Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity.

CONTEXT: Craniopharyngioma is a brain tumor whose high local recurrence rate has for a long time led to a preference for extensive surgery. Limited surgery minimizing hypothalamic damage may decrease the severe obesity rate at the expense of the need for radiotherapy to complete the treatment. OBJECTIVE: We compared weight gain and local recurrence rates after extensive resection surgery (ERS) and hypothalamus-sparing surgery (HSS). DESIGN: Our observational study compared a historical cohort managed with ERS between 1985 and 2002 to a prospective cohort managed with HSS between 2002 and 2010. SETTING: The patients were treated in a pediatric teaching hospital in Paris, France. PATIENTS: Thirty-seven boys and 23 girls were managed with ERS (median age, 8 years); 38 boys and 27 girls were managed with HSS (median age, 9.3 years). MAIN OUTCOME MEASURES: Data were collected before and 6 months to 7 years after surgery. Body mass index (BMI) Z-score was used to assess obesity and the number of surgical procedures to assess local recurrence rate. RESULTS: Mean BMI Z-score before surgery was comparable in the 2 cohorts (0.756 after ERS vs 0.747 after HSS; P = .528). At any time after surgery, mean BMI Z-score was significantly lower after HSS (eg, 1.889 SD vs 2.915 SD, P = .004 at 1 year). At last follow-up, the HSS cohort had a significantly lower prevalence of severe obesity (28% vs 54%, P < .05) and higher prevalence of normal BMI (38% vs 17%, P < .01). Mean number of surgical procedures was not significantly different in the 2 cohorts. CONCLUSIONS: Hypothalamus-sparing surgery decreases the occurrence of severe obesity without increasing the local recurrence rate.

Elevated plasma ferritin and busulfan pharmacodynamics during high-dose chemotherapy regimens in children with malignant solid tumors.

Hepatic veno-occlusive disease (HVOD) is a frequent complication during hematopoietic stem-cell transplantation (HSCT). A strong relationship has been demonstrated between busulfan exposure and HVOD for busulfan-cyclophosphamide and allogeneic HSCT in adults. Busulfan disposition after the first intake was studied in 77 children treated for solid malignancies with high-dose busulfan-containing regimens and autologous HSCT. Busulfan was combined with cyclophosphamide and melphalan (n=30), melphalan (n=27), and thiotepa (n=20). No relationship was observed between busulfan exposure and HVOD. In contrast, plasma ferritin at baseline was higher in patients with HVOD (750 ng/ml (20-3,110)) compared with those without HVOD (189 ng/ml (8-3,967), P=0.012). Multivariate analysis showed that a ferritin level exceeding 300 ng/ml was the only risk factor for HVOD with an odds ratio of 4.0 (confidence interval 95% (1.5-11.2), P=0.0071). A high ferritin level at baseline was explained by the diagnosis of neuroblastoma, related treatments and transfusions.

Extracellular protease activity of different Pseudomonas strains: dependence of proteolytic activity on culture conditions.

AIMS: This study investigated the effect of growth conditions on proteolytic activity of a Pseudomonas strain, named Pseudomonas sp. LBSA1, isolated from bulk raw milk. It was compared with three Pseudomonas chlororaphis and one Pseudomonas fluorescens strain from culture collections. METHODS AND RESULTS: Bacteriae were grown in a minimal salt medium. For all the strains, addition of 1% (v/v) skim milk to the growth medium was sufficient to induce protease production in 48-h culture. Addition of 1 mmol l(-1) calcium chloride permitted the detection of proteolytic activity of four strains in 48-h cultures but not for Pseudomonas sp. LBSA1. The five strains presented two patterns of proteolytic activity when grown in the minimal salt medium supplemented with 2% (v/v) skim milk at various temperatures for 48 h. Two electrophoretic protease patterns were also obtained from the zymogram of extracellular medium for the five strains. CONCLUSIONS: The growth conditions permitting protease production are variable and do not depend on the genus of the producing strain. SIGNIFICANCE AND IMPACT OF THE STUDY: For the first time a study on proteolytic activity of P. chlororaphis strains is reported. Among the tested criteria, zymograms of extracellular medium were the only ones that permitted distinguishing the P. chlororaphis strains from the P. fluorescens strain.

Fermentation of chicory fructo-oligosaccharides in mixtures of different degrees of polymerization by three strains of bifidobacteria.

We estimated and compared the action of three selected strains of bifidobacteria in a semi-synthetic medium for different degrees of polymerization of fructo-oligosaccharides contained in three commercial products derived from chicory inulin: Fibrulose F97 (shorter chains), Fibruline Instant (native chains), Fibruline LC (longer chains). Biomass and production of lactate and acetate were greater when the substrate contained mostly shorter chain fructo-oligosaccharides. Shorter chains were first to be consumed, and one strain could use longer chains. As the degree of polymerization increased, residual fructo-oligosaccharides increased after growth of the strains, and the rate of consumption of fructo-oligosaccharides decreased.

Satisfaction with and usage of a hand neuroprosthesis.

OBJECTIVE: To measure the satisfaction with, clinical impact of, and use of an implantable hand neuroprosthesis. SETTING: Eight different medical centers. PARTICIPANTS: Thirty-four individuals with spinal cord injuries at the C5 or C6 motor level. INTERVENTIONS: Participants were implemented with a hand neuroprosthesis that provides grasp and release. The neuroprosthesis includes a surgically implanted stimulator, implanted electrodes sutured to the hand and forearm muscles, and an externally mounted controller. MAIN OUTCOME MEASURE: A survey was mailed to study participants, who were asked to respond to statements such as "If I had it to do over, I would have the hand system implanted again," using a 5-level Likert scale ("strongly agree" to "strongly disagree"). RESULTS: Eighty-seven percent of participants were very satisfied with the neuroprosthesis, 88% reported a positive impact on their life, 87% reported improvements in activities of daily living, and 81% reported improved independence. Participants reported using the neuroprosthesis a median of 5.5 days per week; 15 participants used the neuroprosthesis 7 days per week, and 5 participants reported not using the device. CONCLUSIONS: The neuroprosthesis was used by most participants. The neuroprosthesis performed satisfactorily, increased users' ability to perform activities of daily living and independence, and improved their quality of life.

Functional neuromuscular stimulation for combined control of elbow extension and hand grasp in C5 and C6 quadriplegics.

Spinal cord injury sustained at the C5/C6 level leaves an individual without voluntary control of the muscles of the forearm, hand, or of the elbow extensors. The objective of this research project was to integrate functional neuromuscular stimulation (FNS) control of elbow extension with a previously developed system that provides hand grasp in order to increase the working volume in space in which users can perform functional tasks. Elbow extension control was achieved by detecting the position of the arm in space and determining the magnitude of the gravitational moment acting to oppose extension. An accelerometer was used as the command control source, and this sensor was placed over the ulna near the elbow joint to detect static (gravitational) acceleration, and therefore the gravitational moment acting about the elbow joint. This value determined the level of electrical stimulation required to activate the triceps muscles to full extension against these forces. Combined FNS control of elbow extension and hand grasp was implemented in two quadriplegic subjects. Both subjects were able to reach and grasp objects at locations in space which were unattainable without triceps activation.