Biomedical subjects
Publications and source records attributed to S H Dikman.
A 60-year-old woman with a mediastinal carcinoid of probable thymic origin, widely metastatic, had associated parathyroid hyperplasia, medullary carcinoma of thyroid with amyloid stroma and an adrenal neuroma. This case demonstrated features of an incomplete Sipple's syndrome (MEA II). The relationship of these diverse tumors is consistent with the concept of the APUD system and neurocristopathies.
Electron microscopic analysis of subendothelial and mesangial alterations in the glomeruli was performed in 15 cases of malignant nephrosclerosis (MNS). 8 cases showed segmental or diffuse subendothelial accumulation of proteinaceous 'fibrinoid' material associated with thickening of glomerular basement membranes. 2 of these cases also showed similar deposits in the mesangium. When severe, this mesangial insudation resulted in almost complete replacement and destruction of the mesangial matrix. Endothelial injury with alteration of glomerular microcirculation and secondary intravascular coagulation is believed to play a role in the development of the glomerular lesions in MNS.
Gaucher's disease has been associated with plasma cell dyscrasias. A patient had Gaucher's disease, nephrotic syndrome, and systemic amyloidosis. Plasmacytosis in the bone marrow, the presence of light chains in the urine and renal glomeruli, and the finding of low circulating immunoglobulin levels suggest that the amyloid in this patient is related to a plasma cell dyscrasia.
A patient with renal amyloidosis and the nephrotic syndrome consequent to extensive infected burns demonstrated both clinical resolution of the nephrotic syndrome and morphologic regression of the renal amyloid deposits over a six year period. The regression of the amyloid deposits was associated with several changes in the glomerular capillary wall resulting in a double capillary wall contour. This case indicates that deposits of amyloid in the kidney may regress and suggests a sequence of events in this resolution.
A 19-year-old woman had multiple functioning extra-adrenal paragangliomas, a pituitary adenoma associated with acromegaly, parathyroid hyperplasia, and pigmentary abnormalities. This case differs from previously described instances of multiple endocrine adenomatosis (MEA) and has features that bridge the classic MEA type 1 and 2 syndromes and possibly Von Recklinghausen disease. The coexistence of pheochromocytoma with acromegaly is extremely rare, and the association with extra-adrenal paragangiliomas appears to be unique. Thyroid parafollicular cell proliferation could not be proved by immunohistochemical or electron microscopical studies. The large number and extensive distribution of paragangliomas, ranging from neck to pelvis, is another unique feature of this case. The concept of neurocrestopathy or of an endocrine polypeptide (APUD) cell system may offer an explanation for the interrelation of these diverse growths.
Three patients had unilateral glomerulonephritis. In two, the protected kidney was associated with stenosis of its renal artery, and in the third patient, a hydronephrotic kidney was spared. These clinical examples of unilateral glomerulonephritis and similar experimental models illustrate the effect of hemodynamic and hydrostatic influence in the manifestation of glomerulonephritis. Glomerulonephritis associated with unilateral arterial or ureteral disease may cause clinical confusion and error in diagnosis and treatment.
Electron microscopic analysis of the mesangial injury in the hemolytic-uremic syndrome was performed in 10 patients. Proteinaceous material similar to that found in the subendothelial region was also seen focally in the mesangium altering the matrix and imparting a reticular appearance. This degenerative process was associated with reparative changes in the glomerular tuft. Many of the mesangial cells were hypertrophied and demonstrated phagocytic activity and peripheral extension of their cytoplasmic processes. Mitotic figures in endothelial as well as mesangial cells were regarded as evidence of a reparative process. Severe mesangial insudation of material containing fibrinogen derivatives resulted in segmental tuft necrosis with almost complete replacement and destruction of the mesangial matrix. On some occasions, a break of the glomerular basement membrane was accompanied by the escape of intraluminal contents into the urinary space, leading to crescentic epithelial cell proliferation.
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