1/68. Optic disc edema in neonatal onset multisystem inflammatory disease (NOMID).PURPOSE: To inform ophthalmologists about neonatal onset multisystem inflammatory disease (NOMID), a rare condition with ophthalmologic manifestations. methods: We report a single case of NOMID with optic disc edema. RESULTS: A 28-month-old child with neonatal rash, arthropathy, central nervous system (CNS) involvement, and optic disc edema was diagnosed with NOMID. CONCLUSIONS: The finding of posterior uveitis or optic disc edema in a child with juvenile onset arthritis may allow the differentiation of NOMID from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 1keywords = multisystem inflammatory disease, inflammatory disease, neonatal onset, multisystem (Clic here for more details about this article) |
2/68. Increased CA 125 serum levels in patients with advanced acute leukemia with serosal involvement.BACKGROUND: CA 125 is a tumor marker used for the diagnosis and monitoring of ovarian carcinoma. This marker also has been found to be increased in patients with serosal effusion derived from nonneoplastic inflammatory disease and in a few instances of advanced non-Hodgkin lymphoma with serosal involvement. methods: CA 125 levels were tested in the serum of 15 patients with acute myeloblastic leukemia (AML) at the time of diagnosis and in 3 patients with advanced leukemia with serosal involvement. In two patients with elevated serum CA 125 levels, a CA 125 assay was performed on leukemic cells and on the supernatant fluid of short term liquid culture. RESULTS: Increased serum CA 125 was found in the three patients with acute leukemia with extramedullary localization and serosal effusion, whereas it was normal in 15 AML patients tested at the time of diagnosis. CA 125 was not detectable in leukemic cell extracts nor in the supernatant fluid of primary cultures. CONCLUSIONS: These results indicate that leukemic cells were unable to produce CA 125 and suggest that its elevation in the serum is likely due to a serosal inflammatory reaction caused by the leukemic infiltration.- - - - - - - - - - ranking = 0.0051195615355801keywords = inflammatory disease (Clic here for more details about this article) |
3/68. Recurrent invasive adenocarcinoma after hysterectomy for cervical adenocarcinoma in situ.BACKGROUND: Unlike its squamous counterpart, therapy for cervical adenocarcinoma in situ with positive endocervical cone margin remains controversial. CASE: A 52-year-old gravida 2, para 1,0,1,1, presented with vaginal bleeding. Gynecologic history was significant for cervical cold knife conization with a positive endocervical margin and endocervical curettage with atypical endocervical cells. Repeat cone biopsy was considered unsafe given the large initial cone specimen. An extrafascial hysterectomy was performed 5 weeks later and pathology confirmed a disease-free cervix. Pap smear performed 1 year later was interpreted as recurrent adenocarcinoma but later downgraded to inflammation. Inspection and random biopsies of the vaginal cuff revealed only inflammation. Two subsequent Pap smears also returned inflammation. Seventeen months after the hysterectomy physical examination revealed a 2 x 3-cm smooth mass at the vaginal cuff. biopsy revealed invasive adenocarcinoma. The patient underwent an upper vaginectomy followed by postoperative pelvic radiation. CONCLUSION: This case suggests that despite extrafascial hysterectomy for presumed adenocarcinoma in situ of the cervix, a residual focus could remain and present later as invasive adenocarcinoma.- - - - - - - - - - ranking = 8.6773727519957E-7keywords = cold (Clic here for more details about this article) |
4/68. Atrophoderma of moulin with preceding inflammation.A 16-year-old Vietnamese man presented to the dermatology Clinic with a 10-year history of bizarre brown patches, which initially started as red asymptomatic "bumps" on the trunk, upper and lower extremities, and face. His past medical history was significant for hypothyroidism and idiopathic urticaria. He was on Eltroxin for hypothyroidism. The family history was noncontributory. physical examination revealed two types of lesion: erythematous, well-circumscribed papules in a linear configuration along with linear hyperpigmented atrophic patches following Blaschko's lines were noted on the lower extremities (Fig. 1), right upper extremity, right flank (Fig. 2), and right jawline. Initial biopsies taken from the papular lesions on the right thigh and right elbow revealed the following changes. The first biopsy showed a slightly thinned epidermis with prominent dilated blood vessels in the superficial dermis. There also appeared to be a slight increase in the amount of collagen in the deep dermis. The findings were reported as in keeping with "epithelial atrophy." The second biopsy from the lesion on the right elbow revealed an acanthotic epidermis. The granular layer was absent in several areas and there was marked overlying parakeratosis. In the dermis, there was a heavy perivascular lymphocytic infiltrate. The appearances were consistent with a psoriasiform dermatitis (Fig. 3). A biopsy taken from the left thigh approximately 18 months later showed slight irregular acanthosis with dermal edema, dilated blood vessels, and a patchy lymphocytic infiltrate. The appearances were compatible with mild inflammation.- - - - - - - - - - ranking = 5.4946303452253E-6keywords = urticaria (Clic here for more details about this article) |
5/68. The CINCA syndrome: a rare cause of chronic arthritis and multisystem inflammatory disorders.Chronic infantile neurological cutaneous articular (CINCA) syndrome is a rare disorder with neonatal onset characterised by a chronic progressive inflammatory process with skin rash, articular and central nervous system involvement. This primary systemic inflammatory disorder should be distinguished from juvenile rheumatoid arthritis (JRA). Although the articular findings are characteristic features of CINCA syndrome, there is a certain degree of variability in the articular involvements which are not always symmetrical nor is the degree of severity uniform. The etiology of CINCA syndrome remains unknown. No single treatment has been found to be effective. This syndrome is known in the American medical literature as infantile onset multisystem inflammatory disease (IOMID).- - - - - - - - - - ranking = 0.20045246054338keywords = multisystem inflammatory disease, inflammatory disease, neonatal onset, multisystem (Clic here for more details about this article) |
6/68. Acute flare-up of conjunctival graft-versus-host disease with eosinophil infiltration in a patient with chronic graft-versus-host disease.Chronic graft-versus-host disease (cGvHD) is a disabling alloimmune disease. Acute flare-ups of alloimmune reactions can occur even at the chronic clinical stage necessitating modulation of immunosuppression therapy. We studied conjunctival lesions of a patient with an acute flare-up in cGvHD. Along with severe GvHD lesions, biopsies showed eosinophils with pathological signs of activation. Since eosinophil recruitment and activation is observed in flare-ups of patients with autoimmune or inflammatory bowel diseases, we suggest that activated eosinophils in target organs may be a marker of evolutive lesions in alloimmune reactions as in other kinds of inflammatory diseases.- - - - - - - - - - ranking = 0.0051195615355801keywords = inflammatory disease (Clic here for more details about this article) |
7/68. Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes.Chronic infantile neurological cutaneous and articular (CINCA) syndrome is a severe chronic inflammatory disease of early onset, characterized by cutaneous symptoms, central-nervous-system involvement, and arthropathy. In the present study, we report, in seven unrelated patients with CINCA syndrome, distinct missense mutations within the nucleotide-binding site of CIAS1, a gene encoding cryopyrin and previously shown to cause Muckle-Wells syndrome and familial cold urticaria. Because of the severe cartilage overgrowth observed in some patients with CINCA syndrome and the implications of polymorphonuclear cell infiltration in the cutaneous and neurological manifestations of this syndrome, the tissue-specific expression of CIAS1 was evaluated. A high level of expression of CIAS1 was found to be restricted to polymorphonuclear cells and chondrocytes. These findings demonstrate that CIAS1 missense mutations can result in distinct phenotypes with only a few overlapping symptoms and suggest that this gene may function as a potential inducer of apoptosis.- - - - - - - - - - ranking = 0.011041863083592keywords = inflammatory disease, articular syndrome, urticaria, cold (Clic here for more details about this article) |
8/68. crohn disease in patients with familial mediterranean fever.crohn disease and familial mediterranean fever (FMF) are inflammatory diseases characterized by abdominal pain and fever. The concurrence of the 2 diseases (FMF-CD) may pose a challenge to diagnosis and treatment. We undertook the present study to determine the prevalence of crohn disease in FMF and to characterize FMF-CD patients clinically and genetically. Using a computerized search, the patients of our FMF clinic were screened for a concomitant diagnosis of crohn disease. patients and their medical records were thoroughly examined, and their dna was genotyped for mutations in the MEFV gene. control groups of ethnically and sex-matched patients suffering from each of the diseases alone, either crohn disease or FMF, were used for comparison. We identified 7 patients with concomitant crohn disease and FMF, which is more than the expected prevalence in the general population (p = 0.03). crohn disease presented at a significantly later age in the FMF-CD group (40.6 /- 10.0 yr versus 26.2 /- 11.4 yr; p < 0.004). Disease severity and other characteristics of crohn disease were comparable to the crohn disease control group. Contrary to the FMF control group patients, FMF in FMF-CD patients was characterized by a higher attack frequency (p < 0.05) and increased prevalence of amyloidosis (p < 0.02). The overall severity score was similar in both groups. In conclusion, crohn disease appears to be more prevalent in FMF and presents later than in patients without FMF. FMF in this group of patients shows a higher attack frequency and is more often complicated by amyloidosis.- - - - - - - - - - ranking = 0.0051195615355801keywords = inflammatory disease (Clic here for more details about this article) |
9/68. New thoughts on sepsis: the unifier of critical care.In the united states, more than $16 billion annually is spent managing patients with severe sepsis and its sequelae. Insight into the inflammatory response, endothelial tissue, and the coagulation cascade suggest promising new treatment regimens that limit morbidity and mortality due to sepsis and multisystem organ failure. This article will discuss new information regarding the pathophysiology of the inflammatory response and sepsis. Current thoughts on clinical management and a promising new agent, Activated protein c, will be presented.- - - - - - - - - - ranking = 0.00011311513584611keywords = multisystem (Clic here for more details about this article) |
10/68. Pathological and virological assessment of acute HTLV-I-associated myelopathy complicated with encephalopathy and systemic inflammation.HTLV-I-associated myelopathy, also known as tropical spastic paraparesis (HAM/TSP), is a chronic inflammatory disease of the spinal cord. Acute cases are uncommon. We report the case of a 41-year-old woman with acute HAM/TSP complicated with encephalitis, an intense inflammatory reaction of the nervous system and lymphocytic infiltration of skeletal muscles, liver, salivary, adrenal and pituitary glands. The immunohistochemical studies of the lymphocytes surrounding blood vessels showed both B- and t-lymphocytes, in similar proportion, with both CD4- and CD8-positive cells. In addition, many perivascular and scattered macrophages were observed. adult T-cell leukemia/lymphoma (ATL) was ruled out. The marrow aspirate was normal. Serial cerebrospinal fluid (CSF) analysis showed presence of htlv-i antibodies, but without intrathecal synthesis of specific antibodies. Determination of HTLV-I viral loads demonstrated increased levels in the CSF relative to the peripheral blood and may be associated with widespread inflammation. The pathological and immunological findings may help understand the role of immune-reactive cells in the pathogenesis of HTLV-I-associated myelopathy.- - - - - - - - - - ranking = 0.0051195615355801keywords = inflammatory disease (Clic here for more details about this article) |
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