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  • Title: Hereditary late-onset lymphedema with pleural effusion and laryngeal edema.
    Author: Herbert FA, Bowen PA.
    Journal: Arch Intern Med; 1983 May; 143(5):913-5. PubMed ID: 6679236.
    Abstract:
    We examined two middle-aged male cousins with unexplained edema of postpubertal onset involving the upper and lower limbs, face, and larynx and, in one of them, a persistent pleural effusion. Scintilymphangiography detected an apparent paucity or absence of lymph nodes in the axillae and above the inguinal ligaments, indicating a defect in the lymphatic systems. Laryngeal edema, confirmed endoscopically, produced changes in one of them in the flow volume loop characteristic of a variable extrathoracic obstruction. A family study showed autosomal dominant transmission of the disorder. The nosology of late-onset lymphedema is briefly discussed, with particular reference to the so-called yellow nail syndrome.
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