A female newborn had the following characteristics; a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestation, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. As the review of the literatures, congenital localized absence of skin has been observed in various subsets of inherited E.B. But, we believe that the term Bart's syndrome should be used to identify patients with good prognosis as the Bart's initial description To the best of our knowedge, this is the first reported case of Bart's syndrome in the korean literature.
Blister ; Cicatrix ; Congenital Abnormalities ; Female ; Humans ; Infant, Newborn ; Inheritance Patterns ; Lower Extremity ; Mucous Membrane ; Prognosis ; Skin

No abstract available.
Family Practice* ; Humans ; Smoke* ; Smoking*

No abstract available.
Adult* ; Humans

A 7 day old female baby suffered from perinatal asphyxia and trauma at birth. Subsequently, she had a violeceous subcutaneous plaque on back. The clinical setting, the skin manifestation and the histologic findings of the lesion were diagnostic of subcutaneous fat necrosis of the newborn. Histologic findings revealed fat necrosis with infiltration of inflammatory cells, foreign body giant cells and several fat cells contain needle shaped cleft in radial arrangement. Four weeks latter, the skin lesion was healed spontaneously.
Adipocytes ; Asphyxia ; Fat Necrosis ; Female ; Giant Cells, Foreign-Body ; Humans ; Infant, Newborn* ; Necrosis* ; Needles ; Parturition ; Skin ; Skin Manifestations ; Subcutaneous Fat*

PURPOSE: Parathyroid injury is the major cause of post-thyroidectomy hypocalcemia. This prospective study aimed to define the relation between the preservation status of the parathyroid glands and post-thyroidectomy hypocalcemia as well as to determine whether the number of preserved parathyroid glands could be a guideline for the management of post-thyroidectomy hypocalcemia. METHODS: We analyzed a total of 76 cases of total thyroidectomy due to malignancy were analysed. Parathyroid gland status at the end of thyroidectomy was classified as preserved intact, discolored (color change or equivocal viability), or autotransplanted. Patients were grouped according to the number of intact preserved parathyroid glands into group 1 (more than two), group 2 (one), or group 3 (zero). The total and ionized serum calcium levels of each group were monitored for three days after surgery. RESULTS: The number of intact preserved parathyroid glands was not found to be closely related to serum calcium level, although ionized serum calcium level was significantly lower in group 1 than group 2 (P<0.001). Hypocalcemia was not found in group 1. Hypocalcemia was found in 31% (10/32) of group 2. Among the 32 patients of group 2, all 4 parathyroid gland were identified in 9 patients and 4 patients were shown to be hypocalcemic. Therefore, the incidence of hypocalcemia for patients in whom only one intact parathyroid was preserved and all 4 parathyroid were indentified was 44% (4/9). Hypocalcemia was transient in all patients. CONCLUSION: The parathyroid glands should be preserved as much as possible to minimize post-thyroidectomy hypocalcemia. The number of intact preserved parathyroid glands can be a good guideline for predicting post-thyroidectomy hypocalcemia and proper care management.
Calcium ; Humans ; Hypocalcemia* ; Incidence ; Parathyroid Glands ; Prospective Studies ; Thyroidectomy*

No abstract available.
Humans ; Neutrophils* ; Phagocytosis* ; Stomach Neoplasms*

PURPOSE: Kawasaki disease is notorious for coronary arterial complication which is usually developed as a febrile disease in early childhood. Increased polymorphonucleus(PMN) cell levels in acute phases may be associated with the pathophysiology of Kawasaki disease. We studied the relationship between coronary arterial dilatation and elastase activity which was excreted from PMN cell and roles as an important factor for vasculitis. METHODS: Ten patients diagnosed with Kawasaki disease in Yonsei University Medical Center were examined between November, 2001 and January, 2002. In addition, 15 patients with other febrile diseases were also examined. Echocardiography was done in patients with Kawasaki disease on the first day of admission and four weeks after the onset of the disease. At each time, venous samples were drawn and separated into plasma and leukocytes. In patients with other febrile disease, samples were drawn on admission. Elastase activities in plasma and neutrophil extracts were measured. RESULTS: The significant increased plasma elastase activity, 6.19+/-0.74 U/mL, found in Kawasaki disease patients compared with the other febrile disease patients, 4.86+/-1.17 U/mL(P<0.05). And there was no significance between the above two diseases in terms of the elastase activity in neutrophil extracts. The relationship between initial elastase activity and the coronary arterial complication which was shown in subacute phase wasn't significant. CONCLUSION: Plasma elastase activity was increased in Kawasaki disease significantly, but the initial plasma elastase activity in the acute phase could not reflect the range of coronary arterial complication.
Academic Medical Centers ; Dilatation* ; Echocardiography ; Humans* ; Leukocyte Elastase* ; Leukocytes ; Mucocutaneous Lymph Node Syndrome* ; Neutrophils* ; Pancreatic Elastase ; Plasma ; Vasculitis

A statistical analysis of the diagnostic value for 244 aspiration biopsy cytology(ABC) among a total 1,043 cases from various sites was performed. ABC, using diagnostic terminology similar to that of a surgical pathology reports, was compared to the final tissue diagnosis. For the entire series, a sensitivity of 91.8%, a specificity of 99.3%, a positive predictive value of 98.9%, a negative predictive value of 94.8%, and an efficacy of the test of 96.3% were shown. There were 8 false negative and 1 false positive diagnosis. The diagnostic accuracy was 89.8%. Those results indicate that the ABC is a considerably highly accurate procedure that should be routinely employed.
Biopsy, Needle ; Child* ; Diagnosis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Intranuclear Inclusion Bodies ; Meningioma ; Pathology, Surgical ; Sensitivity and Specificity

PURPOSE: This study was done to investigate the prevalence of job stress and musculoskeletal symptoms, and to identify the factors that affect work-related musculoskeletal symptoms of the 119 Emergency medical technicians (EMT). METHOD: From August 26 to September 10, 2010, the data were obtained from 456 EMT working in Daejeon city or Choongnam province. For data analysis, descriptive statistics and multiple logistic regression were performed using SAS version 9.1. RESULTS: Physical environment, job demand, interpersonal conflict, and occupational climate were stressful to 119 EMT. The prevalence of musculoskeletal symptoms of female EMT was higher than those of the male EMT's. After gender and age were adjusted, there ware significant relationships between musculoskeletal symptoms and some risk factors including occupational class, hours of intensive musculoskeletal use, previous injury or work-related injury, physical burden, and job stress. Overall, a higher degree of job stress increased musculoskeletal symptoms. CONCLUSION: Job stress is a major cause of musculoskeletal symptoms. To prevent and manage musculoskeletal disease of 119 EMT, there is a need to develop a management program for musculoskeletal symptoms to reduce occupational stress, considering gender differences.
Climate ; Emergencies ; Emergency Medical Technicians ; Female ; Humans ; Logistic Models ; Male ; Musculoskeletal Diseases ; Phenothiazines ; Prevalence ; Risk Factors ; Statistics as Topic

To Evaluate the clinical characteristics of childhood-onset systemic lupus erythemoatosus (SLE) and analyse the factors related to outcome of renal function in lupus nephritis, we reviewed medical records of 18 cases of SLF diagnosed at the Department of Pediatrics. Pusan National University Hospital from January 1981 to December 1990. The results were as follow: 1)Male to female ratio was 1:2.6. 2) Nephropathy was the most common initial impression, and 8 cases were diagnosed as SLE at first. 3) The clinical menifestation. was ANA (+), malar rash, renal disorder, hematologic disorder in frequency. 4) Diffuse proliferative lupus nephritis was the most common in renal histopathologic studies. 5) Among the follow-up cases, active lupus was 39%, remission was 28%. 6) As factors related to outcome of lupus nephrits were, hypertension and proteinuria were siginificant statistically.
Busan ; Exanthema ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Medical Records ; Pediatrics ; Proteinuria