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Pulmonary Manifestations of Gastrointestinal, Pancreatic, and Liver Diseases in Children.
Le Fevre, ER, McGrath, KH, Fitzgerald, DA
Pediatric clinics of North America. 2021;(1):41-60
Abstract
Pulmonary manifestations of gastrointestinal (GI) diseases are often subtle, and underlying disease may precede overt symptoms. A high index of suspicion and a low threshold for consultation with a pediatric pulmonologist is warranted in common GI conditions. This article outlines the pulmonary manifestations of different GI, pancreatic, and liver diseases in children, including gastroesophageal reflux disease, inflammatory bowel disease, pancreatitis, alpha1-antitrypsin deficiency, nonalcoholic fatty liver disease, and complications of chronic liver disease (hepatopulmonary syndrome and portopulmonary hypertension).
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Question 12: What do you consider when discussing treatment adherence in patients with Cystic Fibrosis?
Ohn, M, Fitzgerald, DA
Paediatric respiratory reviews. 2018;:33-36
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Strategies for improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia.
Fitzgerald, DA, Kench, A, Hatton, L, Karpelowsky, J
Paediatric respiratory reviews. 2018;:25-29
Abstract
Post-natal growth in surgical lung conditions, such as congenital diaphragmatic hernia and oesophageal atresia with tracheo-oesophageal fistula, is often sub-optimal in the early years of life when lung growth is occurring. Whilst constitutional, behavioural and mechanical factors may contribute to poor feeding and weight gain, there is a common path of management with greater caloric supplementation that may change growth trajectories and potentially lead to better respiratory, anthropometric and cognitive outcomes. We provide simple, single page, feeding supplementation sheets in three age groups: 0-6months, 6-12months and 12-24months that have proven useful for enhancing weight gain in our patients.
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Question 8: How should distal intestinal obstruction syndrome [DIOS] be managed?
Groves, T, Kench, A, Dutt, S, Gaskin, K, Fitzgerald, DA
Paediatric respiratory reviews. 2017;:68-71
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Question 7: For an infant with an equivocal sweat chloride following newborn screening, how likely is a diagnosis of cystic fibrosis?
Groves, T, Robinson, P, Fitzgerald, DA
Paediatric respiratory reviews. 2016;:48-50
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Liver disease in cystic fibrosis.
Leeuwen, L, Fitzgerald, DA, Gaskin, KJ
Paediatric respiratory reviews. 2014;(1):69-74
Abstract
The survival of patients with cystic fibrosis (CF) has progressively increased over recent decades, largely attributable to early diagnosis through newborn screening and advances in nutritional and respiratory care. As the life expectancy of patients with CF has improved, non-respiratory complications such as liver disease have become increasingly recognized. Biochemical derangements of liver enzymes in CF are common and may be attributed to a number of specific hepatobiliary abnormalities. Among them, Cystic Fibrosis-associated Liver Disease (CFLD) is clinically the most significant hepatic complication and is believed to have a significant impact on morbidity and mortality. However, there remains much conjecture about the extent of the adverse prognostic implications that a diagnosis of CFLD has on clinical outcomes. The purpose of this review is to give an overview of the current knowledge regarding liver disease in children with CF.
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Pulmonary embolism in children.
Dijk, FN, Curtin, J, Lord, D, Fitzgerald, DA
Paediatric respiratory reviews. 2012;(2):112-22
Abstract
Unlike in adults, pulmonary embolism (PE) is an infrequent event in children. It has a marked bimodal distribution during the paediatric years, occurring predominantly in neonates and adolescents. The most important predisposing factors to PE in children are the presence of a central venous line (CVL), infection, and congenital heart disease. Clinical signs of PE are non-specific in children or can be masked by underlying conditions. Diagnostic testing is necessary in children, especially with the lack of clinical prediction rules. Recommendations for tests are derived from adult studies with ventilation/perfusion (V/Q) scintigraphy being well established. There exists an increasing role for computerised tomography pulmonary angiography (CTPA) and magnetic resonance pulmonary angiography (MRPA). Thrombotic events in children are initially treated with unfractionated heparin (UFH) or low molecular weight heparin (LMWH). For the extended anticoagulant therapy LMWH or vitamin K antagonists can be used with duration of treatment recommendations extrapolated from adult data. Mortality rates for PE in children are reported to be around 10%, with death usually related to the underlying disease processes. Exact data about recurrence risk in children is unknown. Because of the difference in aetiology, presentation, diagnostic methods and treatment between adults and children further research is necessary to assess the validity of recommendations for children.
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8.
Viral bronchiolitis for the clinician.
Fitzgerald, DA
Journal of paediatrics and child health. 2011;(4):160-6
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Abstract
Viral bronchiolitis is common, and about 98-99% of infants are managed in the home. Because about 95% of infants < 2 years old are infected with respiratory syncytial virus, however, bronchiolitis is the commonest reason for admission to hospital in the first 6 months of life. It is usually a self-limiting condition lasting around a week in previously well children. About 1% of infants are admitted to hospital, and about 10% of hospitalised infants will require admission to the intensive care unit. Respiratory syncytial virus is isolated from about 70% of infants hospitalised with bronchiolitis. The emphasis of hospital treatment is to ensure adequate hydration and oxygenation. Other than supplemental oxygen, little in the way of pharmacological treatment has been demonstrated to alter the course of the illness or the risk of wheezing in the months following bronchiolitis.
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Improved antioxidant and fatty acid status of patients with cystic fibrosis after antioxidant supplementation is linked to improved lung function.
Wood, LG, Fitzgerald, DA, Lee, AK, Garg, ML
The American journal of clinical nutrition. 2003;77(1):150-9
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Plain language summary
Oxidative stress is elevated in patients with cystic fibrosis (CF). It has been hypothesised that supplementing with antioxidants reduces oxidative stress and therefore the rate of lung deterioration in CF patients. The aim of the study was to examine oxidative stress and antioxidant defences in CF patients after antioxidation supplementation in relation to fatty acid status, dietary intake and clinical status. Children with CF were given a high dose supplement containing 200mg vitamin E, 300mg vitamin C, 25mg beta-carotene, 90 micrograms selenium and 500 micrograms vitamin A for 8 weeks. The control group received low doses of vitamins A and E equal to the RDA, which is part of routine treatment for some CF patients. At the end of the study, the group taking the high dose antioxidant supplement showed significant increases in plasma concentrations of vitamin E, beta-carotene, selenium and glutathione peroxidase compared to the control group. Despite this, there were no significant differences in markers of lung function or wellbeing between the two groups. The researchers did however find correlations between both increased plasma fatty acids and antioxidant (beta-carotene and selenium) status and improved lung function, suggesting that antioxidant supplementation and high fat diets may be beneficial for CF patients. Since increased plasma fatty acids are linked to oxidative stress, the authors point out the importance of reducing oxidative stress in CF patients who are on a high-fat diet.
Abstract
BACKGROUND Oxidative stress, as measured by 8-iso-prostaglandin F(2)(alpha) (8-iso-PGF(2)(alpha)), and depleted antioxidant defenses were shown in stable cystic fibrosis (CF) patients. The plasma fatty acid status of CF patients was linked to oxidative stress after respiratory exacerbations. OBJECTIVE We examined changes in plasma 8-iso-PGF(2)(alpha), antioxidant defenses, plasma fatty acid status, and clinical markers resulting from short-term antioxidant supplementation. DESIGN Forty-six CF patients were randomly assigned to either group A [low dose of supplement (10 mg vitamin E and 500 micro g vitamin A)] or group B [high dose of supplement (200 mg vitamin E, 300 mg vitamin C, 25 mg beta-carotene, 90 micro g Se, and 500 micro g vitamin A)]. Plasma concentrations of 8-iso-PGF(2)(alpha), vitamins E and C, beta-carotene, zinc, selenium, and copper; plasma fatty acid composition; erythrocyte glutathione peroxidase (EC 1.11.1.9) and superoxide dismutase (EC 1.15.1.1) activities; lung function; and dietary intake were measured before and after 8 wk of supplementation. RESULTS Antioxidant defenses in group B improved, whereas those in group A did not: in groups B and A, the mean (+/- SEM) changes (Delta) in vitamin E were 10.6 +/- 1.5 and -1.9 +/- 0.9 micro mol/L, respectively (P < 0.001), (Delta)beta-carotene were 0.1 +/- 0.04 and -0.01 +/- 0.02 micro mol/L, respectively (P = 0.007), (Delta)selenium were 0.51 +/- 0.10 and -0.09 +/- 0.04 micro mol/L, respectively (P < 0.001), and (Delta)glutathione peroxidase activity were 1.3 +/- 0.3 and -0.3 +/- 0.6 U/g hemoglobin, respectively (P = 0.016). There were no significant differences between the groups in Delta8-iso-PGF(2)(alpha), (Delta)vitamin C, (Delta)fatty acid composition, (Delta)superoxide dismutase activity, (Delta)lung function, or (Delta)white cell count. Within group B, (Delta)beta-carotene correlated with (Delta)percentage of forced vital capacity (r = 0.586, P = 0.005), (Delta)selenium correlated with (Delta)percentage of forced expiratory volume in 1 s (r = 0.440, P = 0.046), and (Delta)plasma fatty acid concentrations correlated with (Delta)percentage of forced expiratory volume in 1 s (r = 0.583, P = 0.006) and Delta8-iso-PGF(2)(alpha) (r = 0.538, P = 0.010). CONCLUSIONS Whereas increased beta-carotene, selenium, and fatty acid concentrations are linked to improved lung function, increased plasma fatty acid concentrations are linked to oxidative stress. If oxidative stress is deemed to be important to the clinical outcome of CF patients, means of reducing oxidative stress while maintaining a high-fat, high-energy diet must be investigated.
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Hypothesis: vitamin E complements polyunsaturated fatty acids in essential fatty acid deficiency in cystic fibrosis.
Wood, LG, Fitzgerald, DA, Garg, ML
Journal of the American College of Nutrition. 2003;(4):253-7
Abstract
While several studies have demonstrated essential fatty acid (EFA) deficiency in plasma and tissue lipids of cystic fibrosis (CF) patients, the reasons for this deficiency are not well established. It is believed that reduced EFA intake, malabsorption of fat, altered desaturase/lipase activity and defective cystic fibrosis transmembrane conductance regulator (CFTR) altering utilisation of EFA in epithelial cells contribute to the development of EFA deficiency in CF. It is likely that increased metabolism of arachidonic acid to eicosanoids such as leukotrienes, thromboxane and prostaglandins may also be a contributing factor. Evidence is presented that elevated oxidative damage to EFA and impaired antioxidant defences, in particular vitamin E, may contribute to the development of EFA deficiency in CF. Furthermore, antioxidant supplementation in CF may improve EFA status.