Objective: To elucidate the clinical features of patients with refractory juvenile dermatomyositis (JDM), and to explore the efficacy and safety of tofacitinib in the treatment of refractory JDM. Methods: A total of 75 JDM patients admitted to the Department of Rheumatology and Immunology in Shenzhen Children's Hospital from January 2012 to January 2021 were retrospectively analyzed, and to analyze the clinical manifestations, efficacy and safety of tofacitinib in the treatment of refractory JDM. Patients were divided into refractory group with using of glucocorticoids in combination with two or more anti-rheumatic drugs for treatment, and the presence of disease activity or steroid dependence after a one-year follow-up. The non-refractory group is defined as clinical symptoms disappeared, laboratory indicators were normal, and clinical remission was achieved after initial treatment, and the clinical manifestations and laboratory indexes of the two groups were compared. The Mann-Whitney U test, Fisher's precision probability test was used for intergroup comparison. Binary Logistic multivariate regression analysis was used to identify risk factors for refractory JDM. Results: Among the 75 children with JDM, 41 were males and 34 were females with a age of onset of 5.3 (2.3, 7.8) years. The refractory group consisted of 27 cases with a age of onset of 4.4 (1.5, 6.8) years, while the non-refractory group consisted of 48 cases with a age of onset of 5.9 (2.5, 8.0) years. Compared with 48 cases in the non-refractory group, the proportion of interstitial lesions and calcinosis in the refractory group was higher than that in the non-refractory group (6 cases (22%) vs. 2 cases (4%), 8 cases (30%) vs. 4 cases (8%), both P<0.05). Binary Logistic regression analysis showed that observation group were more likely to be associated with to interstitial lung disease (OR=6.57, 95%CI 1.22-35.31, P=0.028) and calcinosis (OR=4.63, 95%CI 1.24-17.25, P=0.022). Among the 27 patients in the refractory group, 22 cases were treated with tofacitinib, after treatment with tofacitinib, 15 of 19 cases (86%) children with rashes showed improvement, and 6 cases (27%) with myositis evaluation table score less than 48 score both were improved, 3 of 6 cases (27%) had calcinosis were relieved, and 2 cases (9%) had glucocorticoid-dependence children were successfully weaned off. During the tofacitinib treatment, there was no increase in recurrent infection, blood lipids, liver enzymes, and creatinine were all normal in the 22 cases. Conclusions: Children with JDM with calcinosis and interstitial lung disease are more likely to develop refractory JDM. Tofacitinib is safe and effective for refractory JDM.
Child ; Female ; Male ; Humans ; Dermatomyositis/drug therapy* ; Retrospective Studies ; Risk Factors ; Calcinosis ; Glucocorticoids/therapeutic use*

With the population aging and declining incidence of rheumatic heart disease, calcific aortic valve disease (CAVD) has become the most frequent valve disease and the common cause of aortic valve replacement. Patients with CAVD need to cope with a deteriorating quality of life and valve replacement is the only effective clinical option for the patients. Therefore, early pharmacotherapy is of great significance in prevention or slow-down of the progression of CAVD. For years CAVD was considered to be a passive wear and tear process of valves, but now it is recognized as an active and multi-factorial process. Histopathologic studies have revealed that inflammation, disorder of calcium and phosphorus metabolism and dyslipidemia are involved in the process of CAVD. Clinical trials of CAVD pharmacotherapy have been carried out based on those histopathologic studies. Statin, renin-angiotensin inhibitors and anti-osteoporosis drug are well studied in recent years. This article reviews the recent research progress of the pharmacotherapy for CAVD.
Angiotensin Receptor Antagonists ; therapeutic use ; Angiotensin-Converting Enzyme Inhibitors ; therapeutic use ; Aortic Valve ; pathology ; Aortic Valve Stenosis ; complications ; drug therapy ; etiology ; Calcinosis ; complications ; drug therapy ; etiology ; Calcium Metabolism Disorders ; complications ; Disease Progression ; Dyslipidemias ; complications ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; therapeutic use ; Inflammation ; complications ; Phosphorus Metabolism Disorders ; complications ; Quality of Life

In patients with advanced breast cancer, most new calcifications detected on a mammogram after neoadjuvant chemotherapy are benign dystrophic calcifications, but this is not always observed. We present a patient with advanced breast cancer who had paradoxically increased malignant microcalcifications concomitant with primary tumor regression after undergoing neoadjuvant chemotherapy. After the neoadjuvant chemotherapy, the follow-up mammogram revealed that local, fine pleomorphic microcalcifications had markedly increased. Pathologically, these calcifications were ductal carcinoma in situ. We concluded that, in patients with breast cancer undergoing neoadjuvant chemotherapy, newly developed microcalcifications on follow-up mammograms should be carefully evaluated, and any suspicious malignant microcalcifications should be included in surgical excision planning.
Breast Neoplasms* ; Breast* ; Calcinosis ; Carcinoma, Intraductal, Noninfiltrating ; Drug Therapy* ; Follow-Up Studies ; Humans ; Mammography ; Neoadjuvant Therapy

OBJECTIVETo investigate the effect of HBP-A on meniscal injuries and the expressions of genes associated with pathological hypertrophy and calcification of the meniscusinduced by abnormal loading.

METHODSBovine meniscus explants were subjected to 25% strain at 0.3 Hz for 3 h and treated with 0.6 mg/mL of HBP-A. The cell viability in the meniscus explants after 72 hin culture was determined using live/dead staining and the expression levels of genes associated with pathological hypertrophy and calcification of the meniscus (ANKH, ENPP1, ALP, MMP13, and IL-1) were measured using real-time PCR and Western blotting. The conditioned medium was collected for testing sulfated glycosaminoglycan (GAG) release.

RESULTSThe number of dead cells, loss of proteoglycan content, and the expressions of ANKH, ENPP1, ALP and MMP13, and IL-1 at both the mRNA and protein levels were all significantly lower in the meniscus explants treated with 0.6 mg/mL HBP-A than in the explants with only 25% abnormal pressure stimulation (n=3, P<0.05).

CONCLUSIONHBP-A can effectively alleviate meniscal injuries induced by abnormal loading and suppress the expressions of genes related with pathological hypertrophy and calcification of the meniscus, and can serve as a potential drug for treatment of knee osteoarthritis.

Animals ; Calcinosis ; drug therapy ; Cattle ; Glucans ; pharmacology ; Hypertrophy ; Menisci, Tibial ; drug effects ; Osteoarthritis, Knee ; drug therapy ; Real-Time Polymerase Chain Reaction ; Tibial Meniscus Injuries ; drug therapy

No abstract available.
Basal Ganglia Diseases/diagnostic imaging/drug therapy/*etiology ; Calcinosis/diagnostic imaging/drug therapy/*etiology ; Calcium/therapeutic use ; Dietary Supplements ; Female ; Humans ; Hypoparathyroidism/*complications/diagnosis/drug therapy ; Middle Aged ; Tomography, X-Ray Computed ; Treatment Outcome ; Vitamin D/therapeutic use

Tympanosclerosis is the middle ear tissue hyalinization and calcification caused by chronic middle ear inflammation, which mainly results in conductive deafness with unobvious clinical symptom. Etiology is unclear. The treatment is given priority to surgical treatment at present, while long-term effect reported mostly poor. This article analyzed etiology and treatment of the tympanic cavity sclerosis.
Calcinosis ; Chronic Disease ; Ear, Middle ; Hearing Loss, Conductive ; etiology ; Humans ; Myringosclerosis ; complications ; etiology ; therapy ; Otitis Media ; Sclerosis

The metastatic calcification is defined as the deposition of calcium salt in normal tissue with an abnormal serum biochemical environment, such as chronic kidney disease, hyperparathyroidism, and hypercalcemia related with malignancy. Although the metastatic calcification can develop in any organs and tissues, presenting its symptoms and complications are rare. Thus a few cases have been reported. This case shows the metastatic calcification of the small intestine without any peritoneal and mesenteric vascular calcification which was early diagnosed by computed tomography and mesenteric angiography in a patient with abdominal pain, receiving continuous ambulatory peritoneal dialysis due to end stage renal disease. The clinician should early consider the metastatic calcification as differential diagnosis when unidentified calcifications are noted in simple abdominal X-ray such as in the present case, and promptly confirm it by using appropriate diagnostic tests in order to prevent its complications and progression.
Calcinosis/*diagnosis/drug therapy/etiology ; Calcitriol/therapeutic use ; Calcium/blood ; Calcium Carbonate/therapeutic use ; Calcium Channel Agonists/therapeutic use ; Humans ; Intestine, Small/*radiography ; Kidney Failure, Chronic/therapy ; Male ; Mesenteric Artery, Superior/radiography ; Middle Aged ; Peritoneal Dialysis, Continuous Ambulatory/*adverse effects ; Tomography, X-Ray Computed

OBJECTIVETo investigate the clinical characteristics, diagnosis and therapy of Keutel syndrome, and thereby to minimize the misdiagnosis.

METHODData of a case of Keutel syndrome diagnosed at the Provincial Hospital Affiliated to Shandong University were analyzed and related literature were reviewed.

RESULTAn 8-month-26-day-old boy was presented with inspiratory and expiratory stridor and wheezing after movement on lung auscultation. His craniofacial appearance was characterized by midfacial hypoplasia with a broad depressed nasal bridge. The nose was small and flat. A grade 2-3/6 systolic murmur was heard between the second and third ribs at left edge of the sternum. The end phalanges of his fingers were thickened. Chest radiograph showed tracheobronchial cartilage calcification and tracheobronchial stenosis. Echocardiographic examination revealed the right pulmonary stenosis. With endoscopic surgery, antiobstructive and antibiotic therapy clinical symptoms were improved. Three weeks later he died of lung reinfection after he was discharged from our hospital. English literature search with "Keutel syndrome" as the key word at "PubMed" showed 22 articles covering 26 patients, and the clinical symptoms were hearing loss (91%), persistent respiratory symptoms (68%), recurrent otitis media/sinusitis (67%), growth development delay (52%) in turn, and signs were brachytelephalangism (100%), low nasal bridge (95%), midfacial hypoplasia (93%), cardiac murmur (69%), and auxiliary examinations showed abnormal cartilage calcification (100%), pulmonary arterial stenosis (72%), tracheobronchial stenosis (50%).

CONCLUSIONThe diagnosis of Keutel syndrome should be considered in patients with brachytelephalangism, abnormal cartilage calcification, peripheral pulmonary stenosis, and midfacial hypoplasia. Tracheal stenosis was main clinical manifestation in part of patients.

Abnormalities, Multiple ; diagnosis ; diagnostic imaging ; therapy ; Bone and Bones ; diagnostic imaging ; Calcinosis ; diagnosis ; diagnostic imaging ; therapy ; Cartilage ; diagnostic imaging ; Cartilage Diseases ; diagnosis ; diagnostic imaging ; therapy ; Diagnosis, Differential ; Hand Deformities, Congenital ; diagnosis ; diagnostic imaging ; therapy ; Humans ; Infant ; Male ; Pulmonary Valve Stenosis ; diagnosis ; diagnostic imaging ; therapy ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed ; Tracheal Stenosis ; diagnosis ; diagnostic imaging

Although the treatment of aortic coarctation in adults with the use of stents has shown favourable results and reduced complications, there have been few studies involving elderly patients. We highlight the clinical challenges faced in the management of such patients, with attention to severe calcification at the coarctation site. The midterm results were good in our patient, showing an improvement in blood pressure control and maintenance of stent patency.
Aged ; Aortic Coarctation ; therapy ; Blood Pressure ; Calcinosis ; complications ; Cardiology ; methods ; Female ; Geriatrics ; methods ; Hemodynamics ; Humans ; Magnetic Resonance Angiography ; methods ; Myocardial Infarction ; therapy ; Stents ; Tomography, X-Ray Computed ; methods ; Treatment Outcome ; Troponin I ; blood

Pancreatic duct stones are a common complication during the natural course of chronic pancreatitis and often contribute to additional pain and pancreatitis. Abdominal pain, one of the major symptoms of chronic pancreatitis, is believed to be caused in part by obstruction of the pancreatic duct system (by stones or strictures) resulting in increasing intraductal pressure and parenchymal ischemia. Pancreatic stones can be managed by surgery, endoscopy, or extracorporeal shock wave lithotripsy. In this review, updated management of pancreatic duct stones is discussed.
Abdominal Pain/etiology ; Balloon Dilation ; Calcinosis/complications/diagnosis/physiopathology/surgery/*therapy ; Calculi/diagnosis/etiology/physiopathology/surgery/*therapy ; *Endoscopy/instrumentation ; Evidence-Based Medicine ; Humans ; Lithotripsy ; Pancreatic Ducts/physiopathology/*surgery ; Pancreatitis, Chronic/complications/diagnosis/physiopathology/surgery/*therapy ; Sphincterotomy, Endoscopic ; Stents ; Treatment Outcome