Judith Pichler

Growth and bone health in pediatric intestinal failure patients receiving long-term parenteral nutrition

BACKGROUND Children with chronic intestinal failure (IF) treated with long-term parenteral nutrition (PN) may present with low bone mineral density (BMD). The cause may reflect small body size or suboptimal bone mineralization. OBJECTIVE We assessed growth and bone health in children with severe IF. DESIGN Height, weight, and fracture history were recorded. The lumbar spine bone mass was measured in 45 consecutive patients (24 male subjects) aged 5-17 y receiving PN for a median of 5 y. BMD and bone mineral apparent density (BMAD) [ie, adjusted-for-height SD scores (SDSs)] were calculated. RESULTS Diagnoses were short bowel syndrome in 12 patients (27%), intestinal enteropathy in 20 patients (44%), and motility disorder in 13 patients (29%). Mean (±SD) weight, height, and body mass index SDSs were -0.8 ± 1.3, -1.80 ± 1.5, and 0.4 ± 1.3, respectively. The height SDS was less than -2 in 23 children (50%). Patients with enteropathy or intestinal mucosal inflammation (associated with dysmotility or short bowel) were significantly shorter than patients without enteropathy (P = 0.007). The BMD SDS was -1.7 ± 1.6, and the BMAD SDS was -1.4 ± 1.5, independent of primary diagnosis or mucosal inflammation. Nineteen patients (42%) had low BMD (SDS less than -2.0), and 14 patients (31%) had low BMAD. In 25 patients studied at 1-2-y intervals, the BMD SDS fell significantly with time, whereas BMAD declined less, which suggested that a poor bone mineral accretion reflected poor growth. A total of 11 of 37 patients (24%) had nonpathologic fractures (P = 0.3 compared with the general population). CONCLUSIONS Approximately 50% of children were short, and one-third of children had low BMD and BMAD. Children with enteropathy or intestinal mucosal inflammation are at greatest risk of growth failure. Close nutritional monitoring and bespoke PN should maximize the potential for growth and bone mass.

Intestinal failure-associated liver disease in hospitalised children

Objective and aim Liver disease is a potentially life-threatening complication of intravenous/parenteral nutrition (PN). Our aim was to determine the incidence, aetiology and outcome of intestinal failure-associated liver disease (IFALD) in hospitalised children treated with long-term PN (>27 days). Methods Over 4 years all long-term intestinal failure (IF) patients were reviewed for the possible predisposing factors of age, diagnosis, PN lipid, sepsis, length of PN treatment and length of hospitalisation. Outcome measures were IFALD incidence, severity and prognosis. Results Of 60/279 (22%) children aged 0–18 years who developed IFALD, 13 (5%) progressed to type 3/end stage disease. IFALD was associated with younger age (p=0.03), longer treatment (p<0.001), longer hospitalisation (p=0.01), surgical diagnosis (p=0.005) and prematurity (p=0.03). IFALD was not associated with sepsis. Intestinal surgery was associated with IFALD independently of age (p=0.03). Survival was 86%, with three deaths attributed to IFALD (1% of all cases), all of which were surgical. Conclusion IFALD incidence was lower than previously reported in paediatric patients, with surgical neonates at greatest risk.

Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition

PURPOSE Our aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN). METHODS We retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)--persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)--additional elevated total bilirubin (≥ 50 μmol/L); and type 3 (late)--additional clinical signs of end-stage liver disease. RESULTS Eighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex. CONCLUSION Intestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex.

Sepsis and its etiology among hospitalized children less than 1 year of age with intestinal failure on parenteral nutrition.

Infections accompany intestinal failure (IF) more commonly in children than in adults, with reported incidences of 2% to 29%. Appropriate care of the central venous catheter is the most important factor preventing infections; but in addition, bacteria translocate from the dysmotile gut as a possible source of septicemia. The aim of this retrospective analysis was to investigate the rate and the epidemiologic profile of septicemia in the patient group at greatest risk, namely, children less than 1 year of age with IF on parenteral nutrition (PN). Among 63 children less than 1 year of age who were included over a 2-year period, 55% were boys. The overall median age at the start of PN was 0.3 years, with a mean duration of 80 days. Some 68% of patients had at least one episode of septicemia, experiencing a mean of 1.5 episodes (range, 1-12). Also, 19% of children displayed polymicrobial bloodstream infections. The most common Gram-positive pathogens were Staphylococcus spp and Enterococcus spp; the Gram-negative pathogens were Klebsiella spp followed by Enterobacter spp and E. coli. Infants less than 1 year of age with IF >28 days experienced a high (68%) rate of sepsis. There was no difference in the incidence of catheter-related blood stream infection according to the primary underlying diagnosis. The most common pathogens were Staphylococcus spp and Enterococcus spp, similar to etiologies of sepsis among children in intensive care units.

Body composition in paediatric intestinal failure patients receiving long-term parenteral nutrition

Objective Outcome of children with intestinal failure (IF) has improved on treatment with parenteral nutrition (PN). The effects of PN and IF on body composition (BC) are unknown. The aim was to review BC in PN-treated children and those weaned off and to compare with reference data. Design Children on long-term/home PN underwent measurement of regional fat mass (FM) and lean mass (LM) using dual energy X-ray absorptiometry. Underlying diseases were intestinal enteropathy, n=15, short bowel syndrome (SBS), n=8 and intestinal dysmotility, n=11. PN duration was median 10 years. Fat Mass Index (FMI) and Lean Mass Index (LMI) were compared in children with and without intestinal inflammation, steroid treatment and according to PN dependency. Results 34 children aged 5–20 years were studied. They were short, mean height SD score (SDS) −1.8 (p<0.001) and light (mean weight SDS −0.86, p<0.001) with high body mass index (BMI) SDS: mean 0.4 (p=0.04) and low Limb LMI SDS −0.9 (p<0.001). Children with SBS had low FMI SDS −0.8 (p=0.01). BC did not significantly differ between diagnostic groups or with steroid treatment. Patients with intestinal inflammation (n=20) had higher BMI SDS than those without, p=0.007. Totally, PN-dependent children, n=11 had higher BMI SDS, p=0.004, total body FMI SDS, p=0.008 and trunk FMI SDS, p=0.001 compared with patients partially dependent and off PN. Conclusions Significantly low limb LM was seen in all patient groups with high FM in children on total PN. Children with IF requiring PN treatment >27 days may benefit from BC monitoring and PN adjustment according to results in order to maximise linear growth and health in later life.

Comparison of liver function with two new/mixed intravenous lipid emulsions in children with intestinal failure.

Background/objective:The high incidence of liver disease associated with intravenous soybean lipid has led to development and use of alternative intravenous lipid emulsions (ILEs). The aim of this study was to compare two new/mixed ILEs: a medium-chain triglyceride (MCT) combined with soybean (i.e., Lipofundin) and a combination of both these lipids with additional olive and fish oils (SMOF).Subjects/methods:Neonates/premature infants newly starting parenteral nutrition (PN) treatment and children with abnormal liver function tests, alanine transferase (ALT), alkaline phosphatase (ALP), γ-glutamyl transferase (γ-GT) 1.5x upper limit of normal and/or total bilirubin >50 μmol/l for >2 weeks on treatment with PN containing pure soybean ILE (Intralipid 20%; Fresenius Kabi), were started on/changed to either SMOF or Lipofundin. Results of biochemistry and clinical outcome were compared on commencing and discontinuing treatment according to the new ILE used.Results:One hundred and twenty-seven children aged 0–16 (median 0.6) years were included. Fifity-six were given Lipofundin and 71 SMOF. Fifty-three of 127 started PN for the first time and 74 had had previous treatment with Intralipid. During treatment, ALT and ALP levels fell significantly (P<0.008 on SMOF; P<0.05 on Lipofundin), with additional significant reduction in γ-GT with SMOF. Hyperbilirubinaemia incidence decreased from 34% on starting to 24% on discontinuing treatment (P⩽0.05). Infection rate/1000 catheter days, full blood count, serum triglyceride and cholesterol levels were similar with both ILEs.Conclusion:Addition of MCT to soybean ILE was associated with improved liver function. There was an even greater improvement when olive and fish oils were also added with higher incidence of resolution of abnormal liver function tests and reduced inflammation.

Prevalence of undernutrition during hospitalisation in a children’s hospital: what happens during admission?

Background/Objective:The aim of the study was to compare the prevalence of undernutrition in children on presentation to hospital and on discharge.Methods:On a screening week, 141 children aged from birth to 17 years who were hospitalised for ⩾72 h were reviewed on presentation and discharge or after 3 months (if still in hospital) by auditing hospital records. Weight for age standard deviation (s.d.<−2) was used to define undernutrition on admission and discharge. The number of children referred for dietetic advice was recorded.Results:The prevalence of undernutrition on admission was 27% (14% moderate (s.d.: −2 to −3) and 13% severe (s.d.: ⩾−3)) according to weight s.d. and increased to 32% by discharge (11% moderate; 21% severe). The most nutritionally vulnerable children, with a prevalence of undernutrition from 33 to 53% on admission, were aged less than 2 years, inpatients for >1 month and those with multiple medical problems. In all, 74% (n=104) of cases were referred to Dietetics, including 73% (n=79) of those without evidence of undernutrition.Conclusions:Undernutrition is a major problem in children during hospitalisation. The risk of nutritional depletion needs to be identified at the time of admission, especially for children under 2 years and those with multiple medical problems, in order to initiate appropriate nutritional intervention.

Prevalence of Gallstones Compared in Children With Different Intravenous Lipids.

Objective: The aetiology of biliary liver disease in children with intestinal failure treated with long-term parenteral nutrition (PN) is multifactorial. Risks include the lipid component of PN. The aim of the study was to compare prevalence and outcome of gallstones with different types of intravenous lipids. Methods: Liver and biliary tract imaging and relevant clinical details were reviewed in 71 patients (37, 52% boys) treated with PN for >3 months. Types of lipid infused were compared with regard to hepatobiliary abnormalities. Results: In total 369 abdominal ultrasounds were performed in 71 patients of age between 3 months and 17 years. Underlying diagnoses were short bowel syndrome in 20 (28%), small intestinal enteropathy in 34 (48%), and motility disorder in 17 (24%). A total of 67 (94%) children had 362/369 scans on lipid-containing PN. Of the total, 15 (21%) patients had gallstones, 8 (11%) had sludge, and both were detected in 7 (10%) children. The gallstones/sludge resolved in 7 patients (10%) and persisted in 10 (13%). In 6 patients, sludge progressed to form discrete gallstones, and in 9 children, gallstones led to biliary duct dilatation. Four (6%) patients underwent cholecystectomy. Fewer children had abnormalities with the newer mixed lipid emulsion (P = 0.005). There was a higher prevalence of sludge (P = 0.01) on pure soya lipid. Predictors for sludge were young age at PN (P = 0.001), lack of enteral feed (P < 0.001), and motility disorder with stoma (P = 0.002). Conclusions: Hepatobiliary pathology is common in children on PN. The use of mixed lipid was associated with less biliary complications and should be the first choice of treatment in children.

Review of the diagnoses predisposing infants to intestinal failure on hospitalized parenteral nutrition.

During the last 3 decades the use of parenteral nutrition (PN) and the aggressive introduction of enteral feeding in daily practice have transformed the outcome for even the sickest of these infants. More than 90% of infants and children now survive after extensive small bowel resection in the neonatal period. During the last 3 decades the use of parenteral nutrition (PN) and the aggressive introduction of enteral feeding in daily practice have transformed the outcome for even the sickest of these children. The aim of this study was to review the diagnoses (other than infants purely premature) that predispose infants to intestinal failure (IF) and dependency on PN as well as their outcomes. A total of 63 children less than 1 year old received PN for more than 28 days including 35 (56%) boys; 29% of cases were preterm infants with a median gestational age of 26.5 weeks (range, 24-33 weeks). The median age at the start of PN was 0.25 years or 3 months. Median duration of PN treatment was 62 days and median duration of hospitalization was 128 days. Twenty-three (36.5%) children had a primary nondigestive disorder (PNDD) and 40 (63.5%), a primary digestive disorder (PDD). Forty (63.5%) children with severe intestinal failure were successfully weaned off PN; whereas 8 (13%) infants with severe gastrointestinal diseases remained dependent on IV nutrition. Fourteen (22%) patients died. Infants less than 1 year of age with severe intestinal failure have up to a 75% survival rate, with a 65% chance of achieving intestinal autonomy. For children presenting with PDD in infancy, there is a high risk of needing long-term PN.