Features of transfusion haemosiderosis in hereditary anaemia

• Growth retardation in second decade.

• Hypogonadism—delayed or absent sexual maturation.

• Skin pigmentation—slate grey or bronze discolouration.

• Hepatic dysfunction—hepatomegaly, chronic hepatitis, fibrosis, cirrhosis, hepatocellular carcinoma.

• Diabetes mellitus.

• Other endocrine dysfunction—rarely hypothyroidism, hypoparathyroidism, adrenal insufficiency.

• Cardiac dysfunction —cardiomyopathy, heart failure, dysrhythmias (main cause of death).

• Death from heart disease in adolescence.

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