Objective: To analyze the clinical characteristics of children with multisystem inflammatory syndrome (MIS-C) associated with SARS-CoV-2 in China, and to improve the understanding of MIS-C among pediatricians. Methods: Case series study.Collect the clinical characteristics, auxiliary examinations, treatment decisions, and prognosis of 64 patients with MIS-C from 9 hospitals in China from December 2022 to June 2023. Results: Among the 64 MIS-C patients, 36 were boys and 28 were girls, with an onset age being 2.8 (0.3, 14.0) years. All patients suffered from fever, elevated inflammatory indicators, and multiple system involvement. Forty-three patients (67%) were involved in more than 3 systems simultaneously, including skin mucosa 60 cases (94%), blood system 52 cases (89%), circulatory system 54 cases (84%), digestive system 48 cases (75%), and nervous system 24 cases (37%). Common mucocutaneous lesions included rash 54 cases (84%) and conjunctival congestion and (or) lip flushing 45 cases (70%). Hematological abnormalities consisted of coagulation dysfunction 48 cases (75%), thrombocytopenia 9 cases (14%), and lymphopenia 8 cases (13%). Cardiovascular lesions mainly affected cardiac function, of which 11 patients (17%) were accompanied by hypotension or shock, and 7 patients (12%) had coronary artery dilatation.Thirty-six patients (56%) had gastrointestinal symptoms, 23 patients (36%) had neurological symptoms. Forty-five patients (70%) received the initial treatment of intravenous immunoglobulin in combination with glucocorticoids, 5 patients (8%) received the methylprednisolone pulse therapy and 2 patients (3%) treated with biological agents, 7 patients with coronary artery dilation all returned to normal within 6 months. Conclusions: MIS-C patients are mainly characterized by fever, high inflammatory response, and multiple organ damage. The preferred initial treatment is intravenous immunoglobulin combined with glucocorticoids. All patients have a good prognosis.
Male ; Child ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Blood Coagulation ; COVID-19 ; China/epidemiology* ; Connective Tissue Diseases ; Coronary Aneurysm ; Fever ; Systemic Inflammatory Response Syndrome/therapy*

Allergy and Immunology ; Ambulatory Care ; Autoimmune Diseases/therapy* ; COVID-19 ; Communicable Disease Control ; Connective Tissue Diseases/therapy* ; Delivery of Health Care/methods* ; Dermatology ; Humans ; Immunosuppressive Agents/therapeutic use* ; Patient Selection ; SARS-CoV-2 ; Singapore ; Skin Diseases, Vesiculobullous/therapy* ; Telemedicine/methods* ; Tertiary Care Centers ; Vasculitis/therapy*

Raynaud syndrome is a medical condition that causes pain, numbness, and changes in skin color at the distal extremities. Raynaud syndrome can be subdivided into primary Raynaud's and secondary Raynaud's. The former is diagnosed when the cause is unknown and the latter is caused by an underlying condition, such as connective tissue diseases, injury, smoking, or certain medications. Both cancer chemotherapy and β-blockers are relatively common causes of Raynaud syndrome but there are no reports of its association with methimazole administration. The authors encountered a 43-year old woman with hyperthyroidism who developed digital ulcers associated with Raynaud syndrome after a methimazole treatment. Her digital ulcers and Raynaud syndrome were improved after methimazole was replaced with propylthiouracil and conventional therapy. This paper reports this case along with a review of the relevant literature.
Connective Tissue Diseases ; Drug Therapy ; Extremities ; Female ; Humans ; Hyperthyroidism ; Hypesthesia ; Methimazole* ; Propylthiouracil ; Skin Pigmentation ; Smoke ; Smoking ; Ulcer

BACKGROUNDStevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases with high mortality rates. This study was designed to analyze the pathogenic factors, clinical manifestations, complications, treatment, and prognosis of SJS/TEN and to explore the differences between surviving and deceased patients.

METHODSSJS/TEN patients admitted to Beijing Friendship Hospital from January 2006 to December 2015 were included in the study. Patients' data were retrospectively analyzed. Comparative studies were performed on the survival group and the deceased group, and Fisher's exact probability test was used for statistical analysis.

RESULTSAmong the 88 patients included, 40 (45.5%) were male with a mean age of 45 ± 18 years. Forty-eight (54.5%) had SJS, 34 (38.6%) had SJS/TEN, and 6 (6.8%) had TEN. Fifty-three (60.2%) cases were caused by medications, mainly antibiotics (n = 24) followed by traditional Chinese medicines (n = 7). Forty-two cases (47.7%) developed visceral damage. Eighty-two patients improved or recovered and were discharged from hospital, and six patients died. Comparative studies on the survival group and the deceased group showed that the presence of malignant tumor ( χ2 = 27.969,P < 0.001), connective tissue diseases ( χ2 = 9.187, P= 0.002), previous abnormal liver/kidney functions ( χ2 = 6.006, P= 0.014), heart rate >100 times/min ( χ2 = 6.347, P= 0.012), detached skin area >20% ( χ2 = 5.594, P= 0.018), concurrent mucosal involvement at the mouth, eyes, and external genitals ( χ2 = 4.945, P= 0.026), subsequent accompanying liver/kidney damage ( χ2 = 11.839, P= 0.001, and χ2 = 36.302,P < 0.001, respectively), and SCORTEN score >2 ( χ2 = 37.148,P < 0.001) increased the risk of death.

CONCLUSIONSSJS/TEN is mainly caused by medications, and nearly half of patients develop visceral damage. Multiple factors increase the mortality risk.

Adult ; Anti-Bacterial Agents ; therapeutic use ; Connective Tissue Diseases ; metabolism ; pathology ; Eye ; pathology ; Female ; Genitalia ; pathology ; Humans ; Kidney ; metabolism ; pathology ; Liver ; metabolism ; pathology ; Male ; Middle Aged ; Mouth ; pathology ; Retrospective Studies ; Skin ; metabolism ; pathology ; Stevens-Johnson Syndrome ; drug therapy ; metabolism ; pathology

Rhabdomyolysis is defined as a skeletal muscle injury with release of muscle cell constituents into the plasma. It can occur in various diseases and conditions, including muscle strain, drug or alcohol abuse, connective tissue disease, excess exercise, or following surgery. Only one case of rhabdomyolysis has ever been associated with liposuction in Korea. We experienced a case of rhabdomyolysis that developed after liposuction surgery. The patient was a 39-year-old woman presenting with abdominal pain 1 day after liposuction. She was treated with general supportive care, including massive hydration and absolute bed rest. Renal replacement therapy was performed due to pulmonary edema. She, finally, recovered fully. Acute kidney injury caused by liposuction-induced rhabdomyolysis is a rare disease. Therefore, we present this case with a review of the literature.
Abdominal Pain ; Acute Kidney Injury* ; Adult ; Alcoholism ; Bed Rest ; Connective Tissue Diseases ; Female ; Humans ; Korea ; Lipectomy* ; Muscle Cells ; Muscle, Skeletal ; Plasma ; Pulmonary Edema ; Rare Diseases ; Renal Replacement Therapy ; Rhabdomyolysis*

Despite recent advances in understanding of the pathobiology and targeted treatments of pulmonary arterial hypertension (PAH), epidemiologic data from large populations have been limited to western countries. The aim of the Korean Registry of Pulmonary Arterial Hypertension (KORPAH) was to examine the epidemiology and prognosis of Korean patients with PAH. KORPAH was designed as a nationwide, multicenter, prospective data collection using an internet webserver from September 2008 to December 2011. A total of 625 patients were enrolled. The patients' mean age was 47.6 +/- 15.7 yr, and 503 (80.5%) were women. The diagnostic methods included right heart catheterization (n = 249, 39.8%) and Doppler echocardiography (n = 376, 60.2%). The etiologies, in order of frequency, were connective tissue disease (CTD), congenital heart disease, and idiopathic PAH (IPAH) (49.8%, 25.4%, and 23.2%, respectively). Patients with WHO functional class III or IV at diagnosis were 43.4%. In total, 380 (60.8%) patients received a single PAH-specific treatment at the time of enrollment, but only 72 (18.9%) patients received combination therapy. Incident cases during the registry represented 297 patients; therefore, the incidence rate of PAH was 1.9 patients/yr/million people. The 1st-, 2nd-, and 3rd-yr estimated survival rates were 90.8%, 87.8%, and 84.4%, respectively. Although Korean PAH patients exhibited similar age, gender, and survival rate compared with western registries, they showed relatively more CTD-PAH in the etiology and also systemic lupus erythematosus among CTD-PAH. The data suggest that earlier diagnosis and more specialized therapies should be needed to improve the survival of PAH patients.
Adult ; Aged ; Connective Tissue Diseases/complications ; Data Collection ; *Databases, Factual ; Familial Primary Pulmonary Hypertension/*epidemiology/mortality/therapy ; Female ; Heart Defects, Congenital/complications ; Humans ; Internet ; Male ; Middle Aged ; Prognosis ; Prospective Studies ; Pulmonary Artery/*physiopathology ; *Registries ; Republic of Korea/epidemiology ; Survival Rate ; Young Adult

OBJECTIVETo evaluate the effect of Notoginseng Radix on hepatic expression of transforming growth factor beta1 (TGF-beta1) and connective tissue growth factor (CTGF) in rats with alcoholic liver disease (ALD), in order to discuss its protective effect on alcoholic cirrhosis.

METHODFifty SD male rats were divided into the normal control group, the model group, the high-dose and low-dose Notoginseng Radix groups (3.0, 12.0 g x kg(-1)) and the magnesium isoglycyrrhizinate group (24 mg x kg(-1)), with 10 rats in each group. Apart from the control group, other groups were administered with ethanol-cornoil-pyrazole for 14 weeks to establish the alcoholic liver disease model. During the establishment of the model, the high-dose and low-dose Notoginseng Radix groups were administered with 12 g x kg(-1) x d(-1) Notoginseng Radix for 14 weeks, once everyday. Efforts were made to detect liver function, pathology with Masson staining, and the expressions of TGF-beta1, Smad3, Smad7 and CTGF mRNA.

RESULTCompared with the rats in model group, rats in Notoginseng Radix groups showed significant reduction in liver ALT, AST, collagen fiber deposition, and TGF-beta1, Smad3 and CTGF mRNA expressions in liver tissues, with the increase in the expression quantity of Smad7 mRNA. There were differences between the Notoginseng Radix groups. No significant difference was observed between the high-dose Notoginseng Radix group and the magnesium isoglycyrrhizinate group.

CONCLUSIONNotoginseng Radix can affect TGF-beta1/Smads signaling pathway and reduce the expression of CTGF.

Animals ; Connective Tissue Growth Factor ; genetics ; metabolism ; Drugs, Chinese Herbal ; administration & dosage ; Gene Expression ; drug effects ; Humans ; Liver Diseases, Alcoholic ; drug therapy ; genetics ; metabolism ; Male ; Panax notoginseng ; chemistry ; Rats ; Rats, Sprague-Dawley ; Smad3 Protein ; genetics ; metabolism ; Smad7 Protein ; genetics ; metabolism ; Transforming Growth Factor beta1 ; genetics ; metabolism

BACKGROUND: This study aimed at assessing the number of red blood cell (RBC) units transfused at different types of medical institution and examining the characteristics of transfusion recipients. METHODS: We calculated and compared the number of transfusion recipients, total RBC units transfused, and RBC units transfused per recipient. Study data were extracted from insurance benefits reimbursement claims for RBC units at the Health Insurance Review & Assessment Service from 2006 to 2010. RESULTS: Between 2006 and 2010, the number of recipients of RBC units increased from 298,049 to 376,445, the number of RBC units transfused increased from 1,460,799 to 1,841,695, and the number of RBC units transfused per recipient changed from 4.90 to 4.89. The number of recipients aged > or =65 yr increased from 133,833 (44.9%) in 2006 to 196,127 (52.1%) in 2010. The highest number of RBC units was transfused to patients with neoplastic diseases (31.9%) and diseases of the musculoskeletal system and connective tissue (14.4%). More than 80% of the total number of RBC units were transfused at tertiary and general hospitals. However, this composition rate was slightly decreasing, with the composition rate for hospitals increasing from 12.6% to 16.3%. CONCLUSIONS: This study revealed an increase in the number of RBC units transfused over a 5-yr period due to an increase in the number of transfused recipients, especially recipients aged > or =65 yr; moreover, the number of RBC units transfused differed based on medical institution type. These results provide fundamental data on RBC transfusions required for future research.
Adolescent ; Adult ; Age Factors ; Aged ; Child ; Child, Preschool ; Connective Tissue Diseases/therapy ; Databases, Factual ; Erythrocyte Transfusion/*statistics & numerical data/*trends ; Female ; Hospitals, General/statistics & numerical data ; Humans ; Infant ; Infant, Newborn ; Insurance, Health, Reimbursement/*statistics & numerical data ; Male ; Middle Aged ; Musculoskeletal Diseases/therapy ; Neoplasms/therapy ; Republic of Korea ; Sex Factors ; Tertiary Care Centers/statistics & numerical data ; Young Adult

Adult ; Antibodies, Monoclonal, Murine-Derived ; administration & dosage ; therapeutic use ; Connective Tissue Diseases ; complications ; Female ; Humans ; Lung Diseases ; complications ; Middle Aged ; Rituximab ; Thrombocytopenia ; drug therapy ; etiology ; Young Adult

The descriptive cross sectional surveys were carried out among the outstanding clinical physicians in the field of acupuncture and moxibustion by using the survey sampling. The fuzzy comprehensive evaluation techniques was applied to evaluate the opinions of clinical experts in the field of acupuncture and moxibustion in order to classify the spectrum of diseases related to the musculoskeletal and connective tissue. After the statistical analysis on sixty-four diseases with 104 subtypes, the spectrum of diseases were determined as grade I spectrum of diseases with 13 subtypes of diseases, grade II spectrum of diseases with 51 subtypes of diseases, grade III spectrum of diseases with 10 subtypes of diseases, and grade IV spectrum of diseases with 30 subtypes of diseases. The outcomes reveal that acupuncture and moxibustion therapy have been applied widely for musculoskeletal diseases and connective tissue related diseases.
Acupuncture Therapy ; methods ; Bone Diseases ; therapy ; Connective Tissue Diseases ; therapy ; Cross-Sectional Studies ; Humans ; Moxibustion ; methods ; Muscular Diseases ; therapy ; Surveys and Questionnaires