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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.
The Hirschsprung disease is a disease characterized by the inherent absence of ganglion cells in the submucosal and myenteric nerve network in the distal column. In the general population, the incidence is 1/5000 living births. The symptoms of this disease may vary from intestinal obstruction in the newborn period to chronic constipation in older children. Persistant anemia is a very rare symptom. Here; the fact that the five-year-old applied for oral iron treatment with anemia of severe iron deficiency and constipation was presented. In the physical examination there was advanced western distancy and malnutrition. In the baryum column graph, the sigmoid column was traced as a clear dilate, the rectosigmoid column was seen as a transition zone. Hirschprung disease was diagnosed when no ganglion cells were observed in the submucosal and intermyenteric plexus in the rectal biopsy. In children with anemia of severe iron deficiency and congestive complaints, the pre-diagnosis of Hirschsprung disease should also be taken into account. Hirschsprung’s disease is a congenital disorder and is characterized by the absence 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 level of 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.
Alan : Sağlık Bilimleri
Dergi Türü : Uluslararası
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