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At least 91 records · Page 5Linked to original sources
PubMed1983

The hyperimmunoglobulin E recurrent-infection (Job's) syndrome. A review of the NIH experience and the literature.

The hyperimmunoglobulin E recurrent-infection syndrome (HIE) entails a disorder of recurrent bacterial infections of the skin and sinopulmonary tract commencing in infancy or early childhood in the presence of serum levels of IgE which are at least 10 times normal (greater than 2,000 IU/ml). Variable concomitants of HIE are coarse facies, chronic eczematoid rashes, cold cutaneous abscesses, mild eosinophilia, mucocutaneous candidiasis, and a neutrophil chemotactic defect. The bacteria which commonly infect these patients are Staphylococcus aureus and Haemophilus influenzae although Streptococcus pneumoniae and enteric gram-negative rods are seen in some cases. Other than pneumonias, deep-seated infections are unusual, although osteomyelitis, arthritis, and visceral abscesses are seen. Bacteremia and sepsis are rare. Therapy should involve prolonged intravenous antibiotics and early surgery to treat infections which usually seem deceptively benign. HIE patients' neutrophils display a variable chemotactic defect, and their mononuclear cells variably produce an inhibitor of neutrophil chemotaxis. The production of the inhibitor correlates with the in vitro chemotactic defect. The basis of the propensity for recurrent infections is still speculative, and the further study of this syndrome should add new dimensions to our understanding of host defenses against bacterial invaders.

Abscess↗
PubMed1990

Spinal anaesthesia in a child with Job's syndrome, pneumatoceles and empyema.

We present a case of acute bowel obstruction in an immunocompromised child, who also had lobar pneumonia and a giant unilateral pneumatocele. She was successfully managed with subarachnoid anaesthesia for exploratory laparotomy to relieve a colonic obstruction. This proved to be a safe alternative to general anaesthesia with tracheal intubation in this patient and should be considered in infants and children in selected cases whenever a contraindication to general anaesthesia exists.

Anesthesia, Spinal↗
PubMed1980

[Job's syndrome--a case report (author's transl)].

A 4-year old boy with chronic eczema since early infancy had been admitted thrice to the hospital because of recurrent staphylococcal infections including furunculosis, lymphadenitis, pneumonia and pyothorax. Exhaustive immunologic studies revealed normal humoral, cell-mediated and non-specific immunities except extremely high serum IgE level and dysfunctions of granulocytes including decreased Fc and complement receptors, lowered chemotactic response (around the lower range of normal), slightly impaired intracellular killing of staphylococci and possibly impaired reactions to candida and BCG. In spite of long-term antimicrobial prophylaxis, cold abscesses continued to appear and the eczematous skin lesions waxed and waned.

Child, Preschool↗
PubMed1987

Evolution of the hyperimmunoglobulin E and recurrent infection (HIE, JOB's) syndrome in a young girl.

The hyperimmunoglobulin E and recurrent infection syndrome is difficult to diagnose in children with markedly elevated IgE and recurrent superficial Staphylococcus aureus infections who have not presented with a severe infection. The patient, the child of a woman with HIE, had elevated cord blood IgE. In early infancy, she had cutaneous colonization with S. aureus followed by frank impetiginous lesions. Anti-S. aureus IgE was easily detected with a highly specific ELISA assay at 2 years of age (2 years before her presentation with a S. aureus subcutaneous abscess). Thus, the measurement of anti-S. aureus IgE by this technique may be a useful laboratory test for the diagnosis of HIE before the appearance of a severe infection.

Abscess↗
PubMed1987

Elevated urinary histamine in the hyperimmunoglobulin E and recurrent infection (Job's) syndrome: association with eczematoid dermatitis and not with infection.

To test the hypothesis that IgE-mediated release of histamine may be, in part, responsible for the abnormal inflammatory response observed in the hyperimmunoglobulin E (HIE) and recurrent infection syndrome, urine and plasma histamine levels were measured. Twenty-four-hour urinary histamine concentrations from 15 patients with HIE (23.7 +/- 6.9 micrograms/24 hr) were significantly elevated (p less than 0.001) compared to a large historical control population (10.5 +/- 0.7 micrograms/24 hr; n = 97). However, urinary histamine levels in HIE were much less abnormal (p less than 0.01) than in five patients with biopsy-proven systemic mastocytosis (159 +/- 62 micrograms/24 hr) and were not significantly elevated when levels were compared to 13 concurrently studied normal subjects (10.1 +/- 1.7 micrograms/24 hr) and nine patients with chronic granulomatous disease (8.1 +/- 1.2 micrograms/24 hr). Overall, there was no clear relationship between urine histamine values and the presence of infection as well as no significant correlation between urine histamine and total IgE or anti-Staphylococcus aureus IgE. However, urine histamine levels in a subgroup of six patients with HIE with chronic eczematoid dermatitis (42.4 +/- 12.5 micrograms/24 hr) were elevated compared with values from the historical control subjects (p less than 0.001), the concurrent control subjects (p less than 0.01), the patients with chronic granulomatous disease (p less than 0.01), and five patients with HIE who did not have skin manifestations (4.6 +/- 1.1 micrograms/24 hr; p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Dermatitis, Contact↗
PubMed1998

The face of Job.

We evaluated facial features in 9 patients from 7 kindreds with Job syndrome. Consistent features included prominent forehead with deep-set eyes, increased width of the nose, a full lower lip, and thickening of the nose and ears. The mean alar width (Z score = +3.9) and outer canthal distance (Z score = +2.2) were significantly increased. A recognizable face of Job syndrome exists.

Adolescent↗
PubMed2005

Hyper-IgE syndrome is not associated with defects in several candidate toll-like receptor pathway genes.

The genetic basis of hyper-IgE syndrome (HIES), also known as Job syndrome, a primary immunodeficiency associated with recurrent skin and pulmonary infections, is unknown. We hypothesized that HIES is due to a defect in the Toll-like receptor signaling pathway. We used a whole blood cytokine assay to compare inflammatory responses to stimulation with specific Toll-like receptor (TLR) pathway agonists in four individuals with HIES and nine healthy controls. Production of tumor necrosis factor-alpha, interleukin (IL)-1beta, IL-6, and IL-12 was not impaired in response to stimulation with lipopolysaccharide, peptidoglycan, zymosan, lipoteichoic acid, Staphylococcus aureus, Escherichia coli, or Streptococcus pneumoniae. Interferon (IFN)-gamma was reduced in HIES subjects in response to each of these stimuli. We sequenced several candidate genes from the TLR pathway in HIES individuals to determine whether any mutations were associated with this syndrome. No novel mutations or polymorphisms were found in the coding regions of TLR1, TLR2, TLR6, MyD88, or TRAF6. In summary, although HIES individuals had an IFN-gamma secretion defect, they also produced normal levels of several TLR-regulated proinflammatory cytokines. No unique mutations or polymorphisms were observed in several candidate genes from the TLR pathway. Our studies do not support a role for a defective TLR response in HIES individuals.

Adaptor Proteins, Signal Transducing↗
PubMed1989

Regulation of immunoglobulin production in hyperimmunoglobulin E recurrent-infection syndrome by interferon gamma.

The hyperimmunoglobulin E recurrent-infection (Job) syndrome (HIE) is a congenital disorder characterized by high serum IgE, chronic eczematoid dermatitis, and recurrent infections. We examined the effect of interferon gamma (IFN-gamma) on excessive IgE production in HIE patients. Spontaneous in vitro production of IgE by peripheral blood mononuclear cells from HIE patients was elevated compared to normal individuals and correlated with serum IgE. In 9 of 13 patients, IgE production by peripheral blood mononuclear cells was inhibited by 50% by IFN-gamma at 100-1000 units/ml, whereas inhibition by IFN-gamma at 10(4) units/ml ranged from 67 to 93% for these 9 patients. IFN-gamma also inhibited IgG1, IgG3, and IgG4 production by B lymphocytes without inhibiting IgG2 production. IFN-gamma was administered subcutaneously to 5 HIE patients. After 2 weeks of treatment with IFN-gamma (0.05 mg/m2) at three doses per week given on alternate days, peripheral blood mononuclear cells from all 5 HIE patients decreased spontaneous in vitro IgE production (27-62% decrease) with no change in IgG and IgM. One patient had a 58% decrease in serum IgE and another patient had a 50% decrease in serum IgE after the IFN-gamma was increased to 0.1 mg/m2 for three doses per week for a month. In both patients, serum IgE returned to pre-IFN-gamma-challenge levels 1-3 months after completion of treatment, and in vivo IFN-gamma did not affect serum IgG and IgM, although serum IgG4 decreased with changes in serum IgE. Our studies demonstrate that IFN-gamma can regulate production of IgE and some IgG subclasses in humans.

Antibody Formation↗
PubMed1970

Job's syndrome.

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Bible↗