Journal Detail Information

1

Cite

Share

A Case of Klippel-Feil Syndrome with Recurrent Hypoglycemia.

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):110-115.

Klippel-Feil syndrome is characterized by congenital fusion of cervical vertebra which shows classic triad of short neck, low posterior hairline and limited range of motion of the neck. We report a case of Klippel-Feil syndrome not only associated with renal hypoplasia and sensorineural hearing defect but also with repetitive severe hypoglycemia with lactic acidosis.
Acidosis, Lactic ; Hearing ; Hypoglycemia* ; Klippel-Feil Syndrome* ; Neck ; Range of Motion, Articular ; Spine

2

Cite

Share

A Case of Multiple Endocrine Neoplasia Type 2B early Diagnosis by RET Proto-oncogene Analysis and Prophylactic Total Thyroidectomy.

Bum Sik KIM ; Young Jun RHIE ; Hong KOH ; Duk Hee KIM ; Seung Hoon CHOI

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):104-109.

Multiple endocrine neoplasia type 2B (MEN 2B) is a rare autosomal-dominant hereditary syndrome which includes medullary thyroid carcinoma (MTC), pheochoromocytoma, multiple ganglioneuromas, gastrointestinal disorders and marfanoid face. MTC is the main cause of death in patients that have not received early and prophylactic treatment. Clinically useful direct DNA tests have recently been available to identify gene carriers before any clinical or biochemical abnormalities are present. Prophylactic total thyroidectomy is recommended to affected children at an early stage of the disease to prevent the development of metastases of MTC. We report a case of MEN 2B patient who was diagnosed by RET proto-oncogene analysis and performed prophylactic total thyroidectomy.
Cause of Death ; Child ; DNA ; Ganglioneuroma ; Humans ; Multiple Endocrine Neoplasia Type 2b* ; Multiple Endocrine Neoplasia* ; Neoplasm Metastasis ; Proto-Oncogenes* ; Thyroid Neoplasms ; Thyroidectomy*

3

Cite

Share

Analysis of the Inquiry Types of the Pediatric Endocrinologic Diseases Counseled on the Internet.

Moon Kyung KWON ; So Yeon YI ; Young Ho KIM ; Seung YANG ; Kwang Nam KIM ; Jeh Hoon SHIN ; Phil Soo OH

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):98-103.

PURPOSE: On account of the civilization progress and popularization of the internet, we have researched the current public understandings of the pediatric endocrinologic diseases. METHODS: Analyzable 582 cases of 1,160 cases inquired by the online counsel board for the pediatric endocrinologic diseases from March of 2001 to January of 2004 were classified into four groups. RESULTS: There were 187 inquiries related to short stature. Most counsels were taken by their mothers. In the height distribution of them, under 3 percentile were 27 (21.4%). About precocious puberty, there were 82 for girls and 8 for boys of the total 110 inquiries. The number of children among 6-10 year old were 62. The main subject matter of inquiry accounted for the great majority as 67 cases was about breast budding. And there were 117 inquiries related to childhood diabetes. The number of children among 1-5 year old were 43 (49.4%). Lastly, there were 149 inquiries related to the thyroid diseases. The number of children under 1 year old were 55 (45.8%). CONCLUSION: Considering the most cases of the stature under 50 percentile on the current Korean growth curve begun since 1998, a present phase of quite competition of the stature was recognized. And it could be possible to say that the maternal factor for short stature seems more important than the paternal factor, because our data show that the short stature mothers of the short stature children is two folds more than the short stature fathers of them.
Breast ; Child ; Civilization ; Fathers ; Female ; Humans ; Internet* ; Mothers ; Puberty, Precocious ; Thyroid Diseases

4

Cite

Share

Estimation of Bone Age using Ultrasonic Method.

Hyun Jung SHIN ; Byung Kyu CHOE ; Heung Sik KIM ; Hee Jung LEE

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):93-97.

PURPOSE: In evaluating the children with short stature, estimation of bone age is the primary step. Conventionally, radiologist estimates the bone age by comparing the radiographic image (left hand) of the child with the age-standardized atlas or by summing up the points given to individual bones of the hand and wrist. This method needs a skilled radiologist, causes radiation exposure, and it may take time. Recently, commercial ultrasonic method to estimate the bone age (BonAge(R), Sunlight Medical Ltd. Israel) was developed. We intended to compare the estimated bone ages using different methods-by radiographic and ultrasonographic methods. METHODS: One hundred and one boys and 108 girls over 5 years (all Koreans) who visited pediatric department from December 2004 to March 2005 for evaluation of short stature were evaluated. The radiologist estimated the bone age with Tanner-Whitehouse 2 method (Korean version). The ultrasonography was done in outpatient base by residents in pediatric department. We calculated the Pearson's correlation coefficients between each age and sex groups and compared the mean of each group by paired t-test. RESULTS: Ultrasonic bone age estimation was well correlated with radiologic method and was concordant with radiologic estimates in boys under age 12 and premenarchal girls of all ages and in postmenarchal girls under age 14. CONCLUSION: BonAge system could be easily performed without radiation. Further observation to more patients will be needed to see the accuracy of bone age estimation between ultrasonography and radiologic methods in older age group of Korean children.
Child ; Female ; Hand ; Humans ; Outpatients ; Sunlight ; Ultrasonics* ; Ultrasonography ; Wrist

5

Cite

Share

Outcomes of an Community-based Intervention Program for Obese Children.

Jung Yeon SHIN ; Kee Soo HA ; Soo Young KIM ; Hyo Young LEE ; Byung Min CHOI ; Kee Hyoung LEE

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):85-92.

PURPOSE: Childhood obesity is global public health issue and early intervention is essential because the long-term consequences of childhood obesity are well documented. We studied the effects of community-based intervention program for obese children in regard to regulating excess body weight and improving their lifestyle. METHODS: The study population were 101 obese children (age 6-12) who lived in Ansan during year 2003. The Program consists of exercise and nutritional education, and was conducted for 20 weeks (during the first 8 weeks program was performed once every week and once every month after that). We evaluated the changes of weight, body mass index, obesity index, lipid profiles and their lifestyle during the program. RESULTS: After the intensive course during 8 weeks, body mass index, obesity index, LDL-cholesterol decreased significantly. However, those results increased again after 20 weeks program comparable to initial levels. There are no significant differences in total cholesterol, triglyceride and HDL-cholesterol during program. The lifestyle for exercise and eating improved after initial 8 weeks intensive course, but returned to previous state after 20 weeks program finished. CONCLUSION: Our 20 weeks obesity program did not influence body mass index significantly although it showed transient improvement during early intensive course. These results suggest that the importance of long-term intensive programs to maintain educated healthy lifestyle. Hereafter, well-controlled and long term followed clinical researches in larger group will be necessary.
Body Mass Index ; Body Weight ; Child* ; Cholesterol ; Early Intervention (Education) ; Eating ; Education ; Gyeonggi-do ; Humans ; Life Style ; Obesity ; Pediatric Obesity ; Public Health ; Triglycerides

6

Cite

Share

Jong Gyun AHN ; Jeong Mee KIM ; Jeh Hoon SHIN

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):76-84.

PURPOSE: Childhood obesity is associated with complications such as hyperinsulinemia/insulin resistance, dyslipidemia and fatty liver. The aims of this study were to evaluate the difference of measurements between obese and normal weight children and to investigate the factors related to complications of obesity. METHODS: The body mass index (BMI), trunk and total fat percent, lipid profiles, ALT, AST, leptin, LH, FSH, fasting insulin, glucose, IGF-1, IGFBP-3 were measured in 97 children (49 obese children and 48 non-obese controls). We calculated G/I ratio, loginsuin, HOMA-IR, logHOMA-IR, and QUICKI and evaluated the relationship between each anthropometric data and metabolic data. RESULTS: Hyperinsulinemia, dyslipidemia and abnormal ALT showed high prevalence in obese children. In obese, BMI was correlated with G/I ratio (r=-0.309, P<0.05), loginsuin (r=0.381, P<0.05), logHOMA-IR (r= 0.342, P<0.05), QUICKI (r=-0.343, P<0.05), ALT (r=0.451, P<0.01)) and AST (r=0.289, P<0.05). Serum insulin level had positive correlation with BMI (r=0.345, P<0.01), serum triglyceride (r=0.332, P<0.05) and all insulin resistance indices (P<0.01). Serum leptin levels were significantly correlated with BMI (r=0.555, P<0.01), trunk (r=0.463, P<0.01)) and total (r=0.506, P<0.01) fat percent, LDL (r=0.350, P<0.05), total cholesterol (r=0.297, P<0.05). CONCLUSION: BMI is valuable for predicting insulin resistance and prevalence of abnormal ALT or AST. Monitoring of insulin level is helpful to assess the morbidities of obesity. Also, serum leptin level might be an important predictor of obesity.
Body Mass Index ; Child ; Cholesterol ; Dyslipidemias ; Fasting ; Fatty Liver ; Glucose ; Humans ; Hyperinsulinism ; Insulin ; Insulin Resistance ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Leptin ; Obesity ; Pediatric Obesity* ; Prevalence ; Triglycerides

7

Cite

Share

Incidence and Clinical Characteristics of Diabetes Mellitus in Aplastic Anemia Patients Treated with Multiple Transfusions.

So Young PARK ; Eun Young CHA ; Min Ho JUNG ; Nak Gyun CHUNG ; Bin CHO ; Hak Ki KIM ; Byung Churl LEE

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):70-75.

PURPOSE: We investigated the epidemiological and clinical characteristics of diabetes mellitus developed in aplastic anemia patients who have had many blood transfusions. METHODS: We retrospectively reviewed medical records of 170 patients with aplastic anemia who were diagnosed before 15 years of age in Department of Pediatrics, The Catholic University of Korea from 1987 to 2001. We obtained their medical history, family history of diabetes mellitus, clinical onset of diabetes mellitus and the successive history, and coexistence of other disorders. RESULTS: Diabetes mellitus was diagnosed in 8 of 107 patients (7.5%) with severe aplastic anemia in childhood. The mean age of diagnosis of diabetes mellitus was 18.5+/-5.2 years, and the mean duration from the start of blood transfusion to the diagnosis of diabetes mellitus was 7.7+/-2.9 years. Duration of multiple blood transfusions was a major risk factor for the development of diabetes mellitus in severe aplastic anemia patients. There was a wide range of symptoms at clinical onset of diabetes mellitus from asymptomatic hyperglycemia to diabetic ketoacidosis. Incidence of other complications, such as hepatic impairment (88%) and cardiac dysfunction (75%), was high in patients who developed diabetes mellitus. CONCLUSION: Severe aplastic anemia patients treated with prolonged, multiple transfusions have a significant risk of developing insulin-deficient diabetes mellitus. These patients are at risk for other complications, such as hepatic, cardiac, or thyroid disorders. Early prevention of iron overload and screening for transfusion-related complications are very important in these patients.
Anemia, Aplastic* ; Blood Transfusion ; Diabetes Mellitus* ; Diabetic Ketoacidosis ; Diagnosis ; Humans ; Hyperglycemia ; Incidence* ; Iron Overload ; Korea ; Mass Screening ; Medical Records ; Pediatrics ; Retrospective Studies ; Risk Factors ; Thyroid Gland

8

Cite

Share

Clinical Characteristics of Type 2 Diabetes in Children and Adolescents.

Jungi CHOI ; Jung Min KO ; Jung Hyun LEE ; Jin Ho CHOI ; Han Wook YOO

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):64-69.

PURPOSE: The number of children and adolescents with type 2 diabetes is increasing worldwide. This study was undertaken to characterize clinical features of type 2 diabetes mellitus and evaluate the risk factors. METHODS: This study included 27 type 2 diabetes patients out of 204 patients diagnosed as diabetes mellitus from 1994 though 2005 at Asan Medical Center. Clinical characteristics including acanthosis nigricans, obesity index, body mass index (BMI), laboratory data, and treatment methods of patients with type 2 diabetes were reviewed retrospectively. RESULTS: The mean age at diagnosis was 12.3+/-2.4 years. Patients were followed up for a duration of 3.1+/-1.9 years. In family history, 16 patients (59%) had one or more family members of type 2 diabetes. Ten children (37%) had acanthosis nigricans. Mean body mass index and obesity index were 28.4+/-4.9 kg/m2 and 41.6+/-27.1%, respectively. Anti-GAD antibody were not detected in any patient. Hypercholesterolemia was found in 9 patients (36%), and hypertriglyceridemia in 19 patients (76%). Homeostatic model assessment-insulin resistance (HOMA-IR), which was calculated in 10 patients, 6.8+/-7.3. The modes of therapy included insulin alone (30%), oral hypoglycemic agents alone (63%), and combined therapy (7%). No one had developed long-term complications of diabetes during follow-up periods. CONCLUSION: Risk factors such as positive family history, obesity, acanthosis nigricans, and insulin resistance might be associated with type 2 diabetes. This study suggests that modification of obesity could prevent the incidence of type 2 diabetes in children and adolescents.
Acanthosis Nigricans ; Adolescent* ; Body Mass Index ; Child* ; Chungcheongnam-do ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Diagnosis ; Follow-Up Studies ; Humans ; Hypercholesterolemia ; Hypertriglyceridemia ; Hypoglycemic Agents ; Incidence ; Insulin ; Insulin Resistance ; Obesity ; Retrospective Studies ; Risk Factors

9

Cite

Share

Clinical Course and Outcome of Thyroid Carcinoma in Childhood.

Chang Hee SON ; Jung Min KO ; Jung Hyun LEE ; Jin Ho CHOI ; Han Wook YOO

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):57-63.

PURPOSE: Thyroid carcinoma is rarely occurred in childhood and has a good prognosis despite high incidence of relapse. This study was undertaken to investigate clinical, histological characteristics and prognosis of thyroid carcinoma in childhood. METHODS: Eleven patients (6 males and 5 females) less than 21 years of age were enrolled in this study. They have been followed up for a mean duration of 6 years, ranging from 0.4 to 11 years. Clinical characteristics including age at diagnosis, sex, histological type, tumor size, metastasis, staging, treatment modality, outcome and prognosis with thyroid carcinoma in childhood were reviewed retrospectively. RESULTS: The mean age at diagnosis was 11.6+/-2.84 years. The predominant type of histology was papillary carcinoma (82%). Serum thyroglobulin levels were elevated up to 203.6+/-151.17 ng/mL in all patients at diagnosis. A thyroid scan using 99mTc-pertechnetate revealed cold (64%), warm (9%) and hot (18%) nodules. Seven patients (64%) had lymph node metastases, and five patients (45%) had pulmonary metastases at diagnosis. Total or near total thyroidectomy was performed in seven (64%) patients with local or distant metastases. Unilateral lobectomy was performed in four (36%) patients without metastases. Seven (64%) patients with local or distant metastatic lesions have been under radioiodine (131I) ablation therapy. Four (57%) out of seven patients improved, and three (43%) patients were persistent. CONCLUSIONS: Prognosis of thyroid carcinoma in childhood was good despite high incidence of local or distant metastasis. Patients with thyroid carcinoma should be diagnosed early because different treatment modalities and prognosis of patients with thyroid carcinoma could be determined by the presence of metastatic lesion and histological type.
Carcinoma, Papillary ; Diagnosis ; Humans ; Incidence ; Lymph Nodes ; Male ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Retrospective Studies ; Thyroglobulin ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy

10

Cite

Share

Predictors of Transient Hypothyroidism in Neonatal Screening Test.

Ho Kyung LIM ; Ki Hwan KIM ; Sun Hui KIM ; Ha Young NO ; Chan Jong KIM ; Young Jong WOO ; Tai Ju HWANG

Journal of Korean Society of Pediatric Endocrinology.2006;11(1):50-56.

PURPOSE: Congenital hypothyroidism is detected through the neonatal screening test and treated early from neonatal period. Among these patients, however, transient hypothyroidism is included. In this study, we aimed to evaluate the predicting factors of transient hypothyroidism in neonatal screening test. METHODS: We retrospectively reviewed medical records of 40 male and 31 female neonates diagnosed as congenital hypothyroidism by newborn screening from 1996 to 2003. The untreated cases that revealed slightly decreased levels of thyroid hormone on repeated examination and the cases who showed normalized hormone after 2 years of treatment were regarded as transient hypothyroidism. The results of neonatal screening tests and other clinical features of children with transient hypothyroidism were compared to those of children with permanent form. The time to normalization of thyroid hormone levels in the transient hypothyroidism were also reviewed. RESULTS: Among 71 cases of congenital hypothyroidism, 39 cases (54.9%) were transient and 32 cases (45.1%) were permanent hypothyroidism. There were no significant differences in clinical features between two groups, except lower birth weight in the transient group (transient 2.80+/-0.75 kg, permanent 3.22+/-0.57 kg, P=0.02). Free T4 levels were significantly lower and TSH levels were significantly higher in the permanent form than the transient ones (P<0.01). The estimated cut-off point for distinguishing transient hypothyroidism from permanent form was 2.05 ng/dL (sensitivity 54%, specificity 93%) for free T4 and 34 microIU/mL (sensitivity 72%, specificity 87%) for TSH. CONLCUSION: In this study, transient hypothyroidism could be differentiated from permanent hypothyroidism by TSH and free T4 levels of neonatal screening test. Repeated thyroid function tests are thought to be essential to make a diagnosis of not only transient but also permanent hypothyroidism.
Birth Weight ; Child ; Congenital Hypothyroidism ; Diagnosis ; Female ; Humans ; Hypothyroidism* ; Infant, Newborn ; Male ; Mass Screening ; Medical Records ; Neonatal Screening* ; Retrospective Studies ; Sensitivity and Specificity ; Thyroid Function Tests ; Thyroid Gland

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share