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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
PubMed2000

Pulmonary arterial fibromuscular dysplasia: a rare cause of fulminant lung hemorrhage.

Arterial fibromuscular dysplasia (FMD) represents a collection of noninflammatory and nonatherosclerotic vascular diseases with a poorly understood etiology. Classically occurring in renal and cerebral arteries, this entity has also been reported in coronary, carotid, and other medium and small arteries. One case occurring in the pulmonary vasculature has been reported. Fatal hemothorax and lung hemorrhage have multiple causes, including other vascular malformations and connective tissue disorders; however, cases of pulmonary FMD are exceedingly rare. We report what appears to be the second such association, occurring in a 69-year-old man. The patient presented with a 3-week history of increasing dyspnea, fatigue, and productive cough; 3 days of increasing back and chest pain; and syncope. Chest radiograph showed a "white-out" of the left lung. The patient died shortly after admission from a fulminant respiratory disease of undetermined etiology. At autopsy he was found to have a massive left hemothorax resulting from an unsuspected pulmonary arterial fibromuscular dysplasia.

Aged↗
PubMed1999

Infantile disseminated visceral giant cell arteritis presenting as sudden infant death.

The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.

Arteries↗
PubMed1998

Pathology of isolated nonclassical and catastrophic manifestations of Takayasu arteritis.

Takayasu arteritis is usually defined as a chronic, progressive, inflammatory, occlusive disease of the aorta and its branches. However, we should remind Takayasu arteritis as a systemic disease. Here I describe nonclassical and catastrophic manifestations of the Takayasu arteritis, which often go unrecognized until after the event. Especially I stress that we should focus on cardiopulmonary complications in Takayasu arteritis.

Aortic Dissection↗
PubMed1998

Visceral intestinal Buerger's disease.

Buerger's disease is a non-arteriosclerotic, segmental, progressive, inflammatory vaso-occlusive disease of unknown etiology. Buerger's disease occurs almost exclusively in susceptible young men who are habitual tobacco users; usually with onset of symptoms before the age of 40 years. Buerger's disease affects both arteries and veins of principally lower and upper limbs and, rarely, of the viscera. To date, only 16 confirmed cases of visceral-intestinal Buerger's disease have been reported in the English-language literature; and all 16 patients were men. We describe here, for the first time, two young women with intestinal Buerger's disease who died of complications of ischemic bowel disease.

Adult↗
PubMed1998

Segmental mediolytic arteriopathy of the splenic and hepatic arteries mimicking systemic necrotizing vasculitis.

Segmental mediolytic arteriopathy, a rare, noninflammatory arterial disease, is fundamentally a variant of fibromuscular dysplasia. The characteristic angiographic findings of segmental mediolytic arteriopathy include the "string of beads" and microaneurysms which are indistinguishable from those of vasculitis, and the correct diagnosis can be made only after histopathologic evaluation of the arterial lesions. Thrombosis, arterial wall hemorrhage, and dissection are among the complications of segmental mediolytic arteriopathy. We describe herein a patient with segmental mediolytic arteriopathy who presented with hemoperitoneum. The patient underwent urgent surgical repair of a ruptured hepatic artery aneurysm. The postoperative visceral arteriography findings led to a clinical diagnosis of polyarteritis nodosa, and immunosuppressive therapy was initiated. This treatment was stopped as soon as the correct biopsy diagnosis of segmental mediolytic arteriopathy was obtained through outside consultation. The patient recovered without drug treatment and was spared the potentially life-threatening complications of immunosuppression.

Aged↗
PubMed1998

Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients.

We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used.

Adolescent↗
PubMed1997

Classification and histopathologic spectrum of central nervous system vasculitis.

There are many different and diverse causes of central nervous system (CNS) vasculitis, and many nonvasculitic disorders that often mimic CNS vasculitis. CNS vasculitis is usually suspected clinically with compatible or suggestive angiographic findings, but a definitive diagnosis is not possible without biopsy confirmation, especially with CNS vasculitis mimickers. Primary CNS vasculitis, although relatively uncommon, is most important because of its overall unfavorable prognosis. Secondary CNS vasculitis occurs in association with a long list of systemic vasculitic and nonvasculitic disorders with variable brain biopsy findings. Because of the focal and segmental distribution of CNS vasculitis, a positive biopsy is diagnostic for the disease demonstrated, but a single isolated negative biopsy does not necessarily exclude primary or secondary CNS vasculitis.

Brain Diseases↗
PubMed1997

Biopsy diagnosis of systemic vasculitis.

A definitive diagnosis of virtually all vasculitides requires histological documentation. Although each major type of systemic vasculitis may have its own unique features, variability and overlaps still exist, and histopathological specificity is rarely an absolute discriminator. The interpretation of biopsies for the diagnosis of vasculitis remains more an art than a science, and it requires full and complete correlation with historical, clinical, laboratory, and angiographic findings.

Biopsy↗
PubMed1997

Disseminated lipogranulomas and sudden death from self-administered mineral oil injection.

The cosmetic injection of exogenous lipids and more recently of polydimethyl siloxane (injectable silicone) into the scrotum has been described since 1899. Sclerosing lipogranuloma and paraffinoma are terms applied to a complication of this practice in which the injected oils or silicone elicit a marked granulomatous reaction with prominent surrounding fibrosis. Although this complication has been described as a localized process occurring mainly in the scrotal area and regional lymph nodes, few studies have documented systemic manifestations. In this report we describe the autopsy findings of a 48-year-old man who had scrotal and systemic lipogranulomas from repeated self-administered injection of mineral oil. In addition, severe acute pulmonary edema resulted in sudden unexpected death. To our knowledge, this fatal complication of exogenous lipogranuloma has not been previously reported.

Death, Sudden↗
PubMed1997

Intramural sarcoma of the carotid artery with adventitial inflammation and fibrosis resembling 'inflammatory aneurysm'.

A rare case is described of an intramural sarcoma of the right common carotid artery coexisting with adventitial inflammation and fibrosis, resembling 'inflammatory aneurysm', which was resected from a 33-year-old Japanese woman who had presented with a pulsatile mass on the right side of the anterior neck. Grossly, the wall of the carotid artery showed an intimal tear with dissection of the media filled with thrombus. A grayish area, abutting directly onto the dissected space and involving the media and inner adventitia, was composed of alpha-smooth muscle actin-positive and desmin-negative polygonal and spindle cells with large blunt-ended nuclei and coarse granular chromatin arranged into a well-organized interlacing bundle pattern. This portion was thus considered to represent leiomyosarcoma. White to yellow-tan fibrotic tissue present in the adventitial area consisted of extensive lamellar fibrosis with scattered foci of lymphoplasmacytic aggregates and obliterated arteries, and lacked atypical spindle and polygonal cells. These changes accorded with the histopathological findings hitherto described in cases of 'inflammatory aneurysm', which is known to almost exclusively involve the abdominal aorta. We consider this case unique in that the leiomyosarcoma involved an artery other than the aorta, with an 'inflammatory aneurysm'-like reaction in the same site. The possible relationship between these two conditions is discussed.

Adult↗
PubMed1997

Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia.

OBJECTIVE: To report three cases of pulmonary or myocardial disease (or both) and necrobiotic xanthogranuloma. MATERIAL AND METHODS: Giant cell granulomas of the lung and myocardium were demonstrated in three patients who had pulmonary and myocardial lesions of necrobiotic xanthogranuloma in conjunction with skin lesions, leukopenia, paraproteinemia, and complement deficiencies. The patients were two men who were 47 and 64 years of age and a 39-year-old woman. RESULTS: Biopsies of skin and visceral lesions showed asteroid and cytoplasmic inclusions. B-cell lymphoid nodules were found. In one of the male patients, a major clonal T-cell receptor gene rearrangement was detected in the peripheral blood. Prednisone was ineffective in two of the patients. The other patient experienced regression of skin lesions and diminishment of a chest nodule after receiving alkylating agent therapy. CONCLUSION: Establishing the correct diagnosis is important, and apparently it is possible to establish the nature of the myocardial and pulmonary lesions with use of appropriate scans and by biopsy. Successful treatment of necrobiotic xanthogranuloma skin lesions with corticosteroids or alkylating agents (or both) implies that evolution of serious disease that compromises the heart and lungs could be controlled.

Adult↗
PubMed1997

Mesenteric inflammatory veno-occlusive disease (MIVOD): an emerging and unsuspected cause of digestive tract ischemia.

BACKGROUND: Mesenteric inflammatory veno-occlusive disease (MIVOD) is a new clinicopathological entity and an unsuspected cause of digestive tract ischemia in the 17 patients reviewed in this article. PATIENTS AND METHODS: Of this series, MIVOD occurred twice as often in men as in women, and the age of affected patients ranged from 24 to 78 years. Unexplained ischemic bowel disease was the most common clinical presentation of MIVOD. All patients required surgical exploration and underwent resection of ischemic or gangrenous bowel. None of the patients had a known underlying systemic vasculitis, connective tissue disease, inflammatory bowel disease, infection, drug allergy, or ingestion of food contaminants or toxins. RESULTS: In general, a correct diagnosis of MIVOD is possible in virtually all cases only after careful histological examination of the resected specimens because the endoscopic biopsy findings may be inconclusive. The inflammatory infiltrate of active MIVOD may be lymphocytic, necrotizing, granulomatous, or mixed, and thrombosis is almost invariably also present. The late changes of MIVOD, concentric or eccentric myointimal hyperplasia and occlusive phlebosclerosis, represent organized thrombi. CONCLUSION: MIVOD probably occurs more commonly than is generally recognized. To a casual observer, the presence of thrombosis may overshadow the inflammatory component of a veno-occlusive disease, especially in the absence of arterial vasculitis, many cases of MIVOD can conceivably go undiagnosed.

Adult↗