Splenogonadal fusion: a rare cause of testicular enlargement.

Splenogonadal fusion is a rare congenital abnormality found in boys and girls. There is fusion of the developing splenic anlage and the gonadal mesoderm at approximately week 5 of intrauterine life. It commonly presents as a testicular mass treated with an unnecessary orchidectomy. We report two cases of the discontinuous type presenting with testicular swelling and review the literature.

Characteristics of protein and energy metabolism in neonates with necrotizing enterocolitis--a pilot study.

BACKGROUND/PURPOSE: It is assumed that neonates with necrotizing enterocolitis (NEC) are hypermetabolic. However, the dynamics of protein and energy metabolism in neonates with NEC have not been characterized. The purpose of this study was to test the hypothesis that protein turnover and energy expenditure are increased during the acute stage of NEC and later return to normal values. METHODS: A pilot study was performed on six neonates with proven NEC (Bell's stage II or III). Patients were studied in two phases: (1) in the acute stage of their disease and (2) when their clinical condition had stabilized. Whole-body protein turnover was calculated using an intravenous infusion of [1-13C] leucine and by measuring the isotopic enrichment of plasma [13C]alpha-ketoisocaproic acid and 13CO2. Respiratory gas exchange was measured simultaneously by computerized indirect calorimetry. RESULTS: Median gestational age was 36 weeks (range, 28 to 40) with a median postnatal age of 21 days (range, 6 to 47). All patients recovered from the acute episode, although three patients died after recovering from the acute disease from other conditions. The patients studied showed marked variability in protein metabolism kinetics. However, there was no difference in whole-body protein flux between the acute phase (7.6 g/kg/d; range, 5.6 to 18.2) and the recovery phase (7.0 g/kg/d; range, 6.9 to 12.2; P = .89). Furthermore, there was no difference in any of the component parts of wholebody protein turnover. Resting energy expenditure did not change between the acute phase (42.8 kcal/kg/d; range, 34.4 to 52.5) and the recovery phase (51.0 kcal/kg/d; range, 34.9 to 55.3; P = .18). CONCLUSIONS: This pilot study shows that the rates of protein and energy metabolism in neonates with NEC are comparable with reported values in stable neonates. There was no difference in protein or energy dynamics between study phases. The authors speculate that neonates with NEC may divert the products of protein synthesis from growth to tissue repair.

Preputial intussusception or acquired megaprepuce.

Preputial intussusception, or acquired megaprepuce, is a clinical condition whose incidence is increasing. It results from infolding of the outer preputial skin, obstruction of urinary flow, and the development of a phimosis. With time the process continues, resulting in a distinctive clinical picture. Treatment by early circumcision cures the problem and allows resolution of the sequelae.

Effect of major abdominal operations on energy and protein metabolism in infants and children.

PURPOSE: The authors attempted to test the hypothesis that infants and children increase whole-body protein flux and energy metabolism during the early postoperative period. METHODS: Ten infants and children (age range, 2 days to 3 years; weight range, 1.5 to 14.2 kg) who had undergone a major operation were studied. Anaesthesia was standardised, and operative stress score (OSS) recorded. Patients were studied for 4 hours preoperatively and for the first 6 hours after surgery. Respiratory gas exchange was measured by computerised indirect calorimetry. The components of whole-body protein turnover were estimated by giving an intravenous infusion of [1-13C]leucine, and by measuring the isotopic enrichment of plasma [13C]alpha-ketoisocaproic acid by gas chromatograph mass spectrometry and 13CO2 enrichment by isotope ratio mass spectrometry. RESULTS: Median duration of the operation was 73.5 minutes (range, 28 to 285 minutes) with a OSS of 8 (range, 7-17). There were no significant differences in oxygen consumption and resting energy expenditure between the two study phases. The respiratory quotient (RQ) fell from a preoperative value of 0.92 (range, 0.81 to 1.08) to 0.89 (range, 0.79 to 0.95) postoperatively (P = .04). The authors found no significant differences in the rates of whole body protein flux, protein synthesis, amino acid oxidation, and protein degradation between the study phases. CONCLUSIONS: Infants and children do not increase their whole-body protein turnover and metabolic rate after major operations. The observed decrease in RQ reflects mobilisation of endogenous fat. We speculate that the lack of catabolism observed in children is caused by a diversion of protein synthesis from growth to tissue repair.

The effect of complete excision on stage III neuroblastoma: a report of the European Neuroblastoma Study Group.

UNLABELLED: Current opinion is divided about the value of excisional surgery in Evans stage III neuroblastoma. AIMS: To evaluate and correlate the survival of patients with stage III neuroblastoma with the effectiveness of the surgical excision, as assessed by (1) the surgeon (resection data) at the time of operation and (2) the pathologist (excision data). METHODS: The ENSG (European Neuroblastoma Study Group) database of 202 patients from 29 centres with proven stage III were analysed. The data include all patients with neuroblastoma diagnosed between 1982 and 1992 and their subsequent follow-up. RESULTS: Patients were grouped according to the extent of resection (100%, 75% to 99%, and < 75%) and the completeness of excision (complete, microscopic residual, macroscopic residual). There were 123 with resection data, a subgroup of 104 with excision data, and 27 with no excision. There was no statistically significant difference (log rank test) in overall survival (p = 0.11) or event-free survival between the resection subgroups, even when the data from patients without resection were included. Complete excision was associated with a highly significant survival advantage, in terms of overall survival (P = .007) and event-free survival (P = .006). This effect is most obvious among patients with the worst prognosis: older children and those with an abdominal tumour. CONCLUSION: Histological confirmation of complete excision confers a significant survival advantage for patients with stage III neuroblastoma and justifies a painstaking attempt at complete resection.