[CIS PIDD] [cis-pidd] Neonatal Autoinflammatory Syndrome

[Richard Wasserman]

I was asked to see a five week old boy status post truncus repair
(microarray pending) because of recurrent episodes characterized by low
grade fever, profound capillary leak and hypotension (some episodes have
required pressors). The first episode was treated with antibiotics as rule
out sepsis and glucocorticoids because of a concern about hypoadrenalism.
The episode lasts several days and then resolves. All cultures and viral
studies have been negative. During the episodes there is mild
granulocytosis and a mildly elevated CRP of 4.5 (normal <0.5). Fevers have
always been mild. In between episodes the granulocyte count is normal and
the CRP is <0.5. There is mild lymphopenia (ALC about 1300).

The child is not dysmorphic. Specifically, there has never been any rash or
eye findings. Except for the cardiac problem, the exam is normal. His only
other problem is feeding intolerance.thought to be due to bowel edema
during the episodes.

I have never seen NOMID but this doesn't sound like it. I am out of
suggestions for the neonatologists.

Thank you,
Richard Wasserman

-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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