Latent autoimmune diabetes in adults: A distinct but heterogeneous clinical entity

Latent autoimmune diabetes in adults (LADA) accounts for 2%-12% of all cases of diabetes. Patients are typically diagnosed after 35 years of age and are often misdiagnosed as type II Diabetes Mellitus (DM). Glycemic control is initially achieved with sulfonylureas but patients eventually become insulin dependent more rapidly than with type II DM patients. Although they have a type II DM phenotype, patients have circulating beta (β) cell autoantibodies, a hallmark of type I DM. Alternative terms that have been used to describe this condition include type 1.5 diabetes, latent type I diabetes, slowly progressive Insulin Dependent Diabetes Mellitus, or youth onset diabetes of maturity. With regards to its autoimmune basis and rapid requirement for insulin, it has been suggested that LADA is a slowly progressive form of type I DM. However, recent work has revealed genetic and immunological differences between LADA and type I DM. The heterogeneity of LADA has also led to the proposal of criteria for its diagnosis by the Immunology of Diabetes Society. Although many workers have advocated a clinically oriented approach for screening of LADA, there are no universally accepted criteria for autoantibody testing in adult onset diabetes. Following recent advances in immunomodulatory therapies in type I DM, the same strategy is being explored in LADA. This review deals with the contribution of the genetic, immunological and metabolic components involved in the pathophysiology of LADA and recent approaches in screening of this distinct but heterogeneous clinical entity.     

Activity-induced weakness in recessive myotonia congenita with a novel (696+1G>A) mutation.

OBJECTIVE: To investigate the cause of the transient weakness that occurs in recessive myotonia congenita (RMC) following sustained muscle contraction. METHODS: Nerve excitability studies were performed on a 35-year-old male with RMC due to a novel 696+1G>A CLCN1 mutation. The median nerve was stimulated at the wrist and compound muscle action potentials (CMAPs) were recorded from abductor pollicis brevis (APB). Stimulus-response behaviour using two stimulus durations, threshold electrotonus to 100-ms polarizing currents, a current threshold relationship and the recovery of excitability following supramaximal stimulation were recorded at rest. Excitability parameters were also recorded before and after maximal voluntary contraction (MVC) of APB against resistance for 60s. Results were compared to data obtained from 12 normal controls. RESULTS: Baseline axonal excitability parameters were all normal, indicating that axonal function was normal at the point of stimulation. Following one minute of MVC, excitability parameters demonstrated a significant increase in threshold when compared to controls (RMC 54.9%; controls 15.5+/-3.1%). In the RMC patient, this increase in threshold was associated with a 39% reduction in the amplitude of the maximal CMAP, which remained unaffected in controls. CONCLUSIONS: The reduction in maximal CMAP is likely to represent muscle activation failure due to depolarization block, with the increase in threshold possibly reflecting a compensatory attempt by motor axons to overcome prolonged contraction-induced changes in the muscle membrane. SIGNIFICANCE: The prolonged recovery of excitability following sustained muscle contraction is likely to be a contributing factor to symptoms of weakness and fatigue experienced by RMC patients.     

Neurogenic Detrusor Overactivity in Patients With Spinal Cord Injury: Evaluation and Management

Lower urinary tract dysfunction can have a significant impact on patients with spinal cord injury. Over the years, many treatment options have become available. This article reviews the assessment and management of neurogenic detrusor overactivity, with a particular focus on articles from the recent literature. Recent guidelines on the subject will be discussed. Management options include antimuscarinics and bladder emptying measures, botulinum toxin A, and neuromodulation in refractory cases and surgery for intractable cases. Recent and relevant publications in these areas will be summarized and discussed.     

Morbidity profile and functional outcome of modified facial translocation approaches for skull base tumors

The primary objective of this study was to evaluate morbidity associated with facial translocation approaches for skull base and results of various technical modifications. Forty consecutive patients who underwent facial translocation approaches for accessing skull base tumors from July 2005 to June 2010 were included in this study. There were 25 patients who underwent standard facial translocation, 4 patients medial mini, and 11 patients underwent extended facial translocation. Thirteen patients had benign disease and 27 patients had malignant disease. Resection was R0 in 36 and R1 in 4 patients. Most patients had acceptable cosmetic results. None of the patients had problems related to occlusion or speech and swallowing. The commonest complication observed was nasal crusting in 16 patients. Grade 2 trismus and exposure of mini plate was seen in three patients. Two patients developed necrosis of translocated bone. Three patients developed palatal fistula before modification of palatal incision. Facial translocation provides a satisfactory access for adequate clearance of skull base tumors with satisfactory aesthetic and functional results. With modifications of the surgical technique and implementation of new surgical tools, the morbidity of facial translocation approaches will continue to decrease.     

Anterior dural ectasia mimicking a lytic lesion in the posterior vertebral body in ankylosing spondylitis

Anterior dural ectasia is an extremely rare finding in ankylosing spondylitis (AS). The authors describe a unique case of AS in which the patient presented with cauda equina syndrome as well as an unusual imaging finding of erosion of the posterior aspect of the L-1 (predominantly) and L-2 vertebral bodies due to anterior dural ectasia. Symptomatic patients with long-standing AS should be monitored for the presence of dural ectasia, which can be anterior in location, as is demonstrated in the present case.     

Acute bioprosthetic mitral valve stenosis in a patient with HITS

Acute thrombosis involving the left atrium and a bioprosthetic valve during the early postoperative period is an extremely rare complication of heparin-induced thrombocytopenia syndrome (HITS). We present a patient with early bioprosthetic mitral valve stenosis complicated by HITS in a patient with severe mitral regurgitation, atrial fibrillation, and severe left ventricular dysfunction.     

Periprosthetic fracture of the proximal tibia after lateral unicompartmental knee arthroplasty

We report a case of periprosthetic fracture of the proximal tibia after lateral unicompartmental knee arthroplasty following a trivial fall. At the time of surgery, the components were found to be loose; and there was a large uncontained tibial defect with bone loss and communition at the fracture site. The patient was treated by revision total knee arthroplasty and proximal structural tibial allograft, with a satisfactory result at 5-year follow up. Our case illustrates that a bone-conserving unicompartmental knee arthroplasty, if complicated by a periprosthetic fracture, can also present with a difficult surgical problem. Attention to preoperative planning and to availability of structural allograft for such difficult cases is recommended.     

Clipless and sutureless laparoscopic surgery for adrenal and extra-adrenal tumors

BACKGROUND: Newer, energy-based devices like the ultrasonic coagulator (Harmonic Scalpel, Ethicon Endo-Surgery, Inc., Cincinnati, OH) and the LigaSure vessel-sealing system (Valleylab, Boulder, CO) are increasingly being used in advanced laparoscopic procedures. Use of these devices has resulted in shorter operative time, less blood loss, and lower conversion rates. We present our experience with these devices for laparoscopic removal of adrenal and extra-adrenal tumors. METHODS: Ten patients with adrenal tumors and 4 with extra-adrenal tumors were operated on laparoscopically with the ultrasonic shears and LigaSure vessel-sealing system. The entire surgery was carried out using these energy-based devices without using any clips or sutures. RESULTS: No conversions were necessary. None of the patients experienced any major intraoperative or postoperative bleeding. The mean size of the tumor was 6.2 cm (range, 3 to 8). The mean operative time was 123 minutes (range, 80 to 210), and the mean blood loss was 70 mL (range, 10 to 150). Histopathology revealed pheochromocytomas in 7 patients. CONCLUSIONS: Use of the LigaSure vessel-sealing device along with ultrasonic shears for laparoscopic removal of adrenal and extra-adrenal tumors is safe and effective.     

New Technologies for the Surgical Curriculum

The aim of a surgical residency program is to produce competent professionals displaying the cognitive, technical, and personal skills required to meet the needs of society. Current changes to the delivery of healthcare necessitate the development of new models of training. These can be supported with the development of new technologies to train and assess surgical practitioners. This article describes recent developments within Imperial College London with regard to eye tracking, noninvasive brain imaging, and an innovative mentoring scheme for the new surgical curriculum. The concept of eye tracking is described, together with surgical application for this technique in terms of dexterity analysis during minimally invasive procedures. We have also begun to understand spatial localization within the brain cortex during surgical knot-tying tasks. The aim is to develop a map of the cortex with regard to surgical novices and experienced surgeons and then to develop the hypothesis that a translational process of cortical plasticity occurs during training. Finally, the article is intended to describe a training scheme that goes beyond dexterity, and moves toward the development of a successful surgeon through surgical mentoring. It is hoped that some of these tools will enhance the training of future surgeons in order to continue to provide a high-quality service to our patients.     

Traumatic bilateral posterior hip dislocation in 10 year old male child

Traumatic bilateral posterior hip dislocation in skeletally immature patient is reported very less in literature. We report a 10 yr old boy presented to us following farmyard injury with bilateral posterior hip dislocation, which was reduced manually under sedation with uneventful follow-up and complete hip range of motion at 2 year.