• Pediatric Infection and Vaccine
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• v.29 no.2
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• pp.96-104
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• 2022

Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory bone disorder presenting with sterile osteomyelitis, most often presenting in childhood. Although the etiology is understood incompletely, its association with other auto-inflammatory diseases including inflammatory bowel disease (IBD); psoriasis; Wegener's disease; arthritis; and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome suggests that dysregulated innate immunity may play an important role in the pathogenesis. We report a case of a 13-year-old boy with CRMO associated with Crohn's disease (CD) successfully treated with infliximab after failure of non-steroidal anti-inflammatory drug (NSAID) treatment. He initially was diagnosed with CRMO based on symmetric and aseptic bone lesions with no fever, lack of response to antibiotic treatment, vertebral involvement, and normal blood cell counts. Despite five months of NSAID treatment, his musculoskeletal symptoms were aggravated, and he developed gastrointestinal symptoms. Finally, he was diagnosed with CRMO associated with CD. Due to the severity of symptoms, infliximab was initiated and produced symptom improvement. This case supports infliximab as another choice for treatment of bowel symptoms in addition to the bone and joint symptoms of CRMO when other first-line treatments are ineffective.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.391-405
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• 2003

Craniosynostosis, known as a premature fusion of cranial sutures, is a developmental disorder characterized by precocious differentiation and mineralization of osteoblasts in the calvarial sutures. Recent genetic studies have demonstrated that mutation in the homeobox gene Msx2 causes Boston-type human craniosynostosis. Additionally, the phenotype of Dlx5 homozygote mutant mouse presents craniofacial abnormalities including a delayed ossification of calvarial bone. Furthermore transcription of osteocalcin, a mature osteoblast marker, is reciprocally regulated by the homeodomain proteins Msx2 and Dlx5. These facts suggest important roles of osteocalcin, Msx2 and Dlx5 genes in the calvarial bone growth and suture morphogenesis. To elucidate the function of these molecules in the early morphogenesis of mouse cranial sutures, we have first analyzed by in situ hybridization the expression of osteocalcin, Msx2 and Dlx5 genes in the developing parietal bone and sagittal suture of mouse calvaria during the embryonic (E15-E18) stage. Osteocalcin mRNA was found in the periosteum of parietal bones from E15, and gradually more highly expressed with aging. Msx2 mRNA was intensely expressed in the sutural mesenchyme, osteogenic fronts and mildly expressed in the dura mater during the embryonic stage. Dlx5 mRNA was intensely expressed osteogenic fronts and the periostem of parietal bones. To further examine the upstream signaling molecules of transcription factor Msx2 and Dlx5, we have done in vitro experiments in E15.5 mouse calvarial explants. Interestingly, implantation of BMP2-, BMP4-soaked beads onto the osteogenic fronts after 48 hours organ culture induced etopic expressions of Msx2 and Dlx5 genes. On the other hand, overexpression of $TGF{\beta}1$, GDF-6, -7, FGF-2, -4 and Shh did not induce the expression of Msx2 and Dlx5. Taken together. these data indicate that transcription factor Msx2 and Dlx5 play critical roles in the calvarial bone and suture development, and that BMP siganling is involved in the osteogenesis of calvarial bones and the maintenance of cranial sutures through regulating these two transcriotpn factors. Furthermore, different expression patterns between Msx2 and Dlx5 suggest their specific functions in the osteoblast differentiation.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.4
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• pp.651-658
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• 2004

Runx2 is a transcription factor in homologous with Drosophila runt gene and it is essential for bone formation during embryogenesis and a critical gene for osteoblast differentiation and osteoblast function. Runx2-haploinsufficency causes cleidocranial dysplasia (CCD). CCD is an autosomal-dominant inherited disorder characterized by hypoplastic clevicle and delayed ossification in fontanelles and wormian bones. Dental defects are possibly shown to CCD patients : multiple supernumerary teeth, irregular and compressed permanent tooth crowns, hypoplastic and hypomineralized defects in enamel and dentin, an excess of epithelial root remnants, the absence of cellular cementum, and abnormally shaped roots. In addition, delayed eruption of the secondary dentition is a constant finding. The aim of this study is to evaluate the role of Runx2 in the tooth development and eruption through analyzing the expression pattern of Runx2 by in situ hybridization during crown (late bell stage) and root formation of tooth, using postnatal day 1, 4, 7, 14 and 21 mice mandibular molar teeth. mRNA of Runx2-full length is expressed in dental follicle and surrounding tissue at postnatal day1 and 4. At postnatal day 7, it is expressed in ameloblasts of occlusal surface of enamel and bone area surrounding the tooth. In comparison with previous stage, at postnatal day 14, it is expressed in ameloblasts of proximal surface of enamel. At postnatal day 21 it's expression is observed only in bone area. mRNA of Runx2-typeII is not expressed. At postnatal day 1 and 7. At postnatal day 14 and 21, it's expression is observed in the bone area. In this study, we suggest that Runx2 have a relation of ameloblasts differentiation and an important role to tooth eruption made by dental follicle during intraosseous eruption stage. Also we can confirm that Runx2 has a role to bone formation.

• Journal of Life Science
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• v.17 no.12
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• pp.1654-1659
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• 2007

We examined total body irradiation (TBI)-induced effects by complete blood count (CBC) and fluorescence-activated cell sorter analysis (FACS) in the piglet following radiation irritation. A CBC included red blood cell count, white blood cell count, and platelet cell count. Four piglets were examined in this study and each piglet was divided by irradiation dose, two piglets with 4 Gy, two with 6 Gy, one with 8 Gy. All piglets showed leukopenia, thrombocytopenia after irradiation. In 6 and 8 Gy group, three piglets showed severe hemostatic disorder and gastrointestinal disorder suchas diarrhea and anorexia, and they died between 10 and 15 days after radiation irritation. In 4 Gy, two piglets showed no clinical sign after radiation injury, but persistent leukopenia was shown in blood examination. We suggest that a single TBI dose less than 6 Gy is adequate for conditioning piglet for bone marrow transplantation.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.94-98
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• 2010

Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.3
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• pp.372-380
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• 2004

Cleidocranial dysplasia(CCD) is a congenital disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs. Skeletal abnormalities in CCD are delayed closure of cranial suture and fontanelle, presence of wormian bone and clavicle aplasia. And CCD has an effect on the long bones, phalanges, spine, pelvis, muscles, and central nervous system. Dental manifestations include retention of deciduous teeth, multiple supernumerary teeth, delayed or absent eruption of permanent teeth and formation of cysts around nonerupted teeth. But as a result in common with a lack of medical and physical disability patient may have no substantive complaint, there are many masticatory and psychological problem by absent eruption of permanent teeth after exfoliation of deciduous teeth. For this reason CCD is necessary fo early diagnosis and must be improvement of the patient's appearance as well as provision of a functioning masticatory mechanism by treatment of surgical removal of supernumerary teeth, followed orthodontically eruption of the natural permanent teeth at adequate time.

• Journal of Korean Biological Nursing Science
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• v.4 no.2
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• pp.93-112
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• 2002

Recently, the number of the elderly has increased according to the improvement of socioeconomic status and the efficient medical care system. In spite of the development of medicine, the elderly suffers from the various health problems caused by weakness of musculoskeletal system, cardiopulmonary function and immunologic dysfunction. Regular rhythmic exercise program is known to be one of the effective tools to enhance the health condition in the elderly. However, there has been few studies to evaluate the comprehensive effects of rhythmic movement program on the elderly. This study was focused to evaluate the indices of cardiopulmonary function, life satisfaction, calcium, phosphorous, osteocalcin and deoxypyridinoline which are the essential factors of health problems in the elderly women. Twenty six subjects, aged between 68 and 72, who can do the ordinary activities and do not have cardiovascular dysfunction and mental disorder, participated in this study. They were divided into two groups: 13 in the experimental group and 13 in the control group. The experimental group participated in the rhythmic movement program at the welfare center located in G-city. The program were consisted of three sessions a week during 10 weeks. Each session had three parts: warming up(10 minutes), main exercise(40 minutes), finishing(10 minutes). Heart rate, blood pressure and peripheral arterial oxygen saturation were measured for the evaluation of cardiopulmonary function. Serum calcium, phosphorous, osteocalcin and urine deoxypyridinoline were measured as the indices of bony metabolism. Data were analyzed with mean, standard deviation, $x^2$-test, t-test, paired t-test using SPSS PC+ program. The results of this study were as follows. 1) Heart rate of the experimental group showed significant decrease following the rhythmic movement program. Peripheral arterial oxygen saturation of the experimental group showed significant increase following the program. 2) The degree of life satisfaction of the experimental group showed significant increase following the program. 3) Calcium showed significant decrease following the program but remained within normal range. There was no significant difference of phosphorous between two groups. 4) Osteocalcin, the index of bone formation, showed no significant change following the program, but significant increase in the experimental group comparing with the control group. 5) Deoxypyridinoline, the index of bone resorption, in urine of the experimental group showed significant decrease following the program. In conclusion, the rhythmic exercise program in the elderly showed the improvement of physiologic function and favorable effects on life satisfaction and bony metabolism. According to the above results, the regular rhythmic movement program can be strongly recommended for the improvement of health in the elderly women.

• The Journal of Korean Academy of Prosthodontics
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• v.29 no.2
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• pp.285-294
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• 1991

The human temporomandibular joint as a ginglymoarthrodial one has much in common with the other synovial joints of the body, but it does possess an unique charachteristic in that it must accomodate the various occlusal relations of dentition during an end point of closure. For that reason, the movement of the condyle in the temporomandibular joint is susceptible to influences from the nature of occlusion. Undue loading to the temporomandibular joint can be applied on the occasion of premature contacts in centric relation, balancing side interferences, change of occlusal surfaces due to excessive attrition, loss of tooth. Such occlusal disharmonies in association with the systemic and emotional factors may give rise to the temporomandibular disorder. On the other hand, the changes of occlusal patterns in the growing body can also have an effect on the growth of the temporomandibular joint through the alterations of functional stresses. The purpose of this study was to observe histopathologic response of the temporomandibular joint in unilateral chewing on one side exclusively for 10 months. Three dogs showing normal masticatory function were chosen. One dog aged about 12 months was for control, two dogs for experimental specimens were about 12 and 18 months old respectively. For chewing on the left side only, unilateral lower right premolar and molar were extracted in two experimental specimens. And then three dogs were sacrificed 10 months later. Frontal histologic sectioning of joints were done for the observation of the effects of one-side chewing. 24 specimens from three dogs were obtained and fixed in 10% formalin and routinly processed with H-E staining for histologic examination. The light microscopic findings were interpretated as follows: 1. Experimental specimen 1 aged about 22 months: In comparison with control and right non-chewing side, the proliferative and hypertrophic zone were increased at the mesial and lateral part of left chewing side condyle. There was no change of the articular tissue of temporal bone. 2. Experimental specimen 2 aged about 28 months: The articular tissues of adult joint were observed. The differences between the chewing and non-chewing side were not seen in the articular tissues of condyle and temporal bone.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• International Journal of Oral Biology
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• v.32 no.4
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• pp.119-125
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• 2007

Dlx3 is a homeodomain protein and is known to play a role in development and differentiation of many tissues. Deletion of four base pairs in DLX3 (NT3198) is causally related to tricho-dento-osseous (TDO) syndrome (OMIM #190320), a genetic disorder manifested by taurodontism, hair abnormalities, and increased bone density in the cranium. The molecular mechanisms that explain the phenotypic characteristics of TDO syndrome have not been clearly determined. In this study, we examined phenotypic characteristics of wild type DLX3(wtDlx3) and 4-BP DEL DLX3 (TDO mtDlx3) in C2C12 cells. To investigate how wtDlx3 and TDO mtDlx3 differentially regulate osteoblastic differentiation, reporter assays were performed by using luciferase reporters containing the promoters of alkaline phosphatase, bone sialoprotein or osteocalcin. Both wtDlx3 and TDO mtDlx3 enhanced significantly all the reporter activities but the effect of mtDlx3 was much weaker than that of wtDlx3. In spite of these differences in reporter activity, electrophoretic mobility shift assay showed that both wtDlx3 and TDO mtDlx3 formed similar amounts of DNA binding complexes with Dlx3 binding consensus sequence or with ALP promoter oligonucleotide bearing the Dlx3 binding core sequence. TDO mtDlx3 exhibits a longer half-life than wtDlx3 and it corresponds to PESTfind analysis result showing that potential PEST sequence was missed in carboxy terminal of TDO mtDlx3. In addition, co-immunoprecipitation demonstrated that TDO mtDlx3 binds to Msx2 more strongly than wtDlx3. Taken together, though TDO mtDlx3 acted as a weaker transcriptional activator than wtDlx3 in osteoblastic cells, there is possibility that during in vivo osteoblast differentiation TDO mtDlx3 may antagonize transcriptional repressor activity of Msx2 more effectively and for longer period than wtDlx3, resulting in enhancement of osteoblast differentiation.