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Prof. Dr. Agung Pranoto, dr.,MSc.Sp.PD-KEMD

Fakultas Kedokteran / Departemen Ilmu Penyakit Dalam

Tim Peneliti :

  1. Ari Baskoro
  2. Chairul Effendi
  3. Agung Pranoto
  4. Djazuly Chalidyanto
  5. Ratna Konthen --> Peneliti Utama

Tahun : 2009

Halaman Naskah : 1 halaman

Sumber Dana : Deprt - SMF Ilmu Penyakit Dalam FK Unair - RSUD Dr. Soetomo

Besaran Dana : 7000000

SK. Penetapan :

Publikasi : Seminar : Seminar Current Management of Allergy Imunology and Infectious Disease From Biomolecular to Clinical Practice

Kategori Penelitian : Kesehatan

Posting : 25-01-2012

Visitor : 5196

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Title :


Author : Prof. Dr. Agung Pranoto, dr.,MSc.Sp.PD-KEMD

Year : 2009

Abstact :




 Ratna Konthen Ari Baskaro, Chairul Effendi, Putu Gede Konthen,

Agung Pranoto, Djoko Santoso


1. Resident Internal Medicine Department, School of Medicine Airlangga University

2,3,4. Allergy & Clinical Immunology Division, Internal Medicine Department School of Medicine

5. Endocrinology Metabolic and Diabetes Division, Internal Medicine Department School of Medicine

6. Nephrology and Hypertension Division, Internal Medicine Department School of Medicine Internal Medicine Department School of Medicine Airtangga University Dr. Soetomo Teaching Hospital



Hyper‑IgE syndrome (HIES) is a complex primary immunodeficiency disorder characterized by spectrum of abnormalities related to the immune system, bones, connective tissue and teeth. The cause ofv HIES is not known. The disease is also known as Job Syndrome because skin boils, a hallmark of the syndrome, are reminiscent of the biblical character Job, who was smitten by Satan "with sore boils from the sole of his foot unto his crown. "HIES was initially defined as a triad of clinical problems involving skin boils, severse episodes of pneumonia and very high serum IgE levels.

There are a number of clinical features of HIES that relate to underlying abnormalities in the immune system. These include eczema, abscesses, pneumonia, infections with a fungus Galled candida, IgE values in the blood serum that are extremely high and high numbers of a type of white blood cell known as eosinophils.

In the absence of a known gene or definitive test for HIES, the diagnosis must be made on a combination of clinical and laboratory findings. An elevated level of serum IgE alone is not sufficient to make the diagnosis since patients certain conditions such as severe allergic skin rashes occasionally have IgE levels in the HIES range without having HIES. An IgE of over 2,000 lUlml (normal adult value is less than 100 IU/ml) has been used as a cutoff level for HIES when other features including boils and peneumonia are present. In infants, in whom normal IgE levels are very low, an IgE of 10 times the age appropriates level is a reasonable guide for HIES. It should be noted that in some adults with HIES, IgE may decrease and even become normal, The presence of the other clinical features involving the skeleton and teeth can be very useful in diagnosing HIES, and even high IgE levels are not specific, since these can be found in other conditions. Many studies have focused on the immune asolects of HIES, such as the migration of neutrophils toward damaged or infected tissue. However, ni specific immune defect has been found consosistently in all patients with HIES.

Keyword : Syndrome on A Diabetes Mellitus Patient,

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