with recombinant human p50, one member of the classic NF-kB heterodimer, showed decreased gel mobility of theputative NF-kB binding site oligonucleotide. Specificity ofthe binding was demonstrated by competition with unla-belled oligonucleotides representing a consensus NF-kBbinding site and the gp91phox-derived sequence, but notby the recognition sequence for an unrelated DNA-bindingprotein. We conclude that NF-kB is an important transcrip-tion factor for the induction of gp91phox gene expressionand activation of the human NADPH oxidase system.

doi:10.1016/j.clim.2006.04.311

Sa.80. Lamotrigine-Associated ImmunoglobulinDeficiency and Urticaria.Robert Roberts, YehSheng Wang. Pediatrics, UCLA, LosAngeles, CA.

Antiepileptic drugs, particularly phenytoin, has beenassociated with immunoglobulin deficiency. We report on a14 year old male who developed a severe urticarial rashafer taking the drug lamotrigine and found to have nomeasurable immunoglobulin A or immunoglobulin E. Thepatient had been diagnosed with a severe, multiple seizuredisorder localized to the left hemisphere. He underwent aleft hemispherectomy at 34 months which greatly reducedhis seizure frequency. However, antiepileptic medicationswere still required and his was tried on phenytoin,phenobarbital, and carbamazepine. He was switched tolamotrigine in 2002 which was effective in controlling hisseizures. In the fall of 2005, he developed an urticarialrash that becoming progressively worse, particularly atnight. Antihistamines gave little relief but he responded toa course of prednisone. His health was otherwise stableexcept for chronic orthopedic problems. Physical examshowed and evanescent urticarial rash on his abdomen andlegs. Laboratory studies revealed he has an undetectableIgA and IgE. Ig G and IgM levels were with normal limits. Band T-cell subclasses were normal but C-reactive proteinwas elevated. Rashes occur commonly with lamotrigine,and there has been one report of common variableimmunodeficiency. We presume both the urticaria andhypogammaglobulinemia are due to lamotrigine althoughthe parents were reluctant to stop the drug because of thedifficulty in controlling the seizures. The urticaria is notdue to IgE which is very low but must be cell-mediated. Wewill continue to follow the patient closely as rashesinduced by lamotrigine sometimes precedes hemophagocy-tic syndrome. It may be useful to measure immunoglobulinlevels prior to starting lamotrigine so that immunedysfunction can be assessed if it occurs.

doi:10.1016/j.clim.2006.04.312

Sa.81. Differential Effects of GM-CSF UponNeutrophil Activation as Evaluated By FlowCytometric and Morphological Assays.Mickey Ni, Vicky Tai, Robert Roberts. Pediatrics, UCLAMedical Center, Los Angeles, CA.

RATIONALE: Human granulocyte-macrophage colony stim-ulating factor is a cytokine that plays a major role in theactivation and survival of neutrophils, eosinophils, andmonocytes. When applied topically GM-CSF has been shownto improve wound healing particularly in patients withinherit defects in neutrophil function. METHODOLOGY: Inorder to quantify the effects of GM-CSF on neutrophilactivation, we monitored their morphology and utilizedflow cytometric analysis at various concentrations. We firstisolated white blood cells from whole blood by centrifugingthrough a 75% Percoll gradient. After the leukocytes wereextracted from the buffy coat, uniform cultures diluted to1.0 � 106 cells/mL were prepared. These cultures were thensubjected to increasing concentrations of GM-CSF andincubated over various increments of time (1 hour, 2 hours,24 hours). After the allotted time, further activation of theleukocytes was prevented with the addition of gluterade-hyde. Activation through morphology was then determinedby sampling 100 neutrophils to observe morphologicalelongation and the presence of pseudopods at the variousconcentrations of GM-CSF. Flow cytometry was performed tomonitor neutrophil activation through increasing CD16receptor expression. RESULTS: Preliminary results indicatethat GM-CSF induces morphological changes in neutrophilsthat may be monitored observation and by flow cytometry.CONCLUSIONS: With these findings we hope to observe acorrelation between activated neutrophils and concentra-tions of GM-CSF. Hopefully these in-vitro studies can be usedto apply in-vivo with the use of topical GM-CSF in chronicwound healing.

doi:10.1016/j.clim.2006.04.313

Sa.82. Long-Term Enzyme Replacement forAdenosine Deaminase-Deficient Patient-ImmuneFunction and Outcome.Maitham Husain,1 Alessandro Aiuti,2 Adelle Atkinson,1 EyalGrunebaum,1 Chaim Roifman.1 1Allergy and Clinicalimmunology, The hospital for Sick Children, Toronto, ON,Canada; 2Immunology, San Raffaele Telethon Institute forGene Therapy, Milan, Italy.

Introduction: Lack of adenosine deaminase (ADA) activ-ity results in severe combined immune deficiency (SCID)which is fatal without proper treatment. Bone marrowtransplantation for ADA-SCID has had limited success.Alternatively, frequent injections of ADA bound to poly-ethylene glycol (PEG-ADA) were shown to correct some ofthe metabolic and immune abnormalities associated withADA deficiency. However, data on the effectiveness of long-term ADA enzyme replacement therapy is limited. MethodsA patient diagnosed with SCID secondary to ADA deficiencyreceived PEG-ADA. Immune reconstitution was determinedby immunoglobulin levels, specific antibody production,enumeration of lymphocyte subpopulations, response oflymphocytes to mitogenic stimulation, analysis of T-cellreceptor repertoire and formation of T-cell receptorexcision cycles (TREC). Results: During 13 years of weeklyinjections of PEG-ADA no significant infections weredocumented in this patient and she did not require

Abstracts S133