[Familial LCAT deficiency--clinical picture. Case report].

We report a case of familial LCAT deficiency, diagnosed in a 35 year old women. The disease manifested itself by a presence of proteinuria, corneal opacities and haemolytic anaemia with target cells. Suspecion of familial LCAT deficiency was based on renal biopsy, which revealed characteristic serpiginous fibrillar deposits in electron microscopy.

A case of abetalipoproteinaemia in a Polish family.

Abetalipoproteinaemia (ABLP) was diagnosed in a brother and sister, 9 and 13 years old, presenting with symptoms of malabsorption during the neonatal period. Both children showed most of the main clinical features of ABLP, including neurological, and ophthalmic symptoms, and mental retardation. Acanthocytosis of erythrocytes was almost complete in the affected children, while in most of the remaining 11 members of their three-generation family, it was found in less than 50% of red blood cells.

Two siblings with phenotypes mimicking peroxisomal disorders but with discordant biochemical findings.

The authors present a report on two sibling with a nearly identical phenotype mimicking peroxisomal disorder but with totally discordant biochemical findings. In an attempt to confirm the diagnosis of a peroxisomal disorder, plasma levels of very long chain fatty acids (VLCFA) and phytanic acid were determined. In addition, fibroblasts profile of VLCFA and plasmalogen levels were evaluated for Case 2.

[Clinical and morphological picture of adrenal gland carcinoma with characteristics of early embryonal development].

A two year old girl with symptoms of virilization was investigated and an adrenal gland tumor with morphological sings of malignancy was diagnosed. After surgery, irradiation and chemotherapy, a complete recovery was observed. The authors point out the diagnostical difficulties in similar cases.

Human placental trophoblast in regions of high and low environmental pollution. A histochemical and morphological study.

The authors demonstrate the dependence of development of a uniform layer of syncytiotrophoblast with good cytochrome oxidase activity in placental villi on the absence of noxious industrial by-products in the environment of the pregnant woman. The degree of deviation from the developmental norm observed in regions free from industrial pollution can serve as measure of the degree of that pollution in urban-industrial regions.

Influence of D,L-ethionine intoxication on galactosyl transferase activity and morphology of the rat liver Golgi apparatus.

Eight hours after single, intraperitoneal injection of D,L-ethionine to rats, statistically significant increased activity of liver Golgi apparatus UDP-Gal-GlcNAc transferase calculated per g as well as per total liver in comparison with control was found. Specific activity of the enzyme was higher than normal, however not statistically significant. Yield of Golgi-rich membrane fraction was unchanged in comparison with control.

Synthesis and secretion of some plasma proteins by tissue slices of Morris hepatoma 7777 and liver from control and turpentine-injected rats.

Slices of Morris hepatoma 7777 or rat liver isolated from control or turpentine-injected rats were incubated for 2 h with 14C-leucine. Radioactivities incorporated into albumin, alpha-fetoprotein, fibrinogen, alpha 1-AP-globulin, haptoglobin and alpha 1-acid glycoprotein were determined after the proteins had been isolated from the incubation medium or tissue homogenate by immunoprecipitation with monospecific antisera. It was found that hepatoma synthesizes fibrinogen, alpha 1-AP-globulin and alpha 1-acid glycoprotein in the amounts comparable to rat liver, whereas formation of albumin and haptoglobin is reduced 5- to 10-fold.

Comparison of rat plasma glycoprotein composition with biochemical activity and morphology of liver Golgi apparatus in streptozotocin diabetes treated with insulin.

The seromucoid level and its neutral sugar composition were studied and the results compared with Golgi-enriched membrane UDP-Gal leads to GlcNAc transferase activity and the morphology of the liver Golgi apparatus in situ. Four groups of rats were studied: 1. control, 2.

Morphology of the Golgi apparatus in streptozotocin-diabetic rat liver.

The altered morphology of the Golgi apparatus in streptozotocin-diabetic rat liver is described in situ and in preparations of the Golgi-membrane-enriched fraction. The main differences, as compared with controls, are a diminished Golgi apparatus area, the rounding of stacks of cisternae and the absence of secretory vacuoles with VLDL granules. These findings are discussed in view of biochemical alterations found previously in the Golgi-membrane-enriched fraction isolated from streptozotocin-diabetic rat liver.

Short-term action of streptozotocin on biochemical activity and morphology of the rat liver golgi apparatus.

After six hours, the diabetogenic dose of streptozotocin has neither induced the release of hepatocyte cytoplasmic enzyme nor visibly injured the hepatocyte. As a rule, however, these doses of streptozotocin induced various responses in individual rat livers. Morphological studies have shown hypertrophy of the rat liver Golgi apparatus, higher numbers of secretory vacuoles and dilated cisternae.

Ultrastructural alterations in extrinsic eye muscles induced by vincristine.

A comparative morphological analysis of the effects of vincristine on particular types of muscle fibres of the eye and selected trunk muscles of the mouse was performed. Great resistance of the mouse organism to the action of sublethal doses of vincristine has been found. Degenerative changes of great intensity (atrophy of myofibrils, disturbances of the Z line) and the appearance of new changes, not mentioned hitherto among the vincristine myopathies (megamitochondria, intermembranous inclusions, glycogen in mitochondria and very large vacuoles) were observed in the trunk muscles of mice.

Quantitative electron-microscopy analysis of cranial nerves III, IV, and VI and of the extrinsic eye muscles in fog.

A comparative study of the quantitative data of the frog extraocular muscles and the cranial nerves that innervate them was performed. Oculorotatory muscles contain muscle fibres of at least 4 types which are arranged in heterogeneous layers. The zonal arrangement of the muscles does not occur on the cross-sections in the vicinity of muscle insertions.

Morphology of spermatozoa in semen from stallions of normal fertility.

Semen samples were collected from 3 fertile stallions by means of an 'open' artificial vagina and examined under scanning and transmission electron microscopy. The stallion spermatozoon has many features in common with that of other mammals but differs specifically in that it has an asymmetric head, an abaxial position of the tail and an acrosome of small volume. The presence of microtubules in the neck is also a characteristic of stallion spermatozoa.

Biochemical activity and morphology of the Golgi apparatus in alloxan diabetic rats.

In Golgi-enriched membrane fraction isolated from alloxan diabetic rat liver, the protein content is lower but the protein composition is similar to normal. The specific activity of galactosyl transferase in this membrane fraction is higher than normal, but the total activity of the enzyme in the whole liver is normal. Great dispersion is found among the individual values.