Extractions: polycystic kidney disease By Adam Brochert, MD Images (click to view larger image) Polycystic kidney disease (PKD) is an inherited condition that results in abnormally formed kidneys. It also causes other abnormalities throughout the body. What is going on in the body? Polycystic kidney disease has three forms: infantile, childhood, and adult. The forms are named this way because of the time during a person's life when symptoms begin. The earlier symptoms begin, the more severe the disease usually is. All three forms are inherited from either one or both parents, depending on the type of PKD. Polycystic kidney disease results in malformed kidneys, which are filled with small sacs, or cysts, instead of normal kidney tissue. This leads to kidney problems including kidney failure, which can lead to death. Abnormalities in other areas of the body may also occur. What are the signs and symptoms of the disease? Signs and symptoms depend on the type of polycystic kidney disease. Children with the infantile form of PKD often have very large, malformed kidneys at birth. These children are usually critically ill because their lungs don't development correctly due to the PKD. Most affected children die in the first few days of life. Children with the childhood form of PKD usually get symptoms before adulthood. They often have kidney failure which causes:
FamilyFun: Health Encyclopedia: Polycystic Kidney Disease An excerpt on polycystic kidney disease from the Disney Encyclopediaof Baby and Child Care. polycystic kidney DISEASE, This hereditary http://familyfun.go.com/raisingkids/child/health/childhealth/dony79enc_kidn/
Extractions: This hereditary kidney disorder occurs in two main forms. The more common form, known as adult polycystic disease (APCD), usually develops in adulthood but may, in rare cases, manifest itself in infancy or childhood. Much less common is infantile polycystic disease (IPCD), which is usually apparent soon after birth and is accompanied by liver abnormalities. In both forms of the disease, the kidney is riddled with fluid-filled growths called cysts. These cysts, which usually appear in both kidneys, severely impair kidney functioning. In the infantile form of the disease, the kidneys may be either enlarged or underdeveloped. Some infants also are born with a noninherited cystic kidney disorder called multicystic kidney of the newborn. This disorder, which usually affects only one kidney, occurs when the ureter (the tube to the bladder) develops abnormally, causing the kidney to degenerate into an irregular mass of cysts.
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Medicine, Ob/Gyn, Psychiatry, and Surgery Nephrology Last Updated: March 12, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: PKD, autosomal dominant polycystic kidney disease, ADPKD, adult polycystic kidney disease, polycystic kidney disease type 1, PKD1, polycystic kidney disease type 2, PKD2, polycystic kidney disease type 3, PKD3 AUTHOR INFORMATION Section 1 of 11 Author Information Introduction Clinical Differentials ... Bibliography Author: Roser Torra, MD, PhD , Consulting Staff, Hereditary Renal Diseases, Department of Nephrology, Fundacio Puigvert, Barcelona, Spain Editor(s): Laura Lyngby Mulloy, DO , Chair, Associate Professor, Department of Internal Medicine, Division of Nephrology, Hypertension and Transplantation, Medical College of Georgia; Francisco Talavera, PharmD, PhD , Senior Pharmacy Editor, Pharmacy, eMedicine;
Extractions: (advertisement) Home Specialties CME PDA ... Patient Education Articles Images CME Patient Education Advanced Search Link to this site Back to: eMedicine Specialties Radiology Genitourinary Last Updated: September 20, 2002 Rate this Article Email to a Colleague Synonyms and related keywords: Potter type III disease, adult polycystic kidney disease, kidney disease, polycystic kidney disease, renal cyst, ADPKD, ADPCKD, renal failure, hypertension, impaired renal function, AUTHOR INFORMATION Section 1 of 12 Author Information Introduction Differentials X-ray ... Bibliography Author: Ali Nawaz Khan, FRCP, FRCR , Lecturer, Department of Diagnostic Radiology, Faculty of Medicine, University of Manchester Coauthor(s): Muthusamy Chandramohan, FRCR , Special Registrar, Department of Radiology, Manchester Radiology; Sumaira Macdonald, MRCP, FRCR , Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute Ali Nawaz Khan, FRCP, FRCR, is a member of the following medical societies: American Institute of Ultrasound in Medicine British Institute of Radiology British Medical Association Radiological Society of North America ... Royal College of Radiologists , and Royal College of Surgeons of England Editor(s): John L Haddad, MD
Polycystic Kidney Disease (PKD) - Lucile Packard Children's Hospital polycystic kidney Disease (PKD) What is polycystic kidney disease (PKD)? Autosomaldominant PKD is often called the adult polycystic kidney disease. http://www.lpch.org/DiseaseHealthInfo/HealthLibrary/urology/pkd.html
Extractions: What is polycystic kidney disease (PKD)? Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts filled with fluid in the kidneys. PKD cysts can reduce kidney function, leading to kidney failure. PKD can also cause cysts in the liver and problems in other organs, such as the heart and blood vessels in the brain. PKD is the fourth leading cause of kidney failure and affects approximately 500,000 people in the US. According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), about one-half of people with autosomal dominant PKD progress to kidney failure, or end-stage renal disease (ESRD). What are the different types of PKD? There are two primary inherited forms of PKD and one non-inherited form: autosomal dominant PKD (inherited) This is the most common inherited form of polycystic kidney disease, accounting for about 90 percent of all PKD cases. "Autosomal dominant" means that if one parent has the disease, there is a 50 percent chance that the disease will pass to a child, and that both males and females are equally affected. Usually, at least one parent must have the disease for a child to inherit it. In 25 percent of cases, there is no family history of PKD. These cases are new mutations in a family. Parents would not be at increased risk to have additional children with PKD, but the child with PKD would have a 50/50 chance to pass the gene on to their children.
Polycystic Kidney Diseases polycystic kidney Diseases. polycystic kidney disease (PKD) is an inherited disorderthat causes many fluidfilled sacs, called cysts, to form in both kidneys. http://rarediseases.about.com/library/weekly/aa030902a.htm
Extractions: Polycystic kidney disease (PKD) is an inherited disorder that causes many fluid-filled sacs, called cysts, to form in both kidneys. The cysts cause problems because they interfere with the function of the kidney and damage the kidney tissues. The cysts can grow very big (the size of a grapefruit, for example) and/or there can be hundreds, even thousands, of cysts. PKD can also cause cysts to form in the liver, the heart, or blood vessels in the brain.
Medic-Planet Polycystic Kidney Disease polycystic kidney disease See also Bladder infections, Genetic diseases,Kidney diseases, Kidneys, Back to the Index page Polycystic http://www.medic-planet.com/MP_article/internal_reference/Polycystic_kidney_dise
Medic-Planet Polycystic Kidney Disease polycystic kidney disease See also Bladder infections, Genetic diseases,Kidney diseases, Kidneys, Back to the Index page polycystic http://www.medic-planet.com/MP_article/internal_reference/polycystic_kidney_dise
Extractions: The clinical and histopathologic variability of this common genetic disordera leading cause of renal failurecannot be explained by inherited mutation. Instead, the disease evidently progresses by a second hit: somatic mutation superimposed on germline mutation. The source of the mutability appears to be DNA triple-helixing, as mediated by some odd genetic code, the longest polypyrimidine tract ever found in the human genome. Dr. Germino is Assistant Professor of Medicine and Irving Blum Scholar, Johns Hopkins School of Medicine, Baltimore. Throughout the 20th century, the chief epidemiologic impression of polycystic kidney disease (PKD) has been what an early observer described as its outstandingly hereditary character. The autosomal dominant form of the disease is indeed among the most common genetic disorders, with an incidence of roughly one person in every thousand. Its penetrance, moreover, is almost 100%: Virtually every person born with a disease-related mutation eventually shows manifestations of cystogenesis. Evidently beginning in utero and continuing throughout the patient's life, renal cysts increase in size and number, with associated compression of normal renal parenchyma. Typically, significant loss of renal function does not emerge until a patient's 30s or 40s, but in about half of patients, renal failure develops by age 50. Overall, the disease accounts for as much as 5% of all cases of chronic renal failure. In as many as half of patients, end-stage renal disease is manifest by age 60.
Polycystic Kidney Disease polycystic kidney Disease. Cerebral artery aneurysms have also beenassociated with the dominant form of polycystic kidney disease. http://www.kidneyindia.com/polycystic_kid.htm
Extractions: Acute Tubular Necrosis Acute Nephritis Alport Syndrome Analgesic Nephropathy ... Minimal Change Disease MultipleMyeloma Kidney Nephrotic Syndrome Neurogenic Bladder Polycystic Kidney Disease Prostate Disease Prostate Cancer Primary Hyperoxaluria Pyelonephritis Renal Tubular Acidosis Renal Vein Thrombosis RPGN Radiation Kidney SLE/Lupus Nephritis Sexual dysfuctions Transplant Drugs Urinary Incontinence Urinary Tract Infection Uric Acid Kidney Vesicoureteral Reflux Vasculitis Wilms Tumor Polycystic Kidney Disease The overall incidence of polycystic kidney disease in the pediatric population is unknown. Autopsy series have identified polycystic kidneys in 1.3 - 5.9/1000 pediatric patients. Autosomal dominant polycystic kidney disease is far more common than the recessive form. The incidence of the dominant condition in patients of all ages has been estimated from autopsy studies at 1/220 to 1/1100. As anticipated with autosomal inheritance, the male:female ratio of both disorders is approximately 1:1.
POLYCYSTIC KIDNEY DISEASE Columbia University College of P S Complete Home Medical Guide. CPMCnet.Kidney and Urinary Tract Disorders polycystic kidney DISEASE. http://cpmcnet.columbia.edu/texts/guide/hmg27_0005.html
Extractions: Polycystic kidney disease is a hereditary condition in which numerous cysts form on individual nephrons and interfere with kidney function. Although it is not unusual to have one or two cysts within kidney tissue, in most cases they have no function and are harmless (although they may cause confusion and lead to unnecessary diagnostic procedures). In polycystic kidney disease, virtually all the nephrons of both kidneys eventually become cystic or are compressed, distorted, and rendered increasingly ineffective by the pressure of adjacent cysts. In the process, the kidneys enlarge to three or four times normal size as function decreases. A person suffering this condition is unaware of the disease unless some complication— hypertension, blood in the urine, pain caused by bleeding into the kidney, a stone, or infection—calls attention to the kidney, usually long before kidney failure has developed. Eventually, the kidneys grow so large that it is possible to feel them through the skin. Although some patients with polycystic kidney live a normal life span and a significant group live long enough to die of something else, until the advent of dialysis and transplantation, many patients with the disease eventually died of uremic poisoning. While some people who inherit the responsible genes do not encounter significant kidney damage from cysts until their 30s or 40s, others live into their 80s; the average age at which renal failure occurs is 50. Consequently, for carriers of these genes, choosing whether to have children is a difficult decision, but genetic counseling may be helpful.
Renal Cystic Disease Images available as described below range in file size from 50 to 250k. Recessivepolycystic kidney Disease (RPKD). Dominant polycystic kidney Disease (DPKD). http://medlib.med.utah.edu/WebPath/TUTORIAL/RENCYST/RENCYST.html
Extractions: Return to the tutorial menu. Images available as described below range in file size from 50 to 250k. This condition is inherited in an autosomal recessive pattern, giving a 25% recurrence risk for parents having subsequent children. The kidneys are affected bilaterally, so that in utero, there is typically oligohydramnios because of poor renal function and failure to form significant amounts of fetal urine. The most significant result from oligohydramnios is pulmonary hypoplasia, so that newborns do not have sufficient lung capacity to survive, irrespective of any attempt to treat renal failure. RPKD may be termed "Type I" cystic disease in the Potter's classification. Grossly, the kidneys are markedly enlarged and tend to fill the retroperitoneum and displace abdominal contents. The kidneys tend to be symmetrically enlarged. The cysts are quite small and uniform, perhaps 1 to 2 mm on average. Microscopically, the characteristic finding in the later third trimester is cystic change with the cysts elongated and radially arranged. The few remaining glomeruli are not involved by the cysts, and the intervening parenchyma is not increased. In the second trimester, the cysts may not be as well-developed. A helpful finding at autopsy is the presence of congenital hepatic fibrosis, which accompanies RPKD. Normal fetal kidneys, gross
Polycystic Kidney Disease polycystic kidney Disease. (1996) A role for CFTR in human autosomal dominantpolycystic kidney disease. Am.J.Physiol. 270 (Cell Physiol.39)389399. http://www.guggino.org/Polycystic_Kidney_Disease.htm
Extractions: Ion Channels Cystic Fibrosis Gene Therapy Polycystic Kidney Disease Epithelial Cell Biology Polycystic Kidney Disease The laboratory is involved in the study the two genes (PKD1 and PKD2) which are implicated in the disease. Research techniques employed include electrophysiology and confocal microscopy. In addition primary culture studies from patients is underway. Select Publications Hanaoka, K., DeVuyst, O., Schwiebert, E.M., Wilson, P., Guggino, W.B . (1996) A role for CFTR in human autosomal dominant polycystic kidney disease. Am.J.Physiol. Cell Physiol.
Extractions: Polycystic kidney disease (PKD) is the commonest inherited cause of kidney failure in adults. It is genetically acquired and has two forms - the dominant and the recessive form. Most adults with PKD have the dominant form. Recessive form causes problems much earlier (during childhood) and life expectancy is usually not beyond the teenage years. In the dominant PKD form, one parent must have the disease and the chance of passing the gene to the offspring is 50%. There are instances of spontaneous genetic mutations whereby the parents do not carry the flawed gene but the offspring is still afflicted with the condition. This genetic flaw causes the development of multiple cysts to evolve from the nephrons. Cysts are abnormal pouches containing fluid. Eventually the cysts replace normal kidney tissue and the person with this condition suffers end stage kidney failure. Cysts also develop in the liver and pancreas. Signs and symptoms: Dull pain at the side of the abdomen (flank) and back Blood in the urine
Polycystic Kidney Disease Urology. polycystic kidney Disease (PKD). What is polycystic kidney disease(PKD)? polycystic kidney disease (PKD) is a genetic disorder http://www.mcghealthcare.org/urology/polycyst/polycyst.htm
Extractions: Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts filled with fluid in the kidneys. PKD cysts can reduce kidney function, leading to kidney failure. PKD can also cause cysts in the liver and problems in other organs, such as the heart and blood vessels in the brain. PKD is the fourth leading cause of kidney failure and affects approximately 500,00 people in the US. According to the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), about one-half of people with autosomal dominant PKD progress to kidney failure, or end-stage renal disease (ESRD). What are the different types of PKD?
Polycystic Kidney Disease polycystic kidney Disease. Inheritance, autosomal dominant. polycystic kidneyDisease (PKD) is an ideal way to introduce your students to transplants. http://www.usoe.k12.ut.us/curr/Science/core/bio/genetics/polycystic_kidney.htm
Extractions: Polycystic Kidney Disease Inheritance autosomal dominant Occurrence about 600,000 cases in the United States Description a disease which causes cysts to grow on a person's kidneys (and liver); the third leading cause of kidney failure in the United States Testing there is testing available through ultrasound and magnetic resonance imaging Treatment there is currently no cure or effective treatment for PKD; half of all patients develop kidney failure and need either constant dialysis or transplantation Organ Transplants Background: This is also a very relevant activity since most states identify organ transplant donors when an individual receives his or her driver license. Many students will be making these decisions in the near future (or have already made these decisions). Activity: Here are some ideas for students to research. There is a great deal of information available on the Internet. If your school has a computer lab, this would be an ideal topic to have students research on the Internet in groups. Here are some possible topics. (1) Which organs can be transplanted?
