Laboratory Studies

Albumin, prealbumin, and retinol-binding protein levels are classic nutritional markers. However, assessment of malnutrition in patients with liver cirrhosis before transplantation cannot rely completely on these tests because those proteins are produced in the liver. There are also many factors that affect albumin levels in children with chronic kidney disease (CKD); thus, it is important to evaluate each child to assess the degree to which the serum albumin reflects nutritional status.

Consider the following:

Obtain levels of fat-soluble vitamins (eg, vitamins A, D, and E) and eventually correct deficiencies. Patients on maintenance dialysis may have hypervitaminosis A due to loss of clearance of metabolites that normally occurs in a functional kidney.
Obtain prothrombin time (PT) and activated partial thromboplastin time (aPTT).
Obtain cholesterol and triglyceride levels.
Important mineral elements that can be deficient in these patients include zinc, calcium, and iron.
Total lymphocyte count is also a nutritional marker.

In patients with CKD, assess for anemia caused by iron, folate, and erythropoietin deficiency. Patients may be deficient in water-soluble vitamins and minerals; therefore, supplementation should be considered if dietary intake does not meet or exceed dietary reference intakes for children/adolescents, if blood levels are suboptimal, or if the patient shows clinical evidence of deficiency.^[31]Monitor calcium, phosphorus, alkaline phosphatase, and intact parathyroid hormone for secondary hyperparathyroidism or renal osteodystrophy. These patients may need vitamin D replacement in the pretransplantation and posttransplantation periods.

Lipid profile may be affected by immunosuppressants such as corticosteroids, cyclosporine, or mammalian target of rapamycin (mTOR) inhibitors.

Imaging Studies

Radiography to assess for renal osteodystrophy is considered inadequate for assessing pediatric patients; however, it may be used to assess skeletal maturation and vascular calcification due to high calcium-phosphate product (CaXP).^[31]

Although it is important to obtain bone densitometry (dual-energy x-ray absorptiometry [DEXA] scanning) before transplantation to assess the presence of metabolic bone disease in adults, DEXA is of limited usefulness in children. This is due to lack of adequate pediatric reference data and difficult interpretation in patients with impaired growth, altered body composition, or delayed maturation. Therefore, DEXA should not be used to monitor bone mineral density in pediatric patients with CKD.^[31]

Computed tomography (CT) scanning is also not useful to assess renal osteodystrophy in pediatric patients.^[31]

In patients with end-stage renal disease (ESRD) and uncontrollable secondary hyperparathyroidism or renal osteodystrophy, perform a parathyroid scan. If the scan shows parathyroid gland hyperplasia, the patient may need parathyroidectomy before transplantation.

Other Tests

Body composition measurements have been used to assess nutritional status in pediatric patients before and after liver transplantation. These include total body potassium measurement, neutron activation, total body electrical conductivity, and dual-energy x-ray absorptiometry (DEXA) scanning. Unfortunately, only a few centers have these methodologies available. Delayed skin hypersensitivity has been used to assess nutritional status. However, the test is not very accurate in patients with liver transplants.

The criterion standard for assessing bone disease in pediatric patients with CKD is bone biopsy (quantitative bone histomorphometry with double-tetracycline labeling), although it is rarely performed in clinical practice.^[31]

Treatment & Management

Burdelski M, Nolkemper D, Ganschow R, et al. Liver transplantation in children: long-term outcome and quality of life. Eur J Pediatr. 1999 Dec. 158 Suppl 2:S34-42. [QxMD MEDLINE Link].
Melter M, Briscoe DM. Challenges after pediatric transplantation. Semin Nephrol. 2000 Mar. 20(2):199-208. [QxMD MEDLINE Link].
Bartosh SM, Thomas SE, Sutton MM, et al. Linear growth after pediatric liver transplantation. J Pediatr. 1999 Nov. 135(5):624-31. [QxMD MEDLINE Link].
van Mourik ID, Beath SV, Brook GA, et al. Long-term nutritional and neurodevelopmental outcome of liver transplantation in infants aged less than 12 months. J Pediatr Gastroenterol Nutr. 2000 Mar. 30(3):269-75. [QxMD MEDLINE Link].
Sarna S, Laine J, Sipila I, et al. Differences in linear growth and cortisol production between liver and renal transplant recipients on similar immunosuppression. Transplantation. 1995 Oct 15. 60(7):656-61. [QxMD MEDLINE Link].
Pasqualini T, Ferraris JR, Jasper H, et al. Differences in anthropometric parameters and the IFG-I-IGFBP3 axis between liver and renal transplant children. Transplantation. 2000 Aug 15. 70(3):472-6. [QxMD MEDLINE Link].
Infante D, Tormo R, Castro de Kolster C, et al. Changes in growth, growth hormone, and insulin-like growth factor-I (IGF-I) after orthotopic liver transplantation. Pediatr Surg Int. 1998 Jul. 13(5-6):323-6. [QxMD MEDLINE Link].
Fujii H, Chikamoto H, Akioka Y, Hattori M. Final adult height in kidney recipients who underwent highly successful transplantation as children: a single-center experience. Clin Exp Nephrol. 2013 Jul 18. [QxMD MEDLINE Link].
Billing H, Burmeister G, Plotnicki L, Ahlenstiel T, Fichtner A, Sander A, et al. Longitudinal growth on an everolimus- versus an MMF-based steroid-free immunosuppressive regimen in paediatric renal transplant recipients. Transpl Int. 2013 Jun 22. [QxMD MEDLINE Link].
Greer RM, Quirk P, Cleghorn GJ, Shepherd RW. Growth hormone resistance and somatomedins in children with end-stage liver disease awaiting transplantation. J Pediatr Gastroenterol Nutr. 1998 Aug. 27(2):148-54. [QxMD MEDLINE Link].
Franke D, Steffens R, Thomas L, Pavičić L, Ahlenstiel T, Pape L, et al. Kidney transplantation fails to provide adequate growth in children with chronic kidney disease born small for gestational age. Pediatr Nephrol. 2017 Mar. 32 (3):511-519. [QxMD MEDLINE Link].
Englund MS, Tyden G, Wikstad I. Growth impairment at renal transplantation--a determinant of growth and final height. Pediatr Transplant. 2003 Jun. 7(3):192-9. [QxMD MEDLINE Link].
Valanne L, Qvist E, Jalanko H, et al. Neuroradiologic findings in children with renal transplantation under 5 years of age. Pediatr Transplant. 2004 Feb. 8(1):44-51. [QxMD MEDLINE Link].
Qvist E, Pihko H, Fagerudd P, et al. Neurodevelopmental outcome in high-risk patients after renal transplantation in early childhood. Pediatr Transplant. 2002 Feb. 6(1):53-62. [QxMD MEDLINE Link].
Loeb N, Owens JS, Strom M, Farassati F, Van Roestel K, Chambers K, et al. Long-term Follow-up After Pediatric Liver Transplantation: Predictors of Growth. J Pediatr Gastroenterol Nutr. 2018 Apr. 66 (4):670-675. [QxMD MEDLINE Link].
SPLIT research group. Studies of Pediatric Liver Transplantation (SPLIT): year 2000 outcomes. Transplantation. 2001 Aug 15. 72(3):463-76. [QxMD MEDLINE Link].
Ng VL, Alonso EM, Bucuvalas JC, Cohen G, Limbers CA, Varni JW. Health status of children alive 10 years after pediatric liver transplantation performed in the US and Canada: report of the studies of pediatric liver transplantation experience. J Pediatr. 2012 May. 160(5):820-6.e3. [QxMD MEDLINE Link].
Silver HJ, Castellanos VH. Nutritional complications and management of intestinal transplant. J Am Diet Assoc. 2000 Jun. 100(6):680-4, 687-9; quiz 685-6. [QxMD MEDLINE Link].
Sudan DL, Iverson A, Weseman RA, et al. Assessment of function, growth and development, and long-term quality of life after small bowel transplantation. Transplant Proc. 2000 Sep. 32(6):1211-2. [QxMD MEDLINE Link].
Wray J, Radley-Smith R. Developmental and behavioral status of infants and young children awaiting heart or heart-lung transplantation. Pediatrics. 2004 Mar. 113(3 Pt 1):488-95. [QxMD MEDLINE Link].
Chinnock R, Baum M. Somatic growth in infant heart transplant recipients. Pediatr Transplant. 1998 Feb. 2(1):30-4. [QxMD MEDLINE Link].
Cohen A, Addonizio LJ, Softness B, et al. Growth and skeletal maturation after pediatric cardiac transplantation. Pediatr Transplant. 2004 Apr. 8(2):126-35. [QxMD MEDLINE Link].
Boucek MM, Edwards LB, Keck BM, et al. Registry for the International Society for Heart and Lung Transplantation: seventh official pediatric report--2004. J Heart Lung Transplant. 2004 Aug. 23(8):933-47. [QxMD MEDLINE Link].
Department of Health and Human Services, Health Resources and Services Administration, Healthcare Systems Bureau, Division of Transplantation. Organ Procurement and Transplantation Network (OPTN) and Scientific Registry of Transplant Recipients (SRTR). OPTN / SRTR 2011 Annual Data Report. 2012.
North American Pediatric Renal Trials and Collaborative Studies. NAPRTCS 2010 Annual Transplant Report. Available at https://web.emmes.com/study/ped/annlrept/2010_Report.pdf. Accessed: October 15, 2018.
Sarna S, Sipila I, Vihervuori E, et al. Growth delay after liver transplantation in childhood: studies of underlying mechanisms. Pediatr Res. 1995 Sep. 38(3):366-72. [QxMD MEDLINE Link].
Sarna S, Sipila I, Ronnholm K, et al. Recombinant human growth hormone improves growth in children receiving glucocorticoid treatment after liver transplantation. J Clin Endocrinol Metab. 1996 Apr. 81(4):1476-82. [QxMD MEDLINE Link].
Viner RM, Forton JT, Cole TJ, et al. Growth of long-term survivors of liver transplantation. Arch Dis Child. 1999 Mar. 80(3):235-40. [QxMD MEDLINE Link].
Quiros-Tejeira RE, Ament ME, Heyman MB, et al. Does liver transplantation affect growth pattern in Alagille syndrome?. Liver Transpl. 2000 Sep. 6(5):582-7. [QxMD MEDLINE Link].
Ee LC, Beale K, Fawcett J, Cleghorn GJ. Long-term growth and anthropometry after childhood liver transplantation. J Pediatr. 2013 Aug. 163(2):537-42. [QxMD MEDLINE Link].
National Kidney Foundation. KDOQI Clinical Practice Guidelines for Bone Metabolism and Disease in Children with Chronic Kidney Disease. Am J Kidney Dis. 2005. 46(suppl 1):S1-S122.
Sarwal MM, Ettenger RB, Dharnidharka V, et al. Complete steroid avoidance is effective and safe in children with renal transplants: a multicenter randomized trial with three-year follow-up. Am J Transplant. 2012 Oct. 12(10):2719-29. [QxMD MEDLINE Link]. [Full Text].
Grenda R, Watson A, Trompeter R, Tönshoff B, Jaray J, Fitzpatrick M. A randomized trial to assess the impact of early steroid withdrawal on growth in pediatric renal transplantation: the TWIST study. Am J Transplant. 2010 Apr. 10(4):828-36. [QxMD MEDLINE Link].
Nucci AM, Strohm S, Squires RH, Mazariegos GV, Sun Q, Sindhi R. Growth pre- and postimplementation of a steroid-free induction protocol in a large pediatric intestinal transplant population. J Pediatr Gastroenterol Nutr. 2011 May. 52(5):601-6. [QxMD MEDLINE Link].
Kidney Disease: Improving Global Outcomes (KDIGO) Transplant Work Group. KDIGO clinical practice guideline for the care of kidney transplant recipients. Am J Transplant. 2009 Nov. 9 Suppl 3:S1-155. [QxMD MEDLINE Link].
Fine RN, Stablein D. Long-term use of recombinant human growth hormone in pediatric allograft recipients: a report of the NAPRTCS Transplant Registry. Pediatr Nephrol. 2005 Mar. 20(3):404-8. [QxMD MEDLINE Link].
Rodeck B, Kardorff R, Melter M, Ehrich JH. Improvement of growth after growth hormone treatment in children who undergo liver transplantation. J Pediatr Gastroenterol Nutr. 2000 Sep. 31(3):286-90. [QxMD MEDLINE Link].
Puustinen L, Jalanko H, Holmberg C, Merenmies J. Recombinant human growth hormone treatment after liver transplantation in childhood: the 5-year outcome. Transplantation. 2005 May 15. 79(9):1241-6. [QxMD MEDLINE Link].
Martinelli J, Habes D, Majed L, Guettier C, Gonzalès E, Linglart A, et al. Long-term outcome of liver transplantation in childhood: A study of 20-year survivors. Am J Transplant. 2017 Dec 15. [QxMD MEDLINE Link].
Midgley DE, Bradlee TA, Donohoe C, et al. Health-related quality of life in long-term survivors of pediatric liver transplantation. Liver Transpl. 2000 May. 6(3):333-9. [QxMD MEDLINE Link].
Lee H, Vacanti JP. Liver transplantation and its long-term management in children. Pediatr Clin North Am. 1996 Feb. 43(1):99-124. [QxMD MEDLINE Link].
Kelly DA. Current results and evolving indications for liver transplantation in children. J Pediatr Gastroenterol Nutr. 1998 Aug. 27(2):214-21. [QxMD MEDLINE Link].
Fine RN, Martz K, Stablein D. What have 20 years of data from the North American Pediatric Renal Transplant Cooperative Study taught us about growth following renal transplantation in infants, children, and adolescents with end-stage renal disease?. Pediatr Nephrol. 2010 Apr. 25(4):739-46. [QxMD MEDLINE Link].

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Author

Meredith J Aull, BPharm, PharmD Assistant Research Professor of Pharmacology in Surgery, Division of Transplant Surgery, Director of Clinical Research and Quality, Kidney and Pancreas Transplant Program, New York-Presbyterian Hospital, Weill Cornell Medical College

Meredith J Aull, BPharm, PharmD is a member of the following medical societies: American College of Clinical Pharmacy, American Society of Transplantation, NATCO, The Organization for Transplant Professionals

Disclosure: Nothing to disclose.

Coauthor(s)

Sandip Kapur, MD, FACS G Tom Shires, MD, Faculty Scholar in Surgery, Associate Professor of Surgery (Transplantation), Department of Surgery, Division of Transplant Surgery, Weill Medical College of Cornell University; Associate Attending Surgeon, Associate Attending Physician, Department of Transplantation Medicine, New York Presbyterian Hospital-Weill Cornell Medical Center

Sandip Kapur, MD, FACS is a member of the following medical societies: American College of Surgeons, American Society of Transplant Surgeons, American Society of Transplantation, Association for Academic Surgery, Association for Surgical Education, Society of University Surgeons, Transplantation Society, Cell Transplant Society, International Pancreas and Islet Transplant Association, New York Surgical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Mary C Mancini, MD, PhD, MMM

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Additional Contributors

Richard G Ohye, MD Head, Section of Pediatric Cardiovascular Surgery, Associate Professor of Cardiac Surgery, Program Director, Pediatric Cardiac Surgery Fellowship, University of Michigan Medical Center

Richard G Ohye, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Thoracic Surgery, Congenital Heart Surgeons Society, Society of University Surgeons, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Acknowledgements

Marcela Del Rio, MD Assistant Professor, Department of Pediatric Nephrology, Department of Pediatrics, Division of Pediatric Nephrology, Albert Einstein College of Medicine; Consulting Staff, The Children's Hospital at Montefiore

Marcela Del Rio, MD is a member of the following medical societies: American Society of Nephrology and American Society of Pediatric Nephrology

Disclosure: Nothing to disclose.

John A Goss, MD, FACS Professor of Surgery, Chief, Division of Abdominal Transplantation, Baylor College of Medicine; Director of Liver Transplantation, Texas Children's Hospital; Director of Liver Transplantation, St Luke's Episcopal Hospital; Director of Liver Transplantation, Michael E DeBakey Veterans Affairs Medical Center

John A Goss, MD, FACS is a member of the following medical societies: American Association for the Study of Liver Diseases, American Medical Association, American Society of Transplant Surgeons, Association for Academic Surgery, Sigma Xi, Southwestern Surgical Congress, and Texas Medical Association

Disclosure: Nothing to disclose.

Stuart M Greenstein, MD Professor of Surgery, Albert Einstein College of Medicine; Consulting Surgeon, Department of Surgery, Division of Transplantation, Montefiore Medical Center

Stuart M Greenstein, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Society of Transplant Surgeons, American Society of Transplantation, Association for Academic Surgery, International College of Surgeons, Medical Society of New Jersey, National Kidney Foundation, New York Academy of Sciences, and Southeastern Surgical Congress

Disclosure: Nothing to disclose.

Shefali Mahesh, MD, MBBS Fellow, Department of Pediatric Nephrology, Albert Einstein College of Medicine, Montefiore Medical Center

Shefali Mahesh, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and American Society of Transplantation

Disclosure: Nothing to disclose.