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PubMed1986

Cancer is a complication of severe immunosuppression.

Diverse states of immunodeficiency are complicated by the development of various types of cancer. Most striking in nearly every type of immunodeficiency is a high incidence of a neoplasm of the immune system itself, the NHL, which consists of activated pathologic lymphocytes. Morphologically, these are mostly reticulum cell sarcomas, arise from B-cells, tend to be extranodal in distribution, have a predilection for the brain and are probably caused by EBV. If Kaposi's sarcoma is a form of lymphoreticular malignant disease, as some investigators think, the situation becomes even more intriguing. We need to intensively study the various groups of immunosuppressed patients, including those with and without tumors, to seek clues to the cause of neoplasms. Such information may shed light on the causes of similar malignant conditions that occur in the general population and on the role of the immune system in the control of cancer. Hopefully, such knowledge may lead to immunologic methods for the prevention and cure of neoplasms.

Acquired Immunodeficiency Syndrome↗
PubMed1985

The thymus in congenital heart disease.

Some cases of thymus developmental defects (DiGeorge's syndrome) are associated with the development of defects of the heart and great vessels. To see if anomalies of the heart are also associated with anomalies of the thymus, a material of thymus tissue removed during operation for congenital heart disease was compared with thymus tissue from a forensic autopsy material representing the same age group, but without cardiac lesions. Among 27 cases of congenital heart disease, occurrence in the thymus of solid epithelial cords were seen in 6 and cyst- or duct-like structures were found in 7. Among 47 forensic cases, 1 showed cords and another showed duct-like structures. Of these, one had congenital anomalies in the form of hepatic hamartomas and nesidioblastosis, whereas the other had died from morbilli, raising the possibility of congenital immune deficiency. Anyhow, a striking occurrence of minor morphological deviations in the structure of the thymus was found in children with congenital heart disease. Such material therefore may be less suitable for studying the morphology and biology of the normal thymus gland.

Abnormalities, Multiple↗
PubMedDate not supplied

Dental considerations for individuals with Down syndrome.

Down syndrome is one of the most frequently encountered and easily recognizable of all developmental disabilities. Patients with Down syndrome exhibit mental retardation and often present with associated medical conditions, such as cardiac defects, immune deficiencies and musculoskeletal disorders. It is important for the dental practitioner to be familiar with the medical aspects of Down syndrome, as well as the potential behavioral issues. We present a brief review of Down syndrome for the dental provider and suggest that with a little knowledge and some patience, most private practitioners can easily accommodate these patients in their practice.

Dental Care for Persons with Disabilities↗
PubMed1986

Thymus deficiency in an infant with a chromosome t(18;22)(q12.2;p11.2)pat rearrangement.

The finding of an unbalanced t(18;22)pat chromosome rearrangement in a boy with multiple anomalies including apparent absence of the thymus is described. The observation is of interest because of the reported association of chromosome 22 rearrangements with the DiGeorge sequence. In contrast to previous reports of this association, the deletion involving chromosome 22 is confined to the short arm.

Abnormalities, Multiple↗
PubMed1980

A new immunodeficiency disorder in humans involving NK cells.

Immunodeficiency disorders have provided much information on the development and interaction of the various B and T lymphoid components in the immune system of man. As the lymphoid system becomes increasingly divided into functional subsets of cells it will be important to find immunodeficiencies affecting newly discovered cell types. Natural killer (NK) cells are a recently described but ill-defined subpopulation of lymphocytes which is thought to play an important part in surveillance against tumour development. Mice homozygous for the beige gene were found to have a selective deficiency in NK function and were more susceptible to transplantation of syngeneic tumours as predicted. We report here that patients carrying the analogous, autosomal recessive Chediak-Higashi (CH) gene have a profound defect in their ability to spontaneously lyse various tumour cells in vitro by either antibody-dependent or independent mechanisms. Since other cell-mediated cytolytic functions were relatively normal, these results suggest that the beige or Chediak-Higashi gene in both man and mouse controls NK function.

Adult↗
PubMedDate not supplied

[Use of intravenous immunoglobulins in pediatrics].

Intramuscular Immunoglobulin (IMIG) have been used for 40 years in substitution therapy for antibody deficiencies and as prophylaxis for and treatment of several infectious diseases. Modified and intact intravenous immunoglobulin preparations (IVIG) have now been available for more than 10 years: only the intact product express full Fc- mediated functions with a biological half-life of IgG (3-4 weeks). These preparations have constituted an important achievement in the treatment of humoral immunodeficiencies also resulting in a dramatic improvement of the prognosis. The use of IVIG has also modified the therapeutic approach to several secondary and acquired immunodeficiencies. Treatment with IVIG for immune modulation in several diseases is investigated: substantial data indicate a useful role in selected cases of idiopathic thrombocytopenic purpura, Kawasaky disease and in some neurologic diseases. IVIG are substantially safe and severe side effects have been rarely reported.

Acquired Immunodeficiency Syndrome↗
PubMed1993

New and old immunodeficiencies.

Yesterday's immunodeficiencies emphasized the clinical and familial associations of the syndromes and date from the 1920s (ataxia-telangiectasia, chronic mucocutaneous candidiasis), the 1930s (Wiskott-Aldrich syndrome), skipping the 1940s, but blossoming in the 15-y period from 1950 to 1965. In this period, primary immunodeficiencies affecting all the major limbs of the immune system were first described (1950: severe combined immunodeficiency; 1952: X-linked agammaglobulinemia; 1957: chronic granulomatous disease; 1965: C2 deficiency). Today's immunodeficiencies, as detailed in Stiehm's Immunologic Disorders in Infants and Children (Edition 1, 1973; Edition 2, 1980; and Edition 3, 1989) emphasize the immunologic and genetic aspects of immunodeficiency. These increased from 43 syndromes in the 1973 edition (34 primary, nine secondary) to 94 syndromes in the 1989 edition (66 primary, 28 secondary). This means that about two primary and one secondary immunodeficiencies have been uncovered annually. Tomorrow's immunodeficiencies, to be covered in Edition 4, will include new clinical and immunologic observations and molecular and biochemical studies that characterize some unique immunodeficiencies. These include the following six groups of defects: 1) neutropenic syndromes with hypogammaglobulinemia, including the WHIM syndrome; 2) phenotypic genetic syndromes with immunodeficiency including Bloom's syndrome and Schimke's immuno-osseous dysplasia; 3) natural killer cell defects associated with a) other primary immunodeficiencies, b) other nonimmunologic illness, and c) primary natural killer defects; 4) T-cell membrane defects; 5) IL defects; and 6) miscellaneous phagocytic illnesses including periodontitis and the asplenia syndrome.

Allergy and Immunology↗
PubMed1989

[Graft versus host reaction in an infant with DiGeorge syndrome].

An infant with complete DiGeorge syndrome was treated with blood transfusions and fresh frozen plasma because of severe septicemia and anemia. 9 weeks after the first transfusion and 2 weeks after administration of fresh frozen plasma he died of acute graft-versus-host disease. The blood products were routinely irradiated with 25 gray, the fresh frozen plasma was not irradiated. The diagnosis of GvHD was confirmed on autopsy. All cellular blood products including fresh frozen plasma which are used in the treatment of immuno-deficient patients have to be irradiated to avoid GvHD. Currently 50 gray are recommended.

Blood Transfusion↗
PubMed1989

Interstitial deletion of chromosome 22 in a patient with the DiGeorge malformation sequence.

We describe a chromosome 22 deletion in a patient with the DiGeorge malformation sequence as manifested by an interrupted aortic arch, mild thymic hypoplasia, and minor craniofacial anomalies. Although others have reported DiGeorge sequence patients with deletions derived from unbalanced translocations involving the chromosome 22 long arm, the small interstitial deletion described here appears to be unusual for patients with this disorder.

Aorta, Thoracic↗
PubMed1982

Immunodeficiency and syndromes: a nosographic approach.

Immunodeficiency disorders can be classified on clinical grounds into two broad groups according to whether all features are the result of the immune defect (immunodeficiency syndromes) or whether many, even prominent, features cannot be explained by the immune defect (syndromes with immunodeficiency). X-linked agammaglobulinemia and X-linked chronic granulomatous disease are paradigmatic examples of immunodeficiency syndromes. Despite some overlap (for instance extra-immune symptoms, although minor, are present in several variants of severe combined immunodeficiency and chronic granulomatous disease) immunodeficiency syndromes and syndromes with immunodeficiency are easily distinguishable. Together with the pathogenetic classification of the WHO, the present approach to a clinical classification amplifies the operational concept of immunodeficiency also from a therapeutic point of view.

Acrodermatitis↗
PubMed1985

Defence of mucous membranes by antibodies, receptor analogues and non-specific host factors.

Most infections reach man via the mucosal membranes, and more than half of the lymphoid system is found in connection with mucosae. The major antibodies found on mucous membranes are secretory IgA, which function primarily by binding microorganisms and thereby preventing their contact with the host tissues. The optimal mode of immunization to obtain a secretory IgA response is not well defined. Repeated mucosal exposure with antigen may result in oral tolerance, with decreasing circulating antibodies but a remaining secretory IgA response. The secretory IgA response is usually short-lived and can be difficult to boost. IgM as well as IgG antibodies may add to host defence at the mucosal level, but when engaged, they usually induce inflammation in host tissues. Analogues to bacterial receptors on mucosal epithelium may be present in exocrine secretions such as human milk. During an attack on the host, it is possible that such receptor analogues may aid in the prevention of attachment of bacteria to mucous membranes used as an initial site. A number of non-specific host factors support mucosal defence. One of them is lactoferrin. Lactoferrin deficiency seems to result in recurrent bacterial infections, suggesting its importance in normal host defence.

Antibody Formation↗
PubMed1986

Immunogenetics.

The 1985 Catalog of Mapped Genes (Human Gene Mapping 8; 33) has been used to pick out the known, immunologically important genes; these are then discussed in the following order: genes controlling organs, tissues and cells of the immune apparatus, genes determining 'self' structures, genes determining the structures of immunological specificity, genes determining substances with immunoregulatory and effector properties. The symbols for the genes and the biological functions of their products are explained. The genetics of the ABO blood groups, of the HLA-system and of antibody formation are given in rather more detail.

ABO Blood-Group System↗
PubMed2004

Griscelli syndrome.

An eight month old male infant presented with recurrent infections and partial albinism. Initially a possibility of Chediak Higashi syndrome (CHS) was considered, but a negative investigative work up prompted us to look for an alternate diagnosis. A literature search revealed that Griscelli syndrome (GS) has overlapping symptoms and signs. The findings in skin and hair biopsies in Griscelli syndrome are distinctive.

Chediak-Higashi Syndrome↗