Cases reported "Cysts"

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11/169. 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 = 1
keywords = kidney disease, kidney
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12/169. association of cystic nephroma with angiomyolipoma in the same kidney.

    The synchronous occurrence of two different renal tumors in a patient is a rare event. Here we report an incidentally discovered coexistence of cystic nephroma with angiomyolipoma in the right kidney. The patient was a 68-year-old woman without the tuberous sclerosis complex. To the best of our knowledge, this combination of neoplasms has not yet been described before. This rare situation emphasizes the need for careful diagnosis and the correct management of coincident renal neoplasms.
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ranking = 0.0098693306220307
keywords = kidney
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13/169. Isolated visceral leishmaniasis presenting as an adrenal cystic mass.

    A 69-year-old woman presented with a large left retroperitoneal suprarenal mass. Radical resection of the left kidney and the mass revealed a cystic adrenal tumor with a weight of 1500 g. Histologic examination showed that the cyst was composed mostly of partially organized clotted blood. The periphery of the mass consisted of a thin rim of cortical and medullary adrenal tissue with superimposed granulomatous chronic inflammation. The infectious nature of the process was manifested by the scattered intracellular and extracellular Leishmania amastigotes that were found throughout the lesion. The differential diagnosis of cystic adrenal masses and the unusual presentation of visceral leishmaniasis are discussed in this context.
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ranking = 0.0019738661244061
keywords = kidney
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14/169. Ivemark syndrome: asplenia with kidney collecting duct cysts and polysplenia with cerebellar cyst.

    Two newborns, one male and one female, from two different families, with Ivemark syndrome proven at autopsy are reported. One of them had asplenia and another had polysplenia. Both newborns had complex cardiac defects with isomerism of the lungs. The newborn with asplenia had dextrocardia, transposition of the great vessels, stenosis of the pulmonary artery, common atrioventricular canal and patent ductus arteriosus. The newborn with polysplenia had a common atrium, hypoplastic left ventricle and patent ductus arteriosus. The patient with asplenia had cystic dilated collecting ducts of the kidney and the patient with polysplenia had cerebellar cyst. These associate malformations have not been reported previously. Both cases were sporadic.
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ranking = 0.0098693306220307
keywords = kidney
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15/169. 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 = 0.99407840162678
keywords = kidney disease, kidney
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16/169. MRI and proton spectroscopy in Lowe syndrome.

    The oculocerebrorenal syndrome of Lowe (OCRL) is an X-linked disorder characterized by major abnormalities of eyes, nervous system, and kidneys. We report two patients with typical intracranial lesions on MRI. The proton spectroscopy study of the periventricular white matter showed a moderate elevation of the signal at 3.56 ppm in the patient with cystic lesions. This resonance is usually assigned to myo-inositol and interpreted as a glial marker. In our patient it could also represent a true accumulation inside the cysts of phosphatidylinositol 4,5-biphosphate which is not degraded in patients with Lowe syndrome.
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ranking = 0.0019738661244061
keywords = kidney
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17/169. Symptomless cyst formation at the location of a biloma resolved with a single aspiration: case report.

    A biloma is an encapsulated bile collection outside the biliary tree. The underlying causes include iatrogenic, traumatic, and spontaneous injury of the biliary tree. Its diagnosis is based upon clinical history, imaging studies, and needle aspiration with chemical analysis of the fluid. Biloma usually presents as a simple or septated cyst or a cyst with internal contents. The symptomatic biloma if left untreated may result in significant morbidity and even mortality. In past reports, the mainstay of management relied on bile flow diversion and/or bile drainage to achieve elimination of bile as well as complete resolution of the cyst. We report on a 58-year-old woman with a 9.0 x 5.4 cm cyst between the right liver and the upper role of the kidney after wedge resection for a hepatoma in the right lobe. She suffered from abdominal pain and fever. A single percutaneous needle aspiration with chemical analysis confirmed the diagnosis of biloma and relieved her symptoms. The biloma vanished immediately. However, a recurrent symptomless cyst was detected at the same site 6 months later. Its content was proven to be clear transudate, and it was left untreated. If a biloma has no communication with the biliary tree, and the proximal biliary tree has neither stricture nor stones, percutaneous aspiration alone may achieve resolution.
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ranking = 0.0019738661244061
keywords = kidney
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18/169. Successful living donor liver transplantation for polycystic liver in a patient with autosomal-dominant polycystic kidney disease.

    Orthotopic liver transplantation has been recommended for patients with disabling polycystic liver disease (PCLD). Because of the shortage of cadaveric donors, living donor liver transplantation (LDLT) has been developed as an alternative. We describe the case of a woman with PCLD as an extrarenal manifestation of autosomal-dominant polycystic kidney disease (ADPKD) who was successfully palliated by LDLT. The patient was a 48-year-old woman with abdominal distention. Computed tomography showed a massively enlarged liver containing innumerable cysts, as well as bilateral kidney cysts. Hepatic and renal functions were well preserved. Genetic analysis of the family did not exclude linkage to the PKD1 locus. Two and a half years after the first examination, the patient reported severely disabling symptoms caused by the PCLD. Living donor liver transplantation was performed using a right-lobe graft. The recipient and donor were both well 8 months after the transplantation. The excised liver weighed 7.4 kg, and the histopathology revealed multiple cysts and von Meyenburg complexes in the portal areas.
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ranking = 0.83037253414672
keywords = kidney disease, kidney
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19/169. Hepatocyte nuclear factor-1beta: a new kindred with renal cysts and diabetes and gene expression in normal human development.

    The hepatocyte nuclear factor-1beta (HNF-1beta) transcription factor controls endoderm development. Human mutations cause early-onset diabetes mellitus and have recently been associated with dysplastic, hypoplastic, and glomerulocystic kidneys. A new kindred with this "renal cysts and diabetes" syndrome is described, and nephrogenic HNF-1beta expression is defined. The proband had congenital cystic kidneys: over the next 12 yr, his renal function was impaired, but he was normoglycemic. His mother developed diabetes during pregnancy: renal ultrasonography at age 24 yr was normal, but she subsequently developed cysts. Both subjects have a heterozygous frameshift mutation in HNF-1beta that results from a 1-bp insertion in exon 5 (Y352fsinsA). When reverse-transcription PCR and in situ hybridization were used, HNF-1beta mRNA was detected in normal human metanephroi, with the highest levels of transcripts localized to fetal medullary and cortical collecting ducts and low levels of expression in nephrogenic cortex mesenchyme, primitive nephron tubules, and immature glomeruli. These results constitute the first demonstration of HNF-1beta expression during human nephrogenesis and emphasize a disease spectrum associated with HNF-1beta mutation.
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ranking = 0.0039477322488123
keywords = kidney
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20/169. Seminal vesicle cyst associated with ipsilateral renal agenesis and papillary carcinoma of the bladder.

    We report a case of seminal vesicle cyst associated with ipsilateral renal agenesis in a 37-year-old patient with papillary carcinoma of the bladder. ultrasonography showed absence of the right kidney, a bladder tumour and a round retrovesical hypoechogenic mass with posterior acoustic enhancement. It showed low attenuation on CT, low signal intensity on T1-weighted MR images and high signal intensity on T2-weighted MR images. Intravenous urography (IVU) and cystoscopy were also performed. After surgery, it proved to be a seminal vesicle cyst. The embryology, imaging characteristics and differential diagnosis of seminal vesicle cysts are discussed. Associated findings are also described.
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ranking = 0.0019738661244061
keywords = kidney
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