1,721,089 research outputs found
Immunoregulatory T subsets in chronic active viral hepatitis: characterization by monoclonal antibodies.
No full textImmunoregulatory T subsets, defined by monoclonal antibodies, were enumerated in children affected by HBsAg-positive chronic active hepatitis. The helper to suppressor/cytotoxic cells ratio was lower in patients than in age-matched controls. The lower ratio was mainly due to an increase of lymphocytes of the suppressor/cytotoxic phenotype. Helper cells were even fewer in severe chronic hepatitis patients, thereby lowering still further the helper/suppressor ratio. Therapy seemed to influence the ratio in patients affected by moderate active chronic hepatitis, for four of eight children treated with azathioprine and prednisone had a ratio within -1 SD of normal values. The increase of the suppressor/cytotoxic cells in patients affected by chronic hepatitis might be a means for limiting virus-induced cell hyperactivity
Hypogammaglobulinemia and T cell subsets unbalanced in an infant with perinatal B hepatitis infection. An acquired or congenital immunodeficiency syndrome.
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New strategies for the treatment of lysosomal storage diseases
No full textThe lysosomal storage diseases (LSDs) are a group
of inherited metabolic disorders caused by the deficiency of
any of the lysosomal functions, in most cases of lysosomal
hydrolases. LSDs are typically characterized by storage of a
variety of substrates in multiple tissues and organs and by the
variable association of unusual clinical manifestations that
are often responsible for physical and neurological handicaps.
During the past two decades, research in the field of LSDs has
made marked progress, particularly with the development of
a variety of innovative therapeutic approaches. These include
several strategies aimed at increasing the residual activity of
the missing enzyme, such as hematopoietic stem cell transplantation,
enzyme replacement therapy, pharmacological
chaperone therapy and gene therapy. An alternative approach
is based on reducing the synthesis of the stored substrate. More
recently, the improved knowledge on LSD pathophysiology
has indicated additional targets of therapy. The recent progress
made in the treatment of LSDs represents a good model that
may be extended to other genetic disorders
Difetto immunologico combinato in un paziente con epatite cronica da virus B a modico grado di attivita'
No full textAbnormal pattern of distribution of IgG subclasses in children with chronic hepatitis B virus infection.
No full textImmunoregulatory functional abnormalities in children affected by HBsAg-positive chronic active hepatitis: role of prostaglandins in T-mediated suppression.
No full textSuppressor cell function was evaluated in children affected by HBsAg-positive chronic active hepatitis. Circulating concanavalin A- (ConA) precultured lymphocytes failed to suppress the proliferative response of autologous responder cells to a mitogen. In four of eight patients with a failure of ConA-induced suppressor activity, indomethacin added during the induction phase of T suppressor cells abolished the defect, indicating that prostaglandin-producing adherent cells may influence ConA-induced suppressor activity. An inverse relationship between suppressor cell activity and the number of suppressor/cytotoxic subsets defined by the OK T8 monoclonal antibody was found. Our findings strongly support the hypothesis that an abnormality in the immunoregulatory system plays a role in the pathogenesis of HBsAg-related chronic active hepatitis. It is also suggested that non-T regulatory cells are implicated in the immunological abnormality in chronic active hepatitis
DiGeorge Syndrome
No full textDiGeorge syndrome, also known as 22q11.2 deletion syndrome, is the prototype of syndromes due to defective development of the third and fourth pharyngeal pouch. Even though the association between thymic aplasia and congenital hypoparathyroidism was first observed by Sedlackova in 1955 and Lobdell in 1959, only in 1965 were these signs classified as a new syndrome called DiGeorge (DGS) syndrome, from the name of Dr. Angelo DiGeorge, who reported a few infants with congenital absence of the thymus and parathyroid glands. Congenital heart disease (CHD), particularly involving the outflow tract, was later added to the list of the typical symptoms. However, the phenotypic spectrum of the syndrome is very wide (McDonald-McGinn and Sullivan 2011). The DGS phenotype, initially restricted to the presence of all or more than one of the above-mentioned signs, was extended over time even to patients with only a few classic symptoms, not necessarily associated with the presence of endocrine or immunological alterations, such as Velocardio Facial Syndrome or VCSF (MIM192430), or the Conotruncal Anomaly Face Syndrome (CTAFS)/Takao syndrome (MIM217095). VCFS has been defined as the association of palatoschisis, cardiac defects, typical facies, and difficulties in acquiring language and learning skills, while CTAFS is characterized by conotruncal cardiac defects and a peculiar facial appearance. The finding that 22q11.2 deletion can be detected in these subjects confirms that CTAFS, VCFS, and DGS are the same entity. A DGS phenotype may be also found in patients with diabetic or retinoic acid embryopathy and it has also been described in patients carrying mutations in the Chromodomain Helicase DNA-binding Protein 7 gene (CHD7), responsible for CHARGE syndrome, T-box 1 gene (TBX1), or other chromosomal alterations, including the 10p13-14, 11q23ter11, 3p12.3, 17p13, and 4q34.1q35.2 (Corsten-Janssen et al. 2013; Cirillo et al. 2017), suggesting that the biological differentiation process of the organs involved in the syndrome involves a very high number of genes, as expected. A different degree of immunological abnormalities has been described in all these conditions
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
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