• 대한두경부종양학회지
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• 제25권2호
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• pp.138-142
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• 2009

Introduction : Although bilateral exploration has been thought to be the standard therapeutic modality for primary hyperparathyroidism(pHPT) due to the admirable cure rate, questions have remained as to whether bilateral neck exploration for all patients with pHPT is needed because 80-85% of patients with pHPT have a single parathyroid adenoma. If the diseased parathyroid can be determined preoperatively, a directed and minimal operation is appropriate using the recent innovations of preoperative diagnosis and operation techniques for parathyroid diseases, the radio-guided parathyroid surgery(RGPS) is one of the standards. In especial, RGPS has been reported beneficial in special circumstances, such as in recurrent disease, ectopic parathyroid, and in mediastinal parathyroid. It can be also useful for the parathyroid disease in previously explored or irradiated neck. Material and Methods : We experienced 2 cases of pHPT successfully treated by RGPS in who previously underwent extensive neck dissection and concomitant external or internal radiotherapy. We adopted and modified the technique described by Dr. James Norman at the University of South Florida-the minimally invasive parathyroidectomy using intraoperative nuclear mapping with 99mTc-sestamibi scanning and radioactivity detection probe. Results : We acquired the successful results for these patients. Conclusion : RGPS is thought to be the alternative technique for the patients with prior central neck exploration and irradiation.

• 대한두경부종양학회지
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• 제36권1호
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• pp.49-52
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• 2020

The most common cause of primary hyperparathyroidism is a single adenoma accounts for more than 85% and about 1-2% in multiple occurrence. The adenoma arises mainly in the neck and rarely in the mediastinum. The simultaneous occurrence is extremely rare. A 73-year-old man came to our clinic complained about sense of falling forward during last eight months. The brain MRI and vestibular function test showed non-specific findings but total calcium and intact parathyroid hormone levels were markedly elevated. Radiologic studies and sesta-MIBI scan revealed multiple masses in lower paratracheal area and superior mediastinum. We performed mass excision with transcervical approach and all of them were diagnosed as parathyroid adenoma. After surgery, intact PTH and calcium levels returned to the normal range and his symptoms were dramatically improved. We report the unique and rare disease entity with a brief literature review.

• 대한두경부종양학회지
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• 제15권2호
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• pp.194-199
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• 1999

Introduction: Since 1996, Dr. James Norman has successfully performed mimimally invasive radio-guided parathyroidectomy(MIRP) using intraoperative nuclear mapping with $^{99m}Tc$ sestamibi scanning and radioactivity detection probe. Objectives: We aimed to introduce this new surgical technique and evaluate it's efficacy by our own experiences. Method: From May to October 1999, five consecutive patients with primary hyperparathyroidism underwent parathyroidectomy by using modified MIRP technique. $^{99m}Tc$ sestamibi scanning was performed 1.0 or 1.5 hour before operation. After intraoperative localization of the tumor under the guidance of quantitative gamma counting with a NEVIGATOR probe, an unilateral small skin incision(3.0-4.0cm) was placed. Without a skin flap, the strap muscle was directly divided with the use of a Harmonic scalpel. After careful dissection, the parathyroid tumor was removed. Result: In all patients, a single adenoma could be easily detected and removed by this new technique. Mean incision length was 3.2cm(3.0-4.0cm) and operative time ranged from 40 to 110minute. All the patients were discharged within 2 days of surgery without any complication. Conclusion: This new operative technique could become the most minimally invasive alternative to the standard operative procedure for parathyroid adenoma.

• Biomolecules & Therapeutics
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• 제30권4호
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• pp.309-319
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• 2022

Metabolic bone diseases are serious health issues worldwide, since several million individuals over the age of 50 are at risk of bone damage and should be worried about their bone health. One in every two women and one in every four men will break a bone during their lifetime due to a metabolic bone disease. Early detection, raising bone health awareness, and maintaining a balanced healthy diet may reduce the risk of skeletal fractures caused by metabolic bone diseases. This review compiles information on the most common metabolic bone diseases (osteoporosis, primary hyperparathyroidism, osteomalacia, and fluorosis disease) seen in the global population, including their symptoms, mechanisms, and causes, as well as discussing their prevention and the development of new drugs for treatment. A large amount of research literature suggests that balanced nutrition and balanced periodic supplementation of calcium, phosphate, and vitamin D can improve re-absorption and the regrowth of bones, and inhibit the formation of skeletal fractures, except in the case of hereditary bone diseases. Meanwhile, new and improved drug formulations, such as raloxifene, teriparatide, sclerostin, denosumab, and abaloparatide, have been successfully developed and administered as treatments for metabolic bone diseases, while others (romososumab and odanacatib) are in various stages of clinical trials.

• Childhood Kidney Diseases
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• 제26권1호
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• pp.63-67
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• 2022

Nephrocalcinosis often occurs in infants and is caused by excessive calcium or vitamin D supplementation, neonatal primary hyperparathyroidism, and genetic disorders. Idiopathic infantile hypercalcemia (IIH), a rare cause of nephrocalcinosis, results from genetic defects in CYP24A1 or SLC34A1. Mutations in CYP24A1, which encodes 25-hydroxyvitamin D 24-hydroxylase, disrupt active vitamin D degradation. IIH clinically manifests as failure to thrive and hypercalcemia within the first year of life and usually remits spontaneously. Herein, we present a case of IIH wih CYP24A1 mutations. An 11-month-old girl visited our hospital with incidental hypercalcemia. She showed failure to thrive, and her oral intake had decreased over time since the age of 6 months. Her initial serum parathyroid hormone level was low, 25-OH vitamin D and 1,25(OH)₂ vitamin D levels were normal, and renal ultrasonography showed bilateral nephrocalcinosis. Whole-exome sequencing revealed compound heterozygous variants in CYP24A1 (NM_000782.4:c.376C>T [p.Pro126Ser] and c.1310C>A [p.Pro437His]). Although her hypercalcemia and poor oral intake spontaneously resolved in approximately 8 months, we suggested that her nephrocalcinosis and renal function be regularly checked in consideration of potential asymptomatic renal damage. Hypercalcemia caused by IIH should be suspected in infants with severe nephrocalcinosis, especially when presenting with failure to thrive.

• Journal of Yeungnam Medical Science
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• 제14권2호
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• pp.459-466
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• 1997

저자들은 원발성 부갑상선 기능 항진증과 함께 전신성 석회증을 동반한 부갑상선암 환자에서 부갑상선암 제거후 골 스캔상 폐와 위장의 전신성 석회화가 사라진 증례를 경험하여 보고하는 바이다.

• Childhood Kidney Diseases
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• 제13권2호
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• pp.176-188
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• 2009

목적 : 복막투석요법은 말기 신부전 환아에서 신이식 다음으로 추천되는 신대체 요법으로 현재 말기 신부전 환아들의 장기 생존율 향상으로 복막투석요법 환아 수가 점차 증가하고 있다. 현재 한국 내 복막투석요법 소아에 대한 현황을 파악하고자 이번 연구를 시행하였다. 방법 : 2008년 5월 국내 4개 대학병원을 대상으로 전자우편 설문조사를 시행하였고 현재 3개월 이상 복막투석 중인 103명의 소아를 대상으로 자료를 분석하였다. 결과 : 대상 환아 103명의 남녀비는 1.6:1로 남아가 많았고, 평균 연령은 $11.5{\pm}4.9$세(0-19세), 투석시작 전 말기 신부전의 원인 중 원발성 사구체 질환이 34%로 가장 많았다. 복막투석의 형태는 지속적 외래 복막투석법이 42.7%로 가장 많았고, 주간 전체 Kt/V는 $2.1{\pm}0.7$ (0.3-4.1), 복막평형검사상 저 투과성이 36.8%, 저평균 투과성이 31.6%으로 가장 많은 비율을 차지했다. 체중 표준편차점수(Z-score)는 $-1.00{\pm}1.20$(-4.54-+2.50), 신장 표준편차점수(Z-score)는 $-1.55{\pm}1.65$(-9.42-+1.87)였고, 성장호르몬은 24.3%에서 투여 중이었다. 38.8%의 환아에서 이완기 혹은 수축기 혈압 95 백분위수 이상의 고혈압을 보였고, 64.0%의 환아가 항고혈압제 투여 중이었다. 평균 혈청 혈색소는 $10.5{\pm}1.4$ g/dL, 평균 혈청 칼슘은 $9.7{\pm}0.7$ mg/dL, 평균 혈청 인은 $5.4{\pm}1.4$ mg/dL, 평균 부갑상선 호르몬은 $324.2{\pm}342.8$ pg/mL였다. 결론 : 국내 복막투석요법 소아에서 원발성 사구체 질환이 소아 말기 신부전의 가장 흔한 원인이고, 지속적 외래 복막투석법이 가장 흔히 사용되는 복막투석 형태이며, 복막평형검사상 저 투과성과 저평균 투과성이 가장 많았다. 대부분의 환아가 적절한 신장과 체중을 유지하고 있었으나 저신장 환아의 경우 성장 호르몬 치료가 제대로 이루어지지 않고 있었고 여전히 많은 환아에서 빈혈과 고혈압이 관찰되었다. 혈청 칼슘과 인은 비교적 정상범위를 유지하고 있으나 많은 환아가 이차성 부갑상선 기능 항진증을 보였다.

• Tuberculosis and Respiratory Diseases
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• 제47권4호
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• pp.525-532
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• 1999

악성질환에서 체액성 고칼슘혈증은 주로 부갑상선호르몬관련단백질(parathyroid hormone related hormone ; PTHrP)이 매개물질로 작용하여 발생한다. 이러한 PTHrP포 인한 고칼슘혈중이 발생한 경우에는 원발성부갑상선기능항진증을 동반한 경우를 제외하고는 모든 환자에서 부갑상선호르몬(PTH) 농도는 저하되며 PTHrP는 증가하는 것으로 알려져있다. 특히 부갑상선이외의 종양에서 이소성 부갑상선호르몬을 생성한다거나 PTH와 PTHrP를 동시에 산생하는 경우는 매우 드물다. 저자들은 고칼슘혈증을 동반한 편평상피세포 폐암환자에서 혈청 부갑상선호르몬(PTH)과 부갑상선호르몬관련단백질(PTHrP)이 동시에 증가된 1례를 경험하여 이에 보고한다. 61세 남자 환자로서 혈청 칼슘은 7.5 mEq/L로 증가되어 있었으며, iPTH와 PTHrP(C 말단 부위 : 109-138)는 각각 150 pg/mL, 99.1 pmol/L로 모두 상승되어 있었다. 골전이나 부갑상선 기능 장애의 동반여부를 알기 위해서 시행한 골스캔이나 경부초음파, 전산화단층촬영, Tc-99m MIBI 스캔 검사에서 모두 정상소견을 보였다. 결론적으로, 본 증례는 고칼슘혈증을 보인 편평상피상피세포 폐암환자에서 PTH와 PTHrP가 동시에 증가된 경우이다. 이러한 매개물이 동시에 증가한 원인으로 PTH와 PTHrP을 분비하는 타병변이 있을 수 있지만, PTH와 PTHrP를 동시에 분비할 수 있는 주된 기관인 부갑상선에 대한 상기 검사상 특이한 이상소견이 없었던점으로 미루어 보아 편평상피세포 폐암조직으로부터 이소성 PTH와 PTHrP가 동시에 분비되었을 가능성도 있다.