ABSTRACT
In the original version of this article unfortunately two authors were missing: Dr. Jürgen Weidemann and Dr. Daniel Berthold. The correct list of authors is presented above.
ABSTRACT
Studies of chest computed tomography (CT) in patients with primary antibody deficiency syndromes (ADS) suggest a broad range of bronchial pathology. However, there are as yet no multicentre studies to assess the variety of bronchial pathology in this patient group. One of the underlying reasons is the lack of a consensus methodology, a prerequisite to jointly document chest CT findings. We aimed to establish an international platform for the evaluation of bronchial pathology as assessed by chest CT and to describe the range of bronchial pathologies in patients with antibody deficiency. Ffteen immunodeficiency centres from 9 countries evaluated chest CT scans of patients with ADS using a predefined list of potential findings including an extent score for bronchiectasis. Data of 282 patients with ADS were collected. Patients with common variable immunodeficiency disorders (CVID) comprised the largest subgroup (232 patients, 82.3%). Eighty percent of CVID patients had radiological evidence of bronchial pathology including bronchiectasis in 61%, bronchial wall thickening in 44% and mucus plugging in 29%. Bronchiectasis was detected in 44% of CVID patients aged less than 20 years. Cough was a better predictor for bronchiectasis than spirometry values. Delay of diagnosis as well as duration of disease correlated positively with presence of bronchiectasis. The use of consensus diagnostic criteria and a pre-defined list of bronchial pathologies allows for comparison of chest CT data in multicentre studies. Our data suggest a high prevalence of bronchial pathology in CVID due to late diagnosis or duration of disease.
Subject(s)
Bronchi/pathology , Immunologic Deficiency Syndromes/pathology , Thoracic Wall/pathology , Adolescent , Adult , Aged , Bronchiectasis/pathology , Child , Child, Preschool , Common Variable Immunodeficiency/pathology , Female , Humans , Infant , Male , Spirometry/methods , Tomography, X-Ray Computed/methods , Young AdultSubject(s)
Granulomatous Disease, Chronic/diagnosis , Invasive Fungal Infections/diagnosis , Lung/diagnostic imaging , Tomography, X-Ray Computed/methods , Adolescent , Antifungal Agents/therapeutic use , Granulomatous Disease, Chronic/drug therapy , Humans , Invasive Fungal Infections/drug therapy , Lung/pathology , Treatment OutcomeABSTRACT
Necrotizing fasciitis (NF) is a life-threatening, progressive, bacterial soft tissue infection characterized by necrosis of skin, subcutaneous tissues, fasciae, and muscles. It usually occurs in adults and is most often localized to the abdominal wall, the extremities, the perineum, the pelvis, and the thoracic region. Localization to the head and neck area is rarely encountered, especially in pediatric patients. Early diagnosis and prompt, aggressive surgical treatment associated with intravenous, broad-spectrum antibiotic therapy are mandatory to successfully control the disease. To date, only anecdotal cases of cervical NF in the pediatric age have been described. We report a case of cervical NF with mediastinitis in a 13-year-old girl who underwent successful immediate surgery and prolonged intravenous antibiotic therapy. A literature review is also presented with particular emphasis on etiology, clinical and radiological presentation, diagnosis, and treatment of this rare disorder.
Subject(s)
Fasciitis, Necrotizing/diagnosis , Mediastinitis/diagnosis , Adolescent , Combined Modality Therapy , Diagnosis, Differential , Fasciitis, Necrotizing/drug therapy , Fasciitis, Necrotizing/surgery , Female , Humans , Mediastinitis/drug therapy , Mediastinitis/surgery , NeckABSTRACT
PURPOSE: To compare the efficacy of two different MR contrast agents for the detection and diagnosis of focal nodular hyperplasia (FNH). MATERIALS AND METHODS: Fifty patients with 83 FNH lesions detected on spiral CT were studied in two different MRI sessions with Gd-BOPTA (MultiHance) and ferumoxides (Endorem). MRI with Gd-BOPTA was performed precontrast (T1wGRE and T2wTSE sequences) and during the dynamic and late (1-3 hours) phases after injection (T1wGRE sequences only). MRI with ferumoxides (T1wGRE and T2wTSE sequences) was performed before and at least 30 minutes after injection. Hyper- or isointensity of FNH in the late phase was considered typical for Gd-BOPTA, while isointensity or lesion hypointensity was considered typical for ferumoxides. RESULTS: With Gd-BOPTA, 83 FNH lesions (100%) appeared hyperintense during the arterial phase of dynamic MRI. All but one lesion was iso- or slightly hyperintense in the portal-venous and equilibrium phases. In the late phase, 81 FNH lesions were hyper- or isointense to the surrounding parenchyma, with two lesions appearing slightly hypointense. With ferumoxides, a significant (P < 0.001) number (21/83, 25.3%) of FNH lesions (mean diameter = 16.8 +/- 6.6 mm) were not visible. Of the visible FNH lesions, 38/62 were slightly hyperintense, and 24/62 were isointense to the surrounding parenchyma on the T2wTSE images. On the T1wGRE images, 42/62 lesions were isointense, 19/62 were slightly hyperintense, and one lesion was slightly hypointense. Seventeen lesions in 12 patients with previous neoplasia were all detected after Gd-BOPTA administration, whereas only nine of these 17 lesions (52.9%) were detected after ferumoxide administration. Two of these nine lesions showed atypical enhancement features. CONCLUSION: Gd-BOPTA-enhanced MRI is significantly better than ferumoxide-enhanced MRI for the identification and characterization of FNH.
