PURPOSE: Short stature is the most constant finding in Turner syndrome. Short stature in Turner syndrome has lately received considerable attention, mostly because of recent attemp to improve growth by hormonal treatments; growth hormone, oxandrolone, estrogen. The aim of this study was to find out whether growth promoting treatment would improve final height in girls with Turner syndrome. METHODS:Seventy-one girls with the clinical chracteristics Turner syndrome verified by karyotype analysis were entered into this study. The following selection criteria for final adult height were used; Chronological age of more than 14years old, bone age of more than 15years old and growth velocity of less than 0.5cm per 6months. Analysis was performed by means of multiple regression analysis between descriptive data; modality of treatment with oxandrolone and/or estrigen, parental height, karyotype and final adult height. RESULTS: 1) The final adult height of untreated Turner syndrome was 138.9+/-3.9cm. 2)The final adult height in 29 GH treated Turner girls was 143.9+/-6.5cm, significant higher value than 42 GH untreated Turner girls height, 139.8+/-5.2cm(p<0.01). 3) The final height in GH only group and combined group were 141.2+/-6.0cm, 146.2+/-6.2cm, respectively. The combined therapy was more effective than GH therapy(p<0.01). 4) The final height in 32 patients with karyotype of 45,X was 141.6+/-5.6cm, and that of 31 structural aberration group was 140.3+/-6.2cm. There was no significant difference between two groups. But in mosaicism, only numeric abnormalities, the final height 145.9+/-6.1cm was much more higher than other two groups(p<0.05). 5) The final adult height in Turner syndrome was in good correlation with target height. Final adult height(cm)= 1.01*Target height(cm)- 4.97 r=0.51, p<0.05. 6) There was positive correlation between final adult height and height SDS at start GH treatment and negative correlation with age at start GH treatment. The delta height (final height - height at start treatment) correlate with GH treatment duration. CONCLUSIONS:The final adult height in Turner syndrome in a given ethinic or national population varies in the same way as adult height in normal women. Growth hormone therapy may increase final height in Turner syndrome irrespective of ethinic or national difference. Further growth was observed in GH combined with estrogen or oxandrolone.
Adult* ; Estrogens ; Female ; Growth Hormone ; Humans ; Karyotype ; Mosaicism ; Oxandrolone ; Parents ; Patient Selection ; Turner Syndrome*

PURPOSE: A number of studies have been published on the effect of growth hormone therapy over 1-3 years in children with growth hormone deficiency(GHD) & Familial short stature(FSS). So far final height data are seldomly available. Final heights of GH treated children with GHD & FSS were evaluated. METHODS: 10 Children with GHD and 69 children with with FSS were enrolled for the study. They were treated with GH 0.1IU/kg/daily in 10 GHD and 20 children with FSS. They were grown up and reached adult height. 49 children with FSS were not treated at all. Facors influencing final height were investigated. RESULTS: 1) All patients with GHD(Idiopathic 8 cases, Organic 2 cases) had additional gonadotropin deficiency and had multiple pituitary hormone deficiency. 2) At start of GH treatment boys of idiopathic GHD were 9.8 years old and 12.4 years old in girls and their mean height was 114.8cm(-2.8SDS), 123.0cm(-2.9 SDS)in boys and girls respectively. Boy with orgnaic GHD was 11.1 years and 6.7 years old in girl. Their height were 126.0cm(-1.5SDS) and 104cm(-1.2SDS) respectively. 3) Mean final height of idiopathic GHD was 167.6cm(-0.5SDS) in male and 161.0 cm(0.7SDS) and that of organic GHD was 173.0cm(0.5SDS) in male and 157cm (0SDS) in girl. 4) Mean Final height in untreated children with FSS was 159.8+/-.2cm(-1.6 SDS)in male and 149.6+/-.3cm(-1.4SDS) in female. Mean final height of GH treated in FSS was 162.5+/-.1cm(-1.5SDS) in male and 152.0+/-.4cm(-1.2SDS) in female But there was no statiscally difference between untreated and treated children in final height. 5) The age of onset of menarche was 12.74+/-.78 years old in GH treated group (n=12) and 12.45+/-.16 years old in untreated group(n=34). CONCLUSION: The GH administration in patients with GHD has been confirmed for growth promotion. but in case of FSS there was no significant difference between treated and untreated group. More further studies are needed for the confirmation of the efficacy of GH therapy in patients with FSS.
Adult ; Age of Onset ; Child* ; Female ; Gonadotropins ; Growth Hormone* ; Humans ; Male ; Menarche

Craniofacial clefts are rare among facial anomalies with an incidence of 1.5 to 5 per 100,000 births, and 1 per 100 cases of cleft lip and palate. The Tessier No. 7 clefts are unusual lesions that result from failure of the embryonic mandibular and maxillary processes to properly fuse and form the corners of the mouth. We experienced a case of Tessier No. 7 craniofacial cleft in a 1 day-old female patient who presented with a macrostomia and auricular malformation. The diagnosis was established by clinical and radiographic findings. A brief review of literature was made.
Cleft Lip ; Diagnosis ; Female ; Humans ; Incidence ; Macrostomia ; Mouth ; Palate ; Parturition

PURPOSE:Decreased bone mineral density(BMD) has been reported in girls with Turner syndrome. Estrogen therapy is recommanded to improve sexual infantilism and decreased BMD. Short stature is also characteristic finding in patients with Turner syndrome. Treatment modality for short stature has included estrogen, anabolic steroids and growth hormone(GH). Recently GH therapy in GH deficient children could increase BMD in addition to improve short stature. We observed the treatment effects on bone mineral density in patients with Turner syndrome. METHODS: Bone Mineral Density in second to fourth lumbar spine area were measured by dual energy X-ray absorptiometry in 56 girls with Turner syndrome, before and after growth hormone and/or estrogen. All Turner girl was confirmed by clinical and chromosomal examination. RESULTS: 1) There was no significant difference in BMD according to karyotype. 2) The mean BMD of untreated Turner syndrome was 0.752+/-0.122g/cm2. 3) The mean BMD before and after GH treatment were 0.620+/-0.028g/cm2, 0.793+/-0.093g/cm2 respectively. The mean BMD before and after estrogen treatment were 0.761+/-0.125g/cm2, 0.918+/-0.141g/cm2 respectively. In combined group, the BMD were 0.752+/-0.087g/cm2 and 0.939+/-0.134g/cm2. Growth hormone was also effective to improve BMD as well as estrogen. But the changes of BMD were more significant in estrogen and combined group(p<0.05). 4) A significant positive correlation was found between age and BMD(p<0.05). CONCLUSIONS:Estrogen therapy can accelerate epiphysial maturation and compromise final height. Growth hormone therapy in Turner girls was effective for improvement bone mineral density as well as growth improvement. But growth hormone and estrogen combined therapy or Estrogen therapy is more effective to improve bone mineral density in Turner syndrome. Estrogen replacement can be delayed for a while on growth hormone treatment and the appropriated time of estrogen therapy should be elucidated.
Absorptiometry, Photon ; Bone Density* ; Child ; Estrogen Replacement Therapy ; Estrogens ; Female ; Growth Hormone ; Humans ; Karyotype ; Sexual Infantilism ; Spine ; Steroids ; Turner Syndrome*

Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

Electron mlactimpic studies were performed on three primary and two early secondary syphilitic lesions from three patients. AZ of these patients had both secondary syphilitic lesions and chancres. There was no difference in the morphology and location of treponemes in these patients as compared to those who had only a chancre or only a syphilid reported earlier. This study revealed that Treponema pallida were principally located in the intercellular space of keratinocytes and exhacellular space of the dermis. Furthermore, these organisms were observed within histiocytes, endothelial cells, fibroblasts, lymphocytes and even plasma cells. The plasma cells had well developed endoplasmic reticulum, and in some, dilatation of the endoplasmic reticulum was noted. Many t mponemes were demonstrated in and around blood vessels. The vascular endothelial cells were proliferating and the basement membranes were thick.
Basement Membrane ; Blood Vessels ; Chancre* ; Dermis ; Dilatation ; Endoplasmic Reticulum ; Endothelial Cells ; Extracellular Space ; Fibroblasts ; Histiocytes ; Humans ; Keratinocytes ; Lymphocytes ; Microscopy, Electron ; Plasma Cells ; Syphilis ; Syphilis, Cutaneous* ; Treponema pallidum* ; Treponema*

PURPOSE: Headache, especially migraine disorders, have been thought to be pathophysiologically related to epilepsy. We sought to elucidate the differential diagnostic clues between headache and epilepsy by analyzing the clinical characteristics of patients who presented with a chief complaint of headache and who had been subsequently diagnosed with epilepsy. METHODS: We studied 399 patients with headache who had been followed-up in Pediatrics Department of Ilsan Hospital from January 2005 to July 2009. Patients were divided into two groups and analyzed retrospectively. The test group included 15 patients who had been diagnosed with epilepsy. The control group included 305 patients whose symptoms had been diagnosed as primary headache. We compared the type of headache, presence of aura, subtype of aura, family history of migraine disorders, EEG findings, and response to a hyperventilation test between the two groups. RESULTS: 305 patients had primary headache, 20 patients secondary headache, and 74 patients unclassified headache. Between the two groups, there was no statistically significant difference in mean age, sex, type of headache (P=0.23), type of aura (P=0.64), or associated family history (P=0.84). However the epilepsy group had more migraine with aura (P<0.05), more EEG abnormalities (P<0.0001), and more abnormal hyperventilation test (P<0.0001), which were all statistically significant. CONCLUSION: The presence of aura, probably visual aura, and EEG abnormalities or abnormal response in hyperventilation test are very useful differential diagnostic clues for epilepsy in headache patients. Therefore, we highly recommend that EEG are performed in order to rule out the possibility of epilepsy in those children who present with headache and have migraine headache with aura and abnormal result on hyperventilation test.
Child ; Electroencephalography ; Epilepsy ; Headache ; Humans ; Hyperventilation ; Migraine Disorders ; Migraine with Aura ; Pediatrics ; Retrospective Studies

Bone metastasis of hepatocellular carcinoma appears to be peculiar when clinical manifestation of liver disease is not apparent, and initial diagnosis of metastatic hepatocellular carcinoma by fine needle aspiration cytology is rarely obtained. We experienced a case of 45-year-old man with metastatic hepatocellular carcinoma in the sacrum, which was diagnosed by fine needle aspiration cytology. The intrahepatic mass, measuring 1.2cm in diameter and kept unchanged in size for two years, was never proved to be hepatocellular carcinoma histopathologically. The aspirated neoplastic cells were mostly in sheets, showing abundant acidophilic cytoplasm and large, round, centrally located nuclei with single, prominent acidophilic mucleoli. In the cell block section, diagnosis of metastatic well-differentiated hepatocellular carcinoma was made without difficulty, and definite trabecular fashion with sinusoidal endothelial cell lining was found.
Biopsy, Fine-Needle ; Carcinoma, Hepatocellular ; Cytoplasm ; Diagnosis ; Endothelial Cells ; Humans ; Korea* ; Liver Diseases ; Middle Aged ; Neoplasm Metastasis ; Prevalence* ; Sacrum

PURPOSE: This study was done to elicit basic data for effective human resource management by identifying the relationships among job embeddedness, emotional intelligence, social support, and the turnover intention of Nurses. METHODS: Research design was to build a hypothetical causal model between variables and to verify its fitness. The sample for this study was 283 nurses with careers of more than 6 months in one hospital of more than 800 beds located in Seoul. They agreed in writing and this study was approved by the Institutional Review Board. Data were analyzed using SPSS 18.0 and AMOS 18.0 program. RESULTS: Differences in general characteristics for the variables were significant for age, marital status, education, work experience, job title, income, and department. Job embeddedness, emotional intelligence and social support were significantly correlated to turnover intention. Job embeddedness to emotional intelligence and social support showed positive effects and a negative effect to turnover intention. Emotional intelligence to turnover intention showed a positive effect, but social support was not significant. CONCLUSION: Organizations should provide ways to minimize voluntary turnover of a competent workforce and demonstrate their competency. Also it should develop training and management programs to effectively utilize emotional intelligence.
Education ; Emotional Intelligence* ; Ethics Committees, Research ; Humans ; Intention* ; Marital Status ; Research Design ; Seoul ; Writing

PURPOSE: This study was performed to evaluate the efficiency of Platelet-rich plasma (PRP) for acceleration of bone healing process on allograft transplantation after curettage in benign bone tumor. MATERIALS AND METHODS: From December 2007 to February 2009, twenty-one patients who had benign bone tumor and underwent allograft transplantation after curettage were evaluated. Mean follow-up period was 14.6 months (range, 12-26 months). We compared with 13 cases of PRP group and 8 cases of non-PRP group in terms of size of lesion, bone resorption, amount of applied PRP and complications. The mean age at surgery was 23.6 years (range, 4-73 years). The most common diagnosis was simple bone cyst (7) followed by enchondroma (4), giant cell tumor (3), undifferentiated benign bone tumor (3) and so on. RESULTS: The mean size of lesion was 33.5 cm3 (range, 2.3-181.9 cm3) (29.4 cm3 in PRP group and 40.2 cm3 in non-PRP group). The mean volume of injected PRP was 7.4 cc (range, 3-12 cc). Bone union started at 3.0 months (range, 1.5-5.8 months) in PRP group and 5.3 months (range, 4-8 months) in non-PRP group. Three cases for each group were excluded due to recurrence and pathologic fracture. One patient had febrile episode 3 weeks later after surgery which subsided with antibiotics. CONCLUSION: The PRP could accelerate bone union in allograft transplantation after curettage of benign bone tumor. Furthermore, we expect that PRP can accelerate bone union in fracture or non-union.
Acceleration ; Anti-Bacterial Agents ; Bone Cysts ; Bone Resorption ; Chondroma ; Curettage ; Follow-Up Studies ; Fractures, Spontaneous ; Giant Cell Tumors ; Humans ; Platelet-Rich Plasma ; Recurrence ; Transplantation, Homologous ; Transplants