Maternal Hyperglycemia during Pregnancy Increases Adiposity of Offspring

Background@#The effect of intrauterine hyperglycemia on fat mass and regional fat proportion of the offspring of mothers with gestational diabetes mellitus (OGDM) remains to be determined. @*Methods@#The body composition of OGDM (n=25) and offspring of normoglycemic mothers (n=49) was compared using dualenergy X-ray absorptiometry at age 5 years. The relationship between maternal glucose concentration during a 100 g oral glucose tolerance test (OGTT) and regional fat mass or proportion was analyzed after adjusting for maternal prepregnancy body mass index (BMI). @*Results@#BMI was comparable between OGDM and control (median, 16.0 kg/m2 vs. 16.1 kg/m2 ). Total, truncal, and leg fat mass were higher in OGDM compared with control (3,769 g vs. 2,245 g, P=0.004; 1,289 g vs. 870 g, P=0.017; 1,638 g vs. 961 g, P=0.002, respectively), whereas total lean mass was lower in OGDM (15,688 g vs. 16,941 g, P=0.001). Among OGDM, total and truncal fat mass were correlated with fasting and 3-hour glucose concentrations of maternal 100 g OGTT during pregnancy (total fat mass, r=0.49, P=0.018 [fasting], r=0.473, P=0.023 [3-hour]; truncal fat mass, r=0.571, P=0.004 [fasting], r=0.558, P=0.006 [3-hour]), but there was no correlation between OGDM leg fat mass and maternal OGTT during pregnancy. Regional fat indices were not correlated with concurrent maternal 75 g OGTT values. @*Conclusion@#Intrauterine hyperglycemia is associated with increased fat mass, especially truncal fat, in OGDM aged 5 years.

Short-term efficacy of 1-month and 3-month gonadotropin-releasing hormone agonist depots in girls with central precocious puberty

Purpose@#Gonadotropin-releasing hormone agonist (GnRHa) has been the mainstay of central precocious puberty (CPP) treatment for decades, but few reports have compared the efficacy of 1-month and 3-month depot GnRHa formulations. This study investigates the short-term efficacy of 1-month and 3-month GnRHa depots in girls with CPP. @*Methods@#Overall, 150 girls with CPP were included in a retrospective review of medical records. Subjects in group 1 (n=105) were treated with 1-month GnRHa depots for ≥12 months, and those in group 2 (n=45) were treated with 1-month GnRHa depots for 6 months followed by 3-month GnRHa depots for ≥6 months. Anthropometric and biochemical data were compared between the groups at 3-time points (after 0, 6, and 12 months of GnRHa treatment). @*Results@#Demographic and clinical characteristics did not differ between the groups at baseline or after 6 months of GnRHa treatment. After 12 months of GnRHa treatment, patients in the both groups showed no difference in bone age (BA), chronological age (CA), BA–CA difference, height standard deviation score (SDS) for CA and BA, or body mass index SDS for CA and BA. The sexual maturity rate of the breast was prepubertal at 12 months in most of subjects. GnRH-stimulated luteinizing hormone (LH) level was suppressed during GnRHa treatment in both groups at 6 and 12 months, although the LH level in group 2 was higher than that in group 1. @*Conclusion@#Treating CPP with a 3-month GnRHa depot showed short-term efficacy comparable to that with a 1-month depot in anthropometric parameters and pubertal suppression.

Maternal Hyperglycemia during Pregnancy Increases Adiposity of Offspring

Background@#The effect of intrauterine hyperglycemia on fat mass and regional fat proportion of the offspring of mothers with gestational diabetes mellitus (OGDM) remains to be determined. @*Methods@#The body composition of OGDM (n=25) and offspring of normoglycemic mothers (n=49) was compared using dualenergy X-ray absorptiometry at age 5 years. The relationship between maternal glucose concentration during a 100 g oral glucose tolerance test (OGTT) and regional fat mass or proportion was analyzed after adjusting for maternal prepregnancy body mass index (BMI). @*Results@#BMI was comparable between OGDM and control (median, 16.0 kg/m2 vs. 16.1 kg/m2 ). Total, truncal, and leg fat mass were higher in OGDM compared with control (3,769 g vs. 2,245 g, P=0.004; 1,289 g vs. 870 g, P=0.017; 1,638 g vs. 961 g, P=0.002, respectively), whereas total lean mass was lower in OGDM (15,688 g vs. 16,941 g, P=0.001). Among OGDM, total and truncal fat mass were correlated with fasting and 3-hour glucose concentrations of maternal 100 g OGTT during pregnancy (total fat mass, r=0.49, P=0.018 [fasting], r=0.473, P=0.023 [3-hour]; truncal fat mass, r=0.571, P=0.004 [fasting], r=0.558, P=0.006 [3-hour]), but there was no correlation between OGDM leg fat mass and maternal OGTT during pregnancy. Regional fat indices were not correlated with concurrent maternal 75 g OGTT values. @*Conclusion@#Intrauterine hyperglycemia is associated with increased fat mass, especially truncal fat, in OGDM aged 5 years.

Short-term efficacy of 1-month and 3-month gonadotropin-releasing hormone agonist depots in girls with central precocious puberty

Purpose@#Gonadotropin-releasing hormone agonist (GnRHa) has been the mainstay of central precocious puberty (CPP) treatment for decades, but few reports have compared the efficacy of 1-month and 3-month depot GnRHa formulations. This study investigates the short-term efficacy of 1-month and 3-month GnRHa depots in girls with CPP. @*Methods@#Overall, 150 girls with CPP were included in a retrospective review of medical records. Subjects in group 1 (n=105) were treated with 1-month GnRHa depots for ≥12 months, and those in group 2 (n=45) were treated with 1-month GnRHa depots for 6 months followed by 3-month GnRHa depots for ≥6 months. Anthropometric and biochemical data were compared between the groups at 3-time points (after 0, 6, and 12 months of GnRHa treatment). @*Results@#Demographic and clinical characteristics did not differ between the groups at baseline or after 6 months of GnRHa treatment. After 12 months of GnRHa treatment, patients in the both groups showed no difference in bone age (BA), chronological age (CA), BA–CA difference, height standard deviation score (SDS) for CA and BA, or body mass index SDS for CA and BA. The sexual maturity rate of the breast was prepubertal at 12 months in most of subjects. GnRH-stimulated luteinizing hormone (LH) level was suppressed during GnRHa treatment in both groups at 6 and 12 months, although the LH level in group 2 was higher than that in group 1. @*Conclusion@#Treating CPP with a 3-month GnRHa depot showed short-term efficacy comparable to that with a 1-month depot in anthropometric parameters and pubertal suppression.

The durability and effectiveness of sensor-augmented insulin pump therapy in pediatric and young adult patients with type 1 diabetes

Purpose@#Despite the prevalent use of insulin pump therapy worldwide, few studies have been conducted among young patients with type 1 diabetes (T1D) in Korea. We investigated the durability and effectiveness of insulin pump therapy among Korean pediatric and young adult patients with T1D. @*Methods@#This study included 54 patients with T1D diagnosed at pediatric ages (range, 1.1–14.1 years) who initiated insulin pump therapy during 2016–2019 at Seoul National University Children's Hospital and Seoul National University Bundang Hospital. Clinical and biochemical data, including anthropometric measurements, insulin dose, and glycated hemoglobin (HbA1c) levels were obtained from T1D diagnosis to last follow-up. @*Results@#Forty-four patients (81.5%) continued insulin pump therapy with a median pump use duration of 2.9 years (range, 0.2–3.5 years); 10 discontinued the therapy within 12 months (<1 month, n=6; 1–6 months, n=1; and 6–12 months, n=3) due to physical interferences or financial problems. Older age (≥10 years of age) and longer diabetes duration (≥2 years) at the initiation of pump therapy were associated with discontinuation (P<0.05 for both). For patients continuing pump therapy, HbA1c levels significantly decreased after 1 year of therapy (from 8.9% to 8.1%, P<0.001) without changes in the body mass index z-scores or insulin dose. Although 4 patients experienced diabetic ketoacidosis, all recovered without complications. @*Conclusion@#Insulin pump therapy was effective in improving glycemic control in T1D patients during 12 months of treatment. Early initiation of insulin pump therapy after T1D diagnosis was helpful for continuing therapy.

The durability and effectiveness of sensor-augmented insulin pump therapy in pediatric and young adult patients with type 1 diabetes

Purpose@#Despite the prevalent use of insulin pump therapy worldwide, few studies have been conducted among young patients with type 1 diabetes (T1D) in Korea. We investigated the durability and effectiveness of insulin pump therapy among Korean pediatric and young adult patients with T1D. @*Methods@#This study included 54 patients with T1D diagnosed at pediatric ages (range, 1.1–14.1 years) who initiated insulin pump therapy during 2016–2019 at Seoul National University Children's Hospital and Seoul National University Bundang Hospital. Clinical and biochemical data, including anthropometric measurements, insulin dose, and glycated hemoglobin (HbA1c) levels were obtained from T1D diagnosis to last follow-up. @*Results@#Forty-four patients (81.5%) continued insulin pump therapy with a median pump use duration of 2.9 years (range, 0.2–3.5 years); 10 discontinued the therapy within 12 months (<1 month, n=6; 1–6 months, n=1; and 6–12 months, n=3) due to physical interferences or financial problems. Older age (≥10 years of age) and longer diabetes duration (≥2 years) at the initiation of pump therapy were associated with discontinuation (P<0.05 for both). For patients continuing pump therapy, HbA1c levels significantly decreased after 1 year of therapy (from 8.9% to 8.1%, P<0.001) without changes in the body mass index z-scores or insulin dose. Although 4 patients experienced diabetic ketoacidosis, all recovered without complications. @*Conclusion@#Insulin pump therapy was effective in improving glycemic control in T1D patients during 12 months of treatment. Early initiation of insulin pump therapy after T1D diagnosis was helpful for continuing therapy.

Pregnancy Outcomes of Women Additionally Diagnosed as Gestational Diabetes by the International Association of the Diabetes and Pregnancy Study Groups Criteria

BACKGROUND: We investigated the pregnancy outcomes in women who were diagnosed with gestational diabetes mellitus (GDM) by the International Association of the Diabetes and Pregnancy Study Groups (IADPSG) criteria but not by the Carpenter-Coustan (CC) criteria.METHODS: A total of 8,735 Korean pregnant women were identified at two hospitals between 2014 and 2016. Among them, 2,038 women participated in the prospective cohort to investigate pregnancy outcomes. Diagnosis of GDM was made via two-step approach with 50-g glucose challenge test for screening followed by diagnostic 2-hour 75-g oral glucose tolerance test. Women were divided into three groups: non-GDM, GDM diagnosed exclusively by the IADPSG criteria, and GDM diagnosed by the CC criteria.RESULTS: The incidence of GDM was 2.1% according to the CC criteria, and 4.1% by the IADPSG criteria. Women diagnosed with GDM by the IADPSG criteria had a higher body mass index (22.0±3.1 kg/m² vs. 21.0±2.8 kg/m², P<0.001) and an increased risk of preeclampsia (odds ratio [OR], 6.90; 95% confidence interval [CI], 1.84 to 25.87; P=0.004) compared to non-GDM women. Compared to neonates of the non-GDM group, those of the IADPSG GDM group had an increased risk of being large for gestational age (OR, 2.39; 95% CI, 1.50 to 3.81; P<0.001), macrosomia (OR, 2.53; 95% CI, 1.26 to 5.10; P=0.009), and neonatal hypoglycemia (OR, 3.84; 95% CI, 1.01 to 14.74; P=0.049); they were also at an increased risk of requiring phototherapy (OR, 1.57; 95% CI, 1.07 to 2.31; P=0.022) compared to the non-GDM group.CONCLUSION: The IADPSG criteria increased the incidence of GDM by nearly three-fold, and women diagnosed with GDM by the IADPSG criteria had an increased risk of adverse pregnancy outcomes in Korea.

Screening and management of thyroid dysfunction in preterm infants

Preterm infants can suffer various thyroid dysfunctions associated with developmental immaturity of the hypothalamic-pituitary-thyroid axis, postnatal illness, medications, or iodine supply. The incidence of thyroid dysfunction among preterm infants is higher than that among term infants and has been increasing with improvement in the survival of preterm infants. Hypothyroxinemia is frequently observed during the first week of life in extreme preterm neonates, and the incidence of delayed thyrotropin elevation is high at the age of 2–6 weeks. Although the necessity of routine rescreening remains controversial, recent guidelines on screening for congenital hypothyroidism have recommended rescreening of all preterm neonates. Thyroid hormone replacement is recommended for persistent thyrotropin elevation with or without hypothyroxinemia. Hypothyroxinemia without thyrotropin elevation does not require treatment, and some potential risks of levothyroxine supplementation have been reported. Although most thyroid dysfunctions are transient, careful follow-up after discontinuation of levothyroxine is considered so as to avoid missing persistent hypothyroidism.

Factors Associated with the Presence and Severity of Diabetic Ketoacidosis at Diagnosis of Type 1 Diabetes in Korean Children and Adolescents

The aim of this study was to identify the risk factors for presence and severity of diabetic ketoacidosis (DKA) at the onset of type 1 diabetes mellitus (T1DM) in Korean children and adolescents. A retrospective chart review of children and adolescents newly diagnosed with T1DM was conducted in seven secondary and tertiary centers in Korea. Eligible subjects were < 20 years of age and had records on the presence or absence of DKA at the time of T1DM diagnosis. DKA severity was categorized as mild, moderate, or severe. Data were collected on age, height, body weight, pubertal status, family history of diabetes, delayed diagnosis, preceding infections, health insurance status, and parental education level. A total of 361 patients (male 46.3%) with T1DM were included. Overall, 177 (49.0%) patients presented with DKA at T1DM diagnosis. Risk factors predicting DKA at T1DM diagnosis were age ≥ 12 years, lower serum C-peptide levels, presence of a preceding infection, and delayed diagnosis. Low parental education level and preceding infection increased the severity of DKA. These results suggest that alertness of the physician and public awareness of diabetes symptoms are needed to decrease the incidence and severity of DKA at T1DM diagnosis.

Glucose Homeostasis during Fetal and Neonatal Period / 대한주산의학회잡지

Glucose is essential for energy metabolism in human, especially in brain, and is a source of energy storage in the form of glycogen, fat and protein. During fetal life, the predominant source of energy is also glucose, which crosses the placenta by facilitated diffusion. There is very little endogenous glucose production under normal circumstances during fetal life. During labor, the fetus is exposed to physiological challenges that require metabolic adaptation. A healthy infant successfully manages the postnatal transition by mobilizing and using alternative. After birth, there is a rapid surge in catecholamine and glucagon levels, and a steady decrease in insulin, as blood glucose levels decline. These hormonal changes induce enzyme activities that lead to glycogenolysis and gluconeogenesis. During the first 24-48 hours of life, plasma glucose concentrations of neonates are typically lower than later in life. Distinguishing between transitional neonatal glucose regulation in normal neonates and hypoglycemia that persists or occurs for the first time beyond the first 72 hours of life is important for prompt diagnosis and treatment to avoid serious consequences.

Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

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 limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

Clinical and laboratory characteristics of neonatal hypocalcemia

PURPOSE: To describe the clinical characteristics of full-term neonates with hypocalcemia and to suggest factors associated with neonatal hypocalcemia METHODS: The medical records of full-term neonates with hypocalcemia were reviewed. Hypocalcemia was defined as an ionized calcium (iCa) concentration of <4 mg/dL. Parathyroid hormone (PTH) insufficiency was defined as a serum PTH level of <60 pg/mL or a serum phosphorus level higher than the serum calcium level in the presence of hypocalcemia. RESULTS: Fifty-three neonates were enrolled. The median age at diagnosis of hypocalcemia was 3 days. In all the neonates, formula feeding predominance was observed. Thirty-eight neonates (69.8%) were compatible with PTH insufficiency. The number of formula-fed neonates was significantly higher than that of breast-fed patients among neonates with PTH insufficiency (P=0.017). Intact PTH was negatively correlated with serum phosphorus levels. Twelve out of 14 neonates (85.7%) had 25-hydroxy vitamin D (25OHD) levels <20 ng/mL and 9 neonates (64.3%) had 25OHD levels <10 ng/mL. Twenty-one neonates had hypocalcemic tetany. The serum calcium and iCa concentrations of neonates with tetany were 4.2-8.3 mg/dL and 1.85-3.88 mg/dL, respectively. Three neonates showed symptomatic hypocalcemia with calcium levels over 7.5 mg/dL. Among the 16 neonates who underwent electroencephalography (EEG), 12 had abnormalities, which normalized after 1-2 months. CONCLUSION: Formula milk feeding, PTH insufficiency and low serum vitamin D concentration are associated with the development of neonatal hypocalcemia. Symptoms such as tetany and QT interval prolongation can develop in relatively mild hypocalcemia. Moreover, transient neonatal hypocalcemia can cause transient EEG abnormalities.

Blood glucose levels within 7 days after birth in preterm infants according to gestational age

PURPOSE: This study investigated blood glucose levels in preterm babies according to gestational age (GA). METHODS: Subjects were 141 preterm infants with a GA180 mg/dL. RESULTS: During the 7 days after birth, hypo- and hyperglycemia occurred in 29 (29 of 141, 20.6%) and 42 (42 of 141, 29.8%) neonates, respectively. During the first 2 hours, 18 neonates (12.8%) exhibited hypoglycemia, and only 2 (2 of 141, 1.4%) developed hyperglycemia. From 6 to 24 hours, hypo- and hyperglycemia were observed in 0 and 9 (9 of 141, 6.4%) neonates, respectively. Infants small for their GA (SGA) were at risk for hypoglycemia both within 24 hours (odds ratio [OR], 2.718; P=0.045) and during days 2 to 7 (OR, 4.454; P=0.006), and hyperglycemia during days 2 to 7 (OR, 3.200; P=0.005). Low 1-minite Apgar score was risk factor for both hypo- and hyperglycemia during days 2 to 7 (OR, 0.756; P=0.035 for hypoglycemia and OR, 0.789; P=0.016 for hyperglycemia). Both hypo- and hyperglycemia within 24 hours were less common in those who started feeding (OR, 0.294; P=0.013 for hypoglycemia and OR, 0.162; P=0.011 for hyperglycemia). CONCLUSION: Careful blood glucose level monitoring is required in preterm infants, especially SGA infants or those with low Apgar score. Early feeding could be beneficial for maintaining euglycemia.

Adrenocortical Function in Preterm Infants

Adrenal hormones are essential for the timely differentiation and maturation of fetal organs and the regulation of intrauterine homeostasis. These hormones play complex roles during fetal life, and they are believed to provide the cellular communication that coordinates maternal-fetal interactions. Cortisol serves to modulate functional adaptations for extrauterine life in the perinatal period. Serum cortisol levels of preterm infants are similar to basal levels reported for healthy full-term neonates. However, a rise of cortisol production is absent during illness. Compared with a fetus of a similar gestational age, premature maturation of the hypothalamic-pituitary-adrenal (HPA) axis is suggested in preterm infants; however, the inappropriate cortisol value observed in severe illness is due to immaturity of 11beta-hydroxylase activity and an immature response of the brain to stress. Cardiovascular instability associated with adrenal insufficiency is more frequent than formerly reported, and replacing glucocorticoids in the treatment of refractory hypotension is effective without an increase in short term adverse consequences. However, the diagnostic criteria and optimal management have not yet been determined. Thus, further understanding of perinatal adrenal function will provide insight into the improved management of preterm infants.

Adrenal and thyroid function in the fetus and preterm infant / 소아과

Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic vasopressor-resistant hypotension is associated with low levels of circulating cortisol, a result of immaturity of hypothalamic-pituitary-adrenal axis in preterm infants under stress. Over the past few decades, studies in preterm infants have shown abnormal clinical findings that suggest adrenal or thyroid dysfunction, yet the criteria used to diagnose adrenal insufficiency in preterm infants continue to be arbitrary. In addition, although hypothyroidism is frequently observed in extremely low gestational age infants, the benefits of thyroid hormone replacement therapy remain controversial. Screening methods for congenital hypothyroidism or congenital adrenal hyperplasia in the preterm neonate are inconclusive. Thus, further understanding of fetal and perinatal adrenal and thyroid function will provide an insight into the management of adrenal and thyroid function in the preterm infant.

Iodine and thyroid function

Severe iodine deficiency causes hypothyroidism that results in impaired somatic growth and motor development in children. Mild and moderate iodine deficiencies cause multifocal autonomous growth of thyroid, which results in thyrotoxicosis. On the other hand, iodine excess is associated with the development of hypothyroidism and thyroid autoimmunity. In areas of iodine deficiency, a sudden increase in iodine intake is associated with transient hyperthyroidism. Recent studies demonstrated that long-term thyroid function of subjects who experienced both iodine deficiency and iodine excess during childhood tended to be abnormal despite optimization of their current iodine intake. Iodine status in the Korean Peninsula is very unique because people in the Republic of Korea have been shown to have predominantly excessive iodine levels, whereas the Democratic People's Republic of Korea is known to be an iodine-deficient area. Further research is warranted to verify the optimal ranges of iodine intake and to clarify the effects of iodine intake on thyroid disorders in the Korean Peninsula.

Thyrotoxicosis Associated with Autoimmune Thyroiditis in Children and Adolescents

PURPOSE: The aims of this study are to estimate the frequency of thyrotoxicosis associated with autoimmune thyroiditis (TAIT) and to investigate the characteristics of TAIT in children and adolescents. METHODS: 220 subjects who had shown thyroid autoimmunity before the age of 18 were included. All the subjects were divided as autoimmune thyroiditis (AIT), Graves' disease and unspecified group. In addition, AIT was subdivided as TAIT and euthyroid or hypothyroid AIT. Clinical data were review and the variation of thyroid hormone status was investigated among the subjects with TAIT. RESULTS: Of 220 subjects who showed thyroid autoimmunity, 147 subjects were diagnosed as AIT and 47 were as Graves' disease. Among 147 subjects with AIT, 14 subjects (9.5%) had presented TAIT. Among 65 subjects who had initially presented with thyrotoxicosis, 8 subjects (12.3%) were compatible with TAIT. Whereas presenting symptoms of TAIT were similar to those of Graves' disease, Technetium-99m scan had showed thyroid with decreased uptake. The durations of thyrotoxicosis were several weeks. 6 of 14 TAIT subjects showed recurrent thyrotoxicosis, and 4 of 8 patients who had initially presented with thyrotoxicosis showed subclinical or overt hypothyroidism during follow-up. CONCLUSIONS: Possibility of TAIT should not be overlooked when a patient presents thyrotoxicosis, because this condition is not so rare in children and adolescents. Long term follow-up after resolution of thyrotoxicosis is necessary because of a diverse clinical course.

The Relationship between Initial Body Mass Index and Body Mass Index after One Year of Gonadotropin-Releasing Hormone Agonist Therapy in Idiopathic True Precocious Puberty Girls / 대한소아내분비학회지

PURPOSE: The aim of this study is to evaluate the relationship between initial body mass index (BMI) and BMI after gonadotropin-releasing hormone agonist (GnRHa) treatment in idiopathic true precocious puberty girls. METHODS: The subjects were 99 idiopathic true precocious puberty girls treated with GnRHa for more than 1 year. The patients were categorized into two groups according to initial BMI; normal weight group (BMI or = 85 percentile). We investigated chronologic age (CA), bone age (BA), BA advancement (BA-CA), height (Ht), Ht-standard deviation score (Ht_zs), BMI, BMI_zs, predicted adult height (PAH), and PAH_zs before initiation of GnRHa treatment and 1 year later. RESULTS: Baseline CA, BA, BA-CA, Ht_zs, and PAH showed no differences between normal weight group and overweight/obesity group. BMI_zs increased only in normal weight group, and DeltaBMI_zs was negatively correlated with initial BMI_zs (r = -0.501, P < 0.001). PAH_zs increased less in normal weight group (DeltaPAH_zs = 0.30) than in overweight/obesity group (DeltaPAH_zs = 0.66) (P = 0.02), but there was no correlation between initial BMI_zs and DeltaPAH_zs. DeltaBA-CA and DeltaHt_zs were not different between two groups either. Comparing patients with increased BMI_zs with those whose BMI_zs decreased or remained the same, there were no differences in DeltaBA-CA, DeltaHt_zs, and DeltaPAH_zs. On multiple regression analysis, DeltaBMI_zs was negatively correlated with initial BMI_zs, and it showed no correlation with CA, BA, BA-CA, height, and dose of GnRHa. CONCLUSION: BMI_zs increased after 1yr of GnRHa treatment in idiopathic true precocious puberty girls whose initial BMI_zs was normal, and its increment was negatively correlated with initial BMI_zs.

Clinical Picture of Adrenal Insufficiency-associated Hypotension in Preterm Infants

PURPOSE: This study aims to describe the clinical characteristics of adrenal insufficiency-associated hypotension in preterm infants and the effects of hydrocortisone therapy on their cardiovascular system and serum electrolytes. METHODS: Twelve preterm infants less than 32 gestational weeks admitted to neonatal intensive care unit (NICU) of the Seoul National University Bundang Hospital from January 2007 to August 2009 with clinical and laboratory findings suggestive of adrenal insufficiency were analyzed retrospectively. RESULTS: Gestational age was 27.8+/-2.5 weeks and birth weight was 1,110+/-307 g. Postnatal age, postmenstrual age, weight at the onset of adrenal insufficiency-associated hypotension were 19+/-7 day, 30.6+/-2.4 weeks, 1,285+/-365 g. In preterm infants who showed vasopressor resistance, intravenous hydrocortisone was started with a stress dose of 4 mg/kg/day, maintained for 2.2+/-0.7 days, and then tapered. Serum cortisol concentration before hydrocortisone administration was 11.6+/-4.1 mg/dL. Mean blood pressure increased from 25.0+/-5.4 mmHg to 35.0+/-5.3 mmHg, 38.3+/-8.0 mmHg and 41.9+/-6.5 mmHg at time of hydrocortisone administration and 2, 4 and 6 hours after hydrocortisone administration. Urine output increased from 0.9+/-0.6 mL/kg/hr to 4.1+/-3.4 mL/kg/hr. Twelve hours after the administration of hydrocortisone, dopamine requirement decreased from 11.0+/-2.9 microg/kg/min to 8.0+/-2.3 microg/kg/min, and to 5.5+/-3.4 microg/kg/min after 24 hours. Serum sodium concentration was increased from 130+/-4 mEq/L to 136+/-4 mEq/L, serum potassium concentration was decreased from 6.1+/-1.1 mEq/L to 4.6+/-0.6 mEq/L before and 12 hours after hydrocortisone administration. CONCLUSION: In preterm infants with adrenal insufficiency-associated hypotension, hydrocortisone administration improved blood pressure and urine output, decreased vasopressor requirement, and normalized serum electrolyte abnormalities.

Clinical Findings and Characteristics of Gonad at Diagnosis of Ovotesticular Disorders of Sex Development / 대한소아내분비학회지

PURPOSE: Ovotesticular disorder of sex development (OTDSD) is rare condition and defined as the presence of both ovarian and testicular tissues in the same individual. We aimed to investigate clinical characteristics of patients and histologic findings of gonads at diagnosis of OTDSD and to characterize patients diagnosed with gonadal tumor. METHODS: We retrospectively reviewed the records of 20 patients histologically confirmed as OTDSD between 1980 and 2009 in Seoul National University Hospital. Age, chief complaints, karyotype, external and internal genital organs at diagnosis and gonadal histology were investigated. RESULTS: Age at histologic diagnosis of OTDSD was 5.1 years of age (0.1 - 28 yr). Chief complaints at diagnosis included wide spectrum of ambiguous genitalia including hypospadia and clitoromegaly (n = 15), inguinal mass (n = 3) and gynecomastia (n = 2). Thirteen cases (65%) showed 46,XX karyotype and 24 (61.5%) of 39 gonads histologically confirmed ovotestes. Testis was found in right (83.3%), in inguinal or scrotal area (84.2%) and ovary was in left (88.9%), in pelvis or abdomen (88.9%). Three cases (15%) were diagnosed with gonadal tumor, who all had undergone gonadectomy in the second decade due to belated identification of gonad (10 yr) despite early diagnosis, incidentally developed clitoromegaly (12.9 yr), and inguinal mass (28 yr) after surgical correction of clitoromegaly at 2.9 years of age. CONCLUSION: Patients with OTDSD had wide spectrum of ambiguous genitalia including hypospadia and clitoromegaly. This study provides the importance of early diagnosis and gonadectomy in patients with OTDSD considering increased risk for gonadal tumor in the second decade.