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Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease
BACKGROUND: The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair st...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8885120/ https://www.ncbi.nlm.nih.gov/pubmed/35237076 http://dx.doi.org/10.2147/IMCRJ.S345847 |
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author | Ogwang, Eugene Odongo, Charles Newton Namusisi, Jane Okello, Patrick Ambrose Acan, Moses |
author_facet | Ogwang, Eugene Odongo, Charles Newton Namusisi, Jane Okello, Patrick Ambrose Acan, Moses |
author_sort | Ogwang, Eugene |
collection | PubMed |
description | BACKGROUND: The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Hb electrophoresis confirmed the diagnosis of sickle cell anemia. CASE SUMMARY: We present a 9-year-old Ugandan girl who presented with an unexplained stroke that preceded an episode of acute headache, vomiting, followed by focal convulsions and altered consciousness. Clinical findings revealed severe pallor of the conjunctivae and mild scleral icterus. CT demonstrated right cerebral intraparenchymal hemorrhage, multiple high cerebral infarcts on the right and evidence of extra medullary hematopoiesis with a classical HOE sign. Hemoglobin (Hb) electrophoresis confirmed sickle cell disease (SCD). The child was then initiated on hydroxyurea, antibiotics, analgesics and intravenous fluids. She improved and was discharged 16 days later. Follow-up of the child was uneventful. CONCLUSION: HOE sign is a complication of chronic hemolysis usually seen in patients with thalassemia and sickle cell anemia. It is a rare finding that clinicians should be well conversant with, especially in Africa where sickle cell disease is common. |
format | Online Article Text |
id | pubmed-8885120 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Dove |
record_format | MEDLINE/PubMed |
spelling | pubmed-88851202022-03-01 Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease Ogwang, Eugene Odongo, Charles Newton Namusisi, Jane Okello, Patrick Ambrose Acan, Moses Int Med Case Rep J Case Report BACKGROUND: The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Hb electrophoresis confirmed the diagnosis of sickle cell anemia. CASE SUMMARY: We present a 9-year-old Ugandan girl who presented with an unexplained stroke that preceded an episode of acute headache, vomiting, followed by focal convulsions and altered consciousness. Clinical findings revealed severe pallor of the conjunctivae and mild scleral icterus. CT demonstrated right cerebral intraparenchymal hemorrhage, multiple high cerebral infarcts on the right and evidence of extra medullary hematopoiesis with a classical HOE sign. Hemoglobin (Hb) electrophoresis confirmed sickle cell disease (SCD). The child was then initiated on hydroxyurea, antibiotics, analgesics and intravenous fluids. She improved and was discharged 16 days later. Follow-up of the child was uneventful. CONCLUSION: HOE sign is a complication of chronic hemolysis usually seen in patients with thalassemia and sickle cell anemia. It is a rare finding that clinicians should be well conversant with, especially in Africa where sickle cell disease is common. Dove 2022-02-24 /pmc/articles/PMC8885120/ /pubmed/35237076 http://dx.doi.org/10.2147/IMCRJ.S345847 Text en © 2022 Ogwang et al. https://creativecommons.org/licenses/by-nc/3.0/This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/ (https://creativecommons.org/licenses/by-nc/3.0/) ). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). |
spellingShingle | Case Report Ogwang, Eugene Odongo, Charles Newton Namusisi, Jane Okello, Patrick Ambrose Acan, Moses Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title | Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title_full | Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title_fullStr | Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title_full_unstemmed | Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title_short | Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease |
title_sort | hair-on-end sign in a 9-year-old girl presenting with acute stroke in sickle cell disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8885120/ https://www.ncbi.nlm.nih.gov/pubmed/35237076 http://dx.doi.org/10.2147/IMCRJ.S345847 |
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