CIDDI DEMIR EKSIKLIĞI ANEMISININ EŞLIK ETTIĞI HIRSCHSPRUNG HASTALIĞI / SEVERE IRON DEFICIENCY ANEMIA ASSOCIATED WITH HIRSCHSPRUNG’S DISEASE

Yıl 2015, Cilt: 37 Sayı: 2, 24 - 28, 25.12.2015

Yeter Kar , Zeynep Özdemir Ayşe Turhan Mehmet Arda Özcan Bör

Öz

Özet: Hirschsprung hastalığı;  distal kolondaki submukozal ve myenterik sinir ağında gangliyon hücrelerinin doğuştan yokluğu ile karakterize bir hastalıktır. Genel popülasyonda insidansı 1/5000 canlı doğumdur. Bu hastalıkta belirtiler, yenidoğan döneminde intestinal obstrüksiyondan daha büyük çocuklarda kronik kabızlığa kadar değişkenlik gösterebilir. Persistan anemi ise oldukça nadir bir bulgudur. Burada; beş yaşında oral demir tedavisine yanıtsız ağır demir eksikliği anemisi ve kabızlık şikayeti ile başvuran olgu sunuldu. Fizik muayenesinde ileri derecede batın distansiyonu ve malnutrisyonu mevcuttu. Baryumlu kolan grafisinde sigmoid kolon belirgin dilate olarak izelndi, rektosigmoid kolonda geçiş zonu görüldü. Rektal biyopside submukozal ve intermyenterik pleksuslarda gangliyon hücresi görülmemesi üzerine Hirschprung hastalığı tanısı konuldu. Ağır demir eksikliği anemisi ve kabızlık şikayeti ile başvuran çocuk hastalarda ön tanıda Hirschsprung hastalığı da akılda tutulmalıdır.

ANAHTAR KELİMELER: Hirschsprung hastalığı, demir eksikliği anemisi, kabızlık

 

SEVERE IRON DEFICIENCY ANEMIA ASSOCIATED WITH HIRSCHSPRUNG’S DISEASE

Abstract: Hirschsprung’s disease is a congenital disorder and is characterized by the absent of ganglion cells in the myenteric and submucosal plexus of the distal intestine. The incidence is approximately 1 in 5000 live births. Symptoms range from neonatal intestinal obstruction to chronic progressive constipation in older children. Persistent anemia is a very rare symptom. Here, we report a case of a 5 year-old boy who presented with severe iron deficiency anemia unresponsive to oral iron therapy and constipation. Physical examination revealed a severe abdominal distention and malnutrition. An abdominal radiography showed the rectosigmoid transition zone. Hirschsprung’s disease diagnosis was confirmed by rectal biopsy. Hirschsprung's disease should be considered in children presenting with severe iron deficiency anemia and constipation.

KEYWORDS: Hirscprung’s disease, iron deficiency anemia, constipation.

 

Anahtar Kelimeler

ANAHTAR KELİMELER: Hirschsprung hastalığı, demir eksikliği anemisi, kabızlık

Kaynakça

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