BACKGROUND: In epidermolysis bullosa acquisita, it has been recognized that there exists heterogeneity in the clinical and serologic/immunopathologic features. OBJECTIVE: We examined patients with epidermolysis bullosa acquisita to see if there were any associated clinical and serological features which may predict disease activity or prognosis in the disease. METHODS: Clinical and some serologic features were compared. between 2 groups of patients with epidermolysis bullosa acquisita; one with complete remission of the symptoms and signs of the disease for more than 2 years and the other group with persistent disease activities of longer than 5 years.
Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Humans ; Population Characteristics ; Prognosis

PURPOSE: To determine the value of bilobate anterior epidrual extension(AEE) on MRI in differential diagnosis of tuberculous spondylitis, pyogenic spondylitis and malignant tumor. MATERIALS AND METHODS: SE TI-,T2- and Gd-DTPA enhanced Tl-weighted, sagittal and axial MR images of 39 patients(40 vertebral lesions), including 16 tuberculous spondylitis, 7 pyogenic spondylitis(8 lesions, consisting of 2 separate lesion of 1 patient), and 16 malignant vertebral tumors(15 metastases,1 lymphoma) with an AEE were reviewed. The frequency of bilobate AEE shown as double-convexity in the anterior epidural space on axial scans was evaluated in the above vertebral diseases. RESULTS: The bilobate AEE on the axial scans was seen in 12 out of the 16 tuberculous spondytitis(75%) and 1 out of 8 pyogenic spondylitis(13%), and 10 out of the 16 malignant tumor including 15 metastasis and 1 lymphoma(63%). CONCLUSION: The bilobate' ^EE of vertebral lesions is suggestive of tuberculous spondylitis or malignant tumor rather than pyogenic spondylitis. This pattern seems to be related with the preservation of the structures, including midline septurn, F~LL(Posterior longitudinal ligament), lateral membrane and fibrous membrane, limiting and surrounding the extension of the tuberculous spondylitis and malignant tumor, and with the early penetration or disruption of PLL, midline septurn, lateral & fibrous membranes in the pyogenic spondylitis.
Diagnosis, Differential ; Epidural Space ; Gadolinium DTPA ; Magnetic Resonance Imaging* ; Membranes ; Neoplasm Metastasis ; Spondylitis

Polyvesicular vitelline tumor of the ovary is an extremely rare variant of yolk sac tumor. We present a case of pure polyvesicular vitelline tumor in a 43-year-old woman. Light microscopy revealed a predominantly polyvesicular pattern embedded in mesoblastic stroma with the cysts showing two type of lining; tall columnar and cuboidal, or mesothelioid cells. The lining atypical cells showed occasional mitoses and intracytoplasmic PAS positive hyaline globules. In some areas, the cystic space contained a large amount of intraluminal hyaline material. Immunohistochemically, alpha- fetoprotein and alpha-1-antitrypsin were detected as a fine granular deposit in the cytoplasm of epithelial cells and hyaline globules. Electron microscopically, marked specialization of the vesicular lining cells suggested a differentiation toward gut structures and mature yolk sac.
Adult ; Cytoplasm ; Endodermal Sinus Tumor ; Epithelial Cells ; Female ; Fetal Proteins ; Humans ; Hyalin ; Microscopy ; Mitosis ; Ovary* ; Vitellins* ; Yolk Sac

Polyvesicular vitelline tumor of the ovary is an extremely rare variant of yolk sac tumor. We present a case of pure polyvesicular vitelline tumor in a 43-year-old woman. Light microscopy revealed a predominantly polyvesicular pattern embedded in mesoblastic stroma with the cysts showing two type of lining; tall columnar and cuboidal, or mesothelioid cells. The lining atypical cells showed occasional mitoses and intracytoplasmic PAS positive hyaline globules. In some areas, the cystic space contained a large amount of intraluminal hyaline material. Immunohistochemically, alpha- fetoprotein and alpha-1-antitrypsin were detected as a fine granular deposit in the cytoplasm of epithelial cells and hyaline globules. Electron microscopically, marked specialization of the vesicular lining cells suggested a differentiation toward gut structures and mature yolk sac.
Adult ; Cytoplasm ; Endodermal Sinus Tumor ; Epithelial Cells ; Female ; Fetal Proteins ; Humans ; Hyalin ; Microscopy ; Mitosis ; Ovary* ; Vitellins* ; Yolk Sac

A review has been made of mortality trends in Korea from 1958 to 1907 analyzing the data by sex, age and cause of death. The crude death rates and age specific death rates were estimate6 by the model of N. Keyfitz life table which had teen developed by the data of the 1960's national census. The cause specific death rates shown in this article are based on the following: all deaths occurring in the death-registration are expressed as a numberator, while the denominator was estimated from the regular national census data by interpolation method. It is estimated that only an average of about 40% of deaths which occurred during a year were registered during 1958 to 1967. The validity and the reliability of the diagnosis of causes of death seem to be extremely poor in this country. Therefore the cause specific death rates in this article are aimed to reveal trends of causes of registered death and not for the actual level of death rates. For 10 years very interesting mortality trends were observed : 1. The trend in the crude death rates was downward slowly. 2. The estimated death rate for the infant in 1960 was still high up to 100 per 1,000. 3. The rates for mortality attributed to such infectious diseases as pneumonia, bronchitis, gastroenteritis and measles decreased an average 40-60%. 4. The death rates for over-all tuberculosis decreased only 9.8%. 50% of the decrease was contributed by those in the less-than-15 year age group. 5. The death rates for chronic diseases, such as vascular diseases affecting the central nervous system, malignant neoplasm, major heart diseases and all accidents rose about 40-60%. 6. The rank order of the 10 leading causes of death showed large changes over the years, except for pneumonia and tuberculosis which occupyed 1st and End places respectively. Vascular diseases affecting the central nervous system moved from 5th to 3rd place and malignant neoplasm from 6th to 4th palace. The major heart diseases moved from 10th to 6th place and all accidents from 10th to 7th place. On the other hand, gastroenteritis moved from 3rd to 5th place and influenza from 4th to 8th place.
Adolescent ; Bronchitis ; Cause of Death ; Censuses ; Central Nervous System ; Chronic Disease ; Communicable Diseases ; Diagnosis ; Gastroenteritis ; Hand ; Heart Diseases ; Humans ; Infant ; Influenza, Human ; Korea* ; Life Tables ; Measles ; Mortality* ; Pneumonia ; Tuberculosis ; Vascular Diseases

No abstract available.
Base Sequence* ; DNA, Complementary* ; Hantaan virus*

A case oof erythema elevatum diutinum in 30-year old male was described. Reviewing literatures, this disease is very rare and is the first case to be reported in Korea. There were persistant elevated hard brownish red nodulo-infiltrated plaques which were symmetrically distributed especia]ly over the shins, elbows, knees, ear lobes, buttocks, and dorsal surfaces of the hands, feet, fingers, and toes, accompanied by occasional mild itching. These skin eruption were started from the erythematous papulo-vesicular macules which developed on the left shin and wrist about 7 years previously. He has also had a long-standing encapsulated pleural effusion with marked pleural thickening in left lower chest which was diagnosed 3 years ago. The serum IgG was noted to be increased. Histological examination of skin lesions revea]ed leucocytoclastic angiitis and sevrer perivascular fibrosis in older lesions.
Adult ; Buttocks ; Ear ; Elbow ; Erythema* ; Fibrosis ; Fingers ; Foot ; Hand ; Humans ; Immunoglobulin G ; Knee ; Korea ; Male ; Pleural Effusion ; Pruritus ; Skin ; Thorax ; Toes ; Vasculitis ; Wrist

PURPOSE: Chronic lung disease is a cause of significant morbidity and mortality in the neonatal respiratory distress syndrome. Although the exact mechanisms leading to the development of chronic lung disease are unknown, there is increasing evidence indicating that pulmonary inflammation contributes to the pathogenesis. The proinflammatory cytokines, interleukin(IL)-1beta, IL-8 may have a role to play in initiating the inflammatory response that is seen in chronic lung disease. In contrast, IL-10 is an antiinflammatory cytokine. The abnormal persistent inflammatory process in chronic lung disease may be due to dysregulation of these proinflammatory cytokines. So, to describe cytokine profiles of chronic lung disease in neonate, we analysed the IL-1beta, IL-8 and IL-10 levels in bronchoalveolar lavage fluid by ELISA technique. METHODS: We obtained bronchoalveolar lavage samples from 14 respiratory distress syndrome infants without chronic lung disease and 8 respiratory distress syndrome infants with chronic lungisease. The cytokine levels were assayed. RESULTS: The results were as follows: 1) IL-1beta levels were higher in chronic lung disease group(205.16+/-360.21pg/rnl) than group without chronic lung disease(21.21+/-26.14pg/ml)(P<0.05). 2) IL-8 levels were also higher in chronic lung disease group(166.16+/-347.26pg/ml) than group without chronic lung disease(49.85+/-105.27pg/ml)(P>0.05). 3) In contrast, IL-10 levels were lower in chronic lung disease group(78.29+/-138.69pg/ml) than group without chronic lung disease(111.29+/-155.27pg/ml)(P>0.05). CONCLUSION: These results suggest that the dysregulation of cytokines contributes to the pathogenesis of chronic lung disease. The difference in cytokine levels of bronchoalveolar lavage fluid can be used as a predictor for development of chronic lung disease.
Bronchoalveolar Lavage Fluid* ; Bronchoalveolar Lavage* ; Cytokines* ; Enzyme-Linked Immunosorbent Assay ; Humans ; Infant ; Infant, Newborn ; Interleukin-10 ; Interleukin-8 ; Lung Diseases* ; Lung* ; Mortality ; Pneumonia ; Respiratory Distress Syndrome, Newborn

Maternal phenylketonuria(PKU) is a disorder which appears when a pregnant woman with PKU doesn't take low phenylalanine diet, phenylalanine level of the fetus rises and interferes with fetal development and can easily result in abortion, low birth weight, microcephaly, cardiac anomaly or mental retardation. To prevent these effects of untreated maternal PKU, low phenylalanine diet should be taken to maintain the serum phenylalanine level less than 10mg/dl throughout the pregnancy. We experienced a case of maternal PKU in a female baby born to a 26-year-old woman with PKU who didn't take low phenylalanine diet until eighth moth of pregnancy. She showed intrauterine growth retardation, ventricular septal defect and patent ductus arteriosus. We report a case of maternal PKU with a brief review.
Adult ; Diet ; Ductus Arteriosus, Patent ; Female ; Fetal Development ; Fetal Growth Retardation ; Fetus ; Heart Septal Defects, Ventricular ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Intellectual Disability ; Microcephaly ; Moths ; Phenylalanine ; Pregnancy ; Pregnant Women

No abstract available.
DNA*