Richard Idro1, Amelia K Boehme2, Michael Kawooya3, Samson K Lubowa4, Deogratias Munube1, Paul Bangirana5, Robert Opoka1, Ezekiel Mupere1, Angela Lignelli6, Philip Kasirye1, Nancy S Green7, Frank J Minja8. 1. Depts. of Paediatrics and Child Health. 2. Depts. of Neurology. Electronic address: akb2188@cumc.columbia.edu. 3. Radiology, Makerere University College of Health Sciences, Kampala, Uganda; Ernest Cook Ultrasound Research and Education Institute (ECUREI) Mengo Hospital, Kampala, Uganda. 4. Radiology, Makerere University College of Health Sciences, Kampala, Uganda. 5. Psychiatry. 6. Radiology, Columbia University Irving Medical Center, NY, USA. 7. Pediatrics. 8. Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, GA, USA.
Abstract
OBJECTIVE: Children with sickle cell anaemia (SCA) are highly susceptible to cerebrovascular injury. We performed brain magnetic resonance imaging and angiography (MRI-MRA) in Ugandan children with SCA to identify structural cerebrovascular abnormalities and examine their relationship to standardized clinical assessments. METHODS: A sub-sample (n=81) was selected from a cross-sectional study of children attending SCA clinic, including 52 (64.2%) with and 29 (35.8%) without clinically detected abnormalities. Clinical evaluation included assessment for prior stroke, cognitive testing and cerebral arterial transcranial doppler (TCD) flow velocity. MRI-MRA scans were interpreted by at least two neuroradiologists. RESULTS: Mean age was 6.5±2.7 years, with 39 (48.1%) female. Mean haemoglobin was 7.3±0.9 g/dl. Overall, 13 (16.0%) were malnourished. Infarcts and/or stenoses were detected in 55 (67.9%) participants, with stenoses primarily in the anterior circulation. Infarcts were seen in those with normal 17/29 (58.6%) or abnormal 34/52 (65.4%) clinical testing (p=0.181). Neither abnormal MRI nor MRA was associated with age, sex, haemoglobin, or malnutrition. Abnormal MRA was highly associated with infarcts (p<0.0001). Participants with abnormal imaging had two-fold higher proportion of stroke on exam and/or impaired cognition. Stroke on exam was strongly associated with an imaging abnormality after adjusting for age, sex, malnutrition, and haemoglobin (OR 11.8, 95%CI 1.87-74.2). CONCLUSION: Over half of these SCA children had cerebrovascular infarcts and/or arterial stenoses. Cerebrovascular disease was frequently undetectable by clinical assessments. While rarely available in under-resourced settings, MRI-MRA brain imaging is an important tool for defining SCA cerebrovascular disease and for assessing impact of clinical intervention trials.
OBJECTIVE: Children with sickle cell anaemia (SCA) are highly susceptible to cerebrovascular injury. We performed brain magnetic resonance imaging and angiography (MRI-MRA) in Ugandan children with SCA to identify structural cerebrovascular abnormalities and examine their relationship to standardized clinical assessments. METHODS: A sub-sample (n=81) was selected from a cross-sectional study of children attending SCA clinic, including 52 (64.2%) with and 29 (35.8%) without clinically detected abnormalities. Clinical evaluation included assessment for prior stroke, cognitive testing and cerebral arterial transcranial doppler (TCD) flow velocity. MRI-MRA scans were interpreted by at least two neuroradiologists. RESULTS: Mean age was 6.5±2.7 years, with 39 (48.1%) female. Mean haemoglobin was 7.3±0.9 g/dl. Overall, 13 (16.0%) were malnourished. Infarcts and/or stenoses were detected in 55 (67.9%) participants, with stenoses primarily in the anterior circulation. Infarcts were seen in those with normal 17/29 (58.6%) or abnormal 34/52 (65.4%) clinical testing (p=0.181). Neither abnormal MRI nor MRA was associated with age, sex, haemoglobin, or malnutrition. Abnormal MRA was highly associated with infarcts (p<0.0001). Participants with abnormal imaging had two-fold higher proportion of stroke on exam and/or impaired cognition. Stroke on exam was strongly associated with an imaging abnormality after adjusting for age, sex, malnutrition, and haemoglobin (OR 11.8, 95%CI 1.87-74.2). CONCLUSION: Over half of these SCA children had cerebrovascular infarcts and/or arterial stenoses. Cerebrovascular disease was frequently undetectable by clinical assessments. While rarely available in under-resourced settings, MRI-MRA brain imaging is an important tool for defining SCA cerebrovascular disease and for assessing impact of clinical intervention trials.
Authors: Scott D Grosse; Isaac Odame; Hani K Atrash; Djesika D Amendah; Frédéric B Piel; Thomas N Williams Journal: Am J Prev Med Date: 2011-12 Impact factor: 5.043
Authors: Lianna J Marks; Deogratias Munube; Philip Kasirye; Ezekiel Mupere; Zhezhen Jin; Philip LaRussa; Richard Idro; Nancy S Green Journal: Glob Pediatr Health Date: 2018-05-14
Authors: M R DeBaun; L C Jordan; A A King; J Schatz; E Vichinsky; C K Fox; R C McKinstry; P Telfer; M A Kraut; L Daraz; F J Kirkham; M H Murad Journal: Blood Adv Date: 2020-04-28
Authors: Nancy S Green; Deogratias Munube; Paul Bangirana; Linda Rosset Buluma; Bridget Kebirungi; Robert Opoka; Ezekiel Mupere; Philip Kasirye; Sarah Kiguli; Annet Birabwa; Michael S Kawooya; Samson K Lubowa; Rogers Sekibira; Edwards Kayongo; Heather Hume; Mitchell Elkind; Weixin Peng; Gen Li; Caterina Rosano; Philip LaRussa; Frank J Minja; Amelia Boehme; Richard Idro Journal: BMC Pediatr Date: 2019-10-25 Impact factor: 2.125