• Korean Journal of Bronchoesophagology
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• v.5 no.1
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• pp.55-61
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• 1999

The authors recently experienced a case of ectopic thyroid. A 15-year-old female patient visited to ENT department with the complaint of the submental neck mass. On physical examination, the mass was relatively firm, non tender and 3$\times$2cm in size. Oral cavity examination revealed 0.5$\times$0.5cm sized pink colored mass near the foramen cecum area. Suspecting ectopic thyroid, thyroid function test, thyroid scan, neck computed tomogram scan were performed. Thyroid scan revealed a functioning thyroid on the lingual and submental area without normal uptake in the anterior neck area. Thyroid (unction test was normal. Pre-contrast computed tomogram scan revealed an ectopic thyroid in the lingual and submental area. A review of literature concerning ectopic thyroid was discussed.

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.368-380
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• 1999

Purpose: The purpose of this study was to evaluate and compare the scintigraphic findings and diagnostic accuracy of double-phase Tc-99m sestamibi scan in primary and secondary hyperparathyroidism (HPT). Materials and Methods: We retrospectively reviewed 16 cases of primary (18 lesions) and 11 cases of secondary HPT (44 lesions) who underwent Tc-99m-sestamibi scan before the surgical intervention. Scan was performed using LEM camera (Siemens, Germany) after the injection of 740MBq of Tc-99m sestamibi. Routine image consisted of baseline and 3-hour delayed images and each image was obtained using both parallel and pine hole collimator. The study population was 27 patients (male/female=5/22, age: $49.1{\pm}10.8$). Results: Eighteen lesions of primary HPT consisted of 13 adenomas and S hyperplasias, while all lesions of secondary HPT were hyperplasias. Among the case of primary HPT, we could detect all the lesions of 13 adenomas but only 2 lesions of 5 hyperplasias (40%) could be detected by double phase scintigraphy. Three cases of primary lesion showed decreased uptake in delayed images compared with baseline. The sensitivity, specificity, positive predictive value and accuracy of primary and secondary HPT were 58.8% (10/17), 83.3% (10/12), 83.3% (10/12), 75.9% (22/29), and 37.5% (15/40), 50% (2/4), 88.2% (15/17), 38.6% (17/44), respectively. Overall sensitivity, specificity, positive predictive value and accuracy were 43.9% (25/57), 75% (12/16), 86.2% (25/29), and 53.4% (39/73). There were no statistical difference between the weight of primary and secondary HPT lesion (p>0.05). Conclusion: Tc-99m sestamibi scan is fairly good modality to detect parathyroid lesion in patient with primary HPT before the surgical intervention. However, since some of cases may reveal decreased uptake in delayed image, a careful attention to the findings of baseline images may be helpful. Still the low accuracy of sestamibi scan in diagnosis of secondary HPT prohibits routine use of it for this disease.

• Advances in pediatric surgery
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• v.2 no.2
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• pp.133-137
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• 1996

A 17 month-old girl presented in the pediatric clinic on November 27th, 1990 with a neck mass. The mass was 2 cm in diameter, firm in consistency and movable on the upper pole of the thyroid cartilage in the midline. The technetium thyroid scan showed a hot reactivity at the compatible site of the mass, but no other radioactivity in either site of the normal thyroid positions. At her second visit on January 23th, 1996, the mass had enlarged up to 3.5cm in diameter in the same location of the neck. The follow up thyroid scan revealed a walnut sized, snowman-like radioactivity. One of the snowman-like double images seemed to be a lingual ectopic thyroid and the other a midline ectopic thyroid remnant in the infrahyoid level. This interpretation was supported by the computed tomography of the neck, which showed a ligual mass in the foramen cecum area and an another mass in the anterior comis-sure of the larynx in the mildline. Thyroid function test was normal except a slightly increased TSH. As a result of this changing pattern of thyroid radioactive images, a case of a lingual thyroid as well as another midline ectopic thyroid tissue at the infrahyoid level is reported.

• Archives of Craniofacial Surgery
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• v.11 no.1
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• pp.23-27
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• 2010

목적: 모반양 기저세포암 증후군 (Nevoid basal cell carcinoma syndrome) 또는 골린-골츠 증후군은 한국에서는 흔하지 않은 증후군으로 주로 상염색체 우성으로 유전하고 다기관 장애가 나타날 수 있으며 높은 표현율과 다양한 표현도를 특징으로 한다. 모반양 기저세포암 증후군의 진단 기준에는 다발성 기저세포암, 이소성 석회화(ectopic calcification), 손 또는 발바닥 오목 (palma or plantar pits), 치성 각화 낭종(odontogenic keratocysts), 가족력 및 골격계, 신경계, 안, 비뇨생식계 및 심장혈관의 이상 등이 있다. 본원에서 주로 두부의 다발성 기저세포암을 가진 모반양 기저세포암 증후군 환자를 경험하여 보고하고자 한다. 방법: 환자는 2007년 4월 두부의 색소성 모반으로 피부과에서 시행한 펀치 생검에서 기저세포암을 진단받고 의뢰되었으며, 이후 2009년 7월까지 14회의 추가적인 절제 및 조직 검사를 시행하였다. 환자는 갑상샘 유두암종의 재발로 인해 갑상샘 절제술을 2회 시행한 과거력이 있었으며 이학적 검사와 일반 혈액, 소변, 간 기능 및 갑상선 기능 검사를 시행하였고, 흉부와 늑골 방사선 검사, 심전도와 안면부 및 두부 컴퓨터단층촬영과 유전자 검사를 시행하였다. 결과: 두부와 안면부에서 절제한 27개의 병변 중 23개(85%)가 기저세포암으로 진단되었으며, 치성 각화 낭종과 대뇌겸 석회화, 이학적 검사에서 손바닥 오목이 발견되었다. 하복부 초음파에서 난소 낭종이 발견되었으나 조직 검사는 시행되지 않았다. 결론: 한국에서 모반양 기저세포암 증후군에 대한 연구는 주로 치과와 피부과 영역에서 국한되었으며, 특히 치과 영역에서의 보고는 치성 각화 낭종 및 손바닥 오목에 초점을 둔 것이 대부분이었다. 이에 본원에서는 주로 두부의 다발성 기저세포암을 가진 모반양 기저세포암의 환자를 경험하였으며, 초기에 발견된 작은 병변의 제거 시 2mm의 정상 조직을 포함하여 절제하였어도 3년간의 경과관찰 중 재발없이 좋은 결과를 얻을 수 있어 이를 보고하는 바이다.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.7-14
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• 2021

Purpose: Pseudohypoparathyroidism (PHP) is caused by genetic and epigenetic alteration in the GNAS locus, and characterized by the resistance to multiple hormones and the Albright's hereditary osteodystrophy (AHO) phenotype. This study investigated the phenotypic characteristics and molecular features of PHP. Methods: Eight patients who diagnosed as PHP were enrolled at Pusan National University Children's hospital and clinical features, biochemical and genetic findings were retrospectively reviewed. Results: Of a total of 8 patients, 5 were diagnosed with PHP1a, and 3 were diagnosed with PHP1b. Patients with PHP1a had three different mutations in the GNAS gene, and patients with PHPIb had imprinting defect in differentially methylated regions (DMRs) of the GNAS locus. Two novel GNAS variants were identified in patients with PHP1a, including c.313-2A>T and c.1094G>A. All patients with PHP1a displayed AHO features; short stature (80%), brachydactyly (80%), a round face (80%), obesity (40%), heterotopic ossification (60%), and intellectual disability (60%), whereas only one patient (33.3%) with PHP1b showed AHO feature such as a round face. When phenotypic features between PHP1a and PHP1b patients were compared, patients with PHP1b showed a tendency of higher current height standard deviation scores (SDS) compared to patients with PHP1a, (-3.2±2.1 vs.-1.1±0.8; P=0.06) Conclusions: This study summarizes the phenotypic and genetic features of the PHP patients. Although we found considerable clinical overlap between PHP1a and PHP1b, further long-term follow-up is needed to evaluate the growth and development of children with PHP, as well as the effects of end-organ resistances to endocrine hormones.

• Korean Journal of Veterinary Research
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• v.22 no.2
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• pp.211-219
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• 1982

Chickens from 10 to 32 weeks of age were inoculated with P. multocida via seven routs(intravenous, intramuscular, intraperitoneal, subcutaneous, into ear, intranasal, per oral). The development or distribution of disseminated intravascular coagulation (DIC) in multiple organs and the role of P. multocida endotoxins in disease process of fowl cholera were studied. The histological diagnosis of DIC was made by demonstration of fibrinous in arterioles, capillaries, venules and medium-sized blood vessels. The presence of fibrinous thrombi in blood vessels of multiple organs was observed in chickens which died within approximately 3 days post inoculation. Fibrinous thrombi were observed most frequently in the lung(90% of all cases with DIC) followed by liver (70%), kidney (60%), heart(20%), spleen, brain, pancreas, thymus and thyroid gland. The density of fibrinous thrombi (i.e. the number of thrombi per section) was greatest in the lung, followed by spleen, kidney, liver and heart. It is thought that the widespread hemorrhage of acute fowl cholera is also caused by P. multocida endotoxin which initiates DIC in variety of organs. The cause of death for the chickens after infection with acute fowl cholera is probably due to an endotoxin (septic) shock accompanied with DIC in multiple organs.