• Journal of Korean Neurosurgical Society
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• v.30 no.4
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• pp.501-508
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• 2001

Objectives : Hemifacial spasm is painless uncommon disorder characterized by involuntary paroxysmal movement on one side of face. It is known that hemifacial spasm is mainly due to pulsatile compression by vessels at the root exit zone(REZ) of the facial nerve. Microvascular decompression at REZ of the facial nerve has become the standard treatment modality for hemifacial spasm. The authors have analized patients with hemifacial spasm treated with microvascular decompression to evaluate operation result and clinical course after operation. Patients and Methods : From 1992 to 1999, 41 patients with hemifacial spasm underwent this operation. Retrospective analysis of operation results and clinical recovery patterns was done. The length of observation had been more than 6 months in all cases. Results : The ratio of male to female was 1:1.4, and age at operation ranged from 24 to 66 years. Their mean age was 47.6 years and the mean preoperative duration of symptoms was 7.2 years. Most common offending vessels were AICA in 18 cases(48%) and second most common were PICA in 13 cases(31.7%). The rest of them were 3 case in vertebral artery, and 7 cases(13%) in multiple offending vessels. Patterns of improvement after surgery could be divided into 4 clinical types. There was complete recovery in 3 days after operation in 24 cases(58.6%, Immediate complete recovery). There was complete recovery in 3 days after operation, and symptom was recurred partially, which was gradually subsided in 2 weeks after operation in 4 cases(9.8%, Delayed complete recovery type I). There was partial recovery after operation and symptom was compretely disappeared gradually in 6 months after operation in 7 cases(17.1%, Delayed complete recovery type II). Finally, there was partial recovery after operation, and symptom was somewhat remained after 6 months later(14.5%, Delayed partial recovery). Conclusion : In conclusion, microvascular decompression for hemifacial spasm is a safe and reliable treatment modality with good results of improvement and there are 4 recovery patterns in clinical course after operation in our series. Therefore, follow-up observation after microvascular decompression is necessary to evaluate the operative results and complication, especially in the delayed resolved cases.

• Clinical and Experimental Reproductive Medicine
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• v.30 no.3
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• pp.203-206
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• 2003

Objectives: Controversial arguments exists on both the case for and against on the accumulation of mitochondrial DNA (mtDNA) deletion in association to tissue and age. The debate continues as to whether this mutation is a major contributor to the phenotypic expression of aging and common degenerative diseases or simply a clinical insignificant epiphenomenon. The objective of this study was to determine whether the accumulation of mtDNA deletion is correlated with age-related and tissue-specific variation. Materials and Methods: One hundred and fifty-seven tissues from blood, ovary, uterine muscle, and abdominal muscle were obtained from patients ranging in age from 31$\sim$60 years. After reviewing the clinical reports, patients with mitochondrial disorder were excluded from this study. The tissues were obtained at gynecological surgeries with the consent of the patient. Total DNA isolated from blood, ovary, uterine muscle, and abdominal muscle was amplified by two rounds of PCR using two pairs of primers corresponding to positions 8225-8247 (sense), 13551-13574 (antisense) for the area around deleted mtDNA and 8421-8440 (sense), 13520-13501 (antisense) for nested PCR product. A statistical analysis was performed by $x^2$-test. Results: About 0% of blood, 94.8% of ovary, 71.4% of uterine muscle, and 86.1% abdominal muscle harbored mtDNA deletion. When we examined the proportion of deleted mtDNA according to age deletion rate was 90% of ovary, 63.6% of uterine muscle, 77.7% of abdominal muscle in thirties and 100% of all tissue in fifties. Conclusion: The findings of this study suggest that the mtDNA deletion is varied in tissue-specific pattern and increases with aging.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.512-518
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• 2002

Purpose : Cerebral palsies are the most common and severe motor disabilities in childhood. There is currently increased interest in their occurrence and patterns of likely cause for a variety of reasons. Therefore, a retrospective study was carried out to understand the clinical features of cerebral palsy. Methods : A retrospective chart review was conducted of all children with cerebral palsy who were diagnosed at St. Benedict Hospital between March 1999 and March 2001. Results : Cerebral palsy patients were classified into 6 major groups. Of six groups, spastic diplegia is the most common type of cerebral palsy(55.3%). The risk factors of cerebral palsy were placenta previa(1 case), placenta abruption(1 case), cytomegalovirus infection(1 case), prematurity (53 cases), neonatal asphyxia(12 cases), dystocia(2 cases), breech delivery(1 case), multiple birth(5 cases), head trauma(3 cases), meningitis(2 cases) and unknown(26 cases). Among the 59 in the preterm group, 37 patients showed MR or CT images of periventricular leukomalacia. Among the 44 in the term group, 15 patients showed MR or CT images of atrophy. Among 103 patients, 29 patients(28.2%) had a seizure disorder. Conclusion : It is very importent to understand the clinical features and risk factors of cerebral palsy for physicians to diagnose and manage cerebral palsy patient.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.29 no.5
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• pp.330-337
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• 2003

This is a retrospective study on the care of odontogenic infections in admission patients with major bleeding disorders. The study was based on a series of 514 patients treated at Dong San Medical Center, Wonju Christian Hospital and Il San Health Insurance Hospital, from Jan. 1, 2000, to Dec. 31, 2002. The obtained results were as follows : 1. The cardiovascular disease was the most frequent cause of the systemic diseases with major bleeding disorders, and liver disease, cerebrovascular disease and renal failure were next in order of frequency. But, there was the most frequent dental consultation in the liver disease, owing to the many odontogenic infectious diseases. 2. Male prediction (66.3%) was almost existed in the odontogenic infectious patients with major bleeding disorders. But, there was slight female prediction (53.4%) in the cardiovascular disease. 3. The most common age group of the odontogenic infectious patients with major bleeding disorders was the fifty decade(27.2%), followed by the forty, sixty & thirty decade in order. 4. In the contents of chief complaints on the odontogenic infectious patients with major bleeding disorder, peak incidence was occurred as toothache (42.2%), followed by intraoral bleeding, ulcer pain, dental extraction in order. 5. In the diagnosis group of odontogenic infectious diseases, periodontitis, pulpitis and periapical abscess were more common. 6. In the treatment group of odontogenic infectious diseases, the most frequent incidence(44.2%) was showed in primary endodontic drainage(pulp extirpation, occlusal reduction & canal opening drainage) and followed by the incision & drainage, the medications & oral hygiene instruction, scaling, indirect pulp capping in order.

• Journal of the korean academy of Pediatric Dentistry
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• v.47 no.3
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• pp.352-358
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• 2020

Glanzmann's thrombasthenia (GT) is a rare, autosomal recessive inherited congenital disorder, characterized by impaired blood coagulation due to platelet dysfunction. It was first reported by the pediatrician Glanzmann in 1918. GT affects both males and females, and it is more common in regions of the Middle East, India, and France, where intermarriage is common. It has an incidence of about 1 in 1,000,000 people. In South Korea, according to the Division of Rare Diseases, Korea Centers for Disease Control and Prevention, around 200 cases have been reported in 2018. Clinical symptoms include petechia, ecchymosis, epistaxis, and gingival bleeding. The spontaneous loss of deciduous teeth can result in excessive bleeding with that blood transfusion should be considered. Preventing hemorrhages and hemostasis are most important factors in dental treatment. Local bleeding can be controlled by compression, but platelet transfusion can be required by prolonged bleeding. Pediatric dentists can minimize the gingival bleeding by control of the oral hygiene to prevent gingivitis and dental caries. The importance of oral hygiene and periodic recall check-up should be emphasized. During dental treatment, the examination and the treatment plan of patient should be modified to prevention of hemorrhages carefully. A 6-year-old girl with GT was referred for the treatment of dental caries, and resin restoration was performed under nitrous oxide inhalation sedation. After treatment, compression was required for the bleeding control.

• Journal of Genetic Medicine
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• v.2 no.2
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• pp.53-57
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• 1998

Spinal muscular atrophy (SMA) type I is a common severe autosomal recessive inherited neuromuscular disorder that has been mapped to chromosome 5q11.2-13.3. The survival motor neuron (SMN) gene, a candidate gene, is known to be deleted in 96% of patients with SMA type I. Presently, PCR and single strand conformation polymorphism (PCR-SSCP) analyses have been made possible for application to both archival slides and paraffin-embedded tissues. Archival materials represent valuable DNA resources for genetic diagnosis. We applied these methods for the identification of SMN gene of SMA type I in archival specimens for the prenatal diagnosis. In this study, we performed the prenatal diagnosis with chorionic villus sampling (CVS) cells on two women who had experienced neonatal death of SMA type I. DNA extraction was done from archival slide and tissue materials and PEP-PCR was performed using CVS cells. In order to identify common deletion region of SMN and neuronal apoptosis-inhibitory protein (NAIP) genes, cold PCR-SSCP and PCR-restriction site assay were carried out. Case 1 had deletions of the exons 7 and 8, and case 2 had exon 7 only on the telomeric SMN gene. Both cases were found to be normal on NAIP gene. These results were the same for both CVS and archival biopsied specimens. In both cases, the fetuses were, therefore, predicted to be at very high risk of being affected and the pregnancy were terminated. These data clearly demonstrate that archival slide and paraffin-embedded tissues can be a valuable source of DNA when the prenatal genetic diagnosis is needed in case any source for genetic analysis is not readily available due to previous death of the fetus or neonate.

• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.1
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• pp.13-18
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• 2013

CATCH 22 syndrome is a one of the most common chromosome microdeletion syndrome with multiple organ anomalies in humans, with an incidence of approximately 1:4,000 to 1:5,000 live births. It is caused by a microdeletion of 1.5 to 3.0 megabases on the long arm of chromosome 22. The phenotypic spectrum of this disorder is wide and various. A 19-year-old patient who showed delayed growth and development (Height; 110 cm, Weight; 18 kg) was referred to our department for the treatment of dental cavities. She was diagnosed as CATCH 22 syndrome in 2004. Physical examination revealed hypertelorism, a short philtrum, thick reflected lips and a small mouth. She underwent cleft palate surgery at 1 year of age and heart valve surgery due to the cardiovascular abnormality at 13 years of age. Convulsive seizures had persisted until 5 years ago but are well controlled at present. Oral examination showed poor oral hygiene, crowding, prolonged retention on #65, 75 and dental cavities on #16, 21, 65, 26, 36, and 46. Cavity treatment and prophylaxis were performed under general anesthesia. Also continuous follow-up checks have been carrying out with the periodic prophylaxis and dental home education. Problems with numerous cavities and gingivitis which can lead to specific risks are common for CATCH 22 syndrome patients. It is therefore of great importance that these patients are referred to foremost physician and dental specialist for the oral care. In addition, preventive treatment targeting the risk of dental cavities and gingivitis is especially important and, as the syndrome involves many different medical problems, the dental treatment should be carried out in collaboration with the patient's physician.

• Journal of Yeungnam Medical Science
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• v.8 no.2
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• pp.24-34
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• 1991

Delayed emergence of speech or language are frequent causes for concern about development during early childhood. Dealy in evaluation and proper management until school entry in more likely to result in frustration, anxiety and school failure. Many language disturbances and their attendant behavior disorders respond to intensive language therapy. Authors analyzed the medical recoreds of 52 children with delayed language development evaluated during 30 months from January 1986 through June 1988. The results were as follows : 1. The majority of cases were evaluated at the age of 2-2.9 year old(16 cases, 30.8%) and 3-3.9 year old(11 cases, 21.2%) 2. Male to female ratio was 3 : 1 3. The most common cause was mental retardation(53.8%), followed by developmental language disorder(23.1%) and autism(13.5%) 4. The most common associated condition was dysarticulation(17.3%), followed by strabismus(9.6%) and seizures(7.7%) 5. Special education was recommended in cases of 23(44.2%), speech therapy in 12 cases(23.1%) and consultation to child psychiatry in 7 cases (13.5%). Making diagnosis of underlying disorders is not simple because assessment of intelligence in young children is difficult and only a few tests are standardized. More detailed study on children with delayed language development and development of psychometric tests for the handicapped children are necessary, especially in Korea.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.40
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• pp.27.1-27.8
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• 2018

Purpose: Various complications occur when a maxillofacial fracture is malunionized or improperly resolved. Malocclusion is the most common complication, followed by facial deformity, temporomandibular joint disorder (TMD), and neurological symptoms. The purpose of this study was to evaluate the dental treatment of postoperative complications after maxillofacial fracture. Materials and methods: In this study, nine patients with a postoperative complication after maxillofacial fracture who had been performed the initial operation from other units and were referred to the authors' department had been included. Of the nine patients, six had mandibular fractures, one had maxillary fractures, one had maxillary and mandibular complex fractures, and one had multiple facial fractures. All the patients had tooth fractures, dislocations, displacements, and alveolar bone fractures at the time of trauma, but complications occurred because none of the patients underwent preoperative and postoperative dental treatment. Malocclusion and TMD are the most common complications, followed by dental problems (pulp necrosis, tooth extrusion, osteomyelitis, etc.) due to improper treatment of teeth and alveolar bone injuries. The patients were referred to the department of dentistry to undergo treatment for the complications. One of the nine patients underwent orthognathic surgery for a severe open bite. Another patient underwent bone reconstruction using an iliac bone graft and vestibuloplasty with extensive bone loss. The other patients, who complained of moderate occlusal abnormalities and TMDs such as mouth-opening limitation, underwent occlusal treatment by prosthodontic repair and temporomandibular joint treatment instead of surgery. Results: One patient who underwent orthognathic surgery had complete loss of open bite and TMD after surgery. One patient who underwent reconstruction using an iliac bone graft had a good healing process. Other patients were treated with splint, injection, and physical therapy for mouth-opening limitation and temporomandibular joint pain. After treatment, the TMDs were resolved, but the remaining occlusal abnormalities were resolved with prosthetic restoration. Conclusions: Considering the severity of malocclusion and TMJ symptom and the feasibillity of reoperation, nonsurgical methods such as orthodontic and prosthodontic treatments and splint therapy can be used to manage the dental and TMD complication after the trauma surgery. However, reoperation needs to be strongly considered for severe malocclusion and TMD problem.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.5
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• pp.432-440
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• 2022

Purpose: Eating disorders often result in somatic complications, including cardiac abnormalities. Cardiac abnormalities may involve any part of the heart, including the cardiac conduction system, and can lead to sudden cardiac death. The current study aimed to evaluate the incidence of cardiac complications in pediatric patients with eating disorders and their associated factors. Methods: We retrospectively analyzed patients aged 10-18 years who were diagnosed with DSM-V (Diagnostic and Statistical Manual of Mental Disorder-V) eating disorders and underwent electrocardiography (ECG) and/or echocardiography between January 2015 and May 2020. Results: In total, 127 patients were included, of whom 113 (89.0%) were female. The median body mass index (BMI) was 15.05±3.69 kg/m². Overall, 74 patients (58.3%) had ECG abnormalities, with sinus bradycardia being the most common abnormality (91.9%). Patients with ECG abnormalities had significantly lower BMI (14.35±2.78 kg/m² vs. 16.06± 4.55 kg/m², p<0.001) than patients without ECG abnormalities, as well as lower phosphorus and higher cholesterol levels. Among the 46 patients who underwent echocardiographic evaluation, 23 (50.0%) had echocardiographic abnormalities, with pericardial effusion being the most common (60.9%). The median left ventricular mass (LVM) and ejection fraction were 67.97±21.25 g and 66.91±28.76%, respectively. LVM and BMI showed a positive correlation (r=0.604, p<0.001). After weight gain, the amount of pericardial effusion was reduced in 3 patients, and 30 patients presented with normal ECG findings. Conclusion: Cardiac abnormalities are relatively frequent in patients with eating disorders. Physicians should focus on this somatic complication and careful monitoring is required.