Respiratory and allergic disorders in children with severe and partial immunoglobulin A immunodeficiency.

BACKGROUND: Immunoglobulin A deficiency (IgAD) is the most common primary immunodeficiency. Although most people with selective IgAD (sIgAD) are asymptomatic, many patients often suffer from recurrent respiratory infections and different allergic disorders. Our aim was to investigate connection between subtypes of sIgAD and incidence of respiratory and allergic disorders, as well as connection with lung function changes in children. METHODS: Children with IgAD where divided into two groups; severe IgAD in patients was defined as serum IgA level <7 mg/dL, while partial IgA deficiency diagnosis was made when serum IgA levels was higher than 7 mg/dL but at least two standard deviations (SD) below mean normal concentrations for their age. All patients were evaluated by their clinical and laboratory investigation parameters and compared to control group of children. RESULTS: Group of children with IgAD, severe as well as partial, showed higher prevalence of allergic diseases and total number of infections, compared to controls. There was a statistically significant difference in lung function for peak expiratory flow (PEF), the maximal expiratory flow at 50% of the forced vital capacity (MEF50) and fraction of exhaled nitric oxide (FENO) between group of patients with severe as well as partial IgAD and control group, where children with IgAD showed reduced lung function. CONCLUSIONS: Children with sIgAD are at increased risk for higher number of respiratory infections and developing allergic diseases, resulting in significantly lower pulmonary function which is related with the severity of sIgAD.

Diagnosis, clinical features, management, and post-natal follow-up of fetal tachycardias.

OBJECTIVE: To evaluate the diagnosis, clinical features, management and post-natal follow-up in consecutive fetuses identified with tachycardia. METHODS: We reviewed consecutive fetuses with tachycardia identified in a single tertiary institution between January, 2001, and December, 2008. We considered several options for management, including no treatment but close surveillance, trans-placental antiarrhythmic therapy in fetuses presenting prior to 36 weeks of gestation, and delivery and treatment as a neonate for fetuses presenting after 36 weeks of gestation. Data was gathered by a review of prenatal and postnatal documentation. RESULTS: Among 29 fetuses with tachycardia, 21 had supraventricular tachycardia with 1 to 1 conduction, 4 had atrial flutter, 3 had atrial tachycardia, while the remaining fetus had ventricular tachycardia. Of the group, 8 fetuses (27.6%) were hydropic. Transplacental administration of antiarrhythmic drugs was used in just over half the fetuses, delivery and treatment as a neonate in one-quarter, and no intervention but close surveillance in one-sixth of the case. Twenty-six of 29 fetuses (89.7%) were born alive. Only patients with fetal hydrops suffered mortality, with 37.5% of this group dying, this being statistically significant, with the value of p equal to 0.03, when compared to non-hydropic fetuses. Only 3 patients (11.5%) were receiving antiarrhythmic prophylaxis beyond the first year of life. CONCLUSION: A significant proportion of fetal tachycardias recognized before 36 weeks of gestation can be treated successfully by transplacental administration of antiarrhythmic drugs. Fetuses presenting after 36 weeks of gestation can be effectively managed postnatally. The long-term prognosis for fetuses diagnosed with tachycardia is excellent, with the abnormal rhythm resolving spontaneously during the first year of life in most of them.