Donohue syndrome: A review of literature, case series, and anesthetic considerations.

BACKGROUND: Donohue syndrome is a rare autosomal recessive disorder of insulin resistance, causing a functional defect in insulin receptor function, and affecting the ability of the insulin to bind the receptor. Features include severe hyperinsulinism and fasting hypoglycemia, along with severe failure to thrive despite feeding. An accelerated fasting state results in muscle wasting, decreased subcutaneous fat, and an excess of thick skin. A reduced thoracic diameter is accentuated by increased abdominal distension, which impacts on respiratory reserve. Cardiac disease develops early in life, with progressive hypertrophic cardiomyopathy as a result of hyperinsulinism. Prognosis is poor with the majority of patients dying in infancy of sepsis. The aim of this review is to report our experience of providing anesthesia for patients with Donohue syndrome, and inform guidance for safe management of these children, based on a comprehensive literature review. METHODS: A literature search was carried out using PubMed, Medline, and the Cochrane Library, and using the MESH search terms detailed below. Patients were identified by formal request to the department of pediatric endocrinology at Great Ormond Street Hospital. Each patient's notes were searched manually and electronically for both clinical presentation and outcome, and anesthesia records. DISCUSSION: There is currently no published literature relating to anesthetic management of Donohue syndrome. We report a case series of 5 patients with Donohue syndrome who have presented to our institution. This small series of children with this complex disorder demonstrates a clearly increased risk of general anesthesia. Many of the risks relate to restrictive lung disease and abdominal distension which worsens with bag valve mask ventilation and limited respiratory reserve which leads to precipitous desaturation. During induction, a spontaneously breathing technique is recommended. If required, bag valve mask ventilation should be accompanied by constant gastric aspiration. Intubation is challenging, and a difficult airway plan, including a second experienced anesthetist and ENT support, should be in place. These children are predisposed to developing cardiomyopathy and therefore at risk of cardiovascular collapse under anesthesia.

Predicting ICU admissions from attempted suicide presentations at an Emergency Department in Central Queensland.

BACKGROUND: Emergency medicine physicians and psychiatric staff face a challenging job in risk stratifying patients presenting with suicide attempts to predict which patients need intensive care unit admission, hospital admission or can be discharged with psychiatry follow up. AIMS: This study aims to analyse patients who were admitted to the intensive care unit or regular ward for suicide attempt, and the methods they employed in a rural Australian base hospital. METHOD: We conducted a retrospective analysis of patients who presented with suicide attempts to the Rockhampton Base Hospital Emergency Department, Queensland Australia from 1 September 2007 to 31 August 2009. Multivariate logistic regression was undertaken to identify risk factors for ICU and regular ward admission, and predictors of suicide method. RESULTS: There were 570 patients presenting with suicide attempts, 74 of which were repeat suicide attempts. There was a 10- fold increase in the odds of intensive care unit or ICU admission (CI 1.45-81.9, p=0.02) for patients who presented with drug overdose. Increased age (OR=1.02, 95 per cent CI 1.00-1.03, p=0.05), drug overdose (OR=2.69, 95 per cent CI 1.37-5.29, p=0.004), and previous suicide attempt (OR=1.53, 95 per cent CI 1.03-2.28, p=0.03) were significantly correlated with hospital admission. Male patients (OR=2.76, 95 per cent CI 1.43-5.30, p=0.002) and Aboriginal patients (OR=3.38, 95 per cent CI 1.42-8.05, p=0.006) were more likely to choose hanging as a suicide method. CONCLUSION: We identified drug overdose as a strong predictor of ICU admission, while age, drug overdose and history of previous suicide attempts predict hospital admission. We recommend reviewing physician practices, especially safe medication, in suicide risk patients. Our study also highlights the need for continued close collaboration by acute care and community mental health providers for quality improvement.