• Journal of the korean academy of Pediatric Dentistry
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• v.40 no.2
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• pp.133-140
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• 2013

Cyclic neutropenia is a hematologic disorder characterized by a marked decrease in the number of circulating neutrophils occurring at regular intervals and after this period, the level of neutrophils usually recovers to a normal range. The clinical symptoms of cyclic neutropenia include fever, malaise, headaches and oral findings associated with painful soft tissue ulceration where lips, tongue and gingiva are typically involved. A 4 year 1 month old boy was presented to the hospital. His chief complaint was mobility of his teeth and swollen gums. The patient had suffered from cyclic neutropenia. Clinical examination revealed evident decay on all primary teeth except for the mandibular anterior teeth and localized alveolar bone loss around mandibullar right and left first primary molars which have mobility was notable. The patient was diagnosed with multiple dental caries, gingivitis and localized periodontitis associated with cyclic neutropenia and treated based on it. The dental treatment, including regular tooth care and appropriate treatment of dental caries or gingivitis, is essential for patients suffering cyclic neutropenia. Especially, proper care at an early stage is needed for young patients to minimize the unwanted consequences for permanent teeth development.

• Clinical and Experimental Pediatrics
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• v.52 no.6
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• pp.633-642
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• 2009

Neutropenia is defined as an absolute neutrophil count (ANC) of <$1,500/{\mu}L$, and the severity of neutropenia generally can be graded as mild ($1,000-1,500/{\mu}L$), moderate ($500-1,000/{\mu}L$), or severe (<500/$\mu{L}$). This stratification aids in predicting the risk of pyogenic infection because the susceptibility to life-threatening infections is significantly increased in patients with prolonged episodes of severe neutropenia. Especially cancer-related neutropenia carry significant mortality. Neutropenia can develop under various conditions such as decreased bone marrow production, the sequestering of neutrophils, and increased destruction of neutrophils in the peripheral blood. Neutropenia is classified according to the etiology as congenital or acquired, with the latter further defined according to the etiology or pathology. The clinical result is increased risk for infection, which is directly proportional to the severity and duration of the neutropenia. The typical workup of neutropenia starts with a 6-week period in which complete blood counts are measured twice weekly to document the persistence of the neutropenia and whether a cyclic pattern is present. When persistent neutropenia is diagnosed and no spontaneous recovery occurs within 3 months, a more extensive evaluation is advised. Treatment is usually unnecessary for most patients with severe neutropenia, as the majority of patients have a good prognosis. However, for patients who have severe and frequent infections, treatment with filgrastim may prevent infectious complications and improve quality of life.

• Journal of Yeungnam Medical Science
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• v.40 no.3
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• pp.283-288
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• 2023

Severe chronic neutropenia is classified as severe congenital, cyclic, autoimmune, or idiopathic. However, there is a lot of uncertainty regarding the diagnosis of severe congenital neutropenia (SCN) and chronic idiopathic neutropenia, and this uncertainty affects further evaluations and treatments. A 20-year-old man presented with fever and knee abrasions after a bicycle accident. On admission, his initial absolute neutrophil count (ANC) was 30/µL. He had no medical history of persistent severe neutropenia with periodic oscillation of ANC. Although his fever resolved after appropriate antibiotic therapy, ANC remained at 80/µL. Bone marrow (BM) aspiration and biopsy were performed, and a BM smear showed myeloid maturation arrest. Moreover, genetic mutation test results showed a heterozygous missense variant in exon 4 of the neutrophil elastase ELANE: c597+1G>C (pV190-F199del). The patient was diagnosed with SCN. After discharge, we routinely checked his ANC level and monitored any signs of infection with minimum use of granulocyte colony-stimulating factor (G-CSF), considering its potential risk of leukemic transformation. Considering that SCN can be fatal, timely diagnosis and appropriate management with G-CSF are essential. We report the case of a patient with SCN caused by ELANE mutation who had atypical clinical manifestations. For a more accurate diagnosis and treatment of severe chronic neutropenia, further studies are needed to elucidate the various clinical features of ELANE.

• Journal of the Korean Academy of Esthetic Dentistry
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• v.13 no.2
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• pp.7-11
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• 2004

The Schwachman-Diamond syndrome is an autosomal recessive syndrome(1/20,000 births), consisting of pancreatic insufficiency, neutopenia, which may be intermittent, neutrophil chemotaxis defects, metaphyseal dysostosis, failure to thrive and short stature. Patients present in infancy with poor growth and grease, foul-smelling stools that are characteristic of malabsorption. These children can be readily differentiated from those with cystic fibrosis by their normal sweat chloride levels, lack of the cystic fibrosis gene, and characteristic metaphyseal lesions. Pathologically, the pancreatic acini are replaced by fat with little fibrosis. The neutropenia may be cyclic. Recurrent pyogenic infections otitis media, pneumonia, dermatitis(fig 1), sepsis are common and a frequent cause of death. In dental examination, these patients had a poor oral hygine and moderate generalized marginal gingivitis, also show delayed primary tooth exfoliation and oral development. This report illustrates a case that pancreatic agenesis 6 yeas-old boy with various esthetic dental problems has been served the esthetic dental restoration of 6 years.

• International Journal of Oral Biology
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• v.41 no.1
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• pp.17-23
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• 2016

Chronic/cyclic neutropenia, leukocyte adhesion deficiency syndrome, Papillon-$Lef{\grave{e}}vre$ syndrome, and $Ch{\grave{e}}diak$-Higashi syndrome are associated with severe periodontitis, suggesting the importance of neutrophils in the maintenance of periodontal health. Various Toll-like receptor (TLR) ligands are known to stimulate neutrophil function, including FcR-mediated phagocytosis. In the present study, the effect of TLR2 activation on the non-opsonic phagocytosis of oral bacteria and concomitant production of reactive oxygen species (ROS) by human neutrophils was evaluated. Neutrophils isolated from peripheral blood were incubated with Streptococcus sanguinis or Porphyromonas gingivalis in the presence of various concentrations of $Pam_3CSK_4$, a synthetic TLR2 ligand, and analyzed for phagocytosis and ROS production by flow cytometry and chemiluminescence, respectively. $Pam_3CSK_4$ significantly increased the phagocytosis of both bacterial species in a dose-dependent manner. However, the enhancing effect was greater for S. sanguinis than for P. gingivalis. $Pam_3CSK_4$ alone induced ROS production in neutrophils and also increased concomitant ROS production induced by bacteria. Interestingly, incubation with P. gingivalis and $Pam_3CSK_4$ decreased the amounts of ROS, as compared to $Pam_3CSK_4$ alone, indicating the possibility that P. gingivalis survives within neutrophils. However, neutrophils efficiently killed phagocytosed bacteria of both species despite the absence of $Pam_3CSK_4$. Although P. gingivalis is poorly phagocytosed even by the TLR2-activated neutrophils, TLR2 activation of neutrophils may help to reduce the colonization of P. gingivalis by efficiently eliminating S. sanguinis, an early colonizer, in subgingival biofilm.