Interesting Case 11/03/56

อจ.พญ. พนิดา ศรีสันต์ ธิติดา ชัยศุภมงคลลาภ

Interesting Case

เด็กหญิงไทยอายุ 2 ปี 3 เดือน ส่งตัวจากรพ.แห่งหนึ่งด้วย Recurrent pneumonia

PI:

เริ่มป่วยเมื่ออายุ 4 เดือน ไอ หายใจครืดคราด ไม่ได้ admit

อายุ 6 และ 8 เดือน เริ่มหอบ ไอมีเสมหะ ไป admit รพช. เป็นปอดอักเสบ

อายุ 1 ปี 5 เดือน Pneumonia with respiratory failure on ETT 10 วัน

(CXR: hyperinflation, RLL, LUL, LLL infiltration)

อายุ 1 ปี 7 เดือน Pneumonia with respiratory failure on ETT 10 วัน

(CXR: hyperinflation, RML, LUL, LLL infiltration)

อายุ 1 ปี 9 เดือน Pneumonia with respiratory failure on ETT 7 วัน

(CXR: hyperinflation, RUL, RML, LUL infiltration)

อายุ 1 ปี 11 เดือน Pneumonia

(CXR: hyperinflation, RLL, LLL infiltration with atelectasis)

อายุ 2 ปี 2 เดือน Pneumonia

(CXR: hyperinflation, RML, LUL, LLL infiltration)

ระหว่างการป่วยแต่ละครั้ง ยังมีหายใจเร็ว ไอมีเสมหะตลอด ไม่มีไข้

PH:

ไม่เคยมีหูอักเสบ ตุ่มหนอง หรือถ่ายเหลวเรื้อรัง

G&D: ปกติ วัคซีนได้ถึงอายุ 9 เดือน ตัวเล็กมาตลอด กินน้อย

FH: ปฏิเสธ TB contact

1 2 3 4

Physical examination :

T 37.2°_{C , PR 100/min, R 30/min, BP 110/50 mmHg, BW 9.4 kg, Ht 82 cm} GA: alert, tachypnea, dyspnea, no pallor, no jaundice, SpO2 92% RA Heart: normal S1S2, no murmur

Lungs: increased AP diameter, coarse crepitation both lungs Abd : soft, liver and spleen impalpable

Ext : clubbing fingers

CXR

Investigation

CBC : WBC 14510 (N31, L62, Mo1), Hb 11.6, Hct 37.8%, PLT 507,000

LFT : Alb 3.87, Glob 3.9, TB 0.17 , DB 0.14, AST 39, ALT 32, ALP 240

Na 136, K 3.56, Cl 96, HCO

₃

26.5

ABG : pH 7.49, PCO

₂

34.1, PO

₂

71.9, HCO

₃

25.4, BE 2.3

Anti HIV: negative,

Stool fat : negative

UGIS : moderate GER

Bronchiectasis

• Abnormal dilatation of bronchi & bronchioles due to repeated cycles of airway infections & inflammation.

• Thickening, irregular, dilate and herniation (out-pouching of the walls).

• Not a single disease, but the result of many different conditions : genetic, anatomic, systemic causes.

Cycle of infection & inflammation

Cole PJ. Eur J Respir Dis Suppl 1986;147: 6-15.

• Post-infectious

• Bacteria : H.influenza (nontypeable), P. aeruginosa, S. pneumoniae, S. aureus, B. pertussis, K. pneumoniae, M. pneumoniae • Virus : measles, adenovirus, HIV, influenza,

RSV, varicella

• Mycobacteria : TB, MAC • Fungus : Aspergillus

Risk factors

Risk factors

• Mucociliary disorders

• Primary : primary ciliary dyskinesia (PCD) • Secondary : bacterial infection

• Cystic fibrosis (CF) • Obstruction

• Intraluminal : FB, endobronchial mass/tumor • Extraluminal : LN, mass, vascular ring • Rheumatic inflammatory conditions

Risk factors

• Immune dysfunction

• Primary : hypogammaglobulinemia, CVID, SCID, CGD, deficiency of MHC-I, TAP-1, selective Ab • Secondary : malignancy, chemotherapy

• Malnutrition • Extremes of age

• Allergic bronchopulmonary aspergillosis (ABPA)

Risk factors

• Congenital malformation

• William-Campbell syndrome (cartilage deficiency) • Mounier-Kuhn syndrome (tracheobronchomegaly) • Alpha 1-antitrypsin deficiency

• Marfan syndrome • Bronchomalacia

• Congenital lobar emphysema • Miscellaneous

• Aspiration

• Impaired cough : neuromuscular weakness • Smoking, toxic inhalation : NH₃, chlorine

Clinical manifestations

• Chronic cough, usually with purulent sputum • Recurrent pulmonary infections

• Progressive dyspnea, recurrent wheezing • Hemoptysis (bronchial a. neovascularization) • Pleuritic chest pain

• Clubbing fingers

• Crackles, wheezing, rhonchi

Clinical manifestations

• Sinusitis

• Pulmonary hypertension

• Left-to-right shunt (bronchial a. pulmonary a.) • Natural course is dictated by the underlying

etiology, severity at the time of diagnosis, and impact of proactive management programs.

CXR

• Dilated & thickened airways (tram lines) • Ill-defined perihilar linear densities • Irregular elongated opacities, sometimes calcified (mucopurulent plugs)

• Air-fluid level in thick-walled cyst • Surrounding consolidations & atelectasis • Overall lung volume may be increased • Normal 13%

HRCT

• Gold standard for diagnosis, sensitivity 97% • Conventional HRCT :

1-1.5-mm-thick images, every 10 mm. • Internal bronchial Ø >> Ø of accompanying pulmonary artery (signet ring sign)

• Lack of bronchial tapering • Tram-track sign

Tubular (cylindrical) Varicose Saccular (cystic) Reid L. Thorax 1950; 5: 223–247.

varicose

cystic

varicose

Pulmonary function

• Mild to moderate obstructive defect • Progressive worsening airflow obstruction (decrease FEV₁ 1-2% per year)

• Progressive decline in lung diffusion capacity (adjacent lung parenchymal involvement) • Ventilation-perfusion mismatches • Bronchial hyperresponsiveness (30-70%)

Treatments

• General • Medical • Pharmacologic • Nonpharmacologic • Surgical

Pathogenesis & Interventions

General Treatments

• Adequate systemic hydration • Adequate nutrition

• Vaccinations

• Avoid smoking or secondhand smoke • Treat underlying condition if discovered i.e. IVIG for humoral immunodeficiency • Treat associated condition : sinusitis

Pharmacologic Treatments

Antibiotics

• Systemic ATB based on culture & prior infection. • S.pneumoniae, H.influenzae, S.aureus, P. aeruginosa • prolonged course, often >3-4 weeks

• Chronic / prophylaxis with rotating ATB • risk of adverse effects & ATB resistance • Nebulized ATB

• tobramycin, colistin, aztreonam, ciprofloxacin • deliver high concentration into lung with little or no systemic effects.

• Reduce exacerbation & hospitalization in CF

Pharmacologic Treatments

Anti-inflammatory agents

• Systemic corticosteroid for acute flare if BHR & ABPA • Inhaled corticosteroids ? some benefit ?

• sputum volume, QOL, frequency of exacerbation • Long term low-dose macrolides “immunomodulatory” (azithromycin, clarithromycin, erythromycin)

• Θ inflammatory cell migration, cytokine secretion • decrease sputum & reactive O₂ species production • Θ organism’s ability to produce biofilm & toxins • Small but favorable effects on symptoms, but not alter PFT & exacerbation rates (Cochrane Review).

Pharmacologic Treatments

• Promote mobilization of secretions:

• nebulized recombinant human DNase (Pulmozyme) • nebulized acetylcysteine

• hyperosmolar agent: hypertonic saline, mannitol • Bronchodilator + anticholinergic if concomitant BHR. • PPI or H₂-blocker if GERD.

• NSAIDs : ibuprofen, inhaled indomethacin

• reduce persistent neutrophil airway inflammation • Erdosteine (thiol derivative)

• anti-oxidant activity, decrease cough & sputum

Chest physiotherapy (CPT)

Airway clearance techniques, exercise protocols, breathing retraining methods

Bronchial hygiene therapy

Noninvasive airway clearance techniques Lung expansion therapy

Respiratory modalities to increase lung volume, for prevent or correct atelectasis

Surgical Treatment

• Complete resection of affected part of lung : segmentectomy, lobectmy, pneumonectomy • Be considered in localized disease that is

refractory to medical Rx.

• Massive hemoptysis 2° to bronchiectasis & unresponsive to other measures.

• Lung transplantation

Key points of management

• High index of suspicion: chronic wet productive cough  • Establish diagnosis with HRCT  • Search for an underlying cause   • Treat underlying cause  • Treat any exacerbation of respiratory symptoms with  appropriate ATB & augmented chest physiotherapy  • Regular monitor respiratory pathogens to guide Rx choice   • Regular monitor progress: PFT, CXR, consider HRCT, especially  if there are early changes  

Bronchial Hygiene Therapy

1. Postural drainage therapy

- Postural drainage (PD), percussion, vibration 2. Coughing & related expulsion techniques

3. Positive airway pressure (PAP) adjuncts - PEP, CPAP, EPAP

4. High-frequency compression/oscillation methods 5. Mobilization & exercise

Sustained Maximum

Inspiration (SMI)

C A U T I O N

•

Syncope

•

Hyperventilation

•

Respiratory alkalosis

•

Fatigue

Ultrasonic nebulizer

Sustained Maximum Inspiration (SMI)

ทางท่อเจาะคอ

PEP therapy

resistor Face mask

Splinting

airway

during expiration

Positive Expiratory Pressure (PEP) therapy

Collateral ventilation

PEP Mask with Aerosol therapy

PEP Therapy

INDICATION • Atelectasis • Bronchial drainage CONTRAINDICATION • Acute sinusitis • Otitis media • Epistaxis

• Head and face trauma • Increase ICP

• Hemoptysis • pneumothorax

Purse lip breathing: Device

Acapella

Vibratory PEP therapy

Acapella with nebulizer

Ez-PAP

Ez-PAP

EzPAP Therapy

INDICATION

•

Atelectasis

•

Bronchial drainage

•

Decrease WOB

CONTRAINDICATION

• Acute sinusitis • Middle ear disease • Epistaxis

• Head and face trauma • Increase ICP

• Hemodynamic instability • Hemoptysis

• pneumothorax

IPPB

(Intermittent Positive Pressure Breathing)

Non-intubated patients

IPPB Therapy

INDICATION

•

Atelectasis

•

Bronchial drainage

•

Cough training

CONTRAINDICATION • Hypovolemia • Hyperventilation • Tension pneumothorax • Pulmonary hemorrhage

PEP EzPAP

BIPAP CPAP

HFCWO creates a gentle & rapid SQUEEZE & RELEASE action around the chest 5 - 20 times/ sec.

•

ใช้ง่าย

•

สามารถใช้ร่วมกับการพ่นยา

•

Portable

•

ใช้เวลา: 15-30 นาที

Effective Cough Techniques

•

Huff cough (forced expiratory technique)

•

Active Cycle of Breathing Technique (ACBT)

•

Autogenic Drainage

Forced Expiratory Technique

•

“Huff” cough

•

3 second breath hold

•

Open glottis

•

Prevents airway collapse

•

Effective technique for “floppy” airways

•

But difficult for neuromuscular weakness

Active Cycle of Breathing Technique

•

3 steps:

•

Breathing control

•

Thoracic expansion / breath hold

•

Forced expiratory technique

Autogenic Drainage

•

3 phases

•

Unsticking

•

Collecting

•

Evacuating

•

วิธีนี้สอน/เรียนรู้ยากกว่าเทคนิคอื่นๆ

•

ผป.ที่มีอาการหนักมักทําไม่ค่อยได้

Autogenic Drainage

Normal Breathing Complete Exhalation VT RV ERV IRV Cough

UNSTICKING COLLECTING EVACUATING

Autogenic Drainage

ถ้าไอไม่มีประสิทธิภาพ ควรช่วยดูดเสมหะ

Action:

•

ช่วยการไอโดยใช้แรงดันบวกและลบจากเครื่องไปกระทําต่อระบบหายใจของผป.

•

Positive/negative pr. up to 60 cmH₂O

•

ควรให้ผู้ดูแลช่วยทํา

•

Technique independent

•

Portable