The prevalence of obesity is rapidly growing throughout the developing and developed world. Given the seriousness of obesity, it critically needs to develop new therapeutic ways to defend against its growth. Persistent increase in food intake is a primary cause of the energy imbalance. The arcuate nucleus of the hypothalamus is a key region to integrate signals originating from various regions in periphery and leptin resistance in the central nervous system (CNS) contributes to the impaired regulation of food intake. It has been endeavor to treat obesity by understanding the mechanisms of CNS regulation of food intake. Adipose tissue has been regarded as a tumor because of its reversible expansibility and dependency on vasculature. There has been a challenge to starve adipose tissue by inhibiting adipose tissue vasculature. A peptide to cause apoptosis of endothelium only in white adipose tissue greatly loses body weight by reducing food intake independent of the action of leptin. This study provides convincing evidence for a previously unknown relationship between the status of adipose tissue vasculature and the regulation of food intake that may provide a novel way for decreasing body fat. However, the mechanism by which the inhibition of angiogenesis in white adipose tissue decreases food intake and body weight remains unclear. In this review, we describe the potential mechanisms of regulation of food intake induced by inhibition of angiogenesis in white adipose tissue.
Adipose Tissue ; Adipose Tissue, White ; Apoptosis ; Arcuate Nucleus ; Body Weight ; Central Nervous System ; Dependency (Psychology) ; Eating ; Endothelium ; Hypothalamus ; Leptin ; Obesity ; Prevalence

Bone age is important to evaluate growth status and remaining growth. The Greulich and Pyle atlas is widely used and is so far the most common assessment method of bone age. However, this technique has some limitations, especially during puberty : (1) 11.5 and 12.5 years of bone age in girls and 14.5 years of bone age in boys are not represented in the atlas ; (2) Hand and wrist radiographs are difficult to assess between 11 and 13 years of bone age in girls and between 13 and 15 years of bone age in boys. Sauvegrain et al. developed a method to assess bone age by using elbow radiographs(AP& lateral projections) during pubertal age. Between 11 and 13 years of bone age in girls and between 13 and 15 years of bone age in boys, the olecranon apophysis is characterized by clear morphological development. This method is a reliable tool to assess bone age during puberty because significant morphological changes in the elbow happened every six months.
Elbow ; Hand ; Olecranon Process ; Puberty ; Wrist

Kallmann syndrome (KS) is characterized by the association of hypogonadotropic hypogonadism and anosmia. Although the vast majority of KS cases are sporadic, some X-linked recessive (KAL1), autosomal dominant (FGFR1), and autosomal recessive (most commonly GNRHR) modes of inheritance have been described. Two boys were referred to our department because of cryptorchidism and the absence of puberty. Upon laboratory evaluation they were diagnosed with hypogonadotropic hypogonadism. Agenesis of the olfactory bulbs was detected in radiologic tests, and total deletion of the KAL1 gene was detected through multiplex ligation-dependent probe amplification (MLPA). Although cryptorchidism was diagnosed in the siblings, only the older brother suffered from sensorineural hearing loss and right renal agenesis, a feature that had been reported in X-linked KS. We describe herein the clinical heterogeneity of two affected brothers who carry a complete deletion in KAL1; this is the first case of familial Kallmann syndrome due to the complete deletion of the KAL1 gene reported in Korea.
Congenital Abnormalities ; Cryptorchidism ; Hearing Loss, Sensorineural ; Humans ; Hypogonadism ; Kallmann Syndrome ; Kidney ; Kidney Diseases ; Korea ; Male ; Multiplex Polymerase Chain Reaction ; Olfaction Disorders ; Olfactory Bulb ; Population Characteristics ; Puberty ; Siblings ; Wills

We report a case of Turner syndrome associated with idiopathic central diabetes insipidus in a 12-year-old girl, who presented with polyuria and polydipsia after a year. The patient was very short and and centrally obese, and was initially diagnosed with Turner syndrome, hyperlipidema, and diabetes mellitus. A water deprivation test revealed central diabetes insipidus, and sellar magnetic resonance imaging (MRI) showed a thickening of the pituitary stalk, with normal high signal intensity in the posterior pituitary gland. Replacement therapy with desmopressin was initiated, and follow-up sellar MRI findings after two years showed spontaneous regression of the thickened pituitary stalk. There are only few reports of concomitant Turner syndrome with central diabetes insipidus worldwide. Further observation is needed in order to disclose the cause of central diabetes insipidus in patients having Turner syndrome.
Child ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic ; Diabetes Mellitus ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Pituitary Gland ; Pituitary Gland, Posterior ; Polydipsia ; Polyuria ; Turner Syndrome ; Water Deprivation

Kallmann syndrome (KS) is characterized by the association of hypogonadotropic hypogonadism and anosmia. Although the vast majority of KS cases are sporadic, some X-linked recessive (KAL1), autosomal dominant (FGFR1), and autosomal recessive (most commonly GNRHR) modes of inheritance have been described. Two boys were referred to our department because of cryptorchidism and the absence of puberty. Upon laboratory evaluation they were diagnosed with hypogonadotropic hypogonadism. Agenesis of the olfactory bulbs was detected in radiologic tests, and total deletion of the KAL1 gene was detected through multiplex ligation-dependent probe amplification (MLPA). Although cryptorchidism was diagnosed in the siblings, only the older brother suffered from sensorineural hearing loss and right renal agenesis, a feature that had been reported in X-linked KS. We describe herein the clinical heterogeneity of two affected brothers who carry a complete deletion in KAL1; this is the first case of familial Kallmann syndrome due to the complete deletion of the KAL1 gene reported in Korea.
Congenital Abnormalities ; Cryptorchidism ; Hearing Loss, Sensorineural ; Humans ; Hypogonadism ; Kallmann Syndrome ; Kidney ; Kidney Diseases ; Korea ; Male ; Multiplex Polymerase Chain Reaction ; Olfaction Disorders ; Olfactory Bulb ; Population Characteristics ; Puberty ; Siblings ; Wills

Hypercalcemia is not common, and occurs more frequently in children than in adults. Left untreated, hypercalcemia could result in a profound impact on growth and development. We report a case of recurrent idiopathic infantile hypercalcemia with poor weight gain, constipation, and a renal stone. We successfully treated the infantile hypercalcemia with a low-calcium diet and intravenous pamidronate.
Adult ; Child ; Constipation ; Diet ; Diphosphonates ; Growth and Development ; Humans ; Hypercalcemia ; Infant ; Kidney Calculi ; Weight Gain

We report a case of Turner syndrome associated with idiopathic central diabetes insipidus in a 12-year-old girl, who presented with polyuria and polydipsia after a year. The patient was very short and and centrally obese, and was initially diagnosed with Turner syndrome, hyperlipidema, and diabetes mellitus. A water deprivation test revealed central diabetes insipidus, and sellar magnetic resonance imaging (MRI) showed a thickening of the pituitary stalk, with normal high signal intensity in the posterior pituitary gland. Replacement therapy with desmopressin was initiated, and follow-up sellar MRI findings after two years showed spontaneous regression of the thickened pituitary stalk. There are only few reports of concomitant Turner syndrome with central diabetes insipidus worldwide. Further observation is needed in order to disclose the cause of central diabetes insipidus in patients having Turner syndrome.
Child ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic ; Diabetes Mellitus ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Pituitary Gland ; Pituitary Gland, Posterior ; Polydipsia ; Polyuria ; Turner Syndrome ; Water Deprivation

Ketosis-prone diabetes includes heterogeneous disease groups characterized by provoked or unprovoked ketoacidosis (ketosis), with a typical phenotype of autoimmune type 1 diabetes. As the incidence of obesity and type 2 diabetes is universally increasing, the rate of type 2 diabetes in diabetic ketoacidosis in children and adolescents is exptected to increase rapidly. The clinical presentation of atypical ketoacidosis with type 2 diabetes has been reported mostly in adults. We recently experienced a case of a 10-year-old obese girl with new-onset type 2 diabetes who initially presented with severe diabetic ketoacidosis, and introduce it with literature reviews.
Adolescent ; Adult ; Child ; Diabetes Mellitus, Type 2 ; Diabetic Ketoacidosis ; Humans ; Incidence ; Ketosis ; Obesity ; Phenotype

Hypercalcemia is not common, and occurs more frequently in children than in adults. Left untreated, hypercalcemia could result in a profound impact on growth and development. We report a case of recurrent idiopathic infantile hypercalcemia with poor weight gain, constipation, and a renal stone. We successfully treated the infantile hypercalcemia with a low-calcium diet and intravenous pamidronate.
Adult ; Child ; Constipation ; Diet ; Diphosphonates ; Growth and Development ; Humans ; Hypercalcemia ; Infant ; Kidney Calculi ; Weight Gain

PURPOSE: Central precocious puberty (CPP) is defined as any sign of secondary sexual maturation appears at an age lower than two standard deviations of the mean for the average age. This process is driven by activation of hypothalamic gonadotropin releasing hormone (GnRH) secretion. Many genes expressed in the hypothalamus have been identified to play an important role in the onset and the progression of puberty. In this study, the GNRH1, its receptor (GNRHR), and kisspeptin receptor (GPR54) genes were scanned to investigate sequence alterations and their distribution in Korean girls with CPP. METHODS: One hundred and one Korean girls with CPP were recruited as the case group and 51 normal Korean women as the control group. The DNAs were extracted and amplified by polymerase chain reaction (PCR), and the products were sequenced directly. Statistical analyses were performed, and P values of < 0.05 were considered significant. RESULTS: Four polymorphisms were identified; however, no pathological mutation was found. Two of the polymorphisms were previously reported, c.47G > C in GNRH1, and c.1091T > A in GPR54. However, the other two (c.196C > T in GNRH1 and c.546T > C in GNRHR) were novel. There was no polymorphism that was significantly associated with early onset or rapid progression of puberty. CONCLUSION: Although the size of our study population was relatively small, simple genetic variations in GNRH1, GNRHR, and GPR54 genes are not likely to be a substantial factor directly associated with the onset and progression of puberty.
DNA ; Female ; Genetic Variation ; Gonadotropin-Releasing Hormone ; Humans ; Hypothalamus ; Piperazines ; Polymerase Chain Reaction ; Puberty ; Puberty, Precocious ; Sexual Maturation