Endocrinology and Metabolism

Original Article

Hypothalamus and Pituitary Gland
Metabolic Impacts of Discontinuation and Resumption of Recombinant Human Growth Hormone Treatment during the Transition Period in Patients with Childhood-Onset Growth Hormone Deficiency: Yun Jeong Lee, Yunha Choi, Han-Wook Yoo, Young Ah Lee, Choong Ho Shin, Han Saem Choi, Ho-Seong Kim, Jae Hyun Kim, Jung Eun Moon, Cheol Woo Ko, Moon Bae Ahn, Byung-Kyu Suh, Jin-Ho Choi; Endocrinol Metab. 2022;37(2):359-368. Published online April 25, 2022; DOI: https://doi.org/10.3803/EnM.2021.1384

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Background
Discontinuing growth hormone (GH) treatment during the transition to adulthood has been associated with adverse health outcomes in patients with childhood-onset growth hormone deficiency (CO-GHD). This study investigated the metabolic changes associated with interrupting GH treatment in adolescents with CO-GHD during the transition period.
Methods
This study included 187 patients with CO-GHD who were confirmed to have adult GHD and were treated at six academic centers in Korea. Data on clinical parameters, including anthropometric measurements, metabolic profiles, and bone mineral density (BMD) at the end of childhood GH treatment, were collected at the time of re-evaluation for GHD and 1 year after treatment resumption.
Results
Most patients (n=182, 97.3%) had organic GHD. The median age at treatment discontinuation and re-evaluation was 15.6 and 18.7 years, respectively. The median duration of treatment interruption was 2.8 years. During treatment discontinuation, body mass index Z-scores and total cholesterol, low-density lipoprotein, and non-high-density lipoprotein (HDL) cholesterol levels increased, whereas fasting glucose levels decreased. One year after GH treatment resumption, fasting glucose levels, HDL cholesterol levels, and femoral neck BMD increased significantly. Longer GH interruption (>2 years, 60.4%) resulted in worse lipid profiles at re-evaluation. The duration of interruption was positively correlated with fasting glucose and non-HDL cholesterol levels after adjusting for covariates.
Conclusion
GH treatment interruption during the transition period resulted in worse metabolic parameters, and a longer interruption period was correlated with poorer outcomes. GH treatment should be resumed early in patients with CO-GHD during the transition period.

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Case Reports

A Case of Pheochromocytoma Crisis with Acute Myocardial Infarction Induced by Glucocorticoids Administration.: Woo Sun Rou, Sang Kyung Jung, Sung Yun Lee, Yun Jeong Lee, Dong Jun Kim, Young Doo Kim, Hyung Yoon Kim, Sunhee Chang, Jung Hyun Noh; Endocrinol Metab. 2010;25(3):240-244. Published online September 1, 2010; DOI: https://doi.org/10.3803/EnM.2010.25.3.240

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Abstract PDF: The most common symptoms of pheochromocytoma are paroxysmal or sustained hypertension, or symptoms of paroxysmal adrenergic stimulation such as palpitation, headache, and diaphoresis. These patients can on rare occasion reveal or be complicated with cardiovascular symptoms such as arrhythmia, cardiomyopathy, acute coronary syndrome and cardiogenic shock. These cardiac manifestations of pheochromocytoma may delay the diagnosis, which can cause a catastrophic outcome. A pheochromocytoma crisis is provoked by surgery, anesthesia, exercise and, several drugs and it is known to be an endocrine emergency with mortality as high as 85%. Many classes of drugs are well known to precipitate adverse reactions, but the presentation of pheochromocytoma after the administration of steroid has rarely been reported. We report here on a case of pheochromocytoma crisis with acute myocardial infarction after the patient took prednisolone. Furthermore, we discuss the mechanism of glucocorticoid induced crisis and myocardial infarction in pheochromocytoma patients.

Ectopic ACTH Syndrome with Bilateral Pheochromocytoma in Multiple Endocrine Neoplasia Type 2A.: Ji Mi Moon, Yoon Jung Kim, Young Jin Seo, Hye Yoon Choi, Joo Hyong Kim, Ju Ri Park, Yun Jeong Lee, Hee Young Kim, Sin Gon Kim, Dong Seop Choi; J Korean Endocr Soc. 2009;24(4):265-271. Published online December 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.4.265

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4 Crossref

Abstract PDF

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome characterized by the presence of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN 2A arises due to a germline missense mutation of the RET proto-oncogene. Specific RET mutation analysis has revolutionized the diagnosis and therapy of this disorder, and early thyroidectomy may have lowered the morbidity and mortality associated with these diseases. Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of ACTH outside of the pituitary gland; the most common causes are malignancies, but rarely adrenal pheochromocytoma may be the cause.

Citations

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Severe Cushing Syndrome Due to an ACTH-Producing Pheochromocytoma: A Case Presentation and Review of the Literature
Jenan N Gabi, Maali M Milhem, Yara E Tovar, Emhemmid S Karem, Alaa Y Gabi, Rodhan A Khthir
Journal of the Endocrine Society.2018; 2(7): 621. CrossRef
Adrenocorticotropic hormone (ACTH)-producing pheochromocytoma presented as Cushing syndrome and complicated by invasive aspergillosis
Jae Ho Cho, Da Eun Jeong, Jae Young Lee, Jong Geol Jang, Jun Sung Moon, Mi Jin Kim, Ji Sung Yoon, Kyu Chang Won, Hyoung Woo Lee
Yeungnam University Journal of Medicine.2015; 32(2): 132. CrossRef

A Case of Fulminant Type 1 Diabetes Mellitus Complicated with Ischemic Ileitis.: Se Won Oh, Ju Ri Park, Yun Jeong Lee, Hee Yeong Kim, Ji A Seo, Nan Hee Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi, Sin Gon Kim; J Korean Endocr Soc. 2009;24(2):116-120. Published online June 1, 2009; DOI: https://doi.org/10.3803/jkes.2009.24.2.116

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Abstract PDF: Fulminant type 1 diabetes is characterized by diabetes with an abrupt onset, severe metabolic acidosis at diagnosis, a low HbA1c level and negativity for islet cell-related autoantibodies, and this illness has been classified as type 1B diabetes by the WHO. The prevalence of this disease is higher in Japan than any other country and recently, there have been an increasing number of such case reports in Korea. Genetic factors and environmental factors such as virus infection and an immune mechanism have been suggested as the mechanism of the pathophysiology, but this remains to be clarified.

A Case of Graves' Disease with Pheochromocytoma.: Hye Sook Kim, Hyung Joon Joo, Yoon Seok Choi, Eun Sun Kim, Soo Yeon Park, Yun Jeong Lee, Hee Young Kim, Ji A Seo, Sin Gon Kim, Dong Seop Choi; J Korean Endocr Soc. 2007;22(6):465-469. Published online December 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.6.465

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Abstract PDF

Graves' disease is an autoimmune disorder in which thyrotropin-stimulating hormone receptor antibodies stimulate the thyroid gland. Stress hormones such as catecholamine are known to play important roles in the pathogenesis of Graves' disease. Pheochromocytoma with Graves' disease is extremely rare, and no case has been reported within the Republic of Korea. However, according to previous studies conducted abroad, pheochromocytoma influences the pathogenesis of Graves' disease by producing excessive cathecholamine. In the present report, we describe a 65-year-old female patient with paroxysmal hypertension and rapidly progressive body weight loss who was diagnosed as having Graves'disease with pheochromocytoma.

Citations

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Graves' Disease Accompanied by Pheochromocytoma: Report of a Case
Jin-Hwa Kim, Sang-Jun Lee, Ji-Hye Shin, Mi-Ra You, Jae-Sik Jung, Sang-Yong Kim, Hak-Yeon Bae
Journal of Korean Endocrine Society.2009; 24(2): 126. CrossRef

Two Cases of Acromegaly with Empty Sella Syndrome Treated by Long-Acting Release Octreotide.: Dong Jin Kim, Young Jin Seo, Nam Hoon Kim, Hye Soo Chung, Chai Ryoung Eun, Hye Jung Choi, Hye Sook Kim, Sae Jeong Yang, Juri Park, Hye Jin Yoo, Soo Yeon Park, Yun Jeong Lee, Ohk Hyun Ryu, Kye Won Lee, Hee Young Kim, Ji A Seo, Sin Gon Kim, Nan Hee Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi; J Korean Endocr Soc. 2007;22(2):135-141. Published online April 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.2.135

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Abstract PDF: Two cases of typical acromegaly with empty sella syndrome presented to our institution. In the natural course of untreated pituitary adenoma, empty sella syndrome may result from necrosis by infarction or from hemorrhage of the pituitary gland. In our patients, the secretion of growth hormone continued in spite of the existence of empty sella syndrome. In one case, we confirmed the hypersecretion of growth hormone from sella by jugular vein sampling. Medical therapy with somatostatin analogue was attempted because there was no obvious mass in the sella. After 6~12 months of treatment with long-acting release octreotide, clinical features in our patients were improved, and the level of growth hormone and IGF-1 were also normalized.

Original Articles

Comparison of Target Organ Damages between Primary Aldosteronism and Essential Hypertension.: Juri Park, Dong Jin Kim, Sae Jeong Yang, Sook Hae Kim, Soo Yeon Park, Hye Jin Yoo, Yun Jeong Lee, Hee Young Kim, Ohk Hyun Ryu, Kye Won Lee, Ji A Seo, Sin Gon Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi; J Korean Endocr Soc. 2007;22(1):11-18. Published online February 1, 2007; DOI: https://doi.org/10.3803/jkes.2007.22.1.11

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Abstract PDF

BACKGROUND
A number of recent clinical studies have reported marked target organ damages in patients with primary aldosteronism. The aim of this study was to compare the incidence of target organ damages in patients with primary aldosteronism (PA) and essential hypertension (EHT). METHODS: The clinical records of 41 PA patients, over a 20-year period, were retrospectively analyzed. The clinical characteristics and incidence of target organ damages of 33 of the patients in this group were compared with those of 66 patients with essential hypertension, directly matched for age, gender and mean blood pressure. 8 of the PA patients could not be matched with EHT patients for age, gender and mean blood pressure, so were excluded from the comparison. The patients with essential hypertension were sampled from patients who visited for the evaluation of hypertension. RESULTS: Ischemic heart diseases were found in 18.2 and 10.6% of patients with PA and EHT, respectively (P = 0.22). From echocardiograms, left ventricular hypertrophy was found in 93.3% and 61.4% of patients with PA and EHT, respectively (P = 0.017). The degrees of left ventricular hypertrophy were correlated with the levels of serum aldosterone, with an r value of 0.490 (P < 0.005). Cerebrovascular attack was found in 18.2% and 1.5% of patients with PA and EHT, respectively (P = 0.005). Hypertensive retinopathy was found in 50% and 33.3% of patients with PA and EHT (P = 0.255), and nephropathy was found in 42.4% and 25.8% of patients with PA and EHT, respectively (P = 0.074). CONCLUSION: Patients with primary aldosteronism had target organ damages more frequently than with those with essential hypertension, which was independent of blood pressure.

Citations

Citations to this article as recorded by

Changes in the clinical manifestations of primary aldosteronism
Sun Hwa Kim, Jae Hee Ahn, Ho Cheol Hong, Hae Yoon Choi, Yoon Jung Kim, Nam Hoon Kim, Hye Jin Yoo, Hee Young Kim, Ji A Seo, Nan Hee Kim, Sin Gon Kim, Kyung Mook Choi, Sei Hyun Baik, Dong Seop Choi
The Korean Journal of Internal Medicine.2014; 29(2): 217. CrossRef
Comparing the Prevalence of Primary Aldosteronism in Hypertensive Diabetic and Non-diabetic Patients
Yi Sun Jang, Koon Soon Kim, Hye Soo Kim
Journal of Korean Endocrine Society.2009; 24(4): 254. CrossRef
Aldosterone as a Cardiovascular Risk Factor
Soon Jib Yoo
Journal of Korean Endocrine Society.2007; 22(1): 8. CrossRef

Hepatic Injury during Treatment with Antithyroid Drugs in Patients with Hyperthyroidism.: Ki Young Lee, Yun Jeong Lee, Soon Hong Hong, Sung Kwoen Jung, Hwa Eun Lee, Chan Jong Seo, Yon Sil Jung, Sung Kwang Lee, Hong Kyu Kim, Hye Young Park, Moon Ho Kang; J Korean Endocr Soc. 2000;15(4-5):554-560. Published online January 1, 2001

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Abstract PDF: BACKGROUND
Propylthiouracil (PIV) and methimazole (MMI) were widely used for the treatment of hyperthyroidism. Hepatic injury caused by these agents is a rare but serious complication. This study is to investigate the clinical features of hepatotoxicity from antithyroid drugs. METHODS: We reviewed 17 cases of hepatic injury during treatment with antithyroid drugs in patients with hyperthyroidism. Included were 6 cases we experienced and 11 cases reported in Korean literature from 1986 to 1999. We analyzed the clinical features of hepatic injury. RESULTS: Of 17 cases of hepatic injury, 12 were PTU cases and 5 MMI cases. The mean age of PTU cases was 40 years with 6/12 patients over 40 years old and 2/5 MMI cases were over 40 years old. The dose of PTU was 300 mg/d or more in 10/12 cases (83%) and the dose of MMI was 30 mg/d in 3/5 cases (60%). The hepatic injury occurred within 3 months in 8/12 PTU cases (67%) and within 2 months in 4/5 MMI cases (80%). The duration of hepatic injury tended to be longer in MMI cases than in PTV cases (median; 80 vs 41 days, p=0.102). In PTU cases, the duration of hepatic injury was correlated with the duration of drug use before hepatic injury (p<0.05). All of 8 biopsied cases who took PTU had predominantly hepatocellular necrosis. Two biopsied cases who took MMI had cholestatic jaundice and nonspecific abnormality, respectively. Biochemical findings of all MMI cases were compatible with cholestatic jaundice. As to the treatment of hyperthyroidism after hepatic injury, 4/12 PTU cases were treated with RAI therapy, 5 with MMI and one with surgery, and treatment was unknown in two. On the other hand 3/5 MMI cases interestingly entered into spontaneous remission after hepatic injury and 2/5 had RAI therapy. Hepatic dysfunction recurred in each one whom treatment by changing to MMI or PTU was tried on. CONCLUSION: Most of hepatic injury during treatment with antithyroid drugs developed within two to three months of drug use. The hepatic injury related to PTU was mainly cytotoxic whereas that related to MMI was cholestatic. Since there is a cross-reaction between PTU and MMI in hepatotoxicity, RAI therapy or operation shoud be considered as an alternative treatment of hyperthyroidism after hepatic injury.

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