• Journal of the Korean Society of Radiology
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• v.81 no.3
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• pp.676-687
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• 2020

Purpose To analyze the altered brain regions and intrinsic brain activity patterns in trauma-exposed firefighters without posttraumatic stress disorder (PTSD). Materials and Methods Resting-state functional MRI (rsfMRI) was performed for all subjects. Thirty-one firefighters over 40 years of age without PTSD (31 men; mean age, 49.8 ± 4.7 years) were included. Twenty-six non-traumatized healthy controls (HCs) (26 men; mean age, 65.3 ± 7.84 years) were also included. Voxel-based morphometry was performed to investigate focal differences in the brain anatomy. Seed-based functional connectivity analysis was performed to investigate differences in spontaneous brain characteristics. Results The mean z-scores of the Seoul Verbal Learning Test for immediate and delayed recall, Controlled Oral Word Association Test (COWAT) score for animals, and COWAT phonemic fluency were significantly lower in the firefighter group than in the HCs, indicating decreased neurocognitive function. Compared to HCs, firefighters showed reduced gray matter volume in the left superior parietal gyrus and left inferior temporal gyrus. Further, in contrast to HCs, firefighters showed alterations in rsfMRI values in multiple regions, including the fusiform gyrus and cerebellum. Conclusion Structural and resting-state functional abnormalities in the brain may be useful imaging biomarkers for identifying alterations in trauma-exposed firefighters without PTSD.

• Clinical and Experimental Pediatrics
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• v.60 no.12
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• pp.403-407
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• 2017

Purpose: Quadriplegic children with cerebral palsy are more susceptible to osteoporosis because of various risk factors that interfere with bone metabolism. Pamidronate is effective for pediatric osteoporosis, but there are no guidelines for optimal dosage or duration of treatment in quadriplegic children with osteoporosis. We aimed to evaluate the efficacy of low-dose pamidronate treatment in these patients. Methods: Ten quadriplegic patients on antiepileptic drugs (6 male, 4 female patients; mean age, $10.9{\pm}5.76years$), with osteoporosis and gross motor function classification system level V, were treated with pamidronate (0.5-1.0 mg/kg/day, 2 consecutive days) every 3-4 months in a single institution. The patients received oral supplements of calcium and vitamin D before and during treatment. The lumbar spine bone mineral density (BMD) z score and biochemical markers of bone metabolism were measured regularly during treatment. Results: The main underlying disorder was perinatal hypoxic brain damage (40%, 4 of 10). The mean cumulative dose of pamidronate was $4.49{\pm}2.22mg/kg/yr$, and the mean treatment period was $10.8{\pm}3.32months$. The BMD z score of the lumbar spine showed a significant increase from $-4.22{\pm}1.24$ before treatment to $-2.61{\pm}1.69$ during treatment (P=0.008). Alkaline phosphatase decreased during treatment (P=0.037). Significant adverse drug reactions and new fractures were not reported. Conclusion: Low-dose pamidronate treatment for quadriplegic children with cerebral palsy increased lumbar BMD and reduced the incidence of fracture.

• Journal of Dental Rehabilitation and Applied Science
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• v.33 no.3
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• pp.216-222
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• 2017

Intellectual disability is an imperfect disorder in which mental development is permanently retarded and development of intellectual ability is inadequate. Having intellectual disabilities makes it difficult to handle their own work and adapt to social life. For this reason, it is difficult to hygienically manage the patient's oral condition, multiple caries are easily observed, and the periodontal disease incidence rate is high. The patient in this case is a 33-year-old female with a mental retardation first grade who had a problem with meals. In first visit, Patients had decreased occlusal vertical dimension and posterior bite collapse due to periodontitis and multiple caries. This case reports a satisfactory functional and esthetic results by reconstructing occlusion using partial removable dental prosthesis.

• Journal of Dental Rehabilitation and Applied Science
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• v.34 no.3
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• pp.239-245
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• 2018

If complication arises after glossectomy which leads to trouble in forming food bolus or transfer of the food, it is possible that either food bolus may block the airway or dysphagia may occur as the food bolus goes down into the airway. To solve the issue, palatal augmentation prosthesis could be used. In this case, the patient with an oral cancer is having difficulties swallowing food after glossectomy. Through taking impressions of polishing surface of his denture referring his tongue movement, the complete denture for the upper jaw was created using the concept of palatal augmentation prosthesis. This new upper denture increases the palatal-tongue contact pressure, allowing the patient to perform better swallowing and better pronunciation.

• Journal of Yeungnam Medical Science
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• v.27 no.1
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• pp.78-84
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• 2010

Adult-onset Still's disease (AOSD) is an inflammatory disorder that's characterized by daily, spiking high fever, arthritis and an evanescent, salmon-pink rash. AOSD is diagnosed purely on the basis of the typical clinical features of the illness. The symptoms commonly include swelling of the lymph nodes, enlargement of the spleen and liver, and a sore throat. AOSD is difficult to differentiate from systemic lupus erythematosus (SLE) due to the similar clinical manifestations. We report here on a case of a 16-year-old female patient with autism and epilepsy and who complained of daily spiking fever for 20 days. The patient had maculopapular skin rashes on the face and whole body and lymphadenopathy. The liver function tests were elevated mildly. The initial rheumatoid factor (RF) and antinuclear antibody (ANA) tests were negative. We diagnosed her as having adult-onset Still's disease according to the criteria of Yamaguchi. We successfully treated her with oral prednisolone. But her antinuclear antibody test was changed to positive after discharge. So we finally diagnosed her as having SLE.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.66-70
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• 2014

X-linked adrenoleukodystrophy (ALD) is a uncommon metabolic disorder which derived by peroxismal ${\beta}$-oxidation and elevation of serum very long chain fatty acid (VLCFA). VLCFA is mainly accumulated in the myelin of the central nervous system and adrenal cortex, by which the expressed symptoms of this disease are mainly neurologic and endocrinologic (such as adrenal insufficiency). The mutations in the ABCD1 gene causes X-linked ALD, nevertheless its phenotypes and genotypes are poorly coordinated. We report the case of a 12-year-old boy with X-linked ALD who developed vomiting, fatigue and poor oral intake. Severe dehydration and hyponatremia were found in initial physical examination and laboratory test, but his motor/sensory nerve function and mental status were completely normal. We diagnosed ALD with diffuse high-intensity signal in both parietotemporal cerebellar white matter in brain MRI and elevated serum VLCFA. Later, we confirmed a novel c.1635-1G>A (IVS6-1G>A) mutations of the ABCD1 gene. With the discrepancy between its phenotypes and genotypes, various phenotypes could be seen in X-ALD patient. Careful examination and further studies for these patients will be needed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.15 no.1
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• pp.60-64
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• 2019

Swyer-James syndrome (SJS), also known as Swyer-James-MacLeod syndrome and unilateral hyperlucent lung syndrome, is rare acquired pulmonary disorder develops secondary to infectious etiologies in early childhood. Viral respiratory infection such as adenoviruses or Mycoplasma pneumoniae in infancy or early childhood rarely cause Swyer-James syndrome. It is generally characterized on radiographs by a unilateral small lung with hyperlucency and air trapping on expiration. In many cases unaffected lung tissue functions normally, compensating for affected lung portion. Preoperative assessment is needed to determinate individual's pulmonary function. A 4-year-old boy with Swyer-James syndrome visited Seoul National University Dental Hospital Department of pediatric dentistry for caries treatment. Clinical and radiographic examinations revealed multiple carious lesions on deciduous teeth. Considering patient's underling disease, age, and level of cooperation, dental treatment under general anesthesia was scheduled. Dental treatment was done with composite resin and stainless-steel crown. Since ventilation of Swyer-James syndrome patients was diminished because of airway obstruction, close monitoring of ventilation is necessary during dental treatment. Considering pulmonary pathology, general anesthesia rather than sedation is recommended when special behavior management is required for dental treatment. Swyer-James syndrome patients can tolerate general anesthesia and surgery well, according to several reports.

• Journal of Dental Rehabilitation and Applied Science
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• v.31 no.2
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• pp.86-95
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• 2015

Purpose: The aim of the study was to evaluate the subjective symptoms and clinical signs through the TMD-questionnaire, clinical examination and radiography against the many instrumental performers and to investigate the association between playing instruments and TMDs. Materials and Methods: A total of 803 instrumental performers received TMD-related questionnaire and evaluations of prevalence and disease distribution were performed. Among those who reported at least one symptom of TMD, 70 volunteers visited in clinic then received clinical examination and radiography for diagnosis and evaluations of prevalence and disease distribution were performed. 70 subjects were divided into three groups as woodwind, brass wind, string. Comparative analysis of disease distribution was performed. Results: Among 803 instrumental performers, 610 people (75.97%) were reported to one or more symptoms of TMD. The most frequent symptom was click (29.68%).70 subjects underwent a clinical examination and radiography survey results, the most frequent symptom was a click (29.75%). Most commonly diagnosed disease was a myofacial pain (30.53%).Comparison of the three groups, a significant difference was not observed in the clinical sign. But among subject symptom, muscle pains howed significant differences in accordance with the Group (P = 0.024). During the 70 people who underwent clinical examination, 66 people (94.3%) showed moderate to severe attrition, mild to severe tongue ridging, mild to severe cheek ridging assigns of parafunction. Conclusion: Instrumental performers showed a high prevalence of TMD and the most of the musicians with temporomandibular disorder had bruxism or clenching habits.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.601-606
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• 2009

Amelogenesis Imperfecta (AI) is a genetic disorder which retards the development of enamel and it can be classified into three types: hypoplastic, hypomaturation, hypocalcified type. This can occur both in deciduous and permanent dentition. A 8 year 8 month old patient with a chief complaints of delayed eruption on upper anteriors, calculus deposit on lower anteriors and anterior openbite visited the clinic. Anteriors had thin layer of enamel and were very narrow. Especially lower anteriors had rough surface and were in bad shape. Teeth were very hypersensitive to thermal changes. Upper and lower first molars showed severe attrition on the occlusal surface. Radiographs also verified hypoplastic enamel in the whole dentition including the teeth in the tooth bud. The patient was diagnosed as hypoplastic AI, and is being treated at the pediathc and prosthodontic department of the Kyunghee dental university hospital. To improve the function, esthetics, hypersensitivity of the AI patients, restorations on the posteriors and the anteriors with oral hygiene instruction are necessary, Constant follow-up check is needed until full growth and after full growth, cooperative care with the other department is needed.

• Clinical and Experimental Pediatrics
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• v.51 no.11
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• pp.1211-1216
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• 2008

Purpose : Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. Methods : Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. Results : Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2-8). All patients responded to initial treatment with steroids and had a hemoglobin level ${\geq}9g/dL$ with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone (<5 mg per day) was required to maintain hemoglobin ${\geq}9mg/dL$ in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. Conclusion : The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.