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Aim Haemorrhoidal disease can severely affect a patient's quality of life. Its classification is commonly based on morphology of the degree of prolapse; however, this does not take into account the symptoms and impact on the quality of life. The aim of this systematic review was to determine the most appropriate instruments that classify the severity of disease according to symptoms. Method A PRISMA-compliant search was conducted in December 2019 to identify studies that described the validation of a haemorrhoidal symptom score. The measurement properties of the scoring systems were assessed based on the consensus-based standards for the selection of health status measurement instruments (COSMIN) methodology for systematic reviews for patient-reported outcome measures. Results A total of 5288 articles were identified, with five articles included. Three studies developed a scoring system based on a set of core symptoms for a cohort of patients and validated the score against treatment outcomes. One study developed a disease-specific quality of life questionnaire based on symptoms to evaluate disease burden. One study combined both quality of life and symptom measures and tested measurement properties on two cohorts of patients. Only one study demonstrated satisfactory valid, reliable and responsive measurement criteria. Conclusion A single study demonstrated sufficient quality in measurement properties to be recommended for clinical use. Further studies in this area should utilize consensus-based standards for designing and reporting validation research to ensure that the appropriate evidence base is acquired if any further patient-reported outcome measures are to be recommended.

Clinical use of genome-wide sequencing (GWS) requires pre-test genetic counseling, but the availability of genetic counseling is limited. We developed an interactive online decision-support tool, DECIDE, to make genetic counseling, patient education, and decision support more readily available. We performed a non-inferiority trial comparing DECIDE to standard genetic counseling to assess the clinical value of DECIDE for pre-GWS counseling. One hundred and six parents considering GWS for their children with epilepsy were randomized to conventional genetic counseling or DECIDE. Following the intervention, we measured parents' knowledge and empowerment and asked their opinions about using DECIDE. Both DECIDE and conventional genetic counseling significantly increased parents' knowledge, with no difference between groups. Empowerment also increased but by less than 2% in each group. Parents liked using DECIDE and found it useful; 81% would recommend it to others; 49% wished to use it along with a genetic counselor; 26% of parents preferred to see a genetic counselor; 7% preferred DECIDE alone; and 18% had no preference. DECIDE appears equivalent to genetic counseling at conveying information. In addition, it was highly acceptable to the majority of study participants, many of whom indicated that it was useful to their decision-making. Use of DECIDE as a pre-test tool may extend genetic counseling resources.

Background General practitioners with specialty interests (GPwSIs) have been an emerging entity in the last decade or so and aim to improve patient's access to specialist level care in the primary care setting. This is achieved by them providing equivalent quality and outcomes to secondary consultant-led services, while not necessarily providing the same breadth of clinical care as them. In this systematic review, we attempt to address their efficacy for surgical procedures and specialties. Methods PRISMA guidelines were followed and an electronic literature search was performed independently by two authors using predefined search terms across EMBASE, Ovid MedLine, PubMed, PSYCINFO and the Cochrane Library databases. A total of 817 articles were reviewed after which only six were included for the systematic review. Results Of the six articles selected, three studies analysed efficacy of GPwSIs with regard to surgical excision of skin lesions. One study looked at the economic evaluation of a GPwSI-led dermatology service in primary care and included GPwSIs carrying out skin excisions. The remaining two included studies were from the same institution and evaluated hernia repairs at a single centre general practitioner practice. Conclusion There is generally, a paucity of evidence looking at the efficacy of GPwSIs for surgical procedures. While they seem to provide an acceptable standard of specialist care in the primary care setting, they do not appear to save money. However, they provide an alternative workforce and the improved access to care that results from it may offset their higher costs.

Background: The prognostic significance of lymph node evaluation is not well described for rectal cancer due to a lack of reproducibility in nodal counts and variable use of adjuvant and neoadjuvant therapy. The aim of this study was to examine the role of quantitative lymph node evaluation as an independent marker of prognosis in stage III rectal cancer. Methods: New Zealand Cancer Registry data were retrieved for consecutive patients with rectal cancer from January 1995 to July 2003. Cases with node-negative tumours, distant metastases, death within 30 days of surgery and incomplete data fields were excluded. Three nodal stratification systems were investigated – Total Number of Nodes examined (TNN), Absolute number of Positive Nodes (APN) and Lymph Node Ratio (LNR). Univariate and Cox regression analyses were performed with 5-year all-cause mortality as the primary end point. Results: The study identified 895 stage III rectal cancer cases. The mean APN and LNR were significantly higher in patients who died within 5 years. An increasing APN or LNR was associated with a significant increase in 5-year mortality. The APN and LNR were also powerful predictors of 5-year mortality after correcting for other factors using Cox regression. The TNN was of no prognostic significance. Conclusions: Both the APN and LNR are highly effective at independently predicting and stratifying 5-year mortality in stage III rectal cancer. The significant predictive value of the LNR is likely to be a reflection of the APN rather than one functioning in autonomy, given that the TNN was of no prognostic significance.

Summary Objective: To determine the clinical characteristics, surgical challenges, and outcome in children younger than 3 years of age undergoing epilepsy surgery in Canada. Methods: Retrospective data on patients younger than age 3 years who underwent epilepsy surgery at multiple centers across Canada from January 1987 to September 2005 were collected and analyzed. Results: There were 116 patients from eight centers. Seizure onset was in the first year of life in 82%, and mean age at first surgery was 15.8 months (1–35 months). Second surgeries were done in 27 patients, and a third surgery in 6. Etiologies were malformations of cortical development (57), tumor (22), Sturge-Weber syndrome (19), infarct (8), and other (10). Surgeries comprised 40 hemispheric operations, 33 cortical resections, 35 lesionectomies, 7 temporal lobectomies, and one callosotomy. There was one surgical mortality. The most common surgical complications (151 operations in 116 patients) were infection (17) and aseptic meningitis in 13. Of 107 patients with seizure outcome assessed more than one year postoperatively, 72 (67.3%) were seizure free (Engel I), 15(14%) had >90% improvement (Engel II), 12 had >50% improvement (Engel III), and 8 did not benefit from surgery (Engel IV). Development improved in 55.3% after surgery. Conclusion: Epilepsy surgery in children younger than 3 years of age is relatively safe and is effective in controlling seizures. Very young age is not a contraindication to surgery in children with refractory epilepsy, and early surgery may impact development positively.

Summary Purpose: Factors influencing the electroencephalography (EEG) features of absence seizures in newly presenting children with idiopathic generalized epilepsy (IGE) have not been rigorously studied. We examined how specific factors such as state, provocation, age, and epilepsy syndrome affect the EEG features of absence seizures. Methods: Children with untreated absence seizures were studied using video-EEG recording. The influence of state of arousal, provocation (hyperventilation, photic stimulation), age, and epilepsy syndrome on specific EEG features was analyzed. Results: Five hundred nine seizures were evaluated in 70 children with the following syndromes: childhood absence epilepsy (CAE) 37, CAE+ photoparoxysmal response (PPR) 10, juvenile absence epilepsy (JAE) 8, juvenile myoclonic epilepsy (JME) 6, and unclassified 9. Polyspikes occurred in all syndromes but were more common in JME. They were brought out by drowsiness and sleep in fragments of generalized spike and wave (GSW). Polyspikes were more likely to occur during photic stimulation, but were not influenced by age independently. GSW was more likely to be disorganized in JME than JAE, and in JAE than CAE. Increasing age and levels of arousal were more likely to result in organized GSW. Factors specific to each child independently influenced EEG features; the nature of these factors has not been identified. Discussion: The EEG features of absence seizures are influenced by a complex interaction of age, epilepsy syndrome, level of arousal, provoking factors, and other intrinsic factors. Epilepsy syndrome alone cannot predict specific features of GSW; however, JME is more frequently associated with polyspikes and disorganization of the paroxysm.

Acquired brachial-plexus neuropathy outside the immediate neonatal period is uncommon. Pseudopalsy of a limb, associated with osteomyelitis, is well recognized. Acquired brachial-plexus neuropathy as the initial presentation of osteomyelitis of the humerus in the neonatal period is described. Three infants presented at 3, 15, and 21 days respectively, with acute monoplegia consistent with brachial-plexus neuropathy. The infants were afebrile and generally well. Initial radiographs of the humerus were normal and blood cultures grew group-B streptococcus in all infants. Nerve conduction studies were consistent with brachial-plexus neuropathy. Following intravenous antibiotics, there was complete recovery in all infants. Osteomyelitis of the humerus should be considered in infants in whom there are no overt signs of sepsis and who present with brachial-plexus neuropathy. Early diagnosis and appropriate treatment should result in a complete neurological recovery.

Multicenter, retrospective analysis of 70 subjects with TSC following surgery for relief of epilepsy revealed significant associations between younger age at seizure onset, present/prior history of infantile spasms, interictal focality (bilateral versus unilateral), and absence of residual postoperative predominant tuber, and poorer postoperative outcome (p < 0.01). Ictal multifocality, mental retardation, and discordant EEG and MRI data showed a negative trend toward outcome, but were not significant.

Summary: Health-related quality of life (HRQOL) is an important outcome measure in clinical research. Given the psychosocial and behavioral difficulties associated with pediatric epilepsy, evaluating HRQOL in this patient population is of particular importance. Though HRQOL has been examined in pediatric patients receiving focal resection or pharmacological (antiepileptic drug; AED) treatment, it has not been assessed in patients receiving hemispherectomy (HE) for intractable epilepsy. The current study evaluated HRQOL in a sample of pediatric HE cases (N = 26) using previously validated questionnaires relative to surgical (N = 30) and nonsurgical (N = 84) comparison groups. Compared with focal resection and nonsurgical patients, parents of children who received HE reported similar levels of HRQOL. In surgical cases, worse HRQOL was correlated with residual seizure frequency. In both surgical and nonsurgical cases, female gender, higher AED load, and lower functional independence predicted worse HRQOL. Interestingly, HE status (i.e., having undergone HE) predicted fewer epilepsy-related limitations. Consistent with previous findings, AED load, in addition to lower functional abilities, appear particularly detrimental to life quality in pediatric epilepsy. HE, however, is not associated with increased risk for poor HRQOL. When considered in light of the multiple, significant risk factors for poor outcome associated with HE, children who undergo the procedure fare surprisingly well.

Oral melatonin (MLT) has been used by our Vancouver research group in the treatment of paediatric sleep disorders since 1991; slightly over 200 children, mainly with multiple disabilities, who frequently had seizures, have been treated. Three children with markedly delayed sleep onset due to recurring myoclonus were also referred for MLT treatment: two had non-epileptic, and one had epileptic and non-epileptic myoclonus. Low doses of oral MLT (3 to 5 mg) unexpectedly abolished their myoclonus and allowed them to sleep. There were no adverse effects. It appears that certain types of myoclonus, which might be resistant to conventional anticonvulsant medications, may respond to MLT but the mechanism of action is unclear. Further research on this novel treatment is urgently needed.

Frequent infusion of factor concentrates may be challenging in young boys with haemophilia, especially if their disease is complicated by inhibitors. A central venous access device (CVAD) is often placed in young patients in need of repeated infusions for prophylaxis or immune tolerance induction. Although user friendly and capable of providing reliable venous access, these devices are associated with a high complication rate over time. In the haemophilia population, major complications include CVAD-associated infections and deep venous thrombosis, which is most often silent. Established risk factors for catheter-related infection include age less than 6 years at the time of CVAD placement and use of an external CVAD when compared with a totally implantable device such as a port. Avoidance of CVAD-related infections is facilitated by strict adherence to aseptic technique. The risk of deep venous thrombosis appears related to the duration for which the catheter is in place, with the risk increasing beyond 4 years. The promotion of a strict clinic policy in which CVADs are left in place for as short a time as possible should decrease the risk of complications. In rare cases where a totally implantable CVAD cannot be placed for technical reasons, an arteriovenous fistula may provide reliable venous access. In all cases, however, venous access via peripheral veins is preferred over CVADs.

The diagnosis of mitochondrial encephalomyopathies is complex and a system for classification of the diagnosis as definite, probable, and possible has been proposed. The objective of this study was to explore the spectrum of epileptic disorders associated with probable and definite mitochondrial disease in children using this classification system. The patient population with mitochondrial disease and epilepsy was selected from a tertiary care children's hospital. Interictal electroencephalograms and video-EEG recordings were used to characterize seizure types. Ten children fulfilled the criteria for probable or definite mitochondrial disease and had epilepsy. Four had siblings with a similar clinical phenotype. Spasms were the most common seizure type and were the initial seizure type in seven patients and two siblings. Four patients had only partial seizures, with or without generalization, and one patient had seizures that were difficult to classify. Blood lactate concentrations were elevated consistently in patients with partial seizures alone but were occasionally normal in children with spasms. Spasms were the most common presenting seizure type in children with probable and definite mitochondrial disease.

Summary: Purpose: Hyperventilation-induced high-amplitude rhythmic slowing (HIHARS) in children may be associated with clinical episodes of altered awareness. The presence of automatisms has been proposed as a distinguishing feature that helps to differentiate absence seizures from nonepileptic causes of decreased responsiveness. This retrospective, controlled, video-EEG study compared the clinical characteristics of episodes of HIHARS with loss of awareness with those of absence seizures. Methods: The database of a tertiary Children's Hospital was searched for patients studied between April 1993 and April 1997 who had at least one episode of HIHARS with loss of awareness. The absence control group was obtained by selecting the next patient, after an HIHARS study subject, who met the following criteria: (a) had at least one absence seizure occurred during hyperventilation in the EEG recording, and (b) had a diagnosis of idiopathic generalized epilepsy. The video-EEG and medical histories of all patients were reviewed and summarized. Results: We reviewed video-EEG recordings of 77 episodes of HIHARS with loss of awareness from 22 children and 107 absence seizures during hyperventilation from 22 children. Eye opening and eyelid flutter were seen more frequently in absence seizures, whereas fidgeting, smiling, and yawning occurred more frequently during HIHARS episodes. Arrest of activity, staring, and oral and manual automatisms were observed in both groups. Conclusions: Automatisms are common in both HIHARS and absence seizures. Yawning, smiling, and particularly fidgeting occur more commonly and eye opening and eyelid flutter less commonly in HIHARS. However, episodes of HIHARS with loss of awareness clinically mimic absence seizures, and these conditions can be distinguished reliably only by EEG.

Summary: Purpose: Validity studies on health-related quality of life (HRQOL) scales for pediatric epilepsy are few, and cross-validation with other samples has not been reported. This study was designed to assess the validity of three parent-rated measures of HRQOL in pediatric epilepsy: (a) the Impact of Childhood Illness Scale (ICI), (b) the Impact of Child Neurologic Handicap Scale (ICNH), and (c) the Hague Restrictions in Epilepsy Scale (HARCES). Methods: Retrospective data were examined for 44 children with intractable epilepsy. Validity was assessed by evaluating differences across epilepsy severity groups as well as correlations between HRQOL scales and neurologic variables (seizure severity, epilepsy duration, current/prior antiepileptic medications) and psychosocial measures (emotional functioning, IQ, social skills, adaptive behavior). Scale overlap with a global QOL rating also was assessed. Results: The HRQOL measures were moderately to highly intercorrelated. The scales differed in terms of their associations with criterion measures. The HARCES was related to the highest number of neurologic variables and the ICNH to the fewest. All three scales were related to psychosocial functioning and to global quality of life. Conclusions: The results of this study suggest that the three measures are likely adequate measures of HRQOL for use in intractable childhood epilepsy. The measures were highly intercorrelated, and they were all broadly related to criterion measures reflecting specific domains of HRQOL as well as global QOL. Some differences between scales emerged, however, that suggest care in choosing HRQOL instruments for children with epilepsy. Key Words: Quality of life— Epilepsy—Childhood—Validity—Parent ratings.

Summary: Purpose: To analyze the results of surgical treatment of intractable epilepsy in patients with subcortical band heterotopia, or double cortex syndrome, a diffuse neuronal migration disorder. Methods: We studied eight patients (five women) with double cortex syndrome and intractable epilepsy. All had a comprehensive presurgical evaluation including prolonged video-EEG recordings and magnetic resonance imaging (MRI). Results: All patients had partial seizures, with secondary generalization in six of them. Neurologic examination was normal in all. Three were of normal intelligence, and five were mildly retarded. Six patients underwent invasive EEG recordings, three of them with subdural grids and three with stereotactic implanted depth electrodes (SEEG). Although EEG recordings showed multilobar epileptic abnormalities in most patients, regional or focal seizure onset was recorded in all. MRI showed bilateral subcortical band heterotopia, asymmetric in thickness in three. An additional area of cortical thickening in the left frontal lobe was found in one patient. Surgical procedures included multiple subpial transections in two patients, frontal lesionectomy in one, temporal lobectomy with amygdalohippocampectomy in five, and an additional anterior callosotomy in one. Five patients had no significant improvement, two had some improvement, and one was greatly improved. Conclusion: Our results do not support focal surgical removal of epileptogenic tissue in patients with double cortex syndrome, even in the presence of a relatively localized epileptogenic area.

Background The treatment of malnutrition, which is of great concern in paediatric haematology/oncology patients, is fraught with problems. The goals of our study were to document the complications and assess the weight gain with gastrostomy tubes (G-tubes) in this population. Procedure Patient data were acquired by retrospective review of all haematology, oncology, and bone marrow transplant (BMT) patients (n = 44) who received radiologically placed G-tubes at our institution over a 4-year period. Results Forty-four G-tubes were placed (59% peri-BMT). At tube placement, 55% of patients were malnourished and 45% were nourished. Seventy-five percent of patients had the procedure without general anaesthetic. Localized G-tube-site infection was the most common complication (41%). Major complications occurred in 3 patients; 2 patients experienced localized peritonitis, and 1 patient developed a localized collection of pus in the abdominal wall. There were no G-tube-related deaths. At 1 month after the tube insertion, 39% of patients had gained, 54% maintained, and 7% lost weight. At 3 months, 69% had gained, 28% maintained, and 3% lost weight. There was a statistically significant weight gain from the time of the G-tube placement to both 1 month (P < 0.018) and 3 months (P < 0.0001) after G-tube placement. Patients in all diagnosis categories showed improvement from 1 to 3 months. Conclusions We conclude that retrograde tube placement is safe and can frequently be done without general anaesthetic and that G-tube feeding effectively reverses malnutrition and prevents weight loss in this patient population. Med. Pediatr. Oncol. 34:177–182, 2000. © 2000 Wiley-Liss, Inc.

Laparoscopic colonic surgery has an established role in the management of both benign and malignant conditions. Proposed benefits from laparoscopic surgery include decreased pain, decreased metabolic disturbance to the patient and faster recovery. It is now generally accepted that pro-inflammatory mediators, including cytokines, are to a great extent responsible for the metabolic changes associated with injury and surgery, and that these metabolic changes are related to postoperative recovery. Cytokine levels in the serum are decreased after major laparoscopic colorectal surgery compared with open surgery. However, the cytokine concentration in abdominal drain fluid is the same independent of the size of the incision and these concentrations are far higher than those found in the serum suggesting that the peritoneal would from the surgery itself is more important to metabolic events than the skin wound used to access the abdominal cavity to perform the operation. When looked at critically in programmes where patients are optimally managed perioperatively, there appears to be minimal metabolic benefit from performing a major colonic resection using minimal access surgery. Thus, it appears that the wound is critical when the operation involves only minor peritoneal disruption, such as in laparoscopic cholecystectomy, but when large peritoneal defects are created, such as in major colorectal surgery, then the skin wound becomes irrelevant to metabolism and hence recovery. Thus, minimal access does not necessarily equate to minimal invasion and the terms should not be used interchangeably in the context of laparoscopic colorectal surgery.

This scientific statement is the product of the Utstein ’95 Symposium held June 23-24, 1995, at Utstein Abbey, Island of Mosteroy, Rogaland County, Norway. Draft versions were circulated for comment to participants of the Utstein ’95 Symposium; the European Resuscitation Council Executive Committee; the Emergency Cardiac Care Committee of the American Heart Association; the Executive Committees of the Heart and Stroke Foundation of Canada, the Australian Resuscitation Council, and the Resuscitation Councils of Southern Africa; and several outside reviewers. The development of this statement was authorized by the Science Advisory and Coordinating Committee of the AHA and the Executive Committee of the European Resuscitation Council. We do not know the true effectiveness of in-hospital resuscitation. Observed results of the many published studies vary greatly. Studies originate from different settings and have different patient populations. Reports suffer from nonuniform nomenclature and variable inclusion definitions. Patients differ in the extent of comorbid conditions and interventions in place at the time of cardiac arrest. These differences prevent valid interhospital and intrahospital comparisons and make determining the effectiveness of current resuscitation techniques impossible. To develop these guidelines the task force used a consensus development process that originated with the “Utstein style” for reporting outcome data from out-of-hospital resuscitation events. Task force members performed an integrated review of published studies. An initial draft was prepared, discussed, and revised at a 2-day conference. Further drafts were revised and circulated among task force members and discussed face-to-face at three subsequent meetings. The task force defined a set of data elements that are essential or desirable for documenting in-hospital cardiac arrest. Data categories are hospital variables, patient variables, arrest variables, and outcome variables. The “In-Hospital Utstein-Style Template” was developed to summarize these data and recommendations for reporting a specific set of survival rates and outcomes. The task force …

Background: Several previous studies have shown that Gastrografin can be utilized to triage patients with adhesive small bowel obstruction (ASBO) to an operative or a non-operative course. Previous studies assessing the therapeutic effect of Gastrografin have been confounded by post-administration radiology alerting the physician to the treatment group of the patient. Therefore the aim of the present paper was to test the hypothesis that Gastrografin hastens the non-operative resolution of (ASBO). Methods: Patients, diagnosed with ASBO on clinical and radiological grounds, were randomized to receive Gastrografin or placebo in a double-blinded fashion. Patients did not undergo further radiological investigation. If the patient required subsequent radiological intervention or surgical intervention they were excluded from the study. End-points were passage of time to resolution of ASBO (flatus and bowel motion), length of hospital stay and complications. Results: Forty-five patients with ASBO were randomized to receive either Gastrografin or placebo. Two patients were excluded due to protocol violations. Four patients in each group required surgery. Eighteen of the remaining patients received Gastrografin and 17 received placebo. Patients who received Gastrografin had complete resolution of their ASBO significantly earlier than placebo patients (12 vs 21 h, P = 0.009) and this translated into a median of a 1-day saving in time in hospital (3 vs 4 days, P = 0.03). Conclusions: Gastrografin accelerates resolution of ASBO by a specific therapeutic effect.

BACKGROUND Study of involuntary skeletal muscle function (MFA) has been well accepted in the area of nutrition assessment and potentially offers a means for following progress of the critically ill patient. We report on the application of this technique to intensive care patients. METHODS MFA was performed by study of the contraction/relaxation characteristics of the adductor pollicis muscle of the thumb after ulnar nerve stimulation. Serial measurements were made in 16 critically injured patients and 28 patients with severe sepsis and were compared with those obtained from 26 control subjects. Extent of loss of total body protein (TBP) was quantified with in vivo neutron activation. RESULTS Significant difficulties exist in applying this technique to intensive care patients. In the critically injured, only five acceptable traces could be obtained from a possible 58 measurements. For patients with severe sepsis it was possible to obtain an acceptable trace on 12 of 56 occasions. Neuromuscular blockade and lack of patient cooperation were significant impediments to MFA study. Although frequently perceived as unpleasant by these patients, there was no long-term morbidity associated with MFA. No significant differences were seen in maximal relaxation rate at 30 Hz (MMR30) or force frequency ratios (F10/50 and F30/ 50) between trauma patients and controls. In the sepsis patient group, a significantly higher F10/50 was measured (52% +/- 3% severe sepsis vs 40% +/- 1% control subjects, p < .01). Six patients had MFA measured approximately 21 days after the illness, by which stage they had lost 11% of their initial TBP. Compared with control subjects, no significant differences were observed in MRR30 or F30/50, whereas a higher value for F10/50 was measured (48% +/- 1% critical illness vs 40% +/- 1% control subjects, p < .01). CONCLUSIONS The MFA technique is difficult to apply to intensive care patients. No significant disturbance to MFA is seen after critical injury. Severe sepsis results in an elevation of F10/ 50 only. When able to be obtained, MFA results do not reflect the extent of proteolysis but are indicative of the state of cellular energetics.

Most reports of supplementary sensorimotor seizures have been of adults with medically refractory epilepsy. Typically, supplementary sensorimotor seizures have onset in childhood. We describe the electroclinical features in 12 children. Cases were selected from an EEG laboratory population in whom video-EEG was performed routinely on all children. Supplementary sensorimotor seizures were diagnosed when there was bilateral tonic posturing of the upper or lower extremities, preserved consciousness, and lack of postictal confusion. Sensory auras were reported by 8 of 9 children aged > 3 years who had daytime seizures. Speech arrest occurred in all patients in whom it could be assessed, and abnormal vocalization was observed in 7 children. Interictal EEGs were often normal (49% of recordings), and ictal EEG changes could be subtle. An etiology was demonstrated in only 2 children, and brain imaging studies were normal in the other 10 patients. Seizures were controlled with antiepileptic drugs (AEDs) in 6 of the 12 children. The clinical manifestations of supplementary sensorimotor seizures in children are similar to those reported in adults; misdiagnosis is common at all ages.

Summary: Video-EEG monitoring enables correlation of behavioral activity with EEG activity, which is useful in recognition of pseudoepileptic seizures and in investigation of patients for epilepsy surgery. Because most patients are monitored for a prolonged time as in-patients, the cost of the procedure is high. We investigated the value of brief (2–3 h) outpatient video-EEG monitoring in 43 children with frequent seizures, most of whom had symptomatic generalized epilepsy. Indications for monitoring included differentiation of epileptic from nonepileptic behavior, seizure classification, and determination of seizure frequency. Clinical episodes were recorded in 36 of 43 children (83%). A definite diagnosis was established in 9 of the 17 patients investigated to determine the nature of the clinical behavior. Seizures were classified in 1.5 of the 25 patients investigated to determine seizure type, and classification was different from the original in 9 of the 15 children. A change in epilepsy syndrome classification was made in 9 children. The video-EEG allowed diagnosis in 25 of the 43 children (59.5%). Video-EEG appears to be an effective method for outpatient investigation of children with frequent seizures, particularly those with symptomatic generalized epilepsy. ReSUMe Le monitorage video-EEG permet d'etablir des correlations entre l'activite comportementale et l'activite EEG, ce qui est utile dans la reconnaissance de crises pseudo-epileptiques et dans l'evaluation de patients en vue d'une chirurgie de l'epilepsie. La plupart des patients peut beneficier d'une surveillance pendant une periode prolongee, en hospitalisation, et le coet de cette procedure est donc elevee. Les auteurs ont evalue l'intert d'un monitorage video-EEG bref, de 2 a 3 heures, effectuea titre externe chez 43 enfants presentant des crises frequentes, la plupart presentant une epilepsie generalisee secondaire. Les indications pour ce monitorage incluaient le diagnostic differentiel entre les comportements epileptiques et non epileptiques, la classification des crises, et la determination de la frequence des crises. Les episodes cliniques ont ete enregistres chez 36 enfants sur 43 (83%). Un diagnostic precis a eteetabli chez 9 des 17 patients evaluCs en vue d'une diagnostic de la nature du comportement clinique. Une classification des crises a ete obtenue chez 15 des 25 patients evalues en vue d'une ditermination du type des crises et cette classification etait differente de la classification originelle chez 9 des 15 enfants. Un changement de classification du syndrome epileptique a ete effectue chez 9 enfants. La video-EEG a procure une reponse ii question posee chez 25 des 43 enfants (59.5%). La video-EEG semblait etre une methode efficace pour l'evaluation a titre externe d'enfants presentant des crises frequentes, surtout pour ceux qui ont une epilepsie gCnCraliste secondaire. RESUMEN La monitorizacion vidoe-EEG permite estuadiar la correlacion entre el comportamiento y la actividad del EEG util para reconocer los ataques pseudoepilCpticos y tambikn util para la investigacion de enfermos para posible cirugia antiepilkptica. La mayor parte de los pacientes se monitorizan durante largos periodos de tiempo y en regimen de hospitalizacion, lo que eleva mucho los costes de este procedimiento. Los autores han investigado el valor de la monitorizacion vidoe-EEG durante un breve periodo de tiempo (2–3 horas) y en regimen ambulatorio en 43 nifios con ataques frecuentes con generalizaciones secundarias. Las indicaciones para la motorizacion incluian la distincion entre ataques epilepticos y alteraciones del comportamiento no-epilepticas, la clasificacion de los ataques y la determinacion de la frecuencia de los mismos. En 36 de los 43 niiios se registraron ataques clinicos (83%). Se llego a un diagnostico definitivo en 9 de los 17 enfermos investigados para determinar la naturaleza clinica del comportamiento. La clasificacion de los ataques se alcanzo en 15 de los 25 enfermos investigados y determinando el tipo de ataque y fue diferente a la clasificacion original en 9 de los 15 ninos. En nifios se cambio la clasificacion del sindrome epiltptico. El video-EEG proporciono una respuesta valida a la pregunta planteada en 25 de los 43 ninos (59.5%). El video-EEG parece ser un mCtodo eficaz para la investigacidn en regimen ambulatorio de nifios con ataques frecuentes, particularmente los que tienen generalzacion secundaria. ZUSAMMENFASSUNG Mittels Video-EEG-Monitoring konnen Korrelationen zwischen Verhalten und EEG-Aktivitat erfapt werden, urn pseudo-epileptische Anfalle zu diagnostizieren. Ferner konnen Patienten fur epilepsie-chirurgische Eingriffe herausgefunden werden. Bei den meisten Patienten erfolgt das Monitoring stationar und ist daher mit hohen Kosten verbunden. Wir untersuchten bei 43 Kindern mit haufigen Anfallen den Nutzen eines Monitorings von 2 bis 3 Stunden. Es handeke sich meistens urn sekundar-generalisierte Epilepsien; eingeschlossen waren die Untersu-chung von epileptischen und nichtepileptischen Anfallen Anfall-sklassifikation und Anfallshaufigkeit. Bei 36 von 43 Kindern (83%) wurden klinische Auffalligkeiten abgeleitet. Bei 9 von 17 Kindern konnte eine endgultige Diagnose der klinischen Ereignisse erreicht werden. Bei 15 von 25 Kindern wurde der Anfallstypus identifizierter wich gegenuber der Eingangs untersuchung bei 9 von 15 Kindern ab. Bei 9 Kindern konnte eine Zuordnung zu einem epileptischen Syndrom erfolgen. Die diagnostischen Fragen konnten bei 25 Kindern (59.5%) beantwortet werden. Somit stellt das Video-EEG auch bei ambulant vorgestellten Kindern mit haufigen Anfallen, besonders sekundar generalisierten Epilepsien, eine effektive Methode dar.

Application of new genetic techniques has brought remarkable discoveries in the study of genetic diseases. The potential benefits from applying such technology to idiopathic epilepsies include improved understanding of cellular mechanisms and potential new methods of prevention and treatment. The complex problems involved in studying the hereditary epilepsies include: defining of specific phenotypes; detecting genetic and non-genetic heterogeneity; and specifying the appropriate mode of inheritance and penetrance. The gene loci for three primary epilepsies have been localized to specific chromosomal regions, and serve to demonstrate the process used in generalized linkage studies of hereditary epilepsy syndromes. Benign familial neonatal convulsions (BFNC) and Unverricht-Lundborg progressive myoclonus epilepsy are rare single-gene disorders that are sufficiently localized to chromosomal regions that positional cloning studies are likely to succeed. Juvenile myoclonic epilepsy (JME), a common hereditary syndrome with an uncertain mode of inheritance, has been reported to be linked to chromosome 6p. JME presents a challenge for generalized linkage methodology that may be overcome by attending to potential problems reviewed here. The candidate-gene method, combined with studies using animal models, holds promise for understanding these as well as other hereditary epilepsies.

Molybdenum pentacarbonyl-trimethylamine promotes the cyclization of 1-alkyn-4-ols to the isomeric 2,3-dihydrofurans

Background There is an ethnic variation in outcomes for colonic cancer in New Zealand. Whether this disparity is caused by cancer biology or inequitable provision of treatment services after diagnosis has not been elucidated. Method National cancer registry data from 1996 to 2003 were obtained. Incidence and mortality rates for the four major ethnic groups were age-adjusted to the new WHO world population. The impact of age, sex, AJCC stage and site of cancer at diagnosis was compared between ethnic groups using a Cox regression analysis. Results A total of 11 987 colonic cancer registrations were identified. The overall raw 5-year mortality was 53.7%. The age-adjusted incidence in Europeans was more than double that of the Maori, Asian and Pacific populations at 33.0 per 100 000 population/year. Europeans presented at a greater age, with more right sided cancers, and at an earlier stage of disease. The opposite was true for the Maori population. Pacific Islanders and Asians presented at a younger age, but with a similar site, stage and sex distribution to the rest of the population. There were no significant differences in 5 year mortality after diagnosis when age, sex, stage, and site at presentation were controlled for by cox regression analysis. Conclusion These results suggest that age, sex, stage and site at presentation may be more important than inequality in treatment provision after diagnosis in explaining differences in outcomes between the ethnicities. Efforts need to be focused on identifying reasons for the increased risk of colonic neoplasia in Europeans and the later stage disease presentation in the Maori population.

Using a microdissection technique we localized androgen receptors and aromatase activity (AA) in the brain of male guinea-pigs. In addition, we evaluated the effects of castration and androgen replacement on androgen receptor dynamics and induction of AA. In the castrate animal, cytosolic androgen receptor content was highest in the basal hypothalamus, specifically in the median eminence-arcuate nucleus (> 15 fmol mg protein (1)), while lesser levels were found in the preoptic regions and amygdala. Nuclear receptor content was highest (> 150 fmol mg DNA (-1)) in the median eminence-arcuate nucleus, periventricular region of the preoptic area and cortical amygdala. All regions investigated showed a significant decrease in nuclear receptors following castration and an increase with androgen replacement. However, reciprocal changes in cytosolic androgen receptors were not always evident. Aromatase activity was high in the cortical amygdala, medial amygdala, periventricular region of the preoptic area and bed nucleus of the stria terminalis. Castration and androgen replacement had significant stimulatory effects on AA in the ventral medial hypothalamus, median eminence-arcuate nucleus, cortical amygdala and periventricular regions of the preoptic area and anterior hypothalamus. Thus, androgen receptors and AA are unevenly distributed throughout the subcortical regions of the male guinea-pig brain and respond differently to endocrine stimuli. Our data demonstrate that AA is androgen-dependent in some subcortical regions which contain androgen receptors. Even though nuclear receptors in all brain regions were affected by castration and dihydrotestosterone treatment, the events were not always linked to AA regulation. Due to this difference in regulation, AA may serve divergent functions in guinea-pig brain.

Alström disease is a rare disorder; less than 20 cases have been reported. An 11-year-old girl is described with this condition. She has pigmentary retinopathy, sensory neural deafness, obesity, Type II diabetes mellitus, hyperlipidemia, and acanthosis nigricans. However, in addition she developed hepatic dysfunction, pathologically similar to chronic active hepatitis. This may be a further, previously undescribed systemic manifestation of Alström disease.

Introduction: Post-operative fatigue (POF) is an important indicator of recovery after major surgery. Recent data have suggested a relationship between the post-operative peritoneal cytokine response and development of POF. Glucocorticoids decrease the production of cytokines and thus we hypothesised that administration of dexamethasone to patients undergoing colonic surgery may decrease peritoneal cytokine production and POF. Aim: To assess the influence of 8 mg of pre-op IV Dexamethasone on peritoneal cytokine and POF in patients undergoing elective colonic surgery. Method: In a double blinded RCT, 70 consecutive patients undergoing elective, open colonic resection were administered 8 mg I.V. Dexamethasone or saline (Placebo) prior to surgery. All patients were treated within a structured Enhanced Recovery After Surgery program. Results: After exclusions there were 31 patients in the Placebo group and 29 in the Dex group. At baseline there were no differences in the age, ASA, BMI or CR-POSSUM scores. Dexamethasone was associated with a significant reduction in Interleukin-6 (IL-6) and IL-13 in the peritoneum and IL-6 and IL-8 in plasma. Subsequently, patients in the Dex group had lesser POF and fatigue impact scores at days 3 and 7. Patients in the Dex group had less post-op nausea and vomiting. There was no significant increase in complications. Conclusion: Preoperative Dexamethasone administration is associated with a significantly diminished post-operative pro-inflammatory reaction which is associated with clinical benefits in POF and its impact. This intervention may be an effective method of improving outcomes for patients undergoing major colonic surgery within a multimodal enhanced peri-operative care pathway.

Purpose: The most important prognostic factor in colon cancer is the presence or absence of metastases to regional lymph nodes. The aim of this study was to evaluate the relationship between the number of nodes examined in Stage II and III colonic cancers and 5-year mortality in the New Zealand population. Methodology: New Zealand Cancer Registry data were retrieved for consecutive cases of colonic cancer from January 1995–July 2003. Patients with incomplete entries, Stage I tumours and distant metastases were excluded from analysis. Univariate and Cox regression models were used with 5-year mortality as the primary endpoint. Results: 4309 patients were identified. Younger age, female gender, Pacific Island descent and right-sided tumours were associated with significantly higher lymph node retrieval. Cox regression analysis showed that the number of nodes examined was a significant predictor of 5-year mortality after age, gender, ethnicity, and tumour site were controlled for. Five-year survival consistently improved until the 16-node mark, above which survival advantage was minimal in both the Stage II and Stage III subsets. For Stage III cancers, a higher positive-to-all node ratio was associated with a significant increase in mortality. Conclusion: Increased rates of nodal examination are associated with a significantly lower 5-year mortality for Stage II and III colonic cancer, but this survival advantage appears to be minimal after the 16-node mark. The positive-to-all node ratio has been validated as a powerful predictor of survival in Stage III cancers. Our results support the current practice of harvesting and examining as many nodes as possible during attempted curative resection.

Purpose: There is an ethnic variation in outcomes for colonic cancer in New Zealand. Whether this disparity is due to cancer biology or inequitable provision of treatment services after diagnosis has not been elucidated. Methodology: National cancer registry data from 1996 to 2003 were obtained. Incidence and mortality rates for the four major ethnic groups were age-adjusted to the new WHO world population. The impact of age, sex, AJCC stage, and site of cancer at diagnosis was compared between ethnic groups using a Cox regression analysis. Results: 11 987 colonic cancer registrations were identified. The overall raw five year mortality was 53.7%. The age-adjusted incidence in Europeans was more than double that of the Maori, Asian, and Pacific populations at 33.0 per 100 000 population/year. Europeans presented older, with more right sided cancers, and at an earlier stage of disease. The opposite was true for Maori. Pacific Islanders and Asians presented younger, but with a similar site, stage, and sex distribution to the rest of the population. There were no significant differences in 5 year mortality after diagnosis when age, sex, stage, and site at presentation were controlled for by Cox regression analysis. Conclusion: These results suggest that age, sex, stage, and site at presentation may be more important than inequality in treatment provision after diagnosis in explaining differences in outcomes between the ethnicities. Efforts need to be focused on identifying reasons for the increased risk of colonic neoplasia in Europeans and the later stage disease presentation in Maori.

Acetylcholinesterase (AChE) activity was measured in the presence of the specific inhibitor of pseudocholinesterase, iso-OMPA, in plasma from patients with amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA), neuromuscular disease controls, and normal controls. Both AChE and Na-K ATPase activities were measured in erythrocyte ghost membranes from ALS and normal controls. Activities of erythrocyte ghost AChE and Na-K ATPase did not differ between ALS and control patients, suggesting that erythrocyte membranes were normal in ALS. However, the activity of plasma AChE in patients with ALS and PMA was increased significantly over plasma activity in disease controls and normal controls. In addition, in an animal model of human PMA, the Wobbler mouse, plasma AChE activity was increased significantly over littermate controls. The explanation for the increase in plasma acetylcholinesterase was not clear; however, a number of potentially useful clinical points followed from this study. First, there was no relationship between a specific subtype of motor neuron disease and the level of AChE activity. Second, AChE activity appeared to vary directly with the duration of PMA but not with the severity of PMA. This did not correlate with either the duration or severity of ALS. Last, plasma AChE activity was normal in about 30% of patients who had motor neuron disease; therefore, AChE assay had limited use in the diagnosis of ALS or PMA.

Divergent selection has created rat phenotypes of high- and low-capacity runners (HCR and LCR, respectively) that have differences in aerobic capacity and correlated traits such as adiposity. We analyzed visceral adipose tissue of HCR and LCR using label-free high-definition MS (elevated energy) profiling. The running capacity of HCR was ninefold greater than LCR. Proteome profiling encompassed 448 proteins and detected 30 significant (p