Long-term outcome and complications of children born with meningomyelocele

The long-term functional outcome of 101 children born with meningomyelocele between 1971 and 1981 was assessed, by a combination of retrospective chart review and follow-up assessments. The children had been managed at birth using a process ofnonstandardized selection. Eighty-three of the 101 patients survived after a minimum follow-up of 8.6 years, for a mortality rate of 18%. Forty-four of 83 children (53%) were community ambulators, and this correlated well with the presence of intact quadriceps function. Forty-eight children (58%) attended normal school and were grade-appropriate. Sixty-two of 83 patients (75%) were socially continent of urine, and 71/83 (86%) were socially continent of stool. Hydrocephalus was present in 93 of the 101 children in the study, and 85 children were shunted. Half of the shunted children required a shunt revision in the first year of life, and thereafter the rate of revision decreased, so that after 2 years the risk of revision was approximately 10% per year.     

Comparison of propofol induction with thiopentone or methohexitone in short outpatient general anaesthesia

The per- and post-operative characteristics of three different i.v. anaesthetic induction agents were studied double-blindly in 75 patients admitted for outpatient gynaecological dilatation and curettage. All the patients were premedicated with midazolam 0.1 mg/kg i.m. Induction started with alfentanil 0.015 mg/kg i.v. 60 s before either: propofol 2.2 mg/kg i.v., or thiopentone 4.0 mg/kg i.v., or methohexitone 2.0 mg/kg i.v. All the patients received 66% nitrous oxide in oxygen. The propofol patients were significantly better relaxed and had a higher incidence of hypotension during the procedure. The methohexitone patients had higher pulse rates and a higher frequency of hiccups during the procedure. Propofol induction resulted in a faster awakening of the patients and a better recovery function compared with methohexitone for the first 15 min and compared with thiopentone for the first 240 min after the procedure. Postoperative side-effects were less frequent in the thiopentone group, and minor abdominal pain was significantly more frequent in the propofol group. There was no significant difference between the groups for any variable after 240 min postoperatively.     

Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

超声引导下肿瘤内注射^90钇玻璃微球的肝癌综合治疗

超声引导下对２８例肝癌病人进行了肿瘤内注射＾９０钇玻璃微球（Ｙ－９０ＧＴ ＭＳ）为主的综合性治疗（部分病人配合肿块周边注射无水酒精及门静脉穿刺化疗）。随访２～１６个月（平均７．９个月），病人全健在。肿瘤缩小率为９１％，其中显著缩小达７５％；肿块回声呈致密增强，少数呈混合型或等回声型；肿瘤内及周边血流信号显著减少；原甲胎蛋白升高者１３例，１１例显著下降，其中６例降至正常；病人症状减轻，全身情况改善。     

Could bipolar vessel sealers prevent bile leaks after hepatectomy?

BACKGROUND AND AIMS: The aim of this work was to test the feasibility of using a bipolar low thermal acting system inducing collagenic sealing but not protein coagulation to secure hepatic parenchyma cutting. MATERIALS AND METHODS: Thirty consecutive hepatectomies were carried out using kellyclasy plus ligatures and clips (controls), while the following 50 hepatectomies used kellyclasy plus bipolar vessels sealer (BVS). Blood loss, duration of hepatic pedicle clamping, length of hospital stay, and complications were recorded. RESULTS: There was no statistically significant difference in blood loss and duration of clamping between controls and BVS. Specific complications (9/21 in the control group vs 1/49 for the BVS group, p<0.00045) and length of hospital stay (14 days in the control group vs 11 days in the BVS group, p<0.014) were statistically lower in BVS group than in the controls, mainly due to prevention of bile duct leakages. CONCLUSIONS: Our data suggest that BVS may be particularly efficient to achieve bilistasis leading to the highest level of safety in performing hepatectomies. Further studies are now needed to confirm its superiority on the classical biliary ducts occlusion techniques.     

Adult living donor liver transplantation: living donation of the right liver lobe

Background Adult living donor liver transplantation (LDLT) has become a routine treatment option for patients waiting for liver transplantation. In European and North American countries, LDLT for adult recipients is mainly performed with right lobe grafts. Indications, when compared to deceased donor liver transplantation, are controversial. Materials and methods In our institution, patients suffering from hepatocellular carcinoma in cirrhosis, non-resectable hilar cholangiocarcinoma, viral hepatitis associated cirrhosis, as well as cholestatic liver and biliary disease are considered good candidates for LDLT. Results In this overview, donor evaluation, graft selection, and the donor operation with special regard to operative techniques and strategies are discussed. For visualization, a 5-min video sequence of the standard donor operation as performed in our institution is attached. Conclusion Given the ongoing shortage of donor organs, adult LDLT has become a routine treatment option for patients waiting for liver transplantation. The associated inevitable risk for the healthy donor, however, remains ethically controversial. Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Caffeine restores regional brain activation in acute hypoglycaemia in healthy volunteers.

AIMS: Caffeine enhances counterregulatory responses to acute hypoglycaemia. Our aim was to explore its effects on cortical function, which are not known at present. METHODS: Regional brain activation during performance of the four-choice reaction time (4CRT) at different levels of complexity was measured using functional magnetic resonance imaging (fMRI) at euglycaemia (5 mmol/l) and hypoglycaemia (2.6 mmol/l) in the presence and absence of caffeine in six healthy right-handed men. RESULTS: During hypoglycaemia, caffeine enhanced adrenaline responses to hypoglycaemia (2.5 +/- 0.7 nmol/l to 4.0 +/- 1.0 nmol/l, P = 0.01) and restored the brain activation response to the non-cued 4CRT, the linear increases in regional brain activation associated with increased task complexity and the ability to respond to a cue that were lost in hypoglycaemia alone. CONCLUSIONS: Caffeine can sustain regional brain activation patterns lost in acute hypoglycaemia, with some restoration of cortical function and enhanced adrenaline responsiveness. A methodology has been established that may help in the development of therapies to protect against severe hypoglycaemia in insulin therapy for patients with diabetes and problematic hypoglycaemia.     

Staged anterior resection and TEM to preserve rectal function in synchronous malignant and benign rectal lesions

BACKGROUND: Traditionally patients with a high rectosigmoid carcinoma and a synchronous large distal rectal adenoma would be treated by low anterior resection with associated loss of rectal function. METHOD: Four patients with a carcinoma of the upper rectum or distal sigmoid colon and a synchronous distal rectal adenoma were treated by high anterior resection followed by staged Transanal Endoscopic Microsurgery (TEM) thus conserving the distal rectum. Preoperative and postoperative rectal function was assessed using the St. Mark's incontinence score. RESULTS: The proximal carcinomas and distal adenomas were 12-18 cms and 0.5-9 cms respectively from the dentate line. The mean surface area of the distal adenomas was 9.7 cms2. There were no deaths or major complications. There were no recurrences after a mean follow-up of 31.5 months. Rectal function was unchanged in three patients with a minor increase in the score in one. CONCLUSION: Staged high anterior resection and 'rEM offers effective treatment of synchronous rectosigmoid carcinoma and distal rectal adenoma with preservation of rectal function.     

Changes in International Normalized Ratio (INR) and Model for Endstage Liver Disease (MELD) Based on Selection of Clinical Laboratory

Priority for liver transplantation is based on the Model for Endstage Liver Disease (MELD) score, a mathematical function which includes international normalized ratio (INR). We present an analysis to determine the lab-to-lab variation in INR at 14 clinical laboratories across the United States. We performed a survey to identify representative clinical laboratories across the United States, where INR was measured in the determination of MELD score. Five 'standard' samples for INR were formulated and were sent to the 14 clinical laboratories to determine variation in INR and MELD score. Among the 14 clinical laboratories, the range in INR for the five samples was: sample 1 (1.2-2.0), sample 2 (1.4-2.5), sample 3 (1.7-3.4), sample 4 (1.9-3.7) and sample 5 (2.4-5.1). The range in calculated MELD score was: sample 1 (8-14), sample 2 (10-17), sample 3 (12-20), sample 4 (14-21) and sample 5 (16-25). The selection of the clinical laboratory used to determine INR may result in substantial changes in MELD score independent of severity-of-illness. These data suggest that further review of interlaboratory variation in MELD should be undertaken because of the potential impact on prioritization for liver transplantation.     

Phase-sensitive cardiac tagging--REALTAG.

Fully inverting spins, instead of merely saturating them, provides superior contrast for tagging procedures. The resulting improvement in tag contrast-to-noise ratio (CNR) yields higher-precision tag detection. Also, thinner slices and hence reduced tag separations can be employed, providing displacement and strain measurements with better spatial resolution. Alternatively, the improved tag contrast can be used to obtain cine images covering a greater portion of the cardiac cycle. The use of standard magnitude reconstruction for images of these inversion tags causes rectification of the negative-valued signals from the tags, confounding the image interpretation. Therefore, a phase-sensitive reconstruction scheme of the inverted tags must be employed. Here we demonstrate the implementation of inverted tags with phase-sensitive reconstruction in a ramped-flip-angle, steady-state free precession (SSFP) sequence.