PubMed Health⌕ Search

Biomedical subjects

J L Olson

Publications and source records attributed to J L Olson.

15 recordsLinked to original sources
PubMed1992

Indian childhood cirrhosis in an American child.

Indian childhood cirrhosis is a fatal liver disease characterized by a striking accumulation of copper-containing granules within hepatocytes. A two-year-old American boy, the product of a third-cousin marriage, with clinical, biochemical, and histological signs of Indian childhood cirrhosis was studied. Liver biopsies at 22 and 30 months of age revealed a rapid progression from fibrosis to micronodular cirrhosis, with many of the remaining hepatocytes staining strongly for copper and copper-binding proteins. Electron microscopy showed characteristic dense granules containing copper and sulfur by electron probe analysis. Hepatic copper content was 1500 micrograms/g dry weight (normal, 20-50). Urinary copper was 3.6 mumol/d (229 micrograms/24 hours; normal, 15-20), and serum ceruloplasmin was 352 mg/L (normal, 150-320). The case suggests that both genetic and environmental components contribute to the manifestations of Indian childhood cirrhosis, and that the diagnosis of Indian childhood cirrhosis should be considered even in non-Indian infants with progressive liver disease.

Copper↗
PubMed1992

Acute effects of a nucleoside analog dideoxyinosine (DDI) on the pancreas.

Dideoxyinosine (DDI, Videx) is a recently developed nucleoside analog with activity against the human immunodeficiency virus. A significant number of patients with AIDS or AIDS-related complex treated with DDI have developed acute pancreatitis. This study was performed to investigate the acute effects of DDI on the pancreas utilizing an isolated ex vivo perfused canine pancreas preparation. Control preparations remained normal throughout a 4-hr perfusion period. The addition of 12.5 mg of DDI to the perfusate (ca. 100 mumol/liter) did not induce any changes in the preparation. The addition of 62.5 mg of DDI to the perfusate (ca. 500 mumol/liter) did not induce changes in the gross appearance, weight gain, or amylase activity. However, the arterial pressure and the oxygen consumption of the preparation decreased significantly after the administration of DDI. The amount of zymogen in the acinar cells also decreased, as evaluated by electron microscopy. Protein secretion increased temporarily, probably as a result of acinar cell emptying (increased secretion without new synthesis). Water and bicarbonate secretion were also increased during the fourth perfusion hour.

Animals↗
PubMed1992

Acute tubular necrosis associated with Lowe's syndrome: possible role of rhabdomyolysis.

The oculo-cerebral-renal syndrome of Lowe is an X-linked recessive disorder characterized by severe mental retardation, congenital cataracts, renal tubular dysfunction, growth retardation, hypotonia, glaucoma, and rickets. Recently, it has been found that serum concentrations of the muscle enzymes are elevated, providing evidence that there is primary muscle involvement in this disorder. The renal functional abnormalities that occur have also been further delineated. Renal tubular dysfunction presents within the first year of life, followed by a serum creatinine level that increases with age. Renal failure generally occurs in the fourth decade of life. We report two patients with Lowe's syndrome who presented with new onset of acute renal failure (ARF). Workup of their ARF established the diagnosis of acute tubular necrosis with evidence of rhabdomyolysis in one case. These patients were treated aggressively with dialysis and had subsequent recovery of renal function to their baseline state. We suggest that patients with Lowe's syndrome who present with an acute change in their renal function should be treated early with vigorous hydration therapy. If dialysis is indicated, it should be initiated. Furthermore, these patients should be promptly evaluated for evidence of rhabdomyolysis with alkalinization of the urine if possible.

Biopsy↗
PubMed1991

Membranoproliferative glomerulonephritis in dogs with a genetically determined deficiency of the third component of complement.

Renal disease is a common clinical manifestation of genetically determined deficiencies of the complement system in man. Like their human counterparts, dogs with a genetically determined complete deficiency of C3 also develop renal disease. Five of 20 C3-deficient dogs developed clinical evidence of renal failure. However, 14 of the 15 remaining dogs had histological evidence of type I membranoproliferative glomerulonephritis. The lesions were characterized by mesangial cell proliferation, an increase in the mesangial matrix, thickening of the glomerular capillary wall, electron-dense deposits in the mesangium and subendothelial space, and the presence of IgG and IgM. In order to determine the effect of treatment with C3 on the renal disease of C3-deficient dogs, two C3-deficient dogs were infused with normal canine plasma twice weekly for 3 weeks. Their urinary protein excretion rose progressively from less than 200 mg/24 hr to greater than 1000 mg/24 hr; renal function remained normal. Renal biopsies performed 1 week after the last infusion revealed more severe glomerulonephritis and the presence of C3. As controls, a C3-deficient dog was given C3-deficient canine plasma and a normal dog was given normal canine plasma; neither control animal developed proteinuria or changes in their renal biopsy. These observations suggest that renal disease may be more common in humans with complement deficiencies than would be suspected based on clinical assessment. Furthermore, these results suggest that treatment with complement-containing blood products may worsen preexisting renal disease in complement-deficient individuals.

Animals↗
PubMed1979

Adenomatoid odontogenic tumor: ultrastructural demonstration of two cell types and amyloid.

A typical adenomatoid odontogenic tumor removed from a 13-year-old female was studied by light and electron microscopy. The tumor was composed of two types of epithelial cells: Type I cells were cuboidal and occurred in nests or formed ductlike structures and Type II cells were smaller and spindle shaped. The formation of extracellular masses of amyloid was found in association with Type I epithelial cells, and amyloid formation was not observed in association with Type II cells. Results suggest that the lesion is of enamel organ origin, derived from cells of the inner enamel epithelium at the pre-ameloblastic stage, stellate reticulum and stratum intermedium. The origin of this amyloid material is unknown; however, it may be of enamel protein origin which, like amyloid, may have a beta-protein conformation.

Adolescent↗
PubMed1979

Mucoepidermoid carcinoma of the bronchus: an electron microscopic study of the low grade and the high grade variants.

Two cases of mucoepidermoid carcinoma of the bronchus--one a low grade tumor and the other a high grade tumor--are presented with findings by light and electron microscopy. This represents the first report of the ultrastructure of mucoepidermoid carcinoma of the bronchus and demonstrates the ultrastructural similarities between the low grade exophytic tumor confined to the bronchus and the high grade infiltrating tumor with lymph node and pulmonary metastases. The ultrastructural features are similar to those described for mucoepidermoid carcinoma of the salivary gland and are consistent with the proposed origin of the tumor from the submucosal bronchial gland duct. These two cases and a review of previously reported cases indicate that, analogous to mucoepidermoid carcinoma of the salivary glands, mucoepidermoid carcinoma of the bronchus may occur as either a low grade or high grade variant which can be identified on the basis of growth characteristics and histologic features.

Adult↗
PubMed1979

Vasoactive intestinal peptide and its relationship to ganglion cell differentiation in neuroblastic tumors.

Immunohistochemical studies have demonstrated that immunoreactive vasoactive intestinal peptide is present in, and restricted to, the differentiating and mature ganglion cells in a variety of normal and neoplastic neural tissues. In a composite pheochromocytoma-ganglioneuroma (associated with the syndrome of watery diarrhea, hypokalemia, and hypochlorhydria), five ganglioneuroblastomas, five ganglioneuromas (two of which were associated with diarrheal syndromes), an unusual mixed neuroblastoma-ganglioneuroma, and four normal sympathetic ganglia, vasoactive intestinal peptide was present in differentiating and mature ganglion cells. The peptide was also demonstrated in isolated ganglion cells in two pheochromocytomas but was not present in pheochromocytes, Schwann cells, or undifferentiated neuroblastic cells in the neuroblastomas and ganglioneuroblastomas. These studies indicate that the presence and presumably the production of vasoactive intestinal peptide thus reflect a particular line of neuroblastic differentiation and are not merely a reflection of common derivation of these tissues. Our identification of vasoactive intestinal peptide in neurogenic tumors associated with diarrhea supports the contention that the peptide might be an important diarrheogenic factor in these tumors.

Adrenal Gland Neoplasms↗
PubMed1979

Malignant melanoma with renal dense deposits containing tumor antigens.

A patient with disseminated malignant melanoma developed mild renal failure with proteinuria four months following the diagnosis of his neoplasm. Renal biopsy showed a membranoproliferative glomerulonephritis. Electron microscopy revealed intramembranous dense deposits in the glomeruli as well as in tubular basement membranes and Bowman's capsule. Immunofluorescence microscopy demonstrated granular staining for C3 in these same locations and granular deposits of immunoglobulins in the glomerular capillary loops. Light and electron microscopy of the autopsy kidney obtained 5 months later revealed progression of the number, size and density of the deposits. Eluates from both autopsy and biopsy kidney contained melanoma antigen and anti-melanoma antibodies. This case illustrates the association between the dense deposits and neoplasia with the dense deposits representing immune complexes with the tumor as antigen.

Antigen-Antibody Complex↗
PubMed1978

Mediastinal paragangliomas (aortic body tumor): a report of four cases and a review of the literature.

Four cases of paraganglioma originating in the supra-aortic or aortico-pulmonary bodies are described. A review of the reported examples of aortic body tumors and a study of these four cases indicate that there is a high incidence of aggressive tumor growth in the mediastinum, with resultant important morbidity or death in 16 of 35 cases. The morphologic features of aortic body tumors are identical to those of paragangliomas of other locations, and the occurrence of invasive growth and/or metastasis cannot be predicted on histologic grounds.

Adolescent↗
PubMed1978

Pheochromocytomas.

Explore the source record for details and available documents.

Adrenal Gland Neoplasms↗
PubMed1978

Congenital cystic adenomatoid malformation of the lung.

Ultrastructural study of a congenital cystic adenomatoid malformation of the lung revealed a persistence of type 2 pneumocytes lining the alveolar and gland-like spaces. The larger cystic areas were lined by normal bronchiolar-type epithelium and were surrounded by a few smooth muscle cells. The interstitium contained undifferentiated mesenchymal cells. No cartilage or bronchial glands were present. These features suggest that this lesion is developmental in nature, with a failure of maturation resulting from an insult occurring early in embryonic life, prior to the onset of cartilaginous differentiation within the lungs.

Epithelium↗
PubMed1977

Fine structural studies of a human thyroid adenoma, with special reference to psammoma bodies.

The fine structural morphologic features of a microfollicular thyroid adenoma from a 28 year old female were examined. Although the patient had been laking exogenous thyroxine therapy for 14 months, the morphology of the adenoma was characterized by numerous small to medium sized follicles composed of metabolically active, well differentiated columnar cells with numerous colloid droplets, dilated granular endoplasmic reticulum, large numbers of coated vesicles and lysosomes, large colloid containing "lakes," microtubules, microfilaments, and prominent apical microvillous projections. Of special inetrest were small spherical psammomatous calcospherites ecountered in histiocytes and the interstitium. Also noteworthy were ropelike configurations observed in most of the follicular lumina. Structural-functional correlations and potential origins of psammoma bodies and calcospherites are discussed.

Adenoma↗