54. Lymphomatoid Granulomatosis Definition Lymphomatoid granulomatosis (LYG) is a rare angiodestructive, lymphoproliferative disease with significant pulmonary involvement in the form of pulmonary angiitis and granulomatosis. LYG mimics Wegner’s granulomatosis (see…
53. Lymphangioleiomyomatosis Definition Lymphangioleiomyomatosis (LAM) affects women during the childbearing years. It is characterized by proliferation of smooth muscle phenotypic neoplastic cells in the lungs, kidneys, and axial lymphatics. The…
50. Long QT Syndrome Definition Long QT syndrome (LQTS) is prolonged duration of the QT interval in the cardiac cycle. This syndrome can be congenital or acquired. The congenital form…
52. Ludwig’s Angina Definition Ludwig’s angina is cellulitis of the mouth floor. It expands very rapidly and can spread to other areas of the body, such as the mediastinum or…
51. Lown-Ganong-Levine Syndrome Definition Lown-Ganong-Levine (LGL) syndrome is a preexcitation syndrome producing supraventricular tachycardia that is neither atrial fibrillation nor atrial flutter. This syndrome is characterized by a short P-R…
47. Incontinentia Pigmenti Definition Incontinentia pigmenti is a rare, X-linked, dominant inherited disorder involving skin pigmentation. Melanin is lost from the basal cells of the epidermis and collects in the…
44. Hydatid Disease Definition Hydatid disease is a parasitic infestation by a tapeworm of the genus Echinococcus. Incidence The incidence of hydatid disease in the United States is <1:1,000,000, except…
28. Eisenmenger Syndrome Definition Eisenmenger syndrome (ES) is the presence of a bidirectional or exclusively right-to-left cardiac shunt in conjunction with pulmonary hypertension and a patent heart defect. Incidence Congenital…
27. Ehlers-Danlos Syndrome Definition Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders that vary according to clinical and biochemical evidence, inheritance mode, and disorder severity (mild to…
43. Huntington’s Chorea Definition Huntington’s chorea is an incurable, autosomal dominant inherited disorder, with onset as an adult, associated with loss in specific subsets of neurons in the basal ganglia…
