Rotavirus Gastroenteritis is Associated with Increased Morbidity and Mortality in Children with Inherited Metabolic Disorders.

Rotavirus is the leading cause of infantile diarrhoea worldwide in children <5 years1. Although mortality rates are low in Ireland, certain populations are more susceptible to the associated morbidity and mortality of infection. A retrospective chart review of 14 patients with confirmed IMDs who were admitted to Temple Street Children's Hospital between 2010 to 2015 with rotavirus infection were compared with 14 randomly selected age matched controls. The median length of stay was 7 days (SD25.3) in IMD patients versus 1.5 days (SD 2.1) in the controls. IV fluids were required on average for 4.5 days (range 0-17) in IMD patients versus 0.63 days (range 0-3) in controls. This report highlights the increased morbidity of rotavirus infection in patients with IMD compared to healthy children. This vulnerable population are likely to benefit from the recent introduction of the rotavirus oral vaccination in October 2016.

A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders.

Mitochondrial disorders are a clinically and biochemically diverse group of disorders which may involve multiple organ systems. General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders, and there is little guidance for anaesthetists and other clinicians regarding the optimal anaesthetic agents and perioperative management to provide to patients with mitochondrial disease[15]. The aim of this review was to document adverse events and perioperative complications from GA in patients with genetically confirmed mitochondrial disorders. A retrospective chart review of patients with genetically confirmed mitochondrial disorders who had undergone GA was undertaken. The indication for GA, anaesthetic agents utilised, length of admission and post anaesthetic complications were documented and analysed. Twenty-six patients with genetically proven mitochondrial disease underwent 65 GAs. Thirty-four (52%), received propofol as their induction agent. Thirty-three (51%) patients received sevoflurane for the maintenance of anaesthesia, while 8 (12%) received isoflurane and 24 (37%) received propofol. The duration of most GAs was short with 57 (87%) lasting less than 1 h. Perioperative complications occurred in five patients while under GA including ST segment depression, hypotension and metabolic acidosis in one. All five patients were stabilised successfully and none required ICU admission as a consequence of their perioperative complications. The duration of hospital stay post GA was <24 h in 25 (38%) patients. CONCLUSION: No relationship between choice of anaesthetic agent and subsequent perioperative complication was observed. It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique. What is Known: • General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders. • There is a great diversity in the anaesthetic approaches undertaken in this cohort, and little guidance exists for anaesthetists and other clinicians regarding the optimal anaesthetic agents and perioperative management to provide to patients with mitochondrial disease. What is New: • In this study of 26 patients with genetically confirmed mitochondrial disease who underwent 65 GAs, no relationship between choice of anaesthetic agent and subsequent perioperative complication was observed • It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique.

Type 1 Tyrosinaemia.

Tyrosinaemia type 1 (TYR1, OMIM# 276700) is a rare autosomal recessive disease that results from an enzyme defect that leads to a deficiency in fumarylacetoacetase (FAH)1. We present 3 cases of TYR1 in the Irish population over a 9 year period, the only cases known to have been diagnosed in Ireland since 1989. The common presenting symptom was hypoglycaemia and the diagnosis was made by the identification of the pathognomonic biomarker succinylacetone on urine organic acid analysis. We discuss the clinical presentation, biochemical and genetic results including one novel mutation. We also highlight the importance of early initiation of Nitisinone (NTBC), which reduces the complications of TYR1 and the incidence of liver transplantation in this population2.

Complete mesocolic resection and extended lymphadenectomy for colon cancer: a systematic review.

AIM: Complete mesocolic excision (CME) and extended lympha-denectomy (EL) have been proposed as safe procedures for improving colon cancer survival outcomes. The aim of this study was to evaluate the evidence regarding oncological outcomes, morbidity and mortality after such techniques for colon cancer. METHOD: A systematic review of the literature was conducted to evaluate evidence regarding oncological outcomes, morbidity and mortality after CME or EL. Three major databases (PubMed, MEDLINE and the Cochrane Library) were searched. The review included original articles reporting outcomes after CME or EL from January 1950 to July 2012. RESULTS: Twenty-one, predominately retrospective, studies involving 5246 patients (mean age 68.2 years, 56.5% men) were included. Reporting of outcomes was inconsistent. Median follow up was 60 months. The operative mortality rate was 3.2% and the cumulative morbidity rate was 21.5%. The weighted mean local recurrence rate and the 5-year overall and disease-free survival rates were 4.5%, 58.1% and 77.4%, respectively. CONCLUSION: The available data for CME and EL have numerous fundamental limitations that prohibit adoption. Contemporary controlled studies are required before universal recommendation.

Omentoplasty to assist perineal defect closure following laparoscopic abdominoperineal resection.

AIM: This technical note describes laparoscopic production of a well vascularized, omental flap of adequate size to fill the pelvic floor defect in the course of laparoscopic abdominoperineal resection (LAPR). METHOD: The omentum is laparoscopically mobilized and transposed to the pelvis following full LAPR in three discrete stages. RESULTS: Laparoscopic omental mobilization, transfer and buttressing of a primary perineal repair reduces pelvic dead space and facilitates closure following LAPR with minimal additional operative time or complications and a potential reduction in perineal wound associated morbidity. CONCLUSION: Laparoscopic omental mobilization is technically feasible and provides a safe method to aid perineal wound closure.

Omental pedicle flaps following proctectomy: a systematic review.

AIM: Abdominoperineal excision (APR) for cancer carries significant morbidity of the perineal wound. An omental pedicle graft has been used to fill the pelvis and limit attendant complications after radical extirpation of the anorectum. A review of the literature was conducted to determine whether omentoplasty following APR reduces perineal wound complications. METHOD: Three major databases (PubMed, MEDLINE and the Cochrane Library) were searched. The review included original articles reporting outcomes after APR and omentoplasty from January 1950 to July 2012. RESULTS: Fourteen studies involving 891 patients (mean age 61 years, 59.8% men) were included. Median follow-up was 13.5 months. A variety of omentoplasty techniques added a median of 20 min to the operating time. The mean rate of primary wound healing was 66.8%, time to wound healing 24 days and weighted mean wound infection rate 14.4% with omentoplasty compared with 50.1%, 79 days and 18.5% in patients having no omentoplasty. CONCLUSION: Omental mobilization, transfer and buttressing of primary perineal repair following proctectomy reduces perineal wound morbidity with minimal additional operating time or flap-associated morbidity.

Structured care of diabetes in general practice: a qualitative study of the barriers and facilitators.

This qualitative study explored general practitioner's and practice nurse's perceptions of barriers and facilitators to the proposed transfer of diabetes care to general practice. Qualitative data were collected through five focus groups. Participants included GPs (n = 55) and practice nurses (n = 11) representing urban (44%), rural (29%) and mixed (27%) practices, in the Irish Mid-West region. Barriers and facilitators were mentioned 631 times (100%). Barriers were mentioned 461 times (73%), facilitators 170 times (27%). The most frequently identified barriers were lack of financial incentive (119/631; 19%), lack of access to secondary resources (93/631; 15%), lack of staff and increased workload (59/631; 9%) and time constraints (52/631; 8%). Identified facilitators were access to secondary care (49/631;7.8%), the holistic nature of general practice and continuity of care (48/631;7.6%). Although many are enthusiastic, there remains significant reluctance among GPs and practice nurses to take responsibility for diabetes care without addressing these barriers.

Beware the ischiorectal abscess.

INTRODUCTION: Ischiorectal abscesses have been shown to form sinuses with various deep structures but continuity with the spinal canal is extremely rare. PRESENTATION OF CASE: A previously healthy sixty-five year old man presented emergently with rectal pain, weight loss and recurrent severe tension headaches. He had systemic sepsis and resultant coagulapathy (INR 3.4) which precluded investigation of neurological symptoms by lumbar puncture. MRI rectum demonstrated a well circumscribed fluid collection with direct connection to the spinal canal and containing meningeal tissue. It extended inferiorly to the right ischiorectal fossa and abutted the natal cleft. A radiological diagnosis of ischiorectal abscess which had become continuous with a previously existing anterior sacral myelomeningocoele (ASM) was made. He was treated with broad spectrum antibiotics and a neurosurgical opinion was sought. He remained clinically unwell (septic and coagulopathic) until the abscess fistulated through the perianal skin, draining pus mixed with clear fluid (likely CSF) at which point he improved systemically. DISCUSSION: Few general surgeons would be faced with acute management of complicated ASM. Paucity of literature made application of evidence based medicine difficult. In fit healthy patients surgery is the mainstay of treatment as myelomengingoceles do not regress spontaneously. Conservative management is associated with up to 30% mortality (largely due to bacterial meningitis). The patient in this case was adamant that he did not consent to definitive surgical intervention. CONCLUSION: This case highlights challenges encountered in the management of complicated ASM in a general hospital.

The effects of remoxipride and chlorpromazine on eye movements and psychomotor performance in healthy volunteers.

Fifteen healthy male volunteers received single doses of 100 mg immediate release remoxipride (IR), 150 mg controlled release remoxipride (CR), 50 mg chlorpromazine (CPZ), 2 mg lorazepam (LZ), and placebo in a randomised, five-period cross-over study. Both saccadic (SEM) and smooth pursuit eye movements (SPEM) as well as a battery of psychomotor performance tests were assessed at 1.5-h intervals over 9 h following drug administration. The areas under the response-time curves and the maximum effect during the study period were analysed by analysis of variance. The most consistent impairments were produced by LZ. The neuroleptics caused impairments to SEM, and tended to impair critical flicker fusion, continuous attention and both paced and unpaced versions of the digit-symbol substitution test as well as subjective measures of sedation. Only LZ impaired SPEM. Neither paced nor unpaced psychomotor tests distinguished between neuroleptics and benzodiazepines. The low therapeutic doses of IR and CR produced similar impairments to a sub-therapeutic dose of CPZ. Selectivity of pharmacological action does not appear to predict selectivity of effect on psychomotor function.

The strength of lining materials under constraint.

The compressive strength of the lining material Dycal was found to increase when peripheral constraints were applied to its deformation. The stress required to produce fracture under such circumstances increased with the degree of constraint. In each case, by summing all the stresses in a yield criterion (Von Mises) the strength is understood. Materials previously used with caution because of low (compressive) strength may now be used with more confidence when external support and confinement exists.