Fabry’s disease: an example of cardiorenal syndrome type 5

Sharma, Aashish; Sartori, Marco; Zaragoza, José J.; Villa, Gianluca; Lu, Renhua; Faggiana, Elena; Brocca, Alessandra; Lullo, Luca Di; Feriozzi, Sandro; Ronco, Claudio

doi:10.1007/s10741-015-9500-0

Cited by 14 publications

(10 citation statements)

References 200 publications

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“…However, we only recruited patients with a maintained renal function because of the need for gadoliniumbased enhancement. Previous reports have suggested that renal dysfunction might be related to the severity of heart involvement [27,28]. Therefore, an estimation method other than that based on LGE is necessary in order to evaluate the cardiac condition of patients with renal dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Clinical findings of gadolinium-enhanced cardiac magnetic resonance in Fabry patients

Nojiri

Anan

Morimoto

et al. 2020

Journal of Cardiology

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Recently, we reported that nearly one-third of males and onefourth of females had LVH among Fabry patients prior to enzyme replacement therapy (ERT) [9]. We also showed that the appearance of LVH was age-dependent, and the progression rate of left ventricular mass (LVM) was higher in male patients than in female patients [9,10]. LVH itself is a key manifestation of a heart A B S T R A C T Background: Fabry disease is one of the causes of left ventricular hypertrophy (LVH) and can be treated with enzyme replacement therapy or pharmacological chaperone therapy. Late gadolinium enhancement (LGE) in cardiac magnetic resonance (CMR) can identify myocardial fibrosis and be used for the stratification in LVH. However, the details of the prevalence and characteristics of LGE in Japanese Fabry patients have not been reported. Methods: We evaluated myocardial involvement in 26 Fabry patients (10 males, 16 females) using gadolinium-enhanced CMR. LGE areas were analyzed using the previously reported scoring method. Echocardiography was also performed to evaluate the left ventricular function and left ventricular mass. Results: LGE on CMR images was positive in 5 out of 26 patients, and all patients with LGE-positive findings suffered from LVH (2 out of 5 male patients and 3 out of 4 female patients with LVH on echocardiography). LGE was specifically localized at the mid-wall in the infero-lateral area of the left ventricle.LGE-positive patients seemed to be older, and tended to have a larger left ventricular mass index and higher B-type natriuretic peptide level than LGE-negative patients. Conclusions: These results revealed that specific localization of LGE was present in Fabry patients.

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Section: Discussionmentioning

confidence: 99%

Clinical findings of gadolinium-enhanced cardiac magnetic resonance in Fabry patients

Nojiri

Anan

Morimoto

et al. 2020

Journal of Cardiology

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“…CRS type 5 or secondary is characterized when cardiac and renal injury coincides, resulting from a common underlying pathological process such as connective tissue disorders or sepsis[ 25 , 26 ]. Despite the existence of the underlying disease, the hemodynamic and pro-inflammatory mediators are similar to those in the other types[ 27 ].…”

Section: Cardiorenal Syndrome: Typesmentioning

confidence: 99%

Range of adiposity and cardiorenal syndrome

Pazos

2020

WJD

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Obesity and obesity-related co-morbidities, diabetes mellitus, and hypertension are among the fastest-growing risk factors of heart failure and kidney disease worldwide. Obesity, which is not a unitary concept, or a static process, ranges from alterations in distribution to the amount of adiposity. Visceral adiposity, which includes intraabdominal visceral fat mass and ectopic fat deposition such as hepatic, cardiac, or renal, was robustly associated with a greater risk for cardiorenal morbidity than subcutaneous adiposity. In addition, morbid obesity has also demonstrated a negative effect on cardiac and renal functioning. The mechanisms by which adipose tissue is linked with the cardiorenal syndrome (CRS) are hemodynamic and mechanical changes, as well neurohumoral pathways such as insulin resistance, endothelial dysfunction, nitric oxide bioavailability, renin-angiotensin-aldosterone, oxidative stress, sympathetic nervous systems, natriuretic peptides, adipokines and inflammation. Adiposity and other associated co-morbidities induce adverse cardiac remodeling and interstitial fibrosis. Heart failure with preserved ejection fraction has been associated with obesity-related functional and structural abnormalities. Obesity might also impair kidney function through hyperfiltration, increased glomerular capillary wall tension, and podocyte dysfunction, which leads to tubulointerstitial fibrosis and loss of nephrons and, finally, chronic kidney disease. The development of new treatments with renal and cardiac effects in the context of type 2 diabetes, which improves mortality outcome, has highlighted the importance of CRS and its prevalence. Increased body fat triggers cellular, neuro-humoral and metabolic pathways, which create a phenotype of the CRS with specific cellular and biochemical biomarkers. Obesity has become a single cardiorenal umbrella or type of cardiorenal metabolic syndrome. This review article provides a clinical overview of the available data on the relationship between a range of adiposity and CRS, the support for obesity as a single cardiorenal umbrella, and the most relevant studies on the recent therapeutic approaches.

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“…sepsis, diabetes mellitus, hypertension and sarcoidosis) cause simultaneous dysfunction of the heart and kidneys due to different pathophysiological mechanisms (Mehta et al, 2013;Sharma et al, 2015). sepsis, diabetes mellitus, hypertension and sarcoidosis) cause simultaneous dysfunction of the heart and kidneys due to different pathophysiological mechanisms (Mehta et al, 2013;Sharma et al, 2015).…”

Section: Type 5: Other Diseases Affecting Both the Kidney And The Heartmentioning

confidence: 99%

“…Type 5: other diseases affecting both the kidney and the heart Several systemic conditions (e.g. sepsis, diabetes mellitus, hypertension and sarcoidosis) cause simultaneous dysfunction of the heart and kidneys due to different pathophysiological mechanisms (Mehta et al, 2013;Sharma et al, 2015).…”

Section: Type 3: Renal Worsening Leading To Acute Cardiac Dysfunctionmentioning

confidence: 99%

Mechanisms in fluid retention – towards a mutual concept

Mottelson

Lundsgaard

Möller

2019

Clin Physio Funct Imaging

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Fluid retention is a common and challenging condition in daily clinical practice. The normal fluid homoeostasis in the human body is based on accurately counter-balanced physiological mechanisms. When compromised fluid retention occurs and is seen in pathophysiologically different conditions such as liver cirrhosis, heart and kidney failure, and in preeclampsia. These conditions may share pathophysiological mechanisms such as functional arterial underfilling, which seems to be a mutual element in cirrhosis, cardiac failure, cardiorenal and hepatorenal syndromes, and in pregnancy. However, there are also distinct differences and it is still unclear whether kidney dysfunction or arterial underfilling is the initiating factor of fluid retention or if they happen simultaneously. This review focuses on similarities and differences in water retaining conditions and points to areas where important knowledge is still needed.

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Fabry’s disease: an example of cardiorenal syndrome type 5

Cited by 14 publications

References 200 publications

Clinical findings of gadolinium-enhanced cardiac magnetic resonance in Fabry patients

Clinical findings of gadolinium-enhanced cardiac magnetic resonance in Fabry patients

Range of adiposity and cardiorenal syndrome

Mechanisms in fluid retention – towards a mutual concept

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