Objective To improve understanding of the clinical manifestations, diagnosis and treatment of childhood Kasabach-Merritt phenomenon (KMP). Methods The clinical data of 13 patients admitted for KMP to XXX from January 2010 to January 2016 was retrospectively analyzed, with a review of relevant literature. Results The patients were 10 males and 3 females. The age of presentation varied from newborn to 5 months. 12 patients had cutaneous manifestations, like petechiae, ecchymosis, jaundice, skin masses, etc, 1 patient had pleural effusion. The location of lesions varied. The laboratory hallmark consists of profound thrombocytopenia and hypofibrinogenemia with elevated D-dimers. The median time from initial presentation to diagnosis was 60 days. After approaches like surgery, corticosteroids, propranolol, interferon, sirolimus, etc, 10 patients got remission while 3 patients died. 6 patients treated with sirolimushad complete response. Conclusions KMP is characterized with vascular tumor, severe thrombocytopenia and consumptive coagulopathy. Clinically, KMP often presents with early-onset and delay in diagnosis. Surgery is an effective approach for KMP. Sirolimus appears to be a promising treatment for KMP.

Although immune checkpoint inhibitors (ICIs) have great breakthrough in cancer treatment in recent years, most patients have not benefited from it on account of immune microenvironment. Studies have shown that tryptophan metabolism is not only involved in the formation of tumor immunosuppressive microenvironment but also plays an important role in the therapeutic application of ICIs. At present, inhibiting the kynurenine pathway of tryptophan metabolism is now in various stages of clinical trials, while the other two metabolic pathways, 5-HT and the indole pathway, also have aroused wide concern. This article reviews the latest developments in this field.

Objective : o evaluate the association between Crohn's disease (CD) and frailty using a Mendelian randomization (MR) approach, so as to provide the evidence for prevention and control strategies. Methods: Genetic association data for CD were collected through the International Inflammatory Bowel Disease Genetics Consortium, with 20 883 samples and 12 276 506 single nucleotide polymorphism (SNP), and genetic association data for frailty were collected through a meta-analysis including 175 226 samples and 7 589 717 SNPs. A forward MR analysis was performed using the inverse-variance weighted (IVW) method with 37 CD-associated SNPs as instrumental variables, and frailty as the study outcome, and a reverse MR analysis was performed with 13 frailty-associated SNPs as instrumental variables and CD as the study outcome. The heterogeneity was assessed using the Cochran's Q test, and the horizontal pleiotropy was assessed using the MR-PRESSO global test and MR-Egger regression. In addition, the robustness of the results was verified with the leave-one-out. Results: Forward MR analysis results showed that patients with genetically predicted CD had an increased risk of frailty index relative to those without CD (β=0.018, 95%CI: 0.011-0.026, P<0.05). Cochran's Q test detected no heterogeneity (P>0.05), and neither the MR-PRESSO test nor the MR-Egger regression revealed horizontal pleiotropy of instrumental variables (both P>0.05). Leave-one-out analysis showed robustness of the MR analysis results. Reverse MR analysis showed no association between frailty index and the risk of CD (OR=0.740, 95%CI: 0.206-2.661, P>0.05). Conclusions Genetically predicted CD is associated with an increased risk of frailty. It is suggested that screening and prevention of frailty should be reinforced among CD patients.

Objective : To examine the causal relationship between ulcerative colitis (UC) and pancreatitis, to provide basis for early screening of pancreatitis among UC patients. Methods: Genomic data of UC were obtained from 47 745 European individuals pooled by the International Inflammatory Bowel Disease Genetics Consortium, including 156 116 single nucleotide polymorphism (SNP), and genomic data of pancreatitis were obtained from 198 166 European individuals pooled from FinnGen, including 16 380 428 SNPs. Mendelian randomization (MR) analysis was performed using the inverse variance weighted (IVW) method with 72 UC-associated SNPs as instrumental variables and pancreatitis as the study outcome. The heterogeneity was assessed using Cochran Q test, the horizontal pleiotropy was assessed using MR-Egger regression, MR-PRESSO was performed with the exclusion of outliers, and effect of individual SNP on the results was tested with the leave-one-out method. Results: MR analysis results showed that patients with genetically predicted UC had an increased risk of pancreatitis relative to those without UC (OR=1.076, 95%CI: 1.019-1.136, P<0.05). Cochran Q test showed no heterogeneity (P>0.05), and MR-Egger regression did not reveal horizontal pleiotropy of instrumental variables (P>0.05). The MR analysis results were robust after removing SNP one by one. Conclusions Genetically predicted UC is associated with an increased risk of pancreatitis. The screening for pancreatitis risk should be enhanced in patients with UC.

Objective To observe the clinical safety and efficacy of foscarnet prophylaxis and pre-emptive therapy for cytomegalovirus (CMV) infection in allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods Ninety-six patients undergoing allo-HSCT from October 2014 to December 2016 were retrospectively analyzed. Plasma CMV-DNA was monitored with real-time quantitative polymerase chain reaction (RQ-PCR) from beginning to 180 days after transplantation. Foscarnet was used not only for prophylaxis but also for first-line pre-emptive therapy when plasma CMV-DNA turned to positive. Foscarnet was given 60 mg·kg-1·d-1 and 120 mg·kg-1·d-1 respectively in prevention and pre-emptive therapy. Incidences of CMV infection and CMV disease were observed, influencing factors on CMV in faction and the efficacy and safety of foscarnet prophylaxis were analyzed, and survival of patients treated by all-HSCT was evaluated. Results Of the total 96 patients, 42 cases (43.8%) had CMV infection with the median time of 42 days after allo-HSCT. CMV-DNA became negative in 36 patients (85.7%, 36/42) after pre-emptive therapy. Six patients (14.3 %, 6/42) developed CMV disease, including 5 patients with CMV negative and 1 patient died for CMV pneumonia. Haploidentical donor and grade Ⅱ-Ⅳacute graft versus host disease (GVHD) were the risk factors for CMV reactivation (χ2 = 3.834, P< 0.05; χ2 = 16.807, P< 0.001). The side effects of foscarnet prophylaxis were mild without hematologic toxicities. 12 patients (28.6 %) died in 42 patients with CMV infection, and 6 patients (11.1 %) died in 54 patients without CMV infection. The difference of survival rates between both groups was not statistically significant. Conclusion Foscarnet is an effective agent for prophylaxis and pre-emptive therapy in CMV infection after allo-HSCT with mild adverse reactions, especially for patients following with hematopoietic recovering.

Background/Aims: This study aimed to explore the effect of gut microbiota-regulated Kupffer cells (KCs) on colorectal cancer (CRC) liver metastasis. Methods: A series of in vivo and in vitro researches were showed to demonstrate the gut microbiota and its possible mechanism in CRC liver metastasis. Results: Fewer liver metastases were identified in the ampicillin-streptomycin-colistin and colistin groups. Increased proportions of Parabacteroides goldsteinii, Bacteroides vulgatus, Bacteroides thetaiotaomicron, and Bacteroides uniforms were observed in the colistin group. The significant expansion of KCs was identified in the ampicillin-streptomycin-colistin and colistin groups. B.vulgatus levels were positively correlated with KC levels. More liver metastases were observed in the vancomycin group. An increased abundance of Parabacteroides distasonis and Proteus mirabilis and an obvious reduction of KCs were noted in the vancomycin group. P. mirabilis levels were negatively related to KC levels. The number of liver metastatic nodules was increased in the P. mirabilis group and decreased in the B. vulgatus group. The number of KCs decreased in the P. mirabilis group and increased in the B. vulgatus group. In vitro, as P. mirabilis or B. vulgatus doses increased, there was an opposite effect on KC proliferation in dose- and time-dependent manners. P. mirabilis induced CT26 cell migration by controlling KC proliferation, whereas B. vulgatus prevented this migration. Conclusions An increased abundance of P. mirabilis and decreased amount of B. vulgatus play key roles in CRC liver metastasis, which might be related to KC reductions in the liver.

Objective To analyze the real experiences of patients with inflammatory bowel disease(IBD)during dietary restrictions,providing references for healthcare personnel to guide patients in standardizing dietary restriction behaviors.Methods Purposeful sampling was employed to select 14 patients with IBD who were treated at a tertiary A hospital in Shanghai between October 2022 and February 2023 for semi-structured in-depth interviews.Data were analyzed using the Colaizzi's 7-step method in phenomenological research.Results 4 themes and 13 sub-themes were extracted.Theme 1:facing multiple physiological challenges(hunger,nutritional imbalance,fatigue and muscle atrophy).Theme 2:experiencing negative psychological disturbances(craving and struggle for gourmet food,diminished zest for life and increased anxiety,feeling embarrassed during social activities).Theme 3:adopting various coping strategies(self-adjustment and adaptation to dietary restrictions,satisfying oral desires through various avenues,seeking knowledge and guidance on nutrition).Theme 4:gaining more growth and support(improved symptom and quality of life,enhanced awareness of dietary health,improved ability to manage diet,receiving support from peers and family).Conclusion The experience of dietary restrictions in IBD patients is complex and varied.Nursing staff should prioritize nutritional risk screening for IBD patients,pay attention to their mental health,provide patients with scientific and personalized dietary guidance,and strengthen social and family support to assist patients in better self-management of their diet.

Objective To analyze the clinical characteristics,imaging manifestations and prognoses of anti-GABAB receptor antibodies encephalitis.Methods The clinical manifestations,laboratory findings and radiological data of 13 patients with anti-GABAB receptor encephalitis,admitted to our hospital from September 2015 to March 2017,were retrospectively analyzed.Modified Rankin scale (mRs) was performed to evaluate the prognoses (good prognosis:mRs scores ＜ 2;poor prognosis mRs scores≥3).Results These 13 patients had an average age of 58 years (ranged 49-76 years) with a male to female ratio of 12:1.The major clinical features,including epileptic seizure,were found in 12 patients,psychiatric symptoms in 11 patients,cognitive disorder in 7 patients,and disturbance of consciousness in 4 patients.Brain MR imaging showed abnormal signal in 5 patients:4 were located in the hippocampus and amygdaloid,and one in the pons and left temporal lobe.Five patients showed abnormalities in PET-CT,including 4 with temporal hypermetabolism and 1 with cortical hypometabolism.Chest CT showed lung occupying lesions in 4 patients,of which 2 patients were diagnosed as having small cell lung cancer (SCLC) by pathological examination.Ten patients received immunomodulatory therapy,and three were with supportive care.After the average of 8 months of follow-up,7 patients had good prognosis,5 patients had poor prognosis and one patient lost of follow up.Conclusions Anti-GABAB receptor encephalitis frequently occurs in elderly male subjects and the main characteristic includes prominent refractory epilepsy and shows neurological improvement on immunotherapy.It can accompany by SCLC and have a relatively poor prognosis.

Objective:To explore the rapid evaluation of the early pathogen of severe Chlamydophila psittaci pneumonia by bedside diagnostic bronchoscopy, so as to start effective anti-infection treatment before the results of macrogenome next generation sequencing (mNGS) test. Methods:The clinical data of three patients with severe Chlamydophila psittaci pneumonia who were successfully treated in the First Affiliated Hospital of Xinjiang Medical University, the First People's Hospital of Aksu District, and the First Division Hospital of Xinjiang Production and Construction Corps from October 2020 to June 2021 were retrospectively analyzed, including the rapid assessment of early pathogens by bedside diagnostic bronchoscopy and the use of antibiotics to start anti-infection treatment. These patients were successfully treated. Results:The three patients were male, aged 63, 45 and 58 years old, respectively. Before the onset of the penumonia, they had a clear medical history of bird exposure. The clinical manifestations mainly included fever, dry cough, shortness of breath and dyspnea. One case had abdominal pain and lethargy. The results of laboratory examination indicated that the peripheral blood white blood cell count (WBC) of two patients were high [(10.2-11.9)×10 9/L], the percentage of neutrophils increased (85.2%-94.6%) and the percentage of lymphocytes decreased (3.2%-7.7%) in all 3 patients after admission to hospital and entering into intensive care unit (ICU). The procalcitonin (PCT) of 3 patients increased after admission, and still increased when entering ICU (0.3-4.8 ng/L), so did C-reactive protein (CRP, 58.0-162.0 mg/L) and erythrocyte sedimentation rate (ESR, 36.0-90.0 mm/1 h). After admission, serum alanine transaminase (ALT) increased in 2 cases (136.7 U/L, 220.5 U/L), so did aspartate transaminase (AST) in 2 cases (249.6 U/L, 164.2 U/L). ALT (162.2-267.9 U/L) and AST (189.8-223.2 U/L) increased in 3 patients when they entered ICU. The level of serum creatinine (SCr) of 3 patients were normal after admission and entering ICU. The chest computed tomography (CT) findings of 3 patients were acute interstitial pneumonia, bronchopneumonia and lung consolidation, of which 2 cases were accompanied by a small amount of pleural effusion, and 1 case was accompanied by more regular small air sacs. Multiple lung lobes were involved, but mainly one lung lobe. The oxygenation index (PaO 2/FiO 2) of the 3 patients admitting to ICU were 100.0, 57.5 and 105.4 mmHg (1 mmHg ≈ 0.133 kPa), respectively, which met with the diagnostic criteria of moderate and severe acute respiratory distress syndrome (ARDS). All three patients received endotracheal intubation and mechanical ventilation. Under the bedside bronchoscope, the bronchial mucosa of 3 patients were obviously congested and edematous, without purulent secretion, and there was 1 case with mucosal hemorrhage. Three patients underwent bedside diagnostic bronchoscopy, and the evaluation result of the pathogen was that it might be atypical pathogen infection, so they were given moxifloxacin, cisromet and doxycycline intravenously, respectively, and combined with carbapenem antibiotics intravenously. After 3 days, the detection results of mNGS in bronchoalveolar lavage fluid (BALF) showed that only Chlamydia psittaci was infected. At this time, the condition was significantly improved, and PaO 2/FiO 2 was significantly increased. Therefore, the antibiotic treatment scheme remained unchanged, and mNGS only served to verify the initial diagnosis. Two patients were extubated on the 7th and 12th day of admission to the ICU, respectively, while one patient was extubated on the 16th day of admission to the ICU due to nosocomial infection. All 3 patients were transferred to the respiratory ward after the condition was stable. Conclusion:The bedside diagnostic bronchoscopy based on clinical characteristics is conducive to not only the rapid assessment of the early pathogens of severe Chlamydia psittaci pneumonia, but also effective anti-infection treatment before the returning of mNGS test results, which can make up for the lag and uncertainty of the mNGS test results.

Background: and Purpose To examine the clinical and safety outcomes after endovascular treatment (EVT) for acute basilar artery occlusion (BAO) with different anesthetic modalities. Methods: This was a retrospective analysis using data from the Endovascular Treatment for Acute Basilar Artery Occlusion (ATTENTION) registry. Patients were divided into two groups defined by anesthetic modality performed during EVT: general anesthesia (GA) or non-general anesthesia (non-GA). The association between anesthetic management and clinical outcomes was evaluated in a propensity score matched (PSM) cohort and an inverse probability of treatment weighting (IPTW) cohort to adjust for imbalances between the two groups. Results: Our analytic sample included 1,672 patients from 48 centers. The anesthetic modality was GA in 769 (46.0%) and non-GA in 903 (54.0%) patients. In our primary analysis with the PSM-based cohort, non-GA was comparable to GA concerning the primary outcome (adjusted common odds ratio [acOR], 1.01; 95% confidence interval [CI], 0.82 to 1.25; P=0.91). Mortality at 90 days was 38.4% in the GA group and 35.8% in the non-GA group (adjusted risk ratio, 0.95; 95% CI, 0.83 to 1.08; P=0.44). In our secondary analysis with the IPTW-based cohort, the anesthetic modality was significantly associated with the distribution of modified Rankin Scale at 90 days (acOR: 1.45 [95% CI: 1.20 to 1.75]). Conclusion In this nationally-representative observational study, acute ischemic stroke patients due to BAO undergoing EVT without GA had similar clinical and safety outcomes compared with patients treated with GA. These findings provide the basis for large-scale randomized controlled trials to test whether anesthetic management provides meaningful clinical effects for patients undergoing EVT.