• 대한두경부종양학회지
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• 제15권1호
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• pp.61-65
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• 1999

Introduction: The surgical treatment of Graves' disease has several advantage such as rapid and long lasting therapeutic effect, relatively low incidence of postoperative hypothyroidism and recurrence. Because of less frequent but serious postoperative complications, the operation have not been casually used for treatment of Graves' disease. Hypoclacemia, hoarseness and hematomas are common complications after operative treatment. However, the causative mechanisms of these complications have not been well explained. Objective: We aimed to analyze the risk factors affecting the occurance of complications in surgical treatment for Graves' disease and to evaluate what are the causative mechanisms of postoperative complications. Patients and Methods: From January 1985 to December 1998, a total of 668 surgically treated patients for Graves' disease were enrolled in this study. The incidence of postoperative complications were analyzed in relation to types of surgery, preoperaive preparation, specimen weight, bleeding amount and remnant weight. Results: Postoperative complication was developed in 108(16.2%) of 668 patients. The most common complication was transient hypocalcemia in 85 cases(12.7%). Other complications were: Hematoma in 9 cases(1.3%), transient hoarseness in 5 cases(0.7%), permanent hypocalcemia in 6 cases(0.9%), permanent hoarseness in 3 case(0.4%). The important etiologic factors affecting the occurance of complications were preoperative preparation, perioperative thyroid weight, and remnant thyroid weight. Since 1993, the incidence of complication was more decreased than that in before. Conclusion: To decrease the incidence of postoperative complications of Graves' disease, the patients should be selected carefully, sufficient preoperative preparation should be achieved, and the operation should be performed by well-experienced surgeons.

• 한국임상수의학회지
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• 제26권6호
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• pp.603-605
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• 2009

7연령의 암컷 미니어처 슈나우저견이 갑작스런 발작과 근육 떨림 증상을 주증으로 내원하였다. 내원 당시 발열과 헐떡임이 두드러졌으며, 신체검사 상에서 현저한 앞다리 근육 강직증상, 근육부분수축과 뻣뻣한 걸음걸이가 관찰되었다. 실험실 검사에서 심각한 저칼슘혈증과 함께 고인혈증이 특징적으로 관찰되었다. 이와 더불어 관찰된 낮은 농도의 부갑상선 호르몬과 이온화된 칼슘농도, 뇨중 칼슘과 인의 배설 량 측정을 통하여 원발성 부갑상선기능저하증이 진단되었다. 이 환축은 10% 글루콘산칼슘 투여를 통한 초기 치료에 반응이 좋았으며, 비타민 D 유사체와 칼슘 보조제를 통한 유지 치료에 의해 잘 관리 되고 있다. 부작용으로 일시적인 고인혈증이 나타났으나, 이 역시 경구용인 흡착제인 sevelamer hydrochloride를 이용한 치료에 의해 안정화 되었다. 결론적으로 본 증례의 경우 개에서 나타나는 원발성 부갑상선기능저하증의 임상증상과 특징적인 실험실 검사 결과 그리고, 치료 반응에 대한 국내 첫 증례보고이다.

• The Korean Journal of Pain
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• 제30권2호
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• pp.86-92
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• 2017

Osteoblasts, originating from mesenchymal cells, make the receptor activator of the nuclear factor kappa B ligand (RANKL) and osteoprotegerin (OPG) in order to control differentiation of activated osteoclasts, originating from hematopoietic stem cells. When the RANKL binds to the RANK of the pre-osteoclasts or mature osteoclasts, bone resorption increases. On the contrary, when OPG binds to the RANK, bone resorption decreases. Denosumab (AMG 162), like OPG (a decoy receptor), binds to the RANKL, and reduces binding between the RANK and the RANKL resulting in inhibition of osteoclastogenesis and reduction of bone resorption. Bisphosphonates (BPs), which bind to the bone mineral and occupy the site of resorption performed by activated osteoclasts, are still the drugs of choice to prevent and treat osteoporosis. The merits of denosumab are reversibility targeting the RANKL, lack of adverse gastrointestinal events, improved adherence due to convenient biannual subcutaneous administration, and potential use with impaired renal function. The known adverse reactions are musculoskeletal pain, increased infections with adverse dermatologic reactions, osteonecrosis of the jaw, hypersensitivity reaction, and hypocalcemia. Treatment with 60 mg of denosumab reduces the bone resorption marker, serum type 1 C-telopeptide, by 3 days, with maximum reduction occurring by 1 month. The mean time to maximum denosumab concentration is 10 days with a mean half-life of 25.4 days. In conclusion, the convenient biannual subcutaneous administration of 60 mg of denosumab can be considered as a first-line treatment for osteoporosis in cases of low compliance with BPs due to gastrointestinal trouble and impaired renal function.

• Laboratory Medicine Online
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• 제7권2호
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• pp.83-87
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• 2017

Pseudohypoparathyroidism (PHP) is a rare disorder caused by genetic and epigenetic aberrations in the GNAS complex locus resulting in impaired expression of stimulatory G protein ($Gs{\alpha}$). PHP type Ib (PHP-Ib) is characterized by hypocalcemia and hyperphosphatemia due to renal resistance to the parathyroid hormone, and is distinguished from PHP-Ia by the absence of osteodystrophic features. An 11-yr-old boy presented with poor oral intake and cramping lower limb pain after physical activity. Laboratory studies revealed hypocalcemia, hyperphosphatemia, and increased parathyroid hormone levels. The GNAS complex locus was evaluated using the methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) assay. Gain of methylation in the NESP55 domain and loss of methylation in the antisense (AS) transcript, XL, and A/B domains in the maternal allele were observed. Consequently, we present a case of PHP-Ib diagnosed using MS-MLPA.

• Endocrinology and Metabolism
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• 제33권4호
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• pp.485-492
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• 2018

Background: Increasing evidence supports interplay between aldosterone and parathyroid hormone (PTH), which may aggravate cardiovascular complications in various heart diseases. Negative structural cardiovascular remodeling by primary aldosteronism (PA) is also suspected to be associated with changes in calcium levels. However, to date, few clinical studies have examined how changes in calcium and PTH levels influence cardiovascular outcomes in PA patients. Therefore, we investigated the impact of altered calcium homeostasis caused by excessive aldosterone on cardiovascular parameters in patients with PA. Methods: Forty-two patients (mean age $48.8{\pm}10.9$ years; 1:1, male:female) whose plasma aldosterone concentration/plasma renin activity ratio was more than 30 were selected among those who had visited Severance Hospital from 2010 to 2014. All patients underwent adrenal venous sampling with complete access to both adrenal veins. Results: The prevalence of unilateral adrenal adenoma (54.8%) was similar to that of bilateral adrenal hyperplasia. Mean serum corrected calcium level was $8.9{\pm}0.3mg/dL$ (range, 8.3 to 9.9). The corrected calcium level had a negative linear correlation with left ventricular end-diastolic diameter (LVEDD, ${\rho}=-0.424$, P=0.031). Moreover, multivariable regression analysis showed that the corrected calcium level was marginally associated with the LVEDD and corrected QT (QTc) interval (${\beta}=-0.366$, P=0.068 and ${\beta}=-0.252$, P=0.070, respectively). Conclusion: Aldosterone-mediated hypercalciuria and subsequent hypocalcemia may be partly involved in the development of cardiac remodeling as well as a prolonged QTc interval, in subjects with PA, thereby triggering deleterious effects on target organs additively.

• 대한유전성대사질환학회지
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• 제20권2호
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• pp.44-49
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• 2020

가성부갑상샘기능저하증(pseudohypoparathyroidism, PHP)은 부갑상샘호르몬에 대해 표적 기관이 저항성을 가지며, 저칼슘혈증과 고인산혈증을 특징으로 하는 질병이다. PHP의 원인은 자극형 G 단백의 신호전달이 문제인데, α-아형을 인코딩하는 GNAS 유전자와 GNAS 유전자 상부의 각인 이상으로 발생한다. 가족력 없이 발생하는 산발성 PHP 1b형은 GNAS 유전자 상류 다발 지역의 메칠화 이상으로, 그 중 일부는 모계 유전형이 소실되고, 부계 유전형만이 표현된다. 본 논문에서는 간헐적 강직을 주소로 내원한 10.8세 남아에서 발생한 20번 염색체 장완의 부계 단친성 이염색체에 의한 산발성 PHP 1b형의 증례를 고찰해보고자 한다.

• Journal of Yeungnam Medical Science
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• 제14권1호
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• pp.220-226
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• 1997

저자들은 경련을 주소로 내원한 특발성 부갑상선 기능저하증에서 뇌기저핵, 소뇌, 뇌실 주위의 광범위한 석회침착을 보인 환자를 치험하여 방사선학적 뇌단층촬영상 특징적 소견과 그 병태생리 및 임상증상과의 연관관계를 문헌고찰과 함께 보고 한다.

• Asian Pacific Journal of Cancer Prevention
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• 제13권8호
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• pp.3555-3560
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• 2012

The tumour lysis syndrome (TLS) is a group of metabolic abnormalities caused by rapid and unexpected release of cellular components into the circulation as a result of massive destruction of rapidly proliferating malignant cells. It usually develops in patients with hematologic malignancies like acute lymphoid leukemia, non-Hodgkin and Burkitt's lymphoma after initiation of chemotherapy or may, rarely, occur spontaneously. Though TLS is seldom observed in relation to solid tumours, there have been reports of connections with examples such as lung, liver, breast, gastric carcinomas. The clinical manifestations of TLS include hyperuricemia, hyperkalemia, hyperphosphatemia and hypocalcemia. These indications if untreated lead to life-threatening complications such as acute renal failure, cardiac arrhythmias, seizures, and eventually death due to multiorgan failure. Therefore early detection of TLS is of vital importance. This can be accomplished by identification of high risk patients, implementation of suitable prophylactic measures andmonitoring of the electrolyte levels in patients undergoing chemotherapy.

• Clinical and Experimental Pediatrics
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• 제57권1호
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• pp.1-18
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• 2014

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

• 대한임상독성학회지
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• 제5권2호
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• pp.135-137
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• 2007

Hydrofluoric acid is a weak inorganic acid used for etching and as rust removals. Systemic toxicity after oral ingestion induces rapid development of hypocalcemia and hyperkalemia, leading to ventricular fibrillation and finally asystole. We report a case of intentional ingestion of hydrofluoric acid producing an altered mental state at the time of the patient's arrival in the emergency department. The patient died approximately 80 minutes after the exposure with asystol.