Neutrophils promote the release of alpha-6-fucosyltransferase from blood platelets through the action of cathepsin G and elastase.

Human blood platelets release alpha-6-fucosyltransferase during coagulation of blood or after stimulation with thrombin or other agonists that cause platelet activation (Antoniewicz et al., FEBS Lett. 244 (1989) 388-390). However, in the absence of neutrophils the thrombin-stimulated platelets release only a small fraction of alpha-6-fucosyltransferase activity (Koscielak et al., Acta Biochim. Polon. 42 (1995) 35-40). We show that the effect of neutrophils is reproduced by cathepsin G or (less efficiently) by elastase, the two enzymes that are released by neutrophils during coagulation of blood. We have also localized alpha-6-fucosyltransferase to membrane and alpha-granule fractions of platelets that had been disrupted by nitrogen cavitation. It is concluded that thrombin-activated neutrophils release cathepsin G and elastase that promote degranulation of platelets and hence the secretion of alpha-6-fucosyltransferase.

[Tubulointerstitial nephritis related to immunotherapy and immunostimulation: a report of two cases]. / Sródmiazszowe zapalenie nerek zwiazane ze stosowaniem immunoterapii i leków immunostymulujacych--opis 2 przypadków.

Two cases of tubulointerstitial nephritis (TIN) with renal failure related to immunotherapy (case 1) and immunostimulation (case 2) have been described. Case 1: 18 years old male patient with hay fever was admitted because of rapid increase of serum creatinine from 1.1 mg/dl to 5.5 mg/dl, fever, weight loss and anemia which developed during 6 months after second course of immunotherapy. Case 2: 12 years old boy was admitted because of fever, weight loss and rapid progression to renal failure after treatment of pharyngitis with antibiotics and immunostimulant drug. In both patients renal biopsy was performed and TIN with huge lymphocytes T infiltrates was diagnosed. After 6 months treatment with corticosteroids renal function turned back to previous levels in both patients. Pathogenesis and treatment of TIN is discussed.

Short report: erythrocyte membranes from a patient with congenital dyserythropoietic anaemia type I (CDA-I) show identical, although less pronounced, glycoconjugate abnormalities to those from patients with CDA-II (HEMPAS).

Congenital dyserythropoietic anaemias (CDAs) are rare hereditary disorders characterized by ineffective erythropoiesis and multinuclearity of erythroblasts. Three main types of the disease have been described. Glycoconjugate abnormalities in erythrocyte membrane glycoconjugates, consisting of hypoglycosylation of band 3 and accumulation of certain glycosphingolipids including lactotriaosylceramide, neolactotriaosylceramide and polyglycosylceramides, have been described only in patients with CDA type II (CDA-II). We report on identical, although less pronounced, abnormalities in erythrocyte glycoconjugates from a patient with CDA-I. A low degree of hypoglycosylation of band 3 in our patient with CDA-I suggests that hypoglycosylation is not a cause, but, most probably, a consequence of dyserythropoiesis.

Encapsulation of parathyroid cells in hollow fibers: a preliminary report.

The purpose of experiments was to evaluate the survival and functioning of human parathyroid cells after encapsulation in hollow fibers (HFs). The polypropylene HFs K600(PP Accurel (Akzo-Nobel, Germany) of inner diameter 0.6 mm, wall thickness 0.2 mm, original or surface modified were used for encapsulation. Production of parathormone (PTH) by encapsulated cells was measured in vitro. HF were filled with parathyroid cell suspension and tightly closed. Encapsulated cells were cultured for 9 or 33 days in RPMI 1640 containing 10% FCS or in Chang's medium. The level of PTH, produced by encapsulated cells was evaluated in the culture medium with radioimmunoassay test (RIA). The assays were performed every 2-4 days. The result of PTH assay was similar in both types of tested media as well as with unmodified and modified HFs, being 2-4 pg/ml of culture medium per 10(3) encapsulated cells. In conclusion, encapsulation in original or modified HFs ensures diffusion of nutrients from culture medium to encapsulated cells and allows for functioning of cells for at least 33 days in vitro.

Lewis antigens and argyrophilic nucleolar organizer regions staining for assessment of potential malignancy of adenomatous polyps of the gastrointestinal tract in children.

Adenomatous polyps (AP) of the gastrointestinal tract in children are very rare. Because of their potential malignancy, they are of great clinical importance. There is little experience in the management of children with AP. The immunohistochemical expression of the Lewis blood group antigens (BGA) (sialosyl-Le(a), Le(a), Leb, Le(x), and Le(y)) and the number of activated nucleoli with the silver staining method for nucleolar organizer regions (AgNORs) were studied in two children with AP. In a girl with isolated AP of the stomach and colon, it was found that antigens Le(b) and s-Le(a) were expressed extensively in the gastric adenoma, and sialosyl-Le(a) throughout the entire length of the rectal adenoma crypts, but in the AgNORs stain the number of nucleoli ranged from two to four, evidencing changes of a benign character. In the case of familial adenomatous polyposis diagnosed in a 9-year-old boy, in some colonic adenomas the number of activated nucleoli was greater than five, and the Le(b) antigen was expressed in superficial epithelial cells in one of the adenomas. Also, extensive expression of antigens Le(y) and s-Le(a) throughout the entire length of the crypt in another polyp removed was observed. We believe that immunohistochemical study of the intensity and extent of the expression of Lewis BGA in the polyp tissue simultaneously with the determination of the number of activated nucleoli by the AgNORs staining method can be helpful in better analysis of cytological risk factors of a malignant transformation.

Are mu-opioid receptors involved in the control of endothelin-1 release from the pituitary gland in normal and dehydrated rats?

UNLABELLED: The objective of the present study was to investigate whether the endogenous opioids are involved in the control of endothelin-1 release from the pituitary gland. To test this hypothesis we have measured the peripheral plasma concentration of ET-1 as well as the content of immunoreactive ET-1 (irET-1) in the pituitary in response to opioid receptors blockade in euhydrated and 24 h water-deprived Wistar-Kyoto rats. Placebo or naltrexone (50 micrograms/kg body wt.) were given i.v. in both groups. Trunk blood was collected to determine hematocrit, plasma sodium and ET-1 levels (RIA). Immunostaining of ET-1 in the whole pituitary glands was performed by colloidal gold labeling. The quantitative analysis of irET-1 was carried out under a light microscope using a computerized image analyzer (MultiScan). RESULTS: (1) Twenty-four-hour dehydration resulted in marked increase of peripheral concentration of ET-1. Naltrexone injection induced a significant elevation of ET-1 plasma concentration in both, dehydrated and control animals. (2) The content of irET-1 in anterior and intermediate lobes of the pituitary in dehydrated rats was markedly higher than in control group. (3) Naltrexone injection caused a rapid and significant reduction irET-1 within the anterior, intermediate and posterior lobes in dehydrated and control animals. CONCLUSIONS: (1) An elevation of irET-1 in the pituitary gland and peripheral circulation in dehydrated animals may play a role in maintaining of water-electrolyte balance. (2) The mu-opioid system appears to control the ET-1 release from the pituitary in normal and dehydrated animals.

Clinical, biochemical and histological analysis of seven patients with cholesteryl ester storage disease.

Lysosomal acid lipase (LAL) deficiency leads to two phenotypically different diseases: cholesteryl ester storage disease (CESD) and Wolman's disease. Lysosomal acid lipase hydrolyzes cholesteryl esters and triglycerides. Deficiency of LAL results in intralysosomal storage of cholesteryl esters and triglycerides. CESD has a chronic and benign course and is characterized by hepatomegaly and mild hypercholesterolemia. It leads to fibrosis (cirrhosis) and early atherosclerosis. This report presents the clinical, biochemical and microscopic data of seven patients with CESD followed up over 10 years. The physical development of all the study children remained within the normal range; 7 patients had hepatomegaly and 6 also had splenomegaly. Three patients had normal cholesterol, triglycerides and transaminases values; the other four had slightly elevated levels for these parameters. The activity of LAL in all patients was reduced to below 30% of the lower normal value. Histologically, cholesteryl crystals and lipid storage vacuoles in Kupffer cells were present in all examined patients except one. Accumulation of cholesteryl esters was visible on thin-layer chromatography of lipid extracts obtained from liver biopsies.

Serum and salivary antigliadin antibodies and serum IgA anti-endomysium antibodies as a screening test for coeliac disease.

Serum and salivary IgA and IgG antigliadin antibodies were determined by an enzyme-linked immunosorbent assay in 18 children with villous atrophy and 30 children on a gluten-free diet for coeliac disease in whom normal intestinal mucosa was found. Serum IgA anti-endomysium antibodies were also determined by an immunofluorescence method in these children. Serum IgG antigliadin and IgA anti-endomysium antibodies had the highest sensitivity (100 and 94.4%, respectively), followed by serum IgA antibodies to gliadin (72.2%), salivary IgA antigliadin (61.2%) and IgG antigliadin (50%) antibodies. The highest specificity was found for serum IgA anti-endomysium (100%) and IgA antigliadin (96.6%) antibodies and salivary IgA and IgG antigliadin antibodies (93.3%), while serum IgG antigliadin antibodies were found to be least specific (63.3%).

Cultured parathyroid cells allotransplantation without immunosuppression for treatment of intractable hypoparathyroidism.

Cultured, viable and functioning ABO compatible parathyroid cells were allografted in 18 nonimmunosuppressed patients with a postoperative hypoparathyroidism. Variable, but promising biochemical and clinical results were obtained. Clinical and biochemical observations have documented graft function up to 14 months. The mechanism of cessation of function of implanted cultured cell suspension remains unknown. Some parameters, suggest a rejection mechanism but other mechanisms can not be excluded. This suggests that some form of immune modulation may be necessary to improve further PT allograft survival in recipients off immunosuppressive therapy.

[Allotransplantation of the parathyroid in patients without immunosuppression]. / Allotransplantacja przytarczyc u chorych bez immunosupresji.

Eighteen patients with postoperative insufficiency (after thyroid operations) have been treated with cultured, hormonally active, living and ABO the same group type parathyroid cells. Explants had been taken from 2 operated patients with secondary hyperparathyroidism. Hormonal activity of the transplanted parathyroid cells under the fascia of the brachio-radialis muscle has been confirmed by clinical, biochemical tests and the level of PTH in blood serum from the vein of non-grafted arm. Hormonal activity of the graft was variable but lasted up to 14 months.

Pancreatic glucagon levels in infants and children with hyperinsulinemia.

Serum concentrations of pancreatic glucagon were measured in 40 patients with hyperinsulinemic hypoglycemia. In infants with severe disease the glucagon levels were markedly elevated; lower levels were observed in children with episodic hypoglycemia. During diazoxide treatment and after pancreatectomy, the glucagon levels decreased proportionally to suppression of the insulin secretion. Thus the secretion of glucagon seems to be retained in hyperinsulinism in proportion to the insulin hypersecretion, and an increased glucagon level indicates long during and severity of hyperinsulinemic hypoglycemia.

[Diagnostic problems of congenital muscular dystrophies in children]. / Problemy diagnostyczne we wrodzonych dystrofiach miesniowych u dzieci.

Three children with a diagnosis of congenital muscular dystrophy are described. Because of the heterogeneity of these disorders the authors stress the necessity of differentiation with other causes of "floppy infant" syndrome, especially with Werdnig-Hoffmann disease, structural myopathies, and Duchenne progressive muscular dystrophy. An extensive hypodense area on brain CT scan was found in one child.

The value of quantitative measurement of HBeAg and HBsAg before interferon-alpha treatment of chronic hepatitis B in children.

Serum concentrations of HBsAg, HBeAg and hepatitis B virus DNA were measured quantitatively before interferon treatment in 23 children (17 boys, 6 girls) suffering from chronic hepatitis B, and correlated to the outcome of the treatment. Five children remained HBsAg- and HBeAg-positive throughout the treatment and 6 months after the end of the treatment (non-responders), 12 children eliminated HBeAg but not HBsAg (partial responders) and six eliminated HBeAg and HBsAg (complete responders). The five non-responders had significantly higher initial HBsAg and HBeAg concentrations and significantly lower alanine aminotransferase levels than the partial or complete responders. The six complete responders had significantly lower HBsAg concentrations than the partial or non-responders, and seemed to be younger. No significant difference in HBV DNA levels was found in the three response groups. These data suggest that quantitative assays of HBsAg and HBeAg are particularly useful in selecting patients with chronic hepatitis B for interferon therapy.

Expression of antigens homologous to human retrovirus molecules in normal and severely atrophic thymus.

An immunopathologic study of normal and severely atrophic thymuses (STA) was undertaken in order to evaluate the expression of human retrovirus (envelope and core) molecules in thymic epithelial cells (TEC) in HIV negative children. Both normal and STE thymuses disclosed p19, p24, p39, p45 and p55 viral core proteins as well as gp46, gp63 glicoprotein of envelope origin. No evidence of gp160, gp120 and gp41 molecules were observed in TEC which suggested endogenous lack of receptor molecules for HIV. The results are discussed in the context of possible thymus oriented autoimmune reaction in HIV and non-HIV bearing patients and in consequence, severe injury of TEC forming microenvironment.

Calcyclin as a marker of human epithelial cells and fibroblasts.

The distribution of calcyclin in human tissues was studied using polyclonal antibodies against this protein. In all organs examined (breast, heart, intestine, kidney, liver, ovary, placenta, stomach, thymus, and uterus) only epithelial cells and fibroblasts were stained. This suggests that calcyclin expression is related either to proliferation rate or secretion activity. The data show that calcyclin might be considered as a marker of some human epithelial cells and fibroblasts.

Procollagen type III peptide in the assessment of liver fibrosis in children with congenital alpha-1-antitrypsin deficiency.

In 31 children with congenital alpha 1-antitrypsin deficiency the concentration of procollagen type III peptide was determined in a trial establishing liver fibrosis degree and monitoring of fibrosis progression. No correlation was found between the degree liver fibrosis determined by histological examination and the serum concentration of procollagen type III peptide. The concentration of procollagen type III peptide was higher with coexistent cholestasis and in case of inflammatory processes outside the liver (pneumonia). In progressing cirrhosis with inflammatory reaction the concentration was higher than in advanced cirrhosis and non-inflammatory liver fibrosis.