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Biomedical subjects

M A Spycher

Publications and source records attributed to M A Spycher.

At least 37 records · Page 2Linked to original sources

[Primary amyloidosis of the liver].

For 2 years a 72-year-old man had suffered from nonspecific upper abdominal discomfort and hepatomegaly. The gamma-glutamate transaminase concentration was increased to 121 U/l, the erythrocyte sedimentation rate was 80 mm in the first hour. Histological examination of tissue from the enlarged liver (22 cm in the midclavicular line) revealed the diagnosis of amyloidosis. The gastric mucosa, duodenum and rectum were not involved. Two years later ascites developed; six months after this he was again hospitalized in hepatic coma. Now, for the first time, a type IgA-lambda paraprotein was demonstrated by serum immunoelectrophoresis. The patient died of slowly progressing anicteric liver failure after having been ill for a total of 4 1/2 years. At autopsy there were extensive amyloid deposits throughout the liver and spleen so that the structure of these organs was hardly recognizable. The amyloid deposits in the liver were restricted to the glomerular region, while there was no amyloid in the heart. Histochemical tests showed that the deposits were strongly positive to the anti-lambda antibody. This was thus a case of primary (AL-lambda) amyloidosis of the liver and spleen which had taken an unusually prolonged course, because the heart was not involved at all and the kidneys only slightly.

Aged↗

Intestinal Enterocytozoon bieneusi microsporidiosis in an HIV-infected patient: diagnosis by ileo-colonoscopic biopsies and long-term follow up.

A 39-year-old patient with acquired immunodeficiency syndrome was diagnosed as having intestinal Enterocytozoon bieneusi microsporidiosis after persistent watery diarrhea for 30 months and a 16-kg weight loss. Microsporidian parasites were found by light and electron microscopy in tissue specimens of the duodenum, jejunum, and terminal ileum, and by light microscopic examination of stool specimens. When duodenal tissue sections obtained 16 months previously were reviewed retrospectively, E. bieneusi was also found. Until now, diagnosis of intestinal microsporidiosis has been based on examination of bioptic specimens of the upper small intestine because the sensitivity of new coprodiagnostic techniques has not been determined. Our findings of ileal microsporidiosis show that examination of the terminal ileum and ileal biopsy collection in tandem with colonoscopy is indicated for patients infected with human immunodeficiency virus and suffering from unexplained chronic diarrhea. The long-term course of our patient demonstrates that E. bieneusi, although not necessarily life threatening, can cause protracted debilitating diarrhea and wasting in severely immunodeficient patients.

AIDS-Related Opportunistic Infections↗

Ultrastructure of the guinea pig pancreas in acute hypercalcemia.

The effect of acute hypercalcemia on pancreatic ultrastructure and the ultrastructural localization of calcium during hypercalcemia were studied in the guinea pig pancreas. After 3 h of i.v. calcium infusion (0.6 mmol/kg/h), hypertrophy and distention of the Golgi apparatus and an increased number of condensing vacuoles were seen. At 6 h, vacuolar fusion and displacement of zymogen granules occurred. At 9 h, irregular distribution of zymogen granules, indentation of the nucleus with chromatin clumping, and inclusion of intact cell organelles were present. Disruption of the plasma membrane and release of cell organelles into the interstitial space were seen. Control animals receiving saline solution (0.9% NaCl) revealed normal pancreatic ultrastructure. The serum ionized calcium values were 0.65 +/- 0.36 mM in controls and 0.71 +/- 0.14, 0.79 +/- 0.21, and 1.22 +/- 0.50 mM at 3, 6, and 9 h of calcium infusion, respectively. The ultrastructural localization of calcium was performed with the pyroantimonate staining technique after 3 h of calcium and saline infusion. Large calcium deposits were found in calcium-treated animals along the plasma membrane and in the Golgi region. The findings indicate that calcium exerts a strong stimulatory effect that eventually leads to the degeneration of the pancreatic acinar cell.

Acute Disease↗

Pulmonary and intestinal microsporidiosis in a patient with the acquired immunodeficiency syndrome.

The microsporidian protozoan organism Enterocytozoon bieneusi has been found in enterocytes of the small intestine in patients infected with human immunodeficiency virus, and it has been recognized as an important cause of chronic diarrhea in this patient group. We report the first case of a 41-yr-old man with acquired immunodeficiency syndrome in whom microsporidia were detected in bronchoalveolar lavage fluid, transbronchial lung biopsies, stool specimens, and ileal biopsies. He experienced chronic diarrhea, wasting syndrome, chronic cough, and dyspnea. His chest roentgenogram showed a small left posterobasal infiltrate and a small left pleural effusion. The histologic pattern of microsporidia in his bronchial and ileal tissue and the cellular inflammatory reaction with intraepithelial infiltration by lymphocytes were identical to findings described in duodenal and jejunal Enterocytozoon bieneusi microsporidiosis. An association between the presence of microsporidia in the lung and the pulmonary symptoms has yet to be determined. It is not known whether pulmonary microsporidiosis was acquired by the aerosol route, by aspiration, or by hematogenous dissemination from the intestine.

AIDS-Related Opportunistic Infections↗

Hypercalcaemia and pancreatic ultrastructure in cats.

OBJECTIVE: To study the effects of local and systemic infusions of calcium on the ultrastructure of the pancreas in cats. DESIGN: Controlled study. INTERVENTIONS: Three groups of four cats each had local infusions (into the splenic artery) of calcium gluconate 0.6 mmol/kg.hour or potassium chloride 1.1 mmol/kg.hour, or sodium chloride 0.9%, for three hours. Two groups of eight cats each had systemic infusions (into the jugular vein) of either calcium gluconate 0.6 mmol/kg.hour or sodium chloride 0.9%, for twelve hours. In the group that was given calcium, the infusion rate was reduced after three hours to 0.3 mmol/kg.hour to maintain the hypercalcaemic state for a further nine hours. RESULTS: Local infusion of calcium caused destruction of acinar cells with hydropic degeneration of nuclei, discharge of cell organelles into the interstitial spaces, and extravasation of red blood cells but no apparent damage to the capillaries. There were no ultrastructural changes of any importance in the groups that received potassium or sodium chloride. Systemic infusion of calcium resulted in a 1.8 fold increase in the ionised calcium concentration in the serum, progressive signs of overstimulation of the Golgi apparatus with hypertrophy, fusion of condensing vacuoles, and disruption of the acinar cell polarization. This was followed by clumping of nuclear chromatin and destruction of acinar cells. CONCLUSION: Acute pancreatitis in cats can result from stimulation and destruction of acinar cells by hypercalcaemia.

Acute Disease↗

Variation of quantitative and qualitative changes of enchondral ossification in heterozygous achondroplasia.

Whereas the radiologic features of achondroplasia--the most common type of skeletal dysplasia in adults--are clearly defined there is still some debate about the severity and the type of histologic changes. Earlier descriptions reported severe disturbance of enchondral ossification to be typical of achondroplasia: They are, however, misleading by dealing mostly with cases of lethal neonatal dwarfism (e.g. thanatophoric dysplasia). Newer findings confirmed that only minor, quantitative lesions are typical of heterozygous achondroplasia. But even in recent years some observers noted more severe changes of enchondral ossification. An extensive histologic skeletal survey in a newborn achondroplastic male revealed remarkable findings: It clearly showed that in addition to a generalized, but moderate narrowing of the zones of enchondral ossification focal severe changes were present in various epiphyseal plates including clusterlike arrangement of enlarged chondrocytes, vacuolization, premature calcification and important fibrosis of cartilagineous matrix with membranous ossification. Our findings thus enable us to reconcile the seemingly divergent statements made before.

Achondroplasia↗

Perhexiline maleate-induced hepatitis.

We report on a case of perhexiline maleate-induced hepatitis secondary to a long-term administration of recommended daily dosages of 300 mg. The patient had a spectacular weight loss of 29 kg. He developed hepatitis, which subsided after drug withdrawal. Our electron-microscopic findings with the typical inclusion bodies and impaired hydroxylation capacity point to an underlying metabolic disorder as the pathogenetic mechanism.

Angina Pectoris↗

Infantile phytanic acid storage disease, a disorder of peroxisome biogenesis: a case report.

The infantile and classic forms of phytanic acid storage disease belong to the newly recognized group of peroxisomal disorders. In this paper we report the full clinical, morphological and biochemical results in a patient with infantile phytanic acid storage disease. The results indicate a generalized loss of peroxisomal functions due to a deficiency of peroxisomes as demonstrated in hepatocytes and cultured skin fibroblasts.

Acyl Coenzyme A↗

Induction and prevention of chondrocyte hypertrophy in culture.

Primary chondrocytes from whole chick embryo sterna can be maintained in suspension culture stabilized with agarose for extended periods of time. In the absence of FBS, the cells remain viable only when seeded at high densities. They do not proliferate at a high rate but they deposit extracellular matrix with fibrils resembling those of authentic embryonic cartilage in their appearance and collagen composition. The cells exhibit many morphological and biochemical characteristics of resting chondrocytes and they do not produce collagen X, a marker for hypertrophic cartilage undergoing endochondral ossification. At low density, cells survive in culture without FBS when the media are conditioned by chondrocytes grown at high density. Thus, resting cartilage cells in agarose cultures can produce factors required for their own viability. Addition of FBS to the culture media leads to profound changes in the phenotype of chondrocytes seeded at low density. Cells form colonies at a high rate and assume properties of hypertrophic cells, including the synthesis of collagen X. They extensively deposit extracellular matrix resembling more closely that of adult rather than embryonic cartilage.

Animals↗

Formation of double contour as a repair reaction in the human femoral head.

Thirty-two femoral heads from autopsy material with arthrotic changes and 12 surgical specimens of femoral heads with different diseases have been examined with respect to their ability to form a 'double contour'. An additional bony plate within the cartilage, giving rise to the double contour, occurred only in the non-pressure zone of the articular cartilage. There were no major differences between autopsy and surgical material and these changes could also not be found in normal femoral heads. The origin of a double contour could be traced to tissue elements penetrating the subchondral bone from the bone marrow, and was the result of a gradual transformation with chondroid tissue as the starting material. We observed a similar transformation leading to lamellar bone in association with pannus.

Adult↗

Inorganic particulates in pneumoconiotic lungs of hard metal grinders.

Data from the analysis of lung dust in 16 metal grinders who had been exposed to hard metals between five and 44 years is reported. The mean latent time between the first exposure and analysis in biopsy or necropsy specimens was 33.6 years. Mineralogical and elementary analysis by a variety of techniques showed small or trace amounts of hard metal in all lungs. Many specimens, however, did not contain all hard metal components, cobalt, for example, being detected in four cases only. All the lungs contained quartz and silicates and in most of the necropsy cases carborundum and corundum could also be shown. Histologically no specific pattern was found. The appearances included mixed dust nodular pneumoconiosis, diffuse interstitial lung fibrosis, and foreign body and sarcoid like granulomatous changes. In view of the mixed dust exposure of the hard metal grinders and the variable histological appearance we think that the term "mixed dust pneumoconiosis in hard metal grinders" is more appropriate than "hard metal lung" to describe this condition.

Adult↗

[Pneumoconiosis caused by "rare earths" (cer-pneumoconiosis)].

Slowly progressive restriction in respiratory function was observed in five reproduction photographers who had been exposed to fumes of carbon arc lamps for more than a decade. In all cases interstitial lung fibrosis had developed and accumulations of fine granular dust particles were found in the tissue. Combined analyses including X-ray diffraction, infrared spectroscopy, plasma analysis, X-ray microanalysis and electron diffraction revealed the presence of rare earth minerals (mainly cerium compounds) in all lungs. The rare earth minerals originating from the core of the burned carbon rods used in the arc lamps represent the etiological factors responsible for the development of so-called "cerium pneumoconiosis".

Aged↗

The ultrastructure of the erectile tissue in priapism.

The ultrastructure of erectile tissue from the corpora cavernosa penis of patients suffering from stasis priapism and high-flow priapism has been studied. Trabecular interstitial edema was confirmed as the first reaction of the tissue to the hemodynamic impairment. At the cellular level trabecular smooth muscle cells were found to be the first affected by the altered environmental conditions. Their reaction consisted of structural and functional transformation to fibroblast-like cells. Severe cellular damage and widespread necrosis were not seen in high flow priapism; such damage existed in stasis priapism, but only when the priapic episode lasted more than 24 hours. Blood clot formation within the cavernae and destruction of the endothelial lining occurred in stasis priapism lasting over 48 hours. At this time trabecular inflammation became conspicuous and most of the smooth muscle cells were either transformed to fibroblast-like cells or had undergone necrosis. This stage was not reached in high flow priapism, a fact supporting the view that high flow priapism is a more benign and prognostically more favorable form of priapism. Massive smooth muscle cell transformation and the loss of contractile trabecular elements may play an important role in the evolution of irreversible erectile failure following stasis priapism persisting longer than 24 hours.

Adult↗

Focal lacunar resorption in the articular cartilage of femoral heads.

Human articular cartilage taken from 92 femoral heads at autopsy was examined macroscopically and microscopically. Fifty-two showed no changes except for occasional slight degeneration in the non-pressure areas; these changes were visible only microscopically. In the remaining 40 heads, different degrees of osteoarthrosis were seen; half the heads also showed focal lacunar resorptive lesions in the cartilage. The origin of this focal cartilage resorption is discussed and its possible association with necrosis, pannus formation and enzymatic synovial activities. We conclude that there is no evidence of a direct relationship between focal cartilage resorption and osteoarthrosis.

Adult↗

The thickening of basement membrane in synovial capillaries in rheumatoid arthritis.

Synovial tissues from seven rheumatoid arthritis (RA) patients were used for the ultrastructural investigation of capillary cellular components and basement membranes (BM). Attention has been specially paid to the mechanism of BM thickening of the capillaries in the inflammatory sites. The capillary BM were multilamellated in the inflammatory sites. The multilamellation was characteristic not only in the BM surrounding the endothelial cells and pericytes but also in the BM between these two types of cells. Cell debris was frequently encountered between the multilamellated BM. The hyperplasia and various stages of degeneration of the endothelial cells were observed in these regions. Some endothelial cells were activated and occasionally located in capillaries containing degenerated endothelial cells. The high incidence of these findings indicates the following hypotheses. The accelerated rate of death and replenishment of capillary cellular components may play a role in BM thickening in the inflammatory sites of RA synovium. These cells may not only produce one layer of BM in their life-time but may also be activated to produce excessive amounts of BM components to make several layers.

Adult↗