Increasingly, kidney problems are recognized in children with intestinal failure/short bowel syndrome who receive long-term parenteral nutrition. A recent study (H Billing et al JPGN 2018; 66: 751-54) highlights the experience with this issue at a pediatric intestinal rehabilitation center in Germany.
- Among 50 patients with a median age of 4.2 years, 76% had proteinuria
- 30% had chronic kidney disease –indicated by reduced creatinine clearance of <90 min (1.73 squared)/min
- Hypercalciuria was identified in 30 patients (60%)
- Nephrocalcinosis was identified in 9 patients (18%)
The authors note that end-stage renal failure has not been reported in association with intestinal failure, though proteinuria is associated as a risk factor.
My take: This observational study shows a high frequency of kidney issues in children with intestinal failure. With improvements in survival, chronic kidney disease could become a more significant clinical issue.
Tweet below indicates need for careful nutrition input when children are placed on unusual diets, including the ketogenic diet.
D Wendel et al. JPGN 2018; 66: 212-7. This single center retrospective review examined patients who received home TPN prior to liver transplantation. These 18 patients, which represented 41% of their entire transplant cohort of 44 between 2010-2015, all had biliary atresia. Key findings:
- Malnutrition improved or resolved in all but one patient
- 8 catheter-related infections were noted (3.8/1000 catheter days)
- There were no deaths in patients receiving TPN
My take: While there is an increased burden of care with TPN, improved nutrition may improve long-term outcomes.
Related blog posts:
Amber Cove, Dominican Republic
I recently had the opportunity to hear a terrific lecture by David Adamkin (University of Louisville) on neonatal nutrition. Unlike previous lectures that I’ve highlighted on this blog (Neonatal Nutrition Lecture -What We Know Right Now …) which focused on enteral nutrition and breastmilk. This lecture focused on providing early parenteral nutrition to prevent postnatal growth failure.
“Father” of TPN was Stanley Dudrick (1968)
Introduction of TPN dramatically improved survival for many infants. In disorders like gastroschisis, TPN increased survival from ~10% to 90%.
Extreme premature infants have minimal energy reserves
At 24-28 weeks gestational age, fetuses are ‘bathed in amino acids’ and extreme premature infants need early amino acids. At University of Louisville, the neonatologists try to deliver ~3 gm/kg/day of amino acids in 1st 1-2 days in order to match intrauterine growth and prevent growth failure. Half of postnatal weight loss is water; other half is related to proteolysis. To facilitate TPN at all hours, they use a stock solution (4% amino acids at 60 mL/kg/day delivers 2.4 mg/kg/day of protein; 80 mL/kg/day delivers 3.2 mg/kg/day of protein.
Return to Birth Weight Time is Correlated with Growth Failure. Extreme prematurity has been correlated with slower return to birth weight
Lack of correlation between BUN and Protein Intake
BUN increases with any protein intake but not affected by protein intake -issue has to do renal fxn, comorbidity. Smaller & sicker have higher BUN.
- The more premature, then the longer it takes to return birth weight and more growth failure
- Poor growth related to neurodevelopment outcomes
- With higher protein intake, there is better glucose tolerance; protein intake helps with glucose tolerance & lowers chance of hyperkalemia
Recently one of my radiology colleagues, Dr. Laura Hayes, put together (lead author) a presentation (poster) for an upcoming meeting. The main focus of the presentation is a TPN-dependent toddler who presented with refusal to walk due to copper deficiency.
Attached is a link to the presentation: TPN Copper. This link is a power point presentation with numerous radiographs and even bone scan images.
- All TPN components except dextrose have been in periods of shortage over the last few years.
- TPN-dependent patients may not be receiving all the needed components and their physicians may not have been notified of the specific shortage(s).
- Copper deficiency leads to reduced activity of numerous enzymes important for function of bone, blood, skin, nervous system and hair.
- Subperiosteal hemorrhage leads to the periosteal thickening seen in this case and is associated with the bone pain our patient experienced.
- Increased losses of bilious fluid can increase the risk of copper deficiency due to the excretion of copper in bile.
- Other TPN-related deficiencies reviewed include thiamine deficiency (Wernicke’s encephalopathy), Vitamin D deficiency (Rickets), and Vitamin C deficiency (Scurvy).
Another recent case report:
Oestreich AE, Cole CR. Vigorous periosteal reaction secondary to copper deficiency in an infant on total parenteral nutrition. (2013) Pediatr Radiol 43:1411-1413.
Related Blog Posts:
A randomized prospective study of early parenteral nutrition cycling was not helpful in reducing parenteral nutrition associated cholestasis (PNAC): J Pediatr 2012; 161: 229-33.
This study enrolled preterm infants <1250 g in the first five postnatal days to either cycled (n=34) or continuous parenteral nutrition (n=36). Cholestasis was defined as direct bilirubin >2 mg/dL. The study indicated that intralipid 20% was administered in all patients over 18 hours; the exact amount is not clear, though in the cycled group, patients received 3 g/kg/day on postnatal day 3.9 ± 2.6. All patients received dextrose over 24 hours; only the amino acid (TrophAmine) infusion varied: 20 hours compared with 24 hours.
- PNAC in each group was nearly identical, 32% vs 31% as were bilirubin and transaminase values.
- While the study was randomized, the cycled group did have more frequent late-onset sepsis 22 (65%) compared with 14 (39%) which reached statistical significance.
- In addition, a trend towards more frequent non-surgical NEC in cyclical group was noted as well: 6 (18%) compared with 3 (8%).
- PNAC was associated with days until full enteral nutrition/duration of parenteral nutrition and bronchopulmonary dysplasia.
The higher incidence of late-onset sepsis could have occurred randomly and affected the results; alternatively, the higher rate could reflect an increased risk of sepsis due to doubling the frequency of central line access.
This study was only powered to detect a 25% difference between the groups; the authors estimate that a study with 290 patients would be needed in each group to identify a reduction of 10% in the incidence of PNAC. As such, this study leaves open the possibility that cycled parenteral nutrition may be helpful in a smaller percentage, particularly if efforts are made to eliminate central line infections. More promising efforts to minimized PNAC are noted in previous blog entries:
PNAC, PNALD, and IFAC
Optimizing lipids to minimize cholestasis
More on PNAC
Four advances for intestinal failure