The Efficacy of Yttrium-90 Radiosynovectomy in Patients with Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome

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The Efficacy of Yttrium-90 Radiosynovectomy in Patients with Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome

OBJECTIVE Camptodactyly-arthropathy-coxa-vara-pericarditis (CACP) syndrome is an autosomal recessive disorder caused by mutations in PRG4 gene that encodes for proteoglycan 4, the main lubricant for joints and tendon surfaces. It is a non-inflammatory arthropathy, characterized by joint effusions and synovial hypertrophy. So far, there is no effective treatment for this disorder. To evaluate th...

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Camptodactyly, Arthropathy, Coxa vara, Pericarditis (CACP)Syndrome: A Case Report

The camptodactyly-arthropathy-coxa vara-pericarditis syndrome (CACP) is characterized by congenital or early-onset camptodactyly, childhood-onset noninflammatory arthropathy associated with synovial hyperplasia. Some patients have progressive coxa vara deformity and/or noninflammatory pericardial effusion. CACP is inherited as an autosomal recessive mode and the disease gene is assigned to a 1....

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CACP syndrome (Camptodactyly Arthropathy Coxa Vara Pericarditis). Clinical case

Case Report Our patient, a 6 year old girl was diagnosed with the CACP syndrome at the age of 30 months. Her parents were negative for consanguinity. As an infant the patient was diagnosed with camptodactyly. At the age of 18 months frequent swelling of the knee was observed. Examination by rheumatologist confirmed the presence of swelling to elbows, wrists, knees, and ankles, without limitatio...

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Spinal involvement in Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome in two Yemeni sisters

Aim of the work: The objective of this clinical report is to describe the detailed magnetic resonance imaging (MRI) findings of the spine, knee and hip joints in two young sisters with Camptodactyly Arthropathy Coxa-vara Pericarditis (CACP) syndrome. Cases report: In two young sisters, both had normal levels of inflammatory markers and typical features of the CACP syndrome with camptodactyly, s...

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Protein-losing enteropathy in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome

BACKGROUND Camptodactyly-arthropathy-coxa vara-pericarditis (CACP, OMIM: #208250) syndrome is a rare autosomal recessive disease that can be difficult to recognise not only because of its wide clinical variability but also because of its clinical resemblance to juvenile idiopathic arthritis (JIA). PRG4 is the only gene so far known to be associated with CACP syndrome. Children with CACP syndrom...

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ژورنال

عنوان ژورنال: Malecular Imaging and Radionuclide Therapy

سال: 2017

ISSN: 2146-1414

DOI: 10.4274/mirt.29484