scholarly journals Cystic Fibrosis and New Trends by Ophthalmological Evaluation: A Pilot Study

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Marcella Nebbioso ◽  
Serena Quattrucci ◽  
Emanuela Leggieri ◽  
Leopoldo Spadea ◽  
Enzo Maria Vingolo

Background.Cystic fibrosis (CF) is characterized by hypoxia that affects several organic tissues. Retinal ganglion cells may suffer from the hypoxic status, and this may lead to alterations of retinal nerve fiber.Methods.Twenty-two eyes in CF patients were analyzed. A complete ocular evaluation and visual field exams of the 30 central degrees were performed using the frequency doubling technology (FDT). Forced expiratory volume in one second (FEV1%), forced vital capacity (FVC%), oxyhaemoglobin saturation (SpO2%), and hematocrit (Ht%) have been calculated. FDT analyzed parameters were mean deviation (MD) and pattern standard deviation (PSD). Pearson’s correlation was chosen as statistical analysis.Results.Data showed statistically significant relationship between MD and Ht% (rvalue −0.18;P=0.04), MD and FEV1% (rvalue −0.68;P=0.001), and MD and FVC% (rvalue −0.45;P=0.005). Moreover, there were correlations between PSD and Ht% (rvalue 0.29;P=0.03), PSD and SpO2% (rvalue −0.31;P=0.01), PSD and FEV1% (rvalue 0.71;P=0.0005), and PSD and FVC% (rvalue 0.63;P=0.003).Conclusions.The oxygen supply alterations might determine hypoxia of the ganglion cells causing a decrease of receptive optic nerve fiber activity. This method could be also useful to evaluate indirectly pulmonary activity of the CF disease.

2019 ◽  
Vol 188 ◽  
pp. 107781 ◽  
Author(s):  
Diego García-Ayuso ◽  
Johnny Di Pierdomenico ◽  
Francisco J. Valiente-Soriano ◽  
Ana Martínez-Vacas ◽  
Marta Agudo-Barriuso ◽  
...  

2020 ◽  
Author(s):  
Vianney Gruzelle ◽  
Hélène Guet-Revillet ◽  
Christine Segonds ◽  
Stéphanie Bui ◽  
Julie Macey ◽  
...  

Abstract Background: Whereas Burkholderia infections are recognized to impair prognosis in cystic fibrosis (CF) patients, there is no recommendation to date for early eradication therapy. The aim of our study was to analyse the current management of initial colonisations with Burkholderia cepacia complex (BCC) or B. gladioli in French CF Centres and its impact on bacterial clearance and clinical outcome. Methods: We performed a retrospective review of the primary colonisations (PC), defined as newly positive sputum cultures, observed between 2010 and 2018 in five CF Centres. Treatment regimens, microbiological and clinical data were collected. Results: Seventeen patients (14 with BCC, and 3 with B. gladioli) were included. Eradication therapy, using heterogeneous combinations of intravenous, oral or nebulised antibiotics, was attempted in 11 patients. Six out of the 11 treated patients, and 4 out of the 6 untreated patients cleared the bacterium. Though not statistically significant, higher forced expiratory volume in one second and forced vital capacity at PC and consistency of treatment with in vitro antibiotic susceptibility tended to be associated with eradication. The management of PC was shown to be heterogeneous, thus impairing the statistical power of our study. Large prospective studies are needed to define whom to treat, when, and how. Conclusion: Pending these studies, we propose, due to possible spontaneous clearance, to check the presence of Burkholderia one month after PC before starting antibiotics, at least in the milder cases, and to evaluate a combination of IV beta-lactam + oral or IV fluoroquinolone + inhaled aminoglycoside.


1985 ◽  
Vol 19 (10) ◽  
pp. 757-761 ◽  
Author(s):  
Gilles L. Fraser ◽  
August J. Valenti ◽  
Gilbert R. Grimes ◽  
Richard P. Corbin

Fourteen episodes of acute pseudomonal pulmonary exacerbations of cystic fibrosis were treated with high-dose tobramycin (10.1–17.1 mg/kg/d) and carbenicillin (600 mg/kg/d). The Sawchuck-Zaske method of dosing tobramycin was used and resulted in good agreement between the desired and measured peak (8.1 ± 0.5 vs. 8.2 ± 1.5 μg/ml) and trough (0.5 ± 0.2 vs. 1.0 ± 0.4 μg/ml) concentrations. Ninety-three percent of cases improved clinically. Forced expiratory volume in one second and forced vital capacity increased significantly (39.3 ± 24.8, p < 0.001 and 24.1 ± 22.8 percent, p < 0.05, respectively) after treatment. Pseudomonas aeruginosa was eradicated from the sputa of 69 percent of the cases, with recolonization occurring within three months. Significant nephrotoxicity and ototoxicity were not seen. Liver enzymes, however, were elevated in 29 percent of those treated. Combination high-dose carbenicillin and tobramycin satisfies the treatment goals of bacteriological and clinical cure with a minimal degree of toxicity.


2021 ◽  
Vol 22 (4) ◽  
pp. 1724
Author(s):  
Tsunehiko Ikeda ◽  
Kimitoshi Nakamura ◽  
Takaki Sato ◽  
Teruyo Kida ◽  
Hidehiro Oku

Dissociated optic nerve fiber layer (DONFL) appearance is characterized by dimpling of the fundus when observed after vitrectomy with the internal limiting membrane (ILM) peeling in macular diseases. However, the cause of DONFL remains largely unknown. Optical coherence tomography (OCT) findings have indicated that the nerve fiber layer (NFL) and ganglion cells are likely to have been damaged in patients with DONFL appearance. Since DONFL appearance occurs at a certain postoperative period, it is unlikely to be retinal damage directly caused by ILM peeling because apoptosis occurs at a certain period after tissue damage and/or injury. However, it may be due to ILM peeling-induced apoptosis in the retinal tissue. Anoikis is a type of apoptosis that occurs in anchorage-dependent cells upon detachment of those cells from the surrounding extracellular matrix (i.e., the loss of cell anchorage). The anoikis-related proteins βA3/A1 crystallin and E-cadherin are reportedly expressed in retinal ganglion cells. Thus, we theorize that one possible cause of DONFL appearance is ILM peeling-induced anoikis in retinal ganglion cells.


2015 ◽  
Vol 2015 ◽  
pp. 1-11 ◽  
Author(s):  
Alberto Mavilio ◽  
Francesca Scrimieri ◽  
Donato Errico

Objective. To evaluate variability of steady-state pattern electroretinogram (SS-PERG) signal in normal, suspected, and glaucomatous eyes.Methods. Twenty-one subjects with suspected glaucoma due to disc abnormalities (GS), 37 patients with early glaucoma (EG), and 24 normal control (NC) were tested with spectral-domain optical coherence tomography (SD-OCT), standard automated perimetry (SAP), and SS-PERG. Mean deviation (MD), pattern standard deviation (PSD), retinal nerve fiber layer (RNFL), and ganglionar complex cells (GCC) were evaluated. The SS-PERG was recorded five consecutive times and the amplitude and phase of second harmonic were measured. PERG amplitude and coefficient of variation of phase (CVphase) were recorded, and correlation with structural and functional parameters of disease, by means of one-way ANOVA and Pearson’s correlation, was analysed.Results. PERG amplitude was reduced, as expression of retinal ganglion cells (RGCs) dysfunction, in EG patients and GS subjects compared to NC patients (P<0.0001). CVphase was significantly increased in EG patients and GS subjects, compared to healthy (P<0.0001), and it was also correlated with PSD (P=0.0009), GCC (P=0.028), and RNFL (P=0.0078) only in EG patients.Conclusions. Increased intrasession variability of phase in suspected glaucomatous eyes may be a sign of RGCs dysfunction.


2018 ◽  
Vol 23 (2) ◽  
pp. 125-131 ◽  
Author(s):  
Nicholas M. Fusco ◽  
Calvin J. Meaney ◽  
Corey Wells ◽  
Carla A. Frederick ◽  
William A. Prescott

OBJECTIVES This study aimed to compare the change in pulmonary function in children and adolescents with cystic fibrosis (CF) who were infected with methicillin-resistant Staphylococcus aureus (MRSA) treated with either vancomycin (VAN) alone or vancomycin plus rifampin (VAN-RIF). METHODS Included patients were ages 6 to 20 years; hospitalized for an acute pulmonary exacerbation (APE) of CF from May 1, 2012, to April 30, 2014; had a respiratory tract culture positive for MRSA within 1 month of index hospital admission; received at least 48 consecutive hours of VAN or VAN-RIF; and had admission and discharge pulmonary function tests. The primary end point was change in percent predicted forced expiratory volume in 1 second (FEV1). RESULTS A total of 39 encounters met inclusion criteria: 24 in the VAN group (mean age 15.1 years) and 15 in the VAN-RIF group (mean age 13.7 years). There were no between-group differences in mean percent change in FEV1 (32.6% ± 28.8% vs. 21.1% ± 12.1%; p = 0.091), mean percent change in forced vital capacity (22.6% ± 25.8% vs. 14% ± 9.4%; p = 0.127), or return to baseline FEV1 (20 [83.3%] vs. 14 [93.3%] patients; p = 0.631). Median (IQR) length of stay (13 days [11–14 days] vs. 13 days [9–14 days]; p = 0.6) and median (IQR) time to readmission (82 days [43–129 days] vs. 147 days [78–219 days]; p = 0.2) were similar between the VAN and VAN-RIF groups, respectively. CONCLUSIONS Vancomycin monotherapy appears to be adequate when treating APEs of CF in children and adolescents with moderate lung disease and high MRSA VAN minimum inhibitory concentrations. Therefore, the addition of RIF may be unnecessary; however, larger studies are needed to confirm these findings.


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