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- Volume 18(5); October 2003
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Review Articles
- Loss of Function of the Autoimmune Regulator (AIRE) Gene and Development of Poyendocrinopathy.
- Yong Soo Park
- J Korean Endocr Soc. 2003;18(5):439-449. Published online October 1, 2003
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- The New Concept of Hormone Replacement Therapy.
- Ki Ok Han
- J Korean Endocr Soc. 2003;18(5):450-455. Published online October 1, 2003
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Original Articles
- Determination of Glucocorticoid Replacement Therapy and Adequate Maintenance Dose in Patients with Secondary Adrenal Insufficiency.
- Sang Wan Kim, Hye Seung Jung, Seong Hee Kwon, Do Joon Park, Chan Soo Shin, Kyung Soo Park, Seong Yeon Kim, Bo Youn Cho, Hong Kyu Lee
- J Korean Endocr Soc. 2003;18(5):456-464. Published online October 1, 2003
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Abstract
PDF - BACKGROUND
Determination of the adequate dose of glucocorticoid replacement therapy, in patients with secondary adrenal insufficiency, is of great importance to avoid the consequences of under or over treatment. The aims of this study were: 1) to assess the value of adrenal cortical function tests in determining whether glucocorticoid replacement should be given, and 2) to investigate the adequate maintenance dose of glucocorticoid in patients with secondary adrenal insufficiency. METHODS: Forty patients, with secondary adrenal insufficiency, confirmed by the insulin-induced hypoglycemia test (IHT), were studied. All subjects underwent basal serum cortisol measurement, IHT and 250 g rapid ACTH stimulation tests (AST). The clinical usefulness of these tests, for the determination of glucocorticoid replacement therapy, was evaluated in patients with secondary adrenal insufficiency. 26 of the 40 patients had received prednisolone (Pd) (5 mg per day) replacement due to symptoms from adrenal insufficiency. The dose of Pd was serially changed from 5 to 3.75, and then to 6.25 mg per day, every 3 month. The measured lipid parameters, serum osteocalcin and urinary N-telopeptide were measured and the quality of life evaluated by the administration of an Addisonian questionnaire, both before and after the dose changes. RESULTS: 1) For all tests, cut-offs were selected that would provide adequate specificity and sensitivity. When the cut-offs were set to provide 95% specificity, the corresponding sensitivitycut-off values, obtained with basal serum cortisol, peak serum cortisol in IHT and AST were: 88.4% <5 microgram/dL, 80.7% <11 microgram/dL and 76.9% <16 microgram/dL. 2) The urinary type I collagen N-telopeptide, total cholesterol, HDL- and LDL-cholesterol levels were significantly increased, and the serum osteocalcin levels significantly decreased when the daily dose of Pd was increased to 6.25 from 3.75 or 5 mg. The LDL-cholesterol levels especially, were significantly increased, even though the change in the Pd from 3.75 to 5 mg per day was subtle. CONCLUSION: The basal cortisol levels, HPA axis tests and the symptoms of patients may be helpful to determine whether prednisolone replacement therapy should be given. It is suggest that an adequate dose of glucocorticoid replacement therapy should be not exceed Pd 5mg per day, so as not to have adverse effects on the bone and lipid metabolisms.
- The Change of Pulmonary Artery Pressure in Graves'Disease Before and After Treatment.
- Taek Man Nam, Han Soo Cho, Jin Seo Lee, Young Rim Song, Doo Man Kim, Young Cheoul Doo, Cheol Young Park, In Kyung Jeong, Eun Gyung Hong, Seong Jin Lee, Gi Weon Oh, Hyeon Kyu Kim, Jae Myung Yu, Moon Gi Choi, Hyung Joon Yoo, Sung Woo Park
- J Korean Endocr Soc. 2003;18(5):465-472. Published online October 1, 2003
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Abstract
PDF
- BACKGROUND
Exertional symptoms, dyspnea and impaired effort tolerance are common in patients with Graves' disease. Proposed explanations include: high-output left heart failure, ineffective oxygen utilization and respiratory muscle weakness. In addition, pulmonary hypertension has also been reported in patients with Graves' disease. A high prevalence of hypothyroidism and positive thyroid autoantibody were also observed in patients with pulmonary arterial hypertension. Therefore, the pulmonary artery pressure in patients with Graves' disease was evaluated. METHODS: Two-dimensional and Doppler echocardiographic examinations (Hewlett Packard Sonos 2500) were performed to determine the pulmonary artery (PA) pressure in 26 Graves' disease patients, both before and after treatment (23 patients with propylthiouracil and 3 with RAI), and in 10 euthyroid controls. The changes in the PA pressure after treatment were evaluated in 13 patients with Graves' disease, who became euthyroid after treatment. RESULTS: The pulmonary artery pressure was increased in the untreated Graves' disease patients compared to the normal controls (23.5+/-2.32 vs. 29.6+/-10.3 mmHg). 38.5% of the Graves' disease patients (10/26) showed pulmonary arterial hypertension (PA>30 mmHg) and the serum TBII levelwas higher in the Graves' disease patients with pulmonary arterial hypertension than in those with normal PA pressure (P<0.05). In the Graves' patients who became euthyroid after treatment, the PA pressure was significantly decreased. CONCLUSION: 38.5% of the untreated Graves' disease patients showed pulmonary arterial hypertension, and the pulmonary artery pressure was significantly decreased in those who became euthyroid after treatment. The pathogenesis and clinical importance of pulmonary arterial hypertension in Graves' disease requires further studies.
- Relationship with Serum Adiponectin Concentrations and Obesity in Korean Children.
- Hyoun Ah Kim, Hyoung Suk Lee, Chul Sik Kim, Chul Woo Ahn, Yoon Sok Chung, Kwan Woo Lee, Kap Bum Huh, Dae Jung Kim
- J Korean Endocr Soc. 2003;18(5):473-480. Published online October 1, 2003
- 1,306 View
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Abstract
PDF
- BACKGROUND
Adiponectin is an adipocytokine that is highly specific to adipose tissue. In contrast to other adipocytokine, the adiponectin levels are decreased in obesity and/or type 2 diabetes. There are few studies regarding the correlation between the adiponectin concentration and obesity in children. Thus, whether the serum adiponectin concentrations are associated with adiposity in children was investigated. METHODS: One hundred and sixty four subjects were selected from the participants in an ongoing study on the relationship between birth weight and insulin resistance in children. The current weights, heights, body fat percentages, waist circumferences, blood pressures, lipid profiles and insulin resistance, by the HOMA method, were measured in all the subjects. The serum adiponectin concentrations were determined by a validated sandwich ELISA, using a human adiponectin-specific antibody. RESULTS: The serum adiponectin concentration was negatively correlated with the body mass index, waist circumference, systolic blood pressure, insulin resistance by HOMA and serum triglycerides, and positively correlated with the serum HDL cholesterol level. The serum adiponectin concentrations in the boys were significantly lower than in the girls. In a multiple regression analysis, the serum adiponectin concentration was strongly associated with waist circumference and gender. CONCLUSION: It is concluded that there was an inverse relationship between the serum adiponectin concentration and abdominal adiposity in children. However, further studies on independent gender differences on adiponectin are needed.
- A Case of Multiple Endocrine Neoplasia 2A with Germ Line Mutation of RET Gene.
- Hee Young Kim, Ji Yeon Lee, Sung Bum Kim, Kye Won Lee, Ji A Seo, Jeong Heon Oh, Sin Gon Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi, Nan Hee Kim
- J Korean Endocr Soc. 2003;18(5):481-488. Published online October 1, 2003
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- 19 Download
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Abstract
PDF
- Multiple endocrine neoplasia 2A (MEN 2A) is an autosomal dominantly inherited disease, composed of medullary thyroid carcinoma, pheochromocytoma and hyperparathyroidism. The activation of germ-line mutations in the RET proto-oncogene are responsible for MEN 2. The analysis of the RET mutations has replaced the measurement of the calcitonin level in the diagnosis of the MEN carrier state. Specific RET codon mutations correlate with the MEN 2 syndromic variant, the age at onset of the medullary thyroid carcinoma (MTC) and the aggressiveness of the MTC. Herein, our experience of a 47-year-old woman, who had a bilateral pheochromocytoma and MTC, and MEN 2A confirmed by the detection of an RET proto-oncogene mutation at axon 10 on codon 618, is reported. Her sister was found to have the same mutant gene. After a total thyroidectomy and bilateral adrenalectomy, the calcitonin and catecholamine levels were normalized, and the patient discharged without problems. This case is reported, with a review of the literature.
Case Reports
- A Case of Cushing's Syndrome Complicating Pregnancy Adrenalectomized after Delivery.
- Jeong Soo Cho, Jae Seok Jeon, Kyu Jeong Ahn, Byoung Joon Kim, Sung Ho Kwon, Eun Gyu Lee, Won Wook Choi, Sang Won Park, Won Mee Lee
- J Korean Endocr Soc. 2003;18(5):489-495. Published online October 1, 2003
- 1,141 View
- 17 Download
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Abstract
PDF
- Pregnancy is rare in women with Cushing's syndrome, as the associated infertility is related to excess cortisol and/or androgen. However, approximately 100 such cases have been reported, with 50% due to an adrenal cortical adenoma. Establishing a diagnosis and cause can be difficult. Clinically, striae, hypertension and gestational diabetes are common features in pregnancy, with hypertension and diabetes being the most common signs of Cushing's syndrome in pregnant women. Furthermore, biochemically, a normal pregnancy is associated with a several fold increase in plasma cortisol, as the increased cortisol production rate also increases the cortisol binding protein. Untreated, the condition results in high maternal and fetal morbidity and mortality. An adrenal or pituitary adenoma should be excised, but a metyrapone, which is not teratogenic, has been effective in controlling many cases of excess cortisol. Here, a case of Cushing's syndrome, complicating a pregnancy due to an adrenal cortical adenoma, with thorough obstetric and medical management, including a metyrapone, which was adrenalectomized after delivery, is reported.
- Clinical Characteristics of 10 Cases of Korean Osteogenesis Imperfecta.
- Hyoung Suk Lee, Hyon J Kim, Jae Hyun Cho, Seong Won Lee, Hyun A Kim, Joon Hyuck Choi, Young Jun Song, Dae Jung Kim, Kwan Woo Lee, Yoon Sok Chung
- J Korean Endocr Soc. 2003;18(5):496-503. Published online October 1, 2003
- 1,355 View
- 20 Download
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Abstract
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- Osteogenesis Imperfecta (OI) is a relatively rare hereditary disease, which is characterized by multiple bone fractures and spine scoliosis, due to the fragility of bone, and is often associated with blue sclerae, deafness and dentinogenesis imperfecta. Four types of OI can be distinguished, according to the clinical findings. Although mutations affecting type I collagen are responsible for the disease in most patients, the mechanism by which the genetic defects cause abnormal bone development remains to be fully understood. Here, the clinical characteristics of 10 OI patient cases are reported, with a review of the literature. All the cases, including 4 type I, 4 type III and 2 type IV, inherited OI as an autosomal dominant trait. All the subjects had multiple old fractures and decreased bone densities. In this study, the biochemical marker of bone formation, serum alkaline phosphatase, was found to be increased only in the pediatric OI patients, while the biochemical marker of bone resorption, urinary deoxypyridinoline, was increased in all cases. The mobility score was found to correlate with the severity of the type on diagnosis.
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