The Naked Truth

Translation of a story by Kashmiri short story writer, Harikrishna Kaul

A Systematic Review of Immunological Studies of Erythema Nodosum Leprosum.

Erythema nodosum leprosum (ENL) is a painful inflammatory complication of leprosy occurring in 50% of lepromatous leprosy patients and 5-10% of borderline lepromatous patients. It is a significant cause of economic hardship, morbidity and mortality in leprosy patients. Our understanding of the causes of ENL is limited. We performed a systematic review of the published literature and critically evaluated the evidence for the role of neutrophils, immune complexes (ICs), T-cells, cytokines, and other immunological factors that could contribute to the development of ENL. Searches of the literature were performed in PubMed. Studies, independent of published date, using samples from patients with ENL were included. The search revealed more than 20,000 articles of which 146 eligible studies were included in this systematic review. The studies demonstrate that ENL may be associated with a neutrophilic infiltrate, but it is not clear whether it is an IC-mediated process or that the presence of ICs is an epiphenomenon. Increased levels of tumor necrosis factor-α and other pro-inflammatory cytokines support the role of this cytokine in the inflammatory phase of ENL but not necessarily the initiation. T-cell subsets appear to be important in ENL since multiple studies report an increased CD4+/CD8+ ratio in both skin and peripheral blood of patients with ENL. Microarray data have identified new molecules and whole pathophysiological pathways associated with ENL and provides new insights into the pathogenesis of ENL. Studies of ENL are often difficult to compare due to a lack of case definitions, treatment status, and timing of sampling as well as the use of different laboratory techniques. A standardized approach to some of these issues would be useful. ENL appears to be a complex interaction of various aspects of the immune system. Rigorous clinical descriptions of well-defined cohorts of patients and a systems biology approach using available technologies such as genomics, epigenomics, transcriptomics, and proteomics could yield greater understanding of the condition

Comparing Single-Photon Emission Computed Tomography (SPECT), Electroencephalography (EEG), and Magneto-encephalography (MEG) Seizure Localizations in Pediatric Cases of Laser Ablation

Intractable epilepsy may have a more severe effect on children in comparison to adults because the motor, sensorial, and cognitive functions of children are still undergoing development. For this same reason, however, children maintain a greater potential for recovery from intractable epilepsy because of the remaining cerebral plasticity. Thus, after 2 unsuccessful antiepileptic medications, surgical intervention is recommended. Pre-surgical localization of the epileptogenic zone increases seizure-freedom post-surgery by 200% to 300%. Single-photon emission computed tomography (SPECT) is commonly used, with magneto-encephalography (MEG) and electroencephalography (EEG), to localize the epileptogenic zone, because it localizes cerebral blood flow. There is limited research in the efficacy of SPECT localization in pediatric pharmacoresistant epilepsy. Therefore, the objective of this study was to determine the efficacy of SPECT in it’s ability to localize the epileptogenic zone in laser ablation cases of pediatric pharmacoresistant epilepsy. Out of 122 SPECTs conducted at Dell Children’s Medical Center from July 2010 to January 2015, 12 underwent laser ablation. In the 12 cases of laser ablation, SPECT displayed more sensitivity and specificity than EEG and more specificity than MEG when held against the laser ablation outcomes. This study further proves that SPECT is efficacious in epileptogenic zone localization of pediatric pharmacoresistant epilepsy

Alloimmunization and autoimmunization in transfusion dependent thalassemia major patients: Study on 319 patients

Background: The development of anti-red blood cell antibodies (both allo-and autoantibodies) remains a major problem in thalassemia major patients. We studied the frequency of red blood cell (RBC) alloimmunization and autoimmunization among thalassemia patients who received regular transfusions at our center and analyzed the factors, which may be responsible for development of these antibodies. Materials and Methods: The study was carried out on 319 multiply transfused patients with β-thalassemia major registered with thalassemia clinic at our institute. Clinical and transfusion records of all the patients were examined for age of patients, age at initiation of transfusion therapy, total number of blood units transfused, transfusion interval, status of splenectomy or other interventions. Alloantibody screening and identification was done using three cell and 11 cell panel (Diapanel, Bio-rad, Switzerland) respectively. To detect autoantibodies, autocontrol was carried out using polyspecific coombs (IgG + C3d) gel cards. Results: Eighteen patients out of total 319 patients (5.64%) developed alloantibodies and 90 (28.2%) developed autoantibodies. Nine out of 18 patients with alloantibodies also had autoantibodies. Age at first transfusion was significantly higher in alloimmunized than non-immunized patients (P = 0.042). Out of 23 alloantibodies, 52.17% belonged to Rh blood group system (Anti-E = 17%, Anti D = 13%, Anti-C = 13%, Anti-C w = 9%), 35% belonged to Kell blood group system, 9% of Kidd and 4% of Xg blood group system. Conclusion: Alloimmunization was detected in 5.64% of multitransfused thalassemia patients. Rh and Kell blood group system antibodies accounted for more than 80% of alloantibodies. This study re-emphasizes the need for RBC antigen typing before first transfusion and issue of antigen matched blood (at least for Rh and Kell antigen). Early institution of transfusion therapy after diagnosis is another means of decreasing alloimmunization