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PubMed1992

[Abnormality of T lymphocyte subsets in aged persons with kidney deficiency syndrome and its influence on immunologic regulation].

T lymphocyte subsets were investigated in 57 aged persons with Kidney Deficiency Syndrome as well as in 30 normal controls with application of the monoclonal antibody (OKT3, OKT4, OKT8). The results showed that the percentage of OKT3, OKT4 in the aged persons was significantly lower than that of control (P < 0.01), while OKT8 was significantly higher (P < 0.01). The OKT4/OKT8 ratio was significantly lower than that of control (P < 0.01). However, the above-mentioned changes were more evident in the Kidney Yang Deficiency Syndrome than those in Kidney Yin Deficiency Syndrome (P < 0.01). The further analysis indicated that the aged persons with Kidney Yang Deficiency appeared as marked positive correlation (r = 0.64, P < 0.001) between the OKT4/OKT8 ratio and IgA, and they also appeared as marked negative correlation (r = -0.89, P < 0.001 and r = -0.665, P < 0.001 respectively) between the OKT4/OKT8 ratio and C3 or CIC. The results suggested that the imbalance of the cell mediated immunity expressed by the T lymphocyte subsets in aged persons with Kidney Deficiency might be one of the essences of senility.

Aged↗
PubMed1988

Aplastic anaemia complicating adenovirus infection in DiGeorge syndrome.

An 18-month-old child with partial DiGeorge syndrome developed aplastic anaemia during an acute adenovirus infection. Assessment of the child's immune system revealed T-cell subset abnormalities consistent with DiGeorge syndrome. A possible link between the underlying immune deficiency and the observed complication is suggested.

Adenoviridae Infections↗
PubMedDate not supplied

Immunodeficiency diseases: head and neck manifestations.

Infections of the upper respiratory tract are common to both the immunodeficient and the normal child during their development. The most common head and neck manifestations of immunodeficiency disease are recurrent suppurative otitis media, tonsillitis, sinusitis, rhinitis, and nasopharyngitis. Often the head and neck specialist is confronted with a child with one or more of these problems and must institute the appropriate therapy or decide on an avenue for further investigation. This paper outlines the major immunodeficiency state, discusses the immune defects thought to be responsible for the spectrum of clinical findings, and suggests a systematic approach to the evaluation of these difficult diseases. The recognition of immunodeficient individuals is an important step in their treatment so that adjunctive immunological therapy can be provided.

Agammaglobulinemia↗
PubMed2006

Secondary failure of plasma therapy in factor H deficiency.

We report a patient with homozygous factor H deficiency leading to permanent alternate complement activation and early onset of the hemolytic uremic syndrome. He was successfully treated with weekly infusions of fresh frozen plasma over 4 years, displaying normal blood pressure while only treated with an angiotensin converting enzyme (ACE) inhibitor, a steady level of haptoglobin, low-range proteinuria and normal creatinine clearance. By the end of the fourth year of treatment, he dramatically developed a relapse of hemolytic and uremic syndrome, displaying undetectable haptoglobin, nephrotic range proteinuria and progressive renal failure. Despite a ten-fold increase in the dosage of plasma infusion through daily plasma exchange, haptoglobin remained undetectable while circulating antigenic factor H levels reached 22-24% (normal values 65-140%). Three months following the biological onset of the relapse, a bilateral nephrectomy was performed owing to uncontrolled hypertension and rapidly progressive renal failure. The molecular mechanism of plasma resistance remained unclear while antifactor H antibodies were not detected in the plasma. We suggest that protracted administration of exogenous factor H might not be a long-term strategy in homozygous factor H deficiency.

Child↗
PubMed1977

Circulating thymic-hormone activity in congenital immunodeficiency.

Circulating thymic-hormone activity was assayed by measuring Thy 1-2 antigen induction on null lymphocytes from athymic mice incubated with human plasma or serum. Plasma from 19 normal children aged under 10 had inductive activity equivalent to 10-6-16-2 ng thymopoitin/ml. Plasma from 15 infants were severe combined immuno-deficiency, 2 of whom had appreciable immunoglobulin synthesis, and from 2 infants with DiGeorge syndrome had little or no inductive activity. Successful reconstitution with thymus or bone-marrow grafts and with red-cell infusions (if adenosine-deaminase deficiency is present) was followed by a rise in circulating thymic-hormone activity.

Agammaglobulinemia↗
PubMed2000

[Immunology in the medical practice. XXXI. Hereditary immune deficiencies: relationship between clinical symptoms and abnormalities of the immune system].

Severe congenital immunodeficiency diseases occur in approximately 1:10,000 newborns, in Western Europe and North America. They are characterised by recurrent infections, mostly caused by opportunistic micro-organisms, and autoimmune phenomena. In many patients the immune deficiency occurs as part of a syndrome. An immunological dysfunction may be caused by total absence, strong reduction or dysfunction of one or more cellular elements or of cell-associated or cellular secretion products. It is convenient to divide the immune system into a cellular compartment (with specific T and B cells, and non-specific natural killer cells and myelomonocytes), a compartment for cellular interactions (adhesion, costimulation and communication through cytokines) and an aspecific opsonization compartment. In cases of deficient humoral immunity treatment options are antimicrobial drugs and substitution therapy with immunoglobulins, in cases of phagocytic dysfunction the application of intracellularly active antibiotics and in cases of disturbances of cellular immunity allogeneic transplantation of haematopoietic stem cells and in the future perhaps the repair of the genetic defect by somatic gene therapy.

Anti-Bacterial Agents↗
PubMed1988

Spectrum of Di George syndrome in patients with truncus arteriosus: expanded Di George syndrome.

A study of 26 patients with truncus arteriosus showed a high prevalence of facial dysmorphism, aortic arch abnormalities, extracardiac malformations, and significant prenatal risk factors. There was little evidence of parathyroid or thymic abnormalities. However, there was laboratory evidence of immune deficiency, especially T-helper lymphocytes, and clinical evidence of predilection to infection. These findings suggest that patients with truncus arteriosus belong to the spectrum of the Di George syndrome.

Abnormalities, Multiple↗
PubMed1981

Deficiency of autologous mixed lymphocyte reactions and serum thymic factor level in Down's syndrome.

The immune competence of the T lymphocyte system was studied in 28 noninstitutionalized subjects with Down's syndrome (DS) and were compared with sex- and age-matched healthy controls. The ability of enriched T lymphocytes to respond to 3 different T cell stimulants revealed a selective impairment of T lymphocyte subset(s). Subjects with DS showed normal responsiveness in allogeneic mixed lymphocyte reactions, but their response to phytohemagglutinin and in autologous mixed lymphocyte reactions was severely impaired. Non-T cells from DS subjects stimulated equally well both normal and DS allogeneic T lymphocytes. The blood concentration of serum thymic factor in the majority of DS subjects was much lower than that found in age-matched healthy controls. These data support the hypothesis that a deficiency of the T-dependent regulatory system is an intrinsic feature of DS and confirm the precocious aging of the immune system in these subjects.

Adolescent↗
PubMed1987

Embryologic and other developmental considerations of thirty-eight possible variants of the DiGeorge anomaly.

The DiGeorge anomaly (DGA) represents a polytopic developmental field defect that can be caused by a number of different chromosomal, mendelian, toxic, or metabolic factors operating in early embryonic life. If the affected field is thought to be focused on either the fourth branchial arch or the third branchial pouch, with variable cephalad or caudad extension, 38 different combinations of malformations can include DiGeorge anomalies, 24 complete and 14 partial DGA, with the constraints that the field defect must be contiguous (involvement of more than one branchial arch or pouch requires involvement of all intervening arches or pouches) and complete (all derivatives of an affected branchial arch or pouch are deficient). The types and relative frequencies of abnormalities of structures other than the thymus and parathyroid glands in these possible "subsets" of DGA are discussed, and the need for more data on their occurrence in patients diagnosed as having DGA is emphasized.

Branchial Region↗
PubMed1984

Familial DiGeorge syndrome and associated partial monosomy of chromosome 22.

Partial monosomy of 22q due to an unbalanced 4;22 translocation was seen in a 2-month-old male with Type I truncus arteriosus, dysmorphic features, and T-cell abnormalities. The family history revealed a previous sib with Type I truncus arteriosus, thymic aplasia, and parathyroid hypoplasia noted on postmortem examination, consistent with DiGeorge syndrome. Evaluation of the asymptomatic mother of these two patients revealed partial T-cell deficiency and the same unbalanced translocation with deletion of proximal 22q11. These findings provide further evidence that some cases of complete or partial DiGeorge syndrome are associated with monosomy of the proximal long arm of chromosome 22, and they may explain many, if not all, familial cases of the syndrome.

Adult↗