Sickle cell disease

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 02/04/2015

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249 Sickle cell disease

Instruction

Examine this patient’s hands who has recurrent episodes of abdominal pain, precipitated by infection.

Salient features

History

• Bone pain

• Past history of strokes, fits

• Priapism

• Family history of similar problem

• Precipitating factors (infection, dehydration, cold, acidosis or hypoxia)

• Recurrent painful episodes.

Examination

• Afro-Caribbean patient

• Anaemia

• Digits of varying lengths (may be painful) (Fig. 249.1)

• Tell the examiner that you would like to examine the:

• urine for haematuria (renal papillary necrosis)
• leg for arterial ulcers
• abdomen for hepatomegaly
• heart for cardiomegaly and hyperdynamic circulation
• retina for neovascularization (which may cause blindness) (Fig. 249.2).
image

Fig. 249.1 Sickle-cell disease. Infarction in several of the metacarpals and proximal phalanges has resulted in bone destruction and swelling of the soft tissues.

(With permission from Adam et al. 2008.)

image

Fig. 249.2 Sickle-cell disease. (A) Macular ischaemia with the macular depression sign and perifoveal vascular remodelling. (B) Fluorescein angiogram of the same patient demonstrating an irregular and moth-eaten perifoveal capillary network and vascular telangiectasia.

(With permission from Yanoff, Duker 2008.)

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