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Jammed joints and constricted heart: The science of tribology and missing lubricin. A case report on camptodactyly-arthropathy-coxa vara-pericarditis syndrome.
Ann Pediatr Cardiol
October 2024
Department of Paediatric Cardiology, Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India.
An autosomal recessively inherited noninflammatory arthropathy known as camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome was discovered in 1999. It is distinguished by synoviocyte hyperplasia and subcapsular fibrosis of the synovial capsule, which results in a shortage of lubricin production. The resulting lack of joint lubrication induces increased mechanical stress, causing progressive deformities that become evident with weight-bearing and heightened joint activity.
View Article and Find Full Text PDFIndications and outcomes of elective open chest lead extractions.
J Cardiovasc Electrophysiol
November 2024
Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Background: Complications associated with cardiovascular implantable electronic devices may necessitate device and lead removal. An open approach to removal may be electively chosen in cases with high risk of complications or those requiring additional concomitant cardiac surgery. This study aimed to investigate outcomes of patients who underwent elective open lead extractions (OLE) at two large tertiary care centers.
View Article and Find Full Text PDFArticle Synopsis
- Rosai-Dorfman disease (RDD) is a rare condition characterized by painless swelling of lymph nodes and can affect multiple body systems.
- A six-year-old child presented with constrictive pericarditis and heart failure as the first signs of RDD, which is unusual.
- Treatment involved surgery to remove the thickened pericardium and steroid medication, leading to complete resolution of the child's symptoms.
Camptodactyly-arthropathy-coxa vara-pericarditis syndrome and an unusual association with mitral stenosis.
Turk J Pediatr
March 2024
Department of Cardiovascular Surgery, Acıbadem University School of Medicine, İstanbul, Türkiye.
Background: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported.
View Article and Find Full Text PDFTuberculous Pericarditis in Childhood: A Case Report and a Systematic Literature Review.
Pathogens
January 2024
Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties "G D'Alessandro", University of Palermo, 90127 Palermo, Italy.
Tuberculous pericarditis (TBP) is an important cause of pericarditis worldwide while being infrequent in childhood, especially in low-TB-incidence countries. We report a case of TBP and provide a systematic review of the literature, conducted by searching PubMed, Scopus, and Cochrane to find cases of TBP in pediatric age published in the English language between the year 1990 and the time of the search. Of the 587 search results obtained, after screening and a backward citation search, 45 studies were selected to be included in this review, accounting for a total of 125 patients.
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