Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes

Leeuwen, Lisette; Magoffin, Annabel; Fitzgerald, Dominic A.; Cipolli, Marco; Gaskin, Kevin

doi:10.1136/archdischild-2013-304159

Cited by 23 publications

(23 citation statements)

References 33 publications

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“…However, a recent large, single-center analysis of 401 CF infants over a 25-year period and other studies have found no difference in the percentage of patients with meconium ileus who developed CFLD compared with those without liver disease 20-22 . Our results suggest that a history of meconium ileus increases risk for a homogeneous US pattern.…”

Section: Discussionmentioning

confidence: 91%

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

Ling

Palermo

et al. 2015

The Journal of Pediatrics

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Objective To investigate the relationship between abdominal ultrasound (US) findings and demographic, historical and clinical features in children with CF. Study design Children age 3-12 years with CF without known cirrhosis, were enrolled in a prospective, multi-center study of US to predict hepatic fibrosis. Consensus US patterns were assigned by 3 radiologists as normal, heterogeneous, homogeneous, or cirrhosis. Data were derived from direct collection and U.S. or Toronto CF registries. Chi-square or ANOVA were used to compare variables among US groups and between normal and abnormal. Logistic regression was used to study risk factors for having abnormal US. Results Findings in 719 subjects were normal (n=590, 82.1%), heterogeneous (64, 8.9%), homogeneous (41, 5.7%), and cirrhosis (24, 3.3%). Cirrhosis (p=0.0004), homogeneous (p<0.0001) and heterogeneous (p=0.03) were older than normal. More males were heterogeneous (p=0.001). More heterogeneous (15.0%, p=0.009) and cirrhosis (25.0%, p=0.005) had CF-related diabetes or impaired glucose tolerance versus normal (5.4%). Early infection with Pseudomonas aeruginosa (<2 years old) was associated with a lower risk (OR 0.42, p=0.0007) of abnormal. Ursodeoxycholic acid use (OR 3.69, p <0.0001) and CF-related diabetes (OR 2.21, p=0.019) were associated with increased risk of abnormal. Conclusions Unsuspected cirrhosis is seen in 3.3% of young patients with CF, heterogeneous in 8.9%. abnormal US is associated with CF-related diabetes, and early P aeruginosa is associated with normal US. Prospective assessment of these risk factors may identify potential interventional targets.

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Section: Discussionmentioning

confidence: 91%

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

Ling

Palermo

et al. 2015

The Journal of Pediatrics

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“…53 In addition to short-term nutritional and surgical complications, neonatal cholestasis is common. 69 In the long term, CF patients with prior MI have not been shown to differ in pulmonary function, nutritional status, or survival; however, they have higher rates of DIOS in addition to possible postsurgical complications. [70][71][72] Distal Intestinal Obstruction Syndrome DIOS is a CF-specific obstructive process beginning in the ileocecum secondary to an adherent, viscid fecal mass associated with dehydrated intestinal contents and densely packed mucus.…”

Section: Ileocolonic Disease Meconium Ileusmentioning

confidence: 95%

“…Its incidence among infants diagnosed with CF within a statewide newborn screening program is considered low at 5.7%, but is approximately 140-fold greater than that in the general population of term infants. 69,153 MI and complicated MI are significant risk factors for the development of cholestasis, with incidence greater than 25% in infants with MI. 69 In addition to jaundice, other features of CF-associated neonatal cholestasis are often nonspecific.…”

Section: Neonatal Cholestasismentioning

confidence: 99%

Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

Gabel

Galante

Freedman

2019

Semin Respir Crit Care Med

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Cystic fibrosis (CF) is a multiorgan disease, and gastrointestinal (GI) manifestations can contribute to significant morbidity and mortality for individuals with CF. Up to 85% of patients with CF experience GI symptoms, thus addressing the GI aspects of this disease is paramount. With the advent of highly effective CF transmembrane conductance regulator modulators that are increasingly available, many individuals with CF now have significantly improved life expectancy. With these advances, GI manifestations that can be a detriment to quality of life such as gastroesophageal reflux disease, dysbiosis, and chronic abdominal pain have become a priority for patients and caregivers. In addition, as individuals have increased longevity, it has become essential for care providers to be aware of topics such as hepatobiliary disease and colorectal cancer screening. An understanding of the wide scope of GI manifestations in CF can enable providers to optimize the overall health and well-being of their patients. In this review, we aim to provide an up-to-date overview of key aspects of GI and hepatic disease in CF.

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“…About 25% of infants with CF-associated meconium ileus will develop neonatal cholestasis. 93 Cholestasis in CFRLD is likely due to the increased viscosity of bile from CFTR dysfunction. Cholestasis not only leads to liver parenchymal inflammation and fibrosis but also limits the amount of bile acids available for digestion by decreasing the total bile acid content and overall concentration of bile in the small intestines.…”

Section: Cf-related Liver Diseasementioning

confidence: 99%

Growth and Nutrition in Cystic Fibrosis

Brownell

Bashaw

Stallings

2019

Semin Respir Crit Care Med

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Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

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Cholestasis and meconium ileus in infants with cystic fibrosis and their clinical outcomes

Cited by 23 publications

References 33 publications

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

Baseline Ultrasound and Clinical Correlates in Children with Cystic Fibrosis

Gastrointestinal and Hepatobiliary Disease in Cystic Fibrosis

Growth and Nutrition in Cystic Fibrosis

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