Pulmonary Functions in Children With Thalassemia Major.

BACKGROUND: Thalassemia major (TM) is a chronic disease requiring regular transfusions that may result in generalized iron loading, such as in the heart, the liver, endocrine organs, and the lungs. We aimed to determine pulmonary function abnormalities in children with TM in our center. PATIENTS AND METHODS: In this study, pulmonary function tests (PFTs) of 49 patients with TM who received regular blood transfusion and had no history of chronic respiratory disease were evaluated. The relationship between PFTs and the age, the body surface area, pretransfusional hemoglobin, and serum ferritin was evaluated. RESULTS: Among the ß-TM patients included in this study, 61% were male and 39% were female, with a mean age of 10.8±3 years (range, 5 to 17 y). The patients' mean level of ferritin was 3873±2011 ng/dL (range, 676 to 9476 ng/dL). A reduced forced vital capacity (FVC) was found in 33 patients (67%). A reduced forced expiratory volume in 1 second (FEV1) was found in 15 patients (30%). A forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio of >80% was found in all patients. The peak expiratory flow (PEF) was decreased in 23 patients (46.9%). The forced mid-expiratory flow between 25% and 75% of the exhaled vital capacity (MEF25%-75%) was decreased in 5 patients (10%). FVC and FEV1 values in patients with a high ferritin level (>2500 ng/dL) were decreased compared with patients with a low ferritin level (<2500 ng/dL) (P=0.04, 0.03). FVC, FEV1, and PEF parameters were negatively correlated with the age and the body surface area. Age was a predictor of FVC (ß=-0.450, P<0.001), FEV1 (ß=-0.419, P<0.001), and PEF (ß=-0.505, P<0.001), and hemoglobin was a predictor of FEV1/FVC (ß=0.366, P=0.01) and MEF25%-75% (ß=0.323, P=0.003). CONCLUSIONS: Our results concluded that the respiratory system should be evaluated by PFTs even in asymptomatic patients with high serum ferritin levels during the adolescent period annually to prevent the squeal of pulmonary disease in TM. Patients who have abnormal PFTs should be reevaluated for compliance with chelation therapy and the transfusion program.

Negative predictive value of drug provocation tests in children.

BACKGROUND: The negative predictive value of the drug provocation test is important for both the patient and the physician. However, in children, this predictive value is unresolved. METHODS: The study included patients who had drug provocation test with a suspected drug and was diagnosed as 'not allergic to the drug'. Three months after allergy workup, the patients were contacted and asked for reexposure to the tested drug. Patients who have reported reactions during reexposure were reevaluated with skin tests and drug provocation. RESULTS: During the study period, 217 provocations were performed to 203 patients. Of these, 163 patients (80.3%) with 175 negative drug provocation tests could be contacted. Ninety-one (52%) of the 175 cases reported to use the tested drug again, and 11 (12%) of these cases declared that they had a new reaction. Two of the eleven cases refused reevaluation. Nine cases were evaluated by drug allergy workup. Two of the nine cases were classified as allergic after retests. Collectively, the negative predictive value was 95.6% (87 of 91 cases) for all drug challenges. CONCLUSIONS: The negative predictive value of the drug provocation test is abundant in children; however, negative drug provocation tests do not necessarily predict tolerance for the drug.

Serum sickness-like reaction in children due to cefditoren.

We describe the case of a 7-year-old boy with urticaria, fever, and arthritis that appeared 10 days after starting cefditoren therapy for acute tonsillopharyngitis, which was diagnosed as a serum sickness-like reaction due to this medication.