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

The clinical features of 55 cases of autosomal recessive polycystic kidney disease (ARPCKD) have been reviewed. Each had evidence of ARPCKD. The outcomes of 87% were known; 24 had died. Twenty-four of 31 were seen between 1980 and 1986; 7 could not be traced. Forty-five percent presented under 1 month; 38% between 1 month and 1 year; and 9 cases over 1 year. Hyponatraemia occurred in 15 out of 19 aged less than 3 months; hypertension occurred in 65%; splenomegaly in 47% of those surviving more than 3 months. Portocaval shunts were done in 5 aged 2-12 years. Thirteen died of renal failure, 6 under 1 year, and 7 between 1 year and 13 years. Life-table survival rates calculated from birth revealed that 86% were alive at 3 months, 79% at 1 year, 51% at 10 years, and 46% at 15 years. Calculations based on patients who survived to 1 year of age showed that 82% were alive at 10 years and 79% at 15 years. These results reveal an improved prognosis for a condition once assumed to be fatal.
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PMID:Autosomal recessive polycystic kidney disease. 270 87

Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11-22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts.
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PMID:Infantile chronic tubulo-interstitial nephritis with cortical microcysts: variant of nephronophthisis or new disease entity? 270 88

Hypertension is an early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Whether polycystic kidneys have an intrinsic abnormality that leads to sodium retention, volume expansion, and hypertension is uncertain. We studied the natriuretic response to a 4-hour infusion of physiologic saline at a rate of 6.5 ml/kg per hour in 10 patients with ADPKD who had normal renal function and 10 gender- and age-matched control subjects. Baseline 24-hour urinary excretions of sodium and potassium were similar in both groups. The baseline filtration fraction was significantly higher in the patients with ADPKD than in the control subjects. During the infusion of saline, no significant changes in blood pressure, clearance of inulin, or clearance of p-aminohippuric acid were detected. The increase in fractional excretion of sodium over baseline was significantly higher in the patients with ADPKD than in the control subjects. The pressure-natriuresis regression line was significantly shifted to the right in patients with ADPKD who had hypertension. The fractional excretion of potassium was significantly lower in patients with ADPKD than in control subjects. No significant differences in plasma renin activity, aldosterone, or atrial natriuretic factor were detected between the two groups. These observations suggest the presence of subtle abnormalities in the management of renal sodium that might contribute to the development and maintenance of hypertension in patients with ADPKD.
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PMID:Natriuretic response to volume expansion in polycystic kidney disease. 272 63

A review of 20 polycystic kidney disease patients in admitted to Kaohsiung Medical College Hospital is herein reported. In this series 60% of the patients were in the fourth to sixth decades of life at the time of diagnosis. The average age was 50 years. The disease was generally more commonly found in the females than in male subjects. Flank pain and gross hematuria was the most common presenting symptom in these cases. In 70% of the patients hypertension were noted, besides 55% of patients have associated liver cysts and 9% of the patients have associated pancreatic cystic diseases. In a comparison of ultrasonography, CT scans, renal angiography, intravenous pyelography and retrograde pyelography in the evaluation of polycystic kidney disease, it was found that CT scans and ultrasonography had more than a 90 per cent diagnostic accuracy rate, while intravenous pyelography had only a 30 percent accuracy rate. Because of improved treatments such as antibiotic and hemodialysis techniques the five-year survival rate of patients, with polycystic kidney diseases, may be increased.
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PMID:[Diagnosis and treatment of adult polycystic kidney disease]. 277 66

To provide information on the possible influence that hypertension or its treatment might have on the development of cysts in autosomal dominant polycystic kidney disease, we studied the effects of the sodium content of the diet, DOCA-salt hypertension, renovascular hypertension, and the administration of enalapril or furosemide on the development of 2-amino-4-5-diphenylthiazole (DPT)-induced renal cystic disease. DOCA-salt hypertension caused vascular and glomerular lesions and proteinuria, but it did not enhance the development of cysts. Cytogenesis was enhanced in experimental conditions where the renin-angiotensin system is known to be activated. On the other hand, interventions known to suppress the renin-angiotensin system lessened the development of cysts. We hypothesize that this effect might be mediated by intrarenal angiotensin II and its capacity to promote cell growth and to control the postglomerular vascular resistances and the compliance of the renal interstitium.
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PMID:Mechanisms affecting the development of renal cystic disease induced by diphenylthiazole. 284 32

The clinical features and laboratory data of 93 children with polycystic kidney disease were analysed. Family studies showed that the disease was dominant (DPKD) in 17 and recessive (RPKD) in 32 of them. Of the remaining 44 sporadic patients, 1 was classified by histological and/or imaging findings as having DPKD, 41 as having RPKD and 2 could not be classified. The symptoms tended to be more severe in RPKD than in DPKD, but there was much overlap. Death in early life was common in RPKD (55/73) and more rare in DPKD (4/18). If a child with DPKD had disease manifest during the neonatal period, then siblings were usually affected in the neonatal period. Survival to adulthood was seen in both diseases. In the patients who survived the neonatal period, hypertension was more common in RPKD (11/18) than in DPKD (4/14). Symptoms of portal hypertension were present in 2 patients with RPKD and none with DPKD. None of the laboratory investigations discriminated between the two entities. Glomerular filtration rate was diminished more often in RPKD (9/11) than in DPKD (2/8). Some difference was seen in the maximal urine concentrating ability; it was always reduced, often markedly, in RPKD but usually either normal or only moderately disturbed in DPKD. Studies on hepatic function and hepato-cellular damage were usually normal, but bacterial cholangitis was noted in some children with RPKD. The differential diagnosis between DPKD and RPKD needs to be based on the family history, family studies, radiological and/or histological features.
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PMID:Dominant and recessive polycystic kidney disease in children: evaluation of clinical features and laboratory data. 315 29

The effects of hypertension on the course of early chronic renal failure were evaluated in 233 patients with renal disease of diverse etiology, followed for 12-166 months (mean 51.35) on protein-restricted diet. On entry, 174 patients (74.6%) were hypertensive and 59 (25.4%) were normotensive. Serum creatinine levels rose from 2.40 +/- 1.11 to 4.84 +/- 3.26 mg/dl in the overall population. Deterioration of renal function was more evident in hypertensives (percent increase in serum creatinine 112.8, monthly increase 0.053 mg/dl) than in normotensives (percent increase 70.9, monthly increase 0.032 mg/dl). This difference, however, was not statistically significant. Progression of renal failure was significantly faster in hypertensive than in normotensive patients in the groups of polycystic kidney disease and chronic renal failure of unknown etiology. The actuarial renal survival probability at 72 months was 77% in normotensives and 47% in hypertensives. Among the 84 patients (36.1%) who had a fast deterioration of renal failure, 71 (84.5%) were hypertensive. In conclusion, hypertension seems to play an important role in worsening the prognosis of patients with renal parenchymal disease and early chronic renal failure. It is still difficult to separate the exact role of hypertension from the constellation of pathogenetic factors (such as the underlying renal disease, the magnitude and duration of proteinuria, the inadequate dietary contents of protein and phosphate) which may affect the progression of chronic renal disease in man.
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PMID:Role of hypertension on the progression of renal disease in man. 320 72

Long-term imaging and clinical findings are reported in six children whose polycystic kidney disease was detected in infancy or early childhood. Over time (2 years to 20 years) all patients developed portal hypertension from hepatic fibrosis, a problem primarily noted in recessive pattern polycystic kidney disease. Mild renal failure (two patients) was accompanied by serious systemic hypertension in the same patients. In one family, one of the babies also showed dilated right hepatic ducts. Imaging studies included urography and CT although recently ultrasonography was the method of choice. The relative renal and hepatic manifestations in these patients so changed with time that it would seem fallacious to attempt to use rigid classifications based on findings at initial diagnosis.
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PMID:The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease. Initial and follow-up sonographic and radiographic findings. 328 Nov 10

One hundred fifty-four children aged eighteen years or younger from 83 families with autosomal-dominant polycystic kidney disease were studied by ultrasonography or excretory urography. Twenty-three children had bilateral renal involvement with at least five cysts (ADPKD), 28 children were classified as suspicious (SADPKD), and 103 children had no renal involvement (NADPKD) detected by ultrasound. Seventy-four percent of the ADPKD children had signs or symptoms compatible with the diagnosis of ADPKD, compared to 34% of the NADPKD and 36% of the SADPKD children (both P less than 0.05). Three of the 23 ADPKD children had elevated serum creatinines at the time of diagnosis, while all of the NADPKD and SADPKD children had normal renal function. Renal area by ultrasonography (width X depth) was greater among the ADPKD children compared to the SADPKD and NADPKD groups (P less than 0.05). On follow-up 30 of the 37 NADPKD children remained NADPKD, three were reclassified as SADPKD, and four progressed to ADPKD after 18 years of age. All of the NADPKD children had normal renal function on follow-up. Overall, 14 children had suspicious ultrasounds at some point with follow-up ultrasonography and ten (71%) progressed to ADPKD. All SADPKD children maintained normal renal function. Eight of 18 ADPKD children had progression of the disease manifested by development of hypertension and/or decreased renal function. Three children progressed to end-stage renal disease. Five ADPKD children were diagnosed before one year of age, two of them via prenatal ultrasonography. One fetus was aborted after documentation of oligohydramnios.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Autosomal dominant polycystic kidney disease in childhood: a longitudinal study. 329 57

Since the introduction of new non-invasive diagnostic techniques such as abdominal ultrasound and computerized tomography, simple renal cysts are diagnosed with increasing frequency. Over 30% of patients over 50 years of age are found to have simple renal cysts of different size. A cystic renal mass may represent a simple renal cyst without clinical relevance, a cystic renal carcinoma, early evidence of polycystic kidney disease in a young patient, a rare cause of renal hypertension, a source of infection in a symptomatic patient (infected renal cyst), or a manifestation of an infectious disease (renal abscess, echinococcus cyst). The differential diagnosis and management of a cystic renal mass therefore remain a clinical problem. In the past, surgical exploration of a cystic renal mass was frequently performed. Today, modern diagnostic techniques such as ultrasound-guided percutaneous cyst puncture with cytological analysis of the cyst content, or computerized tomography, are considered the methods of choice. They are particularly useful in case of doubt about the dignity of a cystic renal mass. The determination of renal venous renin levels may be useful in differentiating the causal role of a renal cyst in a patient with hypertension. The management of a cystic renal mass depends on the underlying disease.
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PMID:[An incidental finding of renal cysts: routine occurrence or a finding deserving clarification?]. 329 67


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