Biomedical subjects
Publications and source records attributed to A M Luger.
Two children with the clinical and histopathological features of disseminated histiocytosis X became refractory to chemotherapy and a diffuse erythrophagocytic process developed. No cause of the erythrophagocytosis could be found. Pancytopenia and marked erythrophagocytosis persisted until death ensued. The relationship of this disorder to familial erythrophagocytosis is discussed.
The authors report a case of leiomyosarcoma in the mediastinum presenting with superior vena cava obstruction, and also demonstrate the aggressive nature of this tumor in spite of radiotherapy. Where technically feasible, surgery appears to be the treatment of choice. The pathologic and electron microscopic features of this tumor are also described.
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To examine the role of the renal vascular receptor in the control of renin secretion in the rat, a denervated, nonfiltering kidney model (DNFK) was developed. The left kidney was subjected to a 2-hr period of total renal ischemia followed by ureteral ligation and section Denervation was accomplished by stripping all visible nerves and painting the renal vessels with 5% phenol. Forty-eight hours later lissamine green dye was injected iv and failed to appear in either the cortical or medullary tubules, indicating that glomerular filtration had ceased. Histological study of these kidneys revealed diffuse tubular necrosis with extensive intratubular cast formation. Norepinephrine content of the DNFK was reduced 91% compared to the contralateral normal kidney (P less than 0.001). In another group of anesthetized rats with a single DNFK, 15 min of suprarenal aortic constriction (SAC) increased plasma renin activity (PRA) from 3.4 +/- 0.6 to 11.5 +/- 1.6 ng AI/ml/hr; in a time control series, PRA was unchanged. To exclude the influence of adrenal catecholamines in this response, bilateral adrenalectomy was performed in a separate group of animals with a DNFK. In this series, SAC also markedly increased PRA. The present data indicate that in the rat the macula densa, the renal nerves, and adrenal catecholamines were not essential for the hyperreninemia induced by a reduction in renal perfusion pressure.
Sixteen years earlier a 42-year-old woman with an IgA kappa plasma cell neoplasm presented with bleeding disorder. Her prolonged course was complicated by subsequent development of rheumatoid arthritis, vascular purpura, and an acute membranoproliferative glomerulonephritis (MPGN). The paraprotein and its (Fab')2 fragment showed affinity for a test myeloma IgG2 (lambda ) paraprotein. The patient's serum and the IgA-IgG complex separated by gel filtration did not exhibit cryoprecipitation. The complex also did not dissociate by ultracentrifugation. Electron microscopic and immunofluorescent studies of a renal biopsy sample taken during the episode of nephritis showed subendothelial deposits and a lacy fluorescent pattern strongly positive for IgA and IgG. The same immunoglobulins were eluted from the kidney at postmortem. A low concentration of monoclonal IgA kappa (antibody) and excess unbound polyclonal IgG (antigen) were demonstrated in the patient's serum at the time of MPGN, apparently analogous to the conditions necessary for the induction of experimental immune complex nephritis.
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Presented is a unique case of accelerated hypertension in a 26-year-old black man. Clinically, the patient was found to have a left renal artery lesion and lateralizing renal vein renins. At surgery, a stenosing lesion was found in the artery supplying the upper pole of the left kidney. This was caused by a densely fibrotic mass involving the main renal artery. Microscopy revealed a necrotizing arteritis with no other arteries involved. This case of primary necrotizing arteritis of the main renal artery appears to represent a new entity because of its pathological and clinical differences with the known forms of large vessel arteritis. It is possible that this may represent a particularly acute and necrotizing stage of Takayasu's aortitis, or an extremely rare case of classical periarteritis nodosa confined solely to the main renal artery. The renin tissue assays document the hyperreninemia produced by arterial stenosis. The differential diagnosis of the lesion is considered, and the pathophysiology of hypertension due to large vessel arteritis is discussed.
The ultrastructure, differential diagnosis, and biologic behavior of the peripheral pulmonary spindled carcinoid tumor are reviewed. Electron microscopy is useful in distinguishing the spindled carcinoid from a variety of neoplasms with similar histologic features. The spindled morphology is a rare expression of the carcinoid tumor that is almost exclusively confined to the lung periphery. It appears that the spindled carcinoid without atypical features is fully capable of regional lymph node metastases in approximately 20 per cent of the cases.
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A 39-week-old phenotypically female infant was born with hypoplastic left heart syndrome and expired on the third day of life. An autopsy revealed the patient to also have male pseudohermaphroditism and uterus bicornis bicollis. The association of hypoplastic left heart syndrome and male pseudohermaphroditism has been reported in only two previous patients.
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