An atypical presentation of cystic fibrosis: a case report

<p>Abstract</p> <p>Introduction</p> <p>The presentation of cystic fibrosis is dependant upon which organs are affected. Common presentations include chronic respiratory infections and malabsorption. Patients with atypical disease tend to present late in childhood or as adults. Eye manifestations of cystic fibrosis are less well known.</p> <p>Case presentation</p> <p>A 14-year-old Caucasian boy presented with tiredness and difficulty seeing at night, over a period of 6 months. Good vision was only described in bright conditions. There was no history of jaundice, steatorrhea or diarrhoea.</p> <p>Conclusion</p> <p>This is the first reported case of newly diagnosed cystic fibrosis-related liver disease in a teenage boy, whose presenting symptom was night blindness secondary to vitamin A deficiency.</p

The relationship between hemoglobin and [Formula: see text]: A systematic review and meta-analysis.

ObjectiveThere is widespread agreement about the key role of hemoglobin for oxygen transport. Both observational and interventional studies have examined the relationship between hemoglobin levels and maximal oxygen uptake ([Formula: see text]) in humans. However, there exists considerable variability in the scientific literature regarding the potential relationship between hemoglobin and [Formula: see text]. Thus, we aimed to provide a comprehensive analysis of the diverse literature and examine the relationship between hemoglobin levels (hemoglobin concentration and mass) and [Formula: see text] (absolute and relative [Formula: see text]) among both observational and interventional studies.MethodsA systematic search was performed on December 6th, 2021. The study procedures and reporting of findings followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Article selection and data abstraction were performed in duplicate by two independent reviewers. Primary outcomes were hemoglobin levels and [Formula: see text] values (absolute and relative). For observational studies, meta-regression models were performed to examine the relationship between hemoglobin levels and [Formula: see text] values. For interventional studies, meta-analysis models were performed to determine the change in [Formula: see text] values (standard paired difference) associated with interventions designed to modify hemoglobin levels or [Formula: see text]. Meta-regression models were then performed to determine the relationship between a change in hemoglobin levels and the change in [Formula: see text] values.ResultsData from 384 studies (226 observational studies and 158 interventional studies) were examined. For observational data, there was a positive association between absolute [Formula: see text] and hemoglobin levels (hemoglobin concentration, hemoglobin mass, and hematocrit (PConclusionThese findings suggest that [Formula: see text] values are closely associated with hemoglobin levels among both observational and interventional studies. Although our findings suggest a lack of sex differences in these relationships, there were limited studies incorporating females or stratifying results by biological sex

Zinc therapy for night blindness in cystic fibrosis

This is the first report of a supplemented CF patient presenting with clinical vitamin A deficiency to be successfully treated with zinc therapy alone. Therefore in addition to retinol supplementation, normalizing serum zinc levels may be important in maintaining the vitamin A status of CF patients. The interactions and synergistic effects between the two micronutrients are discussed

Risk of bias among interventional articles.

ObjectiveThere is widespread agreement about the key role of hemoglobin for oxygen transport. Both observational and interventional studies have examined the relationship between hemoglobin levels and maximal oxygen uptake () in humans. However, there exists considerable variability in the scientific literature regarding the potential relationship between hemoglobin and . Thus, we aimed to provide a comprehensive analysis of the diverse literature and examine the relationship between hemoglobin levels (hemoglobin concentration and mass) and (absolute and relative ) among both observational and interventional studies.MethodsA systematic search was performed on December 6th, 2021. The study procedures and reporting of findings followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Article selection and data abstraction were performed in duplicate by two independent reviewers. Primary outcomes were hemoglobin levels and values (absolute and relative). For observational studies, meta-regression models were performed to examine the relationship between hemoglobin levels and values. For interventional studies, meta-analysis models were performed to determine the change in values (standard paired difference) associated with interventions designed to modify hemoglobin levels or . Meta-regression models were then performed to determine the relationship between a change in hemoglobin levels and the change in values.ResultsData from 384 studies (226 observational studies and 158 interventional studies) were examined. For observational data, there was a positive association between absolute and hemoglobin levels (hemoglobin concentration, hemoglobin mass, and hematocrit (PPP = 0.006)).ConclusionThese findings suggest that values are closely associated with hemoglobin levels among both observational and interventional studies. Although our findings suggest a lack of sex differences in these relationships, there were limited studies incorporating females or stratifying results by biological sex.</div

The association between change in hemoglobin concentration and change in absolute maximal oxygen uptake () following blood donation or transfusion.

Meta-regression and bubble plot depicting the relationship between the change in hemoglobin concentration and the effect size of the change in absolute (standard paired difference) among independent subgroups following blood transfusion or donation. Different size symbols indicate relative weights used in the meta-regression and are proportional to study sample size.</p