Evaluating for Human Herpesvirus 6 in the Liver Explants of Children With Liver Failure of Unknown Etiology.

BACKGROUND: Liver failure of unknown etiology (LFUE) has a transplant-free survival rate <25%. Human herpesvirus 6 (HHV-6) may be associated with LFUE, but studies are limited by small sample size. METHODS: We identified all children who underwent liver transplant for LFUE at a single quaternary children's hospital; 51/65 cases could be age matched with controls (children who underwent liver transplant for metabolic liver disease). Quantitative polymerase chain reaction for HHV-6 was performed on DNA from formalin-fixed paraffin-embedded liver explant tissue. RESULTS: HHV-6 was detected in 34/51 cases (66.7%) and 19/51 controls (37.3%) (P = .005). Average HHV-6 viral load was 213207 copies/106 cells in positive cases (range: 7293-1102030) and 38115 copies/106 cells in positive controls (range: 1382-122375) (P = .0008). HHV-6 was present significantly more often in cases compared to controls in patients younger than 6 years. In particular, in patients younger than 3 years, HHV-6 was present in 13/27 cases (48.1%) and 2/27 controls (7.4%) (P = .0009). CONCLUSIONS: HHV-6 was detected in liver explants significantly more often and in higher quantities in children transplanted for LFUE compared to controls, suggesting HHV-6 should be evaluated in young children who present with LFUE.

Nutritional Needs and Support for Children with Chronic Liver Disease.

Malnutrition has become a dangerously common problem in children with chronic liver disease, negatively impacting neurocognitive development and growth. Furthermore, many children with chronic liver disease will eventually require liver transplantation. Thus, this association between malnourishment and chronic liver disease in children becomes increasingly alarming as malnutrition is a predictor of poorer outcomes in liver transplantation and is often associated with increased morbidity and mortality. Malnutrition requires aggressive and appropriate management to correct nutritional deficiencies. A comprehensive review of the literature has found that infants with chronic liver disease (CLD) are particularly susceptible to malnutrition given their low reserves. Children with CLD would benefit from early intervention by a multi-disciplinary team, to try to achieve nutritional rehabilitation as well as to optimize outcomes for liver transplant. This review explains the multifactorial nature of malnutrition in children with chronic liver disease, defines the nutritional needs of these children, and discusses ways to optimize their nutritional.

Orthotopic liver transplant for multifocal lymphangioendotheliomatosis with thrombocytopenia.

An eight-yr-old female with a history of multifocal lymphangioendotheliomatosis and thrombocytopenia presented for MVT. The patient had multiple vascular lesions in the skin and stomach in infancy. Although her cutaneous lesions resolved with vincristine and methylprednisolone, her gastric lesions persisted. Eight yr later, she was diagnosed with portal hypertension and decompensating liver function despite therapy with bevacizumab, propranolol, furosemide, and spironolactone. Upon presentation, she was found to have a Kasabach-Merritt-like coagulopathy in association with multiple lesions in her GI tract and persistent gastric lesions. Although treatment with methylprednisolone and sirolimus normalized her coagulation factors and d-dimer levels, she never developed sustained improvement in her thrombocytopenia. Her liver function continued to deteriorate and she developed hepatorenal syndrome. Given better outcomes after OLT in comparison with MVT, she underwent OLT, with the plan to manage her GI lesions with APC post-transplant. Post-transplant, her liver function and coagulopathy normalized, and GI tract lesions disappeared upon screening with capsule endoscopy. The patient is doing well, without recurrence of either GI lesions or thrombocytopenia, at 18 months after transplantation.

Practice patterns of pediatricians and trainees for the management of functional constipation compared with 2006 NASPGHAN guidelines.

OBJECTIVES: The North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition has formulated guidelines for managing functional constipation. There have been no studies that have investigated how pediatricians apply the constipation guideline since it was revised in 2006. The purpose of this study was to examine how pediatricians approach functional constipation and how closely their approaches adhere to the guidelines. METHODS: An anonymous multiple-choice questionnaire was developed by general pediatricians and pediatric gastroenterologists. This was distributed to pediatricians and pediatric residents at 7 academic institutions, and to the American Academy of Pediatrics section on medical students, residents, and fellowship trainees mailing list. RESULTS: A total of 1202 responses were received (952 trainees, 250 attendings). Of these, 84.3% reported being unfamiliar/slightly familiar with the guidelines. The most common initial interventions for constipation without fecal incontinence included fluids (92.1%), fiber (89.5%), juice (77.7%), behavioral interventions (71.2%), follow-up (53.4%), and reducing constipating foods (50.1%). The most common initial interventions for constipation with fecal incontinence included bowel cleanout (73.4%), maintenance medication (70.0%), fluids (67.9%), behavioral interventions (67.6%), fiber (66.1%), and follow-up (57.8%). Osmotics were the most commonly prescribed as needed (83.0%) and maintenance medications (96.8%), with stimulants prescribed PRN by 35.6% and as maintenance by 16.8%. Some individuals (39.7%) reported concern that osmotics could result in dependence, addiction, or electrolyte imbalances, compared with 73.0% for stimulants. CONCLUSIONS: Our results show that more education regarding medication in functional constipation is necessary, including the use of medication reducing time to remission, the necessity of disimpaction, and misconceptions regarding adverse effects.

High long-term local control with sacrectomy for primary high-grade bone sarcoma in children.

BACKGROUND: Sacrectomy may offer curative potential for primary sarcomas of the sacrum. However, it is unclear whether and to what extent sacrectomies achieve local control. QUESTIONS/PURPOSE: We therefore determined the curative potential, walking ability, continence control, and quality of life for children after sacrectomy for primary bone sarcoma. METHODS: We retrospectively reviewed eight children with high-grade primary bone sarcomas treated with sacrectomy. The average age of the patients was 14 years (range, 4-18 years). There were six Ewing sarcomas and two osteosarcomas. Five patients underwent partial and three underwent total sacrectomies with all resection margins free of tumor. We recorded local and distant recurrences, functional (Musculoskeletal Tumor Society [MSTS] score) and oncologic outcome (survival), walking ability, bladder and bowel control, independence, and reintegration in society. The average followup was 80 months (range, 54-204 months). RESULTS: Six patients were alive without evidence of disease at last followup. Three patients had metastases develop and two died of disease 3 and 25 months postoperative. MSTS scores among survivors averaged 17.5 points (range, 5-25 points). Seven of the eight patients were able to ambulate, two independently. All initially were incontinent, but at last followup, two were continent and two were incontinent only under stress. Complications occurred in seven of eight, five of whom required additional surgery. CONCLUSION: Despite the high incidence of neurologic deficits and complications, sacrectomy achieved local control in patients with high-grade bone sarcomas. Our observations suggest long-term survival and adequate quality of life are possible in the pediatric population. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.