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Sung Jun KIM ; Pan Ju LIM ; Seong Hwan BAN ; Dong Hwan LEE ; Dong Kyu JIN ; Seung Mi SONG ; Jung Sim KIM

Journal of Korean Society of Pediatric Endocrinology.2000;5(2):215-219.

Pseudohypoaldosteronism is a disorder in which synthesis of aldosterone is normal but unresponsiveness of the target to the aldosterone activates angiotensin-renin system which in turn elevates levels of renin and aldosterone. This salt-losing syndrome causes life-threatening hyponatremia and hyperkalemia. Despite of the normal renal and adrenal function, due to deficiency of aldosterone function, reabsorption of sodium and excretion of potassium in the kidney is impaired. Sodium loss not only from the kidney but also from sweat gland, salivary gland and colon may occur in some cases. We experienced two cases of pseudohypoaldosteronism in a 3-day-old male and 6-month-old female. The hyponatremia, hyperkalemia, elevation of plasma renin activity and aldosterone concentration were observed without renal and adrenal dysfunction. Brief review and related literatures were also presented.
Aldosterone ; Colon ; Female ; Humans ; Hyperkalemia ; Hyponatremia ; Infant ; Kidney ; Male ; Plasma ; Potassium ; Pseudohypoaldosteronism* ; Renin ; Salivary Glands ; Sodium ; Sweat Glands

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A Case of Neonatal Diabetes Mellitus: Transient or Permanent?.

Dae Yeol LEE ; Ho Keun YI ; Pyoung Han HWANG

Journal of Korean Society of Pediatric Endocrinology.2000;5(2):210-214.

A case of neonatal diabetes mellitus is described. The child presented with low birth weight but was normal in appearance. She was acidotic and ketonuria was observed. The HLA typing was DR1 and 3, and insulin autoantibodies were negative. Genetic analysis with polymorphic DNA markers for chromosome 6 indicated biparental inheritance. She required insulin therapy for the control of hyperglycemia, and insulin dependence continues after 8 months of age.
Autoantibodies ; Child ; Chromosomes, Human, Pair 6 ; Diabetes Mellitus* ; Genetic Markers ; Histocompatibility Testing ; Humans ; Hyperglycemia ; Infant, Low Birth Weight ; Infant, Newborn ; Insulin ; Ketosis ; Wills

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A Case of Persistent Hyperinsulinemic Hypoglycemia Treated with Diazoxide.

Min Sun KIM ; Eun Hye PARK ; Sun Young KIM ; Dae Yeol LEE

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):164-167.

Congenital hyperinsulinism (CHI), the most important cause of hyperglycemia in early infancy, is a heterogenous disease characterized by dysregulation of insulin secretion. Mutations in five proteins have been associated with CHI: sulfonyl urea receptor 1; Kir 6.2; glucokinase; glutamate dehydrogenase and mitochondrial enzyme short-chain 3-hydroxyacyl-coenzyme A dehydrogenase. Early recognition of hypoglycemia, diagnosis of CHI and appropriate management of the hypoglycemia are of the utmost importance to prevent neurologic damage. We report a case of persistent hyperinsulinemic hypoglycemia in 8-month-old male infant. This patient has no mutation in previously mentioned genes. Treatment with diazoxide was successful without any severe side effects in this patient.
Congenital Hyperinsulinism* ; Diagnosis ; Diazoxide* ; Glucokinase ; Glutamate Dehydrogenase ; Humans ; Hyperglycemia ; Hyperinsulinism ; Hypoglycemia ; Infant ; Insulin ; Male ; Oxidoreductases ; Urea

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A Case of Erythromelalgia in Adolescent with Diabetes Mellitus.

Sin Young PARK ; Hae Sun SHIM ; Sung Ryul KWON ; Won PARK ; Young Jin HONG ; Byong Kwan SON ; Ji Eun LEE

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):159-163.

Erythromelalgia is a rare clinical condition of unknown etiology characterized by severe burning pain in the distal limbs. It can be accompanied by pronounced erythema and increased skin temperature precipitated by heat or activity and can be improved by cooling the affected part. It can be divided into two categories, primary, which begins spontaneously at any age, and secondary, which is infrequently associated with diabetes mellitus. However, the significance of this association is little known. We report a rare case of intractable erythromelalgia in an adolescent with diabetes mellitus.
Adolescent* ; Burns ; Diabetes Mellitus* ; Erythema ; Erythromelalgia* ; Extremities ; Hot Temperature ; Humans ; Skin Temperature

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A Case of the Hypercalcemia Induced by the Coenzyme Q10 and Alphacalcidol Treatment in a Patient with Kearns-Sayre Syndrome and Hypoparathyroidism.

Hyun Hee JUNG ; Hyun Hee HWANG ; Duk Hee KIM

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):155-158.

Kearns-Sayre Syndrome (KSS) is rare mitochondrial disorder characterized by chronic progressive external ophthalmoplegia, atypical retinal pigmentation and complete heart block. It is occasionally combined endocrinologic symptoms such as hypoparathyroidism, short stature, diabetes mellitus and hypothyroidism. We reported the effect of Coenzyme Q10 on total serum calcium concentration in 17 years old girl with KSS and hypoparathyroidism. The patients was treated with alfacalcidol (1alpha-OHD3), Coenzyme Q10 and oral calcium agent. Total serum calcium concentration had even remained within normal range and hypercalcemia was developed suddenly after treatment of combination of Coenzyme Q10 and alfacalcidol (1alpha-OHD3). After stop of all medication, her total calcium concentration was decreased to 7.6 mg/dL and remained in normal range with oral calcium (2 g/day) and Coenzyme Q10 (150 mcg/day) daily. The action of Coenzyme Q10 is not clearly defined but, we could explain Coenzyme Q10 activates the capacity of the patient to produce the active form of Vitamin D, 1alpha-OHD3.
Adolescent ; Calcium ; Diabetes Mellitus ; Female ; Heart Block ; Humans ; Hypercalcemia* ; Hypoparathyroidism* ; Hypothyroidism ; Kearns-Sayre Syndrome* ; Mitochondrial Diseases ; Ophthalmoplegia, Chronic Progressive External ; Pigmentation ; Reference Values ; Retinaldehyde ; Vitamin D

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A Case of Sjogren's Syndrome with Hyperthyroidism.

Yun Hye JUNG ; Im Jeong CHOI ; Jin Wha JUNG

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):150-154.

Sjogren syndrome is a chronic, slowly progressive, autoimmune disease in which the exocrine glands are damaged by lymphocytic infiltration, resulting in xerostomia and xerophthalmia. Sjogren syndrome may occur in 2 forms: primary Sjogren syndrome, when the clinical manifestations of the syndrome are seen alone, and secondary Sjogren syndrome, when associated with another autoimmune disease, such as rheumatoid arthritis, systemic lupus erythromatosus, or scleroderma. Approximately one third of patients present with extraglandular manifestations: arthritis, Raynaud phenomenon, lymphadenopathy, lung involvement, vasculitis and peripheral nervous system involvement. About 10-50% of patients with Sjogren syndrome had evidence of thyroid disease, mainly hypothyroidism. Several inflammatory thyroid diseases are also considered to be autoimmune in origin. In this respect, the histologic picture of primary Sjogren syndrome exocrine glands and autoimmune thyroid glands show great similarities. Here, we report a new case of Sjogren syndrome accompanying with hyperthyroidism which affected a 10-year-old girl.
Arthritis ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Child ; Exocrine Glands ; Female ; Humans ; Hyperthyroidism* ; Hypothyroidism ; Lung ; Lymphatic Diseases ; Peripheral Nervous System ; Raynaud Disease ; Sjogren's Syndrome* ; Thyroid Diseases ; Thyroid Gland ; Vasculitis ; Xerophthalmia ; Xerostomia

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Time Trend in Height, Weight, BMI and Waist Circumference of Korean Adolescents; From the Korean National Health and Nutrition Examination Survey (KNHNES), 1998, 2001 and 2005.

You Suk KIM ; Mi Jung PARK

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):142-149.

PURPOSE: The mean height and weight had been increasing rapidly in Korean adolescents. The purpose of this study was to analyze body physique (height, weight, BMI and waist circumference) of Korean adolescents, using KNHNES data. METHODS: The data were obtained from the KNHNES from each year of 1998, 2001 and 2005. Total number of 4164 adolescents (2140 boys, 2024 girls) aged 10 to 19 years were included. Height, weight, and waist circumference were measured and the mean value of body physique was analyzed by sex and age. RESULTS: For height of 10 yr-old boys, there was no difference between 1998, 2001 and 2005, but the height of 13 yr-old boys was increased more than 3 cm in 2005, compared to 1998 and 2001. For height of 10 yr-old girls, more than 2 cm was increased, but, there was no significant difference after the age of 14 year in each 1998, 2001 or 2005. Height of the age of 19 was 173.7 cm in boys and 161.3 cm in girls in 2005, showing no distinct change compared to 1998 or 2001. Weight and BMI showed significant increase in both sexes and all-aged groups for older than the age of 11 years in 2005, compared to 1998 or 2001. Weight increase was more distinct in boys compared to girls. For boys, waist circumference of 2005 were higher than 1998, but lower than 2001. For girls, waist circumference of 2005 were higher than 1998 and 2001 especially in the late teens. CONCLUSION: Rapid increase in height was shown in the early teenage whereas final height showed no significant difference during 1998-2005. The mean weight and BMI have been increased dramatically in 2005, compared to 1998 or 2001 in boys and girls. This phenomenon was shown more remarkably in boys, compared to girls. These findings emphasize importance of obesity management, especially on boys.
Adolescent* ; Female ; Humans ; Nutrition Surveys* ; Obesity ; Waist Circumference*

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The Changes of Plasma Ghrelin Levels After Exercise in Obese Women.

Eun Young KANG ; Min Sun KIM ; Eun Hye PARK ; Ki Jin KIM ; Dae Yeol LEE

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):135-141.

PURPOSE: Ghrelin is the known orexigenic hormone as well as an endogenous ligand for the growth hormone secretagogue receptor and has been shown to be related with the regulation of energy homeostasis. To investigate the changes of plasma ghrelin levels in response to weight loss induced by exercise, we measured fasting plasma ghrelin levels in obese and control groups before and after exercise intervention. METHODS: According to body mass index (BMI), total 32 adult women were divided into obese group (n=14, BMI> or =25 kg/m2) and control group (n=18, BMI<25 kg/m2). All subjects underwent weight reduction exercise intervention for 3 months. Before and after exercise, we measured body compositions, serum lipid profiles, serum glucose, plasma ghrelin levels in all subjects. RESULTS: At baseline, the plasma ghrelin levels in obese group were significantly lower than those in the control group. After the exercise intervention, the plasma ghrelin levels increased significantly from 174.8+/-40.8 to 235.9+/-53.1 pg/mL (P<0.05) in obese group and from 244.4+/-42.6 to 276.5+/-45.1 pg/mL (P<0.05) in control group. The changes of plasma ghrelin levels in obese group (38.7+/-30.8 pg/mL) were significantly higher than those of the control group (14.6+/-20.3 pg/mL, P<0.05). However, there was no significant relationship between the changes of plasma ghrelin levels and various parameters in the obese group after exercise. CONCLUSION: In this study, the plasma ghrelin levels in obese adults were significantly increased than those in the control group associated with exercise-induced weight loss. Further studies are needed to establish the precise roles of ghrelin in the regulation of energy homeostasis in obesity.
Adult ; Blood Glucose ; Body Composition ; Body Mass Index ; Fasting ; Female ; Ghrelin* ; Homeostasis ; Humans ; Obesity ; Plasma* ; Receptors, Ghrelin ; Weight Loss

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The Effect of Metformin in Obese Pediatric Patients with Type 2 Diabetes.

Hyun Hee JUNG ; Tae Hyoung KIM ; Duk Hee KIM

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):127-134.

PURPOSE: Metformin is well-established oral hypoglycemic agent for adults and children with type 2 diabetes mellitus. In adults, metformin is effective as initial monotherapy with lack of weight gain. But, its beneficial role on obesity in children is less clearly defined. We assessed the effects of metformin in obese children with type 2 diabetes. METHODS: We evaluated body weight, height, body mass index, fasting blood sugar, HbA1c, lipid profiles in obese 36 children with type 2 diabetes treated with oral hypoglycemic agents at baseline, after 1 year and 2 years of treatment. We divided the subjects into three groups: metformin monotherapy, combination metformin with sulfonylurea, sulfonylurea monotherapy. The changes from baseline in three groups were measured. RESULTS: The decrease in body mass index, blood sugar & HbA1c was observed after treatment with metformin monotherapy group. Metformin monotherapy group after 1 & 2 years of treatment had a greater effect for body mass index (-1.9+/-1.7 kg/m2, -1.5+/-1.6 kg/m2, P<0.05). CONCLUSION: The Metformin monotherapy for obese children with type 2 diabetes improves glycemic control with benefits of significant improvement in body mass index.
Adult ; Blood Glucose ; Body Height ; Body Mass Index ; Body Weight ; Child ; Diabetes Mellitus, Type 2 ; Fasting ; Humans ; Hypoglycemic Agents ; Metformin* ; Obesity ; Weight Gain

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Clinical and Endocrinologic Characteristics of Children Referred for Precocious Puberty.

Tae Hyoung KIM ; Hee Jung COE ; Seung KIM ; Sun Woo LEE ; Hyun Wook CHAE ; You Suk KIM ; Mi Jung PARK ; So Chung CHUNG ; Eun Kyung YU ; Duk Hee KIM ; Ho Seong KIM

Journal of Korean Society of Pediatric Endocrinology.2007;12(2):119-126.

PURPOSE: Precocious puberty is defined as the onset of secondary sexual characteristics before 8 year of age in girls and 9 year in boys. The purpose of this study is to analyze the spectrum of diagnoses made in a consecutive group of children referred for signs of precocious puberty and evaluate the clinical and endocrinologic characteristics of patients with precocious puberty. METHODS: The charts of all 948 children referred for evaluation of signs of precocious puberty between January 2003 and June 2007 in several referral centers were reviewed. Clinical features including age of onset, presenting symptoms, yearly growth rate, bone age advancement, weight, height, and body mass index were analysed and endocrine investigations included basal and gonadotropin releasing hormone (GnRH)-stimulated levels of luteinizing hormone (LH) and follicle stimulating hormone (FSH) as well as sex hormones. RESULTS: Of the 948 children referred for signs of precocious puberty, 915 (96.5%) were female and 33 (3.5%) were male. The final diagnoses made were early puberty (39%), premature thelarche (31%), true precocious puberty (27%) and precocious pseudopuberty (1%). The increases in yearly growth rate and bone age advancement were significant in true precious puberty group (P<0.05). The height and weight standard deviation score were significantly increased in true precious puberty and premature thelarche group compared to the average level according to gender and age (P<0.05). Endocrinologic studies showed that the level of basal LH, basal estradiol and GnRH-stimulated peak LH, peak FSH, peak LH/basal LH, peak FSH/basal FSH, peak LH/peak FSH ratio was all significantly higher in true precicous puberty group and early puberty group when compared to premature thelarche group (P<0.05). Neurogenic true precocious puberty among true precocious puberty was more common in boys (3 out of 7, 42.8%) than in girls (27 out of 253, 10.7%). Endocrinologic studies did not show any difference between idiopathic precocious puberty and neurogenic precocious puberty. CONCLUSION: The result of this study showed the proportion of true precocious puberty among the children referred for early pubertal signs was rather high. Collectively assessing all available data including detailed history, growth records, physical findings, laboratory studies and radiological imaging is important in the evaluation of a child with concerns of early pubertal maturation. Foregoing extensive studies regarding incidence and causes of true precocious puberty should be needed.
Adolescent ; Age of Onset ; Body Mass Index ; Child* ; Diagnosis ; Estradiol ; Female ; Follicle Stimulating Hormone ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone ; Humans ; Incidence ; Luteinizing Hormone ; Male ; Puberty ; Puberty, Precocious* ; Referral and Consultation

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