ASSOCIATION OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE AND ABDOMINAL AORTIC ANEURYSM
Date Issued
2024-04-04
Author(s)
Sulejman S
Usprcov J
Karanfilovikj A
Abstract
ASSOCIATION OF AUTOSOMAL DOMINANT
POLYCYSTIC KIDNEY DISEASE AND ABDOMINAL
AORTIC ANEURYSM
Z. Shterjova Markovska1, I. Rambabova Bushljetikj1, G.
Severova1, L. Trajcevska1, I. Nikolov1, V. Karanfilovski1,
S. Sulejman1, J. Usprcov1, Z. Janevski1, A. Canevska1, A.
Karanfilovikj1, S. Krstevska Balkanov2, G. Spasovski1
1University clinic of Nephrology, 2University clinic of
Hematology, Faculty of Medicine, University Sts. Cyril
and Methodius Skopje, N. Macedonia
PP
Introduction. Autosomal dominant polycystic kidney
disease (ADPKD) is a systemic disease with multiple
cysts in several organs. Formation of aneurysms of:
the aorta, coronary and cerebral arteries are increasingly reported in the literature as extra-renal
manifestations.
Case report. We report 77-year-old male with
ADPKD and long-standing hypertension, admitted to
our ward due to extreme weakness, malaise and
abdominal pain with severe anemia and elevated
serum levels of creatinine and urea. Treatments with
hemodialysis and blood substitution were started.
Abdomen echo-sonography showed hepatic cysts and
polycystic kidneys. The cysts were filled with clear
content, in the right kidney toward the upper pole,
two larger cysts were notified and next to them а
pulsatile cystic lesion was noted with hemorrhagicfilled content which was highly suspicious for an
aneurismaticlly dilated abdominal aorta. CT angiography of the aorta showed dilated, tortuous aorta with advanced atherosclerosis along its entire length.
The dilatation was evident in the descending part of
the aorta, with infrarenal saccular dilatation before
the bifurcation, that seemed to be thrombosed and
next to it denser content was observed, probably
older hemorrhagy, without imaging signs of acute
extravasation of the contrast. Cardiovascular surgeon
recommended coronography and coronary artery
aneurisms were excluded. Unfortunantly the patient
started to alternete with his consuosnes and brain CT
angiography showed corticoreductive changes, no
aneurism, extra-or intra-axial hemorrhage were observed. Due to the severe general condition, clinical
assessment and advanced age of the patient, the case
was declared as inoperable.
Conclusion. Due to hypertension and associated
connective tissue disorders patients with ADPKD are
prone to develop aortic aneurysms, and should be
questioned as a frequent feature in such patients,
hence early diagnosis and treatment decisions based
on a risk–benefit analysis, remains the cornerstone of
management.
POLYCYSTIC KIDNEY DISEASE AND ABDOMINAL
AORTIC ANEURYSM
Z. Shterjova Markovska1, I. Rambabova Bushljetikj1, G.
Severova1, L. Trajcevska1, I. Nikolov1, V. Karanfilovski1,
S. Sulejman1, J. Usprcov1, Z. Janevski1, A. Canevska1, A.
Karanfilovikj1, S. Krstevska Balkanov2, G. Spasovski1
1University clinic of Nephrology, 2University clinic of
Hematology, Faculty of Medicine, University Sts. Cyril
and Methodius Skopje, N. Macedonia
PP
Introduction. Autosomal dominant polycystic kidney
disease (ADPKD) is a systemic disease with multiple
cysts in several organs. Formation of aneurysms of:
the aorta, coronary and cerebral arteries are increasingly reported in the literature as extra-renal
manifestations.
Case report. We report 77-year-old male with
ADPKD and long-standing hypertension, admitted to
our ward due to extreme weakness, malaise and
abdominal pain with severe anemia and elevated
serum levels of creatinine and urea. Treatments with
hemodialysis and blood substitution were started.
Abdomen echo-sonography showed hepatic cysts and
polycystic kidneys. The cysts were filled with clear
content, in the right kidney toward the upper pole,
two larger cysts were notified and next to them а
pulsatile cystic lesion was noted with hemorrhagicfilled content which was highly suspicious for an
aneurismaticlly dilated abdominal aorta. CT angiography of the aorta showed dilated, tortuous aorta with advanced atherosclerosis along its entire length.
The dilatation was evident in the descending part of
the aorta, with infrarenal saccular dilatation before
the bifurcation, that seemed to be thrombosed and
next to it denser content was observed, probably
older hemorrhagy, without imaging signs of acute
extravasation of the contrast. Cardiovascular surgeon
recommended coronography and coronary artery
aneurisms were excluded. Unfortunantly the patient
started to alternete with his consuosnes and brain CT
angiography showed corticoreductive changes, no
aneurism, extra-or intra-axial hemorrhage were observed. Due to the severe general condition, clinical
assessment and advanced age of the patient, the case
was declared as inoperable.
Conclusion. Due to hypertension and associated
connective tissue disorders patients with ADPKD are
prone to develop aortic aneurysms, and should be
questioned as a frequent feature in such patients,
hence early diagnosis and treatment decisions based
on a risk–benefit analysis, remains the cornerstone of
management.
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