Ömür Ardeniz

Granulomatous disease in common variable immunodeficiency.

Granulomatous disease occurs in 8-22% of patients with common variable immunodeficiency (CVID). We examined the clinical and immunologic information of all 37 of 455 (8.1%) CVID subjects with this complication. The median age at diagnosis of CVID was 26 (2-59). 14 had granulomas 1-18 years before diagnosis of CVID. In 6 detection of granulomas coincided with this diagnosis; for 17, granulomas were documented later. 54% had lung granulomas, 43% in lymph nodes and 32% in liver. 54% of the group had had autoimmune diseases, mostly immune thrombocytopenia and hemolytic anemia. 24% had had a splenectomy. Nineteen (51.3%) required steroid treatment for granulomas; other immune suppressants were used in some. Over 25 years 28.5% died (median age 37.5), but not significantly more when compared to our CVID patients without granulomas (19.8%). Those with lung granulomas had similar mortality to those with granulomas in other tissues.

Desensitization Effect of Preseasonal Seven-Injection Allergoid Immunotherapy with Olive Pollen on Basophil Activation: The Efficacy of Olive Pollen-Specific Preseasonal Allergoid Immunotherapy on Basophils

Background: It has previously been demonstrated that subcutaneous immunotherapy with allergoids positively affects clinical and immunological parameters even after 7 preseasonal injections. However, its effect on basophil activation remains unclear. We investigated the effect of preseasonal allergoid immunotherapy on basophils and concomitantly assessed its clinical and immunological efficacy in olive pollen-monosensitized patients. Methods: This study enrolled 437 consecutive patients with respiratory allergy and positive skin prick tests (SPTs); 212 (48.5%) patients were sensitized to olive pollen, and 33 (7.5%) patients were sensitized to olive pollen only. Of these patients, 23 received preseasonal immunotherapy with an olive pollen allergoid. The olive pollen-specific basophil activation, the titrated nasal provocation test, the nasal symptom score, and olive pollen-specific IgE, IgG1 and IgG4 levels were evaluated before immunotherapy and 8 months after the end of immunotherapy in the follow-up visit. Results: In comparison to baseline evaluation, 7 preseasonal injections of an allergoid resulted in a significant decrease in the percentage of basophils expressing CD63 (29 vs. 7%, respectively, p < 0.0001) and a significant increase in the titrated nasal provocative dose (1/10 vs. 1/1, respectively, p < 0.01). SPT induration diameters caused by an olive pollen extract decreased (12 mm at baseline vs. 5.5 mm at follow-up, p < 0.005), as did nasal symptom score (7 at baseline vs. 3 at follow-up, p < 0.01). Olive pollen-specific IgE (17.5 vs. 50 kU/l, p < 0.012), IgG1 (0.16 vs. 2.9 µg/ml, p < 0.0001) and IgG4 (0.07 vs. 1.92 µg/ml, p < 0.0001) levels significantly increased. Conclusions: Immunotherapy with 7 preseasonal injections of an olive pollen allergoid decreases olive pollen-specific basophil activation over 8 months, an effect observed in vitro and in vivo.

Chlamydia pneumoniae arthritis in a patient with common variable immunodeficiency

BACKGROUND Arthritis is an important and sometimes life-threatening complication in patients with common variable immunodeficiency (CVID). OBJECTIVE To describe a patient with CVID and arthritis due to Chlamydia pneumoniae, which is usually regarded as a respiratory tract pathogen and has not previously been detected in the synovial fluid by cell culture technique. METHODS Routine bacteriologic, virologic, mycologic, and tuberculosis cultures were performed. The patients synovial fluid was examined for fastidious organisms that might be causative pathogens of arthritis, such as chlamydiae, and special cell culture methods were used. Serologic tests were performed to determine viral and bacteriologic etiology. RESULTS The patient had a history of recurrent respiratory tract infections, and the latest exacerbation was followed by arthritis. Cytologic examination of the fluid yielded abundant lymphocytes. Chlamydia pneumoniae was detected in synovial fluid specimens by cell culture technique. Her nasopharyngeal swab and sputum culture specimens were also positive for this pathogen. She was diagnosed as having arthritis caused by C pneumoniae and was given antibiotherapy. CONCLUSION Chlamydia pneumoniae should be kept in mind as a causative pathogen in patients with CVID and arthritis, especially when effusion fluid is full of lymphocytes rather than polymorphonuclear cells and no organism is grown on routine cultures.

Phagocytic and oxidative burst activity of neutrophils in patients with spinal cord injury.

OBJECTIVE To evaluate phagocytic activity and neutrophil oxidative burst functions in patients with spinal cord injury (SCI) because alterations in neutrophil metabolic activity can be one of the causes of immune mechanism damage contributing to repeated bacterial infections. DESIGN A controlled and cross-sectional study. SETTING Departments of physical medicine and rehabilitation and immunology. PARTICIPANTS Patients with SCI (N=34) and 28 healthy controls. INTERVENTIONS Phagocytosis and oxidative burst in whole-blood neutrophils were assessed by flow cytometry. The percentage of phagocytizing cells after in vitro incubation with Escherichia coli, phagocytic activity (mean intensity of fluorescence [MIF]) and the percentage of neutrophiloxidative burst, and the MIF value of the production of reactive oxygen intermediates (ROIs) were analyzed. In addition, clinical assessment including the level of injury, American Spinal Injury Association scores, and functional status were carried out. MAIN OUTCOME MEASURES Not applicable. RESULTS Although the percentage of E. coli phagocytizing neutrophils was not different between groups, the MIF value of absorbed E. coli was significantly lower in patients with SCI than in controls (P<.05). The MIF value of ROI production by neutrophils with both stimulator of phorbol 12-myristate 13-acetate and E. coli was significantly higher in patients with SCI (P<.05). CONCLUSIONS In patients with SCI, decreased phagocytic activity of neutrophils may be a result of a regulatory mechanism to minimize the deleterious effects of increased neutrophil burst activity.

The Turkish Hereditary Angioedema Pilot Study (TURHAPS): The First Turkish Series of Hereditary Angioedema

Background: No published data presently exist concerning hereditary angioedema (HAE) in Turkey. The aim of the study was to initiate a preliminary multicentric evaluation about HAE and to determine the genetic properties of Turkish patients. Methods: Based on records drawn from four medical centers we identified a total of 70 subjects, belonging to 60 unrelated families, fulfilling clinical and laboratory criteria for diagnosis of HAE with C1 inhibitor deficiency. Ten type I patients, and their first-degree relatives, underwent genetic analysis for HAE. Results: The majority of patients were female (60%), the mean age was 37.7 ± 14.1 years. The mean age at the time of first angioedema symptom was 12.5 ± 9.2 years. Mean time lag between first symptom and diagnosis was 26 ± 14.4 years. All but 3 subjects had HAE type I. Family history of angioedema was present in 75.7% of the cases. Cutaneous swelling was reported by 87.1% of the patients, facial edema by 65%, abdominal symptoms by 74.3% and approximately one half (55.7%) had experienced one or more laryngeal attack. Genetic analysis of 10 families demonstrated that 5 carried a mutation that had never been previously described. Conclusion: We found that the clinical features of Turkish HAE patients were consistent with previously described patterns of this rare disease. The most noteworthy feature identified in the study was a significantly long duration between the first symptom appearance and final diagnosis. Our detection of different mutations in 10 patients confirms the allelic heterogeneity of the disease.

Diagnostic value of specific IgE analysis in latex allergy.

Background: The precision of the methods used to diagnose latex allergy is of great importance due to false-positive results. Neither the skin prick test (SPT) nor the latex-specific IgE assay has 100% diagnostic accuracy. We analysed the diagnostic value of latex-specific IgE by the first-ever concomitant use of the SPT and nasal provocation test (NPT). Methods: Twenty-seven latex-sensitive patients (group 1), 46 aeroallergen-sensitive patients (group 2a) and 33 healthy subjects (group 2b) participated in the study. All groups underwent an SPT with latex and aeroallergens and an NPT with latex. Latex-specific IgE and total IgE levels were measured by the ImmunoCAP assay. Results: Latex-specific IgE was positive in 92.6, 30.4 and 9.1% of groups 1, 2a and 2b, respectively. The 11 aeroallergen-sensitive patients in group 1 and all of the patients in group 2a were predominantly sensitised to pollens (grass, weed and tree) and reacted to a lesser degree to house dust mite, moulds and animal dander. Combined pollinosis was remarkably more prevalent in patients with positive latex-specific IgE in group 2a than in those with negative latex-specific IgE (p = 0.001). The NPT was positive in 84.6% of group 1 and negative in all control subjects. The sensitivity, specificity, negative predictive value and positive predictive value of the latex-specific IgE assay were 90.9, 72.2, 96.3 and 50%, respectively. Conclusion: The high rate of false-positive results for latex-specific IgE by ImmunoCAP should be taken into account when making a diagnosis of latex allergy in patients with pollinosis, especially in those sensitised to more than one pollen species.

The frequency of mouse and rat allergy among allergic individuals in Izmir (a preliminary report).

The author’s evidence for this event uses hieroglyphs found on two almost identical but only partially preserved ebony plates at one of the many putative Menes burial sites. In contrast to other clearly perceivable animals, Waddell interprets a half circle hieroglyph on plate no. 1 as a wasp or a hornet, using plate no. 2 for completion (Figs 1 and 2). However, the sting can only be seen on plate no. 1, giving the certainty to be only an accidental wooden scratch. Moreover, Waddell ignored the variation of the hieroglyphs on plate no. 2, where three wasps are noticeable instead of one. Never again have historians followed Waddell’s interpretation. In contrast, the wasps are seen without doubt as hieroglyphic numbers (5). Although Waddell’s fictional account of Menes death after a wasp sting has never been accepted by other Egyptologists it has reverberated through the medical literature on allergy to this very day.

The importance of nasal provocation test in the diagnosis of natural rubber latex allergy.

Background:  Most studies regarding natural rubber latex (NRL) allergy have concentrated on the prevalance using skin prick test (SPT) and specific IgE assay. The objective of this study is to examine the target organ (skin, nasal mucosa) responses in patients with positive SPT to NRL using the nasal provacation test (NPT) and glove use test (GUT).

Vitamin D deficiency in the absence of enteropathy in three cases with common variable immunodeficiency.

Background: Common variable immunodeficiency (CVID) is characterized by hypogammaglobulinemia and a defect in antibody production. Herein we describe 3 patients diagnosed with CVID in whom vitamin D deficiency was detected in the absence of enteropathy. Methods: Biochemical and immunological analysis, serum osteocalcin, parathyroid hormone, 25-OH vitamin D, 1,25(OH)2 vitamin D, vitamin A, vitamin E, urinary calcium, and deoxypyridinoline measurements were carried out. Vitamin D receptor (VDR) expression was examined in the peripheral blood mononuclear cells and hair follicles by reverse transcriptase polymerase chain reaction. VDR gene polymorphism was evaluated by high-performance liquid chromatography. Results: None of the patients presented nutrient deficiencies other than vitamin D. Two of them were free of osteomalacia-related symptoms. VDR expression was found to be lower in the peripheral blood mononuclear cells and hair follicles when compared to the control group. Conclusions: Patients with CVID may present asymptomatic vitamin D deficiency. Vitamin D and VDRs play an important role in the innate immune system and modulate Toll-like receptor-related responses. Delay in diagnosis may predispose these patients not only to irreparable bone loss but also to infections, and autoimmune and malignant disorders, thus emphasizing the importance of prompt intervention.

Laryngeal edema due to European bee-eater (Merops apiaster) in a patient allergic to honeybee

Contact with the antigen may not always be obvious in allergic reactions. Inhalation of the bee allergens has been reported to induce allergic symptoms in beekeepers (1). Bee venom components found in honey can cause severe allergic reactions in sensitive individuals (2). We present a case of anaphylaxis after eating a bee-eater (BE) in a patient allergic to honeybee. European bee-eater (Merops apiaster) is a colorful bird which eminently eats bees, wasps and hornets (250 bees per day). Stings are frequently swallowed and found in their food remains. BEs are apparently immune to venom; they show pain when stung but no other effect is evident. They live in most parts of southern Europe, south-western Asia, northern and southern Africa and are found throughout Turkey (3). A 52-year-old hunter, ate BE meat 1 week before admission to our clinic. He experienced itching in the oral mucosa and difficulties in breathing 30 min after the meal. He was admitted in the emergency room where laryngeal edema was confirmed. He received treatment and was referred to our allergy clinic. He did not have personal or family history of atopy. In the past he had been stung six times by bees. The wasps never caused any reaction. But generalized urticaria, hypotension and unconsciousness had occurred for the last three times after he was stung by honeybees. The last reaction was at 4 months ago. Before the laryngeal edema episode caused by BE he had eaten the bird several times without any clinical reaction. He stopped eating this bird after systemic sting reactions caused by honeybees. Allergy tests were performed 2 months after the reaction. Skin prick test to honeybee venom revealed a wheal of 3 mm greater than the negative control (ALK 300 lg/ml). Serum-specific IgE (Pharmacia UniCAP, Uppsala, Sweden) for Apis mellifera was 17.5 kU/l (class 3). Tests for wasp venom remained negative. In addition to venom allergy via bee stings, venom allergens can cause reaction in sensitive patients when they are encountered unknowingly. Beekeepers may be sensitized to bee venom through inhalation as well as stings (1). Thirtyone percent of beekeepers had experienced nasal or eye symptoms correlated with the history of systemic sting reactions while working at beehives (4). Allergic symptoms ranging from itching in the oral mucosa to anaphylaxis have been reported in patients allergic to honey (5). In these individuals primary sensitization may be due either to the honey itself, to compositae pollen or to bee venom components (2). To our knowledge, this is the first case of laryngeal edema caused by ingestion of BE meat in a patient with a history of honeybee allergy. The mechanisms of this reaction remain unclear. But it is necessary to investigate whether major venom allergens are detectable in the bird’s blood or tissues. In conclusion, patients allergic to bees should avoid eating these birds.