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

• Journal of Gastric Cancer
• /
• v.2 no.3
• /
• pp.140-144
• /
• 2002

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.13 no.1
• /
• pp.23-27
• /
• 2017

Ectodermal Dysplasia is a genetic disorder caused by the abnormal development of two or more structures derived from the ectodermal layer. As an aspect in dentistry, ectodermal dysplasia is characterized by hypodontia, conical or peg-shaped teeth, reduced salivary secretion and decreased vertical dimension. These unfavorable oral conditions make children to have difficulties with mastication, esthetics, and even in social activities. This case report presents an alternative oral rehabilitation treatment of a 4-year-old boy with ectodermal dysplasia. A removable space maintainer with artificial teeth in maxillary arch was delivered according to the initial treatment plan. However, the child failed to adapt the appliance because of his masticatory habit. Then a fixed-type space maintainer was delivered on the patient's esthetic demand and it restored function, esthetics and self-esteem of the patient. The treatment described here suggests that individually customized considerations are essential for the oral rehabilitation of a patient with ectodermal dysplasia.

• Clinical and Experimental Pediatrics
• /
• v.52 no.2
• /
• pp.181-186
• /
• 2009

Purpose : Inflammation plays a potential role in the pathogenesis of bronchopulmonary dysplasia (BPD). Strategies for preventing BPD include respiratory management, antioxidants, nutritional treatment, and others such as anti-inflammatory agents. We aimed to assess the safety, tolerability, and efficacy of montelukast (MK), a cysteinyl leukotriene 1 receptor antagonist, as an add-on therapy in BPD. Methods : In addition to currently available standard measures such as oxygen supplementation, bronchodilators, nutritional support, and/or diuretics, montelukast was administered to 15 preterm infants with BPD. MK was given orally (1 mg/kg/d) for a mean period of 12 weeks. We compared safety and efficacy parameters with historical controls. Results : All 15 patients survived, and no differences were found in the incidence of adverse reactions between the 2 groups. The ventilation index was significantly improved after 2 weeks in MK group compared with historical controls. There were no significant differences in other respiratory parameters (MAP, oxygen dependency, and ventilator dependency) between the groups, but the MK group showed trends of greater improvement. Conclusion : Administration of MK 1 mg/kg/d was well tolerated in preterm BPD patients as an add-on therapy. We demonstrated that after 2 weeks of MK administration of 1 mg/kg/d, MK had beneficial therapeutic effects on BPD patients as an add-on to the standard therapy. Further multicenter randomized controlled clinical trials are needed to confirm the efficacy and safety of MK as a useful supplement to standard therapy for BPD patients.

• The Journal of the Korean bone and joint tumor society
• /
• v.13 no.1
• /
• pp.22-30
• /
• 2007

Purpose: We describe clinical, radiographic, MRI and pathologic findings as well as final outcome after simple curettage and bone graft of cystic fibrous dysplasia (FD) in the long bone, which has been rarely documented in the literature. Materials and Methods: Clinical records, radiographs, MRI and histologic slides of 11 patients with cystic FD in the long bone were retrospectively analyzed. Results: Six patients complained pain for several months, 4 patients presented pain after trivial injury event, and 1 patient suffered pathologic fracture. The mode of involvement was monostotic in 10 patients and polyostotic in l patient. The femur was affected in 7 patients, the humerus in 3, and the radius in 1. Radiography showed prominent, expansive lysis associated with ground-glass density of FD. MRI revealed 2 different signals of FD and cyst. Microscopic examination revealed classic findings of FD and non-specific cystic degeneration. The final outcome was satisfactory in every patient. Local recurrence was not observed. Conclusion: Cystic FD in the long bone seems not as rare as the scarcity of reported cases would indicate. MRI features provide a basis for differential diagnosis between benign cystic change and malignant transformation. Cystic FD would be an indication for surgery and simple curettage with allo-chip-bone graft is effective.

• Clinical and Experimental Pediatrics
• /
• v.50 no.3
• /
• pp.255-261
• /
• 2007

Purpose : The objective of this study is to observe high-resolution computed tomography (HRCT) findings of lung parenchyme in very low birth weight (VLBW) infants between the corrected age of 38-42 weeks who were treated with oxygen after birth, and to compare them to the clinical severity of bronchopulmonary dysplasia (BPD). Methods : The lungs of fourty-four VLBW infants with gestational ages of less than 32 weeks and birth weights of less than 1,500 g who were treated with oxygen after birth were examined using HRCT taken when the corrected age was between 38-42 weeks. Common findings among the infants and the frequency of their occurrences were noted. Total CT scores obtained by the summation of air trapping and actelectasis scores and the ratio of bronchus-to-pulmonary artery diameter were used to quantitatively evaluate HRCT findings and correlate them with the clinical severity of BPD as defined by Jobe-Bancalari diagnostic criteria. Results : 1) The most common findings in HRCT images of the lungs were air trapping (56%), atelectasis (70.5%), linear opacity (77%), and distortion of the bronchopulmonary bundle (65.9%). These findings were more commonly observed in infants with BPD in a mixed pattern than those without (P<0.05). However, abnormal findings were also found in HRCT images of some infants without BPD. In infants with BPD, air trapping, atelectasis and total CT scores were higher than those without BPD. Also infants with BPD had a lower bronchus-to-pulmonary artery diameter than those without BPD (P<0.05). 2) The total CT scores (r=0.799, P<0.0001) and the ratio of bronchus-to-pulmonary artery diameter (r=0.576, P<0.0001) showed a linear correlation with the clinical severity of BPD. Conclusion : HRCT findings in VLBW infants between the corrected age of 38-42 weeks who had been treated with oxygen after birth are useful in revealing pathologic changes in the lung parenchyme and show a good correlation with the clinical severity of BPD.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.35 no.4
• /
• pp.737-743
• /
• 2008

Regional odontodysplasia is a relatively rare developmental anomaly of dental hard tissue with characteristic clinical, radiographic and histologic features. It requires a continuous and multidisciplinary approaches, and the aim of treatment for these patients should include aiding mastication, improving aesthetics, maintaining normal vertical dimension and space, allowing normal jaw growth and eruptional management of affected teeth. This report describes three cases of regional odontodysplasia with 2-5 years of follow-up. Conservative treatment is chosen to preserve the affected teeth as long as possible, and periodic radiographic and clinical examination was done. During this time, all teeth except one showed progressive development. An interesting finding observed in our cases was that each tooth even in the same person showed different degree of tooth development and eruption rate. Thus, we colcluded that the treatment plan for regional odontodysplasia should be conservative and individualized and based on the assessment of each tooth.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.27 no.2
• /
• pp.222-228
• /
• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.28 no.4
• /
• pp.626-632
• /
• 2001

Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).

• The Journal of the Korean bone and joint tumor society
• /
• v.10 no.1
• /
• pp.50-55
• /
• 2004

Fibrodysplasia ossificans progressiva is a very rare genetic disorder, but is possible to diagnose with mass on neck or scalp in early neonate or child and accompanying characteristic congenital malformation of great toe. But because inappropriate treatment and complications from misdiagnosis may aggravate the progress of the disease, so the disorder require careful inspection for accurate diagnosis. We describe a case that was misdiagnosed properly and treated inappropriately and the natural history of the disease in adult.