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Efficacy of pamidronate in pediatric osteosarcoma patients with low bone mineral density

Lim SW, Ahn JH, Choi A, Cho WH, Lee JA, Kim DH, Seo JH, Lim JS

PURPOSE: Most surviving pediatric osteosarcoma patients experience osteoporosis, bone pain, and pathologic fracture during and after therapy. The aim of this study was to evaluate the efficacy and side effects...
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Early onset of puberty in an obese boy with Klinefelter syndrome

Cho BW, Kwon SE, Kim SK, Lee T, Han JY, Lee JE

Klinefelter syndrome (KS) is one of the most common disease entities characterized by X-chromosomal aberration causing the primary hypogonadism in adult men. Patients with KS seem to be typically characterized...
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A patient with Cushing disease lateralizing a pituitary adenoma by inferior petrosal sinus sampling using desmopressin: a case report

Lim JH, Kim SJ, Jung MK, Kim KE, Kwon AR, Chae HW, Kim DH, Kim HS

A 14-year-old girl was referred for evaluation of the etiology of Cushing syndrome. During the previous 2 years, she had experienced weight gain, secondary amenorrhea, growth retardation, and back pain....
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Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

Yu HW, Cho WI, Chung HR, Choi KH, Yun S, Cho HS, Shin CH, Yang SW

Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a...
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Sitosterolemia: a review and update of pathophysiology, clinical spectrum, diagnosis, and management

Yoo EG

Sitosterolemia is an autosomal recessive disorder characterized by increased plant sterol levels, xanthomas, and accelerated atherosclerosis. Although it was originally reported in patients with normolipemic xanthomas, severe hypercholesterolemia have been...
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Recent advances in biochemical and molecular analysis of congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Choi JH, Kim GH, Yoo HW

The term congenital adrenal hyperplasia (CAH) covers a group of autosomal recessive disorders caused by defects in one of the steroidogenic enzymes involved in the synthesis of cortisol or aldosterone...
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Male patients presenting with rapidly progressive puberty associated with malignant tumors

Kim SJ, Ko AR, Jung MK, Kim KE, Chae HW, Kim DH, Kim HS, Kwon AR

In males, precocious puberty (PP) is defined as the development of secondary sexual characteristics before age 9 years. PP is usually idiopathic; though, organic abnormalities including tumors are more frequently...
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Endocrine dysfunctions in children with Williams-Beuren syndrome

Kim YM, Cho JH, Kang E, Kim GH, Seo EJ, Lee BH, Choi JH, Yoo HW

PURPOSE: Williams-Beuren syndrome (WBS) is caused by a hemizygous microdeletion of chromosome 7q11.23 and is characterized by global cognitive impairment, dysmorphic facial features, and supravalvular aortic stenosis. Endocrine dysfunctions have...
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Long-term efficacy of recombinant human growth hormone therapy in short-statured patients with Noonan syndrome

Jeong I, Kang E, Cho JH, Kim GH, Lee BH, Choi JH, Yoo HW

PURPOSE: Noonan syndrome (NS) is characterized by short stature, heart anomalies, developmental delays, dysmorphic features, cryptorchidism, and coagulation defects. Several studies reported the short-term effects of recombinant human growth hormone...
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Relationships of physical fitness and obesity with metabolic risk factors in children and adolescents: Chungju city cohort study

Kim HJ, Lee KJ, Jeon YJ, Ahn MB, Jung IA, Kim SH, Cho WK, Cho KS, Park SH, Jung MH, Lee JH, Suh BK

PURPOSE: The purpose of this study was to investigate the relationships of physical fitness and obesity with metabolic risk factors in children and adolescents. METHODS: This cohort study was conducted in...
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