Cerebellar mutism: the rehabilitation challenge in pediatric neuro-oncology: case studies.

Cerebellar Mutism Syndrome (CMS), also known as posterior fossa syndrome (CMS/PFS), refers to a transient loss of speech followed by dysarthria and is a common complication in the pediatric population following resection of a cerebellar tumor. Recognition and rehabilitation of CMS is critical in the post-operative phases of recovery. Two case studies, including neuropsychological assessments, along with the family and patient's perspectives are reviewed.

Adiposity and plane of nutrition influence reproductive neuroendocrine and appetite responses to intracerebroventricular insulin and neuropeptide-Y in sheep.

Long-term nutritional background is thought to influence hypothalamic appetite and reproductive neuroendocrine responses to short-term nutritional feedback. In order to investigate this phenomenon, the effects of intracerebroventricular administration of insulin or neuropeptide-Y (NPY) on LH secretion and voluntary food intake (VFI) were examined in sheep that were initially thin and kept on an increasing nutritional plane (INP), or initially fat and kept on a decreasing nutritional plane (DNP), for 10 weeks. Intracerebroventricular insulin stimulated LH secretion and suppressed VFI in INP sheep when initially thin, but not when they became fat, and had no effect on LH in DNP sheep when initially fat, and stimulated LH secretion when they became thin. Intracerebroventricular NPY had no effect on LH or VFI in INP sheep when initially thin, decreased LH secretion and increased VFI when they became fat, and decreased LH secretion in DNP sheep when initially fat but had no effect when they became thin. Therefore, sensitivity to insulin increases with low or decreasing nutritional status and decreases with high or increasing nutritional status, whereas sensitivity to NPY increases with high or increasing nutritional status and decreases with low or decreasing nutritional status. In conclusion, reproductive neuroendocrine and appetite responses to acute changes in nutritional feedback signals depend on the individual's longer-term nutritional background.

Comparison of L-serine uptake by human placental microvillous membrane vesicles and placental villous fragments.

Both syncytiotrophoblast microvillous plasma membrane vesicles (MVM) and placental villous fragments are used to characterize the placental uptake of maternal substrate and to investigate changes in uptake associated with pathological conditions. However, the two techniques have not been directly compared. In this study uptake of (14)C-L-serine was compared in placental villous fragments and in MVM prepared from the same placentas. (14)C-L-serine uptake into MVM vesicles was mediated by System L and System A and smaller unidentified Na(+)-dependent and Na(+)-independent components. In villous fragments an unidentified Na(+)-dependent component mediated the majority of (14)C-L-serine uptake followed by System A and System L. The unidentified Na(+)-independent component of L-serine uptake was not detected in villous fragments. The ratio of System A activity to System L activity was similar in villous fragments and MVM vesicles. However, the unidentified Na(+)-dependent component in villous fragments was significantly higher than that in MVM vesicles. This indicates that the main differences in serine uptake mechanisms identified using the two techniques were not due to differences in System A and System L activity but to differences in the unidentified Na(+)-dependent component. This study suggests that uptake of L-serine into MVM vesicles and villous fragments via Systems A and L is comparable, but that this is not true for all components of L-serine uptake.

L-serine uptake by human placental microvillous membrane vesicles.

The human fetus requires more glycine than any other amino acid but placental glycine transfer to the fetus is insufficient to meet fetal demand. L-Serine could represent a major metabolic source of glycine for the human fetus but little is known about the kinetics and physiology of L-serine uptake by the human placenta. We have characterised the amino acid transport systems involved in the uptake of L-serine by the microvillous membrane of the human placental syncytiotrophoblast and compared the uptake rates to those of glycine. L-Serine uptake into microvillous membrane (MVM) vesicles was primarily mediated by system A (MeAIB inhibitable) and system L (BCH inhibitable). Further characterisation using specific substrates of LAT1 and LAT2 found the pattern of L-serine uptake was consistent with that expected for uptake mediated by LAT2. Uptakes were performed with tracer levels of (14)C-L-serine, physiological levels of L-serine, or with physiological levels of amino acids. As amino acid concentrations rose, the proportion of uptake by System L decreased while uptake by uncharacterised Na(+)-independent systems increased. Uptake of Lserine into MVM vesicles had a V(max) of 2.1+/-0.4 nmol/mg protein/min, which was significantly higher than for glycine (V(max) 1.0+/-0.2 nmol/mg protein/min). This indicates that MVM vesicles have a higher uptake capacity for L-serine than glycine, despite a greater demand for glycine over serine for fetal protein synthesis. Further studies are now required to define the fate of L-serine taken up by the placenta and its importance for the fetus.

Randomised, crossover comparison of the single-use SoftSeal and the LMA Unique laryngeal mask airways.

We performed a randomised, crossover, single-blind trial among 168 patients, to compare the single-use SoftSeal and LMA Unique laryngeal mask airways in spontaneously breathing adults. Size-3 and -4 laryngeal mask airways, inserted by experienced anaesthetists, performed equivalently for successful first-time placement (148 (91%) vs 155 (96%) for the SoftSeal and LMA Unique, respectively). The SoftSeal was more often rated as difficult to insert (27 (17%) vs 4 (3%); p < 0.001) and was more likely to show evidence of mucosal trauma after the first insertion (14 (10%) vs 5 (4%); OR 1.3 (95% CI 1.3-11.3); p < 0.05). The fibreoptic view of the larynx was better through the SoftSeal (vocal cords not visible in 27 (17%) vs 44 (27%); p < 0.05) and it more frequently provided a ventilation seal at 20 cmH(2)O (93 (59%) vs 62 (39%); OR 2.15 (CI 1.44-3.21); p < 0.001). In contrast to the LMA Unique(trade mark), its cuff pressure did not increase during nitrous oxide anaesthesia (median (IQR [range]) decrease 3 (- 20-23 [-40-94]) cmH(2)O vs increase of 16 (-2-39 [-54-112]) cmH(2)O; p < 0.01). Both devices were equivalent for the success of first-time insertion and performed satisfactorily clinically. There were some performance differences, but either appears suitable for airway management in spontaneously breathing patients.

Cerebrospinal fluid and serum concentrations of beta-trace protein during pregnancy.

We conducted a prospective observational study among a cohort of 40 term parturients undergoing spinal anaesthesia for elective Caesarean section, to determine the concentration of beta-trace protein in cerebrospinal fluid (CSF) and serum. Serum and CSF samples, taken at the time of dural puncture, were assayed by nephelometry. The mean serum beta-trace protein concentration was 0.39 mg.l(-1) and the mean CSF concentration was 27.9 mg.l(-1), giving a mean ratio of CSF to serum concentration of 76. This ratio is higher than that published for non-pregnant females and for males because of both a higher mean CSF and a lower mean serum beta-trace protein concentration. The concentration correlated positively with both serum creatinine and gestational age. If these concentrations are used to estimate the normal range, we propose that the nephelometric measurement of beta-trace protein might prove a useful diagnostic test for cerebrospinal fluid-cutaneous fistula in parturients.

Persistence of pregnant mare serum gonadotrophin in plasma of Japanese quail (Coturnix coturnix japonica).

Pregnant mare serum gonadotrophin (PMSG) is currently being used to develop a hormone regime that will stimulate reproductive development in Japanese quail (Coturnix coturnix japonica). In this study, the persistence of PMSG in quail plasma was examined after a single injection of the hormone (500 IU). Plasma concentrations of PMSG increased to a peak 12 h after injection and declined to approximately 50% of peak concentrations 24 h after injection. Pregnant mare serum gonadotrophin concentrations declined gradually thereafter and had not returned to basal levels by 96 h after injection. Cloacal diameter, and ovarian and oviducal mass, had increased significantly by 96 h after injection. The persistence of PMSG in quail plasma has implications for the use of the hormone in future regimes stimulating reproductive activity in birds.

Administration of pregnant mare serum gonadotropin to Japanese quail (Coturnix coturnix japonica): dose response over seven days and comparison of delivery by daily injection or osmotic pump.

AIMS: This study is part of a research programme that aims to develop a method of hormone treatment to stimulate breeding in female birds. The aims of this study were to compare dose rates and two different delivery methods, daily injection or osmotic pump, for hormone treatment of Japanese quail (Coturnix coturnix japonica) with pregnant mare serum gonadotropin (PMSG). METHODS: PMSG (0, 5, 10, 20, 40 and 80 IU PMSG/day) was administered to 6-week-old Japanese quail housed under short-day, cool-temperature conditions (8L:16D at 7-10 degrees C) by daily injections or osmotic pump for 7 days. Three additional groups were untreated: one group was dissected at Day 0, and two groups were maintained under either short-day, cool-temperature or long-day, warm-temperature (16L:8D, 20 degrees C) conditions for 7 days. Cloacal diameter was measured daily, and ovarian and oviductal mass and plasma oestradiol concentrations measured at the end of the treatment period. RESULTS: PMSG treatment stimulated ovarian and oviductal growth. After 7 days of treatment with 10-20 IU PMSG, ovarian and oviductal mass were similar to those in birds moved from short to long days. Females treated with the highest doses of PMSG (40 or 80 IU) had significantly larger cloacal diameters and ovarian and oviductal mass than other treated birds or birds maintained under long-day, warm-temperature conditions. Daily injections and osmotic pumps were equally effective methods of delivery. However, there was considerable variation in response to PMSG among individual birds and this was particularly obvious at the higher doses (20-80 IU PMSG). There were no differences in plasma oestradiol concentrations between groups treated using daily injections or osmotic pumps. CONCLUSIONS: A dose of 10 IU PMSG/day was chosen for use in future experiments with Japanese quail, for the first 7 days of treatment. The delivery method of choice for future studies will depend on the practical considerations of the research in question.

Anaesthesia in the Dili General Hospital, East Timor.

An analysis was performed of the anaesthesia caseload of the Dili General Hospital for a four-month period from April 21, 2000 to August 20, 2000.

Psychological and sex features of delayed gut transit in functional gastrointestinal disorders.

BACKGROUND: The relation of demographic and psychological factors to the presence and extent of gut transit impairment in the functional gastrointestinal disorders has received little attention. AIMS: To compare the psychosocial and demographic features of patients with functional gastrointestinal disorders and delayed transit in one region of the gastrointestinal tract with those displaying more widespread delayed transit (that is, delay in two or three regions), and those with normal transit in all three regions. PATIENTS: Of 110 outpatient participants who satisfied standardised criteria for functional gastrointestinal disorders, 46 had delayed transit in one region, 32 had delay in two or three regions, and 17 exhibited normal transit in all regions. METHODS: Transit in the stomach, the small intestine, and the large intestine was assessed concurrently using a wholly scintigraphic technique; psychological status was assessed using established psychometric measures. RESULTS: Patients with delayed transit displayed demographic and psychological features that contrasted with patients with normal transit in all regions. In particular, widespread delayed transit featured female sex, a highly depressed mood state, increased age, frequent control of anger, and more severe gastric stasis, while the features distinguishing normal transit were male sex and high levels of hypochondriasis. CONCLUSION: These data suggest the existence of a distinct psychophysiological subgroup, defined by the presence of delayed transit, in patients with functional gastrointestinal disorders.

Functional gastrointestinal disorders: psychological, social, and somatic features.

BACKGROUND: Psychological, social, and extraintestinal (somatic) disturbances are prominent features of functional gastrointestinal disorders (FGID); little attention, however, has been given to differences in the nature of these disturbances in the various FGID subgroups. AIMS: (1) To determine whether psychological, social, and extraintestinal factors are associated with specific FGID, and/or with the overall severity and extent of FGID disturbance (the number of coexistent FGID subgroups present in any individual); and (2) to determine whether chronic social stressors link gastrointestinal, extraintestinal, and emotional symptomatologies in FGID. PATIENTS: One hundred and eighty eight outpatients, fulfilling standard criteria for one or more functional gastroduodenal or functional bowel disorders. METHODS: Utilising detailed and objective interview and questionnaire methods, detailed gastrointestinal, extraintestinal, psychological, and social data were collected. RESULTS: Chronic stressors and extraintestinal and emotional symptomatologies were prominent features of functional dyspepsia (FD) and irritable bowel syndrome (IBS) alone. These particular features were, however, highly specific for particular FD and/or IBS subgroups. The chronic threat component of social stressors predicted the nature and extent of multisystem (gastrointestinal, extraintestinal, and emotional) symptomatology. CONCLUSIONS: Notable differences between the various FGID subgroups support the symptom based classification of FGID. Chronic stressor provoked psychological and extraintestinal disturbance is most specific for the FD-IBS group of syndromes.

Level of chronic life stress predicts clinical outcome in irritable bowel syndrome.

BACKGROUND: Life stress contributes to symptom onset and exacerbation in the majority of patients with irritable bowel syndrome (IBS) and functional dyspepsia (FD); research evidence is conflicting, however, as to the strength of these effects. AIMS: To test prospectively the relation of chronic life stress threat to subsequent symptom intensity over time. PATIENTS: One hundred and seventeen consecutive outpatients satisfying the modified Rome criteria for IBS (66% with one or more concurrent FD syndromes) participated. METHODS: The life stress and symptom intensity measures were determined from interview data collected independently at entry, and at six and 16 months; these measures assessed the potency of chronic life stress threat during the prior six months or more, and the severity and frequency of IBS and FD symptoms during the following two weeks. RESULTS: Chronic life stress threat was a powerful predictor of subsequent symptom intensity, explaining 97% of the variance on this measure over 16 months. No patient exposed to even one chronic highly threatening stressor improved clinically (by 50%) over the 16 months; all patients who improved did so in the absence of such a stressor. CONCLUSION: The level of chronic life stress threat predicts the clinical outcome in most patients with IBS/FD.

Jejunal sensorimotor dysfunction in irritable bowel syndrome: clinical and psychosocial features.

BACKGROUND & AIMS: The interrelationships between enteric hypersensitivity, dysmotility, and psychosocial factors in the pathogenesis of irritable bowel syndrome (IBS) are poorly understood. The aims of this study were to (1) compare the clinical, jejunal motor, and psychological features in patients with IBS who have heightened sensitivity and those who have normal sensitivity to jejunal balloon distention and (2) examine psychosocial correlates of sensory and/or motor dysfunction. METHODS: Female patients with IBS (n = 24) and healthy controls (n = 9) underwent 24-hour ambulant duodenojejunal manometry, assessment of jejunal sensitivity by balloon distention, and comprehensive psychosocial assessment. RESULTS: In 6 (25%) and 10 (42%) patients, hypersensitivity was present at the threshold for initial perception and at the threshold for pain, respectively. After ingestion of a high-energy standard meal, all patients with heightened sensitivity for perception had abnormalities in the postprandial motor pattern compared with one third of patients with normal sensitivity. In this subgroup with hypersensitivity and postprandial dysmotility, clinical features were not discriminative but an inherently ineffectual coping style featuring both anger hyperreactivity and defensive control of anger was highly characteristic. CONCLUSIONS: In IBS, abnormal postprandial jejunal motor activity is related to jejunal mechanoreceptor-related hypersensitivity, and such sensorimotor dysfunction has a specific psychological profile.

Suppression of anger and gastric emptying in patients with functional dyspepsia.

Psychologic distress and gastric motor dysfunction have both been implicated in the pathogenesis of functional (non-ulcer) dyspepsia (FD). This study assesses the association between psychologic factors and gastric emptying in 28 FD patients. Subjects completed an extensive range of psychologic questionnaires and underwent dual-isotope scintigraphic assessment of solid and liquid gastric emptying. Attempts to resist, control, suppress, and hold in anger, to adopt a fighting spirit whilst dealing with chronic stressors, and manifest unhappiness were predictors of prolonged gastric emptying. These findings suggest that psychologic factors may be important in the aetiology of gastric stasis and subsequent upper gastrointestinal symptoms in patients with functional dyspepsia.

Functions of the two adenovirus early E1A proteins and their conserved domains in cell cycle alteration, actin reorganization, and gene activation in rat cells.

Rat embryo cells were infected with adenovirus type 5 mutants that code for only one of the two early E1A proteins, mutants with defects in one of the two conserved regions common to the two proteins, or mutants with defects in the 46-amino-acid region unique to the 289-amino-acid E1A protein. Cells were scored for altered cell cycle progression, disruption of actin stress fibers, and activation of E2A expression. Mutants lacking either E1A protein were able to cause all of these effects; but mutants lacking a 243-amino-acid protein had less effect, and mutants lacking a 289-amino-acid protein much less effect, than wild-type virus. A mutation in any of the three conserved regions caused a defect in each E1A effect. To investigate the reported function of conserved domain 2 in mitosis, we monitored by fluorescence-activated cell sorter the reduction in Hoechst 33342 fluorescence that occurs when cells divide after undergoing a round of DNA replication in 5-bromodeoxyuridine. A smaller percentage of adenovirus-infected cells than mock-infected cells divided within a given period after completing a round of DNA replication. Viruses with mutations in conserved domain 2 were defective for initiation of cellular DNA replication, as were all other E1A mutants we have examined, but had no specific defect in cell division compared with wild-type virus. Thus, although there may be some specialization of function between the two E1A proteins and between their conserved domains, it was not apparent in the aspects of E1A function and the mutants that we examined.

Anesthetic care of pediatric surgical patients.

Some technical aspects of intraoperative anesthetic care of pediatric surgical patients are discussed. Recent concepts of premedicant, anesthetic and muscle relaxant drugs as related to the pediatric patient are presented. Endotracheal intubation is an integral part of the pediatric anesthetic management. Adequacy of fluid and blood replacement is emphasized. Most current pediatric anesthetic systems incorporate the "T piece" principle. Maintenance of a near normal PaCO2 could be accomplished by allowing partial rebreathing during controlled ventilation. Current status of three useful techniques is presented: deliberate hypotension, hemodilution, and the rapid induction-intubation technique for children with a full stomach. Anesthetic considerations of special problems, such as neurosurgery or cardiac surgery and monitoring, are not discussed.

Preoperative and postoperative care of the pediatric surgical patient.

The preoperative and postoperative care of the pediatric surgical patient is considered under the headings of elective surgery, urgent surgery, surgery for trauma, and other emergency surgery. The specialized care of the neonatal surgical patient is not discussed. The approach outlined is the personal approach of the authors. Points stressed are the importance of preserving normal hydration and blood glucose levels before elective surgery, and the importance of correcting major deficits before urgent or emergency surgery.

Surgical palliation in aortic atresia.

In an infant with aortic atresia, two surgical procedures resulted in a 45 day postoperative survival. An atrial septectomy was initially performed. This was followed by the insertion of a Dacron graft from the main pulmonary artery to the descending thoracic aorta; the pulmonary artery was handed distal to the graft and the ductus arteriosus was ligated. Prostaglandin E1 was infused from the time of the diagnostic study to the second surgical procedure, 48 hours later. A postoperative cardiac catheterization was performed 3 weeks following the operation. Death occurred at 67 days of age from renal failure and a low cardiac output state.