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Bloom Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Bloom syndrome is associated with a spectrum of clinical symptoms: (i) growth deficiency (affecting height, weight, and head circumference); (ii) dysmorphic facies (narrow face with underdeveloped malar and mandibular prominences and retrognathia or micrognathia); (iii) feeding problems (slow feeding, decreased appetite); (iv) skin lesions (red, sun-sensitive rash/telangiectasia/poikiloderma on the nose, cheeks, the dorsa of the hands and forearms; cheilitis, blistering and fissuring of the lips, eyebrow and eyelash hair loss, alopecia areata, and vesicular and bullous lesions with excessive or intense sun exposure; café-au-lait macules and areas of hypopigmented skin); (v) immunodeficiency (low plasma IgM and IgA levels); (vi) infections; (vii) reduced fertility (azoospermia or severe oligospermia; premature menopause); (viii) other anomalies (tracheoesophageal fistula, cardiac malformation, absent thumbs, and absence of a toe and malformation of a thumb); (ix) medical complications (chronic bronchitis and bronchiectasis, pulmonary failure; myelodysplasia; diabetes mellitus; cancer) (Figures 64.2 and 64.3) [3,16,19–21].
The respiratory system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Bronchiectasis is defined as the abnormal and irreversible dilatation of bronchi (Figure 8.4). It may affect one lung segment or be widely distributed. Bronchiectasis is the result of repeated episodes of infection, usually with some degree of lung collapse. The bronchial walls are weakened, lack support, and are expanded by the force of inspiration. Bronchiolitis and bronchopneumonia in childhood, cystic fibrosis, and chronic pulmonary TB are common precursors. An obstructing lung cancer can cause localized bronchiectasis in the distal segment.
Staged Bilateral Uniportal Video-Assisted Thoracoscopic Surgery (U-VATS) Extended Lung Resection for Chronic Bronchiectasis
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
In a meta-analysis of 35 studies that included 4,788 patients with bronchiectasis who underwent surgical management, Fan et al. reported the following outcomes: mortality in 1.5%; morbidity in 17%; symptom resolution in 67%; symptoms improved in 28%; and no symptom improvement in 9% [2]. The low morbidity and mortality and high rates of symptom improvement after surgical treatment in localized bronchiectasis indicates that this should be considered when medical management is ineffective. In a retrospective analysis of 86 patients who underwent surgery for bronchiectasis, Balci et al. observed that, while complete resection independently predicted symptom-free outcome (p < 0.05), an FEV1 of less than 60% of the predicted value, an incomplete resection, and preoperative antibiotic therapy independently predicted postoperative complications (p < 0.05) [1]. In an analysis of the United Network of Organs (UNOS) database by Hayes et al., the authors reported that the survival for non-CF bronchiectasis patients on the lung transplant wait list is much higher than those with CF bronchiectasis, suggesting that they are different pathophysiologies [3]. In non-CF patients, who are more likely to survive longer, surgical resection may be a better option than lung transplantation.
Airway disease in hematologic malignancies
Published in Expert Review of Respiratory Medicine, 2022
Ricardo J José, Burton F. Dickey, Ajay Sheshadri
Bronchiectasis is defined as the permanent abnormal dilatation of one or more bronchi visualized on radiological imaging, often associated with bronchial wall thickening [101]. On axial CT images, the bronchial caliber is larger (a bronchial-artery ratio of greater than 1.5) than the accompanying artery at the same level due to lack of the normal airway tapering. Bronchiectasis, however, is only clinically significant when structural airway abnormalities are accompanied by chronic respiratory symptoms such as persistent cough, sputum production, and recurrent respiratory infections. Bronchiectasis itself is the end result of several pathophysiological processes that trigger Cole’s vicious cycle hypothesis of airway inflammation and/or infection that results in bronchial epithelial injury and subsequently promotes further inflammation, injury and infection [102].
Hemoptysis with no malignancy suspected on computed tomography rarely requires bronchoscopy
Published in European Clinical Respiratory Journal, 2020
Christian Lund Petersen, Ulla Møller Weinreich
The two most frequent non-cryptogenic etiologies for hemoptysis in this study, respiratory tract infection (12.6%) and bronchiectasis (2.2%), are consistently reported as major etiologies in previous studies. However, for both of these, considerable variations in reported frequencies exist due to differences in definitions and reporting methods. Most other studies have reported in terms of pneumonia rather than respiratory tract infection, the definition of which varying markedly between reports. Nielsen et al. reported pneumonia as the etiology in 34% of non-cryptogenic cases [4] and the corresponding proportion was approx. 46% in the French population [13], whereas Thirumaran et al. reported only 10%, in part because acute bronchitis constituted 67% of diagnoses [7]. Similar variations can also be found and correspondingly explained for bronchiectasis [4,5,7,9,10,19]. In our study, radiological findings of bronchiectasis were considerably more prevalent than the final diagnosis of bronchiectasis. Also, 20% of patients with a radiological finding of bronchiectasis had respiratory tract infection recorded as their etiology while bronchiectasis was the recorded etiology in just 11% of patients with bronchiectasis on CT. Thus, a considerable proportion of the respiratory tract infections identified may be associated with underlying bronchiectasis, suggesting an underestimation of the proportion of bronchiectasis as the cause of hemoptysis. Nonetheless, it is widely agreed that respiratory tract infection and bronchiectasis, diagnoses not consistently requiring bronchoscopy, are leading causes of hemoptysis.
Contemporary management of bronchiectasis in children
Published in Expert Review of Respiratory Medicine, 2019
Johnny Wu, Anne B Chang, Danielle F Wurzel
Bronchiectasis is the final common pathway of several other disease entities, e.g. immunodeficiencies, primary ciliary dyskinesia (PCD), aspiration, cystic fibrosis (CF). Although many patients with bronchiectasis have unknown etiology, assessment for and treatment of the underlying conditions early is important as intervention can influence disease outcomes. In a child with characteristic clinical features (Table 1), a CT chest followed by a minimum panel of investigations to exclude an underlying cause is recommended. These would include full blood count, IgG, IgM, IgA and IgE, vaccine responses and sweat test for CF. Spirometry would be performed and sputum or bronchoalveolar lavage collected. Further investigations performed on a case-by-case basis would include, e.g. bronchoscopy, interferon-gamma release assay or Mantoux, HIV antibodies, modified barium swallow for aspiration, nasal nitric oxide and nasal ciliary brushing for PCD. The management of bronchiectasis may vary depending on whether a cause is identified (e.g. removal of foreign body for bronchiectasis secondary to foreign body inhalation, immunoglobulin replacement in hypogammaglobulinemia) [36]. This review will focus on generic treatments for bronchiectasis.