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*

Calcification so of the basal ganglia are rarely found at routine autopsies and in skull radiographs. CT is superior to the plain skull radiographs in detecting intracranial attenuation differences and may be stated to bethe method of choice in the diagnosis of intracranial calcifications. Of 5985 brain CT scans performed in KyungHee Univeristy Hospital during past 3 years, 36 cases were found to have high attenuation lesions suggesting within basal ganglia. 1. The incidence of basal ganglia calcification on CT scan was about 0.6%. 2. Of these 36 cases, 34 cases were bilateral and the remainder was unilateral. 3. The plain skull films of 23 cases showed visible calcification of basal ganglia in 3 cases (13%). 4. No specific metabolic disease was noted in the cases.
Autopsy ; Basal Ganglia ; Brain ; Diagnosis ; Incidence ; Metabolic Diseases ; Methods ; Skull ; Tomography, X-Ray Computed

PURPOSE:The incidence of congenital adrenal hyperplasia(CAH) is 1/5,000- 1/20,000 births and thus the importance of the neonatal screening test is being emphasized. However, the reference value for the term and preterm infants has not yet been established and false positive values are frequent due the immature hypothalamic-adrenal axis of the preterm infants or the stress-induced adrenal dysfunction. Therefore, we analyzed the 17-hydroxyprogesterone(17-OHP) concentration in terms of gestational age, birth weight, and postnatal state to establish the reference range for the Korean term and preterm infants. METHODS:We analyzed the results of the CAH screening test retrospectively, which was performed on 737 neonates(624 fullterm neonates, 113 premature neonates) born between January 1998 through July 1998 in Inje University College of Medicine Sanggye Paik Hospital. Mean gestational age and birth weight of infants were 38.2+/-2.6 weeks and 3,116+/-674kg respectively. 17-OHP screening test was performed on 4.9+/-3.8days after birth by obtaining blood samples from the heelstick of neonates. 17-OHP concentration was measured by the ELISA kit(ICN Co.) and repeated the procedure if the result was higher than 35ng/ml. RESULTS: 1) 17-OHP concentration of the preterm infants was significantly higher than that of the fullterm infants(19.1+/-12.3ng/ml vs 11.7+/-7.8ng/ml, P=0.001). 17-OHP concentration was inversely proportional to gestational age. 2)17-OHP concentration was inversely proportional to birth weight(r=0.22, P>0.01). 17-OHP concentration according to birth weight was as follows.:below 1,500g was 26.7+/-11.7ng/ml, 1,500 to 2,000g was 18.0+/-13.9ng/ml, 2,001 to 2,500g was 17.9+/-10.5ng/ml, 2,501 to 3,000g was 12.1+/-7.9ng/ml, 3,001 to 3,500g was 11.5+/-8.1ng/ml, above 3,500g was 11.4+/-7.5ng/ml. There was a significant decline in the 17-OHP concentration as the birth weight increased. 3) 17-OHP concentration was gradually decreased as sampling date increased. 4) The gender of the infants did not influence the 17-OHP concentration(male 13.0+/-9.1 vs female 12.7+/-9.0). 5)17-OHP concentration were significantly higher in sick preterm infants than healthy preterm infants. 6)Six cases, whose 17-OHP concentration were greater than 35ng/ml, were all preterm and low birth weight infants. Reexamination after one week showed the value within normal range. No CAH cases were diagnosed in the study. CONCLUSION: 17-OHP concentration was inversely proportional to gestational age and birth weight. Therefore, reference ranges of 17-OHP concentration should be subdivided according to gestational age and birth weight. Further research about perinatal risk factors affecting the 17-OHP concentration will be required.
17-alpha-Hydroxyprogesterone* ; Axis, Cervical Vertebra ; Birth Weight ; Enzyme-Linked Immunosorbent Assay ; Female ; Gestational Age ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature* ; Mass Screening ; Neonatal Screening ; Parturition ; Reference Values ; Retrospective Studies ; Risk Factors

Leri-Weill syndrome or Leri-Weill dyschondrosteosis represents a short stature syndrome that is characterized by symmetric shortening of the forearms and lower legs and a bilateral shortening and bowing of the radius with a dorsal subluxation of the distal ulna(Madelung deformity). Recent genetic analyses demonstrated that functional haploinsufficiency of SHOX(short stature homeobox-containing gene) accounts for Leri-Weill syndrome. Further studies are needed to explain phenotypic heterogeneity of SHOX defect. We experienced a case of Leri-Weill syndrome in a 11-year-old girl with short stature, who revealed typical Madelung deformity, mesomelic(middle segment) dysplasia, and a karyotype of 46,XX. In cases with dyschondrosteosis or Turner-characteristic dysmorphic skeletal features, detection of SHOX mutation is recommended.
Child ; Congenital Abnormalities ; Female ; Forearm ; Haploinsufficiency ; Humans ; Karyotype ; Leg ; Population Characteristics ; Radius

OBJECTIVE: To investigate new signal transduction cascade through integrin, FAK and ERK in the suppressed cell proliferation by GnRH-I and -II. METHOD: Human endometrial cancer cells (HEC1A) were cultured under the following condition: DMEM/F12 (10% FBS). GnRH-I and -II were treated time (0, 5, 10, 15, 20, 30 min; 100 nM) and dose (10 nM or 100 nM; 20 min) dependent manner according to experimental purposes. Cell proliferation was measured using [3H] thymidine incorporation assay. Immunoblotting was utilized to detect proteins. RESULTS: GnRH-I and -II inhibited proliferation of HEC1A cells and induced expression of integrin beta3. Phosphorylation of FAK and ERK were induced by GnRH-I and -II. CONCLUSION: GnRH inhibited cell proliferation via the expression of integrin and FAK, ERK phosphorylation.
Cell Proliferation* ; Endometrial Neoplasms* ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Immunoblotting ; Integrin beta3 ; Phosphorylation ; Signal Transduction ; Thymidine

Six different brain tumors in middle cranial fossa are presented which are studied by CT and proved pathologically. The authors experienced rare tumors in middle cranial fossa such as cavernous hemangioma, cysticmeningioma, Schwannoma, Masson's vegetant intravascular hemangioendothelioma and other tumors (arteriovenousmal formation and metastatic adenoid cystic carcinoma) whose CT findings were atypical. The results are as follows; 1. I case of tumors in middle cranial fossa, basal and coronal sections are necessary for further evaluation of the relation with dura and adjacent bone changes. 2. In suspicion of metastasis, bone setting should be done to find out bone involvement. 3. Internal carotid angiography gave little help in the differential diagnosis of tumors in middle cranial fossa.
Adenoids ; Angiography ; Brain Neoplasms ; Cranial Fossa, Middle ; Diagnosis, Differential ; Hemangioendothelioma ; Hemangioma, Cavernous ; Neoplasm Metastasis ; Neurilemmoma

Paragonimiasis is widely distributed in Far East and Southeast Asia, particularly in Korea. The centralnervous system is the most frequent location for paragonimiasis outside the lungs. We analized the computedtomographic findings of 17 cases which were diagnosed pathologically and clinically as cerebral paragonimiasis.The results were as follows; 1. The ratio of male to female was 10:7 and about 88% of cases were under the age of40 years. 2. The common location so cerebral paragonimiasis were the occipital (12 cases ) and temporal (11 cases) lobes. 3. Precontrast CT findings of cerebral paragonimiasis were low density with calcifications in 6 cases,low and isodensities in 4 cases, mixed densities in 3 cases, only low density in 2 cases and only calcification sin 2 cases. Hydrocephalus (7 cases), mass effect (6 cases), atrophic change(6 cases) and cyst formation (3 cases)were associated. 4. The shape of calcifications in CT scan were soap-bubble or ring in 6 cases, nodular or oval in6 cases, stippled in 4 cases and amorphous conglomerated in 2 cases. 5. The contrast-enhanced 8 cases were 5 ringor rim like, 2 nodular and 1 irregular enhancements, while 9 cases were not enhanced.
Asia, Southeastern ; Far East ; Female ; Humans ; Hydrocephalus ; Korea ; Lung ; Male ; Paragonimiasis ; Tomography, X-Ray Computed

BACKGROUND: A large number of children with psychosocial dysfunction are not recognized within schools or primary care settings. In several research, an increasing amount of attention has been paid to the importance of low family support as a predictor of psychosocial dysfunction in children. Our study examined the agreement and relationship between the lack of family support as measured by the Family APGAR and child psychosocial dysfunction as screened by the Pediatric Symptom Checklist(PSC). Therefore, We studied the usefulness of Family APGAR as a screening tool of child psychosocial dysfunction. METHODS: We set 643 children that are engaged in elementary school. We gave a questionnaire to their parents and made them record the questionnaire which contained the sociodemographic data, past history of child and parents, the Family APGAR and the translated Pediatric Symptom Checklist(PSC). Finally, we analysed only 506 complete data. We use the SAS/PC 6.12 that is a statistical analysis program. RESULTS: Children with a lack of family support(APGAR) were 5.1 times as likely to receive scores indicating dysfunction on the PSC(PSC). Families with a lack of support were significantly more likely to report low parental educational achievement and low income. Sixty percent of children from families with a lack of support were identified as having a psychosocial dysfunction by the PSC rating; however, only 24% percent of the children identified with psychosocial dysfunction by the PSC had scores indicating poor family functioning on the Family APGAR. Families with a lack of support had significantly higher total scores on PSC(mean=20.2) than families with adequate support(mean=11.9). The strength of the agreement between the Family APGAR and the PSC was k=.29. CONCLUSION: A lack of family support is associated with child psychosocial dysfunction as assessed by the PSC. However, the Family APGAR was not a sensitive measure of child psychosocial dysfunction, and thus it supplements, but does not replace the PSC.
Child* ; Educational Status ; Humans ; Mass Screening ; Parents ; Primary Health Care ; Surveys and Questionnaires

OBJECTIVE: To investigate the role of ERK and PPAR gamma on the TGF-beta1 induced human endometrial stromal cell decidualization in vitro. METHOD: Endometrial stromal cells are cultured under the following condition: DMEM/F12 (10% FBS, 1 nM E2 and 100 nM P4). TGF-beta1 (5 ng/ml), Rosiglitazone (50 nM), and PD98059 (20 microgram) were added according to experimental purposes. Trypan-Blue and hematocytometer were utilized to count cell number. Enzyme-linked immunosorbent assay (ELISA) and western blotting were utilized to detect proteins. RESULT: TGF-beta1 inhibited proliferation of cultured human endometrial stromal cells and induced expression of PGE2 and prolactin. This effect was mediated by Smad and ERK activation. Administration of rosiglitazone, PPAR gamma agonist, prevented TGF-beta1 effect on cell proliferation. Furthermore, Rosiglitazone inhibited TGF-beta1 induced activation of ERK, consequently reduced PGE2 and prolactin production. CONCLUSION: TGF-beta1 induced decidualization of endometrial stromal cell through Smad and ERK phosphorylation. PPAR gamma acts as a negative regulator of human endometrial cell decidualization in vitro.
Blotting, Western ; Cell Count ; Cell Proliferation ; Dinoprostone ; Enzyme-Linked Immunosorbent Assay ; Humans* ; Peroxisome Proliferator-Activated Receptors* ; Phosphorylation ; PPAR gamma ; Prolactin ; Stromal Cells* ; Transforming Growth Factor beta1*

PURPOSE: To evaluate the extrahepatic spread of peripheral cholangiocarcinoma with CT. MATERIALS AND METHODS: We retrospectively reviewed CT findings of extrahepatic spread in thirty-six patients with peripheral cholangiocarcinoma confirmed adenocarcinoma at surgery(n=5) or percutaneous biopsy(n--31) without primary leion in other organs, and analysed the frequency and distribution of enlarged lymph nodes, direct invasion of adjacent solid organs, and thrombosis of major vessels. RESULTS: Among the 36 cases, enlarged metastatic lymph node was noted in twenty-four(66.7%), direct invasion into adjacent organs in six(16.7%), portal and inferior vena caval thrombosis in five(13.9%). Enlarged lymph nodes were seen in hepatic nodes(n=16, 44.4%), portocaval nodes(n=15, 41.7%), interaortocaval nodes (n=10, 27.8) and celiac nodes in two(5.6%). Direct invasion was detected in the greater omentum(n=6), gallbladder(n:3) and stomach(n=2). Portal vein thrombosis(n:5) and thrombosis of inferior vena cava(n=2) were noted. CONCLUSION: Lymph node metastasis was seen in 67% in patients with peripheral cholangiocarcinoma. Direct invasion to adjacent organs and thrombosis in portal vein or inferior vena cava were occasionally associated.
Adenocarcinoma ; Cholangiocarcinoma* ; Humans ; Lymph Nodes ; Neoplasm Metastasis* ; Portal Vein ; Retrospective Studies ; Thrombosis ; Vena Cava, Inferior