Muhammad Sohaib Ejaz Khan, Arjumand Saleem, Rizwan Aziz Qazi, Shajee Ahmed Siddiqui.
Spectrum of Clinical Presentation in Sickle Cell Disease Patients.
Ann Pak Inst Med Sci Jan ;10(2):67-70.

Objectives: To determine the spectrum of clinical presentation in patients with SCD. Study Design: Cross-sectional study Place and Duration: Department of Internal Medicine, Prince Salman Northwest Armed Forces Hospital, Kingdom of Saudi Arabia. Data was collected for six months. Material and Methods: Total 41 patients of either gender with age more than 15 years who were diagnosed cases of SCD were recruited. The diagnosis of SCD was established by cellulose acetate electrophoresis with citrate agar electrophoresis. All the admissions were recorded during the study period. Diagnosis of different clinical presentations was established by clinical examination and other necessary investigations where needed. Results: Data of 41 patients was analyzed with mean age of 24.27 years ± 4.29 SD. There were 28 males and 13 females with male to female ratio 2.15. Total number of hospital admissions was 260 during the study period. Frequency of different clinical presentations were vaso-occlusive crisis 67.7% (n=176), painful crisis 4.0% (n=11), hemolytic crisis 3% (n=8), priapism 3% (n=8), chest infection 3% (n=8), stroke 2% (n=5), aplastic crisis 2% (n=5), urinary tract infection 1.5% (n=4), sequestration crisis 1.5% (n=4), upper respiratory tract infection 1.5% (n=4), gall bladder stones 1.5% (n=4). Less than 10% (n=23) of times less common presentations like hand & foot disease, dysmennohrea, deep vein thrombosis, avascular necrosis, bone crisis, hemochromatosis leading to cardiomyopathy and cirrhosis, obstructive jaundice, acute cholycystitis, osteomyelitis and acute pancreatitis were seen. Conclusion: Spectrum of clinical presentations varies widely among SCD patients with vaso-occlusive crisis came out to be the most common manifestation followed by painful and hemolytic crisis. By adopting a comprehensive care approach, morbidity and mortality from complications of sickle cell disease can be significantly reduced.

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