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Klin Padiatr ; 226(6-7): 372-4, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25431870

ABSTRACT

UNLABELLED: Sickle cell disease is a chronic hematologic disease with variable but often severe systemic symptoms. In this report, we describe a 6-year-old boy presenting with acute bowel pseudo-obstruction. During this episode, previously undiagnosed sickle cell disease was discovered upon peripheral blood smear analysis. The condition was therefore interpreted as a massive intestinal vaso-occlusive crisis or "girdle syndrome". Conservative treatment with hydration therapy, analgesia and a manual partial exchange transfusion was initiated. The patient fully recovered within 5 days. CONCLUSION: Girdle syndrome is a rare but severe adverse event associated with sickle cell disease that must be considered as differential diagnosis in patients with sickle cell disease.


Subject(s)
Abdomen, Acute/etiology , Anemia, Sickle Cell/diagnosis , Arterial Occlusive Diseases/diagnosis , Emigrants and Immigrants , Intestinal Pseudo-Obstruction/diagnosis , Intestine, Small/blood supply , Arterial Occlusive Diseases/therapy , Child , Exchange Transfusion, Whole Blood , Germany , Ghana/ethnology , Humans , Intestinal Pseudo-Obstruction/therapy , Male
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