1/238. Isolated polycystic liver disease not linked to polycystic kidney disease 1 and 2.Autosomal dominant polycystic liver disease occurs commonly in association with autosomal dominant polycystic kidney disease, types 1 and 2. It may also exist as a separate entity, genetically distinct from autosomal dominant polycystic kidney disease types 1 and 2, as has been recently established to exist in a Belgian family. We report here a large Argentinian family of Spanish-Belgian ancestry with autosomal dominant polycystic liver disease, where proximal and distal markers for both polycystic kidney disease 1 and 2 failed to demonstrate genetic linkage. The data support the notion that polycystic liver disease and autosomal dominant polycystic kidney disease may have separate chromosomal loci.- - - - - - - - - - ranking = 1keywords = polycystic kidney disease, polycystic kidney, polycystic, kidney disease, kidney (Clic here for more details about this article) |
2/238. Fibropolycystic disease of the hepatobiliary system and kidneys.This complicated case of fibropolycystic disease of the hepatobiliary system and kidneys was ably and incisively analyzed by Professor Sheila Sherlock. Her clinical acumen was revealed by her ability to differentiate congenital hepatic fibrosis, Caroli's disease, and adult polycystic disease of the liver and kidney. Interesting histologic features of this case included hepatic fibrosis with intact limiting plates anc central veins and the presence of bile plugs in the ducts, but the absence of bile statsis in the parenchyma. A percutaneous transhepatic cholangiogram demonstrated the dilated intrahepatic and extrahepatic ducts. Washing out the "gunk" from the biliary tract by T-tube drainage has great limitations in this type of case. Therefore, Dr. Adson suggested irrigation of the biliary ductal system using tubed placed transhepatically, plus a wide choledojejunostomy. Dr. Sherlock questioned this surgical approach. The use of chenodeoxycholic acid for this "gunk" was suggested. In spite of the dilated ducts and pathologic changes in the liver, the patient was not jandiced and did not have stones in her biliary tract. The genetics of this patient's problems was discussed.- - - - - - - - - - ranking = 0.085469887534514keywords = polycystic, kidney (Clic here for more details about this article) |
3/238. Portal hypertension due to extensive hepatic cysts in autosomal dominant polycystic kidney disease.Liver cysts are a well-recognized feature of autosomal dominant polycystic kidney disease (ADPKD) and occur in 77% of patients more than 60 years old. Serious sequelae, however, are rare, the two most common complications being pain and cyst infections. Portal hypertension has been reported in ADPKD due to the rare presence of congenital hepatic fibrosis. We report a case of ADPKD in a patient who had portal hypertension due to distortion of portal vein and venules by extensive hepatic cysts.- - - - - - - - - - ranking = 0.56306580979847keywords = polycystic kidney disease, polycystic kidney, polycystic, kidney disease, kidney (Clic here for more details about this article) |
4/238. Neonatal detection and evaluation of infantile polycystic disease by gray scale echography.Infantile polycystic disease (IPCD) is an uncommon pathologic entity involving the kidneys and liver. Gray scale echography can detect this pathologic process within the kidneys, despite the presence of renal failure. In addition, the sonic study may detect associated hepatic abnormalities even though isotopic liver scan is normal. These capabilities make the ultrasonic examination uniquely suited for evaluating patients with IPCD.- - - - - - - - - - ranking = 0.070959121506842keywords = polycystic, kidney (Clic here for more details about this article) |
5/238. Screening for cerebral aneurysm in patients with polycystic liver disease.BACKGROUND: Polycystic liver disease (PCLD) is an autosomal dominant disease characterized by multiple macrocystic lesions throughout the liver. The association between PCLD and cerebral aneurysm is well documented, and approximately 20% of patients with PCLD have demonstrable cerebral aneurysms at autopsy. The prevalence reported from autopsy series, however, may not reflect the true prevalence in patients with PCLD. We undertook this study to evaluate the prevalence and diagnosis of cerebral aneurysms in screening cerebral studies in patients with PCLD. methods: patients were identified by searching the hepatobiliary surgical service data base and hospital medical records. Hospital charts were reviewed to confirm presence of PCLD and to identify screening studies for cerebral aneurysms. RESULTS: Ten patients with PCLD received screening studies of the cerebral vasculature during a 10 1/2-year period. One patient was found to have an asymptomatic cerebral aneurysm. A 45-year-old woman with no other significant medical history was referred for evaluation of PCLD. Screening magnetic resonance angiography (MRA) revealed a 5 mm aneurysm extending anteriorly near the origin of the right ophthalmic artery, without evidence of rupture. cerebral angiography confirmed these findings, and the aneurysm was clipped. CONCLUSIONS: Because cerebral aneurysms can be an important source of morbidity and mortality in PCLD, we recommend screening by MRA or by computed tomographic angiography (CTA) of the cerebral vasculature in all patients who have PCLD.- - - - - - - - - - ranking = 0.056625545327116keywords = polycystic (Clic here for more details about this article) |
6/238. Transverse hepatectomy for symptomatic polycystic liver disease.Polycystic liver disease can result in massive enlargement of the liver with resultant debilitating symptoms of abdominal pain, chronic fatigue, and severely compromised functional status. Fenestration of hepatic cysts has been advocated as a treatment for polycystic disease. However, in patients with predominant small cyst replacement, fenestration alone often results in limited hepatic volume reduction without improvement in patient functional status. liver transplantation has also been previously advocated for polycystic liver disease with predominant small cyst replacement, but, with the severe shortage of donor organs, alternative treatment should be considered. In this report we present a case of massive enlargement of the liver with severe clinical debilitation due to polycystic liver disease. Transverse hepatectomy provided a safe and effective alternative to fenestration or liver transplantation.- - - - - - - - - - ranking = 0.099094704322453keywords = polycystic (Clic here for more details about this article) |
7/238. New syndrome?: Three sibs diagnosed prenatally with situs inversus totalis, renal and pancreatic dysplasia, and cysts.Recently we described a previously apparently undescribed autosomal recessive syndrome in two sib fetuses with situs inversus totalis, cystic dysplastic kidneys and pancreas, bowing of the lower limbs and clavicles, severe intrauterine growth retardation, and oligohydramnios. This syndrome differs from that of Ivemark and related syndromes due to lack of liver involvement. After these two sibs, this consanguineous family had a third child and an early prenatal diagnosis of pancreatic and dysplastic renal cysts was made in the 19.5-week-old fetus. The last case supports the genetic hypothesis.- - - - - - - - - - ranking = 8.8594923973375E-5keywords = kidney (Clic here for more details about this article) |
8/238. infertility treatment in autosomal dominant polycystic kidney disease (ADPKD)--a case report.Autosomal dominant polycystic kidney disease (ADPKD) is a frequently occurring inherited condition with cysts in many organs including the kidneys. However, a combination of seminal vesicle cysts, cystic obstruction of ejaculatory duct and ADPKD is rarely encountered. The following case report presents an infertile ADPKD patient who had seminal vesicle cysts and ejaculatory duct cyst, and describes the treatment by transurethral resection of the ejaculatory duct.- - - - - - - - - - ranking = 0.56315440472244keywords = polycystic kidney disease, polycystic kidney, polycystic, kidney disease, kidney (Clic here for more details about this article) |
9/238. Transjugular intrahepatic portosystemic shunt for the treatment of intractable ascites in a patient with polycystic liver disease.Though polycystic liver disease (PCLD) has historically been considered a contraindication to TIPS, we present a case where technically successful shunt creation was achieved without the need for modification of the standard TIPS procedure, as was required in a previous report.- - - - - - - - - - ranking = 0.070781931658895keywords = polycystic (Clic here for more details about this article) |
10/238. milk of calcium in the inferior calyx of a hydronephrotic kidney in a tetraplegic patient - a diagnosis to be made before scheduling for extracorporeal shock wave lithotripsy.STUDY DESIGN: A Case Report of renal milk of calcium in a tetraplegic subject. OBJECTIVES: To increase the awareness of renal milk of calcium in spinal cord injury (SCI) physicians. Renal milk of calcium contains a colloidal suspension of calcium crystals. Since upright views of the kidneys are not performed in tetraplegic subjects, the renal milk of calcium may be misinterpreted as renal lithiasis by routine radiography taken in supine position. SETTING: Regional spinal injuries Centre, Southport, england. METHOD: In a 41-year-old male with traumatic tetraplegia, X-ray of abdomen in supine position showed multiple opacities in the region of the left kidney. These radio opaque shadows were interpreted as renal calculi. Subsequently, computed tomography (CT) of the kidneys was performed. RESULTS: CT confirmed the presence of calculi in the mid-polar calyx. However, the density situated in the inferior calyx of the hydronephrotic left kidney exhibited a horizontal upper edge. This specific radiological finding as observed in the CT of kidneys, provided the clue to the presence of milk of calcium in the inferior calyx of the hydronephrotic left kidney. CONCLUSION: As plain film of the abdomen in standing position is not performed in SCI patients, physicians caring for SCI patients should have a high index of suspicion for renal milk of calcium. Prompt diagnosis of renal milk of calcium will help to avoid unnecessary surgery, or extracorporeal shock wave lithotripsy.- - - - - - - - - - ranking = 0.00088594923973375keywords = kidney (Clic here for more details about this article) |
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