SummaryWiedemann–Steiner Syndrome (WSS) is a rare condition characterised by short stature, hypertrichosis of the elbow, intellectual disability and characteristic facial dysmorphism due to heterozygous loss of function mutations in KMT2A, a gene encoding a histone 3 lysine 4 methyltransferase. Children with WSS are often short and until recently, it had been assumed that short stature is an intrinsic part of the syndrome. GHD has recently been reported as part of the phenotypic spectrum of WSS. We describe the case of an 8-year-old boy with a novel heterozygous variant in KMT2A and features consistent with a diagnosis of WSS who also had growth hormone deficiency (GHD). GHD was diagnosed on dynamic function testing for growth hormone (GH) secretion, low insulin-like growth factor I (IGF-I) levels and pituitary-specific MRI demonstrating anterior pituitary hypoplasia and an ectopic posterior pituitary. Treatment with GH improved height performance with growth trajectory being normalised to the parental height range. Our case highlights the need for GH testing in children with WSS and short stature as treatment with GH improves growth trajectory.Learning points:Growth hormone deficiency might be part of the phenotypic spectrum of Wiedemann–Steiner Syndrome (WSS).Investigation of pituitary function should be undertaken in children with WSS and short stature. A pituitary MR scan should be considered if there is biochemical evidence of growth hormone deficiency (GHD).Recombinant human growth hormone treatment should be considered for treatment of GHD.

The purpose of this study is to determine the effect of illicit drug and alcohol dependencies on mortality, length of stay, and complications in patients who have been supported with veno-venous extracorporeal membrane oxygenation (VV-ECMO) following respiratory failure not responsive to conventional methods of ventilation. 584 VV-ECMO referrals received at Wythenshawe Hospital were reviewed for evidence of drug dependency. 13 patients were identified as being drug-dependent and having undergone treatment with VV-ECMO. A matched cohort of 13 non-drug-dependent patients was identified using date of birth, pre-ECMO Murray Score, and primary diagnosis. The outcomes were compared. 19 more complications were found amongst the drug-dependent patients compared with the non-drug-dependent cohort (39 vs 20). A mean difference of 1.46 complications per patient was calculated (p = 0.005). Mortality after 180 days was reported in 4 of the drug-dependent patients, compared with one in the matched cohort. Length of stay on ECMO was increased on average by 1.93 days amongst the drug-dependent patients (p = 0.557); however, the sample size was not great enough to achieve statistical significance. Patients with drug dependencies undergoing VV-ECMO have more complications when compared with a cohort of patients with no proven or suspected drug dependencies. Differences in morbidity and mortality were not statistically significant.

Background Exposure to SARS-CoV-2 was widespread in hospitals during 2020. The risk of infection after in-hospital exposure has not yet been quantified and effective strategies to prevent it remain unclear. Methods All incidences of patient-to-patient exposure to SARS-CoV-2 on non-COVID wards between October and December 2020 at a UK hospital trust were identified. Patient contacts were traced, and data collected on SARS-CoV-2 testing, symptoms, and outcomes. Factors associated with acquiring infection and mortality were investigated. Results Of 575 patients exposed, 118 (19.5%) tested positive within 14 days of their exposure, with secondary attack rates (SAR) ranging from 0 to 72%. 68.6% (81/118) of secondary cases had not been in the same bay as the index case. For exposed patients, sharing a bay with the index case and having spent longer on the ward with them were associated with acquiring infection (ORs of 3.8, 95% CI: 1.89, 7.74, and 1.08, 95% CI: 1.01, 1.15 respectively). 71% of secondary cases tested positive while asymptomatic and 94.6% had tested negative earlier in their admission. Conclusions This is the first study to describe the outcomes of a cohort of patients exposed to COVID-19 in hospital. Exposure to COVID-19 in hospital commonly leads to transmission that is not confined to the index case’s bay. This study confirms that asymptomatic testing is important and suggests that an increased frequency of testing may be beneficial. Moreover, we provide factors that can be used to identify the contacts at the greatest risk of acquiring infection.