1/15. Isolated abdominal vasculitis as an atypical presentation of Wegener's granulomatosis.Although current classifications characterize vasculitic syndromes based upon the size of the vessels involved, the histopathology, and the presence or absence of antineutrophil cytoplasmatic antibodies ANCA (1-3), those occasional patients with vasculitis whose features are not typical may evade diagnosis and effective treatment. We report one such patient who presented with bilateral refractory uveitis and abdominal angina who had a positive C-ANCA. Because of his atypical presentation, this patient's disease progressed over 8 yr despite an extensive gastrointestinal evaluation, before a diagnosis of vasculitis was established angiographically, and immunosuppressive therapy was begun.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/15. Relapsing ischemic encephaloenteropathy and cryoglobulinemia.cryoglobulinemia is a rare cause of encephalopathy. The authors report three patients with strikingly similar clinical features of recurrent encephalopathy accompanied by symptoms of gastrointestinal ischemia. In only one patient was cryoglobulinemia ascertained in life during the final illness. The autopsy examinations all showed diffuse cerebral, enteral, and systemic small vessel lesions immunoreactive for immunoglobulins and typical of mixed essential cryoglobulinemia. This unusual relapsing clinical syndrome is readily misinterpreted as of nonorganic origin despite its potentially fatal prognosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/15. Necrotizing angiitis of the small intestine related to AA-amyloidosis: a novel association.A 71-year-old man with intestinal pseudo-obstruction was found to have a diffusely thickened adynamic small bowel with AA-amyloid in submucosal vessels and muscularis propria, foreign body giant cell reaction to amyloid, and necrotizing angiitis. The mucosa was unremarkable. Immunostains demonstrated numerous CD68 monocyte/macrophages and CD8 T cells associated with the amyloid deposits. The patient had no evidence of systemic vasculitis and no underlying cause for AA-amyloidosis was identified. Necrotizing angiitis coexistent with amyloid angiopathy has been reported in brain and temporal arteries, but not in the gastrointestinal tract and not with AA-amyloid. The inflammatory cell infiltrates in this case are consistent with a foreign-body and/or cell-mediated immunologic reaction to AA-amyloid, although a role for these cells in amyloid formation cannot be excluded.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/15. A case of fistula of the right common iliac aneurysm to the appendix.We report a very rare case of spontaneous ilioappendicial fistula with right common iliac aneurysm. After the aneurysm was opened, afferent and efferent vessels were closed following extraanatomical femorofemoral bypass, and the appendectomy was performed. The wall of the aneurysm showed the atherosclerotic change and histologic study of the appendix confirmed the diagnosis of acute appendicitis. Enhanced computed tomography was useful for the diagnosis and the extraanatomical bypass was deemed the most effective operative strategy. The pathogenesis of the fistula was surmised to be related to the appendicitis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/15. Reactive angioendotheliomatosis of the intestine.We present a case of reactive angioendotheliomatosis (RAE) of the colon, featuring intravascular proliferation of endothelial cells with histologic resemblance to glomeruloid hemangioma. A 19-year-old Japanese male with an anal fistula was diagnosed endoscopically with Crohn's disease. Six months later, he was hospitalized for fever and abdominal pain. Emergency resection of ileocecum and splenic flexure of the colon was undertaken to control massive intestinal hemorrhage, and in all parts of the resected colon, foci of many small vessels with intravascular proliferation of endothelial cells were noted throughout the layers. Moreover, solid proliferation of endothelial cells was seen in the submucosa at the base of open ulcers. Two small granulomas, compatible with Crohn's disease, were also evident in the muscle layer of the terminal ileum. No other hemangiomas or hemangioma-like structures were observed with CT scans, and the vascular lesions were histologically diagnosed as RAE. The pathogenesis of this disorder is unknown, and most cases occur in skin with systemic disease. The present case might thus be a first case of RAE of the intestine without cutaneous involvement. Whether there is a relation with coexistent enteritis suggestive of Crohn's disease needs to be clarified.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/15. Inhaled nitric oxide improves pulmonary functions following massive pulmonary embolism: a report of four patients and review of the literature.Acute pulmonary embolism increases pulmonary vascular resistance and may lead to acute right ventricular failure and cardiocirculatory collapse and respiratory failure, possibly resulting in substantial morbidity and mortality. Inhaled nitric oxide (NO) dilates pulmonary blood vessels and has been used to reduce pulmonary vascular resistance in patients with chronic thromboembolic pulmonary hypertension and acute respiratory distress syndrome. This case series describes our experience with inhaled NO administered to four patients suffering from acute massive pulmonary embolism following abdominal surgery. The four described patients recovering from small bowel resection, pancreatoduodenectomy, hemipelvectomy, or recent gastrointestinal bleeding had severe respiratory and hemodynamic deterioration due to pulmonary embolism. Each received inhaled NO (20-25 ppm) via the inspiratory side of the breathing circuit of the ventilator. Pulmonary and systemic blood pressures, heart rate, and lung gas exchange improved in all the patients within minutes after the initiation of NO administration. Inhaled NO may be useful in treating acute massive pulmonary embolism. This potential application warrants further investigation.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/15. Myo-, neuro-, gastrointestinal encephalopathy (MNGIE syndrome) due to partial deficiency of cytochrome-c-oxidase. A new mitochondrial multisystem disorder.A 42-year-old woman had a 10-year history of external ophthalmoplegia, malabsorption resulting in chronic malnutrition, muscle atrophy and polyneuropathy. Computer tomography revealed hypodensity of her cerebral white matter. A metabolic disturbance consisted of lactic acidosis after moderate glucose loads with increased excretion of hydroxybutyric and fumaric acids. Post-mortem studies revealed gastrointestinal scleroderma as the morphological manifestation of her malabsorption syndrome, ocular and skeletal myopathy with ragged red fibers, peripheral neuropathy, vascular abnormalities of meningeal and peripheral nerve vessels. Biochemical examination of the liver and muscle tissues revealed a partial defect of cytochrome-c-oxidase (complex IV of the respiratory chain). This mitochondrial multisystem disorder may represent a separate entity to be classified between the spectrum of myoencephalopathies and oculo-gastrointestinal muscular dystrophy.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/15. Reversible cerebral segmental vasoconstriction.vasoconstriction is not recognized as a cause of cerebrovascular disease except in the vasospasm seen following subarachnoid hemorrhage and possibly in migraine. However, we found four patients to have transient, fully reversible vasoconstriction and dilatation prominently involving arteries around the circle of Willis. All four patients were evaluated for severe headaches and fluctuating or recurring motor or sensory deficits. No cause for the clinical syndromes and angiographic abnormalities was found. Similar patients are reported in the literature under various nosologies. This newly recognized clinical-angiographic syndrome should be differentiated from other known causes of vessel constriction and dilatation; the precipitants of reversible vasoconstriction may then be better defined.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/15. Brown bowel syndrome with manifestation in the gastrointestinal tract and thyroid gland.Brown bowel syndrome (BBS) is a rare disease accompanied by deposits of lipofuscin predominantly in smooth muscle cells of the gastrointestinal tract. To determine whether cells other than smooth muscle cells show pigment deposition we studied biopsies of the stomach, small and large intestine, and thyroid gland of a 52-year-old male with malabsorption syndrome. light and electron microscopy found lipofuscin pigment in vascular endothelial cells and in smooth muscle cells of the muscularis mucosae, arterioles and venules of the gastrointestinal tract and thyroid gland. All other cells of bowel and thyroid gland, such as epithelial cells, schwann cells, nerve fibers, fibroblasts, macrophages and leukocytes, showed no such pigment inclusions. Intracellular lipofuscin deposition is thought to be caused by a deficiency of vitamin e. Such a deficiency existed in our patient and was attributed to a six year history of malabsorption syndrome with complete atrophy of the villi of the intestinal mucosa. lipofuscin pigmentation has been reported in numerous organs of patients with BBS but not, until now, in the thyroid gland or in endothelial cells of lymph vessels. The origin of lipofuscin pigment is not known exactly. The prevailing opinion is that it derives from degenerating mitochondria. Our findings, however, suggest that autophagocytotic processes may also play a role in the formation of lipofuscin.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/15. Digestive tract and renal small vessel hyalinosis, idiopathic nonarteriosclerotic intracerebral calcifications, retinal ischemic syndrome, and phenotypic abnormalities. A new familial syndrome.A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain. Using electron microscopy, we found that the hyalin substance in the intestinal capillaries consisted of several concentric layers of basal membrane-like deposits within a finely granular fluffy material. Huge deposits of this material were present in the subepithelial and mesangial spaces of the kidneys. endothelial cells and, in the kidneys, mesangial cells were markedly abnormal, and a true mesangiolysis pattern was present in 2 patients. The clinical and biologic expression of these vascular changes was variable. diarrhea, rectal bleeding, malabsorption, and protein-losing enteropathy were the main and lethal clinical problems in the proband. hypertension appeared in the early stage of a second pregnancy in 1 sister, and mild proteinuria was found in all 3 affected patients. Peripheral retinal ischemic syndrome and chorioretinal scars were found in the ocular fundi of both affected sisters of the proband. A subarachnoid hemorrhage, due to a right sylvian aneurism, also occurred in both sisters and was lethal in 1 sister. None of the known causes of distal vessel hyalinosis could be ascertained.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
| | Next -> |