[Endoscopic sclerotherapy and esophageal varices].

60 consecutive patients underwent sclerotherapy for hemorrhage from ruptured esophageal varices. Sclerosis was always started within the first 48 hours. 12 patients (20%) died during initial hospitalization, but only 5 from recurrent bleeding.

The relationship of heat-shock proteins, thermotolerance, and protein synthesis.

The relationship of heat-induced inhibition of protein synthesis (HIIPS) and thermotolerance, the transient ability to survive otherwise lethal heat treatments, was studied in HA-1 Chinese hamster fibroblasts exposed to various treatments. A mild heatshock or exposure to sodium arsenite induced a refractoriness to HIIPS, while exposure to the amino acid analog of proline, azetidine, did not. The development and decay of refractoriness to HIIPS after exposure to heat or sodium arsenite paralleled in the increase and decrease of the rate of synthesis of the heat-shock proteins (HSP), and was associated with neither the persistence of elevated levels of HSP nor the persistence of the thermotolerant state.

Miller-Dieker syndrome and monosomy 17p13: a new case.

A 12-year-old boy is described with multiple anomalies and a de novo terminal deletion of 17p13. Based on clinical examination, the Miller-Dieker syndrome was diagnosed.

Evidence for two states of thermotolerance in mammalian cells.

The effect of the inhibition of protein synthesis on the development of thermotolerance in Chinese hamster fibroblasts following a brief heat shock or exposure to sodium arsenite has been examined. Under conditions that inhibit protein synthesis by 95 per cent, significant amounts of thermotolerance develop after a brief exposure to 45 degrees C or continuous exposure to 41 degrees C, without the significant accumulation of heat shock proteins. However, no thermotolerance development in cells treated with sodium arsenite was observed if protein synthesis was inhibited.

Specificity of Mycobacterium tuberculosis phenolic glycolipid (PGL-Tb1) antiserum.

The specificity of Mycobacterium tuberculosis anti-PGL-Tb1 antiserum prepared in rabbits was evaluated by an enzyme-linked immunosorbent assay. It was found that the antiserum immunoreacted with its homologous antigen but not with purified phenolic glycolipids from M. bovis BCG (mycoside B), M.

Cysteine and metalloproteinase activities in serum of Duchenne muscular dystrophic genotypes.

Lysosomal cysteine proteinase (cathepsin B, H, and L) and MMP-7ase muscle metalloproteinase activities were measured in serum from Duchenne muscular dystrophic male patients and their mothers as gene-carriers. The activity of cathepsin H significantly increased in the Duchenne muscular dystrophic (DMD)-hemizygotes group and in the group of DMD heterozygotes. Significant positive correlation was found between the activity of serum creatine kinase (which previously has been proven to be a marker of muscular dystrophy) and of cathepsin L in the DMD-hemizygotes group.

Spontaneous periodic contraction of the ampullar segment of the human fallopian tube in vitro.

Spontaneous mechanical activity of 36 segments from the ampullar part of human Fallopian tubes was studied in vitro. The age of the patients varied between 37-64 years. Outer diameter and axial tension were continuously measured at an intraluminal pressure of 5 mmHg (0.

Serum complement C2 levels in patients suffering from cystic fibrosis (CF).

Serum C2 complement levels were measured in 17 children suffering from CF, 17 with obstructive bronchitis, and 7 control children. No correlation was found between the C2 level and the clinical stage in Shwachman score, the HLA B7 or B18 antigens and the ventilation functional parameters. The mean serum C2 complement level did not differ in the three investigated groups, but in 5 of the 17 CF patients the serum C2 was diminished according to the possibility of C2 complement heterozygosity.

Normal lipid values (cholesterol, triglycerides and HDL-cholesterol) for children in a rural area of Hungary. A screening program.

Screening for serum total cholesterol (Ch), triglyceride (Tg) and HDL-Ch levels was performed in 3 Hungarian rural areas among 3-14-year-old children, divided into three groups: 3-6, 6-10 and 10-14-year-old, respectively. The mean levels as well as the 85, 90 and 95 percentile values of the investigated lipids are given. The serum HDL-Ch levels were very low in all three groups: 0.

Effect of pharmacological agents on the activity of the circular and longitudinal smooth muscle layers of human fallopian tube ampullar segments.

The in vitro determination of the effects of some pharmacological agents on the outer diameter and axial tension of human Fallopian tube ampullar segments showed that some drugs may act on the circular and longitudinal smooth muscle layers relatively separately. PGF2 alpha elicited an excitatory response in both muscle layers while norepinephrine and isoproterenol decreased the activity. Oxytocin and indomethacin had relatively limited effects on the spontaneous motility of the ampullar segments.

Manifestation of angiokeratoma diffusum in a girl patient with heterozygous genotype for Fabry disease.

The case of a 16 years old girl patient having angiokeratoma corporis diffusum with Fabry heterozygosity is published together with the clinical and genetical screening investigations of the family members. The problem of the detection of the heterozygotes is discussed.

Proficiency testing of conventional drug susceptibility tests of Mycobacterium tuberculosis.

Proficiency testing of indirect drug susceptibility tests of Mycobacterium tuberculosis was begun in 1985 by the Laboratory Centre for Disease Control (LCDC) with the participation of Provincial Public Health Laboratories in Canada. Comparable sets of 60 cultures of Mycobacterium tuberculosis representing 30 strains were distributed by LCDC to the participating laboratories to be tested for drug susceptibility against isoniazid, streptomycin, rifampin, and ethambutol using conventional methodologies. Intralaboratory agreement values determined by comparing results obtained on sets of duplicate cultures were high and were found to vary little from drug to drug and from laboratory to laboratory.

A new translocation t(1;4;11) in congenital acute nonlymphocytic leukemia (acute myeloblastic leukemia).

A new translocation t(1;11;4)(1pter----1p32::11q23----11q13::4p16--- -4qter) was found in the peripheral blood of a patient with congenital acute myeloblastic leukemia (AML). It was concluded that this translocation may represent a new mutation, which caused the leukemia with very high leukocytosis, hepatosplenomegaly, leukemic infiltration of the majority of the organs, and a very poor prognosis.

Activity of the lysosomal cysteine proteinases (cathepsin B,H,L) and a metalloproteinase (MMP-7-ase) in the serum of cystic fibrosis homozygous children.

Lysosomal cysteine proteinase (cathepsin B, H, L) and metalloproteinase (MMP-7-ase) activities were measured from serum of 19 cystic fibrosis (CF) homozygotes and of 13 healthy children, as control group. The activity of cathepsin B and H significantly increased in the CF-group.

HLA investigations in cardiomyopathies.

Typization of HLA A, B and C antigens of peripheral lymphocytes was performed in 14 patients suffering from cardiomyopathy and in 10 family members from 10 families. Among the antigens of locus A, the most frequent were the subgroups of HLA A9 (A23 and A24) in 7/14, and those of HLA A10 (A25 and A26) in 3/14; frequent antigens of locus B were the types B5, B7, B12 and B35. In 2 of 10 families the cardiomyopathy was transmitted by autosomal dominant genes, while the other cardiomyopathy cases were sporadic.

Spontaneous and stimulated lymphocyte transformation test in homozygous children with cystic fibrosis.

Spontaneous and non-specific mitogenic stimulated lymphocyte transformation tests of cellular immune function were carried out. The phytohaemagglutinin, concanavalin A and pokeweed mitogen stimulated lymphocyte transformation was significantly diminished in the group of cystic fibrosis (CF) homozygotes as compared to the controls, as an indication of the impaired reactivity of the T-lymphocytes. The spontaneous phytohaemagglutin stimulated lymphocyte transformation ratio was diminished, too.

Ultrastructural study of peripheral lymphocytes and polymorphonuclear leukocytes in children with lysosomal enzymopathies and hyperlipoproteinemia.

On the basis of electronmicroscopic examinations of the peripheral lymphocytes and polymorphonuclear leukocytes (PMNL) in mucopolysaccharidosis of types I and II in Gaucher and Nieman-Pick diseases, in metachromatic leukodystrophy and in hyperlipoproteinemia, the ultrastructural characteristics are described. Pathological findings with vacuoles formations were observed in Gaucher disease and in metachromatic leukodystrophy against the preliminary literature. The ultrastructural pathological changes are reported from the first ultrastructural PMNL examinations in hyperlipoproteinemias.

Serum lipid and lipoprotein levels in premature ageing syndromes: total lipodystrophy and Cockayne syndrome.

Serum lipids and lipoproteins were investigated in two girls who suffered from total lipodystrophy, in their family members, and in two brothers suffering from Cockayne syndrome. Hyperlipoproteinemia type IIb (Fredrickson) was detected in one case with total lipodystrophy. Very low levels of serum high density lipoprotein cholesterol (HDL-Ch) were observed in both of total lipodystrophic cases, in most of their first degree relatives, and in one of patients with Cockayne syndrome.

Radiometric diagnosis of mycobacteria.

The aim of this study was to determine the impact of recently developed rapid radiometric techniques on the clinical diagnostic operations of a reference laboratory for mycobacteria. Selective inhibition by rho-nitro-alpha-acetylamino-beta-hydroxypropiophenone was used to rapidly screen submitted cultures for identification of mycobacterial species other than Mycobacterium tuberculosis. The radiometric drug susceptibility test was applied only to those cultures presumptively identified as belonging to the Mycobacterium tuberculosis complex.