Objective: As of date, Kimura disease (KD) has an unclear etiology, no accepted diagnostic standard, and no definite treatment regimen. In this study, clinical and pathological laboratory characteristics and treatment regimens of patients with KD with different tumor sizes and status of tumor recurrence were analyzed. This was performed to identify the factors, which determine tumor size and recurrence, and to identify effective treatment methods for patients with KD.Methods: A total of 33 hospitalized patients with a definite diagnosis of KD were enrolled in this study.Results: There were 15 patients (45.5%) with a maximum tumor diameter of <3 cm. There were no statistically significant differences in age, gender, clinical symptoms, lesion sites, laboratory indicators, and treatment regimens among patients with a maximum tumor diameter <3 cm or ≥3 cm (P > 0.05). Among the 25 patients who completed the follow-up, there were 18 patients (72%) who had a recurrence of KD. There were no statistically significant differences in age, gender, clinical symptoms, the maximum tumor diameter, lesion sites, laboratory indicators, and initial treatment regimens between patients with or without the recurrence of KD (P > 0.05). There was a statistically significant difference in systolic blood pressure (SBP) between patients with or without the recurrence of KD (P < 0.05). All patients who received only surgical treatment had disease recurrence, 33.3% of patients who received prednisone therapy had no disease recurrence, and 37.5% of patients who received combination therapy showed recurrence.Conclusion: The current study summarized clinical manifestations, pathological features, laboratory indicators, and treatment regimens of patients with KD. There were no significant differences in these aspects among patients with different tumor sizes, and there was no significant difference in these aspects except in the SBP between patients with or without the recurrence of KD, indicating that SBP is a significant clinical factor affecting disease recurrence in patients. Combination therapy with prednisone was found to be superior to surgical treatment.
BackgroundAs one kind of osteochondrodysplasia, progressive pseudorheumatoid dysplasia (PPD) is also known as spondyloepiphyseal dysplasia tarda with progressive arthropathy or arthropathy progressive pseudorheumatoid of childhood. PPD is a very rare disease, especially in China, and has an estimated prevalence of 1/1000000 due to lacking definite prevalence survey. It is an autosomal recessive disorder caused by gene mutation of Wntl inducible signaling pathway protein 3 (WISP3). Its basic pathological change is persistent degeneration and loss of articular cartilage in multiple joints. Its clinical appearances include bone enlargement, platyspondyly, irregular endplate, secondary osteoarthritis, extensive osteoporosis, joint rigidity and function loss. Clinical diagnosis of PPD is made based on clinical appearance and imaging examinations, whereas its definite diagnosis depends on gene sequencing. PPD has no severe effect on life span, but causes high disability rate and very poor prognosis. There are only case reports with limited information in China.Case presentationOne female patient was diagnosed as PPD and secondary osteoarthritis. She had typical clinical appearance and imaging examinations, and received individualized therapeutic regimens. She had a gene mutation (c.72delT, p.T24TfsX4) of WISP3. This gene mutation has not been reported by previous literatures and included in Single Nucleotide Polymorphism Database.ConclusionsAs the first time, this paper reported a patient with PPD caused by new-found gene mutation (c.72delT, p.T24TfsX4) of WISP3.
Objectives This study aimed to assess the factors influencing bone erosion (BE) in patients with gout using dual-energy gemstone spectral imaging computer tomography. Methods We compared the clinical data, laboratory indices, and tissue urate levels at the monosodium urate (MSU) bone interface measured by dual-energy gemstone spectral imaging computed tomography of 87 gout patients with (n = 41) and without (n = 46) BE. Logistic regression analysis was used to investigate the risk factors associated with BE. Results In total, 47.1% of patients with gout had BE. The disease duration, serum uric acid, tissue urate levels, and the presence of tophi were significantly higher (p < .05) in gout patients with BE than in those without BE. Longer disease duration (odds ratio = 1.11, 95% confidence interval: 1.00–1.24, p < .05) and increased tissue urate levels (odds ratio = 1.01, 95% confidence interval: 1.00–1.02, p < .05) were independently associated with BE. Tissue urate levels at the MSU-bone interface were correlated with the presence of tophi (r = 0.62, p < .001), BE (r = 0.51, p < .001), renal calculus (r = 0.24, p = .03), and serum uric acid levels (r = 0.23, p = .03). Conclusions This study found that longer disease duration and elevated tissue urate concentrations at the MSU-bone interface were associated with BE in patients with gout.
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