• Journal of Nutrition and Health
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• 제44권1호
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• pp.29-40
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• 2011

본 연구는 서울 소재 대학병원에서 건강검진을 한 폐경 전 (283명)과 폐경 후 (143명) 여성 총 431명을 대상으로 골밀도, 골대사지표, 여성호르몬 및 영양소와의 관련성에 대해 알아보고자 실시하였으며 그 결과를 요약하면 다음과 같다. 연구 대상자의 평균 나이는 48.4세였고, 폐경 후 여성의 평균 폐경 나이는 50.3세였으며, 폐경 후 경과기간은 5.17년 이었다. 대상자의 평균 신장 체중 허리둘레 체질량지수 체지 방률은 각각 159.2 cm, 55.6 kg, 74.4 cm, $21.9\;kg/m^2$, 29.1% 이었으며, 폐경 후 여성의 나이, 신장, 허리둘레, BMI, 체지방률이 폐경 전 여성보다 유의하게 높았다 (p < 0.001, p < 0.001, p < 0.001, p < 0.01, p < 0.001). 평균 수축기혈압과 이완기혈압은 각각 113.5 mmHg, 69.4 mmHg었으며 폐경 후 여성에서 유의적으로 높았다 (p < 0.001, p < 0.01). 연구 대상자의 골 밀도를 살펴본 결과 폐경 후 여성이 폐경 전 여성보다 요추, 대퇴경부, 대퇴전체 골 밀도가 유의하게 감소되어 있었고 (p < 0.001) OC, CTx, ALP 지표는 폐경 전 여성보다 폐경 후 여성이 유의하게 높은 것으로 나타났다 (p < 0.001). 평균 열량 섭취량은 폐경 전 여성 1,679.4 kcal, 폐경 후 여성 1,621 kcal로 권장량의 93.2%, 90.05%이었으며 탄수화물, 단백질, 지방 섭취량의 에너지 기여 비율은 각각 58 : 18 : 24, 61 : 18 : 21 이었다, 단백질, 지방, 콜레스테롤 섭취량이 폐경 전 여성보다 폐경 후 여성에서 유의하게 높았다 (p < 0.05, p < 0.001, p < 0.001). 일반 영양소 섭취량 중에서 특히 칼륨 섭취량 (폐경 전 후 여성의 칼륨 섭취량은 권장량의 66.69%, 71.54%)이 권장량에 많이 부족한 반면 나트륨 섭취량 (폐경 전 후 여성의 나트륨 섭취량은 권장량의 226.8%, 216.5%)은 권장량보다 훨씬 높았으며 섬유소, 칼륨의 섭취량은 폐경 전 여성이 폐경 후 여성보다 유의하게 높게 나타났다 (p < 0.05, p < 0.05). 폐경 전 여성은 요추 골 밀도와 칼륨 섭취량 사이에서 음의 상관관계를, 폐경 후 여성에서는 요추 골 밀도와 칼슘 섭취량사이에서 양의 상관관계를 나타냈고 CTx와 열량, 지방, 탄수화물, 철분, 칼륨, 비타민 A, 베타카로틴, 나이아신, 엽산, 섬유소 섭취량과 음의 상관관계를 나타냈으며 OC와 나이아신 섭취량 사이에서 음의 상관관계를 나타났다. 요추골 밀도는 단백질, 지방, 칼슘과 양의 상관관계를 나타냈고 FSH 또한 지방, 나이아신 섭취량 사이에서 양의 상관관계를 나타냈다. 이상의 결과를 종합해 볼 때 폐경 전과 폐경 후 여성에서 골 표지자인 OC, CTx, ALP 지표는 골 밀도와 여성 호르몬 농도와 연관되어, 체내의 골 대사에 관련된 중요한 중재자의 하나임을 재확인하였고, 골 밀도를 예측하는 인자는 다소 차이가 있었지만 폐경 전 여성에서 체중, BMI, 폐경 후 여성에서는 칼슘 섭취량, 나이 등이 다른 인자들보다 중요도 및 예측에 있어 상대적인 영향이 더 큰 것을 알 수 있었다. 또한, 영양 섭취 상태는 폐경 전과 폐경 후 여성에서 열량 섭취는 권장량과 비교하였을 때 양호한 편이었으나 그에 반해, 칼륨, 칼슘 섭취는 부족, 나트륨은 과잉 섭취를 하고 있었다. 따라서 폐경 후 골다공증을 예방하고 골 감소를 최소화하기 위해서는 골 밀도에 긍정적 영향을 미친다고 판단된 혈청 칼슘의 충분한 섭취와 적절한 체중유지가 중요하며 반대로 부정적 영향을 미친 나트륨 섭취 감소는 골밀도의 감소를 예방하는데 중요한 역할을 할 수도 있다고 제안한다.

• Molecules and Cells
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• 제38권9호
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• pp.806-813
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• 2015

Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by mutations in the glucocerebrosidase gene (GBA), which encodes the lysosomal enzyme glucosylceramidase (GCase). Deficiency in GCase leads to characteristic visceral pathology and lethal neurological manifestations in some patients. Investigations into neurogenesis have suggested that neurodegenerative disorders, such as GD, could be overcome or at least ameliorated by the generation of new neurons. Bone marrowderived mesenchymal stem cells (BM-MSCs) are potential candidates for use in the treatment of neurodegenerative disorders because of their ability to promote neurogenesis. Our objective was to examine the mechanism of neurogenesis by BM-MSCs in GD. We found that neural stem cells (NSCs) derived from a neuronopathic GD model exhibited decreased ability for self-renewal and neuronal differentiation. Co-culture of GBA-deficient NSCs with BM-MSCs resulted in an enhanced capacity for self-renewal, and an increased ability for differentiation into neurons or oligodendrocytes. Enhanced proliferation and neuronal differentiation of GBA-deficient NSCs was associated with elevated release of macrophage colony-stimulating factor (M-CSF) from BM-MSCs. Our findings suggest that soluble M-CSF derived from BM-MSCs can modulate GBA-deficient NSCs, resulting in their improved proliferation and neuronal differentiation.

• Tuberculosis and Respiratory Diseases
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• 제74권2호
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• pp.70-73
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• 2013

Dyskeratosis congenita is a rare congenital disorder characterized by a triad of reticular pigmentation of the skin, dystrophic nails, and leukoplakia of the mucous membrane. Sometimes it is associated with bone marrow failure, secondary malignancy and interstitial lung disease. Though it is rare, Dyskeratosis congenita is diagnosed relatively easily when clinicians suspect it. It can be diagnosed just by gross inspection with care. Dyskeratosis congenita should be considered as one cause associated with interstitial lung disease. In Korea, interstitial lung disease with dyskeratosis congenita has not been reported. We report a case and review the literature.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제31권3호
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• pp.249-253
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• 2009

Langerhans cell histiocytosis(LCH), previously known as histiocytosis X, is rare, proliferative disorder in which the accumulation of pathologic Langerhans cell leads to tissue infiltration and destruction. The jaw is involved 10~20% of all LCH and most common oral symptoms are jaw swelling or a palpation mass. Most of subjects are males and disease appears to start before the age of 10 years. An 11 years old girl was referred from pediatric department. Histological examination confirmed the diagnosis LCH. She was treated intra-lesional injection of corticosteroid. The overall outcome was excellent. After a follow-up period of 20 month, the patient present with no evidence of residual disease on Panoramic view and whole body bone scan. We report this early recognized LCH case treated by corticoid injection in good progress with literature review.

• Journal of Korean Neurosurgical Society
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• 제59권2호
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• pp.165-167
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• 2016

Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis.

• Imaging Science in Dentistry
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• 제47권2호
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• pp.129-133
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• 2017

Dentinogenesis imperfecta is a dominant autosomal hereditary disorder of dentin formation that affects the deciduous and permanent teeth. Its etiology is characterized by inadequate cell differentiation during odontogenesis. The clinical characteristics of dentinogenesis imperfecta are discolored teeth with a translucency that varies from gray to brown or amber. Radiographically, the teeth exhibit pulp obliteration, thin and short roots, bell-shaped crowns, and periapical bone rarefaction. The aim of this report was to present a case of dentinogenesis imperfecta type II that was followed up over a 17-year period. This report also presents scanning electron microscopy images of the enamel and dentin, showing that both were altered in the affected teeth. The disease characteristics and the treatments that were administered are reported in this study to guide dentists with respect to the need for early diagnosis and adequate follow-up to avoid major sequelae.

• Neonatal Medicine
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• 제25권1호
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• pp.44-48
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• 2018

Platyspondylic lethal skeletal dysplasia, Torrance type (PLSD-T), is one of the pheno-types of type II collagenopathy and is characteristic of severe bone growth disorder. This phenotype may limit the growth and expansion of the lungs, which is known to cause death from respiratory failure during or shortly after birth, but in few less severe cases, patients have been reported to have survived to adulthood. We have experienced a case of PLSD-T in a preterm infant who was delivered via cesarean section at the gestational age of 29 weeks 3 days, with a birth weight of 1.15 kg. Physical examination of the infant revealed characteristic findings of short arms and legs, small thorax, distended abdomen, and cleft palate. On the basis of the subsequent genetic testing, the patient had a heterozygous mutation in the encoded c-propeptide region of collagen, type II, alpha 1 (COL2A1), c.4335G>A ($p.Trp1445^{\ast}$) in exon 52. This is the first case of PLSD-T diagnosed in Korea, and we hereby report the case.

• 대한골관절종양학회지
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• 제14권2호
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• pp.178-181
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• 2008

골막하에 발생하는 결절종은 매우 드물며, 골막 연골종, 지방종, 건초 거대 세포종, 혈종, 기타 염증 그리고 방골성 골육종 등과 감별이 필요하다. 현재까지 보고된 골막하 결절종의 발생 부위는 경골이 가장 흔하며, 요골, 척골, 대퇴골에서도 발생이 보고되었지만, 비골에 발생한 경우는 1예만 보고되어 있다. 이에 저자들은 30세 여자 환자의 우측 원위 비골 부위에 발생한 골막하 결절종을 경험하였기에 이를 문헌 고찰과 함께 보고하는 바이다.

• 대한골관절종양학회지
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• 제15권2호
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• pp.160-164
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• 2009

골반문증은 장관골의 골단 및 골간단에 다발성의 경화성 병변이 발생하는 매우 드문 골 이형성 질환으로 아직 원인이 명확히 알려지지 않았다. 일반적으로 상염색체 우성으로 유전되며 대부분 무증상으로 단순방사선학적 검사에 의해 우연히 발견되는 경우가 대부분이다. 저자들이 경험한 증례는 전형적인 골반문증의 방사선학적 소견 및 임상적 특징을 보이고 있었고, 감별진단을 위하여, 조직검사를 시행하였다.

• 대한암한의학회지
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• 제17권1호
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• pp.27-38
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• 2012

Objective : To present an overview of current reports and guidelines of physical activity and exercise intervention for cancer survivors Methods : We searched Pubmed for the related studies such as randomized controlled trials and observational studies, as well as published guidelines or recommendations for exercise intervention. Results : Physical activity and exercise intervention is considered safe and effective for most cancer survivors. According to the guidelines, patients with peripheral neuropathy, musculoskeletal disorder, or those who are at risk of fracture, should undergo proper medical assessment before starting exercise intervention. Also, patients with bone metastasis, thrombocytopenia, symptomatic anemia, or acute infection may fall into one of the contraindications of exercise intervention. Conclusions : Physical activity and exercise intervention may play a major role in improving physical functioning, quality of life, or treatment-related symptoms of cancer survivors. It is necessary to recognize the benefits and precautions of exercise in caring cancer patients.