The variability of clinical presentation of chronic obstructive pulmonary disease in patients with hereditary alpha-1 antitrypsin deficiency

Four patients with alpha-1 antitrypsin (alpha-1 AT) deficiency are presented: one woman with severe (phenotype PiZ) and 3 men with moderate (phenotype PiMZ) deficiency of alpha-1 AT. The variability of clinical presentation of hereditary emphysema is described. In all patients tobacco smoking history, spirometric and 6-minutes walking tests as well as HRCT of the lung were performed and compared. The influence of smoking on the functional status is underlined.     

Distribution of ganglionic sympathetic neurons supplying the subcutaneous,perirenal and mesentery fat tissue depots in the pig

Previous morphological studies revealed that the adipose tissue is innervated by adrenergic nerve fibers. Furthermore, physiological studies showed that the metabolism of adipose tissue is controlled by the adrenergic component of the nervous system. However, nothing is known on the sources of innervation of different fat tissue depots. Therefore, we decided to study the distribution of ganglionic sympathetic neurons innervating adipose tissue in the pig by means of a retrograde tracing method. We used 9 male and 9 female pigs of approximately 50 kg body weight. The retrograde tracer, Fast Blue (FB), was injected into the subcutaneous, perirenal and mesentery fat tissue depots. Results of the present study showed that numerous centers of the sympathetic nervous system innervate adipose tissue in the pig. FB+ neurons projecting to the subcutaneous fat tissue were placed in the thoraco-lumbar region of the sympathetic chain ganglia (SChG). However, neurons supplying perirenal and mesentery fat tissue depots were found in both the SChG and prevertebral ganglia (PVG). We conclude that different adipose tissue depots (subcutaneous, perirenal and mesentery) have different sources of innervation and that there is no significant difference in the distribution of neurons innervating adipose tissue in male and female pigs.     

Invasive aspergillosis in autopsy material of patients treated at the Institute of Tuberculosis and Chest Diseases during the years 1993-2000

The aim of this paper is an analysis of clinical documentation and results of autopsy of 21 patients (pts) who died of invasive aspergillosis (IA) in the Institute of Tuberculosis and Chest Diseases in years 1993-2000 and the assessment of predisposing factors for IA. In 17 pts IA was the main and in other 4 only an accessory cause of death. All pts were treated with corticosteroids and/or cytostatic drugs--because of lung cancer (11 pts), cancer in other site (2 pts), haematologic disorders (2 pts), Wegener's granulomatosis (1 pt), polymyositis (1 pt), idiopathic pulmonary fibrosis (1 pt) and other diseases (3 pts). In 15 out of 21 pts granulocytopenia was revealed (from 0.008 x 10(9)/L to 0.82 x 10(9)/L) on an average one month before death. In 15 pts IA was limited to the lungs, in 6 others there were also fungal lesions in brain, kidneys, liver, spleen and heart. Pts with disseminated form of IA had significantly lower granulocyte count and were treated with higher doses of corticosteroids than others. Immunosuppressive drugs and granulocytopenia can be regarded as predisposing factors. Fatal course of IA depended also on the late diagnosis.     

The development of the tongue and morphological and cytological changes in taste discs of Rana esculenta

From the 38th developmental stage of the tadpole of Rana esculenta the process of tongue formation consists in the fast growth of the lining of the oral cavity floor anteriorly and faucially. This process is accompanied by the development of taste organs on the dorsal side of the tongue. At developmental stages 39-42 taste disc anlages are covered by a layer of ordinary epithelial cells. At these stages, in some cells of a taste disc single synaptic-like vesicles with an electron-dense core appear. Apart from that, as early as at stage 42 differentiation of the cells of a taste disc can be observed at the ultrastructural level. It is only at the 44th stage that all cell types characteristic for the mature TD can be distinguished in TEM (i.e., taste cells, basal cells and three kinds of associate cells: mucous, wing and sustentacular). Starting from that stage changes in the cell membrane can be observed indicating the presence of afferent synaptic junctions. The antibody used in the experiment was raised against neuron-specific enolase (NSE). At each of the developmental stages investigated (38, 42, 45) nerve fibres within the connective tissue beneath the epithelium of a taste disc anlage were immunopositive for NSE. From stage 42 onwards neural elements present in the basal part of the epithelium of a taste disc anlage were also NSE-positive. Basal cells did not show immuno-reactivity for NSE at any of the developmental stages investigated.     

Wiedemann‐Rautenstrauch (neonatal progeroid) syndrome: New case with normal telomere length in skin fibroblasts

Wiedemann‐Rautenstrauch (neonatal progeroid) syndrome is an autosomal recessive condition with characteristic appearance of premature aging present at birth (aged face, natal teeth, and wrinkled skin). Other features of the syndrome are generalized lipoatrophy with specific fat accumulation in the lateral suprabuttock region, hypotrichosis, macrocephaly (pseudohydrocephalus), and mental retardation. We report on a new case that demonstrates all typical features of the syndrome. The girl is now 16 years and 10 months old and has had follow‐up from birth. We measured terminal restriction fragment (TRF) length to evaluate whether the patient's premature aging process is accompanied by shortening of telomere length in her cultured fibroblasts. Mean TRF of 13.5 kb found in our patient's fibroblasts is not shortened as compared to that of normal fibroblasts. Our results differ from those observed in Hutchinson‐Gilford progeria. © 2001 Wiley‐Liss, Inc.     

Lung cancer--differences of incidence between the sexes

During the last decade increasing incidence of lung cancer among women have been observed in Poland. The aim of the study was to demonstrate differences among men and women with lung cancer. Lung cancer was diagnosed in 785 female and 4619 male in 1995 in Pulmonary Outpatients Departments. Women were younger than man when all histologic types of lung cancer were analysed (59.7 vs 61.9 years p. < 0.001). Particularly younger subjects were those with adenocarcinoma and small cell lung cancer (56.9 and 57.4 years for women and for men respectively 60.2 and 59.6 years, p < 0.001). Although squamous lung cancer was the most prevalent histological type among men (43.7%) and women (24.7%), about two times higher percentage of men had this neoplasm (p. < 0.001). Adenocarcinoma (18% vs 6.6%, p. < 0.001) and small cell lung cancer (18.5% vs 15.5% p. < 0.001) were prevalent in significantly higher percentage among female than male. Nonsmokers were more frequently noticed among women then men (20.4% vs. 1.9%, p. < 0.001), particularly those with adenocarcinoma. Also women smoked less intensively (33.6 pack/years vs. 42.3 pack/years, p < 0.001) except those with squamous cancer. The higher incidence of cancer was observed among mothers (7% vs 3.8% p. < 0.001) and fathers (7.1% vs 5.6%, p. < 0.001) of women than men with lung cancer.     

Activation of hamster mast cells for IgE — Mediated histamine release

The conditions for active sensitization of hamster peritoneal and pleural mast cells and IgE-induced histamine release as well as cell desensitization were defined. Immunization of hamsters with ovalbumin (5 g) in Al/OH/₃ gel (5 mg) with several boosters resulted in sensitization of peritoneal and pleural mast cells; in the presence of extracellular Ca⁺⁺, pH of medium 7.2 and at 37°C these cells released up to 70% of histamine on the challenge with specific antigen. Partial release was observed when the cells were challenged with antigen in the absence of extracellular calcium. The rate of release is high during the first seconds of activation and is complete at 1 min. 30 min preincubation of peritoneal and pleural mast cells in calcium-free conditions (in the presence of 4 mM EDTA) resulted in complete desensitization of cells to subsequent action of antigen in potimal conditions. The present experiments demonstrate, that hamster peritoneal and pleural mast cells can be a useful model system forin vitro studies of the mechanisms of IgE-induced cell activation.     

Fibroblasts from recurrent fibrotic overgrowths reveal high rate of proliferation in vitro - findings from the study of hereditary and idiopathic gingival fibromatosis

ABSTRACT

Purpose: Investigate the content of fibrotic fibrils in gingival tissue and the proliferation of fibroblasts collected from recurrent and non-recurrent hereditary gingival fibromatosis (HGF) and idiopathic gingival fibromatosis (IGF).

Methods: Gingival biopsies were collected from HGF (n = 3) and IGF (n = 3) donors with recurrent and non-recurrent gingival overgrowths and from a control group (Ctrl, n = 3). Hematoxylin staining was performed to evaluate the histomorphology of gingival tissue. Heidenhain’s AZAN trichrome staining served for visualization of fibrotic fibrils in gingiva. Quantitative analysis of the content of fibrotic fibrils in gingival tissue was performed using a polarized light microscope. Proliferation was evaluated at 24 h, 48 h, and 72 h in fibroblast cultures using a cell proliferation ELISA assay based on 5-bromo-2?-deoxyuridine (BrdU).

Results: Numerous blood vessels and fibroblasts were observed in recurrent overgrowths, whereas moderate blood vessels and moderate to scanty fibroblasts were detected in non-recurrent overgrowths. Heidenhain’s staining revealed numerous collagen fibers in both recurrent and non-recurrent overgrowths. Quantitative analysis in a polarizing microscope showed significant accumulation of fibrotic fibrils exclusively in the overgrowths with the recurrence. In all time-points, increased proliferation of cells from all recurrent overgrowths was observed, but not from overgrowths which do not reoccur.

Conclusions: The study revealed that recurrent gingival overgrowths consist of highly fibrotic and dense connective tissue with numerous blood vessels and abundant fibroblasts. We also demonstrated that unlike fibroblasts derived from overgrowths, which did not present recurrence, fibroblasts derived from highly fibrotic and recurrent overgrowths maintain high rate of proliferation in vitro.     

Palmitic acid metabolism in the soleus muscle in vitro in hypo- and hyperthyroid rats

The aim of this study was to establish whether the rate of fatty acid (FA) incorporation and its utilization by the isolated soleus muscle is modified under conditions of thyroid hormone deficit or excess. The rate of palmitic acid (PA) uptake, oxidation and incorporation into intramuscular lipids with increasing PA concentration (0.5–1.5 mM) in the incubation medium were determined. In hypothyroid rats intramuscular triacylglycerol (TG) synthesis was increased, while the rate of PA oxidation to CO₂ and incorporation into mono- and diacylglycerols (MG/DG) and phospholipids (PL) remained unchanged. In rats with triiodothyronine (T₃) excess the rate of all processes studied was enhanced, although the percentage incorporation of PA into different classes of intramuscular lipids was fairly constant and, independently of thyroid state and FA concentration in the medium, was 56–66% for TG, 9-14% for MG/DG and 24–32% for PL. Our results thus indicate that even short-term T₃ excess accelerates the rate of FA uptake and metabolism in the oxidative soleus muscle, whereas in hypothyroid rats only intramuscular TG synthesis is affected.