[CIS PIDD] [cis-pidd] Sibs with low IgG, normal Pneumo Abs

[Brynn Wainstein]

I am looking after a very similar patient. 5 year old boy with recurrent non-severe upper respiratory infections that could almost be 'normal' but are perhaps a bit frequent. He has shown a progressive drop in his IgG from ~7 g/L over a year ago to ~4 g/L and now ~2.8 g/L. IgM normal, IgA low but measurable. But his vaccine responses to pneumococcal and protein antigens are excellent. He is otherwise well but is thought to have non-celiac 'gluten intolerance' so I have wondered about PLE but his stool alpha1AT is not elevated. Reminds me of the question that was asked recently in Miami whether people feel that there is an IgG level below which we should always treat despite normal vaccine responses in a clinically 'well' child? I would appreciate some advice on that question as I have decided not to treat for the time being while the gastroenterologist works out whether he does or does not have an enteropathy. Also his T, NK and B subsets are completely normal.

Regards,

Dr Brynn Wainstein.
Sydney Children's Hospital

-----Original Message-----
From: Sokolic, Robert (NIH/NHGRI) [E] [mailto:sokolicr at mail.nih.gov] 
Sent: Thursday, 9 May 2013 8:26 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] Sibs with low IgG, normal Pneumo Abs

Would also get endoscopic ENT exam for anatomic abnormalities.

Sent from my iThing

On May 8, 2013, at 10:13 PM, "Church, Joseph" <JChurch at chla.usc.edu<mailto:JChurch at chla.usc.edu>> wrote:

Colleagues:

I am following a sister (4yo) and her brother (2½yo) with recurrent URIs and otitis media from <1year of age.  They have not experienced pneumonias or other invasive infections.  Their growth and development have been normal.  Their physical examinations have been normal except for evidence of chronic ear/sinus disease.

Labs on both show normal CBCs etc.

Sister (4+ yo):
                IgG repeatedly (from 1yo) below normal range; currently 484 mg/dl
                IgA 35, IgM 107
                IgE (in past) 17 IU/ml
                Tetanus and Hib antibodies protective
                Mannose binding lectin normal
                Pneumococcal antibodies after earlier Prevnar and then Pneumovax, + for 18 of 23 serotypes tested, stable for > 1 year.

Brother (2 ½ yo):
                IgG repeatedly below normal range; currently 367 mg/dL
                IgA 49, IgM 55
                IgE 17 IU/mL
                Tetanus antibody protective; Hib antibody initially protective, now non-protective.
Pneumococcal antibodies after earlier Prevnar and then Pneumovax at 2yo, + 19 of 23 serotypes tested at 1 month, now +16 of 23 at 4 months post pneumovax.

This past winter was difficult for both kids.

WHAT WOULD YOU SUGGEST:
                          TREAT AT THIS TIME (if I can get insurance to cover)?
                          CHECK ANTIBODIES IN 4 MONTHS?  IF DECREASED TREAT?
                          WHAT IF THEY MAINTAIN THEIR CURRENT IgG LEVELS AND PNEUMOCOCCAL ANTIBODIES, BUT CONTINUE TO BE SICK?

I would appreciate any thoughts or comments.  Thanks.

Joe Church
Children's Hospital Los Angeles




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