Faculty Bibliography

The recent identification of the genes involved in many primary immunodeficiency disorders has led to a significant increase in our understanding of the pathogenesis of these defects. Many of these disorders share a clinical phenotype with common features such as recurrent infections, chronic inflammation, and autoimmunity. Although some of these immune defects have mild presentations and better outcomes, others result in severe infections and significant morbidity and mortality. For these, early diagnosis and treatment are critical. This review provides an overview of the genetic defects and clinical features of primary immune deficiencies due to defects in lymphocytes

BACKGROUND: Casual skin contact or inhalation of peanut butter fumes is reported and feared to cause allergic reactions in highly sensitive children with peanut allergy but has not been systematically studied. OBJECTIVE: We sought to determine the clinical relevance of exposure to peanut butter by means of inhalation and skin contact in children with peanut allergy. METHODS: Children with significant peanut allergy (recent peanut-specific IgE antibody concentration >50 kIU/L or evidence of peanut-specific IgE antibody and one of the following: clinical anaphylaxis, a reported inhalation-contact reaction, or positive double-blind, placebo-controlled oral challenge result to peanut) underwent double-blind, placebo-controlled, randomized exposures to peanut butter by means of contact with intact skin (0.2 mL pressed flat for 1 minute) and inhalation (surface area of 6.3 square inches 12 inches from the face for 10 minutes). Placebo challenges were performed by using soy butter mixed with histamine (contact), and scent was masked with soy butter, tuna, and mint (inhalation). RESULTS: Thirty children underwent the challenges (median age, 7.7 years; median peanut IgE level, >100 kIU/L; 13 with prior history of contact and 11 with inhalation reactions). None experienced a systemic or respiratory reaction. Erythema (3 subjects), pruritus without erythema (5 subjects), and wheal-and-flare reactions (2 subjects) developed only at the site of skin contact with peanut butter. From this number of participants, it can be stated with 96% confidence that at least 90% of highly sensitive children with peanut allergy would not experience a systemic-respiratory reaction from casual exposure to peanut butter. CONCLUSIONS: Casual exposure to peanut butter is unlikely to elicit significant allergic reactions. The results cannot be generalized to larger exposures or to contact with peanut in other forms (flour and roasted peanuts)

We report the case of a 41-year-old man infected with human immunodeficiency virus who had two episodes of aseptic meningitis that occurred 2 weeks apart; the first was associated with ingestion of trimethoprim-sulfamethoxazole (TMP-SMZ) and the second was associated with ingestion of TMP alone. Onset of fever, headache, and flushing was abrupt, followed by somnolence, hearing loss, and aphasia. Analysis of the CSF showed pleocytosis and an elevated protein level. The findings resolved within 48 hours after withdrawal of the drug. We also review 18 previously reported cases of TMP-SMZ- or TMP-induced meningitis, 17 of which occurred in women. In all of these cases, a similar abrupt onset and resolution were noted. Six of the 18 patients had collagen-vascular diseases. All but two of these patients had multiple recurrent episodes of meningitis before the diagnosis was made. We conclude that the diagnosis of TMP-SMZ- or TMP-induced meningitis should be considered when a patient receiving these drugs has recurrent episodes of aseptic meningitis