Surveillance of Primary Immunodeficiency Disorders in Mofid Children's Hospital: A 10-year retrospective experience

Babaie, Delara; Atashpar, Sara; Chavoshzadeh, Zahra; Armin, Shahnaz; Mesdaghi, Mehrnaz; Fahimzad, Alireza; Tabatabaei, Sedigheh Rafiee; Ghanaie, Roxana Mansour; Karimi, Abdollah; Pourmoshtagh, Hassan; Mansouri, Mahboobeh; Darougar, Sepideh

doi:10.5812/pedinfect.61642

Cited by 2 publications

(3 citation statements)

References 12 publications

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“…Regarding small studies conducted around the world, the median delay in diagnosis of PID in children hospitalized in the Mofid Children's Hospital in Iran was 20 months [19], compared with 19 months in Korea [20] and 2.17 years in Mexico [21]. Given the large delay in diagnosing PID in the SR, it is necessary to carry out activities aimed at reducing this delay.…”

Section: оригинальные исследованияmentioning

confidence: 99%

“…The most common infectious complications in patients with PID were pneumonias (43. Ranking of clinical syndromes by predominance of infections is noted in the German register (2019) [15] and the South African register (ASID) [22], as well as in the registers of other countries and regions [15,19,20,22,23]. However, due to the differences in classifications among different years, direct comparisons of prevalence rates are difficult to implement [15].…”

Section: оригинальные исследованияmentioning

confidence: 99%

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Congenital defects in the immune system in the Stavropol region

Barycheva¹,

Khachirova²,

Голубева³

et al. 2021

Medical news of the North Caucasus

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The study analyzed 88 patients with congenital immunodeficiencies registered in the Stavropol Region (SR) during a 10year period (2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016)(2017)(2018)(2019). The results established that the prevalence of primary immunodeficiencies (PID) in the SR was 3.1 per 100,000 population. Children affected by PID predominated (74.7 %) in the cohort of living patients. Twenty-seven nosological forms of PID were verified in the SR register. The most common was defective humoral component of the immune system (44.3 %), followed by combined immune disorders with syndromic manifestations (15.9 %) and phagocytosis defects (10.2 %). The molecular genetic diagnosis was confirmed in 38.6 % of patients. Hematopoietic cell transplantation was performed in 11.4 % of patients. Intravenous immunoglobulin medications were administered in 20.2 % of patients. Mortality in the PID cohort within the SR register was 6.9 %.

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Section: оригинальные исследованияmentioning

confidence: 99%

Section: оригинальные исследованияmentioning

confidence: 99%

Congenital defects in the immune system in the Stavropol region

Barycheva¹,

Khachirova²,

Голубева³

et al. 2021

Medical news of the North Caucasus

View full text Add to dashboard Cite

show abstract

“…In a study on 32 patients (20 males and 12 females) with PID referred to Mofid Children’s Hospital over a 10-year period, CGD was the most frequent PID seen (22%) [35]. The infections observed in the CGD patients included pneumonia, lymphadenitis, inguinal and perianal abscesses, lung abscess, BCGosis, peritonitis, mouth ulcers, sinusitis, mastoiditis, and respiratory infections.…”

Section: Clinical Evidencementioning

confidence: 99%

Common Infections and Target Organs Associated with Chronic Granulomatous Disease in Iran

Mortaz

Azempour

Mansouri

et al. 2019

Int Arch Allergy Immunol

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Recurrent severe bacterial and fungal infections are characteristic features of the rare genetic immunodeficiency disorder chronic granulomatous disease (CGD). The disease usually manifests within the first years of life with an incidence of 1 in approximately 200,000 live births. The incidence is higher in Iran and Morocco where it reaches 1.5 per 100,000 live births. Mutations have been described in the 5 subunits of NADPH oxidase, mostly in gp91^phox and p47^phox, with fewer mutations reported in p67^phox, p22^phox, and p40^phox. These mutations cause loss of superoxide production in phagocytic cells. CYBB, the gene encoding the large gp91^phox subunit of the transmembrane component cytochrome b₅₅₈ of the NADPH oxidase complex, is localized on the X-chromosome. Genetic defects in CYBB are responsible for the disease in the majority of male CGD patients. CGD is associated with the development of granulomatous reactions in the skin, lungs, bones, and lymph nodes, and chronic infections may be seen in the liver, gastrointestinal tract, brain, and eyes. There is usually a history of repeated infections, including inflammation of the lymph glands, skin infections, and pneumonia. There may also be a persistent runny nose, inflammation of the skin, and inflammation of the mucous membranes of the mouth. Gastrointestinal problems can also occur, including diarrhea, abdominal pain, and perianal abscesses. Infection of the bones, brain abscesses, obstruction of the genitourinary tract and/or gastrointestinal tract due to the formation of granulomatous tissue, and delayed growth are also symptomatic of CGD. The prevention of infectious complications in patients with CGD involves targeted prophylaxis against opportunistic microorganisms such as Staphylococcus aureus, Klebsiella spp., Salmonella spp. and Aspergillus spp. In this review, we provide an update on organ involvement and the association with specific isolated microorganisms in CGD patients.

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Surveillance of Primary Immunodeficiency Disorders in Mofid Children's Hospital: A 10-year retrospective experience

Cited by 2 publications

References 12 publications

Congenital defects in the immune system in the Stavropol region

Congenital defects in the immune system in the Stavropol region

Common Infections and Target Organs Associated with Chronic Granulomatous Disease in Iran

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