Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome Resembling Juvenile Idiopathic Arthritis: A Single-Center Experience from Southern Turkey.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the PRG4 gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. Herein, we report 3 patients with CACP syndrome from 2 unrelated families. All patients are female, born to consanguineous parents, and had camptodactyly since the first years of their lives. Two patients had a prior diagnosis of juvenile idiopathic arthritis. Hip changes were present in 2 patients, and 2 of 3 patients had undergone surgery for camptodactyly. Routine echocardiographic evaluations were normal during the 2-year follow-up. This paper represents the third study including CACP patients from Turkey. Clinically, all 3 patients resembled juvenile idiopathic arthritis cases and received unnecessary medication. There is also an ongoing need for improving awareness of CACP and an effective treatment focusing on the lubrication of the joint space in CACP patients.

Serum lubricin levels in patients with juvenile idiopathic arthritis.

OBJECTIVES: Lubricin, encoded by the proteoglycan 4 (PRG4) gene, is mainly responsible for lubricating joints. However, there is expanding evidence on its involvement in inflammatory pathways. Juvenile idiopathic arthritis (JIA) is a heterogeneous group of chronic arthritides with an unknown origin in children aged below 16 years. It is characterized by chronic joint inflammation, including synovial inflammation, and may result in cartilage destruction. We aimed to determine whether serum lubricin levels are affected in JIA patients. MATERIAL AND METHODS: This cross-sectional study included children diagnosed with JIA and 28 healthy controls. The patients were divided into two groups according to the presence of remission at the time of study. Lubricin protein analysis was performed by the enzyme-linked immunosorbent assay method. Serum samples were obtained at the study enrollment, and lubricin levels were measured once, and compared between JIA patients and healthy controls, and between JIA patients with active disease and remission. RESULTS: The study included 52 JIA patients (28 female, 24 male) and 28 healthy controls (18 female, 10 males). The mean age at study enrollment was 11.66 ±4.41 years and 12.72 ±4.52 years in the JIA patient and control groups, respectively. Although median serum lubricin level did not differ between JIA patients (median: 0.66 ng/µl, range: 0.02-3.85 ng/µl) and healthy controls (median: 0.52 ng/µl, range: 0.06-3.84 ng/µl), it was statistically significantly higher in patients with active disease (median: 1.58 ng/µ, range: 0.08-3.85 ng/µl) than both patients in remission (median: 0.57 ng/µl, range: 0.02-3.57 ng/µl) and healthy controls. A low degree positive correlation was also found between serum lubricin levels and erythroid sedimentation rate of the JIA patients (r = 0.383 and p = 0.011). CONCLUSIONS: This is the first study investigating serum lubricin levels in JIA patients, and we found elevated serum lubricin levels in JIA patients with active disease. Further studies are needed to clarify our results.

Isolation of lytic bacteriophages infecting Salmonella Typhimurium and Salmonella Enteritidis.

The objectives of this study were to isolate, purify and determine host range of lytic bacteriophages infecting foodborne the pathogen Salmonella Typhimurium and S. Enteritidis. River/stream water, sewage, raw foods, wastewater from food processing plants, slaughterhouse and fish farms and water from troughs were used for the screening of bacteriophages. The richest sources in terms of phages infecting S. Typhimurium and Enteritidis were found to be sewage, wastewaters of slaughterhouse, food processing and fisheries and streams. A total of 33 S. Typhimurium and 56 S. Enteritidis phages were isolated and purified from the samples. It was demostrated that host ranges of the isolated phages were quite wide. The numbers of bacteria types inhibited by S. Typhimurium or Enteritidis phages were changed among 1-15 and 1-19, respectively. It was found that 75.8% (25 out of 33) and 83.93% (47 out of 56) of isolated S. Typhimurium or Enteritidis phages formed clear plaques and were capable of lysing at least six or two Salmonella serovars. Beside Salmonella serovars, some S. Typhimurium (15 out of 33, 45.5%) and S. Enteritidis phages (5 out of 56, 8.93%) were also infective against E. coli strains. The host ranges of S. Typhimurium phages were wider than those of S. Enteritidis.