scholarly journals Clinical course and risk factors for severe COVID-19 among Italian patients with cystic fibrosis: a study within the Italian Cystic Fibrosis Society

Infection ◽  
2021 ◽  
Author(s):  
Carla Colombo ◽  
Marco Cipolli ◽  
Valeria Daccò ◽  
Paola Medino ◽  
Federico Alghisi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Clinical Course

scholarly journals Association of polymorphic variants of xenobiotic biotransformation genes and glutation metabolizing genes with clinical course of cystic fibrosis, antimicrobial therapy efficacy and adverse reactions

2020 ◽  
pp. 64-65
Author(s):  
О.Г. Новоселова ◽  
Е.И. Кондратьева ◽  
Н.В. Петрова ◽  
В.Д. Шерман ◽  
А.Ю. Воронкова ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Treatment Failure ◽  
Antimicrobial Therapy ◽  
Adverse Reactions ◽  
Clinical Course ◽  
Clinical Manifestations ◽  
Therapy Efficacy ◽  
Xenobiotic Biotransformation ◽  
Polymorphic Variants

Тяжесть клинических проявлений муковисцидоза может быть обусловлена действием генов-модификаторов. Выяснение причин неэффективности терапии и нежелательных побочных реакций, определение факторов риска позволит улучшить прогноз для данной категории больных. Исследованы ассоциации 18 полиморфных вариантов 10 генов ферментов первой и второй фазы биотрансформации ксенобиотиков: CYP2C9 (c.430C>T, c.1075A> C), CYP2C19 (c.681G>A), CYP2D6 (1846G>A), CYP3A4 (c-392C>T), GSTT1 (del), GSTM1 (del), GSTP1 (c.313A>C), GCLC (TVR GAG, c.-129C>T), GCLM (c.-588C>T), NAT2 (c.282C>T, c.341T>C, c.434A>C, c.481C>T, c.590G>A, c.845A>C, c.857G>A) с тяжестью клинических проявлений муковисцидоза. CF clinical variability could be associated with interaction of modifier genes. Сlarification of the causes of treatment failure and adverse reactions, prediction of risk factors could improve the outcome of therapy. Association of 18 polymorphic variants of 10 genes of xenobiotic biotransformation: CYP2C9 (c.430C>T, c.1075A> C), CYP2C19 (c.681G>A), CYP2D6 (1846G>A), CYP3A4 (c-392C>T), GSTT1 (del), GSTM1 (del), GSTP1 (c.313A>C), GCLC (TVR GAG, c.-129C>T), GCLM (c.-588C>T), NAT2 (c.282C>T, c.341T>C, c.434A>C, c.481C>T, c.590G>A, c.845A>C, c.857G>A) with severity of clinical manifestations were analyzed in 333 CF patients.

Risk factors and clinical course of children with cystic fibrosis colonized with Staphylococcus aureus

2020 ◽  
Author(s):  
Jagat Jeevan Ghimire ◽  
Krishna Mohan Gulla ◽  
Kana Ram Jat ◽  
Jhuma Sankar ◽  
Rakesh Lodha ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Staphylococcus Aureus ◽  
Clinical Course ◽  
Age Of Onset ◽  
Clinical Severity ◽  
Significant Deterioration ◽  
Group A ◽  
Pulmonary Exacerbations ◽  
Group B

Abstract Background The literature is limited on staphylococcal infection in children with cystic fibrosis (CF) from tropical countries. We aimed to study the risk factors and clinical course of children with CF infected with Staphylococcus aureus. Methods In this chart review we compared demographic, clinical and spirometry characteristics in CF children with S. aureus alone (group A), both S. aureus and Pseudomonas aeruginosa (group B) and P. aeruginosa alone (group C) colonization. Results We included 79 cases (group A, 22; group B, 19; group C, 38). There was no difference in age of onset of symptoms, age of diagnosis, age of first isolation and spirometry parameters before colonization between the groups. The median duration of follow-up was shorter in group A. After colonization, children in group A and group B had significantly lower mean Shwachman and Kulczycki (SK) scores (44.7±5.4 and 40.8±5.8, respectively) compared with group C (49.9±6.8). Pulmonary exacerbations and hospitalizations were significantly greater in the combined group. After colonization, group A had a significant deterioration in SK score and forced vital capacity (FVC). Conclusions S. aureus colonization, especially in combination with P. aeruginosa, in children with CF was associated with worsening of FVC and clinical severity score and increased pulmonary exacerbations.

scholarly journals Risk Factors for the Progression of Cystic Fibrosis Lung Disease throughout Childhood

2014 ◽  
Vol 11 (1) ◽  
pp. 63-72 ◽  
Author(s):  
Don B. Sanders ◽  
Zhanhai Li ◽  
Anita Laxova ◽  
Michael J. Rock ◽  
Hara Levy ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Disease ◽  
Cystic Fibrosis Lung ◽  
Cystic Fibrosis Lung Disease

Risk Factors for Aspergillus Colonization and Allergic Bronchopulmonary Aspergillosis in Children With Cystic Fibrosis

2010 ◽  
Vol 45 (8) ◽  
pp. 764-771 ◽  
Author(s):  
Virginie Jubin ◽  
Stéphane Ranque ◽  
Nathalie Stremler Le bel ◽  
Jacques Sarles ◽  
Jean-Christophe Dubus
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Allergic Bronchopulmonary Aspergillosis

Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study

2021 ◽  
Author(s):  
Olga Archangelidi ◽  
Paul Cullinan ◽  
Nicholas J. Simmonds ◽  
Emmanouil Mentzakis ◽  
Daniel Peckham ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Case Control Study ◽  
Case Control ◽  
The Uk ◽  
Control Study

Characteristics, outcomes, and risk factors of ICANS after axicabtagene ciloleucel: Does age matter?

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e19556-e19556
Author(s):  
Kitsada Wudhikarn ◽  
Radhika Bansal ◽  
Arushi Khurana ◽  
Matthew Hathcock ◽  
Michael Ruff ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Factor ◽  
Older Patients ◽  
Clinical Course ◽  
International Prognostic Index ◽  
Age Groups ◽  
Central Nervous System Involvement ◽  
Microvascular Disease ◽  
B Cell Lymphoma ◽  
Younger Patients

e19556 Background: CD19 chimeric antigen receptor T cell therapy possesses unique side effects including cytokine release syndrome (CRS) and immune effector cell associated neurotoxicity syndrome (ICANS). Age is a major risk factor for ICANS. However, whether ICANS in older patients is different compared to younger patients is unknown. Herein, we report clinical course, outcomes and risk factors for ICANS in older patients with large B cell lymphoma (LBCL) treated with axicabtagene ciloleucel (axi-cel). Methods: We comprehensively reviewed detailed clinical courses of ICANS in 78 adult patients with LBCL treated with axi-cel between June 2016 and October 2020. Incidence, manifestation, risk factors, treatment, and outcomes of ICANS were compared between patients age ≥60 (n=32) and <60 (n=46) years old. Results: Baseline characteristics were comparable between older and younger patients except higher proportion of high international prognostic index and underlying cerebral microvascular disease in older patients. ICANS was observed in 16 patients in the older and 24 patients in the younger age group, with a 30-day incidence of 52% and 50%, respectively. Median time to CRS and ICANS were similar between 2 age groups. The most common initial neurological findings included aphasia, dysgraphia and encephalopathy in both age groups. Table summarizes the characteristics, clinical course and interventions of ICANS in older and younger patients. In Cox regression model, the presence of CRS was the only factor associated with ICANS in both age groups. Age, history of central nervous system involvement and cerebral microvascular disease were not associated with ICANS. Importantly, all patients had complete resolution of ICANS. No elderly patients in our cohort experienced seizure as a manifestation of ICANS. Conclusions: In our study, older age was not a risk factor for ICANS. CRS was the only factor associated with ICANS in both younger and older patients. Incidence, clinical course and neurological outcomes of ICANS in older patients treated with axi-cel were comparable to younger patients. [Table: see text]

A Therapeutic Trial of Fatty Acid Supplementation in Cystic Fibrosis

PEDIATRICS ◽  
1976 ◽  
Vol 57 (4) ◽  
pp. 474-479
Author(s):  
Robert B. Elliott
Keyword(s):  
Cystic Fibrosis ◽  
Fatty Acid ◽  
Clinical Course ◽  
Therapeutic Trial ◽  
Biochemical Abnormality ◽  
Fatty Acid Supplementation ◽  
Oil Emulsion ◽  
Soya Oil ◽  
One Year ◽  
Better Than

Seven children with cystic fibrosis (CF) have been treated for at least one year with intravenously administered soya oil emulsion. In all, an improvement of at least one biochemical abnormality in character with the disease appeared. The children's clinical course remains benign. This course is remarkably better than that of other children with CF treated without Intralipid in Auckland in the same period, though a placebo effect cannot be discounted. It is postulated that intravenous supplementation with essential fatty acid in CF may in turn partially correct an error of metabolism of prostaglandins present in the disease.

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