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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 127 records · Page 7Linked to original sources

The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis.

Criteria for the classification of Takayasu arteritis were developed by comparing 63 patients who had this disease with 744 control patients with other forms of vasculitis. Six criteria were selected for the traditional format classification: onset at age less than or equal to 40 years, claudication of an extremity, decreased brachial artery pulse, greater than 10 mm Hg difference in systolic blood pressure between arms, a bruit over the subclavian arteries or the aorta, and arteriographic evidence of narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities. The presence of 3 or more of these 6 criteria demonstrated a sensitivity of 90.5% and a specificity of 97.8%. A classification tree also was constructed with 5 of these 6 criteria, omitting claudication of an extremity. The classification tree demonstrated a sensitivity of 92.1% and a specificity of 97.0%.

Adult↗

Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients.

Between 1978 and 1988, three patients at our institution had an initial diagnosis of idiopathic pulmonary fibrosis but later were correctly diagnosed as having pulmonary-renal syndrome and microscopic polyarteritis. These cases involved elderly patients with progressive dyspnea and nonproductive cough, bilateral dry crackling rales, bilateral interstitial infiltrates evident on a chest roentgenogram, and restrictive findings on pulmonary function testing. In two patients, lung biopsy specimens were obtained, and an initial diagnosis of nonspecific pulmonary fibrosis was made. All three patients eventually had microhematuria and renal insufficiency. A revised diagnosis of small-vessel pulmonary-renal vasculitis was based on the demonstration of segmental necrotizing glomerulonephritis in renal biopsy specimens in two patients, thrombotic microangiopathy consistent with healed vasculitis on postmortem examination of the kidney in one patient, and subsequent detection of small-vessel vasculitis on review of the two lung biopsy specimens. Anti-neutrophil cytoplasmic antibodies with perinuclear staining on indirect immunofluorescence microscopy were positive in the two patients in whom determinations were performed. The clinical manifestations of vasculitis were notably scarce--no involvement of the skin, nervous system, or gastrointestinal tract; no episodes of fever; and minimal or absent musculoskeletal symptoms. These cases illustrate the importance of a high index of suspicion for the diagnosis of systemic vasculitis in elderly patients and the need to consider a vasculitis in the differential diagnosis of idiopathic pulmonary fibrosis, especially if an active urinary sediment is present.

Aged↗

Diagnostic histopathology of major systemic and pulmonary vasculitic syndromes.

The vasculitides are a heterogeneous group of diseases with protean and frequently overlapping clinical and pathologic manifestations. They are difficult to classify because the etiology and pathogenesis of most vasculitides are unknown. A definitive diagnosis of vasculitis almost invariably requires histologic documentation of the disease. Although each major vasculitic syndrome may have its own unique and characteristic histologic features, the correct interpretation of biopsies still depends on such factors as the pathologist's interest and experience, tissue selection, sample size, chronologic age of the disease process, and any prior treatment at the time of the biopsy.

Adolescent↗

Disseminated Aspergillus terreus infection in immunocompromised hosts.

Aspergillus terreus is ubiquitous in the environment but has rarely been found to be pathogenic. When recovered from clinical specimens, it is commonly considered a saprophyte. We report two cases of fatal disseminated A. terreus infection. The first patient was receiving corticosteroid therapy for immune thrombocytopenia when the condition developed, and the second patient was receiving immunosuppressive therapy after bone marrow transplantation for myelodysplasia. We also describe the frequency of recovery of A. terreus in our laboratory. The serious pathogenic potential of A. terreus in immunocompromised hosts should be recognized.

Adrenal Cortex Hormones↗

The rise and fall and resurgence of thromboangiitis obliterans (Buerger's disease).

Thromboangiitis obliterans (TAO; or Buerger's disease) is a nonarteriosclerotic, segmental, occlusive inflammatory disease of medium-sized and small arteries and veins of unknown cause, occurring predominantly in young male habitual tobacco users. Less than 2% of the patients with TAO are women or elderly persons. TAO is a treatable disease when diagnosed early and if strict abstinence from tobacco is observed. Otherwise progression and exacerbation of limb ischemia may lead to multiple and repeated amputations, and rarely visceral or cerebral ischemia. Over a 40-year period, 1947 to 1986, the prevalence rate of the diagnosis of TAO has declined steadily from 104/100,000 in 1947 to 13/100,000 in 1986 (an 8-fold decrease) where clinical and pathologic criteria for the diagnosis of TAO have remained unchanged. A similar pattern of decline was observed in two other major medical centers. Twelve women and 97 men with TAO were identified in the 5-year period 1981-1985. The 11% (12 of 109) incidence of female TAO was 10-fold that was previously reported. All 12 women were heavy smokers; 8 (67%) had thrombophlebitis and 6 (50%) had upper limb ischemia. Nine (75%) of 12 women required amputations; delay in diagnosis contributed to additional amputations in 2 patients. The increased prevalence of TAO in women may be attributed to the smoking habit in young women overall, which has increased significantly rather than decreased (as in males) since 1965.

Age Factors↗

Pulmonary hypertension in the CREST syndrome: variant of systemic sclerosis (scleroderma)--a case report.

The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.

Calcinosis↗

Sudden death as the initial manifestation of diffuse pleural mesothelioma.

Malignant mesotheliomas are generally considered pathognomonic of asbestos exposure. The prognosis of mesothelioma varies with clinical staging of the disease. The overall survival of patients from the time of diagnosis is six to 16 months. Although patients with malignant mesotheliomas often have nonspecific symptoms and signs, occult diffuse pleural mesothelioma is uncommon, and one that occurs in a patient with no evidence of asbestos exposure must be exceedingly rare. Sudden death as the initial manifestation of pleural mesothelioma, to our knowledge, has not been previously reported in the literature. One such unusual case is documented here.

Adult↗

Petrified cardiac myxoma masquerading as organized atrial mural thrombus.

To our knowledge, petrified cardiac myxoma is a heretofore undescribed histologic variant of atrial myxomas. It is an obsolescent myxoma that has been converted to a sclerosiderotic nodule by impregnation of the tumor's stromal connective tissue fibers with iron, calcium, and ceroid pigments, presumably following recurrent hemorrhage in the myxoma over time. Four cases of this unusual histologic variant of cardiac myxoma were identified from among 22,150 autopsies performed over the 25-year period, 1954 through 1979. These four cases were asymptomatic incidental postmortem findings discovered in two men (54 and 82 years of age) and two women (66 and 80 years of age). The original autopsy diagnoses of the left atrial lesions were fibrosiderotic nodule (Gamna-Gandy body) in one patient and organized mural thrombi in the other three patients.

Aged↗

Isolated eosinophilic coronary arteritis and eosinophilic myocarditis. A limited form of Churg-Strauss syndrome.

Eosinophilic coronary arteritis is rare, and it occurs principally as a manifestation of cardiac involvement in Churg-Strauss syndrome (allergic granulomatosis and angiitis). A 39-year-old white man, who was an asthmatic, died suddenly and unexpectedly. Autopsy showed isolated eosinophilic coronary arteritis associated with myocardial necrosis, and isolated eosinophilic myocarditis as the cause of death. This limited form of Churg-Strauss syndrome, to our knowledge, has not been reported previously.

Adult↗