A Primer on Home Infusion Administration Methods.

Home infusion therapy is a rapidly growing field in healthcare, allowing patients to receive postacute care at home at a fraction of the cost of an inpatient stay. Understanding the various drug delivery systems can facilitate a more seamless discharge to home with improved patient outcomes. Herein we review several home infusion methods of administration utilized to transition patients from hospital to home care for a variety of therapies.

Coexistent sarcoidosis and lymphangioleiomyomatosis in a patient with cystic lung disease.

A 45-year-old lady presented acutely with pleuritic chest pain, haemoptysis, and dyspnoea. Her background was significant for a 1.4 cm renal angiomyolipoma, and she was an ex-smoker without any relevant family history. A computed tomography (CT) pulmonary angiogram was negative for a pulmonary embolism but demonstrated diffuse cystic change throughout both lungs. A bronchoscopy confirmed a normal endobronchial tree, and pulmonary function tests demonstrated moderate airways obstruction, with reversibility and a normal diffusion capacity for carbon monoxide (DLCO). A video-assisted thoracoscopic surgery (VATS) lung biopsy showed non-caseating granulomas, and serum angiotensin converting enzyme (ACE) was elevated consistent with a diagnosis of pulmonary sarcoidosis. Further sectioning indicated focal areas that stained positive for Human Melanoma Black 45 (HMB-45), confirming lymphangioleiomyomatosis (LAM). A diagnosis of cystic lung disease secondary to coexistent sarcoidosis and LAM was made.

Lithium and nephrotoxicity: Unravelling the complex pathophysiological threads of the lightest metal.

While lithium remains the most efficacious treatment for bipolar disorder, it can cause significant nephrotoxicity. The molecular mechanisms behind both this process and the development of nephrogenic diabetes insipidus still remain to be fully elucidated but appear to involve alterations in glycogen synthase kinase 3 signalling, G2 cell cycle progression arrest, alterations in inositol and prostaglandin signalling pathways, and dysregulated trafficking and transcription of aquaporin 2 water channels. The end result of this is a tubulointerstitial nephropathy with microcyst formation and relative glomerular sparing, both visible on pathology specimens and increasingly noted on non-invasive imaging. This paper will elucidate the current evidence pertaining to the pathophysiology of lithium induced nephrotoxicity.

Fibrillary glomerulonephritis: An apparent familial form?

Fibrillary glomerulonephritis is a rare cause of glomerulonephritis characterized by non-amyloid fibrillary deposits of unknown aetiology. It is generally considered idiopathic but may be associated with secondary causes such as monoclonal gammopathy, hepatitis B and C infections, autoimmune diseases and malignancies. We report two Australian families with apparent familial fibrillary glomerulonephritis inherited in an autosomal dominant pattern, and postulate the existence of a primary familial entity. Family 1 consists of an affected father and daughter; the daughter progressed to end-stage renal failure within 18 months of diagnosis, despite immunosuppressive therapy. The father, however, remains stable at 10 months follow up. Family 2 comprises an affected mother and son; the mother commenced haemodialysis 5 years after diagnosis and subsequently underwent successful renal transplantation. The son is presently stable at last follow-up after 5 years. A further review of the second family history reveals a third family member (maternal father) dying of 'Bright's disease'. We describe their histopathology, clinical progression and treatment outcomes, and provide a review of the current understanding of this heterogeneous condition that is associated with poor renal outcomes.

Setting up and utilizing a service for measuring perioperative transcranial motor evoked potentials during thoracoabdominal aortic surgery and thoracic endovascular repair.

OBJECTIVES: Paraplegia is a complication that may occur following surgery or endovascular stenting of thoracic and thoracoabdominal aortic pathology. Measuring transcranial motor evoked potentials (tcMEPs) has been shown to provide a reliable measure of spinal cord function during such procedures allowing interventions to protect cord function. In the spirit of sharing experience and eliminating the learning curve for others, this manuscript describes our experience of setting up a service for tcMEP monitoring as well as the documents and algorithms for measuring, recording and acting on the patient data, the so-called 'MEP Pathway'. METHODS: Recording and interpretation of tcMEP during thoracoabdominal aortic intervention requires training of staff and close team working in the operating theatre and postoperative intensive care unit. Providing consistent, reliable, specific and sensitive information on spinal cord function and its safe and effective use to alter patient outcomes requires a protocol. The MEP pathway was developed by medical and paramedical staff at our institution based on clinical experience and literature reviews over a 1-year period (2012-2013). RESULTS: The tcMEP pathway comprises six documents that guide staff in: (a) assessing suitability of patients, (b) setting up hardware, (c) preparing algorithms for management, (d) documenting intervention (left heart bypass, cardiopulmonary bypass or endovascular stenting) as well as (e) documenting postoperative intensive care processes. CONCLUSIONS: The tcMEP pathway acts as a guide for safe introduction and use of tcMEPs in thoracoabdominal aortic interventions. tcMEP-led guidance of intraoperative and postoperative management in thoracic aortic surgery is an important adjunct in caring for this patient group.

Liverpool Aortic Surgery Symposium V: New Frontiers in Aortic Disease and Surgery.

Aortic aneurysm disease is a complex condition that requires a multidisciplinary approach in management. The innovation and collaboration among vascular surgery, cardiothoracic surgery, interventional radiology, and other related specialties is essential for progress in the management of aortic aneurysms. The Fifth Liverpool Aortic Surgery Symposium that was held in May 2013 aimed at bringing national and international experts from across the United Kingdom and the globe to deliver their thoughts, applications, and advances in aortic and vascular surgery. In this report, we present a selected short synopsis of the key topics presented at this symposium.

Blunt aortic injury secondary to fragmented tenth thoracic vertebral body.

We present a case of blunt traumatic aortic laceration following a motor vehicle crash. The aortic laceration was 4.5 cm above the coeliac axis and occurred because of an unstable tenth thoracic vertebral body. Open surgery was considered high risk, whereas an endovascular approach with an endoprosthesis placed at the exact anatomic location of the laceration was advocated.

Abnormal Processing of Autophagosomes in Transformed B Lymphocytes from SCARB2-Deficient Subjects.

Mutations of the intrinsic lysosomal membrane protein SCARB2 cause action myoclonus-renal failure syndrome (AMRF syndrome), a rare disease characterized by renal and neurological manifestations. In this study, examination of Cos7 cells transfected with SCARB2 cDNA derived from two patients with AMRF syndrome showed that the resultant protein was truncated and was not incorporated into vesicular structures, as occurred with full-length SCARB2 cDNA. Mutant SCARB2 protein failed to colocalize with lysosomes and was found in the endoplasmic reticulum or the cytosol indicating a loss of function. Cultured skin fibroblast and Epstein-Barr virus-transformed lymphoblastoid B cell lines (LCLs) were created from these two patients. Despite the loss of SCARB2 function, studies with lysosomal-associated membrane protein (LAMP) 1 and LAMP2 demonstrated normal lysosomal numbers in fibroblasts and LCLs. Immunofluorescence microscopy using anti-LAMP1 and anti-LAMP2 antibodies also showed normal lysosomal structures in fibroblasts. There was no change in the morphology of fibroblasts examined by electron microscopy compared with cells from unaffected individuals. By contrast, LCLs from individuals bearing SCARB2 mutations had large intracellular vesicles that resembled autophagosomes and contained heterogeneous cellular debris. Some of the autophagosomes were seen to be extruding cellular contents into the media. Furthermore, LCLs had elevated levels of microtubule-associated protein light chain 3-II, consistent with increased autophagy. These data demonstrate that SCARB2 mutations are associated with an inability to process autophagosomes in B lymphocytes, suggesting a novel function for SCARB2 in immune function.

A Single-Stage Repair of Arch and Descending Thoracic Aortic Aneurysms Using Jotec E-vita Open Plus Hybrid Stent Graft Combined With Antegrade Deployment of Thoracic Endograft.

We report a unique case of a 63-year-old female with extensive peripheral vascular disease who underwent a single-stage surgical repair of the aortic arch and descending thoracic aortic aneurysm utilizing the Jotec E-vita Open Plus hybrid stent graft system combined with antegrade deployment of a thoracic endograft via a median sternotomy.

Polypoid pseudomyxoma of ascending aorta after replacement with a Dacron graft.

We report a 58-year-old man who had a multi-lobulated pseudomyxomatous lesion in his ascending aorta 6 months after his root and ascending aorta was replaced by a Dacron graft.

The safe use of spinal drains in thoracic aortic surgery.

Paraplegia is a devastating complication which may occur following surgery on the thoracic aorta. The use of a cerebrospinal fluid drain (CSFD) has helped reduce the incidence of neurological deficit; however, the management of patients with a CSFD postsurgery requires nurses and doctors to have expertise and awareness of the associated complications. The National Patient Safety Agency (UK) has highlighted a number of cases involving inadvertent spinal injections throughout the UK National Health Service (NHS). To this end we have introduced a protocol or 'care bundle' for safe CSFD care as well as drain management. The protocol was developed by medical and nursing staff at our institution based on clinical experience and literature reviews over a two-year period (2008-2010). Interventions undertaken during the development of the protocol included discussion with the UK National Patient Safety Agency (NPSA). Content of the protocol was reviewed by internal regulatory bodies within the hospital prior to ratification and general dissemination. Clear guidance is given within the policy on the standards expected when caring for the line and managing drainage according to agreed parameters of spinal cord perfusion pressure. The protocol constitutes five documents which guide staff in the care of CSFD, its routine management, documentation and interventions necessary once neurological deficit is detected. Document 1 which is a checklist, communication tool and aide-memoire was developed to ensure effective management, when the patient arrives in intensive care unit (ICU) from theatre. Document 2 ensures that early detection of a neurological deficit is noted and with Document 3 is acted upon immediately to reverse the injury. Document 4 provides information on the safe administration of analgesia via the spinal drain and has reference to the Glasgow Coma Scale. Document 5 is a bespoke observation chart for documenting CSFD pressure and cerebrospinal fluid drainage. In conclusion, the protocol acts as a guide for safe management of the CSFD and directs staff in reacting to detection of neurological deficit.

Mitral valve involvement in Wegener's granulomatosis.

Wegener's granulomatosis is an autoimmune condition resulting in the granulomatous vasculitis of small-to-medium-sized vessels, and is characterized by granulomatous lesions in the renal and respiratory systems. Cardiac involvement in Wegener's granulomatosis has been previously reported. However, involvement of cardiac valves is extremely rare. We present a patient with Wegener's granulomatosis with an extensive mitral mass extending to the aortic valve.

True aneurysm of a Dacron tube graft 19 years after repair of coarctation of the aorta.

We report a 31-year old woman who presented with acute onset of shortness of breath 19 years after multiple repairs of a preductal coarctation of the aorta using a Dacron tube graft. Imaging studies showed an aneurysm had developed in the tube graft. The aneurysmal tube graft was replaced during an open repair.

Successful reversal of delayed paraplegia associated with chronic type A aortic dissection using a spinal drain.

Delayed neurological deficit (DND) following thoracic and thoracoabdominal aortic aneurysm repair refers to any neurological deficit in a patient who is evaluated to be neurologically intact postoperatively, but develops neurological problems several hours or days later which can be reversed if identified and treated immediately. We report a rare case where cerebrospinal fluid drainage reversed DND that happened 18 months following type A dissection repair.

Avoiding the use of helmet continuous positive airway pressure after surgery on thoracic aorta.

Spinal cord ischaemia remains a major problem after surgery of the thoracic aorta. Early detection and avoidance of systemic hypotension can prevent permanent neurological damage. We report a rare case that developed a temporary paraplegia postoperatively, associated with the use of helmet continuous positive airway pressure.

Effect of peri-operative red blood cell transfusion on 30-day and 1-year mortality following coronary artery bypass surgery.

OBJECTIVE: The purpose of this study was to examine the effect of peri-operative red blood cell (RBC) transfusion on 30-day and 1-year mortality following coronary artery bypass grafting (CABG). METHODS: We retrospectively analysed 3024 consecutive patients who underwent isolated CABG between January 1999 and December 2001. Patient records were linked to the National Strategic Tracing Service, which records all mortality in the UK. Thirty-day and 1-year mortality were derived from Kaplan-Meier curves. Confounding variables were controlled for by constructing a propensity score for the probability of receiving a transfusion from core patient characteristics including the lowest recorded laboratory haemoglobin (LL Hb) from a clinical chemistry database (C statistic 0.81). The propensity score and the comparison variable (transfusion versus no transfusion) were included in a Cox proportional hazards analysis, allowing calculation of adjusted hazard ratios (HR) and Kaplan-Meier survival curves. RESULTS: Nine hundred and forty (31.1%) patients received RBC transfusion during or within 72h of surgery. Predictors of the need for transfusion were LL Hb and lower body mass index, use of cardiopulmonary bypass, female sex, number of grafts, renal dysfunction, increased age, extent of disease, and prior CABG; these factors were all included in the propensity score. After 1-year of follow-up, 122 (4.03%) deaths occurred. The crude HR for 1-year mortality in patients transfused was 3.0 (P<0.001). After adjusting for the propensity score, re-operation for bleeding, peri-operative blood loss and post-operative complications, the adjusted 30-day mortality was 1.9% in transfused patients compared to 1.1% in patients not transfused (P<0.05). The adjusted HR for 1-year mortality in patients transfused was 1.88 (P<0.01). CONCLUSIONS: Peri-operative RBC transfusion after CABG is associated with an increased risk of mortality during a 1-year follow-up period, with a large proportion of deaths occurring within 30-days.