The neurourology of tropical spastic paraparesis.

We report the clinical features, urodynamic results and neurophysiological findings in 6 patients with urinary symptoms related to tropical spastic paraplegia. The widespread nature of the pathological changes within the nervous system result in a complex variety of urodynamic and neurophysiological features.     

Pathogenesis and recovery of tetraplegia after electrical injury

The site of neurological damage causing paralysis after electrical trauma remains to be clarified. A patient is described who developed a flaccid tetraplegia after a high voltage electrical injury. The findings on initial examination and neurophysiological investigation showed a very severe generalised sensory-motor polyneuropathy. His subsequent follow up over 60 months showed a remarkable degree of reinnervation and the unmasking of a myelopathy. The degree of reinnervation noted suggests an axonopathy that left the other elements of the peripheral nerves relatively spared. These findings provide the most convincing evidence to date that a generalised polyneuropathy can follow electrical injury and that it results from non-thermal mechanisms such as electroporation.     

Feasibility of Lung Transplantation From Donation After Circulatory Death Donors Following Portable Ex Vivo Lung Perfusion: A Pilot Study

Background

Donation after circulatory death (DCD) has the potential to significantly alleviate the shortage of transplantable lungs. We report our initial experience with the use of portable ex vivo lung perfusion (EVLP) with the Organ Care System Lung device for evaluation of DCD lungs.

Avoidance of Profound Hypothermia During Initial Reperfusion Improves the Functional Recovery of Hearts Donated After Circulatory Death

The resuscitation of hearts donated after circulatory death (DCD) is gaining widespread interest; however, the method of initial reperfusion (IR) that optimizes functional recovery has not been elucidated. We sought to determine the impact of IR temperature on the recovery of myocardial function during ex vivo heart perfusion (EVHP). Eighteen pigs were anesthetized, mechanical ventilation was discontinued, and cardiac arrest ensued. A 15‐min standoff period was observed and then hearts were reperfused for 3 min at three different temperatures (5°C; N = 6, 25°C; N = 5, and 35°C; N = 7) with a normokalemic adenosine–lidocaine crystalloid cardioplegia. Hearts then underwent normothermic EVHP for 6 h during which time myocardial function was assessed in a working mode. We found that IR coronary blood flow differed among treatment groups (5°C = 483 ± 53, 25°C = 722 ± 60, 35°C = 906 ± 36 mL/min, p < 0.01). During subsequent EVHP, less myocardial injury (troponin I: 5°C = 91 ± 6, 25°C = 64 ± 16, 35°C = 57 ± 7 pg/mL/g, p = 0.04) and greater preservation of endothelial cell integrity (electron microscopy injury score: 5°C = 3.2 ± 0.5, 25°C = 1.8 ± 0.2, 35°C = 1.7 ± 0.3, p = 0.01) were evident in hearts initially reperfused at warmer temperatures. IR under profoundly hypothermic conditions impaired the recovery of myocardial function (cardiac index: 5°C = 3.9 ± 0.8, 25°C = 6.2 ± 0.4, 35°C = 6.5 ± 0.6 mL/minute/g, p = 0.03) during EVHP. We conclude that the avoidance of profound hypothermia during IR minimizes injury and improves the functional recovery of DCD hearts.     

Somatic mosaicism and the phenotypic expression of COL2A1 mutations

Mutations in COL2A1, the gene for type II-collagen, can result in a wide variety of phenotypes depending upon the nature of the mutation. Dominant negative mutations tend to result in severe and often lethal skeletal dysplasias such as achondrogenesis type 2, Kniest dysplasia, and spondyloepiphyseal dysplasia congenita. Stickler syndrome, a condition characterized by ophthalmological and orofacial features, deafness and arthritis, usually, but not exclusively, results from haploinsufficiency. Overlapping features of all these disorders can also be seen in the same family. Rare reports have demonstrated that phenotypic variability can be explained in some families by somatic mosaicism. Here, we describe five further examples of somatic mosaicism of COL2A1 mutations illustrating the importance of detailed clinical evaluation and molecular testing even in clinically normal parents of affected individuals.     

What size of left atrium significantly impairs the success of maze surgery for atrial fibrillation?

A best evidence topic in cardiothoracic surgery was written according to a structured protocol. The question addressed was in adults with atrial fibrillation (AF), what preoperative size of left atrium impairs maze surgery success in terms of recurrence of AF. Altogether 422 papers were found using the reported search, of which 12 represented the best evidence to answer the clinical question. The authors, journal. date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Nine of 12 papers found that preoperative left atrial (LA) size was significantly larger in patients who experienced recurrent AF. When left atrial diameter (LAD) was found to be statistically different between sinus conversion and recurrent AF groups the mean LAD was consistently >60 mm in the recurrent AF group, whereas it was <60 mm in all but one of the sinus conversion groups. In terms of a cut-off value for LA size, a left atrial volume index >135 ml/m(2) was found to confer 100% specificity for maze failure and a LAD >60 mm was found to be 100% sensitive for maze failure. A preoperative LAD <48.3 mm was shown in one study to be 100% sensitive for sinus conversion by the maze procedure. Despite much evidence highlighting preoperative LAD as a risk factor for maze failure, relatively few studies seek to define a definitive cut-off value for LA size beyond which the risks of the procedure (such as bleeding, infection or stroke) outweigh the chance of sinus recovery. We conclude that since mean preoperative LAD in AF groups is consistently over 60 mm caution should be exercised when offering these patients the maze procedure. Furthermore, the relationship between preoperative LAD and maze failure appears continuous and so patients should be counselled as to their increased risk of failure the further they deviate from a LAD of 60 mm. There is some evidence for and no available evidence to the contrary that a LAD < 43 mm is associated with complete maze success. Hence, these patients should be offered the maze procedure unless there are alternate strong contraindications.