A case of Ramsay Hunt syndrome, though more often seen at the ENT clinic because of the patients' concern with symptoms in the ear, was seen and treated at the Dermatologic Clinic of Chosun University. A 29 year old male patient presented earache, pinhead to rice sized erythematous vesicular eruption on the external auditory canal, auricle and superior auricular area, and facial paralysis, all involving the right side of the face, duration of which were 7 days, 5 days and 1 day respectiveIy. High doses of prednisolone alone made it possible to remit the otalgia, control the vesicular eruption and resolute the facial paralysis almost completely within 18 days in the order of their appearance, indicating that this is one of the more easily treated diseases for dermatologists, The 22 cases of this syndrome reported in Korean literature were reviewed and the conceptual development of its pathogenesis and definition was described.
Adult ; Ear ; Ear Canal ; Earache ; Facial Paralysis ; Herpes Zoster Oticus* ; Humans ; Male ; Prednisolone

Percutaneous balloon angioplasty has been reported to be useful in the treatment of intracranial atherosclerotic arterial stenosis. However, arterial dissection with increased risk of acute closure and stroke has limited its widespread implementation. Stenting of the intracranial vasculature recently has been shown to be feasible in a variety of circumstance. However, stenting of middle cerebral artery has been limited because of difficulty with tracking stents across the carotid siphon. We report a case of successful percutaneous stenting of a symptomatic middle cerebral artery stenosis using a balloon-expandible flexible coronary stent.
Angioplasty ; Angioplasty, Balloon ; Constriction, Pathologic* ; Middle Cerebral Artery* ; Stents* ; Stroke

A statistical observation was made on 448 in-patients in the Department of Urology Catholic Medical College, during the period from the January 1, 1976 to December 31, 1977.
Urology

Carcinoid tumors are low-grade malignant tumors arising from neuroendocrine cells. Primary renal carcinoid tumors are extremely rare, and only 56 cases have been reported in the literature. Because of the rarity of the lesion, its histogenesis and prognosis are unclear. Here we report a case of a primary renal carcinoid tumor in a 51-year-old man that was found incidentally in a medical examination and was treated by transperitoneal radical nephrectomy.
Carcinoid Tumor ; Humans ; Kidney ; Middle Aged ; Nephrectomy ; Neuroendocrine Cells ; Prognosis

PURPOSE: To evaluate the radiologic findings of carotid-vertebrobasilar anastomosis and associated anomalies. MATERIALS AND METHODS: Thirteen patients with fourteen cases of carotid-vertebrobasilar anastomosis collectedfrom January 1992 to December 1997 were reviewed. Clinical diagnosis refered for cerebral angiography werecerebral infarction(n=3), intracranial hemorrhage(n=2), subarachnoid hemorrhage(n=1), brain tumor(n=3),arteriovenous malformation(n=3) and trigeminal neuralgia(n=1). Cerebral angiograms and clinical symptoms wereretrospectively analyzed. RESULTS: The fourteen carotid-vertebrobasilar anastomsis consisted of eleven cases ofpersistent trigeminal artery and three cases of type I proatantal intersegmental artery. Persistent trigeminalarteries were associated with anterior communicating artery aneurysm(n=1), posterior fossa arteriovenousmalformation(n=2) and persistent trigeminal artery variant(n=5). Type I proatantal intersegmental arteries wereassociated with hypoplastic vertebral arteries(n=2) : only proximal segment in one, and proximal and distalsegments in one case. Only one patient had clinical symptom related to the carotid-vertebrobasilar anastomosiswhich was trigeminal neuralgia. CONCLUSION: Knowledge of the anatomical and radiologic findings ofcarotid-vertebrobasilar anastomosis and associated anomalies will aid in the accurate diagnosis of neurovasculardisease and prevent possible complications during surgical and interventional procedures.
Arteries ; Brain ; Cerebral Angiography ; Diagnosis ; Humans ; Trigeminal Neuralgia

A case of fatal hematemesis due to an aberrant right subclavian arterioesophageal fistula which is a rare complication of the vascular ring is presented. A 42-year-old man with multiple injury by traffic accident presented severe upper gastrointestinal bleeding and was taken emergent operation. He was keeping tracheostomy tube and nasogastric tube for 7 weeks. We could find an aberrant right subclavian arterioesophageal fistula through left thoracotomy which was made by irritation of the prolonged nasogastric tube. We carried division of the aberrant right subclavian artery and fistulectomy. He was doing well postoperatively. But massive bleeding occurred at the fifth postoperative day. We performed emergent reoperation at CCU and found the tear point on the suture site of the aorta, which might be developed due to irritation of the chest tube and/or infection of the surrounding tissues. He was expired at the 8th postoperative day due to ischemic brain damage.
Accidents, Traffic ; Adult ; Aorta ; Brain ; Chest Tubes ; Fistula* ; Hematemesis ; Hemorrhage ; Humans ; Multiple Trauma ; Reoperation ; Subclavian Artery* ; Sutures ; Thoracotomy ; Tracheostomy

BACKGROUND: Myotonic dystrophy type 1 (DM1) is an autosomal-dominant muscular dystrophy caused by expansion of cytosine-thymine-guanine (CTG) trinucleotide repeats in the myotonic dystrophy protein kinase (DMPK) gene. The clinical features of DM1 are multisystemic and highly variable, and the unstable nature of CTG expansion causes wide genotypic and phenotypic presentations. The aim of this study was to characterize the molecular and clinical spectra of DM1 in Koreans. METHODS: The CTG repeats of 283 Korean individuals were tested by PCR fragment analysis and Southern blot. The following characteristics were assessed retrospectively: spectrum of CTG expansions, clinical findings, genotype-phenotype correlation, anticipation, and genetic instability. RESULTS: One-hundred twenty-four patients were confirmed as DM1 by molecular tests, and the CTG expansions ranged from 50 to 2,770 repeats (median 480 repeats). The most frequent clinical features were myotonia, muscular weakness, and family history. Patients with muscular weakness or dysfunction of the central nervous system harbored larger CTG expansions than those without each symptom (P<0.05). The age of onset was inversely correlated with the size of the CTG expansion (gamma=-0.422, P<0.001). The instability of CTG expansion representing as the maximum difference between sibships was observed from 50 to 700 repeats in nine families. Clinical anticipation and the increase in CTG repeat were significantly higher in maternally transmitted alleles (P=0.002). CONCLUSIONS: Molecular genetic tests are not only essential for diagnosis, but also helpful for suggesting the spectrum and relationship between genotype and phenotype in Korean DM1 patients.
Blotting, Southern ; Data Interpretation, Statistical ; Female ; Genotype ; Humans ; Korea ; Male ; Myotonic Dystrophy/*diagnosis/*genetics ; Pedigree ; Phenotype ; Polymerase Chain Reaction ; Protein-Serine-Threonine Kinases/genetics ; Retrospective Studies ; Trinucleotide Repeat Expansion/genetics

BACKGROUND: Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. METHODS: The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. RESULTS: The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1+/-1.9 g/dL, mean corpuscular volume was 93.4+/-11.6 fL, and mean number of reticulocytes was 19,700/mm3. The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). CONCLUSION: The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.
Anemia ; Anemia, Diamond-Blackfan ; Aortic Coarctation ; Bone Marrow ; Congenital Abnormalities ; Diamond ; Erythrocyte Indices ; Heart Septal Defects, Ventricular ; Hemoglobins ; Hospitals, University ; Humans ; Incidence ; Korea ; Male ; Medical Records ; Registries ; Reticulocytes ; Retrospective Studies ; Steroids ; Strabismus ; Thumb ; Transplants

PURPOSE: Wilms tumor is the most common malignant renal tumor in children. We investigated the epidemiology, clinical features and treatment outcome of the children with Wilms tumor in Korea during the recent 10 years. METHODS: Two hundred forty six patients were enrolled between January 1991 and December 2000 from 26 major hospitals in Korea. The data regarding the clinical features including sex, age, pathologic type, prognostic factor and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed, The differences between groups were analyzed by log-rank test. RESULTS: There were 130 males and 116 females. The incidence between the age of 1~4 years was the highest with 66.2%. The annual incidence rate per 1, 000, 000 population varied from 1.9 to 2.1. The 10 years overall survival rate according to sex, clinical stage, pathologic type and relapse were as follows: 88.6% in male, 90.9% in female, 100% in stage I, 94.7% in stage II, 92.1% in stage III, 63.4% in stage IV, 85.7% in stage V, 95.3% in favorable histology, 64.1% in unfavorable histology, 94.8% in non-relapse, and 40.9% in relapse. The relapse rate was 12%. The 10 years overall survival rate of 246 patients were 89.1%. CONCLUSION: Our results could provide the most recent and important clinical information on Wilms tumor of children in Korea.
Child ; Epidemiology* ; Female ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Male ; Medical Records ; Recurrence ; Retrospective Studies ; Survival Rate ; Treatment Outcome ; Wilms Tumor*

PURPOSE: Acute lymphoblastic leukemia (ALL) accounts for approximately 75% of all cases of childhood leukemia. We investigated epidemiology, clinical and laboratory features and treatment outcome of the children with ALL in Korea during recent 5 years. METHODS: One thousand forty nine patients were enrolled between January 1994 and December 1998 from 37 major hospitals in Korea. The data regarding the clinical and laboratory features including age, WBC counts at diagnosis, immunophenotype, morphology, cytogenetics and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed. The differences between groups analyzed by log-rank test. RESULTS: There were 597 males and 452 females. The distribution between the age 2 and 5 years is most common in 46.1%. The annual incidence rate per 100,000 population varied from 1.6 to 2.2. The 5 year event free survival (EFS) rates according to good prognostic factors were as follows: 67% bet ween 1-9 year of age at diagnosis, 69% in under 10,000/mm3of initial WBC count, 74% in early pre-B cell CALLA ( ) immunophenotype, 65% in L3 morphology, 68% in no CNS invasion. Most of patients were treated by CCG treatment protocol. The 5 year EFS was 63%. Main complications were sepsis (21.8%) and hemorrhage (12.5%). The relapse rate was 15.6%. The common causes of death were sepsis, DIC, pneumonia, relapse. CONCLUSION: Our results could provide the most recent and important information about acute lymphoblastic leukemia of children in Korea.
Cause of Death ; Child ; Clinical Protocols ; Cytogenetics ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Epidemiology* ; Female ; Hemorrhage ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Leukemia ; Male ; Medical Records ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Recurrence ; Retrospective Studies ; Sepsis ; Treatment Outcome