[PAGID] Patient Query

[Brynn Wainstein]

I wonder if I can ask about an interesting young boy I have seen? He is
a 7 year old Lebanese boy who is the son of 1st cousins. He developed
severe 'bronchiolitis' at age 1 and required an ICU admission. For the
next year he was in and out of hospital with respiratory infections.
Then he re-presented in 2005 with a liver abscess which was found to be
Staph Aureus and responded to drainage and flucloxacillin. He has had no
further liver or other abscesses. A NBT done in 2005 was normal. He then
disappeared again until January this year when he presented with a
febrile respiratory illness. The symptoms came and went over the next
few months until he was referred to me in May. When I saw him he was
well but I found that he had profound palmar plantar hyperkeratosis and
bilateral fine inspiratory crepitations with no clinical evidence of
chronic lung disease. His teeth and gums are normal and there was
nothing else to find on physical examination. I performed some immune
investigations (attached) and ordered a high res chest CT. When he
returned for review a few weeks later the crepitations had completely
disappeared and his CT was reported as normal with a small area of
atelectasis in the lower lobe. No evidence of bronchiectasis or
fibrosis. I also found that he had a high IgE level (attached) and
eosinophilia but has no clinic evidence of atopy or positive
specific-IgE tests.

We considered PLS but his gums are normal. Could this be HIES? <<A
El-D.pdf>> 

I would appreciate your thoughts and comments.

Thank you.

Regards,

Dr Brynn Wainstein
Paediatric Clinical Immunologist
Staff Specialist 
Sydney Children's Hospital
Tel: +61-2-9382-1515
Fax: +61-2-9382-1580




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