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MORNING REPORT

Lauren Zager—1/23/19
TC presents with 2 weeks of congestion, headache, intermittent
abdominal pain and 1 week of fever, myalgia, malaise minimally
productive cough. Seen at her PCP where she was febrile and
hypoxic to the 80s. CXR showed bilateral pulmonary infiltrates.
She was admitted to an OSH and started on azithromycin and
ceftriaxone.
At the OSH, she continued to have high spiking fever with some
cough but no sputum. She had rapidly worsening WOB and
CHIEF
progressed to respiratory failure requiring intubation. Her labs
were significant for high WBC of 19 with elevated CRP and COMPLAINT:
sputum/saliva cx which was found to be growing fungus. She was
transferred to PCH for further care due to respiratory failure and
concern for invasive fungal infection.
RESPIRATORY
Fungal plate was described as 8-10 blue-green fluffy colonies FAILURE
REVIEW OF SYSTEMS:
Positives: Fever, emesis, headache, cough, and respiratory distress
Negatives: diarrhea, rash, URI symptoms, Sick contacts
Vitals: Temp 36.9, HR 100, RR 38, BP 135/92, SpO2 98% on SIMV
Gen: Adolescent girl laying in hospital bed, ill appearing
HEENT: NC/AC, PERRL, conjunctiva clear, MMM, ETT in place
Cardiac: mild tachycardia, no murmur, central cap refill <3 sec
Resp: bilateral coarse breath sounds, equal air entry, mild subcostal
retractions

PHYSICAL EXAM Abd: soft, NT/ND, no hepatosplenomegaly, BS present


Derm: no rashes, jaundice or pallor
Neuro: medically sedated, withdraws to painful stimuli

CXR on admission to PCH: diffuse patchy, hazy, nodular pulmonary


opacities
• Term birth, NSVD without complication
Past Medical and • Normal development
• Ureteral reflux as a child with intermittent UTIs, most recent 1.5 years ago
Surgical Hx • Salivary gland removal at age 4 due to infection unresponsive to abx
• Vaccinations UTD

Family Hx • No family history of Immunodeficiency/Recurrent infections/Autoimmune disease


• Sibling recently diagnosed with Salmonella diarrhea

• Lives in Idaho with parent, step-parent and 5 siblings.


Social Hx • Father works pruning trees
• Visits other biological parent every other weekend in Wyoming

• Lives on a farm, contact with dogs, cats, chickens, horses, ducks, live stock
• Recently went camping and hunting, no known tick or other insect bites

Exposure Hx • No contact with dead/decomposing animals or animal skins


• Family spread locally produced wood chips with visible mold in the yard
• No raw meat, unpasteurized milk or unpurified water
• No recent travel outside Idaho/Wyoming
DIFFERENTIAL DIAGNOSIS: INFECTIOUS DISEASE
Fungi Viruses Bacteria
 Histoplasmosis  Hantavirus  Mycoplasma
 Blastomycosis  HIV  Legionella
 Coccidioides Cryptococcus  Coxiella (Q fever)
 Aspergillus  Strep pneumo
 Pencillium  MSSA/MRSA
 Candida  Chlamydia psittaci
 Pneumocystis
 Brucellosis
 Rhodococcus equi
 Nocardia
 Burkholderia
 Serratia
DIFFERENTIAL DIAGNOSIS BEYOND INFECTIOUS DISEASE…

Rheum Immunology Pulmonology Other


 Lupus  Hyper-IgE syndrome  Hypersensitivity  Trauma
 HLH/MAS  Hyper-IgM syndrome pneumonitis  Fat embolism
 Goodpastures  Chediak-Higashi  Farmer’s Lung  Bilateral lung contusions
 Granulomatosis w/ syndrome  Bird Fanciers Disease  Burns
Polyangiitis  Chronic Granulomatous
Disease  TRALI
 Common Variable  BMT
Immunodeficiency  Pancreatitis
 Leukocyte Adhesion  Near drowning
Deficiency  Air/Amniotic fluid
embolism
 Drugs/Medications
BAL studies Legionella Urine Antigen NEGATIVE
- Pneumocystis DFA NEGATIVE/PCR NEGATIVE Histoplasma Urine Antigen NEGATIVE
LABS AND MICRO

- Legionella PCR NEGATIVE Coxiella burnetii Abs ALL NEGATIVE


- Galactomannan NEGATIVE HIV 1/2 NONREACTIVE
- Viral Respiratory Panel PCR NEGATIVE Hantavirus antibodies <2.0 (NEGATIVE)
ANA NEGATIVE Mycoplasma pcr throat NEG
Cocci antibody panel: <1:2, IgG 0.6, IgM 0.5 1,3 BDG 199 (POSITIVE)
(NONE DETECTED)
Galactomannan antigen neg, BAL 6/30
Cryptococcus Antigen serum NEGATIVE
Chlamydia Ab panel (IgG/IgM) : all IGMs negative
Galactomannan serum NEGATIVE

OSH sputum cx 6/23: Prelim has Aspergillus fumigatus Neutrophil Oxidative Burst : Positive, consistent with
from outside hospital (cultures at ARUP) autosomal recessive CGD
6/24 BAL Respiratory cultures NGTD,
No AFB after 1 week
6/29 Trach aspirate: Aspergillus
Legionella cx pending
6/30 BAL NGTD
Required VV ECMO cannulation on HD 2
Worsening ARDS to the point of complete “white out” of bilateral
lung
Required escalation to VA ECMO support on HD 7
Diagnosis of Chronic Granulomatous Disease w/ Mulch Pneumonitis
and Pneumonia
Steroids initiated on HD 8 in addition to double aspergillus
coverage and broad antibiotic coverage (meropenem and septra)
Dramatic improvement in clinical status, decannulated from ECMO on
HD 13 and extubated HD 16

OUR PATIENT’S COURSE


CHRONIC GRANULOMATOUS DISEASE
CAUSE—INHERITANCE—DIAGNOSIS
Caused by defects in the phagocyte NADPH oxidase, which constitutes the oxidative burst
X-linked (65%) and Autosomal recessive (35%) inheritance

Nitroblue tetrazolium test (NBT) Flow cytometric reduction of dihydrorhodamine


CHRONIC GRANULOMATOUS DISEASE –
PRESENTATION, COMMON INFECTIONS, TREATMENT

COMMON PRESENTING PARTICULARLY SUSCEPTIBLE


TO CATALASE-POSITIVE PREVENTING INFECTIONS CURE
INFECTIONS ORGANISMS
• Skin/perianal abscesses • Staph aureus • Daily antibacterial and • The only cure for CGD is bone
• Lymphadenitis • Serratia antifungal prophylaxis ex. marrow transplant
• Pneumonia • Burkholderia Septra/Bactrim and an –
• Liver abscesses • Nocardia azole
(Posaconazole/Itraconazole)
• Osteomyelitis • Aspergillus species • Interferon gamma injections,
• Sepsis • Salmonella which stimulate superoxide
• Klebsiella release and improve
• Aerobacter microbicidal function of
• Actinomyces phagocytes