Genital ve Karın Bölge Tutulumuyla Giden Çocukluk Çağında Tekrarlayan Anjioödem: C1 İnhibitör İşlevsel Eksikliğine Bağlı Herediter Anjioödem Tip 2 Hastalığı

Hereditary angioedema is a rare disorder characterized by recurrent angioedema attacks due to C1 inhibitor antigen or functional deficiency. Here, two cases with recurrent swelling on extremities, genital organs and face that were later diagnosed with C1 inhibitor functional deficiency (hereditary angioedema type 2) were presented. The first patient was an 8-year-old boy complaining of hand and foot swelling recurring once a year for the last 4 years. He was more frequently brought to outpatient pediatric clinics because of his recurring complaints in the last 5 months. In prodromal period, he had erythema marginatum-like rash and sometimes described abdominal pain with swelling. The second patient was an 11-year-old girl complaining of abdominal pain and facial swelling recurring in a couple of months for the last 8 years. Her grandfather, father and uncle had the same angioedema. In their laboratory evaluation, complement C4 levels were both found as low at 0.1). Although C1 inhibitor antigen levels were both in reference range, its function tests were detected as low at 31% and 43% (>70%), respectively. Therefore, in cases with pediatric recurrent angioedema without urticaria, hereditary angioedema should be considered. After screened with C4 level, if required, both C1 inhibitor antigen and function tests are evaluated.Herediter anjioödem C1 inhibitor antijen ya da işlevsel eksikliğine bağlı tekrarlayan anjiyoödem ataklarıyla bilinen nadir bir bozukluktur. Burada ekstremitelerde, genital organlarda ve yüzde tekrarlayan şişlikleri nedeniyle C1 inhibitor işlevsel eksikliği (herediter anjiyoödem tip 2) tanısı konulan iki olgu sunulmuştur. Sekiz yaşındaki erkek çocuk el ve ayaklarında şişliklerin yılda bir kere son 4 yıldır tekrarladığından şikayet etmekteydi. Son beş ayda tekrarlayan şikayetlerinden dolayı daha sık pediyatri polikliniğine başvurmuştur. Olgu prodrom döneminde eritema marginatum-benzeri döküntü ve şişlikleri esnasında karın ağrısından şikayet etmekteydi. On bir yaşında kız çocuk olan ikinci olgu son 8 yıldır birkaç ayda bir tekrarlayan karın ağrısı ve yüzde şişmeden şikayet ediyordu. Dede, baba ve amcada benzer anjiyoödem mevcuttu. Olguların laboratuvar değerlendirmesinde, kompleman C4 düzeyi 0,1) g/L olarak düşüktü. Her ikisinde C1 inhibitor antijen seviyesi referans aralığı içinde olmasına rağmen fonksiyon testi sırasıyla %31 ve %43 (>%70) olarak düşük bulundu. Dolayısıyla, çocukluk çağı tekrarlayan ürtikersiz anjiyoödemlerinde, herediter anjiyoödem düşünülmelidir. Kompleman C4 düzeyi ile tarama sonrasında, gerekirse, hem C1 inhibitor antijen ve hem de fonksiyon testleriyle değerlendirilmelidir

Cell-Mediated Cytotoxicity Assays

Cell-mediated cytotoxicity measurements can be divided in two methods, depending on whether radioactive material is used or not. Natural Killer cell-mediated cytotoxicity is routinely measured with a short-term assay by labeling the target cells with radioactive chromium (51Cr). The advantages of this method are (1) highly sensitivity, (2) easy execution, (3) low spontaneous release, and (4) nontoxic markers. The disadvantages include short half-life of the label and handling and disposal of radioactive supplies. Many attempts have been made to adapt this cytotoxicity assay to abolish radioactivity while maintaining its high sensitivity. Consequently, various nonradioactive methods have been developed. One of these methods utilizing the release of enzymes (LDH) as a result of cytolysis or membrane dyes (PKH-26) is usually less sensitive than radioactive assays. MTT-based colorimetric/enzymatic assays are highly sensitive and easy to use but unfortunately works only with adherent tumor cell targets. Fluorescent dyes such as carboxy-fluorescein diacetate can easily accumulate in the cytoplasm of effector or target cells. After cytotoxicity, the release of the dyes into the supernatant or their retention in target cells is calculated. However, spontaneous release of these dyes can be quite high, causing false positive results leading to a decreased sensitivity and restricting their use in short-term assays. More recently, flow cytometric methods using fluorescent monoclonal antibodies such as anti-CD56 for effector and anti-CD33 for target cells have been defined

Subcutaneous Immunoglobulin Replacement Therapy Experience with Intravenous Preparation

Conclusion: In this article, we describe our experience with 6 primary immunodeficiency patients of our clinic in whom we successfully used intravenous immunoglobulin preparation via the subcutaneous route

Use of Stem Cell Therapy in COVID-19

Severe acute respiratory syndrome coronavirus 2 is known to cause the new coronavirus disease 2019 (COVID-19), in which many organs and systems such as the lung, heart, and immune system can be severely affected. Currently, the treatment process is generally based on supportive and palliative care. Some potential drugs are being tested for treatment. At this point, perhaps a promising treatment method for many diseases in the future seems to be stem cell therapy in recent times. Stem cell therapy in COVID-19 may be a treatment method that can play an important role, especially in resistant and severe cases. In this review, we summarized the potential mechanisms of stem cell therapy and developments in this treat ment modality

Development of Hypersensitivity Reactions after Using Different Oral Iron Preparations

Gastrointestinal side effects after using oral iron preparations have been usually observed, but skin reactions and allergic angioedema due to hypersensitivity have been rarely reported in the literature. We delineated 4 patients who were referred to us and who had shown hypersensitivity reactions following oral iron use, and their response to alternate oral iron preparations was evaluated. Initially prick and intradermal skin tests, and then oral provocations, were algorithmically performed in 4 patients to diagnose allergy to oral iron salts. Two of 4 cases showed hypersensitivity reactions after oral provocation. We were able to prevent the possible allergic reaction, which probably depends on IgE-mediated hypersensitivity, by simply changing the initial oral iron preparation to alternate one