1/75. Mobility challenges and solutions for fibrodysplasia ossificans progressiva.Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive soft tissue ossification. Although signs may be present at birth, the first appearance of ectopic bone typically occurs in early childhood. The primary target is the axial musculature. Eventually ectopic bone also occurs in ligaments, fascia, aponeurosis, tendons, and joint capsules of the appendicular skeleton with a proximal to distal predilection. As the disease advances, mobility becomes restricted, and affected individuals are typically limited to bed or chair by their early 30s. This report describes a 30-year-old woman with advanced FOP. She had a fused spine and a fixed pelvis, with hips and knees locked in flexion and feet in plantarflexion. Her upper limb mobility was similarly restricted. She was not able to stand upright or sit independently. The modification of a commercially available power wheelchair that allowed the patient to maintain her employment as a preschool teacher and custom shoes are described. Creative physiatric intervention is essential to liberate human potential for people with FOP.- - - - - - - - - - ranking = 1keywords = ligament (Clic here for more details about this article) |
2/75. Late development of antidesmoglein 1 antibodies in pemphigus vulgaris: correlation with disease progression.The coexistence of antidesmoglein 3 (Dsg3) and antidesmoglein 1 (Dsg1) autoantibodies is well described in patients with pemphigus vulgaris (PV); however, there is little evidence of sequential development of these two autoantibodies. Autoantibody responses to Dsg3 and Dsg1 were studied in seven PV patients over time by enzyme-linked immunosorbent assay, using baculovirus expressed recombinant fusion proteins. All patients had anti-Dsg3 IgG antibodies at presentation. Two patients developed anti-Dsg1 later in the course of the disease. The transition in autoantibody profile was associated with disease progression to generalized PV involving mucous membranes and skin in both patients; one patient initially presented with a predominantly mucosal phenotype, the other with herpetiform pemphigus-like features. These findings demonstrate that there is an extension of autoimmune response from anti-Dsg3 only to both anti-Dsg3 and anti-Dsg1 in some patients, which is associated with an alteration in clinical expression in PV.- - - - - - - - - - ranking = 0.37127070226725keywords = membrane (Clic here for more details about this article) |
3/75. Rapid progression of primary vaginal squamous cell carcinoma in a young hiv-infected woman.BACKGROUND: The association of human immunodeficiency virus (hiv) infection with rapid progression of cervical and anal squamous cell carcinoma has been clearly established by several studies. Human papilloma virus (HPV) infection of the anogenital tract is believed to be the causative agent of cervical, anal, vaginal, and vulvar squamous cell carcinoma. While a myriad of reports exist in the literature pertaining to the rapid progression of cervical and anal carcinoma in hiv-infected patients, no association of hiv infection and vaginal carcinoma has been reported. We present an unusual case of a young woman infected with hiv who was diagnosed with advanced vaginal carcinoma and succumbed to her disease shortly thereafter despite aggressive treatment. CASE: A 40-year-old woman with a 2-year history of hiv infection presented with Stage IVA squamous cell carcinoma of the vagina and a large vesicovaginal fistula from the tumor eroding through the posterior bladder wall. Computed tomography (CT) of the abdomen and pelvis revealed a large tumor replacing the vagina with mild hydronephrosis and diffuse pelvic and inguinal lymphadenopathy. She underwent urinary diversion with a transverse colon conduit followed by pelvic radiation with weekly cisplatin chemosensitization. A repeat CT scan of the abdomen and pelvis upon completion of her treatment revealed progression of disease with multiple liver metastases and gastrohepatic ligament adenopathy. She subsequently died of advanced metastatic vaginal carcinoma 2 months after completion of treatment. CONCLUSION: Due to the rarity of primary vaginal carcinoma, the clinical behavior of this neoplasm in the hiv-infected patient is poorly understood. Our case indicates that, although vaginal carcinoma is a disease of the elderly, young women infected with hiv and HPV are predisposed not only to develop cervical or anal carcinoma but also may be at increased risk for vaginal carcinoma with more aggressive and less responsive disease. Furthermore, although vaginal carcinoma is usually a slow-growing neoplasm, this case illustrates the aggressive behavior of such a tumor when associated with hiv infection.- - - - - - - - - - ranking = 1keywords = ligament (Clic here for more details about this article) |
4/75. Skewfoot in patients with osteogenesis imperfecta.We report skewfoot deformities in two patients who have osteogenesis imperfecta. A discussion will follow proposing etiologies of skewfoot, speculating that the ligamentous laxity often present in children who have osteogenesis imperfecta may predispose the development of skewfoot.- - - - - - - - - - ranking = 1keywords = ligament (Clic here for more details about this article) |
5/75. Do B cells influence disease progression in chronic synovitis? Lessons from primary hypogammaglobulinaemia.We describe a 62-yr-old male patient with primary hypogammaglobulinaemia (PH) who fulfilled the 1987 American Rheumatism association/American College of rheumatology revised diagnostic criteria for rheumatoid arthritis (RA) but, despite persistent symmetrical synovitis, did not develop erosions. virology studies and blood and synovial fluid (SF) cultures were consistently negative; a search for crystals in the SF was unrevealing. Peripheral blood (PB) B cells were absent, whilst the PB CD3( ) cell count was normal. The ratio of naive (CD45RA( )) to memory (CD45R0( )) cells was also normal (1:1) but the CD4:CD8 ratio was reversed. To our knowledge, this is the first report which combines the immunophenotypic analysis of the PB with that of the SF and synovial membrane (SM). This confirmed the absence of B cells and the reversed CD4:CD8 ratio. However, as in other chronic arthropathies, the SF and SM cellular infiltrate consisted almost exclusively of memory T cells, consistent with the preferential localization of this subset to inflamed tissues. This case indicates that synovitis can proceed persistently in the absence of B cells and that the migratory mechanisms of T cells are not altered. However, the case suggests that the absence of B cells and negativity for rheumatoid factor, combined with an increased presence of CD8( ) (suppresser/cytotoxic) T cells in the joint, might contribute to the non-erosive nature of the synovitis.- - - - - - - - - - ranking = 0.37127070226725keywords = membrane (Clic here for more details about this article) |
6/75. Remitting asymmetrical pitting oedema in systemic lupus erythematosus: two cases studied with magnetic resonance imaging.To our knowledge, only three cases of remitting symmetrical pitting oedema in systemic lupus erythematosus have been reported so far. This is the first report of two patients with asymmetrical pitting oedema and systemic lupus erythematosus. The first patient presented two consecutive episodes of unilateral oedema. The first episode involved the distal part of the right lower extremity and remitted spontaneously while the second involved the volar region of the left wrist and distal half of the forearm and promptly responded to steroids. magnetic resonance imaging of the left wrist and forearm showed tenosynovitis of the flexor tendons and subcutaneous oedema. The second patient instead developed a single episode of pitting oedema of the distal part of the right lower extremity that resolved spontaneously. magnetic resonance imaging of the right ankle and foot showed subcutaneous oedema without involvement of the tendon sheaths or osteoarticular and ligamental structures. These two cases suggest that pitting oedema in systemic lupus erythematosus may occasionally be asymmetric and associated or not with tenosynovitis.- - - - - - - - - - ranking = 1keywords = ligament (Clic here for more details about this article) |
7/75. coronary angiography and intravascular ultrasound.Clinicians have long used the size of the lumen and the angiogram as a predictor of coronary events. However, cardiovascular disease is not a disease of the lumen but a disease of the vessel wall. In early stages, atherosclerosis outwardly remodels the external elastic membrane; only late in the disease process does luminal narrowing occur, enabling angiographic detection. This has profound implications for drug therapy, because approximately 70% of patients present with acute myocardial infarction (MI) or sudden death, not angina as the first symptom of coronary disease. Intravascular ultrasound (IVUS) can provide detailed images of the artery and is the only technique currently available that enables physicians to routinely visualize coronary plaques. Due to its sensitivity in measuring plaque volume and content, IVUS may be a useful surrogate marker to evaluate the atherosclerotic process in smaller numbers of patients than required for conventional clinical endpoint trials.- - - - - - - - - - ranking = 0.37127070226725keywords = membrane (Clic here for more details about this article) |
8/75. Sarcomatoid chordoma: chordoma with a massive malignant spindle-cell component.We report a case of chordoma containing a spindle cell sarcomatoid component with a gradual transition from conventional chordoma. Immunohistochemically, many tumor cells in both conventional chordoma and sarcomatoid components were positive for cytokeratins (AE1/AE3, CAM5.2) and epithelial membrane antigen as well as vimentin. This report provides a rare example of sarcomatoid chordoma. Familiarity with this type of bone tumor should help to avoid confusion with dedifferentiated chordoma and other spindle cell sarcomas or carcinomas.- - - - - - - - - - ranking = 0.37127070226725keywords = membrane (Clic here for more details about this article) |
9/75. Successful steroid therapy for cefdinir-induced acute tubulointerstitial nephritis with progressive renal failure.A 58-year-old woman was admitted to our hospital because of renal dysfunction that continued to progress even after withdrawal of cefdinir, the presumed cause of acute renal failure. Renal histologic findings included interstitial fibrosis accompanied by moderate lymphocytic infiltration, and tubular atrophy with reduced numbers of epithelial cells. mesangial cells and glomerular basement membranes were nearly normal. Scintigraphy with 67gallium disclosed diffuse abnormal accumulation in both kidneys. A lymphocyte stimulation test with cefdinir was positive. The patient was diagnosed with acute tubulointerstitial nephritis caused by cefdinir. serum creatinine concentrations continued to rise after withdrawal of the drug, but steroid therapy was effective in normalizing renal function.- - - - - - - - - - ranking = 0.37127070226725keywords = membrane (Clic here for more details about this article) |
10/75. cholesteatoma behind an intact tympanic membrane: histopathologic evidence for a tympanic membrane origin.BACKGROUND: Several theories have been proposed with respect to the origin and pathogenesis of cholesteatoma behind an intact tympanic membrane. CASE REPORT: The authors describe a case of cholesteatoma behind an intact tympanic membrane in a 71-year-old man with a history of tympanic membrane retraction fixed to the incus without evidence of a perforation. The membrane eventually became detached, and remnants of keratinizing squamous epithelium were found on the incus. DISCUSSION: Mechanisms such as metaplasia, ectopic epidermis rests, or ingrowth of meatal epidermis have been proposed to explain the pathogenesis of cholesteatoma behind an intact tympanic membrane. These findings, based on temporal bone histopathology, support the role of an acquired epidermal rest. CONCLUSIONS: This case report provides evidence that cholesteatoma behind an intact tympanic membrane can be established from a resolved retraction of the pars tensa of the tympanic membrane.- - - - - - - - - - ranking = 5.5690605340088keywords = membrane (Clic here for more details about this article) |
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