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Int J Clin Exp Pathol 1(1):84-90;2008
Case Report
Adult Polycystic Kidney Disease: A Disorder of Connective Tissue?
Anwar Ul Haque and Ambreen Moatasim
Department of Pathology, Pakistan Institute of Medical Sciences Islamabad Pakistan
Received 15 June 2007; accepted with revision 15 July 2007; available online 1 January 2008
Abstract: Adult Polycystic Kidney Disease (APCKD) is one of the most common serious inherited disorders. Many affected patients succumb
to the renal and non renal manifestations of this autosomal dominant disease. The disease is characterized by the cysts formation in several
organs, most obvious of which are in the renal parenchyma and that has led to the name of the condition. The other associated features of the
disease include hepatic fibrosis, hepatic cysts, pancreatic cysts, splenic cysts, Berry aneurysms, colonic diverticulae, hernias and cardiac
valvular disorders! The rupture of Berry aneurysm is a sudden and often fatal manifestation and brings to attention some unsuspected cases
of APCKD. We recently examined one surgically removed kidney of APCKD in a 15 year old male patient. In addition to the classical cystic
changes we were impressed with the hitherto unreported vascular changes which resulted from excessive and or atypical renal parenchymal
matrix. The excessive and weak matrix has contributed to dilatations of both renal tubules giving rise to so called cysts and the blood vessels.
Our findings suggest that the APCKD is in reality a connective tissue disorder which is a common denominator between all the manifestations
or pleotropism of the APCKD. (IJCEP706003).
Key Words: Adult Polycystic Kidney Disease, APCKD, Renal Cysts, Hepatic cysts, Hepatic fibrosis, Berry aneurysm, Hernia, Divertiula, Cardiac
valvular disorders
Full Text: PDF
Case Report
Adult Polycystic Kidney Disease: A Disorder of Connective Tissue?
Anwar Ul Haque and Ambreen Moatasim
Department of Pathology, Pakistan Institute of Medical Sciences Islamabad Pakistan
Received 15 June 2007; accepted with revision 15 July 2007; available online 1 January 2008
Abstract: Adult Polycystic Kidney Disease (APCKD) is one of the most common serious inherited disorders. Many affected patients succumb
to the renal and non renal manifestations of this autosomal dominant disease. The disease is characterized by the cysts formation in several
organs, most obvious of which are in the renal parenchyma and that has led to the name of the condition. The other associated features of the
disease include hepatic fibrosis, hepatic cysts, pancreatic cysts, splenic cysts, Berry aneurysms, colonic diverticulae, hernias and cardiac
valvular disorders! The rupture of Berry aneurysm is a sudden and often fatal manifestation and brings to attention some unsuspected cases
of APCKD. We recently examined one surgically removed kidney of APCKD in a 15 year old male patient. In addition to the classical cystic
changes we were impressed with the hitherto unreported vascular changes which resulted from excessive and or atypical renal parenchymal
matrix. The excessive and weak matrix has contributed to dilatations of both renal tubules giving rise to so called cysts and the blood vessels.
Our findings suggest that the APCKD is in reality a connective tissue disorder which is a common denominator between all the manifestations
or pleotropism of the APCKD. (IJCEP706003).
Key Words: Adult Polycystic Kidney Disease, APCKD, Renal Cysts, Hepatic cysts, Hepatic fibrosis, Berry aneurysm, Hernia, Divertiula, Cardiac
valvular disorders
Full Text: PDF
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