ROGERS SYNDROME-THIAMINE RESPONSIVE MEGALOBLASTIC ANEMIA SYNDROME: CASE REPORT
Thiamine responsive megaloblastic anemia (TRMA) encompasses a complex syndrome of anemia, sensorineural hearing loss and Diabetes Mellitus. We report a 06 years old male child with pallor, bilateral sensorineural hearing loss, Diabetes Mellitus, megaloblastic anemia, retinitis pigmentosa and a normal response to water deprivation test. Child responded well to high doses of thiamine treatment. Rogers Syndrome, a spectrum disease, should be kept in mind in differential diagnosis of Diabetes Mellitus and megaloblastic anemia in population with frequent consanguinity. Thorough medical and family history and physical examination are vital for diagnosis. Prompt diagnosis, use of careful clinical monitoring and supportive care can relieve the debilitating symptoms.
Key Words: Diabetes Mellitus, Megaloblastic anemia, Sensorineural hearing loss, Rogers syndrome.
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