Background/aim: In this study, we aimed to assess the clinical and immunological findings of our patients with common variable immunodeficiency (CVID). Materials and methods:We analyzed the records of 31 adult patients with CVID (12 females, 19 males). The patients were classified into clinical and immunophenotypic subgroups for statistical comparisons.Results: Our patients had some clinical signs in considerable frequencies, such as low body weight (45.2%), urinary tract infections (41.9%), various dermatoses (35.5%), and oral aphthae (32.3%). The histological findings in the biopsy specimens of the gastrointestinal tract (nodular lymphoid hyperplasia, villous atrophy, and lymphocytic infiltrates at mucosa) were significantly associated with splenomegaly, hepatomegaly, or low body weight (P = 0.005, 0.045, and 0.007, respectively). The patients with low CD4/CD8 ratios had lower IgG levels and a lower percentage of CD19+ B cells, but a higher percentage of activated T cells (P = 0.023, 0.011, and 0.028, respectively). Conclusion:In adults with CVID, there existed some clinical signs at considerable frequencies, but these are not overemphasized in the literature. The CD4/CD8 ratio is an important factor in antibody production and the clinical presentation of CVID. It seems that the adaptive immune system is on alert and subclinical immune activation insidiously continues in patients with CVID.
Hypertensive patients have strong evidence of endothelial dysfunction. Some novel endothelial dysfunction parameters such as pulse wave velocity (PWV), augmentation index (AIx), and central aortic pressure (CAP) have been investigated as predictive markers of atherosclerosis. It is well known that obesity has relationships with endothelial dysfunction and atherosclerosis. We aimed to investigate relationships between anthropometric measurements and arterial stiffness parameters in essentially hypertensive patients. The study population included 100 patients (56 females, 44 males) newly or formerly diagnosed as essentially hypertensive in an outpatient clinic. Arterial stiffness measurements, including PWV, AIx, CAP, and body mass index (BMI); waist circumference, hip circumference; waist/hip ratio; and triceps, biceps, subscapular, and suprailiac skinfold thicknesses were also applied to all the study patients. Then, the relationships between BMI, anthropometric measurements, and arterial stiffness parameters were investigated. The mean systolic arterial blood pressure of the study population was 135.85 ± 15.27 mm Hg and the mean diastolic arterial blood pressure of the study population was 84.17 ± 9.58 mm Hg. The parameters such as PWV, AIx, and CAP measured for arterial stiffness had correlations between BMI and different anthropometric measurements. The statistically significant correlations were present between PWV and triceps skinfold thickness (TST) (r = 0.377, P < .001) and it was also seen when regression analysis was performed (PWV = 6.41 + [0.072 × TST]; R(2) = 0.142, F[1-98] = 16.23, P < .001). Triceps skinfold thickness among these correlations may be used to estimate the carotid-femoral PWV, which is an indicator of subclinical organ damage due to hypertension.
This present study results support that HLA-Cw7 allele, an inhibitor of KIR ligand, may play a role in the pathogenesis of CVID.
Mastocytosis is a rare group of disorders characterized by abnormal accumulation of mast cells in the skin, bone marrow, and internal organs. In particular, patients with systemic mastocytosis are at an increased risk of frequent and severe episodes of anaphylaxis. Hymenoptera venom allergy is the most common trigger of anaphylaxis in these patients. Immunotherapy is an effective and safe therapy recommended for patients with mastocytosis and venom allergy. Although this therapy can be administered according to different protocols, the preferred protocol for patients with mastocytosis remains unclear. Systemic side effects can occur, in particular, during the up-dosing phase of immunotherapy, making progression to the maintenance phase of therapy challenging. This case report presents the diagnosis and ultrarush immunotherapy process ended with anaphylaxis of a 33-y-old male patient with Apis mellifera allergy.
A 15-year-old male patient presented to the emergency room with chest pain, palpitations and shortness of breath that began 5 min after a wasp sting on his neck. He complained of retrosternal chest pain feeling pressure/squeezing, radiating to the neck that continued for about 45-50 min. The patient had no comorbid disease and had not previously been exposed to bee stings. His initial vital signs were temperature 36 C, heart rate 132 regular beats per minute, respiratory rate 22 breaths per minute, blood pressure 109/68 mmHg and pulse oximetry 91% in room air. There was a mild swelling, redness and tenderness in the wasp sting area. Troponin I level at admission was elevated (66.4 pg/mL; reference range <34.2 pg/mL). Chest radiography was unremarkable. There were no additional findings on electrocardiogram (ECG) except sinus tachycardia. Echocardiography showed good global contractility of the heart and no regional hypokinesia. Nasal oxygen therapy and intravenous 0.9% saline infusion were initiated. A single dose of 45.5 mg pheniramine maleate and 50 mg methylprednisolone intravenous were administered. The patient's complaints began to regress after 15 min and completely resolved in 1 h. The laboratory parameters of the hospitalised patient were monitored (Table 1).The patient was discharged 2 days after hospitalisation. No abnormality was detected in the cardiological examinations during the follow-up. Allergy tests performed at 3 months showed serum tryptase: 4.4 μg/L (ref. range 0-11.4); serum total
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