• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.2
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• pp.53-57
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• 2022

Congenital metabolic disorders are rare inherited disorders resulting from a defect in biochemical and metabolic pathways affecting proteins, fats, carbohydrates metabolism or impaired organelle function. Depending on the abnormality of biochemical metabolism, various precursors and their abnormal metabolites can accumulate in the body and the final products which are critical in normal physiology can be deficient, resulting in disease. Congenital metabolic disorders present complicated medical conditions involving several human organ systems, including nervous system, eyes, liver, and kidneys. Various proteins and lipids are involved in the development and homeostasis of the skin, so many congenital metabolic disorders present abnormal changes in skin and hair. In this review, congenital metabolic diseases related to amino acid and lipid metabolism accompanying skin abnormalities will be discussed.

• Proceedings of the KSLP Conference
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• 1997.11a
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• pp.256-256
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• 1997

조기후두암에서의 치료방법은 방사선치료, 수술적치료등 여러 가지가 있는데 최근들어 사용되고 있는 방법중의 하나가 레이저를 이용하여 병변을 제거하는 방법이다. 서울대학교병원 이비인후과에서 레이저를 이용한 후두미세술을 이용하여 치료한 조기 후두암 환자와 방사선 치료를 이용한 조기후두암환자의 치료 후 성대의 기능을 주관적인 애성의 정도와 Kay voice analysis system을 이용하여 구한 객관적인 척도로 분석하였다. (중략)

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.29 no.1
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• pp.37-40
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• 2018

Background and Objectives : This study aimed to evaluate patients' subjective and objective outcomes after laryngeal microsurgery for benign vocal fold (VF) lesions, and to identify usefulness of surgical treatment. Materials and Methods : The authors reviewed the 102 patients medical records, retrospectively who received laryngeal microsurgery for benign VF lesions from January 2013 to August 2017. Subjective voice were measured using the Voice Handicap Index (VHI). Objective voice were recorded with Multi-Dimensional Voice Program (MDVP) just before surgery, and after at least 3 months of surgery. Results : Benign VF lesions were categorized as VF nodule (n=34, 33%), VF Polyp (n=47, 26%), Intracordal cyst (n=15, 15%), Reinke's edema (n=6, 6%), and VF Papilloma (n=2, 2%). Post-operative voice assessment at VHI scores showed statistically significant reductions in all of functional, physical and emotional parts (p<0.001). MDVP were showed significant improvement of Jitter (P=0.001), Shimmer (p<0.001) and Noise to Harmonic Ratio (NHR) (p=0.001). Conclusion : Laryngeal microsurgery for benign vocal fold lesions is effective treatment with statistically significant improvement at subjective and objective vocal quality assessment.

• Journal of Chest Surgery
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• v.31 no.8
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• pp.792-798
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• 1998

Background: There are various tracheal diseseas which cause the obstruction of the trachea: postintubation tracheal stenosis, tracheal cancer, thyroid cancer, endotracheal tuberculosis, et al. Recently surgical resection and reconstruction of the trachea has been adopted as the safe method for tracheal lesions. Materials and methods: We report our experience and results of resection and reconstruction for various obstructive tracheal lesions in 38cases from 1985 to 1996. Length of resection of the trachea was up to 6 cm. Twenty lesions were approached by cervical collar incision, 12 lesions by cervicosternal incision and 4cases needed transthoracic approach. Surgical procedures consisted of resection and tracheotracheal anastomosis in 32 cases, resection and laryngotracheal anastomosis in 6cases and in addition laryngeal release was necessary to release anastomotic tension in 3cases. Results: The complications were 4 minor wound infections, 2 mild suture line granulomas, 1 vocal cord palsy, 2 pneumonias and 1 systemic candidiasis. Two patients who had poor consciousness and pnemonia and one who developed systemic candidiasis were expired after operation. Conclusion: We suggests resection and reconstruction of trachea is optimal procedure for up to 6cm long tracheal lesions. However, for the patients with poor consciousness or poor general conditions would be the conservative treatment preferred to the tracheal reconstruction because of high serious complications and mortalities.

• Proceedings of the KSLP Conference
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• 2017.04a
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• pp.6-9
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• 2017

• Proceedings of the KOR-BRONCHOESO Conference
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• 1977.06a
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• pp.7.2-7
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• 1977

Laryngeal web was first reported by Fleischmann in 1822, and it causes upper airway obstruction and abnormalities of phonation. Congenital webs result from an arrest of development of the larynx around the tenth week of fetal life. Acquired webs may result from cicatrical strictures of infectious lesion, traumatic and postoperative wounds. The most common site of webbing is glottic, followed by subglottic and supraglottic. We have experienced a case of simultaneous glottic and supraglottic laryngeal webs probably due to tuberculous lesions in 28 aged male who was treated by endolaryngeal microsurgery.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.178-181
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• 2014

Hunter syndrome (mucopolysaccharidosis type II, MPS II) is a X-linked lysosomal storage disease caused by a deficiency in the lysosomal enzyme, iduronate-2-sulfatase (IDS), leading to accumulation of glycosaminoglycans within lysosomes of many organs and tissues. Since the enzyme replacement therapy was approved and available in the treatment of MPS I, II, VI, early diagnosis and early therapy can bring the better prognosis of disease and the better quality of life in patients. We described a 2.5 year old child presented with frequent otitis media and developmental delay including speech impairment, who was diagnosed as Hunter syndrome with IDS NM_000202.5:c. 263G>A(p.Arg88His) mutation.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1978.06a
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• pp.7.1-7
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• 1978

The microsurgery of the laryngeal lesions was introduced by Kleinsasser in 1965. This has been utilized for diagnosis, surgical management and education of the laryngeal lesions. The laryngomic-roscopic technique appear to be useful in the evaluation of the lesions which may be precancerous, since minor degrees of epithelial thickening and alterations in the pattern of fine vessels beneath the epithelium may be observed with greater clarity. With suspension laryngoscopy, the authors experienced 11 cases of the laryngeal lesions: polyp (4 cases), web formation between false cords and true cords (1), papilloma (4), laryngeal trauma (1), and squamous cell carcinoma (1).

• Journal of Chest Surgery
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• v.33 no.7
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• pp.565-569
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• 2000

Background; Post-intubation injury is known to be the most common cause of tracheal stenosis. Treatment strategy for tracheal stenosis varies accoring to the extent of pathologic lesion. Focal mucosal lesion can be treated with laser photoablation, but full thickness tracheal lesion should be treated with resection and anastomosis. Material and Method; From Aptil 1998 to May 1999, twelve patients suffering from tracheal stenosis as a complication of endotracheal intubation were managed by resection and end-to-end anastomosis in the Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital. Result; There was no operative mortality. Five temporary vocal cord paralysis and one wound infection occurred as early complications. During 18 months of follow-up, re-stenosis was not found. Conclusion; Tracheal resection and anastomosis can be considered as an excellent surgical treatment for tracheal stenosis which developed as a complication of endotracheal intubation.

• Korean Journal of Bronchoesophagology
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• v.3 no.1
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• pp.27-36
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• 1997

초기후두암에 있어서 보존적 술식의 장점은 후두 전적출술시와 같은 국소치료효과를 얻을 수 있으면서도 후두의 생리적 기능을 최대한 보존하는데 있다. 성공적인 후두부분절제술을 위해서는 후두내에서 종양의 발생위치 및 그 확산에 대한 연부조직들의 해부학적 역할에 대한 이해가 필요하다. 과거에는 초기후두암환자에서 정상적인 목소리를 보존할 수 있다는 이점 때문에 방사선 치료가 일차적 치료요법으로 많이 시행되어 왔다. Wang 과 Wong은 성대 막양부에 국한된 병변에서 방사선치료에 대한 5년간 조절율(5-year control rate)이 92%, 전연합부를 침범한 경우가 81%, 그리고 후방으로 전파된 경우 76%로 감소된다고 보고하였다. Olofsson 등은 전연합부를 침범한 57례의 환자에서 방사선 치료를 시행하여 85.7%의 5년 생존율을 얻었으나, 57례중 15례에서 재발하여 구제수술(salvage surgery)을 시행하였고, Jesse 등$^{13)}$ 은 전연합부를 침범한 91례의 T1, T2 성문암을 방사선 치료를 시행하여 8.8%의 실패율을 보였으나 22례의 환자에서 재발하여 후에 salvage surgery를 시행하였다. 그러나 Ogura 등은 피열 연골을 침범한 79례의 환자에서 보존적 수술을 시행한 결과 6례의 환자에서 재발하여 90%의 3-years control rate를 얻을 수 있었다고 보고하였다. 이처럼 병변이 전연합부, 피열연골 혹은 후방성문하부로의 침범이 있는 경우는 방사선 치료 효과가 떨어지고, 따라서 방사선 치료에 실패할 가능성이 보다 높기 때문에 비록 방사선 치료 후 실패한 경우에서 구제 수술을 시행할 수는 있지만 후두기능을 보존하려는 궁극적인 목표를 생각할 때 보존적 수술을 우선적으로 시행하는 것이 바람직하다고 할 수 있다. Biller 등은 방사선 요법에서 실패한 성문부 종양에서는 대부분 전적출술을 시행하지만 일부는 보존적 수술을 시행할 수 있다고 보고하였다. 이들은 반대측 성문부에 종양의 침범이 없어야 하고, 전연 합으로의 파급은 있어도 가능하며 성대돌기를 제외한 피열연골이 정상이어야 하고, 종양의 성문하부로의 파급이 5mm 이내라야 하며, 연골에 침범이 없어야 하고, 성대고정이 없어야 하며, 재발 병소가 방사선요법 전의 원발병소와 상관관계가 있어야 하며, 원발병소가 후두부분적출술에 합당할 경우 보존적 술식을 시행할 수 있다고 하였다. 최근에는 다양한 후두 재건술의 발달로 보다 진행된 후두암에 대한 보존적 후두절제술들이 소개되고 있다. 단측 T3,T4 후두암 혹은 경성문암, 이상와에 위치한 암의 경우 음성만을 재건하는 후두근적출술 (near total laryngectomy)을 시행하기도 한다. 본원 이비인후과에서 1986년부터 초기성문암 51례 중 24례(47.1%)와 초기성문상암 17례 중 12례 (70.6%)에 대하여 보존적 술식을 시행하였다. 초기 성문암의 보존적 술식을 시행한 24례중, 2례(8.3%)에서 경부재발, 1례(4.2%)에서 경부재발과 동반된 폐의 원격전이가 있었으나 원발부위의 재발은 관찰되지 않았고, 술후 합병증으로 1례(4.2%)에서 후두협착, 3례(12.5%)에서 수술부위의 육아종형성, 그리고 1례(4.2%)에서 기관지염이 발생하였으나 오연으로 인한 폐렴은 발생하지 않았다. 초기성문 상암의 보존적 술식을 시행한 12례 중 1례(8.3%)에서 원발병소의 재발, 2례(16.7%)에서 경부재발이 있었으며, 술후 합병증으로 3례(16.7%)에서 오연으로 인한 폐렴의 발생이 있었지만 다른 합병증은 발생하지 않았다. 초기성문암 24례 중 재발이 나타난 3례의 환자를 제외한 21례(97.5%)와 초기성문상암 12례 중 재발이 나타난 3례의 환자를 제외한 9례 (75%)에서는 현재까지 재발소견을 보이지 않고 있다. 이러한 결과는 다른 보고자들과 유사한 결과를 보이고 있지만 아직까지 증례가 많지 않기 때문에 생존율을 얻기에는 미흡한 점이 있으며, 향후 지속적인 추적관찰이 필요할 것으로 사료된다.