1/27. Ask-Upmark kidney associated with renal and extrarenal arterial aneurysms.Reports of Ask-Upmark kidney, initially described as a congenital defect in renal development, are uncommon. We report a case with the features of bilateral asymmetrical segmental atrophy in a patient with childhood-onset hypertension. As an adult, she developed cerebral, celiac, and renal artery aneurysms. She underwent successful clipping of the cerebral aneurysm and renal artery repair with preservation of renal function. Novel radiologic techniques make possible the noninvasive diagnosis of segmental atrophy and its complications.- - - - - - - - - - ranking = 1keywords = kidney (Clic here for more details about this article) |
2/27. Does the tuberous sclerosis complex include intracranial aneurysms? A case report with a review of the literature.BACKGROUND: tuberous sclerosis is a protean, genetically determined disease that may involve any organ or tissue and lead to a great number of symptoms and clinical features. OBJECTIVE: diagnosis can be very difficult in cases with incomplete manifestations (formes fruste) lacking the classic signs of the disease. MATERIALS AND methods: We report a case fulfilling the diagnostic criteria for tuberous sclerosis (shagreen patches, hypomelanotic macules, renal cysts and angiomyolipomas, and "migration tracts" in the cerebral white matter) in association with a giant intracranial aneurysm, but lacking mental retardation, epilepsy and facial angiofibroma. RESULTS: Fourteen other cases of tuberous sclerosis and intracranial aneurysms, all but one without any clear sign of polycystic kidney disease, were found in the literature. CONCLUSION: We suggest that vascular dysplasias in general and aneurysms (mainly intracranial) in particular can be added to the other non-primary diagnostic features for the clinical diagnosis of tuberous sclerosis.- - - - - - - - - - ranking = 1164.7011462185keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
3/27. Multiple intracranial aneurysms in a patient with autosomal recessive polycystic kidney disease.Autosomal recessive polycystic kidney disease (ARPKD) is usually characterized by early onset chronic renal failure due to innumerable dilated collecting ducts. Hepatic fibrosis is an obligate sign. Here, for the first time, we report a 31-year-old female with ARPKD who was diagnosed with symptomatic multiple intracranial aneurysms, a manifestation previously only known to be associated with autosomal dominant polycystic kidney disease (ADPKD).- - - - - - - - - - ranking = 2721.9843463955keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
4/27. 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 = 103.71904947573keywords = polycystic (Clic here for more details about this article) |
5/27. Ruptured cerebral aneurysm not detected by magnetic resonance angiography in juvenile autosomal dominant polycystic kidney.Recently, it has been reported that magnetic resonance angiography (MRA) is useful for screening and following up cerebral aneurysms in patients with autosomal dominant polycystic kidney disease (ADPKD). However, a patient was encountered with a ruptured cerebral aneurysm that was not detected by routine MRA. The patient, a 29-year-old man with ADPKD, was followed up at our hospital for more than 5 years. Ten months after an MRA examination, he suddenly developed severe headache. brain computed tomography revealed subarachnoid hemorrhage. Digital subtraction angiography detected an aneurysm with a diameter of approximately 2 mm in the anterior communicating artery. Clipping of the aneurysm was immediately performed and he recovered without sequela after operation. magnetic resonance angiography is useful to detect cerebral aneurysms, but it can not detect aneurysms measuring less than 4 mm.- - - - - - - - - - ranking = 2240.5852130623keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
6/27. A giant internal carotid-posterior communicating artery aneurysm presenting with atypical trigeminal neuralgia and facial nerve palsy in a patient with autosomal dominant polycystic kidney disease: a case report.BACKGROUND: In cases of internal carotid-posterior communicating artery (IC-PC) aneurysm, involvement of the trigeminal nerve at its root is rare, and facial nerve palsy is even more unusual. CASE REPORT: A large, unruptured IC-PC aneurysm was detected in a 56-year-old man with autosomal dominant polycystic kidney disease (ADPKD), but surgery was not performed because of mild renal dysfunction. Two months later, a sudden, severe headache suggested a subarachnoid hemorrhage, which was ruled out by computed tomography and lumbar puncture. Neurological examination revealed complete oculomotor palsy, atypical trigeminal neuralgia, and facial palsy with gustatory disturbance. Magnetic resonance (MR) imaging revealed a partially thrombosed giant aneurysm that directly compressed the trigeminal nerve root, reached the internal auditory canal, and was adjacent to the facial nerve. The neck of the aneurysm was successfully clipped via a subtemporal transtentorial approach. The postoperative course was uneventful, and all neurological symptoms had resolved within 3 months. CONCLUSIONS: We believe that the prosopalgia in this case was atypical trigeminal neuralgia due to direct compression of the trigeminal nerve root by the aneurysmal sac. A contributory cause was stretching of the oculomotor nerve, which contains sensory afferent inhibitory fibers derived from the ophthalmic branch of the trigeminal nerve. The facial palsy was of peripheral type and was accompanied by gustatory disturbance. This is the first reported case of facial palsy caused by an IC-PC aneurysm and also a very rare case of an IC-PC aneurysm clipped by a subtemporal transtentorial approach.- - - - - - - - - - ranking = 5823.5057310927keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
7/27. Intracranial aneurysms in a child with autosomal recessive polycystic kidney disease.Intracranial aneurysms (ICA) are a well-known feature of autosomal dominant polycystic kidney disease. There is only one report about ICA in an adult patient with autosomal recessive polycystic kidney disease (ARPKD). We observed a 2-year, 6-month old girl with ARPKD and multiple ICA. The family history is negative for kidney disease. The diagnosis of ARPKD was based on the typical findings in ultrasonography and computed tomography. Cystic ectasia of biliary ducts 6.3/4.8 cm in diameter was found in the liver. Arterial hypertension in a range of 140/100-170/120 mm Hg was registered. The child has polyuria, polydipsia and enuresis. Blood urea was 15 mmol/l, creatinine in a range of 120 to 75 micromol/l. One episode of vomiting, dizziness and lethargy was the reason for a brain magnetic resonance imaging. Multiple fusiform and saccular aneurysms in the branches of middle and posterior cerebral arteries were seen bilaterally. The girl is growing well without neurological symptoms during an observation period of 1.5 years. blood pressure is well controlled with an ACE inhibitor (enalapril 2.5 mg daily). It was concluded that ICA can be found in patients with ARPKD. blood pressure control is essential to reduce the risk of intracranial hemorrhage.- - - - - - - - - - ranking = 7060.4226284319keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
8/27. Intracranial aneurysms and dolichoectasia in autosomal dominant polycystic kidney disease.BACKGROUND: Intracranial saccular aneurysms (ICA) are a known extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). In order to facilitate the definition of subgroups who are at risk for ICA and to determine the prevalence of ICA in these subgroups we studied ADPKD patients with a positive family history for a cerebral event, including cerebral stroke (group I) and intracranial bleeding or known ICA (group II). methods: Within an enrolment period of 21 months, 43 ADPKD patients from our outpatient clinic and hospital were examined with cerebral magnetic resonance angiography (MRA). RESULTS: ICA were detected in six patients (14%). Three out of 32 patients (9.4%) in group I and three out of 11 patients (27.3%) in group II had an ICA. A dolichoectasia of intracerebral vessels was found in two out of 43 patients (4.7%). CONCLUSIONS: Using MRA a high prevalence of ICA was shown only in patients with a family history of cerebral bleeding or ICA. A family history for cerebral stroke does not imply an elevated risk for ICA. However, dolichoectasia, rare in the normal population, was detected in two patients. We recommend screening for ICA in patients with a positive family history for cerebral bleeding or ICA. Because of potential complications, examiners should direct their attention to dolichoectasia in ADPKD patients.- - - - - - - - - - ranking = 5823.5057310927keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
9/27. Development of de novo intracranial aneurysm in three months: case report and literature review.Summary: Formation of de novo intracranial aneurysms is rare. Their etiology is not known, but they are seen in patients with inherited collagen disorders, polycystic kidney disease, and familial history of aneurysms. Most de novo intracranial aneurysms are found 3-20 years after diagnosis of the initial aneurysm. We report the imaging findings in a 46-year-old man who developed a de novo intracranial aneurysm only 3 months after surgical clipping of another aneurysm.- - - - - - - - - - ranking = 1164.7011462185keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
10/27. A 4-year-old girl with autosomal dominant polycystic kidney disease complicated by a ruptured intracranial aneurysm.In patients with autosomal dominant polycystic kidney disease (ADPKD), intracranial aneurysms (ICAs) are extrarenal manifestations and may result in serious and potentially fatal outcome following rupture. Although ICAs are a well-known complication of ADPKD, nearly all cases of ICA occurring in the context of ADPKD are adults. Here, we report the case of a Japanese girl with ADPKD who developed a subarachnoid haemorrhage (SAH) due to a ruptured ICA at the age of 4 years. CONCLUSION: This report is intended to raise awareness that the use of noninvasive screening techniques such as three-dimensional CT angiography or magnetic resonance angiography to detect intracranial aneurysms should also be performed in paediatric patients with autosomal dominant polycystic kidney disease.- - - - - - - - - - ranking = 6988.2068773113keywords = polycystic kidney disease, polycystic kidney, kidney disease, polycystic, kidney (Clic here for more details about this article) |
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