PURPOSE: To evaluate the clinical characteristics, treatments and outcome of patients with hemorrhagic shock and encephalopathy(HSE) syndrome. METHODS: We performed a clinical study on 14 patients who were diagnosed as hemorrhagic shock and having encephalopathy syndrome in the Department of Pediatrics, from 1984 to 1998. Age, sex, clinical symptoms and physical findings at admission, the most deranged laboratory findings, radiologic findings, treatments and outcome were analyzed. RESULTS: The age of onset was 1.0+/-0.9 years and the male to female ratio was 1: 1.8. At admission, clinical findings included dehydration in 85.7%, shock in 85.7%, fever in 71.4%, vomiting in 71.4%, diarrhea in 64.3%, GI bleeding in 50%, convulsion in 42.9%, and edema in 35.7%. Altered mental state was found in 100%, hepatomegaly in 64.3%, and splenomegaly in 21.4%. Laboratory findings revealed D-dimer positive in 92.9%, the mean hemoglobin level 8.2+/-2.1g/dL, BUN 35.7+/-24.0mg/dL, creatinine 1.9+/-1.5mg/dL, AST 561.0+/-1,412.1IU/L, ALT 858.9+/-1,649.8IU/L, blood glucose 229.5+/-197.4mg/dL, ammonia 195.4+/-129.7pg/dL, and total bilirubin 4.9+/-8.2mg/dL. On serologic tests, rotavirus and Epstein-Barr virus was found in 1 patient(7.1%), respectively. The mortality rate was 78.6%. CONCLUSION: We found that shock and disseminated intravascular coagulation(DIC) played important roles in the pathogensis of HSE syndrome, and encephalopathy, hepatic and renal insufficiency, and respiratory failure were secondary complications resulting from shock and DIC. Despite vigorous treatment, the prognosis was very poor. We feel more efforts should be focused on investigating the etiology and pathophysiology of HSE to prevent as well as develop a specific therapy. (J Korean Pediatr Soc 2000;43:814-819)
Age of Onset ; Ammonia ; Bilirubin ; Blood Glucose ; Creatinine ; Dacarbazine ; Death, Sudden* ; Dehydration ; Diarrhea ; Edema ; Female ; Fever ; Hemorrhage ; Hepatic Encephalopathy ; Hepatomegaly ; Herpesvirus 4, Human ; Humans ; Infant* ; Male ; Mortality ; Pediatrics ; Prognosis ; Renal Insufficiency ; Respiratory Insufficiency ; Rotavirus ; Seizures ; Serologic Tests ; Shock ; Shock, Hemorrhagic* ; Splenomegaly ; Vomiting

Progressive renal fibrosis is considered to be the final common pathway leading to chronic renal insufficiency, however, the mechanism regarding renal fibrosis in renal injury is not well understood. Recently, several kinds of cytokines have been known to be related to fibrosis after renal injury. The interaction between elements regulating fibrogenesis would be better understood by looking at the effect of TGF-beta1 on the synthesis and accumulation of extracellular matrix, especially collagenous proteins. Crescentic glomerulonephritis (CGN) was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG; and their kidneys were analyzed for the development of crescents and fibrosis through sequential renal biopsies. Serum creatinine levels in a time course progressively increased until day 15. We semi-quantitatively assayed the levels of the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA factored for GAPDH mRNA using RT-PCR. We observed a progressive interstitial fibrosis and the expression of collagen I both in the cortex and medulla. The effect of repeated renal biopsy itself on pathology and on the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA in a time course were not significant, but a very mild increase of the expression of alpha1(I) collagen mRNA was noted at day 15. Histology showed a progressive crescent formation and interstitial fibrosis in a time course that roughly paralleled the expression of alpha1(I) collagen mRNA in both cortex and medulla. TGF-beta1 mRNA was hardly expressed at day 0 in cortex as well as in medulla. It was elevated from day 1, peaked at day 7, and then decreased. In medulla, TGF-beta1 mRNA was noticeably expressed at day 1, peaked at day 4, and then decreased. The expression of alpha1(I) collagen mRNA was seen even before inducing CGN. It was gradually and continuously increased until day 15 both in cortex and medulla. These results suggest that the expression of TGF-beta1 mRNA precedes that of alpha1(I) collagen mRNA in the early stage of CGN and has a central role for provoking the accumulation the collagen I, the most representative interstitial extracellular matrix, in the rabbit model CGN induced by anti-GBM antibody. We conclude that the measurement of the expression of TGF-beta1 mRNA and/or alpha1(I) collagen mRNA in a biopsy sample can be a useful predictor for renal outcome.
Animals ; Anti-Glomerular Basement Membrane Disease ; Biopsy ; Collagen ; Creatinine ; Cytokines ; Extracellular Matrix* ; Fibrosis ; Glomerulonephritis* ; Guinea Pigs ; Immunoglobulin G ; Kidney ; Pathology ; Rabbits ; Renal Insufficiency, Chronic ; RNA, Messenger* ; Transforming Growth Factor beta ; Transforming Growth Factor beta1

Most ectopic pregnancies occur in the fallopian tubes. There have been numerous theories to explain the occurrence of ectopic pregnancy in fallopian tubes. The most commonly held view is that the passage of the fertilized ovum through the fallopian tube is delayed or hindered by chronic inflammation and its sequelae. We designed a study to evaluate the details of histopathologic changes and the location of implantation and how they relate to the clinical history. 182 fallopian tube specimens from patients who had undergone total or partial salpingectomy were examined. A high incidence of non-specific inflammation of plicae and wall of tube (31.9%) and salpingitis isthmica nodosa (12.6%) were observed. Other associated findings included acute salpingitis, complex plicae or complex hyperplasia of tubal epithelium, fibrous adhesion with ovary, endometriosis, and calcification. History of previous ectopic tubal pregnancy was found in 8 cases. The cases with serum beta-HCG value above 2,500 I.U./L (group I, n=97) were more frequently noted in those exhibiting myosalpingeal invasion of trophoblast (67 cases) than in those without invasion (30 cases). Of the 182 tubal pregnancies, 117 (64.3%) cases were found in the ampulla and 47 (25.8%) cases in isthmic location. In 117 ampullary pregnancies, the products of conception were found intraluminally in 71 cases (60.7%), and extraluminally in 34 (29.1%) cases, of which the products of conception were found entirely extraluminal. The products of conception, found both within and outside the tubal lumen, were found in 12 cases (10.2%). Of 47 tubes with isthmic pregnancies, 33 cases were intraluminal (70.2%), 12 cases were extraluminal (25.5%), and two cases were mixed (4.3%). In conclusion, significant histopathologic abnormalities accompany a majority of ectopic tubal pregnancy, and myosalpingeal invasion of trophoblast is correlated with high serum beta-HCG. Thus, it is necessary to confirm not only the ectopic placental tissue but also the accompanying details of the other histopathologic findings or the pathologic evaluation of ectopic tubal pregnancy.
Endometriosis ; Epithelium ; Fallopian Tubes ; Female ; Fertilization ; Humans ; Hyperplasia ; Incidence ; Inflammation ; Ovary ; Pregnancy ; Pregnancy, Ectopic ; Pregnancy, Tubal* ; Salpingectomy ; Salpingitis ; Trophoblasts ; Zygote

We investigate the serial change of the bone density of the lengthening sites in distraction osteogenesis in long bone lengthening of the lower extremity by measuring the pixel value of the PACS(Picture Archiving Communication System). The purpose of this study was to find the clinical implication of the pixel value in PACS in the distraction osteogenesis. The number of the distraction sites were 22 in tibia and 16 in femur. The average distraction length was 4.5cm ranged between 2.1cm and 7.0cm in femur, 4.1cm ranged hetween 1.9cm and 6.8cm in tibia. When the image were sent to the PACS workstations, they were directly interfaced to the workstation without any processing. The absolute and the relati ve pixel values of cortical bones of the original and the lengthening sites repr sented in workstation of PACS were obtained by average value measuring 3 times by 3 different persons. The average absoiute pixel value of the original cortical bone near distraction site was not significantly changed, maintaining 575+/-6 in femur, and 570+/-7 in tibia. The absolute pixel vaIues in AP and lateral view were not significantly changed until 6 week/cm, but rapidly increased after 7 week/cm hoth in the tibia and the femur. The relative pixel value of the lengthening sites were more than 95% in three of the four cortices at the time of the removal of the external fixators. in conclusion, the pixel value of the PACS can be a rapid, simple and easy method for detection of the change of the bone density in distraction osteogenesis.
Bone Density* ; Bone Lengthening* ; External Fixators ; Femur ; Humans ; Lower Extremity* ; Osteogenesis, Distraction* ; Tibia

-D- is a very rare haplotype which determines D without C, c, E or e and exalted D activity. The extremely rare homozygote propositi(-D-/-D-) are usually ascertained through their immune antibodies which react with red cells of all common Rh phenotypes. Authors experienced a woman with -D- phenotype for the first time in Korea. She had a history of abortion and intrauterine fetal death. She delivered a baby with severe hemolytic disease of the newborn at the third pregnancy. In spite of intensive medical interventions, the baby died of hydrops fetalis. An immune antibody to high incidence Rh antigen, namely anti-Hro, was demonstrated in the woman's serum. Family study revealed that all of the family had -D- gene complex and one of her sisters also was -D-homozygote. The sister also had anti-Hro in the serum.
Antibodies ; Female ; Fetal Death ; Haplotypes ; Homozygote ; Humans ; Hydrops Fetalis ; Incidence ; Infant, Newborn* ; Korea ; Phenotype* ; Pregnancy ; Siblings

BACKGROUND: The platelet-specific antigens which exist specifically on platelets have their antigenic determinant on platelet membrane glycoprotein. The alloantibodies against this antigens are responsible for neonatal alloimmune thrombocytopenia, posttransfusion purpura and platelet refractoriness in multitransfused patients. Discovering of the fact that the polymorphism of platelet-specific antigens is resulted from a single base pair substitution of genomic DNA stimulated studies on genotyping of platelet-specific antigens on various populations. This study was performed to investigate the genotype frequency of platelet-specific antigens in Koreans. METHODS: Using genomic DNA extracted from venous blood of 200 Koreans, genotype of seven platelet-specific antigen systems were determined through allele-specific polymerase chain reaction and restriction fragment length polymorphism. RESULTS: The genotype frequencies of HPA-1 were a+b- 98.0%, a+b+ 1.5%, a-b+ 0.5%; HPA-2, a+b- 85.5%, a+b+ 13.5%, a-b+ 1.0%; HPA-3, a+b- 28.5%, a+b+ 54.0%, a-b+ 17.5%; HPA-4, a+b- 98.0%, a+b+ 2.0%, a-b+ 0.0%; HPA-5, a+b- 95.5%, a+b+ 4.5%; HPA-6W, a+b- 96.0%, a+b+ 4.0%; HPA-7W were a+b- 100.0%, a+b+ 0.0%. CONCLUSION: The gene frequency of HPA-1b in Koreans was lower than that of Caucasian. As a whole, the genotype frequencies of platelet-specific antigens in Koreans were similar to those of the Japanese. However, we found one HPA-1(a-b+) among 200 Koreans, which is very rare in Japanese. This study will serve as a basic data for the study and management of the patients with diseases associated with platelet-specific antigens and antibody reactions in Koreans.
Antigens, Human Platelet* ; Asian Continental Ancestry Group ; Base Pairing ; Blood Platelets ; DNA ; Gene Frequency ; Genotype* ; Humans ; Isoantibodies ; Membrane Glycoproteins ; Polymerase Chain Reaction ; Polymorphism, Restriction Fragment Length ; Purpura ; Thrombocytopenia, Neonatal Alloimmune

Hydrops fetalis is diagnosed when abnormal fluid collections are manifest in two or more fetal compartments, including abdominal ascites, pleural effusions, pericardial effusions, skin edema, polyhydroamniosis and placental edema. Although fetal hydrops was hystorically most commonly associated with Rh blood group isoimmunization, the availability of Rh immunoglobulin has increased the proportion of fetuses affected due to nonimmune etiologies. We have experienced a case of nonimmune hydrops fetalis at 32 weeks of gestation in a 27-year-old woman and reported that with brief review of related literatures.
Adult ; Ascites ; Edema ; Female ; Fetus ; Humans ; Hydrops Fetalis* ; Immunoglobulins ; Pericardial Effusion ; Pleural Effusion ; Pregnancy ; Skin

Pseudomembranous colitis, thought to be uncommon in children, is a bacterial, toxin-mediated inflammatory process resulting in acute or chronic diarrhea and is characterized by colonic pseudomembranes. It is mediated by toxins produced by Clostridium difficile and is increasingly recognized in pediatric population. Diagnosis is based on positive culture of C. difficile in selective media and positive test of C. difficile toxin. Oral metronidazole or vancomycin are the main treatment options but avoidance of further antibiotics should also be encouraged where possible. We have experienced a case of pseudomembranous colitis in a 4-year-old female presented with septic shock and colitis. This case was diagnosed with positive test of C. difficile toxin B and confirmed by isolation of the organism on cultire in selective media. Symptoms have been ameliorated by discontinuation of antibiotics and administration of metronidazole and oral vancomycin, and ICU care.
Anti-Bacterial Agents ; Child ; Child, Preschool ; Clostridium difficile* ; Clostridium* ; Colitis ; Colon ; Diagnosis ; Diarrhea ; Enterocolitis, Pseudomembranous* ; Female ; Humans ; Metronidazole ; Shock, Septic ; Vancomycin

PURPOSE: Many investigators suggested that changes in hormonal environment in the postmenopausal women functionally or histologically effect the clitoris and vagina. We investigated the mechanism of female se xual dysfunction occurring in the decreased hormonal status, and aimed to establish and experimental base for the hormone replacement in postmenopausal women to correct sexual dysfunction. MATERIALS AND METHODS: Twenty mature female New Zealand white rabbits were randomly divided into three groups; control group, oophorectomy group, estrogen replacement group after oophorectomy. Nitric oxide synthase (NOS) activity and the degree of expression of neuronal NOS (nNOS) and endothelial NOS (eNOS), collagen content in clitoral tissues were analysed. RESULTS: nNOS and eNOS were significantly increased in the oohporectomized group while decreased in the estrogen replacement group (p<0.01). NOS activity showed the similar pattern of change (p<0.05). Oophorectomy induced a significant increase in collagen content, while the ratio of smooth muscle content was increased significantly after the estrogen replacement (p<0.01). CONCLUSIONS: Estrogen deficiency induces collagen synthesis and decreases the content of smooth muscle in clitoris, resulting in a structural relaxation difficulty. We believe that NOS becomes more active to improve the relaxation difficulty and that hormone replacement helps to restore the normal state of smooth muscle relaxation. Considering these findings, we suggest that estrogen down-regulates NOS and its activity.
Clitoris* ; Collagen ; Estrogen Replacement Therapy ; Estrogens* ; Female ; Humans ; Muscle, Smooth ; Neurons ; Nitric Oxide Synthase ; Ovariectomy ; Rabbits ; Relaxation ; Research Personnel ; Vagina

We encountered a case of neonatal altoimmune thrombocytopenia(NAIT) due to anti-HLA-B7+B60+B61. Bilateral cephal hematoma and umbilical hematoma were noted at the time of birth. Purpura developed at the third day. Platelet count was 110,000 at birth and decreased to 66,000/micro liter at the day 4. Prothrombin time and partial prothrombin time were within normal limit. The mother's platelet count was 220,000/micro liter and she had no history of abnormal bleeding. Platelet antibody tests empolying mixed passive hemagglutination and immunofluorescence revealed that the mother's serum was reactive against the platelets from the father and the neonate, but was not reactive with her own platelets. Platelets from eight volunteer group 0 donors were tested with the mother's serum; seven were reactive and one was negative. The positive reactions were lost after chloroquine treatment of platelets. Antigen capture ELISA(ACE) and modified antigen capture ELISA employing monoclonal antibodies against platelet glycoproteins In, IIa, IIb, and IIIa were negative. Mother's serum was tested for lymphocytotoxicity against 49 donor ]ymphocytes and the specificity was found to be anti-HLA-B7+B60+B61. At the 9th day, one unit of platelet concentrate from the mother was transfused and the platelet count of the neonate rose up to 340,000/micro liter. The neonate was discharged at the day of sixteenth and the platelet count remained high thereafter.
Antibodies, Monoclonal ; Blood Platelets ; Chloroquine ; Enzyme-Linked Immunosorbent Assay ; Fathers ; Fluorescent Antibody Technique ; Hemagglutination ; Hematoma ; Hemorrhage ; Humans ; Infant, Newborn ; Mothers ; Parturition ; Platelet Count ; Platelet Membrane Glycoproteins ; Prothrombin Time ; Purpura ; Sensitivity and Specificity ; Thrombocytopenia* ; Tissue Donors ; Volunteers