Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0016199 (flank pain)
 2,189 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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 (ADPKD) is often characterized by end-stage renal disease (ESRD) and problems including pain, hematuria, and infection. Open nephrectomy is curative; however, the morbidity of the procedure is considerable. Between 1995 and 1998, 11 laparoscopic nephrectomies were performed on nine symptomatic patients (five men and four women) with ESRD and ADPKD. Two patients underwent a staged bilateral laparoscopic nephrectomy. All patients presented with abdominal or flank pain and an abdominal mass. Other clinical problems included hypertension in eight patients, urinary tract infections in two patients, and gross hematuria in one patient. Seven patients were receiving long-term dialysis treatment, and two patients had undergone prior renal transplantation. Patients were evaluated for preoperative and postoperative pain, analgesic use, hospital course, and convalescence. The overall average operative time was 6.3 hours, with an average estimated blood loss of 153 mL. Eight nephrectomy specimens were removed by morcellation, and three specimens were removed intact through a 7- to 12-cm incision. The average hospital stay was 3 days, and the average time to normal activity was 5 weeks. With a mean follow-up of 31 months, all nine patients reported elimination of their preoperative pain based on a pain analogue score. Six major and two minor complications occurred, including blood transfusion, a vena cavotomy, splenic cyanosis, pulmonary embolism, clotted arteriovenous fistula, and brachial plexus injury. Incisional hernias occurred in two of the three patients who underwent open removal. One patient noted improvement, and two patients noted resolution of their hypertension postoperatively. Laparoscopic nephrectomy in patients with ADPKD and ESRD offers an effective alternative to open nephrectomy to manage renal-related pain. This procedure provides the benefits of minimal intraoperative blood loss, minimal postoperative pain, brief hospital stay, and rapid convalescence.
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PMID:Laparoscopic nephrectomy in patients with end-stage renal disease and autosomal dominant polycystic kidney disease. 1073 3

The kidney has both afferent (sensory) and efferent (sympathetic) nerves that can influence renal function. Renal innervation has been shown to play a role in the pathogenesis of many forms of hypertension. Hypertension and flank pain are common clinical manifestations of autosomal dominant (AD) polycystic kidney disease (PKD). We hypothesize that renal innervation contributes to the hypertension and progression of cystic change in rodent PKD. In the present study, the contribution of renal innervation to hypertension and progression of renal histopathology and dysfunction was assessed in male Han:SPRD-Cy/+ rats with ADPKD. At 4 weeks of age, male offspring from crosses of heterozygotes (Cy/+) were randomized into either 1) bilateral surgical renal denervation, 2) surgical sham denervation control, or 3) nonoperated control groups. A midline laparotomy was performed to allow the renal denervation (i.e., physical stripping of the nerves and painting the artery with phenol/alcohol). Blood pressure (tail cuff method), renal function (BUN) and histology were assessed at 8 weeks of age. Bilateral renal denervation reduced the cystic kidney size, cyst volume density, systolic blood pressure, and improved renal function (BUN) as compared with nonoperated controls. Operated control cystic rats had kidney weights, cyst volume densities, systolic blood pressures, and plasma BUN levels that were intermediate between those in the denervated animals and the nonoperated controls. The denervated group had a reduced systolic blood pressure compared with the operated control animals, indicating that the renal innervations was a major contributor to the hypertension in this model of ADPKD. Renal denervation was efficacious in reducing some pathology, including hypertension, renal enlargement, and cystic pathology. However, sham operation also affected the cystic disease but to a lesser extent. We hypothesize that the amelioration of hypertension in Cy/+ rats was due to the effects of renal denervation on the renin angiotensin system.
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PMID:Contribution of renal innervation to hypertension in rat autosomal dominant polycystic kidney disease. 1848 Apr 17

Autosomal dominant polycystic kidney disease (ADPKD) is not well described in black Africans while some data suggesting the disease is exceptional in this race. A retrospective study of patients with ADPKD followed in nephrology department of a teaching hospital in Dakar (January 1, 1995 to December 31, 2005) was therefore undertaken. Prevalence of ADPKD was one in 250. Mean age was 47 + or - 5 years with a predominance of male (57%). High blood pressure (HBP) was present in 68% of patients. Other renal manifestations were flank pain, hematuria and proteinuria. Majority of patients had impaired renal function at time of diagnosis. Extra-renal cysts were essentially found in liver (45.5%), pancreas and seminal vesicles. Main complications: ESRD (51%) occurred within a 6 year mean period, urinary tract infection (13%) and cerebral haemorrhage (2%). HBP control, in general needed 2 or more antihypertensive drugs. Fourteen patients died, ten patients had been on haemodialysis and four others died from uremic complications. In conclusion, ADPKD in black African adults is not rare and probably underdiagnosed. Early HBP and ESRD are likely more frequent than in other races. Earlier ultrasound detection and strategies to preserve renal function should be offered to at-risk individuals to improve outcomes.
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PMID:Patterns of autosomal dominant polycystic kidney diseases in black Africans. 2006 98

A 40-year-old Japanese man was admitted to our hospital for investigation of fever and right flank pain starting after watery diarrhea. Salmonella enteritidis was cultured from samples of stool and blood. Although his diarrhea subsided soon, high fever persisted and flank pain got worse. Magnetic resonance imaging (MRI) revealed four areas of abnormal intensity in renal cysts and computed tomography showed the same findings. Renal cysts infection was diagnosed. After ultrasound-guided aspiration of these cysts, his fever subsided. Culture of each aspirate grew Salmonella enteritidis. Although the route of cyst infection in ADPKD generally remains unclear, the clinical course of our patient may indicate that gastrointestinal tract infection progressed to renal cyst infection when bacteremia occurred due to bacterial translocation.
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PMID:The renal cyst infection caused by Salmonella enteritidis in a patient with autosomal dominant polycystic kidney disease: how did this pathogen come into the renal cysts? 2112 36

Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD. Patients with ADPKD have cysts and renal enlargement. Most patients present with hypertension, hematuria or flank pain; the most common extrarenal manifestation is polycystic liver disease. Oligohydramnios, bilaterally enlarged kidneys and decreased urine are featured in utero in ARPKD. Medullary sponge kidney is uncommon and features nephrocalcinosis, recurrent calcium stones and a history of polyuria/nocturia and/or urinary tract infections. Alport syndrome (AS) is an inherited disease of the glomerular basement membrane that is usually inherited as an X-linked dominant trait. Most patients with AS present in the first two decades of life with persistent microscopic or gross hematuria. Later, proteinuria is seen and its presence portends disease progression. Other findings may include sensorineural hearing loss and ocular abnormalities. There are various inherited tubulopathies, including Bartter syndrome, a group of renal tubular disorders that consist of two phenotypes with four genotypes. Patients usually present early in life with salt wasting, hypokalemia and metabolic alkalosis. Other features, depending on genotype, may include polyhydramnios and premature birth. Gitelman syndrome is also a salt-losing tubulopathy characterized by hypokalemic alkalosis. The majority of patients with Gitelman syndrome present during adolescence or early adulthood.
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PMID:Inherited renal diseases. 2508 62

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of kidney disease. Enlarging cysts within the kidneys are the clinical hallmark of the disease. Renal manifestations include varying degrees of kidney injury, urinary tract infections, kidney stones, and hematuria. Extrarenal manifestations can include pain, hypertension, left ventricular hypertrophy, hepatic cysts, intracranial aneurysm, diverticulosis, and abdominal and inguinal hernias. The progression of ADPKD cannot be reversed with current treatment modalities; therefore, therapies target the resulting clinical manifestations. Early detection and management of hypertension are important to delay the progression of renal dysfunction and development of cardiovascular complications. Pain management includes evaluation of concomitant illnesses, use of analgesics, and adjuvant therapy. Fluoroquinolones may be the most useful class of antibiotics for the treatment of urinary tract infections because of their lipophilic properties and bactericidal action against gram-negative pathogens. Nephrolithiasis is twice as common in persons with ADPKD compared with the general population and is suggested by flank pain with or without hematuria. Cystic hemorrhages usually resolve within one week, although microscopic hematuria may still be present. Because of the proliferative effect of estrogen on hepatic cysts, oral contraceptives containing estrogen and menopausal estrogen therapy should be administered at the lowest effective dose or avoided in patients with ADPKD. Intracranial aneurysms are at least twice as common in patients with ADPKD than in the general population. Renal ultrasonography is the diagnostic modality of choice to screen at-risk individuals for ADPKD.
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PMID:Autosomal dominant polycystic kidney disease. 2722 67

Background: Autosomal dominant polycystic kidney disease is the most prevalent hereditary renal disease, associated with progressive renal insufficiency, usually leading to dialysis. It is rarely diagnosed with other renal abnormalities. We present a case of a 35-year-old woman with a duplicated left polycystic kidney, who had recurrent pain and pyelonephritis because of ureteropelvic junction (UPJ) obstruction of the upper moiety. Case Presentation: A 35-year-old female patient initially presented with left flank pain for 7 days. Evaluation demonstrated enlarged bilateral polycystic kidneys with the appearance of a duplicated system of the left kidney and UPJ obstruction of the upper moeity. She underwent endoscopic management, including balloon dilatation and stent placement. After stent removal she had no symptoms, and ultrasonography showed resolution of the upper pole hydronephrosis. Conclusion: Minimally invasive nephron sparing approaches for UPJ obstruction could delay the process of end-stage renal disease development in polycystic kidney disease patients who have additional congenital renal anomalies. Balloon dilatation should be considered as a feasible therapy for UPJ obstruction in polycystic kidney disease patients with duplicated systems.
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PMID:Ureteropelvic Junction Obstruction in a Polycystic Kidney with Duplicated System: Successful Outcome with Endoscopic Management. 3277 45