BACKGROUND: Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited. METHODS: This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed. RESULTS: Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]-3rd quartile [Q3]: 11-44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset ( P  = 0.021) and transitioning to plaque psoriasis ( P  = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1-Q3: 2-41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1-Q3: 13.0-23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP. CONCLUSIONS The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.
Humans ; Male ; Female ; Psoriasis/pathology* ; Adult ; Cross-Sectional Studies ; Adolescent ; Child ; Young Adult ; Quality of Life ; Middle Aged ; China/epidemiology* ; Recurrence ; Risk Factors ; Surveys and Questionnaires ; East Asian People

Objective:To comparative analyze mammographic and clinicopathological findings of ductal carcinoma in situ (DCIS) and DCIS with microinvasion (DCISM), and to investigate the predictive factors for DCISM.Methods:A total of 626 patients with DCISM and DCIS confirmed by surgery and pathology in the Affiliated Hospital of Qingdao University from January 2016 to July 2020 were collected and underwent preoperative mammography. The X-ray findings of DCISM and DCIS patients were classified and diagnosed according to the Breast Imaging Reporting and Data System (BI-RADS) criteria. The differences in clinicopathological and radiographic findings between DCISM and DCIS patients were analyzed using χ 2 test or Fisher exact test. The risk factors of DCISM were evaluated by using univariate and multivariate binary logistic regression analysis. Results:Among the 626 cases, 171 were diagnosed as DCISM, 455 were diagnosed as DCIS. Large diameter (≥2.7 cm), high nuclear grade, comedo type, axillary lymph node metastasis, high Ki67 proliferation index, negativity of estrogen receptor and progesterone receptor were found to be predictors of DCISM in the univariate analysis (all P<0.05). And large diameter (≥2.7 cm)(OR 2.229,95% CI 1.505-3.301, P<0.001), high nuclear grade(OR 1.711,95%CI 1.018-2.875, P=0.043) and axillary lymph node metastasis(OR 4.140,95% CI 1.342-12.773, P=0.013) were found to be independent predictors of DCISM in the multivariate analysis (all P<0.05). Mammographically, the lesion types, the presence and distribution of calcification were statistically significant between DCIS and DCISM patients (χ 2=17.42, 9.65, 9.10, P<0.05). Up to 17.6% (80/455) of DCIS were occult leisions, and DCISM showed more lesions with calcification in mass, asymmetry, and architectural distortion (49.1%, 84/171). Grouped calcifications were usually associated with DCIS (41.5%, 120/289), while regional calcification were commonly found in DCISM (35.9%, 47/131). Conclusions:Lesions with calcification and regional calcification were more likely associated with DCISM on mammography. Large diameter (≥2.7 cm), high nuclear grade and axillary lymph node metastasis were found to be independent predictors of DCISM.

Objective:To evaluate the diagnostic value of digital breast tomosynthesis (DBT) and digital mammography (DM) for radial lesions.Methods:The data of 76 patients (78 lesions) with radial lesions confirmed by operation and pathology on DBT between December 2016 and May 2020 in the Affiliated Hospital of Qingdao University were analyzed retrospectively. Taking pathological results as the gold standard, 78 lesions were divided into benign radial lesions ( n=46) and malignant radial lesions ( n=32), and their DBT features were compared. According to the standard of breast imaging report and data system (BI-RADS), the wheel-spoke structure, central density, overall size, central size and surrounding burr length of the two groups of radial lesions were compared on DBT. Results:The detection rates of DM and DBT for 78 radial lesions were 59.0% (46/78) and 100% (78/78), the difference had statistically significant ( P<0.05). The diagnostic accuracy rates of DM and DBT for 78 radial lesions was 65.2% (30/46) and 74.4% (58/78), the difference had no statistically significant ( P>0.05). The sensitivity, specificity, misdiagnosis rates, missed diagnosis rates of DM and DBT in the diagnosis of malignant radial lesions were 64.3%(18/28) and 84.4%(27/32), 66.7% (12/18) and 67.4%(31/46), 33.3%(6/18) and 32.6%(15/46), 35.7%(10/28) and 15.6%(5/32), respectively. The difference was not statistically significant ( P>0.05). There were significant differences in the overall size of lesions [18.0 (14.9, 29.2) mm, 26.5 (20.2, 34.9) mm], central size [3.5 (2.5, 4.5) mm, 4.5 (3.5, 5.5) mm] and peripheral burr length [(11±6) mm, (13±4) mm] between benign and malignant radial lesions on DBT ( P<0.05). When the central size of the lesion was 5 mm, there was significant difference in the distribution of benign and malignant radial lesions ( P<0.05), and when the overall size of the lesion was 2 cm, there was significant difference in the distribution of benign and malignant radial lesions ( P<0.05). Conclusion:DBT can improve the detection and diagnosis accuracy of radial lesions, and provide an important basis for clinicians to make surgical treatment decisions.

OBJECTIVE To analyse the result of mastoid abnormal MRI singals in infants without clinical symptoms and to evaluate the diagnostic value. METHODS The MRI data of abnormal signals in the middle ear and mastoid of 42 infants(62 ears) were analyzed with 1000 Hz probe tone tympanometry and oto-endoscope. RESULTS Of the infants with abnormal MRI signals of the middle ear and mastoid, 50 ears were secretory otitis media(80.7％), 10 ears had dysfunction of middle ear (16.1％), and 2 ears were not identified. CONCLUSION In the infant who had a abnormal long T2 singals MRI but without clinical symptoms, 80.7％ were caused by SOM or AOM. It is valuable for clinical efficacy evaluation and treatment planning in advance.

Objective To compare the diagnostic value of digital breast tomosynthesis (DBT), digital mammography(DM),and ultrasonography(US)for the non-calcified ductal carcinoma in situ(DCIS) of the breast.Methods To retrospectively analyze the imaging and clinical data of ductal carcinoma in situ which was confirmed by surgical pathology and displayed as non-calcified lesions in mammography in 110 patients.DBT,DM and US were performed in all the 110 cases.The breast imaging report and data system (BI-RADS)classification and breast density classification were evaluated using the 5th edition of BI-RADS. In our study, BI-RADS 4B, 4C, and 5 were regarded to be in agreement with the pathologic findings, BI-RADS 1,2,3,and 4A were considered to be negative.BI-RADS c and d were classified as dense breasts, BI-RADS a and b were classified as fatty breasts.The imaging findings of the non-calcified ductal carcinoma in situ were evaluated.The differences in the detection rate and the diagnostic accuracy among the DBT,DM and US in all cases and in different breast density were compared using χ2 test. Results The detection rates of DBT,DM,and US for non-calcified DCIS in all cases were 84.5%(93/110),70.9%(78/110),95.5% (105/110).Pairwise comparisons among the three techniques showed statistically significant difference(P<0.05). The diagnostic accuracy of DBT, DM, and US were 70.0% (77/110), 44.5% (49/110), and 69.1% (76/110),respectively.The diagnostic accuracy of DBT and US were significantly higher than that of DM(P<0.01). Of the 110 patients, 89 patients were classified as dense breasts and non-dense breasts in the remaining 21 patients.The detection rates of DBT,DM,and US for non-calcified DCIS in dense breasts were 82.0%(73/89),65.2%(58/89),and 96.6%(86/89).Pairwise comparisons among the three techniques showed statistically significant difference(P<0.01).The diagnostic accuracy of DBT,DM,and US for non-calcified DCIS in dense breast were 65.2% (58/89), 38.2% (34/89) and 66.3% (59/89), respectively.The diagnostic accuracy of DBT and US were significantly higher than that of DM in dense breast(P<0.01).The detection rate and diagnostic accuracy for DBT,DM,and US in non-dense breasts were not statistically different(P>0.05).By DBT and DM,most cases of non-calcified DCIS presented as a mass lesion with an irregular shape, indistinct margin,and isodense composition.Conclusion US is more advantageous to the detection of the non-calcified DCIS and the non-calcified DCIS in the dense breast.

Objective To investigate the results and complications of hemispherotomy for drug resistant hemispheric epilepsy.Methods The authors reviewed 5 patients who were diagnosed as drug resistant hemispheric epilepsy and operated in the neurosurgery department of Peking Union Medical College Hospital from 2011 to 2013.All the 5 patients were underwent hemispherotomy after carefully multidisciplinary pre-operation evaluation.Results All patients tolerated the procedure well and the postoperative course was smooth.All the 5 patients didn`t have seizure in the period of following up of 46 to 69 months.Conclusions According to literatures and the authors` experience, hemispherotomy is as safe and efficient for hemispheric epilepsy as hemispherectomy.It is most important advance for hemispherectomy.The procedure of hemispherotomy is complex but not very difficult, illustrating a good prospect of application and extension.

OBJECTIVE To investigate the rescue and treatment of critical children with tracheobronchial foreign body. METHODS From June 2011 to June 2015,there were 2489 children with tracheobronchial foreign bodies treated in Children's Hospital of HeBei Province, among which 11critical children who were rescued as soon as they came to the hospital. The clinical data of the 11critical children were analyzed. RESULTS All the 11 critical cases endured dyspnea of third degree or more severe and presented severe hypoxia, in which 2 children had been performed tracheal intubation before they came to the hospital and 1 child even showed the symptom of respiratory and cardiac arrest. Among these critical cases, the foreign body was removed directly without anesthesia in 1 child. The other 2 children with severe pneumothorax, mediastinal emphysema and subcutaneous emphysema in neck and chest area were treated by excision and drainage of emphysema firstly, and then the foreign bodies were extracted through bronchoscope after general anesthesia. The another 8 children were performed operations of extraction of bronchial foreign body and then the foreign bodies were taken out. All the 11 critical children were rescued successfully and no death cases happened. CONCLUSION Rapid diagnosis and rapid removal of foreign bodies is the key to save the lives of critical children with tracheobronchial foreign bodies.

Objective To analyze the epidemiological characteristic of New Delhi metallo-β-lactamase (NDM)-1-positive bacteria in China and explore its mechanism of drug-resistance.Methods The published papers from 2010 to 2015 about domestic blaNDM-1 were collected and analyzed according to the bacteria species,regional distribution,NDM-1-positive bacteria,infectious source,drug-resistance spectra and transfer mechanism.Results NDM-1-positive bacteria were isola-ted from 25 provinces (municipalities)in China,Guangdong Province was predominant and accounted for 39.49%(P <0.05).The number of Klebsiella pneumoniae and Enterobacter cloacae were predominant in the NDM-1-positive bacteria (P <0.05).Number of patients under the age of ten and among 60 -80 years which infected by NDM-1-positive bacteria were predominant of all the reported patients(P <0.05).The most kinds of samples and diseases were sputum sample (P <0.05)and pulmonary diseases(P <0.05).NDM-1-positive bacteria which found in China had the lowest resistance to amikacin and tigecycline accounted for 7.69% and 2.33%(P <0.05).Conclusion Antibiotic resistant bacteria with NDM-1 resistant gene have become a global public health problem,and significant difference among age,districts and sources,which need active surveillance and more studies to find how it happens and epidemic in the future.

Objective To analyze the distribution and drug susceptibility of the pathogenic bacteria in the airway secretions in children with tracheobronchial foreign bodies so as to assist physicians in clinical prescription.Methods Sputum specimens of 1 125 children with tracheobronchial foreign bodies were collected in removal of the foreign bodies by rigid bronchoscope,and the drug susceptibility test was performed.Results Pathogenic bacteria were detected in 218 (19.4％) of 1 125 sputum specimens.Among the pathogenic bacteria,126 (57.79％) strains were gram-negative bacilli,consisting of 76 (34.86％) strains of Haemophilus influenzae,10 (4.59％) strains of Escherichia coli,7 (3.21％) strains of Sewer enterobacter,7 (3.21％) strains of Pseudomonas aeruginosa,and 6 (2.75％) strains of Klebsiella bacillus; and 92 (42.21％) strains were gram-positive bacilli,consisting of 80 (36.69％) strains of Streptococcus pneumonia and 10(4.59％)strains of Escherichia coli.Most of detected gram-negative bacilli were highly sensitive to cefepime,ceftazidine,imipenem and amikacin,no strains were resistant to meropenem and ciprofloxacin.None of the detected gram-positive bacilli were resistant to cefepime,vancomycin,levofloxacin and teicoplanin.Conclusions The Haemophilus influenzae of gram-negative bacilli and the Streptococcus pneumonia of gram-positive bacilli are the main pathogenic bacteria existing in the airway secretions of children with tracheobronchial foreign bodies.The Haemophilus influenzae were highly sensitive to cephalosporin,imipenem and amikacin,and the Streptococcus pneumonia to cefepime,vancomycin,levofloxacin and teicoplanin.