2017 Clinical practice guidelines for dyslipidemia of Korean children and adolescents

The Committee on Dyslipidemia of Korean Pediatric and Adolescents of the Korean Society of Pediatric Endocrinology has newly developed evidence-based clinical practice guidelines for dyslipidemia in Korean children and adolescents. These guidelines were formulated with the Grading of Recommendations, which include both the strength of recommendations and the quality of evidence. In the absence of sufficient evidence, conclusions were based on expert opinion. These guidelines are based on the 2011 National Heart, Lung, and Blood Institute Guidelines, which focus on the prevention of cardiovascular disease in children and draw from a comprehensive review of evidence. These guidelines contain the definition of and screening process for dyslipidemia and introduce new dietary methods: the Cardiovascular Health Integrated Lifestyle Diet (CHILD)-1, the CHILD-2-low-density lipoprotein cholesterol, and the CHILD-2-triglyceride. Potential drug therapies for dyslipidemia along with their main effects and doses were also included.

2017 Clinical practice guidelines for dyslipidemia of Korean children and adolescents

The Committee on Dyslipidemia of Korean Pediatric and Adolescents of the Korean Society of Pediatric Endocrinology has newly developed evidence-based clinical practice guidelines for dyslipidemia in Korean children and adolescents. These guidelines were formulated with the Grading of Recommendations, which include both the strength of recommendations and the quality of evidence. In the absence of sufficient evidence, conclusions were based on expert opinion. These guidelines are based on the 2011 National Heart, Lung, and Blood Institute Guidelines, which focus on the prevention of cardiovascular disease in children and draw from a comprehensive review of evidence. These guidelines contain the definition of and screening process for dyslipidemia and introduce new dietary methods: the Cardiovascular Health Integrated Lifestyle Diet (CHILD)-1, the CHILD-2-low-density lipoprotein cholesterol, and the CHILD-2-triglyceride. Potential drug therapies for dyslipidemia along with their main effects and doses were also included.

2018 Guidelines for the Management of Dyslipidemia in Korea

No abstract available.

Erratum: 2018 Guidelines for the management of dyslipidemia in Korea

The title and author names are incorrect.

Behavioral and intelligence outcome in 8- to 16-year-old born small for gestational age / 소아과

PURPOSE: We investigated behavioral problems, attention problems, and cognitive function in children and adolescents born small for gestational age (SGA). METHODS: Forty-six SGA children born at term and 46 appropriate for gestational age (AGA) children born at term were compared. Psychiatric symptoms were examined with reference to the Korean-Child Behavior Checklist, Korean-Youth Self Report, and Attention Deficit Hyperactivity Disorder Rating Scale (ADHD-RS). Cognitive function was estimated using the Wechsler Intelligence Scale. Sociodemographic data were recorded from interviews. RESULTS: SGA children had high scores on delinquent behavior, aggressive behavior, and the externalizing scale, and they also showed a propensity for anxiety and depression. The SGA group had a higher mean ADHD-RS score than the AGA group (10.52±8.10 vs.9.93±7.23), but the difference was not significant. The SGA group had a significantly lower verbal intelligence quotient (IQ) than the AGA group, but the mean scores of both groups were within normal limits. CONCLUSION: This study indicates marked behavioral problems, such as delinquency, aggressiveness, and anxiety and depression, as well as low verbal IQ in the SGA group than in the AGA group. Even in cases in which these symptoms are not severe, early detection and proper treatment can help these children adapt to society.

Mixed gonadal dysgenesis in 45,X Turner syndrome with SRY gene

Turner syndrome is the most common chromosomal disorder in girls. Various phenotypic features show depending upon karyotype from normal female through ambiguous genitalia to male. Usually, Turner girls containing 45,X/46,XY mosaicism, or sex-determining region Y (SRY) gene may have mixed gonadal dysgenesis with various external sexual differentiation. We experienced a short statured 45,X Turner girl with normal external genitalia. Because SRY gene was positive, laparoscopic gonadectomy was performed. The dysgenetic gonads revealed bilateral ovotesticular tissues. The authors report a mixed gonadal dysgenesis case found in clinical 45,X Turner patient with positive SRY gene. Screening for SRY gene should be done even the karyotype is 45,X monosomy and external genitalia is normal.

Serum IGF-1 and IGFBP-3 Levels in Central Precocious Puberty Girls Treated with Gonadotropin Releasing Hormone Agonist (GnRHa) / 대한소아내분비학회지

PURPOSE: The purpose of this test was to investigate the changes of serum IGF-1 and IGFBP-3 levels during a one-year gonadotropin releasing hormone agonist (GnRHa) treatment in central precocious puberty (CPP) girls. METHODS: From 2007 to 2009, 26 girls were enrolled in this study. They were diagnosed as having central precocious puberty and were treated with GnRHa (leuprolide actete) for one year. Their height, bone age, and serum IGF-1 and IGFBP-3 levels were evaluated every six months. RESULTS: At the time of diagnosis, their mean serum IGF-1 and IGFBP-3 levels were 302.90+/-102.54 ng/mL and 3,103.58+/-705.08 ng/mL, respectively. At six month after treatment, the serum IGF-1 and IGFBP-3 levels were slightly decreased. One year later, IGF-1 concentrations were higher than before treatment and IGFBP-3 levels were lower (P=NS). This result, however, was not statistically significant. CONCLUSION: Gonadal suppression with gonadotropin releasing hormones inversely influenced circulating IGF-1 and IGFBP-3 levels. However, the serum IGF-1 and IGFBP-3 levels were maintained at relatively steady levels, preserving a normal height velocity.

Prevalence of obesity, hypertension, and hyperlipidemia in Gunpo children of low economic status / 소아과

PURPOSE: This study aimed to assess the prevalence of obesity, hypertension, and hyperlipidemia in children from low-income families in Gunpo and to evaluate whether economic status affects the prevalence of obesity. METHODS: Between October 2007 and March 2008 , 341 children (167 girls and 174 boys; age, 6 to 13 years) were enrolled in this study. All these children came from families who earned minimum wages and who were supported by government. We measured height, weight, and blood pressure and performed laboratory examinations, including total cholesterol, highdensity lipoprotein cholesterol, and triglycerides. Body mass index (BMI) was calculated using weight and height. We compared the prevalence of obesity, hypertension, and hyperlipidemia in children from low-income families with the prevalence of these maladies found in other studies. RESULTS: Prevalence of obesity (BMI> or =95th) was 7.1%. Prevalence of hypertension and hyperlipidemia was 8.2% and 16.7%, respectively. In the obese group, prevalence of hypertension and hyperlipidemia was 25.0% and 45.8%, respectively. Obesity and hyperlipidemia were slightly more frequent in our study than those found in other reports, and the prevalence of hypertension in the obese group was very high compared with statistics from the Korean National Health and Nutrition Examination Survey (25% vs. 19.5%) and those from other reports. CONCLUSION: Low-income status was associated with an increased incidence of obesity, hyperlipidemia, and hypertension. Hypertension showed an especially strong association with economic status, which seemed to correlate with genetic, environmental, and dietary effects.

Clinical and Biochemical Factors Associated with GnRH Stimulation test in the Idiopathic Central Precocious Puberty and Early Puberty Girls / 대한소아내분비학회지

PURPOSE: Gonadotropin releasing hormone (GnRH) stimulation test is the gold standard method for the diagnosis of central precocious puberty. To predict the result of this test, we studied clinical and biochemical factors associated with the test. METHODS: We evaluated 168 girls under 9 years old with the signs of breast budding and more than one year bone age advancement who underwent this test. We defined the result as two criteria. In the first criteria, positive result of the test means peak LH> or =5 IU/L and peak/basal LH> or =2. In the second criteria, positive result means peak LH> or =5 IU/L, peak/basal LH> or =2 and peak LH/FSH> or =1. RESULTS: In the both first and second criteria, 83 and 31 girls had positive results and other 85 and 137 girls had negative results respectively. In both criteria, the patients with positive results turned out to have more advancement in bone age, smaller predicted adult height, smaller paternal height and higher insulin like growth factor-1 (IGF-1) than those of the patients with negative results. Peak LH was positively correlated with bone age advancement (r=0.35, P<0.001) and IGF-1 (r=0.42, P<0.001) and negatively correlated with predicted adult height (r=-0.22, P=0.01). In multiple logistic regression, bone age was significant predictive factor [first criteria, OR 1.43 (95% CI 1.09, 1.87), P=0.01; second criteria, OR 2.46 (95% CI 1.48, 4.08), P=0.01] of the positive results. CONCLUSION: The result of GnRH stimulation test depends on the degree of breast development and advancement in bone age. Also, IGF-1 is the only positively correlated biochemical factor with the test.

The Behavioral and Psychic Masculinizing Scores and Its Related Factors in Female Patients with Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency / 대한소아내분비학회지

OBJECTIVE: We analyzed the degree of behavioral and psychic masculinization as well as factors influencing the masculinization in female patients with CAH caused by 21-hydroxylase deficiency. METHODS: We studied 26 patients with salt-wasting (SW) type, 29 patients with simple virilizing (SV) type and 30 control patients, who were older than 10 years of age. Study participants were given a questionnaire containing 55 items (20 masculine items, 20 feminine items, 15 neutral items) in order to evaluate the degree of feminity and masculinity. RESULTS: SW and SV girls revealed significantly higher masculine scores and lower feminine scores than the control groups (P<0.05). Feminine scores were lower in the SW group compared to those in the SV group (P<0.05). Neutral scores showed no significant difference among three groups. Masculine, feminine, bisexual and undifferentiated personality characteristics exhibit 57.6%, 3.8%, 7.6%, and 30.7% in SW group, respectively; 31%, 17.2%, 20.6%, and 30%, respectively in SV group; 3.3%, 50.0%, 20.0% and 26.6%, respectively in control group. Masculine scores were negatively correlated with age at diagnosis and treatment (r=-0.516, P<0.05), but not with the levels of 17-hydroxyprogesterone at the time of diagnosis and study, advancement of bone age, age at clitoplasty, age at onset of puberty and age at menarche. CONCLUSIONS: Female patients with 21-hydroxylase deficiency, who reveal high masculinizing score, should be closely monitored for the adverse effects of high degree of masculinization, such as polycystic ovarian syndrome. Additionally, it is important to treat adequately after early diagnosis to minimize the degree of masculinization and to develop the prenatal mode of treatment to interrupt the secretion of adrenal androgen.

Insulin Sensitivity in Prepubertal Children born with Low Birth Weight / 대한소아내분비학회지

PURPOSE: We aimed to determine whether prepubertal children born with low birth weight (LBW) have decreased insulin sensitivity, compared with children born with normal birth weight and to know a relationship between insulin sensitivity and adiponectin concentration in those children. METHODS: LBW (n=33) was defined as a birth weight less than 2.5 kg and control (n=20) was defined as a birth weight between 2.5 kg and 4.0 kg. Height and weight were measured for calculation of BMI. Fasting blood samples were taken for the measurements of glucose, insulin, lipid and adiponectin concentration. Body fat mass and abdominal fat ratio were measured. HOMA-IR and QUICKI were calculated, as a mean of insulin sensitivity. RESULTS: Children with LBW showed significantly higher levels of QUICKI and adiponectin than control group (P-1) were more resistant to insulin than shorter children with LBW (height SDS6 yr) children with LBW. In children with LBW, age was negatively correlated with QUICKI adjusted by BMI percentile (r=-0.373, P<0.05). CONCLUSION: These findings suggest that the levels of adiponectin increase to compensate for the diminished insulin sensitivity in younger children with LBW and this phenomenon is faded away with aging. Additionally, the results suggest that LBW children with better postnatal growth have a tendency to have insulin resistance.

Indication of Combined Treatment of GnRH Agonist and GH / 대한소아내분비학회지

No abstract available.

Final Adult Height and Its Influencing Factors in Patients with Congenital Adrenal Hyperplasia Caused by 21-Hydroxylase Deficiency / 대한소아내분비학회지

PURPOSE: We evaluated adult final height and factors influencing adult height outcome in patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency. METHOD: We retrospectively reviewed medical records of 20 male and 22 female patients with 21- hydroxylase deficiency who reached adult final height from 1980 to 2004. We obtained final adult height and analysed possible factors influencing final adult height such as urine 17-ketosteroid (17- KS), plasma 17-OH-progesterone (17-OHP), plasma renin activity, pubertal initiaton age, glucocorticoid dose, age at diagnosis and effect of growth hormone or gonadotropin releasing hormone analogue. RESULT: Mean FH (final height) SDS-MPH (midparental height) SDS were -1.89+/-1.0 in male simple virilization (SV) and -0.83+/-0.8 in female SV patients, -2.27+/-1.3 in male salt wasting (SW) and -1.12+/-1.1 in female SW patients. Mean final adult height and mean FH SDS-MPH SDS were not different between SV and SW patients, but mean FH SDS-MPH SDS in males was significantly lower than that of females (-2.12+/-1.2 vs 0.95+/-0.9, P<0.05). In SW mean FH SDS-MPH SDS were correlated positively with pubertal height gain (r=0.484. P<0.05) and correlated negatively with pubertal initiation age (r=-0.334, P<0.05). but mean FH SDS-MPH SDS were not correlated with age at diagnosis, glucocorticoid dose, urine 17-KS level, plasma 17-OHP level, plasma renin activity, treatment with growth hormone or gonadotropin releasing hormone analogue in both SW and SV. CONCLUSION: Mean final height and mean FH SDS-MPH SDS in 21-hydroxylase deficiency were below the mean for the general population. In SW, adult final height was correlated with pubertal initiation age and pubertal height gain. Therefore it should be important to control early onset of puberty and make improvement pubertal height gain during puberty.

Effect on Final Height of Gonadotropin-Releasing Hormone Agonist (GnRHa) in Children with Congenital Adrenal Hyperplasia / 대한소아내분비학회지

PURPOSE: Object of present study is to identify the effect of gonadotropin-releasing hormone agonis (GnRHa) treatment on final adult height in congenital adrenal hyperplasia (CAH) children with central precocious puberty. METHOD: A retrospective study was conducted of all CAH patients seen between 1977 and 2004, in pediatric endocrinology department, Seoul National University Hospital. All patients treated with glucocorticoid and mineralocorticoid toward to CAH. Treatment group (SW7, SV12) received GnRHa or associated with growth hormone (GH) when they had pubertal changes; breast development before 8 years in girls, testis enlargement (>4 mL) before 9 years in boys and pubertal response in GnRH stimulation test. GnRHa treatment was continued from 1 year to 7.5 years (mean=3.6 yr) while they continued glucocorticoid therapy. We evaluated them every 6 month or yearly until reached final adult height (FH). We compared FH SDS between treatment group and control group. Also final adult height in treatment group compared with pretreatment predicted adult height (By Bayley-Pinneau method). Each group was subdivided into salt wasting group (SW) and simple virilizing group (SV). RESULTS: Treatment group (SW 7, SV 12) was included 19 patients with CAH and control group (SW 6 SV 7) was belonged 13 patients. In treatment group, predicted adult heights are 148.5+/-8.8 cm (-2.8+/-0.8SDS) in SW, 149.8+/-6.7 cm (-2.8+/-1.1SDS) in SV and midparental heights are 165.9 7.7 cm (0.4+/-0.5SDS), 163.3+/-p9.0 cm (-1.40+/-0.8SDS), respectively. Final adult heights are 158.87.4 cm(-0.9+/-1.2SDS) in SW and 156.7+/-7.4 cm (-1.5+/-1.2SDS) in SV. In control group, pretreatment predicted adult height and midparental heights were 154.8+/-12.0 cm (-2.1+/-0.6SDS) in SW, 149.9+/-6.3 cm (-2.3+/-0.7SDS) in SV and 159.8+/-7.2 cm (-1.2+/-0.5SDS) in SW, 158.6+/-5.1 cm (-0.6+/-0.8SDS) in SV. Final heights are 154.4+/-5.3 cm (-2.1+/-0.6SDS) in SW and 153.6+/-4.1 cm (-1.5+/-0.8SDS) in SV. There was no significant difference in comparison of FH between control group and treatment group (By Mann-Whitney test, SW P=0.063, SV P=0.663). But it was significant in comparison predicted adult height and final adult height in treatment group (By Wilcoxon Signed Ranks test P=0.043 in SW, P=0.008 in SV). CONCLUSION: In CAH children with precocious puberty, treatment with GnRH agonist alone treatment is effective to improve final adult height. But the effectiveness is limited. So GH or GnRHa combined with GH therapy is more attempted.

Leptin Levels in Children with Type 1 Diabetes Mellitus / 대한소아내분비학회지

PURPOSE: Leptin is a product of ob gene in adipose tissue. This is a important factor for regulation of body weight, especially body fat mass. So it is interested in correlation of leptin with obesity or various diseases related with obesity, particularly diabetes mellitus. It was demonstrated that leptin level was increased in type 2 diabetes and insulin was associated with increased leptin level. This study is to measure leptin levels in type 1 diabetes without insulin resistance, to validate whether insulin increases leptin level and to investigate correlation of leptin with degree of diabetic control and pubertal progression. METHODS: This study included 114 children(boys; 57, girls; 57) with type 1 diabetes, whose blood samples were taken to measure leptin level using RIA(radioimmunoassay). We observed the correlation of leptin with body mass index, HbA1c, insulin dose, duration of disease, and pubertal degree. RESULTS: Mean values of age, BMI, insulin dose, and HbA1c at the measurement of leptin were 13.0+/-5.04 years-old, 18.9+/-3.5kg/m2, 36U/day, and 8.9+/-2.1%, respectively. Thirty seven children were prepubertal and fifty seven children had pubertal characteristics and the rests were not confirmed. With increment of BMI, serum leptin levels were significantly increased in the all subjects, with the same correlation in pubertal female group, but not in pubertal male. There was a relatively weak correlation(r=0.29) of leptin levels with insulin doses. In prepubertal male, there was a significant positive correlation(r=0.55) of leptin with insulin dose, whereas in pubertal male there was shown negative correlation(r=-0.41). Female patients had a significantly higher leptin levels than male, irrespective of pubertal changes or BMI. There was not a significant correlation of leptin levels with duration of diabetes and HbA1c level. CONCLUSION: It is suggested that blood levels of insulin and sex hormones play an important roles in determining the levels of leptin.

Factors Associated with the Development of Anti-insulin Antibody in Diabetic Children / 대한소아내분비학회지

PURPOSE: Anti-insulin antibodies develop within several months of initiation of insulin therapy in most of diabetic patients. The purpose of this study is to observe the relationship between the clinical factors and development of anti-insulin antibody METHODS: Serum was collected from 116 diabetic patients and 47 nondiabetic children for the measurement of anti-insulin antibody titer by radioimmunoassay (RIA). Retrospective analysis of the medical records of clinical factors were evaluated. RESULTS: There was no relationship of anti-insulin antibody titer with age, duration, HbA1c, insulin dose, and BMI in diabetic children. There was no difference in anti-insulin antibody titer according to the sex, the presence of family history, the presence of DKA, the presence of complications, the presence of puberty, species of insulin, duration of disease in diabetic children. The titers of anti-insulin antibody were significantly higher in type 1 diabetic children(30.3+/-17.9% in type 1 and 16.5+/-7.0% in type 2, P7%. The positive rates of anti-insulin antibody were higher in male patients with diabetes(73.2% in male and 53.3% in female, P7%). CONCLUSION: The results suggests that anti-insulin antibody developed more likely in type 1 DM and less likely in DM patients whose control had been good and who used less insulin doses, which remains to be studied further with more patients for longer duation.

Cellular electrophysiology of fast pathway ablation of rabbit atrioventricular node

Discrete radiofrequency lesion at the atrial insertion site of the tendon of Todaro in the perfused rabbit preparation lengthens A-H interval, mimicking fast pathway input ablation. This study attempts to define the cellular electrophysiology of the ablation region prior to and after the elimination of fast AV node conduction. In six superfused rabbit AV node preparations, the cellular electrophysiology around the region of the atrial insertion to the tendon of Todaro was recorded using standard microelectrode technique prior to and after ablation. Before ablation, the action potentials recorded in the area of proposed lesion were exclusively from atrial or AN cells. At postablation, the superior margin of the lesion was populated with atrial or AN cells. AN, N, or NH cells bordered the lower part of the lesion. Electrophysiology of surviving cells at the edges of the lesion showed no significant changes in their Vmax, APD50 or APD90 and MDP from preablation values. Fast AV node pathway input ablation in the rabbit heart can be accomplished with a singular lesion around the atrial insertion site of the tendon of Todaro, involving atrial or AN cells. The results of the studies imply that inputs to the compact node may act as a substrate for successful ablation of AV node reentry tachycardia.