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Tag Archives: berry aneurysm
Cystic Kidney Diseases
Cystic Diseases of the Kidney
The different types of cystic diseases in the kidney and there associated pathological findings and prognosis.
Adult Polycystic Kidney Disease
Genetics: PKD1, PKD2 mutations encode for polycystin 1 and 2 respectively
Clinical associations:
- berry aneurysms in circle of Willis
- polycystic liver disease with bile duct proliferation
- mitral valve prolapse (MVP)
Pathology: markedly distorted kidney by cysts with no obvious intervening parenchyma although there are functional glomeruli
Prognosis: Chronic renal failure at 40-60 years of age
Autosomal Recessive Childhood Polycystic Kidney Disease
Genetics: PKHD (polycystic kidney and hepatic disease)
Clinical association: liver cysts and bile duct proliferation
Histology
- dilation of tubules lined by cuboidal cells
- hepatic fibrosis
Prognosis: Poor. Death in infancy, childhood.
Posted in Kidney
Tagged Adult Polycystic Kidney Disease, Autosomal Recessive Childhood Polycystic Kidney Disease, berry aneurysm, Bile duct proliferation, Chronic renal failure, Cystic diseases of the kidneys, Cystic Kidney Disease, Hepatic fibrosis, Liver cysts, Mitral valve prolapse, PKD1, PKD2, PKHD, polycystic kidney and hepatic disease, Polycystic liver disease, Polycystin 1, Polycystin 2
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Berry Aneurysm
Berry Aneurysm
Most common locations of Berry Aneurysms:
· Anterior cerebral artery (anterior communicating artery)
· Middle cerebral artery
· Internal carotid artery
Associated systemic conditions for Berry Aneurysms:
· ADPCKD
· Ehlers-Danlos syndrome (type IV)
· Neurofibromatosis type 1
· Marfan syndrome
· Coarctation of aorta
· Fibromuscular dysplasia of extracranial arteries
Predisposing factors for Berry Aneurysms:
- Cigarette smoking and hypertension are
Berry Aneurysm Gross Features
Unruptured aneurysms are a thin-walled arterial outpouchings at branch points of the circle of Willis or a major vessel just beyond. Measure 2-3 mm and are shiny, bright red surface with thin, translucent wall. Atheromatous plaques, thrombosis, or calcification may be found in wall or lumen of aneurysm. Brownish discolouration of brain/meninges indicates previous hemorrhage. Rupture occurs at apex of sac with extravasation of blood to subarachnoid space, substance of brain, or both.
Histological Features of Berry Aneurysms:
Arterial wall adjacent to aneurysm shows intimal thickening and attenuation of media as it approaches the neck of the aneurysm. At the neck of the aneurysm the muscular wall and intimal elastic lamina stop short and are absent from the aneurysmal wall itself. The sac is made up of thickened, hyalinised intima. The adventitia covering the sac is continuous with that of the parent artery
References:
Robbins Basic Pathology 7th ed, edited by Vinay Kumar, Ramzi S. Cotran, and Stanley J. Robbins, 873 pp, Philadelphia, Pa, Sounders, 2003.
Posted in Brain, Vascular
Tagged , aneurysm, aneurysms, berry, berry aneurysm, cigarette, hypertension, marfan syndrome, smoking
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