Wishaw General Hospital

About: Wishaw General Hospital is a healthcare organization based out in Wishaw, Scotland, United Kingdom. It is known for research contribution in the topics: Population & Medicine. The organization has 256 authors who have published 222 publications receiving 4324 citations.

Impact of newborn screening on outcomes and social inequalities in cystic fibrosis: a UK CF registry-based study

Abstract: Background Newborn bloodspot screening (NBS) for cystic fibrosis (CF) was introduced across the UK in 2007 but the impact on clinical outcomes and health inequalities for children with CF is unclear. Methods We undertook longitudinal analyses of UK CF registry data on over 3000 children with CF born between 2000 and 2015. Clinical outcomes were the trajectories of percent predicted forced expiratory volume in one second (%FEV1) from age 5, weight for age and body mass index (BMI) SD-scores from age one, and time to chronic Pseudomonas aeruginosa (cPA) infection. Using mixed effects and time-to-event models we assessed the association of NBS with outcomes and potential interactions with childhood socioeconomic conditions, while adjusting for confounders. Results NBS was associated with higher average lung function trajectory (+1.56 FEV1 percentage points 95% CI 0.1 to 3.02, n=2216), delayed onset of cPA, and higher average weight trajectory intercept at age one (+0.16 SD; 95% CI 0.07 to 0.26, n=3267) but negative rate of weight change thereafter (−0.02 SD per year; 95% CI −0.03 to −0.00). We found no significant association of NBS with BMI or rate of change of lung function. There was no clear evidence of an impact of NBS on health inequalities early in life. Conclusions Children diagnosed with CF by NBS in the UK have better lung function and increased early weight but NBS does not appear to have narrowed early health inequalities.

Novel mutations in the Dubin–Johnson syndrome gene ABCC2/MRP2 and associated biochemical changes

Abstract: A patient with sepsis and jaundice was admitted for diagnosis and treatment. Associated biochemical changes included increased C-reactive protein, conjugated bilirubin and gamma-glutamyltransferase, the duration of which was protracted. High urine coproporphyrin isomer-1 and immunostaining of liver tissue suggested Dubin-Johnson syndrome. DNA sequencing using polymerase chain reaction amplification of the ABCC2 gene revealed the patient to have a compound heterozygous variant of MRP2, a molecule involved in canalicular transport of bilirubin. There was a history of jaundice since infancy.

Hyperamylasaemia and multiple myeloma.

Abstract: This case report describes a 42-year-old Caucasian woman who presented with persistent hyperamylasaemia and no evidence of pancreatic pathology. Further investigations resulted in a diagnosis of light-chain multiple myeloma. Amylase production by epithelial tumours has been well documented but the association with multiple myeloma has only been described in a small number of cases. The link does not appear to be immunoglobulin class-specific but the association with Bence Jones myeloma is unusual. The common features in this group of patients have been extensive extramedullary spread with a high tumour mass and a poor prognosis. This case was similar in that the patient showed very rapid disease activity developing extensive metastatic lesions and treatment ultimately proved unsuccessful. The amylase concentrations have been shown to decrease in response to treatment and increase at times of relapse and it has been proposed that it may be useful as a tumour marker in these patients. This case study adds to the pool of patents with this unusual association.

Fatal and near-fatal encephalopathy with hyponatraemia in two siblings with fluticasone-induced adrenal suppression.

Abstract: AIM: To document previously unreported acute effects of adrenal insufficiency. METHODS: We describe two siblings who presented acutely with hyponatraemia and cerebral oedema following prolonged treatment with high dose inhaled fluticasone. RESULTS: A girl aged 5.5 years presented with vomiting, headache, visual impairment and seizures. She was hyponatraemic but not hypoglycaemic. Her conscious level continued to deteriorate and she died, post mortem examination showing small adrenal glands and cerebral oedema. Four weeks later her 7-year-old brother presented with similar symptoms. Assessment showed hyponatraemia with cerebral oedema. His illness responded to intensive care. A diagnosis of adrenal insufficiency was made retrospectively in both cases. The siblings had been receiving Fluticasone propionate (FP) in doses of up to 2000 microg/day for several years. CONCLUSION: We believe that the hyponatraemia and cerebral oedema was related to cortisol deficiency, leading to impaired excretion of water. We emphasize the need for careful cerebral monitoring in acute adrenal insufficiency presenting with impaired consciousness.