[CIS-PAGID] (no subject)

[Church, Joseph]

Are the episodes associated with eosinophilia (Gleich Syndrome)?

 

Joe Church

Children's Hospital Los Angeles

 

________________________________

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Junker, Anne
Sent: Tuesday, June 21, 2011 2:45 PM
To: 'pagid at list.clinimmsoc.org'
Subject: [CIS-PAGID] (no subject)

 

I would appreciate advice on management of this 4 yo boy who has been
admitted for the 3rd time for rapid onset of generalized edema during
the recovery phase of a mild upper respiratory tract infection with low
grade fever (Jan; Mar-influenza B; June-strep throat).  These episodes
start with periorbital edema which becomes pronounced, then progresses
over 2-3 days to involve face, then limbs.  During episodes he has
maintained his blood pressure but his heart rate increases.  Labs shows
increasing hemoconcentration, to Hb 165 (normal range, 105-135g/L);
falling albumin, to 23 (normal, 34-42), low C3 of 0.45 (normal
0.8-1.54).  With careful fluid management he diureses and recovers over
3-5 days and these values all normalize.  He is otherwise completely
well.  Other studies have shown normal renal function and no
proteinuria, normal C1 esterase, normal C4 and normal Ch50s.  His CRP
and ESR during episodes are low/normal.  He has normal IgG, IgA, IgM and
IgE; normal T, B and NK cell numbers; and normal mitogen-induced
lymphocyte proliferation.  He makes specific antibodies.  He has no
monoclonal gammopathy.  Our working diagnosis is systemic capillary leak
syndrome.  On this last occasion we gave him 1G/kg IVIg and he made a
rapid recovery with dieresis starting during infusion. 

Your thoughts are welcome.

 

Thanks, anne

email: ajunker at cw.bc.ca

 

Anne K. Junker, MD, FRCP(C)

Associate Professor, Pediatrics

Director, Clinical Immunology Service, BC Children's Hospital

Director, Clinical & Population Studies, Child & Family Research
Institute

Director, Maternal Infant Child Youth Research Network of Canada/Reseau
de Recherche en Sante des Enfant et des Meres (MICYRN)

K4-223 4480 Oak Street

Vancouver, British Columbia

Canada  V6H 3V4

phone: 604-875-3591

fax: 604-875-2414

 



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