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The use of two different Health Assessment Questionnaires in Turkish rheumatoid arthritis population and assessment of the associations with disability.

The aim of this study was to compare and evaluate the Health Assessment Questionnaire (HAQ) and Arthritis Impact Measurement Scale (AIMS) in our patient population with rheumatoid arthritis (RA) and also to find some associations with clinical assessment of disability. One hundred and twenty-three consecutive adult patients with RA were included in the study. Pain, and global assessments by patients and physicians were recorded using a 10 cm visual analogue scale. Each patient completed the HAQ and AIMS questionnaires. Correlations among tender and swollen joint counts, erythrocyte sedimentation rate, pain, and AIMS anxiety and depression scores were all investigated. Pearson correlation was used to assess the possible correlations between each questionnaire and clinical variables. Pain and the AIMS subscales of mobility, dexterity, social activity and activities of daily living correlated with global assessments by patients and physicians, and tender joint counts. Depression correlated with pain and disability (HAQ). It was also of note that we observed high intercorrelation between the global assessments of physicians and patients. It was concluded that a measure of functional status, patient global assessment and pain score should be considered as important in the evaluation of RA patients. Measuring psychological well-being also provides further information. The HAQ, with the addition of the anxiety and depression sections of AIMS (CLINHAQ), provides the advantage of a global evaluation of these chronically ill patients.

Activities of Daily Living↗

Segmental myoclonus in a patient affected by syringomyelia.

We describe a patient who has been complaining of brief jerk-like, rhythmic, involuntary movements involving the second digit of the left hand for the last three months. These involuntary jerks produced an adduction movement of the second digit and were unaffected by peripheral sensory stimuli. In addition, the patient experienced loss of dexterity in the left hand. On examination the patient showed hypotrophy of the first dorsal interosseous (FDI) muscle of the left hand and a dissociated sensory loss involving the C8-T1 dermatomes. Magnetic resonance imaging of the brain and spinal cord revealed a tonsilar hemiation characteristic of the Chiari I malformation associated with a syrinx extending from C4 to D5 that did not communicate with the fourth ventricle. The electrophysiological evaluation indicated the presence of a focal myoclonus of spinal origin that is likely to be caused by the syrinx.

Arnold-Chiari Malformation↗

Clinical trials of surgical treatment of malignant diseases.

The Dutch Gastric Cancer Study Group Trial was the first clinical phase III trial to be carried out in the field of cancer surgery. In spite of the excellent quality of the trial, it was heavily criticized for the poor quality of the treatment itself. Actually, the hospital mortality after the new surgical treatment (D2 lymph node dissection for gastric cancer) was unacceptably high. In surgical trials, special attention should be paid to quality issues specific to surgery. The first and the most important issue is the quality of treatment given. Reproducibility, homogeneity, and verifiability are the greatest problems in surgical trials. There are also some patient factors. If the patient is old, or fragile, or obese, the results of the surgical treatment can easily be affected by these factors. The surgeon can also be a prognostic factor, especially in complicated procedures or those requiring experience and training. Experience, including postoperative care, and dexterity affect the results. If surgeons do not know how to manage complications, mortality becomes very high. Because blinding is impossible in surgical trials, the treatment may easily be affected by personal preference or prejudice. To minimize the influence of these hampering factors, the procedures should be defined in as detailed a way as possible. If pretrial training or a feasibility study (phase II) is needed, it should be carried out properly for the patients' sake. An excellent design and excellent statistical analysis cannot lead to meaningful results if the quality of treatment is poor. Nonsense in, nonsense out.

Clinical Trials, Phase III as Topic↗

Upper thoracic sympathetic surgery. Open surgical techniques.

Four open surgical approaches have been used to perform upper thoracic sympathectomy. The posterior approach requires access through the posterior muscles of the back, and rib transection. It is a painful operation that has been practically abandoned in favor of the other techniques. The anterior transthoracic approach consists of a formal thoracotomy and never gained popularity. The supraclavicular approach involves dissection of several important anatomical structures. It requires excellent surgical dexterity, but ensures the easiest postoperative recovery. The last approach involves a small transaxillary thoracotomy. Technically, it is the easier procedure. Both the supraclavicular and the transaxillary approaches were widely used until the advent of thoracoscopic surgery. The results (rate of success, recurrences, and sequelae) were similar for all techniques, depending on the procedure performed on the sympathetic chain, not on the access route. Open approaches for upper dorsal sympathectomy are not used any more except in the very rare cases in which thoracoscopy is unfeasible.

Humans↗

Muscle dimensions in the chimpanzee hand.

We dissected the forearms and hands of a female chimpanzee and systematically recorded mass, fiber length, and physiological cross-sectional area (PCSA) of all muscles including those of intrinsic muscles that have not been reported previously. The consistency of our measurements was confirmed by comparison with the published data on chimpanzees. Comparisons of the hand musculature of the measured chimpanzee with corresponding published human data indicated that the chimpanzee has relatively larger forearm flexors but smaller thenar eminence muscles, as observed in previous studies. The interosseous muscles were also confirmed to be relatively larger in the chimpanzee. However, a new finding was that relative PCSA, which reflects a muscle's capacity to generate force, might have increased slightly in humans as a result of relatively shorter muscle fiber length. This suggests that the human intrinsic muscle architecture is relatively more adapted to dexterous manipulative functions. Shortening of the metacarpals and the intervening interosseous muscles might accordingly be a prerequisite for the evolution of human precision-grip capabilities.

Animals↗

Computer-assisted laparoscopic colon resection with the Da Vinci system: our first experiences.

PURPOSE: Telerobotic surgery is a developing and promising modality that highly improves the laparoscopic dexterity. We have performed more than 100 laparoscopic and thoracoscopic procedures since December 2002 with the aid of the Da Vinci robotic system. This study was designed to assess the value of robots in colonic laparoscopic surgery. We present our first cases of robotic-assisted colectomies. METHODS: Two patients underwent a telerobotic-assisted sigmoidectomy for sigmadiverticulitis. One of these cases was complicated with a sigmoid-bladder fistula. Three other patients were submitted to a colon resection for cancer: sigmoidectomy (n = 2), and right colectomy (n = 1). A four-trocar technique was used for all operations. Tissue dissection of colonic adhesions, mobilization of the colon, management of the fistula, mesenteric dissection and division, and bowel resection were fully performed with the telerobotic system. RESULTS: Three operations were completed using the Da Vinci system without any problems in acceptable times. In two patients, the operation had to be converted to laparotomy because of severe adhesions and locally extended tumor growth. Postoperative courses of all patients were uneventful. Patients were discharged between postoperative Days 9 and 20, and were well six months later. CONCLUSIONS: Colonic telerobotic surgery can be performed safely. Benefits were seen during dissection of the rectum in the small pelvis. A major limitation is a lack of a large operation field especially if there is the need to dissect a colonic flexure in the upper abdomen. The enormous costs and the lack of appropriate instruments can be a major problem in the further expansion of the telerobotic surgery.

Aged↗

Marrow stromal cells from patients affected by MPS I differentially support haematopoietic progenitor cell development.

Bone marrow transplantation is the therapy of choice in patients affected by MPS I (Hurler syndrome), but a high incidence of rejection limits the success of this treatment. The deficiency of alpha-L-iduronidase (EC 1.2.3.76), one of the enzymes responsible for the degradation of glycosaminoglycans, results in accumulation of heparan and dermatan sulphate in these patients. Heparan sulphate and dermatan sulphate are known to be important components of the bone marrow microenvironment and critical for haematopoietic cell development. In this study we compared the ability of marrow stromal cells from MPS I patients and healthy donors to support normal haematopoiesis in Dexter-type long term culture. We found an inverse stroma/supernatant ratio in the number of clonogenic progenitors, particularly the colony-forming unit granulocyte-machrophage in MPS I cultures when compared to normal controls. No alteration in the adhesion of haematopoietic cells to the stroma of MPS I patients was found, suggesting that the altered distribution in the number of clonogenic progenitors is probably the result of an accelerated process of differentiation and maturation. The use of alpha-L-iduronidase gene-corrected marrow stromal cells re-established normal haematopoiesis in culture, suggesting that correction of the bone marrow microenvironment with competent enzyme prior to transplantation might help establishment of donor haematopoiesis.

Adolescent↗

Measuring patient quality of life: is the health utility index useful?

The purpose of this paper is to report Health Utility Index Mark 3 (HUI3) scores, at baseline and at one year, for patients with psychotic disorders. Eighty two randomly selected outpatients from a Montreal teaching hospital completed the HUI3 and other measures of symptoms and side-effects. At baseline, the average Global Utility score was 0.64, which is rated in the "dysfunctional" health status range. Improvements were seen at one year follow-up in the Global, Dexterity, Cognition, and Pain Utility scores. The proportion of individuals rated in the "healthy" health status range improved by 32% from baseline to one year. HUI3 scores were negatively related to measures of psychotic symptoms and side-effects. We propose that the HUI3 should be used to assess health-related quality of life (HRQOL) in patients with psychotic disorders. Scores could be compared with other populations affected with chronic conditions (e.g., Alzheimer dementia, cancer, arthritis, etc.).

Adult↗

Development, reliability and validity of a new measure of overall health for pre-school children.

BACKGROUND: Few comprehensive systems are available for assessing and reporting the overall health of preschool children. OBJECTIVES: (i) To develop a multi-dimension health status classification system (HSCS) to describe pre-school (PS) children 2.5-5 years of age; (ii) to report reliability and validity of the newly developed measure. DESIGN: Existing systems (Health Utilities Index, Mark 2 and 3) were adapted for application to a pre-school population. The new system was tested for acceptability, validity and reliability. PARTICIPANTS: Three cohorts of children and their parents from Canada and Australia were utilized: Cohort 1 (MAC)-101 3-years old very low birthweight (VLBW, <1500 g) and 50 same age term children from Canada; Cohort 2 (AUS)-150 VLBW 3-years old from Australia; Cohort 3 (OMG)-222 3-years old with cerebral palsy (CP) from Ontario. METHODS: Parental intra-rater reliability was evaluated by completion of the HSCS-PS Parent questionnaire (MAC) at the clinic visit and again 14 days later. Health professionals (MAC) completed the HSCS-PS Clinician questionnaire. Percent agreement and Kappa values were used to assess parent-clinician agreement. Concurrent validity was tested in two populations of VLBW children (MAC and AUS) and a reference group of term children (MAC) by exploring the relationships between dimensions of the HSCS-PS and well-recognized norm-referenced measures: the Bayley Scales of Infant Development (BSID-II), the Vineland Adaptive Behavior Scales (VABS) and the Stanford-Binet (SB). Construct validity was tested by comparing ratings on both the HSCS-PS and the Gross Motor Function classification system (GMFCS) using a population of pre-school children with CP. Analyses were done using chi2, ANOVA and correlations with tau-b statistic. RESULTS: The HSCS-PS has 12 dimensions and 3-5 levels per dimension. Response rate for parental intra-rater reliability was 95%, with percent agreement ranging between 86 and 100%. Kappa values for various dimensions ranged from 0.38 to 1.00. Inter-rater reliability between parents and clinicians showed agreement ranging from 72 to 100%. Kappa values ranged from 0.30 to 1.00. CONCURRENT VALIDITY: There was a statistically significant gradient between HSCS-PS Mobility levels and motor scale scores of the BSID-II and VABS. A significant gradient also occurred when comparing HSCS-PS cognition levels to psychometric scores on the BSID-II and SB, as well as HSCS-PS self-care levels compared to VABS Daily Living scores. DISCRIMINATIVE AND CONSTRUCT VALIDITY: Birthweight category was shown to be a significant determinant of proportion of children with multiple HSCS-PS dimensions affected. In addition, HSCS-PS dimension levels were congruent with GMFCS levels where expected: mobility had excellent correlation; self-care, dexterity, speech and cognitive dimensions had moderate correlations. CONCLUSIONS: The HSCS-PS is readily accepted, quick to complete, widely applicable and provides a multi-dimensional description of health status. Preliminary assessments of reliability and validity are promising. The HSCS-PS can discriminate across populations by birthweight and shows strong relationships with standardized psychometric measures in comparable domains. It can pro- vide a summary profile of functional limitations in various populations of pre-school children in a consistent manner across programs and in different settings.

Australia↗

Inter-observer agreement of a comprehensive health status classification system for pre-school children among patients with Wilms' tumor or advanced neuroblastoma.

We assessed inter-observer agreement on a new comprehensive health status classification system for preschool children (CHSCS-PS). Prospective assessments of children aged 2-4.9 years at the time of diagnosis of neuroblastoma (stages 3-4, excluding 4S) or Wilms' tumor (stages II-V) were collected independently from a parent and nurse by self-report during therapy. Responses were used to determine functional status on 10 health domains, as well as an overall disability score. Inter-observer agreement was evaluated by a kappa statistic for agreement about levels within individual domains, and by an intraclass correlation coefficient (ICC) for agreement of overall disability scores. Twenty-four parent/nurse pairs of assessments were collected. Agreement was almost perfect for mobility and self-care, substantial for emotion and pain, and slight for speech. There was high percent agreement for vision, hearing, dexterity, learning and remembering, and thinking and problem solving, but insufficient variability in responses to calculate a kappa statistic. The ICC for overall disability scores between observers was 0.86, indicating strong agreement. Given the need for, and paucity of, instruments for the measurement of health-related quality of life in very young children, these results strongly support further evaluation of the CHSCS-PS.

Child, Preschool↗

Health status in survivors of cancer in childhood and adolescence.

BACKGROUND: Assessing health status in survivors of childhood cancer is increasingly important due to improved survival rates. However, there are limited estimates available for this population based on large samples and compared to population controls. METHODS: In a retrospective cohort study, 2,152 long-term survivors and 2,432 controls, aged 5-37, who had survived cancer during childhood or adolescence were compared on the Health Utilities Index Mark III (HUI3). Descriptive and logistic regression analyses were used to assess the effect of age at diagnosis, type of cancer and therapy received on HUI3 domains. MAJOR FINDINGS: More survivors than controls showed deficits in dexterity, ambulation, hearing, speech and cognition but not in vision, emotion or pain. The largest numbers of survivors reporting excess impairment was found in the cognition attribute. Survivors of central nervous system tumors were most likely to show impairments across multiple domains. Lastly, impairments in cognition were found most commonly in survivors exposed to cranio-spinal radiation at young ages. CONCLUSIONS: Seventy-five percent of childhood cancer survivors and 80% of controls were found to have two or fewer impaired attributes. Those reporting impairments that were most likely to be of clinical relevance were among survivors diagnosed with central nervous system and bone tumours, and those exposed to cranial radiation as young children. Tools assessing health status should be included in prospective trials to more clearly assess the contribution of therapy to reduced long-term health status.

Adolescent↗

Learning curve using robotic surgery.

The da Vinci (Intuitive Surgical, Inc., Sunnyvale, CA) surgical system is being used by an increasing number of surgeons across several surgical specialties. The robotic interface is different not only to open surgery, but also to laparoscopy because it involves remote surgical control, stereoscopic vision, and lack of haptic feedback. As the transition is made from traditional open to robotic surgery, factors such as learning of robotic skills, assessment of proficiency in robotics, and structured training for urologists in practice and residents assumes importance. Understanding how the robotic surgical technique is learned and how such learning can be best assessed will enable us to define protocols for training and set standards for proficiency. Learning curve and surgical dexterity are two parameters that are used to compare surgical learning and training. This article presents the current gold standard for assessing skill training and compares surgical skill acquisition and proficiency using conventional laparoscopy and robotic interfaces.

Clinical Competence↗

Robotic Laparoscopic Fundoplication.

Gastroesophageal reflux disease is a very common disorder, and both medical and surgical treatments have shown outstanding results. Whereas proton pump inhibitors are the mainstay of treatment, laparoscopic fundoplication has become a very attractive alternative due to its efficacy and low morbidity. There are defined patient categories that may benefit more from laparoscopy than medical therapy, but a conclusive comparison between the two is lacking. Robotic laparoscopic fundoplication can be performed safely without increased morbidity. Potential advantages include enhanced precision, improved dexterity, and remote telesurgical applications. Disadvantages include increased cost and prolonged operative times. Further studies and more long-term outcome data are needed to fully evaluate the procedure. Robotic surgery is currently in its infancy and not cost effective but has a very promising future. With further development of automatization and miniaturization features, robotic surgery may prove more efficient than conventional laparoscopy.

Journal Article↗

Complex Regional Pain Syndrome.

Complex regional pain syndrome (CRPS) is a heterogeneous disorder that falls in the spectrum of neuropathic pain disorders. It is maintained by abnormalities throughout the neuraxis (the peripheral, autonomic, and central nervous systems). The pathophysiology of CRPS is not fully known. There are no scientifically well-established treatments. The diagnostic criteria for CRPS at this time are purely clinical, and the use of diagnostic tests has not been demonstrated. The most appropriate management of CRPS uses a multidisciplinary approach, with the inclusion of medical and psychologic intervention, and physical and occupational therapy. The key is gradual, persistent, functional improvement. The rational use of pain therapies must be grounded in a thorough knowledge of the neurobiology of pain, its endogenous modulation, and the clinical presentation. Potential peripheral pathophysiologic targets (and possible treatments) include increased spontaneous firing and responsiveness of peripheral afferent fibers mediated by inflammatory and other algogenic substances (somatosensory blocks, corticosteroids), altered levels of expression and functioning of multiple ion channels (local anesthetics, calcium channel blockers, anticonvulsants), abnormal interneuronal communication, and increased peripheral expression of adrenergic receptors and sympathetic excitation (sympathetic blocks, alpha-adrenergic antagonists, alpha-2 agonists). CRPS is also perpetuated by central mechanisms, with pathophysiologic targets (and possible treatments) including reorientation of dorsal horn terminals (desensitization techniques), functional reduction in inhibitory interneuron activity (tricyclic antidepressants, gabapentin, opioids), central sensitization and increased central excitability (gabapentin, topiramate, spinal cord stimulation, somatosensory blocks), impaired descending nociceptive inhibition (tricyclic antidepressants, opioids), and adaptive changes in the cortical centers underlying the sensory-discriminative and affective-motivational dimensions of pain (psychologic, physical, and occupational therapies). The treatment choices should be aimed at remodulating, normalizing, disrupting, or preventing the progression of abnormalities in pain processing. Sympathetic nerve blocks should be performed at least once to assess if sympathetically maintained pain is present. To the extent that peripheral somatosensory nerve blocks can diminish nociceptive input to the central nervous system, these techniques may help reduce the nociceptive sensitization of spinal neurons. Pain relief, however it is achieved and however temporary it is, is intended to facilitate participation in functional therapies to normalize use and to improve motion, strength, and dexterity. Psychologic therapies, such as biofeedback and cognitive-behavioral techniques targeting pain, stress, and mood disorders, are valuable adjunctive treatments for pain control and can facilitate functional improvement.

Journal Article↗

Starr-Edwards aortic prosthesis: a 20-year retrospective study.

Over the years the Starr Edwards prosthesis has demonstrated a high durability and improved survival in patients with severe aortic stenosis or insufficiency. While the ideal valve prosthesis is not yet available, the Starr-Edwards valve in most instances demonstrates an adequate hemodynamic performance, does not degenerate throughout the human life span, is biocompatible, and is inserted reliably without requiring unique technical dexterity. Despite occasional valve-related complications with early models, beneficial effects of valve replacement are clearly seen in patients with a reversible myocardial dysfunction, a lower level New York Heart Association functional class (II to III), and who survive the early postoperative period and the first year. Long-term complications seen with the Starr-Edwards valve are primarily thromboembolism, endocarditis, hemolysis, and anticoagulation related complications. Despite good operative results, the major cause of deaths in these patients remains cardiac.

Adult↗

Effect of sleep deprivation on the performance of surgical residents.

Surgical residents were tested under sleep-deprived and nonsleep-deprived conditions. Three performance domains were tested: factual recall, the ability to concentrate, and manual dexterity. Sleep deprivation was defined as less than 3 hours of sleep in a 24 hour period. A randomized, repeated measures design was used. Split-plot analysis of variances was used to analyze the mean scores, and it revealed that there were no significant differences in performance in the sleep-deprived condition compared with performance in the nonsleep-deprived condition in any of the three domains tested. Moderate sleep deprivation appeared to be well tolerated by the surgical residents studied in this investigation.

Adult↗

Surgical handicraft: teaching and learning surgical skills.

Surgeons choose their profession with a strong desire to excel at manual therapeutic skills. Although we mime our mentors, we have often received the torch of technique in the absence of a systematic program to optimally develop our manual dexterity. The operating room is the ultimate arena to refine one's technical ability, but a surgical skills laboratory should assume increasing importance in introducing the trainee to the many nuances of the fine manual motor skills necessary for optimal surgical technique. Surgical educators should address the science of surgical handicraft in a manner similar to the science of preoperative and postoperative surgical principles that have been espoused over the past 40 years. Although it has been euphemistically said that "you can teach a monkey to operate," few of us have broken the process down into the basic elements to accomplish such a goal. In view of the increasing complexity of operations and equipment, the constraints on animal laboratories and teaching caseloads, and the mounting economic and medico-legal pressures, the development of optimal surgical skills should be a major objective of every surgical training program. By developing novel programs and scientifically evaluating the results of such endeavors, surgical faculties may find increased academic rewards for being a good teacher.

Functional Laterality↗

Coupled-enzyme and direct assays for uroporphyrinogen III synthase activity in human erythrocytes and cultured lymphoblasts. Enzymatic diagnosis of heterozygotes and homozygotes with congenital erythropoietic porphyria.

Rapid and reproducible assays for uroporphyrinogen III synthase (URO-S; EC 4.2.1.75) have been developed and used to determine the enzymatic activity in human erythrocytes and cultured lymphoid cells. In the coupled-enzyme assay, porphobilinogen was first converted to hydroxymethylbilane, the natural substrate for URO-S, by hydroxymethylbilane synthase which was conveniently obtained from heat-treated erythrocyte lysates. In the direct assay, synthetic hydroxymethylbilane was used as substrate. In both assays, the uroporphyrinogen reaction products were oxidized to their respective uroporphyrin isomers, which were then resolved and quantitated by reversed-phase high-pressure liquid chromatography. Both assays were optimized for pH, substrate concentration, and linearity with time and protein concentration. The mean URO-S activities in normal human erythrocyte lysates determined by the coupled-enzyme and direct assays were 7.41 +/- 1.35 and 7.64 +/- 1.73 units/mg protein, respectively. In normal human cultured lymphoid cells, the mean activities were 13.7 +/- 1.39 and 17.6 +/- 1.15 units/mg protein for the coupled-enzyme and direct assays, respectively. In four families with congenital erythropoietic porphyria, both assays reliably identified the markedly decreased URO-S activities in erythrocytes and cultured lymphoid cells from affected homozygotes and the half-normal activities in these sources from obligate heterozygotes. The coupled-enzyme assay was easier to perform and was suited for clinical diagnostic assays and for monitoring enzyme purification procedures, while the direct assay, which required substrate preparation and technical dexterity, was best for kinetic studies of URO-S.

Cells, Cultured↗