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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 19 recordsLinked to original sources
PubMed1992

Malignant angioendotheliomatosis (intravascular lymphomatosis) clinically simulating primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (PACNS) and malignant angioendotheliomatosis (MAE) are two unrelated and lethal diseases that frequently affect the brain. Both are uncommon and both require histologic documentation of their characteristic lesions for a definitive diagnosis. Because brain biopsies are not routinely included in the diagnostic workup of non-neoplastic CNS diseases, a number of unconfirmed nonvasculitic conditions may mimic PACNS. To my knowledge, MAE simulating PACNS clinically and angiographically has not been previously reported, and 3 such cases are described herein.

Brain Diseases↗
PubMed1992

Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature.

The clinical and pathologic features of 15 new cases of the uncommon primary or granulomatous angiitis of the central nervous system (PACNS) are described. To date, only 108 such cases have been reported in the English literature. Clinically, most PACNS patients have been young or middle-aged (mean age, 45 years; range, 3 to 96 years), with men outnumbering women slightly by a ratio of 4 to 3. The most frequent presenting complaints are headache, weakness, and confusion; less common complaints are aphasia, dysphasia, nausea or vomiting, loss of memory, and seizure disorder. There is usually no evidence of a systemic disease; the erythrocyte sedimentation rate is almost invariably normal, and there are no diagnostic laboratory tests. The cerebral angiogram usually shows multifocal, segmental stenosis or irregularity of small and medium-sized leptomeningeal and intracranial blood vessels, often with a beading or aneurysmal appearance, and alterations in blood flow in the affected regions. Anatomically, the angiitis is focal and segmental in distribution. An isolated negative biopsy, therefore, does not rule out the disease. Histologically, PACNS may be granulomatous, necrotizing, or lymphocytic in character, and mixed morphologic types often occur. Large- and small-vessel thrombosis is common. Acute lesions frequently coexist with healing or healed lesions. Involvement of extracranial blood vessels occurs only rarely. Past or current herpes zoster infection and Hodgkin's lymphoma are the most noteworthy clinical associations of PACNS, but whether they are causally related remains uncertain.

Adult↗
PubMed1992

Magnetic resonance imaging of facial vascular anomalies.

In 36 patients, facial vascular anomalies were studied with 46 magnetic resonance (MR) examinations, 9 angiograms, and 5 computed tomographic scans. All lesions were categorized into classic pathologic groups on the basis of radiologic and pathologic studies, clinical examination, and behavior. Overall, 2 juvenile hemangiomas, 3 capillary malformations (port-wine stains), 18 venous malformations, 9 lymphatic malformations, and 4 arteriovenous malformations were found. MR imaging was superior to computed tomography and angiography for demonstrating the precise anatomic extent of the facial vascular anomalies and their relationship to the adjacent soft tissues but was inferior to computed tomography for demonstrating radiopaque structures such as trophic bone changes and phleboliths. MR imaging was also inferior to angiography in determining the nidus and the exact nature of collateral vascular structures in arteriovenous malformations. MR studies confirmed the clinically suspected diagnosis of facial vascular anomalies and demonstrated typical characteristics for each type of lesion. MR imaging is an ideal initial technique to triage patients with facial vascular anomalies for appropriate management, including observation, endovascular therapy, or surgical excision.

Adolescent↗
PubMed1992

Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg-Strauss syndrome.

Isolated eosinophilic coronary arteritis expressed as a limited variant of the Churg-Strauss syndrome (allergic granulomatosis and angiitis) is a rare condition. Equally as rare is the entity of isolated spontaneous coronary arterial dissection associated with eosinophilic arteritis. A 57-year-old woman with a history of asthma and recurrent hypersensitivity (anaphylactoid) reactions to various exogenous allergens was found dead in her home; no premonitory complaints had been noted during the preceding days. Autopsy revealed focal occlusion of the left anterior descending and first diagonal coronary arteries by discrete dissecting hematomas of the media as the cause of sudden and unexpected death. Histologically, the affected arterial wall showed eosinophilic inflammation characteristic of this limited expression of the Churg-Strauss syndrome. To our knowledge, sudden cardiac death caused by arterial dissection in isolated eosinophilic coronary arteritis has not previously been reported.

Arteritis↗
PubMed1992

Diagnostic value and limitations of orbital biopsy in Wegener's granulomatosis.

Patients with Wegener's granulomatosis may present with ophthalmic involvement in either the classic or limited forms. Although the overall clinical picture and serologic testing for antineutrophil cytoplasmic antibody (ANCA) are important, biopsy is often necessary for a definitive diagnosis. Accurate interpretation of these orbital biopsies is essential. The authors reviewed the histopathologic features of 13 orbital biopsies in patients with well-documented Wegener's granulomatosis. The classic triad of vasculitis, tissue necrosis, and granulomatous inflammation was seen in 7 of 13 biopsies (54%). Vasculitis in combination with other microscopic findings was seen in 4 of 13 (31%) biopsies. Giant cells were seldom seen. Two biopsies showed only perivascular infiltrates. Based on this study, it appears that a spectrum of histopathologic features can be seen in orbital biopsies in Wegener's granulomatosis. This study underscores the importance of clinical correlation, the application of the ANCA test, and comparison with extraorbital biopsies, if available, when interpreting orbital biopsies in the diagnosis of Wegener's granulomatosis.

Adolescent↗
PubMed1992

Retroperitoneal polyarteritis nodosa presenting as ureteral obstruction.

Polyarteritis nodosa (PAN) may be systemic or isolated in distribution and may involve virtually any organ or tissue in the body. Retroperitoneal PAN with ureteral obstruction as the first manifestation of the disease, to our knowledge, has not been previously reported. Two such unusual cases are described.

Adolescent↗
PubMed1992

Primary antiphospholipid syndrome with multiorgan arterial and venous thromboses.

A 57-year-old man with no evidence of infection, vasculitis or connective tissue disease died with multiple organ thromboses after an acute illness. He was found to have lupus anticoagulant, IgG anticardiolipin antibody, false positive rapid plasma reagin, prolonged partial thromboplastin time, and thrombocytopenia. Venous and arterial thrombi leading to necrosis were found in his scrotum, testicles, upper and lower extremities, adrenals, kidneys, lungs, and brain. No other explanation could be found for his fatal illness, thus suggesting the primary antiphospholipid syndrome (APS). This is a documented case of primary APS associated with multiorgan arterial and venous thromboses of large and small vessels, presenting as a fulminant and fatal acute illness.

Antibodies, Anticardiolipin↗
PubMed1992

Sudden death associated with aortitis and fibrosclerosing disease of the conduction system.

Aortitis is known to complicate a number of autoimmune diseases and syphilis. In most patients with autoimmune disease, arthritis is the initial presentation followed by aortic insufficiency. We report two cases of ostensibly healthy, middle-aged men in whom the initial manifestation of aortitis was sudden death. In each patient, there was extension of inflammation from the aorta into the atrioventricular node. These cases emphasize the importance of examining the conducting system in cases of sudden death associated with aortitis and no grossly evident cause of death. To our knowledge, this is the first report of aortitis presenting as sudden death.

Aortitis↗
PubMed1992

Dissection of the aorta and coronary arteries associated with acute cocaine intoxication.

Acute myocardial ischemia, cardiac arrhythmias, and conduction disturbances are the most common cardiovascular complications of cocaine and its alkaloidal freebase "crack." Cocaine abuse-related acute aortic dissection has been reported only rarely: three cases in the last 5 years. Described herein, to our knowledge, is the first case of cocaine intoxication-associated acute aortic dissection that also involved the coronary arteries, causing sudden death.

Adult↗
PubMed1992

Atypical eosinophilic fasciitis localized to the hands and feet: a report of four cases.

We describe 4 patients who had episodic localized swelling of an extremity, 2 of the hand and 2 of the foot, in combination with peripheral eosinophilia and a biopsy characteristic of eosinophilic fasciitis (EF). Surgical excision produced marked improvement in the patients' symptoms. Whether these patients, despite a characteristic biopsy, can be included in the spectrum of EF remains uncertain.

Adult↗
PubMed1992

Vasculitis simulators and vasculitis look-alikes.

A variety of common and uncommon nonvasculitic disorders may mimic vasculitis clinically, angiographically, or even histologically. These are the vasculitis simulators and vasculitis look-alikes. It is important to distinguish these vasculitis simulators from true vasculitis in order to avoid the unnecessary and harmful side effects of corticosteroids and cytotoxic agents, the mainstay of drug therapy for vasculitis.

Autoimmune Diseases↗
PubMed1991

Granulomatous angiitis of the spinal cord associated with Hodgkin's disease.

A 28-year-old man had a 5-month history of focal and generalized neurologic symptoms culminating in a thoracic myelopathy. Evaluation revealed granulomatous angiitis of the spinal cord in association with occult nodular sclerosing Hodgkin's disease. In previous reports, manifestations indicative of intracranial involvement have dominated the clinical presentation of granulomatous angiitis associated with Hodgkin's disease. Successful therapy for Hodgkin's disease may result in marked improvement of associated granulomatous angiitis, whereas the lack or failure of therapy results in a uniformly fatal outcome. Definitive antemortem diagnosis of granulomatous angiitis requires a biopsy of involved tissue. The cause of granulomatous angiitis, as well as the nature of its association with Hodgkin's disease, remains unexplained.

Adult↗