### How should the diagnosis of bronchiectasis be determined?

#### Recommendations – Imaging

#### Good practice points

CT imaging protocol 

CT features of bronchiectasis 

General 

### In whom should the diagnosis of bronchiectasis be suspected?

#### Recommendations

https://thorax.bmj.com/content/thoraxjnl/74/Suppl_1/1.full.pdf

British Thoracic Society Guideline for bronchiectasis in adults

Background and objective

Bronchiectasis is a chronic respiratory disease with diverse causes that may differ in clinical features and thus treatment options. However, few large-scale studies on the aetiology of bronchiectasis are currently available. This study aims to determine aetiology and clinical features of bronchiectasis in a Chinese Han population.



Methods

This prospective study enrolled adult patients diagnosed with bronchiectasis as confirmed by high-resolution computed tomography at five general hospitals in Shandong from January 2010 to August 2014. Causes of bronchiectasis were sought by analysis of clinical history and auxiliary examinations (including serum immunoglobulin determination, saccharin test, Aspergillus skin prick test, autoantibody detection and electronic bronchoscopy).



Results

A total of 476 adult patients with bronchiectasis were included, and all patients were of Chinese Han ethnicity. Idiopathic (66.0%) was the most common cause, followed by post-tuberculosis (16.0%). Other uncommon causes included post-infective (3.8%), immunodeficiency (3.8%), allergic bronchopulmonary aspergillosis (4.0%), rheumatic diseases (4.4%) and primary ciliary dyskinesia (0.9%). Patients with post-tuberculosis bronchiectasis had a higher frequency of upper lobe involvement (P   0.05).



Conclusions

In a Chinese Han population in Shandong, idiopathic bronchiectasis is the most common form of bronchiectasis followed by post-tuberculosis bronchiectasis. Patients with different causes differ in distribution and pattern of bronchiectasis on computed tomography.

Aetiology and clinical characteristics of patients with bronchiectasis in a Chinese Han population: A prospective study.

Cystic fibrosis (CF) in the Asian population is less frequently reported due to under-diagnosis and lack of centralized CF patient registries. Clinical studies on CF cases from Asia have documented a severe course of the disease. The spectrum of the cystic fibrosis transmembrane conductance regulator (CFTR) variants in this population is quite heterogeneous. In total, 166 variants have been reported on approximately 3700 Asian CF chromosomes. The frequency of F508del among Asians is low compared with Caucasians. Recent in vitro studies have shown promise of small molecule correction and potentiation of 45 different CFTR variants. Of these variants, 16 (including G551D and F508del) have also been observed among Asian CF individuals. We suggest undertaking molecular studies extensively to annotate CFTR variants that will help Asian CF individuals to benefit from the precision medicine gaining momentum in the Western countries.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/resp.12656

Epidemiology and genetics of cystic fibrosis in Asia: In preparation for the next-generation treatments:

This article considers the issue of personalized drug discovery for the orphan disease cystic fibrosis (CF) to deliver a candidate for therapeutic development. CF is a very complicated disease due to numerous anomalies of the gene leading to progressive severity and morbidity. Despite extensive research efforts, 20 years after the cloning of the CF gene, CF patients are still waiting for a curative treatment as prescribed medications still target the secondary manifestations of the disease rather than the gene or the CF transmembrane conductance regulator (CFTR) protein. New therapeutics aimed at improving mutant CFTR functions, also known as 'protein repair therapy' are nevertheless hoped and predicted to replace some of the currently used therapy, while improving the quality of life as well as life expectancy of CF patients. Although there is substantial variability in the cost of treating CF between countries, a protein repair therapy should also alleviate the financial burden of medical costs for CF patients and their families. Finding new drugs or rediscovering old ones for CF is critically dependent on the delivery of molecular and structural information on the CFTR protein, on its mutated version and on the network of CFTR-interacting proteins. The expertise needed to turn compounds into marketable drugs for CF will depend on our ability to provide biological information obtained from pertinent models of the disease and on our success in transferring safe molecules to clinical trials. Predicting a drug-induced response is also an attractive challenge that could be rapidly applied to patients.

Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date.

Cystic fibrosis (CF) is a rare disease most commonly seen in Caucasians. Only a few Chinese CF patients have been described in literature, taking into account the large population of China. In this systematic review, we collected the clinical and genetic information of 71 Chinese CF patients based on all available data. Compared with Caucasians, Chinese CF patients often present atypical symptoms, mainly displaying symptoms of pulmonary infection with fewer digestive symptoms. An ethnicity-specific CFTR variant spectrum was also observed in CF patients of Chinese origin, with p.Gly970Asp as the most common mutation while p.Phe508del, the most common pathogenic mutation in CF patients of Caucasian origin, is rare, suggesting the necessity of a Chinese-specific CFTR variant screening panel. Besides, multiplex ligation-dependent probe amplification analysis should be routinely considered, especially for those with unidentified mutations. Potential under-diagnosis of CF in Chinese patients might be caused by a combination of atypical clinical features and genetic heterogeneity in Chinese CF patients, the inaccessibility of sweat and genetic testing facilities, and the one-child policy in China. With the approval of promising small molecule correctors and potentiators, molecular characterization of Chinese-specific CFTR mutations will help to realize more precise treatment for Chinese CF patients.

/pdf/clinical-and-genetic-characteristics-of-cystic-fibrosis-in-7b6xps8dy1.pdf

Clinical and genetic characteristics of cystic fibrosis in CHINESE patients: a systemic review of reported cases.

Background Cystic fibrosis (CF) is rare in Chinese. We investigated the mutations in the gene of cystic fibrosis transmembrane conductance regulator (CFTR) in a Chinese CF patient and reviewed the clinical features, gene mutations in Chinese CF cases. Methods Blood samples were collected from a previously reported CF girl and her parents. The 24 coding exons of CFTR of the proband were amplified and sequenced. Results A Chinese girl of 16 years old was diagnosed as CF at the age of 14. She had recurrent productive cough with bronchiectasis in bilateral upper lobes, parasinusitis and otitis media, but without pancreatic involvement. Her sweat chloride was (108.9 +/- 3.3) mmol/L. A heterozygous novel missense mutation of 699 C --> A which results in the amino acid change of N189K was identified in exon 5. In addition, a heterozygous 3821 - 3823 delT mutation in exon 19 was found in CFTR. The mutation 699C --> A was inherited from her father, and the 3821 - 3823 delT mutation was from her mother. Twenty patients with CF in Chinese reported from 1974 to 2004 were also reviewed. DelF508 mutation was not found in the nine cases whose CFTR mutations were analyzed. Conclusions The CF proband carries two heterozygous mutations (699C --> A and 3821 - 3823 delT) in CFTR. 699C --> A mutation is a novel mutation which is not reported previously. Review of reported Chinese cases suggests that the genotype of Chinese CF may be different from those of white cases. More studies are needed to understand the spectra of CFTR and clinical CF features in Chinese.

A novel CFTR mutation found in a Chinese patient with cystic fibrosis.

OBJECTIVE To report a case of cystic fibrosis (CF) in a Chinese and to review the clinical features, gene mutations, diagnosis and outcome of CF in Chinese patients METHODS A 14-year-old Chinese girl presenting with recurrent productive cough since birth with parasinusitis and otitis media was confirmed to have CF in Peking University First Hospital Chest CT scan showed bronchiectasis, more severe in the right upper lobe Sweat tests were taken three times, and the values of Na(+) and Cl(-) were (1266 +/- 54) mmol/L and (1089 +/- 33) mmol/L, respectively The examination of the pancreas showed no remarkable cystic changes on CT scan and there was no pancreatic insufficiency Sixteen patients with CF in Chinese reported from 1974 to 1999 were reviewed RESULTS Sixteen of the 17 patients (7 males and 9 females, aged from 6 months to 25 years) had clinical data available for analysis Eleven of them had died, nine before the age of 13 They all suffered from frequent episodes of pneumonia, while 14 had malnutrition and 4 had jaundice The diagnostic procedures included clinical features, sweat test and autopsy Four of them had DNA screened and four kinds of novel mutations in the cystic fibrosis transmembrane conductance regulator gene were found CONCLUSIONS Chinese patients with CF show similar clinical manifestations to patients in the European and North American populations, but the CFTR mutation was different

A case report of cystic fibrosis and review of 16 cases of cystic fibrosis in Chinese patients

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A novel CFTR mutation found in a Chinese patient with cystic fibrosis.

A case report of cystic fibrosis and review of 16 cases of cystic fibrosis in Chinese patients