Nutrition for Children with Cholestatic Liver Disease

Los, Esther; Lukovac, Sabina; Werner, Anniek; Dijkstra, T.; Verkade, Henkjan J.; Rings, Edmond H. H. M.

doi:10.1159/000098533

Cited by 4 publications

(6 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In CDG presenting with cholestasis (such as ALG8-CDG, COG6-CDG, COG7-CDG, CCDC115-CDG and ATP6AP1-CDG), nutritional interventions such as in other causes of cholestasis can be necessary [ 2 ]. These entail the supplementation of fat-soluble vitamins and MCT (medium-chain triglycerides) that are more easily absorbed in the absence of bile [ 36 ].…”

Section: Resultsmentioning

confidence: 99%

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG)

2017

View full text Add to dashboard Cite

Congenital disorders of glycosylation (CDG) are a group of more than 130 inborn errors of metabolism affecting N-linked, O-linked protein and lipid-linked glycosylation. The phenotype in CDG patients includes frequent liver involvement, especially the disorders belonging to the N-linked protein glycosylation group. There are only a few treatable CDG. Mannose-Phosphate Isomerase (MPI)-CDG was the first treatable CDG by high dose mannose supplements. Recently, with the successful use of d-galactose in Phosphoglucomutase 1 (PGM1)-CDG, other CDG types have been trialed on galactose and with an increasing number of potential nutritional therapies. Current mini review focuses on therapies in glycosylation disorders affecting liver function and dietary intervention in general in N-linked glycosylation disorders. We also emphasize now the importance of early screening for CDG in patients with mild hepatopathy but also in cholestasis.

show abstract

Section: Resultsmentioning

confidence: 99%

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG)

2017

View full text Add to dashboard Cite

show abstract

“…According to the above considerations, it is evident that CCLD negatively affects the nutritional status in infancy (i.e., when growth rates are the highest), thus compromising clinical outcomes for cholestatic children who have end-stage liver disease [ 68 ], and is present in about 80% of cases [ 69 ]. CCLD increases the mortality and morbidity associated with underlying diseases and significantly influences the outcomes of liver transplantation in children [ 70 ].…”

Section: Issues In the Nutritional Management Of Children With Cholestasismentioning

confidence: 99%

“…Although some causes of neonatal cholestasis have no specific treatment, affected children may benefit from appropriate nutritional support to prevent malnutrition and to correct macro/micronutrient deficiencies. This is paramount because a better pre-transplant nutritional status is associated with better post-transplant outcomes, and lower mortality and morbidity [ 18 , 68 , 119 , 120 , 121 ]. The nutritional needs of children with liver disease are outlined schematically in Figure 5 .…”

Section: Pre- and Post-transplant Nutritional Status Of Children With End-stage Cholestatic Liver Diseasementioning

confidence: 99%

Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview

et al. 2021

View full text Add to dashboard Cite

Despite recent advances, the causes of and effective therapies for pediatric chronic cholestatic diseases remain elusive, and many patients progress to liver failure and need liver transplantation. Malnutrition is a common complication in these patients and is a well-recognized, tremendous challenge for the clinician. We undertook a narrative review of both recent and relevant older literature, published during the last 20 years, for studies linking nutrition to pediatric chronic cholestasis. The collected data confirm that malnutrition and failure to thrive are associated with increased risks of morbidity and mortality, and they also affect the outcomes of liver transplantation, including long-term survival. Malnutrition in children with chronic liver disease is multifactorial and with multiple potential nutritional deficiencies. To improve life expectancy and the quality of life, patients require careful assessments and appropriate management of their nutritional statuses by multidisciplinary teams, which can identify and/or prevent specific deficiencies and initiate appropriate interventions. Solutions available for the clinical management of these children in general, as well as those directed to specific etiologies, are summarized. We particularly focus on fat-soluble vitamin deficiency and malnutrition due to fat malabsorption. Supplemental feeding, including medium-chain triglycerides, essential fatty acids, branched-chain amino acids, and the extra calories needed to overcome the consequences of anorexia and high energy requirements, is reviewed. Future studies should address the need for further improving commercially available and nutritionally complete infant milk formulae for the dietary management of this fragile category of patients. The aid of a specialist dietitian, educational training regarding nutritional guidelines for stakeholders, and improving family nutritional health literacy appear essential.

show abstract

“…Nutritional intervention in children with CLDC under 24 months of age has been performed for several decades (45,46). Protocols with special formulas delivered PO with an increase in the energy density based on cereals, glucose polymers, and fats (4,47), the use of lipids that do not require bile acids for absorption, such as medium-chain triglycerides (4,9,32,34,36,47), and the use of branched-chain amino acids to improve insulin resistance and favor an increase in muscle mass (70)(71)(72) are used by most liver units or pediatric gastroenterology departments. In some studies, conducted on small samples of patients with CCLD, it was shown that in infants fed these types of formula administered PO ad libitum, the ingested amounts were similar or slightly greater than those in healthy infants of the same age, however, this amount of energy and nutrients was not enough to achieve adequate catch-up growth.…”

Section: Nutritional Interventionmentioning

confidence: 99%

“…Osteodystrophy associated with CCLD has a multicausal etiology, including malabsorption of vitamin D, poor bone accretion of calcium, and systemic chronic inflammation with increased expression of IL-4 ( 28 – 31 ). In addition to decreased cell mass ( 31 ), decreased availability of energy, and impaired fat absorption ( 32 ), these factors seem to be related to the linear growth and head circumference growth retardation that is almost universally present in these patients, particularly in those with biliary atresia ( 2 , 20 , 31 , 33 ).…”

Section: Pathophysiology Of Secondary Malnutrition In Infants With Ccldmentioning

confidence: 99%

Secondary Malnutrition and Nutritional Intervention in Cholestatic Liver Diseases in Infants

Larrosa‐Haro

Caro-Sabido²

2021

Front. Nutr.

View full text Add to dashboard Cite

We aimed to conduct an updated review on the pathophysiology, diagnosis, and nutritional intervention of CCLD and secondary malnutrition in infants. Protein-energy malnutrition, impaired linear growth, fat-soluble vitamin deficiencies, and hepatic osteodystrophy can occur in up to 80% of cases. The proposed pathophysiological mechanisms include insufficient energy intake, lipid- and fat-soluble vitamin malabsorption, increased energy expenditure, altered intermediate metabolism, hormonal dysregulation, and systemic inflammation. The current approach to diagnosis is the identification of the deviation of growth parameters, body composition, and serum concentration of micronutrients, which determines the type and magnitude of malnutrition. Currently, liver transplantation is the best therapeutic alternative for the reversal of nutritional impairment. Early and effective portoenteroanatomosis can extend survival in patients with biliary atresia. Medical and dietary interventions in some storage and metabolic diseases can improve liver damage and thus the nutritional status. A proportion of patients with biliary atresia have fat-soluble vitamin deficiencies despite receiving these vitamins in a water-soluble form. With aggressive enteral nutrition, it may be possible to increase fat stores and preserve muscle mass and growth. The nutritional issues identified in the pre- and post-transplantation stages include muscle mass loss, bone demineralization, growth retardation, and obesity, which seems to correspond to the natural history of CCLD. Due to the implications for the growth and development of infants with CCLD with this complex malnutrition syndrome, innovative projects are required, such as the generation of prediction and risk models, biomarkers of growth and body composition, and effective strategies for nutritional prevention and intervention.

show abstract

Nutrition for Children with Cholestatic Liver Disease

Cited by 4 publications

References 23 publications

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG)

Nutritional Therapies in Congenital Disorders of Glycosylation (CDG)

Malnutrition in Pediatric Chronic Cholestatic Disease: An Up-to-Date Overview

Secondary Malnutrition and Nutritional Intervention in Cholestatic Liver Diseases in Infants

Contact Info

Product

Resources

About