Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes

Boster, Julia M.; Feldman, Amy G.; Mack, Cara L.; Sokol, Ronald J.; Sundaram, Shikha S.

doi:10.1002/lt.26339

Cited by 12 publications

(14 citation statements)

References 43 publications

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“…The first handicap in our study was trying to establish a cut-off point to define sarcopenia in children under 1 year of age. Previous published studies were based on a control group in this population ( 13 , 26 ), and recently Lurz et al published curves of tPMA for a Canadian population of 779 children, but did not include children under 1 year of age ( 25 ). Metzger et al published curves of tPMA in a group of 782 patients in the US and also included a small set of children under 1 year of age, but showing only p25-p50 values and no clear cut-off points ( 27 ).…”

Section: Discussionmentioning

confidence: 99%

“…Multiple factors contribute to malnutrition and sarcopenia in cholestatic pediatric patients: (a) Decreased energy intake secondary to anorexia, nausea, gastroesophageal reflux disease, vomiting and early satiety; (b) Decreased absorption of fat and fat-soluble vitamins that is aggravated by portal hypertension-induced enteropathy ( 7 ); (c) Altered nutrients metabolism with a decrease in protein synthesis and its use, with abnormal plasma amino acid profiles with low levels of branched-chain amino acids; (d) A hypermetabolic state, with higher energy needs above 40% from the basal ones ( 13 ); (e) Growth hormone (GH) resistance, with elevated GH levels and low-circulating insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding proteins ( 14 ), which may play a role in the development of sarcopenia in children with BA ( 15 ); (f) Physical deconditioning and systemic inflammation that may worsen muscle loss ( 16 , 17 ); (g) Alterations in microbiome in BA children that may contribute to malnutrition ( 18 ).…”

Section: Introductionmentioning

confidence: 99%

“…Anthropometric measurements are employed in daily care and changes in weight, weight-for-length, and body mass index (BMI) must be interpreted cautiously due to organomegaly, fluctuating ascites, edema, and fluid retention. Other measurements commonly used in children with chronic liver disease are mid-upper arm circumference (MUAC) that reflects lean and adipose mass, and triceps skin fold that reflects adipose tissue ( 13 ). Although both have been considered as sensitive markers of malnutrition, their measurement requires trained personnel and should be performed serially to evaluate the adequacy and impact of nutritional interventions ( 7 ).…”

Section: Introductionmentioning

confidence: 99%

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Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia

Lledín

Parrón‐Pajares²,

Morais³

et al. 2023

Front. Pediatr.

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BackgroundSarcopenia in adult cirrhotic patients is associated with increased morbidity and mortality whereas in children it is still being studied. Anthropometric variables in cirrhotic children are not reliable for assessing muscle mass as they may be altered by ascites, edema, and organomegaly. Measuring the area of the psoas showed good correlation with muscle mass in adults. We aimed to study in cirrhotic infants undergoing liver transplantation the association of the psoas area with liver transplant prognosis as well as with several analytical and anthropometric parameters used to evaluate nutritional status.MethodsRetrospective cohort of 29 infants with cirrhosis due to biliary atresia who underwent abdominal CT scan as a pre-transplant study. We measured the psoas muscle index (PMI) at L4-L5 since it best correlates with muscle mass in pediatric patients. As there are no validated cut-off points to define sarcopenia in children under one year of age, PMI was recorded as a continuous variable and correlated with different prognostic, clinical, and analytical variables. The SPSS 17.0 package was used for statistical analysis and a P < 0.05 was considered significant.Results29 infants (10 boys, 19 girls) were studied. 62% were Caucasian and the rest were South American. The mean age at CT scan was 8.5 months (range 3–15 months). There was a negative correlation between PMI and days of admission prior to liver transplant, previous infections, and bone fractures. Among the analytical parameters, cholinesterase, albumin, and prealbumin correlated positively with PMI (P < 0.05). No relationship was observed with anthropometric parameters: weight, height, BMI, brachial perimeter, or bioimpedance. During surgery, patients with lower PMI had a greater need for plasma transfusion, and in the immediate postoperative period, there was a longer stay in intensive care, more days of mechanical ventilation, and more days of hospital admission (P < 0.05). On the contrary, no relationship was found with other complications: bleeding, re-interventions, biliary leaks, rejection, thrombosis, re-transplantation, or infections.ConclusionsThe decrease in muscle mass is associated with increased morbidity in infants with biliary atresia undergoing liver transplantation. Muscle mass in these patients cannot be adequately assessed with anthropometric measurements commonly used in the clinic.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia

Lledín

Parrón‐Pajares²,

Morais³

et al. 2023

Front. Pediatr.

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“…The infant was fed a deeply hydrolyzed formula containing high and medium chain triglycerides (MCT). Fat malabsorption can lead to essential fatty acid (EFA) deficiency along with impaired absorption of fat-soluble vitamins (A, D, E, and K) and trace minerals (e.g., calcium, magnesium, iron, and zinc) from BA ( 9 ). We added fat-soluble vitamins and trace minerals to intravenous nutrition to support Kasai's perioperative nutritional status.…”

Section: Discussionmentioning

confidence: 99%

Meconium Peritonitis, Intestinal Atresia Combined With Biliary Atresia: A Case Report

Han

Chen

et al. 2022

Front. Pediatr.

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Meconium peritonitis (MP) combined with intestinal atresia (IA) is a rare neonatal condition, and it is even rarer in combination with biliary atresia (BA). We describe a case of an infant who developed short bowel syndrome after partial intestinal resection due to MP and IA, along with a Santullienterostomy. During continuous enteral and parenteral nutrition, the stool color became paler. BA was identified by elevated direct bilirubin (DBIL), gamma-glutamyltransferase (GGT), serum matrix metalloproteinase-7 (MMP-7), and hepatobiliary ultrasound; then, Kasai portoenterostomy (KPE) was performed promptly. The Roux-en-Y limb was adjusted intraoperatively to preserve the maximum length of the small intestine while closing the enterostomy. After the operation, the infant gradually adapted to enteral nutrition, his bilirubin level returned to normal, and his weight gradually caught up to the normal range. Although rare, BA should be suspected when MP is combined with IA and when the stool becomes paler in color in the enterostomy state.

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“…Sullivan et al (30) also demonstrated better anthropometric outcomes when comparing 25 patients with BA who had PN and 22 patients without PN, with a mean duration of PN of 2.9 months, after a same mean duration of EN. Using PN is also recommended in case of EN failure by the 2019 guidelines published by Mouzaki et al (12), as well as a recent review by Boster et al, who advised first using oral feeds, second nasogastric tube feeding when oral intake is insufficient, and third PN in case of poor growth despite NG feeds (31). Our results reinforce the importance to set up an early EN prior to transplantation in order to prevent the onset of malnutrition and consequences of advanced chronic liver diseases and avoid the use of PEN.…”

Section: Discussionmentioning

confidence: 99%

Efficacy and tolerance of enteral nutrition in children with biliary atresia awaiting liver transplantation

et al. 2022

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ObjectivesMalnutrition is common in children with biliary atresia (BA) awaiting liver transplantation (LT). Few studies have evaluated the effectiveness of enteral nutrition (EN) in these patients. The objective of this work was to assess the efficacy and tolerance of EN in children with BA awaiting LT.MethodsA total of 31 patients with BA followed between 1995 and 2018 were retrospectively included. Anthropometric indicators (weight, length, and head circumference) and adverse effects of EN were noted at the start (T0) and the end (T1) of EN. The z-scores for anthropometric indicators were compared between T0 and T1.ResultsThe median age at T0 was 7 months (interquartile range [IQR] 5–9), and the median duration of EN was 9 months (IQR 3–17). The z-scores for anthropometric variables improved from T0 to T1: –1.6 (IQR –2.5 to –1.0) to –0.5 (IQR –1.8 to 0.3) for median weight for age; –1.3 (IQR –2.4 to 0) to –0.4 (IQR –2.0 to 0.7) for length for age; –0.9 (IQR –2.3 to –0.3) to –0.3 (IQR –1.2 to 0.1) for weight for length; and –1.2 (IQR –2.1 to –0.6) to –0.2 (IQR –1.6 to 0.4) for body mass index (p < 0.05 for all comparisons). Nearly all (94%) of the patients had a weight-for-length z-score > –2 at the end of EN; 23% had adverse effects and 10% had complications leading to the cessation of EN.ConclusionEN is effective and well tolerated in infants with BA awaiting LT.

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Malnutrition in Biliary Atresia: Assessment, Management, and Outcomes

Cited by 12 publications

References 43 publications

Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia

Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia

Meconium Peritonitis, Intestinal Atresia Combined With Biliary Atresia: A Case Report

Efficacy and tolerance of enteral nutrition in children with biliary atresia awaiting liver transplantation

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