Primary pulmonary lymphangiectasia in infancy and childhood
نویسندگان
چکیده
منابع مشابه
Primary pulmonary lymphangiectasia in infancy and childhood.
Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period. The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-...
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heterogenously hyperintense and hypointense on with a renal lymphangiectasia. At 2.5 years of followup, she remains asymptomatic without radiological change. Renal lymphangiectasia is a rare, benign tion of the perirenal lymphatic system. The lymphatic structures that surround the kidney fail to establish normal communication with the rest of the lymphatic system. The physiopathological process...
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Thirty-five children with pulmonary stenosis were catheterized from 1 day to 9 years of age and recatheterized after 2 weeks to 15 years. Right ventricular systolic pressure rose in 24 and the increase was greater in those under than over 5 years old. Pulmonary valve orifice area per square metre of body surface area increased in 12, but did not change in 3, and fell in 17; absolute decrease in...
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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 ...
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This is an opportune time to reappraise primary pulmonary hypertension. The disease is still incurable but recent laboratory studies are beginning to provide clues to its pathogenesis, and we now know that survival is improved by sustained intravenous prostacyclin treatment and by chronic anticoagulation. In 1991, before these treatments were generally available, the median survival was given a...
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ژورنال
عنوان ژورنال: European Respiratory Journal
سال: 2004
ISSN: 0903-1936,1399-3003
DOI: 10.1183/09031936.04.00014004