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Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report

INTRODUCTION: Cyclic neutropenia (CyN) is a rare hematological disease, and patients with CyN often experience an early onset of severe periodontitis and are forced to undergo tooth extraction. Here, we report a case of a patient with CyN who showed different periodicity and oscillations of neutroph...

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Autores principales: Aota, Keiko, Kani, Koichi, Yamanoi, Tomoko, Momota, Yukihiro, Ninomiya, Masami, Yumoto, Hiromichi, Azuma, Masayuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6775366/
https://www.ncbi.nlm.nih.gov/pubmed/31574885
http://dx.doi.org/10.1097/MD.0000000000017372
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author Aota, Keiko
Kani, Koichi
Yamanoi, Tomoko
Momota, Yukihiro
Ninomiya, Masami
Yumoto, Hiromichi
Azuma, Masayuki
author_facet Aota, Keiko
Kani, Koichi
Yamanoi, Tomoko
Momota, Yukihiro
Ninomiya, Masami
Yumoto, Hiromichi
Azuma, Masayuki
author_sort Aota, Keiko
collection PubMed
description INTRODUCTION: Cyclic neutropenia (CyN) is a rare hematological disease, and patients with CyN often experience an early onset of severe periodontitis and are forced to undergo tooth extraction. Here, we report a case of a patient with CyN who showed different periodicity and oscillations of neutrophil count compared with her mother, despite sharing the same novel genetic mutation. PATIENT CONCERNS: A 17-year-old Japanese girl who had been diagnosed with CyN shortly after birth presented to our hospital with a complaint of mobility of her teeth and gingivitis. Upon presentation, an intraoral examination was performed and revealed redness and swelling of the marginal and attached gingiva. Radiographs revealed extreme resorption of the alveolar bone and apical lesions in her mandibular lateral incisors. The patient's hematologic data demonstrated a lack of blood neutrophils (0/μL). The patient had no history of dental extraction, and her mother also had a history of CyN. DIAGNOSES: The patient was diagnosed with severe periodontitis that was associated with CyN. Gene testing showed a novel heterozygous mutation in exon 4 of the ELANE gene (c.538delC, p.Leu180Ser fsX11). INTERVENTIONS: Based on the clinical findings, we planned to extract the patient's mandibular lateral incisors. Although the tooth extraction was scheduled considering the cyclic variation in neutrophil count, the patient's neutrophil count was 0/μL on the day before the planned extraction. Therefore, granulocyte-colony stimulating factor (G-CSF) was administered to increase the patient's neutrophil count. On the day of the patient's admission for the tooth extraction, she presented with fever (body temperature, 38.5°C), tonsillitis, and stomatitis. The extraction was subsequently delayed, and the patient was administered antibiotics and G-CSF for 4 days. At this time, the neutrophil count increased to 750/μL, and the tooth extraction was carried out safely. OUTCOMES: The postoperative course was uneventful, and the healing process at the extraction site was excellent. CONCLUSION: There is a possibility that the periodicity and oscillations of neutrophil count may change with growth in patients with CyN. Therefore, it is important to frequently examine and treat patients with fluctuating neutrophil levels for the management of invasive dental treatment in patients with CyN.
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spelling pubmed-67753662019-10-07 Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report Aota, Keiko Kani, Koichi Yamanoi, Tomoko Momota, Yukihiro Ninomiya, Masami Yumoto, Hiromichi Azuma, Masayuki Medicine (Baltimore) 5900 INTRODUCTION: Cyclic neutropenia (CyN) is a rare hematological disease, and patients with CyN often experience an early onset of severe periodontitis and are forced to undergo tooth extraction. Here, we report a case of a patient with CyN who showed different periodicity and oscillations of neutrophil count compared with her mother, despite sharing the same novel genetic mutation. PATIENT CONCERNS: A 17-year-old Japanese girl who had been diagnosed with CyN shortly after birth presented to our hospital with a complaint of mobility of her teeth and gingivitis. Upon presentation, an intraoral examination was performed and revealed redness and swelling of the marginal and attached gingiva. Radiographs revealed extreme resorption of the alveolar bone and apical lesions in her mandibular lateral incisors. The patient's hematologic data demonstrated a lack of blood neutrophils (0/μL). The patient had no history of dental extraction, and her mother also had a history of CyN. DIAGNOSES: The patient was diagnosed with severe periodontitis that was associated with CyN. Gene testing showed a novel heterozygous mutation in exon 4 of the ELANE gene (c.538delC, p.Leu180Ser fsX11). INTERVENTIONS: Based on the clinical findings, we planned to extract the patient's mandibular lateral incisors. Although the tooth extraction was scheduled considering the cyclic variation in neutrophil count, the patient's neutrophil count was 0/μL on the day before the planned extraction. Therefore, granulocyte-colony stimulating factor (G-CSF) was administered to increase the patient's neutrophil count. On the day of the patient's admission for the tooth extraction, she presented with fever (body temperature, 38.5°C), tonsillitis, and stomatitis. The extraction was subsequently delayed, and the patient was administered antibiotics and G-CSF for 4 days. At this time, the neutrophil count increased to 750/μL, and the tooth extraction was carried out safely. OUTCOMES: The postoperative course was uneventful, and the healing process at the extraction site was excellent. CONCLUSION: There is a possibility that the periodicity and oscillations of neutrophil count may change with growth in patients with CyN. Therefore, it is important to frequently examine and treat patients with fluctuating neutrophil levels for the management of invasive dental treatment in patients with CyN. Wolters Kluwer Health 2019-09-27 /pmc/articles/PMC6775366/ /pubmed/31574885 http://dx.doi.org/10.1097/MD.0000000000017372 Text en Copyright © 2019 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 5900
Aota, Keiko
Kani, Koichi
Yamanoi, Tomoko
Momota, Yukihiro
Ninomiya, Masami
Yumoto, Hiromichi
Azuma, Masayuki
Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title_full Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title_fullStr Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title_full_unstemmed Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title_short Management of tooth extraction in a patient with ELANE gene mutation-induced cyclic neutropenia: A case report
title_sort management of tooth extraction in a patient with elane gene mutation-induced cyclic neutropenia: a case report
topic 5900
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6775366/
https://www.ncbi.nlm.nih.gov/pubmed/31574885
http://dx.doi.org/10.1097/MD.0000000000017372
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