[CIS-PAGID] (no subject)

[Junker, Anne]

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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