UNASSIGNED: Anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis is a rare autoimmune encephalitis, and clinical and experimental information regarding this disease is limited. We conducted this study to comprehensively describe the clinical characteristics, ancillary test results, neuroimaging results, and treatment response in a group of Chinese patients with anti-DPPX encephalitis for better understanding this disease.
UNASSIGNED: We recruited 14 patients who tested positive for anti-DPPX antibodies in the serum and/or cerebrospinal fluid from 11 medical centers between March 2021 and June 2023. This retrospective study evaluated data on symptoms, autoantibody test, auxiliary examinations, treatments, and outcomes.
UNASSIGNED: The average age at diagnosis was 45.93 ± 4.62 years (range: 11-72 years), and 9 of the 14 patients were males. The main symptoms included cognitive impairment (50.0%, 7/14), central nervous system hyperexcitability (42.9%, 6/14), gastrointestinal dysfunction (35.7%, 5/14), and psychiatric disorders (35.7%, 5/14). Notably, we discovered specific findings on 18F-fluorodeoxyglucose positron-emission tomography (PET)/magnetic resonance imaging in two patients. Co-existing autoantibodies were identified in two patients. Parainfection was identified in four patients. One patient had other autoimmune diseases, and one had tumor. Eleven patients received immunotherapy and most patients improved at discharge. Surprisingly, three male patients but no female patients relapsed during the 6 months of follow-up.
UNASSIGNED: The development and outcome of anti-DPPX encephalitis are variable. Male patients were predominant in our cohort. The most common symptoms were the classical triad of prodromal gastrointestinal dysfunction, cognitive and mental disorders, and central nervous system hyperexcitability. Infections, immune dysregulation, and tumors may be important etiologies. Long-term monitoring of disease development should be done in male patients. Overall, our results highlight novel clinical characteristics of anti-DPPX encephalitis.

OBJECTIVE: To explore the influence of postoperative Hirschsprung-associated enterocolitis (post-HAEC) on long-term outcomes and to identify risk factors of post-HAEC.
METHODS: The medical records of 304 eligible patients diagnosed with Hirschsprung\'s disease (HSCR) were reviewed. We analyzed the clinical characteristics of post-HAEC and its influence on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were identified separately.
RESULTS: The overall incidence of post-HAEC was 29.9% (91/304). We categorized early HAEC as occurring within postoperative 3 months (n = 39) and recurrent HAEC as occurring ≥ 3 episodes within postoperative 6 months (n = 25). Patients with early HAEC were more likely to experience worse nutritional status, defecation function, and quality of life compared to those with late or no episodes (P < 0.05). Similarly, the adverse influences of recurrent HAEC on these outcomes were also significant (P < 0.05). The risk factors for early HAEC included preoperative undernutrition, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days. For recurrent HAEC, risk factors were preoperative malnutrition, non-parental caregivers, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days.
CONCLUSIONS: Classification of post-HAEC based on the first episode time and frequency was necessary. The earlier or more frequent episodes of post-HAEC have detrimental influences on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were different.

OBJECTIVE: To explore the clinical manifestations, endoscopic findings, histopathological changes, treatment, and prognosis of eosinophilic gastrointestinal disease (EGID) in children, with the aim of enhancing awareness among pediatricians about this condition.
METHODS: Data of 267 children with EGID were prospectively collected from January 2019 to July 2022 at Jiangxi Children\'s Hospital, Hunan Children\'s Hospital, and Henan Children\'s Hospital. The age of onset, symptoms, physical signs, laboratory examination results, endoscopic findings, histopathological changes, and treatment outcomes were observed.
RESULTS: Among the 267 children with EGID, the majority had mild (164 cases, 61.4%) or moderate (96 cases, 35.6%) clinical severity. The disease occurred at any age, with a higher prevalence observed in school-age children (178 cases). The main symptoms in infants were vomiting and hematemesis, while in toddlers, vomiting and bloody stools were prominent. Abdominal pain and vomiting were the primary symptoms in preschool and school-age children. Nearly half (49.4%) of the affected children showed elevated platelet counts on hematological examination, but there was no significant difference in platelet counts among children with mild, moderate, and severe EGID (P>0.05). Endoscopic findings in EGID children did not reveal significant specificity, and histopathological examination showed no specific structural damage. Among them, 85.0% (227 cases) received acid suppression therapy, 34.5% (92 cases) practiced dietary avoidance, 20.9% (56 cases) received anti-allergic medication, and a small proportion (24 cases, 9.0%) were treated with prednisone. Clinical symptoms were relieved in all patients after treatment, but three cases with peptic ulcers experienced recurrence after drug discontinuation.
CONCLUSIONS: Mild and moderate EGID are more common in children, with no specific endoscopic findings. Dietary avoidance, acid suppression therapy, and anti-allergic medication are the main treatment methods. The prognosis of EGID is generally favorable in children.
目的: 探讨儿童嗜酸粒细胞性胃肠道疾病（eosinophilic gastrointestinal disease, EGID）的临床表现、内镜下改变、病理组织学改变、治疗及预后特点，以提高儿科医师对该疾病的认识。方法: 前瞻性收集2019年1月—2022年7月江西省儿童医院、湖南省儿童医院、河南省儿童医院确诊的267例EGID患儿资料，观察其发病年龄、症状、体征、实验室检查、内镜下改变、病理组织学改变及治疗的预后情况。结果: 267例EGID患儿中，临床分度以轻度（164例，61.4%）和中度（96例，35.6%）为主。各年龄均可发病，以学龄期儿童（178例）为主。婴儿期主要表现为呕吐、呕血，幼儿期主要表现为呕吐、便血，学龄前期和学龄期主要表现为腹痛、呕吐。血常规示近一半（49.4%）患儿出现血小板升高，但轻、中、重度EGID患儿血小板计数比较差异无统计学意义（P>0.05）。EGID患儿消化道内镜下无显著特异性改变，组织病理亦无特异性结构损伤。其中85.0%（227例）予以抑酸治疗，34.5%（92例）予以饮食回避，20.9%（56例）予以抗过敏药物治疗，少部分（24例，9.0%）使用泼尼松治疗。治疗后患儿临床症状均得到缓解，3例以消化性溃疡为表现者停药后症状出现反复。结论: 儿童EGID以轻、中度多见，内镜下改变无明显特异性，饮食回避及抑酸、抗过敏药物为主要治疗方法，预后良好。.

OBJECTIVE: To analyze posttraumatic glaucoma regarding its demographics, presentations, different causes, surgical modalities, and hospitalization burden among patients in southern China.
METHODS: This retrospective study investigated all individuals with posttraumatic glaucoma admitted to the Zhongshan Ophthalmic Center of Sun Yat-Sen University from January 2012 through December 2021.
RESULTS: Out of 2211 cases, 64.82% had closed globe injury (CGI), 28.22% had open globe injury (OGI), and 6.96% had chemical injury (CI). The mean age of all patients was 44.45 ± 19.45 years old. Males (83.36%), rural patients (56.17%), and farmers (27.14%) predominantly had posttraumatic glaucoma. The most common external injury mechanism was blunt objects (37.82%). Compared with the other two groups, the majority of surgical modalities were cataract extraction (27.12%) in the CGI group, combined anterior-posterior surgery (34.79%) in the OGI group, and cyclocryotherapy/cyclophotoagulation (49.1%) in the CI group. The CI group had higher times of hospitalization (3.542 ± 0.242) and hospitalization duration (8.373 ± 0.743 days), whereas the OGI group had more operation expense ($ 1476.729 ± 11.047) and medical consumables expense per head ($ 962.578 ± 25.801).
CONCLUSIONS: Blunt injury, males, adults, farmers, and rural patients were high-risk factors for posttraumatic glaucoma. Chemical-induced glaucoma management requires a longer hospitalization period, while OGI requires more medical expenditure. This knowledge provides a new reference for clinicians to accurately diagnose and intervene in posttraumatic glaucoma. It also suggests that more education and long-term surveillance are needed regarding the presence of glaucoma after ocular trauma.

OBJECTIVE: To elucidate the sex-specific differences in demographic features, clinical characteristics, and quality of life in Chinese patients with psoriatic arthritis (PsA).
METHODS: A total of 1,074 patients with PsA registered between December 2018 and June 2021 from the Chinese REgistry of Psoriatic ARthritis (CREPAR) cohort were selected. The baseline data on demographics, clinical characteristics, commonly used laboratory tests, comorbidities, and quality of life assessments were collected for this cross-sectional analysis.
RESULTS: A total of 1,074 patients were included in this study, 585 (54.47%) of them were male and 489 (45.53%) were female. The age at PsA onset in male patients was earlier than that in female patients (38.10 ± 12.79 vs 40.37 ± 13.41, p = 0.005). For clinical characteristics, male patients presented with higher rates of axial involvement (43.89% vs 37.74%, p = 0.044) and nail involvement (66.15% vs 58.08%, p = 0.006), while female patients presented with higher rates of peripheral arthritis (89.57% vs 83.93%, p = 0.007). For laboratory tests, men presented with a higher percentage of HLA-B27 positivity than women (24.65% vs 16.70%, p = 0.002) and had higher levels of CRP (median 9.70 vs 5.65, p < 0.001). Regarding disease assessment indices, male patients scored higher in PASI and BASFI (median 5.00 vs 3.00, p = 0.007 and 1.80 vs 1.40, p = 0.012, respectively). No sex difference was found in rates of achieving remission. Factors associated with disease remission were also analyzed in both sexes.
CONCLUSIONS: Demographic and clinical characteristics tend to vary between male and female patients with PsA. Male patients reported more functional limitations in daily life. Key Points • The demographic and clinical features vary greatly between male and female patients with PsA. • Male patients reported more functional burden in daily life as measured by BASFI.

BACKGROUND: Serum tumor markers (STM), extensively used for the diagnosis, monitoring and prognostic assessment of tumors, can be increased in some non-malignant lung diseases. To date, there is a paucity of studies regarding the clinical characteristics of non-cystic fibrosis bronchiectasis patients with positive STMs.
OBJECTIVE: To investigate the clinical characteristics and indicators of bronchiectasis with positive STMs.
METHODS: The clinical data of 377 bronchiectasis patients was retrospectively collected from January 2017 to December 2019 from Beijing Chaoyang Hospital. Patients were divided into the STM negative group, the single STM positive group and the ≥2 STMs positive group according to the number of the positive STMs. The clinical characteristics are described and compared separately. The multivariate logistic regression analysis model was used to investigate the indicators regarding positive STMs.
RESULTS: Patients in the ≥2 STMs positive group were older (P = 0.015), had higher mMRC scores (P < 0.001) and developed higher fever (P = 0.027). Additionally, these patients also had lower Albumin/Globulin Ratio (A/G), albumin (ALB), prealbumin (PAB) (P < 0.001, P < 0.001, P < 0.001, respectively) and higher CRP, ESR and Fbg (P < 0.001, P < 0.001 and P < 0.001, respectively). Age (OR 1.022, 95%CI 1.003-1.042; P = 0.026) and the number of affected lobes (OR 1.443, 95%CI 1.233-1.690; P < 0.001) were independently associated with one and ≥ 2 positive STMs in bronchiectasis patients.
CONCLUSIONS: The ≥2 positive STMs are associated with a higher inflammation status and severer radiologic manifestations in bronchiectasis patients.