Objective To analyze and compare the auxiliary value and significance of γ-interferon release assay (IGRA) in the diagnosis of tuberculosis. Methods A retrospective analysis was conducted to collect the test results of 462 suspected tuberculosis patients who underwent IGRA detection in the outpatient department of Tianjin Tuberculosis Control Center from January 2020 to December 2021. According to the diagnostic results, they were divided into a tuberculosis group of 229 cases (203 cases of pulmonary tuberculosis and 26 cases of extrapulmonary tuberculosis) and a non-tuberculosis group of 233 cases. The auxiliary diagnostic value of IGRA for tuberculosis was analyzed and compared with two methods of Mycobacterium tuberculosis culture and Xpert MTB/RIF. Results The positive rates of IGRA, Mycobacterium tuberculosis culture, and Xpert MTB/RIF in TB patients were 76.42%, 29.26% and 40.62%, respectively, compared with the non-TB group (38.20%, 0.00%, 0.00%), with statistically significant differences (P<0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of IGRA alone in the detection of tuberculosis were 76.42%, 61.80%, 69.29%, and 72.73% respectively, those of Mycobacterium tuberculosis culture were 29.26%, 98.28%, 94.37%, and 63.43%, and those of Xpert MTB/RIF were 40.60%, 100%, 100%, and 63.14%. The positive rates of IGRA were 76.85% and 73.08% in patients with pulmonary tuberculosis and extrapulmonary tuberculosis, respectively, with no statistical significance (P>0.05). The positive rates of IGRA in bacterial positive patients and non-tuberculosis patients were 79.34% and 38.20%, respectively, with statistical significance (χ2=54.526, P<0.001). The positive rates of IGRA in patients with and without tuberculosis were 73.15% and 38.20%, respectively, with significant difference (χ2=36.456, P<0.001). Conclusions IGRA has a relatively high sensitivity in the diagnosis of tuberculosis and also has certain advantages in the screening of extrapulmonary tuberculosis and mycobacterium-negative It provides important reference basis for the clinical diagnosis of tuberculosis.

Psoriasis is an autoimmune skin disease in which dendritic cells (DCs) trigger the progression of psoriasis by complex interactions with keratinocytes and other immune cells. In the present study, we aimed to load celastrol, an anti-inflammatory ingredient isolated from Chinese herbs, on mannosylated liposomes to enhance DC uptake as well as to induce DC tolerance in an imiquimod-induced psoriasis-like mouse model. Mannose was grafted onto liposomes to target mannose receptors on DCs. The results demonstrated that compared with unmodified liposomes, DCs preferred to take up more fluorescence-labeled mannosylated liposomes. After loading celastrol into mannose-modified liposomes, they effectively inhibited the expression of maturation markers, including CD80, CD86 and MHC-II, on DCs both in vitro and in vivo. Additionally, after intradermal injection with a microneedle, celastrol-loaded mannose-modified liposomes (CEL-MAN-LPs) achieved a superior therapeutic effect compared with free drug and celastrol-loaded unmodified liposomes in the psoriasis mouse model in terms of the psoriasis area and severity index, histology evaluation, spleen weight, and expression of inflammatory cytokines. In conclusion, our results clearly revealed that CEL-MAN-LPs was an effective formulation for psoriasis treatment and suggested that this treatment has the potential to be applied to other inflammatory diseases triggered by activated DCs.

Psoriasis is an autoimmune inflammatory disease, where dendritic cells (DCs) play an important role in its pathogenesis. In our previous work, we have demonstrated that topical delivery of curcumin-loaded poly (lactic-

Objective:To analyze the clinical characteristics and diagnosis and treatment of children with Kasabach-Merritt phenomenon (KMP).Methods:A retrospective analysis was conducted on the clinical data and follow-up data of 8 patients diagnosed KMP in Beijing Children′s Hospital, Capital Medical University from January 2016 to January 2019.The clinical data included laboratory examination, diagnosis, treatment and prognosis.Results:Among the 8 children with KMP, 6 cases were male and 2 cases were female.The median onset age was 4 (0-17) months, 2 cases of neonatal onset.The median onset to the diagnosis time was 59 (34-140) days; 6 cases with bone destruction; 6 cases had misdiagnosis and mistreatment history, they were misdiagnosed as idiopathic thrombocytopenic purpura, Evans syndrome, abnormal bone and joint development; 4 cases were Kaposiform hemangioendothelioma; 8 cases were used alone or combined with the application of hormones, Sirolimus, and Vincristine, 7 patients underwent interventional therapy.All patients survived with a median follow-up period of 487 (112-1 033) days.Median time of platelet count returned to normal was 24.5 (7-60) days, and median time of fibrinogen returned to normal was 20 (7-30) days.Median time of D-dimer dropped to a normal was 105 (40-240) days.Conclusions:Children with concurrent platelet count and coagulation abnormalities should be considered with KMP.Doctors need to identify the potential visceral vascular lesions.Early diagnosis and treatment are important, which can improve the clinical prognosis of patients.

Objective:To provide clinical references for the diagnosis and treatment of hemophilic pseudotumor (HPT) in maxillofacial region.Methods:Fourteen cases of HPT in maxillofacial region from the Department of Stomatology, Beijing Children′s Hospital from Jan 2009 to Jan 2019 were collected. Two cases were lost for follow-up and 12 patient,all boys, were finally followed up and included in the study. The patients aged from 13 months to 10 years old. The medical history, clinic manefestitions and the features of the radiology examination were recorded. The patients were treated by using replacement treatment first. If the conservative treatment was not effective, the patients then received operation combined with pereoperation replacement thearapy. The patients were followed up for 13 months to 10 years.There were 11 cases of hemophilia A, and 1 case of hemophilia B. Two cases were severe type, the others (10/12) were mild and moderate types. Only 1 case was diagnosed as hemophilia initially. Nine cases (9/12) were misdiagnosed as malignant tumors, 1 case was misdiagnosed as osteomyelitis and 1 case was misdiagnosed as hemangioma. Only 3 cases had identified history of trauma before.Results:All cases were treated with replacement therapy first, among which 10 cases were effective, 8 cases were cured by conservative therapy, 1 case had residual soft tissue fistula after conservative treatment and 1 case recurrented after conservative treatment for 8 months. Two patients with poor efficacy to the replacement treatment were performed operations and finally were cured.Conclusions:The misdiagnosis rate of HPT in maxillofacial region was high. The conservative factor replacement therapy could achieve good results in most children and could be used as the preferred treatment. If the conservative treatment was not effective, the surgical treatment was also a safe option.

Objective:To understand the relationship between joint bleeding and joint disease in hemophilia children, and to provide a theoretical basis for clinical treatment and prognosis.Methods:The patients with severe hemophilia A between 1 and 7 years old and with relevant nodal bleeding records were selected.All the patients admitted in Beijing Children′s Hospital, Capital Medical University, and Chengdu New Century Women′s and Children′s Hospital since June 2016 to January 2017.All the joint bleeding of each child was taken as the study joint, and the joint bleeding was collected during the last 3 months.The joints were assessed by using ultrasound, X-ray, magnetic resonance imaging(MRI) and Hemophilia Joint Health Score (HJHS) scoring systems.The correlation analysis was conducted between the joint bleeding, ultrasound, X-ray, MRI and HJHS scores.The correlation analysis was conducted for baseline ultrasound, X-ray, MRI and HJHS scores.Results:(1) There were 18 patients enrolled.The mean age was (5.6±1.8) years old.There were 30 joints bleeding in the observation period in total, with the annul median joint bleeding times of 4 (4-16 times), and the annul median target joint bleeding times of 8 (4-16 times). (2) Joint bleeding times of was correlated with ultrasound and X-ray evaluation ( r=0.390, P=0.033; r=0.517, P=0.008), and not correlated with HJHS or MRI(all P>0.05). (3) There was significantly positive correlation among ultrasound, X-ray, HJHS and MRI [ r=0.815(ultrasound vs.X-ray), r=0.510(ultrasound vs.HJHS), r=0.812(ultrasound vs.MRI), r=0.666(X-ray vs.HJHS), r=0.911(X-ray vs.MRI), r=0.781(HJHS vs.MRI), all P<0.01]. (4) There was no correlation between times and assessment for joints whose ultrasound and /or MRI in joints with abnormal ultrasound and /or MRI evaluation( P>0.05). Conclusions:The results of joint bleeding and joint evaluation are inconsistent.Joint bleeding can not truly reflect the situation of joint diseases.The assessment of hemophilia should include comprehensive evaluation of joint structure, function, activity ability and other aspects to guide the treatment of haemophi-lia children.

Objective:To observe the pronunciation of consonants among children with developmental speech sound disorder and explore the correlation between mispronounced consonants and short-term memory so as to determine the pathogenesis of the disorder.Methods:Thirty-six children with developmental speech sound disorder and aged 4 to 13 years were evaluated. Their pronunciation of consonants at the phoneme and lexical levels was tested to record the error types and error rate. Twelve of the children were then randomly chosen to form a voice disorder group. Another 10 healthy counterparts constituted a control group. The short-term memory of both groups was assessed and any correlation between pronunciation and short-term memory was analyzed.Results:The children with a developmental speech sound disorder differed significantly from the controls in terms of the numbers of errors in articulating blade-alveolar, blade-palatal and velar consonants. On the phoneme level, the highest substitution error rate occurred when pronouncing lingua-palatal consonants (42.86%), followed by supradental consonants (32%). The highest distortion and non-acquisition error rates were with blade-palatal consonants (14%) and lingua-palatal consonants (9.5%). On the vocabulary level, the highest substitution, distortion, ellipsis and non-acquisition error rates appeared when pronouncing lingua-palatal and velar consonants, velar and blade-palatal consonants, supradental consonants as well as blade-palatal consonants. Significant differences were found between the phoneme and lexical levels in the substitution of supradental and blade-palatal consonants as well as in the ellipsis of blade-alveolar consonants. They were moderately associated with pronunciation level. There was, however, no significant difference in working memory span between the two groups, and no significant correlation was observed between working memory span and pronunciation level.Conclusion:The mispronunciation of consonants by children with developmental speech disorders is higher at the lexical than at the phoneme level. They mainly substitute lingua-palatal and velar consonants and elide supradental consonants, which may be related to short-term memory span.

Objective To explore the effects of early enteral nutrition (EN) combined with probiotics on intestinal flora and immune function in patients with severe ischemic stroke. Methods Sixty-nine severe ischemic stroke patients were admitted and continuously enrolled in Taizhou First People's Hospital from June 2017 to June 2018, and they were randomly divided into an EN combined with probiotics group (35 cases) and a simple EN group (34 cases). Early EN support was given to both groups and probiotics (Live Combined Bifidobacterium, Lactobacillus and Enterococcus capsules) was added to the EN combined with probiotics group, 0.42 g each time, 3 times a day for 14 days. The changes of serum inflammatory markers [hypersensitive C-reactive protein (hs-CRP), procalcitonin (PCT), tumor necrosis factor-α (TNF-α), interleukin-10 (IL-10)], intestinal flora (Bifidobacterium, Lactobacillus, Clostridium, Enterobacter, Enterococcus, Bacteroides), intestinal mucosal barrier [endotoxin, D-lactic acid, diamine oxidase (DAO), intestinal fatty acid binding protein (I-FABP) ], and immune indexes [immunoglobulins (IgA, IgG, IgM), human leukocyte DR antigen (HLA-DR)] were observed in two groups of patients after treatment. Results With the prolongation of time, Bifidobacteria, Lactobacilli, HLA-DR and IgA, IgG, IgM after EN in both groups all decreased first and then had a tendency of increase, all reaching the lowest value on the EN 3rd day and then gradually elevated arriving at the peak value on the EN 14th day, and the levels in EN combined with probiotics group were significantly higher than those in the simple EN group [Bifidobacterium (×107 cfu/g): 8.31±1.49 vs. 7.49±1.32, Lactobacillus (×107 cfu/g): 8.04±1.45 vs. 7.19 ±1.37, HLA-DR: (67.22±9.11)% vs. (61.21±9.69)%, IgA (mg/L): 170.34±40.13 vs. 149.54±38.76, IgG (g/L):4.88±0.88 vs. 4.31±0.86, IgM (mg/L): 879.47±100.82 vs. 821.52±97.75, all P < 0.05]. With the prolongation of time, the Clostridium, Enterobacter, Enterococcus, Bacteroides, hs-CRP, PCT, TNF-α, endotoxin, D-lactic acid, DAO, I-FABP after En in both groups all increased first and then had a tendency of decrease, reaching the highest level on the EN 3rd day, then gradually decreased arriving at the valley value on the EN 14th day, and the levels in the EN combined with probiotics group were significantly lower than those in the simple EN group [Clostridium (×107 cfu/g): 5.23±0.87 vs. 5.79±0.91, Enterobacter (×107 cfu/g): 7.45±1.21 vs. 8.62±1.32, Enterococcus (×107 cfu/g): 7.32±1.05 vs. 8.12±1.23, Bacteroides (×107 cfu/g): 9.16±1.35 vs. 9.87±1.42, hs-CRP (mg/L): 18.45±12.98 vs. 25.47±15.55, PCT (ng/L): 3.24±1.21 vs. 4.18±1.32, TNF-α (ng/L): 9.43±8.69 vs. 13.59±9.45, IL-10 (μg/L): 39.45±10.72 vs. 48.52±11.42, endotoxin (U/L): 6.74±2.12 vs. 9.21±3.28, D-lactic acid (mg/L): 98.74±20.74 vs. 114.78±19.89, DAO (mg/L): 21.45±8.49 vs. 29.47±9.41, I-FABP (ng/L): 1.4±0.2 vs. 1.5±0.2, all P < 0.05]. Conclusion Early EN combined with probiotics can effectively regulate the intestinal flora and intestinal mucosal barrier function, reduce the level of inflammatory response and enhance the body immunity in patients with severe ischemic stroke.

Objective: To evaluate the efficacy and safety of imatinib in the treatment of newly diagnosed chronic myeloid leukemia during chronic phase (CML-CP) in children and to analyze the difference of the efficacy and safety between imported original imatinib (Gleevec) and domestic generic imatinib (Xinwei). Methods: Clinical data of 35 children with newly diagnosed CML-CP in Beijing Children′s Hospital from January 2014 to January 2018 were collected, among which 15 cases were treated with the imported original imatinib (original drug group) and 20 cases were treated with the domestic generic imatinib (generic drug group). The hematological, cytogenetic and molecular reactions and safety of the treatments were monitored at months 3, 6 and 12. Chi square test or rank sum test was used for the comparison between two groups. Results: A total of 35 cases were treated for over 3 months, 31 cases were treated for over 6 months and 25 cases were treated for over 12 months. At 3 months, main cytogenetic response was obtained in 15 (100%) cases in the original drug group and 16 (80%) cases in the generic drug group respectively (χ²=3.387, P=0.119). At 6 months, complete cytogenetic response was obtained in 12 (80%) cases in the original drug group and 10 (63%) cases in the generic drug group (χ²=1.435, P=0.390). At 12 months, BCR-ABL^IS ≤ 0.1% was obtained in 11 (92%) cases in the original drug group and 10 (77%) cases in the generic drug group (χ²=1.009, P=0.593). There was no significant difference at all stages (all P>0.05). Hematologic toxicity occurred in 7(20%) cases. The non-hematologic adverse reactions include nausea in 8 (23%) cases, pain in 8 (23%) cases, edema in 6 (17%) cases, emesis in 2 (6%) cases, fever in 2 (6%) cases, weakness in 1 (3%) case, rash in 1 (3%) case. The adverse reactions were easy to control and no drug toxicity related deaths occurred. There was no significant difference in the adverse reactions between original drug group and generic drug group (P>0.05). Conclusions Imatinib had a good efficacy and safety in the early treatment of newly diagnosed CML-CP in children. The efficacy and safety of generic imatinib is similar to that of imported imatinib.

Objective: To know the detection rate of hereditary thrombocytopenia (HT) in children with chronic thrombocytopenia and its clinical and laboratory characteristics for an early clinical identification and diagnosis of HT in future. Methods: Data of the children with thrombocytopenia, who had been treated in Beijing Children′s Hospital from April 2016 to May 2018 and whose present history lasted for more than 1 year and had poor response to immunotherapy were retrospectively collected.HT was screened in these patients by adopting next generation sequencing (NGS). Finally, clinical and laboratory characteristics of these children with HT were summarized and analyzed. Results: A total of 161 children with chronic thrombocytopenia were included.Forty-three cases (26.7%) were found to have gene mutations.The genetic rules of the mutant gene, the family verification and the clinical manifestations of the proband and some related laboratory tests were analyzed and 24 cases (14.9%) can be diagnosed as HT.Among the HT patients, the proportion of males and females was 159, and the median onset of age was 0.58 years, which was significantly lower than that of non-HT cases (the median onset of age was 4.36 years), and the difference was statistically significant (P<0.001); the proportion of mucosal hemorrhage and visceral hemorrhage (31.8% and 13.7%) of HT was significantly higher than non-HT cases (15.3% and 0.6%), and the difference was statistically significant (P<0.001). Fifty percent of (12/24 cases) cases of HT had positive family history; according to the ave-rage platelet volume and platelet morphology in peripheral blood smear, HT could be divided into small platelet HT, po-sitive platelet HT and large platelet HT.Some cases had well response to immunotherapy but seemed easy to relapse du-ring the withdrawal period, while the others responded poorly to therapy.Different clinical manifestations of HT suggest different pathogenesis, which can be divided into the related types of megakaryocyte differentiation defect, megakaryocyte maturation defect, platelet release defect and platelet survival time shortening. Conclusions The pathogenesis and cli-nical phenotype of HT was different.Some of them were effective for immunotherapy, which were easily confused with immune thrombocy-topenla(ITP). It is clinically necessary to perform NGS in children with thrombocytopenia at early onset, abnormal platelet morphology, prolonged disease course or severe mucosal/visceral hemorrhage, in order to recognize HT timely to avoid delay in diagnosis and poor prognosis.