scholarly journals MANEJO Y SEGUIMIENTO CLÍNICO DE PACIENTE CON SINDROME DE ALPORT FASE IV

2021 ◽  
Vol 32 (1) ◽  
pp. s1-s2
Author(s):  
Cesar Igor Vásconez ◽  
Alejandro Xavier Campoverde ◽  
Victoria Trejo Martínez

Introduction Alport syndrome (AS) is a hereditary disease of the basement membranes caused by mutations in type IV collagen. This disease is characterized by the presence of progressive hereditary nephropathy associated with sensory deafness, as well as ocular lesions. AS has an incidence of 1 in every 50,000 live births, constituting 1 to 2% of the cause of chronic kidney disease (CKD) in Europe and 3% of CKD in the American pediatric population; it is considered to be the cause of terminal uremia in 0.6 to 4.6% of terminal patients in the United States and Europe. At the moment there is no casuistry in this regard in Ecuador. At present there is no specific treatment for Alport Syndrome, however the drugs Angiotensin Converting Enzyme Inhibitors (ACEI) and angiotensin II receptor antagonists (ARBs) have demonstrated efficacy and safety over blocking the system. renin angiotensin aldosterone, thus reducing the presence of proteinuria and slowing the deterioration of CKD. Case description A 28-year-old male patient with a personal history of HT and 6-member SA relatives: 2 nephews, 1 brother and 3 carrier nieces. The physical examination revealed the presence of moderate bilateral hearing loss. He presents kidney disease from the age of 10 without adequate control. Alport Syndrome was diagnosed in 2012 by means of a renal biopsy, evidence of hematuria and by family history. He is currently receiving Hemodialysis treatment in a daily short session modality, which consists of 5 weekly sessions from Monday to Friday of 2 hours each; he is kept under hypotensive treatment of 10mg QD of Amlodipine (Calcium Channel Blocker), and 10mg BID of Carvedilol (Beta Blocker), together with 12.5mg BID of Losartan (Angiotensin II Receptor Antagonist), The patient was followed up throughout the year 2020 until January 2021, calculating the Glomerular Filtration Rate month by month to be able to show whether there is a significant progressive deterioration of his residual kidney function. Conclusion It is mentioned that the residual renal function has not been affected thanks to the control of the Arterial Hypertension of the patient where an ARA II drug (Losartan) is added, which slows the progressive renal deterioration that is typical of Alport Syndrome. The incorporation of the patient to the modality of daily short session of hemodialysis has helped to have a compensation of the renal function, obtaining a continuous clearance, resembling the function of a healthy kidney as closely as possible. In order for a hemodialysis treatment to have better results compensating for residual renal function, consecutive and longer sessions should be established, however this is a complicated issue depending on the availability of time and tolerance of the patient.

2004 ◽  
Vol 43 (6) ◽  
pp. 1056-1064 ◽  
Author(s):  
Hiromichi Suzuki ◽  
Yoshihiko Kanno ◽  
Soichi Sugahara ◽  
Hirokazu Okada ◽  
Hidetomo Nakamoto

Toxins ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 289
Author(s):  
Adamasco Cupisti ◽  
Piergiorgio Bolasco ◽  
Claudia D’Alessandro ◽  
Domenico Giannese ◽  
Alice Sabatino ◽  
...  

The retention of uremic toxins and their pathological effects occurs in the advanced phases of chronic kidney disease (CKD), mainly in stage 5, when the implementation of conventional thrice-weekly hemodialysis is the prevalent and life-saving treatment. However, the start of hemodialysis is associated with both an acceleration of the loss of residual kidney function (RKF) and the shift to an increased intake of proteins, which are precursors of uremic toxins. In this phase, hemodialysis treatment is the only way to remove toxins from the body, but it can be largely inefficient in the case of high molecular weight and/or protein-bound molecules. Instead, even very low levels of RKF are crucial for uremic toxins excretion, which in most cases are protein-derived waste products generated by the intestinal microbiota. Protection of RKF can be obtained even in patients with end-stage kidney disease (ESKD) by a gradual and soft shift to kidney replacement therapy (KRT), for example by combining a once-a-week hemodialysis program with a low or very low-protein diet on the extra-dialysis days. This approach could represent a tailored strategy aimed at limiting the retention of both inorganic and organic toxins. In this paper, we discuss the combination of upstream (i.e., reduced production) and downstream (i.e., increased removal) strategies to reduce the concentration of uremic toxins in patients with ESKD during the transition phase from pure conservative management to full hemodialysis treatment.


2018 ◽  
pp. 107-118 ◽  
Author(s):  
V. I. Podzolkov ◽  
A. E. Bragina ◽  
T. I. Ishina ◽  
L. V. Bragina ◽  
L. V. Vasilyeva

The current population is characterized by a high prevalence of risk factors for the development of chronic kidney disease: hypertension, diabetes, obesity, metabolic syndrome, physical inactivity, smoking. The development of severe complications and a close connection with potentially fatal cardiovascular disorders make this disease a socially and economically significant problem. Treatment of chronic kidney disease in advanced stages belong to nephrologist duties. However, the success of preventive interventions depends on the time of their onset, which makes it relevant to identify the disease. The use of nephroprotective approaches by physicians of different specialties (general practitioners, cardiologists, gerontologists, nephrologists, endocrinologists) can significantly improve the prognosis of both those at risk of developing renal dysfunction and the existing disease. The review presents data on the clinical and laboratory efficacy of angiotensin-renin blocker use, as well as the combination of angiotensin II receptor blockers with calcium antagonists. Using the combination of the angiotensin II receptor blocker irbesartan and amlodipine as an example, we demonstrated the possibilities of nephroprotective therapy in patients with renal dysfunction.


Author(s):  
Kathleen E. Adair ◽  
Jeffery L. Heileson ◽  
Matthew N. Peterson ◽  
Rodney G. Bowden ◽  
Jeffrey S. Forsse

Objective: Dietary guidelines from the Kidney Disease Outcomes Quality Initiative (KDOQI) and the United States Department of Agriculture (USDA) are advised to individuals with mid-spectrum (stages G3a and G3b) chronic kidney disease (CKD), yet typical diets in individuals with CKD remain understudied. The purpose of this study is to assess the self-reported dietary pattern of subjects with diagnosed mid-spectrum CKD and compare the normal dietary intakes to the KDOQI and USDA recommendations. Methods: A cross-sectional analysis of 20 participants with mid-spectrum CKD (n = 6 male [M]; n = 14 female [F]) was conducted to assess subjects’ self-reported dietary intakes for an average of 5 days. Micro and macronutrient analyses were compared to the KDOQI and USDA guidelines by sex to assess nutrition, and an exploratory stepwise multiple linear regression model was used to identify predictors of poor renal function;p-values were considered significant at the α = 0.05 level. Results: All subjects met the recommended caloric intake, but the average consumptions of protein (F = 0.86 ± 0.29g/kg body weight/day, M = 1.18 ± 0.45g/kg body weight/day), saturated fat (F = 12.17 ± 2.28%, M = 13.86 ± 1.20%), and sodium (F = 3.78 ± 2.51g, M = 4.21 ± 0.39g) were high (p < 0.05 for all). The average fiber intakewas low (F = 13.64 ± 4.09g, M = 14.82 ± 7.28g) as well as folate, vitamins D and K, zinc, and calcium intakes compared with the recommendations (p < 0.05 for all). The only significant contributor to higher renal function in the exploratory regression analysis was male sex (p = 0.035).


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