Tuberous sclerosis complex(TSC)is a rare genetic disease that can lead to benign dysplasia in multiple organs such as the skin, brain, eyes, oral cavity, heart, lungs, kidneys, liver, and bones. Its main symptoms include epilepsy, intellectual disabilities, skin depigmentation, and facial angiofibromas, whilst incidence is approximately 1 in 10 000 to 1 in 6000 newborns. This case presents a middle-aged woman who initially manifested with epilepsy and nodular depigmentation. Later, she developed a lower abdominal mass, elevated creatinine, and severe anemia. Based on clinical features and whole exome sequencing, the primary diagnosis was confirmed as TSC. Laboratory and imaging examinations revealed that the lower abdominal mass originated from the uterus. CT-guided biopsy pathology and surgical pathology suggested a combination of leiomyoma and abscess. With the involvement of multiple organs and various complications beyond the main diagnosis, the diagnostic and therapeutic process for this patient highlights the importance of rigorous clinical thinking and multidisciplinary collaboration in the diagnosis and treatment of rare and challenging diseases.

Objective To compile nursing guidelines for diabetic patients undergoing ultrasound-guided islet transplantation through percutaneous portal vein puncture and catheterisation,providing valuable insights for the care of such patients during the surgical procedure.Methods Between December 2017 to September 2023,a total of 27 patients underwent 44 surgical procedures for ultrasound-guided islet transplantation via percutaneous portal vein puncture and catheterisation at our hospital.Nursing assistance was provided preoperatively,intraoperatively and postoperatively for all the procedures.Results All 27 patients who had undergone 44 surgical procedures successfully went through the ultrasound-guided islet transplantation via percutaneous portal vein puncture and catheterisation.Among the 44 surgical procedures,3(6.8%)resulted in upper abdominal and liver area pain,nausea and vomiting during surgery,8(18.2%)had transient increase of portal vein pressure during transplantation,and 6(13.6%)encountered active bleeding following the removal of the portal vein catheters.None of the patients developed delayed portal vein bleeding or complication such as portal vein thrombosis after the surgery.Conclusions Nursing interventions play a crucial role in ensuring the successful outcomes of ultrasound-guided islet transplantation.Following measures are the keys and they play an important roles in ensuring the smooth completion of ultrasound-guided islet transplantation:preoperatively,carefully assess the condition of recipients and provide them with psychological supports and patient education.Intraoperatively,closely monitor the vital signs,portal vein pressure and blood glucose as well as to prevent complications.Postoperatively,implement the nursing measures to prevent the recipients from postoperative bleeding of portal vein.

Objective To observe the feasibility of 5.0T MR for cardiac imaging.Methods Three patients with heart diseases and 17 healthy volunteers were prospectively enrolled.Cardiac MR(CMR)cine sequence and black blood sequence imaging were performed using 5.0T and 3.0T MR scanner,respectively.The image quality and artifacts degrees were compared between 5.0T and 3.0T CMR images,and the consistency of left ventricular parameters obtained using 5.0T and 3.0T scanners was analyzed.Results No significant difference of image quality nor artifacts degrees was found between 5.0T and 3.0T CMR images(all P＞0.05).The left ventricular end diastolic volume(EDV),end systolic volume(ESV),ejection fraction(EF),stroke volume(SV)and end diastolic mass(EDM)derived from cine images acquired at different fields were in a good agreement(all ICC＞0.75,all P＜0.001).Conclusion 5.0T MR could be used for cardiac imaging,with image quality of cine and black blood sequences comparable to that of 3.0T MR.

【Objective】 To study the changes of platelet components(PC), apheresis platelets (AP) and pooled platelet concentrates (PPC) production of 19 provincial blood centers before and during the COVID-19 epidemic. 【Methods】 The data related to the collection of AP and the preparation of PPC from 2016 to 2021 of 19 provincial blood centers was collected. The production of PC, AP and PPC during the four years before the epidemic (i.e. 2016-2019) and during the COVID-19 epidemic (i.e. 2020 and 2021) were calculated respectively, and the change of production was analyzed. 【Results】 The total production of PC in 19 blood centers steadily increased from 2016 to 2019, with a decrease of 4.16% in 2020 and an increase of 15.60% in 2021, exceeding the output before the COVID-19 epidemic. In 2020, the production of PC of 42.11% (8/19) blood centers decreased compared with 2019, while 94.74% (18/19) in 2021 increased compared with 2020. The changes of AP output was basically consistent with the trend of PC. The total production of PPC in 2017 and 2018 both doubled compared to the previous year, while decreased by 67.98% in 2019, increased by 30.38% in 2020 and decreased by 27.08% in 2021. 【Conclusion】 The total production of PC kept increasing steadily between 2016 and 2019, but decreased in 2020 under the COVID-19 epidemic, with some blood centers being significantly affected. In 2021, with the strong support from government and various measures by blood centers, the total production of PC increased.

Objective:To evaluate the efficacy of oral pyrotinib in treatment of patients with human epidermal growth factor receptor 2 (HER2)-positive metastatic breast cancer in the real world, and to explore its influencing factors.Methods:The clinical data of 148 patients with HER2-positive metastatic breast cancer treated with oral pyrrolitinib in Shanxi Cancer Hospital from September 2018 to December 2020 were retrospectively analyzed. The efficacy was evaluated according to the efficacy evaluation criteria for solid tumors, version 1.1, and the adverse effects were graded according to the National Cancer Institute common terminology criteria of adverse effects, version 4.0. The Kaplan-Meier method was used to draw progression-free survival (PFS) curves, the patients were stratified by different clinical characteristics, and log-rank test was used for univariate analysis of PFS; the multivariate analysis of PFS was performed using Cox proportional hazards model.Results:The objective response rate (ORR) of 148 patients was 71.6% (106/148), and the disease control rate (DCR) was 89.2% (132/148). The overall median PFS time was 11.0 months (95% CI 10.1-11.9 months), and the median PFS of 19 patients with brain metastases was 10.0 months (95% CI 7.4-12.6 months). The differences in PFS between patients stratified by disease-free interval (DFI), the number of metastatic site and Eastern Cooperative Oncology Group (ECOG) score were statistically significant (all P < 0.05), but the difference in PFS between patients with negative and positive hormone receptor was not statistically significant ( P > 0.05). Multivariate Cox regression analysis showed that DFI (>1 year vs. ≤1 year: HR = 5.254, 95% CI 0.728-37.933, P = 0.046) and ECOG score (≥2 points vs. 0-1 point: HR = 2.454, 95% CI 1.261-4.788, P = 0.008) were independent influencing factors of PFS. The most common ≥grade 3 adverse effects were diarrhea (31 cases, 20.9%) and hand-foot syndrome (38 cases, 25.8%). Conclusions:The pyrotinib has definite efficacy and good safety in the treatment of HER2-positive metastatic breast cancer in the real world, especially for patients with DFI > 1 year and ECOG score 0-1 point, the efficacy and safety are particularly good.

Cost data caliber governance is key to fine cost management. To tackle the troubles in cost data management at multiple campuses of one hospital, the authors built a multi-campus cost data caliber governance mode. By means of enhanced top-level design, the mode carried out data governance by such measures as the establishment of data dictionary mapping library, standardizing department names and caliber, classification of charging items of medical services, precisely matching between fixed assets and charging items, interconnecting the management system of charging library and the procurement library of consumables, as well as precisely matching surgical disease types and charging items. These measures accomplished the consistency and comparability of cost data across campuses, building an automated, streamlined, standardized and integrated data governance mode for reference of hospitals with multiple campuses in need of cost management.

With the wide application of tyrosine kinase inhibitor (TKI), to obtain treatment-free remission (TFR) has gradually become the long-term goal for patients with chronic myelogenous leukemia (CML). Self-renewing leukemia stem cells during disease progression are related with the recurrence, and surveillance of residual leukemic cells is hypothesized to be one of the critical factors in successful TFR. On the way to obtain TFR, many breakthroughs have been made in innate and adaptive immunity of CML cells. This paper reviews the immune function of CML patients, the role of the immune markers in maintaining TFR, and the exploration of TKI combined with new immunomodulator therapy to achieve a greater degree of TFR.

Chronic myelogenous leukemia (CML) is a myeloproliferative disease characterized by heterotopic Ph chromosome. Tyrosine kinase inhibitor (TKI) has significantly improved the prognosis of patients with CML. Cytogenetic and molecular monitoring for assessing the therapeutic efficacy of TKI to guide disease management has become an important component of CML therapy. However, the expanded genomic analysis of the disease diagnosis, transformation and drug resistance has not been fully explored in CML research. The paper reviews the frequency and type of gene mutations at initial diagnosis and the time of treatment failure and transformation, and investigates the relationship between genetic mutations and the prognosis of CML patients in the diagnosis and treatment.

Objective:To investigate the relationship between BRAF V600E gene mutation and ultrasonography manifestations as well as the lesion invasiveness in thyroid cancer.Methods:A total of 153 patients pathologically diagnosed as thyroid cancer after surgery who underwent thyroidectomy in Shanxi Provincial Cancer Hospital from January 2018 to October 2019 were selected, including 146 cases of papillary thyroid carcinoma. Ultrasonography was performed before operation. Paraffin embedded tissue after operation was used to detect BRAF V600E gene mutation. According to the results of BRAF V600E gene detection, patients were divided into mutation group and non-mutation group. The ultrasonic characteristics of the two groups were compared. The relationship of BRAF V600E gene mutation with ultrasonic characteristics, clinicopathological characteristics as well as cervical lymph node metastasis was analyzed by using logistic regression.Results:There were 130 cases (85.0%) of BRAF V600E gene mutation and 23 cases of BRAF V600E gene non-mutation in 153 patients with thyroid cancer. Among 146 cases with papillary thyroid carcinoma, there were 128 cases (87.7%) of BRAF V600E gene mutation. The percentage of patients with the unclear boundary between thyroid lesions and capsule in BRAF V600E gene mutation group was higher than that of patients in non-mutation group, and the difference was statistically significant [46.9% (60/128) vs. 11.1% (2/18), χ 2 = 8.261, P = 0.004]. There were no significant differences in age, gender, nodule long diameter, aspect ratio, nodal location, internal calcification, internal echo, echo uniformity, cystic solid, nodal shape, boundary clarity, blood flow signal, the number of tumor site, lymph node metastasis and nodular goiter between BRAF gene mutation group and non-mutation group (all P > 0.05). The results of logistic regression analysis showed that only the boundary clarity between thyroid lesions and capsule was an independent influencing factor of BRAF V600E gene mutation ( OR = 14.400, 95% CI 1.847-112.246, P = 0.011), tumor lesion size was an independent influencing factor of cervical lymph node metastasis in papillary thyroid carcinoma ( OR = 2.714, 95% CI 1.335-5.517, P = 0.006). Conclusions:In papillary thyroid carcinoma, BRAF V600E gene mutation is related with lesion and the unclear boundary between the tumor and capsule, but not related with lymph node metastasis. The size of the tumor lesion is associated with lymph node metastasis.

Objective: To investigate the effects of maternal exposure to ambient fine particles (PM_2.5) in Fuzhou during pregnancy on immune responses to ovalbumin (OVA) in neonatal rats and the possible mechanisms. Methods: Pregnant Sprague-Dawley rats were randomly assigned into four groups (ten in each): filtered air (FA) plus normal saline (NS), airborne PM_2.5 plus NS (PM_2.5-NS), FA plus OVA (FA-OVA) and PM_2.5 plus OVA (PM_2.5-OVA) groups. Pregnant dams in the PM_2.5 exposure groups were placed in a PM_2.5 exposure chamber in which the PM_2.5 concentration was equal to the ambient air from the beginning of gestation till delivery, whereas the other dams inhaling air without particulate matters were put into a clean chamber. OVA sensitization was conducted through intraperitoneal injection of OVA at 50 μg per dam at 4 and 9 days of gestation, followed by inhalation of atomized 1% OVA for 30 min at 18, 19 and 20 days of gestation. Dams without OVA sensitization were given NS in the same way. Levels of interleukin (IL)-4, IL-5 and interferon-γ (IFN-γ) in neonatal rats' plasma were measured by enzyme-linked immunosorbent assay just after birth. Protein levels of transcription factors GATA-3 and T-bet in lung were analyzed by Western-blotting. Changes in microRNA(miR)-146a and miR-146b in spleen were detected by real-time polymerase chain reaction. Histological changes in lung were observed under light microscope. One-way analysis of variance and LSD test were used as statistical methods. Results: (1) IL-4 level in plasma was significantly increased in PM_2.5-NS [(18.56±7.04) ng/L], FA-OVA [(34.04±7.06) ng/L] and PM_2.5-OVA groups [(45.67±8.18) ng/L] as compared with that in FA-NS group [(10.51±2.88) ng/L], and the level of IL-4 in PM_2.5-OVA group was higher than that in PM_2.5-NS and FA-OVA groups (F=54.667, P<0.001). Significantly increased IL-5 level in plasma was found in FA-OVA and PM_2.5-OVA groups as compared with that in FA-NS group (F=6.253, P=0.023). Among the four groups, FA-OVA group showed significantly increased IFN-γ level in plasma (F=28.604, P<0.001). (2) GATA-3 level in lung tissues was significantly increased in PM_2.5-NS (31.09±3.54), FA-OVA (35.24±5.00) and PM_2.5-OVA groups (47.81±3.63) as compared with that in FA-NS group (24.19±3.12), and higher in PM_2.5-OVA group than in PM_2.5-NS and FA-OVA groups (F=96.581, P<0.001). T-bet level was significantly lower in FA-OVA and PM_2.5-OVA groups than in FA-NS group. Moreover, PM_2.5-OVA group showed decreased T-bet level as compared with that in PM_2.5-NS and FA-OVA groups (F=30.852, P<0.001). (3) Expression of miR-146a in spleen was significantly enhanced in PM_2.5-NS (1.72±0.27), FA-OVA (1.56±0.37) and PM_2.5-OVA groups (3.06±0.52) than in FA-NS group (1.05±0.25). Moreover, PM_2.5-OVA group showed enhanced expression of miR-146a as compared with that in PM_2.5-NS and FA-OVA groups (F=42.276, P<0.001). Changes in the expressions of miR-146b were similar to those in miR-146a (F=28.776, P<0.001). (4) Stenosis or disappearance of alveolar spaces, accompanied with infiltration of inflammatory cells in interstitial substance and congestion in alveolar septum, was seen in FA-OVA and PM_2.5-OVA groups and conditions in the latter group were more severe. Conclusions Intrauterine exposure to ambient PM_2.5 negatively affects fetal lung development and immunological function in rats, especially when the dams are sensitized with OVA during pregnancy.