Development of a parent-report computer-adaptive test to assess physical functioning in children with cerebral palsy II: upper-extremity skills

The specific aims of this study were to (1) examine the psychometric properties (unidimensionality, differential item functioning, scale coverage) of an item bank of upper-extremity skills for children and adolescents with cerebral palsy (CP); (2) evaluate a simulated computer-adaptive test (CAT) using this item bank; (3) examine the concurrent validity of the CAT with the Pediatric Outcomes Data Collection Instrument (PODCI) upper-extremity core scale; and (4) determine the discriminant validity of the simulated CAT with Manual Ability Classification System (MACS) levels and CP type (i.e. diplegia, hemiplegia, or quadriplegia). Parents ( n =180) of children and adolescents with CP (spastic diplegia 49%, hemiplegia 22%, or quadriplegia 28%) consisting of 102 males and 78 females with a mean age of 10 years 6 months (SD 4y 1mo, range 2–21y), and MACS levels I through V participated in calibration of an item pool and completed the PODCI. Confirmatory factor analyses supported a unidimensional model using 49 of the 53 upper-extremity items. Simulated CATs of 5, 10, and 15 items demonstrated excellent accuracy (intraclass correlation coefficient [ICCs] >0.93) with the full item bank, had high correlations with the PODCI upper-extremity core scale score (ICC 0.79), and discriminated among MACS levels. The simulated CATs demonstrated excellent overall content coverage over a wide age span and severity of upper-extremity involvement. The future development and refinement of CATs for parent report of physical function in children and adolescents with CP is supported by our work.     

An integrated strategy for the diagnosis of neuronal ceroid lipofuscinosis types 1 (CLN1) and 2 (CLN2) in eleven Latin American patients

The neuronal ceroid lipofuscinoses (NCLs) are a family of progressive neurodegenerative diseases that are characterized by the cellular accumulation of ceroid lipofuscin-like bodies. NCL type 1 (CLN1) and type 2 (CLN2) are caused by deficiencies of the lysosomal enzymes palmitoyl-protein thioesterase 1 (PPT-1) and tripeptidyl peptidase 1 (TPP-1), respectively. In this study, 118 Latin American patients were examined for NCL using an integrated multidisciplinary program. This revealed two patients affected by CLN1 and nine by CLN2. Both CLN1 patients had a juvenile-onset phenotype with mutation studies of one patient demonstrating the known mutation p.Arg151X and a novel mutation in intron 3, c.363-3T>G. Six of the CLN2 patients presented with the 'classical' late-infantile phenotype. The remaining three patients, who were siblings, presented with a 'protracted' phenotype and had a higher level of residual TPP-1 activity than the 'classical' CLN2 patients. Genotype analysis of the TPP1 gene in the 'classical' CLN2 patients showed the presence of the known mutation p.Arg208X and the novel mutations p.Leu104X, p.Asp276Val, and p.Ala453Val. The siblings with the 'protracted' phenotype were heterozygous for two known TPP1 mutations, p.Gln66X and c.887-10A>G. This multidisciplinary program is also being used to diagnose other NCL types.     

A One-Stop Carpal Tunnel Clinic

INTRODUCTION

By December 2008, 90% of referrals requiring hospital admission will need to be seen and treated within the 18-week patient pathway. Previously, patients within our trust with suspected carpal tunnel syndrome had to wait 3 months to see a specialist in clinic and, once assessed, would have to wait up to a further 6 months for an open carpal tunnel decompression under local anaesthetic (OCTD/LA). We set up a one-stop clinic, where patients would have their out-patient consultation and surgery on the same day. We evaluated the clinic in order to assess whether it led to reduced waiting times whilst maintaining good clinical outcome and patient satisfaction.

PATIENTS AND METHODS

Patients were selected on the basis of the standard referral letter alone. Those selected were then assessed by a single surgeon in the clinic. The patients deemed appropriate underwent an OCTD/LA and were discharged the same day. Patients were followed up with a patient satisfaction and Boston questionnaire.

RESULTS

Forty-six patients underwent 63 OCTD/LA, waiting an average of 2.2 months (9 weeks) from referral. There was high patient satisfaction and improvement in symptoms following treatment in the clinic.

CONCLUSIONS

We believe a one-stop carpal tunnel clinic can be an efficient and cost-effective way of treating this common condition.     

Role of previous hospitalization in clinically-significant MRSA infection among HIV-infected inpatients: results of a case-control study

Background  

HIV-infected subjects have high incidence rates of Staphylococcus aureus infections, with both methicillin-susceptible and methicillin-resistant (MRSA) strains. Possible explanations could include the high burden of colonization, the behavioral risk factors, and the frequent exposures to health care facilities of HIV-infected patients. The purpose of the study was to assess the risk factors for clinically- significant methicillin-resistant Staphylococcus aureus (CS-MRSA) infections in HIV-infected patients admitted to Infectious Diseases Units.     

Clinical and Neuropathological Variability in Clinically Isolated Central Nervous System Whipple's Disease

Central nervous system Whipple's disease (CNS‐WD) with clinically isolated neurological involvement is a rare condition fatal without an early diagnosis. We aimed to present clinical and neuropathological features of three cases of pre‐ or post‐mortem polymerase chain reaction confirmed CNS‐WD with distinct clinical presentation, outcome and pathological findings. One patient had an acute onset with spinal and brainstem involvement and died without CNS‐WD diagnosis after 14 weeks. Neuropathology showed extensive inflammatory and necrotizing lesions with abundant foamy periodic‐acid‐Schiff (PAS)+ macrophages. The second patient had a subacute evolution with late CNS‐WD diagnosis and death occurring 18 months after onset despite antibiotic treatment. Brain examination showed inflammatory lesions in the brainstem, thalamus and cerebellum, and abundant foamy PAS+ macrophages. The third case was diagnosed within 4 weeks of onset and treated with an excellent response. He died after a disease‐free period of 24 months of unrelated causes. Neuropathology showed cystic residual lesions devoid of microorganisms without inflammatory reaction. CNS‐WD may have an acute or subacute course with variable response to treatment. Accordingly, subjacent lesions may be those of a severe acute necrotizing encephalitic process or subacute inflammatory lesions involving diencephalic, brainstem, cerebellar and spinal regions. Chronic, cavitary brain lesions may be sequelae of a successful treatment. Early diagnosis should allow appropriate treatment and improve prognosis.     

Clinical investigation of G‐Bond resin‐based adhesive to non‐carious cervical lesions over five years

Background: The aim of this study was to evaluate the clinical performance of G‐Bond all‐in‐one adhesive with Gradia Direct resin composite placed in non‐carious cervical lesions (NCCLs) over a five‐year period. Methods: Forty‐seven restorations were placed in NCCLs in 10 subjects (age 45–75 years) after written informed consent was obtained. Institutional ethical approval for the trial was obtained before recruitment. Restorations were placed according to the manufacturer’s instructions and using 50% phosphoric acid to etch uncut enamel margins. Patients were recalled annually for five years and restorations reviewed for presence and marginal staining. Photographic records were obtained prior to restoration, immediately after placement and at each recall. Results: At five years, 6 of the original 10 subjects were available for recall, meaning 27 restoration sites could be evaluated. All restorations remained intact apart for one partial failure at four years. This resulted in a cumulative retention rate of 97.5% of restorations at five years. Marginal staining occurred around seven restorations during the study. Staining tended to be isolated to a few patients. Conclusions: It was concluded that G‐Bond with Gradia Direct resin composite showed excellent results over the five years of the study. This material combination seems very suitable for the restoration of NCCLs.     

Somesthetic,gustatory, olfactory function and salivary flow in patients with neuropathic trigeminal pain

Oral Diseases (2010) 16 , 482–487 Objectives: To determine somesthetic, olfactory, gustative and salivary abnormalities in patients with burning mouth syndrome (BMS), idiopathic trigeminal neuralgia (ITN) and trigeminal postherpetic neuralgia (PHN). Subjects and Methods: Twenty patients from each group (BMS, ITN, PHN) and 60 healthy controls were evaluated with a systematized quantitative approach of thermal (cold and warm), mechanical, pain, gustation, olfaction and salivary flow; data were analyzed with ANOVA, Tukey, Kruskal–Wallis and Dunn tests with a level of significance of 5%. Results: There were no salivary differences among the groups with matched ages; the cold perception was abnormal only at the mandibular branch of PHN (P = 0.001) and warm was abnormal in all trigeminal branches of PHN and BMS; mechanical sensitivity was altered at the mandibular branch of PHN and in all trigeminal branches of BMS. The salty, sweet and olfactory thresholds were higher in all studied groups; the sour threshold was lower and there were no differences of bitter. Conclusion: All groups showed abnormal thresholds of gustation and olfaction; somesthetic findings were discrete in ITN and more common in PHN and BMS; central mechanisms of balance of sensorial inputs might be underlying these observations.