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1/27. 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.
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ranking = 1
keywords = hepatic
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2/27. 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.
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ranking = 0.037653817318685
keywords = obstruction
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3/27. Ileo-ileo-colic intussusception secondary to an ileal lipoma.

    adult intussusception is extremely uncommon and constitutes approximately 5-16% of all intussusceptions. We describe a case of ileo-ileo-colic intussusception secondary to an ileal lipoma occurring in a 22 year old man who in addition had autosomal dominant polycystic kidney disease. CT scan played a pivotal role in not only demonstrating the cause of intestinal obstruction (intussusception) but also showed that a lipoma was the lead point precipitating the intussusception.
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ranking = 0.037653817318685
keywords = obstruction
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4/27. 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.
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ranking = 0.5
keywords = hepatic
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5/27. A case of successful management of portosystemic shunt with autosomal dominant polycystic kidney disease by balloon-occluded retrograde transvenous obliteration and partial splenic embolization.

    We describe a patient with autosomal dominant polycystic kidney disease who was successfully managed for severe abdominal distension, impaired liver function and a portosystemic shunt by interventional therapies. The patient's intra-hepatic portal vein was compressed and narrowed by multiple liver cysts, which resulted in a decrease of the portal blood flow and portal hypertension due to a huge gastro-renal shunt These haemodynamic changes were assumed to contribute to insufficient protein synthesis in the liver. Therefore, we first repeatedly performed minocycline hydrochloride instillations to treat the multiple liver cysts. Then, we conducted a partial splenic embolization to prevent elevation of the portal vein pressure prior to balloon-occluded retrograde transvenous obliteration which was performed to increase the portal blood flow. The portal blood flow markedly increased, and protein synthesis in the liver also recovered and the clinical symptoms improved. The patient has been monitored for more than two years up to the present and her liver function parameters have remained within the normal range. renal insufficiency is known to be a major prognostic factor in autosomal dominant polycystic kidney disease. In some cases, however, liver involvement with multiple cysts may result in a fatal outcome. In such cases, interventional therapies, as provided to this patient, should be considered.
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ranking = 0.16666666666667
keywords = hepatic
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6/27. intestinal obstruction in autosomal dominant polycystic kidney disease.

    A 42-year-old woman with autosomal dominant polycystic kidney disease (ADPKD) was admitted to our hospital on April 29, 1999, with complaints of abdominal pain. A diagnosis of intestinal obstruction was reached on the basis of clinical findings and X-ray evidence. A computed tomography scan of the abdomen showed massively enlarged kidneys, especially the right kidney, which seemed to compress the small intestine. The patient underwent percutaneous aspiration of the largest cysts on the surface of the right kidney. The symptoms, in this rare case of intestinal obstruction by an enlarged kidney in ADPKD, were alleviated the day after the aspiration procedure.
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ranking = 0.22592290391211
keywords = obstruction
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7/27. Diagnosis of renal and hepatic cyst infections by 18-F-fluorodeoxyglucose positron emission tomography in autosomal dominant polycystic kidney disease.

    BACKGROUND: infection of a renal or hepatic cyst is a serious complication of autosomal dominant polycystic kidney disease (ADPKD). Although crucial for successful management, early diagnosis is difficult, largely because of nonspecific symptoms and limitations of conventional imaging techniques. Because of an increased metabolic rate, inflammatory cells take up large amounts of glucose. 18-F-fluorodeoxyglucose (FDG), therefore, represents a promising agent for detection of cyst infections using positron emission tomography (PET). methods: The authors studied the results of 7 FDG PET scans in 3 ADPKD patients suspected of renal or hepatic cyst infection. Two PET scans were performed in patient A (PET 1 and 2), one PET scan was performed in patient B (PET 3), and 4 PET scans were performed in patient C (PET 4, 5, 6 and 7). RESULTS: FDG PET identified the infected cysts in 2 episodes of renal cyst infection (PET 2 and 3), 2 episodes of hepatic cyst infection (PET 6 and 7), and 1 episode of both renal and hepatic cyst infection (PET 1). In patient C, FDG PET was normal after 6 weeks of antibiotic treatment for hepatic cyst infection (PET 4) and again at a time when hepatic cyst infection was suspected, but eventually colchicine intoxication was diagnosed (PET 5). CONCLUSION: In these patients, FDG PET proved very helpful in diagnosing and in excluding renal and hepatic cyst infections. It is concluded that FDG PET is a promising new imaging technique enabling early identification of renal and hepatic cyst infections in ADPKD patients.
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ranking = 2
keywords = hepatic
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8/27. Intravascular embolization therapy in a patient with an enlarged polycystic liver.

    Because the kidneys in patients with autosomal dominant polycystic kidney disease (ADPKD) are usually supplied by well-developed arteries, the authors attempted renal contraction therapy in such patients with renal transcatheter arterial embolization (TAE) using intravascular coils. In most patients with marked nephromegaly, renal TAE was effective. However, in patients with marked hepatomegaly without significant nephromegaly, renal TAE was not effective, and hepatic treatment was always required. In June 2001, the authors obtained approval for a new treatment,"TAE for enlarged polycystic liver," from an ethics committee discussing new treatment at their hospital. A 56-year-old man undergoing hemodialysis for 2 months was referred to the authors' institute with the complaint of severe abdominal distension and loss of appetite in March 2001. Most of the liver (about 90%) had been replaced by multiple cysts, and near-intact hepatic parenchyma was quite scarce. The kidneys were quite small compared with the liver. angiography results showed that almost all hepatic arterial branches were well developed, although most of the portal vein was obstructed, and only the left medial portal vein was spared; the former corresponded to the hepatic region replaced by multiple cysts and the latter to the preserved intact hepatic parenchyma. The target of TAE was the hepatic arterial branches of the former. Microcoils were used as embolization material. Two years after TAE, abdominal distension has markedly decreased because of decrease in liver size (to 54% of the previous value), and muscle and fat volume in the thoracic region have increased owing to improvement of appetite. ascites became obscure. Nutrition and activities of daily living have improved. Hepatic TAE may be an option for treatment of patients in poor condition with symptomatic polycystic liver.
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ranking = 1
keywords = hepatic
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9/27. thrombosis in inferior vena cava (IVC) due to intra-cystic hemorrhage into a hepatic local cyst with autosomal dominant polycystic kidney disease (ADPKD).

    A 76-year-old man with autosomal dominant polycystic kidney disease (ADPKD) was admitted complaining of severe edema of bilateral lower extremities. Computed tomography (CT) of his abdomen revealed an enlarged hepatic cyst with intra-cystic hemorrhage, and massive thrombosis in the inferior vena cava (IVC). The extrinsic mechanical stress on the IVC seemed to induce thrombosis within the IVC, and the thrombosis was likely the cause of severe edema in lower extremities. In this report, we present a rare case of IVC thrombosis due to an enlarged cyst in ADPKD successful treated with anticoagulant therapy.
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ranking = 0.83333333333333
keywords = hepatic
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10/27. Polycystic liver disease with obstructive jaundice: treatment with ultrasound-guided cyst aspiration.

    A 59-year-old woman with polycystic liver (PCLD) and kidney disease (PCKD) presented with jaundice, fever, and abdominal pain. A computered tomographic (CT) scan performed immediately after an endoscopic retrograde cholangiopancreatogram (ERCP) clearly demonstrated a hepatic cyst obstructing the biliary system. The cyst was then aspirated under ultrasound guidance with complete relief of obstruction.
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ranking = 0.20432048398535
keywords = hepatic, obstruction
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