• Journal of Korean Neurosurgical Society
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• 제37권1호
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• pp.29-33
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• 2005

Objective: Congenital dermal sinus is a rare congenital disease that results from the failure of the neuroectoderm to separate from the surface ectoderm during the process of neurulation, where there is communication between the skin and the deeper structures. Their pathogenesis, clinical course and treatment strategy are well known. We analyze our series and compare our results with other series. Methods: Twenty patients were diagnosed as congenital dermal sinus and confirmed pathologically from October 1986 to July 2003 at our hospital. We studied the patients' clinical manifestations, radiological findings and pathological profiles. Results: Seven cases were located in the suboccipital area and 13 cases were located in the spinal area. Interestingly, 4 of 13 spinal lesion cutaneous openings were located lower than the 3rd sacral body level. 8 of 20 lesions were terminated at neural structures, 4 of 20 lesions were terminated at the intradural portion and others terminated at the extradural portion. Nine anomalies were combined with the dermal sinus, including 4 lipomas, 2 Currarino's triad, 1 encephalocele, 1 myelomeningocele and 1 diastematomyelia. Eleven patients had dermoid tumors. Conclusion: Congenital Dermal Sinus must be surgically removed immediately if they are diagnosed. The surgical procedure of congenital dermal sinus is complete removal, but in some cases, complete removal is impossible. In those cases, we removed all epithelial tissues. We consider sacrococcygeal dimple almost invariably have no connection with intraspinal structures. But, if other cutaneous manifestations are combined with cutaneous pits, it can communicate with the sacrococcygeal dimple.

• 대한유전성대사질환학회지
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• 제5권1호
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• pp.76-87
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• 2005

Various disorders cause hyperammonemia during childhood. Amongthem are those caused by inherited defects in urea synthesis and related metabolic pathways. These disorders can be grouped into two types: disorders of the enzymes that comprise the urea cycle, and disorders of the transporters or metabolites of theamino acids related to the urea cycle. Principal clinical features of these disorders are caused by elevated levels of blood ammonium. Additional disease-specific symptoms are related to the particular metabolic defect. These specific clinical manifestations are often due to an excess or lack of specific amino acids. Treatment of urea cycle disorders and related metabolic diseases consists of nutritional restriction of proteins, administration of specific amino acids, and use of alternative pathways for discarding excess nitrogen. Although combinations of these treatments are extensively employed, the prognosis of severe cases remains unsatisfactory. Liver transplantation is one alternative for which a better prognosis is reported.

• Annals of Clinical Neurophysiology
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• 제15권2호
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• pp.68-70
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• 2013

Atopic myelitis (AM) is a relatively mild form of myelitis associated with allergic diathesis, and present with predominant sensory manifestations. Lhermitte's sign has been considered as a relatively non-specific clinical sign suggesting demyelinating lesion in cervical cord. Here we report a patient with recurrent AM who presented with isolated Lhermitte's sign, both in first and second attacks. This report suggests that either the diagnosis or recurrence of AM can be frequently underdiagnosed because of its predominant sensory manifestations.

• Journal of Korean Neurosurgical Society
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• 제64권2호
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• pp.238-246
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• 2021

Objective : To retrospectively analyze the clinical characteristics and surgical experience of 10 adults with spinal extradural meningeal cysts (SEMCs) in the thoracolumbar spine which may further provide evidence for surgical decision-making. Methods : Ten adults with SEMCs in the thoracolumbar spine were surgically treated and enrolled in this study. Clinical manifestations, imaging data, intraoperative findings and postoperative outcome were recorded. Results : Clinical manifestations of SEMCs included motor and sensory dysfunction of the lower limbs and urination and defecation disturbance. The cysts presented as intraspinal occupying lesions dorsal to the spine, ranging from the T8 to L3 level. Defects of eight cases were found on preoperative magnetic resonance imaging (MRI). Selective hemilaminectomy or laminectomy were used to reveal the defect within the cyst, which was further sutured with microscopic technique. The final outcome was excellent or good in seven cases and fair in three cases. No recurrence was observed during follow-up. Conclusion : SEMCs are rare intraspinal cystic lesions. Radiography and MRI are clinically practical methods to assess defects within SEMCs. Selective hemilaminectomy or laminectomy may reduce surgical trauma. Detection and microscopic suturing of the defects are the key steps to adequately decompress the nervous tissue and prevent postoperative recurrence.

• Clinical and Experimental Pediatrics
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• 제59권12호
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• pp.471-476
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• 2016

Purpose: Chest radiography is often performed on patients hospitalized with typical clinical manifestations of bronchiolitis. We aimed to determine the proportion of subjects with pathologic chest radiographic findings and the clinical predictors associated with pathologic chest radiographic findings in young children admitted with the typical presentation of bronchiolitis. Methods: We obtained the following data at admission: sex, age, neonatal history, past history of hospitalization for respiratory illnesses, heart rate, respiratory rate, the presence of fever, total duration of fever, oxygen saturation, laboratory parameters (i.e., complete blood cell count, high-sensitivity C-reactive protein [hs-CRP], etc.), and chest radiography. Results: The study comprised 279 young children. Of these, 26 had a chest radiograph revealing opacity (n=24) or atelectasis (n=2). Multivariate logistic regression analysis showed that after adjustment for confounding factors, the clinical predictors associated with pathologic chest radiographic findings in young children admitted with bronchiolitis were elevated hs-CRP level (>0.3 mg/dL) and past history of hospitalization for respiratory illnesses (all P<0.05). Conclusion: The current study suggests that chest radiographs in young children with typical clinical manifestations of bronchiolitis have limited value. Nonetheless, young children with clinical factors such as high hs-CRP levels at admission or past history of hospitalization for respiratory illnesses may be more likely to have pathologic chest radiographic findings.

• Clinical and Experimental Pediatrics
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• 제54권10호
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• pp.409-413
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• 2011

Purpose: Few studies have been conducted on the recent status of infectious mononucleosis (IM) in Korean children. The aim of this study was to evaluate the recent trend in the clinical manifestations of Epstein-Barr virus (EBV)-associated IM as well as the clinical differences according to age. Methods: A retrospective study was performed on 81 children hospitalized with EBV-associated IM who fulfilled the serological criteria for the diagnosis of EBV infection (viral capsid antigen immunoglobulin M positive). The patients were divided into 3 age groups: <5 years, 5 to 9 years, and ${\geq}10$ years. We evaluated the recent trend in clinical manifestations and the differences in clinical and laboratory findings among the 3 age groups. Results: Thirty (37%) children were under 5 years of age, 38 (46.9%) were 5 to 9 years of age, and 13 (16%) were 10 years of age or older. The differences in the symptoms and signs among the 3 age groups were not statistically significant, except for headache. The mean duration of fever was 7.7 days (range, 0 to 18 days). A comparison of liver enzyme elevation among the age groups showed an association with advancing age (26.6%, 63.1%, and 76.9%, respectively, P=0.04) Conclusion: This study showed that EBV-associated IM in Korean children continues to occur mostly in children under 10 years of age. In children with EBV-associated IM, the incidence of headache and liver enzyme elevation, the duration of fever, and the proportion of females to males were all positively associated with advancing age.

• Imaging Science in Dentistry
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• 제54권1호
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• pp.13-24
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• 2024

Purpose: Pycnodysostosis (PYCD), an autosomal recessive syndrome, is characterized by an imbalance in bone remodeling that produces various clinical and radiographic craniofacial manifestations. This review represents a systematic examination of these manifestations, as well as oral features associated with PYCD. Materials and Methods: A systematic review was conducted across 8 databases from February to March 2023. The search strategy focused on studies reporting cases of PYCD that examined the clinical and radiographic craniofacial and oral characteristics associated with this syndrome. Results: The review included 84 studies, encompassing a total of 179 cases of PYCD. More than half of the patients were female (55.3%), and the mean age was 14.7 years. Parental consanguinity was reported in 51.4% of the cases. The most common craniofacial clinical manifestation was a prominent nose, observed in 57.5% of cases. Radiographically, the most frequently reported craniofacial characteristics included the presence of an obtuse mandibular angle (84.3%) and frontal cranial bosses(82.1%). Clinical and radiographic examinations revealed oral alterations, with micrognathia present in 62.6% of patients and malocclusion in 59.2%. Among dental anomalies, tooth agenesis was the most commonly reported, affecting 15.6% of patients. Conclusion: Understanding the clinical and radiographic craniofacial features of PYCD is crucial for dental professionals. This knowledge enables these clinicians to devise effective treatment plans and improve patient quality of life.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.310-316
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• 2014

Purpose: Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment. Methods: The clinical characteristics and the results of the GH treatment were reviewed retrospectively for 30 PWS patients diagnosed by molecular genetic testing and clinical manifestations. Results: The mean age at diagnosis with PWS was 13.7 months (2-47 months of age). All patients showed the characteristics of facial dysmorphism, including brown hair and almond-shaped eyes. Most patients showed developmental delays/mental retardation (93.3%), cryptorchidism (75%), feeding problems in infancy (73.3%), and neonatal or infantile hypotonia (66.7%). Among 30 patients, 14 PWS infants and toddlers had been treated with GH for more than two years. Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age. There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment. Conclusion: Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

• Tuberculosis and Respiratory Diseases
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• 제44권6호
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• pp.1382-1389
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• 1997

저자들은 감기증상 후에 빠른 시간내에 급성호흡부전증에 이르렀던 환자에서 신증후성 출혈열과는 다르게 주로 호흡기와 순환기를 침범하는 한타바이러스 폐 증후군 1예률 경험하였기에 고하는 바이다.

• 한방안이비인후피부과학회지
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• 제27권4호
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• pp.58-66
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• 2014

Objectives : The purpose of this study was to investigate the clinical manifestations and influences of occupation, concomitant allergic diseases, various aggravating factors and seasonal changes for patients with dyshidrotic eczema. Methods : A total of 219 patients who showed clinical manifestations of dyshidrotic eczema were included in this study. We retrospectively reviewed the clinical characteristics of all the patients. Results : 1. Among 219 patients, there were 76 male patients (34.7%), 143 female patients (65.3%), male to female ratio was 1: 1.88. The average age of the patients at admission was $31.9{\pm}9.8$ years, had a lot of 20s and 30s the distribution. 2. The average of duration of the admission to the clinic after the onset was $4.01{\pm}4.42$ years. 3. Common accompaning diseases dyshidrotic eczema patients have were allergic rhinitis(87 cases), and atopic dermatitis(55 cases), sweating(45 cases), and asthma(10 cases) in order. 4. The most commonly occurring sites for dyshidrotic eczema patients are fingers(60.3%), palm(53.4%), dorsum of hand(19.2%) and nails(5.5%) in order. 5. The most commonly contacted materials for dyshidrotic eczema patients are water(130 cases), cosmetics(40 cases) and rubber gloves(34 cases) in order. The aggravating factors of dyshidrotic eczema patients are emotional stress(110 cases), fatty food(22 cases) and alcohol(22 cases) in order. 6. The number of patients showing seasonal changes in symptoms was 111. The season with the most severe symptom was summer with 76 cases, which was statistically significant. Conclusions : This study demonstrated the various clinical characteristics of Korean patients with dyshidrotic eczema.