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Multiorgan involvement in thalassaemia major
  1. S Ghai1,
  2. R Sharma1,
  3. S Ghai1,
  4. B Kulshreshtha2
  1. 1Department of Radio-diagnosis, All India Institute of Medical Sciences, New Delhi, India
  2. 2Department of Endocrinology
  1. Correspondence and reprint requests to:
 Dr Sangeet Ghai; 
 sangeet_ghai{at}yahoo.com

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Answers on p 361.

A 16 year old girl with a history of β-thalassaemia major since birth was admitted as an emergency to hospital with a three day history of dyspnoea on exertion, facial puffiness, and carpopedal spasm. Physical examination revealed a thin, short girl with frontal bossing. The child was pale with greyish skin pigmentation, had not attained menarche, and there were no secondary sexual characteristics. There was an early systolic murmur along the left sternal border. Abdominal examination revealed hepatosplenomegaly.

Magnetic resonance imaging (MRI) was done and the scans are shown in fig 1.

Figure 1

(A) Fast spin echo T2-weighted coronal MRI of the sella; (B) spin echo T1-weighted sagittal MRI of the sella; and (C) gradient echo T2-weighted coronal MRI.

QUESTIONS

  1. What are the findings on the MRI images?

  2. What is the diagnosis?

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