• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.3
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• pp.555-561
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• 1998

Hypophosphatasia is a rare metabolic disorder which manifests characteristics such as abnormal mineralization of bone and dental tissues, diminished serum and tissue alkaline phosphatase, and increased urinary secretion of PEA. It inherited as an autosomal recessive or dominant trait and occurs in all races. In general, hypophosphatasia can be classified in 4 subtypes which are the perinatal, infantile, childhood, adult type depending upon the age at presentation and severity. In young children with Hypophosphatasia the long bones show irregular defects, and the skull showes poor calcification. In older children with premature closure of the skull sutures there may be multiple lucent area called gyral or convolutional markings, described as resembling beaten copper, presumably resulting from increased intracranial pressure. Examination of the jaws reveals a generalized lucency of the maxilla and mandible. the cortical bone and lamina dura are thin, and the alveolar bone may be deficient. Clinical features of Hypophosphatasia include premature loss of deciduous teeth, especially incisors, hypoplasia or aplasia of root cementum, enamel hypoplasia, irregular calcification of dentin, large pulp chamber, and resorption of marginal alveolar bone and roots. Our report involves a patient with a chief complaint of early loss of both Mx. and Mn. deciduous incisors. After conducting a through clinical and radiographic examination this patient was referred to pediatrics under the suspicion of hypophosphatasia, the diagnosis proved to be correct and successful results were accomplished through a denture made to improve esthetics and function.

• Archives of Craniofacial Surgery
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• v.24 no.2
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• pp.59-65
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• 2023

Background: Fibrous dysplasia (FD) is a localized bone disorder in which fibro-osseous tissue replaces the normal bone structure. Patients with craniofacial FD often present with gradual swelling, deformity, and compromised vision or hearing. We previously introduced "the core extirpation method," a novel surgical technique that is minimally invasive like traditional bone shaving but has longer-lasting effects. This study presents the long-term outcomes of our core extirpation method. Methods: We conducted a retrospective analysis of patients who underwent core extirpation for FD of the zygomaticomaxillary region from 2012 through 2021. Computed tomography (CT) scans were performed 6 to 12 months before the operation, immediately before and after the operation, and during follow-up visits. We performed all operations using the upper gingivobuccal approach, and we extirpated the core of the lesion while preserving the cortical structures of the zygoma and the maxilla to maintain symmetrical facial contour. Results: In 12 patients with lesions in the growth phase, anteroposterior/mediolateral (AP/ML) length discrepancies and the volume increased between preoperative and immediate postoperative CT scans. All patients' immediate postoperative AP/ML discrepancies were stable up to 12-17 months postoperatively. Postoperative volume showed continuous lesion growth; the median volume growth rate was 0.61 cc per month. Conclusion: In this article, we present our experiences managing FD using the minimally invasive core extirpation technique, which entails small expected blood loss and can be performed as day surgery. It provides similar cosmetic outcomes as traditional bone shaving but with longer-lasting results. Although there are some limitations with the study's retrospective nature and small sample size, our 4-year follow-up results show promising results of the core extirpation method in well-indicated patients.

• Journal of The Korean Dental Society of Anesthesiology
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• v.2 no.1 s.2
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• pp.33-37
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• 2002

The oculo-cerebro-renal syndrome of Lowe (Lowe syndrome) is an X-linked recessive disorder involving the eyes, nervous systems, and kidneys. The clinical manifestation of this syndrome is characterized by congenital cataracts, glaucoma, seizure disorder, psychomotor growth retardation, hypotonia, renal tubular acidosis, aminoaciduria, rickets, and osteoporosis. We report a 5-year old boy underwent general anesthesia for the treatment of multiple dental carries. During intraoperative period, marked metabolic acidosis was noted and such acidosis was partially corrected by hyperventilation. We suggest that patients with Lowe's syndrome should be attention and treated to possible anesthetic hazards such as metabolic acidosis due to renal tubular dysfunction, rise of intraocular pressure in patient with glaucoma, the fragility of the bone structures due to rickets and osteoporosis.

• The Journal of Pediatrics of Korean Medicine
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• v.24 no.3
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• pp.81-91
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• 2010

Objectives: The basic cause of developmental disability is congenital weakness, which is a disorder of the kidney according to the Oriental medicine definition. I suggest the oriental medicine music therapy, which can improve congenital weakness and recover the kidney dysfunction. Methods: This study focused autism and Asperger syndrome in terms of Oriental medicine, and also considered view points from the Western medicine. Conclusions 1. The kidney monitors vital elements which were produced from the bone marrow. Therefore, the growth and the development of a skeletal structure are related to the strength and weakness of kidney, which is measured in Qi score. 2. In a case of the deficiency of kidney, an essence due to congenital defect, it shows the symptoms of the developmental disability such as sluggish reaction and physical movements, falling of memory, hearing, and eyesight. 3. For the kidney disorder, "Eum music therapy", one of the oriental medicine music therapies, can promote development of the kidney and kidney-Qi score for the children with developmental disability.

• The Journal of the Korean bone and joint tumor society
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• v.3 no.2
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• pp.127-130
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• 1997

Tumoral calcinosis is a rare disorder of unknown etiology, which is characterized by the developement of large calcified masses overlying the large joints in otherwise healthy subjects. If histologic tests are not confirmed, the condition may be treated erroneously as bursitis. This report describes the case of a 10-year-old man with tumoral calcinosis of the knee, a joint very rarely affected by this unusual disorder. Microscopically the tumor consists of a stroma of chronic inflammatory tissue surrounding cystic spaces containing calcium-rich material. We made excision alone, and the patient made an uneventful recovery with no evidence of recurrence up to 13 months postoperatively.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.819-822
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• 2010

Purpose: Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder. It is characterized by complex neoplastic syndrome with multisystemic manifestations, involving six major features. This article presents a rare genetic disorder and usage of the author's methods for odontogenic keratocyst, developed in the maxillary sinus. Methods: A 67-year-old man was presented with large calcified maxillary mass and multisystemic manifestations and findings that matched with basal cell nevus syndrome. The calcified maxillary mass was removed via the versatile maxillary window and maxillary bone segment was repositioned. Results: Histopathologic findings revealed that maxillary and mandibular lesions were odontogenic keratocysts and the skin lesions were basal cell carcinoma. Conclusion: Basal cell nevus syndrome is a rare genetic disease that requires surveillance and care for basal cell carcinoma and multisystemic problems. The author's method was satisfactory for maxillary odontogenic keratocyst in the aspect of the approach and reconstruction.

• Journal of Physiology & Pathology in Korean Medicine
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• v.27 no.5
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• pp.509-519
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• 2013

Through the study on judgment of Body form and settle Energy flow(立形定氣) before diagnose the patients, the results are as follows. The observation of the body form is to determine prosperity and deficiency of each internal organ. It is necessary to distinguish Body form loss(形脫) and Body form fullness(形充). Fat man(肥人), Thin man(瘦人), Creamy man(膏人), Muscular man(肉人), Small Fat man(脂人) are discriminated by fat distribution, fat content, and muscle mass. The observation of the body form means the observation of structure disorder, color change, develop part at body, head and face. The observation of the body form that is to determine prosperity and deficiency of each internal organ is from the limited knowledge of the anatomy. The observation of face color is considered by blood perfusion, blood oxygenation and accumulation of carotinoid, bilirubin and change of melanin in the facial skin. The prosperity and the deficiency of energy flow is considered by symptom combined with growth (<40 years) and aging (>40 years). The prosperity of energy flow includes the anger, anxious emotion and the deficiency of energy flow includes the fear, depressive emotion. The breathing type is expiratory exhalation like asthma patients in the prosperity of energy flow. The deficiency of energy flow is weakness to overcome the disease. The prosperity and the deficiency of energy flow are considered by body metabolic ratios (Basal metabolic Rate: BMR, Resting metabolic rate: RMR, Physical activity ratios: PASs). Development of subcutaneous fat is good in the person of prosperous energy flow. The person of prosperous energy flow is hard to overcome to heat weather than cold weather. The person of deficiency of energy flow has tendencies of low blood pressure, insufficiency of blood flow in the peripheral and being shocked. The person of deficiency of energy flow has tendencies of chronic fatigue syndrome or automatic nerve disorder. If the patient who has deficiency of energy flow has severe weight loss should be checked for the presence of disease. The observation of small and large of bone is to check the development and disorder of bone growth and aging. The observation of thickness and weakness of muscle is to check the development of muscle, particularly biceps, gastrocnemius, and rectus abdominal muscle. The observation of thickness and weakness of skin is to check the ability of regulating body temperature by sweating.

• Journal of Yeungnam Medical Science
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• v.14 no.1
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• pp.209-219
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• 1997

Objectives: Anterior approach to achieve arthrodesis of the cervical spine has become a widely accepted and often-used approach since its earliest reports by Bailey and Badgley, Smith and Robinson and Cloward. However, anterior interbody fusion in the presence of the posterior instability may be complicated by the bone graft dislodgement, kyphotic defomity or nonunion. As an attemp to prevent this undesirable complication, additional methods such as skeletal traction, halo appratus or even posterior fusion has been utilized. Therefore, The cervical spine locking plate(CSLP) with the anterior intervertebral body bone grafting provide immediate cervical stabilization and widely successful in achieving fusion. Material and methods: This study analysed 14 patients who underwent a single anterior procedure and application of CSLP for the treatment of the cervical spinal disorder. Eleven patients were disc herniations and three patients were traumatic lesion. The average age of the patient was 47 years and the mean follow up periods was 20 months ranging from 13 to 27 months. Results: Ambulation was started 2nd day after the operation with the aid of the Philadelpia orthoses. Bone union was observed 13 cases on average 12 weeks after operation. The one case was nonunion with plate breakage without clinical symptom. Conclusion: Anterior fusion with CSLP are thought to be a safe and valuable method for treating cervical spine disorder.

• 한국노년학
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• v.39 no.2
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• pp.231-240
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• 2019

The study aimed to identify risk factors for falls as well as hospitalization status according to disease and demographic characteristics of demented inpatients by investigating the in-depth Injury Patient Surveillance System data collected by Korea Centers for Disease Control and Prevention(KCDC). Older adults over 60 years old who were diagnosed with dementia were included(n=1,732). Their data were analyzed after being assigned to either a fall group or a non-fall group. STATA was used for statistical analyses, such as frequency analysis, chi-square (χ²) test, and logistics regression. It was found that 8.0% of the demented inpatients experienced falls. According to the analysis on category of fall and non-fall group were statistically significant difference in age and Charlson Comorbidity Index(CCI) and bone density deficiency. Based on the logistic regression analysis of factors affecting falls, older adults over 80 are 2.386 times more likely to fall and based on a target with a CCI of 0, the risk of falls is 0.421 times lower, finally based on those without bone density disorder, the fall risk for those with bone density disorder was 3.581 times higher. Therefore, we expect that the important about the factors relating to falls identified in this can not only be found valuable for educating inpatients with dementia and care-givers, but also be used as reference that supports clinical professionals to make decisions on falls management for patients with dementia.

• Imaging Science in Dentistry
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• v.46 no.4
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• pp.259-265
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• 2016

Fibrous dysplasia (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Mainly, FD is found in children, and by adulthood it usually becomes quiescent. Our case showed FD of more than 14-year duration in the left maxilla. Our evaluation was that growth ceased in adulthood and had achieved the static stage. Because FD cases in elderly patients are rarely reported, we hereby present a monostotic FD case in a 65-year-old female. We presented sequential radiographic images and scintigraphic images of this case, and combined them with a literature review that emphasized the progression of the disease.