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Autosomal dominant polycystic kidney disease (ADPKD) is a frequent cause of ESRD, but its frequency in blacks has not been well delineated and its course and the effects of sickle hemoglobin in this disease in blacks have not been previously reported. The occurrence of ADPKD in blacks and whites was determined in two ESRD populations: all ESRD patients seen over a 16-yr period in one area of Southeast Tennessee and all ESRD patients in 15 hemodialysis units in Tennessee and Atlanta, GA. The frequency of sickle hemoglobin was determined and compared in a group of nonrelated blacks with ESRD with and without ADPKD. The age at onset of ESRD and factors that might affect ADPKD such as gender, hypertension, and hemoglobin type were examined. ADPKD was a less frequent cause of ESRD in blacks than whites (1.4 versus 6.8%). However, after adjusting for the population rate, the incidence rates in blacks and whites were similar (0.48 and 0.47 of 100,000). There was a higher incidence of sickle hemoglobin in nonrelated blacks with ADPKD versus other black ESRD patients (50 versus 7.5%; P < 0.005). Blacks had an earlier onset of ESRD than whites (43.2 versus 55.4 yr; P < 0.0001), as did blacks with sickle-cell trait versus blacks without (38.2 versus 48.1 yr; P < 0.003). In this population, hypertension and gender had no effect on the onset of ESRD. ADPKD accounted for a smaller percentage of blacks than whites with ESRD because of the high percentage of blacks with renal disease from other causes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Autosomal dominant polycystic kidney disease in blacks: clinical course and effects of sickle-cell hemoglobin. 801 76

Autosomal dominant polycystic kidney disease (ADPKD) is a common systemic genetic disease which comprises 8 to 10% of patients treated by dialysis and transplantation. Breakthroughs in molecular genetics and cell biology have led to new insights into cyst formation and growth. Until the specific genetic defects are identified, the management of this disorder will necessarily be empiric. This paper discusses current management strategies in ADPKD focusing on hypertension, hematuria, pain and infection. Special considerations for management of end-stage renal failure in patients with ADPKD are also reviewed.
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PMID:Clinical management of autosomal dominant polycystic kidney disease. 836 Nov 35

Autosomal dominant polycystic kidney disease (ADPKD) is an important cause of medical morbidity in the United States that affects one-half million persons and accounts for ESRD in about 10% of the chronic dialysis population. In addition to its effects on the kidney, the disease has important manifestations in the cardiovascular system (aneurysms, hypertension) and the gastrointestinal tract (hepatic cysts). Clinically important renal complications can develop as the disease progresses that require specialized attention, such as urinary tract infection, pain, and nephrolithiasis. The underlying cellular defect that causes ADPKD has eluded investigators thus far, but abnormalities in cellular proliferation, the tubular basement membrane, and cell fluid secretion appear important in pathogenesis. Factors that mediate progressive interstitial fibrosis and failure of renal function are undefined, although rigorous control of blood pressure appears to be an important therapeutic measure. Recent advances in molecular biology have localized the abnormal gene to chromosome 16 in 90% of families, making early genetic screening of asymptomatic family members possible in many cases. A positive diagnosis may have important effects on employment status, as well as health insurance, so that family members sometimes refuse to be assessed for the presence of the disease. Because of such complex social factors, counseling of an asymptomatic individual by his or her physician is required when considering the use of screening tests for ADPKD. Inadequate patient education may still represent an impediment to early detection, genetic counseling, and timely treatment of disease complications.
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PMID:Autosomal dominant polycystic kidney disease. 849 Jan 15

Autosomal dominant polycystic kidney disease is one of the most commonly inherited diseases in the United States. It affects nearly 500,000 Americans and accounts for 5 to 10 percent of patients with end-stage renal disease. Diagnosis is usually made in middle age, when complications such as hypertension, pain and hematuria develop. Renal complications include hypertension, cyst infection and hemorrhage, hematuria and flank pain. Other manifestations and related conditions include polycystic liver disease, cerebral aneurysm, cardiac valve abnormalities and diverticulosis. The severity and course of the disease vary in individual patients. Management involves the control of hypertension and treatment of complications. Genetic counseling is important. Dialysis and renal transplantation often are successful treatments in patients who develop renal failure.
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PMID:Autosomal dominant polycystic kidney disease. 859 59

Autosomal dominant polycystic kidney disease is a relatively common familial disorder which frequently affects multiple organ systems. The condition is associated with hypertension, cardiac valvular abnormalities, cerebral berry aneurysms, and chronic renal failure. We describe an aviator with a long history of hypertension that was found to be due to autosomal dominant polycystic kidney disease. Following an extensive evaluation, he was found capable of continuation of flying duties. The epidemiology, pathogenesis, diagnosis, evaluation, and aeromedical implications of autosomal dominant polycystic kidney disease in an aviator are discussed.
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PMID:Autosomal dominant polycystic kidney disease and hypertension in the aviator. 968 84

The present study concerns 63 patients with autosomal polycystic kidney disease. In 35 patients the disease was linked to chromosome 16, and in the remaining 28 it was not linked to this chromosome. A comparative clinical analysis of the clinical manifestations of each of the forms was carried out using the objective criteria of age at onset of the initial clinical manifestations of ADPKD, age at onset of the hypertension syndrome, age at onset of the initial chronic renal failure and beginning of haemodialysis. The results show that the clinical manifestations of ADPKD in the patients with the unlinked from are milder and with a better prognosis.
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PMID:Comparative analysis of clinical manifestations of autosomal dominant polycystic kidney disease depending on linkage to chromosome 16. 900 54

Among all inherited cystic kidney diseases, the commonest are polycystic kidney diseases, which include 2 diseases characterized by their pathological characteristics and their mode of inheritance, namely autosomal dominant or recessive. Autosomal dominant polycystic kidney disease is usually diagnosed in adulthood and is related at least to 2 different genes; PKD1 gene on chromosome 16 accounts for 85% of cases. This frequent disease (1 in 1,000 people) leads to end-stage renal failure in most patients at a mean age of 55 years. Renal ultrasonography allows its detection at an early stage, during childhood or adolescence, and even in utero in some cases. Autosomal recessive polycystic kidney disease, related to a single gene mapped to chromosome 6, is a rare disease, usually diagnosed during infancy because of enlarged kidneys and hypertension. The early occurrence of advanced renal failure is uncommon and only 1/3 of patients require renal replacement therapy during childhood. The term "polycystic kidney disease" should be limited to these 2 diseases; however there are many other inherited conditions including renal cysts like tuberous sclerosis or Hippel-Lindau's disease in adults, and several malformative syndromes in children.
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PMID:[Cystic kidney diseases]. 936 10

Inherited kidney diseases are frequently encountered in adults; the diagnosis is often made and they usually progress to renal failure at this age. Autosomal dominant polycystic kidney disease is the most prevalent. It is one of the most common inherited diseases, involving 1 in 400 to 1,000 individuals. Renal cysts growth is responsible for hypertension and renal failure; polycystic kidney disease represents 6 to 7% of the causes of end-stage renal failure in adults. The disease also encompasses extra-renal localisations, i.e. liver cysts and intra-cranial aneurysms. Multiple renal cysts may be found in other inherited disorders, such as tuberons sclerosis and von Hippel-Lindau disease. Alport syndrome is the second most prevalent inherited kidney disease, characterized by various abnormalities of type IV collagen molecules. Molecular diagnosis is possible in some families, which makes genetic counselling more reliable. Finally renal involvement is frequent in a great variety of inherited metabolic (Fabry's disease, glycogen storage disease type 1, hyperuricemic nephropathy) or non-metabolic (nail-patella or Bardet-Biedl syndrome) diseases.
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PMID:[Hereditary kidney diseases in adults]. 936 16

Autosomal dominant polycystic kidney disease (ADPKD) progresses to end-stage renal insufficiency before the age of 73 in approximately 48% of affected individuals. Why the disease, characterized by innumerable cysts arising in proximal and distal tubules, eliminates functioning non-cystic parenchyma in some patients and spares other is a mystery. The cysts initiate in early childhood in fewer than 1% of renal tubules as a consequence of the focal expression of mutated DNA. Tubule cells proliferate, causing segmental dilation, in association with the abnormal deposition of extracellular matrix proteins. Most of the cysts separate from the parent tubules and fill with fluid by cAMP-mediated chloride secretion. Risk factors associated with accelerated loss of renal function include: genotype (PKD Type 1 progresses more rapidly than PKD Type 2); gender (males progress more rapidly than females); race (black patients progress more rapidly than whites); hypertension; proteinuria. The relation between kidney size and progression to renal failure is debated. Progressive PKD is associated with the cellular expression of proto-oncogenes (fos, myc, ras, erb), growth factors (EGF, HGF, acid and basic FGF), chemokines (MCP-1. osteopontin), metalloproteinases, and apoptotic markers, and the interstitial accumulation of Types I and IV collagen, laminin, fibronectin, macrophages and fibroblasts, the magnitudes of which increase with age. Cyst activating factor (CAF), a neutral lipid identified in cyst fluid that stimulates fluid secretion and proliferation of renal epithelial cells and monocyte chemotaxis, has recently been identified as a potential progression factor. In those patients destined to develop renal failure there is loss of non-cystic parenchyma in association with mass replacement by fluid-filled cysts in a network of interstitial fibrosis. The decline in renal function is probably the consequence of processes leading to interstitial fibrosis, as in other nephropathies, rather than due to simple mechanical displacement of parenchyma by cysts.
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PMID:Mechanisms of progression in autosomal dominant polycystic kidney disease. 940 32

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary disease. Genetic molecular methods can make the diagnosis of at least three different types of ADPKD. ADPKD concerns young people and complications such as hypertension and decreased renal function occur more frequently if onset is early, if it is a type 1, and if the patient is a woman. The pregnant woman with autosomal dominant cystic disease is at particular high risk of obstetrical complications. Prenatal diagnosis is possible.
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PMID:[Autosomal dominant polycystic kidney. Apropos of 2 cases. Review of the literature]. 941 67


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