Four case reports of Chinese cystic fibrosis patients and literature review

2017 ◽  
Vol 52 (8) ◽  
pp. 1020-1028 ◽  
Author(s):  
Juan Xu ◽  
Yong Yin ◽  
Lei Zhang ◽  
Jing Zhang ◽  
Shuhua Yuan ◽  
...  
2020 ◽  
Vol 14 ◽  
pp. 175346662096164 ◽  
Author(s):  
Isabel C. Eraso ◽  
Saveria Sangiovanni ◽  
Eliana I. Morales ◽  
Liliana Fernández-Trujillo

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction (HR) mediated by antigens to Aspergillus fumigatus. It is estimated that 2–15% of patients with cystic fibrosis (CF) and between 1% and 5% of asthmatics develop ABPA, affecting approximately 4.8 million people worldwide. The goals of treatment are controlling inflammation, reducing the number of exacerbations and limiting the progression of lung damage. Systemic steroids are therefore used as the mainstay therapy, along with antifungal medications. However, many patients do not respond or develop side effects to treatment. In this scenario, biological drugs such as Omalizumab, Mepolizumab, Benralizumab and Dupilumab have been implemented in clinical practice, even though there is a lack of scientific evidence to support their use. We performed a literature review of the studies carried out which analyzed biologics for the management of ABPA in adult populations with asthma and CF. To our knowledge this is the first literature review that included all biologics. We included a total of 32 studies, all but one were descriptive studies, and the vast majority evaluated the use of Omalizumab. Biologics appeared to have more benefit for patients with ABPA and asthma than CF, specifically at decreasing the frequency of acute exacerbations and by having a steroid-sparing effect. Although a decrease in serum IgE level is considered a measure of therapy success, values may not decline as expected in the context of a significant clinical improvement, highlighting the importance of measuring patient-oriented outcomes. As evidence comes mainly from case series and case reports, randomized controlled trials are needed to evaluate further the safety and efficacy of biologics in ABPA. The reviews of this paper are available via the supplemental material section.


Lupus ◽  
2020 ◽  
pp. 096120332096570
Author(s):  
Juliana P Ocanha-Xavier ◽  
Camila O Cola-Senra ◽  
Jose Candido C Xavier-Junior

Reticular erythematous mucinosis (REM) was first described 50 years ago, but only around 100 case reports in English have been published. Its relation with other inflammatory skin disorders is still being debated. We report a case of REM, including the clinical and histopathological findings. Also, a systematic review of 94 English-language reported cases is provided. The described criteria for clinical and histopathological diagnosis are highlighted in order to REM can be confidently diagnosed.


2021 ◽  
Vol 3 ◽  
pp. 100071
Author(s):  
Vanesa Rodríguez-Fernández ◽  
Lucía Cameselle-Cortizo ◽  
María José Lamas González ◽  
Gonzalo José De Castro Parga ◽  
Javier Valdés-Pons ◽  
...  

Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.


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