Objective: Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) is an autoinflammatory syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis and adenopathy. Our ...country is located in a geography where Familial Mediterranean fever (FMF) is common. In this study, it was aimed to investigate the effect of MEFV mutation carrying on the clinical features and treatment of PFAPA syndrome. Methods: The files of patients with PFAPA syndrome who were followed up in the pediatric rheumatology clinic for at least 6 months between 2019 and 2021 were retrospectively reviewed. Demographic data, clinical findings, frequency and duration of attacks, MEFV gene analysis results were recorded in the prepared forms. Results: Forty-three (51.8%) of the patients were girls. The median age of symptom onset was 1.5 (0.3-7) years, the age at diagnosis was 3.5 (0.5-8) years, and the time to diagnosis was 1.5 (0-7) years. MEFV gene was analysed in 68 (82%) patients, and heterozygous mutations were detected in 25 (36%) patients. The most frequent mutation was E148Q. There was no significant difference between the two groups in terms of mean age at diagnosis, age of symptom onset, duration of attack and frequency of attacks. Conclusion: In this study, the effect of MEFV mutation carriyng, which is very common in our country, on the course of the disease and the response to colchicine could not be demonstrated. Considering that FMF is common in our country, MEFV gene analysis should be considered in patients with findings inconsistent with the clinical and course of PFAPA syndrome.
Category:
Lesser Toes; Midfoot/Forefoot
Introduction/Purpose:
This review aimed to evaluate the effects of corticosteroid injections for Morton's neuroma using an algorithmic approach to assess the ...methodological quality of reported studies using a structured critical framework.
Methods:
Several electronic databases were searched for articles published until April 2020 that evaluated the outcomes of corticosteroid injections in patients diagnosed with Morton's neuroma. Data searches, extraction, analysis, and quality assessments were performed according to the PRISMA guideline, and the clinical outcomes were evaluated using various outcome measures.
Results:
With 3-12 months of follow-up, corticosteroid injections provided a satisfactory outcome according to Johnson satisfaction scores without two studies. Visual analogue scale scores showed maximal pain reduction between 1 week and 3 months post-injection. We found that 140 subjects out of 469 (29.85%) eventually underwent surgery after receiving corticosteroid injections due to persistent pain.
Conclusion:
Corticosteroid injections showed a satisfactory clinical outcome in patients with Morton's interdigital neuroma in spite of almost 30% of included subjects eventually underwent operative treatment. Our recommendation for the future study includes using more objective outcome parameters, such as foot and ankle outcome scores or foot and ankle ability measures. Moreover, studies about the safety and effectiveness of multiple injections at the same site are highly necessary.
The acid-catalyzed cyclization of benzylidenes based on 16-dehydropregnenolone acetate (16-DPA) was studied. It was found that these compounds readily undergo regioselective interrupted Nazarov ...cyclization with trapping chloride ion and an efficient method of the synthesis of d-annulated pentacyclic steroids based on this reaction was proposed. The structures of the synthesized pentacyclic steroids were determined by NMR and X-ray diffraction. It was found that the reaction affords a single diastereomer, but the latter can crystallize as two conformers depending on the structure. Antiproliferative activity of synthesized compounds was evaluated against two breast cancer cell lines: MCF-7 and MDA-MB-231. All tested compounds showed relatively high antiproliferative activity. The synthetic potential of the protocol developed was illustrated by the gram-scale experiment.
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