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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