Characterization of microvascular disease in pediatric sickle cell disease using nailfold capillaroscopy

Sickle cell disease (SCD) is a disorder with repetitive vaso-occlusive crises resulting in microvascular obstruction and tissue ischemia that may lead to multi-organ ischemia and dysfunction. Nailfold videocapillaroscopy (NFC) is an imaging technique utilized in clinical rheumatology to visualize ca...

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Published inMicrovascular research Vol. 136; p. 104150
Main Authors Bharara, Rabani, Browne, Rasheda, Seydafkan, Shabnam, Salciccioli, Louis, Rehman, Muzammil, Zhang, Yaoping, Tena, Meseret, Malhi, Princy, Hanono, Monique, Chen, Shannon X., Daich, Jonathan, Lazar, Jason M.
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LanguageEnglish
Published United States Elsevier Inc 01.07.2021
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Abstract Sickle cell disease (SCD) is a disorder with repetitive vaso-occlusive crises resulting in microvascular obstruction and tissue ischemia that may lead to multi-organ ischemia and dysfunction. Nailfold videocapillaroscopy (NFC) is an imaging technique utilized in clinical rheumatology to visualize capillaries located near the fingertip. To characterize NFC abnormalities in the setting of pediatric SCD, we performed NFC using a video capillaroscope on 8 digits in 44 stable SCD patients and 65 age matched healthy controls. Mean capillary number was lower (6.4 ± 1.3 vs 7.5 ± 1.8, p = 0.001) in the SCD group compared to controls. The percentage of dilated capillaries was similar (7.1 ± 8.3 vs. 5.9 ± 8.2, p = 0.4). The large majority of capillaries visualized in the SCD and control groups were normal capillary types per the EULAR definition, with a similar percentage of normal, nonspecific capillary morphologies and abnormal types. Regarding normal capillary sub-types, the SCD group and controls exhibited similar percentages of stereotype hairpin shapes, and tortuous or once or twice crossing type capillaries. On multivariate analyses, mean capillary number was independently associated with SCD after adjusting for age, body mass index, systolic blood pressure and gender. In conclusion, pediatric SCD is associated with lower capillary number but similar percentage of dilated capillaries and morphology on NFC. In our SCD cohort, capillary number was unrelated to our available markers of disease severity, including history of sickle crises, previous hospitalization for crises or Hemoglobin F levels. •Sickle cell disease and abnormal nailfold capillaroscopy in pediatric patients.•There is lower capillary density, but similar morphologic features to normals.•Nailfold capillary abnormalities are unrelated to biomarkers of disease severity.
AbstractList Sickle cell disease (SCD) is a disorder with repetitive vaso-occlusive crises resulting in microvascular obstruction and tissue ischemia that may lead to multi-organ ischemia and dysfunction. Nailfold videocapillaroscopy (NFC) is an imaging technique utilized in clinical rheumatology to visualize capillaries located near the fingertip. To characterize NFC abnormalities in the setting of pediatric SCD, we performed NFC using a video capillaroscope on 8 digits in 44 stable SCD patients and 65 age matched healthy controls. Mean capillary number was lower (6.4 ± 1.3 vs 7.5 ± 1.8, p = 0.001) in the SCD group compared to controls. The percentage of dilated capillaries was similar (7.1 ± 8.3 vs. 5.9 ± 8.2, p = 0.4). The large majority of capillaries visualized in the SCD and control groups were normal capillary types per the EULAR definition, with a similar percentage of normal, nonspecific capillary morphologies and abnormal types. Regarding normal capillary sub-types, the SCD group and controls exhibited similar percentages of stereotype hairpin shapes, and tortuous or once or twice crossing type capillaries. On multivariate analyses, mean capillary number was independently associated with SCD after adjusting for age, body mass index, systolic blood pressure and gender. In conclusion, pediatric SCD is associated with lower capillary number but similar percentage of dilated capillaries and morphology on NFC. In our SCD cohort, capillary number was unrelated to our available markers of disease severity, including history of sickle crises, previous hospitalization for crises or Hemoglobin F levels. •Sickle cell disease and abnormal nailfold capillaroscopy in pediatric patients.•There is lower capillary density, but similar morphologic features to normals.•Nailfold capillary abnormalities are unrelated to biomarkers of disease severity.
Sickle cell disease (SCD) is a disorder with repetitive vaso-occlusive crises resulting in microvascular obstruction and tissue ischemia that may lead to multi-organ ischemia and dysfunction. Nailfold videocapillaroscopy (NFC) is an imaging technique utilized in clinical rheumatology to visualize capillaries located near the fingertip. To characterize NFC abnormalities in the setting of pediatric SCD, we performed NFC using a video capillaroscope on 8 digits in 44 stable SCD patients and 65 age matched healthy controls. Mean capillary number was lower (6.4 ± 1.3 vs 7.5 ± 1.8, p = 0.001) in the SCD group compared to controls. The percentage of dilated capillaries was similar (7.1 ± 8.3 vs. 5.9 ± 8.2, p = 0.4). The large majority of capillaries visualized in the SCD and control groups were normal capillary types per the EULAR definition, with a similar percentage of normal, nonspecific capillary morphologies and abnormal types. Regarding normal capillary sub-types, the SCD group and controls exhibited similar percentages of stereotype hairpin shapes, and tortuous or once or twice crossing type capillaries. On multivariate analyses, mean capillary number was independently associated with SCD after adjusting for age, body mass index, systolic blood pressure and gender. In conclusion, pediatric SCD is associated with lower capillary number but similar percentage of dilated capillaries and morphology on NFC. In our SCD cohort, capillary number was unrelated to our available markers of disease severity, including history of sickle crises, previous hospitalization for crises or Hemoglobin F levels.
ArticleNumber 104150
Author Browne, Rasheda
Chen, Shannon X.
Bharara, Rabani
Salciccioli, Louis
Lazar, Jason M.
Hanono, Monique
Rehman, Muzammil
Zhang, Yaoping
Malhi, Princy
Daich, Jonathan
Tena, Meseret
Seydafkan, Shabnam
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  givenname: Rabani
  surname: Bharara
  fullname: Bharara, Rabani
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
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  givenname: Rasheda
  surname: Browne
  fullname: Browne, Rasheda
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
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  givenname: Shabnam
  surname: Seydafkan
  fullname: Seydafkan, Shabnam
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
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  surname: Salciccioli
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  givenname: Muzammil
  surname: Rehman
  fullname: Rehman, Muzammil
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
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  givenname: Yaoping
  surname: Zhang
  fullname: Zhang, Yaoping
  organization: Department of Pediatrics, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, Box 49, Brooklyn, NY 11203, USA
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  givenname: Meseret
  surname: Tena
  fullname: Tena, Meseret
  organization: Department of Pediatrics, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, Box 49, Brooklyn, NY 11203, USA
– sequence: 8
  givenname: Princy
  surname: Malhi
  fullname: Malhi, Princy
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
– sequence: 9
  givenname: Monique
  surname: Hanono
  fullname: Hanono, Monique
  organization: Department of Pediatrics, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, Box 49, Brooklyn, NY 11203, USA
– sequence: 10
  givenname: Shannon X.
  surname: Chen
  fullname: Chen, Shannon X.
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
– sequence: 11
  givenname: Jonathan
  surname: Daich
  fullname: Daich, Jonathan
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
– sequence: 12
  givenname: Jason M.
  surname: Lazar
  fullname: Lazar, Jason M.
  email: jason.lazar@downstate.edu
  organization: Department of Medicine, Division of Cardiovascular Medicine, State University of New York Downstate Health Sciences University, 450 Clarkson Avenue, MSC 1199, Brooklyn, NY 11203, USA
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Keywords Microvascular
Nailfold capillaroscopy
Sickle cell disease
Capillary
Language English
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Snippet Sickle cell disease (SCD) is a disorder with repetitive vaso-occlusive crises resulting in microvascular obstruction and tissue ischemia that may lead to...
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StartPage 104150
SubjectTerms Adolescent
Anemia, Sickle Cell - diagnostic imaging
Capillary
Case-Control Studies
Child
Child, Preschool
Cross-Sectional Studies
Female
Humans
Male
Microscopic Angioscopy
Microvascular
Microvascular Density
Microvessels - diagnostic imaging
Nailfold capillaroscopy
Nails - blood supply
Predictive Value of Tests
Sickle cell disease
Title Characterization of microvascular disease in pediatric sickle cell disease using nailfold capillaroscopy
URI https://dx.doi.org/10.1016/j.mvr.2021.104150
https://www.ncbi.nlm.nih.gov/pubmed/33647341
https://search.proquest.com/docview/2495409825
Volume 136
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