Primary Immunodeficiency Disorders in Iran: Update and New Insights from the Third Report of the National Registry

Aghamohammadi, Asghar; Mohammadinejad, Payam; Abolhassani, Hassan; Moeini, Babak; Movahedi, Masoud; Gharagozlou, Mohammad; Parvaneh, Nima; Zeiaee, Vaheid; Mirsaeedghazi, Bahram; Chavoushzadeh, Z.; Mahdaviani, Seyed Alireza; Mansouri, Mahboubeh; Yousefzadegan, Sedigheh; Sharifi, Bahareh; Zandieh, Fariborz; Hedayat, Ehsan; Nadjafi, A; Sherkat, Roya; Shakerian, Behzad; Shabestari, Mahnaz Sadeghi; Hosseini, Reza Farid; Jabbari‐Azad, Farahzad; Ahanchian, Hamid; Behmanesh, Fatemeh; Zandkarimi, Mohammad Reza; Shirkani, Afshin; Cheraghi, Taher; Fayezi, Abbas; Mohammadzadeh, Iraj; Amin, Reza; Aleyasin, Soheila; Moghtaderi, Mojgan; Ghaffari, Javad; Arshi, Saba; Javahertrash, Naser; Nabavi, Mohammad; Bemanian, Mohammad Hassan; Shafiei, Alireza; Kalantari, Najmoddin; Ahmadiafshar, Akefeh; Khazaei, Hossein Ali; Atarod, Lida; Rezaei, Nima

doi:10.1007/s10875-014-0001-z

Cited by 105 publications

(83 citation statements)

References 45 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Owayed and Al-Herz [31] found the rate of otitis media to be lower (59 of 202 patients, 29.2%). In the third report of the Iranian registry, we reported 46 cases in 731 patients (6.3%) in whom the first presentation of PID was otitis media [3].…”

Section: Otitis Mediamentioning

confidence: 96%

“…PIDs range from mild to severe forms of immunodeficiency such as severe combined immunodeficiency (SCID). Although the main clinical presentations are recurrent and chronic infections, patients also have an increased incidence of autoimmunity, lymphoproliferative disorders, and cancers [1][2][3][4]. The incidence and prevalence of PIDs are unknown, although they are estimated to affect between 1:10 000 and 1:600 individuals in over 40 countries [5].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

View full text Add to dashboard Cite

Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs. Key words: Primary immunodeficiency disorders. Pulmonary complications. Upper respiratory tract. Lower respiratory tract. ResumenLas inmunodeficiencias primarias (PIDs) son enfermedades causadas por uno o más defectos del sistema inmunológico. Estos pacientes presentan con frecuencia infecciones recidivantes y/o severas así como otro tipo de complicaciones. Las patologías respiratorias son la principal y más frecuente manifestación y complicación de las PIDs. Las complicaciones de estas patologías pulmonares constituyen una de las principales causas de morbimortalidad entre los pacientes que sufren PIDs. El diagnóstico temprano y el tratamiento adecuado pueden prevenir, o al menos retrasar, la aparición de las complicaciones respiratorias en estos pacientes. Dado que el espectro de las enfermedades pulmonares es muy amplio, hemos dividido estas complicaciones entre aquellas que afectan a las vías aéreas superiores (sinusitis, otitis media y angioedema laríngeo, etc.) y las que afectan a las vías aéreas bajas (neumonía, bronquitis, bronquiectasias, enfermedades pulmonares intersticiales, neumonía organizada, adenopatías pulmonares y neoplasias, hiperreactividad bronquial, disgenesia pulmonar y las debidas a los efectos secundarios del tratamiento instaurado). Este artículo revisa las manifestaciones respiratorias que se observan más frecuentemente en los pacientes con PIDs. Palabras clave: Inmunodeficiencias primarias. Complicaciones pulmonares. Vías respiratorias altas. Vías respiratorias bajas.

show abstract

Section: Otitis Mediamentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani

Abolhassani

et al. 2017

J Investig Allergol Clin Immunol

View full text Add to dashboard Cite

show abstract

“…This means a ratio of about one fifth from the total cases of PID who are at increased risk for serious adverse events of BCG vaccination at birth. In fact, more than one third of patients with PID should be considered susceptible to BCG because of susceptibility of infants and children with congenital phagocytic disorder (with prevalence of about 17.4%) to develop systemic BCG disease (18). According to this report systemic BCG disease was found in 17 cases (2.3 %) as the first presentation of PID, during the last seven years (2006 to 2013) (18).…”

Section: Discussionmentioning

confidence: 81%

“…In fact, more than one third of patients with PID should be considered susceptible to BCG because of susceptibility of infants and children with congenital phagocytic disorder (with prevalence of about 17.4%) to develop systemic BCG disease (18). According to this report systemic BCG disease was found in 17 cases (2.3 %) as the first presentation of PID, during the last seven years (2006 to 2013) (18). Considering the numerous recent reports of serious systemic adverse events of BCG vaccination (5,7,(10)(11)(12)(19)(20)(21) and also based on experience in our referral tertiary center, the probability of greater incidence of these side effects as a result of primary immune deficiency comes to mind.…”

Section: Discussionmentioning

confidence: 99%

Diffuse Hyper Pigmented Subcutaneous Nodules as a Primary Manifestation of Disseminated Bacillus Calmette-Guerin Disease in Young Infants

Tabatabaei

Amanati

Karimi

et al. 2015

Arch Pediatr Infect Dis

View full text Add to dashboard Cite

Introduction:There are increasing reports of serious adverse events of bacillus Calmette-Guerin (BCG) vaccination in infants with unrecognized primary immunodeficiency disorders (PIDs) in our country. Among these adverse events skin manifestations occur less frequently and are less noticed. Case Presentation: We report on an 11-months-old boy with prolonged fever and diffuse hyperpigmented subcutaneous nodules. Due to lymphopenia, oral thrush and severe adverse reaction to BCG vaccination, the possibility of primary immunodeficiency was considered for him and immunological investigations were done. Conclusions: Subcutaneous nodules in the absence of a local reaction at the site of BCG vaccination may be the sole manifestation of disseminated BCG disease.

show abstract

“…Due to the prevalence of consanguinous marriages, the incidence of autosomal-dominant PIDs is very high. Moreover, about 70 -90 percent of patients may die before they are diagnosed (5).…”

Section: Introductionmentioning

confidence: 99%

Estimating the Cost of Immunoglobulin Replacement Therapy in Primary Immunodeficiency Patients

et al. 2017

Self Cite

View full text Add to dashboard Cite

Background: Immunoglobulin (Ig) replacement therapy is used for treating a variety of primary immunodeficiency diseases (PIDs). Ig replacement therapy is the most important therapy for these diseases, since it protects the body against infections and reduces autoimmune disease symptoms. The purpose of this research was to estimate the cost of subcutaneous and intravenous Ig therapy in Iran. Methods: This study is carried out from the perspective of Iran's healthcare system and all the medical costs are calculated. Cost variables include personnel, equipment, and supplies. The data required for cost estimation are obtained from the records of children's Medical center of Tehran for 2015. Personnel and medical treatment costs are calculated based on relative value units. For SCIg, cost items are extracted from the literature. Results: The total cost of IVIg and SCIg for the health system in the first year of treatment is $1370 and $121 respectively. The results indicate that SCIg reduces costs and is the preferred treatment for PID patients. Conclusions: SCIg therapy significantly reduces the costs of the healthcare system compared to IVIg therapy, and this is enough economic justification for introduction of this treatment in the Iranian healthcare system. SCIg is also critical in reducing the direct costs of patients.

show abstract

Primary Immunodeficiency Disorders in Iran: Update and New Insights from the Third Report of the National Registry

Cited by 105 publications

References 45 publications

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Diffuse Hyper Pigmented Subcutaneous Nodules as a Primary Manifestation of Disseminated Bacillus Calmette-Guerin Disease in Young Infants

Estimating the Cost of Immunoglobulin Replacement Therapy in Primary Immunodeficiency Patients

Contact Info

Product

Resources

About