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Atheromatous embolism is a systemic disease resulting from cholesterol crystal embolization in many organs, including the kidneys. To characterize atheroembolic renal disease (AERD), we retrospectively evaluated 11 patients with acute renal failure after vascular surgery, vascular radiology investigations, and anticoagulation at Miyazaki Medical College from 1994 to 2001. The diagnosis of cholesterol atheromatous embolism was confirmed by tissue examination or clinical grounds. The patients were all elderly men (average age of 66.8 years) with a history of hypertension (55%), diabetes mellitus (45%), hyperlipidemia (45%), and coronary artery disease (18%). Seven patients had livedo reticularis, and 4 had blood eosinophilia. Clinically, 7 patients were managed conservatively and 5 of them improved, whereas 4 patients required dialysis and developed chronic renal failure or died. The serum creatinine levels of the improved patients were significantly lower (1.28+/-0.3 mg/dl, p < 0.005) than the non-improved ones (7.70+/-3.6). The number of eosinophils was significantly higher in the improved patients (576+/-295 /ml, p < 0.05) than in the non-improved ones (208+/-206). However, no significant difference was observed in the levels of serum cholesterol and C-reactive protein among these patients. Since the population at risk for AERD is growing, we should recognize this disease as a cause of acute renal failure.
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PMID:Atheroembolic renal disease: clinical findings of 11 cases. 1256 May 89

The commonest clinical presentation of both immunoalergic interstitial nephritis (IIN) and atheroembolic renal disease (ATD) is an acute renal failure accompanied by skin lesions and eosinophilia. As a consequence, differential diagnosis between both entities is often very difficult. We have performed a comparative retrospective study of those patients diagnosed as having IIN or ATD in our Hospital in the period 1980-2000. A total of 42 patients have been diagnosed of IIN and 16 of ATD. Demographic data, as well as clinical and laboratory parameters and outcomes of every studied patient were analysed. We found a significantly higher prevalence of male sex (100% vs 57%, p < 0.01), previous history of hypertension (100% vs 55%, p < 0.01), chronic renal insufficiency (56% vs 17%, p < 0.01), ischemic heart disease (56% vs 14%, p < 0.001), peripheral ischemic disease, endovascular procedures (87% vs 7%, p < 0.001) and anticoagulant treatments (25% vs 5%, p < 0.001) among patients with ATD as compared with IIN, respectively. On the contrary, previous infections (45% vs 12%, p < 0.01) and exposure to new drugs (100% vs 40%, p < 0.001) were significantly more frequent among IIN patients in compare with ATD. ATD patients showed skin lesions consisting of livedo reticularis and digital infarcts (63% vs 31%, p < 0.05) accompanied by blood pressure increase (100% vs 24%, p < 0.001), whereas IIN patients showed fever (41% vs 19%, p < 0.05) and cutaneous rash as significant clinical manifestations, respectively. The number of ATD patients with proteinuria > 1 g/24 h was significantly higher, but no differences between both groups in the prevalence of urinary sediment abnormalities were observed. The prevalence of absolute eosinophilia was high in both groups (88% among ATD patients, 64% among IIN patients; pNS). Prognosis of both entities was clearly different: Almost all patients with ATD died (69%) or evolved to end-stage renal failure, whereas most patients with IIN showed a recovery of renal function after withdrawal of responsible drugs and steroid treatment. In summary, the analysis of clinical and laboratory data allows an initial differential diagnosis in patients suspected as having IIN or ATD.
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PMID:[Immunoallergic interstitial nephritis vs. cholesterol atheroembolism. Differentiating characteristics]. 1277 76

Sneddon's syndrome is a rare combination of generalised livedo reticularis and cerebrovascular accidents. Its clinical presentation varies widely and its aetiology is still not known. 60 to 80% of patients are female. First symptoms of the syndrome are mostly repetitive cerebral strokes, but reduced perfusion of the skin, seen as blue or red-brown mottling, precedes the strokes. The vascular disease is generalised and often accompanied by arteriosclerosis, systemic arterial hypertension, valvular heart disease and the presence of antiphospholipid antibodies. The diagnostic procedures are complicated and have to exclude other autoimmunological diseases. Therapeutic options are anticoagulatory therapy with warfarin, ASS or heparin, reduction of endothelial proliferation with ACE-inhibitors, and improvement of microvascular perfusion with prostaglandine. The increased anaesthesiological risk with these patients is due to the acute risk of thromboembolism and ischaemic cerebral and cardiovascular insults. The anaesthetic management must provide stable perfusion pressures for cerebral and myocardial arteries and avoid increasing risk factors for thromboembolism such as increased blood viscosity or stasis due to improper positioning of the patient. The choice of anaesthetic drugs is dependent on good controllability for haemodynamic stability. The high risk of patients with Sneddon's syndrome justifies a more invasive haemodynamic monitoring and postoperative surveillance on an intensive care unit. This case report describes the anaesthesiological considerations for, and management of, a patient with Sneddon's syndrome who was admitted to hospital for vaginal hysterectomy.
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PMID:[Perioperative management of a patient with Sneddon syndrome--a case report]. 1287 40

Cholesterol crystal embolism (CCE) is caused by the shedding of cholesterol crystals into the bloodstream, and it has been recently recognized as a serious complication after vascular procedures. Our case of CCE, which was diagnosed by skin and renal biopsies, occurred in a patient with hypertension and diabetes mellitus, 3 months after coronary angiography, with the development of renal failure and blue toes. After low-density lipoprotein apheresis (LDL-A), the skin lesions, including livedo reticularis and pain from the acrocyanotic toes, dramatically improved, with partial recovery of renal function. Following the administration of low-dose corticosteroid and candesartan--an angiotensin II type 1 receptor antagonist (ARB)--the eosinophilia disappeared and renal function improved gradually with a decrease in urinary protein excretion. Therefore, a combination therapy of LDL-A, low-dose corticosteroid, and an ARB is a possible treatment for CCE, although the possibility of spontaneous recovery of renal function cannot be eliminated for this patient.
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PMID:Acute renal failure due to cholesterol crystal embolism treated with LDL apheresis followed by corticosteroid and candesartan. 1458 47

We report on a 54-year-old woman with Sneddon's syndrome manifested by livedo reticularis, fetal losses, hypertension, and high antinuclear antibody titres. At the age of 42 years she developed tremor of the trunk, limbs, and head only in the standing position that interfered with walking, followed some years later by cognitive decline and a parkinsonian syndrome. T2-weighted brain magnetic resonance imaging showed high signal in cortical areas, basal ganglia, midbrain, and cerebellum.
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PMID:Tremor as the first neurological manifestation of Sneddon's syndrome. 1538 13

Cholesterol crystal embolization (cholesterol embolism, cholesterol embolic disease) is a multiorgan disease, which is a severe iatrogenic complication from an invasive vascular procedure, such as manipulation of the aorta during angiography or vascular surgery, and after anticoagulant and fibrinolytic therapy. The diagnosis is made postmortem in two-thirds of cases. Cholesterol crystal embolism is an increasing and still underdiagnosed disease. Pathognomonic is the constellation of acral pain, nonhealing ulcerations and necrosis, livedo racemosa with intact peripheral arterial pulses and sudden onset of renal failure and arterial hypertension. Biopsy of the affected organs is essential for diagnosis. We report the case of a 66-year-old man who following coronary arteriography with PTCA and implantation of stents developed acral necrosis and cyanosis, livedo racemosa and acute irreversible renal failure.
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PMID:[Cholesterol embolism syndrome after coronary angiography]. 1559

Renal thrombotic manifestations have been reported since the earliest descriptions of the antiphospholipid (Hughes) syndrome (APS). The spectrum of clinical features associated with antiphospholipid nephropathy continues to widen. This review will highlight recent developments such as the prevalence of hypertension, livedo reticularis and renal artery stenosis as well as the ultrastructural changes seen in antiphospholipid nephropathy. The increasing risks of renal transplantation in antiphospholipid antibody positive patients is also discussed leading some authors to question whether these patients should undergo transplantation at all.
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PMID:Renal manifestations of the antiphospholipid syndrome. 1573 87

Anti-phosphatidylethanolamine antibodies (aPE) belong to the group of anti-phospholipid antibodies (aPL) and are directed against neutral phospholipid, connected with co-factor protein, while cardiolipin antibodies (aKL) are directed against negative phospholipid. The paper presents a study of prevalence and clinical significance of IgG aPE in 28 patients (22 women and 6 men, mean age 47.6 +/- 11.6 years) with Sneddon's syndrome (SS), which consists in cerebrovascular disturbances and extensive livedo reticularis. IgG aPE were detected by immune-enzyme assay. The upper normal limit, calculated as mean + 3SD after studying 19 healthy donors, was 0.303 optic density units. aPE were found in 15 (54%), aKL and/or lupus anticoagulant (LA)--in 6 (21%) patients with SS. aPE were found in 10 (46%) out of 22 aKL- and LA-negative patients. Among the aPE-positive patients there was a higher incidence of cortic dementia (53% vs. 8%, p = 0.02), the widening of cortical sulci, detected by means of computed tomography and magnetic resonance imaging (73% vs. 31%, p = 0.05), and mild renal syndrome (73% vs. 16%, p = 0.03). Besides, they displayed a higher rate of headaches (87% vs. 62%), chorea (33% vs. 8%), epilepsy (27% vs. 8%), non-carrying of pregnancy (91% vs. 50%), peripheral venous thrombosis (27% vs. 15%), coronary heart disease (47% vs. 31%), cardiac valvular thickening, detected by means of EchoCG (93% vs. 69%), arterial hypertension (87% vs. 54%), thrombocytopenia (20% vs. 0), anemia (40% vs. 15%); however, the difference was not significant. The results show that aPE detection, performed in addition to detection of classic immunological antiphospholipid syndrome markers (aKL and LA), increases the portion of aPE-positive patients with SS by 33%. aPE are often (in 46% of cases) found in aKL- and LA-negative patients with SS. aPE is likely to be the most significant factor of thrombosis in small arteries of the brain cortex and kidneys, which could explain their association with dementia and renal syndrome.
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PMID:[Anti-phosphatidylethanolamine antibodies in patients with Sneddon's syndrome]. 1598 83

Antiphospholipid syndrome spans many medical disciplines. Classic criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical complications of thrombosis or pregnancy loss. Other common associated manifestations include livedo reticularis, thrombocytopenia, valvular heart disease, and nephropathy with renal insufficiency, hypertension, and proteinuria. Treatment of serious complications with anticoagulation is standard; generally warfarin for thrombosis and aspirin/heparin for pregnancy prophylaxis. Detailed recommendations regarding precise intensity and duration of anticoagulation are still a subject of debate.
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PMID:Antiphospholipid syndrome: review. 1600 69

A 74-year-old man with hypertension and diabetes mellitus was admitted to our hospital because of acute exacerbation of chronic renal failure after treatment with urokinase for a cerebral infarction. A percutaneous renal biopsy was performed to examine the cause of renal damage, revealing glomerulosclerosis and cholesterol clefts in the small arteries. Subsequently eosinophil was increased to 21% and livedo reticularis was found in the patient's foot. A skin biopsy was performed, and cholesterol clefts were again found in the small arteries. For the reason, our diagnosis was cholesterol crystal embolism. Although 30 mg of prednisolone was administered, the patient's renal function did not improve and maintenance hemodialysis therapy was necessary. This is a rare case of cholesterol crystal embolism caused by urokinase without any invasive vascular procedures.
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PMID:[Case of cholesterol crystal embolism occurring after treatment of cerebral infarction with urokinase]. 1677 2


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