1/178. 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) |
2/178. Striking cholestatic liver disease: a distinct manifestation of advanced primary amyloidosis.In patients with systemic amyloidosis, amyloid fibrils are typically deposited in numerous organs, including the kidneys, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinical liver disease is relatively rare. The patient described here had cholestatic liver disease as the primary manifestation of primary systemic amyloidosis. review of the literature suggests that prominent liver disease with cholestasis is unusual but probably underreported in patients with amyloidosis and appears to be restricted to patients with the primary form of amyloidosis. Nonetheless, cholestatic hepatic amyloidosis is characterized by distinct clinical, laboratory, and pathologic features; recognition of this process is critical because it identifies patients with widespread organ involvement and portends a poor prognosis.- - - - - - - - - - ranking = 5.9458544946283E-5keywords = kidney (Clic here for more details about this article) |
3/178. 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 = 0.081487856553302keywords = polycystic, kidney (Clic here for more details about this article) |
4/178. 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 = 0.56584190239736keywords = polycystic kidney disease, polycystic kidney, polycystic, kidney disease, kidney (Clic here for more details about this article) |
5/178. 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 = 0.067728171492913keywords = polycystic, kidney (Clic here for more details about this article) |
6/178. 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 = 0.054087403522416keywords = polycystic (Clic here for more details about this article) |
7/178. 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 = 0.094652956164228keywords = polycystic (Clic here for more details about this article) |
8/178. 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 = 0.06760925440302keywords = polycystic (Clic here for more details about this article) |
9/178. Sonographic pattern of recessive polycystic kidney disease in young adults. Differences from the dominant form.BACKGROUND: To study the sonographic pattern of autosomal recessive polycystic kidney disease (ARPKD) in early adulthood in order to identify imaging criteria to diagnose this disease and to distinguish between recessive and autosomal dominant polycystic kidney disease (ADPKD) in that age group. methods: An abdominal ultrasound was performed on four ARPKD subjects (with a mean age of 20.2) and on 33 ADPKD subjects in early adulthood (29 without renal failure with a mean age of 20.5, and four with renal failure with a mean age of 26.5). Linkage studies with ADPKD and ARPKD markers were compatible with the clinical diagnosis in all cases. RESULTS: The renal sonographic features in ARPKD subjects included multiple small cysts in a normal-sized kidney, increased cortical echogenicity and loss of corticomedullary differentiation. In ADPKD subjects without renal failure, sonographic features included few or multiple cysts of different sizes, in normal-sized kidneys in 22 out of 29 patients (75.8%), normal cortical echogenicity and conserved corticomedullary differentiation, except in patients with nephromegaly. All ADPKD subjects with renal failure had nephromegaly and loss of corticomedullary differentiation. The hepatic sonographic features in ARPKD patients included portal fibrosis and in some cases Caroli's disease, while in ADPKD patients a normal hepatic echostructure was detected in all but one case, in addition to simple hepatic cysts in a few cases. CONCLUSIONS: The evaluation of the sonographic features of the kidneys and those of the liver may help in the differential diagnosis between ARPKD and ADPKD in early adulthood.- - - - - - - - - - ranking = 0.67918865851167keywords = polycystic kidney disease, polycystic kidney, polycystic, kidney disease, kidney (Clic here for more details about this article) |
10/178. Unusual presentation of polycystic liver disease in a young man.Polycystic disease of the liver is usually asymptomatic. The main symptoms of cystic disease are those of an enlarging liver or due to compression of the adjacent organs caused by a large cyst. Less than 5% of these patients present with clinically challenging and life threatening complications. We present a patient with polycystic disease of the liver who developed an aneurysm arising from the right hepatic artery presenting with surgical obstructive jaundice. In spite of intraperitoneal rupture of the aneurysm the patient was managed successfully.- - - - - - - - - - ranking = 0.06760925440302keywords = polycystic (Clic here for more details about this article) |
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