No abstract available.
Trophoblastic Neoplasms* ; Trophoblasts*

PURPOSE:Mutations of the PROP1 (Prophet of PIT1) gene lead to combined pituitary hormone deficiency (CPHD), which is characterized by a deficiency of growth hormone (GH), thyroid stimulating hormone (TSH), luteinizing hormone (LH)/follicle stimulating hormone (FSH), prolactin (PRL) and adrenocorticotropic hormone (ACTH). We studied the PROP1 mutations in Korean children with hypopituitarism. METHODS:Twelve patients with congenital hypopituitarism were recruited from the Ajou University Hospital, Korea. The pituitary phenotype ranged from isolated growth hormone deficiency (IGHD) to CPHD. Clinical data, including endocrine and neuroradiological data were obtained from the medical records, and the DNA was collected and screened for mutations within PROP1 using polymerase chain reaction (PCR). The PCR products were sequenced directly. RESULTS:Nine patients had abnormal pituitary gland and three patients showed normal pituitary gland in magnetic resonance imaging (MRI). Endocrinologically, seven patients had IGHD and five had CPHD. Three of CPHD had GHD and hypogonadotrophic hypogonadism and the other two had GHD, central hypothyroidism, and ACTH deficiency. We identified no mutations in the PROP1 gene. However, three known polymorphisms were identified: The 27T->C (Ala9Ala) and the associated IVS1+3A->G in exon 1 were found in 25% of patient alleles sequenced. The 424G->A (Ala142Thr) in exon 3 change was identified in 4% of those sequenced. CONCLUSION:This study corresponds to the previous reports that PROP1 mutations are rare in sporadic cases of CPHD. The low mutation frequency in Korean patients may be due to ethnic-specificity or other candidate genes causing this disease. It is necessary to study PROP1 gene and the involved genes in more Korean patients.
Adrenocorticotropic Hormone ; Alleles ; Child ; DNA ; Exons ; Growth Hormone ; Humans ; Hypogonadism ; Hypopituitarism ; Hypothyroidism ; Korea ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Medical Records ; Mutation Rate ; Phenotype ; Pituitary Gland ; Polymerase Chain Reaction ; Prolactin ; Thyrotropin

PURPOSE: The incidence of type 1 diabetes mellitus is increasing worldwide, the complications such as growth impairment, thyroid dysfunction, microvascular disease can be accompanied in type 1 diabetes mellitus. So, it is important to study initial clinical characteristics of type 1 diabetes mellitus for long-term managements of such complications. METHODS: Forty children and adolescents with type 1 diabetes mellitus who had been followed up in the department of pediatrics, Ajou university hospital from December 1997 to June 2006 were enrolled in this study. Sex, Onset Age, onset season, body mass index, family history, serum blood glucose, urine ketone, admission duration, HbA1c, serum and 24 hours urine C-peptide, islet cell antibody, daily insulin dose, insulin like growth factor-I, thyroid function test, lipid profile, bone age of patients were reviewed retrospectively. RESULTS: Forty patients were enrolled:19 boys and 21 girls. The mean age of diagnosis was 9.2+/-3.5 years. There were no seasonal variation in onset time. The body mass index were 15.9+/-2.7 kg/m2. In family history, 1 patient had type 1 and 16 patients (40%) had type 2 diabetes. Blood glucose was 509+/-172 mg/dL. Urine ketone was positive in 77.8% of patients. HbA1c was 12.5+/-2.4%. Serum C-peptide was 0.76+/-0.65 ng/mL and there was statistically significant positive relationship between patient's age and serum C-peptide level (P<0.05). Daily insulin dose was 0.89+/-0.28 unit/kg/day and negatively correlated with serum C-peptide level (P<0.05), but there were no differences in daily insulin dose between prepubertal and pubertal patients. In 82.4% of patients, at least one of 3 antibody (glutamic acid decarboxylase antibody, islet cell antibody, insulin auto antibody) were detected. Serum C-peptide were higher in GAD Ab positive group than in negative group (P<0.05). In 63.6% of patients, Insulin like growth factor-I levels were lower than normal control group. Thyroid dysfunction were found in 7.5% of patients. 48.7% of patients were accompanied with dyslipidemia. In 51.9% of patients, bone age was advanced by at least 6 months. CONCLUSION: For the most part, our results were consistent with previous studies. But some points like, 1) there was no seasonal variation in onset time, 2) serum C-peptide was not low, 3) there were no differences in daily insulin dose between prepubertal and pubertal patients, differed from previous studies.
Adolescent ; Age of Onset ; Blood Glucose ; Body Mass Index ; C-Peptide ; Child ; Diabetes Mellitus, Type 1* ; Diagnosis* ; Dyslipidemias ; Female ; Humans ; Incidence ; Insulin ; Islets of Langerhans ; Pediatrics ; Retrospective Studies ; Seasons ; Thyroid Function Tests ; Thyroid Gland

PURPOSE: IGF-I and IGFBP-3 are usually used for the diagnosis of growth hormone deficiency. Until now we don't have normal values of IGF-I and IGFBP-3 in Korea. Therefore, we evaluated the normal values of IGF-I and IGFBP-3 in healthy Korean children and adolescents and compared with reference values in Pediatric endocrinology which was published by the Korean society of pediatric endocrinology. We also evaluated correlation of age, sex, height, body mass index and bone age with serum IGF-I and IGFBP-3 levels. METHODS: We studied the serum levels of IGF-I and IGFBP-3 in 919 and 686 normal healthy children and adolescents respectively who were examined for the growth evaluation at the department of Pediatrics, Ajou University Hospital from January, 1994 to September, 2004. IGF-I and IGFBP-3 were measured using IRMA method. RESULTS: Serum levels of IGF-I and IGFBP-3 were increased with age from early childhood into adolescence. Serum IGF-I correlated significantly with age, sex and BMI but serum IGFBP-3 correlated significantly only with age and sex (P<0.05). IGF-I levels in our data were lower than reference values in Pediatric Endocrinology during early childhood but were higher in puberty. IGFBP-3 levels were higher throughout childhood and puberty. CONCLUSION: There was a significant difference between our data and reference values which were evaluated in U.S.A., so longitudinal antegrade large scale study must be undertaken to obtain the normal reference range of healthy Korean children and adolescents. We believe that one should take into account the patient's age, sex, height, BMI and bone age when measuring serum IGF-I and IGFBP-3 levels for the evaluation of short stature.
Adolescent* ; Body Height* ; Child* ; Diagnosis ; Endocrinology ; Growth Hormone ; Humans ; Insulin-Like Growth Factor Binding Protein 3* ; Insulin-Like Growth Factor I* ; Korea ; Pediatrics ; Puberty ; Reference Values*

A mass excision surrounding the bronchial stump was performed to exclude malignancy in a 42-year-old man who had undergone a right lower lobectomy for lung cancer. The mass was identified as a cicatricial fibroma. Cicatricial fibromatosis, which is desmoid fibromatosis that arises in a surgical scar, is a well-known clinical condition. It consists of histologically benign neoplasms. Their occurrence after thoracic surgery is extremely rare. Biopsy or excision of suspicious lesions is very important for diagnosis. R0 resection remains the principal outcome for intra-thoracic desmoid fibromatosis. We report that a cicatricial fibromatosis in the subcarinal space was removed after suspicion of local recurrence at the bronchial stump follwing lobectomy for lung cancer.
Adult ; Biopsy ; Cicatrix ; Diagnosis* ; Fibroma* ; Fibromatosis, Aggressive ; Humans ; Lung Neoplasms* ; Lung* ; Recurrence* ; Thoracic Surgery

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.
Adult ; Breast ; Gonadotropin-Releasing Hormone ; Humans ; Insulin ; Insulin-Like Growth Factor I ; Logistic Models ; Luteinizing Hormone ; Puberty ; Puberty, Precocious

Eleven types of nuts and seeds were analyzed to determine their energy (326-733 mg), moisture (1.6-18.3 mg), carbohydrate (8.8-70.9 mg), protein (4.9-30.5 mg), lipid (2.5-69.8 mg), and ash (1.2-5.5 mg) contents per 100 g of sample. Energy content was highest in pine nuts (733 mg/100 g), carbohydrate level was highest in dried figs (70.9 mg/100 g) and protein was highest in peanuts (30.5 mg/100 g). The amino acid compositions of nuts and seeds were characterized by the dominance of hydrophobic (range = 1,348.6-10,284.6 mg), hydrophilic (range = 341.1-3,244.3 mg), acidic (range = 956.1-8,426.5 mg), and basic (range = 408.6-4,738.5 mg) amino acids. Monounsaturated fatty acids (MUFA) were highest in macadamia nuts (81.3%), whereas polyunsaturated fatty acids (PUFA) were highest in the walnuts (76.7%). Macadamia nuts did not contain any vitamin E, whereas sunflower seeds contained the highest level (60.3 mg/kg). Iron (Fe) content was highest in pumpkin seeds (95.85 +/- 33.01 ppm), zinc (Zn) content was highest in pistachios (67.24 +/- 30.25 ppm), copper (Cu) content was greatest in walnuts (25.45 +/- 21.51 ppm), and lead (Pb) content was greatest in wheat nuts (25.49 +/- 4.64 ppm), significantly (P < 0.05). In conclusion, current commercial nuts and seeds have no safety concerns, although further analysis of Pb contents is necessary to ensure safety.
Amino Acids ; Arachis ; Copper ; Cucurbita ; Dietary Sucrose ; Fatty Acids, Monounsaturated ; Fatty Acids, Unsaturated ; Helianthus ; Iron ; Juglans ; Korea ; Macadamia ; Nuts ; Pistacia ; Seeds ; Triticum ; Vitamins ; Zinc

PURPOSE: Gonadotropin releasing hormone agonist (GnRH agonist) and growth hormone (GH) treatment is widely used in precocious puberty to delay rapid pubertal growth. We studied the effect of these agents on the predicted adult height in girls with precocious puberty. METHODS: 41 girls were selected who had precocious sexual development, with onset from 5 to 8 years of age. They were divided into two groups. Twenty four girls treated only with GnRH agonist were compared with 17 girls treated with GnRH agonist and GH. We analyzed chronologic age (CA), bone age (BA) and predicted adult height (PAH) during and after treatment. RESULTS: Before treatment, there were no differences for CA, BA, body mass index (BMI), PAH and Tanner stage between two groups. After treatment, the PAH [153.7+/-6.85 cm (-1.31+/-1.25 SDS) vs 158.8+/-5.82 cm (-0.30+/-1.24 SDS)] for both age groups were significantly increased. The difference between initial PAH and follow up PAH was significantly increased when the initial PAH was low (P=0.015), and the duration of treatment was long (P=0.009). CONCLUSION: In girls with precocious puberty, GnRH agonist delayed bone maturation and increased PAH. PAH increased more when GnRH agonist and GH treatment was used as opposed to GnRH agonist treatment alone. GH combination therapy should be considered if the initial PAH was very low.
Adult* ; Body Mass Index ; Female* ; Follow-Up Studies ; Gonadotropin-Releasing Hormone* ; Gonadotropins* ; Growth Hormone* ; Humans ; Puberty, Precocious* ; Sexual Development

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

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