Cases reported "Vascular Diseases"

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1/39. Systemic infection with Alaria americana (trematoda).

    Alaria americana is a trematode, the adult of which is found in mammalian carnivores. The first case of disseminated human infection by the mesocercarial stage of this worm occurred in a 24-year-old man. The infection possibly was acquired by the eating of inadequately cooked frogs, which are intermediate hosts of the worm. The diagnosis was made during life by lung biopsy and confirmed at autopsy. The mesocercariae were present in the stomach wall, lymph nodes, liver, myocardium, pancreas and surrounding adipose tissue, spleen, kidney, lungs, brain and spinal cord. There was no host reaction to the parasites. Granulomas were present in the stomach wall, lymph nodes and liver, but the worms were not identified in them. hypersensitivity vasculitis and a bleeding diathesis due to disseminated intravascular coagulation and a circulating anticoagulant caused his death 8 days after the onset of his illness.
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2/39. Disseminated intravascular meconium in a newborn with meconium peritonitis.

    A 3-day-old premature infant with meconium peritonitis, periventricular leukomalacia, and pulmonary hypertension died with respiratory insufficiency. An autopsy disclosed intravascular squamous cells in the lungs, brain, liver, pancreas, and kidneys. Numerous pulmonary capillaries and arterioles were occluded by squamous cells, accounting for pulmonary hypertension. brain parenchyma surrounding occluded cerebral vessels showed infarct and gliosis. A mediastinal lymph node filled with squamous cells alluded to the mechanism by which these cells from the peritoneal cavity likely entered the bloodstream--namely, via diaphragmatic pores connecting with lymphatics. Thus, disseminated intravascular meconium rarely may complicate meconium peritonitis and have devastating consequences.
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keywords = kidney
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3/39. Intravascular papillary endothelial hyperplasia in the kidney of a child.

    Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion which is thought to represent an unusual form of organizing thrombus. A case of IPEH in the kidney of a 7-year-old girl is described. She suffered from intermittent flank pain and gross hematuria for 6 months. On radiological examinations, well-defined hypoechoic lesions were identified in the medullary portion of the left kidney. A well-demarcated, sponge-like mass was noted on gross examination. It was an intravascular mass lined by a fibrous capsule of various thicknesses. It was characterized by papillary fronds lined with benign endothelial cells. This is the first description of a renal IPEH in a child.
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keywords = kidney
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4/39. Dramatic worsening of vascular calcifications after kidney transplantation in spite of early parathyroidectomy.

    vascular calcification is a common feature in chronic dialysis patients, but their clinical significance is debated and the role of kidney transplantation (TP) in the natural history of their development has received scanty attention. We will describe a case of dramatic worsening of vascular calcifications during TP in a young patient in spite of early and successful parathyroidectomy (PTX), and will discuss other causes which might be putatively linked to vascular damage during the time of TP. A 37-year-old man on regular dialytic treatment (RDT) for 11 years, received his first cadaveric transplantation in January 1993. He underwent PTX 6 months after TP because of the lack of decreasing in parathyroid hormone values despite normal graft function. Although PTX was effective, a dramatic worsening was evident in large as well as in medium and small-sized arteries during the following three years of TP. In February 1997, few months after starting dialysis again because of the recurrence of his primary membranoproliferative glomerulonephritis (MPGN), the patient experienced myocardial infarction followed by aorto-coronary bypass (right coronary artery and anterior descending coronary artery) and leg "claudicatio". Though a role for parathyroid hormone in vascular disease has been commonly accepted, the case here reported clearly shows that blunting parathyroid gland activity may be unable to avoid the worsening of a process of vascular disease during the time of TP. Many other factors--linked to the time of TP--may be involved in vascular diseases, such as nephrotic syndrome, dyslipidemia, hypertension and drugs. In the case of our patient, a clear cut risk factor for his progressive atherosclerosis can be designated hyperlipidema and other disturbancies secondary to a nephrotic syndrome due to relapse of MPGN, together with persistent hypertension. This is the first case report in the English literature which clearly demonstrates that TP may add fuel to the fire of vascular disease also in young people and even in the absence of parathyroid hyperactivity, perhaps on the basis of a favorable genetic background. Furthermore, the history of our patient demonstrates that vascular calcifcation heralds major cardiovascular diseases.
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keywords = kidney
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5/39. Polycystic kidney disease associated with cervical arteriovenous shunt and bilateral jugular vein occlusion.

    A 59-year-old man with abnormal vascular features (intracranial aneurysm, a cervical arteriovenous shunt, bilateral internal jugular vein occlusions, and left transverse sinus hypoplasia), as well as left optic atrophy was suspected to have familial polycystic kidney disease. The possibility of autosomal dominant polycystic kidney disease complicated by ehlers-danlos syndrome type IV due to the coexistence of vasculopathy and polycystic kidneys was considered. However, the negative results of a skin fibroblast culture rendered the diagnosis of ehlers-danlos syndrome type IV unlikely. The cause of left optic atrophy in our patient remains unclear although it was suspected to be a secondary consequence of papilledema, which was caused by intracranial hypertension.
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ranking = 160.00732622129
keywords = kidney disease, kidney
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6/39. Reversed intrapulmonary right-to-left shunt after banding of the patent ductus venosus.

    Diffuse pulmonary microvascular arteriovenous communication developed in an 8-year-old girl with a patent ductus venosus. Tc-99m macroaggregated albumin (MAA) pulmonary perfusion scintigraphy with total-body imaging demonstrated multiple lung perfusion deficits and abnormal tracer uptake in systemic organs with hepatic radioactivity greater than the kidneys, suggesting the presence of right-to-left shunt and abnormal hepatic hemodynamics. I-123 iodoamphetamine transrectal portal scintigraphy revealed a large portosystemic venous shunt. The follow-up Tc-99m MAA perfusion scans after banding of the patent ductus venosus revealed partial improvement of the perfusion deficits and right-to-left shunt, indicating the possible reversibility of this pulmonary shunt complication.
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keywords = kidney
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7/39. Chronic post-thrombotic obstruction of the inferior vena cava: both renal veins being the cause of painless gross hematuria from pelviureteral mucosal varices in normal functioning kidney.

    A 31-year-old man presented with painless gross hematuria. His serum-creatinine level was within the normal range. Abdominopelvic CT showed an infrahepatic calcified fibrous cord, which was suggestive of inferior vena cava (IVC) remnant. Extensive venous collateralization around both kidneys and venous drainage of the extremities via the inferior epigastric and internal thoracic veins were shown. We report a case of chronic post-thrombotic obstruction of the IVC, involving both renal veins, which was believed to be the cause of painless gross hematuria from mucosal varices of the pelviureteral system in normal functioning kidney.
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ranking = 6
keywords = kidney
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8/39. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis.

    calciphylaxis is characterized by an extensive media-calcification of cutaneous and subcutaneous arterioles and capillaries. Recent studies have provided evidence that vascular calcification is a process with similarities to bone metabolism. Bone morphogenic protein-4 (BMP-4) is physiologically involved in bone development and repair. The presence of BMP-4 in atherosclerosis and in sclerotic heart valves led us to suggest that BMP-4 is also involved in calciphylaxis. A 47-year-old male patient developed end-stage renal failure due to chronic glomerulonephritis. He has had two kidney transplants with an immunosuppressive regimen consisting of cyclosporine A and steroids. He was admitted to our hospital because of an increase in serum creatinine (Cr) and he subsequently developed progressive dermal ulcerations. A skin biopsy led to the diagnosis of calciphylaxis. immunohistochemistry for BMP-4 of a skin specimen from our patient showed strong cytoplasmic immunoreactivity of intradermal cells with clear spatial association to arterioles and hair follicles. Whereas there are identified inhibitors and promoters of vascular calcification, the presence of BMP-4 has not been demonstrated in calcific uremic arteriolopathy. In contrast to atherosclerosis, BMP-4 in calciphylaxis cannot be found in vascular media, but in intradermal cells at the border of arterioles and hair follicles. Therefore, in calciphylaxis BMP-4 can play the role of a cytokine, a growth factor or a media-calcification promoter.
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keywords = kidney
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9/39. Immunosuppressive therapy for kidney transplant prevents vaso-occlusive crisis in a haemoglobin SC disease patient.

    Although the molecular basis of sickle cell disease (SCD) is well established, the wide variability in clinical manifestations still puzzles haematologists and clinicians. Recently, SCD started to be considered by different groups as a chronic inflammatory condition, where the inflammatory tendency of each individual could drive more or less severe clinical features. Here we describe a haemoglobin SC disease patient (heterozygous to both HbS and HbC variants) that experienced several vaso-occlusive crises before underwent a successful kidney transplantation. Since then (16 years ago), she is on uninterruped immunosuppressive therapy, and do not experienced any severe vaso-occlusive crisis. Considering SCD associated morbidity as a result of exacerbated immune responses, we suggest that the immunosuppressive therapy directed to the kidney graft maintenance is actually also helping in the control of the chronic inflammatory responses associated to SCD.
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ranking = 6
keywords = kidney
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10/39. sirolimus-induced thrombotic microangiopathy is associated with decreased expression of vascular endothelial growth factor in kidneys.

    The aim of this study was to examine the clinical characteristics, the histological features and the renal expression of vascular endothelial growth factor (VEGF) of five patients with sirolimus-associated thrombotic microangiopathy (TMA). sirolimus-induced TMA occurs preferentially in kidneys with concomitant endothelial injury: it was observed in three patients with acute cellular rejection on calcineurin inhibitor-free regimen, in one patient with chronic graft rejection on a calcineurin inhibitor-free protocol and in one patient with chronic calcineurin inhibitor nephrotoxicity. We found that renal VEGF expression during sirolimus-induced TMA was significantly lower than VEGF expression in normal transplanted kidneys (p < 0.01). Decreased expression of VEGF seems to be a consequence of sirolimus treatment since (i) analysis of two biopsies performed after the switch of sirolimus to calcineurin inhibitor showed reappearance of VEGF expression, (ii) no decreased expression of VEGF was found in five kidneys with classical TMA and, (iii) an increased expression of VEGF was observed in seven kidneys with acute cellular rejection on a sirolimus-free immunosuppressive regimen (p < 0.01). The potential role of sirolimus-induced downregulation of VEGF as a predisposing factor to the development of TMA is discussed.
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ranking = 8
keywords = kidney
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