No abstract available.
Bronchiectasis*

No abstract available.
Bronchiectasis*

Kartagener's syndrome is an inherited condition characterized by triad of chronic paranasal sinusitis, situs inversus, and bronchiectasis. Since 1976, Afzelius found a lack of dynein arm in immotile spermatozoa by electron microscopy, numerous recent studies have focused on the ultrastructural defect in the cilia and reported that the variety type of ultrastructural defect in immotile cilia syndrome. We report a female patient who had the Kartagener's triad with rare multiple ultrastructural defect of cilia in one patient. The electron microscopic examination showed partial dynein arm defect, loss of radial spoke, microtubular transposition, and giant cilia.
Arm ; Bronchiectasis ; Cilia ; Ciliary Motility Disorders ; Dyneins ; Female ; Humans ; Kartagener Syndrome* ; Microscopy, Electron ; Sinusitis ; Situs Inversus ; Spermatozoa

Primary ciliary dyskinesia is a disease characterized by unexplained neonatal respiratory distress, otitis media, chronic sinusitis, and chronic bronchiectasis. In approximately half of cases, situs inversus totalis or other laterality defects are found. The incidence ranges from 1 in 4,000 to 1 in 40,000 live births. Early diagnosis is important and the disease is finally confirmed by electron microscopic biopsy. The treatment of primary ciliary dyskinesia is based on appropriate procedures. We present a rare case of a 20-year-old male with bronchiectasis, chronic otitis media and chronic sinusitis.
Biopsy ; Bronchiectasis ; Early Diagnosis ; Humans ; Incidence ; Kartagener Syndrome* ; Live Birth ; Male ; Otitis Media ; Sinusitis ; Situs Inversus ; Young Adult

PURPOSE: Bronchiectasis in children is still one of the most common causes of childhood mortality in developing countries. The aim of this study was to investigate the epidemiological characteristics, clinical features, underlying etiologic factors, and distinct change in the management of patients with bronchiectasis at Asan Medical Center Children's Hospital of Seoul. METHODS: A retrospective study of children diagnosed with bronchiectasis was conducted between January 1999 and December 2008. All patients underwent a comprehensive examination to identify etiologic factors. Data analysis in terms of age at onset, initial presenting symptoms, underlying etiology, distinct change in treatment, distribution of pulmonary involvement on computed tomography (CT), and causative microbiological flora triggering secondary infections was performed. RESULTS: The median age at the time of the diagnosis of bronchiectasis was 7.6 years (range, 2 months to 18 years). Persistent coughing was the most common symptom. The underlying etiologies identified in 79 patients (85.8%) included bronchiolitis obliterans (32.6%), childhood respiratory infection (20.6%), interstitial lung disease (17.3%), immunodeficiency (8.6%), and primary ciliary dyskinesia (4.3%). In 53 children (67%), the identified cause led to a distinct and individualized change in management. The distribution of CT abnormalities had no correlation with the underlying cause of bronchiectasis. CONCLUSIONS: Selected Korean children with bronchiectasis were reviewed to identify diverse underlying etiologies. All children with bronchiectasis should be comprehensively investigated because identifying underlying causes may have a major impact on their management and prognosis.
Bronchiectasis ; Bronchiolitis Obliterans ; Child ; Coinfection ; Cough ; Developing Countries ; Humans ; Kartagener Syndrome ; Lung Diseases, Interstitial ; Prognosis ; Retrospective Studies ; Statistics as Topic

OBJECTIVE: Cystic fibrosis (CF) is a rare congenital disease in Korea, and its clinical and imaging findings are unclear. The objective of our study was to describe the clinical and CT features of CF in Korea and compare its features with those of other diseases mimicking CF. MATERIALS AND METHODS: From November 1994 to December 2014, a presumptive diagnosis of CF was made in 23 patients based on clinical or radiological examination. After the exclusion of 10 patients without diagnostic confirmation, 13 patients were included in the study. A diagnosis of CF was made with the CF gene study. CT findings were evaluated for the presence and distribution of parenchymal abnormalities including bronchiectasis, tree-in-bud (TIB) pattern, mucus plugging, consolidation, and mosaic attenuation. RESULTS: Of the 13 patients, 7 (median age, 15 years) were confirmed as CF, 4 (median age, 19 years) had primary ciliary dyskinesia, 1 had bronchiectasis of unknown cause, and 1 had chronic asthma. CT of patients with CF showed bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging in all patients, with upper lung predominance (57%). In CT of the non-CF patients, bilateral bronchiectasis, TIB pattern, mosaic attenuation, and mucus plugging were also predominant features, with lower lung predominance (50%). CONCLUSION: Korean patients with CF showed bilateral bronchiectasis, cellular bronchiolitis, mucus plugging, and mosaic attenuation, which overlapped with those of non-CF patients. CF gene study is recommended for the definitive diagnosis of CF in patients with these clinical and imaging features.
Asthma ; Bronchiectasis ; Bronchiolitis ; Cystic Fibrosis* ; Diagnosis ; Fibrosis* ; Humans ; Kartagener Syndrome ; Korea* ; Lung ; Mucus ; Thorax* ; Tomography, X-Ray Computed*

No abstract available.
Bronchiectasis* ; Bronchography*

No abstract available.
Bronchiectasis* ; Bronchography*

Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.
Adult ; Bronchiectasis ; Cough ; Female ; Humans ; Kartagener Syndrome* ; Korea ; Lung ; Lung Diseases* ; Mucociliary Clearance ; Mucus ; Mycobacterium Infections, Nontuberculous ; Mycobacterium* ; Nontuberculous Mycobacteria ; Respiratory Tract Infections ; Sinusitis ; Situs Inversus ; Sputum

Kartagener's syndrome is an autosomal recessively inherited condition characterized by triad of situs inversus, bronchiectasis, and chronic sinusitis. And recently it was classified as a subclass of dyskinetic cilia syndrome, which has a defect in mucociliary transport resulting from immotile or dyskinetic beating of cilia. Electron microscopic examination of the cilia from sperm tails, nasal and bronchial epithelium of patients reveals the partial or the complete absence of dynein arms or radial spoke, or microtubule disposition. This patient had all the triad of Kartagener's syndrome and showed two extracentral microtubules on the electron microscopic examination of the nasal mucosa. Most patients have dynein arms defect, but it is rare to have extracentral microtubules. So we report one case of Kartagener's syndrome with extracentral microtubules confirmed by electron microscopy.
Ammonia ; Apoptosis ; Arm ; Bronchiectasis ; Cilia* ; Dyneins ; Epithelium ; Helicobacter pylori ; Humans ; Kartagener Syndrome* ; Microscopy, Electron ; Microtubules* ; Mucociliary Clearance ; Nasal Mucosa ; Sinusitis ; Situs Inversus ; Sperm Tail