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At least 19 recordsLinked to original sources
PubMed2006

Hyperimmunoglobulin E syndrome (job syndrome) discovered in a patient following corrective spine surgery: case report and review of the literature.

STUDY DESIGN: A case report of the hyperimmunoglobulin E syndrome (Job syndrome) presenting in the context of late postoperative infection after corrective surgery for scoliosis. OBJECTIVE: To describe the clinical presentation and treatment of a patient with Job syndrome, and its implications for spine surgeons. SUMMARY OF BACKGROUND DATA: Job syndrome classically presents with a triad of increased serum immunoglobulin E, multiple abscesses, and pneumonia with pneumatocele formation. In recent years nonimmunologic manifestations have been described, including scoliosis, joint hypermobility, eosinophilia, and atopy. METHODS: A 15-year-old female presented with local swelling and fever 2 years after anterior lumbar discectomy and fusion with spinal instrumentation involving T11-L3 levels. Computerized tomography revealed paravertebral, psoas, and pulmonary abscesses. The implants were removed and antibiotic therapy instituted. Further investigation revealed features of the hyperimmunoglobulin E syndrome (Job syndrome). RESULTS: The patient's symptoms resolved, as did markers of inflammation. CONCLUSIONS: Job syndrome is a primary immunodeficiency often associated with scoliosis. Given the implications for surgical outcome in immunodeficient patients, the diagnosis should be considered and, blood tests instituted in patients with scoliosis with any of the associated history and physical findings of Job syndrome.

Adolescent↗
PubMed1992

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome).

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

Aspergillosis↗
PubMed1990

[The hyper IgE syndrome. Job's syndrome].

Since 1972, 150 cases of the hyper-IgE-syndrome have been reported. The clinical manifestations are rather homogeneous with recurrent subcutaneous infections and infections in the respiratory tract, mostly with juvenile onset. Laboratory tests show minor eosinophilia and elevated immunoglobulin E-levels are always seen, partly specific to Staphylococcus aureus. Also varying decreases of polymorphonuclear leucocyte-chemotactic response are seen. There is no specific treatment of the disorder, only treatment of current infections has proved valuable. A case history is presented.

Humans↗
PubMed1991

Use of recombinant human interferon gamma to enhance neutrophil chemotactic responses in Job syndrome of hyperimmunoglobulinemia E and recurrent infections.

Recombinant human interferon gamma enhances neutrophil respiratory burst and bactericidal activity in patients with chronic granulomatous disease. Mononuclear leukocytes of patients with the hyperimmunoglobulinemia E syndrome (Job syndrome) produce low or undetectable levels of this lymphokine. For these reasons we have restudied neutrophil chemotaxis in a group of our patients with the syndrome and determined the effect of recombinant human interferon gamma on the responses. Each of the patients had neutrophil chemotactic responses ranging from 22% to 55% of simultaneous control values (p less than 0.001). After incubation with interferon gamma, a significant improvement in chemotactic responsiveness was observed in the neutrophils of each of the patients (mean 301% of baseline chemotaxis; p less than 0.008). These data suggest the need for a double-blind, placebo-controlled trial of interferon gamma in a larger group of patients with the syndrome of hyperimmunoglobulinemia E and recurrent infections.

Adolescent↗
PubMed1996

[Job syndrome (hyper-IgE) and hypo-IgA. A rare association of immunodeficiencies].

Job' syndrome and IgA immunodeficiency are a rare dysfunction of the immune system. In this work, we reported a case of a young woman who had recurrent episodes of bacterial infections in the urinary tract and genital, generalized erythematous eczematous patches and stomatitis of oral mucosa and fever. During the hospitalization, laboratory data showed high immunoglobulin IgE and low IgA levels. The T-lymphocyte presented a reduction of CD8+ cells. Tests of granulocyte function have showed a global deficit in the in vitro and in vivo chemotaxis. The correlation between these two clinic conditions is not completely clarified but it is possible to hypothesize that CD8+ lymphocytes produce an inhibition factor of chemotaxis. Job' syndrome is characterized by a selective reduction of CD8+ cells subpopulation which have an immunoregulatory function on the production of IgE by plasmacells. In the ipoIgA, an intrinsic inability of B-IgA cells to proliferate and to differentiate produce a defect in the IgA production. In these two clinic disorders there is an effective dysfunction of immune system. It is possible to hypothesize that an effective defect of CD8+ cells and an immaturity of B-cells may coexist in our patient. That justifies an abnormal production of Ig and a defect in granulocyte chemotaxis.

Adult↗
PubMed1983

[The concept of Job syndrome].

The so-called Job's syndrome belongs to the group of diseases designated "chronic granulomatous disease", characterized by recurrent suppurative infections of the skin, eczema, nail dystrophy, conjunctivitis, lymphadenitis and pulmonary infections. Leucocyte function is defective; the nature of the defect has not yet been elucidated. High immunoglobulin E levels are characteristic. The historical background to this syndrome is outlined.

Child↗
PubMed1991

[Hyperimmunoglobulinemia E (Job) syndrome].

A patient with hyperimmunoglobulin E (Job's) syndrome is presented. The authors review the clinical and immunological characteristics of the disease and sum up the different explanations for the pathogenesis of the syndrome.

Anti-Bacterial Agents↗
PubMed1980

Job's syndrome.

Job's syndrome is characterized by the clinical features of fair skin, red hair, recurrent cold staphylococcal skin abscesses with concurrent other bacterial infections and skin lesions. This case report chronicles a classic presentation of Job's syndrome. A brief review of the otolaryngological presentations of immune deficiencies is presented. Other than its relationship to the manifestations of other immunological defects, Job's syndrome is of interest to the otolaryngologist because of the head and neck infections represented in its clinical expression. Familiarity with phenotypic and laboratory manifestations of such diseases will enable earlier recognition and treatment of this syndrome.

Abscess↗
PubMed1995

Fungal pulmonary abscess in an adult secondary to hyperimmunoglobulin E (Job's) syndrome.

Job's syndrome is characterized by recurring bacterial infections of the skin and sinopulmonary tract. Laboratory evaluation reveals consistent elevation of circulating immunoglobulin E levels. The syndrome has been reported as a rare cause of bacterial pulmonary abscess and pneumatocele formation in childhood; here we present a case of cavitating fungal abscess in an adult with Job's syndrome.

Adult↗
PubMed2002

Meconium thorax: A case of Bochdalek hernia and cecal perforation in a neonate with Job's syndrome.

Meconium peritonitis most often is associated with congenital intestinal obstruction and meconium ileus. Uncommonly, other etiologies are identified. Hyperimmunoglobulin E syndrome (Job's syndrome) is a rare genetic disorder that is characterized by recurrent staphylococcal respiratory and skin infections in addition to elevated serum IgE levels. There have been 2 previously reported cases of intestinal complications associated with Job's syndrome. The current case is the third such case and is the first report of meconium peritonitis in a patient with hyperimmunoglobulin E syndrome. The patient presented with a meconium thorax as a result of a concurrent congenital diaphragmatic hernia.

Cecum↗
PubMed2004

Recurrent Staphylococcus aureus chalazia in hyperimmunoglobulinemia E (Job's) syndrome.

PURPOSE: To report a case of recurrent Staphylococcus aureus chalazia in a patient with hyperimmunoglobulinemia E syndrome (Job's Syndrome). DESIGN: Case report. METHODS: Three separate surgical incisions and curettages of multiple, recurrent chalazia of the right upper eyelid were performed over a course of 3 months. Cultures and pathologic specimens were obtained. Postoperative treatment consisted of oral erythromycin and amoxicillin/clavulonate, topical tobramycin/dexamethasone ointment, and warm compresses. RESULTS: Pathology of the tarsus confirmed the diagnosis of multiple chalazia. Cultures of the chalazia contents were positive for Staphylococcus aureus. No further recurrence was observed following the third surgical procedure over a 3-month follow-up period. CONCLUSION: Characteristic Staphylococcus aureus skin infections in immunodeficient patients with hyperimmunoglobulinemia E syndrome can involve the eyelids and may be recurrent despite appropriate medical and surgical therapy.

Adolescent↗
PubMed1999

[Hyperglobulinemia E syndrome with recurrent infections (Job's syndrome)].

INTRODUCTION: To provide definition and nosology of hyperimmunoglobulinemia E previously termed Job's syndrome or Buckleys' syndrome in the literature. Generalization of IgE dosages makes it more commonly diagnosed, though sometimes diagnosis may not be accurate. CURRENT KNOWLEDGE AND KEY POINTS: The clinical picture is dominated by recurring cutaneous and visceral bacterial infections, particularly infections due to staphylococci, and severe generalized eczema, which may be associated with osteoporosis. Biochemistry includes a high level of total and specific IgE and immunological abnormalities dominated by frequent absence of immune antibodies to staphylococci and disorders of neutrophil chemotaxis. Current pathogenic studies show dysregulation of TH1 and TH2 lymphocytes in favor of TH2 activation with depressed TH1 activation, leading to an imbalance in cytokine synthesis. FUTURE PROSPECTS AND PROJECTS: New therapeutical possibilities with alpha and gamma interferon.

Antiviral Agents↗