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Biomedical subjects

J T Lie

Publications and source records attributed to J T Lie.

At least 91 records · Page 5Linked to original sources
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↗
PubMed1991

De novo systemic vasculitis in a renal transplant recipient.

Abdominal pain, oligoarthritis, macular skin rash, and urine sediment with more than 100 erythrocytes per high-power field and proteinuria developed in a renal transplant recipient who had no prior history of an underlying connective tissue disease. A polyarteritis-type necrotizing vasculitis was detected in the small bowel mesentery. A search for other etiologic factors revealed none. This case demonstrates that de novo vasculitis can develop in renal transplant recipients despite adequate immunosuppressive regimens and may respond to increased dosages of corticosteroids.

Combined Modality Therapy↗
PubMed1991

Causes of death in patients with tuberous sclerosis.

Of the 355 patients with tuberous sclerosis complex (TSC) examined at the Mayo Clinic, 49 had died (9 of causes other than TSC). We attempted to determine what pattern of organ involvement occurred most often in the 40 patients who died of TSC. One baby died of cardiac failure due to cardiac rhabdomyomas, and one child died of rupture of an aneurysm of the thoracic aorta. Eleven patients died of renal disease, which was the commonest cause of death. Ten patients died as a result of brain tumors, and four patients (who were 40 years of age or older) died of lymphangiomyomatosis of the lung. Thirteen patients with severe mental handicaps died of either status epilepticus or bronchopneumonia; in all but one of these patients, the only source of information was the death certificate. Survival curves show a decreased survival for patients with TSC in comparison with that for the general population. Patients with TSC need lifelong follow-up for early detection of potentially life-threatening complications.

Adolescent↗
PubMed1991

Eosinophilic fasciitis associated with use of L-tryptophan: a case-control study and comparison of clinical and histopathologic features.

We investigated the relationship between use of L-tryptophan and development of eosinophilic fasciitis by two methods: a retrospective patient survey and a case-control study of patients with eosinophilic fasciitis diagnosed at the Mayo Clinic between 1977 and 1989. Before 1986, no traceable patients with eosinophilic fasciitis had taken L-tryptophan. Between Jan. 1, 1986, and July 31, 1989, 8 of 34 patients had ingested L-tryptophan (P less than 0.001). In the case-control study, traceable patients with eosinophilic fasciitis were matched with patients who had systemic sclerosis or rheumatoid arthritis and healthy control subjects who had undergone a general medical examination. Of the 60 matched control subjects, 2 had used L-tryptophan. Thus, the odds ratio was 19, indicating a 19-fold greater likelihood of use of L-tryptophan in patients with eosinophilic fasciitis than in the control group. A retrospective assessment of clinical features, response to treatment, and blinded review of biopsy specimens of skin and fascia in patients who had eosinophilic fasciitis with or without exposure to L-tryptophan disclosed no significant differences in the two groups. This retrospective study confirms a strong association between consumption of L-tryptophan and development of eosinophilic fasciitis in some patients. No clinical or histopathologic features were detected that distinguished this disorder on the basis of previous exposure to L-tryptophan.

Adult↗
PubMed1991

Pulmonary hypertension and familial Mediterranean fever: a previously unrecognized association.

Familial Mediterranean fever is an autosomal recessive inherited disorder characterized by recurrent episodes of fever accompanied by inflammation of the peritoneum, pleura, synovial membranes, and skin. The disorder predominantly affects persons of Mediterranean origin. The most serious complication of the disease is amyloidosis, which is the cause of death in a substantial proportion of adult patients with the disorder. Only one previous report has described pulmonary hypertension in a patient with systemic amyloidosis associated with multiple myeloma. Herein we describe the first known occurrence of pulmonary hypertension due to pulmonary amyloidosis in a 48-year-old woman with familial Mediterranean fever. Postmortem examination showed extensive deposits of amyloid in the pulmonary vessels, alveolar capillary walls, and myocardium, which explained the hypoxia, hypotension, and terminal cardiac arrhythmias that were the immediate cause of death in this patient.

Amyloidosis↗
PubMed1991

Histopathologic features of the L-tryptophan-related eosinophilia-myalgia (fasciitis) syndrome.

Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.

Collagen↗
PubMed1991

Angiitis of the central nervous system.

Angiitis signifies an inflammation of the blood vessels that can cause damage to vessel walls, vascular occlusion, and ischemic injury to organs served by these vessels, and it can occur in the central nervous system. All central nervous system vasculitides but one have been linked to the presence of various underlying disorders. This review covers primary and spinal cord granulomatous angiitis of the central nervous system, as well as secondary angiitis of the central nervous system and its associated underlying disorders.

Central Nervous System Diseases↗