Effectiveness of Workplace Interventions in Return-to-Work for Musculoskeletal, Pain-Related and Mental Health Conditions: An Update of the Evidence and Messages for Practitioners.

Purpose The objective of this systematic review was to synthesize evidence on the effectiveness of workplace-based return-to-work (RTW) interventions and work disability management (DM) interventions that assist workers with musculoskeletal (MSK) and pain-related conditions and mental health (MH) conditions with RTW. Methods We followed a systematic review process developed by the Institute for Work & Health and an adapted best evidence synthesis that ranked evidence as strong, moderate, limited, or insufficient. Results Seven electronic databases were searched from January 1990 until April 2015, yielding 8898 non-duplicate references. Evidence from 36 medium and high quality studies were synthesized on 12 different intervention categories across three broad domains: health-focused, service coordination, and work modification interventions. There was strong evidence that duration away from work from both MSK or pain-related conditions and MH conditions were significantly reduced by multi-domain interventions encompassing at least two of the three domains. There was moderate evidence that these multi-domain interventions had a positive impact on cost outcomes. There was strong evidence that cognitive behavioural therapy interventions that do not also include workplace modifications or service coordination components are not effective in helping workers with MH conditions in RTW. Evidence for the effectiveness of other single-domain interventions was mixed, with some studies reporting positive effects and others reporting no effects on lost time and work functioning. Conclusions While there is substantial research literature focused on RTW, there are only a small number of quality workplace-based RTW intervention studies that involve workers with MSK or pain-related conditions and MH conditions. We recommend implementing multi-domain interventions (i.e. with healthcare provision, service coordination, and work accommodation components) to help reduce lost time for MSK or pain-related conditions and MH conditions. Practitioners should also consider implementing these programs to help improve work functioning and reduce costs associated with work disability.

Continuous subcutaneous insulin infusion (CSII) therapy at Derby Teaching Hospitals: sustained benefits in glucose control.

AIM: In the short term, continuous subcutaneous insulin infusion (CSII) has been associated with improved glycaemic control, reduced hypoglycaemia and improved quality of life (QOL). However, limited data are available on its long-term benefits, particularly in the UK. We aimed to assess the impact of CSII on longer term outcomes. METHOD: Patient-level data were obtained for CSII users at Derby Teaching Hospitals, UK. Patient confidence and satisfaction questionnaires using the Likert scale were used to assess confidence in self-management. Comparative statistics were conducted using Pearson's chi-square and Student's t-tests. RESULTS: Some 258 CSII users were identified (60.1% female, mean age 43.9 ± 13.4 years). Overall, there was significant decrease in HbA1c from 78 mmol/mol (9.3 ± 2.0%) at baseline, to 69 mmol/mol (8.5 ± 1.3%) at 6 months [mean difference (md): -0.64; 95% confidence interval (95% CI): -0.91 to -0.37; P < 0.0001]; which was sustained at 6 years of follow-up (HbA1c : 66 mmol/mol, 8.2 ± 1.3%; md: -1.07%; 95% CI: -1.45 to -0.69; P < 0.0001). One hundred and twenty-one patients (47%) responded to the survey, of whom 95 (78.5%) reported a reduction in the frequency of hypoglycaemia; 102 (84.3%) were satisfied with the quality of care received in the insulin pump service. CONCLUSION: CSII therapy led to a sustained long-term improvement in glycaemic control in addition to a reduction in self-reported hypoglycaemia.

Fancy a cup of scald? - The role of hot beverage burns in paediatric burns admissions in Ireland.

Burns and scalds are preventable injuries in children that typically occur in the home. This study aimed to examine the role of hot beverage scalds in paediatric burn admissions in order to identify key target audiences for future safety strategies. Using the Hospital Inpatient Enquiry System (HIPE) a retrospective study of paediatric burn admissions in 2014 examined demographics, cause and severity of injury and location of occurrence. There were 233 paediatric discharges (age 0-18 yrs.) with a principal diagnosis of burn injury; 57% of these occurred in children under three years and 95% of these occurred in the home. Scalds caused 74% of burn injuries; hot beverages accounted for least 33% of these of which 77% were partial thickness and 73% were upper body burns. Effective hot beverage scald prevention strategies, targeted towards caregivers in the home, are required.

[Clinical pharmacy: Evaluation of physician's satisfactions and expectations in a French regional hospital]. / La pharmacie clinique en milieu hospitalier : une enquête de satisfaction auprès des prescripteurs.

INTRODUCTION: The purpose of the study was to evaluate physician's satisfaction with the clinical pharmacy activities in a French regional hospital. METHODS: Data were collected by face-to-face interviews carried out by a public health intern with physicians from 14 different departments of medicine and surgery. A specifically designed questionnaire was used for this study. This contained 18 closed-ended questions, 3 open-ended questions and 6 questions relating to the multidisciplinary analysis of prescriptions of elderly patients. RESULTS: The questionnaire was proposed to 78 physicians, of which 62 replied (participation rate of 79%). Thirty-seven percent were interns (23/62), 19% were assistants (12/62) and 44% were senior physicians (27/62). Clinical pharmacy satisfaction levels were generally very high. In regard to clinical skills, 87% of the physicians were satisfied with pharmacists' competencies and 91% by the pertinence of transmitted information. Ninety-five percent of the physicians were also satisfied by the logistical aspect and the relationship with pharmacists (reactivity, availability and communication). Analysis of the open-ended questions showed that physicians were in favour of the increased presence of clinical pharmacists on the wards. CONCLUSIONS: This study shows a high level of physician satisfaction in relation to the clinical pharmacy activities in our hospital, and should be viewed as a strong endorsement of the work of the clinical pharmacy. This study highlights some areas of improvement such as increase presence of the clinical pharmacists on the wards. In order to assess periodically our activity, this study must be repeated in the future.

Pulmonary radiofrequency ablation in a district general hospital: is it a safe and effective treatment?

AIM: To analyse the technical success of ablation therapy and the incidence of complications in patients treated with pulmonary ablation and to assess factors affecting local disease control and patient survival in a subgroup with metastatic colorectal cancer. MATERIALS AND METHODS: Technical success and complications in all patients undergoing lung ablation between June 2009 and July 2015 were recorded. Overall survival and local disease control in a subgroup with metastases from a colorectal primary were calculated. Factors influencing outcome were explored. RESULTS: Two hundred and seven pulmonary ablations were performed in 86 patients at 156 attendances. Technical success was achieved in 207/207 (100%). Thirty and 90-day mortality was 0%. The major complication rate was 13/86 (15%). One hundred and one metastases were treated in 46 patients with a colorectal primary. This group had a mean ± standard error survival time of 53.58±3.47 months with a 1, 2, 3, 4, and 5-year survival rate of 97.4%, 91.3%, 81.5%, 59.8%, and 48%. There was no statistically significant difference in survival regarding time to development of metastatic disease, the total number of lesions ablated, the initial number of lesions ablated, the maximum size of lesion treated, or unilateral versus bilateral disease. Patients with extrapulmonary disease were found to have a shorter survival from the primary diagnosis. Seventy-eight (77.2%) of the 101 lesions were stable after first RFA. Local relapse was more likely when a metastasis was close to a large (>3 mm) vessel. CONCLUSION: RFA is a safe and effective procedure that can be performed without on-site cardiothoracic support. Good outcomes depend upon careful patient selection. This study supports its use in oligometastatic disease.

Inter-laboratory study of human in vitro toxicogenomics-based tests as alternative methods for evaluating chemical carcinogenicity: a bioinformatics perspective.

The assessment of the carcinogenic potential of chemicals with alternative, human-based in vitro systems has become a major goal of toxicogenomics. The central read-out of these assays is the transcriptome, and while many studies exist that explored the gene expression responses of such systems, reports on robustness and reproducibility, when testing them independently in different laboratories, are still uncommon. Furthermore, there is limited knowledge about variability induced by the data analysis protocols. We have conducted an inter-laboratory study for testing chemical carcinogenicity evaluating two human in vitro assays: hepatoma-derived cells and hTERT-immortalized renal proximal tubule epithelial cells, representing liver and kidney as major target organs. Cellular systems were initially challenged with thirty compounds, genome-wide gene expression was measured with microarrays, and hazard classifiers were built from this training set. Subsequently, each system was independently established in three different laboratories, and gene expression measurements were conducted using anonymized compounds. Data analysis was performed independently by two separate groups applying different protocols for the assessment of inter-laboratory reproducibility and for the prediction of carcinogenic hazard. As a result, both workflows came to very similar conclusions with respect to (1) identification of experimental outliers, (2) overall assessment of robustness and inter-laboratory reproducibility and (3) re-classification of the unknown compounds to the respective toxicity classes. In summary, the developed bioinformatics workflows deliver accurate measures for inter-laboratory comparison studies, and the study can be used as guidance for validation of future carcinogenicity assays in order to implement testing of human in vitro alternatives to animal testing.

Explorative study of the sensitivity and specificity of the pronator quadratus fat pad sign as a predictor of subtle wrist fractures.

OBJECTIVE: To investigate the value of post-traumatic pronator quadratus (PQ) fat pad sign as a reliable predictor of subtle wrist fractures. MATERIALS AND METHODS: This was a prospective study of 68 patients undergoing X-ray for traumatic wrist injuries and subsequent MRI. The reliability of a positive PQ fat pad sign on X-ray, defined as either raised, interrupted or obliterated, was evaluated in detection of subtle wrist fractures. RESULTS: Out of 68 patients, 28 had a positive PQ sign without any obvious bony injuries on plain radiographs; of these, the PQ fat pad was obliterated in 11, disrupted in 12, and raised in five cases. Fractures defined as cortical interruption or trabecular fractures were revealed in 13/28 (46 %) patients with a positive PQ sign but only in 7/40 (18 %) patients with a negative sign. With regards to different types of abnormal PQ fat planes, fractures were present in 7/12 patients with a disrupted plane (58 %), 6/11 patients with an obliterated plane (54 %), and none of the patients with a raised plane. The overall sensitivity and specificity of a positive PQ sign in the prediction of occult wrist fractures were 65 and 69 %, respectively. CONCLUSIONS: Our findings indicate that a positive pronator quadratus (PQ) fat pad sign is not a reliable predictor of subtle fractures of the wrist, although we believe that it is a useful radiographic sign in justifying MRI for further clarification of possible joint abnormalities including occult fracture and cortex interruption.

Improving the hospital 'soundscape': a framework to measure individual perceptual response to hospital sounds.

Work on the perception of urban soundscapes has generated a number of perceptual models which are proposed as tools to test and evaluate soundscape interventions. However, despite the excessive sound levels and noise within hospital environments, perceptual models have not been developed for these spaces. To address this, a two-stage approach was developed by the authors to create such a model. First, semantics were obtained from listening evaluations which captured the feelings of individuals from hearing hospital sounds. Then, 30 participants rated a range of sound clips representative of a ward soundscape based on these semantics. Principal component analysis extracted a two-dimensional space representing an emotional-cognitive response. The framework enables soundscape interventions to be tested which may improve the perception of these hospital environments.

The National Newborn Bloodspot Screening Programme (NNBSP) process review 2010.

The National Newborn Bloodspot Screening Programme (NNBSP) incorporates screening for several conditions where early identification helps prevent serious disability or mortality. A national group was established (2009) to plan for the inclusion of screening for cystic fibrosis (CF); as part of this process a review of the existing NNBSP was undertaken. Information was obtained through consultation with the Director of the National Newborn Bloodspot Screening Laboratory (NNBSL) and survey of maternity units (20), Local Health Office (LHO) areas (32), and practicing domiciliary midwives (16). Response rate: hospitals 100%; LHO areas 100%; domiciliary midwives 56%. Potential for strengthening quality assurance was identified e.g. single comprehensive screening register, consistent and timely procedures for checking results. Recommendations, many of which have since been implemented, included overall governance structure, liaison officer, quality assurance programme, ring-fenced funding, standardised unique identifier, dedicated laboratory IT system, secure web-based transmission of results and fail-safe method of tracking samples.

Offspring searching for their sperm donors: how family type shapes the process.

BACKGROUND: This study examines the findings from the largest survey to date of donor-inseminated (DI) offspring and focuses on respondents' learning of the method of their conception and their desire to contact their donor. METHODS: Online questionnaires were completed by 741 DI offspring, of whom 61.8% have heterosexual parents and 38.2% have lesbian parents. Respondents were recruited via the Donor Sibling Registry, a non-profit US-based international registry that facilitates communication between donor-conceived offspring and their non-biological and biological relatives. Data were collected on family composition, offspring's feelings regarding the method of their conception, communication within families, donor anonymity and their search for their donors. This investigation focuses on the relationship between family type (single or dual-parent and lesbian or heterosexual parent/s) and offspring's reactions to learning of their DI conception. RESULTS: Offspring of lesbian parents learned of their DI origins at earlier ages than offspring of heterosexual parents. In the latter families, disclosure tended to occur earlier in single-parent than in dual-parent families. Disclosure was most likely to be confusing to offspring of heterosexual parents, particularly when it occurred at an older age. The vast majority of offspring in all types of families desired contact with their donor; however, comfort in expressing curiosity regarding one's donor was lowest in dual-parent heterosexual families, with about one-quarter reporting an inability to discuss their origins with their social father. CONCLUSIONS: Although the findings are not based on a random sample, the desire among offspring surveyed here is for greater openness and contact with their donor. A variety of strategies are needed for offspring of heterosexual couples to benefit optimally from the general trend toward openness in gamete donation.

Ketamine as an analgesic in the pre-hospital setting: a systematic review.

BACKGROUND: Pain is a common presenting complaint and there is considerable debate regarding the best practice for analgesia in the pre-hospital environment for trauma patients with severe pain. METHODS: A review of the literature was conducted using a number of electronic medical literature databases from their earliest record to the latest available at the time the search was conducted (May 2010). Medical Subject Headings, keywords and a pre-hospital search filter were used to yield relevant literature. RESULTS: The search strategy yielded a total of 837 references. Seven hundred and fifty of these references were excluded as they did not meet the inclusion criteria. Of the 87 articles short listed for abstract or full-text review, six reported on ketamine use as an analgesic agent in the pre-hospital setting. Two papers were prospective randomized-controlled trials, and the number of patients included in the studies ranged from 4 to 164. Three studies aimed to report on the effectiveness of ketamine for pain intensity reduction; two concluded that ketamine provided safe and effective pain relief and one reported that ketamine reduced the amount of morphine required but was not associated with a reduction in pain intensity. One study identified a significantly higher prevalence of adverse effects following ketamine administration. The other studies reported no significant side effects and concluded that ketamine was safe. CONCLUSION: Ketamine is a safe and effective analgesic agent. The addition of ketamine as an analgesic agent may improve the management of patients presenting with acute traumatic pain in the pre-hospital setting.

Barriers to incident notification in a regional prehospital setting.

BACKGROUND: The identification and monitoring of critical incidents or adverse events and error reporting is a relatively new area of study in the prehospital setting. In 2005, we commenced a prospective descriptive study of the implementation of a Critical Incident Monitoring process in a rural/regional pre-hospital setting. The objective of the project was to describe the nature and incidence of errors detected in the management of prehospital trauma with the ultimate aim of identifying processes to reduce or mitigate such incidents. This paper describes the barriers to reporting critical incidents identified during the 3-year study. METHOD: This study used a qualitative approach involving the triangulation of a number of ethnographic methodologies, including unscripted focus groups, informal interviews and qualitative aspects of surveys utilised in a broader research project. Prevailing themes were fed back to participants in an iterative process to further explore perceptions and beliefs regarding these concepts. The final analysis of themes is descriptively presented. RESULTS: A number of barriers were identified and categorised into seven themes. These themes were; Burden of reporting, fear of disciplinary action, fear of potential litigation, fear of breaches of confidentiality and fear of embarrassment, concern that 'nothing would change' even if the incident was reported, lack of familiarity with process and impact of 'blame culture'. CONCLUSION: There are numerous barriers to reporting critical incidents. One of the key approaches which may alleviate many of the barriers to reporting is shifting to a systems based focus rather than an individual 'shame and blame' approach. The underlying barriers lie in the culture of the profession, and appear consistent across other health care disciplines.

Eyes Toward Tomorrow Program Enhancing Collaboration, Connections, and Community Using Bioinspired Design.

The goal of our Eyes Toward Tomorrow Program is to enrich the future workforce with STEM by providing students with an early, inspirational, interdisciplinary experience fostering inclusive excellence. We attempt to open the eyes of students who never realized how much their voice is urgently needed by providing an opportunity for involvement, imagination, invention, and innovation. Students see how what they are learning, designing, and building matters to their own life, community, and society. Our program embodies convergence by obliterating artificially created, disciplinary boundaries to go far beyond STEM or even STEAM by including artists, designers, social scientists, and entrepreneurs collaborating in diverse teams using scientific discoveries to create inventions that could shape our future. Our program connects two recent revolutions by amplifying Bioinspired Design with the Maker Movement and its democratizing effects empowering anyone to innovate and change the world. Our course is founded in original discovery. We explain the process of biological discovery and the importance of scaling, constraints, and complexity in selecting systems for bioinspired design. By spotlighting scientific writing and publishing, students become more science literate, learn how to decompose a biology research paper, extract the principles, and then propose a novel design by analogy. Using careful, early scaffolding of individual design efforts, students build the confidence to interact in teams. Team building exercises increase self-efficacy and reveal the advantages of a diverse set of minds. Final team video and poster project designs are presented in a public showcase. Our program forms a student-centered creative action community comprised of a large-scale course, student-led classes, and a student-created university organization. The program structure facilitates a community of learners that shifts the students' role from passive knowledge recipients to active co-constructors of knowledge being responsible for their own learning, discovery, and inventions. Students build their own shared database of discoveries, classes, organizations, research openings, internships, and public service options. Students find next step opportunities so they can see future careers. Description of our program here provides the necessary context for our future publications on assessment that examine 21st century skills, persistence in STEM, and creativity.

Perspectives on environmental impacts and a land reclamation strategy for solar and wind energy systems.

Global energy demands and environmental concerns are a driving force for use of alternative, sustainable and clean energy sources. Solar and wind are among the most promising sources and have been developing steadily in recent years. However, these energy developments are not free of adverse environmental consequences, which require appropriate reclamation procedures. The environmental issues caused by solar and wind plants were reviewed in this paper by summarizing existing studies and synthesizing the principles that could underlie development of reclamation practices. The major environmental drawback of solar and wind energy plants are bird mortality, biodiversity, and habitat loss; noise; visual impact; and hazardous chemicals used in solar panels. Available mitigation measures to minimize these adverse environmental impacts, and appropriate reclamation protocol for the disturbed ecosystems, including key research needs are discussed. We include socio-economic perspectives of solar and wind energy, such as policy related to re-powering initiatives, decommissioning, and reclamation liability. The intent of this paper is to provide current perspectives on environmental issues associated with solar and wind energy development, strategies to mitigate environmental impacts, and potential reclamation practices to solar and wind energy planners and developers.

Dynamics of capping protein and actin assembly in vitro: uncapping barbed ends by polyphosphoinositides.

Bursts of actin polymerization in vivo involve the transient appearance of free barbed ends. To determine how rapidly barbed ends might appear and how long they might remain free in vivo, we studied the kinetics of capping protein, the major barbed end capper, binding to barbed ends in vitro. First, the off-rate constant for capping protein leaving a barbed end is slow, predicting a half-life for a capped barbed end of approximately 30 min. This half-life implies that cells cannot wait for capping protein to spontaneously dissociate from capped barbed ends in order to create free barbed ends. However, we find that phosphatidylinositol 4,5-bisphosphate (PIP2) and phosphatidylinositol 4-mono-phosphate (PIP) cause rapid and efficient dissociation of capping protein from capped filaments. PIP2 is a strong candidate for a second messenger regulating actin polymerization; therefore, the ability of PIP2 to remove capping protein from barbed ends is a potential mechanism for stimulating actin polymerization in vivo. Second, the on-rate constant for capping protein binding to free barbed ends predicts that actin filaments could grow to the length of filaments observed in vivo during one lifetime. Third, capping protein beta-subunit isoforms did not differ in their actin binding properties, even in tests with different actin isoforms. A major hypothesis for why capping protein beta-subunit isoforms exist is thereby excluded. Fourth, the proposed capping protein regulators, Hsc70 and S100, had no effect on capping protein binding to actin in vitro.

An enzyme trafficking defect in two patients with primary hyperoxaluria type 1: peroxisomal alanine/glyoxylate aminotransferase rerouted to mitochondria.

Most patients with the autosomal recessive disease primary hyperoxaluria type 1 (PH1) have a complete deficiency of alanine/glyoxylate aminotransferase (AGT) enzyme activity and immunoreactive protein. However a few possess significant residual activity and protein. In normal human liver, AGT is entirely peroxisomal, whereas it is entirely mitochondrial in carnivores, and both peroxisomal and mitochondrial in rodents. Using the techniques of isopycnic sucrose and Percoll density gradient centrifugation and quantitative protein A-gold immunoelectron microscopy, we have found that in two PH1 patients, possessing 9 and 27% residual AGT activity, both the enzyme activity and immunoreactive protein were largely mitochondrial and not peroxisomal. In addition, these individuals were more severely affected than expected from the levels of their residual AGT activity. In these patients, the PH1 appears to be due, at least in part, to a unique trafficking defect, in which peroxisomal AGT is diverted to the mitochondria. To our knowledge, this is the first example of a genetic disease caused by such interorganellar rerouting.

Mammalian alanine/glyoxylate aminotransferase 1 is imported into peroxisomes via the PTS1 translocation pathway. Increased degeneracy and context specificity of the mammalian PTS1 motif and implications for the peroxisome-to-mitochondrion mistargeting of AGT in primary hyperoxaluria type 1.

Alanine/glyoxylate aminotransferase 1 (AGT) is peroxisomal in most normal humans, but in some patients with the hereditary disease primary hyperoxaluria type 1 (PH1), AGT is mislocalized to the mitochondria. In an attempt to identify the sequences in AGT that mediate its targeting to peroxisomes, and to determine the mechanism by which AGT is mistargeted in PH1, we have studied the intracellular compartmentalization of various normal and mutant AGT polypeptides in normal human fibroblasts and cell lines with selective deficiencies of peroxisomal protein import, using immunofluorescence microscopy after intranuclear microinjection of AGT expression plasmids. The results show that AGT is imported into peroxisomes via the peroxisomal targeting sequence type 1 (PTS1) translocation pathway. Although the COOH-terminal KKL of human AGT was shown to be necessary for its peroxisomal import, this tripeptide was unable to direct the peroxisomal import of the bona fide peroxisomal protein firefly luciferase or the reporter protein bacterial chloramphenicol acetyltransferase. An ill-defined region immediately upstream of the COOH-terminal KKL was also found to be necessary for the peroxisomal import of AGT, but again this region was found to be insufficient to direct the peroxisomal import of chloramphenicol acetyltransferase. Substitution of the COOH-terminal KKL of human AGT by the COOH-terminal tripeptides found in the AGTs of other mammalian species (SQL, NKL), the prototypical PTS1 (SKL), or the glycosomal PTS1 (SSL) also allowed peroxisomal targeting, showing that the allowable PTS1 motif in AGT is considerably more degenerate than, or at least very different from, that acceptable in luciferase. AGT possessing the two amino acid substitutions responsible for its mistargeting in PH1 (i.e., Pro11-->Leu and Gly170-->Arg) was targeted mainly to the mitochondria. However, AGTs possessing each amino acid substitution on its own were targeted normally to the peroxisomes. This suggests that Gly170-->Arg-mediated increased functional efficiency of the otherwise weak mitochondrial targeting sequence (generated by the Pro11-->Leu polymorphism) is not due to interference with the peroxisomal targeting or import of AGT.

Formation of a molten globule intermediate early in the kinetic folding pathway of apomyoglobin.

Hydrogen exchange pulse labeling and stopped-flow circular dichroism were used to establish that the structure of the earliest detectable intermediate formed during refolding of apomyoglobin corresponds closely to that of a previously characterized equilibrium molten globule. This compact, cooperatively folded intermediate was formed in less than 5 milliseconds and contained stable, hydrogen-bonded secondary structure localized in the A, G, and H helices and part of the B helix. The remainder of the B helix folded on a much slower time scale, followed by the C and E helices and the CD loop. The data indicate that a molten globule intermediate was formed on the kinetic folding pathway.

Familial juvenile hyperuricemic nephropathy: report on a new mutation and a pregnancy.

BACKGROUND: Familial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disease caused by mutations in the uromodulin gene (UMOD) and leading to gout, tubulointerstitial nephropathy and end-stage renal disease. CASE REPORTS AND RESULTS: A Latvian family suffering from FJHN is described. The father of the family developed ESRD at age 36. His daughter was diagnosed with gout and chronic kidney disease at age 14 years. A renal biopsy revealed tubulointerstitial disease; 2 sons were diagnosed at age 9 and 4 with elevated uric acid levels and reduced fractional uric acid excretion. Urinary uromodulin was normal in the younger boy, but markedly decreased in the 2 other patients. Genetic analysis revealed a previously undescribed D196Y mutation in the UMOD gene. The female patient became pregnant at age 23. During pregnancy serum creatinine decreased from 2.0 to 1.5 mg/dl and blood pressure remained low. Analysis of the baby's umbilical cord blood and a mouth swab showed the presence of the D196Y mutation. Its urinary uromodulin excretion was in the low normal range. CONCLUSION: The uromodulin excretion pattern observed in the investigated family suggests that urinary uromodulin decreases in FJHN from low normal values at childhood to extremely low levels in early adulthood. In addition, this first report on pregnancy in a patient with FJHN shows normal adaptation despite markedly reduced renal function.

Measuring acute pain in the prehospital setting.

Severe pain is a common presenting symptom for emergency patients. One major challenge in the management of severe pain is the objective measurement of pain. Due to the subjective nature of pain, it can be very difficult for clinicians to quantify pain intensity and measure the qualitative features of the pain experience. A number of measurement tools have been validated in the acute care setting, with some appropriate for use in the prehospital setting. This paper reviews the characteristics required of a prehospital acute pain measure and appraises the relative utility of a number of currently used pain measures. At present, the verbal numerical rating scale appears the most appropriate pain measure to administer in the prehospital setting for adult patients as it is practical and valid. Either the Oucher scale or the faces pain scale is suitable for prehospital care providers to assess pain in children.