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Biomedical subjects

M Haltia

Publications and source records attributed to M Haltia.

At least 127 records · Page 7Linked to original sources
PubMed1990

Amyloid in familial amyloidosis, Finnish type, is antigenically and structurally related to gelsolin.

Immunohistochemical studies of six patients with familial amyloidosis, Finnish type, showed that their amyloid deposits did not react with polyclonal antibodies against the amyloid proteins of other, established forms of systemic or cerebral amyloidosis. However, strong immunoreactivity was observed with rabbit antiserum raised against a low molecular weight purified amyloid subunit isolated from one of the patients. This immunoreactivity was abolished by absorption with the low molecular weight amyloid fraction. The amino terminal sequence of the amyloid protein subunit was homologous to gelsolin, an actin-modulating protein, and the amyloid deposits in tissues reacted with a monoclonal antibody against gelsolin. These studies show that the amyloid protein in familial amyloidosis, Finnish type, is not related to previously identified forms of amyloid, including prealbumin (transthyretin) variants, but represents a novel amyloidogenic protein related to gelsolin, a plasma and cytoplasmic protein.

Actins↗
PubMed1989

Rabies: ocular pathology.

Ocular pathology in the first European case of human bat-borne rabies is described. The patient was a 30-year-old bat scientist who seven weeks after bat bite developed neurological symptoms and died 23 days later. Rabies virus antigens were detected in brain smears. After extensive virological studies the virus turned out to be a rabies-related virus, closely resembling the Duvenhage virus isolated from bats in South Africa in 1980. By light microscopy focal chronic inflammatory infiltration of the ciliary body and of the choroid was found. PAS-positive exudate was seen in the subretinal and in the outer plexiform layers of the retina, and retinal veins showed endothelial damage and perivascular inflammation. Many of the retinal ganglion cells were destroyed. The presence of rabies-related viral antigen in the retinal ganglion cells was shown by positive cytoplasmic immunofluorescence, though electron microscopy failed to identify definite viral structures in the retina. By immunohistochemistry glial fibrillary acidic protein was observed in the Müller's cells, which are normally negative for this antigen but express it as a reactive change when the retina is damaged. Synaptophysin, a constituent of presynaptic vesicles of normal retinal neurons, was not detected in the retina.

Adult↗
PubMed1988

Dolichol and dolichyl phosphate in the neuronal ceroid-lipofuscinoses and other diseases.

Although, compared to age-matched control samples, nonphosphorylated dolichols are significantly increased in the cerebral cortex of children with the late infantile and juvenile types of neuronal ceroid-lipofuscinosis (NCL), dolichyl phosphates are increased to a much greater extent in infantile, late infantile, and juvenile forms of this disease group. Dolichyl phosphates in the cerebral cortex, expressed as a percentage of the combined nonphosphorylated and phosphorylated compounds, ranged from 59 to 85 (mean 71) in NCL, whereas in the non-NCL disease group the range is 18-36 (mean 25). This marked proportional increase in dolichyl phosphates is not unique to NCL but is also found in the brain of GM1-gangliosidosis and Tay-Sachs disease patients. In the liver from NCL patients, dolichyl phosphates are not a major proportion of the total dolichol compounds (1-9%). However, in the kidney and heart, dolichyl phosphates are again markedly increased, and this is associated with large storage of ceroid. Although to a lesser extent than in NCL, dolichols and dolichyl phosphates are significantly increased over levels in age-matched control samples in the temporal cortex in Alzheimer disease. Our interpretation of these results is that storage in secondary in secondary lysosomes in NCL and the gangliosidoses leads to a decrease in the catabolism of dolichyl phosphate compounds in the Golgi saccules or primary lysosomes.

Alzheimer Disease↗
PubMed1988

Microangiopathy with encephalopathy, hearing loss and retinal arteriolar occlusions: two new cases.

Two young women developed encephalopathy, hearing loss and retinal arteriolar occlusions. Their behaviour became immature and cognitive functions were severely impaired. One of the patients underwent brain biopsy, which showed several microinfarcts in both white and grey matter and microangiopathic changes, with thickened arteriolar segments staining intensely for laminin and fibronectin. These findings support the concept of a new type of microangiopathy involving the brain, inner ear and retina.

Adult↗
PubMed1988

Fatal encephalitis caused by a bat-borne rabies-related virus. Clinical findings.

The clinical findings are described in the first reported European case of fatal encephalitis of bat origin caused by a rabies-related virus. A bat zoologist developed the symptoms of rabies 51 days after his last exposure to a bat bite. The clinical disease of 23 days duration was a combination of the paralytic and 'furious' forms of rabies. Serial BAEP and EEG recordings, CT and MR scans of the brain, as well as CSF findings, demonstrated severe ascending destruction of the brain. An unusual progression from isolated brainstem death to cortical brain death occurred. Neuropathologically, the brain showed severe lytic changes. The presence of rabies-related virus antigens in brain smears was shown using a panel of fluorescent antibodies. The virus was inoculated into and isolated from suckling mice. The virus had a close resemblance to European bat rabies isolates, which belong to the group of rabies-related viruses. Of particular concern is whether the virus can spread from bats to terrestrial animals and whether the European type of bat rabies constitutes a danger to man.

Adult↗
PubMed1988

Congenital fiber type disproportion in an adult: a morphometric and microchemical study.

A 20-year-old man with marfanoid habitus had a history of congenital hypotonia and muscle weakness. Muscle biopsy showed extreme fiber type disproportion. There was total absence of Type 2B fibers. The severely hypertrophic Type 2A fibers showed twice the normal concentration of creatine phosphate at rest. These advanced morphometric, histochemical and biochemical changes may be interpreted as compensatory phenomena, which may explain the patient's pronounced functional improvement with advancing age.

Adenosine Triphosphatases↗
PubMed1988

Descriptive epidemiology of Creutzfeldt-Jakob disease in Finland.

In 1974-84 30 patients died with a diagnosis of Creutzfeldt-Jakob disease (CJD) in Finland. Sixteen of the patients were pathologically confirmed and 14 were probable cases; 6 were familial (20%). One further familial patient was alive at the end of 1984. The median age of the 30 patients at death was 59.5 years (range 46-73 years). The familial patients were significantly younger than sporadic cases (median and range 49.5 and 46-57 versus 61.5 and 51-73; P less than 0.01). Only 5 of the 24 sporadic patients were men (male to female ratio 1:3.8). The annual number of new cases as well as the death rate increased in the late 1970's (annual death rate 0.57 per million in 1974-84 and 0.91 in 1979-84). This probably reflects the growing awareness of CJD among neurologists, rather than a real increase of the incidence and death rate. Annual age-specific death rate per 1 million population in 1974-84 reached a peak value of 2.57 in the age group of 60-64 years. In sporadic CJD the age-specific death rate of women was higher than that of men in all age groups. A chronic medical condition, precedent or concomitant with CJD, was seen in 15 patients.

Aged↗
PubMed1988

Epidermolysis bullosa simplex associated with muscular dystrophy with recessive inheritance.

Epidermolysis bullosa with unusually severe clinical features was associated with progressive muscular dystrophy in two siblings. Light and electron microscopic examination revealed an intraepidermal cleavage confirming that this mechanobullous disease belonged to the epidermolysis bullosa simplex group. This may represent a new disease entity inherited in an autosomal-recessive fashion.

Adolescent↗
PubMed1987

Erythropoietin in capillary hemangioblastoma. An immunohistochemical study.

Capillary hemangioblastoma is a tumor known to be associated with secondary polycythemia. Therefore, specimens from ten hemangioblastomas were studied by immunohistochemistry for the presence of erythropoietin, renin substrate, and for various endothelial, histiocytic and glial markers. In all tumors scattered cells among the stromal cells showed a positive-staining reaction with both anti-erythropoietin and anti-renin substrate. The same cells also stained positively for alpha-1-anti-trypsin. It is concluded that, in addition to the capillary endothelial cells, pericytes and stromal cells, capillary hemangioblastomas harbor cells containing and perhaps producing renin substrate and/or erythropoietin or a substance with similar antigenic determinants.

Angiotensinogen↗