PURPOSE:There is a clear sexual dimorphism in circulating concentration of leptin in adulthood. However, we don' know when such dimorphism begins and how much pubertal development influences on it. So we examined body composition and circulating concentrations of leptin according to Tanner stage(TS). METHODS:We examined 112 children(M; 56, F; 56, Age; 8.5-17 yr) to evaluate the relationship of leptin and body composition. Body composition was determined by bioelectric impedence measurements(BIA) and by anthropometry. Leptin was measured by human specific RIA. Leptin level was analysed according to TS, body mass index(BMI), fat mass(FM), and lean body mass. RESULTS:BMI and free FM was correlated with TS in both sexes. FM was closely correlated with TS in girls but not in boys(M; r=0.08, P=0.54. F; r=0.73, P>0.001). Leptin levels increased in girls with advanced TS(r=0.355, P<0.01), but decreased in boys(r=-0.339, P<0.01). A strong exponential relationship was observed for leptin levels with BMI, FM, and percentage body fat as determined by BIA. There was significant sexual dimorphism of leptin level at TS VI/V. Because leptin level was significantly related FM, leptin level was normalized to FM(Leptin/FM). Leptin/FM of females(0.67+/-.27 ng/mL/kg) was also significantly higher then that of males(0.31+/-.15 ng/mL/kg)(P<0.001). CONCLUSION: These data suggest that plasma leptin levels increase in girls and decrease in boys after TS II as pubertal development proceeds; they show a significant gender difference, especially late puberty, even after adjustment for FM. Sexual dimorphism in leptin during puberty reflects not only differential changes in body composition but also different leptin resistance; reference ranges of leptin could be modified by TS and gender.
Adipose Tissue ; Adolescent* ; Anthropometry ; Body Composition* ; Child* ; Female ; Humans ; Leptin* ; Plasma ; Puberty ; Reference Values

PURPOSE:There is a clear sexual dimorphism in circulating concentration of leptin in adulthood. However, we don' know when such dimorphism begins and how much pubertal development influences on it. So we examined body composition and circulating concentrations of leptin according to Tanner stage(TS). METHODS:We examined 112 children(M; 56, F; 56, Age; 8.5-17 yr) to evaluate the relationship of leptin and body composition. Body composition was determined by bioelectric impedence measurements(BIA) and by anthropometry. Leptin was measured by human specific RIA. Leptin level was analysed according to TS, body mass index(BMI), fat mass(FM), and lean body mass. RESULTS:BMI and free FM was correlated with TS in both sexes. FM was closely correlated with TS in girls but not in boys(M; r=0.08, P=0.54. F; r=0.73, P>0.001). Leptin levels increased in girls with advanced TS(r=0.355, P<0.01), but decreased in boys(r=-0.339, P<0.01). A strong exponential relationship was observed for leptin levels with BMI, FM, and percentage body fat as determined by BIA. There was significant sexual dimorphism of leptin level at TS VI/V. Because leptin level was significantly related FM, leptin level was normalized to FM(Leptin/FM). Leptin/FM of females(0.67+/-.27 ng/mL/kg) was also significantly higher then that of males(0.31+/-.15 ng/mL/kg)(P<0.001). CONCLUSION: These data suggest that plasma leptin levels increase in girls and decrease in boys after TS II as pubertal development proceeds; they show a significant gender difference, especially late puberty, even after adjustment for FM. Sexual dimorphism in leptin during puberty reflects not only differential changes in body composition but also different leptin resistance; reference ranges of leptin could be modified by TS and gender.
Adipose Tissue ; Adolescent* ; Anthropometry ; Body Composition* ; Child* ; Female ; Humans ; Leptin* ; Plasma ; Puberty ; Reference Values

BACKGROUND: Pulmonary arterial hypertension (PAH) in systemic lupus erythematosus (SLE) has very poor prognosis and the prevalence of the disease is also not well delineated. This study was designed to assess the prevalence of PAH in asymptomatic SLE patients and to analyze the potential association between the presence of PAH and SLE including disease activity, organ involvement, and serology. METHODS: Asymptomatic 50 SLE patients and 50 healthy controls of a similar age and sex were assessed by an echocardiographic and clinical study. The diagnosis of PAH was made when the right ventricular systolic pressure (RVSP), measured by 2-dimensional echocardiography, was > or =40 mmHg. RESULTS: Compared to control subjects, lupus patients had significantly increased RVSP (mean+/-SD, 34.9+/-8.9 vs 31.2+/-3.6 mmHg, p=0.008). Patients with SLE were divided into three groups: RVSP> or =40 mmHg, RVSP=30~39 mmHg, RVSP<30 mmHg. Seven patients (14%) had RVSP> or =40 mmHg, 36 patients (72%) had RVSP=30~39 mmHg and 7 patients (14%) had RVSP<30 mmHg. In the healthy controls, none of them had RVSP> or =40 mm Hg. Of the controls, 43 (86%) had RVSP=30~39 mmHg and 7 (14%) had RVSP<30 mmHg. There was no statistical difference in organ involvement, Raynaud's phenomenon, vasculitis, anti-RNP and anti-cardiolipin Ab among the three SLE groups. However, lupus disease activity (checked by SLEDAI-2K) was associated with PAH (p=0.014). CONCLUSIONS: The prevalence of PAH as seen on echocardiograms in SLE patients was 14%. Organ involvement, Raynaud's phenomenon, vasculitis, antiphopholipid antibodies and anti-RNP except SLEDAI-2K were not associated with pulmonary arterial pressure.
Antibodies ; Arterial Pressure ; Blood Pressure ; Diagnosis ; Echocardiography ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Lupus Erythematosus, Systemic* ; Prevalence ; Prognosis ; Vasculitis

PURPOSE: The purpose of this study was to describe the clinical outcome after pancreatcetmy and its relationship with pathological appearances and clinical features in patients with persistent hyperinsulinemic hypoglycemia of infancy(PHHI). METHODS: Medical records of 10 patients(9 males and 1 female, mean age:40.4+/-1.5 months) who were diagnosed as PHHI and underwent pancreatectomy from 1988 to 2000 were reviewed. Clincal and biochemical data were recorded. Subjects were classified arbitrarily into early-onset or late-onset group according to age of onset. Pathologic appearance of pancreas was divided into 2 forms:diffuse or focal. The former had a focal pancreatic adenomatous hyperplasia and the latter was characterized by increased number of betacells with similar distribution seen in normal neonates. RESULTS: One patient had focal, and nine had diffuse lesions. After near-total pancreatectomy, 4 patients(40.0%) showed complete response, 4(40.0%) had persistent hypoglycemia, and 2(20.0%) developed diabetes mellitus. As neurological sequelae, 6 patients(60.0%) had persistent seizures, and 6(60.0%) had delayed motor and speech development. No clinical or biochemical factors related to postoperative outcome were found. CONCLUSION: This data indicate that early diagnosis of patients who present with hypoglycemic symptoms in infancy, especially early in life, and development of more effective therapy are warranted, because there is no clinical or biochemical factor predicting final outcome after near-total pancreatectomy and only 40% of patients with PHHI remained euglycemic after surgery with possible severe neurological sequelae.
Age of Onset ; Congenital Hyperinsulinism* ; Diabetes Mellitus ; Early Diagnosis ; Female ; Humans ; Hyperplasia ; Hypoglycemia ; Infant, Newborn ; Male ; Medical Records ; Pancreas ; Pancreatectomy* ; Seizures

PURPOSE: Pseudohypoparathyroidism(PHP) is caused by a defect of G protein and receptor despite of normal parathyroid hormone(PTH) secretion. It is a rare disorder characterized by hypocalcemia, hyperphophatemia, elevated PTH levels and albright hereditory osteodystrophy(AHO). We retrospectively reviewed the clinical characteristics of PHP. METHODS: We reviewed clinical features, laboratory findings, and outcome to treatment of 8 PHP patients, diagnosed at Seoul National University Hospital from 1988 to rool. RESULTS: Male to Female ratio was 1.7:1 and mean age at diagnosis was 11.8 years old. The initial average height SDS was 0.13+/-.08 and the average weight SDS was 0.43+/-.31. The most common symptom was seizure. Only one patient had typical AHO, three patients had mental retardation. Brain MRI or CT showed basal ganglia calcification in 3 patients. All patients treated with vitamin D and calcium supplementation could maintained normal serum levels of calcium and phophorus. CONCLUSION: PHP should be suspected in patient with seizure of unknown origin, aged above 5 year-old. AHO and mental retardation could be adjuvant signs to the diagnosis of PHP. But definite diagnosis could be made by laboratory work up.
Basal Ganglia ; Brain ; Calcium ; Child, Preschool ; Diagnosis ; Female ; GTP-Binding Proteins ; Humans ; Hypocalcemia ; Intellectual Disability ; Magnetic Resonance Imaging ; Male ; Pseudohypoparathyroidism* ; Retrospective Studies ; Seizures ; Seoul ; Vitamin D

Demyelinating syndrome can rarely occur in Sjogren's syndrome or rheumatoid arthritis. We describe a patient of Sjogren's syndrome with multiple sclerosis-like features whose rheumatoid arthritis has been managed for 3 years. The patient presented paraparesis and urinary retention, and improved with high-dose corticosteroid therapy.
Arthritis, Rheumatoid* ; Humans ; Multiple Sclerosis* ; Paraparesis ; Sjogren's Syndrome* ; Urinary Retention

Neisseria meningitidis is an important cause of sporadic meningitis and fatal sepsis. Skin manifestations like urticaria, maculopapular eruption, and petechial rash occur in the majority of patients with meningococcal infections. However, meningococcal cellulitis is extremely rare and has not been reported in patients with systemic lupus erythematosus (SLE). SLE patients who have low complement level are more susceptible to meningococcal infections than healthy population with normal complement level. We report a case of cellulitis associated with meningococcal infection, which manifested as knee arthritis, skin erythema, and low grade fever mimiking SLE flare in a 54 year-old female patient who had long standing SLE and hypocomplementemia.
Arthritis ; Cellulitis* ; Complement System Proteins ; Erythema ; Exanthema ; Female ; Fever ; Humans ; Knee ; Lupus Erythematosus, Systemic* ; Meningitis ; Meningococcal Infections ; Middle Aged ; Neisseria meningitidis ; Sepsis ; Skin ; Skin Manifestations ; Urticaria

Neisseria meningitidis is an important cause of sporadic meningitis and fatal sepsis. Skin manifestations like urticaria, maculopapular eruption, and petechial rash occur in the majority of patients with meningococcal infections. However, meningococcal cellulitis is extremely rare and has not been reported in patients with systemic lupus erythematosus (SLE). SLE patients who have low complement level are more susceptible to meningococcal infections than healthy population with normal complement level. We report a case of cellulitis associated with meningococcal infection, which manifested as knee arthritis, skin erythema, and low grade fever mimiking SLE flare in a 54 year-old female patient who had long standing SLE and hypocomplementemia.
Arthritis ; Cellulitis* ; Complement System Proteins ; Erythema ; Exanthema ; Female ; Fever ; Humans ; Knee ; Lupus Erythematosus, Systemic* ; Meningitis ; Meningococcal Infections ; Middle Aged ; Neisseria meningitidis ; Sepsis ; Skin ; Skin Manifestations ; Urticaria

PURPOSE: Obesity is closely related to insulin resistance, compensatory hyperinsulinemia and dyslipidemia in adults. We identified the effect of obesity measured by BMI and insulin resistance on dyslipidemia in children and adolescents. METHODS: The fasting serum insulin, glucose, total cholesterol, triglyceride, HDL- and LDL-cholesterol were measured and insulin resistance(HOMA-IR) was calculated in 35 children with simple obesity(age : 10.6+/-2.8 years; male 20, female 15; BMI : 27.1+/-5.4 kg/m(2)). RESULTS: The hypertriglyceridemia(37%), hyperinsulinemia(54%) and HDL-hypocholesterolemia(5.7 %) were observed. HOMA-IR was well expressed by fasting insulin. As BMI increased, there was a statistically significant increase in insulin resistance and insulin level in both sexes. BMI was not related with lipid profile in both sexes. Triglyceride was correlated with only insulin level and insulin resistance index in boys. In girls, there was no correlation between triglyceride, HDL-cholesterol and insulin(insulin resistance). CONCLUSION: These results suggest that hypertriglyceridemia was dependent on insulin resistance in pre-adult males. Monitoring of insulin resistance and those risk factors known to become a part of insulin resistance syndrome should become part of routine medical care for obese children.
Adolescent ; Adult ; Child* ; Cholesterol ; Dyslipidemias* ; Fasting ; Female ; Glucose ; Humans ; Hyperinsulinism ; Hypertriglyceridemia ; Insulin Resistance ; Insulin* ; Male ; Obesity* ; Risk Factors ; Triglycerides

PURPOSE: Puberty is a rapid changing periods of body composition and endocrine system. Already leptin was known to be closely related to body composition and was known to change according to Tanner stage. Ghrelin, a natural GH secretagogue, was also known to be related with weight change, fat utilization, insulin secretion, and influenced by sex steroid. But the relationship between ghrelin and other parameter according to pubertal development is not established yet. METHODS: Cross-sectional study of 101 peripubertal children (54 males and 47 females) aged 8.5 to 17.0 years. We examined body composition (free fat mass, fat mass, % body fat) by bioelectric impendence measurements. Total ghrelin, leptin, insulin was measured by radioimmunoassay (RIA). Relationship between ghrelin, leptin, insulin and body composition data was analysed according to Tanner stage controlling gender using SPSS ver 11.0. RESULTS: Ghrelin concentration is only correlated with Tanner stage (r=-0.21, P<0.05) and GIR (r=0.29, P<0.01). When we divided subjects by sex, even though there was no difference of ghrelin level between both sex, only male is correlated with Tanner stage (r=-0.28, P<0.05) and GIR (r=0.36, P<0.01). There was no difference of ghrelin level between each Tanner stage and no correlation was observed with other body composition and endocrine parameter including leptin. CONCLUSION: These data suggest that Ghrelin levels decrease during puberty at least in boys as pubertal development proceeds but it is minimal. During puberty, ghrelin levels are stable. Ghrelin may have no relationship with other pubertal change. The relation of ghrelin and insulin sensitivity need to be further investigated.
Adolescent ; Body Composition ; Child ; Cross-Sectional Studies ; Endocrine System ; Ghrelin* ; Humans ; Insulin ; Insulin Resistance ; Leptin ; Male ; Puberty* ; Radioimmunoassay