Transcranial doppler and brain MRI in children with sickle cell disease and high hemoglobin F levels

Abstract

The mild phenotype among Kuwaiti patients with SCD is reflected in normal TCD velocities and a low prevalence of SBI in children with the disease.

Transcranial Doppler Ultrasound in Peninsular Arab Patients With Sickle Cell Disease

“…The earlier study from Kuwait that documented transcranial Doppler findings in pediatric patients with SCD and the predominant AI haplotype did not report any patient with abnormal or conditional velocities in all of the arteries of the circle of Willis that were interrogated. We were not certain whether this finding was a reflection of the rarity of cerebral vasculopathy or whether there is, indeed, a regional or ethnic variation.…”

“…The study patients were consecutive patients seen in the Pediatric Hematology Clinics of Al‐Amiri and Mubarak Al‐Kabeer Hospitals in Kuwait, Royal Hospital in Muscat, Oman, Center for Hereditary Blood Diseases, Basra Maternity and Children Hospital in Basra, Iraq, and Tawam Hospital in Al Ain, United Arab Emirates. Although the Kuwaiti patients were part of a previous study, the patients from the other 3 countries had not been reported before. The patients were in a steady state at the time of the study (ie, no crisis, acute illness, or blood transfusion within the preceding 6 weeks).…”

“…Table shows reported transcranial Doppler values from the United States, France, Brazil, Tanzania, Nigeria, and Iran in which the proportions with abnormal values ranged from 1.6% to 20.7%. However, the 2 studies from the Arabian Peninsula (Oman and Kuwait) did not record any abnormal values, whereas the study from Iran reported 3% . The question therefore arises of whether the results from these countries could be reproduced among other Peninsular Arabs, and whether this variation is related to the β S haplotypes, HbF level, and hydroxyurea therapy or race/ethnicity.…”

Pattern of Renal Blood Flow and Renovascular Parameters in Adult Patients With Sickle Cell Disease

“…This was attributed to the high HbF level, which is generally greater than 30% up to the age of 4 years and remains at about 15% to greater than 20% among adults . This delays the vasculopathy that causes many of the SCD complications such as overt stroke, silent infarcts, acute chest syndrome, priapism, and leg ulcers . In addition, the coexistent β‐thalassemia trait, another modulatory factor, is found in about 40% of Kuwaiti patients …”

“…9 This delays the vasculopathy that causes many of the SCD complications such as overt stroke, silent infarcts, acute chest syndrome, priapism, and leg ulcers. [10][11][12][13] In addition, the coexistent β-thalassemia trait, another modulatory factor, is found in about 40% of Kuwaiti patients. 6 Sickle cell disease is a progressive disease; the rate of development of various complications is variable; and many patients develop organ damage in adulthood.…”