1/60. Soleus rupture: a differential diagnosis of calf thrombosis.Soleus rupture may present with clinical features similar to those of calf thrombosis. It is postulated that the signs are the result of compression of the posterior tibial vein as it passes through the narrow space adjacent to the fibrous origin of soleus. A distinctive venographic sign of compression of the posterior tibial vein by a soft tissue mass at the level of the origin of the soleus is described.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
2/60. Arterial tortuosity syndrome.We describe a patient with arterial tortuosity syndrome (ATS), a rare disorder comprising generalized tortuosity and elongation of all major arteries, soft skin, joint laxity, severe keratoconus, and diffuse tortuosity of the carotids and of intracranial arteries. The patient's probably affected brother and sister died at an early age. Cytochemical studies excluded Ehlers-Danlos type IV and type VII syndromes. We review 11 previously described patients.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
3/60. Spontaneous cerebral haemorrhage without hypertension in non-mosaic 45X Turner's syndrome.Cerebral haemorrhage without hypertension, arteriosclerosis or clotting defect has not been reported in patients with Turner's syndrome before. In a 51 year old female patient with non-mosaic Turner's syndrome, acute aphasia and right-sided hemiplegia occurred, due to left-sided basal ganglia haemorrhage. The history for hypertension was negative, blood pressure was normal throughout hospitalisation as well as during 24 h monitoring, and all tests for secondary hypertension were negative. There was no indication of arteriosclerosis or a clotting defect. Since there were hypermobile joints, hyperextensible skin and ectatic ascending aorta and brachiocephalic trunk on angiography, a general connective tissue defect was assumed, making arteries more vulnerable to physiologically increased blood pressure and rupture of intracerebral arteries with consecutive bleeding.- - - - - - - - - - ranking = 21.569724671357keywords = connective (Clic here for more details about this article) |
4/60. Polyneuropathy and anasarca: evidence for a new connective-tissue syndrome and vasculopathic contribution.A 41-year-old woman manifested a polyneuropathy, anasarca, pseudotumor cerebri, hyperhidrosis and hyperpigmentation of the skin, generalized lymphadenopathy, distal esophageal dysphagia, pleuritis, platelike pulmonary atelectasis, fluctuating renal insufficiency, hepatosplenomegaly, amenorrhea, and slight fever suggesting a connective-tissue disorder. Extensive clinical and laboratory evaluation did not support the initial impression of progressive systemic sclerosis or systemic lupus erythematosus but did show nonnecrotizing vascular changes, mild polyclonal gammopathy, and low thyroxin levels similar to the syndrome of polyneuropathy and endocrine disturbances recently reported from japan. The impressive response to moderate-dose corticosteroids and exacerbation on withdrawal require diagnostic awareness of this insidiously progressive multisystem disorder. A bland vasculopathic process resulting from metabolic or immunologic disturbances appears to be the best explanation for this new syndrome, which has previously been recognized only in japan.- - - - - - - - - - ranking = 107.84862335679keywords = connective (Clic here for more details about this article) |
5/60. A case of primary sarcoma of the pulmonary artery.A 39-year-old male was admitted complaining of nonproductive cough and dyspnea on exertion. death occurred eight months after onset of the symptoms. autopsy examination showed that the pulmonary trunk and left main pulmonary artery were markedly dilated and completely occluded by a tumor. The tumor had infiltrated into the left upper lobe and mediastinal lymph nodes, and metastatic nodules were found in both lungs and in the left adrenal gland. Small foci of infarction were noted in the lower lobes of both lungs. The tumor cells were of two types; pleomorphic spindle cells and bizarre multinucleated giant cells. Immunohistochemically, they were positive for vimentin, myosin, and lysozyme, but negative for desmin and muscle-specific actin. The cytoplasm of the tumor cells was showed by electron microscopy to contain microfilaments, dense bodies, and pinocytotic vesicles. We diagnosed this case as undifferentiated sarcoma of the pulmonary artery. Approximately 100 cases of pulmonary artery sarcoma have been reported. Histopathologically, almost all of the reported cases showed both spindle cells and pleomorphic giant cells, indicating a biologically anaplastic neoplasm.- - - - - - - - - - ranking = 3.4464692652927keywords = neoplasm (Clic here for more details about this article) |
6/60. Exclusion of candidate genes in a family with arterial tortuosity syndrome.Arterial tortuosity syndrome (ATS) is a rare hereditary disorder with variable clinical presentation including tortuosity and elongation of the major arteries, often associated with pulmonary artery stenosis, pulmonary hypertension, and skin and joint laxity, suggestive of a connective tissue disorder. ATS is transmitted in an autosomal recessive mode, but the causal gene is unknown. We report an Italian pedigree with three inbred families in which five patients show signs of ATS. In particular, four adult patients present arterial tortuosity and elongation of the main arteries. Two of these patients, with the most severe degree of arterial tortuosity, also show severe peripheral stenosis of the main pulmonary artery. The fifth young patient shows a severe pulmonary valve stenosis in the absence of arterial tortuosity. All patients show signs of ehlers-danlos syndrome (EDS): soft skin with abundant subcutaneous tissue and joint laxity, hernias, and disorganization of the extracellular matrix (ECM) of fibronectin (FN) and of actin microfilaments in cultured skin fibroblasts. Linkage analysis of the genes involved in EDS and other connective tissue disorders, excluded COL1A1, COL1A2, COL2A1, COL3A1, COL5A1, COL5A2, COL5A3, COL6A1, COL6A2, ADAMTS2, ELN, FN1, TNXA, and TNXB as candidate genes in the family under study, thus indicating that ATS is a distinct clinical and molecular entity.- - - - - - - - - - ranking = 44.139449342714keywords = connective, soft (Clic here for more details about this article) |
7/60. A new syndrome of long-term idiopathic, severe CD4 lymphocytopenia: isolated paraparesis and conjunctival ischemic microangiopathy.An extraordinary case report of an adult patient followed-up for a decade with an extremely severe idiopathic CD4 T-lymphocytopenia (as expressed by an absolute CD4 count of 8-25 cells/microL), associated with an isolated paraparesis and a conjunctival ischemic microangiopathy is described, and discussed on the grounds of the available literature. Despite such a severe and prolonged immunodeficiency, no opportunistic disease occurred, in a observation period longer than ever reported to date. The neurological disorder was diagnosed concurrently with idiopathic CD4 lymphocyte depletion, while the ocular complication occurred two years later, but remained stable thereafter. Both disorders remained stable during the subsequent eight years. Despite extensive and repeated instrumental and laboratory workout, only very limited immunological abnormalities were detected (besides the extremely low CD4 lymphocyte count), and no apparent explaination was found for the disabling paraparesis syndrome. Idiopathic CD4 lymphocytopenia, whose pathogenesis deserves careful investigation, has been associated with a very broad spectrum of signs and symptoms, ranging from negligible or no disturbances, to severe lymphoproliferative disorders, different opportunistic infections, and other focal diseases, including neurological pathologies. However, the association of a long-lasting profound peripheral CD4 lymphocyte depletion in absence of any opportunistic infection or neoplasm, and isolated paraparesis and conjunctival microangiopathy, represents an absolutely unique finding, especially due to the apparently stable course of the above-mentioned syndrome.- - - - - - - - - - ranking = 3.4464692652927keywords = neoplasm (Clic here for more details about this article) |
8/60. growth of a leiomyosarcoma of the inferior vena cava.Until recently, leiomyosarcoma of the inferior vena cava was rarely diagnosed preoperatively. The authors describe a patient who was treated for 12 months on the basis of a presumptive diagnosis of thrombosis of the inferior vena cava. Abdominal ultrasonography and computed tomography demonstrated interval growth, and venous biopsy revealed a malignant spindle-cell neoplasm. Surgical excision confirmed the presence of a leiomyosarcoma, which had grown slowly for at least several months before entering a phase of accelerated growth.- - - - - - - - - - ranking = 3.4464692652927keywords = neoplasm (Clic here for more details about this article) |
9/60. femoral vein occlusion and spontaneous collateral ectasia presenting as recurrent hernia of the groin: a case report.femoral vein occlusion is not a common complication even after repeated hernia repair. We describe a case of a 14-year-old boy with a visible and soft, yet irreducible, mass below the inguinal ligament after 3 previous inguinal hernia repairs and heart catheterization in infancy. Further examination showed dilated venous collaterals, bypassing an occluded common femoral vein via the testicular sheaths and across the pelvic floor. We discuss etiology, diagnostic pitfalls, therapeutic options, and possible future complications, with a literature review.- - - - - - - - - - ranking = 1keywords = soft (Clic here for more details about this article) |
10/60. Sarcoma of the pulmonary artery: report of two cases and a review of the literature.Primary tumours of the pulmonary arteries are rare neoplasms seldom diagnosed during the patient's life time. We report on two cases of pulmonary artery sarcomas diagnosed during life time of the respective patients in intra-operative frozen sections by histopathological examination. Case 1 was of a 55-year-old man with a fibrosarcoma originating from the main pulmonary trunk. Case 2 was of a 43-year-old woman with a malignant fibrous histiocytoma originating from the right pulmonary artery. In both patients a radical tumour resection under cardiopulmonary bypass was attempted. Both patients, however, had a local tumour recurrence and died 18 months (patient 1) and 6 months (patient 2) after surgery. A review of pulmonary artery sarcomas is given.- - - - - - - - - - ranking = 3.4464692652927keywords = neoplasm (Clic here for more details about this article) |
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