Two single group, prospective, baseline-controlled feeding studies in infants and children with chronic diarrhea fed a hypoallergenic free amino acid-based formula.

BACKGROUND: Infants and children with chronic diarrhea (CD) often require specialized foods or parenteral nutrition (PN) to achieve adequate nutrient intakes to support growth and development. We assessed the efficacy of an amino acid-based formula (AAF) in supporting growth and improving symptoms in infants and children with CD from multiple etiologies. METHODS: Two studies were conducted: CD study in children (CD-C) and CD study in infants (CD-I). Each was a single group, baseline-controlled study in which each subject served as his/her own control. At enrollment, all subjects had CD lasting > 2 weeks and had ≥ 4 stools/day. Subjects were fed an AAF for 80 days starting at SD5, and were assessed at SD 28 and 84. RESULTS CD-C: 18 of 19 subjects completed the study. At enrollment, the mean age was 5.6 ± 0.7 years, the most common diagnosis was short bowel syndrome (SBS) (n = 13), and 5 subjects with SBS were on PN. Subjects achieved significant increases in weight-for-age z-scores (p = 0.026). Over 50% of subjects achieved improvements in clinical outcomes targeted most frequently by their physicians. Of the five subjects on PN at enrollment, four had substantial weight gain and four had their PN requirements decreased. CD-I: 22 of 27 subjects completed the study. At enrollment, the mean age was 3.3 ± 0.3 months, the most common diagnosis was food allergy (n = 20), and no subjects were on PN. Subjects achieved significant increases in weight-for-age z-scores (p = 0.0023), significant decreases in the number of stools/day (p = 0.0012), and improvements in stool consistency (p = 0.0024). Over 80% of subjects achieved improvements in the clinical outcomes targeted most frequently by their physicians. CONCLUSIONS: Infants and children with CD fed an AAF for three months displayed significant improvements in weight-for-age z-scores and clinical symptoms. Children dependent on PN also grew well and four of five decreased their dependence on PN. TRIAL REGISTRATION: Both trials were registered on ClinTrials.gov (CD-C, NCT01812629; CD-I, NCT01820494).

Evaluation of a free amino acid-based formula in infants with presumptive food protein-induced proctocolitis.

OBJECTIVE: Food protein-induced proctocolitis usually occurs early in life and is characterized by blood-streaked stools and pain during defecation in an otherwise healthy infant. While many infants with food protein-induced proctocolitis respond well to a casein hydrolysate formula, some require an amino acid-based formula. The objective of the study was to measure the change in physician-rated symptom score from enrollment to study completion in infants with presumptive food protein-induced proctocolitis fed with a specific amino acid-based formula. METHODS: In this study, infants ≤6 months of age diagnosed with presumptive food protein-induced proctocolitis received an amino acid-based formula for 42 days. Intake, stool patterns, weight, stool occult blood, and questionnaires assessing infant feeding and stool patterns and parental formula satisfaction were collected. RESULTS: The full analysis set included 43 infants. The mean age at enrollment was 59 ± 5 days. A significant improvement was observed from enrollment to exit in physician-rated symptom score (9.1 ± 0.5 to 4.8 ± 0.5, p < 0.0001), the number of infants with occult blood in stool, and weight-for-age Z-scores during the study. Parental satisfaction with the formula was high. CONCLUSION: The results confirm that the amino acid-based formula studied is efficacious for managing symptoms of presumptive food protein-induced proctocolitis.

Repeat surgical bowel lengthening with the STEP procedure.

BACKGROUND: Feasibility of repeat lengthening has been demonstrated in animals, but in humans, none of the three clinical case reports have achieved enteral autonomy after secondary lengthening with serial transverse enteroplasty (STEP). PATIENTS AND METHODS: Institutional Review Board approved retrospective review of a single center series of repeat intestinal lengthening after prior Bianchi lengthening or STEP. Outcome measures examined include patient survival, weaning from parenteral nutrition, and need for intestinal transplantation. RESULTS: Repeat lengthening with STEP was performed 16 times in 14 patients including 2 adults and 7 male patients after prior Bianchi (n=7) and prior STEP (n=7). Median time after initial lengthening to reSTEP was 12 months (2 months-15 years). Median remnant bowel length before initial lengthening was 35 cm (15-110), before reSTEP was 56.5 cm (27-100) and final length after reSTEP was 90 cm (39-120). Survival is 100% with a median follow-up of 14.5 months (1-65 months). Discontinuation of parenteral nutrition (PN) after reSTEP was achieved in 6 of 14 (43%) patients at a median of 3 months (0.5-13 months) after reSTEP. Intestinal transplant salvage was performed in four patients at 7, 9, 13 months, and 4.6 years, respectively, after reSTEP. All four of those undergoing intestinal transplantation have also subsequently discontinued PN. CONCLUSIONS: Repeat lengthening is technically feasible after either of the available surgical lengthening procedures (Bianchi and STEP). In the stable patient, who is unable to wean from PN, repeat lengthening led to discontinuation of TPN in almost half of these carefully selected patients and does not preclude intestinal transplantation.

Comparison of intestinal lengthening procedures for patients with short bowel syndrome.

OBJECTIVE: Review the clinical results of 24 years of intestinal lengthening procedures at one institution. METHODS: Retrospective review of a single center experience comparing the outcome of 2 intestinal lengthening procedures (Bianchi and serial transverse enteroplasty [STEP]) in terms of survival, total parenteral nutrition (TPN) weaning, and complications. RESULTS: Sixty-four patients, including 14 adults, underwent 43 Bianchi and 34 STEP procedures between 1982 and 2007. Three patients had prior isolated liver transplants. The median (range) remnant bowel length before first lengthening was 45 (11-150) cm overall; (Bianchi=44 cm, STEP=45 cm) and 68 (20-250) cm after lengthening; (Bianchi=68 cm, STEP=65 cm). Actual survival is 91% overall (Bianchi 88%, STEP 95%) with median follow-up of 3.8 years (Bianchi=5.9 years, STEP=1.7 years). Average enteral caloric intake in pediatric patients was 15 kcal/kg before lengthening and 85 kcal/kg at 1 year after lengthening. Sixty-nine percent of patients are off TPN at most recent follow-up, including 8 who were weaned from TPN after intestinal transplantation. Liver disease (when present) was reversed in 80%. Surgical complications occurred in 10%, more commonly requiring reoperation after Bianchi than STEP. Intestinal transplantation salvage was required in 14% at a median of 2.9 years (range=8 months to 20.7 years) after lengthening. CONCLUSIONS: Surgical lengthening with both Bianchi and STEP procedures results in improvement in enteral nutrition, reverses complications of TPN and avoids intestinal transplantation in the majority with few surgical complications. Intestinal transplantation can salvage most patients who later develop life-threatening complications or fail to wean TPN.

Neonatal and infant feeding: effect on bone density at 4 years.

OBJECTIVE: The aim of this study was to determine whether the type of feeding during the first 4 months of life affects bone mineral density at 4 years of age. METHODS: Healthy 4-year-old children were recruited from the offices of primary health care providers. After confirming the type of infant feeding by history, dual energy x-ray absorptiometry analysis was obtained at the University of Nebraska Medical Center and evaluated by a radiologist blinded as to feeding type. RESULTS: One hundred and seventy-eight children completed the study (58% male, 85% Caucasian; mean age, 4.5 years). All children had exclusively consumed human milk (n = 57), an infant formula containing no palm olein oil (n = 56) or an infant formula containing palm olein oil (n = 65) during the first 4 months of life. At 4 years of age, no significant differences were noted in bone mineral content or bone mineral density (P = 0.51 and 0.89, respectively) among the three feeding groups as measured by dual energy x-ray absorptiometry. Total body bone mineral content and bone mineral density varied by gender, with males having significantly higher values than females regardless of feeding type (P = 0.028 and P < 0.001, respectively). CONCLUSION: There is no association between the use of palm olein formula during the first 4 months of life and subsequent bone mineral content and bone mineral density in healthy 4-year-old children.