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  • 1. Case Presentation October 13 th , 2009 Breathing Easier Neha R. Vagadia, D.O. Fellow in Pulmonary/Critical Care Medicine Tufts Medical Center
  • 2. History and Physical
    • CC: Shortness of Breath
    • HPI: Pt is a 31 year old male with history of Hypertrophic Cardiomyopathy, IgA Deficiency who presents to OSH with 5 days of worsening shortness of breath. States that began to have symptoms of cough, fever/chills 3 days prior to admission. Symptoms similar to pneumonias in past, but this is significantly worse.
    • ROS: No significant wt changes. Generalized fatigue. No chest pain. Some mild chest tightness. Positive dyspnea, particularly on exertion. No abdominal pain. No N/V/D. No rashes. No lower extremity edema.
  • 3. History
    • Past Medical History:
      • Hypertrophic Cardiomyopathy
        • Gradient of 25%
      • IgA deficiency
      • 2 previous pneumonias
    • Social History:
      • No smoking history
      • No IVDA or ETOH use
      • Lives with wife – who is a nurse. Newborn child of 8 weeks.
    • Medications :
      • None
    • Allergies :
      • NKDA
    • Family History :
      • CAD, DM
  • 4. Physical Exam
    • Vital Signs: T: 101.3, P: 100, BP: 92/60, RR: 40
    • 85% on a Non-rebreather.
    • General: Young male, WD, WN male in apparent distress.
    • Skin: Diaphoretic, no rash obvious. Clearly flushed.
    • HEENT: Non-icteric, EOMI. PERLA. Oropharynx negative for erythema or exudate.
    • Neck: Supple. No LAD. No JVD
    • Lungs: Diffuse ronchi in the RUL. Diminished breath sounds on the Left > right. No wheezes or rales.
    • CVS: S1, S2 normal with RRR.
    • Abdomen: Positive BS. Mildly obese. NT, ND. No hepatosplenomegaly
    • Extremities: No edema, no cyanosis, or clubbing.
  • 5. Diagnostics 15.6 13 37.5 168 Segs 72 Bands 26 134 4.7 97 28 20 1.05 214 Ca – 8.9, Mg – 2.0, Phos – 2.4, CK: 1016, Troponin: 0.10 Lactate: 1.9, LDH: 1521 LFT’s: AST -115, AP – 60, ALT – 37, TB: 0.8 Amylase: 135, Lipase: 38 IgA and IgG levels were confirmed low. EKG: Sinus Tachycardia. Regular rhythm
  • 6. Hospital Course
    • Patient arrived here in clear respiratory distress.
    • ABG done and finally due to increased work of breathing and clear hypoxia  patient intubated.
    • ABG: 7.47/36/66/26/ on non-rebreather.  7.33/51/79/26 on 100% Fi02
  • 7. Diagnostic Data - CXR
    • CXR -
  • 8. What next? Bronchoscopy Chest CT
  • 9. Bronchoscopy 6/10
    • BAL fluid differential sent but nothing remarkable.
    • Gram stain was negative.
    • Rest of Microbiology – pending
  • 10. Chest CT – 6/10
  • 11. Chest CT – 6/10
  • 12. Chest CT – 6/10
  • 13. Chest CT – 6/10
  • 14. Chest CT – 6/10
  • 15. Chest CT – 6/10
  • 16. Chest CT – 6/10
  • 17. Chest CT – 6/10
  • 18. Chest CT – 6/10
  • 19. Chest CT – 6/10
  • 20. Chest CT – 6/10
  • 21. Chest CT – 6/10
  • 22. Chest CT – 6/10
  • 23. Chest CT – 6/10
  • 24. Chest CT – 6/10
  • 25. Chest CT – 6/10
  • 26. Chest CT – 6/10
  • 27. Chest CT – 6/10
    • Extensive areas of pulmonary parenchymal opacification, with only a small portion of the upper lobes remaining aerated. Consistent with extensive collapse and/or pneumonia, involving both lower lobes and much of the right middle lobe and both upper lobes with hemorrhage not excluded.
    • Mildly prominent mediastinal lymph nodes, perhaps reactive.
  • 28. Things to consider in this patient’s management…
  • 29. IgA Deficiency Prevalence
    • United States
    • Selective IgA deficiency  most common primary immunodeficiency,
    • Prevalence ranging from 1 in 223-3000
  • 30. Clinical Manifestations
    • Vast majority of IgA deficient individuals (85 to 90 percent) are asymptomatic
    • Some are predisposed to develop one or more of the following:
      • Recurrent Sino pulmonary infections (those with concurrent IgG deficiency)
      • Autoimmune antibodies and/or disorders
      • Gastrointestinal infections and disorders (those with absent secretory IgA)
      • Anaphylactic transfusion reactions (those with anti-IgA antibodies)
    • Those with concomitant IgM or IgG subclass deficiency present more frequently with recurrent infections.
  • 31. IgA Deficiency Defect
    • Humoral immune deficiency
      • Impaired molecular defect intrinsic to B cells or a failure of interactions between B and T cells.
    • Helps the phagocytic arm of the immune system located in the mucosal areas, which is why thought to prevent against recurrent respiratory and gastrointestinal infections.
    • Binds foreign antigens which then binds to a receptor located on neutrophils, eosinophils, and macrophages. The organism is then ingested and destroyed.
  • 32.  
  • 33. Bronchoscopy results
  • 34. The Flu and IgA Deficiency
    • Bernard P. Arulanandam, et al:
      • The Journal of Immunology, 2001, 166: 226–231.
        • IgA deficient mice displayed impaired T cell priming to the H1N1 subunit vaccine, with concomitant reduction in recall memory responses due to a defect in APC function.
        • Provided evidence that a major role of IgA is to facilitate presentation of Ag to mucosal T cells.
        • IL-12 treatment can overcome IgA deficiency by providing adequate T cell priming during vaccination
  • 35. Treatment
    • For those with recurrent infections: General focus is management of infections.
    • Six month course of daily prophylactic antibiotics can be used for those with continued infections.
    • If this fails trial of gamma globulin replacement therapy may be warranted.
  • 36.  
  • 37.  
  • 38. Influenza
    • Influenza A and B are two types of influenza viruses that cause epidemic disease
    • Influenza A
      • Hemagglutinin
      • Neuraminidase
      • H1N1, H3N2, reassortment H1N2,
    • Influenza B
      • Yamagata and Victoria
    • Antigenic shift occurs when a new subtype of influenza A virus appears and can result in the emergence of a novel influenza A virus i.e.. H1N1
  • 39. H1N1
    • This novel virus is derived partly from influenza A viruses that circulate in swine and is antigenically distinct from human influenza A (H1N1) viruses in circulation since 1977.
    • First detected in people in the United States in April 2009
  • 40. Treatment
    • Most people who get the flu (either seasonal or 2009 H1N1) will have mild illness and will recover in less than 2 weeks. They do not need medical care or antiviral drugs.
    • Empiric therapy and therapy should be considered in those with suspected influenza and either severe symptoms or potential for complications.
    • Those at higher risk for complications including:
      • Children younger than 2 years old;
      • Persons aged 65 years or older
      • Pregnant women
      • Persons of any age with certain chronic medical or immunosuppressive conditions (blood disorders, kidney disease, cancer, chronic lung disease, liver disease, heart disease, neurologic disorders, or weakened immune systems)
  • 41. Treatment
    • When indicated, should be initiated as early as possible  within 48 hours of illness for most benefit.
    • Treatment should not wait for laboratory confirmation of influenza.
    • Laboratory testing can delay treatment and a negative rapid test for influenza does not rule out influenza.
      • The sensitivity of rapid tests in detecting 2009 H1N1 has ranged from 10% to 70%.
    • Antiviral chemoprophylaxis 
      • Persons at higher risk for influenza-related complications
      • Those who have had contact with someone likely to have been infected with influenza.
  • 42. Treatment
    • H1N1 influenza viruses likely will be the most common influenza viruses circulating this season, but circulation of seasonal influenza viruses is also expected. 
    • Currently circulating 2009 H1N1 viruses  susceptible to oseltamivir and zanamivir, but resistant to amantadine and rimantadine;
    • Antiviral treatment regimens might change according to new antiviral resistance or viral surveillance information.
  • 43. Prevention:
    • Influenza vaccination is the most effective method for preventing influenza virus infection.
    • Beginning each September, or even earlier if vaccine is available, the flu vaccine should be offered
    • Both the Live, Intranasal Influenza Vaccine (LAIV) and Trivalent Inactivated Influenza Vaccine (TVIV) contain strains of influenza viruses that are antigenically equivalent to the annually recommended strains: one influenza A (H3N2) virus, one influenza A (H1N1) virus, and one influenza B virus.
  • 44. Seasonal Vaccination
    • Trivalent Inactivated Influenza Vaccine (TIV)
      • TIV is injected into the muscle of the upper arm or thigh.
      • It can be used for people 6 months of age or older, including healthy people, those with chronic medical conditions, and pregnant women.
      • The viruses in the injectable influenza vaccine are inactivated so they do not cause influenza.
    • Live, Intranasal Influenza Vaccine (LAIV)
      • LAIV is given as a nasal spray. It can be used for healthy people 2-49 years of age who are not pregnant.
  • 45. Summary of seasonal influenza vaccination recommendations 2009: Adults
    • People recommended for vaccination based on their risk of complications from influenza or because they are in close contact with someone at higher risk of influenza complications include:
      • Children aged 6 months until their 5th birthday,
      • Pregnant women,
      • People 50 years of age and older,
      • People of any age with certain chronic health conditions (such as asthma, diabetes, or heart disease),
      • People who live in nursing homes and other long-term care facilities,
      • Household contacts of person at high risk for complications from influenza,
      • Household contacts and out of home caregivers of children less than 6 months of age, and
      • Health care workers.
  • 46. Vaccination
    • People who should NOT be vaccinated include:
      • People who have a severe allergy to chicken eggs,
      • People who have had a severe reaction to an influenza vaccination,
      • People who have developed Guillian-Barré syndrome within 6 weeks of getting an influenza vaccine,
      • Children less than 6 months of age (influenza vaccine is not approved for this age group), and
      • People who have a moderate to severe illness with a fever (they should wait until they recover to get vaccinated).
  • 47. 2009 H1N1 Influenza Vaccine
    • Pregnant women
    • Persons who live with or provide care for infants aged <6 months (e.g., parents, siblings, and daycare providers)
    • Health-care and emergency medical services personnel
    • Children and young adults aged 6 months-24 years
    • Persons aged 25--64 years who have medical conditions that put them at higher risk for influenza-related complications.
  • 48. 2009 H1N1 Influenza Vaccine
    • A 2009 H1N1 &quot;flu shot&quot; —
      • Inactivated vaccine (containing killed virus) muscle injection.
      • The indications are the same as for seasonal flu shots.
      • Approved for use in people 6 months of age and older, including healthy people, people with chronic medical conditions and pregnant women.
    • The 2009 H1N1 nasal spray flu vaccine —
      • live attenuated influenza vaccine (live, weakened viruses) .
      • The indications are the same as for seasonal nasal spray vaccine. LAIV is approved for use in healthy* people 2 years to 49 years of age who are not pregnant..
  • 49. What to look out for
    • The symptoms of novel H1N1 and seasonal influenza are similar: Fever, cough, headache, sore throat, anorexia, myalgias, lethargy, and sometimes vomiting and diarrhea. However, 10% to 40% of persons with novel H1N1 influenza may not be febrile
    • Of Boston residents hospitalized with confirmed novel H1N1 influenza, 49% had underlying asthma and 37% required ICU care
    • One thing that appears to be different from seasonal influenza is that adults older than 64 years do not yet appear to be at increased risk of 2009 H1N1-related complications thus far about one-third of adults older than 60 may have antibodies against this virus
  • 50. Hospital Course Continued
    • Patient treated with empiric antibiotics – broad spectrum. Then placed on oseltamivir for 5 days with the results of the bronchcoscopy.
    • Flu swab was negative but culture was positive for Influenza A and presumed H1N1.
    • Patient remained on the vent for 4 weeks.
    • Went on to get trach and peg transferred out to an LTAC.
    • Now awake, walking, decannulated and able to hold his 4 month old child.
  • 51. Follow up CT scan 6/28
  • 52. Follow up CT scan 6/28
  • 53. Follow up CT scan 6/28
  • 54. Follow up CT scan 6/28
  • 55. Follow up CT scan 6/28
  • 56. Follow up CT scan 6/28
  • 57. Follow up CT scan 6/28
  • 58. Follow up CT scan 6/28
  • 59. Follow up CT scan 6/28
  • 60. Follow up CT scan 6/28
  • 61. Follow up CT scan 6/28
  • 62. Thank you.
    • Special thanks to:
    • Dr. Alex White
    • Dr. Heidi O’Connor
    • Dr. Geraldine Finlay
    • Dr. Eric Garpestad
    • Please remember to HANDWASH!!
  • 63. References
    • IgA deficiency: clinical correlates and responses to pneumococcal vaccine E. Edwards et al. / Clinical Immunology 111 (2004) 93–97
    • Bernard P. Arulanandam, et al. IgA Immunodeficiency Leads to Inadequate Th Cell Priming and Increased Susceptibility to Influenza Virus Infection. Journal of Immunology. 2001 226-229
    • Frederick M. Schaffer, MD. Clinical assessment and management of abnormal IgA levels. Annals Of Allergy, Asthma & Immunology. Volume 100, March, 2008.
    • BOSTON PUBLIC HEALTH COMMISSION ▪ INFECTIOUS DISEASE BUREAU Communicable Disease Control Division 1010 Massachusetts Avenue ∙ Boston, MA 02118