Ege Hemofili Konseyi 20 yıllık verileriyle Türkiye hemofili bakımı ve tedavisi sürecinin değerlendirilmesi
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Tarih
2022
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Yayıncı
Ege Üniversitesi, Tıp Fakültesi
Erişim Hakkı
info:eu-repo/semantics/embargoedAccess
Özet
Hemofili hastalığı yeterince iyi tedavi edilmezse kalıcı eklem sakatlıkları ve özürlü toplum bireyleri ortaya çıkabilir. Eklem problemleri erken dönemde ele alındığında tıbbi olarak tedavi edilebilirken daha ileri dönemde ancak ortopedik yaklaşımlarla tedavi edilebilir.
Bu çalışmanın amacı 20 yıllık bir süredir tüm Türkiye’deki hemofili hastalarına hizmet veren ve tedavilerini üstlenen Ege Hemofili Eklem Konseyi’nin arşiv dokümanlarını inceleyerek başvuran hastaların demografik ve ortopedik özelliklerinin ilk 10 yıl ve ikinci 10 yıldaki değişimleri de ortaya koyarak değerlendirmektir. 2000-2021 yılı arasındaki resmi kayıtlar ve hastaların dosyalarındaki ilgili bilgiler geriye yönelik olarak incelendi. 20 yıllık bir süre içinde 583 hastanın 1580 kez konseyde değerlendirildiği görüldü. Hastaların 551’i (%95) erkekti. Yaş ortalaması ise ilk 10 yıllık dönemde 19 ± 9 yıl iken ikinci dekadda 26 ± 13 yıl idi. Tanı dağılımına bakıldığında hemofili A (n=426) (%73) ve hemofili B (n=81) (%13) ön planda iken von Willebrand hastalığı (n=17) ve nadir faktör eksiklikleri (n=15) ancak %14’lük bir hasta grubunu oluşturabildi. Faktör VIII/IX düzeyleri ise %93 hastada %1 veya altında bulundu. İnhibitör saptanan olgular ise %10 oranda tespit edildi. Hastaların %58'i devlet hastanelerinden sevk edilmişti. %42’si ise üniversite gruplarından geliyordu. Ege Üniversitesi Çocuk ve Erişkin Hematoloji Üniteleri’nden gelen %34 hasta mevcut iken Ege dışındaki %66 hasta diğer ünitelerden geliyordu. İzmir %31, ve tüm Ege Bölgesi %56'lık bir orandaydı. Kalan tüm hastaların Anadolu’nun her yanından gelen hastalar olması nedeniyle grubumuzun ülkemizdeki tüm hemofili hastalarını temsil yeteneği ve gücü olduğu düşünülmektedir. Konseye başvuran hastaların çoğunun profilaksi değil kanadıkça tedavi programında olduğu görüldü (%28 vs. %72). İlk 10 yıllık dönemdeki bu oranlar kanadıkça tedavi lehine olarak %24 vs. %76 iken daha sonraki dekadda %32 vs. %68 oldu. Hemofilik artropati dışında en sık ek hastalıklar pes planus ve Perthes hastalığı oldu. Hastaların %95'inde sorun hemofilik artropati idi. Hedef eklem gelişme oranı eklem bazında değerlendirildiğinde sırasıyla diz (%58 ve %56), ayak bileği (%17 ve %24), dirsek eklemi (%19 ve %10) bulundu. Tek hedef eklem gelişme oranı %73 iken, iki eklemde hedef eklem tanımlanan %20 olgu vardı. İnhibitörlü gruptaki hedef eklem gelişme dağılımı oranları benzer olarak gözlendi.
Yardımcı ortopedik alet kullanım oranının ikinci on yılda giderek azaldığı saptandı. Hemofili konseylerinde alınan kararların hayata geçirilmesi tıbbi tedavi için %100 iken, radyoizotop sinovektomi için %82, major ortopedik operasyonlar için ise %46 olarak hesaplandı. Tıbbi tedavi başarı oranı ise %50.2’de kaldı. Sonuç olarak 20 yıllık Ege Hemofili Konseyi kayıt ve sonuçlarının değerlendirilmesi ile ülkemizdeki hemofili tedavisinin kalitesine yönelik bir değerlendirme yapılabildi. Başlangıçta nadir olan profilaksi uygulamasının giderek artması dikkati çekti. Konsey için başvuran tüm hastalara tıbbi ve cerrahi tedavi olarak kendi merkezimizde tedavi ve takip imkanı verilmesi dikkat çekici bulundu. Hastaların yarısına yakın bir kısmı cerrahi girişimlerden yararlandı.
Hemophilia can cause persistent arthropathy and disable persons in the community if appropriate therapy and prophylaxis were not applied. Joint problems are easily treated with secondary prophylactic programs whereas progressive joint problems generally eed minor or major orthopedic interventions. The aim of this study was evaluation of records of the Ege Hemophilia Joint Council which has continued since years of 2000-2021. Twenty year of experience reevaluated the first ten years and second ten years of period for demographics and orthopedic approaches in patients with hemophiliac arthropathy. The minutes and records of Ege Hemophilia Joint Council were evaluated retrospectively in their hospital files. For twenty years of period 583 patients were recorded with 1580 admissions. 95% of patients (55/583) were male. Mean age was 19 ± 9 years and 26 ± 13 years consecutively for two decades. Hemophilia-A group (n=426)(%73), and hemophilia-B group (n=81)(% 13) are the largest groups of patients. von Willebrand disease (n=17) and rare factor deficiencies (n=15) were the minorities. Factor VIII and IX activity was %1 and below %1 in the majority of patients (%93). The Inhibitor development frequency was %10 for all groups. Most of the patients (%58) referred from State Hospitals. Other patients came from University hospitals from different regions of Turkey. One third of patients came from Ege University Child and Adult hemophilia center. Ege Region of Turkey consists half of the patients. All other patients came from every region of Anatolian cities. This point denotes patients who came to the Ege Hemophilia Council as generally representative as all Turkish patients with hemophilia for the outcome of hemophilic arthropathy. Episodic therapy was the first approach (%76) for the first decade and proceeded to %68 for the last decade. Prophylaxis percentages for patients were %24 and % 32, consecutively. Most frequent additional disorders were pes planus and Perthes disease. 95 percent of patients, problem was hemophilic arthropathy. Target joint availability was the first for knees (%58 and % 56) and then for ankles (%14 and %24) and lastly was elbows (%19 and %10). One target joint frequency was %73 and two target joint availability was %20 of patients. Additional orthopedic tools gradually decreased from the first to second decades. Decision types of hemophilia joint council were medical (initiation of prophylaxis or dose adjustments during prophylaxis), minor surgical (radioisotope synovectomy) and major orthopedic operations in hemophilia patients. Realizations were %100 for medical therapy, % 83 for radioisotope synovectomy and %46 for major orthopedic operations. Success rate for medical therapy was %50.2. As a conclusion, Ege Hemophilia Joint Council data is easily reflected as national data with many patients with hemophilic arthropathy. Rarity of prophylaxis was observed in the first decade. Most of the patients were treated with episodic treatment. Main reason for the arthropathies was the low rate of prophylaxis at that time. The most interesting and important point was that all patients coming to the Joint Council were able to approach a kind of treatment of hemophilic arthropathy. All elective and major surgeries were able to be done at the half of patients.
Hemophilia can cause persistent arthropathy and disable persons in the community if appropriate therapy and prophylaxis were not applied. Joint problems are easily treated with secondary prophylactic programs whereas progressive joint problems generally eed minor or major orthopedic interventions. The aim of this study was evaluation of records of the Ege Hemophilia Joint Council which has continued since years of 2000-2021. Twenty year of experience reevaluated the first ten years and second ten years of period for demographics and orthopedic approaches in patients with hemophiliac arthropathy. The minutes and records of Ege Hemophilia Joint Council were evaluated retrospectively in their hospital files. For twenty years of period 583 patients were recorded with 1580 admissions. 95% of patients (55/583) were male. Mean age was 19 ± 9 years and 26 ± 13 years consecutively for two decades. Hemophilia-A group (n=426)(%73), and hemophilia-B group (n=81)(% 13) are the largest groups of patients. von Willebrand disease (n=17) and rare factor deficiencies (n=15) were the minorities. Factor VIII and IX activity was %1 and below %1 in the majority of patients (%93). The Inhibitor development frequency was %10 for all groups. Most of the patients (%58) referred from State Hospitals. Other patients came from University hospitals from different regions of Turkey. One third of patients came from Ege University Child and Adult hemophilia center. Ege Region of Turkey consists half of the patients. All other patients came from every region of Anatolian cities. This point denotes patients who came to the Ege Hemophilia Council as generally representative as all Turkish patients with hemophilia for the outcome of hemophilic arthropathy. Episodic therapy was the first approach (%76) for the first decade and proceeded to %68 for the last decade. Prophylaxis percentages for patients were %24 and % 32, consecutively. Most frequent additional disorders were pes planus and Perthes disease. 95 percent of patients, problem was hemophilic arthropathy. Target joint availability was the first for knees (%58 and % 56) and then for ankles (%14 and %24) and lastly was elbows (%19 and %10). One target joint frequency was %73 and two target joint availability was %20 of patients. Additional orthopedic tools gradually decreased from the first to second decades. Decision types of hemophilia joint council were medical (initiation of prophylaxis or dose adjustments during prophylaxis), minor surgical (radioisotope synovectomy) and major orthopedic operations in hemophilia patients. Realizations were %100 for medical therapy, % 83 for radioisotope synovectomy and %46 for major orthopedic operations. Success rate for medical therapy was %50.2. As a conclusion, Ege Hemophilia Joint Council data is easily reflected as national data with many patients with hemophilic arthropathy. Rarity of prophylaxis was observed in the first decade. Most of the patients were treated with episodic treatment. Main reason for the arthropathies was the low rate of prophylaxis at that time. The most interesting and important point was that all patients coming to the Joint Council were able to approach a kind of treatment of hemophilic arthropathy. All elective and major surgeries were able to be done at the half of patients.
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