Case Presentation

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Case Presentation

• October 13th, 2009
• Breathing Easier
• Neha R. Vagadia, D.O.
• Fellow in Pulmonary/Critical Care Medicine
• Tufts Medical Center

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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.

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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

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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 gt 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.

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Diagnostics
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134
97
20
Segs 72 Bands 26
15.6
168
214
4.7
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1.05
37.5
Ca 8.9, Mg 2.0, Phos 2.4, CK 1016,
Troponin 0.10 Lactate 1.9, LDH 1521 LFTs
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
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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

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Diagnostic Data - CXR

• CXR -

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What next?

• Bronchoscopy
• Chest CT

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Bronchoscopy 6/10

• BAL fluid differential sent but nothing
  remarkable.
• Gram stain was negative.
• Rest of Microbiology pending

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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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Chest CT 6/10
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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.

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Things to consider in this patients management
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IgA DeficiencyPrevalence

• United States
• Selective IgA deficiency ? most common primary
  immunodeficiency,
• Prevalence ranging from 1 in 223-3000

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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.

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IgA DeficiencyDefect

• 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.

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Bronchoscopy results
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The Flu and IgA Deficiency

• Bernard P. Arulanandam, et al
• The Journal of Immunology, 2001, 166 226231.
• 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

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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.

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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

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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

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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)

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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.

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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.

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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.

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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.

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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.

http//www.cdc.gov/flu/professionals/vaccination/v
ax-summary.html
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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).

http//www.cdc.gov/flu/professionals/vaccination/v
ax-summary.htm
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2009 H1N1 Influenza Vaccine

• Pregnant women
• Persons who live with or provide care for infants
  aged lt6 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.

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2009 H1N1 Influenza Vaccine

• A 2009 H1N1 "flu shot"
• 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..

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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

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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.

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Follow up CT scan 6/28
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Follow up CT scan 6/28
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Follow up CT scan 6/28
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Thank you.

• Special thanks to
• Dr. Alex White
• Dr. Heidi OConnor
• Dr. Geraldine Finlay
• Dr. Eric Garpestad
• Please remember to HANDWASH!!

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References

• IgA deficiency clinical correlates and responses
  to pneumococcal vaccine E. Edwards et al. /
  Clinical Immunology 111 (2004) 9397
• 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.
• http//www.cdc.gov/flu/weekly/
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