1. January 24, 2011

2.  Always look for clubbing  Suprasternal and intercostal retractions may be normal following exercise  Subcostal retractions always pathologic  Accessory muscles  Respiratory alternans- alternating abdominal and chest expansion Respiratory muscle fatigue

3.  1-2 months of age  Afebrile pneumonitis  Congestion, wheezing, fine diffuse crackles  Paroxysmal, staccato cough  Inclusion conjunctivitis in 50%  CBC may have eosinophil predominance

4.  Which CBC results support the diagnosis of Bordetella pertussis? A.High white count with lymphocytosis B.High white count with eosinophilia C.High white count with high bands D.Low white count

5.  3 Phases Catarrhal Paroxysmal  Severe paroxysmal coughing episodes  Followed by cyanosis and apnea  Inspiratory whoop Convalescent  Apnea may be presenting sign  CXR nonspecific  Elevated WBCs with lymphocytosis

6.  Dx with PCR  Treat only to reduce spread Macrolide (treat contacts also)  Tdap vaccine Adults 19-64 years old, one Tdap  At least 2 years after Td

7.  You diagnose a 4 year old patient with 3 rd episode of pneumonia. A dense, opaque area has been seen on CXR in same location all three times. What is most likely diagnosis?  APulmonary Sequestration  BBronchogenic cyst  CCongenital Cystic Adenomatoid Malformation  DRetained foreign body

8.  Think of underlying structural defect!!

9.  Bronchopulmonary Sequestration  Bronchogenic Cyst  Congenital Cystic Adenomatoid Malformation

10.  Portion of lungs perfused by systemic arteries  Lacks normal connection with tracheobronchial tree  Intralobar: Most common lower lobes Anywhere in thorax  Extralobar: Subdiaphragmatic or retroperitoneal Associated with other anomalies

12.  Occur any point along the tracheobronchial tree Centrally located  Does not communicate, fluid fill with wall composed of tissue resembling large airways  Typically present 2 nd decade of life with recurrent wheezing, cough, pneumonia

14.  Previously known as Congenital Cystic Adenomatoid Malformation (CCAM)  Hamartomatous lesion Cystic and adenomatous elements  Resp distress, recurrent PNA, asymptomatic  Surgical resection is symptomatic Controversial if asymptomatic

16.  A 21year old patient is in the ER for pneumonia, which has been recurrent. He has also been treated multiple times for chronic sinusitis. He has required multiple sets of ear tubes for OM, even as an adolescent. He has been married for a year, and has been trying unsuccessfully to have a child. On review of systems, he has had normal growth and development, no bowel or bladder complaints. On exam: bibasilar crackles and ronchi, nasal discharge, nasal polyps. CV exam normal. What is the most likely underlying diagnosis A. Cystic Fibrosis B. Kartagener Syndrome (Primary ciliary dyskinesia) C. Aquired ciliary dyskinesia D. Lymphocytic interstitial pneumonitis

17.  Triad: situs inversus, sinusitis, bronchiectasis Situs inversus in only 50%  Recurrent OM  Nasal polyposis  Recurrent Pneumonia  Infertility

18.  Far more common  May follow LRTI Adeno Mycoplasma RSV Influenza  Need bx of resp epithelium for confirmation

19.  Potentially fatal  Associated with: Cardiac dz Glomerulonephritis (Goodpasture syndrome) Infantile hypersensitivity to cow’s milk Collagen vascular diseases Idiopathic

20.  Dyspnea, cough, hemoptysis, cyanosis, fever, iron-def anemia  Hematemasis or melena  May develop jaundice or clubbing  CXR: variable Transient infiltrates Similar to miliary TB

21.  Dx: hemosiderin-laden macrophages (suggestive) Lung bx definitive

22.  School age children and adults  May imitate viral URI  Progress to lower resp tract Persistent Cough variable, may become productive May progress to consolidation Lower lobes  Physical findings minimal (crackles/wheezing)  Mycoplasma PCR available  Primarily clinical diagnosis

23.  Usually following LRTI  May persist for weeks or months  Loud “honking” “barking”  Disturbing to family members, classmates  Always disappears with sleep  Worse with: Stress Attention to cough  Extensive evaluation may exacerbate problem

26.  Most common cause of inspiratory stridor kids  Acute viral illness Parainfluenza Almost any viral resp illness  Clinical findings Coryza 1-2 days Harsh cough Hoarseness stridor

27.  Mostly self limited  Airway obstruction possible  Treatment Dexamethasone Racemic Epi nebs (vaponeb)  Warm or cool humidified air: no significant benefit

28.  Child treated for croup returns 3 days later looking toxic, stridor has returned Bacterial Tracheitis

29.  An 12 month old has been having daily stridor and intermittent ronchi. Mom was told that her child has laryngomalacia. What is your diagnosis?  A. Laryngomalacia  B. Laryngeal web  C. Hemangioma  D. Chronic aspiration  E. Vascular ring

30.  Congenitally floppy glottis  Symptoms Stridor usually by 1 st week of life Varies with activity May be biphasic Worse supine  Usually self limited  Improvement at 6-10 months of age

31.  Diagnosis Clinical Airway flouro or direct visualization if concerned  Treat for GE Reflux  Refer to ENT if failure to thrive or other complications

32.  Congenital Improves with age  Acquired Endotracheal intubation Problematic May require tracheostomy

33.  Laryngeal papillomatosis Rare Life threatening Presents in first decade Vocal cords or entire tracheobronchial tree Clinical features  Hoarseness  Stridor  Brassy cough

34.  Hemangiomas Stridor, brassy cough Cutaneous hemangiomas may offer clues Now treated with beta-blockers

35.  Laryngeal webs, cysts, laryngoceles Uncommon Clinical manifestations  Resp distress  Feeding difficulties  Stridor  Cyanosis

36.  Clinical Findings Weak or absent cry Hoarseness Insp stridor with/without distress Feeding difficulties  Diagnosis Flexible bronchoscopy

37.  Unilateral Causes  Cardiomegaly  Vascular ring May be subltle  Bilateral Causes  Hydrocephalus  Myelomeningocele  Arnold-chiari malformation

39.  A 13 year old athlete has had wheezing and shortness of breath at multiple track meets this season.  A. PFT’s consistent with asthma  B. PFT’s consistent with restrictive lung disease  C. PFT’s consistent with vocal cord dysfunction  D. Normal PFTs

40.  Often mistaken for exercise-induced asthma  Many have BOTH  Vocal cords adduct during inspiration  Psychogenic origin  Highly pressured adolescent female athlete Competitive event  Sensation of dyspnea “trouble getting air IN” Localized to throat

41.  Diagnosis Clinical suspicion PFT’s Flexible nasolaryngoscopy  Treatment Speech therapists, breathing exercises Psychology may be needed

42.  Increased wheezing in previously well controlled asthmatic or CF patient  Insidious presentation Low-grade fevers Fatigue Weight loss Productive cough  Physical Wheeze Bibasilar crackles clubbing

43.  Imaging Consolidation or atelectasis Dilated bronchi radiating from hila  Diagnosis Skin prick to Aspergillus fumigatus antigen Elevated total and specific IgE Isolation of A.fumigatus from sputum Worsened PFT’s (obstruction)

44.  Recurrent wheezing or stridor  CXR: Right sided aortic arch Tracheal deviation to the Left  Barium swallow Inexpensive screening test  MRI Delineates airway and vascular anatomy Necessary prior to surgery

45.  Don’t forget about Goiter Mediastinal mass

48.  CXR: Large, hyperlucent area Few bronchovascular markings Compression atelectasis of adjacent lobes LUL most common  Middle lobe also seen  Treatment Conservative if infant thriving Resection for prolonged resp distress, recurrent infxn

49.  Any resp infection may cause severe illness  May develop Chronic resp insufficiency Pulmonary HTN Cor pulmonale  Most continue with reactive airways

50.  Bronchioles become inflamed/fibrosed  Non-reversible obstructive lung disease  Causes Idiopathic Post adenoviral Toxic inhalation Conjunction with other diseases  (rheumatoid arthritis) Post-transplant

51.  CXR: hyperinflation decreased vascularity increased interstitial markings areas of atelectasis and consolidation  PFTs: fixed lower airway obstruction  Prognosis is poor