1/100. Treatment of linear scleroderma with oral 1,25-dihydroxyvitamin D3 (calcitriol) in seven children.Linear scleroderma is a connective tissue disorder that characteristically involves the skin. skin induration and pigmentary changes present in a linear distribution. Severe functional and cosmetic disability may occur, especially in growing children. No effective therapy for the fibrotic stage of scleroderma is available at present. Recently a beneficial effect of oral 1, 25-dihydroxyvitamin D3 (calcitriol) treatment was reported in adults. calcitriol has a dose-dependent inhibition on fibroblast proliferation and collagen synthesis and has immunoregulatory activities. We assessed the efficacy of oral calcitriol treatment in seven pediatric patients with linear scleroderma. During the treatment dietary calcium intake was restricted. calcium, inorganic phosphate, creatinine, and urea in the serum and urine was monitored. The urinary calcium:creatinine ratio was measured. The effects of the treatment were evaluated using a clinical scoring system. No side effects were observed. Five of the seven patients showed a good to excellent improvement of their lesions. One of them partly relapsed after 19 months, but showed an excellent response to a second therapy session with calcitriol. One patient with rapidly progressive disease failed to respond to therapy. Our results indicate that calcitriol can be an effective agent for treating localized scleroderma in children.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
2/100. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases.- - - - - - - - - - ranking = 2keywords = connective (Clic here for more details about this article) |
3/100. A morbid course in a girl with mixed connective tissue disease.We describe an 18-year-old girl with a 13-year history of mixed connective tissue disease whose clinical course was unique: she ultimately developed end-stage kidney and bowel disease that led to a protracted morbid clinical course. We report this case to alert pediatric nephrologists to the importance of early recognition of possible intestinal disease in these patients. Unfortunately, no therapy is currently known to reverse the pathological process in the bowel, but multiorgan transplantation might be an option if the numerous medical complications of end-stage bowel disease can be successfully controlled.- - - - - - - - - - ranking = 29.649800103019keywords = mixed connective, connective (Clic here for more details about this article) |
4/100. Pneumatosis intestinalis in association with connective tissue disease.Pneumatosis intestinalis in association with connective tissue diseases is an unusual combination whose pathogenesis is not yet understood. Furthermore, steroid medication, often used to treat these diseases, may itself cause pneumatosis. Three cases of scleroderma, systemic lupus erythematosus, and amyloidosis in association with pneumatosis and without prior steroid therapy are presented. The small vessel occlusive pathologic processes in these diseases may cause focal areas of mucosal ischemia resulting in small, perhaps transient ulcerations that allow gas to enter the gut wall from the lumen.- - - - - - - - - - ranking = 5keywords = connective (Clic here for more details about this article) |
5/100. octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases.Chronic intestinal pseudoobstruction (CIPO) is a rare syndrome that may occur in association with connective tissue diseases (CTD). Effective management is a major challenge. We report 3 cases in which subcutaneous octreotide was efficacious in the treatment of digestive symptoms in CIPO. In 2 of the 3 cases, previous treatment with domperidone, cisapride, or erythromycin had been unsuccessful. All 3 patients underwent a regimen of oral antibiotics along with octreotide to stimulate small bowel motility. The effects of octreotide were evident within 48 hours after the first injection in all patients. In 2, the efficacy seemed to decrease after 1 week and 6 months respectively, but increasing the dosage led to another remission. CIPO in CTD is a severe condition that can evolve regardless of the underlying disease activity. octreotide appears to be efficacious in improving both clinical symptoms and manometric patterns. When its therapeutic effect diminishes, increasing the dosage can be useful.- - - - - - - - - - ranking = 5keywords = connective (Clic here for more details about this article) |
6/100. Thrombotic thrombocytopenic purpura as an initial presentation of limited systemic sclerosis.Thrombotic thrombocytopenic purpura (TTP) is a rare complication of scleroderma (systemic sclerosis, SSc). In the 5 reports documenting the association of TTP and SSc, the TTP syndrome developed on a background of well established SSc. We describe a 51-year-old woman with a 5 month history of an evolving connective tissue disease syndrome who presented initially with TTP, followed 4 months later by limited cutaneous SSc and Raynaud's phenomenon.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
7/100. scleromyxedema with dermato-neuro syndrome.scleromyxedema is a rare connective tissue disease of unknown cause characterized by a generalized papular eruption, dermal fibroblast proliferation, and monoclonal paraproteinemia. A paroxysmal triad consisting of high fever, seizures, and coma with a flu-like prodrome can rarely occur in patients with scleromyxedema and is termed "dermato-neuro syndrome." We describe a 41-year-old patient with scleromyxedema in whom the dermato-neuro syndrome developed.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
8/100. Pseudoscleroderma associated with lung cancer: correlation of collagen type i and connective tissue growth factor gene expression.Pseudoscleroderma as a paraneoplastic syndrome is a rare disease. We report here a patient with lung cancer (undifferentiated squamous cell carcinoma), who developed acrosclerosis. Using in situ hybridization, marked expression of alpha1(I)-collagen and connective tissue growth factor (CTGF) mRNA was found in fibroblasts scattered throughout the dermis. However, transforming growth factor (TGF)-beta1 expression was not detected. The pattern of CTGF gene expression and collagen synthesis was similar to that in systemic scleroderma. The absence of TGF-beta1 mRNA could indicate that tumour-derived factors induce the expression of CTGF.- - - - - - - - - - ranking = 5keywords = connective (Clic here for more details about this article) |
9/100. mixed connective tissue disease.Three patients with mixed connective tissue disease (MCTD) had clinical features that included a high incidence of Raynaud phenomenon, arthritis, myositis, and swollen hands. The diagnostic laboratory test result was the presence of high titers of antibody to extractable nuclear antigen. These antibody titers are notably reduced or abolished in patients with MCTD when the tanned red blood cells that are used in the test are pretreated with ribonuclease. Speckled antinuclear antibodies were present in all patients. patients with MCTD have a low incidence of renal disease, are responsive to treatment with prednisone, and have a good prognosis.- - - - - - - - - - ranking = 9.9299600206037keywords = mixed connective, connective (Clic here for more details about this article) |
10/100. Bibliographical study of the concurrent existence of anticentromere and antitopoisomerase I antibodies.Some connective tissue diseases are characterised by specific autoantibodies. Although anticentromere or antikinetochore antibodies (ACA), and antitopoisomerase-I or anti-Scl-70 antibodies (ATA), have disease-specific meanings for systemic sclerosis and its CREST variant, respectively, the clinical significance of their concurrent existence has not been clarified. We investigated this condition in our case and with reference to the literature. For this purpose published reports between 1980 and 1998, where both ACA and ATA were measured simultaneously, were analysed by a medline search. In 10 papers 24 patients had both antibodies. In a further 25 reports, covering 3509 subjects who had either ACA or ATA, no concurrent existence was found. Prevalences of ACA (P(ACA)) and ATA (P(ATA)) in exclusive cases varied from 8.8% to 54.5%, and from 11.8% to 87.5%, respectively, whereas P(ACA) varied from 20.0% to 56.6%, and P(ATA) from 16.8% to 63.7% in the reports with patients positive for both. The actual prevalence of simultaneous presence was between 0.7% and 5.6%, significantly lower than the expected probabilities if both antibodies were to occur independently (p<0.005). In concurrently positive cases visceral involvement was characteristic, especially affecting the vascular system, with deterioration of oesophageal function and cutaneous lesions. We suggest that ATA and ACA do not coexist by chance, and that clinical characteristics with coexistence have a significance for the classification of scleroderma.- - - - - - - - - - ranking = 1keywords = connective (Clic here for more details about this article) |
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