No abstract available.
Lupus Erythematosus, Cutaneous* ; Scalp*

High dose intravenous gammaglobuline (IVLG) therapy is effective in some of the autoimmune diseases. Although the exact mechanism of action of IVIG is uncertain, the action as a neutralizing antibody against unknown etiologic agents, the action of blocking of Fc receptors of effector cells, or the action as a antiidiotypic antibody are suggested. We report a case of 12 year old girl with systemic juvenile rheumatoid arthritis who was treated with high dose IVIG and got a remission. In August 1990 she was admitted to our hospital. because of intermittent fever, transient rash and multiple arthralgia. Under the diagnosis of systemic juvenile rheumatoid arthritis, aspirin (4.0g/day) had been given with symptom improvement. She was readmitted in October 1990 because of aspirin intoxication and acute fulminant hepatitis. She was discharged after recovery and any medicine was not prescribed. In November 1990 she was admitted because of epigastric pain, vomiting, intermittent fever, multiple arthritis, and mild hepatomegaly. Total parenteral alimentation had been given under the diagnosis of superior mesenteric artery syndrome and gold sodium thiomalate (Myochrysine, 5 and 10 mg, two weekly IM injection) was given in conjunction with prednisolone (30 mg/day) and naproxen (375 mg/day). She was admitted again in February 1991 due to the fever, coughing, rash, and hepatosplenomegaly. Pneumonia and leukopenia (2100/mm(3)) were found and gold sodium thiomalate injection was discontinued. Gammaglobulin 1 g/kg/day was given intravenously for 2 consecutive days with dramatic symptom improvement. Five more monthly IV gammaglobulin had been given and the side reaction of injection were nausia, fever, and headache which were controlled by the decrease of infusion rate. Four months after the last IVIG injection she had no symtom of arthritis and the hepatosplenomegaly was decreased. Hemoglobin level was increase to 12.2 mg/dL form 6.2mg/dL and ESR was decrease to 15mm/h. The oral prednisolne and ibuprofen were stopped one year after th last IVIG injection. All the laboratory parameters of arthritis and physical examinations had been normal for more than two year after the stop of all the medications until March of 1994. We suggest that high dose intravenous gammaglobulin can be one of treatments for severe systemic juvenile rheumatoid arthritis.
Antibodies, Neutralizing ; Arthralgia ; Arthritis ; Arthritis, Juvenile ; Aspirin ; Autoimmune Diseases ; Child ; Cough ; Diagnosis ; Exanthema ; Female ; Fever ; Gold Sodium Thiomalate ; Headache ; Hepatitis ; Hepatomegaly ; Humans ; Ibuprofen ; Immunoglobulins, Intravenous ; Leukopenia ; Naproxen ; Physical Examination ; Pneumonia ; Prednisolone ; Receptors, Fc ; Superior Mesenteric Artery Syndrome ; Vomiting

STUDY DESIGN: Thirty-fiye thoracolumbar and lumbar burst fractures treated with pedicle screw instrumentation were assessed on loss reduction. OBJECTIVE: To compare the mechanical results of simple posterior fixation and posterior fixation with anterior augmentation by anterior interbody fusion, disc space fusion or longer construct. SUMMARY OF BACKGROUND DATA: Althrough the pedicle screw instrumentation provides good mechanical stability, two segment fixation without anterior column augmentation may not be adequate to withstand the repeated axial load. METHODS: Fifteen patients were treated with two segment fixation alone(Group 1), seven with anterior interbody fusion(Group 2), another seven with disc space fusion(Group 3) and remaining six with longer construct involving two segments abode the fracture(Croup 4). Change of anterior body height, upper disc height, lower disc height, kyphotic angle, inter-screw angle and gross screw bend- ing were measured using post-operative and follow-up lateral radiographs. RESULTS: Comparing the two groups(Croup 1 Ys. Group 2, 3, 4), the Group 1 showed definitely more loss of reduction than those groups which had anterior augmentation. But, loss of anterior body height was not significantly different. Comparing the four groups, loss at upper and lower disc height was significantly greater in the Group 1 than the Group 2. Change of kyphotic angle was significantly higher in the Group 1 than the Group 4 and change of inter-screw angle was also higher in the group 1 than every other group. Seyen patients in the Group 1 showed gross screw bending, but none in the Group 2, 3 and 4. All the seven patients with screw bending showed loss of inter-screw angle more than 5 . CONCLUSIONS: These results mean that anterior column augmentation is necessary for the treatment of thoraco-lumbar burst fracture. All the three methods(Group 2,3,4) are effective to prevent excessive loss of correction and screw bending. The best method should be selected considering the characters of each fracture.
Body Height ; Follow-Up Studies ; Humans

The CRST syndrome, first reported by Winterbauer, represents a benign variant of scleroderma and consists of calcinosis(C), Raynauds phenomenon(R), sclerodactyly(S) and telangiectasia(T). The authors report a case of CRST syndrome confirmed by both clinical and histopathological findings. A 62-year-old female developed subcutaneous nodules on both elbow and knee joints, Raynauds phenomenon, sclerodactyly with acrosclerosis and scleroderrnatous changes on both hands and forearms and telangiectasia on the face, neck, and hands of 15 years duration, but she had no difficulty in swallowing. The authors treated a case of CRST syndrome with weekly intra-arterial administration of reserpine and were impressed not only by the marked loosening of the skin but also by the striking effect on the Raynauds phenornena; the latter disappeared within a few weeks of treatment.
CREST Syndrome* ; Deglutition ; Elbow ; Female ; Forearm ; Hand ; Humans ; Knee Joint ; Middle Aged ; Neck ; Reserpine* ; Skin ; Strikes, Employee ; Telangiectasis

For sequential phenotypic changes including enzyme altered hepatocytic foci, hyperplastic nodules, hepatocellular adenomas and carcinomas were produced in Sprague-Dawley rats by Solt-Farber method (administration of diethylnitrosamine and acetylaminofluorene (AAF), and partial hepatectomy). The immunohistochemical expressions of glutathione S transferase P (GST-P) and bromodeoxyuridine (BrdU) were assessed for selective proliferative activity in the enzyme altered foci and the subsequently developed lesions by double immunohistochemical staining technique. Immunoreactive areas against GSTP gradually increase from early period of carciogenesis. BrdU labeling in such areas remained high during the first week. but decreased thereafter. BrdU labeling index remained low in the GSTP negative area throughout the experimental period. This suggests that cells in the enzyme altered foci keep away from the suppressor effect of AAF in contrast to the normal cells in which their growth are inhibited by AAF. BrdU labeling index remained very low in both hyperplastic nodule and adenoma which were prevalent during the mid-experimental period, but increase markedly in carcinoma. The long period of low BrdU labeling index seems to correspond to the "slowly growing step of persistent nodule" during hepatocarcinogenesis. The differentiation index, a ratio of S phase fraction between GSTP positive and negative areas, was low in adenoma-developing period and high in carcinoma-developing period. C-Ha-ras p21 was not expressed in foci of enzyme altered hepatocyte and hyperplasia, but highly positive in carcinoma. This indicates that the c-Ha-ras may involve the late step of hepatocarcinogenesis.
Rats ; Animals ; Adenoma ; Carcinoma, Hepatocellular

Sirenomelia is a severe form of caudal regression syndrome that results in a fusion of the lower extremities, which is not compatible with life. A various spectrum of anomalies affecting primarily the musculoskeletal, genitourinary and gastrointestinal systems also can occur. This rare malformation has a reported incidence rate of approximately 1 in 60,000 births, with a range of 1 to 1 percent of all malformed infants. We experienced a sirenomelic case with combined anomalies of genitourinary, cardiovascular and gastrointestinal systems. Maternal obstetric history revealed oligohydramnios and intrauterine fetal growth retardation, and the baby was spontaneously delivered at 37 weeks of gestational age, but died I hour after birth.
Infant ; Male ; Female ; Humans ; Incidence

No abstract available.
Anemia, Hypoplastic, Congenital*

A retrospective study was conducted to investigate the effects of CNS prophylaxis with 1,800 cGy cranial irradiation on standing height growth of children with acute lymphoblastic leukemia (ALL). Seventeen longterm survivors (DFS over 5 years ) of ALL and Non-Hodgkins Lymphoma who had been admitted at pediatric department of Inje University Seoul Paik Hospital from January 1982 to September 1988 were studied. The long term survivors were divided into two groups by prophylactic treatment modality; intrathecal chemotherapy alone (Group I, 7 cases), intrathecal chemotherapy with cranial irradiation (Group II, 10 cases). The heights of each groups were compared normal standard growth of the sane aged children by mean SD score (The SD score was calculated with the use of the following formula). SD score=(x-x)/SD (x; height measurement, x; mean height for age of the normal population SD; standard deviation). The results were as follows; 1) The mean SD scores of Group I and Group II at diagnosis were -0.30 and -0.17, which were smaller than height of normal child. 2) In Group 1, the mean SD scores after the end of treatment were -0.14 of 1 year, 0.03 at 2 years and 0.01 at 5 years, which suggests that were normal height velocity and catch-up growth in these patients (P<0.05). The height of 1 year after the beginning of treatment were particularly smaller than that of the normal children (P<0.05). 3) The mean SD scores 5 years after the end of treatment were 0.10 in Group I and -0.17 in Group II, which shows strong tendency to statistical difference among these two groups. If larger numbers of patients are evaluated, it, however, may turn significant. In conclusion, 1,800 cGy cranial irradiation in ALL children significantly affects on height growth. We recommend to evaluate growth hormone for patient with 1,800 cGy cranial irradiation and to treat them with growth hormone.
Child* ; Cranial Irradiation* ; Diagnosis ; Drug Therapy ; Growth Hormone ; Humans ; Lymphoma, Non-Hodgkin ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Seoul ; Survivors

Gastric lesion in familial polyposis coli is commonly presented with fundic gland hyperplastic polyps, but duodenal and gastric adenomas together with their carcinomatous transformation have been rarely described in familial polyposis coli mostly by case reports. We present three cases of gastric adenomas in familial polyposis coli with one in synchronous development of gastric adenocarcinoma in Korea. All three cases had the family history related to familial polyosis coli and received proctocolectomy because of synchronous development of colonic adenocarcinoma. One patient developed gastric polyposis and adenocarcinoma 8 years after colectomy, and the remaining two presented with multiple polyps either in the stomach or duodenum synchronously at the time of the diagnosis of familial polyposis coli with colonic adenocarcinoma. None disclosed any evidence of Gardner's syndrome. We conclude that association of gastric adenomas in familial oplyposis coli is not uncommon and gastric adenoma-carcinoma sequence is an another important participating mechanism to understand the histogenesis of gastric carcinoma in Korea.
Adenocarcinoma ; Adenoma

Extramammary Paget's disease is a dermatosis which pathologically and clinically resembles Paget's disease of the breast. The disease occurs in regions with apocrine gIands; most often in the anogenital area, seldom in the axilla. umbilicus, mucous membrane of the mouth, nose, ear and Moll's gland of tne eyelid. In this work the effect of bleomycin for extramammary Paget's disease was studied on ultrastructural aspect. The material was a case of extramammary Paget's disease of 7 years standing occuring in the scrotum and the pubic area by serial sections after IV injection of 60mg, 120mg and 240mg of bleomycin, and examined under the electron microscope. The results obtained are summarized as follows: 1. The size of Paget's cell and its nucleus was gradually decreased. 2. The numbers of lysosome, dense body, autophagic vacuolization and tonofilament was decreased. From these fidings, it is suggested that bleomycin induces retrograde degenerative change of Pagct's cell, and cell necrosis finally.
Axilla ; Bleomycin* ; Ear ; Eyelids ; Intermediate Filaments ; Lysosomes ; Mouth ; Mucous Membrane ; Necrosis ; Nose ; Paget Disease, Extramammary ; Paget's Disease, Mammary ; Scrotum ; Skin Diseases ; Umbilicus