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Summary: We describe the case of a 24-year-old male with hyper-IgE syndrome (HIES) which was diagnosed at 4 years of age and died from a very rare cardiac complication. He had typical clinical and laboratory manifestations of HIES, including total serum IgE as high as > 100,000 IU/mL. Stem cell transplantation was not available. During the 20-year follow-up, he suffered numerous various infections of the skin and deep organs, partial lung resection, as well as multiple bone fractures. At age 24, he developed acute decompensated heart failure associated with elevated serum troponin I and brain natriuretic protein. Two-dimensional echocardiogram revealed global hypokinesis of the left ventricle with estimated ejection fraction 20-25%, and catheterization revealed ectasia of multiple coronary arteries. Endomyocardial biopsy showed lymphocytic myocarditis, focal necrosis, mild fibrosis, and myxoid degeneration, but cultures were negative. The patient improved on corticosteroid therapy and was discharged on heart failure therapy and external defibrillator. Six weeks later, he developed supraventricular tachycardia and persistent global hypokinesis and was treated with amiodarone. A trial of intravenous immunoglobulin was initiated and was repeated as outpatient every four weeks for four times. However, his cardiac function did not improve and he developed severe hypotension and pulseless electrical activity arrest. Resuscitation was unsuccessful. To the best of our knowledge, this is the first reported case of HIES complicated with lymphocytic myocarditis. Both immunologists and cardiologists need to be aware of such a complication and practice caution in using immunosuppressants when the patient's immune status is markedly compromised.

Background: Studies on serum IgE levels during pregnancy are limited., Objective: To investigate the course of serum total IgE levels during pregnancy and postpartum., Methods: 159 pregnant subjects provided 218 serum samples during various stages of pregnancy and the postpartum period. Serum total IgE geometric means were compared at various trimesters and postpartum. In addition, the postpartum IgE data were analysed according to the method of delivery. Analysis was also done according to history of allergy., Results: The geometric mean serum total IgE was 20.5 IU/ml in the first trimester, 20.8 IU/ml in the second and 22.2 IU/ml in the third. Postpartum serum IgE level showed a lower mean, 14.9 IU/ml during the early postpartum period (less than 30 days) compared to 30.3 IU/ml during the late postpartum period (30 days-25 weeks). However this was not statistically significant. Serum IgE in the postpartum period also did not differ according to method of delivery. A history of allergy was positive in 98 samples, negative in 61 and unclear in 59. Using analysis of variance, none of these three groups showed significant change in serum total IgE level during pregnancy or postpartum., Conclusion: In this cross-sectional study, serum total IgE levels showed no statistically significant changes during pregnancy or postpartum. This finding would be of greater weight if reproduced in a larger number of subjects with multiple serial samples at fixed regular time intervals during pregnancy and postpartum., (Copyright © 2010 SEICAP. Published by Elsevier Espana. All rights reserved.)

Background: The role of genetics in allergy development is well accepted. However, studies could not delineate the mode of inheritance or what is specifically being inherited. The purpose of this study was to determine the effect of genetics on the development of allergy manifestation, serum IgE level, and sensitization to specific allergens., Methods: Fifty-eight twin sets (age 7 months to 11 years) were evaluated for allergy by medical history, family history, physical examination, serum total IgE level, and percutaneous testing to selected common allergens., Results: In 25 monozygotic (MZ) sets, concordance of atopy was significantly higher than in 33 dizygotic (DZ) sets (84.6% vs 62.5%). The age at onset tended to be earlier when the mother was allergic than when the father was (23.5 months vs 30.5 months). When both twins were allergic, the intra-pair difference in age at onset was within <6 months in 50% of MZ sets versus 31.8% in DZ sets. Total IgE level in twins showed a very strong correlation in MZ sets (r 0.92), but only a moderate correlation among DZ sets (r 0.57). Skin test positivity to specific allergens did not show a significant concordance between twins in either group., Conclusion: Our study indicates that the genetic influence was strongest on the inheritance of IgE phenotype, the development of the atopic tendency, the age of onset, and to some extent on the specific allergy manifestation. The effect seemed less on determining the specific offending allergen(s), suggesting possible roles of epigenetic and environmental factors., (Copyright 2009 SEICAP. Published by Elsevier Espana. All rights reserved.)

The atopic status of patients with leprosy was assessed by medical history, physical examination, serum total IgE, and specific IgE antibodies to common allergens (by skin testing and RAST). Tests for specific IgE antibody to Mycobacterium leprae were performed by RAST and immunoblotting technique. We studied 28 patients with leprosy and 49 control subjects. The two groups did not differ significantly in the prevalence of atopic disease. The IgE level was significantly higher in the patients, however, than in the control subjects, whether there was atopy (296.1 versus 96.3 IU/ml) or not atopy (72.9 versus 18.9 IU/ml). Neither RAST nor immunoblotting technique detected significant levels of IgE antibodies to M. leprae. Our data indicate that leprosy was associated with increased total IgE level, but clinical atopy in patients with leprosy was similar to that in control subjects. The observed IgE increase in patients with leprosy appears to be generally nonspecific.

Serum IgE levels in healthy blood donors who had no history of atopy were measured by a paper-disc RIA and analyzed according to the donors' smoking habits. The IgE geometric mean for regular smokers was 41.7 IU/ml, which was significantly higher than that for nonsmokers (19.3 IU/ml) or rare smokers (22.7 IU/ml). Whereas 28% of smokers had IgE levels greater than 200 IU/ml, none of the rare smokers or nonsmokers did. IgE levels in smokers showed a moderate inverse correlation with the degree of smoking. The mean IgE level was 189.8 IU/ml in those who smoked 1-9 cigarettes/day but only 32.8 IU/ml in those who smoked 10-19 cigarettes per day and 11.1 IU/ml in those who smoked 20 or more cigarettes/day. The number of years a person smoked did not seem to significantly influence the IgE level. The mean IgE level in ex-smokers (50.5 IU/ml) was much lower than in current light smokers but was still higher than in nonsmokers. There was a moderate inverse correlation between IgE levels and duration of cessation of smoking. Our data suggest a characteristic pattern for the influence of cigarette smoking on serum IgE level, namely, a striking rise associated with light smoking and a remarkable drop in heavy smokers, and such changes seemed reversible after the habit was stopped. Smoking status, therefore, appears to be an important consideration in interpreting serum IgE levels and in revising the "norms" of IgE levels.

Nineteen patients with heterophil-positive infectious mononucleosis were followed with serial blood sampling for an average of 14.6 mo. A preillness serum sample, obtained more than 2 mo before onset of illness, was available from 9 patients. A total of 141 samples were tested under identical conditions for total IgE levels by a paper-disc radioimmunoassay. Hematologic and serologic studies were also done. IgE changes showed a definite pattern, consisting of an elevation early in illness, rapidly followed by a significant drop reaching a nadir by the third month, then gradually returning to the preillness level (PIL) over a period of about a year. On the average, the peak was about 3 times the PIL, and the nadir about half the PIL. The IgE peak always coincided with the peak of atypical lymphocytes, and in no instance did it occur later than the peaks of other hematologic or serologic changes. No definite relationship was noted between IgE levels and the severity of illness. Heterophil-positive infectious mononucleosis is the first well-defined infectious disease found to be associated with such a pattern of IgE response.

Using radioimmunoassay techniques, we measured IgE and IgD levels in paired colostrum and plasma samples obtained within 4 days postpartum. In colostrum, IgE was detected in concentrations of 0.5-6 IU/ml in 16 out of 39 samples (41%) and less than 0.5 IU/ml in the remainder, whereas IgD was detected in all samples in concentrations of 2-2000 micrograms/dl. Only a moderate correlation was found between colostral and plasma levels of both IgE (r = +0.60) and IgD (r = +0.74). The correlation coefficient between IgE and IgD in plasma was 0.23, whereas in colostrum it was only 0.05. The colostrum:plasma ratio of IgE varied strikingly from that of IgD; the ratio of IgD was 0.1-22.2 times that of IgE. The findings argue against passive transfer of IgE and IgD from the circulation of milk and suggest possible local mammary production of either or both of these two immunoglobulins.

We studied 19 young children (aged 15 to 39 months) who had had a systemic allergic reaction after imported fire ant (IFA) sting. Skin testing was performed with whole body extract (WBE) of both Solenopsis species; positive reactions to S. invicta was noted in 94% and to S. richteri in 100%. Serum was tested for total IgE level and for specific IgE antibodies by RAST with WBE and venom (V) of S. invicta. In vitro results were compared with those of 19 age and sex-matched atopic control subjects. The range of total serum IgE level was 31 to 1720 IU/ml (geometric mean 160 IU/ml), compared with 7.5 to 6756 IU/ml (geometric mean 85 IU/ml) in the control subjects. The difference between the two groups, however, was not statistically significant (p greater than 0.05). In the IFA-allergic group, the IFA WBE RAST mean binding was 13.5% (range 2.0% to 30.2%), and the IFA V RAST mean was 29.2% (range 7.8% to 46.7%). The control sera demonstrated low levels of activity to both IFA WBE RAST (0.7% to 4.8%) and IFA V RAST (0.7% to 5.9%). IFA WBE RAST demonstrated that 26% of the IFA-allergic patients had counts within the range of the atopic control subjects. In contrast, the IFA V RAST clearly separated all IFA-allergic patients from their age and sex-matched atopic control subjects. A scoring system for IFA V RAST is proposed. These results demonstrate superiority of IFA V over IFA WBE RAST in the diagnosis of allergy to S. invicta.