PURPOSE: The reported clinical manifestations in Turner syndrome were different by karyotypes, countries and races. So, We evaluated the physical findings & medical problems in patient with Turner syndrome according to karyotype. METHODS: We examined prospectively 53 cases that diagnosed as Turner syndrome by chromosome study at the Pediatric Endocrine Clinic, YUMC from May 1999 to Aug 1999. According to the karyotype, they were divided into 3 groups:45, X(22 cases, 41.5%), mosaicism(18 cases, 34%) and structural aberration group(13 cases, 24.5%), and 53 cases were reevaluated by prepared protocol of 22 abnormal physical findings and 4 medical problems. Chi-square test was used for statistic analysis. RESULTS: 1)In case of 45, X group, they had short stature(100%), cubitus valgus(95.5%), widely spaced nipples(86.4%), shield chest(72.7%), low posterior hair line(68.2%), in case of mosaicism group, they had short stature(100%), cubitus valgus(72.2%), high arched palate(66.7%), widely spaced nipples(61.1%), short neck(61.1%) and in case of structural aberration group, they had short stature(100%), high arched palate (92.3%), widely spaced nipples(92.3%), low posterior hair line(76.9%), shield chest (76.9%), in frequency order. Short stature was the most common finding in Turner syndrome. The incidence of high arched palate was significantly higher in structural aberration group(P<0.05). 2)In case of 45, X group, they had otitis media(63.6%), thyroid problem(4.5%) and no renal problem and cardiac problem, in case of mosaicism group, they had otitis media(38.9%), thyroid problem(5.6%) and no renal problem and cardiac porblem and in case of structural aberration group, they had otitis media(61.5%), thyroid problem(15.4%), renal problem(7.7%) and cardiac problem(7.7%) in frequency order, they had no statistical difference in among three groups. CONCLUSION: In case of 45, X group, the incidence of cubitus valgus, webbed neck were more common findings and of high arched palate, micrognathia were less common findings compared to other group. In case of mosaicism group, the incidence of micrognathia, epicanthal fold were more common findings and wide spaced nipples, antimongoloid fissure were less common findings compared to other group. In case of structural aberration group, the incidence of high arched palate is most common finding among 3 group(P<0.05) and pigmented nevi and epicanthal fold were less common findings compared to other group. The incidence of medical problems such as otitis media and thyroid problem were than other foreign country reports.
Continental Population Groups ; Hair ; Humans ; Incidence ; Karyotype* ; Mosaicism ; Neck ; Nevus, Pigmented ; Nipples ; Otitis ; Otitis Media ; Palate ; Prospective Studies ; Thorax ; Thyroid Gland ; Turner Syndrome*

BACKGROUND: Growth factors play important roles in wound healing. However, the evidence for the effects of growth factors on post-thyroidectomy scars is limited. OBJECTIVE: We performed a prospective study to assess the preventive and therapeutic effect of a multi-growth factor (MGF)-containing cream on post-thyroidectomy scars. METHODS: Twenty-one patients with thyroidectomy scars applied MGF cream twice a day. We assessed the changes in erythema, pigmentation, skin elasticity, and skin hydration status using the erythema index, melanin index, cutometer, and corneometer, respectively. In addition, Vancouver scar scale (VSS) and patient satisfaction were assessed at 10 days after surgery (baseline), 2 weeks, 6 weeks, and 12 weeks after baseline. RESULTS: The mean total VSS scores were significantly lower at 6 weeks (3.24±1.51 vs. 1.91±1.38) and 12 weeks (3.24±1.51 vs. 1.71±1.59) compared to the baseline. The degree of pigmentation was significantly lower at 12 weeks compared to the baseline, and the skin elasticity, and the skin hydration status were significantly higher at 12 weeks compared to the baseline. Over 85% of the patients were satisfied with the use of MGF cream without any adverse effect. CONCLUSION: MGF cream might have additive or supportive effect for scar formation after thyroidectomy.
Cicatrix* ; Elasticity ; Erythema ; Humans ; Intercellular Signaling Peptides and Proteins ; Melanins ; Patient Satisfaction ; Pigmentation ; Prospective Studies ; Skin ; Skin Pigmentation ; Thyroidectomy ; Wound Healing

Intracranial lipoma is a rare tumor. It is considered as congenital malformation, caused by poor differentiation and abnormal persistence of the primitive meninges. More frequently intracranial lipomas are central and located in the midline especially in corpus callosum. We report the two cases of intracranial lipoma, one case was detected in cerebellum by antenatal sonography and the other one was detected in right temporal cistern by cranial sonography during the postnatal follow-up.
Cerebellum ; Corpus Callosum ; Follow-Up Studies ; Lipoma* ; Meninges

Orbital complication of an isolated sphenoid sinus lesion is rare. We recently experienced one case of optic neuropathy secondary to sphenoid sinus Aspergillosis. The patient presented with a headache and diplopia. Imaging and histology showed a sphenoid sinus lesion, andthe patientunderwent endoscopic sinus surgery. Because of the disease progression after the surgery,the patient was administered an anti-fungal agent underclinical suspicion ofan invasive fungal sinusitis infection before receiving histologic confirmation of tissue invasion by fungal hyphae. The disease stabilized in three months by using approximately one month of anti-fungal agent. The type of treatment required for sphenoid sinus lesions depends on the extent of the disease upon initial examination as well ason the rapidity of diseaseprogression.
Aspergillosis* ; Diplopia ; Disease Progression ; Headache ; Humans ; Hyphae ; Optic Nerve Diseases* ; Orbit ; Sinusitis ; Sphenoid Sinus*

No abstract available.
Female ; Humans

Focal nodular hyperplasia (FNH) is a benig nepithelial tumor of the liver. The etiology of FNH is unknown, but recent evidence suggests that FNH may represent a localized, hyperplastic response to a pre-existing vascular malformat ion. There is a high probability of as sociat edlesions , most commonly hepatic hemangiomas, meningioma, as trocytoma, and arterial dysplasia in various organs . In the present report we describe a FNH with aberrant lymphatics in a 24-year-old woman. In operation field, lymphatics were located on the site of falciform ligament. Histologically, aberrant lymphatics were composed of well vas cularized complex lymphatic channels and the mass were typical FNH. In this case, the role of aberrant lymphatics in the development of FNH was unclear . But as the FNH frequently as sociated with ot her anomalies , we think the aberrant lymphatic as such anomaly that have not been reported.
Carcinoma, Hepatocellular* ; Female ; Focal Nodular Hyperplasia ; Hemangioma ; Humans ; Ligaments ; Liver ; Meningioma ; Young Adult

Pacemaker twiddler's syndrome is reported as a very rare complication of permanent pacemaker implantation. There was a recent report suggesting that the incidence of pacemaker twiddler's syndrome increase recently presumably as a result of the implantation of thinner and smaller pacemaker system than before. We experienced a case of pacemaker twiddler's syndrome complicated 3 times with the conventional method of implantation or replacement during 14 months after the first implantation(Optims MP 158C and Pacing lead 400, Telectronic)on June 13th 1987. This case was an 18 year-old high school girl who had suffered frequent syncope for 2 years and extertionl dyspnea for 5 years due to congenital complete heart block, of which block site was proved to be AV nodal by His bundle electrogram. Pacemaker twiddler's syndrome developed 3 times;firstly 6 weeks after the first implantation in the right subclavicular fossa, secondly 10 weeks after the replacement of the twisted pacing lead, thirdly 10 months after the change of implantation site to the left subcalvicular fossa with the replacement of the twisted and fractured lead. Finally, the pacemaker generator was anchored to the clavicular periostium and pectoralis fascia at several points by using Dacron pouch.
Adolescent ; Dyspnea ; Electrophysiologic Techniques, Cardiac ; Fascia ; Female ; Heart Block ; Humans ; Incidence ; Polyethylene Terephthalates ; Syncope

The only clinically avilable levo-isomer type of beta-recepter blocker is penbutolol sulfate, and it is already accepted as one of beta-receptor blockers for initial antihypertensive drug therapy according to the report of 1988 Joint National Committee on Detection, Evaluation, and Tratment of High Blood Pressure. To evaluate the antihypertensive efficacy, effect on the quqlity of life, and side effects of penbutolol recently introduced into Korea, penbutolol was administered to 29 essential hypertensive(mild 9, moderate10, and severe 10) patients for 12 weeks or longer. The result of the clinical analysis are as follows; 1) The mean age was 50.0+/-10.9(M+/-SD), and the sex distribution between male and female was16:13. 2) The blood pressure lowering effects of penbutolol as a monotherapy were marked in 16, moderate in 6, and insignificant in 2 cases. The systolic blood pressure was significantly decreased from 179.1+/-20.2 to 135.4+/-16.5mmHg(P<0.005), and the diastolic blood pressure from 112.6+/-13.5 to 84.0+/-11.9mmHg(P<0.005)after 12 weeks' penbutolol therapy. 3) The heart rate was significantly decreased from70.3+/-13.3 to 65.5+/-9.1 per minute(P<0.05). 4) The quality of life was improved markely in 5(17.2%) and slightly in 8 cases(29.6%). 5) There were no significant laboratory changes after 12 weeks' penbutolol therapy. 6) Two out of three cases with non-specific ST segment and T wave changes in EKG and two out of 9 cases with EKG were normalized, 2 cases of LAH with strain were improved. 7) The side effects of penbutolol were dizziness in 4, sexual dysfunction in 2, and skin rash in 1 case. 8) Final multifarious assessment of penbutolol therapy showed that it was very useful in 11(37.9%), useful in 4(13.8%) and slightly useful in 7 cases(24.1%). These reult suggest that penbutolol is a first-line antihypertensive agent with an effective antihypertensive action, improving quality of life, with no significant laboratory changes and few side effects.
Blood Pressure ; Dizziness ; Drug Therapy ; Electrocardiography ; Exanthema ; Female ; Heart Rate ; Humans ; Hypertension ; Joints ; Korea ; Male ; Penbutolol ; Pheniramine ; Quality of Life ; Sex Distribution

Acer mono is known to contain bioactive substances that exhibit beneficial effects in osteoporosis, gastric ulcers, hepatic damage, and pathologic angiogenesis. The current study aimed to investigate the effects of Acer mono extract on the invasive activities and cell-cycle progression of human fibrosarcoma cells. Cytotoxicity of Acer mono extract was assessed by MTT assay, in-vitro invasiveness of HT1080 fibrosarcoma cells was measured using matrigel assay, expression of invasion- and cell-cycle-related proteins was analyzed by western blot analysis, and that of E2F target genes was quantified using qRT-PCR. Acer mono extract did not show distinct cytotoxicity in the experimental concentrations used. Invasiveness of HT1080 fibrosarcoma cells and expression of cyclin D1 and CDK4 in them were significantly reduced in a dose-dependent manner after treatment with Acer mono extract. Acer mono extract showed inhibitory effects on the G1/S transition during cell-cycle progression; the active phosphorylated Rb protein level was decreased, and expression of E2F target genes was downregulated by the Acer mono extract. Our data collectively demonstrated that Acer mono extract exerts inhibitory effects on the invasiveness and cell-cycle progression of HT1080 human fibrosarcoma cells.

Acer mono is known to contain bioactive substances that exhibit beneficial effects in osteoporosis, gastric ulcers, hepatic damage, and pathologic angiogenesis. The current study aimed to investigate the effects of Acer mono extract on the invasive activities and cell-cycle progression of human fibrosarcoma cells. Cytotoxicity of Acer mono extract was assessed by MTT assay, in-vitro invasiveness of HT1080 fibrosarcoma cells was measured using matrigel assay, expression of invasion- and cell-cycle-related proteins was analyzed by western blot analysis, and that of E2F target genes was quantified using qRT-PCR. Acer mono extract did not show distinct cytotoxicity in the experimental concentrations used. Invasiveness of HT1080 fibrosarcoma cells and expression of cyclin D1 and CDK4 in them were significantly reduced in a dose-dependent manner after treatment with Acer mono extract. Acer mono extract showed inhibitory effects on the G1/S transition during cell-cycle progression; the active phosphorylated Rb protein level was decreased, and expression of E2F target genes was downregulated by the Acer mono extract. Our data collectively demonstrated that Acer mono extract exerts inhibitory effects on the invasiveness and cell-cycle progression of HT1080 human fibrosarcoma cells.