scholarly journals Adult Patients Affected by Cystic Fibrosis in Therapy with Cystic Fibrosis Transmembrane Regulator Modulators and Lung Transplant: Renal Function, Metabolic and Nutritional Status

2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Silvia Lai ◽  
Sandro Mazzaferro ◽  
Anna Paola Mitterhofer ◽  
Francesca Tinti ◽  
Enea Bonci ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Renal Function ◽  
Nutritional Status ◽  
Lung Transplant ◽  
Conservative Therapy ◽  
Control Group ◽  
Inflammatory Status ◽  
Significant Difference ◽  
Cystic Fibrosis Transmembrane ◽  
Cftr Modulators

Background. Cystic fibrosis (CF) is one of the most frequent genetic diseases. The discovery and implementation of new therapies prolonged the survival of CF patients in the last years. Evaluation of long-term complications could be useful to improve the outcome of these patients. Aim of the Study. To evaluate renal function, metabolic, nutritional, and inflammatory status in CF patients on cystic fibrosis transmembrane regulator (CFTR) modulators therapy as well as lung transplant recipients (LRs) and patients on conservative therapy (control group). Materials and Methods. We performed a prospective, longitudinal study on 69 CF patients. Clinical and laboratory parameters (metabolic and nutritional indices and inflammatory markers) were evaluated in all patients before starting CFTR therapy or transplant (T0) and after 3 years (T1). Results. We enrolled 69 CF patients (42 males). Patients were distributed into three groups. The average age was 35.01 ± 10.57 years for the control group (group 0), 32.47 ± 9.40 years for patients on CFTR modulators therapy (group 1), and 38.93 ± 7.14 years for LRs (group 2). At T1, we showed a significant difference among the three groups in terms of renal function indices: creatinine, eGFR, serum nitrogen as well as serum uric acid, sodium, and potassium ( p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , and p < 0.001 , respectively), particularly in LRs patients. Significant differences were found in nutritional status parameters among the three groups: total protein, serum albumin, serum fibrinogen, serum transferrin, and white blood cell counts ( p < 0.001 , p = 0.037 , p = 0.04 , p = 0.003 , and p = 0.007 , respectively), particularly in LRs compared with other groups. Moreover, we found significant differences in metabolic profile (HbA1c, p = 0.026 ) and inflammatory status, with a significant difference in C-reactive protein values, neutrophil counts, and neutrophil-lymphocyte ratio (NLR) among the three groups ( p < 0.001 , p = 0.005 , and p = 0.026 , respectively). Conclusions. Our study showed a reduced renal function and poor nutritional status in LRs, along with worse metabolic control. Moreover, we showed a lower inflammatory status in patients on CFTR modulators therapy. Therefore, we suggest early and careful monitoring of renal function, metabolic, and nutritional parameters in CF patients, whether they are on conservative therapy, CFTR modulators therapy, and LRs patients.

scholarly journals Serum chemerin level, cytokine profile and nutritional status in children with cystic fibrosis

2019 ◽  
Author(s):  
Katarzyna Sznurkowska ◽  
Katarzyna Kaźmierska ◽  
Tomasz Śledziński ◽  
Maciej Zagierski ◽  
Anna Liberek ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Interleukin 10 ◽  
Fat Free Mass ◽  
Control Group ◽  
Healthy Children ◽  
Mass Percentage ◽  
Tnf Α ◽  
Significant Difference ◽  
Factor Α

Background: Cystic fibrosis (CF) is characterized by malnutrition and chronic inflammation predominantly occurring in lungs. Evidence suggests a relation between inflammatory activity and nutritional status. Proinflammatory cytokines, playing crucial role in pulmonary destruction in CF, are regarded as a component of the pathogenesis of illness-related malnutrition. Chemerin - a novel marker of a crosstalk between nutrition and inflammation, has not been investigated in children with cystic fibrosis. The aim of this study was to determine serum level of chemerin, interleukin-1b (IL-1b), interleukin-6 (IL-6), tumor necrosing factor α (TNF-α) and interleukin-10 (IL-10) and to verify if they correlate with the nutritional status in children with CF. Methods: The study included 72 pediatric patients with cystic fibrosis. The control group was comprised of 30 healthy children. Nutritional status parameters: Body Mass Index (BMI), fat mass percentage (FM %) and fat free mass percentage (FFM%) have been assessed in all the subjects basing on bioimpedance and anthropometry according to Slaughter. Serum concentrations of chemerin and cytokines were estimated with ELISA. Results: No statistically significant difference in serum chemerin was found between the studied and the control group. We have documented a significantly higher level of IL-1b, IL-6, TNF-α and IL-10 in CF patients when compared to healthy controls. Neither the chemerin nor the cytokine levels correlated with parameters of nutritional status in our cohort. No statistically significant correlation was found between the serum chemerin and the inflammatory cytokines: IL-1b, IL-6, and TNFα. Conclusions: Our results show that chemerin is not associated with the nutritional status in children with cystic fibrosis. Chemerin has no impact on the levels of IL-1b, IL-6, TNFα in CF patients. IL-1b, IL6, TNFα and also IL10 are upregulated in cystic fibrosis.

scholarly journals Clinical advantages of a newly launched anti-thrombotic PMMA membrane for the nutritional status and dialysis-related symptoms in older chronic dialysis patients: a multicenter pilot study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Ikuto Masakane ◽  
Ichiro Yamaguchi ◽  
Yuji Matsumoto ◽  
Ken Ataka ◽  
Yuta Oyama ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Nutritional Status ◽  
Randomized Clinical Trials ◽  
Hemodialysis Patients ◽  
University Hospital ◽  
Control Group ◽  
Dialysis Patients ◽  
Inflammatory Status ◽  
Significant Difference ◽  
Secondary Outcomes

Abstract Background Malnutrition that is associated with inflammation is a key factor of poor outcome in chronic hemodialysis patients, especially in older dialysis patients. Polymethyl methacrylate (PMMA) membrane has been reported to improve the inflammatory status by removing pro-inflammatory cytokines via adsorption. However, older dialysis patients occasionally have multiple uncomfortable dialysis-related symptoms, which decrease their quality of life and survival rate. We investigated whether a new PMMA membrane, Filtryzer NF, can improve malnutrition and dialysis-related symptoms in older hemodialysis patients. Methods Patients over 70 years of age who were dialyzed using a polysulfone (PS) membrane were enrolled and randomly allocated into one of two groups: control or NF. In the NF group, the PS dialyzers were changed to NF, whereas in the control group, the PS membrane was continuously used. The primary outcome was the malnutrition–inflammation score (MIS). Secondary outcomes were C-reactive protein, normalized protein catabolism rate, percent of creatinine generation rate, arm circumference, and eight dialysis-related symptoms. The primary and secondary outcomes were measured every 3 months for 1 year. Results Fifty-four hemodialysis patients were randomly assigned to the NF group (n = 28) or the control group (n = 26). During the 12-month study period, 11 and 10 patients were withdrawn from the NF and control groups, respectively. There was no significant difference in the MIS between the groups during the study or between the beginning and the end of the study within each group. For the secondary outcomes, there was a significant reduction in the total score of dialysis-related symptoms in the NF group but not in the control group. During the study period, the total dialysis-related symptoms score in the NF group was significantly decreased from 6 (range, 1–16) to 3 (range, 0–11) (median [minimum–maximum], p < 0.05). Other secondary outcomes were not different between the groups or between the beginning and the end of the study. Conclusions This study showed no advantage for the nutritional status in older hemodialysis patients using NF compared with PS. However, our results indicated that NF may improve several dialysis-related symptoms. To clarify this clinical finding, large-scale prospective randomized clinical trials are required. Trial registration This study was registered with the Clinical Trials Registry of the University Hospital Medical Information Network (registration ID, UMIN000032990).

scholarly journals Study of the effectiveness of supplementary feeding in patients with inherited enzyme deficiency

2019 ◽  
Vol 81 (2) ◽  
pp. 239-244
Author(s):  
L. V. Ulyanova ◽  
V. S. Ledneva ◽  
N. S. Burdina ◽  
M. I. Talykova ◽  
A. S. Ivannikova ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Pancreatic Enzyme ◽  
Diet Therapy ◽  
Enzyme Deficiency ◽  
Control Group ◽  
Anthropometric Parameters ◽  
Group 2 ◽  
The Right ◽  
Supplementary Nutrition

The successful provision of optimal nutrition in children with serious diseases depends on the right diet, as well as the addition of specialized mixtures for its correction. The aim of the study was to study the effectiveness of supplemental nutrition in hereditary fermentopathy (cystic fibrosis) in children. The work included a three-year follow-up of 69 children aged 3 to 15 years suffering from cystic fibrosis. Patients were divided into two groups: 37 patients with cystic fibrosis group 1, receiving a modified version of treatment with additional enteral nutrition and 32 patients with cystic fibrosis group 2, receiving traditional treatment. The study conducted a comparative analysis of changes in trophic status in patients using nutritional support mixture produced in the Russian Federation – "Nutrien-standard"in diet therapy. Laboratory, functional and anthropometric parameters were monitored once a month. A significant increase in the physical development of patients was obtained only in the second year of use in the diet of supplementary nutrition, p<0.05. At the 3rd year of treatment, the results of positive dynamics of nutritional status in both groups were confirmed, while the increase in body mass index in the main group was 6.7%, and in the control group-only 1% (p<0.05). It is noted that the use of this mixture allows to achieve positive dynamics of nutritional status in 27% of patients and in 73% of cases to completely eliminate it. The obtained results prove the expediency of additional use of "Nutrient-standard" mixture in the complex therapy of patients with hereditary pancreatic enzyme deficiency, cystic fibrosis

scholarly journals Progress in precision medicine in cystic fibrosis: a focus on CFTR modulator therapy

Breathe ◽  
2021 ◽  
Vol 17 (4) ◽  
pp. 210112
Author(s):  
Daniel H. Tewkesbury ◽  
Rebecca C. Robey ◽  
Peter J. Barry
Keyword(s):  
Cystic Fibrosis ◽  
Precision Medicine ◽  
Real World ◽  
Chloride Ion ◽  
Transmembrane Conductance Regulator ◽  
Therapeutic Approaches ◽  
Transmembrane Conductance ◽  
Cftr Protein ◽  
Cystic Fibrosis Transmembrane ◽  
Cftr Modulators

The genetic multisystem condition cystic fibrosis (CF) has seen a paradigm shift in therapeutic approaches within the past decade. Since the first clinical descriptions in the 1930s, treatment advances had focused on the downstream consequences of a dysfunctional cystic fibrosis transmembrane conductance regulator (CFTR) chloride ion channel. The discovery of the gene that codes for CFTR and an understanding of the way in which different genetic mutations lead to disruption of normal CFTR function have led to the creation and subsequent licensing of drugs that target this process. This marks an important move towards precision medicine in CF and results from clinical trials and real-world clinical practice have been impressive. In this review we outline how CFTR modulator drugs restore function to the CFTR protein and the progress that is being made in this field. We also describe the real-world impact of CFTR modulators on both pulmonary and multisystem complications of CF and what this will mean for the future of CF care.

Evaluation of Renal Function and Plasma Osmolality among Covid-19 Patients in Sudan

2021 ◽  
Vol 15 (12) ◽  
pp. 3576-3578
Author(s):  
Shomos A. Mubarak ◽  
Alneel A. A. Alameen ◽  
Husham O. Elzein ◽  
Mohamed Siddig Ibrahim ◽  
Maha B. Hassan ◽  
...  
Keyword(s):  
Renal Function ◽  
Elevated Serum ◽  
Plasma Osmolality ◽  
Serum Osmolality ◽  
Control Group ◽  
Plasma Urea ◽  
Significant Difference ◽  
The Mean ◽  
Serious Disease ◽  
Sodium And Potassium

Objective: COVID-19 is a very serious disease and is considered a pandemic by the WHO. The aim of this study is to determine the renal function and serum osmolality among COVID-19 patients in Khartoum state, Sudan. Method: This is retrospective study was conducted in Gebra Hospital, Khartoum state, Sudan from April 2021 to August 2021. The study enrolled 50 patients with Covid-19 and 50 healthy individuals as a control group. After all the enrolled participants signed the informed consent the samples were analyzed for Creatinine, Urea, Sodium, and potassium. By using an A25 fully automated chemistry analyzer, and serum Osmolality (mOsm / kg) was calculated according to Smithline and Gardner formula. Results: The study revealed a significant increase of the mean of Urea (P.value = 0.001), Creatinine (P.value = 0.001), however significant decrease in the mean of e GFR (P.value<0.05) on the first day of the admission. There was a significant increase in the mean of plasma Urea, Creatinine, and eGFR on the 7th day of admission when compared with the first day, P.value = (0.0001), (0.001), (0.001), respectively. The study revealed a significant difference in plasma Osmolality in COVID-19 patients (280.73 ±16.10 mOsm/kg) when compared with the control group (288.13 ±3.42 mOsm/kg), P.value = 0.0. Conclusion: COVID-19 does not affect only the lungs; it can also affect the kidney. This study concluded that there were an elevated serum creatinine, blood urea, and low glomerular filtration, and there was a significant difference in serum osmolality in COVID- 19 than in the healthy group. Keywords: COVID -19; Serum Osmolality; eGFR; Renal function; Sudan.

scholarly journals PREVALENCE OF HEPATIC STEATOSIS AMONG CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS AND ITS ASSOCIATION WITH NUTRITIONAL STATUS

2019 ◽  
Vol 37 (4) ◽  
pp. 435-441
Author(s):  
Amanda Oliva Gobato ◽  
Ana Carolina Junqueira Vasques ◽  
Antonio Fernando Ribeiro ◽  
Roberto Massao Yamada ◽  
Gabriel Hessel
Keyword(s):  
Cystic Fibrosis ◽  
Nutritional Status ◽  
Children And Adolescents ◽  
Hepatic Steatosis ◽  
Statistical Tests ◽  
The Body ◽  
Significant Difference ◽  
Group A ◽  
The Mean ◽  
Group B

ABSTRACT Objective: To determine the prevalence of hepatic steatosis (HS) in children and adolescents with cystic fibrosis (CF) and associate it with nutritional status. Methods: Cross-sectional study with children and adolescents with CF diagnosis. Weight and height were used to calculate the body mass index (BMI) and subsequent classification of the nutritional status. The midarm circumference (MAC), triceps skinfold thickness (TSF) and midarm muscle circumference (MAMC) were used to evaluate body composition. Abdominal ultrasonography was performed for diagnosis of HS. The statistical tests used were Student’s t test, Mann-Whitney test and chi-square test with significance level of 5%. Results: 50 patients with CF were evaluated, 18 (36%) were diagnosed with HS (Group A) and 32 (64%) without HS (Group B). The mean age of Group A was 13,2±4,9 years old and Group B 11,7±4,9; for BMI, the value for Group A was 18,0±4,1 and Group B was 15,7±3,8; the TSF of Group A was 8,4±3,5 mm and Group B was 7,0±2,5 mm. For these variables, there was no significant difference between the groups. The mean of MAC and MAMC differed significantly between the groups, being higher in the HS group, with p values of 0,047 and 0,043. Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.

Vitamin D Treatment Attenuates Heart Apoptosis After Coronary Artery Bypass Surgery: A Double-Blind, Randomized, Placebo-Controlled Clinical Trial

2020 ◽  
Vol 25 (4) ◽  
pp. 338-345 ◽  
Author(s):  
Erfan Tasdighi ◽  
Manouchehr Hekmat ◽  
Mahmoud Beheshti ◽  
Ramin Baghaei ◽  
Seyed Mohsen Mirhosseini ◽  
...  
Keyword(s):  
Vitamin D ◽  
Coronary Artery ◽  
Coronary Artery Bypass ◽  
Artery Bypass ◽  
Bypass Graft ◽  
Control Group ◽  
Controlled Clinical Trial ◽  
Double Blind ◽  
Inflammatory Status ◽  
Significant Difference

Background: Vitamin D plays an important role in immune system and in the regulation of inflammatory cytokines. Coronary artery bypass graft (CABG) with cardiopulmonary bypass (CPB) is associated with an extensive inflammatory response. The aim of this study is to examine the effect of vitamin D treatment on the apoptosis and inflammatory changes developed after CABG. Methods: This trial was conducted on 70 patients undergoing CABG with CPB. Patients were randomly administered either in placebo or in the group of orally consuming 150 000 IU vitamin D daily for 3 consecutive days before surgery. The right atrium sample was taken to assess caspases 2, 3, and 7 activity using immunohistochemistry method. The serum level of interleukin-10 (IL-10) and insulin-like growth factor 1 (IGF-1) were compared at intervals. Results: The average number of positive cells for caspases 2 and 3 were less in vitamin D group ( P = .006 and P < .001, respectively). There was an increase in serum levels of IL-10 after 3 days from vitamin D treatment before surgery (vitamin D group = 4.4 ± 4.9 ng/mL and control group = 1 ± 0.5 ng/mL, P = .001). After operation, IL-10 increased in both groups, higher level in vitamin D group ( P < .001). The comparison of serum IGF-1 showed significant difference after 3 days ( P = .006) and remained higher in vitamin D group after CPB ( P < .001). Conclusions: These findings suggest the apoptosis rate after CPB can be reduced by vitamin D. Vitamin D treatment may improve the inflammatory status before and after surgery. Further studies are needed to confirm the antiapoptotic property of vitamin D and clinical implication.

scholarly journals Serum Hepcidin Level as a Marker of Iron Status in Children with Cystic Fibrosis

2018 ◽  
Vol 2018 ◽  
pp. 1-8
Author(s):  
Monika Kałużna-Czyż ◽  
Urszula Grzybowska-Chlebowczyk ◽  
Halina Woś ◽  
Sabina Więcek
Keyword(s):  
Cystic Fibrosis ◽  
Iron Status ◽  
Full Blood Count ◽  
Control Group ◽  
Hepcidin Level ◽  
Significant Difference ◽  
Serum Hepcidin ◽  
Liver Markers ◽  
Group Control Group ◽  
Active Inflammation

Introduction. Iron deficiency is common in patients with cystic fibrosis. Conventional iron status markers are often abnormal in patients with CF, reflecting inflammation and/or infection, rather than actual iron stores. The aim was to evaluate serum hepcidin levels against selected iron status markers, assuming that hepcidin may be a more sensitive indicator of iron management in patients with active inflammation, such as those with CF. Material and Methods. 46 children with cystic fibrosis and 31 healthy controls were enrolled. Hepcidin concentration was evaluated, along with the following other blood assays: full blood count, Fe, ferritin, transferrin, TIBC, liver markers, and CRP. Results. Higher ferritin and CRP levels as well as lower TIBC levels significantly predicted hepcidin levels in the study group, control group, and the entire sample. There was no significant difference in hepcidin levels between the patients and controls. Children with exacerbations had significantly higher hepcidin levels than those with stable disease. These findings support the serum hepcidin level as useful in assessing iron status in children with cystic fibrosis. It may also be useful in early detection and monitoring of treatment of exacerbations.

scholarly journals Iron Homeostasis and Inflammatory Status in Mice Deficient for the Cystic Fibrosis Transmembrane Regulator

PLoS ONE ◽  
2015 ◽  
Vol 10 (12) ◽  
pp. e0145685 ◽  
Author(s):  
Jean-Christophe Deschemin ◽  
Sarah Allouche ◽  
Franck Brouillard ◽  
Sophie Vaulont
Keyword(s):  
Cystic Fibrosis ◽  
Iron Homeostasis ◽  
Cystic Fibrosis Transmembrane Regulator ◽  
Inflammatory Status ◽  
Cystic Fibrosis Transmembrane
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