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Aghamohammadi, Asghar; Moein, M; Farhoudi, A.; Pourpak, Zahra; Rezaei, Nima; Abolmaali, Kamran; Movahedi, Masoud; Gharagozlou, Mohammad; Ghazi, Bahram Mir Saeid; Mahmoudi, Maryam; Mansouri, Davood; Arshi, Saba; Trash, Naser Javaher; Akbari, Hedayatallah; Sherkat, Roya; Hosayni, Reza Farid; Hashemzadeh, Ahmad; Mohammadzadeh, Iraj; Amin, Reza; Kashef, Sara; Alborzi, Alireza; Karimi, Abdollah; Khazaei, Hossein Ali

doi:10.1023/a:1020660416865

Cited by 75 publications

(14 citation statements)

References 7 publications

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“…The Iranian Primary Immunodeficiency Registry reported 440 patients with PID seen over a period of 20 years. There was a predominance of antibody production and phagocytic defects over other PID as compared with other registries, and only 3 patients with complement deficiencies were identified 12. In other Asian countries, like Taiwan, over a 20-year period, 37 patients were diagnosed with PID.…”

mentioning

confidence: 84%

Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

Al‐Tamemi

Elnour

Dennison

2012

World Allergy Organization Journal

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Background:Primary immunodeficiency diseases (PIDs) are considered rare but are generally assumed to be more common in Middle Eastern countries. The prevalence and characteristics of PIDs are unknown in Oman.Methods:Sultan Qaboos University Hospital is the national referral center for PID in Oman during the study period. Patients were diagnosed and classified according to the clinical and laboratory criteria of PID reported by the International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. A registry was created, and patient data were analyzed between July 2005 and July 2010.Results:Over a 5-year period, there were a total of 90 patients, with an estimated prevalence of 4.5 cases per 100,000. The most common form of immunodeficiency was phagocyte disorders (42%), mainly chronic granulomatous disease, followed by predominantly antibody disorders (18%), other well-defined PID syndromes (13%), and combined immunodeficiencies (12%). The median age of onset of symptoms was 9 months. The median age of diagnosis was 24 months. Consanguinity was present in 81% of patients. The most common infectious presentation was pneumonia (42%), followed by deep abscess (27%) and BCGosis (12%). A total of 25% of patients required intravenous immunoglobulins treatment, 4% required gamma interferon therapy, and 11% underwent bone marrow transplantation. Of all PID patients, 90% survived treatment, whereas 10% did not.Conclusions:The estimated minimum prevalence of PID in Oman is 4.5 cases per 100,000, with a predominance of phagocyte disorders. Consanguinity is a significant factor; pneumonia and deep abscesses were the main infectious presentations. The overall survival rate was 90%. Strategies are needed to improve the care for PID patients and to increase the awareness among parents and physicians.

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confidence: 84%

Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

Al‐Tamemi

Elnour

Dennison

2012

World Allergy Organization Journal

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“…Most studies are geographically limited and based on survey data or record review of diagnosed and/or registered cases. 17–21 The number of undiagnosed cases is unknown because a comprehensive population-based screening process for defined primary immunodeficiency has not been developed anywhere in the world.…”

Section: Epidemiologymentioning

confidence: 99%

International Consensus Document (ICON): Common Variable Immunodeficiency Disorders

Bonilla

Barlan

Chapel

et al. 2016

The Journal of Allergy and Clinical Immunology: In Practice

687

803

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The International Collaboration in Asthma, Allergy and Immunology initiated an international coalition among the American Academy of Allergy, Asthma & Immunology; the European Academy of Allergy and Clinical Immunology; the World Allergy Organization; and the American College of Allergy, Asthma & Immunology on common variable immunodeficiency. An author group was formed and then divided into individual committees. Within the committee, teams of authors were subgrouped to generate content for specific sections of the document. Content was derived from literature searches, relevant published guidelines, and clinical experience. After a draft of the document was assembled, it was collectively reviewed and revised by the authors. Where evidence was lacking or conflicting, the information presented represents the consensus expert opinion of the group. The full document was then independently reviewed by 5 international experts in the field, none of whom was among the authors of the original. The comments of these reviewers were incorporated before submission for publication.

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“…In Europe, a similar registry-based effort to define the natural history of patients with CD40 ligand (CD40L) deficiency revealed a significant burden from liver disease and unusual malignancies that had not been previously appreciated (4). These early efforts spurred the expansion of registries for collections of PIDD patients on every continent (5–10). Collection of clinical, laboratory, and outcome data provide a real-world health survey of patients with rare PIDD disorders and form the foundation for evolving outcomes-based research (11).…”

Section: Introductionmentioning

confidence: 99%

USIDNET: A Strategy to Build a Community of Clinical Immunologists

et al. 2014

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Objectives Information about patients with primary immune deficiencies can be scarce because of the rarity of the disorders. Individual centers rarely have sufficient patients to educate trainees and garner collective wisdom. Registries for many diseases have proven their worth by providing essential information on disease spectrum, treatments and natural history. This study describes the construction and use of a registry for patients with primary immune deficiencies and other efforts to improve knowledge and care for affected patients and their families. Methods Registry demographics and data were extracted using proprietary reporting tools. Educational efforts and cell repository data were collected from centralized source material. Results The USIDNET Registry contains 3459 patients, with common variable immune deficiency being the most represented. Pilot studies identified strengths and weaknesses of the data. Visiting Professor and Visiting Scholar Programs have been successful, encouraging trainees at all levels to pursue a career in Immunology. Conclusions USIDNET’s comprehensive and integrated approach provides resources that strengthen the field of primary immune deficiencies, as shown by utilization by 312 distinct sites or individuals. The reach of USIDNET’s efforts is extended through the educational resources.

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Cited by 75 publications

References 7 publications

Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

Primary Immunodeficiency Diseases in Oman: Five Years' Experience at Sultan Qaboos University Hospital

International Consensus Document (ICON): Common Variable Immunodeficiency Disorders

USIDNET: A Strategy to Build a Community of Clinical Immunologists

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