The clinical symptom complex characterized by syncopal attacks and sudden death in patients with electrocardiographic anomalies, especially a prolonged Q-T interval is known as the Romano-Ward syndrome. When a similar symptom complex is accompanied by congenital deafness, it is called Jervell and Lange-Nieisen syndrome. This study was undertaken to investigate the investigate the incidence of the latter syndrome in the Seoul area. A total of 1,013 children with congenital deafness who attend schools for the deaf were studied by analyzing their electrocardiograms. A corrected Q-T interval of> or =0.44 seconds was defined as a prolonged Q-T interval. The overall incidence of long Q-T syndrome observed in the deaf in the Seoul area was 0.49% as compared to 0.25% reported from elsewhere in the world. The mean age of patients with long Q-T syndrome was 11.3+/-5.9 years and the first syncopal attacks was 4.6+/-1.1 per patient. A valsalva maneuver and exercise tests in the test group were associated with significant changes in T wave configuration and prolonged Q-T intervals while similar changes were not observed in the control group. A larger survey is needed to derive more statistically significant conclusions.
Child* ; Deafness* ; Death, Sudden ; Electrocardiography ; Exercise Test ; Humans ; Incidence ; Romano-Ward Syndrome ; Seoul ; Valsalva Maneuver

Endotheline-1 (ET-1) is a potent vasoconstrictor peptide with 21 amino acid residues. ET-1 is thought to have a key role in vasoconstriction, and cardiac, renal, and endocrine actions of the peptide. Creatine Kinase(CK), also referred to as ATP-creatine-N-phosphotransferase, consists of either the B or M type. CK is found almost exclusively in muscle(MM), myocardium(MB), and brain(BB). It is to be an almost specific index of injury of myocardium and brain in hypoxic damage. I measured the ET-1, CK isoenzyme in neonates among asphyxia group (14 cases), as a control devide into two group. 1st group(birth weight (2500 g, n=9) and 2nd group (body weight 2500g, n=11). 1) There was no significant difference between asphyxia and control 1st, 2nd group during postnatal 24 hrs include cord blood on irET-1(p< 0.05). 2) There was significant difference between asphyxia and control 1 st, 2nd group during postnatal 24 hrs include cord blood on CK and CK and CK isoenzyme (p<0.001). 3) There was significant difference between asphyxia and control 1st, 2nd group on CK-MB and CK-BB proportion (p< 0.001). 4) There was significant difference between cord blood and postnatal 24 hrs on total CK level (P< 0.001).
Asphyxia* ; Brain ; Creatine Kinase* ; Creatine* ; Fetal Blood ; Humans ; Infant, Newborn* ; Myocardium ; Plasma* ; Vasoconstriction

No abstract available.
Heart Diseases* ; Heart*

Paraffinoma is a well known complication of previous paraffin injection into the subcutaneous layer that presents as various conditions including firm mass formation, edema, induration, ulceration, and skin necrosis. Paraffinoma can mimic neoplasm on physical examination and imaging studies and may complicate ultrasonographic diagnoses due to typical posterior shadowing and high echogenicity. When paraffinomas involve around the thyroid gland, the diagnosis of thyroid tumors is very difficult. We present a case of thyroid cancer, the evaluation of which was complicated by the presence of cervical paraffinoma.
Edema ; Hydrazines ; Necrosis ; Paraffin ; Physical Examination ; Shadowing (Histology) ; Skin ; Thyroid Gland ; Thyroid Neoplasms ; Ulcer

Congenital origin intestinal obstruction are important disease due to required emergency operation. So accurate and rapid diagnosis needed for decreased mortality and morbidity. Radiologic must defect to accurate obstruction site and also associated other congenital anomalies. And also embryological basis are very important role to the diagnosis of these diseases. We were analyzed radiologically and clinically 25 cases with congenital origin intestinal obstruction with review of literature. 1. Hypertrophic pyloric stenosis 6 cases, midgut malrotation 4 cases, congenital megacolon 8 cases, imperforated anus 5 cases, ileal atresia 1 case and duodenal atresia 1 case. 2. Male and female radio was 16:9. Especially on hypertrophic pyloric stenosis, 5 cases were male infants. 3. All cases of hypertrophic pyloric stenosis represented string sign and also pyloric beak sign, shoulder sign on UGI. 4. 1 case duodenal atresia showed double bubble sign on simple abdomen x-ray and ileal atresia showed mechanical small bowel obstruction sign with microcolon. 5. On midgut malrotaton, cecum was located in right upper abdomen on 4 cases. And 2 cases were associated with Ladd's band, 1 case with volvulus and 1 case with mesenteric defect. 6. Involved site of all congenital megacolon were localized to rectosigmoid colon. 7. On 5 cases imperforated anus, 3 cases were low type and 2 case high type. Rectoperitoneal and rectourogenital fistula were demonstrated on 4 cases.
Abdomen ; Anal Canal ; Animals ; Beak ; Cecum ; Colon ; Diagnosis ; Emergencies ; Female ; Fistula ; Hirschsprung Disease ; Humans ; Infant ; Infant, Newborn* ; Intestinal Obstruction* ; Intestinal Volvulus ; Male ; Mortality ; Pyloric Stenosis, Hypertrophic ; Shoulder

Several investigators have attempted to clarify th well-known phenomenon of anergy in lepromatous leprosy during past two decades, utilizing various methods of immunologic assessrvent, including response to skin test antigens, active skin sensitization with strong allergens, skin homograft survival rate, imrnunopathology of lymphnodes, in vitro blastogenic response by antigens or mitogens of lymphocytes, lymphokine production in vitro and measurement of peripheral T and B cell ratio. Howcver, there is no general agreement as to the cellular irnmunologic status of leprosy patients b tween various investigators. The present study was undertaken to evaluate the ability to mount cutaneous hypersensitivity reactions to various skin test antigens and to investigate active sensitization with DViCB in patients with leprosy. Ten polar lepromatous (LL) and 12 polar tuberculoid (TT) p-tients who have been treated at Department of Dermatology, National Iviedical Center and Seoul Nationa,l University Hospital were the subjects. The subjects have rcceived regular antileprosy chemotherapy with DDS and the average duration of treatment in LL and TT groups was 6. 2 and 4. 7 years, respectively. The control group included 10 healthy physicians and nurs-s. Skin test antigens includ=d lepromin (1 x10' bacilli,ml'), PPD (Parke-Davis 5ppJ/0.1ml), SK-SD (Lederle, 40 u SK and 10 u SD/0.1 ml), Candidin (Hollister stier Lab 1: 1000 dilution) and DNCB aceton solution in the concentrations of 1000ug,/0.1ml for sensitization and 100ug/0.1ml for challenge, respcctively. Skin reactions were read 48 hours after intraderrnal injection of 0.1 rnl of each antigen anci th.' metho4 of DNCB sensitization was same as described elsewhere. The result showed that in polar lepromatous leprosy patients, the skin reactivity to various antigens were generally decroased, as cornpared to both th healthy control group and polar tuberculoicl patients, especially to lepromin, PPD and I')NCB sensitization (p<0.05, respectively). We concluded thxt lepromatous leprosy patients were especially unresponsive to mycobacterial antigcns(lepromin and PPD) and to newly administered antigen (DNCB) and tbe possible mechanism was discussed.
Allergens ; Allografts ; Antigens ; Dermatology ; Dinitrochlorobenzene ; Drug Therapy ; Humans ; Hypersensitivity* ; Lepromin ; Leprosy* ; Leprosy, Lepromatous ; Lymphocytes ; Mitogens ; Research Personnel ; Seoul ; Skin Tests* ; Skin* ; Survival Rate

Anti-T cell monoclonal antibodies(OKT series: OKT4a OKT8A, OKT1 1) ir,amunoperoxidase technique studies were performed for the presence of T cells and their subpopulations in cutaneous lesions of early papules and late plaques from 10 patients with psoriasis. In this semiquantitative examination, T cells reactive to OKT11 antiserum constituted about 70 percents of total leukocytes found in the dermis, both in early papular lesions and late plaque lesions. The number of helper/inducer T cells was about equal to the number of suppressor/cytotoxic T cells in early papular lesions, but helper/inducer T cells were predominant in plaque lesions. Although in situ functions of T cell and their subpopulations can not precisely be deduced, this different proportions of the helper/inducer and suppressor/cytotoxic T cells found in this study might be related to the immunopathologic mechanisms pertainmg to the persistence and the expansion of psoriatic lesions.
Dermis ; Humans ; Leukocytes ; Psoriasis* ; T-Lymphocytes*

Bullous pemphigoid is a chronic and relatively benign subepidermal blistering disease and is generally considered not to be a sulfa-responsive dermatosis. As a rule, bullous dermatosis that excellently responded to sulfapyridine and sulfones was considered by some to be diagnostic of dermstitis herpetiformia. In 1977, Person and Rogers described 6 cases of bullous pemphigoid responded to sulfapyridine and sulfones. These sulfa-responsive cases were younger than ordinary bullous pemphigoid patients and the histopathologic findings showed prevalence of the neutrophilic infiltration. In this report, the patient who diagnosed as bullous pemphigoid by immunofluorescent studies has younger onset (38 years of age) than usual bullous pemphigoid and histopatbologic findings showed dense neutrophilic infiltrations. The patient was treated with 100-200mg of DDS for 2 months and the skin lesions completely disappeared.
Blister ; Humans ; Neutrophils ; Pemphigoid, Bullous* ; Prevalence ; Skin ; Skin Diseases ; Sulfapyridine ; Sulfones

To characterize the immunopathologic phenotype of the cells in the lesional ti.:sue and to further examine the mechanism of the development of the annular lesions in leprosy, we have studied immune cells (T lymphocyte and its subsets, Langerhans cells, and HLA-DR antigen expressing cells) at different anatomical sites inside, active border, and outside normal skin in the annular lesions of leprosy. We took biopsy specimens from 4 patients of BT type, then processed the specimens by the staining methods eif indirect immunoperoxidase with monoclonal antibodies. In the active border the number of T cell was over 50g of the total cells infiltrated in the dermis. Helper T cells were dominant in number, and about three fourths of the cells were positive for HLA-DR staining. In two patients they had expression of DR antigen on the surfaces of the keratinocytes in the epidermis, in contrast to that of the inside, even the intensities were not. strong. At the inside of the annular lesions T cells were about 40% and the ratio of helper/suppressor T cell was approxiinately 1: 1, However, HLA-DR positive immune cells were not more than 10g among the total infiltrates. Langerha,ns cells were increased in number and in size either in the border or at the inside of the annular lesions. With these results we presume that the T cell mediated imrnune responses against Mycobacterium leprae may play an important role in the formation and extension of the annular lesions in leprosy.
Antibodies, Monoclonal ; Biopsy ; Dermis ; Epidermis ; HLA-DR Antigens ; Humans ; Keratinocytes ; Langerhans Cells ; Leprosy* ; Lymphocytes ; Mycobacterium leprae ; Phenotype ; Skin ; T-Lymphocytes ; T-Lymphocytes, Helper-Inducer

PURPOSE: Actinomycosis is still a relatively rare infection, characterized by multiple abscesses, draining sinuses and the appearance of sulfur granules, which are valuable in aiding the diagnosing the discharge of involved tissues. In most instances, the onset of an abdominal disease is preceded by inflammatory or a traumatic incident resulting in the perforation of the mucosa of the gastrointestinal tract. Intensive and prolonged antimicrobial therapy, and wide surgical excision of involved tissues are the two general principles of therapy. METHODS: We experienced 8 cases of abdominal actinomycosis after a laparotomy between March 1997 and February 2002. RESULTS: The results were as follows: 1) There was a 1: 1 male to female ratio of abdominal actinomycosis, and a mean age of 47 years. 2) The clinical features were different for each involved organ, -but, most of the clinical symptoms were nonspecific to suspect actinomycosis. 3) The frequently involved organs were mainly located lower abdomen, such as the appendix and cecum, sigmoid colon and small bowel. 4) In 60% of the patients, the predisposing factors were identifiable, these being: a previous abdominal operation, IUD and abdominal injury. 5) The preoperative diagnoses included: acute appendicitis, and periappendiceal and intra-abdominal abscesses. The pre-exploratory diagnoses were made by ultrasound and abdominal CT. 6) Explorations were performed in all patients, depending on their diagnosis, to afford the proper surgical treatment and correct diagnosis. After the operation, all the patients were treated with oral antibiotics for long period. CONCLUSION: The authors conclude that pre-exploratory cytological or culture studies, with careful history taking, for low abdominal tumors or abscesses may increase the rate of correct diagnosis, as could proper explorations.
Abdomen ; Abdominal Abscess ; Abdominal Injuries ; Abscess ; Actinomycosis* ; Anti-Bacterial Agents ; Appendicitis ; Appendix ; Causality ; Cecum ; Colon, Sigmoid ; Diagnosis ; Female ; Gastrointestinal Tract ; Humans ; Laparotomy ; Male ; Mucous Membrane ; Sulfur ; Tomography, X-Ray Computed ; Ultrasonography