Pediatric Immunology: Phagocyte Defects


Vignette #4
·         A 3-week old girl is brought to the clinic because her parents are concerned about some swelling and redness around her umbilical cord stump. She is otherwise well and has been feeding appropriately, she has about 6 wet diapers and 3 soiled diapers per day. She was born term to a G2P2  mother via planned C-section, birth weight was 7#9(54%ile). Stable, family history is noncontributory, Presently baby is running a slight fever at 100.4oF but is otherwise vitally stable, she appears well /NAD. She weighs 8#12. On exam, you note mild erythema surrounding the intact umbilical stump. Exam otherwise unremarkable. Electrolytes are normal CBC is notable for a WBC of 58,000 µL and elevated ANC. peripheral smear appears normal. Quantitative immunoglobulins, C3, and C4 levels are still all within normal parameters.

Leukocyte Adhesion Deficiency
·         Rare, autosomal recessive disorder affecting the expression of integrins on the cell surface neutrophils
·         Certain integrins are needed for WBC
·         Without these certain integrins, the body cannot respond to sites of infection and otherwise innocuous infection becomes severe.
·         Pts typically present early on the life w/delayed umbilical cord detachment and omphalitis.

·         Their WBC count and ANC will be high through any infection.

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