1/8. Rapid increase in bone mineral density in a child with osteoporosis and autoimmune hypoparathyroidism treated with PTH 1-34.We describe a 16-year-old girl with autoimmune polyglandular syndrome type 1 including hypoparathyroidism, who had osteoporosis that improved rapidly with parathyroid hormone replacement therapy. patients with hypoparathyroidism usually have high bone mass. Our patient developed vertebral compression fractures at age 10, shortly after hypoparathyroidism was diagnosed. She continued to have low lumbar bone mass until age 16, when a dual energy x-ray absorptiometry (DEXA) revealed a Z score of - 2.2 SD. Several factors including decreased physical activity, total body magnesium depletion, and intermittent ketoconazole and short-term prednisone treatment, may have contributed to the development and progression of osteoporosis. Therapy with synthetic human parathyroid hormone (PTH) 1-34 rapidly normalized lumbar bone mass, as assessed by DEXA.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/8. Autoimmune pancreatitis presenting as a mass in the head of the pancreas: a diagnosis to differentiate from cancer.We report a case of autoimmune pancreatitis presenting as a mass in the head of the pancreas that was successfully diagnosed without pancreaticoduodenectomy. The patient was a 64-year-old man who had no complaint. A routine physical checkup unexpectedly revealed mild diabetes and a low-echoic mass in the pancreatic head. The diagnosis was made by noting irregular narrowing of the main pancreatic duct, hypergammaglobulinemia, and increased immunoglobulin g levels. An open wedge biopsy of the mass was performed; this showed a marked fibrosis with lymphocyte- or macrophage-predominant inflammatory infiltrates. Immunohistochemical study revealed that the remnant acinar cells expressed Fas (CD95) ligand and not Fas. We review some of the literature and discuss various features and diagnostic clues of autoimmune pancreatitis. awareness of this pathologic condition may prevent confusion with pancreatic malignancy and unnecessary surgery.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/8. Open-label study of clarithromycin in patients with undifferentiated connective tissue disease.OBJECTIVE: The macrolide family of antibiotics (erythromycin, clarithromycin, and others), have both antimicrobial and immunomodulatory effects. This study explored the effect of clarithromycin on the clinical course of patients with undifferentiated connective tissue disease (UCTD) in a 12-week open-label study. methods: The diagnosis of UCTD was based on symptoms/signs of connective tissue disease, and the presence of 1 or more positive autoimmune disease tests, but with insufficient criteria to make a definitive diagnosis. Screening and monthly follow-up visits over 12 weeks included the following: history and physical examination; concurrent medications; the 68/66 tender/swollen joint count; visual analog scores 0 to 100 mm for patient and physician global assessment of disease activity, and patient pain; antinuclear antibody panel, rheumatoid factor, erythrocyte sedimentation rate, c-reactive protein, and blood chemistry. RESULTS: Seven patients with rheumatic disease were treated with clarithromycin; 6 of 7 had symptomatic relief. Two subjects treated empirically before the decision to perform an open-label study responded favorably. Four of 5 patients who completed the prospective open-label study had mean maximal improvements from baseline of 78, 75, and 79% in patient pain, patient global, and investigator global assessments, respectively. pain relief occurred as early as 1 week. Drug withdrawal with rechallenge in 2 patients resulted in flare followed by recapture of symptomatic relief. CONCLUSIONS: clarithromycin, a macrolide antibiotic, led to clinical improvement in patients with UCTD. Efficacy and safety data support further investigation of macrolide antibiotic use as a primary or adjunctive treatment in various connective tissue diseases.- - - - - - - - - - ranking = 9.1698765755872keywords = physical examination, physical (Clic here for more details about this article) |
4/8. exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II arnold-chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/8. Benign hypergammaglobulinemic purpura of Waldenstrom associated with sjogren's syndrome. Case report and review of immunologic aspects.Since its first description, fewer than 150 cases of benign hypergammaglobulinemic purpura of Waldestrom have been reported. There is a preponderance of females with this disorder, which is characterized by long-standing purpuric vasculitic lesions usually in the lower extremities, increased sedimentation rate, anemia, leukopenia, and hyperglobulinemia with a normal clotting process. Numerous associations have been shown between this and other systemic disorders such as sjogren's syndrome, systemic lupus erythematosus, a rheumatoid arthritis. A 40-year-old woman is described who had an 18-year history of recurrent purpura and increased IgG levels. skin biopsy showed acute vasculitis, and immunofluorescent study revealed fibrinogen precipitation and C3 deposition. Serologic evaluation demonstrated the presence of rheumatoid factor and antinuclear antibodies (1:80). Raji assay showed increased circulating immune complexes, and the T cell subsets were normal. The purpura was associated with symptoms and physical findings of sjogren's syndrome. On the basis of serologic and skin biopsy findings, an autoimmune origin of the disease is postulated.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/8. Approach to the patient with abnormal liver enzymes.Elevated serum enzyme values are often the earliest indicators of liver injury in asymptomatic patients. Abnormal AST and ALT point to a hepatocyte disorder; abnormal alkaline phosphatase suggests a biliary tract disorder. Selected case histories illustrate how the three enzyme levels, in conjunction with the history and physical examination, can guide the rest of the workup.- - - - - - - - - - ranking = 9.1698765755872keywords = physical examination, physical (Clic here for more details about this article) |
7/8. Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus.Autoimmune myelofibrosis is an uncommon disorder in which patients present with anemia and thrombocytopenia in conjunction with limited clinical manifestations of autoimmune disease or an exacerbation of previously established SLE. The presence of leukoerythroblastosis in a patient with SLE may suggest the presence of myelofibrosis. Conversely, the absence of splenomegaly in a patient with presumed idiopathic myelofibrosis may suggest an autoimmune etiology. patients with autoimmune myelofibrosis universally have a positive ANA test and frequently have either elevated anti-dna titers or a positive LE cell preparation. Because physical manifestations of autoimmune disease may not be evident at presentation, all patients found to have myelofibrosis should have an ANA test. Peripheral blood cytopenias in autoimmune myelofibrosis frequently respond to glucocorticoids but regression of bone marrow fibrosis may be incomplete. Hematologic response to treatment parallels that of the associated autoimmune disease.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/8. The pathophysiology of pure red cell aplasia: implications for therapy.To determine the utility of marrow culture in defining the natural history and therapeutic response of pure red cell aplasia we have studied 37 patients. patients were evaluated at the University of washington before specific therapies (n = 21) or at the time of treatment failure in = 16). Evaluation included a medical and drug exposure history, a physical examination, a chest x-ray or computed tomography to rule out thymoma, lymphocyte immunophenotype studies, anti-nuclear antibody and rheumatoid factor determinations, marrow cytogenetics, and marrow progenitor cell cultures. Retrospective Southern analyses to detect human parvovirus B19 was performed in the 27 patients for whom sera was stored. Clinical follow-up was obtained to document therapeutic responses. Normal burst forming unit-erythroid (BFU-E) growth (>30 bursts/10(5) marrow mononuclear cells [MMNC]) in culture proved an outstanding predictor of clinical response, as 27 of 29 individuals with normal frequencies of erythroid bursts in culture responded to immunomodulating therapies (sensitivity 96%, specificity 78%, predictive value 93%, P = .0001 with two-tailed chi square analysis). overall, 28 patients responded to either immunomodulating therapies or drug withdrawal. Twenty-four patients obtained a normal hematocrit (complete response [CR] and 4 additional patients became transfusion independent (partial response). Although responding patients often required several therapies, 20 of 24 (83%) patients who obtained a CR have sustained a normal hematocrit without maintenance therapy at the time of last follow-up (median 5 years). In contrast, of 8 patients with poor in vitro BFU-E growth (<6 bursts/10(5) MMNC), 7 failed to respond to any therapy and all died (median survival time 17 months). Our data suggest that in individuals, from whom BFU-E mature appropriately in culture, immunosuppressive drugs should be used sequentially until a CR is obtained and a durable remission is the expected outcome.- - - - - - - - - - ranking = 9.1698765755872keywords = physical examination, physical (Clic here for more details about this article) |