1/11. An intrasylvian "fibroma" in a child with cystic fibrosis: case report.OBJECTIVE AND IMPORTANCE: Intracranial fibrous tumors are uncommon during childhood. An unusual case of benign intrasylvian "fibroma" that has remained clinically and radiographically stable more than 3 years after a subtotal resection is described. CLINICAL PRESENTATION: A 9-year-old girl with cystic fibrosis presented with new-onset focal seizures referable to a large calcified left sylvian fissure mass. INTERVENTION: An open biopsy with subtotal resection of the lesion revealed a benign process characterized by exuberant fibrocollagenous tissue intermeshed with chronic inflammatory cells and foreign body giant cells, encompassing islands of gliotic brain tissue. Immunohistochemical analysis showed staining for epithelial membrane antigen and reticulin within some of the spindle cells, although the majority were nonreactive. The majority of tumor cells exhibited staining for laminin; CD34 staining was absent. Ultrastructural studies were also suggestive of a fibroblastic rather than a meningothelial origin of the lesion, with elongated cells separated by abundant extracellular collagen. Although dense adherence of the mass to the pial surface and the middle cerebral artery vessels precluded a complete resection, the patient remains seizure-free without anticonvulsant therapy more than 3 years postoperatively with no evidence of growth of the lesion. CONCLUSION: The lesion in this patient bears morphological similarity to a rare group of tumors referred to as "intracerebral fibromas," although a variety of other rare mesenchymal neoplasms were also considered within the differential diagnosis. However, the absence of any definite neoplastic features, the finding of chronic inflammatory changes, and the lack of growth of the residual tumor during an extended follow-up interval indicate that the mass may represent either an extremely indolent neoplasm or a nonneoplastic process. The differential diagnosis of intracranial fibrous tumors is contrasted with that of the reported case.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/11. Simultaneous liver and pancreas transplantation in patients with cystic fibrosis.BACKGROUND: Improved survival in patients with cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, cirrhosis and pancreatic insufficiency, including CF-related diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and mortality. Liver transplantation is the treatment of choice for cirrhosis in this setting, but the addition of an isolated simultaneous pancreas transplant in patients with CFRD has not been reported. methods: Two female patients with CF underwent simultaneous pancreas and liver transplantation. Both had pancreatic insufficiency, CFRD, cirrhosis, and preserved renal function. In each case, the liver and pancreas were procured from a single cadaveric donor. The liver transplant was performed first. A lower midline extension was added for improved exposure of the iliac vessels. The donor pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. immunosuppression included rabbit anti-thymocyte globulin, tacrolimus, mycophenolate mofetil, and early steroid withdrawal. RESULTS: Both patients recovered well with normal liver function, resolution of portal hypertension, and normal blood glucoses independent of insulin. As a result of the enteric exocrine drainage of the pancreas, they are now independent of supplemental pancreatic enzymes. CONCLUSIONS: Simultaneous liver and pancreas transplantation in CF patients provides the advantages of normalization of glucose and improved nutrition for patients requiring liver transplantation and should be considered in CF patients with CFRD who require liver transplants.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/11. Pulmonary cystic disorder related to light chain deposition disease.light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic kappa light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/11. A pathologic study of allergic bronchopulmonary aspergillosis.A lung biopsy specimen was obtained from a 10-year-old boy with cystic fibrosis and allergic bronchopulmonary aspergillosis. light microscopy revealed a marked inflammatory process that was largely bronchocentric. Infiltrating cells included lymphocytes, plasma cells, monocytes, and numerous eosinophils. elastin layers were intact in blood vessels and markedly disrupted in bronchioles. By immunofluorescent, major basic protein was demonstrated in eosinophils, was freely deposited outside of eosinophils, especially in the interlobular septum, and was taken up by macrophages. A number of lymphocytes stained positively for IgE. Through an immunoperoxidase stain, septate hyphae of aspergillus were clearly observed in the lung parenchyma. A significant increase in interleukin-2 positive-staining T cells was observed with an approximate 2:1 ratio of helper to suppressor cells. The use of newer immunohistologic techniques has enabled us to gain additional insights into the pathogenesis of allergic bronchopulmonary aspergillosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/11. Longterm histopathologic follow-up of bronchial arteries after therapeutic embolization with polyvinyl alcohol (Ivalon) in patients with cystic fibrosis.We used light microscopy to examine, at autopsy, bronchial arteries in three patients with cystic fibrosis who died, respectively, 10, 16, and 28 months after bronchial artery embolization with barium sulfate-impregnated polyvinyl alcohol (PVA) to control hemoptysis. PVA was not identified beyond the midsegmental bronchus in any patient. Persistent focal fibrovascular occlusion was noted in two patients, and recanalized and/or partially obstructed vessels were associated with PVA in all. The histologic reaction to PVA included fibrosis, mild chronic inflammation, localized foreign body reaction, and, in two patients, focal calcification of PVA spicules. Within the inflammatory milieu were numerous macrophages containing BaSO4. Extensive vascular mural destruction and fibrosis associated with PVA were also observed. Both PVA and BaSO4 were also frequently present in the perivascular connective tissue. These findings indicate that, although longterm occlusion persists after therapeutic arterial embolization with PVA, focal recanalization also occurs. The extent of vascular mural injury following PVA embolization in humans has been previously underestimated by animal experiments. Finally, perivascular deposition of PVA represents a common reaction to diverse foreign body emboli in both systemic and pulmonary arteries.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/11. Recurrent cutaneous vasculitis in cystic fibrosis.We treated a patient with cystic fibrosis who experienced recurrent episodes of palpable purpura and arthralgias associated with exacerbations of her pulmonary disease. skin biopsy demonstrated the classic findings of leukocytoclastic vasculitis, and C3 deposits were detected in the dermal blood vessels. Chronic bacterial infection and antibiotics are possible sources of antigen involved in immune complex formation in patients with chronic lung diseases.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/11. vocal cord paralysis associated with cystic fibrosis.The course of the left recurrent laryngeal nerve through the chest brings it in close proximity with the heart and great cardiac vessels. Diseases of the heart and the great vessels are known to cause vocal cord paralysis, probably by mechanical injury to the recurrent laryngeal nerve. pulmonary artery hypertension and dilation occur in up to 80% of patients with cystic fibrosis. We report a case of a 23-year-old woman with cystic fibrosis and left vocal cord paralysis. We believe that sudden pulmonary artery expansion produced recurrent laryngeal nerve injury and vocal cord paralysis. This is only the second association of unilateral vocal cord paralysis and cystic fibrosis in the medical literature. The pathophysiology of the cardiovocal syndrome is discussed.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
8/11. Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis.Cardiomyopathy in cystic fibrosis (CF) is an unusual heart disease, mainly characterized by a multifocal fibrosis of the left ventricle. The disorder chiefly occurs in the age group of 1-2 years and leads to fatal cardiac failure. The causal pathogenesis of the disease has not been discovered up to now. In two cases of CF-associated cardiomyopathy we found an oedema (mainly lymphoedema) of the myo- and epicardium and a lymph stasis in lymph vessels and lymph nodes of the heart. Based on a comparative study using animal models we speculate that a) CF may be complicated by a disorder of cardiac lymph circulation, and b) chronic cardiac lymphoedema of the heart in CF can cause focal myocardial damage with fibrosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/11. A pathological study following bronchial artery embolization for haemoptysis in cystic fibrosis.A detailed post-mortem study is reported on an adult with cystic fibrosis who died in respiratory failure three weeks after selective bronchial artery embolization with gelatin sponge to treat severe haemoptysis. Bronchial arteriography during the procedure showed complete occlusion of the vessel supplying the righ upper lobe and following this the bleeding stopped. However, post-mortem angiography of the same vessel demonstrated disappearance of much of the injected material with contrast bypassing the remaining gelatin to fill the peripheral bronchial arterial bed. This report therefore adds pathological evidence to previous clinical reports which have suggested that this procedure may not produce permanent occlusion of the embolised bronchial artery.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
10/11. Transverse myelitis: a reversible complication of bronchial artery embolisation in cystic fibrosis.The case history is presented of a young woman with cystic fibrosis and life threatening haemoptysis. angiography revealed enlarged bronchial vessels, one of which supplied the contralateral lung. Transverse myelitis developed following bronchial artery embolisation but recovery was rapid and nearly complete. Haemoptysis did not recur during four years of follow up.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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