The occurence of pseudarthroses associated with neurofibromatosis is a well documented phenomenon, with the majority of reported cases in the tibia but quite rare in forearm,Only ten cases in the English-literature and one case in Korean-literature of this lesion revealed appearing in the bones of the forearm till now. Of these, four in solitary pseudarthroses of the ulna(4.5.15), five in a solitary lesion of the radius(7.8.10.16.17), and only two cases was on the both bones of the forearm(9.14). We are presenting the case report of a Korean adult male with the clinical stigmata and a positive familial history of neurofibrormotosis who had congenital pseudarthroses of both the radius and ulna of the right forearm. Therefore, the present case is the third to be reported. We treated him with by production of an one-bone forearm using a dual a onlay bone graft positioned in neutral of supination and pronation. A stable, functional extremity with good union of pseudarthroses were encountered.
Adult ; Christianity ; Extremities ; Forearm ; Humans ; Inlays ; Male ; Neurofibromatoses ; Neurofibromatosis 1 ; Pronation ; Pseudarthrosis ; Radius ; Supination ; Tibia ; Transplants ; Ulna

PURPOSE: To compare photon thunderbird with deep match (technique 1) with 3-field technique with electron inguinal boost (technique 2) in acute skin toxicity, toxicity-related treatment breaks and patterns of failure in elective inguinal radiation therapy (RT) for curative chemoradiation in anal cancer. MATERIALS AND METHODS: Seventeen patients treated between January 2008 and September 2010 without evidence of inguinal and distant metastasis were retrospectively reviewed. In 9 patients with technique 1, dose to inguinal and whole pelvis area was 41.4 to 45 Gy and total dose was 59.4 Gy. In 8 patients with technique 2, doses to inguinal, whole pelvis, gross tumor were 36 to 41.4 Gy, 36 to 41.4 Gy, and 45 to 54 Gy, respectively. The median follow-up period was 27.6 and 14.8 months in group technique 1 and 2, respectively. RESULTS: The incidences of grade 3 radiation dermatitis were 56% (5 patients) and 50% (4 patients), dose ranges grade 3 dermatitis appeared were 41.4 to 50.4 Gy and 45 to 54 Gy in group technique 1 and 2, respectively (p = 0.819). The areas affected by grade 3 dermatitis in 2 groups were as follow: perianal and perineal areas in 40% and 25%, perianal and inguinal areas in 0% and 50%, and perianal area only in 60% and 25%, respectively (p = 0.196). No inguinal failure has been observed. CONCLUSION: Photon thunderbird with deep match technique and 3-field technique with electron inguinal boost showed similar incidence of radiation dermatitis. However, photon thunderbird with deep match seems to increase the possibility of severe perineal dermatitis.
Anus Neoplasms ; Chemoradiotherapy ; Dermatitis ; Electrons ; Follow-Up Studies ; Humans ; Incidence ; Neoplasm Metastasis ; Pelvis ; Retrospective Studies ; Skin

No abstract available.
Fetus* ; Humans ; Pregnancy, Twin* ; Twins*

No abstract available.
Fetus* ; Humans ; Pregnancy, Twin* ; Twins*

PURPOSE: We reviewed the treatment outcomes and prognostic factors for patients with anal canal carcinoma who were treated with curative intent chemoradiotherapy (CRT) at Severance Hospital from 2005 to 2011. MATERIALS AND METHODS: Data for 38 eligible patients treated during this period were reviewed. All patients were treated with curative intent using radiotherapy (RT) with (n = 35) or without concomitant chemotherapy (n = 3). Among 35 patients who received CRT, most of the chemotherapeutic regimens were either 5-fluorouracil (5-FU) plus mitomycin C (23 patients) or 5-FU plus cisplatin (10 patients). Recurrence-free survival (RFS), colostomy-free survival (CFS), overall survival (OS), and locoregional control (LRC) rates were calculated using the Kaplan-Meier method and survival between subgroups were compared using the log-rank test. Cox's proportional hazard model was used for multivariate analysis. RESULTS: Over a median follow-up period of 44 months (range, 11 to 96 months), 3-year RFS, CFS, OS, and LRC were 80%, 79%, 85%, and 92%, respectively. In multivariate analysis, tumor size >4 cm was an independent predicting factor for poorer RFS (hazard ratio [HR], 6.35; 95% confidence interval [CI], 1.42 to 28.5; p = 0.006) and CFS (HR, 6.25; 95% CI, 1.39-28.0; p = 0.017), while the presence of external iliac lymph node metastasis was an independent prognosticator for poorer OS (HR, 9.32; 95% CI, 1.24 to 70.3; p = 0.030). No treatment-related colostomies or deaths occurred during or after treatment. CONCLUSION: Curative intent CRT resulted in excellent outcomes that were comparable to outcomes in previous randomized trials. No severe treatment-related toxicities were observed.
Anal Canal ; Anus Neoplasms ; Carcinoma, Squamous Cell ; Chemoradiotherapy ; Cisplatin ; Colostomy ; Fluorouracil ; Follow-Up Studies ; Humans ; Lymph Nodes ; Mitomycin ; Multivariate Analysis ; Neoplasm Metastasis ; Proportional Hazards Models

Erythromelanosis follicularis faciei et coli is a pigmentary disorder of unknown cause originally described by Kitamura et al in 1960. We describe two cases of erythromelanosis follicularis faciei et colli occured in adolescence. Clinical features of these patients were consistent with the clinical changes described in previous cases having clinical triad of erythema, hyperpigmentation and follicular papules. This pigmentary disorder may not be an uncommon disease, however it might be neglected from the concern of the dermatologists.
Adolescent ; Erythema ; Humans ; Hyperpigmentation

The May-Hegglin anomaly is a rare autosomal dominant trait characterized by platelet abnormalities in the peripheral blood and large (up to 5mu) pale blue staining inclusions in the cytoplasm of neutrophils, eosinophils, basophils, and monocytes. We experienced a case of May-Hegglin anomaly in a 7 year old male and another case out of his family. His initial complaints at the admission were petechiae and intermittent epistaxis. Diagnosis was confirmed by peripheral blood smear and family study. We report the case with brief review of related literature.
Basophils ; Blood Platelets ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Epistaxis ; Humans ; Male ; Monocytes ; Neutrophils ; Purpura

Various anatomical defects have been described in the surviving twin who had a stillborn, macerated monozygotic co-twin with Disseminated Intravascular Coargulation. The etiology is thought to be placental transfer of emboli or thromboplastic material through placental vascular anastomoses. We experienced a case of monozygotic twin with deceased co-twin at 30 weeks of gestation and confirmed to have antenatal periventricular germinal matrix and intraventricular hemorrhage, multicystic periventricular leukomalacia and diffuse encephalomalacia by neurosonography on first day of life despite of no clinical evidence of brain damage. The pathologic findings of placenta revealed infarct with massive fibrin deposition. A brief review of related literature is presented.
Brain* ; Encephalomalacia ; Fibrin ; Hemorrhage ; Humans ; Infant, Newborn ; Leukomalacia, Periventricular ; Placenta ; Pregnancy ; Twins ; Twins, Monozygotic*

No abstract available.
Hepatitis*

Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogrens syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.
Arthritis, Rheumatoid ; Esophageal Motility Disorders ; Humans ; Hypertension, Pulmonary* ; Liver Cirrhosis, Biliary* ; Lupus Erythematosus, Systemic ; Scleroderma, Diffuse* ; Sjogren's Syndrome ; Thyroiditis, Autoimmune