1/10. Acquired factor viii autoantibody: four cases demonstrating the heterogenous nature of this condition and problems involved in diagnosis and treatment.The development of an autoantibody to human factor viii is rare and presents many problems for diagnosis and treatment. We have seen several cases at our institution recently with widely heterogenous clinical and laboratory presentations. A wide range of treatment modalities were used in these cases with no gold standard of treatment or widely accepted guidelines existing. This has prompted us to examine all cases of this condition presenting at Fremantle Hospital over the last decade. We describe four cases which demonstrate the heterogeneity of this condition and its treatment and review the recent literature on the subject.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/10. Case of multiple myeloma mimicking an infectious disease with fever, intrahepatic cholestasis, renal failure, and pulmonary insufficiency.We describe a case of multiple myeloma (MM) presenting with high fever, inflammatory chemistry abnormalities, simultaneous acute renal failure, cholestatic hepatitis, and acute lung failure. The extremely aggressive course and pulmonary involvement in the form of pulmonary alveolar proteinosis (PAP) are discussed, stressing the unusual nature of the findings and the variable picture of MM.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
3/10. Postinfectious encephalomyelitis with localized basal ganglia involvement.The diagnosis of postinfectious encephalomyelitis with symmetric lesions in the basal ganglia was confirmed by magnetic resonance imaging in 2 patients. A 7-year-old patient experienced severe dystonia and hyperreflexia; magnetic resonance imaging demonstrated bilateral lesions in the putamina and basis pontes. The other patient, a 2-year-old female, manifested hypotonia, facial grimacing, and athetosis. Symmetric lesions in the globus pallidus and substantia nigra were demonstrated by imaging studies. The nature and monophasic course of illness in these 2 patients, as well as the symmetric involvement of specific regions of the basal ganglia, may result from an immune-mediated postinfectious demyelinating process.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
4/10. Postinfectious glomerulonephritis. Subtypes, clinico-pathological correlations, and follow-up studies.APGN (WHO: diffuse endocapillary proliferative glomerulonephritis) has long been considered one of the best described kidney afflictions, clinically characterized by the sudden development of a nephritic syndrome after a latency period following a nasopharyngeal infection or pyoderma. Proliferation of mesangial and endothelial cells in the endocapillary space, aggregation of polymorphonuclear granulocytes in the capillary lumina, and deposition of predominantly subepithelial immune complexes on the glomerular basement membrane (so-called "humps") are to the present day considered characteristic of renal morphology. However, the nature of the antigen (or antigens) as well as the determining mechanisms in the pathogenesis of APGN still are unclear. Considerable disagreement also exists regarding the prognosis. An analysis of APGN is once again presented to elucidate whether the morphological picture of APGN is really as uniform as has been generally assumed. A large number of kidney biopsies was examined and subjected to the triad of light microscopy (LM), immunofluorescence microscopy (IFM) and electron microscopy (EM). The findings, which were recorded at an early stage of APGN in all cases (i.e. during the first 9 weeks), were related to clinical data, age (childhood or adulthood), and etiology (e.g. streptococci, staphylococci). In addition, clinical and morphological follow-up over a period of up to 10 years in those cases, which had been carefully documented in the early stages, afforded an insight into the dynamics and the prognosis of APGN. light microscopy of APGN showed a certain spectrum of variation even during the rather limited period of 9 weeks, due to the varying number of granulocytes and a varying degree of cell proliferation, as we could show semiquantitatively. With the triad of methods, especially by IFM and EM, three separate morphological patterns were distinguishable: the starry sky pattern, the garland pattern and the mesangial pattern. Based on clinico-pathological correlations, these patterns were shown to permit the nosological subdivision of APGN. The following features merit special emphasis: The starry sky pattern occurred most often during the first few weeks, the mesangial pattern increased in frequency after the 3rd week, and the garland pattern could occur at any time. In the starry sky and garland patterns immunoglobulins (mainly IgG) generally appeared in combination with C 3. The mesangial pattern was characterized by C 3 appearing alone. These three immunohistological patterns, which also showed transitional and combined forms, had certain characteristic features by electron microscopy.(ABSTRACT TRUNCATED AT 400 WORDS)- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
5/10. Proliferation of T gamma cells with killer-cell activity in two patients with neutropenia and recurrent infections.Two patients with severe granulocytopenia and recurrent infections of the skin and oropharynx had excess T lymphocytes with receptors for the Fc portion of IgG (T gamma cells) in blood and bone marrow. The abnormal T gamma cells killed antibody-sensitized target cells in vitro (killer-cell activity) but did not suppress immunoglobulin production by B lymphocytes (suppressor-cell activity). T gamma lymphocytes from normal persons showed both killer-cell activity and suppressor-cell activity. In the serum of one patient, granulocyte antibodies, possibly of an autoimmune nature, were detected. The clinical picture in conjunction with the hematologic and immunologic findings characterized the disease of both patients as a distinct entity among the chronic lymphoproliferative diseases of T-cell origin.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
6/10. The white blood cell scan in orthopedics.A new nuclear scanning technique was found more specific for bone, joint, and soft tissue infections than any previously described scanning technique. The leukocyte scan, whereby a patient's own cells are labeled with a radioactive tagging agent (111In oxine), can distinguish an active infectious process from other pain-inducing conditions. Ninety-seven 111In labeled autologous leukocyte scans were performed in 88 patients. The findings in 17 of 40 patients scanned for possible acute osteomyelitis, six of nine for suspected septic arthritis, and six for possible soft tissue infections, were positive. Subsequent clinical courses verified the infectious nature of these processes in all patients. patients who had chronic osteomyelitis (14), bony metastases (four patients), heterotopic ossification (three), and degenerative arthritis (two) demonstrated negative findings. Of the seven patients scanned for acute long-bone fractures, one demonstrated positive findings. Nine scans demonstrated positive findings without determined causes. The leukocyte scan is a useful addition to the diagnostic tools of the orthopedic surgeon.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
7/10. Myelodysplastic syndrome and thrombocytosis: a random association?We describe a case characterized by the onset of bone marrow hypoplasia. After treatment with steroid and anabolic compounds, it evolved into a myelodysplastic syndrome (MDS) as demonstrated by morphological and karyotypic analysis. Despite the dysplastic nature of the disorder, a unique feature was its association with a high platelet count. The pathogenesis of the thrombocytosis could not be clearly identified. In fact, the course of the disease was complicated by severe infections that, together with therapy, could have played some role in stimulating thrombopoiesis. However, since MDS can precede or follow a chronic myeloproliferative disease, it is also possible that the platelet elevation in our patient could have been sustained by a primitive thrombocyte disorder.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
8/10. The management of rheumatoid nodules.Rheumatoid nodules are present in approximately 25% of patients with seropositive rheumatoid arthritis (RA) and are the most common extra-articular lesions of this disease. Current methods of treatment include a variety of antirheumatic agents, surgical excision, or observation. Treatment options for the rheumatoid nodule are limited, due in part to the highly variable nature of the nodule. This series describes 4 seropositive RA patients, all with extensive rheumatoid nodules. Three were successfully treated by surgical excision, and 1 was successfully treated with conservative management. Surgical treatment is effective. Indications include local pain, nerve compression, limited range of motion, erosion, and infection.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
9/10. Case study: an infection-triggered, autoimmune subtype of anorexia nervosa.OBJECTIVE: Certain cases of anorexia nervosa (AN) may be similar to the recently described subtype of childhood-onset obsessive-compulsive disorder hypothesized to be one of the pediatric infection-triggered autoimmune neuropsychiatric disorders (PITANDs). METHOD: Three clinical cases are reported. The first patient is a 12-year-old boy whose AN worsened acutely after a group A beta-hemolytic streptococcal (GABHS) infection. His symptoms were alleviated after antibiotic treatment. Two other patients with possible PITANDs-related AN are described. RESULTS: An infection-triggered process may contribute to the pathogenesis of a subtype of AN. CONCLUSIONS: Future research is needed to explore the nature of PITANDs and their relationship with AN.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
10/10. The infectious complications of sarcoidosis: a current perspective.The incidence of indections requiring hospitalization was determined in 122 patients with sarcoidosis. The group was remarkably free of infection except for three patients with aspergillus mycetoma occurring in areas of long-standing parenchymal involvement with cystic degeneration. There was a single instance of complicating pulmonary tuberculosis, and the only extrathoracic infection was a single instance of disseminated herpes zoster. This study confirms that aspergillosis, not tuberculosis, is currently the most common infectious complication of sarcoidosis. Although previous case reports have suggested an increased incidence of invasive fungal infection in patients with sarcoidosis, there is little to support this concept. None of the patients in the present study group developed these fungal infections during a mean 7.2-year follow-up. The clinical presentation of many of the previously reported cases suggests that the entire course of the granulomatous illness was infectious in nature rather than sarcoidosis with complicating infection.- - - - - - - - - - ranking = 0.25keywords = nature (Clic here for more details about this article) |
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