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1/162. Polycystic kidney disease in tuberous sclerosis complex: case report.

    tuberous sclerosis complex (TSC) is an inherited neurocutaneous disorder characterised by seizures, mental retardation, cutaneous lesions and visceral harmatoma. We describe a 4 1/2-year old boy in whom in addition to the commonly described features of TSC, adult-type polycystic kidneys, a scantily reported occurrence, was an associated feature.
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keywords = kidney disease, kidney
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2/162. Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.

    Autosomal dominant polycystic kidney disease (ADPKD) is characterized by both renal and non-renal disorders. Extrarenal involvement includes noncystic manifestations such as cardiovascular abnormalities, colonic diverticula and intracranial aneurysms. Familial sensorineural hearing loss (SNHL) has been included in the definition of Alport's syndrome. However, other types of nephropathy have been occasionally associated with hereditary deafness. The association of ADPKD with hereditary SNHL has not been previously documented. We report a family with ADPKD associated with bilateral sensorineural deafness in a pedigree of four affected members in four generations.
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ranking = 1.2499999849169
keywords = kidney disease, kidney
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3/162. Traumatic hematuria in patients with polycystic kidney disease.

    Autosomal dominant polycystic kidney disease (PKD) is the most prevalent hereditary disorder in this country and a common cause of chronic renal failure. patients frequently present with hematuria as the initial manifestation of PKD. We describe a patient with gross hematuria after blunt trauma who was found to have previously undiagnosed PKD. We review present diagnostic and treatment modalities and suggest potential management strategies for surgeons caring for patients presenting with traumatic hematuria and PKD.
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ranking = 1.2499999849169
keywords = kidney disease, kidney
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4/162. 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 = 1.2499999849169
keywords = kidney disease, kidney
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5/162. Fetal polycystic kidney disease in oro-facio-digital syndrome type I.

    We report a girl with oro-facio-digital syndrome type I (OFD I) associated with polycystic kidney disease (PKD), which was identified on fetal US and fetal MRI. After birth, the diagnosis of this X-linked dominant disorder, which is lethal in males, was achieved by recognition of facial dysmorphism, lingual hamartomas, postaxial polydactyly, brain malformations, and the existence of her deceased male sibling with similar malformations. adult PKD is a common feature in heterozygous females with OFD I. However, fetal PKD has been reported only in a lethal homozygous male. Our observation expands our knowledge about the phenotypic variations of PKD in OFD I.
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ranking = 1.2499999849169
keywords = kidney disease, kidney
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6/162. dialysis-associated renal cystic disease resembling autosomal dominant polycystic kidney disease: a report of two cases.

    Acquired renal cystic disease is common in patients receiving dialysis. Characteristically, the kidneys are small or, less often, normal in size, and the cysts are usually less than 0.6 cm in diameter. We present here 2 patients who, after 5 and 7 years on hemodialysis, developed marked renal enlargement, with large cysts in the kidneys and, in 1 patient, in the liver as well; the appearance on ultrasonography and computed tomography was indistinguishable from autosomal dominant polycystic kidney disease. Before starting dialysis the first patient was a 19-year-old man who developed renal shutdown from crescentic glomerulonephritis, and the second patient was a 33-year-old man who developed end-stage renal failure from malignant hypertension. Neither patient had renal cysts at the onset of end-stage renal failure.
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ranking = 1.2500000090498
keywords = kidney disease, kidney
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7/162. Unilateral renal cystic disease in adults.

    Unilateral renal cystic disease (URCD) is morphologically indistinguishable from autosomal dominant polycystic kidney disease (ADPKD) except for its unilaterality. Unlike ADPKD, URCD patients show neither a genetic background nor progressive deterioration in renal function; thus, the differential diagnosis of URCD from ADPKD is important. Only a few cases of URCD have been reported. This study reports two cases of URCD in adults together with a literature review. We identified these two cases using abdominal computerized tomography and family screening with renal ultrasonography.
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ranking = 0.24999999698339
keywords = kidney disease, kidney
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8/162. Renal asymmetry in children with autosomal dominant polycystic kidney disease.

    Although for decades autosomal dominant polycystic kidney disease (ADPKD) was considered a disease of adults, our recent longitudinal studies on children from ADPKD families have shown that the disease is evident by ultrasound imaging in approximately 75% of children who are carriers of the ADPKD1 gene, the most common form of ADPKD. Here we report that, in contrast to adults, the disease appears to be unilateral initially in approximately 17% of children. Asymmetric enlargement of the kidneys is also frequently observed. This renal asymmetry can be extreme and lead to diagnostic confusion. We present 2 unusual cases of asymmetric renal involvement that we have observed during the last 10 years. The first is a 14-year-old boy who was scheduled for a nephrectomy to relieve pain and whose family requested a second opinion. The second is a 10-year-old girl who was diagnosed with ADPKD in utero by prenatal ultrasound. After birth, 1 kidney progressively developed cysts and enlarged, whereas the other had only a few tiny cysts and remained normal in size. A review of the literature shows that presentations like these often lead to a nephrectomy or surgical biopsy. A carefully obtained family history and examination of both parents with ultrasound can help to avoid unnecessary invasive procedures. If pain is a prominent symptom, it can be treated by cyst aspiration if there are only a few cysts or a single dominant cyst. The molecular mechanism for extremely asymmetric renal disease remains to be elucidated.
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ranking = 1.2500000090498
keywords = kidney disease, kidney
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9/162. Transcatheter renal arterial embolization therapy on a patient with polycystic kidney disease on hemodialysis.

    We report a patient with autosomal dominant polycystic kidney disease (ADPKD) undergoing long-term hemodialysis who underwent transcatheter arterial embolization (TAE) of the renal arteries to shrink enlarged kidneys. In 1983, the patient started hemodialysis because of chronic renal failure secondary to ADPKD. However, renal size continued to increase. In January 1997, he was admitted to our hospital with abdominal distension and anorexia, in addition to progression of anemia. Upper gastroendoscopy showed an esophageal ulcer and severe external compression of the stomach. Renal angiography using the Seldinger technique showed stretched and deformed segmental renal arteries with massive enlargement of the kidneys. TAE with stainless steel coils was performed on both renal arteries. With a rapid and progressive decrease in kidney size, anorexia and anemia were improved, and the gastrointestinal compression was eliminated. In some patients with ADPKD, renal size continues to increase even after the initiation of dialysis. In about 10 years, patients develop gastrointestinal complications, such as dysphagia, ileus, severe constipation, and intestinal perforation. Surgical procedures such as nephrectomy are not satisfactory. This report shows that TAE is a safe and effective therapy for patients with ADPKD with massively enlarged kidneys.
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ranking = 1.2500000331828
keywords = kidney disease, kidney
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10/162. Unilateral renal cystic disease.

    Unilateral renal cystic disease (URCD) is a distinct entity that is one of the renal cystic diseases. URCD consists of a cluster of multiple cysts in part or most of one kidney with no association of cystic disease in the contralateral kidney. URCD is a nonfamilial, nonprogressive disorder and is not related with autosomal dominant polycystic kidney disease (ADPKD). We report a case of URCD with six-year CT follow-up. Confinement of the cystic disease to one kidney with an absence of cysts in other organs such as liver or pancreas distinguish URCD from ADPKD. Absence of an encapsulated mass and intervening normal renal parenchyma between the cysts can differentiate URCD from cystic renal tumors.
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ranking = 0.25000003318276
keywords = kidney disease, kidney
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