Rhinologic and sinonasal changes in PR3 ANCA pulmonary vasculitis.

BACKGROUND: Over 70-95% patients with PR3 ANCA pulmonary vasculitis present with upper respiratory tract symptoms or sings. Nasal cavity usually presents with obstruction and chronic refractory infections (rhinosinusitis) which commonly manifest as bloody discharge or crusting obstruction. Mucopurulent discharge may occur in the acute phase or remission, along with other symptoms suggesting sinusitis. Later on, saddle nose deformities can occur due to collapse of the nasal septum. Other common destruction areas are the maxillary ostia, erosion of the tubinates or damage of soft palate. OBJECTIVE: The aim of the study was to characterize pathologies of nasal and sinonasal CT scans in patients with PR3 pulmonary ANCA vasculitis and to establish the CT diagnostic criteria for WG. Between 2005-2009 sinonasal CT visualization was performed in 35 patients (19 female, 16 male) with PR3 ANCA positive WG. RESULTS: Bony destruction of the nasal cavity was revealed in 15 (42.8%), damage or distortion of the paranasal sinuses in 20 (57.1%), the mastoid cells in 7 (20%), and the orbits in 7 (20%) patients. Sclerosing osteitis of the nasal cavity and paranasal sinuses were observed in 11 (31.4%) and in 24 (68.5%), respectively. Bony thickening of the nasal cavity was shown in 5 (14.2%) patients and of the paranasal sinuses in 7 (20%) (unilateral in 2 and bilateral in 5 patients). Seven patients (20%) had orbital masses; all unilateral. Septal perforation was observed in 11 (31.4%) and saddle nose deformity in 7 (20%) patients. CONCLUSIONS: Maxillary sinuses are regions which are most frequently affected during the course of PR3 ANCA pulmonary vasculitis. CT imagines may be a useful supplement to clinical and activity scoring of WG disease with pulmonary involvement.

Combined Therapies for Lysosomal Storage Diseases.

Lysosomal storage diseases (LSDs) is a group consisting of over 50 disorders caused mostly by dysfunctions of lysosomal proteins and resultant accumulation of particular compounds inside cells and extracellular volumes in affected organisms. Genetic diseases are among the most difficult targets for medical treatment. Nevertheless, understanding of molecular bases of LSDs made it possible to develop novel procedures of treatment, employing molecular medicine. Although various therapeutic approaches have been proposed, and some of them were introduced into clinical practice, none of them was found to be effective in correcting all symptoms in treated patients. Central nervous system and skeleton appear to be the most difficult targets to be improved. Therefore, a proposal appeared that perhaps no single therapeutic procedure may be fully effective in treatment of LSD patients, and only combination of two or more approaches could be a successful therapy. In this review, we present and discuss current stage of various combination therapies for LSDs, based on already available published data.

[Effect of detergents on the solubilization of 5'-nucleotidase from the thyroid gland]. / Wplyw detergentów na solubilizacje 5'-nukleotydazy z tarczycy.

The effect of Triton X-100, sodium deoxycholate and saponin upon the solubilization of 5'-nucleotidase, the membrane enzyme derived from pig thyroid was studied. Triton X-100 at the concentration of 0.1% did not cause enzyme solubilization, whereas at the concentration of 1.0% it caused only partial release of the former from the membranes. Saponin (1.0% concentration) brought about a marked (about threefold) increase in the enzyme activity which resulted from the exposing active loci of the enzyme, however it did not cause total solubilization of it. 1% sodium deoxycholate increased the enzyme activity by 5 times and also caused its almost total solubilization (over 90%). The results indicate the 5'-nucleotidase is strongly bound to the cell membranes and the non-ionic detergents like Triton X-100 and saponin do not fit for the solubilization of this enzyme.

[Liver microsomal enzyme function in patients with duodenal ulcer treated with cimetidine]. / Czynnosc enzymów mikrosomalnych watroby u pacjentów z choroba wrzodowa dwunastnicy, leczonych cymetydyna.

The functional status of hepatocytes was evaluated using the breathing test with 14C-aminopyrine in a group of patients with duodenal ulcer treated with cimetidine, and the reversibility of liver microsomal system activity changes was assessed at various time intervals after the completion of treatment with the above mentioned drug. Thirty patients with duodenal ulcer treated with cimetidine were examined: before the treatment (Ia), after one day of treatment (Ib), after four weeks of treatment (Ic), after eight weeks of treatment (Id), one week after completion of treatment (Ie), and four weeks after completion of treatment. A significant decrease was found of the value of the breathing test already after one day of treatment with cimetidine which was more pronounced after four and eight weeks of treatment. An impaired microsomal system activity was shown even one week after completion of the treatment with cimetidine. The obtained results indicate the necessity of caution when other drugs are administered to patients treated with cimetidine, and this refers particularly to drugs impairing liver function, or metabolized in this organ.

[Evaluation of microsomal enzyme function in the liver in patients with duodenal ulcer treated with famotidine]. / Ocena czynnosci enzymów mikrosomalnych watroby u osób z choroba wrzodowa dwunastnicy leczonych famotydyna.

The studies of microsomal liver enzyme activity by means of a respiratory test with 14C-aminopyrine (ABT) were carried out in 20 patients with duodenal ulcer treated for eight weeks with famotidine. The respiratory test was performed before the introduction of treatment (group I), after four weeks of treatment (group II), and after eight weeks of treatment (group III). The mean value of the respiratory test before treatment was 5.32% of the dose/h. After four weeks of treatment with famotidine a significant decrease was found of the test value (x = 4.79% of the dose/h). An even stronger inhibitory action of famotidine on the microsomal system was shown after eight weeks of the treatment (x = 4.46% of the dose/h). These results evidence a depressive effect of famotidine on the activity of liver monooxygenases. In patients taking this drug, other drugs metabolised in the hepatocyte microsomal system should be used with high caution, as well as those which are known to be inhibitors of this system.

[Transforming growth factors and their role in chronic liver diseases]. / Transformujace czynniki wzrostu i ich rola w przewleklych chorobach watroby.

The transforming growth factors are endogenous polypeptide substances having own cell receptors. Among them two main factors have been isolated--transforming growth factor alpha (TGF alpha) and beta (TGF beta). TGF alpha is secreted by activated proliferating hepatocytes and by certain neoplastic cells. It stimulates the synthesis of DNA and migration of hepatic epithelial cells. In chronic liver diseases it is produced in increased amounts and stimulates then proliferation and regeneration. TGF beta is released by non-parenchymal liver cells, thrombocytes, and neoplastic cells. It inhibits the synthesis of DNA in many cells, inhibits the synthesis of albumins and fibrinogen and regulates the production of acute phase proteins. In chronic liver diseases it increases the production of fibronectin and collagen, inhibiting at the same time their enzymatic breakdown. TGF beta participates in the development and progression of hepatic fibrosis.

[Mixed tumor of the lacrimal gland (case history over a 20-year period)]. / Guz mieszany gruczolu lzowego (opis przypadku o 20-letnim przebiegu).

A case of the tumor of the orbita is presented. It caused large exophthalmus and partial damage of orbital bones without any loss in the visual acuity in the 20-year period of increasing. Before operation a tumor of the lacrimal gland or an angioma was suspected. A histopathological examination of the whole removed tumor revealed the presence of mixed tumor cells.