No abstract available.
Bartter Syndrome*

A synophthalmia, another form of cyclopia, in which the element of the two eyes are partially fused to form an apparently single eye in the middle of the forehead. The synophthalmia is a result of complex, neural plate misdevelopment syndrome involving the eye, brain, skull and face. It is well known that synophthalmia is due to heterogenous causes, most of which chromosomal imbalances. We experienced a case of synophthalmia associated with proboscis, alobar holoprosencephaly and chromosomal anomaly 46, XX, -15,t (15 q, 21 q). Diagnosis was confirmed by brain MRI and autopsy, The patient died about 20 hours of age and autopsy was done. A brief review of the literatures was also presented.
Autopsy ; Brain ; Diagnosis ; Forehead ; Holoprosencephaly ; Humans ; Magnetic Resonance Imaging ; Neural Plate ; Skull

Psoriasis is a disease caused by hyperproliferation of keratinocytes. Pathogenesis of psoriasis is still unclear, but many reports suggest that psoriatic keratinocytes themselves may have some factors of pathogenesis. The author developed artificial psoriatic skin by culturing keratinocytes of psoriasis skin over collagen lattice which was constructed with collagen and normal fibroblasts. After the keratinocytes had grown to full layers of stratification, expression patterns of differentiation marks and ultrastructural changes were investigated by immunohistochemistry and electron microscope. The results were very similar to those of psoriasis skin in vivo as follows. Cytokeratin (CK)10, marker of initiation of differentiation of keratinocytes, was expressed in the spinous layer. CK14, marker of basal cells of stratified squamous epithelium, was expressed in the basal and spinous layer. CK16 and CK17, markers of fast turnover of squamous epithelium, were expressed in the spinous layer. Involucrin, marker of terminal differentiation of squamous epithelium, was expressed weakely over the lower spinous layer. In immuno electron microscopical study, involucrin was expressed but confined to cornified cell envelops in the horney layer. Mitochondria, rER and ribosomes were abundant in the basal layer. They continued to appear in the upper spinous layer but intermediate filaments were scarce. Keratohyalin granules were visible in some parts of the granular layer zone, but the granules were smaller and fewer. In the horney layer, cells were thicker than normal and there were many lipid droplets within the cells. Intercellular spaces were enlarged at the basal layer but disappeared in the upper spinous layer. In these results, non systematic expression of differentiation markers and ultrastructural changes suggest that psoriasis is a disease caused by hyperproliferation of keratinocytes concurrent with unstable maturation and degeneration. Artificial psoriatic skin, in exclusion of systemic or dermal effects, showed very similar results with psoriasis skin in vitro. So it was concluded that psoriasis keratinocytes had some factors of pathogenesis and this kind of model on artificial psoriatic skin can be used for further studying of psoriasis.
Antigens, Differentiation ; Collagen ; Epithelium ; Extracellular Space ; Fibroblasts ; Immunohistochemistry ; Intermediate Filaments ; Keratinocytes ; Keratins ; Mitochondria ; Psoriasis* ; Ribosomes ; Skin ; Skin, Artificial

We made a clinical observation of 63 cases with miliary tuberculosis at the pediatric department of Hanyang University Hospital in Seoul, Korea, during the period from January, 1973 to September, 1979. The result were as folowing: 1. The incidence of miliary tubeculosis in children was 0.57%(63 cases/11,040) of total admission cases. The incidence was increased for the last 2 years, that is 0.54% in 1978 and 0.96% in 1979 respectively. 2. The highest incidence by age was noticed as 79.4% in the group under the age of 3 years with the 1 case of 2 months old. 3. The seasonal prevalence were winter and spring showing incidence of 63% 4. The source of infection was found in 49.2% of all cases, and majority (58.1%) of the infection was caused by their parents. 5. Only 9.5% of cases had received BCG innoculation. 6. The chief compalints on admission were revealed in the order of fever (71.4%), coughing (66.7%), vomiting (49.2%), irritability (25.4%), etc. 7. The principal clinical features on admission were marked emaciation (46.0%), abnormal neurologic sign (46.0%), hepatosplenobegaly (34.9%) in order. 8. The tuberculin skin test with 5TU PPD was noticed positive reaction in 54%. 9. The causative organism was isolated in 42.3% of all 26 observed cases. 10. Hematologic findings revealed mild degree of anemia (63.9%), leukocytosis (75.5%), increased ESR (63.4%). 11. Miliary density on X-ray were disappeared within 4 months following treatment in 73.9% of all 23 observed cases. 12. The complications or combined diseases were tuberculous meningitis (41 cases), cervical lymphadenopathy (10 cases) and tuberculous peritonitis (4 cases), etc. 13. The mortality rate was 14.3% (9 cases/63).
Anemia ; Child* ; Cough ; Emaciation ; Fever ; Humans ; Incidence ; Infant ; Korea ; Leukocytosis ; Lymphatic Diseases ; Mortality ; Mycobacterium bovis ; Neurologic Manifestations ; Parents ; Peritonitis, Tuberculous ; Prevalence ; Seasons ; Seoul ; Skin Tests ; Tuberculin ; Tuberculosis, Meningeal ; Tuberculosis, Miliary* ; Vomiting

BACKGROUND/AIMS: Spontaneous bacterial peritonitis (SBP) is a major problem associated with liver cirrhosis which has high mortality. Increased production of inflammatory mediators, such as tumor necrosis factor-a (TNF-a) and interleukin- (IL-) may be associated with development of renal impairment, one of the most important prognostic parameters in SBP. The aim of this study is to investigate the changes of these cytokines in ascitic fluid and plasma in patients with SBP and the relationship between these cytokines and development of renal impairment. METHODS: Forty patients with liver cirrhosis and ascites were studied 21 with SBP and 19 with sterile ascites. TNF-a and IL- levels in ascitic fluid and plasma were determined by ELISA at the time of diagnosis in both groups and 48 hours after antibiotics treatment in SBP patients. RESULTS: TNF-and IL- levels in ascitic fluid and plasma were significantly higher in patients with SBP than those without SBP (ascitic fluid TNF-a: 2.5+/-0.5 vs. 1.6+/-0.2; plasma TNF-a: 2.3+/-0.5 vs. 1.5+/-0.2; ascitic fluid IL-: 3.8+/-0.5 vs. 3.0+/-0.4; plasma IL-: 3.4+/-0.5 vs. 2.3+/-0.3, log pg/mL)(p<0.001). In patients with SBP, levels of TNF-a and IL- in ascitic fluid and plasma decreased 48 hours after antibiotics treatment. Eleven patients with SBP (11/21, 52%) developed renal impairment. Patients with renal impairment had significantly higher ascitic fluid and plasma TNF-a levels than those without renal impairment (median 2.5 vs. 2.1 for ascitic fluid, p=0.006; median 2.4 vs. 2.0, log pg/mL for plasma, p=0.04). Although four out of eleven (36%) patients who developed renal impairment died during hospitalization, all the patients without renal impairment survived (p=0.09). CONCLUSION: Our results suggest that the levels of TNF-a and IL- in ascitic fluid and plasma are increased in SBP and elevated levels of TNF-a in ascitic fluid and plasma may be associated with development of renal impairment, thus indicating poor prognosis in patients with SBP.
Anti-Bacterial Agents ; Ascites ; Ascitic Fluid* ; Cytokines ; Diagnosis ; Enzyme-Linked Immunosorbent Assay ; Hospitalization ; Humans ; Liver Cirrhosis ; Mortality ; Necrosis* ; Peritonitis* ; Plasma* ; Prognosis

BACKGROUND: Lipoprotein (a) concentration is mainly determined by apo (a) genotype, but elevated in the atherosclerotic vascular disease more than in normal group with the same apo (a) phenotype. It has been known that Lp (a) has independent metabolism in contrast with other lipoproteins and that the use of cholesterol lowering agent such as HMG-CoA reductase inhibitor for 6 months does not change the level of Lp (a). The results of several studies suggests that Lp (a) may be related to inflammation of atherosclerotic plaque and therefore, long term use of cholesterol lowering agents make plaque stable by reduction of inflammation at plaque. We hypothesized that there is a relationship between long term use of HMG-CoA reductase inhibitor and change of Lp (a) level. We prospectively measured Lp (a), lipids and inflammatory markers before and after long term use of HMG-CoA reductase inhibitor to examine our hypothesis. METHODS: Forty-nine subjects (M:F=28:21, age=59.1+/-12.0) with hyperlipidemia were administered HMG-CoA reductase inhibitor for 15 months (minimum 6 months, maximun 44 months), and Lp (a), lipids and inflammatory markers were measured before and after use of the HMG-CoA reductase inhibitor. In control group (ninty-nine subjects, M:F=60:39, age=61.2+/-9.2), these parameters were measured more than 6 months. RESULTS: In the hyperlipidemia group who were given HMG-CoA reductase inhibitor, baseline levels of total cholesterol, TG, LDL were significantly elevated more than those of the control group, but Lp (a) and inflammatory markers were not significantly different. After use of HMG-CoA reductase inhibitor, the level of Lp (a) was reduced significantly (before 28.9+/-29.3 mg/dl, after 20.0+/-19.0 mg/dl, p=0.009), but not significantly in the control group. There was a minimal relation between baseline Lp (a) levels and percent changes of Lp (a) levels. Total cholesterol and LDL levels reduced significantly after use of the drug, but inflammatory markers did not. CONCLUSION: These data showed that Lp (a) level in the hyperlipidemia group after the long term use of HMG-CoA reductase inhibitor decreased significantly. We suggest that these changes of Lp (a) level may be one of reliable markers for plaque stability in atherosclerotic vascular disease.
Atherosclerosis ; Cholesterol ; Genotype ; Hyperlipidemias ; Inflammation ; Lipoprotein(a) ; Lipoproteins ; Metabolism ; Oxidoreductases* ; Phenotype ; Plaque, Atherosclerotic ; Prospective Studies ; Vascular Diseases

PURPOSE: The prognosis for patients with stage IV gastric cancer is very poor. However, recently, some studies have reported benefits from a gastric resection for metastatic gastric cancer. This clinical study was performed to evaluate the effectiveness of a noncurative gastrectomy in treating stage IV gastric cancer with hepatic metastasis. MATERIALS AND METHODS: A retrospective analysis was performed on 98 gastric cancer patients who had undergone gastric resection, in spite of hepatic metastasis, between January 1990 and December 1996 at the Department of Surgery in 11 General Hospitals in Korea. RESULTS: The average age was 58 years old, and the male-to-female ratio was 69: 29. The laboratory tests were unable to predict hepatic metastasis. In 54 cases, hepatic metastasis was not identified before the surgery. The most common location of gastric cancer was antrum (72 cases). The most common gross type was Bonmann type III (78 cases). The serosa-exposed cases were 80. The peritoneal seeding was combined in 17 cases. A total gastrectomy was performed in 18 cases and a distal gastrectomy in 80. Lymph-node dissection was performed in 23 Dl, and 51 D2 cases. Hepatic resection was performed in 36 cases, The frequent histologic types were moderately differentiated and poorly differentiated tubular adenocarcinoma. Postoperative adjuvant chemotherapy was done in 70 cases. The complication rate (7%) was low. The median survival time was 15 months, with mean survival time of 18 months. The 2-year and 3-year survival rates were 23%, and 7%, respectively. In the univariate analysis, good survival was closely related to limitation of hepatic metastasis to one lobe, a few metastases to both lobes, negativity of peritoneal seeding and lymph node dissection more than D2 (p<0.05), CONCLUSIONS: An aggressive gastric resection for stage IV gastric cancer with hepatic metastasis might be beneficial in lengthening the survival period. A prospective study is needed, especiaUy one with an exact evaluation and analysis of the quality of life between the gastrectomy and nonresection groups.
Adenocarcinoma ; Chemotherapy, Adjuvant ; Gastrectomy* ; Hospitals, General ; Humans ; Korea ; Lymph Node Excision ; Middle Aged ; Neoplasm Metastasis* ; Prognosis ; Quality of Life ; Retrospective Studies ; Stomach Neoplasms* ; Survival Rate

Plasma pH can be altered during pregnancy and at labor. Membrane excitability of smooth muscle including uterine muscle is suppressed by the activation of K+ channels. Because contractility of uterine muscle is regulated by extracellular pH and humoral factors, K+ conductance could be connected to factors regulating uterine contractility during pregnancy. Here, we showed that TASK-2 inhibitors such as quinidine, lidocaine, and extracellular acidosis produced contraction in uterine circular muscle of mouse. Furthermore, contractility was significantly increased in pregnant uterine circular muscle than that of non-pregnant muscle. These patterns were not changed even in the presence of tetraetylammonium (TEA) and 4-aminopyridine (4-AP). Finally, TASK-2 inhibitors induced strong myometrial contraction even in the presence of L-methionine, a known inhibitor of stretchactivated channels in myometrium. When compared to non-pregnant myometrium, pregnant myometrium showed increased immunohistochemical expression of TASK-2. Therefore, TASK-2, seems to play a key role during regulation of myometrial contractility in the pregnancy and provides new insight into preventing preterm delivery.
4-Aminopyridine ; Acidosis ; Animals ; Contracts ; Female ; Hydrogen-Ion Concentration ; Lidocaine ; Membranes ; Methionine ; Mice ; Muscle, Smooth ; Muscles ; Myometrium ; Plasma ; Pregnancy ; Quinidine ; Relaxation ; Uterine Contraction ; Uterus