Kroni̇k Hastalığı Olan Çocuğun İzlemi̇ ve Toplumda Bakımı

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DOI :10.26650/B/CH32.2024.010.022   IUP :10.26650/B/CH32.2024.010.022    Tam Metin (PDF)

IgA Vasküli̇ti̇ (Henoch Schönlein Purpurasi) Olan Çocuğun İzlemi̇ ve Toplumda Bakımı

Gülşah Kavrul Kayaalp, Nuray Aktay Ayaz

IgA vasküliti çocukluk çağında en sık görülen vaskülittir. En sık 3-15 yaşları arasında görülür. Etiyolojisi tam olarak aydınlatılamamıştır. Küçük damar duvarlarında IgA baskın immün komplekslerin birikimi ve lökositoklastik vaskülit ile karakterizedir. En sık etkilenen organ deridir. Tipik döküntü, alt ekstremitelerde daha belirgin olmak üzere simetrik, palpabl purpuradır. Gastrointestinal tutulum sıktır. Uzun dönem mortalite ve morbiditeyi belirleyen en önemli organ tutulumu böbrek tutulumudur. Renal tutulum değişken bir spektrumda görülebilmektedir. Artrit genellikle büyük eklemleri tutan kendi kendini sınırlayan artrit şeklinde görülür. IgA vasküliti olan çocuklarda nadiren merkezi sinir sistemi vasküliti, pulmoner hemoraji, interstisyel pnömoni, epistaksis, kardit, adrenal kanama ve testis tutulumu görülebilir. Tanı klinik olarak konulmaktadır. Döküntünün atipik olduğu durumlarda diğer tanıları dışlamak için deri biyopsisi yapılabilir. IgA vasküliti olan çocuklar organ tutulumu açısından izlenmelidir. Böbrek tutulumu açısından tüm çocuklar en az iki yıl boyunca tam idrar tahlili, böbrek fonksiyonu ve kan basıncı ölçümleri ile takip edilmelidir. Gastrointestinal tutulum açısından karın ağrısı, hematemez, hematokezya ve melena varlığı takip edilmelidir. Döküntü, artrit ve artralji varlığında destek tedavisi verilir. Şiddetli karın ağrısı, şiddetli böbrek tutulumu, nörolojik bulgular veya çok şiddetli cilt bulguları varlığında yatırılarak izlenir. Ciddi gastrointestinal tutulum ve böbrek tutulumu varlığında steroidler kullanılır. Şiddetli vakalarda, siklofosfamid veya siklosporin gibi immünsüpresif ilaçlar düşünülmelidir. Nüks görülebileceğinden uzun süreli takip gerekmektedir. Başlangıçta, şiddetli böbrek tutulumu olan çocuklarda renal nüksler daha sık görülmektedir. Takipte sağlıklı beslenme, steroid kullanmakta olan çocuklarda düşük sodyumlu diyet, uzun süreli steroid tedavisi alanlarda kalsiyum ve D vitamini desteği, tüm olgularda uygun fiziksel aktivitenin desteklenmesi ve bağışıklama diğer önemli faktörlerdir. Tüm çocukların aşılanma durumu kontrol edilmelidir.


Anahtar Kelimeler: IgA vasküliti, çocuk, Henoch Schönleın Purpurası
DOI :10.26650/B/CH32.2024.010.022   IUP :10.26650/B/CH32.2024.010.022    Tam Metin (PDF)

Follow-up and Community Care of the Child with the Diagnosis of IgA Vasculitis (Henoch Schönlein Purpura)

Gülşah Kavrul Kayaalp, Nuray Aktay Ayaz

IgA vasculitis (IgAV) is the most common vasculitis in childhood, occurring most frequently between the ages of 3 and 15. However, its etiology has not been fully elucidated. Characterized by the accumulation of IgA-dominated immune complexes in the small vessel walls and leukocytoclastic vasculitis, IgAV most frequently affects the skin, presenting as a symmetrical, palpable purpura rash, particularly on the lower extremities, as well as the gastrointestinal tract. Kidney involvement primarily determines long-term mortality and morbidity. Arthritis usually presents as selflimiting that involves large joints. Central nervous system vasculitis, pulmonary hemorrhage, interstitial pneumonia, epistaxis, carditis, adrenal hemorrhage, and testicular involvement rarely occur. Although a skin biopsy may be performed in cases where the rash is atypical to rule out other conditions, it is essential to monitor all patients with IgAV for organ involvement. For renal involvement, regular follow-ups including full urinalysis, renal function assessment, and blood pressure measurements for at least 2 years are recommended. For gastrointestinal involvement, abdominal pain, hematemesis, hematochezia, and melena should be closely monitored. Supportive treatment is indicated for patients presenting with a rash, arthritis, and arthralgia. Meanwhile, hospitalization and close monitoring are necessary for patients with severe abdominal pain, severe kidney involvement, neurological findings, or very severe skin manifestations. Steroids are used for severe gastrointestinal and renal involvement. For severe cases, immunosuppressive agents (cyclophosphamide or cyclosporine) should be considered. Long-term follow-up is necessary due to recurrence; for example, patients with severe renal involvement are more prone to renal recurrences. A healthy diet and low-sodium intake are recommended for patients using steroids, calcium and vitamin D supplementation for those receiving long-term steroid therapy, and continuation of appropriate physical activity for all patients. Importantly, as immunization guidelines should be adhered to, vaccination status of all patients should be checked.


Anahtar Kelimeler: IgA vasculitis, child, Henoch Schönlein Purpura

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