory volume in the first second, forced vital capacity,forced expiratory flows at 75% of exhaled vital capacity) of CF children over 6 years old were analyzed. The relationship between sweat conductivity and pulmonary function was compared between the two groups (80‐120mmol/L vs.>120mmol/L). Results The age of CF group was 9 (7, 12) years old, 19 males (42%) and 26 females(58%); the age of non‐CF group was 8 (5,11) years old, 106 males (53%) and 94 females(47%). The results of sweat conductivity test showed that sweat conductivity in CF group 108(99, 122) mmol/L was significantly higher than that in non‐CF group 43(36, 52) mmol/L (χ2=207, P<0.01). A cut‐off value of 80 mmol/L for CF diagnosis showed a sensitivity of 93.3% and a specificity of 98.5%. The receiver operating characteristic curve analysis suggested the best conductivity cut‐off value for the diagnosis of CF was at 83.5 mmol/L,with a sensitivity of 93.3% and a specificity of 100%,and an area under the curve of 0.993 (95% confidence interval 0.985-1.000). The best conductivity cut‐off value to rule out CF diagnosis was at 63.5 mmol/L,with a sensitivity of 97.8% and a specificity of 90.5%. There was no correlation between the level of sweat conductivity and the extent of pulmonary function decline. Conclusions Sweat conductivity testing can be used for the screening of CF in Chinese children. A diagnosis of CF should be considered if the value is greater than 80 mmol/L.

Objective:To analyze the clinical features of cystic fibrosis (CF) associated allergic bronchopulmonary aspergillosis (ABPA) in children.Methods:A retrospective study was performed in 22 children who were diagnosed with CF associated ABPA in Beijing Children′s Hospital affiliated to Capital Medical University from March 2010 to March 2020. The clinical features, imaging characteristics, laboratory results and the prognosis were reviewed.Results:A total of 22 cases met the diagnostic criterion, including 12 males and 10 females. The age of diagnosis was (10.4±2.8) years and the age of onset was (5.5±4.4) years. Clinical manifestations included cough and expectoration (22 cases), recurrent wheezing (15 cases), hemoptysis (7 cases), failure to thrive (12 cases), pancreatitis (10 cases), hepatomegaly (7 cases), splenomegaly (4 cases) and steatorrhea (4 cases). CT scans of all the patients showed pulmonary infiltrates and central bronchiectasis, combined with mucoid impaction in 17 cases and high density mucus plug in 12 cases. Eosinophilia was found in 18 patients. Total IgE and serum levels of A. fumigatus-specific IgE were elevated in all 22 patients. Positive culture of sputum or bronchoalvedar lauage fluid for fungus were in 15 cases, with single Aspergillus infection in 8 cases and mixed Aspergillus infection in 3 cases. The predominant bacteria found in the airways were Pseudomonas aeruginosa (17 cases), followed by staphylococcas. aureus (6 cases) and stenotrophomonas. maltophilia (5 cases). Pulmonary function revealed obstructive ventilation dysfunction in 4 cases, mixed dysfunction in 11 cases, and small airway dysfunction in 4 cases. Regarding the treatment, 3 were treated only with systemic corticosteroid, while the remaining 19 cases also received antifungal agents.The follow up continued for 1-7 years, and 6 maintained remission, 10 had recurrent episodes, 1 died, and 5 lost to follow up. Conclusions:CF associated ABPA is extremely rare in China. The overlapping clinical, radiographic, and immunologic features of these two diseases make the diagnosis challenging. Systemic corticosteroids are considered the first-line therapy for these patients, and adjuvant antifungal agents may be helpful. Recurrence rate in our center is high.

Objective:To explore the early clinical clues for diagnosis of chronic granulomatous disease (CGD).Methods:One hundred and thirty-nine children with CGD seen in Beijing Children′s Hospital from January 2007 to October 2020 were included in this study. The clinical features including age of onset, first presentations, reason for being hospitalized, etiology, imaging features, clues for early diagnosis of all patients were evaluated retrospectively. According to the time of diagnosis, the patients were divided into two groups, cases diagnosed before 2015 and after 2015 and 2015. The time of diagnosis, the length of stay and the hospital charges were compared between the two groups. T test and χ 2 test were used for statistical analyses. Results:One hundred and nineteen of the cases were males and 20 were females. The age of onset was 4 months (8 d to 14 years), and 103 cases (74.1%) had onset before 1 year of age. The age at diagnosis was 1.8 years (21 d to 14.7 years), and the time of diagnosis delay was 1 year (7 d to 13.7 years). One hundred and thirty-five cases (97.1%) had pulmonary infection as the main reason for hospitalization, of whom 76 cases (56.3%) had positive pulmonary etiology. One hundred and thirty-six patients (97.8%) were referred cases, of whom 5 were suspected of CGD before referral, and the misdiagnosis rate was as high as 96.3% (131/136). Eight early clues for diagnosis were found, the frequency from high to low, large bacillus Callmette-Guer scar in 99 cases (70.5%), left axillary lymphadenopathy or calcification in 73 cases (52.5%), skin or other lymph node infections in 58 cases (41.7%), skin scars in 50 cases (36.0%), multiple lung nodules in 42 cases (30.2%), perianal abscess in 35 cases (25.2%), pulmonary Aspergillus infection in 26 cases (18.7%) and pulmonary Burkholderia infection in 15 cases (10.8%). A total of 120 cases of CGD were diagnosed by respiratory burst test during hospitalization, including 55 cases diagnosed before 2015 and 65 cases diagnosed after 2015. After using these 8 early diagnosis clues, the cases diagnosed after 2015 had shorter time of diagnosis and the length of stay and lower hospitalization charge than cases diagnosed before 2014, and the difference was statistically significant ((25±7) vs. (10±5) d, (29±7) vs. (18±6) d, (3.7×10 4±1.2×10 4) vs. (3.2×10 4±1.2×10 4) Yuan, t=13.763, 9.262, 2.381, all P<0.05). Conclusions:Patients with CGD are younger at onset and the diagnosis is delayed. Pulmonary infections are the most common. Large BCG scar, left axillary lymphadenopathy or calcification, skin or other lymph node infections, skin scars, multiple lung nodules, perianal abscesses, pulmonary Aspergillus infection and Burkholderia infection can help early diagnosis of CGD.

Objective:To investigate and summarize pediatric patients with severe Mycoplasma pneumoniae pneumonia (MPP) presenting with varied clinical and chest imaging features in order to guide the individualized treatment. Methods:This was a retrospective cohort study. Medical records of clinical, imaging and laboratory data of 505 patients with MPP who were admitted to the Department Ⅱ of Respirology Center, Beijing Children′s Hospital, Capital Medical University from January 2016 to October 2023 and met the enrollment criteria were included. They were divided into severe group and non-severe group according to whether lower airway obliterans was developed. The clinical and chest imaging features of the two groups were analyzed. Those severe cases with single lobe ≥2/3 consolidation (lobar consolidation) were further divided into subtype lung-necrosis and subtype non-lung-necrosis based on whether lung necrosis was developed. Comparison on the clinical manifestations, bronchoscopic findings, whole blood C-reactive protein (CRP) and other inflammatory indicators between the two subtypes was performed. Comparisons between two groups were achieved using independent-sample t-test, nonparametric test or chi-square test. Univariate receiver operating characteristic (ROC) curve analyses were performed on the indicators such as CRP of the two subtypes. Results:Of the 505 cases, 254 were male and 251 were female. The age of the onset was (8.2±2.9) years. There were 233 severe cases, among whom 206 were with lobar consolidation and 27 with diffuse bronchiolitis. The other 272 belonged to non-severe cases, with patchy, cloudy infiltrations or single lobe <2/3 uneven consolidation or localized bronchiolitis. Of the 206 cases (88.4%) severe cases with lobar consolidation, 88 harbored subtype lung-necrosis and 118 harbored subtype non-lung-necrosis. All 206 cases (100.0%) presented with persistent high fever, among whom 203 cases (98.5%) presented with inflammatory secretion obstruction and plastic bronchitis under bronchoscopy. Of those 88 cases with subtype lung-necrosis, there were 42 cases (47.7%) with dyspnea and 39 cases (44.3%) with moderate to massive amount of pleural effusion. There were 35 cases (39.8%) diagnosed with lung embolism during the disease course, of which other 34 cases (38.6%) were highly suspected. Extensive airway mucosal necrosis was observed in 46 cases (52.3%), and the level of their whole blood CRP was significantly higher than that of subtype non-lung-necrosis (131.5 (91.0, 180.0) vs. 25.5 (12.0, 43.1) mg/L, U=334.00, P<0.001). They were regarded as subtype "lung consolidation-atelectasis-necrosis". Of those 118 cases with subtype non-lung-necrosis, 27 cases (22.9%) presented with dyspnea and none were with moderate to massive amount of pleural effusion. Sixty-five cases (55.1%) presented with plastic bronchitis and localized airway mucosal necrosis was observed in 32 cases (27.1%). They were deemed as subtype "lung consolidation-atelectasis". ROC curve analyses revealed that whole blood CRP of 67.5 mg/L on the 6-10 th day of disease course exhibited a sensitivity of 0.96, a specificity of 0.89, and an area under the curve of 0.97 for distinguishing between these two subtypes among those with lobar consolidation. Conclusions:Pediatric patients with severe MPP present with lobar consolidation or diffuse bronchiolitis on chest imaging. Those with lobar consolidation harbor 2 subtypes as "lung consolidation-atelectasis-necrosis" and "lung consolidation-atelectasis". Whole blood CRP of 67.5 mg/L can be applied as an early discriminating indicator to discriminate between these two subtypes.

Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with cystic fibrosis (CF). Methods: This is a retrospective study. Sweat conductivity tests were conducted in 45 CF children (CF group) and 200 non-CF children (non-CF group) diagnosed with other chronic pulmonary diseases at the No. 2 Department of Respiratory Medicine, Beijing Children′s Hospital from May 2014 to June 2018. Pearson′s chi-square test was used to assess the differences between CF and non-CF groups. A receiver operating characteristic curve was constructed to calculate the best cut-off value to diagnose or rule out CF. The pulmonary function parameters (forced expiratory volume in the first second, forced vital capacity,forced expiratory flows at 75% of exhaled vital capacity) of CF children over 6 years old were analyzed. The relationship between sweat conductivity and pulmonary function was compared between the two groups (80-120mmol/L vs.>120mmol/L). Results: The age of CF group was 9 (7,12) years old, 19 males (42%) and 26 females(58%); the age of non-CF group was 8 (5,11) years old, 106 males (53%) and 94 females(47%). The results of sweat conductivity test showed that sweat conductivity in CF group 108(99, 122) mmol/L was significantly higher than that in non-CF group 43(36, 52) mmol/L (χ²=207, P<0.01). A cut-off value of 80 mmol/L for CF diagnosis showed a sensitivity of 93.3% and a specificity of 98.5%. The receiver operating characteristic curve analysis suggested the best conductivity cut-off value for the diagnosis of CF was at 83.5 mmol/L,with a sensitivity of 93.3% and a specificity of 100%,and an area under the curve of 0.993 (95% confidence interval 0.985-1.000). The best conductivity cut-off value to rule out CF diagnosis was at 63.5 mmol/L,with a sensitivity of 97.8% and a specificity of 90.5%. There was no correlation between the level of sweat conductivity and the extent of pulmonary function decline. Conclusions Sweat conductivity testing can be used for the screening of CF in Chinese children. A diagnosis of CF should be considered if the value is greater than 80 mmol/L.

Child ; Humans ; Granulomatous Disease, Chronic/complications* ; Pneumonia