High risk of septic complications following surgery for Crohn's disease in patients with preoperative anaemia, hypoalbuminemia and high CRP.

AIM: Bowel resection in Crohn's disease still has a high rate of complications due to risk factors including immune suppression, malnutrition and active inflammation or infection at the time of operating. In this study, we use serological levels and inflammatory markers to predict the potential of complications in patients undergoing resections for complicated Crohn's disease. METHODS: All patients undergoing laparoscopic bowel resection for Crohn's disease from 5th of November 2012 to 11th of October 2017 were included in this retrospective observational study. Patients were divided into 4 groups scoring 0, 1, 2 or 3 depending on their pre-operative haemoglobin concentration (Hb), C-reactive protein (CRP) and albumin (Alb) where 1 point was given for an abnormal value in each as detailed in the definitions. They were then grouped into a low risk group comprised of those scoring 0 and 1, and a high risk group for those scoring 2 and 3 and data was collected to compare outcomes and the incidence of septic complications. RESULTS: Seventy-nine patients were included. Eleven (13.9%) and 2 (2.5%) patients had 2 or 3 abnormal values of CRP, Alb and Hb and were categorized as high risk. High risk patients had a significantly higher rate of post-operative septic complications (30.7%) compared with low risk patients (10.6%) p value < 0.0001. CONCLUSION: Pre-operative CRP, haemoglobin and albumin can serve as predictors of septic complications after surgery for Crohn's disease and can therefore be used to guide pre-operative optimisation and clinical decision-making.

The effectiveness of long-term dietary therapy in the treatment of adult Refsum disease.

OBJECTIVE: To evaluate the long-term effectiveness of dietary therapy with regular dietetic reinforcement for adult Refsum disease. METHODS: Retrospective case note analysis of records of plasma phytanic acid and hospital admission of 13 patients with adult Refsum disease who attended the specialist centre and repeatedly received dietary instruction for a minimum of 10 years. RESULTS: Patients undergoing review had attended for 11-28 years totalling 237 years. Median baseline phytanic acid concentrations at presentation were 1631 (370-2911) micromol/l and declined by 89+/-11% to 85 (10-1325) micromol/l. Levels of phytanic acid were completely normalised (<30 micromol/l) in 30%; partially normalised (30-300 micromol/l) in 50% and remained >300 pmol/l in 15%. The time required for phytanic acid levels to halve was 44.2+/-15.9 months in patients compliant with diet. No patient required admission or plasmapheresis/apheresis during this period for acute neuro-ophthalmological complications despite occasional spikes in phytanic acid levels attributable to intercurrent illness, surgery, sudden weight loss or psychological illness. INTERPRETATION: Dietary modification with regular reinforcement in Adult Refsum Disease can significantly reduce phytanic acid levels with time.

Adult Refsum disease: a form of tapetoretinal dystrophy accessible to therapy.

Adult Refsum disease is characterized by an elevated plasma phytanic acid level and high concentrations of phytanic acid in a variety of tissues. Besides tapetoretinal degeneration, additional symptoms are anosmia, skeletal malformations, chronic polyneuropathy, cerebellar ataxia, sensorineural hearing loss, ichthyosis, and cardiac abnormalities. A diet low in phytanic acid ameliorates polyneuropathy and ataxia and slows or even stops the other manifestations. In order to be able to apply dietary therapy, as many patients as possible (even better if all of them are) have to be identified at an early stage. The ophthalmologist plays a crucial role in achieving this goal because of the early manifestation of the tapetoretinal degeneration.