POS-217 RENAL AMYLOIDOSIS IN AN EXCEPTIONNAL ASSOCIATION OF GLANZMANN’S THROMBASTHENIA AND CROHN'S DISEASE

Crohn's disease (CD), a polygenic disorder, is the major subtype of inflammatory bowel disease. There heterogeneity and many patients present extraintestinal manifestations. However, association crohn's with another genetic has not been previously described. We report exceptional CD Glanzmann’s thrombasthenia which rare inherited bleeding disorder. Indeed, diagnosis in patient kidney amylosis, we think secondary to CD, revealed two diseases Tunisian family. A 38 years old man was admitted August 2020 our Nephrology department for management severe nephrotic syndrome normal renal function. He born first-cousin consanguineous parents diagnosed Glanzmann's since childhood. His family history remarkable: mother maternal uncle had his sister have also thrombasthenia. At examination,blood pressure 09/60 mmHg anasarque complained chronic diarrhea. Given biopsy high risk procedure this Glanzmann thrombasthenia, decided perform salivary gland Congo red staining showed presence characteristic amyloid deposits. During hospitalization presented persistent diarrhea without fever. Enteroscann prominent wall thickening mucosal hyperemia encompassing 10 cm segment ileum. Colonoscopy found discontinuous distribution longitudinal ulcers small aphthous ulcerations typical CD. Biopsy performed because high. The amyloidosis as complication made. Treatment symptomatic introduction systemic corticosteroid therapy treatment Evolution marked by occurrence acute failure conjunction covid 19 infection. reported uncommon complicating remarkable marriage. It an hereditary never literature. believe that explanation must certainly exist. investigation ongoing.