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Imaging
Findings
Features reveal bilateral renal infarcts, pulmonary and vertebral infarcts.
Papillary necrosis is noted in the left kidney (Fig 2).
Diagnosis
Sickle Cell Anaemia
Discussion
Sickle cell disease refers to a group of genetic disorders characterized
by the presence of sickle hemoglobin (HbS), anemia, and acute and chronic
tissue injury secondary to the blockage of blood flow by abnormally shaped
red blood cells.
The major causes of morbidity and mortality in patients with sickle cell
disease as they grow older is organ and tissue failure due to the cumulative
effects of chronic hemolysis and sickling. The heart enlarges in response
to chronic anemia, and cardio myopathy results. Coronary vessel disease
is unusual, and pulmonary infarcts can lead to progressive loss of pulmonary
function and cor pulmonale. Bone infacrts are also common and result in
osteopenia and secondary arthritis. Avascular necrosis of the femoral
head develops in approxiamtely 10 % to 15 % of the patients. Sickling
in the kidney results in microinfarctions and progressive loss of renal
function. Hematuria amd hyposthenuria ( decreased ability to concentrate
urine) are also noted. Priapism occurs in 5% of male patients which can
be recurrent and cause impotence. In female patients, complications during
pregnancy, particularly miscarriage, stillbirth, placental infarcts, preeclampsia,
and premature labor, occur more often than they do in the general population.
Dr.
Bhujang Pai, Hinduja Hospital, Mumbai
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