• Title/Summary/Keyword: Developmental Anomalies

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14세 여아에서 발견된 선천성 낭종성 선종성 기형 1예 (A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung)

  • 이명인;손소희;이대준;하동열;지영구;이계영;김건열;최영희;조정희;서필원;김삼현
    • Tuberculosis and Respiratory Diseases
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    • 제43권5호
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    • pp.805-811
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    • 1996
  • 저자들은 14세 여아에서 호흡곤란과 흉통이 발생하였으나, 긴장성 기흉등으로 잘못 인식되었던 선천성 낭종성 선종성 기형(CCAM)을 진단하여 수술적 방법으로 치료한 증례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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Pierre Robin Sequence 환아의 증례보고 (PIERRE ROBIN SEQUENCE: A CASE REPORT AND REVIEW OF THE LITERATURE)

  • 이정은;송제선;김성오;이제호
    • 대한장애인치과학회지
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    • 제5권2호
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    • pp.92-95
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    • 2009
  • 1. 이 증례는 Pierre Robin Sequence로 진단받은 14개월 남아로 PRS의 3요소 중 하악왜소증, 설하수증의 소견을 보이고 있었으며 기도확보는 측와위를 통하여, 섭식은 PEG 도관삽입술을 시행하여 유지하고 있었다. 2. PRS는 해부학적 폐쇄 질환이기도 하지만 성장 장애이기도 하므로 여러 분야의 다원적 접근 및 치료계획이 필요하며, 다른 증후군들과 연관되어 심장질환, 신경질환, 안과질환, 청력문제 등이 동반될 수 있으므로 치과 진료실 에서 PRS 환아의 진료시 이 점을 유의해야할 것이다.

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Steatocystoma multiplex: A case report of a rare entity

  • Shin, Nan-Young;Kang, Ju Hee;Kim, Jo-Eun;Symkhampa, Khantaly;Huh, Kyung-Hoe;Yi, Won-Jin;Heo, Min-Suk;Lee, Sam-Sun;Choi, Soon-Chul
    • Imaging Science in Dentistry
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    • 제49권4호
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    • pp.317-321
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    • 2019
  • Steatocystoma multiplex is an uncommon benign skin disease, which typically manifests as numerous intradermal cysts that can be scattered anywhere on the body. Although usually asymptomatic, it can be significantly disfiguring. One type of steatocystoma multiplex is known to be associated with the autosomal dominant inheritance of a mutation in the gene coding for keratin 17 (KRT17). In such cases, it is often concurrent with other developmental abnormalities of the ectoderm-derived tissues, such as the nails, hair, and teeth. To the best of our knowledge, few cases have been reported of steatocystoma multiplex of the oral and maxillofacial region. This report describes a case of steatocystoma multiplex of both sides of the neck and multiple dental anomalies, with a focus on its clinical, radiological, and histopathological characteristics, as well as the possibility that the patient exhibited the familial type of this condition.

Cloned Placenta of Korean Native Calves Died Suddenly at Two Months after Birth Displays Differential Protein Expression

  • Kim Hong Rye;Kang Jae Ku;Lee Hye Ran;Yoon Jong Taek;Seong Hwan Hoo;Jung Jin Kwan;Park Chang Sik;Jin Dong Il
    • Reproductive and Developmental Biology
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    • 제29권2호
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    • pp.63-68
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    • 2005
  • Cloned calves derived from somatic cell nuclear transfer (SCNT) have been frequently lost by sudden death at 1 to 3 month following healthy birth. To address whether placental anomalies are responsible for the sudden death of cloned calves, we compared protein patterns of 2 placentae derived from SCNT of Korean Native calves died suddenly at two months after birth and those of 2 normal placentae obtained from AI fetuses. Placental proteins were separated using 2-Dimensional gel electrophoresis. Approximately 800 spots were detected in placental 2-D gel stained with coomassie-blue. Then, image analysis of Malanie III (Swiss Institute for Bioinformatics) was performed to detect variations in protein spots between normal and SCNT placentae. In the comparison of normal and SCNT samples, 8 spots were identified to be up-regulated proteins and 24 spots to be down-regulated proteins in SCNT placentae, among which proteins were high mobility group protein HMG1, apolipoprotein A-1 precursor, bactenecin 1, tropomyosin beta chain, $H^+-transporting$ ATPase, carbonic anhydrase II, peroxiredoxin 2, tyrosine-rich acidic matrix protein, serum albumin precursor and cathepsin D. These results suggested that the sudden death of cloned calves might be related to abnormal protein expression in placenta.

3개월 이하의 영아에서의 개심술 (Open Heart Surgery During the first 3 Months of Life)

  • 서경필
    • Journal of Chest Surgery
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    • 제26권3호
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    • pp.180-185
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    • 1993
  • From February 1982 to December 1991, 49 neonates and 105 infants in less than 3 months of age underwent open heart surgery in Seoul National University Hospital. There were 98 males and 56 females, and their mean ages were 16 days in neonatal group and 67 days in early infant group. Their body weight and height were less than 3 percentile of normal developmental pattern. In order of decreasing incidence, the corrected conditions included Transposition of great arteries with or without ventricular septal defect [43], isolated ventricular septal defect [34], Total anomalous pulmonary venous return [21], Pulmonary atresia with intact ventricular septum [9] and others [47]. Various corrective or palliative procedures were performed on these patients; Arterial switch operation [36], patch closure for ventricular septal defect [34], Repair of total anomalous pulmonary venous return [21], RVOT reconstruction for congenital anomalies with compromised right ventricular outflow tract [17]. Profound hypothermia and circulatory arrest were used in 94 patients [ 61% ]: 42 patients [ 85.7% ] for neonatal group and 52 patients [ 49.5% ] for early infant group. The durations of circulatory interruption were within the safe margin according to the corresponding body temperature in most cases [ 84% ]. The hospital mortality was 36.4% ; 44.9% in neonatal group and 32.4% in infant group 1 to 3 months of age. The mortality was higher in cyanotic patients [ 46.6% ], in those who underwent palliative procedures [ 57.8% ], in patients whose circulatory arrest time was longer than safe periods [ 60% 0] and in patients who had long periods of cardiopulmonary bypss and aortic crossclamping. In conclusion, there has been increasing incidence of open heart surgery in neonates and early infants in recent years and the technique of deep hypothermia and circulatory arrest was applied in most of these patients, and the mortality was higher in cyanotic neonates who underwent palliative procedures and who had long cardiopulmonary bypass , aortic cross-clamping and circulatory arrest.

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증례 보고-Talon Cusp (CASE REPORTS : TALON CUSP)

  • 민유진;김종수
    • 대한소아치과학회지
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    • 제31권1호
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    • pp.46-51
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    • 2004
  • Talon cusp는 전치부의 치관부에 부가적으로 생성된 교두양 구조물로서 치과이상중 하나이다. Talon cusp는 비심미성, 교합 장애, 치아변위, 발육부의 우식으로 인한 치수 괴사 및 치근단 병변 등을 야기할 수 있다. 그밖에 교모나 치주적인 문제, 혀에의 자극, 그리고 턱관절 동통등의 문제점을 야기할 수 있다. 따라서, Talon cusp의 정확한 조기 진단이 필요하며 각각의 증례에 맞는 처치를 통해 Talon cusp로 인한 문제점을 방지할 수 있다. 본 증례는 각각 영구전치와 유전치에 형성된 Talon cusp로서 이차 상아질을 유도하면서 점차적으로 교두를 삭제하는 치료와 근관 치료를 동반해 교두를 완전 절단하는 치료를 시행하였다.

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Evaluation of the lateral ventricle using MRI in normal micropigs

  • Choi, Mihyun;Lee, Namsoon;Yi, Kangjae;Kim, Junyoung;Choi, Mincheol
    • 대한수의학회지
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    • 제51권3호
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    • pp.227-231
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    • 2011
  • This study was undertaken to assess the lateral ventricle, which was some portion of brain and related to congenital anomalies, from 1, 2, 4, and 8 months of age in healthy micropigs. They were induced general anesthesia and performed magnetic resonance imaging (MRI) with a 0.3 Tesla magnet. Each age group was evaluated by three subjects such as lateral ventricular volume, ventricular volume ratio and asymmetry. T1 weighted transverse images were acquired for calculation of lateral ventricular and corresponding brain parenchyma areas. The ratio of bilateral ventricle areas used to analyze the asymmetry. The mean ventricular volumes of each month were $676.74{\pm}25.58mm^3$ (1 month-old), $630.64{\pm}143.84mm^3$ (2 month-old), $992.12{\pm}106.03mm^3$ (4 month-old) and $1172.62{\pm}237.57mm^3$ (8 month-old), respectively. The ventricular volume ratio was the smallest at 2 month-old and re-increased from that age. The ratio was significantly different between 2 month-old and other age groups (p < 0.05). The value of bilateral area ratio showed within 1.5 in all experimental animals. Consequently the lateral ventricle showed a positive correlation with aging and symmetric shapes in both sides. The developmental pattern of the lateral ventricle provides basic data in micropigs as an experimental animal model for physiological and neurosurgical approach.

Somatic embryogenesis from the axillary meristems of peanut (Arachis hypogaea L.)

  • Singh, Shweta;Hazra, Sulekha
    • Plant Biotechnology Reports
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    • 제3권4호
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    • pp.333-340
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    • 2009
  • Developmental anomalies in the plumule meristem of peanut (Arachis hypogaea L.) somatic embryos resulted in poor shoot differentiation and reduced plant recovery. Existing meristems with caulogenic potential have never been tested for embryogenesis in peanut. The present experiment was designed to test the mature zygotic embryo axis derived plumule with three meristems for somatic embryogenesis. Embryogenic masses and embryos developed from the caulogenic meristems in the axils. Exposure of 2 weeks in primary medium with $90.5{\mu}M$ 2,4-D suppressed the shoot tip differentiation temporarily which then regained the ability to form the shoot on withdrawal of 2,4-D. Exposure of 4 weeks in primary medium with $90.5{\mu}M$ 2,4-D suppressed the shoot tip differentiation irreversibly. No shoot formation was noted from the tips in any of the cultures which were in secondary medium with $13.6{\mu}M$ 2,4-D. Development of somatic embryos directly from axillary meristems was confirmed histologically. Conversion frequency of these embryos was 11%. Thus, in this report, we describe a method to obtain somatic embryos from the determined organogenic buds of the axillary meristem, by culturing the nodal explant vertically on embryo induction medium. It also displays the possibility of obtaining both embryogenic and organogenic potential in two parts of the same explant simultaneously. The possibility of extending this approach for genetic transformation in in vivo system through direct DNA delivery or Agrobacterium injection in meristems can also be explored. Using Agrobacterium rhizogenes, we have demonstrated the possibility of gene transfer in the axillary meristems of seed-derived plumule explant.

누난 증후군 : 증례보고 (NOONAN SYNDROME : A CASE REPORT)

  • 이수언;최성철;김광철;나성식;박재홍
    • 대한장애인치과학회지
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    • 제7권2호
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    • pp.123-126
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    • 2011
  • 본 증례는 누난 증후군으로 진단 받은 환아에 대한 내용으로, 성장 지연, 작은 키, 좁고 높은 악궁 등의 특징적인 소견을 보였다. 체중은 8.8kg이었으며 신장은 100cm으로 2세 아이들의 성장 양상과 비슷한 수준을 나타내고 있었다. 이소맹출 되고 있는 매복 상악 측절치에 의해 상악 중절치의 맹출이 방해 받고 있었으며, 다수의 치아우식증이 관찰되었다. 이에 상악궁의 심한 총생이 예상되며, 측절치 치배로 인해 영구 상악 중절치 발육에 영향을 줄 것을 고려하여 전신마취 하에 측절치의 발거 및 치아우식증의 치료를 시행하였다. 주기적인 관찰기간 동안 영구 중절치의 발육에 큰 문제가 나타나지 않았으며, 정상적인 성장이 이루어지는 것을 관찰할 수 있었다.

유합된 하악 영구 중절치와 측절치의 치료에 관한 증례보고 (TREATMENT OF FUSED PERMANENT MANDIBULAR LATERAL AND CENTRAL INCISORS: REPORTS OF CASES)

  • 한정재;김대업;이광희
    • 대한소아치과학회지
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    • 제24권4호
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    • pp.795-804
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    • 1997
  • Developmental dental morphological anomalies are fusion, gemination, twinning, concrescence and etc. They may cause many problems in conservative, periodontal and esthetic aspects. Fusion is a condition where two separate tooth buds unite at some stage in their development to form a bifid crown. If tooth contact occurs early, at least before the start of calcification, the two teeth may be completely unites to form a single large tooth. If tooth contact occurs after the time when a portion of the tooth crown has completed its formation, there may be union of the roots only. In fusion the dentin is always confluent. Fusion teeth is more common in the deciduous than in the permanent dentition. Fused teeth are relatively rare, and are mostly mandibular anterior teeth. Fusion of normal and supernumerary teeth or between normal teeth may occurs. The exact etiology factor of fusion is unknown, but genetic and environmental factors seems to be related. A variety of complications and subsequent treatments have been suggested on this teeth. Periodontal conditions may arise due to a groove formed at the line of fusion of the two teeth. Crowding or Protrusion with potential for malocclusion or delayed eruption of adjacent teeth. Fusion teeth appear in the anterior region, they usually cause esthetic problems. Treatments vary depending on the problem, the location, and the extent of fusion. Treatment of fused teeth has been reported from endodontic, orthodontic, periodontic, surgical and multidisciplinary. This report presents the esthetic improvements by separation of two clinical crowns. Bone reduction or endodontic treatments are not required.

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