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A retrospective analysis was conducted on a MonoMAC syndrome case admitted in October 2022 to the First Affiliated Hospital of Zhejiang University School of Medicine. The patient, a 16-year-old female with a history of persistent monocytopenia and mild anemia for several years, experienced recurrent symptoms of cough, expectoration, and fever, leading to multiple visits to the hospital. The diagnosis of MonoMAC syndrome was confirmed through comprehensive assessments including routine blood tests, pathogen metagenomic sequencing, lung and bone marrow biopsies, and next-generation sequencing of peripheral blood. The patient underwent haploidentical hematopoietic stem cell transplantation, with a smooth course of transplantation, achieving neutrophil engraftment on + 16 d and platelet engraftment on + 17 d, eventually restoring normal monocyte and NK cell counts. MonoMAC syndrome patients often initially present with infectious symptoms, and the diagnosis can be established based on significant monocytopenia in routine blood tests, history of non-tuberculous mycobacterial infections, and GATA2 germline mutations. Allogeneic hematopoietic stem cell transplantation may be required for some patients to improve their prognosis.

Objective: To summarize the resistance of rifampicin-resistant Mycobacterium tuberculosis to anti-tuberculosis drugs in group A. Methods: In the retrospective study, a total of 1 226 clinical isolates from suspected multidrug-resistant pulmonary tuberculosis patients in Beijing TB control system from 2016 to 2021 were identified as Mycobacterium tuberculosis (MTB) strains by MPB64 antigen detection test. Rifampicin-resistant tuberculosis (RR-TB) strains were screened by the phenotypic drug susceptibility using the proportion method. The drug susceptibilities of Levofloxacin(LFX), Moxifloxacin(MFX), Bedaquiline(BDQ) and Linezolid(LZD)were detected by the phenotypic drug susceptibility with microplate method. The drug resistance rate, drug resistance level and minimum inhibitory concentration (MIC) distribution of four anti-tuberculosis drugs in group A were analyzed. We calculated the demographic distribution of RR-TB, multidrug-resistant tuberculosis(MDR-TB), pre-extensively drug resistant tuberculosis (pre-XDR-TB), extensively drug resistant tuberculosis (XDR-TB) patients and the cross resistance of LFX and MFX, then summarized the drug-resistance spectrum of BDQ-resistant and LZD-resistant strains and the treatment outcome of RR-TB patients. Measurement data were expressed as rate or composition ratio,χ 2 test was used between and within groups, and P <0.05 was considered statistically significant. Results: Among the 1 226 suspected multidrug-resistant pulmonary tuberculosis patients, the detection rates of RR/MDR/pre-XDR/XDR-TB patients were 20.8%(255/1 226), 15.2%(186/1 226), 5.7%(70/1 226), 0.5%(6/1 226), respectively. There were statistically significant differences in the distribution of patients with the four types of drug resistance in terms of age and treatment history ( χ 2 =14.95, P =0.020; χ 2 =15.91, P =0.001). The drug resistance rates of LFX, MFX, BDQ and LZD in RR-TB patients were 27.5% (70/255), 27.5% (70/255), 0.4% (1/255) and 2.4% (6/255), respectively. The MICs of LFX, MFX and LZD-susceptible MTB were mainly at 0.25 mg/L, and the MIC of BDQ-susceptible MTB was mainly concentrated at 0.03 mg/L. 25.1% (64/255) of the RR MTB were resistant to both LFX and MFX, and 6 strains were resistant to LFX or MFX, showing incomplete two-way cross resistance. One BDQ-resistant strain and six LZD-resistant strains were detected. The treatment success rate of RR-TB patients was 74.4% (151/203), and there were statistically significant differences in treatment outcomes between resistant and sensitive patients on the LFX-containing treatment regimen (Fisher's exact test, P =0.012). Conclusions: The prevalence of fluoroquinolones (LFX and MFX) resistance in rifampicin-resistant MTB is very serious. LFX and MFX show incomplete bidirectional cross-resistance. BDQ and LZD have the most promising future in the treatment of MDR-TB. Improve drug-resistance testing will help to further improve the success rate of treatment.

Objective: To evaluate the efficacy and safety of intravenous iron supplementation in patients with recurrent iron deficiency anemia (IDA) . Methods: This retrospective analysis of 90 patients with recurrent IDA from May 2012 to December 2021 was conducted, comparing the efficacy and safety of the intravenous iron therapy group and the oral iron therapy group. Results: Among the 90 patients with recurrent IDA, 20 were males and 70 were females, with a median age of 40 (range: 14-85) years. A total of 60 patients received intravenous iron supplementation and 30 received oral iron supplementation. The hematologic response rates in the intravenous iron group were significantly higher than those in the oral iron group at 4 and 8 weeks after treatment [80.0% (48/60) vs 3.3% (1/30) and 96.7% (58/60) vs 46.7% (14/30), all P <0.001, respectively]. The median increase in hemoglobin levels was also significantly higher in the intravenous iron group than in the oral iron group [38 (4, 66) g/L vs 7 (1, 22) g/L at week 4 and 44.5 (18, 80) g/L vs 19 (3, 53) g/L at week 8, all P <0.001]. The intravenous iron group had a significantly higher proportion of patients who achieved normal hemoglobin levels than the oral iron group (55.0% vs 0 and 90% vs 43.3%, all P<0.001, respectively). Iron metabolism indicators were tested before and after 8 weeks of treatment in 26 and 7 patients in the intravenous and oral iron groups, respectively. The median increase in serum ferritin (SF) levels in the intravenous iron group 8 weeks after treatment was 113.7 (49.7, 413.5) μg/L, and 54% (14/26) of these patients had SF levels of ≥100 μg/L, which was significantly higher than the median increase in SF levels in the oral iron group [14.0 (5.8, 84.2) μg/L, t =4.760, P <0.001] and the proportion of patients with SF levels of ≥100 μg/L ( P =0.013). The incidence of adverse reactions was 3.3% (2/60) in the intravenous iron group, which was significantly lower than that in the oral iron group [20.0% (6/30), P =0.015]. Conclusion: Intravenous iron supplementation is more effective for hematologic response, faster hemoglobin increase, and higher iron storage replenishment rates compared with oral iron supplementation in patients with recurrent IDA, and it is well tolerated by patients.

Objective: To explore the clinical manifestations, imaging features, pathological features, diagnosis and treatment of pulmonary mucosal-associated lymphoid tissue(MALT)lymphoma concurrent with lung squamous cell carcinoma, and to improve the understanding of this disease. Methods: Using "Pulmonary mucosa-associated lymphoid tissue, squamous cell carcinoma" as the search term, from January 1, 1983 to August 31, 2020, a total of 3 cases were retrieved in the PubMed database. In the Wanfang database, using "Lung mucosa-associated lymphoid tissue, lung squamous cell carcinoma" as the search term, from January 1, 1990 to August 31, 2020, a total of 1 related document was retrieved. In the CNKI database, "(lung) mucosa-associated lymphoid tissue lymphoma, (lung) squamous cell carcinoma" was used as the search term, and no relevant case reports were retrieved. Results: A 64-year-old man was admitted to the hospital because of chest tightness and shortness of breath for 10 days, cough and fever for one day. Enhanced CT of the chest showed a soft tissue mass shadow in the right lower hilar area, with obstruction of the adjacent bronchus, and local mild enhancement, suggesting of right lower lung cancer. In addition, the CT scan also showed consolidated shadows in the lower lobes of both lungs, scattered nodules, multiple lymphadenopathy in the mediastinum, and a small amount of pleural effusion on the right. Under bronchoscopy, a cauliflower-like neoplasm was seen at the opening of the lower right basal section, about 7 mm×8 mm, and biopsy showed that part of the mucosal structure was destroyed, with disappearance of the squamous epithelial layer, and the nuclei were large and deeply stained, and some were distributed in nests, with poor keratinization and a small amount of necrosis, and fibrous tissue reaction. Immunostaining revealed that the tumor was positive for p40, CK5/6 and EGFR and negative forTTF-1, NapsinA, PD-L1, p53, with about 30% Ki-67 positive cells. A puncture biopsy of the right lower lobe showed that the alveolar cavity was filled with nested lymphoid cells, consisting of small lymphocytes, central cell-like cells and monocyte-like cells, with occasionally large cells. Immunostaining revealed CD 20 + , CD 79a + , scattered CD 3 +, Bcl2+, SMA vascular+, Bcl6-, CK-, CD10-, CyclinD1-, with about 3% Ki-67 positive cells. The histopathological examinations confirmed the diagnosis of mucosal-associated lymphoid tissue extranodal marginal zone lymphoma(MALT lymphoma),and lung squamous cell carcinoma. Conclusions: Pulmonary mucosa-associated lymphoid tissue lymphoma complicated with lung squamous cell carcinoma is rare and easy to be missed and misdiagnosed. Chest CT imaging shows single or multiple nodules, mass shadows or consolidation, often accompanied by air-bronchial signs in the lesion, bronchiectasis, ground glass density around the lesion, hilar and mediastinal lymphadenopathy. Occasionally, pleural effusion can be seen. Lung biopsy is the gold standard for diagnosis.

Objective: To explore the clinical characteristics of bronchiectasis(BR)coexisting in patients with rheumatoid arthritis (RA). Methods: One hundred and forty-eight bronchiectasis patients were retrospectively analyzed. These cases were all diagnosed in the Respiratory Department of Shanghai Pulmonary Hospital and Shanghai Gongli Hospital of Pudong New Area during Jan. 2012 to Dec.2015.The patients consisted of 74 males and 74 females, aging from 45 to 79 [mean(65±11)] years. In these patients, coexisting rheumatoid arthritis was found in 34 males and 36 females, aging from 45 to 79[mean(68±12)] years(RA-BR group). Patients with bronchiectasis alone consisted of 40 males and 38 females, aging from 49 to 76 [mean(63±10)] years (BR alone group). Data between the 2 groups of patients were compared, including general clinical features, serum anti-cyclic citrullinated peptide antibodies (anti-CCP), rheumatoid factor (RF), chest high-resolution CT (HRCT), and lung function . FACED scores were used to assess the severity of bronchiectasis. Meanwhile, we analyzed the correlation between anti-CCP and FACED scores in the 2 groups. Results: We observed an increase of serum anti-CCP in RA-BR patients compared with BR alone patients (196±68 versus 64±26, P <0.05). In addition, FACED scores in RA-BR patients were higher than those in BR patients (5.2±1.8 versus 3.1±1.4 , P < 0.05). Positive correlations between serum anti-CCP levels and FACED scores ( r =0.678, r =0.461, P <0.05) in both RA-BR and BR alone groups were observed in this study. Conclusions: The disease severity scores of RA-BR patients were higher than those of patients with BR alone. Levels of serum anti-CCP may act as a predictor for the diagnosis and prognosis of bronchiectasis in patients with RA.

A comparison is made between mandibular growth of the operated side and the unoperated side after vertical ramus osteotomy by measurements of the area and linear distances of bilateral mandibles, and digital image processing technique from the radiographs. The results show that slightly retarded growth of the mandible of the experimental side in growing Wistar rats occurs after osteotomy in the mandibular ramus, but obvious asymmetry is not found Deficiencies of mandibular growth in the operated side are similar in both groups of upward and downward repositioning of the proximal segment.

In this study, we repaired the extracranial facial nerve defect of rabbit using an autogenous vein graft and compared the results with conventional autogenous nerve graft in function and morphology. A 1 cm segment of the extracranial morphology. A 1 cm segment of the extracranial facial nerve was removed in 30 rabbits, the defect was repaired by a segment of vein or by a segment of nerve or without repair. The gross observation, histologic and histochemical examination, electrophysiological study and nerve fascicle graphics analysis were performed at 4, 8, 12, 20 weeks. The results of this study demonstrated that an autogenous vein as a non-nervous tissue graft can repair the extracranial facial nerve defect. 20 weeks after operation, the results with vein graft were superior to nerve graft.

Hemodynamics, hemorrheology and blood gases were studied in 20 patients with stable chronic cor pulmonale. Blood viscosity was affected by hematocrit and blood gases, which acted on pulmonary pressure, pulmonary vascular resistance and right cardiac output. Blood viscosity was measured in the shear stress rate of 5.75, 11.5, 23, 46, 115 and 230 s-1. The decrease in plasma pH and PaO2 elevated high shear stress blood viscosity, pulmonary arterial pressure, total pulmonary vascular resistance and decreased right cardiac output, O2 administration decreased in high shear stress blood viscosity, but increased in low shear stress blood viscosity. Blood viscosity was positively correlated with hematocrit, which varied in different shear stress rate, the relative coefficient was the highest when the shear stress rate was in 11.5 s-1 (r = 0.9288 P less than 0.001). Pulmonary arterial pressure and pulmonary vascular resistance were positively correlated with high shear stress blood viscosity but uneffected by low shear stress blood viscosity. The increasing in high shear stress blood viscosity decreased the right cardiac output. Rigidity of red blood cell may play an important role in regulating pulmonary circulation and right heart function.

The clinicopathologic features of thirty-six cases of basal cell carcinoma in the facial region are reported. There were 26 men and 10 women. The ages of these patients ranged from 14 to 72 years and the largest number of cases (67.74 percent) were encountered in the fifth and sixth decades of life. The average duration was approximately 6 years before patients were treated. The tumors were most frequent in the nose and upper lip. Most of them were histopathologically classified as the solid type. Moderate and extensive irregular peripheral palisading and squamous differentiation were less frequent in the primary basal cell carcinoma.