Royal Berkshire Hospital NHS Foundation Trust

Reading, United Kingdom

Royal Berkshire Hospital NHS Foundation Trust

Reading, United Kingdom
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Yusuf I.H.,Royal Berkshire Hospital NHS Foundation Trust | Sandford V.,Royal Berkshire Hospital NHS Foundation Trust | Hildebrand G.D.,Royal Berkshire Hospital NHS Foundation Trust | Hildebrand G.D.,King Edward VII Hospital
Journal of AAPOS | Year: 2013

Pyridoxine-dependent epilepsy (PDE) is a cause of neonatal epileptic encephalopathy not previously known to cause ophthalmic disease. We describe the novel observation of a 5-year-old girl with pyridoxine-dependent epilepsy and bilateral cataracts. PDE is the result of mutations in the ALDH7A1 gene encoding antiquitin, an enzyme protective against cellular dehydration and osmotic stress. Accumulating metabolic precursors in PDE have been shown to be cataractogenic in vitro, and experimental pyridoxine deficiency has been associated with lenticular opacities in vivo. The association of ALDH7A1 haploinsufficiency in PDE and congenital cataract may offer insight into the relationship between osmotic stress and fetal cataract development. Bilateral progression of cataracts in this child suggests ongoing metabolic dysregulation within the crystalline lens despite pyridoxine supplementation at doses sufficient to control seizure activity. Copyright © 2013 by the American Association for Pediatric Ophthalmology and Strabismus.


Yusuf I.H.,Royal Berkshire Hospital NHS Foundation Trust | Sahare P.,Royal Berkshire Hospital NHS Foundation Trust | Hildebrand G.D.,Royal Berkshire Hospital NHS Foundation Trust | Hildebrand G.D.,King Edward VII Hospital
Journal of AAPOS | Year: 2013

Children treated for toxoplasma retinochoroiditis may experience a range of severe adverse drug responses. Drug reaction with eosinophilia and systemic symptoms syndrome is a life-threatening idiosyncratic drug reaction with a 10% mortality. We present a case of drug reaction with eosinophilia and systemic symptoms syndrome in a child on standard combination treatment with oral sulfadiazine, pyrimethamine, folinic acid, and steroids for toxoplasma retinochoroiditis. Early clinical recognition and appropriate treatment led to a complete recovery and no longterm sequelae. The parents of children during sulfadiazine treatment should be counseled on the potential significance of nonspecific illness. Copyright © 2013 by the American Association for Pediatric Ophthalmology and Strabismus.


Bhole M.V.,Russells Hall Hospital | Sadler R.,Churchill Hospital | Ramasamy K.,Churchill Hospital | Ramasamy K.,Royal Berkshire Hospital NHS Foundation Trust | Ramasamy K.,National Health Research Institute
Annals of Clinical Biochemistry | Year: 2014

In the last decade, the introduction of the serum-free light-chain (sFLC) assay has been an important advance in the diagnosis and management of plasma cell dyscrasias, particularly monoclonal light-chain diseases. The immunoassay was developed to detect free light chains in serum by using anti-FLC antibodies which specifically recognised epitopes on light chains that were 'hidden' in intact immunoglobulins. Since its introduction in 2001, there have been several publications in the English language literature discussing the clinical utility as well as analytical limitations of the sFLC assay. These studies have highlighted both positive and negative aspects of the assay particularly with regard to its sensitivity and specificity and the technical challenges that can affect its performance. The contribution and significance of the sFLC assay in the management of light-chain myeloma, primary amyloid light-chain (AL) amyloidosis and non-secretory myeloma are well recognised and will be addressed in this review. The aim of this article is to also review the published literature with a view to providing a clear understanding of its utility and limitations in the diagnosis, prognosis and monitoring of plasma dyscrasias including intact immunoglobulin multiple myeloma (MM) and monoclonal gammopathy of unknown significance (MGUS). The increasing interest in using this assay in other haematological conditions will also be briefly discussed. © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.


Lota A.S.,Royal Berkshire Hospital NHS Foundation Trust | Altaf F.,Royal Berkshire Hospital NHS Foundation Trust | Shetty R.,Royal Berkshire Hospital NHS Foundation Trust | Courtney S.,Royal Berkshire Hospital NHS Foundation Trust | And 2 more authors.
Journal of Bone and Joint Surgery - Series B | Year: 2010

Necrotising fasciitis is a rare but severe infection of soft-tissue associated with rapid progression, systemic toxicity and high mortality. Monomicrobial necrotising fasciitis caused by Pseudomonas aeruginosa is exceptionally uncommon with only 12 cases reported in the literature. We describe a fatal case with an atypical presentation in a patient following spinal decompression for a metastasis from prostate cancer. ©2010 British Editorial Society of Bone and Joint Surgery.


Hill G.N.,Royal Berkshire Hospital NHS Foundation Trust | O'Leary S.T.,Royal Berkshire Hospital NHS Foundation Trust
Knee Surgery, Sports Traumatology, Arthroscopy | Year: 2013

Purpose: The use of patient reported outcome measures has gained increasing prominence in reporting surgical outcomes following primary anterior cruciate ligament reconstruction (ACLR). Many peer-reviewed journals now require 'end-result' outcomes in excess of 24 months following surgery for publication. As such, there is less focus on early recovery when the greatest rate of change is experienced and when key rehabilitation decisions are made relating to restricted activity and return to sports. We sought to examine the early recovery profile of patients following primary ACLR, determine the presence of any plateau effect of recovery and establish a source of reference for future study. Method: One hundred and sixty-five patients undergoing primary ACLR were identified from a prospective database. The Knee Injury and Osteoarthritis Outcome Score (KOOS) was recorded pre-operatively and at the 3-, 6- and 12-month follow-up reviews. Mean scores were developed to plot a standard 'recovery profile' of statistical analysis for the presence of any plateau effect. Results: There were significant improvements in all mean KOOS domains at 12 months following ACLR (P < 0.001) and between each recording point (P < 0.003), discounting any plateau effect. Rates of graft rupture and other surgical complications were low (1.2 and 1.8 %). The recovery profile of mean KOOS scores illustrated a reduced rate of recovery over time with sports/recreation and knee-related quality of life KOOS domains demonstrating the greatest sensitivity to change. Conclusions: This study profiles the early recovery of patients following primary ACLR using the KOOS demonstrating continued recovery of function throughout the full first 12 months with no evidence of a plateau effect. The early results in ACLR have not previously been reported in a study of this size and provide important data upon which key rehabilitation decisions can be based. Level of evidence: Therapeutic study-case series with no comparison group, Level IV. © 2012 Springer-Verlag Berlin Heidelberg.


Abbas A.,University of Southampton | Jones H.,Coventry University | Kingston G.T.,Royal Berkshire Hospital NHS Foundation Trust | Zurek A.,Foundation Medicine
British Journal of Radiology | Year: 2011

Malignant peripheral nerve sheath tumours are rare and aggressive softtissue sarcomas of ectomesenchymal origin. These tumours commonly occur in patients with neurofibromatosis Type 1 with a cumulative lifetime risk of 10%. The vast majority of cases present with clinical evidence of a soft-tissue mass with or without features of nerve irritation and loss of function arising from the lesion of origin. The primary presentation of a malignant peripheral nerve sheath tumour with a pneumothorax in the absence of widespread metastatic disease in a patient with no medical or family history of neurofibromatosis has never been reported in the literature. We present a unique case of a systemically well 34-year-old male who presented with clinical evidence of a right-sided pneumothorax. The chest radiograph identified the right-sided pneumothorax and revealed an apical pleural mass that was confirmed by intravenous contrast-enhanced CT of the thorax. The patient was referred for videoassisted thorascopic surgical pleurodesis and biopsy of the lesion. Histopathology analyses confirmed the diagnosis of malignant peripheral nerve sheath tumour. To the best of our knowledge, no such case reports have been published in the literature. A diagnosis of malignant peripheral nerve sheath tumour should be considered as one of the rarer possibilities in patients presenting with pneumothoraces in association with apical pleural lesions. © 2011 The British Institute of Radiology.


Balakrishnan I.,Royal Free Hampstead NHS Trust | Awad-El-Kariem F.M.,East and North Hertfordshire NHS Trust | Aali A.,Ealing Hospital NHS Trust | Kumari P.,Hillingdon Hospital NHS Foundation Trust | And 10 more authors.
Journal of Antimicrobial Chemotherapy | Year: 2011

Background: Temocillin, a β-lactam stable against most β-lactamases [including extended-spectrum β-lactamases (ESBLs) and derepressed AmpC cephalosporinases (dAmpC)], has been suggested as an alternative to carbapenems when Pseudomonas can be excluded. Aims: To assess temocillin clinical and microbiological cure rates (CCR and MCR) in infection caused by ESBL/dAmpC-producing Enterobacteriaceae and the effects of different dosage regimens. Methods: Data were collected retrospectively from patients treated for at least 3 days with temocillin for urinary tract infection (n=42), bloodstream infection (n=42) or hospital-acquired pneumonia (n=8) in six centres in the UK. Results: Data on 92 infection episodes were collected. Overall CCR and MCR were 86% and 84% respectively; ESBL/dAmpC status had no effect. Significantly higher CCR and MCR occurred in patients treated with temocillin at optimal dosage [2 g twice daily or renally adjusted equivalent (ORAE)] compared with those treated with a suboptimal dosage (<2 g twice daily ORAE) (CCR 91% and MCR 92% versus CCR 73% and MCR 63%). This difference was more pronounced in the ESBL/dAmpC-positive subset (CCR 97% and MCR 97% versus CCR 67% and MCR 50%). Conclusions: Clinical and microbiological efficacies of temocillin are unaffected by ESBL/dAmpC production, confirming its potential application as a carbapenem-sparing agent. Both CCR and MCR are optimized by a regimen of 2 g twice daily ORAE in ESBL/dAmpC-positive infection. © The Author 2011. Published by Oxford University Press on behalf of the British Society for Antimicrobial Chemotherapy. All rights reserved.


PubMed | Royal Berkshire Hospital NHS Foundation Trust
Type: Case Reports | Journal: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus | Year: 2013

Children treated for toxoplasma retinochoroiditis may experience a range of severe adverse drug responses. Drug reaction with eosinophilia and systemic symptoms syndrome is a life-threatening idiosyncratic drug reaction with a 10% mortality. We present a case of drug reaction with eosinophilia and systemic symptoms syndrome in a child on standard combination treatment with oral sulfadiazine, pyrimethamine, folinic acid, and steroids for toxoplasma retinochoroiditis. Early clinical recognition and appropriate treatment led to a complete recovery and no longterm sequelae. The parents of children during sulfadiazine treatment should be counseled on the potential significance of nonspecific illness.


PubMed | Royal Berkshire Hospital NHS Foundation Trust
Type: Case Reports | Journal: Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus | Year: 2013

Pyridoxine-dependent epilepsy (PDE) is a cause of neonatal epileptic encephalopathy not previously known to cause ophthalmic disease. We describe the novel observation of a 5-year-old girl with pyridoxine-dependent epilepsy and bilateral cataracts. PDE is the result of mutations in the ALDH7A1 gene encoding antiquitin, an enzyme protective against cellular dehydration and osmotic stress. Accumulating metabolic precursors in PDE have been shown to be cataractogenic in vitro, and experimental pyridoxine deficiency has been associated with lenticular opacities in vivo. The association of ALDH7A1 haploinsufficiency in PDE and congenital cataract may offer insight into the relationship between osmotic stress and fetal cataract development. Bilateral progression of cataracts in this child suggests ongoing metabolic dysregulation within the crystalline lens despite pyridoxine supplementation at doses sufficient to control seizure activity.


PubMed | Royal Berkshire Hospital NHS Foundation Trust
Type: Journal Article | Journal: Knee surgery, sports traumatology, arthroscopy : official journal of the ESSKA | Year: 2013

The use of patient reported outcome measures has gained increasing prominence in reporting surgical outcomes following primary anterior cruciate ligament reconstruction (ACLR). Many peer-reviewed journals now require end-result outcomes in excess of 24months following surgery for publication. As such, there is less focus on early recovery when the greatest rate of change is experienced and when key rehabilitation decisions are made relating to restricted activity and return to sports. We sought to examine the early recovery profile of patients following primary ACLR, determine the presence of any plateau effect of recovery and establish a source of reference for future study.One hundred and sixty-five patients undergoing primary ACLR were identified from a prospective database. The Knee Injury and Osteoarthritis Outcome Score (KOOS) was recorded pre-operatively and at the 3-, 6- and 12-month follow-up reviews. Mean scores were developed to plot a standard recovery profile of statistical analysis for the presence of any plateau effect.There were significant improvements in all mean KOOS domains at 12months following ACLR (P<0.001) and between each recording point (P<0.003), discounting any plateau effect. Rates of graft rupture and other surgical complications were low (1.2 and 1.8%). The recovery profile of mean KOOS scores illustrated a reduced rate of recovery over time with sports/recreation and knee-related quality of life KOOS domains demonstrating the greatest sensitivity to change.This study profiles the early recovery of patients following primary ACLR using the KOOS demonstrating continued recovery of function throughout the full first 12months with no evidence of a plateau effect. The early results in ACLR have not previously been reported in a study of this size and provide important data upon which key rehabilitation decisions can be based.Therapeutic study-case series with no comparison group, Level IV.

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