1/9. Familial amyloidotic polyneuropathy (ATTR Val30Met) with widespread cerebral amyloid angiopathy and lethal cerebral hemorrhage.We report an autopsy case of familial amyloidotic polyneuropathy (FAP) with cerebral hemorrhage. A 38-year-old woman with a typical FAP pedigree started developing severe diarrhea and sensori-motor polyneuropathy at the age of 28 years; autonomic nervous system, heart and renal dysfunction manifested themselves in the following years. Genetic analysis revealed a single amino acid substitution at codon 30 of transthyretin (ATTR Val30Met). Ten years after her initial symptoms, the patient died of a sudden convulsive attack and respiratory failure. autopsy revealed lethal cerebral hemorrhages and uremic lungs. Histochemical and immunohistochemical analyses revealed TTR-derived amyloid protein in every tissue examined, particularly in glomeruli and peripheral vessels. Severe meningo-cerebrovascular amyloidosis was also detected. Because uremia causes oxidative damage to the vascular system and amyloid formation is closely associated with oxidative stress, it is possible that uremic endothelial damage facilitated an unusual cerebral amyloid deposition. In typical FAP (ATTR Val30Met), cerebral amyloid angiopathy does not usually have clinical manifestations. However, cerebral amyloid angiopathy should be considered to explain FAP symptoms when some risk factors such as uremic vascular damage are accompanying features.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/9. Uraemic gangrene syndrome: is parathyroidectomy always necessary?This report presents a case of uraemic gangrene syndrome with multiple ulcerations and calcium (Ca) deposits in the vessel walls. High-dosage oral calcium carbonate (CaCO3) treatment alone resulted in rapid wound healing with decreased serum phosphorus (P), Ca x P product and parathormone and only slightly increased Ca levels. We suggest that this should be the first choice of treatment in such cases because positive clinical response can achieve symptom-free status for the patient until kidney transplantation, thus making parathyroidectomy unnecessary.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/9. Mycotic aneurysms and death in a hemodialysis patient.A patient with newly diagnosed end-stage renal disease (ESRD) received a femoral catheter for hemodialysis (HD). Shortly thereafter he developed fever, and blood cultures grew methicillin-resistant staphylococcus aureus. The catheter was removed and the patient was treated with both vancomycin and rifampin; however, blood culture positivity persisted. The cerebrospinal fluid showed sterile meningitis. Subsequent imaging studies demonstrated aortic valve endocarditis and multiple mycotic aneurysms that appeared to include the intra- and extracranial vessels. The patient eventually died from sepsis. This case illustrates the aggressive and invasive nature of systemic infection with S. aureus and underscores the high morbidity and mortality associated with infections related to HD catheters.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/9. Fulminant pulmonary calciphylaxis and metastatic calcification causing acute respiratory failure in a uremic patient.calciphylaxis is a rare and life-threatening disorder characterized by small-vessel mural calcification with intimal proliferation, fibrosis, and thrombosis, resulting in tissue ischemic necrosis. Although it has been viewed as a systemic disease involving mainly the dermis, subcutaneous fat, or muscle, calciphylaxis of other organs rarely is reported. We describe the case of a 25-year-old uremic woman who rapidly developed massive pulmonary calcification that led to acute respiratory failure after the initiation of hemodialysis therapy. Chest radiography and high-resolution computed tomography showed typical pulmonary calcification. Pulmonary calciphylaxis and metastatic calcification were confirmed further by lung tissue biopsy. No skin or muscle calciphylaxis was discovered. Despite multiple factors precipitating pulmonary calciphylaxis in this patient, we speculate that hemodialysis was the main culprit in accelerating the development of fulminant pulmonary calciphylaxis and metastatic calcification. Alteration in the local environment from an acid to an alkaline condition and a relatively high dialysate calcium level in the presence of systemic hyperphosphatemia are believed to have facilitated the deposition of calcification. This case highlights the importance of "visceral calciphylaxis" and early identification of its causes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/9. thrombosis following desmopressin for uremic bleeding.An elderly patient with evidence of atherosclerosis and uremic bleeding diathesis developed two foci of cerebral thrombosis immediately after an infusion of desmopressin (DDAVP). Because large molecular weight multimers of von Willebrand factor (vWF) have been demonstrated to cause platelet aggregation under conditions of elevated fluid shear stress as occurs in atherosclerotic vessels, we investigated his plasma vWF at the time of the event and compared it to baseline values obtained 2 weeks later. Unusually large vWF multimers induced by the DDAVP infusion were present and likely contributed to the thrombotic process. Consequently, we believe DDAVP should be given with greater caution to patients with atherosclerosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
6/9. Amyloid vascular disease and contracted kidneys--report of a case with review of literature.A case of systemic amyloidosis associated with bronchiectasis is presented. At necropsy, contracted kidneys and centrilobular necrosis of the liver were observed. Systemic blood vessels had heavy deposition of amyloid, and the possibility of visceral ischemia and the development of contracted kidneys due to amyloid vascular disease as a pathogenetic relationship, was discussed against the background of a review of the literature.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/9. Tumour-like growth of parathyroid autografts in uraemic patients.Total parathyroidectomy with autotransplantation of parathyroid tissue into the forearm musculature has been recommended as surgical treatment for renal hyperparathyroidism. Five of 41 patients, in whom this procedure was performed, developed hyperparathyroidism 7 to 33 months after surgery due to graft hyperplasia. Grafts had to be removed. Whereas about 20--25mg were implanted, the removed grafts weighed 0.9--3.1g. Morphological examination showed signs of accelerated growth, infiltration of adjacent structures and invasion of blood vessels. For total removal, repeated and extensive surgery was necessary. Conservative treatment failed to prevent tumour-like growth of autografts. We no longer recommend parathyroidectomy with autotransplantation of parathyroid tissue as the method of choice for the surgical treatment of renal hyperparathyroidism.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/9. Epidural hemorrhage, a rare complication of otitis media.Extradural hemorrhage caused by otitis media is so rare that only four cases were found in a search of the literature. Certain striking similarities and the singular absence of a major demonstrable bleeding vessel in all cases appear more than coincidental and we have proposed a theory to explain the cause of the hemorrhage.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/9. Parasitic lumbar arterial blood supply in renal angiomyolipoma.A young woman with azotemia was found to have an angiomyolipoma which had parasitic blood supply from a lumbar artery. Parasitic lumbar arterial blood supply from a lumbar artery. Parasitic lumbar arterial blood supply has previously been considered a strong indication of malignancy. Since this benign lesion had not invaded the adjacent retroperitoneal structures, the phenomenon tends to confirm the postulate that parasitic blood supply to a lesion may occur through hypertrophy of small anastomotic channels normally present between adjacent vessels but not normally seen in angiography.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |