BACKGROUND: The high level of susceptibility of patients with liver cirrhosis (LC) to septicemic infection is thought to be due to decreased reticuloendothelial function and irnpairment of several components of cell mediated and humoral immunity. It is well known that LC may cause many characteristic skin changes including jaundice, spider angioma, palmar erythema, xanthelasrna and nail changes such as clubbing and white nails. OBJECTIVE AND METHODS: During a 7 month period frorn April to October 1995, 100 patients who had been diagnosed as having LC were evaluated for the duration of their LC, the associated diseases they had apart from LC and various skin manifestations. Also, we performed the tuberculin test in 20 patients with LC and evaluated the correlation of the tuberculin test with severity of LC divided into 3 groups according to the Childs classification. RESULTS: The results are summarized as follows ; 1. The most frequent duration of LC was under 5 years (74%). 2. The most common associated disease of patients with LC was upper GI bleeding including esophageal varix (24 cases). Also, diabetes mellitus (13 cases) and hepatic coma (11 cases) were observed. 3. Jaundice (82 cases) was the most frequent skin rnanifestation of patients with LC. In addition, vascular changes (81 cases) and various nail changes (69 cases) were observed. Onychomychosis was the most common nail change of patients with LC. 4. 60% of the patients who were given the tuberculin test showed anergy, and the frequency of anergy increased in proport on to the severity of LC. However, it was not significant statistically (p> 0.05 ). CONCLUSIONS: Some of the skin manifestations help in the diagnosis of LC. Also, increased frequency of tuberculin test anergy in proportion to the severity of LC is thought to be related to decreased cell mediated immunity, although statistically it is not significant.
Child ; Classification ; Diabetes Mellitus ; Diagnosis ; Erythema ; Esophageal and Gastric Varices ; Hemangioma ; Hemorrhage ; Hepatic Encephalopathy ; Humans ; Immunity, Cellular ; Immunity, Humoral ; Jaundice ; Liver Cirrhosis* ; Liver* ; Skin Manifestations* ; Skin* ; Spiders ; Tuberculin Test

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.
Actins ; Desmin ; Female ; Fibroblasts ; Follow-Up Studies ; Humans ; Infant, Newborn ; Myocytes, Smooth Muscle ; Myofibroblasts ; Myofibromatosis* ; Necrosis ; Parturition ; Pericytes ; Thorax

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.
Antibodies ; Antibodies, Monoclonal ; Biotin ; Bowen's Disease ; Coloring Agents ; Diagnosis ; Melanoma ; Molecular Weight ; Skin ; Streptavidin

A study was carried out to evaluate the house rats in southern Korea as reservoir host of intestinal trematodes, in 13 different areas: 7 in inland and 6 in brackish-water zones, during the period from August 1980 to August 1981. A total of 170 house rats was captured; 101 rats from inland and 69 from brackish-water zones. They consisted of 129 Rattus norvegicus, 22 R. rattus rufescens, and 19 unidentified. The infection status of the rats were as follows: Total 29 rats (17.1%) were infected by one or more kinds of intestinal trematodes; 27 from inland and 2 from brackish-water zones by areas. Rats in inland were more heavily infected. The intestinal trematodes collected from rats were identified as Echinostoma hortense, E. cinetorchis, Plagiorchis muris, Fibricola seoulensis, Clonorchis sinensis and Metagonimus yokogawai. As a reservoir host, the rats were very important in Echinostoma hortense and E. cinetorchis infection. Rats infected with these trematodes were found widely in southern Korea, and its infection rates were the highest among the discriminated flukes. In this study, no rats were found to be infected with Heterophyes and Pygidiopsis even in endemic areas. The negligible importance of rats as reservoir host of these heterophyid should be reevaluated in the future.
parasitology-helminth-trematoda ; rat- Rattus norvegicus-Rattus rattus rufescens ; zoonosis ; epidemiology ; Echinostoma hortense ; Echinostoma cinetorchis ; Plagiorchis muris ; Fibricola seoulensis ; Clonorchis sinensis ; intestine ; Metagonimus yokogawai

Nemaline myopathy is a rare congenital m opathy, characterized by nonprogressive or slowely progressive muscle weakness associated with rod-like structures in muscle fibers and characteristic dysmorphic features. We report the first farnilial nemaline myopathy in two generations of the same family, confirmed by muscle biospy in an 11-year-old boy and his mother. The patients had hypotonia and slowly progressive muscle weakness of the four limbs associated with characteristic facial dysmorphism and skeletal deformities. Light and electron microscopic study of a muscle biopsy showed numerous nemaline rods in both patients.
Biopsy ; Child ; Congenital Abnormalities ; Extremities ; Family Characteristics ; Humans ; Male ; Mothers ; Muscle Hypotonia ; Muscle Weakness ; Myopathies, Nemaline*

PURPOSE: To investigate the various ictal perfusion patterns and find the relationships between clinical factors and different perfusion patterns. MATERIALS AND METHODS: lnterictal and ictal SPECT and SPECT subtraction were performed in 61 patients with partial epilepsy. Bath positive images showing ictal hypoperfusion and negative images revealing ictal hypoperfusion were obtained by SPECT subtraction. The ictal perfusion patterns of subtracted SPECT were classified into focal hypoperfusion, hypoperfusion-plus, combined hypoperfusion-hypoperfusion, and focal hypoperfusion only. RESULTS: The concordance rates with epileptic focus were 91.8% in combined analysis of ictal hypoperfusion and hypoperfusion images of subtracted SPECT, 85.2% in hypoperfusion images only of subtracted SPECT, and 68.9% in conventional ictal SPECT analysis. Ictal hypoperfusion occurred less frequently in temporal lobe epilepsy (TLE) than extratemporal lobe epilepsy. Mesial temporal hypoperfusion alone was seen only in mesial TLE while lateral temporal hypoperfusion alone was observed only in neocortical TLE. Hippocampal sclerosis had much lower incidence of ictal hypoperfusion than any other pathology. Some patients showed ictal hypoperfusion at epileptic focus with ictal hypoperfusion in the neighboring brain regions where ictal discharges propagated. CONCLUSION: Hypoperfusion as well as hypoperfusion in ictal SPECT should be considered for localizing epileptic focus. Although the mechanisrn of ictal hypopertusion could be an intra-ictal early exhaustion of seizure focus or a steal phenomenon by the propagation of ictal discharges to adjacent brain areas, further study is needed to elucidate it.
Baths ; Brain ; Epilepsies, Partial* ; Epilepsy ; Epilepsy, Temporal Lobe ; Humans ; Incidence ; Pathology ; Perfusion* ; Sclerosis ; Seizures ; Tomography, Emission-Computed, Single-Photon*

PURPOSE: The detection of epileptogenic lesion plays an important role in the management of patients with partial epilepsy. Although the development of MRI improved the examination of cerebral hemispheres greatly, many patients with neocortical temporal lobe epilepsy (TLE) or extratemporal lobe epilepsy (extra-TLE) still show no lesion in conventional two-dimensional (2D) images. To increase the yield of MRI in those patients, we performed three-dimensional (3D) surface-projection rendering (SPR) of the cerebral hemispheres. METHODS: Conventional 2D MRI (T1, T2, FLAIR, thin slice SPGR) and 3D SPR were performed in 24 patients with neocortical TLE and extra-TLE, and 20 normal subjects. Sulcogyral patterns were evaluated blindly to clinical information. The locations of the epileptogenic zone, ictal onset zone (IOZ) and irritative zone (IRZ) were determined by intracranial EEG monitoring and epilepsy surgery. RESULTS: The 2D MRI identified epileptogenic lesions in five of the 10 neocortical TLE (50%) and five of the 14 extra-TLE (35.7%). 3D SPR revealed abnormal sulcogyral patterns in 9 of the 10 neocortical TLE (90%) and 9 of the 14 extra-TLE (64.3%). Cortical deformation zones with sulcogyral anomalies included the whole area of IOZ in 10 (55.5%) and IRZ in 6 (33.3%), overlapped with IOZ in 7 (38.9%) and IRZ in 11 (61.1%), were connected to IOZ in 1 (5.6%) and IRZ in 1 (5.6%). CONCLUSION: 3D SPR of volumetric MRI data can detect epileptogenic structural lesions of neocortical epilepsy that are not visible in the conventional 2D images.
Brain* ; Cerebrum ; Electroencephalography ; Epilepsies, Partial ; Epilepsy* ; Epilepsy, Temporal Lobe ; Humans ; Magnetic Resonance Imaging*

In the course of immunoscreening of Clonorchis sinensis cDNA library, a cDNA CsRP12 containing a tandem repeat was isolated. The cDNA CsRP12 encodes two putative peptides of open reading frames (ORFs) 1 and 2 (CsRP12-1 and -2). The repetitive region is composed of 15 repeats of 10 amino acids. Of the two putative peptides, CsRP12-1 was proline-rich and found to have homologues in several organisms. Recombinant proteins of the putative peptides were bacterially produced and purified by an affinity chromatography. Recombinant CsRP12-1 protein was recognized by sera of clonorchiasis patients and experimental rabbits, but recombinant CsRP12-2 was not. One of the putative peptide, CsRP12-1, is designated CsPRA, proline-rich antigen of C. sinensis. Both the C-termini of CsRP12-1 and -2 were bacterially produced and analysed to show no antigenicity. Recombinant CsPRA protein showed high sensitivity and specificity. In experimental rabbits, IgG antibodies to CsPRA was produced between 4 and 8 weeks after the infection and decreased thereafter over one year. These results indicate that CsPRA is equivalent to a natural protein and a useful antigenic protein for serodiagnosis of human clonorchiasis.
Amino Acid Sequence ; Animals ; Antigens, Helminth/*genetics/isolation & purification ; Base Sequence ; *Cloning, Molecular ; Clonorchis sinensis/genetics/*immunology ; DNA, Helminth ; Gene Library ; Human ; Molecular Sequence Data ; Rabbits ; Recombinant Proteins ; *Repetitive Sequences, Nucleic Acid ; Support, Non-U.S. Gov't

To observed whether the specific IgG antibody test using ELISA was useful in diagnosis of presently ill patients of paragonimiasis, a total of 95 sera were tested. The sera were collected from 21 egg positive cases, 8 from positive reactors of intradermal test, 7 from Clonorchis infected, 9 from other parasitic diseases and 50 from apparently non-infected cases. By the result, the sensitivity of the test was 86% and the specificity was 100%. There were no cross reactions between Paragonimus antigen and other parastic infections. Specific IgG antibody test by micro-ELISA was concluded to be useful for mass screening of the presently ill paragonimiasis in the field.
parasitology-helminth-cestoda ; Spirometra sp. ; sparganum ; sparganosis ; immunology ; diagnosis ; ELISA

PURPOSE: The prognosis of non-Hodgkins lymphoma (NHL) in elderly patients seems to be poorer than that in patients aged less than 60 years. This may be due to the lower tolerance for combination chemotherapy in the elderly. Aggressive combination chemo-therapy, which is the treatment of choice in intermediate and high grade NHL of adulthood, may be associated with unpredictab1y severe and lethal toxicity and worsened quality of life in the elderly. We investigated the treatment responses, toxicities and prognostic factors of NHL in elderly patients treated with combination chemotherapy. MATERIALS AND METHODS: We treated 116 elderly (>60 yrs) patients with NHL between January 1986 and June 1996 with adriamycin-containing regimens, such as CHOP (cyclo- phosphamide, adriamycin, vincristine, prednisolone), BACOP (bleomycin, adriamycin, cyclophosphamide, vincristine, prednisolone), and mBACOP (methotrexate, bleomycin, adriamycin, cyclophosphamide, vincristine, prednisolone). Patients in this study ranged from 60 to 81 (median 67) years of age. Fifty-five percent of patients were in stage I or II and the rest (45%) were in stage III or IV. The histologic grade was predominantly (91%) of intermediate and high grade type. RESULTS: The treatment responses were complete (CR) in 55% and partial (PR) in 25%. The median durstion of CR was 32 (3-132) months. The CR rate was significantly higher in patients treated with RDI (relative dose intensity) > 75% than that in the patients treated with RDI < 75% (p 0.003), but there was no significant difference in CR rate between treatment regimens (p-0.38). At a median follow up of 48-months (range, 12 to 132 months), the estimated 5-year ovetall survival was 46%. Ann Arbor Stage (I, II vs III, IV), ECOG performance (0-1 vs 2-3), RDI (>75% vs <75%) and the treatment response were important prognostic factors in the univariate analysis, and the treament response (CR vs non-CR) was the only independent prognostic parameter in the multivariate analysis. The most frequent and severe toxicity associated with chemotherapy was infection with or without neutropenia. The rate of severe infection was significantly decreased in the patients supported with G/GM-CSF but not in the dose-reduction group (RDI<75% vs >75%). CONCLUSION: Our data suggests that achievement of the CR after combination chemotherpy is the most important prognostic factor in the elderly patients with NHL. Suboptimal chemotherapy (RDI<75%) reduced the complete remission rate without reducing the likelihood of developing severe toxicities. Optimal chemotherapy with supportive cares involving the use of hematopoietic growth factors may be needed to improve the treatment response and the survival in the elderly patients with aggressive NHL.
Aged ; Bleomycin ; Cyclophosphamide ; Dimethoate ; Doxorubicin ; Drug Therapy ; Drug Therapy, Combination ; Follow-Up Studies ; Hodgkin Disease* ; Humans ; Intercellular Signaling Peptides and Proteins ; Lymphoma, Non-Hodgkin ; Multivariate Analysis ; Neutropenia ; Prognosis* ; Quality of Life ; Vincristine