Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Six patients with the acquired immune deficiency syndrome (AIDS) had exacerbations or recurrences of previously quiescent atopic disease when they developed immunodeficiency. Four developed a different atopic illness from that suffered previously. Atopic symptoms developed within three months after the patients developed AIDS or during prodromal illness. Two of the patients were treated with recombinant interferon gamma: both showed a striking improvement in symptoms and cellular immunity. These results indicate that cellular immunity, through interferon gamma, may have a role in regulating atopic disease.
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PMID:Atopic manifestations in the acquired immune deficiency syndrome: response to recombinant interferon gamma. 310 72

Fifty children with IgA deficiency were folllowed for 1 to 4 years from 1975 to 1978. Thirty-five had complete deficiency of serum IgA (less than 2.5 IU/ml) and 15 partial deficiency (serum IgA below the 10th centile for age). Patients with another associated immunodeficiency, such as ataxia-telangiectasia, were not included. Most children with complete deficiency of IgA had recurrent respiratory and/or gastrointestinal infections, about half with onset in the first year of life, while partial deficiency of IgA has probably little if any importance for anti-infectious immunity but is important in the pathogenesis of atopy. Atopic diseases were frequent in both groups. Chromosomal abnormalities were found in 2 patients: trisomy 21 in one and in the other a ring chromosome 18. No important defects in cellular immunity were detected but some isolated, borderline abnormalities were often present.
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PMID:Selective IgA deficiency: clinical and immunological evaluation of 50 pediatric patients. 644 75

The paper presents the analysis of T-cell immunity in asthmatics and bronchitis chronics. Immunodeficiency in one-third of them was characterized by thymic failure. T-cell insufficiency was registered in 50% and 37% of atopic and bacterial asthma patients, respectively, in 40% and 25% of those with chronic obstructive bronchitis and nonobstructive one, respectively. Imbalance of immunoregulatory cells in atopic asthma in 87% of relevant patients and in 37% of chronics with obstructive bronchitis was indicated by enhanced suppressor activity. In patients with bacterial asthma and chronic nonobstructive bronchitis (in 40% and 25%, respectively) helper-cell activity rose. A cAMP concentration proved reduced in helper cells and helper + suppressor subpopulations in bacterial asthma and obstructive bronchitis chronics, respectively. Atopic bronchial asthma was associated with low suppressor subpopulation. Variants of immunocorrection in the above conditions are presented for discussion.
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PMID:[The cellular immunity and cyclonucleotide level in the immunoregulatory cells of patients with bronchial asthma and chronic bronchitis]. 790 63

The incidence of positive circulating specific immunoglobulin E (IgE) antibodies to latex and evidence of clinical latex sensitivity appears to be increasing since its first description in 1979. Although heightened medical awareness may be a factor, exposure to latex products, particularly rubber gloves, has increased since the discovery of the human immunodeficiency virus (HIV). Atopic individuals are at greater risk of developing latex sensitivity. We identified seven children with atopic eczema who were known to have clinically significant latex allergy and examined the relationship of prior exposure to latex gloves. All children had significant serum levels of specific IgE to latex. Before developing clinical symptoms of latex allergy, all had been exposed to latex in the form of gloves during either inpatient or outpatient treatments of their skin. Exposure of atopic individuals to latex gloves could be a major risk factor for sensitization and could increase the incidence of serious reactions.
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PMID:Latex allergy in atopic children. 1035 33

Atopic is the most common of the dermatitides seen in infancy and childhood, but there are numerous other diseases that can mimic the skin findings. These include seborrheic dermatitis, immunodeficiency, and psoriasis in infancy; scabies, tinea corporis infection, perioral, nummular, contact, and molluscum dermatitis in childhood. It is sometimes extremely difficult to differentiate between ichthyosis and AD, and it is also important to differentiate AD from erythrodermic conditions including acrodermatitis enteropathica, biotin deficiency, and Netherton syndrome. A rare condition in children that may mimic AD is mycosis fungoides.
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PMID:The differential diagnosis of atopic dermatitis in childhood. 1666 89

Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by atopic manifestations and susceptibility to infections with extracellular bacteria and fungi, which frequently occur in the skin and lung. Atopic manifestations in HIES include extremely high serum IgE levels, eczema and eosinophilia. Most of the extracellular bacterial infections are associated with disproportionally milder inflammation than normal, which was originally described as having a 'cold abscess'. Non-immunological abnormalities are also observed in most patients with HIES, including a distinctive facial appearance, scoliosis, hyper-extensive joints and retained primary teeth. Recent studies have demonstrated that hypomorphic mutations in signal transducer and activator of transcription 3 result in the classical multisystem form of HIES, whereas a null mutation in tyrosine kinase 2 causes the autosomal recessive form of HIES that is associated with viral and mycobacterial infections. Analyses of cytokine responses in both types of HIES have revealed defects in signal transduction for multiple cytokines including IL-6 and IL-23, leading to impaired T(h)17 function. These results suggest that the defect in multiple cytokine signals is the molecular basis of the immunological and non-immunological abnormalities in HIES and that the susceptibility to infections with extracellular bacteria and fungi in HIES might be associated with the defect in T(h)17 cell differentiation.
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PMID:Defects in Jak-STAT-mediated cytokine signals cause hyper-IgE syndrome: lessons from a primary immunodeficiency. 1908 64

Hyper-IgE syndrome (HIES) is a primary immunodeficiency disorder characterized by atopic manifestations and susceptibility to infections with extracellular bacteria and fungi. Atopic manifestations include atopic dermatitis-like skin lesion and extremely high serum IgE levels. Most of the extracellular bacterial infections are caused by Staphylococcus aureus, which is associated with milder inflammation compared to normal. Recent studies have revealed that the most cases of the HIES are caused by dominant negative mutations in STAT3 gene. Cutaneous manifestations of HIES includes newborn rash, eczematoid dermatitis, cold abscesses, mucocutaneous candidiasis, and coarse texture of the facial skin. Impaired Th17 cell development due to the defective IL-6 signaling in T cells and impaired induced regulatory T (iTreg) cell generation due to defective IL-10 signaling in dendritic cells may, at least in part, account for the cutaneous pathology of HIES.
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PMID:Cutaneous manifestations of Hyper IgE syndrome. 2244 39