BACKGROUND: Recent clinial observations have suggested that colchicine, which is in frequent use in gout can affect the conneciive tissue metabolism in skin and other ti.ues. OBJECTIVE: This study suggest that further development of colchiine might provide a novel means of modulating collagen gen expression in patients with fibrotic disease. METHOD: We examed the effect of colchicine on the expres, on of collagen, fibronectin and collagenase gene by skin filroblast culture hy Nort.hern and dot-blot iybridization. Result : The rate of transcription of genomic DNA corresponding o type I collagen and libvonectin was reduced in colchicine-treated cultures but collagenase was not. reduced. Canclusion : The microti.ikile disruptive agent, colchicine, reduced the expression of type I collagen and fibronectin in a dose-(lependent manner. This st.udy suppor that colchicine is one of the promising antifibrogenic drugs curvently being tested as a treatment, of hun an fibrotic disease.
Colchicine* ; Collagen Type I ; Collagen* ; Collagenases ; DNA ; Fibroblasts ; Fibronectins ; Gene Expression* ; Gout ; Humans ; Metabolism ; Skin

PURPOSE: The purpose of this study was to evaluate clinical manifestation, etiology and prognosis of hypocalcemic infants who were admitted with seizure. METHODS: We reviewed medical records of 32 infants admitted at the Asan Medical Center with hypocalcemic seizure retrospectively. We classified patients into vitamin D deficiency group(n=7, 21.9%), transient hypoparathyroidism group(n=4, 12.5%), relative hypoparathyroidism with hyperphosphatemia group(n=16, 50%), and others(n=5, 15.6%) according to the laboratory results. RESULTS: Of the 32 patients, 29 patients were improved. There were no differences in gestational age and birth weight among the three groups. In the vitamin D deficiency group, age of onset was later than those of the transient hypoparathyroidism group and relative hypoparathyroidism with hyperphosphatemia group(51.6+/-2.7 vs 8.3+/-.5, 8.2+/-.6 days). In the age when all laboratory results were normalized, transient hypoparathyroidism group was younger than those of vitamin D deficiency group and relative hypoparathyroidism group(33.2+/-4.6 vs 93.6+/-8.5, 77.1+/-2.4 days). In the total treatment period, relative hypoparathyroidism with hyperphosphatemia group was longer than those of vitamin D deficiency group and transient hypoparathyroidism group(68.9+/-3.5 vs 42.0+/-5.0, 25.0+/-4.3 days). Others included two 22q11.2 deletion syndrome patients, a congenital hypoparathyroidism, a pseudohypoparathyroidism, and an early neonatal hypocalcemia. CONCLUSION: Transient hypoparathyroidism and hyperphosphatemia were major causes of neonatal hypocalcemia. And high calcitonin and peripheral organ resistance to parathyroid hormone act on hypocalcemia. In infants after one month, vitamin D deficiency was also an important cause of hypocalcemia. Most of the patients were improved within 1-2 months after proper management, but relative hypoparathyroidism with hyperphosphatemia group needed longer treatment. So, it is necessary to perform a systematic study for several complex causes that explain above fact.
Birth Weight ; Calcitonin ; Chungcheongnam-do ; DiGeorge Syndrome ; Gestational Age ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Infant* ; Medical Records ; Parathyroid Hormone ; Prognosis ; Pseudohypoparathyroidism ; Retrospective Studies ; Seizures ; Vitamin D Deficiency

Lupus vulgaris is most prevalent on exposed parts, especially the face but can also develop on exetremities. Lupus vulgaris originates from tuberculosis elsewhere in the body by hematogenous, lymphatic, or contiguous spread. A 19-year-old male patient came to our department. The patient had had many recurrent oozing and verrucous plaques and crusts on the left foot for one year. A skin biopsy from the lesion on the left dorsum of the foot showed scattered well defined granulomas consisting of the epithelioid cell clusters with Langerhans and foreign body type giant cells in the mid dermis. Caseation necrosis was slight. There were no bacilli on AFB staining. The multi test CMI for tuberculin was highly positive. A chest X-ray did not show any abnormal findings. The presence of Mycobacterium tuberculosis DNA was demonstrated by polymerase chain reactions (PCR) for detection of mycobacterial DNA from a routinely prepared paraffin-embedded skin specimen. Herein we report a very atypical case of lupus vulgaris confirmed by PCR.
Biopsy ; Dermis ; DNA ; Epithelioid Cells ; Foot ; Giant Cells, Foreign-Body ; Granuloma ; Humans ; Lupus Vulgaris* ; Male ; Mycobacterium tuberculosis ; Necrosis ; Polymerase Chain Reaction ; Skin* ; Thorax ; Transplants* ; Tuberculin ; Tuberculosis ; Young Adult

BACKGROUND: Neurofibroma, the hallmark of neurofibromatosis, is a cutaneous or subcutaneous lesion, with a variable clinical presentation. Histologically, neurofibroma consists of proliferation of nerve derived cellular elements, together with an abundant, collagenous extracellular matrix. Specifically, neurofibroma has been shown to contain 30-50% collagen in its matrix. Objective 5. METHODS: We examined the expression of extracellular matrix genes (collagen, fibronectin, laminin), TGF-b mRNA and Ha-ras oncogene mRNA by using Northern and slot-blot hybridization and immunoperoxidase stains. Result: In Northern blot analysis, Ha-ras and TGF-b genes revealed respectively, 8.8kb and 2. 5kb sized mRNA transcripts in neurofibroma. These parameters were normal in the control. The expression of these genes were 1.9, 2.0 fold increased in neurofibroma. In slot-blot analysis, expression of type I collagen showed fibronectin genes to be 2,401+210, 540+43, respectively, in neurofibroma. So there were 3.7 fold, 2.1 fold, differences respectively, compared to the normal control. However, there were no significant changes of type IV collagen and laminin Bl mRNA levels between neurofibroma and normal skin tissues. Irnmunoperoxidase staining by rnonoclonal anti type IV collagen antibody in neurofibroma showed type IV collagen to be diffusely and weakly stained in tissue. On staining by monoclonal anti-laminin antibody, laminin was stained in a matrix and around vessels. CONCLUSION: The increased expression of extracellular matrix genes may suggest that there is a subpopulation of fibroic cells in neurofibroma which are stimulated by TGF-b. Ha-ras genes which might have accumulated with the differentiation of neural tissue may be related to the pathogenesis of neurofibroma tissue formation. Further studies are needed to determine whether the other factors are related to the pathogenesis of neurofibroma.
Blotting, Northern ; Collagen ; Collagen Type I ; Collagen Type IV ; Coloring Agents ; Extracellular Matrix* ; Fibronectins ; Genes, ras* ; Laminin ; Neurofibroma ; Neurofibromatoses* ; RNA, Messenger ; Skin

No abstract available.
Nuchal Cord* ; Ultrasonography*

No abstract available.
Gwangju* ; Humans

BACKGROUND: Methods to detect and quanitify Mycobacterium leprae(M. leprae)are needed for studies involving the epidemiology, pathogenesis, and chemotherapy of leprosy. Serological assays and skin tests lack the sensitivity and specificity to serve as diagnostic tool for M. leprae infection. The polymerase chain reaction(PCR) based on the selective amplification of an 530-bp frangment of the gene encoding the proline-rich antigen of M. leprae was performed with sections of fixed or frozen biopsy samples from leprosy patients. OBJECTIVE: This study was done to investigate the applicability of PCR for the detection of low numbers of M. leprae in tissues and peripheral blood. METHODS: The PCR was used to amplify a 530-base-pair M. leprae DNA with the thermoxtable Taq DNA polymerase. RESULTS: The In frozen skin tissues and peripheral blood of leprosy patients. relatively high detection rates of PCR products was achieved by using direct gel analysis as well as Southern blot hybridization. CONCLUSION: These results suggest that PCR amplification for the detection of M. leprae may be useful for the epidemiologic study of large papulations as well as coinical astudies on the individual patients.
Biopsy ; Blotting, Southern ; DNA ; Drug Therapy ; Epidemiologic Studies ; Epidemiology ; Humans ; Leprosy ; Mycobacterium leprae* ; Mycobacterium* ; Polymerase Chain Reaction* ; Sensitivity and Specificity ; Skin ; Skin Tests ; Taq Polymerase

Pleomorphic xanthoastrocytoma is histologically characterized by marked cellular pleomorphism of lipid-laden neoplastic astrocytes and bizarre giant cells showing mitotic figures and high cellularity. Inspite of its ominous-looking microscopic features, howerver, the prognosis is usually favorable. This tumor develops mainly in the supratentorial area of young people and frequently involves the leptomeninges. We experienced a case of pleomorphic xanthoastrocytoma in 18 year-old-male. In addition to the cellular pleomophism, the prominent reticulin fibers surround the individual tumor cells or the tumor cells nests. Immunohistochemical staining and electron microscopy revealed glial fibrillary acidic protein(GFAP) expression and pericytoplasmic basal lamina in the tumor cells.

BACKGROUND: The aim of this study is to evaluate the therapeutic effect of splenectomy in children with chronic idiopathic thrombocytopenic purpura(ITP) who do not respond to corticosteroids and intravenous immunoglobulin(IVIG). METHODS: Fifteen patients(7 boys and 8 girls) who were admitted to St. Mary's Hospital under the diagnosis of ITP from December 1990 to February 1997 were enrolled In this study. All had chronic forms(more than 6 months after diagnosis) and were refractory to corticosteroids(prednisolone 1~2mg/kg) and IVIG(400mg/kg for 5 days or 1 g/kg for 2 days). We you-finely employ pneumococcal vaccine in 13 patients before splenectomy slnce 1992. Antiplatelet antibody was examed in 10 patients. We measured platelet count at preoperative time, postoperative 1 day, 1 month, and 3 months or more. Hematologic results were analyzed according to Berchtold and McMillan's criteria (1) complete response(CR) >120 x 10(9)/l; (2) partial response(PR) >50 x 10(9)/l (3) no response(NR) < 50 x 10(9)/l. RESULTS: The median age at operation was 10 yr(6~16). The median time interval between diagnosis and splenectomy was 2 yr 2 mo(6 mo~5 yr 5 mo). Antiplatelet antibody was positive in 2 cases of 10 cases(20%). Of 15 cases, 12 cases were corticosteroid resistant and 3 cases were corticosteroid dependent. The postsplenectomy platelet count(median 407 x 10(9)/l of 1 day; 254 x 10(9)/l of 1 month; 227 x 10(9)/l of 3 months or more) was significantly higher than presplenectomy platelet count(median 9 x 10(9)/l)(P<0.05). All patients showed complete or parial response throughout the follow up period. Accessory spleen was found in 1 case and removed during operation. There were no serious complications following splenectomy except mild fever in 2 cases(14%). CONCLUSION: Splenectomy appears to be an effective and relatively safe treatment for patients with chronic ITP who have had inadequate response to conventional therapies.
Adrenal Cortex Hormones ; Blood Platelets ; Child ; Diagnosis ; Fever ; Follow-Up Studies ; Humans ; Platelet Count ; Purpura, Thrombocytopenic* ; Spleen ; Splenectomy*

OBJECTIVE: In order to compare the clinical effect and frequency of side effects between d-penicillamine and bucillamine, a chemical derivative of d-penicillamine, we conducted a randomized, controlled clinical trial. METHODS: 22 and 24 patients were allocated in each treatment arm, after stratification according to functional class, anatomical stage, and concomittant steroid use. We evaluated tender joint count, swollen joint count, tenderness score, duration of morning stiffness, and grip strength every 4 weeks. Westergren ESR, hemoglobin, leucocyte and platelet count were also checked every 4 weeks, and C-reactive protein(CRP)quantitation and rheumatoid factor(RF) titration were checked at baseline and 16 weeks. Study period spanned 16 weeks. RESULTS: At the end of the trial, both swollen joint count and tenderness score decreased significantly from baseline in both groups, whereas tender joint count decreased siginficantly in bucillamine-treated group only. Grip strength also improved significantly in bucillamine group. Duration of morning stiffness decreased in both groups. When we arbitrarily defined response as more than 30% decrease in both tender and swollen joint count from baseline, 27% of bucillamine group and 33% of d-penicillamine group responded. The response rate did not differ significantly between the 2 groups. ESR and RF decreased in both groups, whereas CRP decreased significantly only in bucillamine group. The frequency of side effects lasting more than 1 day and possibly related to drug was 14% and 37% in bucillamine and d-penicillamine group, respectively. The difference was statistically not significant. CONCLUSIONS: Bucillamine is as effective as d-penicillamine in the treatment of rheumatoid arthritis. The frequency of side effect tended to be lower.
Arm ; Arthritis, Rheumatoid* ; Hand Strength ; Humans ; Joints ; Penicillamine* ; Platelet Count