The Clinical Manifestations and Immunological Characteristics of Selective IgA Deficiency in Children / 소아알레르기및호흡기학회지

PURPOSE: Selective immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency disease. The aim of the present study is to analyze clinical manifestations and immunological characteristics of selective IgAD in children. METHODS: Twenty-one children aged 1-14 years with the serum IgA level of 2 standard deviation (2 SD) below normal for age who had recurrent infection were enrolled in this study at Uijeongbu St. Mary's Hospital of the Catholic University of Korea, between January 2006 and August 2007. Recurrent infection was defined as the presence of at least 6 febrile infection episodes per year before this study. Among IgA deficient patients, we excluded panhypogammaglobulinemic patients. RESULTS: The average frequency of admission was 2.2+/-2.0 per year. The most common causative disease at admission was lower respiratory infection (53.6%). Immunoglobulin G (IgG) subclass deficiency was corcurrent in 9 patients (42.9%): deficiency of IgG2 in 1, IgG3 in 5, IgG4 in 1 and IgG3+IgG4 in 2. Five patients (23.8%) had a decrease in CD3+ cells and 8 patients (38%) had a decrease in CD4+ cells. One patient progressed to common variable immunodeficiency (CVID). Serum IgA levels of 76.5% of the patients increased to 2 SD above normal for age after 15.9+/-4.5 months. CONCLUSION: The patients with IgAD in this study showed frequent respiratory and gastrointestinal infections and combined with other immunodeficiencies including IgG subclass deficiency. Our data suggest that it is necessary to examine the immunological status including IgA level in patients with frequent infections and that those with IgAD should be observed for the potential progression to CVID.

The Significance of (99m)Technetium Dimercaptosuccinic Acid(DMSA) Scan as a Substitute for Voiding Cystourethrography(VCUG) in Evaluating Children with first Febrile Urinary Tract Infection

PURPOSE: We studied the value of clinical signs, laboratory findings and (99m)technetium dimercaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). METHODS: A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. RESULTS: Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. CONCLUSION: An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. Therefore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.

Lifestyles and Gastrointestinal Disorders in Those Who Have a Postprandial Lying-down Habit

BACKGROUND: A habit of lying-down after eating is known as a risk factor of reflux esophagitis. However the association between gastrointestinal disorders and a postprandial lying-down habit has not yet been identified. Some people believe that lying-down after meals is helpful for their health. We intended to investigate the relationship between such a habit and the health lifestyles, gastrointestinal symptoms, disorders using a questionnaires and gastrofiberscopic results of patients who visited our general health screening center. METHODS: We examined 1,030 subjects (the frequent postprandial lying-down group was 576 persons (57.7%), the rare group was 436 persons (42.3%) who visited our general health screening center for a routine check-up including a gastrofiberoscopy during the period between January 2001 to December 2002. After frequency matching by age and sex, health lifestyles (such as smoking, alcohol consumption, exercise, eating habits, night time sleep duration and awakening frequency, nap, and coffee intake), gastrointestinal symptoms (dyspepsia, epigastric pain, heart burn, chest pain, and constipation) and gastrofiberoscopic findings were compared using results from the questionnaires and gastrofiberscopic findings. Factors such as gastritis and reflux esophagitis were set as dependent variables, while postprandial lying-down was set as an independent variable. The frequent postprandial lying-down group and the rare group were analysed through stepwised multiple logistic regression analysis. RESULTS: Subjects with a frequent postprandial lying- down habit showed a significantly greater degree in alcohol consumption (P=0.010), irregular eating habits (P<0.001), eating between meals (P<0.001), napping (P<0.001), and night time awakening frequency (P= 0.016) than the rare group did. In addition, chest pain (P=0.031) and constipation (P=0.010) were more common in the frequent postprandial lying-down group. However, prevalence of dyspepsia (P=0.147), epigastric pain (P=0.085), and heartburn (P=0.700) showed no differences between the two groups. Being controlled with age, sex, education level, body mass index, and life style factors, postprandial lying-down group showed higher prevalence ratios in erythematous gastritis (OR 1.59; P=0.090), atrophic gastritis (OR 1.71; P= 0.059), and reflux esophagitis (OR 1.78; P=0.103). CONCLUSION: A postprandial lying-down habit is associated with undesirable lifestyles and some gastrointestinal disorders. Therefore, a modification of the postprandial lying-down habit should be recommended. Further investigation is needed to clarify the causal relationships between a postprandial lying down habit and gastrointestinal symptoms and disorders.