Hogan-1st Case Flashcards
3.5 month old with 12 week history of cough. Admitted to outside hospital at 1 month of age for for “bronchitis”, thrush and diarrhea and given IV antibiotics. 3 different IV antibiotics tried each trial lasted 10 days.
She has been increasingly blue at home for 3 weeks.
Increasing difficulty in breathing
Diarrhea presumed from IV antibiotics
What are you worried that this child might have? Where do you refer the child?
Worried about immunodeficiency b/c of the repeated infections. Perhaps infections in lungs to the point that she is hypoxic. Diarrhea consistent to SCID.
**Worried about SCID maybe & refer to the ER for emergency of hypoxia.
Why might this child be suspected of having a heart defect?
b/c the oxygen saturation was 45%–crazy low!!!
Why would you be concerned about a 3.5 mo old have an IgG level of 61 mg/dl?
You would be concerned b/c this is a low level. & up until 6 mo of age–the mom’s IgG should be enough to sustain the child. is there a consumptive problem going on b/c of all of the kid’s infections???
Height <5%, HC 50%
What does this show?
Failure to thrive w/ a normal head circumference.
What does candida esophagitis make you think?
T cell problem. maybe HIV from mom
What is the sail sign on a neonatal chest X-ray?
this indicates the presence of a thymus.
if it is absent–probably have serious T cells problems (and therefore B cells problems)
So you have this kid with terrible recurrent lung infections…what do you do next?
you get a lung biopsy so you know exactly which organisms is infecting them.
hopefully by bronchoscopy
a positive silver stain of the lung indicates what?
pneumocystis carnii
How might you initially treat this child?
IV Foscarnet: anti-viral for CMV pneumonitis
Amphotericin B: anti-fungal for invasive Candida esophagitis
Bactrim/IV Solumedrol: Pneumocystis carinii pneumonitis per HIV protocol at time.
**soon a bone marrow transplant from mom b/c close MHC I match via histocompatibility
What are the symptoms of T cell deficiencies that this child displays?
onset before 6 mo opportunistic infection no lymph nodes hepatosplenomegaly failure to thrive diarrhea
How could you see graft v. host disease in an infant?
if there is a messy vaginal birth–sometimes T cells are transferred from mom to kid.
What type of immunodeficiency is HIV?
secondary immunodeficiency
Are you worried if a 3.5 mo kid has IgA<7?
No, b/c of the young age. Any IgA is pretty good.
No functional IgG against CMV
No functional IgM against CMV
Which of these is concerning for a 3.5 mo kid?
the no fcnl IgM is more concerning. b/c she should be making this by now, not IgG yet.
What does this flow cytometry indicate? CD3 – CD4- CD8- CD 19 + CD 56 -
They don’t have a B cell problem.
They seem to have a T cell problem and an NK problem.
