Calculated Globulin Is Clinically Useful as a Screening Test for Antibody Deficiency in Turkish Adult Patients.

INTRODUCTION: Heterogeneous clinical features of antibody deficiency (AD) may cause diagnostic delays. Calculated globulin (CG) (total protein minus albumin) has been proposed as a screening test to prevent morbidity due to diagnostic delays in AD. Our aim was to validate CG as a screening test for AD in Turkish adult patients by comparing its role with gamma globulin analysis in protein electrophoresis. METHODS: Fifty serum samples were randomly collected for each level of CG from 15 to 25 g/L and tested for serum IgG, IgA, IgM levels and protein electrophoresis. Cut-off values predicting low IgG levels were calculated for electrophoretically determined gamma globulin and CG. Additionally, the data of 47 patients followed up in our clinic with a diagnosis of primary antibody deficiency (PAD) were retrospectively analyzed. RESULTS: A total of 550 adult patients were included in the study. The CG value predicting patients with IgG <6 g/L as a screening test was determined as <20 g/L with 83.8% sensitivity and 74.9% specificity. The gamma globulin value which predicted patients with the same IgG value of 89.0% sensitivity and 89.4% specificity was determined as <7 g/L. In the retrospective analysis, 37 of 47 patients (78.7%) with PAD had a CG value of <20 g/L at the time of the diagnosis and all 13 patients (100%) whose gamma globulin values were measured at the time of the diagnosis had a gamma globulin value of <7 g/L. CONCLUSION: The determined CG cut-off value of <20 g/L can be used as a screening test in Turkish adult patients.

Successful desensitization to imiglucerase of an adult patient diagnosed with type I Gaucher disease.

Gaucher disease is the most common lysosomal storage disorder, and enzyme replacement therapy, such as administration of imiglucerase, is the standard therapy. Anaphylaxis to imiglucerase is rarely reported. Here, we report a 26-year-old female who was diagnosed with type 1 Gaucher disease and referred to our Allergy Outpatient Clinic because of an anaphylactic reaction due to imiglucerase enzyme therapy. A desensitization protocol was administered with two different dilutions with an increasing rate of administration delivered in 10 consecutive steps by intravenous infusion in an intensive care setting. No reactions occurred during the procedure, and the total final dose of 2,000 U was successfully administered. To our knowledge, this is the first adult case with successful desensitization to imiglucerase. Desensitization protocols to drugs in chronic disease patients for whom no alternative therapies are available can be lifesaving.

Anaphylaxis in a tertiary adult allergy clinic: a retrospective review of 516 patients.

BACKGROUND: Anaphylaxis is a life-threatening acute allergic reaction that can occur at any age. OBJECTIVE: To determine the frequency, triggering factors, and clinical features of anaphylaxis among adult patients who were referred to a tertiary health care facility. METHODS: A retrospective medical chart review was performed including all patients referred to the outpatient clinic of the adult allergy department in our university hospital between January 1, 2008 and December 30, 2011 to determine cases involving anaphylaxis. RESULTS: A total of 516 (2.11%) patients among 24,443 admissions were diagnosed with anaphylaxis. Although the second highest frequency of anaphylaxis cases took place in 2008, a gradual rise in the frequency was determined from 2009 to 2011. Drugs (90.7%) were the most frequent cause, followed by Hymenoptera stings (5.4%), foods (1.6%), latex (0.4%), and exercise (0.2%) respectively. The clinical manifestations during anaphylaxis reported by patients were cutaneous (n = 292, 56.6%), respiratory (n = 253, 49%), cardiovascular (n = 212, 41%), neuropsychiatric (n = 60, 11.6%), and gastrointestinal (n = 52, 10.1%), respectively. Approximately one fifth of the patients received epinephrine, whereas 43% of patients did not receive epinephrine during their treatment in the emergency room. An epinephrine auto-injector was prescribed to 42 patients (8.1%). CONCLUSION: In this study, the second pattern of National Institute of Allergy and Infectious Disease (NIAID) and the Food Allergy and Anaphylaxis Network (FAAN) diagnostic criteria for anaphylaxis predominated among adult patients. Drugs were the leading triggering factor, followed by Hymenoptera stings, foods, latex, and exercise, respectively. Atopy, asthma, and allergic rhinitis were rarely detected.

Nickel dental alloys can induce laryngeal edema attacks: a case report.

Nickel is a strong immunological sensitizer and may result in contact hypersensitivity. Case reports of allergic reactions to intraoral nickel have occasionally been reported in the published work and these allergic reactions are generally of a delayed type (type IV). Here, we present a case of a nickel allergic patient displaying frequent laryngeal edema attacks which required treatment with epinephrine injections followed by parenteral corticosteroid doses. Her complaints ceased after the removal of the dental bridge and the foods containing nickel. In summary, we propose that in the case of recurrent laryngeal edema attacks without any explainable cause, an allergic reaction due to nickel exposure should be taken into consideration.