Immunodeficiency, centromeric region instability and facial anomalies (ICF) syndrome diagnosed in an adult who is now a long-term survivor.

We describe a 42-year-old British man of Indo-Caribbean origin with immunodeficiency, centromeric region instability and facial anomalies (ICF) syndrome. Most patients with ICF syndrome die of infection at a young age, usually in the first or second decade of life. The patient was born 3.5 weeks premature to non-consanguineous parents. He had a mild bird-like face abnormality, but had no other congenital malformations, cognitive impairment or developmental delays. He had recurrent ear and chest infections during childhood and developed bronchiectasis. Investigations revealed IgG, IgA and IgM deficiencies with a normal lymphocyte count and normal T cell proliferation to in vitro mitogenic stimulation. Following several unsuccessful attempts to make a diagnosis during childhood, a recent chromosomal analysis showed centromeric region instability of chromosomes 1 and 16, diagnosing ICF syndrome. The patient receives immunoglobulin replacement for hypogammaglobulinameia and has chest physiotherapy and antibiotics for bronchiectasis. Recently, he developed liver cirrhosis of unknown cause.

Optimizing secondary prevention: Statin prescribing across East and West London in accordance with NICE guidelines.

OBJECTIVES: Statins are a well-known primary and secondary prevention drug for cardiovascular disease and NICE guidelines have been issued to identify key indicators for their use. An audit looking into statin prescribing for medical inpatients was carried out in two geographically distinct London hospitals. DESIGN: A prospective inpatient audit of medical prescription charts was performed. Blood results were reviewed for the inpatients during their admission to identify any contraindications for statin usage (rhabdomyolysis). The medical notes were also reviewed for patient refusal of statin therapy. SETTING: The study was carried out at two distinct hospitals in London. Whipps Cross University Hospital (WCUH) and Chelsea & Westminster Hospital (CWH) are located in East London and West London, respectively. Acute medical, surgical, obstetrics and gynaecology, paediatric and palliative wards were excluded. PARTICIPANTS: A total of 309 inpatient medical notes and prescription chart data were collected from WCUH (n = 211) and CWH (n = 98). MAIN OUTCOME MEASURES: High percentage of hospitalized patients are not prescribed statins despite clear clinical indications for their use. Regardless of geographical and socioeconomic factors between hospitals, statin prescribing remains suboptimal. RESULTS: The patient demographics in both hospitals were very similar; the mean age at WCUH was 78 ± 15 1SD while at CWH the mean age was 74 ± 15 1SD. The results showed that approximately one-third of patients (30% at WCUH and 33% at CWH) had at least one indication for statin therapy according to NICE guidelines and yet they were not prescribed a statin. Ten percent of patients at WCUH and 13% of patients at CWH had ischaemic heart disease (IHD) and yet were not prescribed statins. CONCLUSION: Statin prescription is often overlooked in secondary care with patients being discharged without the appropriate assessment (NICE guidelines), which subsequently means repeat prescriptions are not provided by the GP. This study is the first to show that this problem is not due to resources or geography, but is inherent within the NHS system. Consequently, a revised prescription chart checking system has been suggested for pharmacists and junior doctors.