PURPOSE: Sex hormone-binding globulin (SHBG) modulates the availability of biologically active free sex hormones. The regulatory role of SHBG might be important in the relationship between hormone levels and the modification of lipid profiles in girls with precocious puberty. However, few studies have evaluated the relationship of SHBG, free estradiol index (FEI), and lipid levels in these girls. METHODS: One hundred and nine girls less than 8 years of age with pubertal development were enrolled. FEI was calculated with SHBG and estradiol (E2). We analyzed SHBG between peak luteinizing hormone (LH)> or =5 (IU/L) (group 1) and LH<5 (IU/L) (group 2) through a gonadotropin releasing hormone stimulation test. RESULTS: Body mass index (BMI) standard deviation score (SDS) was higher in group 2 than in group 1 (P=0.004). Serum SHBG levels did not differ and FEI was not higher in group 1 (P=0.122). Serum cholesterol, HDL, and LDL did not differ; however, triglyceride levels were higher in group 2 (P=0.023). SHBG was negatively correlated with bone age advancement, BMI, BMI SDS, and FEI, and was positively correlated with HDL. However, SHBG was not correlated with E2 or peak LH. CONCLUSION: Serum SHBG itself might not be associated with precocious puberty in girls, but it might be related to BMI and lipid profiles. Further studies are needed to reveal the relationship between sex hormone and obesity in girls with precocious puberty.
Body Mass Index ; Cholesterol, HDL ; Estradiol ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone ; Luteinizing Hormone ; Obesity ; Puberty, Precocious ; Sex Hormone-Binding Globulin

PURPOSE: Sex hormone-binding globulin (SHBG) modulates the availability of biologically active free sex hormones. The regulatory role of SHBG might be important in the relationship between hormone levels and the modification of lipid profiles in girls with precocious puberty. However, few studies have evaluated the relationship of SHBG, free estradiol index (FEI), and lipid levels in these girls. METHODS: One hundred and nine girls less than 8 years of age with pubertal development were enrolled. FEI was calculated with SHBG and estradiol (E2). We analyzed SHBG between peak luteinizing hormone (LH)> or =5 (IU/L) (group 1) and LH<5 (IU/L) (group 2) through a gonadotropin releasing hormone stimulation test. RESULTS: Body mass index (BMI) standard deviation score (SDS) was higher in group 2 than in group 1 (P=0.004). Serum SHBG levels did not differ and FEI was not higher in group 1 (P=0.122). Serum cholesterol, HDL, and LDL did not differ; however, triglyceride levels were higher in group 2 (P=0.023). SHBG was negatively correlated with bone age advancement, BMI, BMI SDS, and FEI, and was positively correlated with HDL. However, SHBG was not correlated with E2 or peak LH. CONCLUSION: Serum SHBG itself might not be associated with precocious puberty in girls, but it might be related to BMI and lipid profiles. Further studies are needed to reveal the relationship between sex hormone and obesity in girls with precocious puberty.
Body Mass Index ; Cholesterol, HDL ; Estradiol ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone ; Luteinizing Hormone ; Obesity ; Puberty, Precocious ; Sex Hormone-Binding Globulin

PURPOSE: This study compares the prevalence of pubertal menstrual disorders in type 1 diabetes mellitus (DM) patients with that in normal subjects. METHODS: A questionnaire was used to obtain data on pubertal development and menstrual history from 43 type 1 DM patients, who were diagnosed at the pediatrics department of Severance Children's Hospital, Yonsei University, and 56 normal control subjects. We evaluated the estrogen levels in all type 1 DM patients. RESULTS: The age at onset of puberty did not significantly differ between the 2 groups. However, the prevalence of oligomenorrhea in type 1 DM patients (21.21%) was significantly higher than that in the control group (2.08%; P<0.05). In addition, the incidence of amenorrhea in type 1 DM patients (20.59%) was significantly higher than that in the control group (2.08%; P<0.05). Pubertal development in type 1 DM patients was delayed due to elevated levels of glycosylated hemoglobin (HbA1c). The delay (9 months) in pubertal development in the patients diagnosed with type 1 DM at prepubertal ages was shorter than that (14.8 months) in the patients diagnosed with type 1 DM at postpubertal age (P<0.05). The level of LH, FSH were higher and E2 level was lower in DM patients compared to control group, especially in diabetics with amenorrhea (P<0.05). CONCLUSION: We observed a high frequency of delayed pubertal development and menstruation irregularities in patients who were diagnosed with type 1 DM at the prepubertal age or in type 1 DM patients with high levels of HbA1c. This finding can be attributed to the decreased plasma levels of E2 or due to the decreased E2 activity in patients with type 1 DM. Further evaluation of hormonal changes in type 1 DM patients is essential.
Amenorrhea ; Diabetes Mellitus ; Diabetes Mellitus, Type 1 ; Estrogens ; Female ; Hemoglobin A, Glycosylated ; Humans ; Incidence ; Menstruation ; Menstruation Disturbances ; Oligomenorrhea ; Pediatrics ; Plasma ; Prevalence ; Puberty ; Surveys and Questionnaires

Spot urinary albumin to creatinine ratio (ACR) measurement has been suggested as a surrogate to 24-hr urine collection for the assessment of microalbuminuria, and cystatin C (cysC) is known as an advantageous marker for renal function. The aim of this study was to evaluate the clinical values of spot urinary ACR and serum cysC for the assessment of diabetic nephropathy instead of 24-hr urine microalbumin in children and adolescents with diabetes. A total of 113 children and adolescents (age 12-19 yr, M:F = 47:66) with type 1 or 2 diabetes were enrolled. We evaluated the validity of spot urine ACR and serum cysC, and then compared them to 24-hr urine microalbumin and creatinine clearance. Spot urine ACR was correlated with 24-hr urine albumin excretion (R2 = 0.828, P = 0.001) and creatinine clearance (R2 = 0.249, P = 0.017). The ROC curve analysis of serum cysC demonstrated higher diagnostic accuracy than that of serum creatinine (AUC 0.732 vs 0.615). Both the measurements of spot urine ACR and serum cysC might better predict the presence of diabetic nephropathy than 24-hr urine microalbumin in childhood diabetic patients.
Adolescent ; Albuminuria/*urine ; Child ; Creatinine/*urine ; Cystatin C/*blood ; Diabetes Mellitus, Type 1/*diagnosis ; Diabetes Mellitus, Type 2/*diagnosis ; Diabetic Nephropathies/*diagnosis ; Female ; Glomerular Filtration Rate ; Hemoglobin A, Glycosylated/analysis ; Humans ; Kidney Function Tests ; Male ; Predictive Value of Tests ; ROC Curve

BACKGROUND: Both the Trendelenburg position and pneumoperitoneum with carbon dioxide have been reported to increase intracranial pressure (ICP) and to alter cerebral blood flow or cerebral blood volume. Also anesthetic agents have variable effects on cerebral hemodynamics and ICP. The present study was conducted to determine whether regional cerebral oxygen saturation (rSO2) values differ between propofol and sevoflurane anesthesia during laparoscopic surgery in the Trendelenburg position. METHODS: Thirty-two adult women undergoing gynecological laparoscopic surgery were divided into sevoflurane and propofol groups. rSO2 values were recorded at 10 min after induction in the neutral position (Tpre), 10 min after the pneumoperitoneum in the Trendelenburg position (Tpt) and 10 min after desufflation in the neutral position (Tpost). For analysis of rSO2, we did ANOVA and univariate two-way ANCOVA with covariates being mean arterial pressure and end tidal carbon dioxide tension. RESULTS: Between sevoflurane and propofol groups, the change in rSO2 was significantly different even after ANCOVA. rSO2 at Tpt (76.3 +/- 5.9% in sevoflurane vs 69.4 +/- 5.8% in propofol) and Tpost (69.5 +/- 7.1% in sevoflurane vs 63.8 +/- 6.6% in propofol) were significantly higher in the sevoflurane group compared with the propofol group. In the propofol group, rSO2 at Tpost was significantly lower than at Tpre (71.1 +/- 4.8%) and cerebral oxygen desaturation occurred in two patients (14.3%). CONCLUSIONS: Significantly lower rSO2 values were observed in the propofol group during gynecological laparoscopic surgery. The possibility of cerebral oxygen desaturation should not be overlooked during propofol anesthesia even after desufflation of the abdomen in the neutral position.
Abdomen ; Adult ; Anesthesia ; Anesthetics ; Antigens, Ly ; Arterial Pressure ; Blood Volume ; Carbon Dioxide ; Female ; Head-Down Tilt ; Hemodynamics ; Humans ; Hypoxia, Brain ; Intracranial Pressure ; Isoantigens ; Laparoscopy ; Methyl Ethers ; Oxygen ; Pneumoperitoneum ; Propofol ; Prostaglandins, Synthetic ; Spectroscopy, Near-Infrared

This study was done to characterize the natural course of C-peptide levels in patients with type 1 diabetes and identify distinguishing characters among patients with lower rates of C-peptide decline. A sample of 95 children with type 1 diabetes was analyzed to retrospectively track serum levels of C-peptide, HbA1c, weight, BMI, and diabetic complications for the 15 yr after diagnosis. The clinical characteristics were compared between the patients with low and high C-peptide levels, respectively. The average C-peptide level among all patients was significantly reduced five years after diagnosis (P < 0.001). The incidence of diabetic ketoacidosis was significantly lower among the patients with high levels of C-peptide (P = 0.038). The body weight and BMI standard deviation scores (SDS) 15 yr after diagnosis were significantly higher among the patients with low C-peptide levels (weight SDS, P = 0.012; BMI SDS, P = 0.044). In conclusion, C-peptide level was significantly decreased after 5 yr from diagnosis. Type 1 diabetes patients whose beta-cell functions were preserved might have low incidence of diabetic ketoacidosis. The declines of C-peptide level after diagnosis in type 1 diabetes may be associated with changes of body weight and BMI.
Adolescent ; Body Mass Index ; Body Weight ; C-Peptide/*blood ; Child ; Child, Preschool ; Diabetes Complications ; Diabetes Mellitus, Type 1/blood/*diagnosis ; Diabetic Ketoacidosis/epidemiology ; Diabetic Retinopathy/epidemiology ; Female ; Follow-Up Studies ; Hemoglobin A, Glycosylated/analysis ; Humans ; Incidence ; Infant ; Male ; Peripheral Nervous System Diseases/epidemiology ; Retrospective Studies

PURPOSE: We observed the frequencies of microvascular complications and their related factors in patients with type 1 diabetes. METHODS: Two hundred seventy one patients (111 males and 160 females, mean age 21.9 +/- 5.8 years) with type 1 diabetes were included. Subjects were all at least 10 years old and had diabetes for at least two years. Three types of microvascular complications (diabetic nephropathy, retinopathy, and neuropathy) were evaluated, and their frequencies and risk factors were analyzed. RESULTS: The overall prevalence of microvascular disease was 83/271 (30.6%). Microalbuminuria had developed in 39 patients (14.4%), persistent microalbuminuria in 31 patients (11.4%), and proteinuria in 12 (4.4%). Diabetic retinopathy had developed in 35 patients (12.9%) and neuropathy in 39 patients (13.7%). The mean HbA1c for 10 years was significantly higher in the patients with microvascular complications (10.5 +/- 2.8% vs. 8.4 +/- 1.4%). The rate of intensive management in the non-complication group was higher than in the microvascular complication group. The incidence of microalbuminuria was greater in males than females, but there were no gender differences in either diabetic retinopathy or neuropathy. Puberty and the duration of diabetes may be associated with microvascular complications. CONCLUSION: The incidence of microvascular complications in type 1 diabetes is declining. Sex, puberty, and the duration of diabetes as well as prolonged hyperglycemia are all related to microvascular complications. Therefore, intensive insulin management and regular screening for microvascular complications should be performed in patients who have either entered into puberty or have had diabetes for more than 5 years.
Diabetes Complications ; Diabetes Mellitus, Type 1 ; Diabetic Retinopathy ; Female ; Humans ; Hyperglycemia ; Incidence ; Insulin ; Male ; Mass Screening ; Prevalence ; Proteinuria ; Puberty ; Risk Factors

Hypomagnesemia may arise from various disorders such as renal magnesium wasting, familial hypomagnesemia, inadequate intake and increased gastrointestinal loss. Hypomagnesemia and hypocalcemia were found in a month-old female patient with generalized tonic-clonic seizure. Twenty-four hour urine collection samples were used to assess renal magnesium wasting; fractional excretion of 24-hr urine magnesium was less than 1.45%, i.e., within the normal limits. The patient had no history of chronic diarrhea or failure to thrive, which supports the conclusion that intake was adequate. She had no family history of hypocalcemia, hypomagnesemia, or seizures. Here, we report a case of idiopathic hypomagnesemia.
Diarrhea ; Failure to Thrive ; Female ; Humans ; Hypocalcemia ; Magnesium ; Seizures ; Urine Specimen Collection

PURPOSE: Noonan syndrome (NS) is characterized by short stature, congenital heart defects, mild mental retardation, and characteristic faces. We investigated the efficacy of growth hormone (GH) treatment and the adverse effect compared to sex and age-matched patients with growth hormone deficiency (GHD). METHODS: We included patients whose Noonan scores were over 60, treated with GH in Severance Children's Hospital. We analyzed height and height velocity before and during GH treatment in 14 NS patients (0.81 +/- 0.13 U/kg/wk) and also in 42 patients with sex- and age-matched GHD as a control group (0.78 +/- 0.17 U/kg/wk) at intervals of 3 months. RESULTS: At the start of GH treatment, mean age was 10.0 +/- 2.4 years, and mean height was 123.3 +/- 13.5 cm, and the height SDS was -2.79 +/- 0.85 in NS, while the mean age was 10.3 +/- 2.6 years, mean height was 119.6 +/- 13.5 cm, and the height SDS was -3.43 +/- 1.56 in GHD. Mean duration was 3.8 +/- 2.1 years in NS and 4.9 +/- 2.4 years in GHD. Mean height SDS increased from -2.79 SDS to -1.94 SDS in NS (p = 0.007) and from -3.43 SDS to -1.82 SDS in GHD (p 0.0001). Growth velocity increased from 3.7 +/- 1.2 cm/yr to 8.5 +/- 2.5 cm/yr (p < 0.0001) and 6.5 +/- 2.9 cm/yr (p = 0.016) during the first and second years of GH treatment, respectively, in NS and from 3.4 +/- 1.5 cm/yr to 8.8 +/- 2.3 cm/yr (p < 0.0001) and 8.1 +/- 3.2 cm/yr (p < 0.0001) in GHD. No severe adverse effects were observed during treatment. CONCLUSION: GH treatment in the NS patients increased growth velocity significantly, especially during the 1st year of treatment. GH treatment in NS is thought to be effective and relatively safe.
Growth Hormone ; Heart Defects, Congenital ; Humans ; Intellectual Disability ; Noonan Syndrome ; Treatment Outcome

Multiple endocrine neoplasia (MEN) 2B is charaterized by tumors of endocrine glands, consisting of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuromas of the tongue, lips and other sites. Especially, MTC is the main cause of death in patients who have not received early prophylactic treatment, and MTC in MEN 2B represents more aggressive progress than that of MEN 2A. We encountered two cases of multiple endocrine neoplasia type 2B. One was a 13 month old boy who had familial history of MEN 2B without any symptoms, and the other was a 6-year old boy who manifested multiple mucosal neuromas of the tongue which had been aggravated in four months. Their genetic analysis revealed a point mutation 918th cordon in the RET proto-oncogene. Both of them underwent an operation for prophylactic total thyroidectomy and the 6 year old boy's specimen turned out to be thyroid medullary carcinoma. We encountered two cases of MEN 2B with prophylactic thyroidectomy by early diagnosis of RET proto-oncogene, and report the cases with review of literature.