Cystic Fibrosis Kidney Disease: 10 Tips for Clinicians

Nowakowski, Alexandra C. H.

doi:10.3389/fmed.2018.00242

Cited by 4 publications

(6 citation statements)

References 18 publications

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“…The above-mentioned causes can significantly accelerate the disease-related decline in the glomerular filtration rate. Additionally, treating lung disease in CF may result in toxicity and drug-drug interaction complications, especially with aminoglycosides [25,26]. Renal pathologies in CF patients have been considered rare, but recently, renal problems such as diabetic glomerulopathy, amyloidosis, postinfectious glomerulonephritis, membranous nephropathy, chronic interstitial nephropathy or tubular damage have been observed more and more often [27].…”

Section: Discussionmentioning

confidence: 99%

Renal Function in Patients with Cystic Fibrosis: A Single-Center Study

Rachel

Galiniak

Biesiadecki

et al. 2022

IJERPH

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Cystic fibrosis (CF) is the most common incurable autosomal recessive disease affecting the Caucasian population. As the prognosis for life extension of CF patients improves, co-morbidities, including kidney disease, become more common. Identifying those at the highest risk of kidney injury is therefore extremely important. The aim of this study was to evaluate the biomarkers of renal function in 50 CF patients using the estimated glomerular filtration rate (eGFR) based on creatinine and cystatin C equation as well as serum creatinine (sCr), serum cystatin C (CysC), serum urea and urinary neutrophil gelatinase-associated lipocalin (uNGAL) concentrations. sCr, CysC, urea and uNGAL were estimated. eGFR was calculated according to the CKD-EPI formula. CysC was significantly increased, while eGFR was significantly lower in the CF group than in the controls (p < 0.001 and p < 0.01, respectively). There was no significant difference in the sCr, urea and uNGAL concentrations between patients with CF and healthy subjects. For the purpose of our analysis, in order to assess renal function in patients with CF in clinical practice, the concentration of serum CysC and eGFRCKD-EPI should be determined. Patients with CF presented with renal function impairment pictured by increased serum CysC and decreased eGFR values compared to controls. Unchanged uNGAL concentrations suggested preserved tubular function despite aminoglycoside treatment. Further prospective studies are needed to clarify whether kidney impairment observed in the course of CF progresses.

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Section: Discussionmentioning

confidence: 99%

Renal Function in Patients with Cystic Fibrosis: A Single-Center Study

Rachel

Galiniak

Biesiadecki

et al. 2022

IJERPH

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“…The low prevalence is presumably due to the onset of CFKD rather late in life. Along with improving treatments and increased longevity, however, the prevalence of kidney complications has been on the rise, and about 20% of pwCF over the age of 55 years have CFKD (Lai et al, 2019; Nazareth & Walshaw, 2013; Nowakowski, 2018; Schechter & Stecenko, 2011).…”

Section: Models Of Other Organs and Tissues Affected Later In Lifementioning

confidence: 99%

“…The spectrum of renal diseases in adult patients with CF has proven to be heterogeneous (Yahiaoui et al, 2009). Symptoms of CFKD include pain in the lower back, fluid retention in the lower body, electrolyte imbalance, asymptomatic nephrocalcinosis, hypercalciuria, nephritis, renal stones, and bacterial infections (Nowakowski, 2018;Yahiaoui et al, 2009). To ameliorate the disease, and possibly avoid transplantation, it is necessary that the disease is diagnosed in a timely manner.…”

Section: Kidneysmentioning

confidence: 99%

Mathematical models of cystic fibrosis as a systemic disease

Olivença

Davis

Kumbale

et al. 2023

WIREs Mechanisms of Disease

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Cystic fibrosis (CF) is widely known as a disease of the lung, even though it is in truth a systemic disease, whose symptoms typically manifest in gastrointestinal dysfunction first. CF ultimately impairs not only the pancreas and intestine but also the lungs, gonads, liver, kidneys, bones, and the cardiovascular system. It is caused by one of several mutations in the gene of the epithelial ion channel protein CFTR. Intense research and improved antimicrobial treatments during the past eight decades have steadily increased the predicted life expectancy of a person with CF (pwCF) from a few weeks to over 50 years. Moreover, several drugs ameliorating the sequelae of the disease have become available in recent years, and notable treatments of the root cause of the disease have recently generated substantial improvements in health for some but not all pwCF. Yet, numerous fundamental questions remain unanswered. Complicating CF, for instance in the lung, is the fact that the associated insufficient chloride secretion typically perturbs the electrochemical balance across epithelia and, in the airways, leads to the accumulation of thick, viscous mucus and mucus plaques that cannot be cleared effectively and provide a rich breeding ground for a spectrum of bacterial and fungal communities. The subsequent infections often become chronic and respond poorly to antibiotic treatments, with outcomes sometimes only weakly correlated with the drug susceptibility of the target pathogen. Furthermore, in contrast to rapidly resolved acute infections with a single target pathogen, chronic infections commonly involve multi‐species bacterial communities, called “infection microbiomes,” that develop their own ecological and evolutionary dynamics. It is presently impossible to devise mathematical models of CF in its entirety, but it is feasible to design models for many of the distinct drivers of the disease. Building upon these growing yet isolated modeling efforts, we discuss in the following the feasibility of a multi‐scale modeling framework, known as template‐and‐anchor modeling, that allows the gradual integration of refined sub‐models with different granularity. The article first reviews the most important biomedical aspects of CF and subsequently describes mathematical modeling approaches that already exist or have the potential to deepen our understanding of the multitude aspects of the disease and their interrelationships. The conceptual ideas behind the approaches proposed here do not only pertain to CF but are translatable to other systemic diseases.This article is categorized under: Congenital Diseases > Computational Models

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“…The deterioration of fibrosis can lead to the destruction of organ structure and functional decline or even failure, which significantly threatens human health ( 58 ). Kidney fibrosis is the main pathological feature of chronic kidney failure and manifests as tissue structure disorganisation ( 59 , 60 ). Piñeiro-Hermida et al ( 61 ) revealed an association between the development of idiopathic pulmonary fibrosis and telomere dysfunction and telomerase mutations.…”

Section: Kidney Fibrosismentioning

confidence: 99%

Roles of telomeres and telomerase in age‑related renal diseases (Review)

Wang

et al. 2020

Mol Med Rep

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age-related renal diseases, which account for various progressive renal disorders associated with cellular and organismal senescence, are becoming a substantial public health burden. However, their aetiologies are complicated and their pathogeneses remain poorly understood. Telomeres and telomerase are known to be essential for maintaining the integrity and stability of eukaryotic genomes and serve crucial roles in numerous related signalling pathways that activate renal functions, such as repair and regeneration. Previous studies have reported that telomere dysfunction served a role in various types of age-related kidney disease through various different molecular pathways. The present review aimed to summarise the current knowledge of the association between telomeres and ageing-related kidney diseases and explored the contribution of dysfunctional telomeres to these diseases. The findings may help to provide novel strategies for treating patients with renal disease. Contents 1. introduction 2. Structural renal changes induced by ageing 3. rcc 4. ageing-associated cKd 5. Kidney fibrosis 6. aKi 7. renal cysts 8. conclusion

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Cystic Fibrosis Kidney Disease: 10 Tips for Clinicians

Cited by 4 publications

References 18 publications

Renal Function in Patients with Cystic Fibrosis: A Single-Center Study

Renal Function in Patients with Cystic Fibrosis: A Single-Center Study

Mathematical models of cystic fibrosis as a systemic disease

Roles of telomeres and telomerase in age‑related renal diseases (Review)

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