{"id":28671,"date":"2024-06-29T14:33:13","date_gmt":"2024-06-29T11:33:13","guid":{"rendered":"https:\/\/www.klimikdergisi.org\/?p=28671"},"modified":"2024-06-29T15:33:25","modified_gmt":"2024-06-29T12:33:25","slug":"primer-immun-yetmezliklerde-gelisen-infeksiyonlar","status":"publish","type":"post","link":"https:\/\/www.klimikdergisi.org\/tr\/2024\/06\/29\/primer-immun-yetmezliklerde-gelisen-infeksiyonlar\/","title":{"rendered":"Primer \u0130mm\u00fcn Yetmezlik Hastalar\u0131nda \u0130mm\u00fcnoglobulin Replasman Tedavisi Alt\u0131nda Geli\u015fen \u0130nfeksiyonlar: 5 Y\u0131ll\u0131k Deneyim"},"content":{"rendered":"<h2 class=\"p1\">G\u0130R\u0130\u015e<\/h2>\n<p class=\"p2\">Daha \u00f6nce primer imm\u00fcn yetmezlik (P\u0130Y) olarak adland\u0131r\u0131lan do\u011fu\u015ftan ba\u011f\u0131\u015f\u0131kl\u0131k hatalar\u0131 (\u201cinborn errors of immunity\u201d &#8211; IEI), do\u011fal ve\/veya adaptif imm\u00fcn sistem bile\u015fenlerini etkileyen kal\u0131tsal bozukluklard\u0131r; infeksiyonlara duyarl\u0131l\u0131k ve imm\u00fcn disreg\u00fclasyon ile karakterize olabilirler (1). Uluslararas\u0131 \u0130mm\u00fcnoloji Dernekleri Birli\u011fi (International Union of Immunological Societies-<span class=\"Apple-converted-space\">\u00a0 <\/span>IUIS) IEI Komitesi yapt\u0131\u011f\u0131 son g\u00fcncelleme ile P\u0130Y\u2019leri fenotiplerine g\u00f6re 10 s\u0131n\u0131fa ay\u0131rm\u0131\u015ft\u0131r (2). Primer imm\u00fcn yetmezli\u011fin temsil etti\u011fi hastal\u0131k grubu heterojen olmakla birlikte, klinik olarak en belirgin bulgu artm\u0131\u015f infeksiyonlard\u0131r. Bu infeksiyonlar\u0131n en \u00f6nemli \u00f6zellikleri ise tekrarlamalar\u0131, tedaviye iyi cevap vermemeleri, a\u011f\u0131r ve komplike seyretmeleridir (3,4). Bunun sonucu olarak da hastalar artm\u0131\u015f morbidite ve mortalite ile kar\u015f\u0131 kar\u015f\u0131ya kalmaktad\u0131r (1,3,4). Bu nedenle infeksiyon hastal\u0131klar\u0131na kar\u015f\u0131 koruyucu tedbirlerin uygulanmas\u0131 hayati \u00f6nem ta\u015f\u0131maktad\u0131r. Bu koruyucu tedbirlerin ba\u015fl\u0131calar\u0131; imm\u00fcnoglobulin replasman tedavisi (\u0130GRT), antimikrobiyal ila\u00e7 profilaksisi, etkilenen imm\u00fcn sistem bile\u015fenine \u00f6zel bireyselle\u015ftirilmi\u015f bir a\u015f\u0131lama program\u0131, beslenme deste\u011fi ve hijyen kurallar\u0131na uyumdur (5,6).<\/p>\n<p class=\"p3\">Yeti\u015fkin P\u0130Y hasta grubunda en yayg\u0131n semptomatik fenotip, primer antikor eksikli\u011fi (PAE) alt grubunda yer alan yayg\u0131n de\u011fi\u015fken imm\u00fcn yetmezlik (common variable immunodeficiency &#8211; CVID)\u2019tir; bu hasta grubunda <i>Haemophilus influenzae <\/i>tip B veya <i>Streptococcus pneumoniae<\/i> ba\u015fta olmak \u00fczere kaps\u00fcll\u00fc bakteriler tekrarlayan sinopulmoner infeksiyonlar\u0131n ba\u015fl\u0131ca etkenleridir (7-9). S\u00f6z konusu hasta grubunda merkezi sinir sistemi, sindirim sistemi, cilt ve yumu\u015fak doku infeksiyonlar\u0131 da g\u00f6r\u00fclebilir. Her ne kadar bu hastal\u0131k antikor eksikli\u011fi \u00f6n planda olan bir bozukluk olmakla birlikte de\u011fi\u015fen derecelerde T h\u00fccre disfonksiyonu CVID\u2019e e\u015flik etmekte ve bu hastalar\u0131 viral ve bazen de f\u0131rsat\u00e7\u0131 infeksiyonlara duyarl\u0131 hale getirebilmektedir (2). Ayr\u0131ca ge\u00e7 bas\u0327lang\u0131\u00e7l\u0131 kombine imm\u00fcn yetmezlik (late-onset combined immune deficiency &#8211; LOCID) CVID\u2019nin yak\u0131n zamanda tan\u0131mlanm\u0131s\u0327 bir varyant\u0131 olup f\u0131rsat\u00e7\u0131 infeksiyonlar (<i>Pneumocystis jiroveci, Cytomegalovirus- CMV<\/i>) ve\/veya d\u00fcs\u0327\u00fck CD4<sup>+ <\/sup>T lenfosit say\u0131lar\u0131 (&lt;200 h\u00fccre\/\u03bc) ile ba\u015fvuran yetis\u0327kin hastalarla kar\u015f\u0131m\u0131za \u00e7\u0131kar (10). \u0130nfeksiyona yol a\u00e7an ajan\u0131n t\u00fcr\u00fc, P\u0130Y\u2019den etkilenen imm\u00fcn sistem bile\u015feni konusunda klinisyenlere yol g\u00f6sterici olabilmektedir (2,3).<\/p>\n<p class=\"p3\">\u0130mm\u00fcnoglobulin replasman tedavisi P\u0130Y\u2019li hastalarda infeksiyon s\u0131kl\u0131\u011f\u0131n\u0131 belirgin olarak azaltmakla birlikte baz\u0131 hastalar kal\u0131c\u0131 akci\u011fer hasar\u0131na neden olabilen solunum yolu infeksiyonlar\u0131n\u0131 ge\u00e7irmeye devam ederler (7,11,12). Bu nedenle ideal \u00e7ukur imm\u00fcnoglobulin G (IgG) seviyesinin hastalar\u0131n klinik \u00f6zelliklerine (infeksiyon say\u0131s\u0131, \u015fiddeti, bron\u015fektazi varl\u0131\u011f\u0131, vb. fakt\u00f6rler) g\u00f6re bireyselle\u015ftirilerek takip edilmesi \u00f6nerilmektedir (13). Primer imm\u00fcn yetmezli\u011fin erken tan\u0131 ve tedavisi ile bir\u00e7ok komplikasyonun yan\u0131 s\u0131ra \u00f6zellikle<b> <\/b>infeksiyonlar ve infeksiyonlar\u0131n etkiledi\u011fi organlarda meydana gelen kal\u0131c\u0131 hasarlar \u00f6nlenebilir (6). Bu nedenle bu \u00f6zel hasta grubunun erken tan\u0131 alabilmesi i\u00e7in fark\u0131ndal\u0131\u011f\u0131n art\u0131r\u0131lmas\u0131 olduk\u00e7a \u00f6nemlidir. Ara\u015ft\u0131rmam\u0131z\u0131n amac\u0131, P\u0130Y hasta grubumuzdaki tan\u0131 \u00f6ncesi infeksiyon s\u0131kl\u0131\u011f\u0131n\u0131 ve tuttu\u011fu b\u00f6lgeleri tan\u0131mlamak ve tedavi alt\u0131ndaki de\u011fi\u015fimleri saptamakt\u0131r.<\/p>\n<h2 class=\"p4\">Y\u00d6NTEMLER<\/h2>\n<p class=\"p2\">\u00c7al\u0131\u015fmam\u0131z<b><i> <\/i><\/b>2017-2022 y\u0131llar\u0131 aras\u0131nda Eri\u015fkin \u0130mm\u00fcnoloji ve Alerji Hastal\u0131klar\u0131 Klini\u011fi\u2019nde \u0130GRT alan P\u0130Y tan\u0131l\u0131, 18 ya\u015f\u0131ndan b\u00fcy\u00fck hastalar ile ger\u00e7ekle\u015ftirilmi\u015f olup demografik \u00f6zellikler, laboratuvar ve g\u00f6r\u00fcnt\u00fcleme y\u00f6ntemi sonu\u00e7lar\u0131, k\u00fclt\u00fcr sonu\u00e7lar\u0131 ile klinik bulgular\u0131 i\u00e7eren veriler elektronik kay\u0131t sisteminden ve hasta dosyalar\u0131ndan retrospektif olarak al\u0131nd\u0131. Tespit edilen 51 hastan\u0131n alt\u0131s\u0131n\u0131n verileri eksikti; \u00e7al\u0131\u015fma 45 hastan\u0131n verileri ile ger\u00e7ekle\u015ftirildi.<\/p>\n<p class=\"p3\">\u00c7al\u0131\u015fma kapsam\u0131ndaki hastalar, Avrupa Ba\u011f\u0131\u015f\u0131kl\u0131k Yetmezli\u011fi Derne\u011fi (European Society for Immunodeficiencies &#8211; ESID) taraf\u0131ndan belirlenen kriterlere g\u00f6re tan\u0131 alm\u0131\u015f olup IUIS-IEI Komitesi taraf\u0131ndan \u00f6nerilen fenotiplemeye uygun olarak s\u0131n\u0131fland\u0131r\u0131lm\u0131\u015ft\u0131r (2,14). Klini\u011fimizde tedavi alan P\u0130Y tan\u0131l\u0131 hastalar, rutin olarak 3-4 haftada bir tedavi almak i\u00e7in ba\u015fvurduklar\u0131 planl\u0131 vizitlerde veya farkl\u0131 \u015fikayetlerle ba\u015fvurduklar\u0131 plans\u0131z vizitlerde infeksiyon ve infeksiyon d\u0131\u015f\u0131 komorbiditeler a\u00e7\u0131s\u0131ndan detayl\u0131 olarak de\u011ferlendirilmi\u015ftir.<\/p>\n<p class=\"p3\">Hastalar\u0131n tan\u0131 almadan \u00f6nce ya\u015fad\u0131klar\u0131 f\u0131rsat\u00e7\u0131 ve\/veya inat\u00e7\u0131 infeksiyonlar, infeksiyon b\u00f6lgeleri ve say\u0131lar\u0131 ile tan\u0131 sonras\u0131ndaki takip s\u00fcre\u00e7lerinde ald\u0131klar\u0131 tedaviler (antibiyotik profilaksisi, IgG uygulamas\u0131n\u0131n yolu\/dozu, a\u015f\u0131lama durumu); tedavi s\u00fcrecindeki infeksiyonlar, infeksiyon b\u00f6lgeleri, infeksiyon say\u0131lar\u0131, hastaneye yat\u0131\u015f durumlar\u0131 ve infeksiyon sonras\u0131 antimikrobiyal profilaksi tedavilerinde yap\u0131lan de\u011fi\u015fikliklere ili\u015fkin t\u00fcm veriler analiz edildi.<\/p>\n<p class=\"p3\">D\u00fcnya Tabipler Birli\u011fi Helsinki Bildirgesi\u2019ne uygun olarak y\u00fcr\u00fct\u00fclen \u00e7al\u0131\u015fma i\u00e7in Ankara \u00dcniversitesi T\u0131p Fak\u00fcltesi \u0130nsan Ara\u015ft\u0131rmalar\u0131 Etik Kurulu\u2019ndan 30 Aral\u0131k 2022 tarih ve \u013011-684-22 karar numaras\u0131yla onay al\u0131nd\u0131.<\/p>\n<h3 class=\"p5\">\u0130statistiksel Analiz<\/h3>\n<p class=\"p2\">Verilerin analizi, SPSS (Statistical Package for the Social Sciences) versiyon 27.0 program\u0131 (IBM Corp., Armonk, NY, ABD) ile yap\u0131ld\u0131. Tan\u0131mlay\u0131c\u0131 analizler kategorik de\u011fi\u015fkenlerde s\u0131kl\u0131k ve y\u00fczde, s\u00fcrekli de\u011fi\u015fkenlerde ise ortalama\u00b1standart sapma (SS) veya ortanca (minimum-maksimum) de\u011ferleri ile sunuldu. S\u00fcrekli de\u011fi\u015fkenlerin normal da\u011f\u0131l\u0131ma uygunlu\u011fu g\u00f6rsel (histogram ve olas\u0131l\u0131k grafikleri) ve analitik y\u00f6ntemlerle (Kolmogorov-Smirnov\/Shapiro-Wilk testleri) incelendi. Kategorik de\u011fi\u015fkenlerde ba\u011f\u0131ms\u0131z grup kar\u015f\u0131la\u015ft\u0131rmalar\u0131 \u03c7<sup>2<\/sup> veya Fisher testleri kullan\u0131larak yap\u0131ld\u0131. S\u00fcrekli de\u011fi\u015fkenler ise Mann-Whitney U veya Student t testi ile analiz edildi. Tan\u0131 \u00f6ncesi ve tedavi sonras\u0131 infeksiyon oranlar\u0131 McNemar, y\u0131ll\u0131k infeksiyon say\u0131lar\u0131 ise Wilcoxon testi kullan\u0131larak kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131. \u0130statistiksel anlaml\u0131l\u0131k i\u00e7in tip-1 hata d\u00fczeyi %5 olarak belirlendi.<\/p>\n<h2 class=\"p6\">BULGULAR<\/h2>\n<h3 class=\"p7\">Hasta \u00d6zellikleri<\/h3>\n<div id=\"attachment_28959\" style=\"width: 1078px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28959\" class=\"wp-image-28959 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1.png\" alt=\"\" width=\"1068\" height=\"1080\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1.png 1068w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1-257x260.png 257w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1-534x540.png 534w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil1-768x777.png 768w\" sizes=\"auto, (max-width: 1068px) 100vw, 1068px\" \/><\/a><p id=\"caption-attachment-28959\" class=\"wp-caption-text\"><strong>\u015eekil 1.<\/strong> Do\u011fu\u015ftan Ba\u011f\u0131\u015f\u0131kl\u0131k Hatalar\u0131 Olan Hastalar\u0131m\u0131zda Klinik S\u0131n\u0131fland\u0131rma<\/p><\/div>\n<p class=\"p2\">\u00c7al\u0131\u015fmaya dahil edilen toplam 45 hastan\u0131n [25 (%55.6) erkek ve 20 (%44.4) kad\u0131n] ya\u015f ortalamas\u0131 38.4\u00b111.6 y\u0131l, ortalama v\u00fccut k\u00fctle indeksi (VK\u0130) 22.8\u00b14.7 kg\/m<sup>2<span class=\"Apple-converted-space\">\u00a0 <\/span><\/sup>idi. En s\u0131k kar\u015f\u0131la\u015f\u0131lan imm\u00fcn yetmezlik tan\u0131s\u0131 (%88.8) primer antikor eksikli\u011fi idi ve 5 (%11.1) hastada genetik tan\u0131 \u00e7al\u0131\u015f\u0131lm\u0131\u015ft\u0131 (\u015eekil 1). Hastalar\u0131n ortalama tan\u0131 ya\u015f\u0131 26.8\u00b114.8 y\u0131l olup ortanca tan\u0131 gecikme s\u00fcresi 6 (0-33) y\u0131l idi. Bron\u015fektazi<span class=\"Apple-converted-space\">\u00a0 <\/span>tan\u0131s\u0131 alan hasta say\u0131s\u0131 20 (%44.4) olup; 40 (%88.9) hastaya intraven\u00f6z imm\u00fcnoglobulin (\u0130V\u0130G), 5 (%11.1) hastaya ise subkutan imm\u00fcnglobulin tedavisi uygulanmaktayd\u0131. En s\u0131k kullan\u0131lan antimikrobiyal profilaksinin trimetoprim-sulfametoksazol (TMP-SMX) (%66.7) oldu\u011fu g\u00f6r\u00fcld\u00fc. Tablo 1\u2019de hastalar\u0131n demografik, klinik \u00f6zellikleri ve tan\u0131 s\u0131ras\u0131nda ortanca IgG, IgA ve IgM d\u00fczeyleri, lenfosit say\u0131lar\u0131 ve periferik lenfosit alt gruplar\u0131 ile tedavi sonras\u0131 ortanca \u00e7ukur IgG d\u00fczeyleri ve antimikrobiyal profilaksileri ayr\u0131nt\u0131l\u0131 olarak verildi. Toplam 41 hastan\u0131n a\u015f\u0131lama verilerine eri\u015filmi\u015f olup hastalar\u0131n %78\u2019inin koronavirus hastal\u0131\u011f\u0131 2019<i> <\/i>(COVID-19) a\u015f\u0131s\u0131, %46.3\u2019\u00fc pn\u00f6mokok a\u015f\u0131s\u0131, %41.5\u2019i ise mevsimsel inaktif influenza a\u015f\u0131s\u0131 (\u0130\u0130A) oldu\u011fu tespit edildi. \u0130mm\u00fcnoglobulin replasman tedavisi alan 17 (%37.8) hasta infeksiyon nedeniyle hastanede yatm\u0131\u015f, 2 (%4.4) hasta infeksiyon nedeniyle kaybedilmi\u015f ve 14 (%31.1) hastan\u0131n tedavisinde infeksiyon sonras\u0131 de\u011fi\u015fiklik yap\u0131lm\u0131\u015ft\u0131.<\/p>\n<div id=\"attachment_28967\" style=\"width: 1083px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28967\" class=\"wp-image-28967 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1.png\" alt=\"\" width=\"1073\" height=\"2495\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1.png 1073w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1-112x260.png 112w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1-232x540.png 232w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo1-1-768x1786.png 768w\" sizes=\"auto, (max-width: 1073px) 100vw, 1073px\" \/><\/a><p id=\"caption-attachment-28967\" class=\"wp-caption-text\"><strong>Tablo 1.<\/strong> Hastalar\u0131n Demografik ve Klinik \u00d6zellikleri (N=45)<\/p><\/div>\n<div id=\"attachment_28969\" style=\"width: 1083px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28969\" class=\"wp-image-28969 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1.png\" alt=\"\" width=\"1073\" height=\"920\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1.png 1073w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1-303x260.png 303w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1-630x540.png 630w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo2-1-768x658.png 768w\" sizes=\"auto, (max-width: 1073px) 100vw, 1073px\" \/><\/a><p id=\"caption-attachment-28969\" class=\"wp-caption-text\"><strong>Tablo 2.<\/strong> Tedavi \u00d6ncesi ve Tedavi Alt\u0131nda Klinik Parametrelerin Kar\u015f\u0131la\u015ft\u0131r\u0131lmas\u0131 (N=45)<\/p><\/div>\n<div id=\"attachment_28972\" style=\"width: 2197px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28972\" class=\"wp-image-28972 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3.png\" alt=\"\" width=\"2187\" height=\"1436\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3.png 2187w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3-390x256.png 390w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3-810x532.png 810w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Tablo3-768x504.png 768w\" sizes=\"auto, (max-width: 2187px) 100vw, 2187px\" \/><\/a><p id=\"caption-attachment-28972\" class=\"wp-caption-text\"><strong>Tablo 3.<\/strong> Tedavi Alt\u0131nda \u0130nfeksiyon Nedenli Hastane Yat\u0131\u015f\u0131 Olan ve Olmayan Hastalar\u0131n \u00d6zelliklerinin Kar\u015f\u0131la\u015ft\u0131r\u0131lmas\u0131 (N=45)<\/p><\/div>\n<div id=\"attachment_28978\" style=\"width: 1079px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28978\" class=\"wp-image-28978 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2.png\" alt=\"\" width=\"1069\" height=\"1068\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2.png 1069w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2-260x260.png 260w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2-541x540.png 541w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil2-768x767.png 768w\" sizes=\"auto, (max-width: 1069px) 100vw, 1069px\" \/><\/a><p id=\"caption-attachment-28978\" class=\"wp-caption-text\"><strong>\u015eekil 2.<\/strong> Tedavi Alt\u0131nda \u0130nfeksiyon Nedenli Hastane Yat\u0131\u015f\u0131<\/p><\/div>\n<div id=\"attachment_28980\" style=\"width: 1078px\" class=\"wp-caption alignright\"><a href=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3.png\"><img loading=\"lazy\" decoding=\"async\" aria-describedby=\"caption-attachment-28980\" class=\"wp-image-28980 size-full\" src=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3.png\" alt=\"\" width=\"1068\" height=\"1100\" srcset=\"https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3.png 1068w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3-252x260.png 252w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3-524x540.png 524w, https:\/\/www.klimikdergisi.org\/wp-content\/uploads\/2024\/06\/KD.C37.S2_4779_Sekil3-768x791.png 768w\" sizes=\"auto, (max-width: 1068px) 100vw, 1068px\" \/><\/a><p id=\"caption-attachment-28980\" class=\"wp-caption-text\"><strong>\u015eekil 3.<\/strong>Bron\u015fektazi Geli\u015fen ve Geli\u015fmeyen Hastalarda \u0130mm\u00fcnoglobulin D\u00fczeyleri (Gruplar aras\u0131ndaki fark istatistiksel olarak anlaml\u0131d\u0131r)<\/p><\/div>\n<h3 class=\"p5\">Tedavi \u00d6ncesi ve Sonras\u0131 Kar\u015f\u0131la\u015ft\u0131rma<\/h3>\n<p class=\"p2\">Tan\u0131 \u00f6ncesi 43 (%95.6) hastada tekrarlayan infeksiyon \u00f6yk\u00fcs\u00fc ve\/veya bulgular\u0131 varken, tedavi alt\u0131nda 31 (%68.9) hastada infeksiyon meydana gelmi\u015fti (<i>p<\/i>=0.004). Tan\u0131 sonras\u0131 d\u00fczenli \u0130GRT alan hastalarda, tan\u0131 \u00f6ncesine g\u00f6re pn\u00f6moni ve \u00fcst solunum yolu infeksiyonu (\u00dcSY\u0130) s\u0131kl\u0131\u011f\u0131nda anlaml\u0131 d\u00fczeyde d\u00fc\u015f\u00fc\u015f tespit edildi [s\u0131ras\u0131yla; n=34 (%75.6) vs n=10 (%22.2), <i>p<\/i>&lt;0.001; n=30 (%66.7) vs n=14 (%31.1), <i>p<\/i>=0.003]. Tedavi alt\u0131nda 9 (%20) hastada semptomatik COVID-19 infeksiyonu geli\u015fmi\u015fti (pandeminin hen\u00fcz ortaya \u00e7\u0131kmad\u0131\u011f\u0131, tedavi \u00f6ncesi d\u00f6nem ile kar\u015f\u0131la\u015ft\u0131rma yapmak uygun olmad\u0131\u011f\u0131 i\u00e7in <i>p<\/i> de\u011feri verilmedi). Tan\u0131 \u00f6ncesinde hastalar\u0131n y\u0131lda ortanca infeksiyon say\u0131s\u0131 4 (0-10) iken tedavi sonras\u0131nda bu say\u0131 1 (0-3)\u2019e gerilemi\u015fti (<i>p<\/i>&lt;0.001) (Tablo 2).<\/p>\n<h3 class=\"p5\">Hastane Yat\u0131\u015f\u0131 Olan Hastalar\u0131n ve \u0130nfeksiyonlar\u0131n \u00d6zellikleri<\/h3>\n<p class=\"p2\">\u0130nfeksiyon nedeniyle hastaneye yat\u0131\u015f \u00f6yk\u00fcs\u00fc olan 17 (%37.8) hastadan be\u015finin infeksiyon nedeniyle birden fazla hastaneye yat\u0131\u015f \u00f6yk\u00fcs\u00fc oldu\u011fu g\u00f6r\u00fcld\u00fc. Bu be\u015f hastadan ilki<i> <\/i>CVID tan\u0131l\u0131yd\u0131 ve bron\u015fektazi ile otoimm\u00fcnite (vitiligo, \u00e7\u00f6lyak hastal\u0131\u011f\u0131) gibi iki komorbiditesi vard\u0131. Hasta tip 1 solunum yetmezli\u011fi nedeniyle uzun s\u00fcreli oksijen tedavisi alm\u0131\u015ft\u0131; \u0130V\u0130G ve TMP-SMX profilaksi tedavisine uyum g\u00f6stermeyen hastan\u0131n \u00e7oklu hastane yat\u0131\u015flar\u0131, pn\u00f6monisi ve balgam k\u00fclt\u00fcrlerinde geni\u015flemi\u015f spektrumlu beta-laktamaz (GSBL) \u00fcreten\u00a0<i>Escherichia coli<\/i>, <i>Klebsiella<\/i>\u00a0spp<i>., <\/i><span class=\"s3\"><i>Pseudomonas<\/i><\/span>\u00a0<i>aeruginosa<\/i>\u00a0\u00fcremeleri oldu\u011fu tespit edildi. Meropenem, ertapenem ve piperasilin-tazobaktam kullan\u0131m \u00f6yk\u00fcs\u00fc mevcuttu. Son ba\u015fvurusunda sepsis nedeni ile vefat etmi\u015fti. \u0130kinci hasta CVID tan\u0131l\u0131yd\u0131 ve bron\u015fektazi ile otoimm\u00fcnite (otoimm\u00fcn hepatite ba\u011fl\u0131 siroz ve hipersplenizme sekonder pansitopeni) komorbiditeleri vard\u0131. Hasta \u0130V\u0130G tedavisi ile izlemde n\u00f6tropenisi ve lenfopenisi oldu\u011fu d\u00f6nemde perianal apse, atipik pn\u00f6moni ve <i>Salmonella<\/i> infeksiyonlar\u0131 ge\u00e7irmi\u015fti; COVID-19 pandemisinin ba\u015f\u0131nda COVID-19 pn\u00f6monisi nedeniyle kaybedilmi\u015fti. \u00dc\u00e7\u00fcnc\u00fc<i> <\/i>hasta \u201c<span class=\"s4\">late onset combined immune deficiency<\/span>\u201d (LOCID) tan\u0131l\u0131 olup bron\u015fektazi komorbiditesi vard\u0131. Hasta pandemi s\u0131ras\u0131nda \u0130V\u0130G ve TMP-SMX profilaksi tedavilerini b\u0131rakm\u0131\u015f, solunum s\u0131k\u0131nt\u0131s\u0131 ile acil servise ba\u015fvurmu\u015ftu. Hasta bilateral CMV pn\u00f6monisi ile hastaneye yat\u0131r\u0131lm\u0131\u015f; tedavi sonras\u0131nda d\u00fczenli \u0130V\u0130G, TMP-SMX ve valgansiklovir profilaksi tedavileri ile taburcu edilmi\u015fti. Tedavi alt\u0131nda bir defa hastaneye yat\u0131\u015f gerektiren <i>Influenza A<\/i> <i>virus<\/i> infeksiyonu, bir defa ayaktan y\u00f6netilebilen COVID-19 infeksiyonu ge\u00e7irmi\u015fti. D\u00f6rd\u00fcnc\u00fc hasta NFkB eksikli\u011fi tan\u0131l\u0131yd\u0131 ve bron\u015fektazi ile<span class=\"Apple-converted-space\">\u00a0 <\/span>otoimm\u00fcnite (adrenal yetmezlik) komorbiditeleri vard\u0131; \u0130V\u0130G ve TMP-SMX profilaksisi ile takip edilen hastan\u0131n pn\u00f6moni nedeniyle birden fazla hastaneye yat\u0131\u015f\u0131 mevcuttu. Balgam k\u00fclt\u00fcrlerinde<i> <\/i><span class=\"s3\"><i>P.<\/i><\/span>\u00a0<i>aeruginosa<\/i>\u00a0\u00fcremeleri olup meropenem kullan\u0131m\u0131 ve devam\u0131nda karbapenem direnci ile amikasin kullan\u0131m\u0131 mevcuttu. Taburculukta profilaksi ama\u00e7l\u0131 inhale tobramisin tedavisi ba\u015flanm\u0131\u015f ancak hasta tedavi uyumsuzlu\u011fu nedeniyle kullanmam\u0131\u015ft\u0131. Be\u015finci hasta<i> <\/i>CVID tan\u0131l\u0131yd\u0131 ve mukokutan\u00f6z kandidiyazis, otoimm\u00fcnite (adrenal yetmezlik, hipoparatiroidi) komorbiditesi vard\u0131; \u0130V\u0130G, TMP-SMX ve posakonazol profilaksisi alm\u0131\u015ft\u0131. Posakonazol profilaksi kullan\u0131m\u0131na uyum g\u00f6stermedi\u011fi d\u00f6nemlerde oral <i>Candida <\/i>infeksiyonu nedenli antifungal tedavi ama\u00e7l\u0131 hastane yat\u0131\u015f\u0131 mevcuttu.<\/p>\n<h3 class=\"p5\">Hastane Yat\u0131\u015f\u0131 ile \u0130li\u015fkili Fakt\u00f6rler<\/h3>\n<p class=\"p2\">D\u00fczenli \u0130GRT alt\u0131nda infeksiyon nedeniyle hastane yat\u0131\u015f\u0131 olan 17 hasta [pn\u00f6moni (n=10),<span class=\"Apple-converted-space\">\u00a0 <\/span>sel\u00fclit\/abse (n=2), \u00fcriner sistem infeksiyonu (n=2), ishal (n=1), kriptik tonsillit (n=1) ve malign otitis eksterna (n=1)] tespit edildi. Hastaneye yat\u0131\u015f gerektiren infeksiyonlar\u0131 olan (n=17) ve olmayan (n=28) hastalar kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda; cinsiyet, ya\u015f, tan\u0131 ya\u015f\u0131, tan\u0131 esnas\u0131nda \u00f6yk\u00fcde tekrarlayan pn\u00f6moni varl\u0131\u011f\u0131, sigara kullan\u0131m\u0131, tan\u0131 an\u0131nda IgG d\u00fczeyi, CD19 lenfosit say\u0131s\u0131, \u00e7ukur IgG d\u00fczeyi ve TMP-SMX profilaksisi kullan\u0131m\u0131 a\u00e7\u0131s\u0131ndan istatistiksel olarak anlaml\u0131 d\u00fczeyde farkl\u0131l\u0131k saptanmad\u0131. Hastane yat\u0131\u015f\u0131 olan hastalar\u0131n ortanca tan\u0131 gecikme s\u00fcresi ve tan\u0131 \u00f6ncesi y\u0131ll\u0131k infeksiyon say\u0131s\u0131 hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re y\u00fcksek g\u00f6r\u00fclmekle birlikte fark istatistiksel olarak anlaml\u0131 d\u00fczeyde de\u011fildi (s\u0131ras\u0131yla; <i>p<\/i>=0.129 ve<i> p<\/i>=0.09). Komorbidite ve organ tutulumlar\u0131 incelendi\u011finde, hastane yat\u0131\u015f\u0131 olan hastalarda otoimmun hastal\u0131k s\u0131kl\u0131\u011f\u0131n\u0131n hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re istatistiksel olarak anlaml\u0131 d\u00fczeyde y\u00fcksek oldu\u011fu g\u00f6r\u00fcld\u00fc (%76.5 ve %35.7; <i>p<\/i>=0.008). A\u015f\u0131lama oranlar\u0131na bak\u0131ld\u0131\u011f\u0131nda; hastane yat\u0131\u015f\u0131 olan grupta d\u00fczenli olarak mevsimsel \u0130\u0130A ile a\u015f\u0131lanma oran\u0131n\u0131n hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re anlaml\u0131 d\u00fczeyde d\u00fc\u015f\u00fck oldu\u011fu tespit edildi (%20 ve %53.8; <i>p<\/i>=0.034) (Tablo 3). Bununla birlikte, hastane yat\u0131\u015f\u0131 olan hastalar\u0131n VK\u0130 ortalamas\u0131 hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re anlaml\u0131 d\u00fczeyde d\u00fc\u015f\u00fckt\u00fc (s\u0131ras\u0131yla; 20.5 ve 24.1 kg\/m<sup>2<\/sup>; <i>p<\/i>=0.019; \u015eekil 2).<\/p>\n<h3 class=\"p5\">Bron\u015fektazi ile \u0130li\u015fkili Fakt\u00f6rler<\/h3>\n<p class=\"p2\"><span class=\"s5\">Bron\u015fektazisi olan (n=20) ve olmayan (n=25) hastalar kar\u015f\u0131la\u015ft\u0131r\u0131ld\u0131\u011f\u0131nda, bron\u015fektazi ile takipli hastalarda tan\u0131 \u00f6ncesi pn\u00f6moni ge\u00e7irme oran\u0131 %95 iken bron\u015fektazisi olmayan grupta bu oran %60 olarak saptand\u0131 (<i>p<\/i>=0.012). Komorbidite ve organ tutulumlar\u0131 incelendi\u011finde ise bron\u015fektazisi olanlarda lenfadenomegali ve splenomegali g\u00f6r\u00fclme s\u0131kl\u0131\u011f\u0131 bron\u015fektazisi olmayan hastalara g\u00f6re anlaml\u0131 d\u00fczeyde y\u00fcksek izlendi (s\u0131ras\u0131yla; <i>p<\/i>=0.014 ve <i>p<\/i>=0.033). Bron\u015fektazili hastalarda ortanca IgG, IgA ve IgM d\u00fczeyleri bron\u015fektazisi olmayanlara g\u00f6re d\u00fc\u015f\u00fck g\u00f6r\u00fclmekle birlikte bunlardan sadece IgM fark\u0131 istatistiksel olarak anlaml\u0131 bulundu (<i>p<\/i>=0.003; \u015eekil 3).<\/span><\/p>\n<h2 class=\"p4\">\u0130RDELEME<\/h2>\n<p class=\"p2\">\u00c7al\u0131\u015fmam\u0131za dahil olan 45 hastada [(25 (%55.6) erkek ve 20 (%44.4) kad\u0131n, ortalama ya\u015f 38.4\u00b111.6 y\u0131l)], d\u00fczenli \u0130GRT ile y\u0131lda ortanca infeksiyon say\u0131s\u0131n\u0131n 4 (0-10)\u2019ten 1 (0-3)\u2019e dramatik bir \u015fekilde azald\u0131\u011f\u0131n\u0131 g\u00f6zlemledik.<\/p>\n<p class=\"p3\"><span class=\"s5\">Demografik verilere bakt\u0131\u011f\u0131m\u0131zda, global \u00f6l\u00e7ekte yap\u0131lan epidemiyolojik \u00e7al\u0131\u015fmalar, P\u0130Y prevalans\u0131n\u0131n co\u011frafi b\u00f6lgeye, etnik k\u00f6kene ve P\u0130Y\u2019in fenotipine g\u00f6re farkl\u0131l\u0131k g\u00f6sterdi\u011fini ortaya koymaktad\u0131r. \u00dclkemizin verilerinin de yer ald\u0131\u011f\u0131 ve 4918 hastan\u0131n de\u011ferlendirildi\u011fi bir ara\u015ft\u0131rmaya g\u00f6re; Orta Do\u011fu ve Kuzey Afrika b\u00f6lgelerinde P\u0130Y prevalans\u0131n\u0131n 0.8-30.5\/100 000 aras\u0131nda de\u011fi\u015fti\u011fi, akraba evlili\u011fi oran\u0131n\u0131n %20-56 ve aile bireylerinde P\u0130Y \u00f6yk\u00fcs\u00fcn\u00fcn %20-30.5 aras\u0131nda oldu\u011fu bildirilmi\u015ftir (15). \u00dclkemizde yap\u0131lan \u00e7al\u0131\u015fmalarda ise akraba evlili\u011fi oran\u0131 %12.9-30 aras\u0131nda saptanm\u0131\u015ft\u0131r (16,17). \u00c7al\u0131\u015fmam\u0131zdaki hastalar\u0131n akraba evlili\u011fi ve aile bireylerinde P\u0130Y \u00f6yk\u00fcs\u00fc oran\u0131 \u00fclkemizden bildirilen sonu\u00e7larla benzerdir.<\/span><\/p>\n<p class=\"p3\">Primer imm\u00fcn yetmezlik tan\u0131s\u0131 alan hastalar s\u0131k tekrarlayan, tedaviye ge\u00e7 yan\u0131t veren, a\u011f\u0131r ve komplike seyreden infeksiyonlar ya\u015famalar\u0131na ra\u011fmen ge\u00e7 tan\u0131 almaktad\u0131rlar; buna ba\u011fl\u0131 olarak bu hastalarda bron\u015fektazi ba\u015fta olmak \u00fczere etkilenen organlarda kal\u0131c\u0131 fonksiyon kay\u0131plar\u0131 ya\u015famaktad\u0131r (1,3,4). Hastalar\u0131m\u0131z\u0131n %95.6\u2019s\u0131nda tan\u0131 \u00f6ncesi y\u0131lda ortanca 4 (0-10) s\u0131k tekrarlayan, tedaviye ge\u00e7 yan\u0131t veren, a\u011f\u0131r ve\/veya komplike seyreden infeksiyon tan\u0131s\u0131 vard\u0131; tan\u0131 gecikmesi ortanca 6 (0-33) y\u0131ld\u0131. Hastalar\u0131m\u0131zda bron\u015fektazi g\u00f6r\u00fclme oran\u0131 Oksenhendler ve arkada\u015flar\u0131n\u0131n (7) bulgusuna (%44.4) yak\u0131nd\u0131; ancak oran\u0131n daha y\u00fcksek ve d\u00fc\u015f\u00fck oldu\u011fu<span class=\"Apple-converted-space\">\u00a0 <\/span>\u00e7al\u0131\u015fmalar da mevcuttur (16,18).<\/p>\n<p class=\"p3\">Heterojen hasta grubumuzdaki en s\u0131k kar\u015f\u0131la\u015f\u0131lan fenotip (%88.8 ile PAE; %71.1 ile alt grubu olan CVID), eri\u015fkinlerde en s\u0131k kar\u015f\u0131la\u015f\u0131lan semptomatik fenotip olan PAE idi (8).Hastalar\u0131n hepsi \u0130GRT alm\u0131\u015f olup kullan\u0131lan Ig dozu klinik takip parametreleri ve \u00e7ukur IgG d\u00fczeylerine g\u00f6re ayarlanm\u0131\u015ft\u0131; infeksiyonu olmayan sekiz hasta d\u0131\u015f\u0131ndaki hastalara (37, %82.2) antimikrobiyal profilaksi (30 hasta TMP-SMX) \u00f6nerilmi\u015fti. \u00c7al\u0131\u015fmam\u0131zdaki hastalar\u0131n antimikrobiyal profilaksi kullan\u0131m oran\u0131, Mu\u015fabak ve arkada\u015flar\u0131 (16) ile Thickett ve arkada\u015flar\u0131n\u0131n (19) (s\u0131ras\u0131yla %43 ve %41.9) \u00e7al\u0131\u015fmalar\u0131n\u0131n sonu\u00e7lar\u0131na g\u00f6re daha y\u00fcksekti.<\/p>\n<p class=\"p3\"><span class=\"s5\">Bu durum, \u00e7al\u0131\u015fmam\u0131zdaki hastalar\u0131n \u0130GRT alt\u0131nda daha fazla infeksiyon ge\u00e7iren bir grup oldu\u011fu \u015feklinde yorumlanabilir; ancak merkezimizde P\u0130Y ile takipli hastalarda \u0130GRT\u2019den ba\u011f\u0131ms\u0131z bir \u015fekilde kar\u015f\u0131la\u015f\u0131lan infeksiyonlarda daha erken antibiyotik profilaksisi ba\u015flamaya y\u00f6nelik bir e\u011filimin oldu\u011fu da d\u00fc\u015f\u00fcn\u00fclebilir. Bu yakla\u015f\u0131m\u0131n olas\u0131 nedeni P\u0130Y ile takipli hassas hasta pop\u00fclasyonunda mortalite ve morbiditeyi azaltmakt\u0131r.<\/span><\/p>\n<p class=\"p3\">\u00c7al\u0131\u015fmam\u0131zda, en s\u0131k kar\u015f\u0131la\u015f\u0131lan infeksiyonlar mevcut verilerle uyumlu olarak \u00fcst ve alt solunum yolu infeksiyonlar\u0131 idi. \u0130mm\u00fcnoglobulin replasman tedavisinin \u00f6ncesi ve sonras\u0131nda infeksiyon g\u00f6r\u00fclme s\u0131kl\u0131\u011f\u0131 a\u00e7\u0131s\u0131ndan di\u011fer \u00e7al\u0131\u015fmalarla da uyumlu bir \u015fekilde \u00e7al\u0131\u015fmam\u0131zda da ortanca infeksiyon say\u0131s\u0131, pn\u00f6moni ve \u00dcSY\u0130 g\u00f6r\u00fclme s\u0131kl\u0131\u011f\u0131 istatistiksel olarak anlaml\u0131 d\u00fczeyde idi ve dikkat \u00e7ekici bir oranda azalm\u0131\u015ft\u0131 (7,11). Pandemi s\u00fcresince en az bir kez nazofarengeal s\u00fcr\u00fcnt\u00fc ile elde edilen SARS-CoV-2 polimeraz zincir reaksiyonu testi ile do\u011frulanm\u0131\u015f COVID-19 s\u0131kl\u0131\u011f\u0131 %20 idi. Tedavi \u00f6ncesi d\u00f6nem, \u00e7o\u011fu hastada pandemi \u00f6ncesine denk geldi\u011finden COVID-19 \u00f6zelinde tedavinin etkinli\u011fi a\u00e7\u0131s\u0131ndan kar\u015f\u0131la\u015ft\u0131rma yapmak uygun olmayacakt\u0131r. Tedavi alt\u0131nda hastaneye yat\u0131\u015f gerektiren infeksiyonu olan ve olmayan hastalar\u0131m\u0131z\u0131n klinik \u00f6zelliklerini kar\u015f\u0131la\u015ft\u0131rd\u0131\u011f\u0131m\u0131zda; hastane yat\u0131\u015f\u0131 olanlarda mevsimsel \u0130\u0130A ile a\u015f\u0131lanma oran\u0131 ve VK\u0130 ortalamas\u0131 hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re anlaml\u0131 d\u00fczeyde d\u00fc\u015f\u00fck bulundu. Bu bulgular P\u0130Y\u2019li hastalar\u0131n takip s\u00fcreci i\u00e7in yap\u0131lan \u00f6nerilenlere de uygun olarak (6,20-22); yat\u0131\u015f gerektiren infeksiyon komplikasyonlar\u0131ndan korunmak i\u00e7in hastalar\u0131n enerji-protein malnutrisyonu a\u00e7\u0131s\u0131ndan her vizitte d\u00fczenli olarak takip edilmesinin ve destek sa\u011flanmas\u0131n\u0131n, imm\u00fcn yetmezlik bile\u015fenine uygun a\u015f\u0131lama yap\u0131lmas\u0131n\u0131n olduk\u00e7a \u00f6nemli oldu\u011funu bize g\u00f6sterdi.<\/p>\n<p class=\"p3\">Yap\u0131lan di\u011fer \u00e7al\u0131\u015fmalarla (7,11,12) benzer \u015fekilde, hasta grubumuzda da \u0130GRT infeksiyon s\u0131kl\u0131\u011f\u0131n\u0131 azaltmakla birlikte solunum yolu infeksiyonlar\u0131 g\u00f6r\u00fclmeye devam etmi\u015fti. Primer imm\u00fcn yetmezli\u011fi olan hastalarda hastaneye yat\u0131\u015f gerektiren infeksiyonlar\u0131 inceledi\u011fimizde \u00f6ncelikli olarak kar\u015f\u0131m\u0131za \u00e7\u0131kan infeksiyon ajanlar\u0131 GSBL+<i> E. coli<\/i>, <i>Klebsiella<\/i>\u00a0spp., <span class=\"s3\"><i>P.<\/i><\/span>\u00a0<i>aeruginosa, CMV<\/i> ve SARS-CoV-2 idi. Komorbidite ve organ tutulumlar\u0131 incelendi\u011finde, hastane yat\u0131\u015f\u0131 olan hastalarda otoimm\u00fcn hastal\u0131k s\u0131kl\u0131\u011f\u0131n\u0131n hastane yat\u0131\u015f\u0131 olmayanlara g\u00f6re istatistiksel olarak anlaml\u0131 d\u00fczeyde y\u00fcksek oldu\u011fu g\u00f6r\u00fcld\u00fc. Bu hastalara \u00f6zellikle bron\u015fektazi daha s\u0131k e\u015flik etmekteydi. Bu durum, daha \u00e7ok \u00f6n planda antikor eksikli\u011fi ile takipli olan hastalar\u0131n multipl komorbiditesi olan alt grubunda, e\u015flik eden daha a\u011f\u0131r T h\u00fccre disfonksiyonu olabilece\u011fini d\u00fc\u015f\u00fcnd\u00fcrmektedir.<\/p>\n<p class=\"p3\"><span class=\"s6\">Oksenhendler<\/span> ve arkada\u015flar\u0131n\u0131n (7) \u00e7al\u0131\u015fmas\u0131na benzer \u015fekilde hastaneye yat\u0131\u015f gerektiren infeksiyonlar\u0131 olan ve olmayan iki grubun \u00e7ukur IgG d\u00fczeylerini kar\u015f\u0131la\u015ft\u0131rd\u0131\u011f\u0131m\u0131zda istatistiksel olarak anlaml\u0131 d\u00fczeyde fark saptamad\u0131k. \u00c7al\u0131\u015fmam\u0131zda \u0130GRT\u2019nin \u00f6nemi \u00f6ne \u00e7\u0131kmakla birlikte, \u00f6zellikle antimikrobiyal profilaksiye uyumsuzlu\u011fun infeksiyon riskini art\u0131rd\u0131\u011f\u0131n\u0131 g\u00f6zlemledik. Ayr\u0131ca; bron\u015fektazisi olan ve olmayan hastalar aras\u0131nda tan\u0131 \u00f6ncesi pn\u00f6moni ge\u00e7irme oran\u0131 istatistiksel olarak anlaml\u0131 d\u00fczeyde y\u00fcksekti. Bu bulgu, erken tan\u0131 ve tedavinin komplikasyonlar\u0131n \u00f6nlenmesinde olduk\u00e7a \u00f6nemli oldu\u011funu g\u00f6stermektedir.<\/p>\n<p class=\"p3\">\u00c7al\u0131\u015fman\u0131n retrospektif yap\u0131lmas\u0131, s\u0131n\u0131rl\u0131 \u00f6rneklem b\u00fcy\u00fckl\u00fc\u011f\u00fc,<span class=\"Apple-converted-space\">\u00a0 <\/span>tan\u0131 \u00f6ncesindeki infeksiyon b\u00f6lgelerinin ve say\u0131lar\u0131n hastalar\u0131n y\u0131llarca s\u00fcregelen \u00f6yk\u00fclerine dayal\u0131 elde edilmesi, baz\u0131 hastalar\u0131n ya\u015fad\u0131\u011f\u0131 yerlerin il d\u0131\u015f\u0131nda olmas\u0131 nedeniyle d\u0131\u015f merkezdeki yat\u0131\u015f s\u00fcre\u00e7lerine dair detayl\u0131 bilgi ve belgelere eri\u015fim eksikli\u011fi ve s\u0131n\u0131rl\u0131 genetik test imkanlar\u0131 \u00e7al\u0131\u015fmam\u0131z\u0131n en \u00f6nemli k\u0131s\u0131tl\u0131l\u0131klar\u0131d\u0131r. Ayr\u0131ca, hastalar\u0131n tedavilere uyumunun sonu\u00e7lar\u0131; antimikrobiyal profilaksi \u00f6ncesinde ve sonras\u0131nda infeksiyon say\u0131lar\u0131n\u0131n yan\u0131 s\u0131ra hastane yat\u0131\u015flar\u0131n\u0131n etkisini de\u011ferlendirmemizde ciddi k\u0131s\u0131tl\u0131l\u0131klar yaratt\u0131. Ancak \u00e7al\u0131\u015fmam\u0131z P\u0130Y\u2019li hastalar\u0131n tedavisinde maln\u00fctrisyonun ve\/veya malabsorbsiyonun \u00f6nemine, klinik takipte \u00f6nemli bir sorun olu\u015fturan tedavi uyumsuzlu\u011funa ve tan\u0131 alana kadar ge\u00e7irilen uzun s\u00fcrelerin infeksiyonla ilgili komplikasyonlar\u0131 art\u0131rd\u0131\u011f\u0131na dikkat \u00e7ekmektedir. Bu durum, CVID tan\u0131s\u0131n\u0131n s\u0131k bir komplikasyonu olan (bizim \u00e7al\u0131\u015fmam\u0131zda hasta \u00f6yk\u00fcs\u00fcne dayal\u0131 gastrointestinal sistem tutulumu s\u0131kl\u0131\u011f\u0131 %24.4 idi) CVID ili\u015fkili enteropatiye y\u00f6nelik uygun y\u00f6netimin \u00f6nemine dikkat \u00e7ekmektedir.<\/p>\n<p class=\"p3\"><span class=\"s5\">Bu \u00e7al\u0131\u015fmada P\u0130Y hastalar\u0131n\u0131n tan\u0131 alma s\u00fcrecindeki semptomlar\u0131, tan\u0131sal gecikmelerden kaynaklanan morbidite oranlar\u0131 ve \u0130GRT, a\u015f\u0131lama ve antimikrobiyal profilaksi sonras\u0131nda meydana gelen de\u011fi\u015fiklikler sunuldu. Sonu\u00e7 olarak; P\u0130Y\u2019in erken tan\u0131s\u0131, tedavisi ve hastalar\u0131n tedavi uyumlar\u0131n\u0131n art\u0131r\u0131lmas\u0131 ile infeksiyonlar ba\u015fta olmak \u00fczere bir\u00e7ok komplikasyonun \u00f6n\u00fcne ge\u00e7ilebilir. Bu nedenle, P\u0130Y\u2019e ili\u015fkin fark\u0131ndal\u0131\u011f\u0131n hem toplumda hem de her uzmanl\u0131k alan\u0131ndan hekimlerde art\u0131r\u0131lmas\u0131, hastalar\u0131n erken d\u00f6nemde tan\u0131 almalar\u0131n\u0131 sa\u011flayacak ve hastaneye yat\u0131\u015f\u0131, mortaliteyi, morbiditeyi azaltacakt\u0131r.<\/span><\/p>\n","protected":false},"excerpt":{"rendered":"<p>G\u0130R\u0130\u015e Daha \u00f6nce primer imm\u00fcn yetmezlik (P\u0130Y) olarak adland\u0131r\u0131lan do\u011fu\u015ftan ba\u011f\u0131\u015f\u0131kl\u0131k hatalar\u0131 (\u201cinborn errors of immunity\u201d &#8211; IEI), do\u011fal ve\/veya adaptif imm\u00fcn sistem bile\u015fenlerini etkileyen kal\u0131tsal bozukluklard\u0131r; infeksiyonlara duyarl\u0131l\u0131k ve imm\u00fcn disreg\u00fclasyon ile karakterize olabilirler (1). Uluslararas\u0131 \u0130mm\u00fcnoloji Dernekleri Birli\u011fi (International Union of Immunological Societies-\u00a0 IUIS) IEI Komitesi yapt\u0131\u011f\u0131 son g\u00fcncelleme ile P\u0130Y\u2019leri fenotiplerine g\u00f6re [&hellip;]<\/p>\n","protected":false},"author":6,"featured_media":28822,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[5129],"tags":[5924,5923,4874],"class_list":["post-28671","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-ozgun-arastirma","tag-immunoglobulin-replasman-tedavisi","tag-primer-immun-yetmezlik","tag-solunum-yolu-infeksiyonlari"],"acf":[],"_links":{"self":[{"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/posts\/28671","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/comments?post=28671"}],"version-history":[{"count":4,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/posts\/28671\/revisions"}],"predecessor-version":[{"id":28911,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/posts\/28671\/revisions\/28911"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/media\/28822"}],"wp:attachment":[{"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/media?parent=28671"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/categories?post=28671"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.klimikdergisi.org\/tr\/wp-json\/wp\/v2\/tags?post=28671"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}