12 research outputs found

    Congenital pulmonary lymphangiectasia

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    <p>Abstract</p> <p>Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.</p

    A systematic review of CD14 and toll-like receptors in relation to asthma in Caucasian children

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    The aetiology of childhood asthma is complex. An early dysfunction in immunological development of the innate immune system in combination environmental factors possibly triggers asthma. CD14 and toll-like important components of the innate immune system. The aim of this review was to obtain a better insight into the relation between CD14 and toll-like receptors and childhood asthma in Caucasians. We searched EMBASE for relevant articles. In total, 44 articles were included. The the selected studies was independently assessed by the first two authors the Newcastle-Ottawa quality assessment scale. Toll-like receptor 2, receptor 6, toll-like receptor 9, and toll-like receptor 10 appear to association with childhood asthma in Caucasians. The evidence for a CD14 with childhood asthma is limited. In conclusion, there is no evidence yet for a role of CD14 and toll-like receptors in relation to asthma. Future studies should include haplotype analysis and take factors into account to further clarify the role of CD14 and toll-like on childhood asthma
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