• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.1
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• pp.14-18
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• 2017

X-linked dominant hypophosphataemic rickets(XLHR) is a hereditary disease generally transmitted as an X-linked dominant trait. Oral manifestations include poorly mineralized dentin, enlarged pulp chambers and root canals, early tooth loss. Also, spontaneous dental abscesses could occur in the absence of a history of trauma or caries. This report describes the clinical finding and dental treatment of a child, who came to the hospital for treatment caries but was refered to pediatrics because the child showed clinically short of status, bow-leg and radiographically enlarged pulp chamber and canal, there as diagnosed as XLHR. After the diagnosis, 13 years follow-up and treatment were performed.

• Imaging Science in Dentistry
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• v.43 no.1
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• pp.25-30
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• 2013

Purpose: This study was performed to determine the prevalence of bifid mandibular condyles (BMCs) in asymptomatic and symptomatic temporomandibular joint (TMJ) subjects with no traumatic history, and to assess their impact on clinical and radiographic manifestations of TMJ. Materials and Methods: A total of 3,046 asymptomatic and 4,378 symptomatic patients were included in the study. Cone-beam computed tomography (CBCT) images were reviewed for bifid condyles. T-tests were used to compare the frequency of BMCs when stratified by symptom, gender, and side. In BMC patients, the clinical features of pain and noise, osseous changes, and parasagittal positioning of the condyles were compared between the normally shaped condyle side and the BMC side using chi-squared tests. Results: Fifteen (0.49%) asymptomatic and 22 (0.50%) symptomatic patients were found to have BMCs. Among the bilateral cases, the number of condyles were 19 (0.31%) and 25 (0.29%), respectively. No statistically significant differences were found between asymptomatic and symptomatic patients, between female and male patients, or between the right and left sides (p>0.05). Compared with the normally shaped condyle side, the BMC side showed no statistically significant differences in the distribution of pain and noise, parasagittal condylar position, or condylar osseous changes, with the exception of osteophytes. In the symptomatic group, osteophytes were found more frequently on the normally shaped condyle side than the BMC side (p<0.05). Conclusion: BMCs tended to be identified as an incidental finding. The presence of BMC would not lead to any TMJ symptoms or cause osseous changes.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.13 no.1
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• pp.82-87
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• 1991

Hemifacial microsomia is a term used to describe a facial anomalies caused by the defect of anatomic structures originated from the first and the second branchial arches. The defect area encompasses some facial areas including mandible, facial muscles, masticatory muscles, cranial nerves, auricles, etc., and the degree of manifestations of the anomalies is extmely diverse. A 20-year-old man complaining of facial asymmetry and malocclusion visited our hospital. An orthognathic surgery was performed for the correction of hard tissue anomalies and then autogenous dermis-fat autotransplantation was done for the improvement of remaining soft tissue defect. The result was esthetically good and the case was presented here.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.92-96
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• 2018

Chronic mucocutaneous candidiasis (CMC) is characterized by increased susceptibility to chronic and recurrent infections of the skin, mucous membranes, and nails by Candida species. It is a primary immunodeficiency disorder that is difficult to diagnose because of its heterogeneous clinical manifestations and genetic background. A 20-month-old boy who did not grow in height for 3 months was diagnosed as having hypothyroidism and he had hepatitis which was found at 5 years old. He presented with persistent oral thrush and vesicles on the body, the cause of which could not be identified from laboratory findings. No microorganism was detected in the throat culture; however, the oral thrush persisted. Immunological tests showed that immunoglobulin (Ig) subclass IgG and cluster of differentiation (CD)3, CD4, and CD8 levels were within normal limits. We prescribed oral levothyroxine and fluconazole mouth rinse. The patient was examined using diagnostic exome sequencing at the age of 6 years, and a c.1162A>G (p.K388E) STAT1 gene mutation was identified. A diagnosis of CMC based on the STAT1 gene mutation was, thus, made. At the age of 8 years, the boy developed a malar-like rash on his face. We conducted tests for detection of antinuclear antibodies and anti-dsDNA antibodies, which showed positive results; therefore, systemic lupus erythematosus (SLE) was also suspected. Whole exome sequencing is important to diagnose rare diseases in children. A STAT1 gene mutation should be suspected in patients with chronic fungal infections with a thyroid disease and/or SLE.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.1
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• pp.139-144
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• 2009

Laron syndrome was first described by Dr. Laron. Administration of exogenous growth hormone failed to stimulate insulin-like growth factor-I(IGF-I) production which was related to postnatal growth, because these patients lacked receptors in the liver for this hormone. The diagnosis of this syndrome is based on the typical features of GH resistance such as normal or elevated serum GH, low serum IGF-I, and impaired IGF-I response to hGH. Laron syndrome patients showed characteristically severe postnatal growth failure and markedly reduced adult height. This report describes the oral and maxillofacial manifestations of children associated with Laron syndrome. Children with Laron syndrome have several dental and skeletal irregularities. Relatively little is known of the direct effect of Laron syndrome on dental development. Further research should be needed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.153-161
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• 2006

Purpose: This study was designed to investigate rotavirus infection by comparing the clinical characteristics in neonates and infants. Methods: We enrolled 104 neonates and 250 infants wiht gastroenteritis and a rotazyme test positive reaction at the Soonchunhyang University Bucheon Hospital from February 2001 to January 2003. Results: The seasonal peaks of infection in infants occurred from February to June. However, in neonates, it occurred from October to December due to nursery outbreaks. Diarrhea, vomiting, fever and convulsions were significant symptoms in infants; however, metabolic acidosis with dehydration, jaundice, irritability, apnea, bloody stool, gastric residual, grunting, poor oral intake, lethargy as well as fever and diarrhea were more common in the neonates. Upper respiratory infection, pneumonia and bronchitis were present in the infants; however, necrotizing enterocolitis was more commonly observed in the in neonates. Among the patients with rotaviral infection, formula feeding was more popular than breast milk feeding in both the neonates and infants; however, this finding was not statistically significant. Conclusion: Rotavirus can be a significant pathogen in neonates as well as infants. Neonates suffering from fever, poor oral intake, lethargy and apnea should be investigated for rotaviral infection. A new vaccine, rotaviral specific immunoglobulin and treatment guidelines are needed for eradicating rotavirus infection. Further studies on isolation, infection pathway, immune response and treatment of rotavirus are needed.

• Journal of the korean academy of Pediatric Dentistry
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• v.49 no.2
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• pp.180-187
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• 2022

The aim of this study was to investigate the risk factors associated with sleep disordered breathing (SDB) by comparing intraoral factors, body mass index (BMI), and medical history with pediatric sleep questionnaire (PSQ) findings. Seven hundred eighty-seven subjects aged between 7 to 11 years old were included. Their caregivers were asked to complete questionnaires. Oral manifestations including Angle's classification, overjet, and Brodsky tonsil grade were examined. Children with PSQ scores of more than 0.33 points were classified into the SDB high-risk group. Among the 787 subjects, 34 (4.3%) were classified into the SDB high-risk group. Children with allergic rhinitis, atopic dermatitis, excessive overjet, or large tonsil size had a significantly higher risk for SDB versus those without. Also, there was a significant difference in SDB risk according to BMI status. Gender, gestational age, breastfeeding, and Angle's classification were not associated with SDB. Children at high risk for SDB were predisposed to tonsillar hypertrophy, allergic rhinitis, obesity, and atopic dermatitis. Children with these factors could be candidates for early intervention to prevent the progression of SDB.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.2
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• pp.262-266
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• 2004

The parathyroid hormone plays a major role in the metabolism of calcium and phosphorus. In hypoparathyroidism the parathyroid glands are atrophied or absent associated with autoantibodies against parathyroid tissue. Pseudohypoparathyroidism is a metabolic disease caused by the disturbance in peripheral action of parathormone, but parathormone level is normal. In general, patients with pseudohypoparathyroidism have short stature, round face, brachydactylia, obesity, mental retardation, cataracts & ectopic calcifications on soft tissues. Dental manifestations are enamel hypoplasia, delayed eruption, blunting of root apex, hypodontia, pulp calcification, thickened lamina dura, excessive caries & malocclusion. In this case, intraoral examination showed enamel hypoplasia on the erupted permanent teeth & hypermobility on the remaining deciduous teeth. From the radiographic view severe dental anomalies were observed on canines and shortening and blunting of root apex was observed on mandibular incisors. Pathologic root resorption was also observed on deciduous teeth.

• Journal of Life Science
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• v.19 no.8
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• pp.1047-1054
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• 2009

Oral squamous carcinoma (OSC) cells present resistance to chemotherapeutic agents-mediated apoptosis in the late stages of malignancy. Advances in the understanding of bacterial toxins have produced new strategies for the treatment of cancers. It was demonstrated here that Pseudomonas aeruginosa exotoxin A (PEA) significantly decreased the viability of chemoresistant YD-9 cells in the apoptosis mechanism. Apoptotic manifestations were evident through changes in nuclear morphology and generation of DNA fragmentation. PEA treatment induced caspase-3, -6 and -9 cleavage, and activation. These events preceded proteolysis of the caspase substrates poly (ADP-ribose) polymerase (PARP), DNA fragmentation factor 45 (DFF45), and lamin A in YD-9 cells. The reduction of mitochondrial membrane potential, release of cytochrome c and SmacjDlABLO from mitochondria to cytosol, andtranslocation of AlF into nucleus were shown. While p53, p21 and $14-3-3{\gamma}$ were upregulated, cyclin Band cdc2 were downregulated by PEA treatment. Taken together, PEA induces apoptosis in chemoresistant YD-9 cells via activation of caspases, mitochondrial events and regulation of cell cycle genes.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.3 no.2
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• pp.91-96
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• 2007

Cleidocranial dysplasia(CCD) is a congenital genetic disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs, CCD was reported by Morand at first in 1766, And later, it was named cleidocranial dysostosis, cleidocranial dysplasia, Marie-sainton syndrome and mutational dysostosis. It is autosomal dominant disorder and there is no prevalence between man and woman. Until recent days, mutation of Runx2 in chromosome6p21 has known to be a main factor causing CCD. The specific clinical features of CCD are aplasia or hypoplasia of one or both clavicles and incomplete closing of fontanels and cranial sutures. Dental manifestations include retention of deciduous teeth, delayed eruption of permanent teeth, supernumerary teeth and cyst. Because there is no mental retardation and physical disability in CCD patients, they usually can not recognize their dental abnormality by the time of abolescence. So, after exfoliation of deciduous teeth, they usually live with edentulous status. It usually drives CCD patients to suffer from esthetic and functional problem. For this reason, CCD patients must be early diagnosed and improved in their appearance as well as masticatory function. So, surgical removal of supernumerary teeth and orthodontic eruption of the natural permanent teeth at adequate time is necessary.