Internal Medicine

0521779407-20 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:22

1418 Thalassemia Thiamine Deficiency

ulcers, folate deficiency, hypersplenism, progressive anemia, iron

overload secondary to increased iron absorption)

complications and prognosis

Complications

■Beta thalassemia major/intermedia:

➣osteoporosis

➣hypersplenism

➣cholecystitis

➣iron overload from increased intestinal absorption

➣if untreated severe anemia: heart failure, bone deformities, poor

growth, fatigue, hypogonadism.

■Untransfused beta thalassemia intermedia may develop folate defi-

ciency

Prognosis

■Alpha or beta thalassemia trait is not associated with morbidity or

mortality.

■Beta thalassemia intermedia follows a variable course, and may

develop increasing complications with age.

■Untransfused beta thalassemia major is fatal in childhood.

■Alpha thalassemia major is fatal in utero or in the neonatal period.

■In transfused patients, mortality is mainly related to complications

of blood transfusions and iron overload.

THIAMINE DEFICIENCY

ELISABETH RYZEN, MD

history & physical

History

■diet high in polished rice, alcoholism, lactation, pregnancy, malab-

sorption, liver disease, prolonged dextrose infusions

Signs & Symptoms

■irritability, sleep disturbances, abdominal complaints, peripheral

neuropathy, cardiomyopathy (beriberi), Wernicke-Korsakoff syn-

drome with mental confusion, ophthalmoplegia, coma