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Umemura K, Mita A, Ohno Y, Masuda Y, Yoshizawa K, Kubota K, Notake T, Hosoda K, Yasukawa K, Kamachi A, Goto T, Tomida H, Yamazaki S, Shimizu A, Soejima Y. Long-term Catch-up Growth and Risk Factors for Short Adult Height After Pediatric Liver Transplantation: A Retrospective Study. Transplantation 2024; 108:732-741. [PMID: 37691167 DOI: 10.1097/tp.0000000000004795] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/12/2023]
Abstract
BACKGROUND Children requiring liver transplantation generally have severe growth retardation. Recipients experience posttransplantation catch-up growth, although some show short adult heights. We aimed to determine decades-long catch-up growth trends and risk factors for short adult height following liver transplantation. METHODS We analyzed long-term height Z scores and risk factors for short adult height in a single-center retrospective cohort of 117 pediatric liver transplantation recipients who survived >5 y, with 75 of them reaching adult height. RESULTS Median age at transplantation was 1.3 y, and the most common primary diagnosis was biliary atresia (76.9%). Mean height Z scores pretransplantation and 1, 3, and 8 y after transplantation were -2.26, -1.59, -0.91, and -0.59, respectively. The data then plateaued until 20 y posttransplantation when mean adult height Z score became -0.88, with a median follow-up of 18.6 y. Nineteen recipients did not show any catch-up growth, and one quarter of recipients had short adult height (<5th percentile of the healthy population). Multivariate analysis identified old age (odds ratio, 1.22 by 1 y; P = 0.002), low height Z scores at transplantation (odds ratio, 0.46 by 1 point; P < 0.001), and posttransplantation hospital stay ≥60 d (odds ratio, 4.95; P = 0.015) as risk factors for short adult height. In contrast, prolonged steroid use after transplantation was not considered a significant risk factor. CONCLUSIONS Although tremendous posttransplantation catch-up growth was observed, final adult height remained inadequate. For healthy physical growth, liver transplantation should be performed as early as possible, before growth retardation becomes severe.
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Affiliation(s)
- Kentaro Umemura
- Division of Gastroenterological, Hepato-Biliary-Pancreatic, Transplantation and Pediatric Surgery, Department of Surgery, Shinshu University School of Medicine, Matsumoto, Japan
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Fang LZ, Jin HX, Zhao N, Wu YP, Shi YQ. Successful management of severe hypoglycemia induced by total parenteral nutrition in patients with hepatocellular injury: Three cases reports. World J Clin Cases 2024; 12:157-162. [PMID: 38292637 PMCID: PMC10824187 DOI: 10.12998/wjcc.v12.i1.157] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/07/2023] [Revised: 11/07/2023] [Accepted: 12/19/2023] [Indexed: 01/02/2024] Open
Abstract
BACKGROUND Glucose imbalance is common in total parenteral nutrition (TPN). Hypoglycemia seems to be less frequent than hyperglycemia, but it influences the clinical outcome to a greater extent. Therefore, it should be effectively prevented and treated. However, there is no relevant report on how to treat hypoglycemia caused by TPN in patients with liver cell injury. CASE SUMMARY We present three patients with liver cell injury who developed severe hypoglycemia during or after TPN infusion. The causes of severe hypoglycemia and glucose-raising strategies were discussed. According to the physiological characteristics of the hepatocellular injury, the ratio of nutrition components prescribed in TPN was appropriately adjusted for the three cases. We simultaneously reduced the dose of insulin and fat emulsion, and increased the dose of glucose in TPN. The blood glucose level was restored to normal range and clinical symptoms were eliminated. CONCLUSION When hypoglycemia occurs during or after TPN in patients with hepatocellular injury, physicians need to simultaneously reduce insulin and fat emulsion, and increase glucose, and correct severe hypoglycemia in time to reduce its adverse consequences.
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Affiliation(s)
- Ling-Zhi Fang
- Department of Pharmacy, Hebei Key Laboratory of Clinical Pharmacy, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
| | - Hui-Xin Jin
- Department of Pharmacy, Hebei Key Laboratory of Clinical Pharmacy, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
| | - Na Zhao
- Department of Pharmacy, Hebei Key Laboratory of Clinical Pharmacy, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
| | - Yu-Pei Wu
- Department of Pharmacy, Hebei Key Laboratory of Clinical Pharmacy, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
| | - Ying-Qin Shi
- Department of Pharmacy, Hebei Key Laboratory of Clinical Pharmacy, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
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3
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Mansour A, Motamed S, Hekmatdoost A, Karimi S, Mohajeri-Tehrani MR, Abdollahi M, Jelodar R, Sajjadi-Jazi SM. Factors related to hypermetabolism in individuals with type 2 diabetes mellitus and non-alcoholic fatty liver disease. Sci Rep 2023; 13:3669. [PMID: 36871124 PMCID: PMC9985614 DOI: 10.1038/s41598-023-30945-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2022] [Accepted: 03/03/2023] [Indexed: 03/06/2023] Open
Abstract
Considering the progressive prevalence and co-occurrence of type 2 diabetes mellitus (T2DM) and non-alcoholic fatty liver disease (NAFLD), as well as the current evidence suggesting the elevated levels of basal metabolic rate (BMR) among these individuals, the present study aimed to identify factors determining hypermetabolism in such subjects. This cross sectional study was conducted in 30 to 53-year-old individuals with concurrent T2DM and NAFLD (controlled attenuation parameter score ≥ 260 dB/m). Resting energy expenditure (REE) was determined by an indirect calorimetry device. Hypermetabolism was defined as an elevated measured REE > 110% of the predicted REE. The multivariate logistic regression test was used for detecting factors associated with hypermetabolism. Between September, 2017, and March, 2018, a total of 95 eligible participants (64.40% male) with both T2DM and NAFLD were included, while 32.63% of them were classified as hypermetabolic. Overall, the mean recruitment age ± standard deviation and median (interquartile range) body mass index were 44.69 ± 5.47 years and 30.20 (27.80-33.30) kg/m2, respectively. Demographic, anthropometric and biochemical variables did not vary significantly across two groups except for total body water, low-density lipoprotein cholesterol and dipeptidyl peptidase 4 (DPP-4) inhibitors (p < 0.05). According to the results of multivariable logistic regression analyses, hypermetabolism had a positive association with adiponectin (odds ratio [OR] 1.167, 95% confidence interval [CI] 1.015-1.342, p = 0.030), physical activity (OR 1.134, 95% CI 1.002-1.284, p = 0.046), alanine transaminase (OR 1.062, 95% CI 1.006-1.122, p = 0.031) and diastolic blood pressure (OR 1.067, 95% CI 1.010-1.127, p = 0.021). However, fat free mass was inversely related to hypermetabolism (OR 0.935, 95% CI 0.883-0.991, p = 0.023). Adiponectin, alanine transaminase, physical activity, diastolic blood pressure and fat free mass were independently associated with hypermetabolism in subjects with NAFLD and T2DM.
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Affiliation(s)
- Asieh Mansour
- Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran
| | | | - Azita Hekmatdoost
- Department of Clinical Nutrition & Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Sara Karimi
- Department of Clinical Nutrition & Dietetics, Faculty of Nutrition Sciences and Food Technology, National Nutrition and Food Technology Research Institute, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Mohammad Reza Mohajeri-Tehrani
- Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran
| | - Mohammad Abdollahi
- Digestive Disease Research Center, Digestive Disease Research Institute, Tehran University of Medical Sciences, Tehran, Iran
| | - Reihane Jelodar
- Department of Internal Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
| | - Sayed Mahmoud Sajjadi-Jazi
- Endocrinology and Metabolism Research Center, Endocrinology and Metabolism Clinical Sciences Institute, Tehran University of Medical Sciences, Tehran, Iran.
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Knoll J, Altintas B, Gahl WA, Parisi M, Gunay-Aygun M. Growth in Joubert syndrome: Growth curves and physical measurements with correlation to genotype and hepatorenal disease in 170 individuals. Am J Med Genet A 2021; 188:847-857. [PMID: 34951506 DOI: 10.1002/ajmg.a.62593] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2021] [Revised: 10/24/2021] [Accepted: 10/29/2021] [Indexed: 11/09/2022]
Abstract
Joubert syndrome (JS) is a genetically heterogenous disorder of nonmotile cilia with a characteristic "molar tooth sign" on axial brain imaging. Clinical features can include developmental delay, kidney failure, liver disease, and retinal dystrophy. Prospective growth and measurement data on 170 individuals with JS were collected, including parental measurements, birth measurements, and serial measures when available. Analysis of growth parameters in the context of hepatorenal disease, genotype, and other features was performed on 100 individuals assessed at the National Institutes of Health Clinical Center. Individuals with JS had shorter stature despite normal growth velocity and were shorter than predicted for mid-parental height. Individuals were lighter in weight, resulting in a normal body mass index (BMI). Head circumference was larger, averaging 1.9 Z-scores above height. At birth, head circumference was proportional to length. Individuals with variants in CPLANE1 had a larger head circumference compared to other genotypes; individuals with evidence of liver disease had lower weight and BMI; and individuals with polydactyly had shorter height. Here we present growth curves and physical measurements for Joubert syndrome based on the largest collection of individuals with this disorder to aid in clinical management and diagnosis.
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Affiliation(s)
- Jasmine Knoll
- Division of Genetics and Genomics, Boston Children's Hospital, Boston, Massachusetts, USA.,Department of Pediatrics and McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA
| | - Burak Altintas
- Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - William A Gahl
- Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA
| | - Melissa Parisi
- Intellectual and Developmental Disabilities Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
| | - Meral Gunay-Aygun
- Department of Pediatrics and McKusick-Nathans Institute of Genetic Medicine, The Johns Hopkins Hospital, Baltimore, Maryland, USA.,Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA
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Aneja A, Scott E, Kohli R. Advances in management of end stage liver disease in children. Med J Armed Forces India 2021; 77:129-137. [PMID: 33867627 DOI: 10.1016/j.mjafi.2021.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2021] [Accepted: 03/05/2021] [Indexed: 02/07/2023] Open
Abstract
End stage liver disease (ESLD) is an irreversible condition that is a management challenge to the paediatrician. The aetiology and natural history of ESLD in children is not only distinct from adults but also variable depending upon the age of presentation. Children are especially vulnerable to developmental delay, frailty and malnutrition. Nutritional support is the cornerstone of management of these children as it has a significant impact on the clinical course and survival, both before and after transplantation. Further, the complications of ESLD in children including but not limited to, ascites, portal hypertension, spontaneous bacterial peritonitis and encephalopathy raise unique management challenges. In this review we provide a concise review of and highlight recent advances in the management of paediatric ESLD.
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Affiliation(s)
- Aradhana Aneja
- Classified Specialist (Pediatrics) & Pediatric Gastroenterologist, Army Hospital (R&R), New Delhi, India
| | - Elizabeth Scott
- Transplant Dietitian, Division of Gastroenterology, Hepatology & Nutrition, Children's Hospital Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA, USA
| | - Rohit Kohli
- Head of Division, Division of Gastroenterology, Hepatology & Nutrition, Children's Hospital Los Angeles, 4650 Sunset Boulevard, Los Angeles, CA, USA
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Body Composition of Infants With Biliary Atresia: Anthropometric Measurements and Computed Tomography-based Body Metrics. J Pediatr Gastroenterol Nutr 2020; 71:440-445. [PMID: 32694399 DOI: 10.1097/mpg.0000000000002859] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVES Biliary atresia (BA) causes neonatal cholestasis that requires hepatoportoenterostomy or liver transplantation (LT) for long-term survival. Nutritional optimization is necessary as sarcopenia and sarcopenic obesity have been associated with adverse clinical outcome. Currently, mid upper arm circumference (MUAC) is considered the most accurate indicator. The aim of the study was to determine computed tomography (CT)-based body metrics in infants with BA and to evaluate its correlation with MUAC. METHODS We retrospectively analyzed all BA infants below 2 years of age who underwent CT as part of LT screening at our hospital between 2006 and 2019. Measured variables were indexed with length and included: MUAC, total psoas muscle surface area (tPMSA), cross-sectional skeletal muscle area (CSMA), and total abdominal fat area. Intraclass correlation coefficients and Pearson coefficients were calculated. CSMA-to-abdominal fat area ratio was divided in quartiles, the lowest quartile group was considered sarcopenic obese. RESULTS Eighty infants with a median age of 4.6 months at LT screening were included. Intraclass correlation coefficients were: tPMSA = 0.94, CSMA = 0.92, and total abdominal fat area = 0.99. Correlation between MUAC z-score and indices of tPMSA, CSMA, and total abdominal fat area were r = 0.02, r = 0.06, and r = 0.43, respectively. The cut-off for sarcopenic obesity was CSMA-to-abdominal fat area ratio below 0.93. CONCLUSIONS In BA infants, it is possible to determine CT-based body metrics during LT screening with very strong interobserver agreement. Poor correlation between CT-based body metrics and MUAC suggests that CT-based body metrics provide additional information on body composition in BA infants, such as relative muscle mass.
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Chen X, Wang J, Lu Y, Xie X, Gu Y, Latour JM, Zhang Y. Feeding practices in 6-24-month-old children with chronic cholestatic liver diseases: a mixed-method study. BMC Pediatr 2020; 20:395. [PMID: 32838748 PMCID: PMC7445932 DOI: 10.1186/s12887-020-02290-8] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2020] [Accepted: 08/12/2020] [Indexed: 11/10/2022] Open
Abstract
Background Children with chronic cholestatic liver diseases have a high risk of malnutrition. However, nutritional management in China has received little attention, and there has been limited evidence regarding improving these practices. This study aimed to evaluate the feeding status of chronic cholestatic children aged 6–24 months and to explore their parents’ experiences with feeding practices. Methods A mixed-method study was conducted among parents of 74 children with chronic cholestatic liver diseases. The Infant and Young Child Feeding Index (ICFI) was used to collect quantitative data of feeding practices. In-depth interviews with parents were performed to generate qualitative data. Multivariate analysis was conducted to identify predictors of inappropriate feeding practices. Qualitative data were analyzed using thematic analysis. Results Only 16.2% of the children had appropriate feeding practices. In terms of dairy products, the rate of breastfeeding, medium-chain triglyceride formula feeding, and regular formula feeding were 25.7, 39.2 and 44.6% respectively. The complementary feeding rate was 68.8%, and the age of adding complementary foods was 6.9 ± 1.7 months. Consumption of foods from animal sources was suboptimal, 66.7% of the children aged 6–8 months and 45.5% of the children aged 9–11 months had carbohydrates as a single source of complementary foods and had no intake of meat, eggs or bean products, while in the age group 12–24 months, 52.0% of the children had eggs less than 2 days/week, 60.0% had meat less than 2 days/week, and 60.0% had no intake of bean products. Low literacy of the primary care provider was significantly related to inappropriate feeding practices (adjusted OR 5.52; 95% CI 1.29, 23.66). The result of the interviews indicated that parents generally lacked the scientific knowledge of feeding and thought that the intake of animal source foods and oils would be a burden to the liver and cause damage to the liver functions. Conclusion Parents generally lacked science-based feeding knowledge and the feeding practices in 6–24-month-old children diagnosed with chronic cholestatic liver diseases fell short of the recommendations in current nutritional guidelines. Appropriate interventions targeting social and cultural family circumstances should therefore be included in supporting parents in feeding practices to improve children’s clinical outcomes.
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Affiliation(s)
- Xiao Chen
- Department of Liver Disease, Children's Hospital of Fudan University, Shanghai, People's Republic of China.,Department of Nursing, Zhongshan Hospital of Fudan University, No.180, Fenglin road, Shanghai, People's Republic of China
| | - Jianshe Wang
- Department of Liver Disease, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Yi Lu
- Department of Liver Disease, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Xinbao Xie
- Department of Liver Disease, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Ying Gu
- Department of Nursing, Children's Hospital of Fudan University, Shanghai, People's Republic of China
| | - Jos M Latour
- School of Nursing and Midwifery, Faculty of Health and Human Sciences, University of Plymouth, Plymouth, UK
| | - Yuxia Zhang
- Department of Nursing, Zhongshan Hospital of Fudan University, No.180, Fenglin road, Shanghai, People's Republic of China.
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Nutrition Support of Children With Chronic Liver Diseases: A Joint Position Paper of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2019; 69:498-511. [PMID: 31436707 DOI: 10.1097/mpg.0000000000002443] [Citation(s) in RCA: 49] [Impact Index Per Article: 8.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Chronic liver disease places patients at increased risk of malnutrition that can be challenging to identify clinically and treat. Nutrition support is a key aspect of the management of these patients as it has an impact on their quality of life, morbidity, and mortality. There are significant gaps in the literature regarding the optimal nutrition support for patients with different types of liver diseases and the impact of these interventions on long-term outcomes. This Position Paper summarizes the available literature on the nutritional aspects of the care of patients with chronic liver diseases. Specifically, the challenges associated with the nutritional assessment of these subjects are discussed, and recently investigated approaches to determining the patients' nutritional status are reviewed. Furthermore, the pathophysiology of the malnutrition seen in the context of chronic liver disease is summarized and monitoring, as well as treatment, recommendations are provided. Lastly, suggestions for future research studies are described.
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Resting Energy Expenditure of Children With End-stage Chronic Liver Disease Before and After Liver Transplantation. J Pediatr Gastroenterol Nutr 2019; 69:102-107. [PMID: 30908388 DOI: 10.1097/mpg.0000000000002344] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
OBJECTIVES Our objective was to test the hypothesis that children with end-stage chronic liver disease (ESCLD) are hypermetabolic when compared to healthy children, and that this hypermetabolism persists for at least 6 months after liver transplant. METHODS Seventeen patients with end-stage chronic liver disease and 14 healthy controls had their resting energy expenditure measured (mREE) by indirect calorimetry. Weight, height, and body mass index were converted to standard deviation (SD) scores. Children older than 5 years had air displacement plethysmography and patients older than 5 years also had whole body dual-energy X-ray absorptiometry with characterization of fat mass (FM), fat-free mass (FFM), and bone-free fat free (lean) mass. RESULTS When compared to the prediction equation 44% of the patients and 50% of the healthy controls were hypermetabolic. The younger patients (0-5 years) had a lower mREE than the healthy controls but were significantly lighter and shorter than their healthy counterparts. mREE correlated strongly for all children with age, weight, height, and FFM. There was a strong negative correlation between age and mREE/kg in both patients (rs = -0.94, P < 0.01) and controls (rs = -0.91, P < 0.01). Almost 84% of the variance in mREE was explained by age (P < 0.001). There were no significant differences between resting energy expenditure (REE)/FFM between the 2 groups. mREE/kg before liver transplant correlated with mREE/kg after transplant (Pearson r = 0.83, P < 0.01). CONCLUSIONS REE mostly reflected the size of the child. The patients were not hypermetabolic when compared to the healthy children. The main determinant of REE/kg after transplant was REE/kg before transplant.
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Abstract
Malnutrition occurs when nutrient intake does not meet the needs for normal body functions and as a consequence leads to alterations of growth and development in children. Chronic illness puts children at risk for developing malnutrition. Because of children's rapid periods of growth and development, early diagnosis, prevention, and management of malnutrition are paramount. The reasons for malnutrition in children with chronic disease are multifactorial and are related to the underlying disease and non-illness-associated factors. This review addresses the causes, evaluation, and management of malnutrition in pediatric congenital heart disease, chronic kidney disease, liver disease, and cystic fibrosis.
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Affiliation(s)
- Catherine Larson-Nath
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of Minnesota, Minneapolis, Minnesota, USA
| | - Praveen Goday
- Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Medical College of Wisconsin, Milwaukee, Wisconsin, USA
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11
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Ruuska SM, Lääperi MT, Hukkinen M, Jalanko H, Kolho KL, Pakarinen MP. Growth of children with biliary atresia living with native livers: impact of corticoid therapy after portoenterostomy. Eur J Pediatr 2019; 178:341-349. [PMID: 30519970 PMCID: PMC6437119 DOI: 10.1007/s00431-018-3302-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2018] [Revised: 11/05/2018] [Accepted: 11/27/2018] [Indexed: 12/24/2022]
Abstract
We addressed growth of biliary atresia (BA) patients living with native livers between ages 0-6 and effects of post-surgical corticosteroid treatment on growth. Growth charts of 28 BA patients born in Finland between 1987 and 2017 were retrospectively evaluated. Dosage and length of corticosteroid treatment and hydrocortisone substitution were reviewed. At birth, BA patients were shorter (median height - 0.6 (interquartile range (IQR) - 1.3 to - 0.1) SDS, n = 28, P < 0.001) than general population. Height remained stable during early childhood (median height - 0.6 (IQR - 1.4 to 0.1) SDS for girls and - 0.4 (IQR - 1.6 to 0.2) SDS for boys at 6 years of age). Patients were of normal height adjusted weight at 6 years with a median age and sex-adjusted body mass index (ISO-BMI) of 20.9 (IQR 19.3 to 25.0) for girls and 22.1 (IQR 20.7 to 25.6) for boys. Higher (≥ 50 mg/kg) cumulative post-portoenterostomy prednisolone dosage resulted in 0.18 SDS lower height per treatment week (β - 0.18, SE 0.04, P < 0.001) compared to lower dosage (< 50 mg/kg).Conclusion: BA patients grow normally during early childhood. As high postoperative corticosteroid dosage has a short-term negative effect on height, very high dosages should be avoided. What Is Known: • Growth of biliary atresia patients has mostly been shown to be within normal limits • Corticosteroids may decrease growth rate What Is New: • Biliary atresia patients surviving with their native livers are shorter than general population and their mid-parental target height at birth • A high (> 50 mg/kg) cumulative prednisolone dosage has a negative transitory impact on height gain after portoenterostomy.
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Affiliation(s)
- Satu Maria Ruuska
- Department of Gastroenterology, Children's Hospital, Helsinki University Hospital, PL 347, 00029 HUS, Helsinki, Finland. .,Pediatric Liver and Gut Research Group, Children's Hospital, Helsinki University Hospital, Helsinki, Finland.
| | - Mitja Tapani Lääperi
- 0000 0000 9950 5666grid.15485.3dPediatric Research Center, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland
| | - Maria Hukkinen
- 0000 0000 9950 5666grid.15485.3dPediatric Liver and Gut Research Group, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland ,0000 0000 9950 5666grid.15485.3dSection of Pediatric Surgery, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland
| | - Hannu Jalanko
- 0000 0000 9950 5666grid.15485.3dDepartment of Pediatric Nephrology and Transplantation, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland
| | - Kaija-Leena Kolho
- 0000 0001 2314 6254grid.502801.eUniversity of Tampere, Tampere, Finland ,0000 0004 0628 2985grid.412330.7Tampere University Hospital, Tampere, Finland
| | - Mikko P. Pakarinen
- 0000 0000 9950 5666grid.15485.3dPediatric Liver and Gut Research Group, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland ,0000 0000 9950 5666grid.15485.3dPediatric Research Center, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland ,0000 0000 9950 5666grid.15485.3dSection of Pediatric Surgery, Children’s Hospital, Helsinki University Hospital, Helsinki, Finland
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12
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Abstract
More than one-half of children with chronic liver disease suffer from malnutrition, which leads not only to a poor quality of life and even possibly catastrophic complications, but also to poor outcomes after a liver transplantation. These children have increased metabolic demands but often decreased intake with malabsorption and altered nutrient utilization, all of which make it difficult to keep up with nutritional demands. Assessment of a patient's nutritional status should be timely, and it should be performed routinely and proactively. When specific nutritional needs are identified, these should be addressed with a multidisciplinary team approach and with the close guidance of an experienced pediatric dietician. The assessment includes anthropometric and laboratory assessments, in addition to a careful physical examination and a detailed patient history. The specific nutritional needs vary, but generally dietary intervention focuses on increasing caloric intake, supplementation with medium-chain triglycerides, and prevention of essential fatty acid and fat-soluble vitamin deficiencies. [Pediatr Ann. 2018;47(11):e445-e451.].
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13
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Haafiz AB. A mechanism based approach to management of children with end-stage liver disease. Expert Rev Gastroenterol Hepatol 2017; 11:1085-1094. [PMID: 28803487 DOI: 10.1080/17474124.2017.1367662] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Due to parallel advances in surgical and acute care disciplines, liver transplantation (LT) has revolutionized the outlook for children with end-stage liver disease (ESLD). Contrary to advances in technical aspects of LT and the peri-operative care, pre-transplant management of ESLD remains quite a formidable challenge. Areas covered: This review provides mechanisms based management strategies to address common complications of ESLD including malnutrition, amended metabolic pathways, gastrointestinal dysfunction, and development of ascites. Clinically relevant discussion of each paradigm is followed by an account of high impact therapeutic interventions which can be used as guides for formulating management plans. A tabulated summary of the suggested interventions is also provided. Indeed, execution of a dynamic plan tailored to the evolution of pathophysiologic derangements can further enhance outcomes of pediatric LT. Expert commentary: LT has evolved as a dependable therapeutic option for a variety of fatal pediatric liver diseases. However, relative organ shortage remains a formidable challenge. Similarly, consumer expectations continue to grow for sustained improvement of graft and patient survival after LT. In this environment, the level of sophistication applied to the management ESLD before LT stands out as a major opportunity with lasting impact on the future of pediatric LT.
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Affiliation(s)
- Allah B Haafiz
- a Pediatric Transplant Hepatology, Organ Transplant and Hepatobiliary Surgery , King Abdullah Specialized Children Hospital , Riyadh , KSA
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14
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Yang CH, Perumpail BJ, Yoo ER, Ahmed A, Kerner JA. Nutritional Needs and Support for Children with Chronic Liver Disease. Nutrients 2017; 9:nu9101127. [PMID: 29035331 PMCID: PMC5691743 DOI: 10.3390/nu9101127] [Citation(s) in RCA: 45] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/22/2017] [Revised: 10/08/2017] [Accepted: 10/11/2017] [Indexed: 12/31/2022] Open
Abstract
Malnutrition has become a dangerously common problem in children with chronic liver disease, negatively impacting neurocognitive development and growth. Furthermore, many children with chronic liver disease will eventually require liver transplantation. Thus, this association between malnourishment and chronic liver disease in children becomes increasingly alarming as malnutrition is a predictor of poorer outcomes in liver transplantation and is often associated with increased morbidity and mortality. Malnutrition requires aggressive and appropriate management to correct nutritional deficiencies. A comprehensive review of the literature has found that infants with chronic liver disease (CLD) are particularly susceptible to malnutrition given their low reserves. Children with CLD would benefit from early intervention by a multi-disciplinary team, to try to achieve nutritional rehabilitation as well as to optimize outcomes for liver transplant. This review explains the multifactorial nature of malnutrition in children with chronic liver disease, defines the nutritional needs of these children, and discusses ways to optimize their nutritional.
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Affiliation(s)
- Christine H Yang
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Lucile Packard Children's Hospital, Palo Alto, Stanford, CA 94304, USA.
| | - Brandon J Perumpail
- Department of Medicine, Drexel University College of Medicine, Philadelphia, PA 19129, USA.
| | - Eric R Yoo
- Department of Medicine, Santa Clara Valley Medical Center, San Jose, CA 95128, USA.
| | - Aijaz Ahmed
- Division of Gastroenterology and Hepatology, Stanford University School of Medicine, Stanford, CA 94305, USA.
| | - John A Kerner
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Lucile Packard Children's Hospital, Palo Alto, Stanford, CA 94304, USA.
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Affiliation(s)
- Wednesday Marie A Sevilla
- Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA
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Abstract
Liver transplantation originated in children more than 50 years ago, and these youngest patients, while comprising the minority of liver transplant recipients nationwide, can have some of the best and most rewarding outcomes. The indications for liver transplantation in children are generally more diverse than those seen in adult patients. This diversity in underlying cause of disease brings with it increased complexity for all who care for these patients. Children, still being completely dependent on others for survival, also require a care team that is able and ready to work with parents and family in addition to the patient at the center of the process. In this review, we aim to discuss diagnoses of particular uniqueness or importance to pediatric liver transplantation. We also discuss the evaluation of a pediatric patient for liver transplant, the system for allocating them a new liver, and also touch on postoperative concerns that are unique to the pediatric population.
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Carpenter A, Ng VL, Chapman K, Ling SC, Mouzaki M. Predictive Equations Are Inaccurate in the Estimation of the Resting Energy Expenditure of Children With End-Stage Liver Disease. JPEN J Parenter Enteral Nutr 2016. [DOI: 10.1177/0148607115597666] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Affiliation(s)
- Andrea Carpenter
- Department of Clinical Dietetics, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Vicky Lee Ng
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
- SickKids Transplant Center, Toronto, Ontario, Canada
| | - Karen Chapman
- Clinical Research Unit, Hospital for Sick Children, Toronto, Ontario, Canada
| | - Simon C. Ling
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
| | - Marialena Mouzaki
- Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
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Santetti D, de Albuquerque Wilasco MI, Dornelles CTL, Werlang ICR, Fontella FU, Kieling CO, dos Santos JL, Vieira SMG, Goldani HAS. Serum proinflammatory cytokines and nutritional status in pediatric chronic liver disease. World J Gastroenterol 2015; 21:8927-8934. [PMID: 26269683 PMCID: PMC4528036 DOI: 10.3748/wjg.v21.i29.8927] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/20/2014] [Revised: 01/06/2015] [Accepted: 02/13/2015] [Indexed: 02/06/2023] Open
Abstract
AIM: To evaluate the nutritional status and its association with proinflammatory cytokines in children with chronic liver disease.
METHODS: We performed a cross-sectional study with 43 children and adolescents, aged 0 to 17 years, diagnosed with chronic liver disease. All patients regularly attended the Pediatric Hepatology Unit and were under nutritional follow up. The exclusion criteria were fever from any etiology at the time of enrollment, inborn errors of the metabolism and any chronic illness. The severity of liver disease was assessed by Child-Pugh, Model for End-stage Liver Disease (MELD) and Pediatric End Stage Liver Disease (PELD) scores. Anthropometric parameters were height/age, body mass index/age and triceps skinfold/age according to World Health Organization standards. The cutoff points for nutritional status were risk of malnutrition (Z-score < -1.00) and malnutrition (Z-score < -2.00). Interleukin-1β (IL-1β), IL-6 and tumor necrosis factor-α levels were assessed by commercial ELISA kits. For multivariate analysis, linear regression was applied to assess the association between cytokine levels, disease severity and nutritional status.
RESULTS: The median (25th-75th centile) age of the study population was 60 (17-116)-mo-old, and 53.5% were female. Biliary atresia was the main cause of chronic liver disease (72%). With respect to Child-Pugh score, cirrhotic patients were distributed as follows: 57.1% Child-Pugh A, a mild presentation of the disease, 34.3% Child-Pugh B, a moderate stage of cirrhosis and 8.6% Child-Pugh C, were considered severe cases. PELD and MELD scores were only above the cutoff point in 5 cases. IL-6 values were increased in patients at nutritional risk (34.9%) compared with those who were well-nourished [7.12 (0.58-34.23) pg/mL vs 1.63 (0.53-3.43) pg/mL; P = 0.02], correlating inversely with triceps skinfold-for-age z-score (rs = -0.61; P < 0.001). IL-6 levels were associated with liver disease severity assessed by Child-Pugh score (P = 0.001). This association remained significant after adjusting for nutritional status in a linear regression model.
CONCLUSION: High IL-6 levels were found in children with chronic liver disease at nutritional risk. Inflammatory activity may be related to nutritional status deterioration in these patients.
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Carpenter A, Pencharz P, Mouzaki M. Accurate estimation of energy requirements of young patients. J Pediatr Gastroenterol Nutr 2015; 60:4-10. [PMID: 25238120 DOI: 10.1097/mpg.0000000000000572] [Citation(s) in RCA: 40] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
The provision of optimal nutritional care is based on accurate estimations of patients' resting energy expenditure. The latter can be calculated with the use of predictive equations or measured with indirect calorimetry (IC). Owing to their ease of use, mathematical equations have largely replaced IC in clinical practice. This article examines the limitations and predictive inaccuracy of commonly used equations in pediatrics, which may contribute to the provision of poor nutritional care and directly affect patient outcomes. In addition, the role of IC is discussed and the physiology of nutrient metabolism, in terms of energy expenditure, is reviewed.
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Affiliation(s)
- Andrea Carpenter
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Canada
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20
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Kyle UG, Shekerdemian LS, Coss-Bu JA. Growth failure and nutrition considerations in chronic childhood wasting diseases. Nutr Clin Pract 2014; 30:227-38. [PMID: 25378356 DOI: 10.1177/0884533614555234] [Citation(s) in RCA: 54] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023] Open
Abstract
Growth failure is a common problem in many children with chronic diseases. This article is an overview of the most common causes of growth failure/growth retardation that affect children with a number of chronic diseases. We also briefly review the nutrition considerations and treatment goals. Growth failure is multifactorial in children with chronic conditions, including patients with cystic fibrosis, chronic kidney disease, chronic liver disease, congenital heart disease, human immunodeficiency virus, inflammatory bowel disease, short bowel syndrome, and muscular dystrophies. Important contributory factors to growth failure include increased energy needs, increased energy loss, malabsorption, decreased energy intake, anorexia, pain, vomiting, intestinal obstruction, and inflammatory cytokines. Various metabolic and pathologic abnormalities that are characteristic of chronic diseases further lead to significant malnutrition and growth failure. In addition to treating disease-specific abnormalities, treatment should address the energy and protein deficits, including vitamin and mineral supplements to correct deficiencies, correct metabolic and endocrinologic abnormalities, and include long-term monitoring of weight and growth. Individualized, age-appropriate nutrition intervention will minimize the malnutrition and growth failure seen in children with chronic diseases.
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Affiliation(s)
- Ursula G Kyle
- Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas
| | - Lara S Shekerdemian
- Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas
| | - Jorge A Coss-Bu
- Section of Critical Care Medicine, Department of Pediatrics, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas
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21
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Mouzaki M, Ng V, Kamath BM, Selzner N, Pencharz P, Ling SC. Enteral Energy and Macronutrients in End-Stage Liver Disease. JPEN J Parenter Enteral Nutr 2014; 38:673-681. [DOI: 10.1177/0148607114522488] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/30/2023]
Affiliation(s)
- Marialena Mouzaki
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto
- Department of Paediatrics, University of Toronto, Toronto
| | - Vicky Ng
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto
- Department of Paediatrics, University of Toronto, Toronto
| | - Binita M. Kamath
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto
- Department of Paediatrics, University of Toronto, Toronto
| | - Nazia Selzner
- Division of Medicine, University Health Network, Toronto
| | - Paul Pencharz
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto
- Department of Paediatrics, University of Toronto, Toronto
| | - Simon C. Ling
- Division of Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto
- Department of Paediatrics, University of Toronto, Toronto
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22
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Squires RH, Ng V, Romero R, Ekong U, Hardikar W, Emre S, Mazariegos GV. Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Hepatology 2014; 60:362-98. [PMID: 24782219 DOI: 10.1002/hep.27191] [Citation(s) in RCA: 142] [Impact Index Per Article: 12.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/22/2014] [Accepted: 04/22/2014] [Indexed: 12/16/2022]
Affiliation(s)
- Robert H Squires
- Department of Pediatrics, University of Pittsburgh School of Medicine; Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA
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23
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Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 2014; 59:112-31. [PMID: 25222807 DOI: 10.1097/mpg.0000000000000431] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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24
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Variability of resting energy expenditure in infants and young children with intestinal failure-associated liver disease. J Pediatr Gastroenterol Nutr 2014; 58:637-41. [PMID: 24361903 PMCID: PMC5025756 DOI: 10.1097/mpg.0000000000000288] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
OBJECTIVE The aim of the study was to determine, in a cohort of young children with intestinal failure (IF), whether the estimates of basal metabolic rate (BMR) by standard equations can approximate measured resting energy expenditure (REE) by indirect calorimetry (IC). METHODS IC was performed using the dilutional canopy technique. REE measurements were compared with standard, age-based estimation equations (World Health Organization) for BMR. Subjects were classified as hypermetabolic (REE > 110% BMR), hypometabolic (REE < 90% BMR), or normal (REE = 90%-110% BMR). RESULTS Twenty-eight IF patients (11 girls, 17 boys) had an underlying diagnosis of necrotizing enterocolitis (n = 10) or a congenital gastrointestinal defect (n = 18). Median age was 5.3 months. Median interquartile range (IQR) REE was 46 (42-58) kcal · kg · day. Median (IQR) total energy intake provided 209% (172%-257%) of REE, with parenteral nutrition providing 76% (23%) of total energy intake. REE was variable, with 39% (n = 11) of measurements hypermetabolic, 39% (n = 11) hypometabolic, and the remaining 21% (n = 6) normal. Although REE was well correlated with estimated BMR (r = 0.82, P < 0.0001), estimated BMR was not consistently an adequate predictor of REE. BMR over- or underestimated REE by >10 kcal · kg · day in 15 of 28 (54%) patients. REE was not significantly correlated with severity of liver disease, nutritional status, total energy intake, or gestational age. CONCLUSIONS Energy expenditure is variable among children with IF and IF-associated liver disease, with approximately 80% of our cohort exhibiting either hypo- or hypermetabolism. Standard estimation equations frequently do not correctly predict individual REE. Longitudinal studies of energy expenditure and body composition may be needed to guide provision of nutrition regimens.
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25
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Young S, Kwarta E, Azzam R, Sentongo T. Nutrition assessment and support in children with end-stage liver disease. Nutr Clin Pract 2013; 28:317-29. [PMID: 23466471 DOI: 10.1177/0884533612474043] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/06/2023] Open
Abstract
Malnutrition is a treatable complication in children with end-stage liver disease (ESLD). Biliary atresia and other cholestatic disorders are the most frequent cause of ESLD in children. No single variable provides adequate information about nutrition status, yet effective nutrition support is the one intervention known to improve pre- and posttransplant outcomes. A proactive approach consisting of screening anthropometry interpreted using appropriate growth references, recognition of clinical manifestations associated with micronutrient deficiency, and timely aggressive nutrition support is of a paramount importance to maximize anabolism and optimize outcomes. This article presents the principles of nutrition assessment, intervention, and monitoring in children with ESLD.
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Affiliation(s)
- Sona Young
- University of Chicago, Chicago, IL 60637, USA
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26
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Emre S, Umman V, Cimsit B, Rosencrantz R. Current concepts in pediatric liver transplantation. THE MOUNT SINAI JOURNAL OF MEDICINE, NEW YORK 2012; 79:199-213. [PMID: 22499491 DOI: 10.1002/msj.21305] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Liver transplantation is the definitive treatment for end-stage liver disease in both children and adults. Advances over the last 2 decades have resulted in excellent patient and graft survival rates in what were previously cases of fatal disorders. These developments have been due to innovations in surgical technique, increased surgical experience, refinements in immunosuppressive regimens, quality improvements in intraoperative anesthetic management, better understanding of the pathophysiology of the liver diseases, and better preoperative and postoperative care. Remarkably, the use of split-liver and living-related liver transplantation surgical techniques has helped mitigate the well-recognized national organ shortage. This review will discuss the major aspects of pediatric liver transplantation as it pertains to indication for transplantation, recipient selection and listing for orthotopic liver transplantation, pre-orthotopic liver transplantation care of children, optimal timing of orthotopic liver transplantation, surgical technical considerations, postoperative care and complications, and patient and graft survival outcomes.
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Affiliation(s)
- Sukru Emre
- Yale University School of Medicine, New Haven, CT, USA.
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27
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Sullivan JS, Sundaram SS, Pan Z, Sokol RJ. Parenteral nutrition supplementation in biliary atresia patients listed for liver transplantation. Liver Transpl 2012; 18:120-8. [PMID: 21987426 PMCID: PMC3245380 DOI: 10.1002/lt.22444] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
The objective of this study was to determine the impact of parenteral nutrition (PN) on the outcomes of biliary atresia (BA) patients listed for liver transplantation (LT). We retrospectively reviewed the charts of all BA patients at our institution who underwent hepatoportoenterostomy and were listed for LT before the age of 36 months between 1990 and 2010. The initiation of PN was based on clinical indications. Twenty-five PN subjects and 22 non-PN subjects (74% female) were studied. The median PN initiation age was 7.7 months, the mean duration was 86 days, and the mean amount of energy supplied by PN was 77 kcal/kg/day. Before PN, the triceps skinfold thickness (TSF) and the mid-arm circumference (MAC) z scores were decreasing. After PN, TSF (P < 0.001) and MAC (P < 0.001) improved significantly. The PN group had lower MAC and TSF scores than the non-PN group at the time of LT listing. Between listing and LT, MAC and TSF improved in the PN group and worsened in the non-PN groups; as a result, the 2 groups had the same z scores at LT. The PN group had a higher incidence of gastrointestinal bleeding and ascites before LT, but there were no differences in the rates of pre-LT bacteremia, days in the intensive care unit after LT, or patient or graft survival. In conclusion, PN improves the nutritional status of malnourished BA patients awaiting LT, and this is associated with post-LT outcomes comparable to those of patients not requiring PN.
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Affiliation(s)
- Jillian S Sullivan
- Section of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, and the Digestive Health Institute, Children’s Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado
| | - Shikha S Sundaram
- Section of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, and the Digestive Health Institute, Children’s Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado
| | - Zhaoxing Pan
- Department of Pediatrics, Children’s Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado,Department of Biostatistics and Informatics, Colorado School of Public Health, Aurora, Colorado,Clinical and Translational Sciences Institute, University of Colorado Denver, Aurora, Colorado
| | - Ronald J Sokol
- Section of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, and the Digestive Health Institute, Children’s Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado,Clinical and Translational Sciences Institute, University of Colorado Denver, Aurora, Colorado
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28
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Smart KM, Alex G, Hardikar W. Feeding the child with liver disease: a review and practical clinical guide. J Gastroenterol Hepatol 2011; 26:810-5. [PMID: 21299619 DOI: 10.1111/j.1440-1746.2011.06687.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Nourishing children with liver disease is a challenging task; however, appropriate assessment and well-timed nutritional interventions are paramount for a good long-term outcome in these patients. An appropriate balance of macronutrients, micronutrients, and vitamins is important, as is the route of administration. This review aims to highlight the practical points in nutrition assessment and also provides a guide to the various nutritional interventions available for children with chronic liver disease.
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29
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Saron MLG, Godoy HT, Hessel G. Nutritional status of patients with biliary atresia and autoimmune hepatitis related to serum levels of vitamins A, D and E. ARQUIVOS DE GASTROENTEROLOGIA 2009; 46:62-8. [PMID: 19466312 DOI: 10.1590/s0004-28032009000100016] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/25/2008] [Accepted: 05/20/2008] [Indexed: 11/22/2022]
Abstract
CONTEXT Chronic liver disease may induce to malabsorption of lipids and fat-soluble vitamins, leading to injury of nutritional status. OBJECTIVES To evaluate the nutritional status of pediatric-age patients with autoimmune hepatitis and biliary atresia related to serum levels of vitamins A, D and E and the disease severity. METHODS This controlled transverse study, evaluated the patients with autoimmune hepatitis and biliary atresia and a reference group paired by sex and age. The patients underwent anthropometric evaluation, alimentary inquiry and determination of serum levels of vitamins A, D and E by high performance liquid chromatography. The Mann-Whitney test, Spearman correlation coefficients and variance analysis (ANOVA) were utilized for data treatment, regarding significant difference if P<0.05. RESULTS The highest nutritional deficit was observed in patients with biliary atresia, mainly with cholestasis. The serum levels of vitamins A and E for the reference group changed as a function of age. The serum levels of vitamins A, D and E were higher in reference group than in patients with biliary atresia and autoimmune hepatitis together or separately. There were not difference in the serum levels of vitamins A, D and E between biliary atresia groups with cholestasis and without cholestasis. It was verified correlation between weight/age, triceps skinfold thickness, subscapular skinfold thickness, midarm circumference, midarm fat area values and vitamin A serum levels, as well as between all anthropometric indicators and vitamin E in patients with autoimmune hepatitis and biliary atresia. CONCLUSION The patients with biliary atresia and cholestasis presented the highest nutritional injury. The patients with biliary atresia and autoimmune hepatitis presented lower serum levels of vitamins A, D and E that in control group. There is a directly proportional correlation between vitamin serum levels, mainly vitamin E, and all anthropometric variables of biliary atresia and autoimmune hepatitis groups.
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Affiliation(s)
- Margareth L G Saron
- Department of Pediatrics, Universidade Estadual de Campinas, Campinas, SP, Brazil
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30
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Cleghorn G. The role of basic nutritional research in pediatric liver disease: An historical perspective. J Gastroenterol Hepatol 2009; 24 Suppl 3:S93-6. [PMID: 19799706 DOI: 10.1111/j.1440-1746.2009.06078.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
The advent of liver transplantation for end-stage liver disease (ESLD) in children has necessitated a major rethink in the preoperative preparation and management from simple palliative care to active directed intervention. This is particularly evident in the approach to the nutritional care of these patients with the historical understanding of the nutritional pertubations in ESLD being described from a single pediatric liver transplant center. ESLD in children is a hypermetabolic process adversely affecting nutritional status, metabolic, and non-metabolic body compartments. There is a complex dynamic process affecting metabolic activity within the metabolically active body cell mass, as well as lipid oxidation during fasting and at rest, with other factors operating in conjunction with daily activities. We have proposed that immediately ingested nutrients are a more important source of energy in patients with ESLD than in healthy children, among whom energy may be stored in various body compartments.
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Affiliation(s)
- Geoffrey Cleghorn
- The School of Medicine, University of Queensland, Herston, Queensland 4006, Australia.
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31
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Nightingale S, Ng VL. Optimizing nutritional management in children with chronic liver disease. Pediatr Clin North Am 2009; 56:1161-83. [PMID: 19931069 DOI: 10.1016/j.pcl.2009.06.005] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Malnutrition is common in infants and children with chronic liver disease (CLD) and may easily be underestimated by clinical appearance alone. The cause of malnutrition in CLD is multifactorial, although insufficient dietary intake is probably the most important factor and is correctable. Fat malabsorption occurs in cholestatic disorders, and one must also consider any accompanying fat-soluble vitamin and essential fatty acid deficiencies. The clinician should proactively evaluate, treat, and re-evaluate response to treatment of nutritional deficiencies. Because a better nutritional state is associated with better survival before and after liver transplantation, aggressive nutritional management is an important part of the care of these children.
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Affiliation(s)
- Scott Nightingale
- SickKids Transplant Center, Division of Gastroenterology, Hepatology, and Nutrition, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
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32
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Erlichman J, Hohlweg K, Haber BA. Biliary atresia: how medical complications and therapies impact outcome. Expert Rev Gastroenterol Hepatol 2009; 3:425-34. [PMID: 19673629 DOI: 10.1586/egh.09.30] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. Untreated, BA is a uniformly fatal disease and, yet, even with our existing therapies, at least 50% of children with BA will undergo liver transplantation by the age of 2 years. Current treatment strategies are, at best, palliative; they focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. The purpose of this article is to discuss the current treatment paradigm for BA and to assess the impact these strategies have on outcomes. As more children with BA survive into adulthood with their native liver, it is important to understand which factors predict good and poor outcomes.
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Affiliation(s)
- Jessi Erlichman
- Division of GI, Hepatology and Nutrition, The Childrens' Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, USA
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33
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Alonso EM, Shepherd R, Martz KL, Yin W, Anand R. Linear growth patterns in prepubertal children following liver transplantation. Am J Transplant 2009; 9:1389-97. [PMID: 19459820 DOI: 10.1111/j.1600-6143.2009.02634.x] [Citation(s) in RCA: 41] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Factors impacting linear growth following pediatric liver transplantation (LT) are not well understood. This longitudinal analysis examines predictors of linear growth impairment in prepubertal children included in Studies of Pediatric Liver Transplantation. In 1143 children with serial measurements, mean height scores increased from -1.55 at LT to -0.87 and -0.68 at 24 and 36 months post LT with minimal subsequent catch up growth observed until 60 months. Subgroup analysis of height measurements at 24 months (n = 696), 33.8% were below 10th percentile at 24 months post LT. Multivariate analysis revealed linear growth impairment more likely in patients with metabolic disease (OR 4.4, CI: 1.83-10.59) and >18 months of steroids exposure (OR 3.02, CI: 1.39-6.55). Higher percentiles for weight (OR 0.80, CI: 0.65-0.99) and height (OR 0.62, CI: 0.51-0.77) at LT decreased risk. Less linear catch up was observed in patients with metabolic disease, non-Biliary atresia cholestatic diseases and lower weight and higher height percentiles prior to LT. Prolonged steroid exposure and elevated calculated glomerular filtration rate and gamma-Glutamyltransferase following LT were associated with less catch up growth. Linear growth impairment and incomplete linear catch up growth are common following LT and may improve by avoiding advanced growth failure before LT and steroid exposure minimization.
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Affiliation(s)
- E M Alonso
- Children's Memorial Hospital, Pediatrics, Chicago, IL, USA.
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Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs 2009; 17:1911-24. [PMID: 19012506 DOI: 10.1517/13543780802514120] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
BACKGROUND Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction before 2 months of age. Untreated BA is a uniformly fatal disease and even with our current therapies only 50% of children with BA will be transplant-free by 2 years of age. Despite descriptions of this disorder dating back to the 1800s our current therapies are palliative. They focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. OBJECTIVE To present the literature supporting current treatment strategies and potential future therapies. METHOD Each of the aspects of care is described and the literature about nuances of care is provided. CONCLUSION Therapies will not improve outcomes until novel treatments are introduced, such as those suggested, which may intervene in the inflammatory or fibrotic steps of the disease process.
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Affiliation(s)
- Barbara A Haber
- Associate Professor of Pediatrics The Childrens Hospital of Philadelphia, Division of GI, Hepatology & Nutrition, Philadelphia, PA 19104, USA.
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35
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Schneider ACR, Pinto RB, Silveira TRD. [Nutritional risk and malnutrition determination by anthropometry in cirrhotic children and adolescents]. ARQUIVOS DE GASTROENTEROLOGIA 2008; 44:345-9. [PMID: 18317655 DOI: 10.1590/s0004-28032007000400012] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
BACKGROUND The malnutrition is a frequent finding in adults with cirrhosis, but the prevalence of nutritional risk and malnutrition is little known in pediatric patients. AIM To evaluate through anthropometry the presence of nutritional risk and malnutrition in cirrhotic pediatric patients regularly attended at the Pediatric Gastroenterology Service of "Hospital de Clínicas" of Porto Alegre, RS, Brazil. METHODS Cross-sectional study with 42 cirrhotic children and adolescents aged between 3 months and 18 years. The nutritional evaluation was made by the determination of the weight/age, height/age, body mass index and triceps skinfold thickness and arm muscle circumference measurements. Patients considered in nutritional risk were < or = -1,28 Z score which corresponds to < or = 10th percentile, and those under -2,0 Z and < or = 3th percentile were in malnutrition status. According to Child-Pugh criteria, 22 patients were classified as A (mild severity), 15 (moderate) B and 5 C (intense). RESULTS The mean weight/age, height/age and body mass index Z scores were, respectively, - 0,38 +/- 1,4 SD, - 0,83 +/- 1,16 SD and 0,17 +/- 1,3 SD. Patients in nutritional risk were 3/42 (weight/age), 8/42 (height/age), 12/37 (triceps skinfold thickness), 9/37 (arm muscle circumference), 2/38 (body mass index); in malnutrition status were 6/42 (weight/age), 7/42 (height/age), 4/37 (triceps skinfold thickness) and 4/37 (arm muscle circumference) and 3/38 (body mass index). CONCLUSION The prevalence of nutritional risk was 32.4% and chronic malnutrition was 16.7%. The index which better reflected the nutritional risk in these patients was triceps skinfold thickness. Chronic malnutrition status occurrence was greater in the height/age index.
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Affiliation(s)
- Ana Cláudia Reis Schneider
- Setor de Gastroenterologia Pediátrica, Departamento de Pediatria do Hospital de Clínicas de Porto Alegre, RS, Brazil
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36
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Bluck LJC. Doubly labelled water for the measurement of total energy expenditure in man – progress and applications in the last decade. NUTR BULL 2008. [DOI: 10.1111/j.1467-3010.2008.00695.x] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Affiliation(s)
- Estella M Alonso
- Department of Pediatrics, Northwestern University Feinberg School of Medicine, Children's Memorial, Hospital, Chicago, IL 60614, USA.
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Baker A, Stevenson R, Dhawan A, Goncalves I, Socha P, Sokal E. Guidelines for nutritional care for infants with cholestatic liver disease before liver transplantation. Pediatr Transplant 2007; 11:825-34. [PMID: 17976116 DOI: 10.1111/j.1399-3046.2007.00792.x] [Citation(s) in RCA: 53] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
Infants with cholestatic liver disease are at risk of various nutritional deficiencies. They require regular review with appropriate interventions to prevent complications and ensure optimum possible status if liver transplantation becomes necessary. We propose evidence based and internationally agreed guidelines for their care with recommendations for therapeutic strategies and for service organization.
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Affiliation(s)
- Alastair Baker
- Paediatric Liver Department, King's College Hospital, London, UK.
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DeRusso PA, Ye W, Shepherd R, Haber BA, Shneider BL, Whitington PF, Schwarz KB, Bezerra JA, Rosenthal P, Karpen S, Squires RH, Magee JC, Robuck PR, Sokol RJ, for the Biliary Atresia Research Consortium. Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium. Hepatology 2007; 46:1632-8. [PMID: 17929308 PMCID: PMC3881187 DOI: 10.1002/hep.21923] [Citation(s) in RCA: 71] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
UNLABELLED Malnutrition is a significant clinical problem in infants with biliary atresia. The natural history of poor growth and its potential association with early transplantation or death in children with biliary atresia was determined. Serial weight- and length-for-age z-scores were computed as part of a retrospective study of 100 infants who underwent hepatoportoenterostomy (HPE) for biliary atresia at 9 U.S. pediatric centers between 1997 and 2000. Poor outcome was defined as transplantation or death by 24 months of age (n = 46) and good outcome was defined as survival with native liver at 24 months of age with total serum bilirubin less than 6 mg/dL (n = 54). Growth velocity was significantly slower in the poor outcome group compared to the good outcome group (P < 0.001 for both weight and length). Mean weight z-scores were significantly lower by 6 months after HPE in the poor outcome group (-2.1 +/- 1.4) compared to the good outcome group (-1.2 +/- 1.4) (P < 0.001). In a subgroup with total bilirubin between 2 and 6 mg/dL at 3 months after HPE (n = 28), the weight z-scores at 3 months after HPE were significantly lower in the poor outcome group (-2.0 +/-1.2) compared to the good outcome group (-1.0 +/- 1.2) (P = 0.04) despite similar bilirubin concentrations. CONCLUSION Growth failure after HPE was associated with transplantation or death by 24 months of age. The combination of intermediate bilirubin concentrations and poor mean weight z-scores 3 months after HPE was also associated with poor clinical outcome.
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Affiliation(s)
- Patricia A. DeRusso
- Johns Hopkins School of Medicine and The Johns Hopkins Children's Center, Baltimore, Maryland
| | - Wen Ye
- The University of Michigan, Ann Arbor
| | - Ross Shepherd
- Washington University School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri
| | - Barbara A. Haber
- The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
| | | | | | - Kathleen B. Schwarz
- Johns Hopkins School of Medicine and The Johns Hopkins Children's Center, Baltimore, Maryland
| | | | - Philip Rosenthal
- University of California, San Francisco, San Francisco, California
| | - Saul Karpen
- Texas Children's Hospital and Baylor College of Medicine, Houston, Texas
| | | | | | - Patricia R. Robuck
- National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland
| | - Ronald J. Sokol
- University of Colorado School of Medicine and The Children's Hospital, Denver, Colorado
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Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology 2007; 46:566-81. [PMID: 17661405 PMCID: PMC3888317 DOI: 10.1002/hep.21790] [Citation(s) in RCA: 181] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Biliary atresia is the most common cause of end-stage liver disease in the infant and is the leading pediatric indication for liver transplantation in the United States. Earlier diagnosis (<30-45 days of life) is associated with improved outcomes following the Kasai portoenterostomy and longer survival with the native liver. However, establishing this diagnosis is problematic because of its rarity, the much more common indirect hyperbilirubinemia that occurs in the newborn period, and the schedule for routine infant health care visits in the United States. The pathogenesis of biliary atresia appears to involve immune-mediated fibro-obliteration of the extrahepatic and intrahepatic biliary tree in most patients and defective morphogenesis of the biliary system in the remainder. The determinants of the outcome of portoenterostomy include the age at surgery, the center's experience, the presence of associated congenital anomalies, and the postoperative occurrence of cholangitis. A number of screening strategies in infants have been studied. The most promising are early measurements of serum conjugated bilirubin and a stool color card given to new parents that alerts them and their primary care provider to alcholic stools. This report summarizes a National Institutes of Health workshop held on September 12 and 13, 2006, in Bethesda, MD, that addressed the issues of outcomes, screening, and pathogenesis of biliary atresia.
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Affiliation(s)
- Ronald J Sokol
- Department of Pediatrics, University of Colorado School of Medicine, The Children's Hospital, Denver, CO, USA.
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Abstract
Patients with cirrhosis develop metabolic derangements of protein, carbohydrate, and lipid metabolism. Malnutrition is commonplace and is associated with morbidity and mortality. Specific nutrient deficiencies may occur and enteral or parenteral nutritional support may improve outcome in appropriately selected patients. Parenteral nutrition itself has been associated with hepatic dysfunction, although the preponderance of evidence suggests that hepatic dysfunction is more a function of the underlying disorder and malabsorption. Intravenously infused organic nutrients may be metabolized differently than the same nutrient consumed enterally. The pathophysiology of total parenteral nutrition-associated liver disease is discussed as well as potential management options.
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Affiliation(s)
- A L Buchman
- Division of Gastroenterology, Intestinal Rehabilitation Center, Feinberg School of Medicine, Northwestern University, 676 N. St. Clair Street, Chicago, Illinois, USA.
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Mager DR, Wykes LJ, Roberts EA, Ball RO, Pencharz PB. Mild-to-moderate chronic cholestatic liver disease increases leucine oxidation in children. J Nutr 2006; 136:965-70. [PMID: 16549458 DOI: 10.1093/jn/136.4.965] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Malnutrition is prevalent in children with chronic cholestatic liver disease. Using the noninvasive indicator amino acid oxidation (IAAO) technique, we recently determined that mild-to-moderate chronic cholestatic (MCC) liver disease increases the need for branched-chain amino acids (BCAA) in children. To examine the underlying mechanisms responsible for this increased need for BCAA in liver disease, we measured L-[1-(13C)]-leucine oxidation in the postabsorptive and fed states in 10 children with MCC liver disease (8.8 +/- 3.5 y) and in 11 healthy children (9.4 +/- 2.2 y). The oxidation of L-[1-(13C)]-leucine to 13CO2 [F13CO2 in micromol/(kg.h)] was determined after a primed, continuous oral administration of the tracer. Total BCAA in diet was provided at 300 mg/(kg.d) to ensure that leucine oxidation was measured when leucine intake was in excess of requirements. In the postabsorptive state, the rate of release of 13CO2 from 13C-leucine oxidation (F13CO2) and whole-body leucine oxidation were significantly higher in children with MCC liver disease (P < 0.05). However, F13CO2 and whole-body leucine oxidation did not differ in the fed state. We conclude that the increased need for dietary BCAA in MCC liver disease is mediated in part by increased leucine oxidation in the postabsorptive state.
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Affiliation(s)
- Diana R Mager
- Department of Nutritional Sciences, University of Toronto, Toronto, Canada
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Mager DR, Wykes LJ, Roberts EA, Ball RO, Pencharz PB. Branched-chain amino acid needs in children with mild-to-moderate chronic cholestatic liver disease. J Nutr 2006; 136:133-9. [PMID: 16365072 DOI: 10.1093/jn/136.1.133] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Protein-energy malnutrition is prevalent in children with chronic cholestatic liver disease. Supplementation of branched-chain amino acids (BCAA) in infants and children with chronic liver disease has been associated with significant improvement in growth and nitrogen balance, suggesting that BCAA requirements are increased in chronic liver disease. The goal of the present study was to determine the total BCAA requirement in children with mild-to-moderate chronic cholestatic (MCC) liver disease using indicator amino acid oxidation (IAAO). Total BCAA requirements were determined in 6 children (6.3 +/- 3.7 y, mean +/- SD) with MCC liver disease. Children were randomly assigned to receive 7 graded intakes of total BCAA. Individual BCAA in the test diet were provided in the same proportions as those present in egg protein to minimize the potential interactive effects of individual BCAA on assessment of requirement. The total BCAA requirement was determined by measuring the oxidation of l-[1-13C] Phe to 13CO2 [F13CO2 in micromol/(kg x h)], after a primed, continuous oral administration of the tracer and using a 2-phase linear regression crossover regression analysis. The estimated mean requirement and the upper limit of the 95% CI for total BCAA establishing using the IAAO in children with MCC liver disease were 209 and 272 mg/(kg x d), respectively. Total BCAA estimated average requirements using the IAAO were significantly higher than mean requirements established previously for healthy children (P < 0.05).
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Affiliation(s)
- Diana R Mager
- Department of Nutritional Sciences, University of Toronto, Toronto, Canada
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Utterson EC, Shepherd RW, Sokol RJ, Bucuvalas J, Magee JC, McDiarmid SV, Anand R. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr 2005; 147:180-5. [PMID: 16126046 DOI: 10.1016/j.jpeds.2005.04.073] [Citation(s) in RCA: 161] [Impact Index Per Article: 8.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2004] [Revised: 02/09/2005] [Accepted: 04/19/2005] [Indexed: 12/19/2022]
Abstract
OBJECTIVES To test the hypothesis that risk analysis from the time of listing for liver transplantation (LT) focuses attention on areas where outcomes can be improved. STUDY DESIGN Competing outcomes and multivariate models were used to determine significant risk factors for pretransplantation and posttransplantation mortality and graft failure in patients with biliary atresia (BA) listed for LT and enrolled in the Studies of Pediatric Liver Transplantation (SPLIT) registry. RESULTS Of 755 patients, most were infants (age < 1 year). Significant waiting list mortality risk factors included infancy and pediatric end-stage liver disease (PELD) score > or = 20, whose components were also continuous risk factors. Survival posttransplantation (n=567) was 88% at 3 years. Most deaths were from infection (37%). Posttransplantation mortality risk factors included infant recipients, height/weight < -2 standard deviations (SD), use of cyclosporine versus tacrolimus and retransplantation. Graft failure risks included height/weight < -2 SD, cadaveric partial donors, donor age < or = 5 months, use of cyclosporine versus tacrolimus, and rejection. CONCLUSIONS Referral for LT should be anticipatory for infants with BA with failed portoenterostomies. Failing nutrition should prompt aggressive support. Post-LT risk factors are mainly nonsurgical, including nutrition, the relative risk of infection over rejection, and the choice of immunosuppression.
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Affiliation(s)
- Elizabeth C Utterson
- Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri 63110, USA
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