[Anti-HMGCR immune-mediated necrotizing myopathy: A case report].

The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.

[Pathologic features of paraspinal muscle biopsies in patients with adolescent idiopathic scoliosis].

OBJECTIVE: To characterize the paraspinal muscles of adolescent idiopathic scoliosis (AIS) patients, and to further explore its etiology. METHODS: Clinical records and paraspinal muscle biopsies at the apex vertebra region during posterior scoliosis correction surgery of 18 AIS were collected from November 2018 to August 2019. Following standardized processing of fresh muscle tissue biopsy, serial sections with conventional hematoxylin-eosin (HE) and histochemical and immunohistochemical (IHC) with antibody Dystrophin-1 (R-domain), Dystrophin-2 (C-terminal), Dystrophin-3 (N-terminal), Dystrophin-total, Myosin (fast), major histocompatibility complex 1 (MHC-1), CD4, CD8, CD20, and CD68 staining were obtained. Biopsy samples were grouped according to the subjects' median Cobb angle (Cobb angle ≥ 55° as severe AIS group and Cobb angle < 55° as mild AIS group) and Nash-Moe's classification respectively, and the corresponding pathological changes were compared between the groups statistically. RESULTS: Among the 18 AIS patients, 8 were in the severe AIS group (Cobb angle ≥55°) and 10 in the mild AIS group (Cobb angle < 55°). Both severe and mild AIS groups presented various of atrophy and degeneration of paraspinal muscles, varying degrees and staining patterns of immune-expression of Dystrophin-3 loss, especially Dystrophin-2 loss in severe AIS group with significant differences, as well as among the Nash-Moe classification subgroups. Besides, infiltration of CD4+ and CD8+ cells in the paraspinal muscles and tendons was observed in all the patients while CD20+ cells were null. The expression of MHC-1 on myolemma was present in some muscle fibers. CONCLUSION: The histologic of paraspinal muscle biopsy in AIS had similar characteristic changes, the expression of Dystrophin protein was significantly reduced and correlated with the severity of scoliosis, suggesting that Dystrophin protein dysfunctions might contribute to the development of scoliosis. Meanwhile, the inflammatory changes of AIS were mainly manifested by T cell infiltration, and there seemed to be a certain correlation between inflammatory cell infiltration, MHC-1 expression and abnormal expression of Dystrophin. Further research along the lines of this result may open up new ideas for the diagnosis of scoliosis and the treatment of paraspinal myopathy.

[Early diagnosis of pulmonary embolism in patients with skin and soft tissue defects after trauma].

Objective: To investigate the early diagnosis method of pulmonary embolism in patients with skin and soft tissue defects after trauma. Methods: From January 2011 to July 2014, 5 patients with skin and soft tissue defects and pulmonary embolism after trauma were admitted to Department of Plastic Surgery and Burns of the Affiliated Drum Tower Hospital of Nanjing University Medical School, including 4 males and 1 female, aged 26-68 years. The medical records of the 5 patients were retrospectively analyzed. Hierarchical screening of patients with suspected pulmonary embolism was performed after admission for 4-45 days. Computed tomography pulmonary angiography (CTPA) was performed immediately in 2 patients who had hemodynamic disorder and were able to tolerate CTPA, and pulmonary embolism was confirmed. Clinical risk assessment was conducted for the other 3 patients who had no obvious hemodynamic disorder and only had clinical manifestations of pulmonary embolism such as chest tightness and dyspnea. Among the 3 patients, two of them were assessed as high risk possibility by clinical risk assessment and diagnosed with pulmonary embolism by CTPA immediately. The other one patient's clinical risk assessment was moderate risk possibility, but D-dimer was positive, and the patient was diagnosed with pulmonary embolism by CTPA immediately. Wound exudation of all patients was collected within 1 week after admission for microbial culture, and wound debridement and skin grafting were performed according to the wound condition. The color Doppler ultrasonography of blood vessel on lower extremity was performed to determine deep venous thrombosis of lower extremity after appearance of symptoms of pulmonary embolism. The patient was immediately given urokinase or recombinant tissue plasminogen activator by intravenous infusion for thrombolysis after definite diagnosis of pulmonary embolism. The activated partial thromboplastin time (APTT) was monitored after treatment, and standardized anticoagulation began when APTT was equal to or lower than 70 seconds. The treatment results of patients, D-dimer measurement value, bed time before definite diagnosis of pulmonary embolism, number of patients underwent wound debridement during hospitalization, definite diagnosis time of pulmonary embolism after wound debridement, and number of patients with deep venous thrombosis of lower extremity and wound infection were recorded. Results: Wounds with skin and soft tissue defects of all patients were completely healed, all skin grafts survived well, pulmonary embolism recovered well after timely treatment, and the trunk and branches of involved pulmonary artery recovered blood supply. The course of disease ranged from 1 month to 3 months. The measurement value of D-dimer was 2.4-31.7 mg/L, and the measurement values of D-dimer of 4 patients were equal to or higher than 5.0 mg/L. The bed time before definite diagnosis of pulmonary embolism was 4-46 days, with an average of 23.2 days. Four patients underwent wound debridement during hospitalization. The definite diagnosis time of pulmonary embolism after the wound debridement was 14-40 days, with an average of 20.5 days. Four patients were diagnosed with deep venous thrombosis of lower extremity. All patients had wound infection, and the bacteria causing wound infection included Pseudomonas aeruginosa of 2 cases, Staphylococcus aureus of 2 cases, and Enterococcus faecalis of 1 case. Conclusions: In the diagnosis process of pulmonary embolism in patients with skin and soft tissue defects after trauma, D-dimer positive, long-term bed rest, experiencing operation during hospitalization, and with deep vein thrombosis and wound infection can be regarded as the key points for diagnosis. When a patient has clinical symptoms of pulmonary embolism and the above conditions, the clinician should promptly perform hierarchical screening, select the corresponding examination to confirm pulmonary embolism, and immediately perform thrombolysis for the patient with pulmonary embolism according to the patient's tolerance, thereby improving patient survival rate.

[Experience of wound treatment on extremely severe mass burn patients involved in August 2nd Kunshan factory aluminum dust explosion accident].

Objective: To explore experience of wound treatment of extremely severe mass burn patients involved in August 2nd Kunshan factory aluminum dust explosion accident. Methods: On August 2nd, 2014, 98 extremely severe burn mass patients involved in August 2nd Kunshan factory aluminum dust explosion accident were admitted to 20 hospitals in China. The patients with complete medical record were enrolled in the study and divided into microskin graft group with 56 patients and Meek skin graft group with 42 patients. Split-thickness skin in area of residual skin were resected to repair wounds of patients in microskin graft group and Meek skin graft group by microskin grafting and Meek miniature skin grafting, respectively. The residual wound size on 28 days post injury and wound infection after skin grafting of patients in the two groups, and position of donor site of all patients were retrospectively analyzed. Data were processed with t test and chi-square test. Results: The size of residual wound of patients in Meek skin graft group on 28 days post injury was (59±13)% total body surface area (TBSA), which was obviously smaller than that in microskin graft group [(70±14)%TBSA, t=4.379, P<0.05]. Twenty-nine patients in microskin graft group and 11 patients in Meek skin graft group suffered from obvious wound infection after skin grafting. Wounds of patients in two groups were repaired with residual skin around wound in head, trunk, groin, armpit, and uncommon donor sites of scrotum (4 patients), vola (10 patients), and toe or finger web (8 patients). Conclusions: Meek skin graft is the first choice for wound repair of extremely severe burn mass patients, with faster wound healing, less wound infection. Uncommon donor sites of scrotum, vola, and toe or finger web can also be used for wound repair in case of lack of skin.

[Dynamic investigation of nutritional risk in patients with malignant tumor during hospitalization].

Objective: To prospectively investigate the changes in nutritional status of patients with malignant tumors during hospitalization by using nutritional risk screening (NRS2002), and to analyze the correlation between the nutritional status and clinical outcomes . Methods: This was a prospective and parallel research done by multi-center collaboration from 34 hospitals in China from June to September 2014.Hospitalized patients with malignant tumors inthese departments (Department of Gastroenterology, respiratory medicine, oncology, general surgery, thoracic surgery and geriatrics)were investigated. Only the patients with age≥ 18 years and hospitalization time between 7-30 days were included. During hospitalization, the physical indexes of human bodywere measured, and the NRS 2002 scores, and monitored the nutritional support at the time points of admission and 24 hours before discharge were recorded.And whether there was a nutritional risk in hospitalized patients and its association with clinical outcomes were investigated. Results: A total of 2 402 patients with malignancies were enrolled in this study. Seventy fourpatients who did not complete NRS2002 were eliminated, and 2 328 patients were included. The number of the main diseases was the top five, including 587 cases of colorectal cancer, 567 cases of lung cancer, 564 cases of gastric cancer, 146 cases of esophageal cancer, and 119 cases of liver tumor. At the time of discharge, compared with admission, the BMI, body weight, grip and calf circumferences of patients with malignant tumor were significantly decreased (P<0.05). The total protein, albumin, prealbumin and hemoglobin were significantly lower than those at admission (P<0.05). In 2 328 patients who were completed nutritional risk screening, the rate of malnutrition at admission was 11.1% (BMI =18.5, 258/2 328) and the rate of malnutrition at discharge was 10.9% (BMI =18.5, 254/2 328), there were no significant differences (χ(2)=0.019 7, P=0.888). There were 1 204 patients with nutritional risk at admission (51.7%, NRS2002 score≥3)and 1 352 patients with nutritional risk at discharge (58.1%, NRS2002 score≥3), with significant differences (χ(2)=49.9, P<0.001). The incidence of nutritional risk in patients with colorectal, stomach, and lung tumors at discharge was significantly higher than that at admission (P<0.05). The infective complications and other complications of patients with nutritional risk were significantly greater than those without nutritional risk at admission and at discharge.ICU hospitalization stay of patients with nutritional risk was increased significantly than those without nutritional risk at admission(P=0.042). Hospitalization expenses of patients with nutritional risk was increased significantly than those of patients without nutritional risk at discharge(P<0.01). Conclusion: The patients with malignant tumor have a higher incidence rate of malnutrition at both admission and discharge and malnutritionhas correlation with adverse clinical outcomes.The aboveindicators did not improve significantly at discharge.Doctors should pay more attention to the nutritional status (screening and evaluation)of patients before discharge and use appropriate and adequate nutrition support in order to prevent the weight loss and improve the life quality of patients.

[Tumor necrosis factor and interleukin 6 in hepatitis C virus infection].

Tumor necrosis factor (TNF) was determined with ELISA in 72 patients with hepatitis C and hepatitis B virus infections. Interleukin 6 (IL-6) was determined at the same time. TNF and IL-6 were significantly increased in patients with hepatitis C virus infections when compared with normal subjects and hepatitis B patients. There was a significant positive correlation between TNF and IL-6. It is suggested that increased TNF and IL-6 may play an important role in pathogenesis of hepatitis C virus infection.