Is respiratory involvement in microvillus inclusion disease a part of multisystem disease? Dr Hemant Bhavsar 1, Dr Satish Rao 2, Dr Katharine Foster 2, Dr Girish Gupte 2 1 Leicester Royal Infirmary, Leicester UK 2 Birmingham Children’s Hospital NHS Trust, Birmingham UK Author’s Introduction Method Results Conclusions Microvillus inclusion disease (MVID) is a congenital disorder with lack of microvilli and intracellular vacuole formation leading to irreversible intestinal failure. In recent years, improvement in parenteral nutrition (PN) and availability of Intestinal transplantation (ITx) has offered hope for long term survival. Liver dysfunction, Renal Fanconi Syndrome and Diabetes are described to be associated with MVID. Improved survival in these patients has offered an opportunity to study other likely systemic manifestations in MVID. Retrospective review of case notes and radiological investigations in patients referred with MVID for ITx over 20 years ( ). Demographic details, management of MVID and evidence of chronic respiratory signs and symptoms in these patients were noted. Radiological findings were confirmed by paediatric radiologist. Nineteen cases were included. (10 females and 9 males). Ten underwent transplantation (3 liver and ITx, 7 isolated ITx). Median age at transplantation was 38 months (8-55). 6/10 died post-transplantation at median age of 46.5 months (12-81). 8/9 died pre-transplantation at median age of 26.5 months (3-83). One (late onset) weaned off PN at 11 years. Seven patients (3 transplant survivors, one late onset MVID, 2 post transplant and one pre- transplant deaths) developed chronic respiratory symptoms (cough and haemoptysis) with chest radiographs showing atelectasis and bronchial wall thickening. Three had chest Computer Tomography (CT) done which confirmed bronchiectatic changes. In MVID, respiratory system involvement has not been described previously. We wonder if there is an underlying predisposition to respiratory system involvement in MVID as a part of the multisystem disorder. We speculate that this could be due to loss of ciliary function by mechanism similar to loss of microvilli on enterocytes. Clinicians following up children with MVID should be vigilant about the potential for respiratory co-morbidity. E-Poster No 194 Objective To describe the prevalence of respiratory complications in patients with MVID with or without ITX Table 1: MVID patients who developed respiratory signs and symptoms Acknowledgement We would like to thank all the referral Hospitals and paediatric gastroenterology colleagues within UK and Europe for the help in management of MVID children who have been referred for ITx NoAge (years)SexTransplantation statusCXR/ CT chest findings 15Male ITx (at 16 months) CT: Bronchiectasis 28FemaleITx (at 51 months) CXR & CT: Mediastinal lymphadenopathy, areas of atelectasis both lungs, nodularity within lungs 39Male ITx (at 40 months) CT: Bronchiectasis 415Female Not transplanted (Late onset MVID) CXR: marked bilateral perihilar bronchial wall thickening extending into both lower lobes medially 5Died at 7FemaleITx (at 4 years)CT: Bronchiectasis 6Died at 1MaleLITx (at 12 months)CXR: persistent right upper lobe changes 7Died at 7FemaleNot transplantedCXR: extensive collapse/ consolidation CXR of a child with MVID during ITX assessment CT chest atelectasis