No abstract available.
Hypoparathyroidism*

No abstract available.
Diabetes Mellitus* ; Insulin* ; Molecular Medicine*

No abstract available.
Hydrocortisone* ; Plasma*

No abstract available.
Molecular Biology* ; Transcription Factors*

An A to G mutation at nucleotide 3243 or 8344 of the mitochondrial genome has been associated with insulin dependent diabetes mellitus(IDDM) and noninsulin dependent diabetes mellitus(NIDDM) in some patients whose family members are frequently affected in maternally inherited fashion. The hypothesis is entertained that defective oxidative phosphorylation system(OXPHOS) caused by mitochondrial DNA mutations would hamper the insulin secretion from pancreas beta islet cells, which requires large amount of ATP energy. Recently, a number of study have been reported to examine the frequecy of these mutations in diabetic populations. In this study, efforts have been directed to investigate the frequency of MELAS tRNALeu(3243) and MERRF tRNALys(8344) mutations in 53 Korean IDDM patients. Total genomic DNA extracted from patients' lymphocytes have been amplified using two sets of mitochondrial specific primers to cover the regions of nt 3243 or 8344. PCR-RFLP anlaysis using Apa I for MELAS(3243) or Ban II for MERRF(8344) were utilized to screen the presence of these mutations in 53 IDDM patients. Two positive controls have been directly sequenced to confirm the presence of these mutations. The results showed that none of IDDM patients(0/53) screened carried these mutations. In conclusion, mitochondrial DNA mutations of MELAS(3243) or MERRF(8344) may be very rare causative factor in developing IDDM, though a large number of IDDM patients are needed to be screened.
Adenosine Triphosphate ; Diabetes Mellitus, Type 1* ; DNA ; DNA, Mitochondrial* ; Genome, Mitochondrial ; Humans ; Insulin ; Islets of Langerhans ; Lymphocytes ; Mass Screening* ; MELAS Syndrome* ; MERRF Syndrome* ; Oxidative Phosphorylation ; Pancreas ; RNA, Transfer*

No abstract available.

PURPOSE:Precocious puberty is the development of secondary sexual characteristics before the age of 8 years in girls and 9.5 years in boys. It is usually associated with premature, rapid skeletal maturation and closure of the epiphyseal plates, resulting in short stature compared with genetic height potential and can produce significant psychological distress for patients. We examined effects of treatment with long-acting gonadotropin-releasing hormone(GnRH) agonist on somatic and skeletal growth in patients with central precocious puberty(CPP). MATERIALS & METHODS:Two male and seven female patients were diagnosed as having central precocious puberty(CPP) on the basis of onset age of secondary sexual characteristics, bone age, results of GnRH stimulation test and levels of sex hormones. They were treated with Triptorelin or Leuprorelin acetate(80-100ug/kg, IM every 4 weeks) for 1 year. The patients have been analyzed in terms of changes in auxological parameters including height velocity(HV), HV SDS CA, height SDS CA, height SDS BA and predicted adult height(PAH) SDS before and 1 year after treatment with GnRH agonist. RESULTS:The growth velocity a year after treatment was decreased to 4.1+/-0.9 from 7.5+/-1.2cm/year(P<0.01) and the height velocity standard deviation score(SDS) for chronologic age decreased to -1.6+/-0.4 from 2.8+/-0.8(P<0.01). The height SDS for chronologic age was increased to 2.0+/-0.7 from 3.8+/-1.0 a year after treatment (P<0.01). However, no significant difference were observed in height SDS for bone age(-1.9+/-0.2 from -2.1+/-0.3)(p>0.05) and predicted adult height SDS(-2.2+/-0.5 from -2.3+/-0.4)(p>0.05) one year after treatment. CONCLUSION: We observed a remarkable growth deceleration a year after treatment with GnRH agonist in CPP patients. However, the results of this study shows no benefit of GnRH agonist treatment in improving predicted adult height. It is still not clear whether GnRH agonist treatment will eventually help the patients with CPP achieve a final adult height within the range of their genetic target height or not. Further extensive long-term study using strict selection criteria for GnRH agonist treatment is required to address this issue.
Adolescent ; Adult ; Age of Onset ; Deceleration ; Female ; Gonadal Steroid Hormones ; Gonadotropin-Releasing Hormone* ; Growth Plate ; Humans ; Leuprolide ; Male ; Patient Selection ; Puberty ; Puberty, Precocious* ; Triptorelin Pamoate

No abstract available.
Plasma* ; Reye Syndrome*

PURPOSE:Craniopharyngioma is one of the most important intracranial tumors in pediatric age which causes multiple pituitary hormone deficiencies. We have studied clinical characteristics in patients with craniopharyngioma, focusing on changes of endocrine function, change of growth pattern, and change of body mass index before and after surgery. In addition, we wanted to know the effect of growth hormone therapy on growth velocity and body mass index, and to identify contributing factors for spontaneous growth in spite of growth hormone deficiency. METHODS:A total number of 17 patients who were diagnosed as having craniopharyngioma at the Department of Pediatrics, Asan Medical Center during the period of January, 1991 to December, 1997 were included in this study. Retrospectively, we reviwed medical records as to their endocrine function tests and auxoloogical data before, after surgery. RESULTS: 1)The number of patients of male and female were 9 and 8 respectively. Mean age was 7.3+/-4.3 years. 2)Preoperatively, 3 patients were thyroid hormone deficient, 3 patients were corticosteroid deficient, and 3 patients suffered from diabetes inspidus. 3)Postoperatively, GH and TSH deficiency were found in 100%, ACTH in 88.2%, ADH in 82.3%, and LH/FSH in 60%, 53% respectively. 4) Mean growth velocity in 14 GH deficient patients without growth hormone treatment for 2 years were 3.5+/-2.4cm/year during the 1st year and 3.1+/-1.7cm/year during the 2nd year. Although height standard deviation score(Ht. SDS) was decreasing trend as -0.24+/-2.19 at diagnosis, -0.94+/-1.73 at 1 year later, and -0.76+/-1.76 at 2 years later, it was not statistically significant. To our suprise, 4 out of 14 patients achieved greater growth velocity than nomal in spite of growth hormone deficiency during the 1st year after operation. 5) Although the mean body mass index of 14 GH deficient patients without GH treatment was increasing trend as 17.9+/-3.5 at diagnosis, 19.0+/-4.5 at 1 year later, and 19.9+/-4.8 at 2 year later, it was not statistically significant. 6) The mean Ht. SDS increased significantly in 7 patients treated with growth hormone(P<0.05) for 2 years, but change of body mass index was not significant. 7) Comparision of postoperative serum prolactin levels and changes of body mass index between spontaneous growth and stunted growth group did not reveal significant difference. CONCLUSION: Since most patients with craniopharyngioma become multiple pituitary hormone deficient after operation, it is important to predict and detect pituitary dysfunction to manage it effectively. Although patients with postoperative GH deficiency responded well to GH treatment, further study is needed to clarify what are the main contributing or prognostic factors for spontaneous growth without growth hormone treatment.
Adrenocorticotropic Hormone ; Body Mass Index ; Chungcheongnam-do ; Craniopharyngioma* ; Diagnosis ; Female ; Growth Hormone ; Humans ; Male ; Medical Records ; Obesity ; Pediatrics ; Prolactin ; Retrospective Studies ; Thyroid Gland

Purpose : As the recombinant human growth hormone has been widely available, a lots of parents having short statured children are interested in promoting growth of them whatever the etiologies of short stature they have. However, the growth hormone therapy for growth-promoting effect is only justified in well-established indications such as growth hormone deficiency, fumer syndrome, and chronic renal insufficiency. This study was undertaken to classify the children with chief complaint of short stature by its cause and giving the basic epidemiologic data for it so that the size of population in which growth hormone is indicated can be estimated. Methods : According to Ranke's etiologic classification, we categorized the 579 children who visited our pediatric endocrinology clinic with chief complaint of short stature during the period of March 1994 to August 1996. In this prospective study, history regarding growth was taken, physical examination and laboratory tests including bone age, thyroid function, blood chemistry were carried out. The auxological data were analyzed. Additional chromosomal study or growth hormone provocative tests were performed when needed. Results : Out of 579 patients, 360(62.2%) were classified as normal and 127(21.9%) were classified as normal variants which consist of familial [74(12.8%)], constitutional [48(8.5%)], and mixed familial & constitutional short stature[5(0.9%)]. Pathologic short stature was found in only 80(13.8%). Those are growth hormone deficiency(28), Tumer syndrome(16), intrauterine growth retardation(14) in order. Other etiologies list varieties of dysmorphism, skeletal dysplasia, chromosomal disorders. Conclusions : This results suggest the vast majority of children with chief complaint of short stature are normal or normal variants. Only 7.8% of children who visited our clinic were indicated for growth hormone therapy.
Chemistry ; Child* ; Chromosome Disorders ; Classification* ; Endocrinology ; Growth Hormone ; Human Growth Hormone ; Humans ; Parents ; Physical Examination ; Prospective Studies ; Renal Insufficiency, Chronic ; Thyroid Gland