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

Autosomal dominant polycystic kidney disease affect adults starting the 5th decade of life. It is caused by at least two different gene defects, one gene being located on the short arm of chromosome 16. It's more frequent at women and clinically, near renal cysts, appear others systemic manifestations as hepatic cysts, intracranial aneurysms, cardiac valvular lesions or diverticula. The diagnosis is set up on urography and ultrasonography and the treatment attempt to slow down the evolution to renal failure and to prevent the complications as infections, obstructions and hypertension, that may aggravate the prognosis. Lately there are discussions about a preventive treatment consisting in genetic counseling.
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PMID:[Polycystic kidney disease with autosomal dominant transmission]. 130 23

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disorder leading to terminal renal failure. About 8% of the dialysis patients suffer from ADPKD, the gene frequency in the general population being about 1:1000. Many facts contribute to the hypothesis that arterial hypertension plays a major role in the pathophysiology of ADPKD. We observed a prevalence of 30% of hypertension in patients with ADPKD and normal serum-creatinine, and of 80% in patients with terminal renal failure. The time of onset of abnormalities of blood pressure regulation is of great interest, since an increase of blood pressure, even in the normotensive range, accelerates the rate of progression. To answer this question, we examined the time of onset of abnormalities in blood-pressure regulation in 23 probands and 23 control patients in a cross-sectional study. The results document abnormal circadian blood-pressure changes and higher blood pressures, although still within the normotensive range, in asymptomatic carriers of the ADPKD-trait, even before and more definitely after onset of puberty. Even at an age when circadian blood pressure is not significantly different, there is an increased LVM as evidence of target organ damage. The findings suggest that (intermittent) increases in blood pressure and blood-pressure-dependent target organ damage precede overt hypertension and renal failure by years or decades.
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PMID:[Ambulatory 24-hour blood pressure monitoring of children and young adults with autosome dominant polycystic kidney degeneration]. 151 6

Autosomal dominant polycystic kidney disease is a common (approximately 1 in 400 individuals in the United States) inherited disorder, in which hypertension is the most often associated disorder. Although the development of hypertension originates with expansion of renal cysts, it most likely has its pathogenesis in the renal vasculature. Evidence is now accumulating that the renin-angiotensin-aldosterone system is important in the development and maintenance of hypertension in this disorder. End-organ damage including left ventricular hypertrophy and progressive renal insufficiency appear to be related to the presence of hypertension in ADPKD. A focus on early detection and adequate control of hypertension in ADPKD, therefore, may be associated with improved cardiovascular and renal outcomes as well as increased patient survival in ADPKD.
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PMID:Pathogenesis of hypertension in autosomal dominant polycystic kidney disease. 176 38

Autosomal dominant polycystic kidney disease (ADPKD) is the commonest hereditary nephropathy. We collected 92 cases in VGH. Diagnosis was confirmed by intravenous pyelogram, renal sonogram, or renal CAT scan. The incidence of having positive family history was just only 28.3%. Patients were diagnosed at the mean age of 54 +/- 11 years (26-74 years). The common clinical findings were hypertension (73.9%), abdominal mass, proteinuria, anemia, azotemia, abdominal or back pain and pyuria in orders. Hypertension might present in the early stage with normal renal function (near 40%). Polycystic liver was the major extrarenal lesion (57.6%), but the incidence of abnormal liver function was only 10.1%. Enlarged kidneys were not always palpable, even at end stage of renal function (mean age 56 +/- 9 years, 89.4% kidney palpable). Patient's urine amount was usually nonoliguric, even in uremic stage (82.9%). Sepsis was the first cause of death. Cardiovascular disease and uremia were followed in sequence. Their expired mean age was 61 +/- 7 years (53-74 years).
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PMID:[Autosomal dominant polycystic kidney disease clinical analysis in VGH--Taipei]. 217 45

Autosomal dominant polycystic kidney disease is the third cause of end-stage chronic renal failure (CRF) requiring dialysis and transplantation. Over a 5-year period we collected 21 cases of that disease. The patients' mean age at the time of diagnosis was 48 years and the sex ratio 1.1. Pain was the most frequent signal symptom, being present in 43 p. 100 of the patients. Varying degrees of renal impairment were found in 61 p. 100 of the cases, and arterial hypertension in 38 p. 100. The diagnosis, suspected on clinical grounds, was confirmed by ultrasonography in 95 p. 100 of the patients. Beside CRF and hypertension, the main complications were microscopic haematuria (38 p. 100) and urinary tract infection (24 p. 100). In two patients the disease was associated with hepatic polycystosis. Treatment was symptomatic for CRF (4 patients were put on periodical haemodialysis) and for the other complications. On the basis of this series, we discuss the profile and prognosis of polycystic kidney disease in our environment, and notably its effects on renal function, and we underline the usefulness of familial investigations and the need for genetic counselling.
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PMID:[Dominant renal polycystic disease]. 252 23

Autosomal dominant polycystic kidney disease (ADPKD) has been shown to be associated with a greater than 50 percent incidence of hypertension prior to deterioration in renal function as assessed by glomerular filtration rate. The present study provides evidence for increased cardiac pre-load, as assessed by plasma atrial natriuretic factor (ANF) and cardiac index, in hypertensive as compared to normotensive ADPKD. The hypertensive ADPKD patients exhibited an increased renal vascular resistance as compared to the normotensive patients in spite of comparable glomerular filtration rates. It is hypothesized that the renal involvement of hypertensive ADPKD patients causes an impaired renal response to the observed increase in cardiac index, and also may release a venoconstrictor (such as angiotensin) which contributes to the enhanced cardiac pre-load and thus the hypertension.
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PMID:Hypertension in autosomal dominant polycystic kidney disease. 297 94

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

Once viewed as hopelessly incurable disorders and the dustbin for careers in academic medicine, the polycystic kidney diseases have emerged as prime targets of pathophysiologic study and palliative and definitive treatment in the era of molecular medicine. Polycystic kidney disease (PKD) may be hereditary or acquired. The major inherited types are autosomal dominant (AD) and autosomal recessive (AR). ADPKD is caused by at least two (and possibly three) genes located on separate chromosomes, while ADPKD-1 is due to a 14 kb transcript in a duplicated region on the short arm of chromosome 16 very near the alpha-globin gene cluster and the gene for one form of tuberous sclerosis. ADPKD-2 has been assigned to the long arm of chromosome 4. ARPKD is due to a mutated gene on both copies of the long arm of chromosome 6. Cysts originate in renal tubules. Proliferation of tubule epithelial cells modulated by endocrine, paracrine, and autocrine factors is a major element in the pathogenesis of renal cystic diseases. In addition, fluid that is abnormally accumulated within the cysts is derived from glomerular filtrate and, to a greater extent, by transepithelial fluid secretion. Abnormal synthesis and degradation of matrix components associated with interstitial inflammation are additional features in the pathogenesis of renal cystic diseases. The ADPKD genotypes are characterized by bilateral kidney cysts, hypertension, hematuria, renal infection, stones, and renal insufficiency. ADPKD is a systemic disorder; cysts appear with decreasing frequency in the kidneys, liver, pancreas, brain, spleen, ovaries, and testis. Cardiac valvular disorders, abdominal and inguinal hernias, and aneurysms of cerebral and coronary arteries and aorta are also associated with ADPKD. Treatment is supportive: dietary regulation of salt and protein intake, control of hypertension and renal stones, and dialysis and transplantation at the end stage. ARPKD is a relatively rare disease that causes clinical symptoms at birth, with significant mortality in the first month of life. The cysts develop primarily in the collecting ducts because of a failure in the maturation process. Early complications include Potter's syndrome; excessive size of the kidneys, causing respiratory dysfunction; hypertension; and renal insufficiency. Hepatic fibrosis is an associated extrarenal problem that results in significant morbidity in young children and adolescents. Treatment includes supportive care, dialysis, and renal transplantation. Acquired cysts (solitary/simple) are commonplace in older persons. Multiple cysts may be seen in association with potassium deficiency, congenital disorders, metabolic diseases, and toxic renal injury.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Polycystic kidney disease: etiology, pathogenesis, and treatment. 758 86

Autosomal dominant polycystic kidney disease is the most common genetic disorder encountered by nephrologists. Clinically relevant areas of which the physician must be knowledgeable are reviewed, emphasizing recent genetic advances with clinical implications for patients and their families. Recent information on hypertension, infection, pain and discomfort, hepatic cysts, and intracranial aneurysms is summarized.
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PMID:Clinical aspects of autosomal dominant polycystic kidney disease. 760 41

Polycystic kidney disease is a rather common genetic disorder, with an estimated amount of 8 to 10% of patients in the dialysis population. Meanwhile the defective gene of autosomal dominant polycystic kidney disease [ADPKD], another common terminus for this disorder, has been localized on the short arm of chromosome 16. The genetic disorder is not strictly localized on the kidney, whereas other organ systems like cardiac valves, brain arteries, liver, colon, etc. may be involved in the disease process. Hypertension is an early and common feature of the disease and its probably an important factor for progression of renal failure in ADPKD. Not all carriers of the ADPKD-trait progress to endstage renal failure, about 50% at the age of 50 years. Patients with ADPKD have a good prognosis in renal replacement therapy programs such as dialysis or renal transplantation.
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PMID:[Cystic kidneys (autosomal dominant polycystic kidney disease)]. 778 92


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