Energy expenditure and intake in children with sickle cell disease during acute illness.

BACKGROUND: Children with sickle cell disease have frequent bouts of pain and infection which may increase energy expenditure, decrease energy intake and lead to a subsequent energy deficit. METHODS: Two groups of African-American children with sickle cell disease-SS genotype were enrolled in this study upon hospital admission for a sickle cell disease related illness: a younger (<6 years, n=14, 7 M) and older group (> or =6 years, n=17, 8 M). Body composition and dietary intake were assessed, and sleeping (younger) or resting energy expenditure (older) were measured by indirect calorimetry at admission and one month later at steady state. RESULTS: Energy expenditure was not different between the two timepoints for younger children, but was slightly elevated at steady state (+50 kcal/d, P=0.049) in the older group. After controlling for gender, changes in fat-free mass and dietary intake, the significance disappeared. Energy intake in both groups was significantly depressed at admission compared to follow-up (P<0.01). CONCLUSIONS: These children and adolescents did not expend excess energy during their acute illness, however, an energy deficit was observed secondary to poor energy intake. Since 20% of patients with sickle cell disease have multiple hospitalizations per year, these results provide justification for the development and evaluation of nutrition care protocols to maintain adequate caloric intake during hospitalization and recovery.

Posttransplantation lymphoproliferative disorders in pediatric patients undergoing liver transplantation.

OBJECTIVES: To study the clinicopathologic and molecular genetic findings in posttransplantation lymphoproliferative disorders (PTLDs) following pediatric liver transplantation and to determine the applicability of a recently proposed consensus classification system. DESIGN: The clinical, pathologic, and molecular genetic findings of 11 PTLDs that occurred in 10 patients are presented. These 10 patients were derived from a group of 121 pediatric patients who underwent liver transplantation at the University of California, San Francisco. The PTLDs were classified using the proposed Society for Hematopathology scheme. Clonality was determined by immunohistochemical detection of monotypic immunoglobulin or by using polymerase chain reaction-based methods to detect monoclonal immunoglobulin heavy-chain gene rearrangements. Epstein-Barr virus (EBV) was detected by immunohistochemistry, in situ hybridization, or polymerase chain reaction. Epstein-Barr virus typing and the presence of LMP1 gene deletions were also analyzed by polymerase chain reaction. RESULTS: There were 3 early lesions, 4 polymorphic PTLDs, and 4 monomorphic PTLDs. Monoclonality was demonstrated in 8 of 9 cases assessed. Epstein-Barr virus was present in all cases; of 9 cases assessed by polymerase chain reaction, the virus was type A in 8 and type B in 1. No EBV LMP1 gene deletions were identified. The corresponding liver explants were negative for EBV in 8 cases and positive in 1 case. Greater than 3 foci of disease and monomorphic PTLD were associated with decreased actuarial survival (P <.05). CONCLUSIONS: The prognosis of pediatric patients with PTLD is favorable for early lesions and polymorphous PTLD, particularly in patients with localized disease. Multifocal disease and monomorphic PTLD are associated with an unfavorable prognosis.

Particle embolization of hepatic neuroendocrine metastases for control of pain and hormonal symptoms.

PURPOSE: To evaluate treatment outcome with respect to the indication for treatment in patients with neuroendocrine tumors metastatic to the liver undergoing hepatic artery embolization with polyvinyl alcohol (PVA) particles. MATERIALS AND METHODS: Charts and radiographs were reviewed of 35 patients undergoing 63 separate sessions of embolotherapy between January 1993 and July 1997. Patient demographics, tumor type, indication for embolization, and complications were recorded. Symptomatic and morphologic responses to therapy were noted, as well as duration of response. RESULTS: Fourteen men and 21 women underwent embolization of 21 carcinoid and 14 islet cell tumors metastatic to the liver. These patients underwent 63 separate episodes of embolotherapy. Of 48 episodes that could be evaluated, response to treatment was noted following 46 episodes (96%). The duration of response was longest in patients treated for hormonal symptoms with (17.5 months) or without (16 months) pain, and was shortest (6.2 months) when the indication was pain alone. Complications occurred after 11 of the 63 embolizations (17%), including four (6%) deaths. Cumulative 5-year survival following embolotherapy was 54%. CONCLUSION: Hepatic artery embolization with PVA particles is beneficial for patients with neuroendocrine tumors metastatic to the liver and may be used for control of pain as well as hormonal symptoms. This therapy should be used cautiously when more than 75% of the hepatic parenchyma is replaced by tumor.