Mutations in the SLC35C1 gene, contributing to significant differences in fucosylation patterns, may underlie the diverse phenotypic manifestations observed in leukocyte adhesion deficiency type II patients.

Congenital disorders of glycosylation (CDG) are a large family of genetic diseases resulting from defects in the synthesis of glycans and the attachment of glycans to macromolecules. The CDG known as leukocyte adhesion deficiency II (LAD II) is an autosomal, recessive disorder caused by mutations in the SLC35C1 gene, encoding a transmembrane protein of the Golgi apparatus, involved in GDP-fucose transport from the cytosol to the Golgi lumen. In this study, a cell-based model was used as a tool to characterize the molecular background of a therapy based on a fucose-supplemented diet. Such therapies have been successfully introduced in some (but not all) known cases of LAD II. In this study, the effect of external fucose was analyzed in SLC35C1 KO cell lines, expressing 11 mutated SLC35C1 proteins, previously discovered in patients with an LAD II diagnosis. For many of them, the cis-Golgi subcellular localization was affected; however, some proteins were localized properly. Additionally, although mutated SLC35C1 caused different α-1-6 core fucosylation of N-glycans, which explains previously described, more or less severe disorder symptoms, the differences practically disappeared after external fucose supplementation, with fucosylation restored to the level observed in healthy cells. This indicates that additional fucose in the diet should improve the condition of all patients. Thus, for patients diagnosed with LAD II we advocate careful analysis of particular mutations using the SLC35C1-KO cell line-based model, to predict changes in localization and fucosylation rate. We also recommend searching for additional mutations in the human genome of LAD II patients, when fucose supplementation does not influence patients' state.

Early alveolar bone grafting has a negative effect on maxillary dental arch dimensions of pre-school children with complete unilateral cleft lip and palate.

OBJECTIVE: To evaluate maxillary dental arch dimensions in pre-school children with a complete unilateral cleft lip and palate (CUCLP) after early alveolar bone grafting. MATERIAL AND METHODS: Intercanine and intermolar widths, length of dental arch and mesiopalatal inclination of both maxillary segments were measured directly on the dental casts of 42 children (27 boys and 15 girls; mean age = 5.2 years, SD 0.5; Early-grafted group), 30 children (18 boys and 12 girls; mean age = 5.8 years, SD 0.8; Non-grafted group), and 40 children (25 boys and 15 girls, mean age = 5.8, SD 0.4; non-cleft Control group). Children from Early-grafted and Non-grafted groups had a CUCLP repaired with a one-stage closure of the entire cleft. An alveolar bone grafting was performed in the Early-grafted group between 2 and 4 years (mean = 2.4, SD 0.6). A one-way anova model with post hoc Tukey's multiple comparison procedures were used to identify intergroup differences. RESULTS: The mesiopalatal inclination of the lesser segment in the Early-grafted group was decreased in comparison with the Non-grafted and Control groups. The intercanine width had a tendency to be reduced in the Early-grafted group relative to Non-grafted group. CONCLUSIONS: Early bone grafting results in a larger collapse of the lesser segment than bone grafting carried out between 9 and 12 years of age.

Comparison of intestinal absorption of selected calcium organic salts through the rat jejunum in vitro.

The calcium penetration rates through the rat's ileum were determined for calcium fumarate, calcium gluconate and calcium chloride in solution by means of a set for determination of drug penetration through membranes. The highest penetration rate of calcium was observed for the calcium fumarate solutions, a lower one for calcium gluconate solutions and the lowest--for calcium chloride solutions. The calcium penetration rate was independent from the calcium doses in the studied concentration range. The obtained results suggest that calcium fumarate, till now not used in therapy might find an application in the corrective therapy of calcium deficiency.

The kinetic study of the selenium yeast stability.

The kinetics of degradation of selenium yeast was investigated at different temperatures. The effect of temperature has been determined and from this data, applying of Arrhenius-law, the stability of selenium yeast at 25 degrees C has been predicted and the t10 was determined. The changes in selenium content of the selenium yeast were determined by the fluorimetric method. It was proved, that the decomposition observed the first-order kinetic equations. The shelf life of selenium yeast predicted from the Arrhenius plot exceeded 1126 days. In the other hand the stability of selenium preparation in normal storage conditions (T = 25 degrees C) was determined to compare with predicted values. These results were found in agreement with experimental ones obtained at room temperature. No discernible physical changes of selenium preparations were observed after the storage in normal conditions (25 degrees C).

Allergy to animal fur and feathers among zoo workers.

OBJECTIVES: This study determined the prevalence of respiratory symptoms and immediate hypersensitivity to feather and fur allergens and pulmonary function among zoological garden workers. METHODS: Skin-prick test (SPT) reactivity to common and epithelium (hair) animal fur and feather allergens were examined in 68 zoological garden workers. All subjects responded to a questionnaire and underwent spirometry. Total and antigen-specific IgE were estimated among subjects claiming respiratory symptoms. RESULTS: Forty-five subjects revealed positive SPTs with any inhalant allergen. Twelve reacted to feather extracts and 18 reacted to animal fur extracts. IgE specific for occupational allergens was seen in the serum of five subjects with SPTs positive to feather allergens and in the serum of 12 subjects with SPTs positive to fur allergens. Nose or eye symptoms were reported most frequently. Rhinitis and asthma were reported by atopic subjects more often than by non-atopic subjects. Occupational asthma due to feathers was diagnosed in 2% of zoo workers, and to fur in 10% of subjects working in contact with birds and furred animals. DISCUSSION: The results suggest that occupational asthma caused by feathers is very rare in contrast to asthma caused by animal fur. Atopy predisposes to the development of allergic diseases caused by animal fur and feathers.

Allergy to laboratory animals in children of parents occupationally exposed to mice, rats and hamsters.

Sensitization to laboratory animals (LA) has a high prevalence among laboratory workers. It is unknown whether transportation of LA allergens can be a risk factor for sensitization of subjects outside the laboratory environment. The aim of the study was to investigate the prevalence of sensitization to LA among children whose parents were and were not occupationally exposed to LA. The first group consisted of 50 children (age 12.3+/-4.3 yrs) whose parents were occupationally exposed to mice, rats and hamsters. The second group consisted of 40 children (age (mean+/-SD) 10.8+/-3.0 yrs) whose parents were not occupationally exposed to LA. Children having LA at home were eliminated from the study. All children responded to a questionnaire, underwent spirometry and were also tested with skin prick tests with the use of common allergens and prick tests with hair extracts from mouse, hamster and rat. Total immunoglobulin (Ig)E levels and the presence of specific IgE against LA were also estimated. Children of parents occupationally exposed to LA presented significantly more positive skin prick tests against allergens from the hair of laboratory animals compared to children of nonexposed parents. Five children from the first group were also found to have specific IgE against LA, with three of these five children complaining of rhinitis and cough while visiting their parents' workplace. It is concluded that the observed increased sensitization to laboratory animals among children of occupationally exposed parents could be the result of poor hygienic conditions at their parents' workplace. Hence, parents' job seems to be an additional risk factor of sensitization and should be taken into consideration when recording an allergic history.

Pharmacokinetics of ferrous fumarate in rats.

There have been evaluated changes of iron concentration in serum dynamics after single oral administration of ferrous fumarate to rats. Pharmacokinetics parameters were determined and the results were compared to those ferric gluconate.

Occupational respiratory diseases in laboratory animal workers: initial results.

UNLABELLED: Laboratory-animal allergy (LAA) is a well-known occupational hazard to workers employed in biological or medical research institutes and in the pharmaceutical industry. The aim of this study was to focus on the problem of LAA and to assess factors predisposing to sensitization among subjects occupationally exposed to animals. Sixty workers were examined in our study. They responded to a questionnaire and underwent spirometry (Vital Capacity, VC and Forced Expiratory Volume in one second, FEV1). In addition, Peak Expiratory Flow (PEF) and the histamine provocation test were estimated in 5 subjects that had been hospitalized in the Department of Occupational Diseases. Skin prick tests with common allergens and with hair extracts from laboratory animals were performed, and total IgE levels and specific IgE antibodies were also measured. Among 60 subjects who had been working with animals, 26 had positive skin prick tests for one or more of the common allergens. Five subjects supposed to have occupational bronchial asthma and four with occupational allergic rhinitis showed positive skin prick tests for one or more animal allergens, increased total IgE levels and specific serum IgE antibodies. All these subjects had smoked for years. CONCLUSIONS: 1) Laboratory animal allergy develops within first years of exposure; 2) atopy and smoking predispose to laboratory animal sensitization and to a development of bronchial asthma and allergic rhinitis.

[Bronchial asthma with inflammation of the nose mucous membrane induced by occupational exposure to methyl methacrylate in a dental technician ]. / Astma oskrzelowa z zapaleniem blony sluzowej nosa w nastepstwie zawodowej ekspozycji na metakrylan metylu u technika dentystycznego.

A case report of a 40-year-old female dental technician with a 13-year history of methyl methacrylate exposure is presented. Symptoms of dyspnea, wheezing, coughing and rhinorrhea occurred 6-8 months after the first occupational contact with methyl-methacrylate containing substances. Skin tests performed with a battery of common allergens produced negative results. While performing a provocation test with methyl-methacrylate, the patient developed severe stridor and dyspnea with concomitant decrease in I second forced expiratory volume (FEV1) and peak respiratory flow (PEF). The increase in leukocytes, eosinophils, basophils, albumin, eosinophil cationic protein (ECP) and mast cell tryptase occurred in nasal lavage fluid after bronchial provocation test. The authors conclude that methyl-methacrylate may cause asthma (probably non-atopic) in persons occupationally exposed to its effect.

[Laboratory animals as a cause of occupational allergy]. / Zwierzeta laboratoryjne jako przyczyna uczulen zawodowych.

Animal allergens are the strongest occupational allergens which sensitize the respiratory tract. Allergy to the animal is the most important occupational health hazard among people working with experimental animals in university and other research laboratories. The most common manifestations of allergy to laboratory animals are: bronchial asthma, rhinitis, contact urticaria, angioedema and contact dermatitis. The major source of allergen is the excreta and secreta of such animals as: rat, mouse, guinea pig, rabbit, dog, cow and horse. Among risk factors responsible for the development of animal allergy are: atopy, tobacco smoking and allergy to domestic pets. The diagnosis of laboratory animal allergy is usually based on a medical history. The objective evidence to support the diagnosis can be obtained from skin testing, a specific immunologic response and work related changes in peak flow rate. Reduction in the airborne levels of animal allergens not only at home, but also at work (proper ventilation, filter masks, elimination of domestic animals) and reduction of factors responsible for the development of bronchial hyperreactivity (avoidance of smoking), can contribute to decreasing the incidence of diseases.

Bioavailability of buserelin and gonadorelin in suspensions.

There have been determined parameters defining biological availability of gonadorelin and buserelin marked 125 J. Hormonal preparations were hypodermically administered to the rats in suspensions containing zinc ions of the molar ratio of the hormone: zinc 1:5. It has been stated that buserelin in suspension is better available to an organism than gonadorelin .

[Treatment of lateral cross-bite with active plates in conditions of raised dental occlusion and by the quad-helix method]. / Leczenie zgryzu krzyzowego bocznego plytami aktywnymi w warunkach podniesionego zwarcia oraz metoda quad-helix.

The purpose of the paper was to estimate the effects of the fixed elevation of occlusion on lateral cross-bite treatment. There was also comparative studies in the group of children treated with the quad-helix appliances. Own results were obtained from the studies of 36 children with lateral cross-bite whose occlusions were regulated. The children were divided into 3 groups:--in the first group 13 children had the occlusion arised with the metal dental crowns widened by metal flat surface on the buccal side. They were cemented on first permanent upper molars (Fig. 1);--in the second group 12 children had the occlusion arised by the mass type "Composite" covering first permanent lower molar teeth (Fig. 2); in both groups children were treated by upper active plate;--in the third group 11 children had the appliance quad-helix used to widen the upper dental arch (Fig. 3). The obtained results (Tax. 1-4) show that stable elevation of the occlusion makes the treatment shorter, especially in the children where the cooperation is worse. For the patients with bigger narrowness of the upper dental arch it is directed to raise the occlusion with the metal dental crowns with flat widened surfaces. In the cases when the narrowness is smaller it is sufficient to raise the occlusion by the mass type "Composite" (Fig. 2). The quad-helix appliance is the most effective method of the treatment in the case of deciduous and mixed teeth, because the time of treatment is short. In the treatment of lateral cross-bites the most important matter is to liquidate the disproportion between the width of the upper and lower dental arches (by widening the maxillae) and to obtain the regular bite.

Preparation and properties of buserelin suspensions.

Obtaining and some properties of suspensions with buserelin-there were obtained buserelin suspensions with zinc ions with pH 7.2-7.5 and hormone: zinc molar ratio from 1:2.5 to 1:500. The obtained buserelin suspensions show moderated hormone release in vitro and prolonged action in vivo. The pulsating buserelin release from suspensions has been found in vitro and in vivo.

Bioavailability of selenium from selenium preparations in rats.

In the study the relative bioavailability as well as the extent of bioavailability of selenium after a single intragastric administration of selenium yeast and sodium selenite have been determined. Also, the mean values of biopharmaceutical parameters of the tested selenium preparations have been presented.

Pharmacokinetics and distribution of selenium in blood and organs of rats.

The dynamics of selenium concentration changes in blood, organs and tissues of rats after a single intragastric administration of selenium yeast and sodium selenite was determined. The course of selenium concentration changes in blood was described, the pharmacokinetic parameters were estimated according to the trivalent equation of dicompartment model and the selenium content in liver, kidneys, small intestine, spleen, heart, brain, stomach, testicles and prostate was determined 48 h after the administration of selenium preparations.

The influence of applying magnesium preparation on the content of chosen ions of human plasma.

We have analyzed the influence of the administration of the "Calcimag" preparation, containing Mg+2 and Ca+2 ions, on the content of chosen blood plasma ions: Mg+2, Na+, K+, CI-, Ca+2 and phosphorus subject to the age of the volunteers. The preparation was administrated three times a day one tablet. The tendency to the decrease of magnesium concentration in plasma has been noticed subject to the age of the individuals. The administration of magnesium-calcium preparation caused statistically significant increase of magnesium in plasma for persons with hypomagnesemia and slight but characteristic increase for those with normomagnesemia.

[Backshifting of lower canines in occlusion regulation]. / Cofanie klów dolnych w przebiegu regulacji zgryzu.

In 17 patients the lower canines were shifted back during treatment of prognathic malocclusion and cross-bites. Stable devices with retraction loops, rubber or spring traction were used. The duration of active treatment was about 3 months, on average. During the treatment gaps after the removed premolars were closed completely, although this is nearly impossible when removable devices are applied.