Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

Yang, So Yeon; Lee, Kyung Soo; Jae, Min; Kim, Tae Jung; Yoon, Haesung

doi:10.3348/kjr.2017.18.1.260

Cited by 7 publications

(10 citation statements)

References 22 publications

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“…The follow-up visit of one case revealed a small airway lesion with mosaic perfusion, which was the manifestation of small airway involvement, and the upper lobes of both lungs were more common. This result is basically consistent with the research results of imaging characteristics of CF patients reported by Yang et al [ 13 ]. Sinusitis is another manifestation of CF involving the upper respiratory tract.…”

Section: Discussionsupporting

confidence: 93%

Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China

Chu

Shuai

Huang

et al. 2021

Evidence-Based Complementary and Alternative Medicine

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Objective. To explore the clinical manifestations, imaging features, and gene mutation characteristics of 6 children with cystic fibrosis (CF) so as to improve the understanding and diagnosis awareness of CF in children and reduce the missed diagnosis and misdiagnosis. Methods. The clinical manifestations, imaging, and gene mutation data of six children with CF were collected and retrospectively analyzed. Results. Among the 6 cases of CF, there were 4 males and 2 females. Among the 6 children with CF, 5 cases presented with recurrent respiratory tract infection. Etiology suggested 3 cases of Pseudomonas aeruginosa and 2 cases of Staphylococcus aureus. 3 cases had pancreatic exocrine dysfunction, manifested as diarrhea and aliphatic diarrhea, of which 1 case had high lipase in blood examination, and pancreatic ultrasound showed rough and enhanced pancreatic echo, considering pancreatic cystic fibrosis. 2 cases of CF combined with pseudo-Bartter syndrome (PBS); 1 case involved only the biliary tract and started with cholestasis without other systemic involvement. In 2 cases of sweat test, sweat chloride ions were all >60 mmol/L. 3 cases underwent fiberoptic bronchoscopy, and a large number of sticky secretions were visible under the bronchoscopy. CT of the chest revealed thickening of the bronchial wall (3 cases), bronchiectasis (1 case), atelectasis (1 case), and thin bronchial lumen (2 cases). 1 patient was found to have small airway lesions and mosaic perfusion during follow-up. All 6 children with CF underwent genetic testing. A total of 12 CF transmembrane conductance regulator (CFTR) gene mutations were found, of which 4 mutations were not reported in the literature. Conclusion. CF is a disease caused by CFTR mutation. The incidence of this disease in China is low, and the clinical manifestations have great differences. The main symptoms are respiratory symptoms. Some children have gastrointestinal symptoms and/or PBS, and some children only show a single systemic lesion.

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Section: Discussionsupporting

confidence: 93%

Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China

Chu

Shuai

Huang

et al. 2021

Evidence-Based Complementary and Alternative Medicine

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“… 17 Only a small number of CF cases have been reported in Korea. 14 15 16 17 18 20 21 22 23 24 We also confirmed that Korean CF patients were extremely rare in the present study, with some patients previously reported. 16 17 25 Accordingly, the rarity caused the unavailability of a CF-specific diagnostic tool, SCT.…”

Section: Discussionsupporting

confidence: 92%

“…Among Korean patients, 20 cases have been described, with the first case being diagnosed in 1988. 14 15 16 17 18 However, there have been no Korean studies on clinical features and CFTR gene mutations. This study aimed to describe the clinical features and CFTR mutations in Korean pediatric cases of CF.…”

Section: Introductionmentioning

confidence: 99%

Multicenter Surveillance of Cystic Fibrosis in Korean Children

Kim

Hong

Ahn

et al. 2022

Allergy Asthma Immunol Res

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Purpose Cystic fibrosis (CF), caused by mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene, is rare among non-Caucasians. We aimed to identify the clinical features and CFTR mutations in Korean children. Methods We included 18 pediatric patients with CF diagnosed using sweat chloride test or genetic analysis for 30 years. HEK293 cells were transfected with wild-type CFTR , ΔF508- CFTR , and L441P- CFTR mutant plasmids for 24 hours and treated with CFTR correctors (VX809 and VX661). Results The median age at diagnosis was 9.2 years. Eleven patients had growth retardation, and 6 had a respiratory failure at diagnosis. Genetic analysis was used for all patients, while sweat testing was for 8 patients. At diagnosis, the median z scores of forced expiratory volume in one second (FEV1), FEV1/forced vital capacity, and forced expiratory flow at 25%–75% of forced vital capacity were −3.61 (−5.78, 1.78), −3.38 (−4.40, −0.60), and −4.45 (−5.78, 0.54), respectively. Two patients were treated with dornase alfa and only one with CFTR modulator. Patients were followed up for 3.7 years as a median. Four patients died at 10.6 years, with 4.2 years of post-diagnosis survival. The most common mutation was exon 16-17b deletion (19.4%). Among 11 single nucleotide variants, c.1322T>C (p.Leu441Pro, L441P) was detected in 4 patients. In the functional assay, L441P-CFTR correction was well restored by CFTR correctors compared with ΔF508. Conclusions CF is extremely rare in Korean children and is caused by different mutations from those commonly observed in Caucasians. Early diagnosis and treatment availability may improve outcomes. CFTR modulators may be effective for Asian patients with rare CFTR mutations, c.1322T>C (p.Leu441Pro).

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“…In total, 50 KJR (9101112131415161718192021222324252627282930313233343536373839404142434445464748495051525354565758), 492 ER, and 254 Radiology articles reported original research studies analyzing patient data. Of these, 44 (88%; 95% CI: 75.8–94.8%) (910121314151617181920212324262728293031323435363738394041424344474849505152535455565758), 359 (73%; 95% CI: 68.9–76.7%), and 211 (83.1%; 95% CI: 78–87.2%) articles, respectively, revealed the start and end of data collection to the level of calendar month.…”

Section: Resultsmentioning

confidence: 99%

“…Of these, 44 (88%; 95% CI: 75.8–94.8%) (910121314151617181920212324262728293031323435363738394041424344474849505152535455565758), 359 (73%; 95% CI: 68.9–76.7%), and 211 (83.1%; 95% CI: 78–87.2%) articles, respectively, revealed the start and end of data collection to the level of calendar month. The point estimate value of this proportion was slightly larger in KJR than in ER and Radiology, although the difference was not statistically significant.…”

Section: Resultsmentioning

confidence: 99%

Age of Data in Contemporary Research Articles Published in Representative General Radiology Journals

2018

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ObjectiveTo analyze and compare the age of data in contemporary research articles published in representative general radiology journals.Materials and MethodsWe searched for articles reporting original research studies analyzing patient data that were published in the print issues of the Korean Journal of Radiology (KJR), European Radiology (ER), and Radiology in 2017. Eligible articles were reviewed to extract data collection period (time from first patient recruitment to last patient follow-up) and age of data (time between data collection end and publication). The journals were compared in terms of the proportion of articles reporting the data collection period to the level of calendar month and regarding the age of data.ResultsThere were 50, 492, and 254 eligible articles in KJR, ER, and Radiology, respectively. Of these, 44 (88%; 95% confidence interval [CI]: 75.8–94.8%), 359 (73%; 95% CI: 68.9–76.7%), and 211 (83.1%; 95% CI: 78–87.2%) articles, respectively, provided enough details of data collection period, revealing a significant difference between ER and Radiology (p = 0.002). The age of data was significantly greater in KJR (median age: 826 days; range: 299–2843 days) than in ER (median age: 570 days; range: 56–4742 days; p < 0.001) and Radiology (median age: 618; range: 75–4271 days; p < 0.001).ConclusionKorean Journal of Radiology did not fall behind ER or Radiology in reporting of data collection period, but showed a significantly greater age of data than ER and Radiology, suggesting that KJR should take measures to improve the timeliness of its data.

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Chest CT Features of Cystic Fibrosis in Korea: Comparison with Non-Cystic Fibrosis Diseases

Cited by 7 publications

References 22 publications

Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China

Analysis of Clinical Manifestations, Imaging Features, and Gene Mutation Characteristics of 6 Children with Cystic Fibrosis in China

Multicenter Surveillance of Cystic Fibrosis in Korean Children

Age of Data in Contemporary Research Articles Published in Representative General Radiology Journals

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