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Randomized controlled trial of topical corticosteroid and home-based narrowband ultraviolet B for active and limited vitiligo: results of the HI-Light Vitiligo Trial.
No full textBACKGROUND
Evidence for the effectiveness of vitiligo treatments is limited.
OBJECTIVES
To determine the effectiveness of (i) handheld narrowband UVB (NB-UVB) and (ii) a combination of potent topical corticosteroid (TCS) and NB-UVB, compared with TCS alone, for localized vitiligo.
METHODS
A pragmatic, three-arm, placebo-controlled randomized controlled trial (9-month treatment, 12-month follow-up). Adults and children, recruited from secondary care and the community, aged ≥ 5 years and with active vitiligo affecting < 10% of skin, were randomized 1 : 1 : 1 to receive TCS (mometasone furoate 0·1% ointment + dummy NB-UVB), NB-UVB (NB-UVB + placebo TCS) or a combination (TCS + NB-UVB). TCS was applied once daily on alternating weeks; NB-UVB was administered on alternate days in escalating doses, adjusted for erythema. The primary outcome was treatment success at 9 months at a target patch assessed using the participant-reported Vitiligo Noticeability Scale, with multiple imputation for missing data. The trial was registered with number ISRCTN17160087 on 8 January 2015.
RESULTS
In total 517 participants were randomized to TCS (n = 173), NB-UVB (n = 169) and combination (n = 175). Primary outcome data were available for 370 (72%) participants. The proportions with target patch treatment success were 17% (TCS), 22% (NB-UVB) and 27% (combination). Combination treatment was superior to TCS: adjusted between-group difference 10·9% (95% confidence interval 1·0%-20·9%; P = 0·032; number needed to treat = 10). NB-UVB alone was not superior to TCS: adjusted between-group difference 5·2% (95% CI - 4·4% to 14·9%; P = 0·29; number needed to treat = 19). Participants using interventions with ≥ 75% expected adherence were more likely to achieve treatment success, but the effects were lost once treatment stopped. Localized grade 3 or 4 erythema was reported in 62 (12%) participants (including three with dummy light). Skin thinning was reported in 13 (2·5%) participants (including one with placebo ointment).
CONCLUSIONS
Combination treatment with home-based handheld NB-UVB plus TCS is likely to be superior to TCS alone for treatment of localized vitiligo. Combination treatment was relatively safe and well tolerated but was successful in only around one-quarter of participants
A case series of cerebral venous thrombosis as the first manifestation of homocystinuria.
No full textBackground
Cerebral venous thrombosis (CVT) is an important cause of stroke particularly in younger patients and potentially fatal if diagnosis is delayed. The presentation of symptoms is highly variable and consequently the diagnosis and underlying cause is often delayed or overlooked. Homocystinuria, a rare autosomal recessive disorder is an identified risk factor for CVT.
Purpose
A timely diagnosis and treatment of the underlying cause of CVT could result in improved outcome and prevent further events. This case series describes the clinical course of six adults presented with unprovoked CVT, in whom the diagnosis of underlying homocystinuria was delayed with adverse consequences. We aim to highlight the importance of recognising homocystinuria as an underlying cause of CVT and offer a practical approach to the diagnosis and management.
Methods
This is a retrospective case series of a cohort of 30 consecutive patients seen in a UK tertiary referral centre.
Result
Six out of 30 patients presented with CVT prior to homocystinuria diagnosis. The mean and range of age at the time of the first CVT episode was 22.6 (range 11-31) years. The mean ±SD age at diagnosis of homocystinuria as the underlying cause was 26 ± 4.2 years. The time between first CVT and diagnosis of homocystinuria ranged from 1.6 to 11 years resulting in a delay to introduction of effective treatment and, in some cases, a further large vessels thrombotic event.
Conclusion
Physician awareness of homocystinuria as an underlying cause for an unprovoked CVT will facilitate timely introduction of effective treatment to prevent a further event
Cytomegalovirus serological testing in potential allogeneic haematopoietic stem cell transplant recipients: A British Society for Haematology Good Practice Paper.
No full textStructural Endpoints and Outcome Measures in Uveitis.
No full textMost uveitis entities are rare diseases but, taken together, are responsible for 5-10% of worldwide visual impairment which largely affects persons of working age. As with many rare diseases, there is a lack of high-level evidence regarding its clinical management, partly due to a dearth of reliable and objective quantitative endpoints for clinical trials. This review provides an overview of available structural outcome measures for uveitis disease activity and damage in an anatomical order from the anterior to the posterior segment of the eye. While there is a multitude of available structural outcome measures, not all might qualify as endpoints for clinical uveitis trials, and thorough testing of applicability is warranted. Furthermore, a consensus on endpoint definition, standardization, and "core outcomes" is required. As stipulated by regulatory agencies, endpoints should be precisely defined, clinically important, internally consistent, reliable, responsive to treatment, and relevant for the respective subtype of uveitis. Out of all modalities used for assessment of the reviewed structural outcome measures, optical coherence tomography, color fundus photography, fundus autofluorescence, and fluorescein/indocyanine green angiography represent current "core modalities" for reliable and objective quantification of uveitis outcome measures, based on their practical availability and the evidence provided so far
Randomized Trial of Ciprofloxacin Doxycycline and Hydroxychloroquine Versus Budesonide in Active Crohn's Disease.
No full textBACKGROUND
Increased mucosa-associated E. coli are present in Crohn's disease, but their role in pathogenesis is uncertain.
AIMS
To assess efficacy and safety of an antibiotic/hydroxychloroquine combination effective against E. coli inside macrophages.
METHODS
Adults with moderately active disease (CDAI > 220-450 plus C reactive protein ≥ 5 mg/l and/or fecal calprotectin > 250 μg/g) were randomized to receive (open-label) oral budesonide (Entocort CR 9 mg/day 8 weeks, 6 mg/day 2 weeks, 3 mg/day 2 weeks) or oral ciprofloxacin 500 mg bd, doxycycline 100 mg bd, hydroxychloroquine 200 mg tds for 4 weeks, followed by doxycycline 100 mg bd and hydroxychloroquine 200 mg tds for 20 weeks. Primary endpoints were remission (CDAI ≤ 150) at 10 weeks, remission maintained to 24 weeks, and remission maintained to 52 weeks. Patients not responding (CDAI fall by > 70) by 10 weeks were invited to crossover onto the alternative therapy.
RESULTS
Fifty-nine patients were recruited across 8 sites. Including crossover, 39 patients received antibiotics/hydroxychloroquine and 39 received budesonide. At 10 weeks, 24 weeks, and 52 weeks on initial therapy, only 2/27, 2/27, and 1/27 were in remission on antibiotics/hydroxychloroquine compared with 8/32, 1/32, and 1/32 on budesonide (P = 0.092 at 10 weeks). Withdrawals by 10 weeks due to adverse events were seen in 15 receiving antibiotics/hydroxychloroquine and 6 budesonide. Results including crossover were more promising with 9/24 patients receiving antibiotics/hydroxychloroquine per protocol in remission by 24 weeks. No correlation was seen between response to antibiotics/hydroxychloroquine and ASCA/OmpC antibody status or disease location.
CONCLUSION
Overall results with this antibiotic/hydroxychloroquine combination were unimpressive, but long-term remission is seen in some patients and justifies further study
European Liver Transplant Registry: donor and transplant surgery aspects of 16,641 liver transplantations in children.
No full textBACKGROUND & AIMS
The European Liver Transplant Registry (ELTR) has collected data on liver transplant procedures performed in Europe since 1968.
APPROACH & RESULTS
Over a 50 years period (1968 - 2017), clinical and laboratory data were collected from 133 transplant centers and analyzed retrospectively (16,641 liver transplants in 14,515 children). Data were analyzed according to 3 successive periods (A: before 2000, B: 2000 to 2009, and C: since 2010), studying donor and graft characteristics, and graft outcome. The use of living donors steadily increased from A to C [A: n=296 (7%), B: n=1131 (23%) and C: n=1985 (39%); P=0.0001]. Overall, the 5-year graft survival rate has improved from 65% in group A to 75% in group B (p<0.0001), and to 79% in group C (B vs C, p<0.0001). Graft half-life was 31 years, overall; it was 41 years for children who survived the first year after transplant. The late annual graft loss rate in teen-agers is higher than children aged < 12 years, and similar to that of young adults. No evidence for accelerated graft loss after age 18 year was found.
CONCLUSIONS
Pediatric liver transplantation has reached a high efficacy as a cure, or treatment, for severe liver disease in infants and children. Grafts that survived the first year had a half-life time similar to standard human half-life. Transplantation before or after puberty may be the pivot-point for lower long-term outcome in children. Further studies are necessary to re-visit some old concepts regarding transplant benefit (survival time) for small children, the role of recipient pathophysiology versus graft aging, and risk at transition to adult age
A Capacitive Cochlear Implant Electrode Array Sensing System to Discriminate Fold-Over Pattern.
No full textPurpose During insertion of the cochlear implant electrode array, the tip of the array may fold back on itself and can cause serious complications to patients. This article presents a sensing system for cochlear implantation in a cochlear model. The electrode array fold-over behaviors can be detected by analyzing capacitive information from the array tip. Method Depending on the angle of the array tip against the cochlear inner wall when it enters the cochlear model, different insertion patterns of the electrode array could occur, including smooth insertion, buckling, and fold-over. The insertion force simulating the haptic feedback for surgeons and bipolar capacitance signals during the insertion progress were collected and compared. The Pearson correlation coefficient (PCC) was applied to the collected capacitive signals to discriminate the fold-over pattern. Results Forty-six electrode array insertions were conducted and the deviation of the measured insertion force varies between a range of 20% and 30%. The capacitance values from electrode pair (1, 2) were recorded for analyzing. A threshold for the PCC is set to be 0.94 that can successfully discriminate the fold over insertions from the other two types of insertions, with a success rate of 97.83%. Conclusions Capacitive measurement is an effective method for the detection of faulty insertions and the maximization of the outcome of cochlear implantation. The proposed capacitive sensing system can be used in other tissue implants in vessels, spinal cord, or heart
Extended Scalp Flaps for Extensive Soft Tissue Scalp Defects as a Day Surgery Procedure Under Local Anesthetic: A Single Centre Experience.
No full textCutaneous malignancies are on the rise, associated with an increased number in scalp cancers that require wide local excision (WLE) to ensure clearance; the inelastic nature of the scalp poses a particular challenge when dealing with such large defects. A series of 68 cases with large scalp defects following WLE for the clearance of squamous cell carcinoma, atypical fibroxanthoma, dermatofibrosarcoma protuberans, and melanoma skin cancers are presented. These cases were treated in one center under local anesthesia and underwent extended scalp flaps to close the resulting defect primarily without the use of skin grafts for the flap donor site on the scalp. Extended scalp flap is a safe and reproducible solution for extensive scalp defects, which results in quicker wound healing with cosmetically superior results, and can be performed safely and comfortably under local anesthesia in the day case setting
Miscellaneous Tumours of Bone.
No full textThere are several tumors that do not easily fit into the specific classifications of primary bone tumors. These tumors include tumors of neural, adipocytic, smooth muscle lineage, and some of uncertain lineage. The pathologic features with recent updates of these tumors are discussed here
Nonadherence in Hypertension: How to Develop and Implement Chemical Adherence Testing.
No full textNonadherence to antihypertensive medication is common, especially in those with apparent treatment-resistant hypertension (true treatment-resistant hypertension requires exclusion of nonadherence), and its routine detection is supported by clinical guidelines. Chemical adherence testing is a reliable and valid method to detect adherence, yet methods are unstandardized and are not ubiquitous. This article describes the principles of chemical adherence testing for hypertensive patients and provides a set of recommendations for centers wishing to develop the test. We recommend testing should be done in either of two instances: (1) in those who have resistant hypertension or (2) in those on 2 antihypertensives who have a less than 10 mm Hg drop in systolic blood pressure on addition of the second antihypertensive medication. Furthermore, we recommend that verbal consent is secured before undertaking the test, and the results should be discussed with the patient. Based on medications prescribed in United Kingdom, European Union, and United States, we list top 20 to 24 drugs that cover >95% of hypertension prescriptions which may be included in the testing panel. Information required to identify these medications on mass spectrometry platforms is likewise provided. We discuss issues related to ethics, sample collection, transport, stability, urine versus blood samples, qualitative versus quantitative testing, pharmacokinetics, instrumentation, validation, quality assurance, and gaps in knowledge. We consider how to best present, interpret, and discuss chemical adherence test results with the patient. In summary, this guidance should help clinicians and their laboratories in the development of chemical adherence testing of prescribed antihypertensive drugs