Parenteral nutrition is the technique of providing the body’s nutritional requirements intravenously. The nutrients are water, glucose, electrolytes, amino acids, lipids, vitamins, minerals and trace metals. Home parenteral nutrition (HPN) is the method of administering this support at home. It is a technique which was developed just over two decades ago. Some infants and neonates who started on this technique in the mid 1970’s are now 18-20 years old. HPN is administered by parents or other caretakers of the child at home through an indwelling semi-permanent central venous catheter (CVC). HPN is supervised and monitored by a multi-disciplinary team consisting of physicians, nurses, pharmacists, dietitians (nutritionist) and social workers. This technique allows patients requiring long-term parenteral nutrition support to be cared for at home, thereby decreasing hospital stay, reducing the cost of care, promoting a near normal life style and avoiding complications associated with prolonged hospitalization.

Trace elements are considered essential if their deficiency consistently results in impairment of function. Trace element deficiency may produce both specific as well as nonspecific effects. Trace element deficiency may produce both specific as well as nonspecific effects. Conversely, an excess of a trace metal, which arises either through a failure of intrinsic control mechanisms or through excessive exposure, may produce toxicity, which is either immediately apparent or latent and, ultimately of potential significance. Iron is known as an important component of porphyrin-based compounds which are bound in protein such as hemoglobin and myoglobin. In smaller amounts within the body tissue, iron is associated with enzymes and mitochondria which have significant metabolic functions. Zinc is a necessary element needed for the function of more than 70 enzymes from different species. The greatest concentrations of copper are in the liver and brain, with smaller amounts in the heart, kidney, spleen, and skeleton.

Background. Advances in intestinal transplantation (ITx) have resulted in improved survival and the opportunity to examine nutritional outcomes. The aim of this study was to describe detailed, long-term nutritional results and identify positive predictors of growth and weight gain following pediatric ITx. Methods. A single-center retrospective, Institutional Review Board-approved review of a prospective database was conducted. Inclusion criteria were ITx recipients 18 years or younger with survival of 6 months or more. Outcomes included anthropometric measurements and biochemical markers at 6, 12, 24, 36, and 48 months post-ITx. More than 25 ITx-related variables were analyzed as potential predictors of growth and weight gain. Statistical analysis was performed using chi-square test, t test, and analysis of variance. Results. Between November 1991 and April 2007, 50 children received 55 ITx; 33 patients met eligibility criteria. Median age at ITx was 2.2 years, follow-up time was 3.8 years, and time from ITx to cessation of total parenteral nutrition was 31 days. The most common micronutrient deficiencies post-ITx were zinc, iron, and copper. Serum protein levels improved significantly over time. Weight gain occurred within 6 months and vertical growth within 12 months, although limited catch-up growth was seen. Early predictors of weight gain and growth included shorter hospitalization and absence of rejection. Long-term predictors were low steroid dosage, infrequent hospitalization, and the use of peptide-based formulas. Conclusions. This represents one of the largest and most comprehensive long-term studies on nutritional outcomes in pediatric ITx. Overall, positive growth and weight gain were seen as were micronutrient deficiencies. Numerous long-term nutritional challenges exist which require a multidisciplinary approach and future prospective studies.

Postinfectious gastroparesis (PIGP) is a subgroup of idiopathic gastroparesis rarely reported in adolescents. This study describes 3 adolescent females with severe PIGP, who each underwent extensive workup prior to referral to a pediatric gastroenterologist. PIGP may be an underrecognized disorder in pediatrics, particularly in adolescents, and if untreated, can lead to significant morbidity.

As outcomes after ITx improve, greater emphasis is needed on HRQOL. The primary aims of this study were to (i) assess the feasibility of measuring HRQOL in pediatric ITx recipients, (ii) measure HRQOL using validated instruments, and (iii) compare HRQOL in ITx recipients to healthy normal (NL) children. The CHQ and Pediatric Quality of Life (PedsQL4.0) instruments were administered to both patients and parents at outpatient visits. All 24 eligible patients were enrolled. The median age at study enrollment was 6.0 yr (range: 2-18 yr), and the median time from transplant to study enrollment was 2.8 yr (range: 0.5-11.8 yr). The majority of subjects were male (58%), Latino (58%), and liver-inclusive (92%) recipients. For CHQ and PedsQL4.0, parental responses were significantly lower in multiple categories including physical health and social functioning compared to healthy norms. Patient responses were not different from NL using CHQ but using PedsQL4.0 were significantly lower in the school functioning subcategory and psychosocial health summary score. HRQOL as reported by children and families after ITx is significantly lower in multiple categories compared to NL.

A 13-month-old girl with vomiting and cough is admitted through the emergency department for failure to thrive.

Background. Infants (<12 months) who require liver transplantation (LTx) represent a particularly challenging and understudied group of patients. Methods. This retrospective study aimed to describe a large single-center experience of infants who received isolated LTx, illustrate important differences in infants versus older children, and identify pretransplant factors which influence survival. More than 25 pre-LTx demographic, laboratory, and operative variables were analyzed using the Log-rank test and Cox proportional hazards model. Results. Between 1984 and 2006, 216 LTx were performed in 186 infants with a mean follow-up time of 62 months. Median age at LTx was 9 months, the majority had cholestatic liver disease, were hospitalized pre-LTx, and received whole grafts. Leading indications for re-LTx (n=30) included vascular complications (43%) and graft nonfunction (40%), whereas leading causes of death were sepsis and multiorgan failure. One-, 5-, and 10-year graft and patient survivals were 75%/72%/68% and 79%/77%/75%, respectively. Relative to older pediatric recipients, infants had worse overall patient survival (P=0.05). The following were significant univariate predictors of graft loss: age less than 6 months and reduced cadaveric grafts; and of patient loss: age less than 6 months, calculated CrCl less than 90, pre-LTx hospitalization, pre-LTx mechanical ventilation, repeat LTx, infants transplanted for reasons other than cholestatic liver disease, and patients transplanted between 1984 and 1994. Conclusions. Long-term outcomes for infants undergoing LTx are excellent and have improved over time. As the largest, single-center analysis of LTx in infants, this study elucidates a unique set of predictors that can aid in medical decision making.

OBJECTIVES:To determine the efficacy of amitriptyline (AMI) in treating irritable bowel syndrome (IBS) in adolescents.STUDY DESIGN:Adolescents 12 to 18 years with newly diagnosed IBS were surveyed with a symptom checklist, pain rating scale, visual analog scale, and IBS quality of life (QOL) questionnaire. Subjects were randomized in a double-blinded fashion to receive AMI or placebo, and again completed surveys at 2, 6, 10, and 13 weeks.RESULTS:Thirty-three patients (24 female) were enrolled. Patients receiving AMI were more likely to experience improvement from baseline in overall QOL at 6, 10, and 13 weeks (P = .019, .004, and .013). Patients receiving AMI were also more likely to experience a reduction in IBS-associated diarrhea at 6 and 10 weeks (P = .029 for both), a reduction in periumbilical pain at 10 weeks (P = .018), and a reduction in right lower quadrant pain at 6, 10, and 13 weeks (P = .014, .039, and .004).CONCLUSION:AMI significantly improves overall QOL in adolescents with IBS and should be a therapeutic option for adolescents with this disorder.

The 2nd edition provides "best practice" guidelines written in collaboration with key home health care professionals. Comprehensive and easy-to-use, the new second edition provides a practical approach to establishing safe, medically necessary, family-centered home care for pediatric patients.

The smallest and youngest pediatric patients with the highest ASA level are at the greatest risk for complications. What is a complication? Webster's dictionary defines a complication as “a difficult factor or issue often appearing unexpectedly and changing existing plans, methods, or attitudes.” In this issue of GIE, Thakkar et al 1 Thakkar K. El-Serag H.B. Mattek N. et al. Complications of pediatric EGD: a 4-year experience in PEDS-CORI. Gastrointest Endosc. 2007; 65: 213-221 Abstract Full Text Full Text PDF PubMed Scopus (143) Google Scholar analyzed 10,236 upper GI endoscopy procedures performed in 9234 patients from 13 pediatric facilities utilizing the Pediatric Endoscopy Database System Clinical Outcomes Research Initiative (PEDS-CORI). They identified complications and analyzed their occurrence with respect to procedure indications, American Society of Anesthesiologists (ASA) class, sex, age, anesthesia type, and unplanned interventions. Because of the lack of specific details and explicit criteria for reported complications, it is difficult to know whether what were reported to be complications actually were significant clinical problems. The smallest and youngest pediatric patients with the highest ASA level are at the greatest risk for complications.

BACKGROUND:Pediatric liver transplantation (PLTx) is the standard of care for treatment of liver failure in children. Unfortunately, there are few studies with substantial numbers of patients that identify outcomes predictors. The goal of this study was to determine factors that influence outcomes in a large, single-center cohort of PLTx. STUDY DESIGN:This retrospective review between 1984 to 2006 included all recipients 18 years of age and younger undergoing PLTx. Multiorgan graft recipients were excluded (n = 48). Data sources included transplantation center database and hospital medical records. Outcomes measures were overall patient and graft survival. Demographic, laboratory, and perioperative variables were analyzed. Univariate and multivariate statistical analysis was undertaken using log-rank test and Cox's proportional hazards model. A p value < 0.05 was considered significant at the multivariate level. RESULTS:Eight hundred fifty-two PLTx were performed in 657 children; 55% were girls, 45% were Hispanic, and median age was 29.5 months. Biliary atresia and acute liver failure were the most common causes of liver disease. Fifty-two percent were hospitalized before PLTx. Graft types were whole (75%) and segmental (25%). Indications for re-PLTx (n = 195) included graft nonfunction (22%), immunologic (34%), and vascular complications (35%). Overall 1-, 5-, and 10-year survival rates were 85%, 81%, and 78% (patient), and 78%, 72%, and 67% (graft). Independent significant predictors of worse patient survival were renal function, pretransplantation ventilator dependence, and causes of liver disease. Independent significant predictors of worse graft survival were renal function and warm ischemia time. CONCLUSIONS:As one of the largest, single-center analyses of PLTx, this study enables accurate statistical analysis and demonstrates excellent longterm outcomes. Independent prognosticators of graft survival were renal function and warm ischemia time, and those for patient survival were renal function, mechanical ventilation, and causes of liver disease. These factors can aid in the medical decision making required for optimal use of scarce donor organs.

PURPOSE:To evaluate tacrolimus in 3 situations: for the induction of remission in children with severe steroid-resistant ulcerative colitis (UC); for steroid sparing in children with steroid-dependent UC in whom treatment with other immunosuppressants fails; and for the maintenance of remission in children with steroid-dependent and steroid-resistant UC.PATIENTS AND METHODS:We retrospectively evaluated 18 consecutive patients (13 with pancolitis) who were treated with oral tacrolimus at our institution from May 1999 to October 2005. Nine patients had steroid-resistant UC and 9 patients were steroid-dependent. We started patients initially on tacrolimus 0.2 mg/kg divided twice daily, with a goal plasma trough level of 10 to 15 ng/mL for the first 2 weeks, and then titrated doses to achieve plasma levels between 7 and 12 ng/mL after induction.RESULTS:Of the 18 patients in this study, 17 showed a positive response to tacrolimus therapy (ie, cessation of diarrhea and other symptoms) and 5 showed a prolonged response to tacrolimus. The mean time from initiation of tacrolimus therapy until response was 8.5 days. The mean duration of response was 260 days. Eleven of 18 patients required colectomy, including all of the patients with steroid-resistant UC, but only 2 of 9 who were steroid-dependent. The mean time from initiation of tacrolimus until colectomy was 392 days.CONCLUSIONS:It is possible that tacrolimus may benefit selected patients with steroid-dependent UC, including those who are intolerant of 6-mercaptopurine or azathioprine. Conversely, patients with steroid-resistant UC are unlikely to sustain a prolonged clinical response to tacrolimus and seem to require colectomy eventually. Careful considerations of risk versus benefit, as well as close monitoring for adverse effects, are essential in all patients.