Objective: To summarize the original CT features of Pneumocystis Jirovecii pneumonia in patients with hematological diseases. Methods: A retrospective analysis was carried out in 46 patients with proven pneumocystis pneumonia (PJP) in the Hospital of Hematology, Chinese Academy of Medical Sciences between January 2014 and December 2021. All patients had multiple chests CT and related laboratory examinations, imaging typing were conducted based on the initial CT presentation, and the distinct imaging types were analyzed against the clinical data. Results: In the analysis, there were 46 patients with proven pathogenesis, 33 males, and 13 females, with a median age of 37.5 (2-65) years. The diagnosis was validated by bronchoalveolar lavage fluid (BALF) hexamine silver staining in 11 patients and clinically diagnosed in 35 cases. Of the 35 clinically diagnosed patients, 16 were diagnosed by alveolar lavage fluid macrogenomic sequencing (BALF-mNGS) and 19 by peripheral blood macrogenomic sequencing (PB-mNGS) . The initial chest CT presentation was categorized into 4 types, including ground glass (GGO) type in 25 cases (56.5%) , nodular type in 10 cases (21.7%) , fibrosis type in 4 cases (8.7%) , and mixed type in 5 cases (13.0%) . There was no substantial discrepancy in CT types among confirmed patients, BALF-mNGS diagnosed patients and PB-mNGS diagnosed patients (χ(2)=11.039, P=0.087) . The CT manifestations of confirmed patients and PB-mNGS diagnosed patients were primarily GGO type (67.6%, 73.7%) , while that of BALF-mNGS diagnosed patients were nodular type (37.5%) . Of the 46 patients, 63.0% (29/46) had lymphocytopenia in the peripheral blood, 25.6% (10/39) with positive serum G test, and 77.1% (27/35) with elevated serum lactate dehydrogenase (LDH) . There were no great discrepancies in the rates of lymphopenia in peripheral blood, positive G-test, and increased LDH among different CT types (all P>0.05) . Conclusion: The initial chest CT findings of PJP in patients with hematological diseases were relatively prevalent with multiple GGO in both lungs. Nodular and fibrosis types were also the initial imaging findings for PJP.目的： 总结血液病患者肺孢子菌肺炎（PJP）的初始肺CT影像特征。 方法： 回顾性分析2014年1月至2021年12月在中国医学科学院血液病医院诊断为PJP的46例血液病患者的临床及影像资料。所有患者均行多次胸部CT及相关实验室检查，依据初始肺CT表现进行影像分型，并分析不同临床特征分组下的不同影像分型分布。 结果： 46例患者中，男33例，女13例，中位年龄37.5（2~65）岁。肺泡灌洗液（BALF）六胺银染色确诊11例、临床诊断35例，临床诊断患者中16例经BALF宏基因组二代测序（BALF-mNGS）诊断、19例经外周血宏基因组二代测序（PB-mNGS）诊断。初始胸部CT表现分为4型，包括磨玻璃型25例（56.5%）、结节型10例（21.7%）、纤维化型4例（8.7%）、混合型5例（13.0%）。确诊、BALF-mNGS诊断和PB-mNGS诊断患者的CT类型构成比差异无统计学意义（χ(2)=11.039，P=0.087）。确诊患者和PB-mNGS诊断患者CT表现以磨玻璃型为主（63.6%、73.7%），BALF-mNGS诊断的患者CT表现以结节型为主，占37.5%。63.0%（29/46）的患者外周血淋巴细胞减少，25.6%（10/39）血清G试验阳性，77.1%（27/35）血清LDH水平升高。不同CT类型患者的外周血淋巴细胞减少率、G试验阳性率和LDH升高率的差异均无统计学意义（P值均>0.05）。 结论： 血液病患者PJP初始肺CT表现多样，以两肺多发磨玻璃影多见，结节型与纤维化型也是PJP初始CT表现。.
To propose the concept of rhinogenic otitis media and explore its pathomechanism through analyzing the diagnosis and treatment on secretory otitis media caused by unhealthy nasal cavity structure.Conservative treatment and correlative operation under nasoscope were undertaken in 176 otitis media patients with unhealthy nasal cavity structure.Of 176 cases, 156 cases recovered completely (88.64%), 18 cases got effective treatment (10.23%), and 2 cases got ineffective treatment (1.14%).One important cause of the secretory otitis media is unhealthy nasal cavity structure, so correcting the unhealthy nasal cavity structure is the main ways to treat rhinogenic otitis media.
Using delayed degradation bone matrix gelatin (BMG) to repair the defect of laryngeal frame for the goal of laryngeal external form and function recovery.Partial laryngectomy and total laryngectomy were performed in nine cases with laryngeal cancer, then delayed degradation BMG be implanted in the defect of laryngeal frame or inside sterno hyoid muscle to build the laryngeal external form. In the same time the delayed degradation BMG be implanted in the crico-arytenoid muscle and around fibre with nerve ending, then embedding and sewing up to recover the swallow function and to prevent deglutition disorder.The delayed degradation BMG were applied in nine cases to rebuild new larynx, five of them were performed vertical partial laryngectomy and two total laryngectomy. The effect is satisfied after 15 years followed up. Eight of them rebuild the laryngeal external form and function, one patient need put on tube for long-term and cramming to speaking.Applying delayed degradation BMG to repair the laryngeal defect not only rebuild the laryngeal external form, but also recover the laryngeal function. It is an ideal bioactivity material and best choice for the new larynx rebuilding in the operation now.
Variants in the solute carrier family 40 member 1 (SLC40A1) gene are the molecular basis of ferroportin disease, which is an autosomal dominant hereditary hemochromatosis. Here, we present a patient with pure red cell aplasia (PRCA) and large granular lymphocytic leukemia (LGLL) associated with an extremely high levels of serum ferritin and iron overload syndrome. Whole exon sequencing revealed a novel heterozygous variant in SLC40A1 (p.T419I), which was found in his daughter as well. A series of functional studies in vitro of the T419I variant in ferroportin were conducted and the results revealed a reduced capacity of iron export from cells without changes in protein localization and its sensitivity to hepcidin. Intracellular iron storage in mutated cells was significantly higher than that of wild-type. These findings suggest that the novel variant p.T419I can cause the classical form of ferroportin disease and an elevated intracellular iron level indicates a potential novel pathogenic mechanism underlying PRCA and LGLL.
The therapeutic efficacy of combined antiangiogenic and immune therapy was tested against weakly immunogenic and highly metastatic 4T1 breast tumor using SU6668, an angiogenesis inhibitor and recombinant murine (rm) B7.2-IgG fusion protein, an immunostimulator. SU6668 inhibits the tyrosine kinase activity of three angiogenic receptors VEGFR2 (Flk-1/KDR), PDGFRbeta, and FGFR1, which play a crucial role in tumor-induced vascularization. rmB7.2-IgG is a fusion protein of the extracellular domain of B7.2, and the hinge and constant domains of murine IgG2a. Intermolecule disulfide linkages are maintained so that it forms a dimer. Our studies showed that three weekly immunizations of BALB/c mice bearing 0.5-0.8 cm 4T1 breast tumors with rmB7.2-IgG and irradiated 4T1 tumor cells (B7.2-IgG/TC) resulted in a significant inhibition of tumor growth and formation of pulmonary metastases. T-cell depletion experiments revealed that both CD4(+) and CD8(+) T lymphocytes are required for stimulation of the antitumor and antimetastatic immune response by B7.2-IgG/TC. Treatment of mice with SU6668 substantially inhibited tumor vascularization but did not inhibit tumor infiltration by T lymphocytes or the T-cell response to rmB7.2-IgG therapy. On the contrary, tumors in mice immunized with B7.2-IgG/TC and treated with SU6668 had higher numbers of tumor infiltrating T cells than tumors of other groups. SU6668 treatments initiated either on day 3 or day 10 after inoculation of 4T1 tumor cells resulted in a significant inhibition of tumor growth. Similarly, three weekly immunizations with B7.2-IgG/TC starting either on day 7 or 12 inhibited growth of 4T1 tumors. However, the most potent antitumor effects were observed in mice treated with a combination of SU6668 and B7.2-IgG/TC. Analysis of pulmonary metastases revealed that combined therapy also had a more potent antimetastatic effect than each modality alone. These results indicate that antiangiogenic and immune therapies using SU6668 and B7.2-IgG/TC are compatible, and manifest complementary antitumor and antimetastatic effects. Combined antiangiogenic and immune therapy might represent a new strategy for cancer treatment.
A biopsy of gastrocnemius muscle from a patient with mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome was studied histologically in semithin sections stained by hematoxylin-and-eosin (H&E) and toluidine blue, and ultrathin sections by transmission electron microscopy (TEM). H&E stain demonstrated typical ragged-red fibers (RRFs) and affected fibers in fascicles. Toluidine-blue stain showed an irregular meshwork in the center of RRFs. TEM demonstrated damaged myofibrils and variations in mitochondrial structure in RRFs and affected fibers. Dense mitochondria were compacted with cristae and pleomorphic electron-dense inclusions. Lucent mitochondria included paracrystalline inclusions with a parking lot appearance. At high magnification, the paracrystalline inclusions were composed of plates that paralleled and connected with mitochondrial cristae. These observations indicated that electron-dense granular and paracrystalline inclusions resulted from cristal degeneration and overlapping in mitochondria in MELAS syndrome.
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