Physical restraint is a commonly used technique on PICU in the UK. There is considerable variation in clinical practice and current clinical guidelines which are available do not deal specifically with children. Prospective randomized trials would be necessary to fully investigate the role of physical restraints amongst critically ill children.

We report the use of nasal continuous positive airway pressure (nCPAP) for neonatal back transfer of 51 babies. nCPAP may be used to provide safe respiratory support during back transportation of infants. Babies transferred on nCPAP were significantly smaller and of younger postmenstrual age than historical comparators who had to wait for transfer until independent of nCPAP. nCPAP transferred babies spent significantly less time on the intensive care unit.

No abstract

Wolcott–Rallison syndrome (WRS) is a rare autosomal recessive disorder due to mutations in the EIF2AK3 gene. It is characterized by permanent neonatal diabetes mellitus, skeletal dysplasia, liver impairment, neutropenia and renal dysfunction. Liver is the most commonly affected organ and liver failure is the commonest cause of death in this syndrome. The EIF2AK3 gene encodes a transmembrane protein PERK, which is important for the cellular response to endoplasmic reticulum (ER) stress. The absence of PERK activity reduces the ER’s abilities to deal with stress, leading to cell death by apoptosis. On acquiring febrile illness, affected patients suffer from liver injury, which may progress into liver failure and death. Renal involvement is less common and is mainly in the form of functional renal impairment at the advanced stage of the disease. Structural renal anomalies have not been reported in WRS. We report a 6-month-old girl who presented with neonatal diabetes on day 1 of life. Her genetic testing confirmed WRS due to missense mutation in the EIF2AK3 gene (c.2867G > A, p.Gly956Glu). Parents are first-degree cousins and both are heterozygous carriers to the mutation. 2 paternal uncles had the same mutation and died of liver disease at 1 and 14 years of age. Neither had a renal disease. She presented with hematuria during a febrile illness at the age of 5 months. Ultrasound scan showed right ectopic multicystic dysplastic kidney (MCDK). To the best of our knowledge, this is the first patient with WRS who is reported to have an MCDK disease.Learning points:Neonatal diabetes should be considered in babies presenting with early hyperglycemia particularly if there is a family history.Genetic diagnosis in neonatal diabetes enables disease confirmation, genetic counseling and anticipation of potential complications during concomitant situations such as acute illness, trauma or major surgery.There is lack of phenotype–genotype correlation in Wolcott–Rallison syndrome.Structural kidney abnormality, in our case MCDK, can be seen in WRS.

A 43-year-old woman, whose pregnancy was complicated by the presence of a large single solid intra-abdominal fetal mass, was referred from the private sector into our fetal maternal unit at the Corniche Hospital, Abu Dhabi at 36 weeks postmenstrual age.Investigations subsequently confirmed that this mass was a congenital hepatoblastoma, one of the very rare embryonic tumours. The baby had chemotherapy and surgical excision of the tumour. Fifteen months later, the alpha feto-protein levels remain normal and follow-on MRI scans do not show recurrence or any residual disease.To our knowledge, this is the first case of congenital hepatoblastoma in the United Arab Emirates (UAE). In the UAE, the interphase between private health insurance schemes and medical (public and private) care within a growing health economy enhances access to unique services such as cancer treatments within specialised centres.