Currarino triad--diagnostic dilemma and a combined surgical approach.

PURPOSE: The authors present 2 families with 3 cases of Currarino triad, diagnostic difficulties, their familial occurrence, and genetic mapping, with emphasis on a combined pediatric surgical and pediatric neurosurgical approach in managing these children. RESULTS: The main presentation was intractable constipation. In the first family there was a 4-generation pedigree with recurrence of Currarino triad. The mother and the child have the condition. Family 2 screening showed a 3-generation pedigree with presence of Currarino triad in 3 members. Patients 2 and 3 are cousins whose fathers are affected by spina bifida occulta and Currarino triad, respectively. In patient 1, the diagnosis was made after inadvertent rupture of an anterior meningocele during posterior myectomy. In patient 2, the presacral mass was found on examination under anesthesia, and the planned anorectal myectomy for intractable constipation was abandoned. Patient 3 was a cousin of patient 2, and the diagnosis was considered when she presented with intractable constipation at the age of 7 months. Magnetic resonance scan was useful in showing the presence of presacral mass, spinal abnormalities, and tethered cord. A combined pediatric and neurosurgical approach optimized the extirpation of the presacral mass with minimal complications. Surgical treatment was individualized according to the estimation of the operative risk factors. All patients have a normal bladder function. Patient 1 has required laxatives and enemas for intermittent constipation. She has associated learning difficulties but is otherwise well. Patient 2 and 3, aged 10 and 2 years, respectively, are awaiting closure of colostomy. They are thriving and well. CONCLUSIONS: The authors recommend a combined pediatric and neurosurgical assessment and management for all cases of Currarino triad. Family screening is obligatory. The authors suggest the use of a magnetic resonance scan or computerized axial tomography myelogram to define the presence of anosacral and spinal cord anomalies in patients with intractable constipation.

Idiopathic constipation in childhood is associated with thickening of the internal anal sphincter.

This study was undertaken to search for a rational basis for the use of anal dilatation and internal sphincterotomy as the treatment for chronic intractable constipation in children. Sixteen children, age 5 months to 13 years, who had constipation resistant to conservative treatment were compared with 39 age-matched controls. History and current symptoms were assessed using a standard questionnaire. Internal and external and sphincter morphology was assessed on clinical examination and by anal endosonography, using a 10-MHz rotating endoprobe to provide accurate measurement of the various components of the anal canal. The control group showed a linear correlation between the thickness of the internal anal sphincter and both age and weight, increasing from 0.4 mm in infancy to 0.9 mm in adolescence. Children who had constipation displayed significant thickening of the internal sphincter (range, 0.5 to 1.9 mm, P = .005) which was independent of the length of the history (P = .103). There was no difference in the morphology of the external anal sphincters between the groups. The finding of a hypertrophied internal anal sphincter could provide a rational basis for anal dilatation and internal sphincterotomy as treatment for idiopathic constipation.

Long-term effects of gastric transposition in children: a physiological study.

Gastric transposition (GT) has become a reliable alternative operation for oesophageal replacement in children. The aims of this study were to assess the long-term results of the operation and to study the function of the intrathoracic stomach. Current symptoms were assessed using a questionnaire and linear analogue scales. Lung function was measured using spirometry and plethysmography, and the results were corrected for height and expressed as a percentage of the predicted values for normal children. Gastric emptying was assessed using a dual isotope radiolabelled test meal (incorporating solid and liquid phases). Full anthropometric and haematologic data also were collected. The results are expressed as medians and interquartile ranges. Seventeen children were examined at least 5 years after GT; the median age was 9 years. Two children frequently had symptoms during swallowing. Four children had significant diarrhoeal episodes, and two had significant postprandial weakness or dizziness. Unexplained breathlessness was noted by four children. All but one child had lung function values that were lower than the mean predicted value for height. For example, the total lung capacity was 68%, and forced vital capacity (FVC) was 64%. However, the ratio of forced expiratory volume in 1 second (FEV1) to FVC was normal. The gastric emptying study showed that the intrathoracic stomach in all subjects served as a conduit (rather than a reservoir) for both liquids and solids. Rapid emptying (> 50%) in both phases occurred within 5 minutes of ingestion in 82% of the group. Thirteen children were between the 3rd and 97th percentiles for height, and 11 in this range for weight. Five children were anaemic (< 11.5 g/dL). In 11 of the tested samples, the serum ferritin was low, indicating depleted iron stores. GT is compatible with an entirely normal life and has allowed satisfactory growth and nutrition for the majority of subjects in this study group.

Pragmatic randomised controlled trial of local corticosteroid injection and naproxen for treatment of lateral epicondylitis of elbow in primary care.

OBJECTIVE: To compare the clinical effectiveness of local corticosteroid injection, standard non-steroidal anti-inflammatory drugs, and simple analgesics for the early treatment of lateral epicondylitis in primary care. DESIGN: Multicentre pragmatic randomised controlled trial. SETTING: 23 general practices in North Staffordshire and South Cheshire. PARTICIPANTS: 164 patients aged 18-70 years presenting with a new episode of lateral epicondylitis. INTERVENTIONS: Local injection of 20 mg methylprednisolone plus lignocaine, naproxen 500 mg twice daily for two weeks, or placebo tablets. All participants received a standard advice sheet and co-codamol as required. MAIN OUTCOME MEASURES: Participants' global assessment of improvement (five point scale) at four weeks. Pain, function, and "main complaint" measured on 10 point Likert scales at 4 weeks, 6 months, and 12 months. RESULTS: Over 2 years, 53 subjects were randomised to injection, 53 to naproxen, and 58 to placebo. Prognostic variables were similar between groups at baseline. At 4 weeks, 48 patients (92%) in the injection group were completely better or improved compared with 30 (57%) in the naproxen group (P<0.001) and 28 (50%) in the placebo group (P<0.001). At 12 months, 43 patients (84%) in the injection group had pain scores 0.05). CONCLUSIONS: Early local corticosteroid injection is effective for lateral epicondylitis. Outcome at one year was good in all groups, and effective early treatment does not seem to influence this.

Evidence-based recommendations for the role of exercise in the management of osteoarthritis of the hip or knee--the MOVE consensus.

OBJECTIVES: Exercise is an effective and commonly prescribed intervention for lower limb osteoarthritis (OA). Many unanswered questions remain, however, concerning the practical delivery of exercise therapy. We have produced evidence-based recommendations to guide health-care practitioners. METHODS: A multidisciplinary guideline development group was formed from representatives of professional bodies to which OA is of relevance and other interested parties. Each participant contributed up to 10 propositions describing key clinical points regarding exercise therapy for OA of the hip or knee. Ten final recommendations were agreed by the Delphi technique. The research evidence for each was determined. A literature search was undertaken in the Medline, PubMed, EMBASE, PEDro, CINAHL and Cochrane databases. The methodological quality of each retrieved publication was assessed. Outcome data were abstracted and effect sizes calculated. The evidence for each recommendation was assessed and expert consensus highlighted by the allocation of two categories: (1) strength of evidence and (2) strength of recommendation. RESULTS: The first round of the Delphi process produced 123 propositions. This was reduced to 10 after four rounds. These related to aerobic and strengthening exercise, group versus home exercise, adherence, contraindications and predictors of response. The literature search identified 910 articles; 57 intervention trials relating to knee OA, 9 to hip OA and 73 to adherence. The evidence to support each proposition is presented. CONCLUSION: These are the first recommendations for exercise in hip and knee OA to clearly differentiate research evidence and expert opinion. Gaps in the literature are identified and issues requiring further study highlighted.

The topical NSAID felbinac versus oral NSAIDS: a critical review.

Musculoskeletal disorders such as soft tissue injuries have traditionally been treated with oral NSAIDs, despite the significant side-effects associated with their clinical use. However, four separate multicentre, double-blind, double-dummy clinical trials have shown that the efficacy of the topical NSAID, felbinac, is equivalent to that of the oral NSAID, ibuprofen, in the treatment of soft tissue injuries, and to that of oral ibuprofen or fenbufen in mild to moderate osteoarthritis. In general practice the incidence of side-effects with felbinac is low, while oral NSAIDs have been associated with significant problems, particularly in the gastrointestinal system. Consequently, the cost of treating side-effects is reduced with felbinac treatment compared with oral NSAIDs, making it a logical treatment alternative from an economic view point as well as for reasons of efficacy and safety.

Prospective evaluation of nonsurgical versus surgical management of appendiceal mass.

PURPOSE: Prospective evaluation was undertaken of surgical findings, complications, morbidity, and hospital stay between initial nonsurgical management versus early surgical intervention of an appendicular mass. METHODS: A prospective, nonrandomized study was conducted of 82 consecutive patients (mean age, 6.9 +/- 3.3 years) presenting with an appendicular mass over a 5-year period. They were categorized as group 1, 58.5% (48 of 82) nonsurgically managed and an interval appendectomy performed at a mean period of 8.6 +/- 4.6 weeks and group 2, 41.5% (34 of 82) appendectomy at presentation. RESULTS: An appendix was identified in all 82 patients in both groups at operation. In-group 1, recurrent episodes of abdominal pain necessitated interval appendectomy in 39.6% (19 of 48) patients at a mean 4.3 +/- 0.8 versus 11.5 +/- 3.7 weeks in 60.4% (29 of 48) who underwent scheduled interval appendectomy. Periappendiceal abscesses present at interval appendectomy in group 1 was (38 of 48) 79.2% versus 100% (34 of 34) at appendectomy in group 2. Adhesions at interval appendectomy in group 1 was 81.3% (39 of 48) versus 100% (34 of 34) at appendectomy in group 2. In-group 1, superficial wound infection was observed in 0 versus 4 wound infections in group 2. Overall morbidity rate between group 1 and group 2 was statistically significant (P <.05). Total mean hospital stay in group 1 was 13.2 +/- 1.5 versus 4.8 +/- 0.4 days in group 2. Of the 48-interval appendectomy specimens, 37 of 48 (77%) appendices had a patent lumen, and 11 of 48 (23%) showed fibrosis and obliteration of appendicular lumen. There was no correlation (r = 0.22) between the histopathologic findings and the interval between abscess treatment and interval appendectomy. CONCLUSIONS: Early surgical intervention was beneficial over nonoperative management in this cohort of patients. Interval appendectomy is recommended after nonsurgical management of an appendicular mass.

The pharmacokinetics of single and multiple doses of tiaprofenic acid in elderly patients with arthritis.

We have studied the single dose and steady-state pharmacokinetic of tiaprofenic acid in ten elderly arthritic patients living in the community (5 men and 5 women) taking 200 mg tid for 8 days. The mean area under the plasma concentration-time curves to 8 h (AUC (0-8)) did not alter significantly from day 1 to day 8 (77.25 to 79.61 micrograms X ml-1 X h). The mean terminal phase half-life (t1/2) was 2.05 h and 2.25 h on Days 1 and 8 respectively in patients in whom the calculations were possible (7 patients on Day 1 and 6 patients on Day 8). The median observed time of maximum concentration (tmax) and the mean observed maximum plasma concentration (Cmax) of 100 min and 21.3 micrograms X ml-1 respectively on Day 1 were not significantly different from the values obtained on Day 8 (tmax 120 min; Cmax 20.7 micrograms X ml-1). The kinetic data suggest that there should be no significant accumulation of tiaprofenic acid in elderly ambulant people suffering from arthritis.

The pharmacokinetics of sustained release tiaprofenic acid in elderly arthritic patients.

Tiaprofenic acid is a non-steroidal anti-inflammatory drug which is highly protein bound and excreted mainly by the kidneys. Previous pharmacokinetic studies with tiaprofenic acid in conventional formulations in elderly arthritic patients have shown no evidence of accumulation of this drug over periods of up to 12 weeks. A sustained release formulation of the drug was given to 14 elderly arthritic patients over 4 weeks and plasma profiles obtained at the beginning and end of treatment over a 24 h period. Area under the curve (AUC), maximum plasma concentration (Cmax) and plasma concentration at 24 h (C24) were calculated and elimination half life (t1/2) was estimated. AUCs to infinity at week 0 were compared with AUCs to infinity at week 4 as a measure of drug accumulation. No significant differences were found for any of these parameters between the beginning and end of treatment. The mean t1/2 for both weeks was approximately 4.4 h. There was no evidence of any accumulation.

The effects of age on the pharmacodynamics and pharmacokinetics of two formulations of verapamil.

The present study, undertaken in general practice, was designed to evaluate the effects of age on the pharmacodynamics and pharmacokinetics of a conventional and a slow-release formulation (Securon SR) of verapamil. Two groups of 12 patients with essential hypertension were treated in an open, randomized, crossover study. One group was younger than 65 years, mean 58 (range 50-64 years) and the other was 65 years and older, mean 72 (range 66-77 years). The patients were titrated through three steps with the two different formulations to efficacy or to maximal dosage. During a 4-week drug-free run-in period the mean blood pressures were 167 +/- 14/103 +/- 4, 168 +/- 18/105 +/- 6, and 168 +/- 18/105 +/- 5 mm Hg at 0, 2, and 4 weeks, respectively, for the whole group. The final blood pressure at control showed a fall with sustained-release verapamil for systolic blood pressure (SBP) and diastolic blood pressure (DBP) of 15 +/- 16 and 17 +/- 5 mm Hg for the younger group and 14 +/- 16 and 14 +/- 6 mm Hg for the older group. For the conventional formulation the falls of SBP and DBP were 14 +/- 18 and 18 +/- 8 mm Hg for the younger group and 19 +/- 16 and 13 +/- 9 mm Hg for the older group. There were no significant differences between the responses to the two formulations nor between the two age groups. The pharmacokinetics of both formulations were investigated at steady state and no significant effects of age were observed. However, there were significant differences between the time-concentration profiles for the formulations. These findings suggest that there are no significant effects of age on the pharmacodynamics and pharmacokinetics of verapamil.

Radiographic change is common in new presenters in primary care with hip pain. PCR Hip Study Group.

OBJECTIVE: To determine the prevalence of radiographic osteoarthritis in subjects with hip pain newly presenting to primary care. METHODS: The study was cross-sectional in design, set in 35 general practices across the UK. It included 195 men and women aged 40 yr and over (median 63 yr) presenting with a new episode of hip pain. Hip radiographs were scored for minimum joint space (MJS) and overall-Croft's modification of the Kellgren and Lawrence (Croft)-grade of osteoarthritis. RESULTS: In all, definite evidence of radiographic change in the painful joint was common: Croft grade > or =2 in 44%, > or =3 in 34%. MJS of 2.5 mm or less was seen in 30% of whom half were below 1.5 mm. There were no significant gender differences in radiographic severity. CONCLUSIONS: Radiographic change is common in patients newly presenting with hip pain and many already have advanced disease.

Health impact of pain in the hip region with and without radiographic evidence of osteoarthritis: a study of new attenders to primary care. The PCR Hip Study Group.

OBJECTIVES: To assess the health impact of hip pain at the time of first presentation to primary care, and the influence on this of radiographic evidence of osteoarthritis. SUBJECTS AND METHODS: Cross sectional survey of 195 patients (63 male, 132 female), aged 40 years and over, presenting with a new episode of hip pain, recruited from 35 general practices across the UK. Health status at presentation was determined by a structured questionnaire on symptoms, healthcare use, and health related quality of life (SF-36). Pelvic radiographs were assessed blindly for hip osteoarthritis using standard scoring systems. RESULTS: The overall impact on health was substantial. Before their first consultation, three quarters of patients needed analgesics, half used topical creams or ointments, and one in eight used a walking stick. Most of these impact measures were, however, unrelated to the degree of radiographic change, though use of a walking stick was increased in those with the most severe damage. Health status, as judged by the SF-36, was also impaired for measures of physical function and pain, but the impact on the "mental health", "general health", and "vitality" dimensions was small. There was a weak relation between the SF-36 scores and radiographic change, with many domains unrelated to the severity of radiographic damage. CONCLUSIONS: This study is the first to show the therapeutic impact and pattern of impairment in health status resulting from hip pain at the time of first presentation to the healthcare services. Unlike many regional pain syndromes seen in primary care, such as back pain, hip pain does not impact on wider aspects of quality of life, such as general health status, mental health, or vitality. Furthermore, any impact of hip pain in this group is not markedly influenced by the degree of structural damage. Further follow up is required to determine whether such damage influences the persistence of any adverse impact.

Syndrome of symptomatic adult acetabular dysplasia (SAAD syndrome).

OBJECTIVES: To determine the prevalence of acetabular dysplasia in subjects presenting with hip pain to primary care and its relationship with radiographic osteoarthritis (OA) of the hip. DESIGN: Cross sectional analysis of a prospective cohort. SETTING: 35 general practices across the UK. SUBJECTS: 195 patients (63 male, 132 female) aged 40 years and over presenting with a new episode of hip pain RESULTS: The prevalence of acetabular dysplasia in this study of new presenters with hip pain was high (32%). There was no significant relationship between acetabular dysplasia and radiographic OA overall. CONCLUSIONS: The high prevalence of acetabular dysplasia across all grades of OA severity suggests that dysplasia itself may be an important cause of hip pain ("symptomatic adult acetabular dysplasia").

Predicting radiographic hip osteoarthritis from range of movement.

OBJECTIVES: The primary objective was to test the hypothesis that new attenders in primary care with hip pain and radiographic osteoarthritis (OA) have a decreased range of movement compared with those without OA. The secondary objective was to define the planes of movement and thresholds that were the most discriminatory for OA. METHODS: Men and women aged 40 yr and over presenting with a new episode of hip pain were recruited from 36 general practices across the UK. A standardized radiographic and clinical examination was performed. The discriminating ability of the range of movement for each plane to identify those with radiographic OA was assessed using receiver operating characteristic curves. RESULTS: New hip pain attenders with radiographic OA had restricted movement at the hip compared with those without radiographic change. Restriction in internal rotation was the most predictive and flexion the least predictive of radiographic OA. At this cut-off, restriction in any single plane had a sensitivity of 86% for moderate and 100% for severe OA (specificity was 54 and 42% respectively). Restriction in all three planes had greater discrimination (sensitivity was 33% for mild to moderate OA and 54% for severe OA; specificity was 93 and 88% respectively). CONCLUSIONS: Restriction in range of movement was predictive of the presence of OA in these new presenters to primary care with hip pain, and the results of this examination could be used to inform decisions regarding radiography.

Ocean circulation off east Antarctica affects ecosystem structure and sea-ice extent.

Sea ice and oceanic boundaries have a dominant effect in structuring Antarctic marine ecosystems. Satellite imagery and historical data have identified the southern boundary of the Antarctic Circumpolar Current as a site of enhanced biological productivity. Meso-scale surveys off the Antarctic peninsula have related the abundances of Antarctic krill (Euphausia superba) and salps (Salpa thompsoni) to inter-annual variations in sea-ice extent. Here we have examined the ecosystem structure and oceanography spanning 3,500 km of the east Antarctic coastline, linking the scales of local surveys and global observations. Between 80 degrees and 150 degrees E there is a threefold variation in the extent of annual sea-ice cover, enabling us to examine the regional effects of sea ice and ocean circulation on biological productivity. Phytoplankton, primary productivity, Antarctic krill, whales and seabirds were concentrated where winter sea-ice extent is maximal, whereas salps were located where the sea-ice extent is minimal. We found enhanced biological activity south of the southern boundary of the Antarctic Circumpolar Current rather than in association with it. We propose that along this coastline ocean circulation determines both the sea-ice conditions and the level of biological productivity at all trophic levels.