No abstract available.
Parturition* ; Seasons*

PURPOSE:Differential diagnosis of sexual precocity has been aided by new imaging techniques and endocrine evaluation. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS:A total 95 children(87 girls and 8 boys) who were diagnosed with sexual precocity at St. Mary's Hospital, Catholic University Medical College from 1984 to 1996, were studied. All underwent standard anthropometric measures. They were assinged to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: 1) The most common cause of sexual precocity was premature thelarche, 50 cases(52.6%) of all children. 2) Central precocious puberty was noted in 7 girls and 3 boys(female to male ratio 2.3:1). The causes were not identified in 6 cases(85.7%) in girls, but the causes were not found in 1 cases(33.3%) in boys. The idiopathic central precocious puberty occurs at least two fold more frequently in girls than in boys. 3) Incomplete precocious puberty was noted in 29 girls and 5 boys(female to male ratio 5.8:1). In the causes of 29 girls with incomplete precocious puberty, congenital adrenal hyperplasia were found in 23 cases, autonomous functional ovarian cyst in 5 cases and McCune-Albright syndrome in 1 case. In the causes of 5 boys with incomplete precocious puberty, virilizing adrenal cortical tumor were found in 3 cases, congenital adrenal hyperplasia in 1 case, and hepatoblastoma in 1 case. CONCLUSION: Sexual precocity occurs at least ten fold more frequently in girls than in boys. Premature thelarche is the most common form of sexual precocity.
Adrenal Hyperplasia, Congenital ; Child ; Diagnosis ; Female ; Fibrous Dysplasia, Polyostotic ; Hepatoblastoma ; Humans ; Incidence* ; Male ; Ovarian Cysts ; Puberty, Precocious ; Ultrasonography

Prader-Willi(PW)syndrome is characterized by obesity, hypotonia, mental retardation, hypogonadism, short stature, excessive eating and characteristic facial appearance. Diabetes mellitus has been considered a component of PW syndrome. Recently this syndrome is caused by the absence of paternally derived genes normally located on chromosome segment 15 q11-q13 or may be the result of maternal uniparental disomy with the absence of paternally derived 15 q11-q13 region. The developement of probes containing segments of DNA from chromosome region 15 q11-q13 provides the oppotunity to confirm the diagnosis of PW syndrome by fluorescence in situ hybridization(FISH). We experienced a 15-year-old boy of PW syndrome with diabetes mellitus, who revealed mental retardation, hypogonadism, obesity and microdeletion of chromosome 15 q11-q13 comfirmed by FISH.
Adolescent ; Chromosomes, Human, Pair 15* ; Diabetes Mellitus ; Diagnosis ; DNA ; Eating ; Fluorescence ; Humans ; Hypogonadism ; Intellectual Disability ; Male ; Muscle Hypotonia ; Obesity ; Prader-Willi Syndrome* ; Uniparental Disomy

We present a case of lymphangioma circumscriptum in a 17 year-old girl according to tihe clinical and histopathological findings. This case is unusual in that lesions developed on the vulva and thigh without preceding lymphedema. Our patient had a plaque of grouped vesicle-like papules resembling frog's apawn on The both labia majora of vulva and several scattered, skin tag like soft papules on the right upper thigh of theree years duration. Histopathologic findings showed variable sized, dilatated lymphatic channels lined by single layer of normal endothelial cells confined to the only upper dermis.
Adolescent ; Dermis ; Endothelial Cells ; Female ; Humans ; Lymphangioma* ; Lymphedema ; Skin ; Thigh ; Vulva*

Small ovarian cysts(<0.7 cm in diameter) are common in prepubertal children. Occasionally larger cysts occur in young girls with isosexual incomplete precocious puberty in the absence of LH and FSH elevation. The mechanism of production of these autonomous functioning cysts is unknown. A 3 year 6 month-old girl was referred to our department of pediatrics because of severe vaginal bleeding. On examination, she was tall and had breast enlargement of Tanner stage II. On hormonal study, FSH(0.38mIU/ml) was suppressed and estradiol(62.2pg/ml) was markedly elevated, and GnRH stimulation failed to evoke an increase in gonadotropin. Bone age was advanced. Pelvic ultrasonography showed both huge ovarian cysts. Brain MRI showed no abnormalities. We underwent both ovarian cystectomy and histological examination showed follicular cyst in left ovary and luteal cyst in right ovary. After ovarian cystectomy vaginal bleeding stopped and hormonal abnormalities were normalized. We report a case of autonomous ovarian cysts presenting severe vaginal bleeding treated with ovarian cystectomy.
Brain ; Breast ; Child ; Cystectomy ; Female ; Follicular Cyst ; Gonadotropin-Releasing Hormone ; Gonadotropins ; Humans ; Infant ; Magnetic Resonance Imaging ; Ovarian Cysts* ; Ovary ; Pediatrics ; Puberty, Precocious ; Ultrasonography ; Uterine Hemorrhage*

Adrenal cortical carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as virilization, Cushing syndrome, hyperaldosteronism or feminization. We experienced a case of adrenal cortical carcinoma in a 7yr old boy who showed features of virilization such as rapid growth rate, penile enlargement, and pubic hair. This case was diagnosed with typical hormonal findings and abdominal MRI and confirmed by pathologic findings. He was successfully treated by total left adrenalectomy and has been followed up without problem over eight months.
Adrenalectomy ; Adrenocortical Carcinoma* ; Adult ; Child ; Cushing Syndrome ; Feminization ; Hair ; Humans ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Rare Diseases ; Virilism

The thyroid gland is highly sensitive to the carcinogenic effects of ionizing radiation. The development of benign thyroid nodules, thyroid carcinoma and thyroid dysfunction following radiotherapy during childhood has been documented. Recently we experienced two cases of thyroid tumor after irradiation. One is a 14-year-old boy with thyroid follicular carcinoma who had received mantle irradiation(3,000cGy) eight years previously for Hodgkin' disease. The other is a 15-year- old boy with thyroid follicular adenoma who had received craniospinal irradiation (3,600cGy) seven years previously for CNS relapse of acute lymphoblastic leukemia. We recommend frequent sonographic evaluation and early suppression of thyroid stimulation in an attempt to arrest the development of neoplastic changes in long term survivors of childhood malignancies who received radiation therapy.
Adenoma ; Adolescent ; Craniospinal Irradiation ; Humans ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Radiation, Ionizing ; Radiotherapy ; Recurrence ; Survivors ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroid Nodule ; Ultrasonography

Recognition of transient forms of neonatal hypothyroidism is very important to prevent the complications of congenital hypothyroidism. Transplacental passage of TSH-binding inhibitory immunoglobulins(TBII) may result in transient congenital hypothyroidism. Transient neonatal hypothyroidism was found in a daughter of 25-yr-old mother who was receiving levothyroxine for primary hypothyroidism due to Hashimoto's thyroiditis. The neonate was treated with thyroxine which was discontinued at 24 months of age. Thyroid scanning during the neonatal period failed to identify functional thyroid tissue, suggesting thyroid agenesis, whereas thyroid scan performed on subsequent follow-up revealed a normal gland. Sequential measurements of serum autoantibodies directed towards the TSH receptor were made in the patient and her mother. High titers of blocking antibodies were present in the mother(TBII, 82.1%) and newborn(TBII, 85.5%) at 19 days after birth. The levels remained persistently high in the mother, whereas they declined and undetectable in the patient at 23 months of age. The above laboratory and clinical data were compatible with blocking nature of TBII, resulting in transient neonatal hypothyroidism and an athyreotic appearance on scan. The TBII measurement can be a useful predictor of neonatal hypothyroidism as well as confirming the nature of the disease in newborn.
Antibodies, Blocking ; Autoantibodies ; Congenital Hypothyroidism ; Follow-Up Studies ; Humans ; Hypothyroidism* ; Immunoglobulins* ; Infant, Newborn ; Mothers ; Nuclear Family ; Parturition ; Receptors, Thyrotropin ; Thyroid Dysgenesis ; Thyroid Gland ; Thyroiditis ; Thyroxine

PURPOSE:Neopterin is a marker of activation of the T-lymphocyte/monocyte axis. We measured serum neopterin concentration to investigate whether serum neopterin levels are increased in children with Graves' disease and whether serum neopterin measurement can be used as a marker of disease activity in Graves disease. METHODS:Twenty children with Graves' disease(3 boys and 17 girls) and 15 healthy children(7 boys and 8 girls) are enrolled in this study. Serum neopterin concentrations are measured by radioimmunoassay. RESULTS:Neopterin concentration in children with Graves' disease(1.59+/-1.25ng/ml) is not higher than that of healthy children(1.51+/-0.73ng/ml). Neopterin concentration is not influenced by thyroid function and remission state. CONCLUSION: Serum neopterin level in children with Graves' disease can not be used as a marker of activity.
Axis, Cervical Vertebra ; Child* ; Graves Disease* ; Humans ; Neopterin* ; Radioimmunoassay ; Thyroid Gland

PURPOSE:Hypochondroplasia is a skeletal dysplasia characterized by poor childhood growth and an inadequate pubertal growth spurt. Final height attainment of hypochondroplasia has been reported to range between 120 and 152cm. Increased availability of growth hormone with the introduction of recombinant human growth hormone has allowed for clinical trials in a number of growth hormone sufficient children with growth problems. The purpose of this study was to assess the growth promoting effect of human growth hormone in children with hypochondroplasia. METHODS:Five patients with hypochondroplasia diagnosed by clinical and radiological findings between 1993 and 1997 at our hospital was aged 3 and 1/2 -11 and 1/2 years. Each patients continuously received human growth hormone 0.6-0.7U/Kg/week, intramuscularly or subcutaneously in 6-7 divided dose for 2 years. Standard auxologic assessment was carried out every 3 month interval in the first year after commencement of therapy and then same assessment was 6 monthly. Bone age was assessed 6 monthly using Gleurich-Pyle method. RESULTS:Mean height velocity of pretreatment and year 1 and 2 of GH treatment were 3.9+/-0.7, 6.5+/-1.8 and 5.7+/-1.5cm/year, respectively. Mean height standard deviation score for chronological age of pretreatment and year 1 and 2 of GH treatment were -2.7+/-0.3, -2.4+/-0.3 and -2.2+/-0.4, respectively. The increase in the height velocity diminishes over the subsequent year. The increment of bone age after GH treatment were same as the increments of chronological age. CONCLUSION: Short-term GH therapy increases the height velocity of children with hypochondroplasia, but the effect of GH therapy on final height remains unknown.
Child* ; Growth Hormone* ; Human Growth Hormone ; Humans