The coexistence of acute aortic dissection with autosomal dominant polycystic kidney disease - description of two hypertensive patients

Pęczkowska, Mariola; Januszewicz, Andrzej; Grzeszczak, W; Moczulski, Dariusz; Janaszek−Sitkowska, Hanna; Kabat, Marek; Biederman, Andrzej; Hendzel, Piotr; Prejbisz, Aleksander; Cendrowska-Demkow, Iwona; Zieliński, Tomasz; Januszewicz, Magdalena

doi:10.1080/08037050410016492

Cited by 10 publications

(7 citation statements)

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“…The study selection process is represented in Figure 3. We report data from 27 cases ( Table 1) of ADPKD patients with AD, of which 25 were previously identified from the literature [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] and two from our clinical experience. A schematic representation of age, hypertension and CKD stage distribution in the study population is reported in the Figure 4.…”

Section: Resultsmentioning

confidence: 99%

“…However, additional manifestations are often described, including: intracranial, coronary and other arterial aneurysms, arterial dolichoectasias, aortic or cervicocephalic arteries dissections, and heart valve diseases . Although ADPKD‐AD association has been previously reported, only few cases are available in the literature and a systematic review has never been made. We report two cases of AD in ADPKD patients from our experience and perform a systematic analysis of the literature.…”

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confidence: 99%

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Aortic dissection in patients with autosomal dominant polycystic kidney disease: A series of two cases and a review of the Literature

et al. 2015

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ABSTRACT:Aortic dissection (AD) is the most common life-threatening disease involving the aorta. It is rarely associated with systemic disorders such as Autosomal Dominant Polycystic Kidney Disease (ADPKD), a genetic syndrome characterized by cystic degeneration of kidneys, possible presence of cysts in other organs and extra-renal manifestations, including cardiovascular disorders. We performed a systematic literature search focused on the occurrence of AD associated with ADPKD (25 cases identified), and reported two cases from our experience. We selected data on sex, age, family history of ADPKD and/or AD, habitus, hypertension, renal function, presence of hepatic/pancreatic/splenic cysts, clinical presentation of AD, AD type according to the Stanford classification, treatment and outcome. Furthermore we compared this dataset with the data of the overall population with AD from the International Registry of Acute Aortic Dissection (IRAD). Stanford A type AD was documented in 62% of patients. As expected, the initial manifestation of AD was most commonly chest and back pain (80%). The mean age of AD occurrence appears significantly reduced in ADPKD patients compared to the general population with AD (49 ± 12 vs 62 ± 14, P < 0.001). Of note, our analysis shows a remarkably higher frequency of hypertension (90%) compared to the overall AD population (75%), although not significantly (P = 0.133). AD should be always ruled out in ADPKD subjects with chest and back pain symptoms, despite them being young and at lower risk. A careful preventive monitoring as well as an optimal blood pressure control may reduce the risk of AD and improve the outcome of these patients.

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Section: Resultsmentioning

confidence: 99%

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confidence: 99%

Aortic dissection in patients with autosomal dominant polycystic kidney disease: A series of two cases and a review of the Literature

et al. 2015

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“…PKD1 may also have a role in the development and function in endothelial- and mesenchymal-derived cells. PKD1 mutations lead to vascular (intracranial and aortic aneurysms) [8], [9], [10], [11] and lung (bronchiectasis) abnormalities [12]. PKD1 and PKD2 are required for placental development [13].…”

Section: Introductionmentioning

confidence: 99%

Conditional Mesenchymal Disruption of Pkd1 Results in Osteopenia and Polycystic Kidney Disease

et al. 2012

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Conditional deletion of Pkd1 in osteoblasts using either Osteocalcin(Oc)-Cre or Dmp1-Cre results in defective osteoblast-mediated postnatal bone formation and osteopenia. Pkd1 is also expressed in undifferentiated mesenchyme that gives rise to the osteoblast lineage. To examine the effects of Pkd1 on prenatal osteoblast development, we crossed Pkd1 flox/flox and Col1a1(3.6)-Cre mice, which has been used to achieve selective inactivation of Pkd1 earlier in the osteoblast lineage. Control Pkd1 flox/flox and Pkd1 flox/+, heterozygous Col1a1(3.6)-Cre;Pkd1 flox/+ and Pkd1 flox/null, and homozygous Col1a1(3.6)-Cre;Pkd1 flox/flox and Col1a1(3.6)-Cre;Pkd1 flox/null mice were analyzed at ages ranging from E14.5 to 8-weeks-old. Newborn Col1a1(3.6)-Cre;Pkd1 flox/null mice exhibited defective skeletogenesis in association with a greater reduction in Pkd1 expression in bone. Conditional Col1a1(3.6)-Cre;Pkd1 flox/+ and Col1a1(3.6)-Cre;Pkd1 flox/flox mice displayed a gene dose-dependent decrease in bone formation and increase in marrow fat at 6 weeks of age. Bone marrow stromal cell and primary osteoblast cultures from homozygous Col1a1(3.6)-Cre;Pkd1 flox/flox mice showed increased proliferation, impaired osteoblast development and enhanced adipogenesis ex vivo. Unexpectedly, we found evidence for Col1a1(3.6)-Cre mediated deletion of Pkd1 in extraskeletal tissues in Col1a1(3.6)-Cre;Pkd1 flox/flox mice. Deletion of Pkd1 in mesenchymal precursors resulted in pancreatic and renal, but not hepatic, cyst formation. The non-lethality of Col1a1(3.6)-Cre;Pkd1 flox/flox mice establishes a new model to study abnormalities in bone development and cyst formation in pancreas and kidney caused by Pkd1 gene inactivation.

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“…3,7,8) Among the vascular manifestations of ADPKD, relatively few cases of thoracic aortic dissection have been published in English literature, [8][9][10][11][12][13][14][15][16][17] when compared with reports of intracranial aneurysms, which are well-established extrarenal manifestations of ADPKD and occur in approximately 6% of patients without a family history of aneurysms and in 16% of those with such a family history. 18) Marfan syndrome is a well-known systemic connective tissue disorder that leads to life-threatening cardiovascular complications.…”

Section: )mentioning

confidence: 99%

“…11) Some authors have warned the medical community of the presence of aortic dissection in patients with ADPKD and concomitant hypertension. 10,11,13) Through these reports, physicians must pay attention to the possibility of devastating entity behind ADPKD.…”

Section: )mentioning

confidence: 99%

Dissecting Aneurysm in A Patient with Autosomal Dominant Polycystic Kidney Disease

Fukunaga

Yuzaki

Nasu

et al. 2012

ATCS

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Autosomal dominant polycystic kidney disease (ADPKD) is primarily associated with renal failure, but it also causes systemic diseases, including cysts of other systemic organs and cerebral or visceral aneurysm. To make matters worse, life-threatening aortic diseases are associated with ADPKD in some cases. However, only a few reports of ADPKD-associated with thoracic aortic dissection have been published. Herein, we present a case of dissecting aneurysm in a patient with hypertension and ADPKD. He had been followed up for type B aortic dissection for six years. Preoperative creatinine level ranged from 2.1 to 2.4 mg/dl. We performed replacement of the thoracic aorta with prosthetic graft successfully, and postoperatively, dialysis was not required. It is very important for us to recognize the relationship between ADPKD and thoracic aortic dissection, which can cause high mortality and morbidity rates.

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The coexistence of acute aortic dissection with autosomal dominant polycystic kidney disease - description of two hypertensive patients

Abstract: Aortic dissection is a rare but potentially disastrous complication, and clinicians should always consider this when dealing with patients with ADPKD who present with chest pain or collapse.

Cited by 10 publications

References 11 publications

Aortic dissection in patients with autosomal dominant polycystic kidney disease: A series of two cases and a review of the Literature

Aortic dissection in patients with autosomal dominant polycystic kidney disease: A series of two cases and a review of the Literature

Conditional Mesenchymal Disruption of Pkd1 Results in Osteopenia and Polycystic Kidney Disease

Dissecting Aneurysm in A Patient with Autosomal Dominant Polycystic Kidney Disease

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