X‐Linked Agammaglobulinemia: A Survey of 33 Iranian Patients

Moin, Mostafa; Aghamohammadi, Asghar; Farhoudi, A.; Pourpak, Zahra; Rezaei, Nima; Movahedi, Masoud; Gharagozlou, Mohammad; Ghazi, Bahram Mir Saeid; Ali, Zahed; Abolmaali, Kamran; Mahmoudi, Maryam; Emami, Leila; Bashashati, Mohammad

doi:10.1081/imm-120027687

Cited by 55 publications

(50 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In one study, we demonstrated that 81.1% of XLA patients manifested acute otitis media. Although this was the second most common infectious disease among Iranian XLA patients, chronic otitis media was the most common complication among chronic manifestations [35]. We found a lower rate of otitis media in CVID patients with hypogammaglobulinemia than in those with agammaglobulinemia [17,36].…”

Section: Otitis Mediamentioning

confidence: 43%

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: 43%

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

“…In another study, done by Busse et al (14), among 50 patients with CVID, 42 patients (84%) had at least one episode of pneumonia before diagnosis, and by using computed tomographic scanning, 18 out of these 42 patients showed bronchiectasis. Infections are less severe in CVID patients than in XLA patients (40).…”

Section: Discussionmentioning

confidence: 99%

“…This delay markedly decreased over time, perhaps due to better diagnostic tools or methods; an increase in the knowledge of our physicians about primary immunodeficiency disorders, especially CVID; and the beginning of the registry program in Iran. In another study by Moin et al of X-linked agammaglobulinemia patients in Iran, diagnostic delay also decreased over time (40).…”

Section: Discussionmentioning

confidence: 99%

Clinical and Immunological Features of 65 Iranian Patients with Common Variable Immunodeficiency

Aghamohammadi

Farhoudi

Moin

et al. 2005

Clin Vaccine Immunol

Self Cite

112

View full text Add to dashboard Cite

Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by hypogammaglobulinemia and recurrent bacterial infections. The records of 65 patients with CVID (37 males and 28 females) in the age range of 24 to 537 months were reviewed. By the year 2003, 11 patients had died and seven patients could not be located. The total follow-up period was 221 patient-years. The median diagnostic delay (time between onset and diagnosis) in our patient group was 60 months. At the time of diagnosis, the baseline serum immunoglobulin G (IgG), IgM, and IgA levels were below the level normal for the patients' age; the medians for this group were 120, 10, and 0 mg/dl, respectively. All of the patients presented with infectious diseases at the time of onset, the most common of which were otitis media, diarrhea, pneumonia, and sinusitis. Acute and recurrent infections were also found in almost all of the patients, particularly involving respiratory and gastrointestinal systems. The most common infections, before diagnosis and during follow-up, were pneumonia, acute diarrhea, acute sinusitis, and otitis media. CVID should be considered in any patient with a history of recurrent infections and decreased levels of all serum immunoglobulin isotypes.

show abstract

“…However, for subgroups of patients, commonly encountered infectious organisms also include Staphylococcus aureus, Pseudomonas spp., Mycoplasma spp., Enterobacteriaceae, Campylobacter, Giardia, and enteroviruses. In rare cases of CVID and XLA, severe infections with opportunistic pathogens such as Pneumocystis jirovecii and other fungi can occur (32,74,90,103,141).…”

Section: Infections and Primary Antibody Deficienciesmentioning

confidence: 99%

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

2009

View full text Add to dashboard Cite

SUMMARY Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.

show abstract

X‐Linked Agammaglobulinemia: A Survey of 33 Iranian Patients

Cited by 55 publications

References 27 publications

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Clinical and Immunological Features of 65 Iranian Patients with Common Variable Immunodeficiency

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

Contact Info

Product

Resources

About