Inmunodeficiencias congénitas del receptor de antígeno de los linfocitos T

T-cell receptor (TCR) immunodeficiencies of humans are low-prevalence autosomal recessive diseases characterized by impaired surface TCR expression and selective T lymphopenia (milder in CD3γ, TCRα or CD247 deficiency, and severe in individuals lacking CD3δ or CD3?). The congenital absence of TCR components has a differential impact on T-cell development and function depending on the affected TCR chain and on the species, with human patients being, in some cases, rather different from mouse counterparts.     

Lack of effect of glutamine administration to boost the innate immune system response in trauma patients in the intensive care unit

Introduction  

The use of glutamine as a dietary supplement is associated with a reduced risk of infection. We hypothesized that the underlying mechanism could be an increase in the expression and/or functionality of Toll-like receptors (TLR), key receptors sensing infections. The objective of this study was to evaluate whether glutamine supplementation alters the expression and functionality of TLR2 and TLR4 in circulating monocytes of trauma patients admitted to the intensive care unit (ICU).     

Metabolic bone disease in inflammatory bowel disease

Osteoporosis is associated with a high morbidity rate and is a risk factor for fractures. Patients with inflammatory bowel disease are at increased risk of developing osteopenia and osteoporosis. Corticosteroid use, malnutrition, and proinflammatory cytokines are unique risk factors for bone loss in ulcerative colitis and Crohn disease. Bone mineral density is assessed by dual-energy X-ray absorptiometry and reported as a T score or number of standard deviations away from the mean. A T score < 1 SD below the mean is normal, 1 to 2.5 SD below the mean is consistent with osteopenia, and greater than 2.5 SD below the mean is defined as osteoporosis. Treatment includes a combination of basic preventative measures, for example, weight-bearing exercise, calcium, vitamin D, and pharmacologic agents, (e.g., bisphosphonates).     

Our experience in the treatment of hyperhidrosis of the upper limbs by video-assisted thoracoscopy: an analysis of our first 100 procedures

Sympathectomy of the thoracic chain is an effective surgical procedure for treating axillary and palmar hyperhidrosis. The procedure has been performed with minimal invasion and good results in recent years through the use of videothoracoscopic surgery. This paper describes the technique and our experience with a series of 50 patients between 16 and 48 years old. The earliest approach was unilateral in successive operations. The procedure was later performed bilaterally, at first with the patients in sequential lateral decubitus positions and later in semi-seated position. Complications were 1 case of incomplete Claude-Bernard-Horner syndrome that resolved spontaneously two months after surgery; 1 failure when sympathectomy was performed without location of the chain, obliging rapid re-operation; laminar pneumothorax in 12% of the series; compensatory hyperhidrosis in 26%; 10% with chest pain due to intercostal involvement, resolving with time; and slight bleeding in 8%. Outcome was excellent, with complete disappearance of axillary and palmar perspiration. Patient satisfaction was 9.2/10 one year after surgery. Mean hospital stay was less than 36 h.     

The importance of histology in the evaluation of pulmonary transplantation: carcinomatous lymphangitis

Pulmonary carcinomatous lymphangitis is a form of neoplastic metastatic spread to the lungs, which represents a poor prognosis for the patient. The physician may be confronted by a differential diagnosis with diffuse pulmonary interstitial affections requiring specific treatment, including lung transplantation. We present the case of a patient, diagnosed with pulmonary interstitial disease with rapidly progressive worsening of lung function, who was considered for lung transplantation. Videothoracoscopic lung biopsy demonstrated the existence of carcinomatous lymphangitis, which completely changed the therapeutic direction.     

Perineostomy: the last oportunity

Objective

To review the technique and outcome of perineal urethrostomy or urethral perineostomy and to identify factors related to the procedure failure.

Material and methods

We studied 17 patients who underwent perineal urethrostomy between 2009-2013 in a single hospital. Success was defined as no need for additional surgical treatment or urethral dilatation. We reviewed the clinical data related to age, weight, previous urethral surgery, diabetes, hypertension, ischemic cardiopathy, lichen sclerosus and other causes and studied their association with the procedure failure (univariate analysis). We completed the analysis with a multivariate test based on binary regression.

Results

The average follow-up was 39.41 months. From all the causes, we found Lichen Sclerosus in 35%, idiopathic etiology in 29% and prior hypospadia repair in 18%. Postoperative failure occurred in 3 patients, with a final success of 82.4%. The binary regression model showed as independent risk factors ischemic cardiopathy (OR: 2.34), and the presence of Lichen Sclerosis (OR: 3.21).

Conclusions

The success rate with the perineal urethrostomy technique shows it to be a valid option above all when we preserve the urethral blood supply and plate. Lichen sclerosus and ischemic vascular problems are risk factors to re-stenosis.     

Pathologic features of early inflammatory bowel disease

Often the pathologic changes of IBD are subtle and may not be present in a proportion of biopsy specimens. In cases of early disease, the changes may be missed, and additional specimens should be taken after a period of time. Modifying factors, such as prebiopsy treatment and coexisting disease, should be considered. A forum to review cases and allow for communication between gastroenterologists and pathologists is especially useful for clinicopathologic correlation and assignment of a working diagnosis to each case. Careful attention to the pathologic features of early UC and CD would be most useful when evaluating new therapies for IBD.