Yoon Jung Oh,M.D. Departments of Pulmonary and Critical Care Medicine Ajou University School of...
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Transcript of Yoon Jung Oh,M.D. Departments of Pulmonary and Critical Care Medicine Ajou University School of...
Yoon Jung Oh,M.D.
Departments of Pulmonary and Critical Care Medicine
Ajou University School of Medicine
BronchiectasisBronchiectasisBronchiectasisBronchiectasis
Abnormal and permanent dilatation of medium sized( ≥ 2mm) bronchus by destruction of muscular, cartilage and elastic tissue component
Three patterns of bronchiectasis (by Reid in 1950)⑴ Cylindrical bronchiectasis⑵ Cystic bronchiectasis⑶ Varicose bronchiectasis
Involved site⑴ Lt.lower lobe post.basal segment (common) ⑵ Rt.middle lobe⑶ bilateral (30%)
Definition
Case M/30
상기 30 세 남환은 수일 전 감기를 앓은 후 내원 당일 고열과
화농성 객담을 동반한 기침을 주소로 내원하였다 .
환자는 부비동염으로 이비인후과에서 치료를 자주 받았다하
며 , 1 년전에도 폐렴으로 입원한적이 있었다 한다 . 평소
간헐적인 객혈이 있었으나 결핵의 기왕력은 없었다 .
P/E moist crackles on left lower lung field
Bronchiectasis
Cystic Bronchiectasis
Cystic Bronchiectasis
Bronchogram HRCT
Bronchiectasis
Cylindrical Bronchiectasis
Varicose Bronchiectasis
• Bronchopulmonary infections
• Hereditary abnormalities
• Immunodeficiency states
• Bronchial obstruction
• Congenital anatomic defects
• Miscellaneous disorders
Pathogenesis and Predisposing Factors
Post pneumonia (Measle, Pertussis, Tbc)1. Mechanical obstruction(foreign body)2. Cystic fibrosis3. -globulin deficiency(cong./aquired)4. Immotile cilia syndrome
1. Post pneumonia(viral, aspiration)2. Pulmonary tuberculosis3. Asthma, ABPA(allergic bronchopulmonary aspergillosis)4. Toxic fume or congenital5. Neoplasm, Chagas’ disease
연령에 따른 원인
Childhood
Adulthood
Bronchopulmonary infections
• Childhood diseases
• Other bacterial infections
• Other viral infections
• Miscellaneous infections
pertussis; measles
S.aureus, Klebsiella, M.tuberculosis, H.influenaze
adenovirus, influenza, H.simplex, HIV
Mycotic infections
Mycolplasmal infections
Nontuberculous mycobacteria
Hereditary Abnormalities
• Ciliary defects of respiratory mucosa
Kartagener’s syndrome : bronchiectasis, situs inversus, sinusitis Immotile cilia syndrome : male infertility, defect in dynein arm or radical spoke • 1-Antitrypsin deficiency : panacinar emphysema, bronchiectasis
• Cystic fibrosis
Kartagener’s Syndrome
• Triad bronchiectasis, sinusitis, situs inversus
• Incidence : 1/68,000
• Inheritance : Autosomal recessive trait
• Pathogenesis * absent or defective dysnein arms * functional abnormality (e.g., perhaps dysnein ATPase)
Kartagener’s syndrome
Immotile Cilia Sydrome
Diagnostic Criteria
1. Recurrent & Chronic URI and LRI
: rhinitis, sinusitis, otitis, bronchitis, bronchiectasis
2. Absence or near absence of tracheobronchial or nasal mucocili
ary transport
3. Total or near-total absence of dynein arms of the cilia in nasal
or bronchial mucosa
4. Sterility in males
MT : microtubulesC : central sheathR : radial spokesD : dysnein armsN : nexin
Normal Cilia
Schematic cross section views of cilia
A : Normal cilium
Nine outer pairs of MT
around a central pair
B – D : congenital ciliary defects
B : lacking dysnein arms
C : missing radial spokes
D : abnormal transposition
Tuberculosis and Bronchiectasis
1. Marked degree of caseation necrosis of bronchial walls, particularly when upper lobes are invaded
2. Scarring of larger bronchi can produce bronchial stenosis
3. Extraluminal obstruction of larger bronchi by tuberculous hilar lymphadenopathy
4. Penetration by a calcified tuberculous node into the airway and broncholith formation
5. Healed tuberculous cavities
Clinical Features of Bronchiectasis
Symtoms
sputumchronic coughhemoptysisdyspneafeverchest pain
Signs
cracklesfinger clubbingcyanosiscor pulmonale
• History / Physical examination
• Plain chest X-ray : normal finding in 20%
• HRCT(high resolution CT)
• Bronchogram
Diagnosis
Indications① Preoperative evaluation of unilateral or segmental disease previously identified on CT② Postoperativ evaluation of surgical airway complications such as dehiscence or fistula formation
Differential Diagnosis
• Chronic bronchitis
• Endobronchial tuberculosis
• Endobronchial adenoma
• Right middle lobe syndrome
• Cystic fibrosis
• Allergic bronchopulmonary aspergillosis
• Pulmonary sequestration
• Congenital abnormalities of the trachiobronchial tree
Aim of Treatment
1. Elimination of an identifiable underlying problem
2. Improved clearance of tracheobronchial secretions
3. Control of infection
4. Reversal of airflow obstruction
1. Treatment of predisposing factors hypogammaglobulinemia administration immunogl
obulin tuberculosis anti-tbc medication ABPA steroid2. Bronchial drainage : most important PDPV(postural drainage, percussion, vibration) PEEP deep brathing, continued coughing3. Antibiotics
Treatment : Medical
Treatment : Surgical
Indication
Localized bronchiectasis
Massive/frequent hemoptysis
Intractable infection
Long standing collapse
Adequate residual lung function
Diffuse fibrosis or COPD
Bilateral bronchiectasis
Bronchiectasis complicating asthma
Low FEV1/FVC
Persisting predisposing factors
(e.g. immunodeficiency)
Contraindication
Complications
• Recurrent pneumonia, sinusitis• Lung abscess• Pleuritis, empyema• Emphysema• Brain abscess(rare, but 10% of mortality)• Amyloidosis• Cor pulmonale(common in Korea)• Massive hemoptysis(rare)
• Incidence : Caucasian 1/1,500 – 2,000 Asian 1/90,000• Inheritance : Autosomal recessive trait• Pathogenesis Single gene disorder defective c-AMP mediated regulation of chloride channels -> failure to secete chloride toward the lumen -> unusually viscid mucous secretions -> chronic pulmonary disease and pancreatic insufficiency
Cystic Fibrosis
Cystic fibrosis
1. Sweat chloride test > 60 mEq : confirmative 50 – 60 mEq : highly probable < 50 mEq : normal2. Chronic pulmonary disease (99%)3. Pancreatic insufficiency(80-90%)4. CXR increased interstitial marking (98%) cystic bronchiectasis (64%) hyperinflation(58%)
Diagnosis of cystic fibrosis
Treatment of cystic fibrosis
Infection control pseudomonas(80%) S.aureus, H.influenza Bronchial drainage postural drainage, percussion and vibration forced exhalation technique positive expiratory pressure