Research progress of axial myopathy / 中华神经科杂志

Axial myopathy is a general term for a group of myopathy involving the axial muscles. It refers to a group of skeletal myopathy in which paraspinal muscles are individually or significantly affected, with or without the involvement of whole body skeletal muscles. Axial muscle weakness is often ignored in clinical practice. The evaluation of axial muscle is mainly the evaluation of the paraspinal muscles (erector spinae) in current literature. The clinical manifestations of paraspinal muscle weakness are dropped head syndrome and camptocormia. Physical examination and skeletal muscle magnetic resonance imaging, especially the whole body muscle magnetic resonance scan, are vital for the evaluation of axial muscle. It is of great clinical significance to increase attention to the diagnosis and differentiation of axial myopathy, which is helpful to avoid missing treatable diseases and improve the understanding and early recognition of associated myopath.

Clinical analysis of skeletal muscle and small fiber involvement associated with anti-contactin-associated protein-like 2 antibodies positive Morvan syndrome / 中华神经科杂志

Objective:To analyze the clinical data of a patient with anti-contactin- associated protein-like 2 (CASPR2) antibodies-related Morvan syndrome (MoS) and the related literature, and summarize the clinical characteristics of MoS patients.Methods:Clinical data of a CASPR2 antibodies-related MoS patient who was admitted in the Department of Neurology, the First Medical Center of Chinese People′s Liberation Army General Hospital in June 2021 were collected. CASPR2 IgG was detected by cell-based assay. Positron emission tomography/computed tomography (PET/CT), skin sympathetic response (SSR) and other examinations were performed. Clinical profiles of MoS patients were summarized by database retrieval.Results:The patient was a 55-year-old man presenting with peripheral nerve hyperexcitability, autonomic dysfunctions, neuropsychiatric symptoms and pain. Physical examination showed cognitive impairment, muscle quivering and absent deep-tendon reflexes. There was no family history of MoS and poisons exposure in this patient. Auxiliary examination showed serum creatine kinase was elevated (570 U/L) and antinuclear antibodies were positive (granular-type 1∶320). Other rheumatic and immunological antibodies, erythrocyte sedimentation rate, autoantibody profile, tumor marker, thyroid function, etc, were normal. Cerebrospinal fluid (CSF) protein and immunoglobulin were slightly higher. CASPR2 antibodies were positive in both serum and CSF (serum: 1∶100, CSF: 1∶10). Needle electromyography showed myokymic discharges, motor and sensory nerve conduction velocities were normal. SSR showed no waveform was elicited from both hands and feet. Cranial magnetic resonance imaging suggested scattered ischemic changes in the brain. PET/CT showed local metabolism increased slightly in soft tissues of bilateral shoulder and back, right lumbar and back muscles and bilateral gluteus medius. A total number of 232 cases of MoS patients were found in literature reports, most of which were male. The most common clinical manifestations were sleep disorders, and cognitive deficits accounted for 32.3%. Among them, skeletal muscle involvement was found in only 1 case by PET, and 4 patients had SSR abnormalities. Most of the patients had favorable neurological outcomes after the immunotherapy.Conclusions:MoS, as an autoimmune syndrome, may present with high uptake of skeletal muscle in PET/CT examination. Skeletal muscle involvement is a rare clinical manifestation of this disease. SSR as an electrophysiological test to evaluate autonomic neuropathy, its clinical value should be further strengthened.

Retrospective study on clinical manifestation, thigh MRI and electrophysiology characteristics of immune-mediated necrotizing myopathy / 中华内科杂志

Objective:To summarize the clinical, thigh magnetic resonance (tMRI) and electromyographic (EMG) characteristics in patients with immune-mediated necrotizing myopathy (IMNM).Methods:A total of 32 IMNM patients who were admitted to the Department of Neurology from April 2019 to April 2021 were enrolled at the First Medical Centre of Chinese PLA General Hospital. According to the type of antibody, the patients were divided into anti-SRP antibody positive (SRP +) group, anti-HMGCR antibody positive (HMGCR +) group and seronegative (SN) group. The gender, age, course of disease, myositis antibodies, extramuscular manifestations, EMG were collected and analyzed among three groups. The characteristics of skeletal muscle were assessed by tMRI inflammatory edema and fat infiltration scores. Analysis of variance, Kruskal-Wallis test and Chi-square test were used to compare the differences in different clinical characteristics and tMRI scores among the three groups. When there was a statistical difference among the three groups, the comparison between the two groups was corrected by the Bonferroni method. Result:(1) Of the 32 patients, 20 were females (62.5%).The median age of onset was 47±14 years, 25 (78.1%) patients had an acute or subacute course.There were 17 (53.1%) with SRP +, 8 (25.0%) with HMGCR +, and 7 (21.9%) with MSAs (myositis specific antibodies) negative. Anti-Ro52 antibody was the most common combined antibody (12/32, 37.5%), among which 10 were in SRP +group.(2) The CK of all patients were elevated, median was 5 948 (4 229, 7 664) U/L. There was no statistical difference of MMT scores among three groups. The proximal limb score was lower than distal limb ( P<0.01). The axial muscle score was lower than the distal limb score ( P<0.05).(3) Extramuscular manifestations of HMGCR + group were lower than those of the other two groups (12.5% vs. 71.4% and 76.5%, P<0.017). Rash (60.0% vs.14.3%, P<0.05) and interstitial pulmonary diseases (70.0% vs. 14.3%, P<0.05) were more common in patients with anti-SRP coexistence with anti-Ro52 than those with isolated anti-SRP. Connective tissue disease was more common in SN group (57.1% vs. 11.8% and 0, P<0.017).(4) tMRI showed fascial edema of SN group was more obvious than that of the other two groups ( P<0.017). There was no statistical difference in the degree of fat infiltration and inflammatory edema among three groups, but SRP + group had more cases of early fat infiltration.(5) Myotonic potentials (25.0% vs. 0 and 0, P<0.017) and compound repetitive discharges (CRDs) (50.0% vs. 5.9% and 0, P<0.017) were common in HMGCR + group. Proteomic analysis found significantly different expressed proteins in skeletal muscle of patients with myotonic potentials or CRDs were associated with cytoskeleton, cell junction and extracellular matrix. Conclusion:IMNM with pure anti-SRP antibody positive and anti-HMGCR positive were mainly affected by skeletal muscles. Those who were co-positive for anti-SRP antibody and anti-Ro52 antibody had more extramuscular manifestations, which might be a special subtype of SRP + group. This study proposed for the first time that myofascial inflammatory edema is an early sign of SN-IMNM injury. EMG of HMGCR +group were more prone to myotonia potential and CRDs.

Exploratory study of tracheal extubation in operating room after single-lung transplantation / 器官移植

Objective To evaluate the feasibility and safety of tracheal extubation in operating room for patients with end-stage chronic obstructive pulmonary disease (COPD) after single-lung transplantation. Methods Clinical data of 57 recipients who underwent single-lung transplantation due to end-stage COPD were retrospectively analyzed. According to the evaluation indexes of tracheal extubation in operating room established by our hospital, 17 recipients eligible for tracheal extubation in operating room were assigned into the operating room extubation group (OR extubation group) and 40 recipients receiving tracheal extubation in intensive care unit (ICU) were allocated in the ICU extubation group. The evaluation results of intraoperative tracheal extubation and postoperative recovery were compared between two groups. Results Compared with the ICU extubation group, recipients in the OR extubation group had higher oxygenation index, lower arterial partial pressure of carbon dioxide (PaCO2), lower blood lactic acid level, less fluctuation range of blood pressure and fewer cases receiving extracorporeal membrane oxygenation (ECMO) during operation (all P < 0.05). Two recipients in the OR extubation group received repeated tracheal intubation at 6 and 8 h after returning to ICU, and tracheal extubation at postoperative 6 and 9 d. In the OR extubation group, time of postoperative mechanical ventilation, length of postoperative ICU and hospital stay of the recipients were shorter than those in the ICU extubation group (all P < 0.05). The incidence of grade 3 primary graft dysfunction (PGD), atrial tachyarrhythmia, continuous renal replacement therapy and 1-year survival rate did not significantly differ between two groups (all P > 0.05). Conclusions The tracheal extubation regimen in the operating room for COPD patients after single-lung transplantation established by our hospital is safe and feasible, which shortens the time of postoperative mechanical ventilation, the length of postoperative ICU and hospital stay, whereas does not increase the incidence of postoperative complications.

Application of capillary electrophoresis in the diagnosis and differential diagnosis of benign and malignant monoclonal gammopathies / 中华检验医学杂志

Objective:To evaluate the value of capillary electrophoresis in the diagnosis and differential diagnosis of benign and malignant monoclonal gammopathies (MGs).Methods:A retrospective analysis of the capillary electrophoresis test results of 2 445 newly diagnosed patients at the Affiliated Hospital of Qingdao University from January 2016 to June 2020 was carried out. Capillary zone electrophoresis and immunosubtractive assay were used to detect serum monoclonal protein (MP). The clinical diagnosis and other information of the patients were collected from the clinical database of the Affiliated Hospital of Qingdao University. Kruskal-Wallis rank sum test was used to compare the different amount of monoclonal protein among multiple groups. Receiver operator characteristic curve (ROC) was used to analyze the diagnostic sensitivity and specificity of the monoclonal protein of each type. Youden index was used to calculate the cut-off values.Results:Among the 2 445 patients, 1 183 were positive for monoclonal protein, of which 944 cases were diagnosed as malignant MG, 174 were monoclonal gammopathy of undetermined significance (MGUS), and 65 cases were monoclonal gammopathy of renal significance (MGRS). The percentages of M protein types from high to low is immunoglobulin G(IgG)-κ, IgG-λ, IgA-λ, IgA-κ, free λ light chain, free κ light chain, IgM-κ, double clone, and IgM-λ. The levels of MP of IgG, IgA, IgM and FLC in the malignant MG group were all higher than those in the MGUS group, with statistical significance( P<0.01). The MP levels of IgG and IgA types in malignant MG group were higher than that in MGRS group ( P<0.01). ROC curve analysis showed that the MP of IgG, IgA, IgM and FLC types had good diagnostic efficacy for malignant MG ( P<0.01), and their AUC values were 0.947 (95 %CI 0.926-0.968), 0.930 (95 %CI 0.895-0.966), 0.844 (95 %CI 0.722-0.967) and 0.865 (95 %CI 0.781-0.950), respectively. For IgG, the cut-off value was 14.24 g/L, and the diagnostic sensitivity and specificity were 88.5% and 90.1%, respectively. The cut-off value of IgA was 8.88 g/L, and the sensitivity and specificity of IgA were 87.9% and 81.4%, respectively. For IgM, the cut-off value was 26.93 g/L, and the sensitivity was 64.4% and the specificity was 90.9%. For FLC, the cut-off value, diagnostic sensitivity, and specificity was 7.08 g/L, 85.9%, and 77.8%, respectively. Conclusions:Capillary electrophoresis immunotyping technique can be used to diagnose malignant MG such as multiple myeloma (MM) and non-Hodgkin′s lymphoma (NHL), as well as to screen and track diseases like MGUS and MGRS. Serum MP level can be used to distinguish malignant MG from benign MG effectively.

Analysis of short-term outcomes for lobar lung transplantation / 中华器官移植杂志

Objective:To explore the clinical value of lobar lung transplantation for end-stage lung disease patients in organ donation era.Methods:Clinical data were analyzed retrospectively for 14 cases with lobar lung transplantations between January 2016 and December 2019, including clinical outcomes and postoperative complications.Results:Eleven cases(78.6 %)had a positive etiology examination in bronchial secretion or tissue culture. There were unilateral lobar lung transplantation (n=2), bilateral lobar lung transplantation(n=2)and unilateral lobar lung transplantation and contralateral lung transplantation(n=10). Intra-operative ECMO(n=11)postoperative ECMO(n=5)were required. All survived during a 30-day perioperative period. The median time of postoperative ECMO was 1(1~11)day, the median time of extubation 4.5(0~182)day and the median time of stay in ICU 11(2~186)day. Re-operation was required for 1 case due to active bleeding in thoracic cavity. There were 10 survivors and 4 deaths. The causes of death were bronchus fistula(n=2), pulmonary infection(n=1)and renal failure(n=1)respectively.Conclusions:Lobar lung transplantation is efficacious for selected patients with end-stage lung disease.

Research progress on early acute kidney injury after lung transplantation / 器官移植

Acute kidney injury (AKI) is one of the common early complications after lung transplantation, which not only increases the short-term and long-term fatality of lung transplant recipients, but also significantly increases the incidence of long-term chronic renal insufficiency after surgery. In recent years, early AKI after lung transplantation has attracted high attention along with the rapid development of lung transplantation in China. In this article, research progresses on diagnosis, incidence, risk factors, prevention and treatment of early AKI after lung transplantation around the globe were reviewed, aiming to better identify the risk factors and poor prognosis of early AKI after lung transplantation, and provide theoretical and practical guidance for early clinical interventions.

Lung transplantation for cystic fibrosis: a case report and literature review / 器官移植

Objective To investigate the clinical efficacy and prognosis of lung transplantation in the treatment of cystic fibrosis (CF). Methods Clinical data of one patient with end-stage CF undergoing allogeneic bilateral lung transplantation were retrospectively analyzed. Clinical characteristics, diagnostic methods and treatment strategies of the CF recipient were summarized. Results The recipient had suffered from relevant symptoms since childhood including repeated cough and purulent sputum for 30 years, complicated with recurrent pulmonary infection combined with acute exacerbation, chronic sinusitis and extremely severe malnutrition. Prior to lung transplantation, the patient had to depend upon the invasive ventilator due to respiratory muscle weakness, and admitted to intensive care unit (ICU) for a long time. Imaging examination revealed multiple cystic columnar bronchiectasis accompanied with infection in bilateral lungs. The diagnosis of CF was further confirmed by sweat test and gene detection. The recipient underwent bilateral lung transplantation on August 17, 2017 and received rehabilitation treatment. The lung function was gradually restored to normal. The recipient had obtained the same quality of life to the healthy counterparts since the date of manuscript submission (over 2 years). Conclusions Lung transplantation is an efficacious treatment for end-stage CF, which can not only save patients' lives, but also significantly improve the quality of life of patients.

Series study on the relationship between air quality index and tuberculosis incidence in Beijing / 中华流行病学杂志

Objective To analyze the effect of air quality index (AQI) on the incidence of tuberculosis (TB) in Beijing,and to provide evidence for setting up a better program regarding prevention and control of tuberculosis.Methods Generalized additive model (GAM) was used to analyze the association between AQI and the incidence of tuberculosis in Beijing,from January 1,2014 to November 9,2016.Confounding factors as meteorological conditions and time trends were under control.Results In Beijing,a total of 14 533 TB cases with definite dates of onset were collected during the study period,with 36 children excluded from the study.Finally,14 497 cases were included in the study,including 9 513 men and 4 984 women,with 11 290 adults (15-59 years old) and 3 207 elderly (≥60 years old).Data from the optimal single-day lag effect of GAM showed that,with every 1 0 increase of AQI,the percent of increase on the onsets of overall,male,female and adult;tuberculosis cases were 0.85％ (95％CI:0.26％-1.44％),0.83％ (95％CI:0.24％-1.42％),0.93％ (95％CI:0.24％-1.62％) and 0.88％ (95％CI:0.29％-1.46％),respectively.The optimal lag time of the single-day effects were 15 days (lagl5),but 16 days (lag16) for male.The optimal cumulative lag effect showed that with every 10 AQI increase,the percent of increase on the onsets of overall,male,female and adult tuberculosis cases were 1.92％ (95％CI:0.23％-3.16％),1.94％ (95％CI:0.15％-3.72％),2.04％ (95％CI:0.10％-3.97％) and 2.00％ (95％CI:0.30％-3.69％),respectively,with the optimal lag time of cumulative delayed effects as 17 days (lag0_17),18 days (lag0_18),16 days (lag0_16) and 17 days (lag0_17),respectively.However,there were no statistical significances noticed in the elderly cases.Conclusion There was a positive correlation between AQI and the number of TB cases in Beijing,and the effects of AQI on the number of TB cases in different genders and age groups were different.

Series study on the relationship between air quality index and tuberculosis incidence in Beijing / 中华流行病学杂志

Objective To analyze the effect of air quality index (AQI) on the incidence of tuberculosis (TB) in Beijing,and to provide evidence for setting up a better program regarding prevention and control of tuberculosis.Methods Generalized additive model (GAM) was used to analyze the association between AQI and the incidence of tuberculosis in Beijing,from January 1,2014 to November 9,2016.Confounding factors as meteorological conditions and time trends were under control.Results In Beijing,a total of 14 533 TB cases with definite dates of onset were collected during the study period,with 36 children excluded from the study.Finally,14 497 cases were included in the study,including 9 513 men and 4 984 women,with 11 290 adults (15-59 years old) and 3 207 elderly (≥60 years old).Data from the optimal single-day lag effect of GAM showed that,with every 1 0 increase of AQI,the percent of increase on the onsets of overall,male,female and adult;tuberculosis cases were 0.85％ (95％CI:0.26％-1.44％),0.83％ (95％CI:0.24％-1.42％),0.93％ (95％CI:0.24％-1.62％) and 0.88％ (95％CI:0.29％-1.46％),respectively.The optimal lag time of the single-day effects were 15 days (lagl5),but 16 days (lag16) for male.The optimal cumulative lag effect showed that with every 10 AQI increase,the percent of increase on the onsets of overall,male,female and adult tuberculosis cases were 1.92％ (95％CI:0.23％-3.16％),1.94％ (95％CI:0.15％-3.72％),2.04％ (95％CI:0.10％-3.97％) and 2.00％ (95％CI:0.30％-3.69％),respectively,with the optimal lag time of cumulative delayed effects as 17 days (lag0_17),18 days (lag0_18),16 days (lag0_16) and 17 days (lag0_17),respectively.However,there were no statistical significances noticed in the elderly cases.Conclusion There was a positive correlation between AQI and the number of TB cases in Beijing,and the effects of AQI on the number of TB cases in different genders and age groups were different.

Report of 27 culture-positive fungal endophthalmitis / 中国感染与化疗杂志

Objective To report the clinical features and treatment outcomes of 27 patients with fungal endophthalmitis (27 eyes)over a five year period.Methods The authors retrospectively reviewed the etiology,direct smear examination,fungal cul-ture and treatments of 27 patients with culture-proven fungal endophthalmitis at the affiliated Hospital of Medical College Qing-dao University from 2007 to 2012.Results Exogenous infection was defined in 21 patients (77.8%),including 20 associated with penetrating wound,and 1 following cataract surgery.Endogenous infection was found in 6 patients (22.2%),including 3 associated with recent use of high-dose steroids,1 after abortion,1 following pelvic fracture,and 1 with long-term use of im-munosuppressive agents.Fungal hyphae were found in 17 smears of 27 samples (63.0%)by direct microscopic examination. The fungal strains cultured from 27 samples belonged to 8 genus and 12 species.The most common isolates were Aspergillus , Fusarium and Candida species.A.flavus (22.2%)and A.fumigatus (18.5%)were the predominant Aspergillus species. F .moniliforme (14.8%)and F .oxysporum (11 .1 %)were the most predominant Fusarium species.Two eyes were eviscer-ated immediately due to the serious condition.Among the other 25 eyes,22 (88.0%)got improvement after at least one of such treatments as intravitreal injection of antifungal agent,vitrectomy or penetrating keratoplasty (PK).Conclusions Exoge-nous fungal endophthalmitis is the most common type of fungal endophthalmitis in this hospital.Penetrating wound is the main cause of such infections.Microscopic examination of hyphae and fungal culture were effective for the diagnosis of fungal endophthalmitis.Aspergillus is the predominant pathogens, followed by Fusarium.Intravitreal injection of antifungal a-gent combined with vitrectomy is an effective treatment of fungal endophthalmitis.