Title of article
Job’s syndrome with an atypical presentation
Author/Authors
Tasneem, Abbas Ali Sindh Institute of Urology and Transplantation (SIUT) - Department of Hepatogastroenterology, Pakistan , Sarfaraz, Sabahat Sindh Institute of Urology and Transplantation (SIUT) - Department of Molecular Biology and Immunology, Pakistan , Hassan, Syed Mujahid Sindh Institute of Urology and Transplantation (SIUT) - Department of Hepatogastroentrology, Pakistan , Luck, Nasir Hassan Sindh Institute of Urology and Transplantation (SIUT) - Department of Hepatogastroentrology, Pakistan , Anis, Sabiha Sindh Institute of Urology and Transplantation (SIUT) - Department of Molecular Biology and Immunology, Pakistan , Abbas, Zaigham Sindh Institute of Urology and Transplantation (SIUT) - Sindh Institute of Urology and Transplantation (SIUT) - Department of Hepatogastroentrology, Pakistan
From page
1427
To page
1429
Abstract
Hyper Immunoglobulin E syndrome also called Job s or Buckley Syndrome is a rare primary immunodeficiency disease characterized by elevated serum IgE levels ( 2000 IU/ml), recurrent infections and eosinophilia. Other features include coarse facies and non-immunologic abnormalities of the dentition, bones, vasculature and connective tissues. We are reporting a case of a twenty four years old male with coarse facies who presented with severe pallor and upper gastrointestinal bleeding. Investigations revealed markedly elevated serum IgE levels (11,800 IU/ml), severe anaemia, esophageoduodenal erosions, Helicobacter pylori (H. pylori) gastritis and oro-esophageal candidiasis.
Keywords
Hyper IgE syndrome , H.pylori infection , Job s syndrome
Journal title
Journal of the Pakistan Medical Association (Centre) JPMA
Journal title
Journal of the Pakistan Medical Association (Centre) JPMA
Record number
2653203
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