Hypogammaglobulinemia: a diagnosis that must not be overlooked.

Humoral immunological defects are frequent and important causes of hypogammaglobulinemia, leading to recurrent infections, autoimmunity, allergies, and neoplasias. Usually, its onset occurs in childhood or during the second and third decades of life; however, the diagnosis is made, on average, 6 to 7 years afterwards. As a consequence, antibody defects can lead to sequelae. Here we describe the clinical-laboratory characteristics, treatment, and prognoses of patients with hypogammaglobulinemia. An observational, cross-sectional, and retrospective study of patients attending the recently established outpatient group of Clinical Immunology between 2013 and 2018 was carried out. Patients with IgG levels below 2 standard deviations from the mean values for the age and/or impaired antibody response were included. Eight patients (3 F and 5 M; median age=41 years (16-65), average symptom onset at 25 years (1-59), and time to diagnosis of 10 years were included. The main infections were: sinusitis in 7/8, pneumonia in 6/8, otitis in 2/8, tonsillitis and diarrhea in 2/8, and diarrhea in 2/8 patients. Hypothyroidism was identified in 4/8 (50%) patients. Rhinitis was found in 7/8 (87.5%) and asthma in 3/8 (37.5%) patients. The tomographic findings were consolidations, atelectasis, emphysema, ground glass opacity, budding tree, bronchial thickening, and bronchiectasis. Immunoglobulin reposition was used between 466 and 600 mg/kg monthly (514.3 mg·kg-1·dose-1). Prophylactic antibiotic therapy was included in 7/8 (87.5%) patients. Airway manifestations prevailed in patients with hypogammaglobulinemia. There is a need for educational work to reduce the time of diagnosis and initiation of treatment, avoiding sequelae.

Hypogammaglobulinemia: a diagnosis that must not be overlooked

Humoral immunological defects are frequent and important causes of hypogammaglobulinemia, leading to recurrent infections, autoimmunity, allergies, and neoplasias. Usually, its onset occurs in childhood or during the second and third decades of life; however, the diagnosis is made, on average, 6 to 7 years afterwards. As a consequence, antibody defects can lead to sequelae. Here we describe the clinical-laboratory characteristics, treatment, and prognoses of patients with hypogammaglobulinemia. An observational, cross-sectional, and retrospective study of patients attending the recently established outpatient group of Clinical Immunology between 2013 and 2018 was carried out. Patients with IgG levels below 2 standard deviations from the mean values for the age and/or impaired antibody response were included. Eight patients (3 F and 5 M; median age=41 years (16-65), average symptom onset at 25 years (1-59), and time to diagnosis of 10 years were included. The main infections were: sinusitis in 7/8, pneumonia in 6/8, otitis in 2/8, tonsillitis and diarrhea in 2/8, and diarrhea in 2/8 patients. Hypothyroidism was identified in 4/8 (50%) patients. Rhinitis was found in 7/8 (87.5%) and asthma in 3/8 (37.5%) patients. The tomographic findings were consolidations, atelectasis, emphysema, ground glass opacity, budding tree, bronchial thickening, and bronchiectasis. Immunoglobulin reposition was used between 466 and 600 mg/kg monthly (514.3 mg·kg-1·dose-1). Prophylactic antibiotic therapy was included in 7/8 (87.5%) patients. Airway manifestations prevailed in patients with hypogammaglobulinemia. There is a need for educational work to reduce the time of diagnosis and initiation of treatment, avoiding sequelae.

Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases).

Hereditary angioedema (HAE) is a rare autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). The disease is characterized by subcutaneous and submucosal edema in the absence of urticaria due to the accumulation of bradykinin. This descriptive study aimed to evaluate the clinical characteristics of patients with a confirmed diagnosis of HAE referred to our Outpatient Clinic between December 2009 and November 2017. Fifty-one patients (38 F, 13 M) with a mean age of 32 years (range: 7-70 y) were included. Family history of HAE was reported in 70% (36/51) of the cases; 33/46 patients became symptomatic by 18 years of age. The median time between onset of symptoms and diagnosis was 13 years (3 mo-50 y). The most frequent triggering factors for attacks were stress (74.4%), trauma (56.4%), and hormonal variations (56%). The main symptoms were subcutaneous edema in 93.5% (43/46) of patients, gastrointestinal symptoms in 84.8% (39/46), and obstruction in the upper airways in 34.8% (16/46). Hospitalization occurred in 65.2%, of whom 13.3% had to be transferred to the Intensive Care Unit. Prophylactic treatment was instituted in 87% (40/46) of patients, and 56.5% (26/46) required additional treatment to control attacks. Owing to our data collection over a period of 8 years, a significant number of patients were identified by this HAE reference center. Despite early recognition and prophylactic treatment, a high percentage of patients were hospitalized. HAE is still diagnosed late, reinforcing the need for more reference centers specialized in diagnosis and educational projects for health professionals.

Anxiety induced by simulated public speaking and stroop color word test in healthy subjects: effects of different trait-anxiety levels.

1. The objective of the present study was to compare the effects of two anxiety-inducing tests, simulated public speaking (SPS) and the stroop color word test (SCWT), in healthy subjects with different trait-anxiety levels. 2. The mean (+/- SD) trait-anxiety score of 524 university students, measured by Spielberger's state-trait anxiety inventory (STAI), was 40.8 +/- 8.9. Based on these scores, 26 students from our sample were divided into low (score less than 1 SD from the mean, N = 10), medium (score between -1 and +1 SD from the mean, N = 7) and high (score more than 1 SD from the mean, N = 9) trait anxiety. 3. Each subject was submitted to the SPS and SCWT tests in the same experimental session. The sequence of test presentation was randomized between subjects. No effect of test order presentation was found. 4. SPS induced significant increases in the anxiety factor of Norris' visual analogue mood scale (VAMS) in all groups, without difference between them. The SCWT, on the other hand, did not induce an increase in subjective anxiety in any group. In the high trait group, however, there was a general increase in anxiety feelings that was evident even before the test, and might have been caused by the presence of the experimenter. 5. The results suggest that SPS is a more effective anxiety-inducing test, and is not dependent on previous trait-anxiety levels.

Anxiety induced by simulated public speaking and stroop color word test in healthy subjects: affects of different trait-anxiety levels

The objective of the present study was to compare the effects of two anxiety-inducing tests, simulated public speaking (SPS) and the stroop color word test (SCWT), in healthy subjects with different trait-anxiety levels. 2. The mean (+ or - SD) trait-anxiety score of 524 university students, measured by Spielberger's state-trait anxiety inventory (STAI), was 40.8 + or - 8.9. Based on these scores, 26 students from our sample were divided into low (score less than 1 SD from the mean, N = 10), medium (score between -1 and + 1 SD from the mean, N = 7) and high (score more than 1 SD from the mean, N = 9) trait anxiety. 3. Each subject was submitted to the SPS and SCWT tests in the same experimental session. The sequence of test presentation was randomized between subjects. No effect of test order presentation was found. 4. SPS induced significant increases in the anxiety factor of Norris' visual analogue mood scale (VAMS) in all groups, without difference between them. The SCWT, on the other hand, did not induce an increase in subjective anxiety in any group. In the high trait group, however, there was a general increase in anxiety feeling that was evident even before the test, and might have been caused by the presence of the experimenter. 5. The results suggest that SPS is a more effective anxiety-inducing test, and is not dependent on previous trait-anxiety levels