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Article: An infant with severe congenital neutropenia presenting with persistent omphalitis: Case report and literature review

TitleAn infant with severe congenital neutropenia presenting with persistent omphalitis: Case report and literature review
Authors
KeywordsSevere congenital neutropenia
Issue Date2010
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
Citation
Hong Kong Journal Of Paediatrics, 2010, v. 15 n. 4, p. 289-298 How to Cite?
AbstractSevere congenital neutropenia (SCN) is a rare, heterogeneous group of inherited disorders of neutrophil precursors presenting with pyogenic infections and severe neutropenia in infancy. We hereby describe an infant with persistent omphalitis and severe neutropenia. The diagnosis of SCN was confirmed by typical bone marrow findings. However, the response to usual dose of granulocyte colony-stimulating factor (G-CSF) was suboptimal. The requirement of high dose G-CSF suggests a higher risk of malignant transformation into myelodysplastic syndrome or acute myeloid leukaemia, and close monitoring for clonal changes and progression to frank leukaemia is needed. In this article we discussed the clinical approach to omphalitis, a condition commonly encountered by paediatricians, as well as differential diagnosis of neutropenia in neonates and infants. We also summarise the molecular etiologies and pathogenesis underlying SCN, which has only been unraveled in the past few years. While majority of patients with SCN respond to G-CSF which significantly reduces the risk of infections and mortality, dosage of G-CSF should be carefully titrated. Patients requiring high-dose G-CSF should be closely monitored for developing malignant transformation, which is uniformly associated with poor prognosis despite chemotherapy and hematopoietic stem cell transplantation.
Persistent Identifierhttp://hdl.handle.net/10722/129321
ISSN
2015 Impact Factor: 0.194
2015 SCImago Journal Rankings: 0.123
References

 

DC FieldValueLanguage
dc.contributor.authorLee, PPWen_HK
dc.contributor.authorLee, TLen_HK
dc.contributor.authorHo, MHKen_HK
dc.contributor.authorChong, PCYen_HK
dc.contributor.authorSo, CCen_HK
dc.contributor.authorLau, YLen_HK
dc.date.accessioned2010-12-23T08:35:19Z-
dc.date.available2010-12-23T08:35:19Z-
dc.date.issued2010en_HK
dc.identifier.citationHong Kong Journal Of Paediatrics, 2010, v. 15 n. 4, p. 289-298en_HK
dc.identifier.issn1013-9923en_HK
dc.identifier.urihttp://hdl.handle.net/10722/129321-
dc.description.abstractSevere congenital neutropenia (SCN) is a rare, heterogeneous group of inherited disorders of neutrophil precursors presenting with pyogenic infections and severe neutropenia in infancy. We hereby describe an infant with persistent omphalitis and severe neutropenia. The diagnosis of SCN was confirmed by typical bone marrow findings. However, the response to usual dose of granulocyte colony-stimulating factor (G-CSF) was suboptimal. The requirement of high dose G-CSF suggests a higher risk of malignant transformation into myelodysplastic syndrome or acute myeloid leukaemia, and close monitoring for clonal changes and progression to frank leukaemia is needed. In this article we discussed the clinical approach to omphalitis, a condition commonly encountered by paediatricians, as well as differential diagnosis of neutropenia in neonates and infants. We also summarise the molecular etiologies and pathogenesis underlying SCN, which has only been unraveled in the past few years. While majority of patients with SCN respond to G-CSF which significantly reduces the risk of infections and mortality, dosage of G-CSF should be carefully titrated. Patients requiring high-dose G-CSF should be closely monitored for developing malignant transformation, which is uniformly associated with poor prognosis despite chemotherapy and hematopoietic stem cell transplantation.en_HK
dc.languageengen_US
dc.publisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.aspen_HK
dc.relation.ispartofHong Kong Journal of Paediatricsen_HK
dc.subjectSevere congenital neutropeniaen_HK
dc.titleAn infant with severe congenital neutropenia presenting with persistent omphalitis: Case report and literature reviewen_HK
dc.typeArticleen_HK
dc.identifier.emailLee, PPW:ppwlee@hku.hken_HK
dc.identifier.emailSo, CC:scc@pathology.hku.hken_HK
dc.identifier.emailLau, YL:lauylung@hkucc.hku.hken_HK
dc.identifier.authorityLee, PPW=rp00462en_HK
dc.identifier.authoritySo, CC=rp00391en_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.scopuseid_2-s2.0-78149439771en_HK
dc.identifier.hkuros183273en_US
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-78149439771&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume15en_HK
dc.identifier.issue4en_HK
dc.identifier.spage289en_HK
dc.identifier.epage298en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridLee, PPW=14048822200en_HK
dc.identifier.scopusauthoridLee, TL=24483772800en_HK
dc.identifier.scopusauthoridHo, MHK=36554116300en_HK
dc.identifier.scopusauthoridChong, PCY=36652345700en_HK
dc.identifier.scopusauthoridSo, CC=7102919978en_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK

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