URORADIOLOGY


Case 12 :
An eight-year-old female presents with abdominal lump.
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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