No abstract available.
Adolescent* ; Humans ; Male* ; Shock, Septic*

Monilethrix is a rare hereditary disorder of hair which was described by smith in 1879. The scalp hair is most frequently affected but the body or sexual hair are rarely affected. The affected hair shows periodic constrictions that break easily, so the hair could not grow more than a few millimetere long. It is thought that the node of the affected hair is normal and the thin internodal segment is deficient in hair matrix. Keratosis pilaris is generally an associated feature. We experienced a case of monilethrix with keratosis pilaris. The 4 years old male patient with monilethrix affected only scalp hair with keratosis. The family history was non-contributory.
Child, Preschool ; Constriction ; Hair ; Humans ; Keratosis* ; Male ; Monilethrix* ; Scalp

Neuroblastoma stage IV-S patients have frequent spontaneous remission and high survival rate. Many investigators have recommended minimal or no therapeutic intervention ; however, some patient do experience progressive disease and ultimately die of neuroblastoma. We experienced a case of stage IVS neuroblastoma with N-myc amplification and coagulopathy. This patient has treated with combination chemotherapy and radiation therapy, then remained disease free for 1 year on the follow up till March, 1997.
Drug Therapy, Combination ; Follow-Up Studies ; Humans ; Neuroblastoma* ; Remission, Spontaneous ; Research Personnel ; Survival Rate

The retinoic acid and retinoid(Ro 11-1430) were alternately applied topically on forty patients with acne vulgaris. Therapeutic effects and side reactions of this regimen were compared with our previous studies of the treatment with retinoic acid alone and with retinoid alone. Compared with the treatment with retinoic acid alone, the therapsutic effect of the alternating treatment was relatively superior as indicated by the markedly reduced severity of local side reactions.
Acne Vulgaris* ; Humans ; Tretinoin

Developmental venous anomalies are congenital anomalies of the intracranial venous drainage and clinically asymptomatic. Cavernous hemangiomas are vascular malformations composed of dilated vascular channels lined with a single layer of endothelial cells without any intervening normal neural tissue. Although, cavernous hemangioma may be clinically silent, but frequently cause variable neurologic manifestations; intracranial hemorrhage, seizures, and focal neurologic deficits. Frequent association of these two diseases has already been reported and it has been suggested that cavernous hemangiomas not the developmental venous anomalies that cause the acute clinical symptoms. A 4 year old girl with sudden onset of headache, vomiting, gait disturbance and signs of unilateral cerebellar dysfunction 2 days before admission showed a 2.5x2.5cm sized hematoma with heterogenous signal intensity both in CT and MRI in the left cerebellar hemisphere. Four-vessel cerebral angiography revealed characteristic findings of developmental venous anomalies. Pathologic findings of the surgically removed hematoma disclosed typical feature of cavernous hemangioma. Based on the findings in this case and review of the literatures, we concluded that the possibility of other diseases such as cavernous hemangiomas must be considered in patients who has intracranial hemorrhage due to the developmental venous anomalies.
Cerebellar Diseases ; Cerebral Angiography ; Child, Preschool ; Drainage ; Endothelial Cells ; Female ; Gait ; Headache ; Hemangioma, Cavernous* ; Hematoma ; Hemorrhage* ; Humans ; Intracranial Hemorrhages ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Seizures ; Vascular Malformations ; Vomiting

The purpose of this study w-s to predict the respose to the chincap therapy from the initial cephalometric measurements and to obtain the indication of chincap therapy. 40 patients selected for this study were classified into two groups by the occlusal stability after completion of permanent dentition and the iiu4n ovement of facial profile, after chincap therapy. One was g response group which consisted of 25 children and the other was poor response group with 15 patients. Various measurements of the craniofacial structure in the initial lateral cephalogram were calculated and analyzed by t-test and discriminant analysis. The results were, as follows 1. Good response group had more horizontal growth pattern in initial stage of treatment, and the contributing measurements were Bjork sum, anterior-posterior facial height ratio, gonial angle, lower gonial angle and occlusal plane to AB plane angle. 2. The critical points and predictive values of the influential skeletal measurements were calculated. 3. The discriminant function was obtained from three. major influential measurements; Bjork sum, gonial angle and occliml plane to AB plane angle, and this function could discreminate correctly in 85% of this samples.
Child ; Dental Occlusion ; Dentition, Permanent ; Humans

PURPOSE: This treatment planning study was undertaken to evaluate the impact of beam angle configuration of intensity-modulated radiotherapy (IMRT) on the dose of the normal liver in hepatocellular carcinoma (HCC). MATERIALS AND METHODS: The computed tomography datasets of 25 patients treated with IMRT for HCC were selected. Two IMRT plans using five beams were made in each patient; beams with equidistance of 72degrees (Plan I), and beams with a 30degrees angle of separation entering the body near the tumor (Plan II). Both plans were generated using the same constraints in each patient. Conformity index (CI), homogeneity index (HI), gamma index, mean dose of the normal liver (Dmean_NL), Dmean_NL difference between the two plans, and percentage normal liver volumes receiving at least 10, 20, and 30 Gy (V10, V20, and V30) were evaluated and compared. RESULTS: Dmean_NL, V10, and V20 were significantly better for Plan II. The Dmean_NL was significantly lower for peripheral (p = 0.001) and central tumors (p = 0.034). Dmean_NL differences between the two plans increased in proportion to gross tumor volume to normal liver volume ratios (p = 0.002). CI, HI, and gamma indices were not significantly different for the two plans. CONCLUSION: The IMRT plan based on beams with narrow separations reduced the irradiated dose of the normal liver, which would allow radiation dose escalation for HCC.
Carcinoma, Hepatocellular ; Humans ; Liver ; Radiotherapy, Intensity-Modulated ; Tumor Burden

A fine needle aspiration biopsy specimen of a retroperitoneal mass in a 26-year-old man who had had an orchiectomy for seminoma was submitted for cytologic evaluation. Cytologic features of the specimen included uniform neoplastic cells found singly or in groups of several cells intermingled with lymphocytes in a characteristic foamy, lacelike background. These cells varied from 10 to 20 m in diameter. The nuclei were round to ovoid with fine or reticular chromatin and one or more prominent nucleoli. The poorly defined cytoplasm stained pale-blue or blue with cytoplasmic vacuoles. The cytologic appearance was consistent with seminoma. Documented reports of the cytological appearance of seminoma are rare. The diagnosis of primary gonadal seminoma by fine needle aspiration biopsy is probably not indicated since the treatment of a primary gonadal tumor, regardless of its histogenesis, requires surgical resection. However, fine needle aspiration biopsy is extremely valuable in the diagnosis of extragonadal as well as metastic and recurrent seminoma.
Adult ; Biopsy ; Biopsy, Fine-Needle ; Chromatin ; Cyclosporine* ; Cytoplasm ; Diagnosis ; Endothelin-1* ; Gonads ; Humans ; Liver ; Lymphocytes ; Nephrosis, Lipoid* ; Orchiectomy ; Plasma* ; Seminoma ; Vacuoles

PURPOSE: The aim of this study was to investigate treatment results, toxicity and efficacy of hyperfractionated radiation therapy combined with paclitaxel for paraaortic node recurrence in cervix cancer. MATERIALS AND METHODS: Between September 1997 to March 1999, 12 patients with paraaortic node recurrence in cervix cancer who previously received radical or postoperative radiotherapy were treated with hyperfractionated radiation therapy combined with paclitaxel. Of these, 2 patients who irradiated less than 30 Gy were excluded, 10 patients were eligible for this study. Median age was 5 1 years. Initial FlGO stage was 1 stage IB1, 2 stage IIA, 7 stage IIB. For initial treatment, 7 patients received radical radiotherapy and 3 received postoperative radiotherapy. The paraaortic field encompassed the gross recur rent disease with superior margin at T 12, and inferior margin was between L5 and S 1 with gap for previously pelvic radiation field. The radiation field was initially anterior and posterior opposed field followed by both lateral field. The daily dose was 1.2 Gy, twice daily fractions, and total radiotherapy dose was between 50.4 and 60 Gy(median, 58.8 Gy). Concurrent chemotherapy was done with paclitaxel as a radiosensitizer. Dose range was from 20 mg/m to 30 mg/m (median, 25 mg/m'), and cycle of chemotherapy was from 3 to 6 (median, 4.5 cycle). Follow-up period ranged from 3 to 21 months. RESULTS: Interval between initial diagnosis and paraaortic node recurrence was range from 2 to 63 months (median, 8 months). The 1 year overall survival rate and median survival were 75% and 9.5 months, respectively. The 1 year disease free survival rate and median disease free survival were 30% and 3 7 months, respectively. At 1 month after treatment, 4 (40%) achieved a complete response and 6 (63%) experienced a partial response and all patients showed response above the partial response. There was distant metastasis in 6 patients and pelvic node recurrence in 2 patients after paraaortic node irradialion. There was 2 patients with grade 3 to 4 leukopenia and 8 patients with grade 1 to 2 nausea/ vom ting which was usually tolerable with antiemetic drug. There was no chronic complication in abdomen and pelvis during follow up period. CONCLUSION: Hyperfractionated radiation therapy combined with paclitaxel as a radiosensitizer showed high response rate and few complication rate in paraaortic node recurrence in cervix cancer. Therefore, present results suggest that hyperfractionated radiation therapy combined with paclitaxel chemotherapy can be used as optimal treatment modality in this patients.
Abdomen ; Cervix Uteri* ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Leukopenia ; Neoplasm Metastasis ; Paclitaxel* ; Pelvis ; Radiotherapy ; Recurrence* ; Survival Rate ; Tolnaftate ; Uterine Cervical Neoplasms*

Ulcerative colitis, one of the inflammatory bowel disease, is primarily managed medically with combinations of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, the indications and outcome for surgical management of pediatric ulcerative colitis patients were studied from medical records retrospectively. Twenty-one patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were subjected to the study. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management primarily after diagnosis and 8 patients (38%) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53%) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure owing to sclerosing cholangitis respectively. In 8 patients who received surgical management, 1 patient underwent surgery due to sigmoid colon perforation and 7 patients due to intractability of medical management. The perforated case received colon segmental resection and the other 7 patients recieved total protocolectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. One patient is still on medical management because of mild persistent hematochezia after surgery. The other operated patients are showing good prognosis without any management. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation is present. Total protocolectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.
Child* ; Cholangitis, Sclerosing ; Chronic Disease ; Colitis, Ulcerative* ; Colon ; Colon, Sigmoid ; Diagnosis ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; Humans ; Inflammatory Bowel Diseases ; Liver Failure ; Medical Records ; Pneumonia ; Prognosis ; Retrospective Studies ; Seoul ; Sepsis ; Steroids ; Ulcer*