Infection outcomes in patients with common variable immunodeficiency disorders: Relationship to immunoglobulin therapy over 22 years

Lucas, Mary; Lee, Martin; Lortan, Jenny; López‐Granados, Eduardo; Misbah, Siraj; Chapel, Helen

doi:10.1016/j.jaci.2010.02.040

Cited by 370 publications

(321 citation statements)

References 29 publications

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“…Advantages of SCIG include greater independence, no need for vascular access; decreased systemic side effects and more stable serum IgG levels (8)(9)(10). Disadvantages include requiring frequent dosing and limitation of the volume that can be administered (11)(12)(13).…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous Immunoglobulin Replacement Therapy Experience with Intravenous Preparation

Özdemir¹

2018

Asthma Allergy Immunol

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ÖZAmaç: İntravenöz immünoglobulin preparatları primer immünyetmezlik tedavisinde uzun yıllardır kullanılmaktadır. Son zamanlarda, subkutanöz yolla immünoglobulin verilimi ön plana çıkmıştır. Subkutan yolla verilen immünoglobulin daha stabil serum immünoglobulin düzeyleri sağlar ve sistemik yan etki sıklığı intravenöz yola göre daha düşüktür. Hastanın kendisinin ya da ebeveyninin evde bu metotu uygulayabilmesi yaşam kalitesini artırır. Subkutanöz verilim için üretilen immünoglobulin preparatları Avrupa ve ABD'de 2006'dan beri mevcuttur. Subkutan immünoglobulin preparatlarının Türkiye'de bulunmamasına rağmen, yakın zamanda %10 konsantrasyonundaki üç değişik intravevöz ürününün kullanım talimatına subkutan kullanım yolu eklenmiştir. Amacımız, bu intravenöz immünoglobulin preparatlarından birini subkutan yolla kullanmak ve bu uygulamanın avantaj ve dezavantajlarını ortaya koymaktır. Gereç ve Yöntem: Kliniğimizden yapılacak işlem anlatılmış ve onamları alınmış altı primer immünyetmezlik hastası seçilmiştir. Bu hastalar, son bir kaç yıldır intravenöz immünoglobulin ile tedavi edilmektedir. Bu hastaların aylık immünoglobulin dozları hesaplanıp yaklaşık olarak haftalık dozlara bölündü. İlk bir kaç uygulama hastalar ve/veya aileleri işlemi öğrenene kadar tıbbi gözetim altında hastanede gerçekleştirilmiştir. Bulgular: Küçük hasta örneğimizde daha az yan etki, daha iyi yaşam kalitesi ve daha az enfeksiyon oranları ile sonuçlar umut verici olmuştur. Sonuç: Makalede, intravenöz immünoglobulin preparatını başarıyle subkutan yolla kullandığımız kliniğimizin 6 primer immünyetmezlik hastası üzerindeki deneyimimizi anlatmaktayız . ABSTRACTObjective: Intravenous immunoglobulin preparations have been used in the treatment of primary immunodeficiency for many years. Recently, immunoglobulin administration via the subcutaneous route has become popular. Subcutaneously administered immunoglobulin provides more stable serum immunoglobulin levels and has a lower incidence of systemic adverse effects than the intravenous route. This method increases the quality of the patient's life by self administration or parental administration at home. Immunoglobulin preparations designed for subcutaneous administration have been available in Europe and the US since 2006. Although subcutaneous immunoglobulin preparations are not available in Turkey, the subcutaneous administration route has recently been included in the instructions of the three intravenous products with 10% concentration. Our aim was to use one of these intravenous immunoglobulin preparations via the subcutaneous route and describe its advantages and disadvantages. Materials and Methods: Six primary immunodeficiency patients were selected from our clinic. The procedure was described and informed consent obtained. They had been treated with intravenous immunoglobulin for the last few years. Their monthly immunoglobulin dosage was calculated and roughly divided into weekly doses. The first few administrations took place under medical supervision at the hospital until the patients and/...

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Section: Discussionmentioning

confidence: 99%

Subcutaneous Immunoglobulin Replacement Therapy Experience with Intravenous Preparation

Özdemir¹

2018

Asthma Allergy Immunol

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“…이전 연구 결과에 따르면 혈청 IgG 치 가 높을 경우 감염 횟수가 줄어든다고 하였으나 [19][20][21][22] 일부 연구에서는 IgG의 농도나 감염 횟수와는 상관관계가 없다 고 보고하였다 [23][24][25][26] . 다만 혈중 IgG 농도가 400 mg/dL 미 만으로 저하된 경우 폐렴과 같은 질병의 이환율이 증가된 다고 한다 15,18,19,27) . Orange 등 …”

Section: 진단검사unclassified

Clinical Manifestations and Treatment in Korean Patients with X-Linked Agammaglobulinemia

Cho

Kim

2017

Pediatr Infect Vaccine

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Purpose: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the Bruton's tyrosine kinase (Btk) gene. The aim of this study was to investigate the clinical manifestations, molecular features, and treatment status of XLA in Korean patients at Seoul National University Children's Hospital. Methods: Fourteen Korean boys with XLA showing serum agammaglobulinemia, non-detectable to less than 2% of peripheral B-cells, and mutation of the Btk gene were enrolled. We observed the clinical features, laboratory findings, status of treatment, and complications in these XLA patients. Results: All XLA patients had a history of recurrent bacterial infections before diagnosis, and 20% of them had a neutropenia. Of the XLA patients 35.7% had a family history of XLA and 75% of their mothers were carriers. Btk gene analysis showed variable gene mutations in Xq22 including 9 amino acid substitutions, 3 frameshifts, 1 premature stop codon, and 1 splice defect. After intravenous immunoglobulin replacement therapy, infection episodes decreased, but complications such as bronchiectasis and chronic sinusitis remained. Conclusions: In patients less than 4 years of age with recurrent infection, analysis of serum gamma globulin levels and the Btk gene are recommended for the early diagnosis of XLA and for the appropriate prevention of recurrent infection.

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“…The main advantage of this method is that high Ig doses can be administered via each infusion, and doses are usually given every three to four weeks (8). On the other hand, infusions must be done in a hospital or at home by a nurse or a trained individual.…”

Section: Introductionmentioning

confidence: 99%

Estimating the Cost of Immunoglobulin Replacement Therapy in Primary Immunodeficiency Patients

et al. 2017

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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.

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Infection outcomes in patients with common variable immunodeficiency disorders: Relationship to immunoglobulin therapy over 22 years

Cited by 370 publications

References 29 publications

Subcutaneous Immunoglobulin Replacement Therapy Experience with Intravenous Preparation

Subcutaneous Immunoglobulin Replacement Therapy Experience with Intravenous Preparation

Clinical Manifestations and Treatment in Korean Patients with X-Linked Agammaglobulinemia

Estimating the Cost of Immunoglobulin Replacement Therapy in Primary Immunodeficiency Patients

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