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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency of renal vascular lesions (RVL) and their relevance in the progression of renal damage were evaluated by the Pathology Group of the "Gruppo Italiano per lo Studio della Nefrite Lupica" (GISNEL). Of 285 patients with lupus nephritis collected from 20 nephrology centers in Italy and classified according to World Health Organization (WHO) criteria, 79 cases (27.7%) with RVL were identified and classified as follows: (1) lupus vasculopathy (n = 27); (2) hemolytic-uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) malignant hypertension-like lesions (n = 24); (3) vasculitis (n = 8); (4) arterio-arteriosclerosis (n = 20). At the time of renal biopsy, patients with RVL had mean serum creatinine levels significantly higher than patients without RVL (201.8 +/- 195.9 mumol/L [2.2 +/- 2.2 mg/dL] v 108.1 +/- 108.0 mumol/L [1.2 +/- 1.2 mg/dL]; P less than 0.01). Hypertension was more frequent in patients with RVL than in those without (68.4% v 30.5%; P less than 0.01). The probability of kidney survival assessed according to the Kaplan-Meier method at 5 and 10 years was, respectively, 74.3% +/- 5.9% and 58.0% +/- 8.9% in patients with RVL, compared with 89.6% +/- 2.7% and 85.9% +/- 3.7% in patients without RVL. However, the two groups did not differ significantly as regards overall survival, the probability of survival at 5 and 10 years being 86.5% +/- 4.5% and 78.8% +/- 6.6% in patients with RVL and 92.2% +/- 2.2% and 83.3% +/- 4.4% in patients without RVL.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal vascular lesions as a marker of poor prognosis in patients with lupus nephritis. Gruppo Italiano per lo Studio della Nefrite Lupica (GISNEL). 186 81

Relapsing hemolytic and uremic syndrome is infrequent in pediatric patients. The case of an infant who had HUS at 11 months of age and subsequently developed two relapses at 3 years and 3 1/2 years of age is reported. Outcome of the first episode was favorable, with no elements suggesting an increased risk for subsequent relapses. Conversely, the second and third episodes were followed by gradual deterioration of renal function and persistence of severe arterial hypertension. To clarify the epidemiology, clinical manifestations, histology, and outcome of HUS, the 24 pediatric cases of relapsing HUS reported in the English-language medical literature were reviewed. In this group, mean age at onset was 4 years 1 month (range 4 months-9 years) and number of relapses ranged from 1 to 14. Evidence of microangiopathy was the most common histologic finding (15/22) but non-specific lesions were seen in some instances. Opinions vary as to the prognosis of relapsing HUS; our case suggests that relapses are of adverse prognostic significance.
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PMID:[A new case of relapsing hemolytic and uremic syndrome]. 195 8

We examined 61 patients an average of 9.6 years (range 5 to 18 years) after an episode of childhood hemolytic-uremic syndrome. Twenty-four (39%) had one or more abnormalities. Seven (11%) had proteinuria and six (10%) had low creatinine clearance as solitary abnormalities. Eight (13%) had both proteinuria and reduced creatinine clearance; three (5%) had a combination of hypertension, proteinuria, and low creatinine clearance. Abnormalities sometimes appeared after an interval of apparent recovery. Logistic regression analysis showed that duration of anuria was the best predictor of disease at follow-up. No patients who had anuria lasting longer than 8 days or oliguria exceeding 15 days escaped chronic disease. However, 45% of those with disease had no anuria, and a third had no oliguria. Physicians should therefore be cautious in assuming recovery from HUS on the basis of a single evaluation and should periodically evaluate patients for an extended period.
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PMID:Long-term outcome and prognostic indicators in the hemolytic-uremic syndrome. 194 99

Despite great improvement in patient and graft survival, the long-term morbidity and mortality in renal transplant recipients are still significant. Cardiovascular disease accounts for much of the mortality in long-term survivors; screening before the transplant procedure and adequate control of hypertension should help improve patient survival. Many of the gastrointestinal complications are due to overimmunosuppression and sepsis. Adequate management must include withdrawal of all immunosuppressive medications in order to save the patient's life. Liver disease is usually of viral origin; patients with chronic active hepatitis or cirrhosis should remain on dialysis. Chronic rejection is the major cause of graft loss in long-term survivors; it is unresponsive to antirejection treatment and its progression may be mediated by nonimmunologic mechanisms. Correctable problems such as renal artery stenosis and ureteral obstruction should be ruled out before a late deterioration in graft function is disregarded as chronic rejection. Post-transplant diabetes, osteonecrosis, cataracts, and nephrotoxicity are directly related to the various immunosuppressive drugs currently used. The lowest dose compatible with graft acceptance should help reduce the incidence of these nonfatal but significant complications. Recurrence of disease is a common histologic finding in many transplant recipients but, except for a few diseases such as HUS, FSGS, and oxalosis, it usually does not lead to graft failure. Successful transplantation restores fertility in many uremic patients. Adequate counseling on contraception is imperative in order to avoid unwanted pregnancies and to delay parenthood for at least 1 year. Current immunosuppressive agents are not teratogenic, no dose adjustments are necessary, and an ill-advised decrease in medication may precipitate a rejection episode. Premature delivery is the major problem in these patients and can be avoided by maintaining adequate graft function and controlling hypertension and infections. It is evident from this review that most of the long-term morbidity and mortality seen in renal allograft recipients are due to overimmunosuppression with sepsis or to side effects of the individual drugs, steroids being a common denominator in almost all cases. New immunosuppressive protocols must aim not only to improve patient and graft survival but also to avoid the many complications that limit the full rehabilitation of these patients.
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PMID:Problems in the long-term renal allograft recipient. 226 90

A case is reported of a 23-year-old multiparous normotensive woman who developed HUS (hemolytic uremic syndrome) on 2 occasions. Both episodes had been preceded by relatively short periods of estrogen-progestogen contraceptive usage. Neither episode was accompanied by arterial hypertension. This syndrome is characterized by acute renal failure associated with histological lesions of thrombotic microangiopathy. Laboratory findings, treatment, and kidney transplant in both of the episodes are described. The 1st instance occurred 5 months postpartum and 4 months after starting the OC (oral contraceptive) use. The 2nd instance occurred 10 months after starting OC usage. In the intervening 8 years, the patient had used a pure progestogen contraceptive without problems. A table presents findings from a review of the literature regarding an association between HUS and estrogen use.
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PMID:Recurrent hemolytic uremic syndrome during oral contraception. 616 23

A group of 21 children with HUS underwent serial estimations of PRA and blood pressure. At time of administration PRA was 13.2 divided by 1.59 ng/ml/hr (mean + SE), in seven who were hypertensive the mean was 14.1 +/- 3.2 ng/ml/hr and despite the wide range PRA was considered to be elevated in 6 of this group. The 14 normotensive children had a mean PRA of 12.8 +/- 1.8 ng/ml/hr, only 1 patient having a value within the normal range. In 9 children PRA was normal once diuresis had been established (9 +/- 1.4 ng/ml/hr) and only two patients of this group were hypertensive at time of the study. PRA measured in 8 children 4 weeks after presentation was 7 +/- 1.6 ng/ml/hr, and 3 hypertensive children having a mean value of 7.5 ng/ml/hr and the 5 normotensive children a mean of 2.5 ng/ml/hr. The data indicate that activation of the renin-angiotensin system in HUS occurs irrespective of hypertension. Furthermore blood pressure levels were not correlated to the degree of hydration.
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PMID:Systemic hypertension and plasma renin activity in children with the hemolytic-uremic syndrome. 675 46

Thirty-one children with diarrhea-associated hemolytic uremic syndrome were retrospectively studied to determine if expression of the P1 blood type was related to the severity of the acute illness or to the prognosis. No differences were found in the clinical variables studied in the patients who were P1 positive compared to the patients who were P1 negative. The clinical variables studied included the age, hemoglobin, white blood cell count (WBC), and presence of central nervous system involvement at presentation, the duration of elevated WBC, thrombocytopenia, hemorrhagic colitis, and anuria, and the follow-up incidence of proteinuria, hypertension and decreased GFR. Expression of the P1 phenotype does not appear to exert a protective influence in patients with D+ HUS.
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PMID:The P1 blood group and the severity of diarrhea-associated hemolytic uremic syndrome. 785 Oct 28

Thus I would like to conclude by saying that an idiopathic form of obliterative renal arteriopathy account for the rare presentation of severe hypertension and progressive renal failure with or without overt hemolytic anemia and thrombocytopenia in children. It can be labelled as primary malignant nephrosclerosis (NScl) or atypical HUS, based on primary thrombotic angiopathy. This, essentially intimal changes, is seen in diverse conditions and appears to result from primary endothelial injury followed by intimal exudation, thrombosis, and repair by fibrosis. Persistent or recurrence of this process form the basis of progressive obliterative arteriopathy. The result is renal ischemia and renin-angiotensin mediated hypertension. Establishment of a vicious circle would further accelerate HT and lead to end stage renal failure. Early recognition and prompt therapeutic intervention might prove beneficial.
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PMID:Thrombotic microangiopathy with hypertension and acute renal failure in children (a typical hemolytic uremic syndrome). 869 75

The short-term prognosis of the "typical", "post-enteropathic" form of infantile HUS is usually good, with a complete recovery of renal function. However, the extent of the renal damage observed on some biopsies may raise concern for the long-term prognosis. Therefore, we studied the outcome of 29 patients affected with classical HUS in infancy or early childhood and followed-up for 15-28 years (m = 18 yrs). Initial renal symptoms ranged from a moderate renal failure with normal diuresis to a 12-day anuria: 21 children had to be treated by peritoneal dialysis. Twenty-five patients underwent a renal biopsy shortly after recovery: lesions of glomerular thrombotic microangiopathy (TMA) were found in 14 patients, and patchy cortical necrosis was diagnosed in the other 11. At latest examination 10 patients had no renal abnormality, 12 had residual renal symptoms (hypertension in 7, proteinuria in 4 and midly reduced GFR in 1), 3 were in chronic renal failure (CRF), and 4 had reached end-stage renal failure (ESRF) 16-24 years after onset; 2 of these latter 4 had a normal GFR at 10-year examination. The long-term evolution was not correlated with the initial clinical severity but appeared well correlated with the extent of the histological damage: 10 of the 11 patients with cortical necrosis have either ESRF (4), CRF (3) or renal sequelae (3), and 4 of the 5 patients with TMA involving more than 50% of glomeruli present with moderate sequelae, whereas the 9 patients with TMA involving less than 50% of glomeruli are symptom-free or have mild sequelae. Thus, the risk of renal failure 20 years after a seemingly cured childhood HUS is not negligible, and renal histology is the best indicator of long-term prognosis.
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PMID:Long-term (15-25 years) outcome of childhood hemolytic-uremic syndrome. 883 49

Atypical, non-diarrhoea associated haemolytic uraemic syndrome (D-HUS) is a heterogeneous disorder with a generally poor outcome, although this view has now been questioned. The clinical and laboratory features of 23 children with D-HUS, representing a third of all patients with HUS seen during the last 26 years, were examined. The median age was 4.9 years (range 3 days-13.8 years). Twenty one children (91%) survived the initial phase. All patients except six infants aged < 18 months required dialysis (74%). Hypertension (43%), cardiomyopathy (43%), and cerebral convulsions (48%) were common. Nineteen (83%) children were followed up for a median period of 5.5 years (range 0.5-23.4). Only five (26%) patients, among them four infants, recovered completely. Six (32%) patients had one to 10 recurrences, including two siblings with neonatal onset, and eight (42%) developed end stage renal failure. Five children underwent cadaveric renal transplantation, with recurrence and subsequent graft failure in two. Four children died, resulting in an overall mortality of 26%. Atypical HUS is heterogeneous with regard to epidemiology, pathophysiology, and outcome. Children with a recurrent, familial, or neonatal course have worse outcomes; in contrast, infants not requiring dialysis in the acute phase have a better prognosis.
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PMID:Heterogeneity of atypical haemolytic uraemic syndromes. 924 50


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