Objective To clarify the epidemiological and clinical features of Birt–Hogg–Dubé syndrome (BHDS) in Chinese patients. Methods We identified reports on Chinese patients with BHDS by searching the China Academic Journals Database, Wanfang Chinese Database, and PubMed databases, either in Chinese or English languages published from January 1, 2008 to December 31, 2020. Studies without sufficient clinical data were excluded and cases under 18 years old were excluded. Results Twenty papers were included and comprised 120 families with 221 cases. Most families with BHDS were reported from institutions in Beijing (66.7%) and Jiangsu Province (15.8%); 80.8% of cases were reported within the past five years. The average duration from clinical presentation to diagnosis was 9.6 years. The average age was 47.0 ± 13.9 years (range, 18–84 years) and the ratio of male to female was 1:1.6. The most common manifestations of BHDS were multiple pulmonary cysts (92.4%), spontaneous pneumothorax (71.0%), skin lesions (18.1%) and renal tumors (3.6%). Pulmonary cysts were predominantly distributed in the lower lobe on chest CT imaging. Family history of spontaneous pneumothorax was identified in 84.7% of the families and average number of pneumothoraxes was 1.8 (range, 1–6). The FLCN gene mutation c.1285dupC/delC in exon 11 was the most frequent mutation observed (17.4% of patients). The recurrence rate of pneumothorax after conservative treatment (including tube thoracostomy) was 29/41 (71%) while the pneumothorax recurred after surgical treatment (pulmonary bullectomy or pleurodesis) in only 4/37 (11%). Conclusions Although BHDS has been increasingly reported in the recent years, only minority of families were reported from institutions outside of Beijing and Jiangsu Province. The dominant clinical manifestations were pulmonary cysts associated with recurrent pneumothorax, while skin lesions and renal tumors were less commonly reported. Delayed diagnosis along with suboptimal management appear to represent critical challenges for Chinese patients with BHDS.
Aims: A novel coronavirus SARS-CoV-2 has resulted in an ongoing global pandemic of Coronavirus disease 2019 (COVID-19). However, the outcomes of recovered patients have not been well defined. Methods: This is a prospective observational follow-up study of survivors with COVID-19 from a designated tertiary center in Hefei, China. We examined chest computed tomography (CT) scanning, pulmonary function, 6-min walk distance (6MWD), and 36 item Short Form General Health Survey (SF-36). Results: Among 81 enrolled patients, 62 (77%) patients and 61 (75%) patients, respectively, completed 1-month and 3-month follow-ups. Abnormal CT findings were still present in 73% of patients at 1 month and 54% at 3 months, whereas chest CT scan scores improved progressively at 1-month (5.0 ± 5.1) and 3-month follow up (3.0 ± 4.5) compared with that during hospitalization (11 ± 6.8). Mild restrictive pulmonary impairment was detected in 11% and 10% of patients at 1-month and 3-month follow up, respectively. The 6MWD was 523 ± 77 m in male patients and 484 ± 58 m in female patients, which was significantly lower than in healthy controls (606 ± 68 m, 568 ± 78 m, p < 0.001). SF-36 scores were significantly impaired in the domains of role physical (RP), role emotional (RE), and social functioning (SF) compared with the normal age-matched population. RP was improved at 3-month compared with 1-month follow up in the 41–64 years group ( p < 0.01). Multivariable analysis showed that older age (over 40 years) and steroid administration during hospitalization were independently associated with worse chest CT scores at 3-month follow up. Conclusions: At 3 months, chest CT abnormalities were present in one half of COVID-19 survivors and worse chest CT scores were independently associated with older age and steroid administration during hospitalization. Residual pulmonary function impairments were modest, whereas exercise capacity and SF-36 scores were significantly lower than the general population. Support program and further follow-up evaluations may be needed. The reviews of this paper are available via the supplemental material section.
An ongoing outbreak of Coronavirus Disease 2019 (COVID-19) has spread around the world. However, the clinical characteristics and outcomes of patients with COVID-19 related to different modes of exposure have not been well defined. We aimed to explore the clinical features and outcomes of COVID-19 related to one-time community exposure versus continuous household exposure. Retrospective case–control study involving COVID-19 patients admitted to a tertiary designated center in China was performed. Patients were enrolled if they had known exposure history of one-time community exposure or continuous household exposure. Twenty patients were compared in terms of demographic characteristics, clinical presentation, chest CT images, laboratory results, treatments, and clinical outcomes at 1-month follow-up. There were 10 patients in one-time community and continuous household exposure groups respectively. Males compromised 80% and 40% while the median ages were 37.5 and 51 years old in the 2 groups, respectively. Fever and cough were most common symptoms. Ground-glass opacities were presented on chest CT scan in 90% and 70% of the patients, and the median CT scores were 7 and 16 on admission, respectively. Three patients ranked severe in the community exposure group while 7 patients were severe or critical in household exposure group. On 1-month follow-up, all patients were improved clinically but COVID-19 IgG antibody detected positive. Median follow-up CT scores were 0 and 13 while pulmonary function test abnormalities were 0/9 and 2/7 in the 2 groups, respectively. COVID-19 patients with one-time community exposure tended to be mild in severity and had better outcomes, comparing to those with continuous household exposure.
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