Efficacy and Safety of Therapeutic Plasma Exchange in Children with Neuroimmunological Disorders: A Limited Unicentral Study.

Objectives: Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE's safety and feasibility data in these conditions. Materials & Methods: The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE's efficacy was studied in these patients. Results: The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients. Conclusion: Patients with PNID tolerated therapeutic plasma exchange, which was a safe process.

Allogenic hematopoietic stem cell transplantation in an Iranian patient with osteopetrosis caused by carbonic anhydrase II deficiency: A case report.

BACKGROUND: Osteopetrosis is a group of geneticall heterogeneous disorders resulting from impaired osteoclast function and bone resorption. The identification of specific genetic mutations can yield important prognostic and therapeutic implications. Herein, we present the diagnosis and successful application of hematopoietic stem cell transplantation (HSCT) in a patient with osteopetrosis caused by carbonic anhydrase II deficiency (Intermediate osteopetrosis). CASE PRESENTATION: Herein, we describe a 2.5-year-old male patient born to consanguineous parents who presented at 8-month-old with hydrocephaly, brain shunt, and developmental delay. Later at 9 months old, he was found to have eye disorder such as nystagmus, fracture of the elbow, abnormal skeletal survey, normal cell blood count (CBC), and severe hypocellularity in the bone marrow. Further evaluation showed renal tubular acidosis type 2. Whole-exome sequencing revealed a pathogenic homozygous variant in intron 2 of the carbonic anhydrase 2 gene (CA2) gene (c.232 + 1 G>T). The diagnosis of intermediate autosomal recessive osteopetrosis was established, and allogenic HSCT from his mother, a full-matched related donor (MRD), was planned. The conditioning regimen included Busulfan, Fludarabine, and Rabbit anti-thymocyte globulin. Cyclosporine and Mycophenolate Mofetil were used for graft-versus-host-disease prophylaxis. He Engrafted on day +13, and 95% chimerism was achieved. He is currently doing well without immunosuppressive therapy, now 12 months post HSCT, with normal calcium level and improving visual quality and FISH analysis revealed complete donor chimerism. DISCUSSION: HSCT could be a promising curative treatment for intermediate osteopetrosis and can provide long-term survival. Ongoing challenges in various aspects of HSCT remain to be addressed.

New Presentation of CD27 Deficiency; Coronary Ectasia and COVID-19.

CD27 is a costimulatory receptor involved in the maturation of the innate and adaptive immunity. CD27, through interaction with CD70, plays a role in the control of Epstein-Barr virus (EBV) infection. CD27 deficiency leads to an immune dysregulation disease characterized by EBV susceptibility. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) might put patients with primary immunodeficiency at risk for adverse outcomes. Chromogenic in situ hybridization (CISH) study was performed to detect EBV in the lymphoma tissue. Genetic analysis of the patient was done with Whole Exome Sequencing and detected variant was confirmed with PCR-Sanger sequencing. Here we report a 20-month-old boy with CD27 deficiency who developed lymphoma and coronary artery ectasia and had been infected with SARS-CoV-2. Clinical and laboratory findings were incompatible with atypical Kawasaki syndrome or multisystem inflammatory syndrome in children (MIS-C). As CD27 deficiency is a rare immune defect, publishing clinical data about the identified patient(s) can shed light on our knowledge about the related phenotype and the spectrum of clinical manifestations associated with CD27 deficiency. Thus, our findings expanded the spectrum of manifestations beyond EBV infection, highlighting this unusual cardiac sequela that could be related to EBV infection, lymphoma, or an underlying disease.

Rapid Desensitization for Hypersensitivity Reactions to Chemotherapeutic Drugs; A Case Series.

Usage of cancer chemotherapeutics drugs can be associated with adverse drug reactions. When IgE-mediated drug reactions are formed following administration of a chemotherapeutics drug that is a drug of choice, drug desensitization protocols can be helpful. HSR can be allergic or nonallergic, but the clinical manifestations are similar. RDD is effective when used appropriately, however it is often over utilized instead of performing a drug challenge. RDD is both an acceptable approach and a high-risk treatment modality in patients, in whom the offending agent is the first choice in chemotherapy. The safety of this modality has been acceptable in large studies. The side effects are often less frequent and less severe by repeating the protocol. We present 4 cases of successful desensitization in cancer patients, who have developed IgE- mediated reactions to their major chemotherapy drug.

Educational ranking of medical universities in Iran (ERMU).

Background: Several international ranking systems are available for examining the performance of universities internationally. However, it seems that in these rankings the educational functions are relatively discounted. In this study, we presented the theoretical framework, methodology, and results of 2 rounds of ERMU (educational ranking of medical universities) in Iran. Methods: The indicators were designed through literature reviews, expert panels, and consensus among professionals using Delphi method. Six domains, including quantitative expansion, educational management scheme, educational management, qualitative development, and alignment with the National Master Plan for Science and Education were assessed. Nineteen educational processes and outcome-based indicators were defined at the end. Some of them, such as the number of educational disciplines, were normalized based on the number of academic staff. Using exploratory factorial analysis, the interval validity of the final instrument was assessed. Also, the internal consistency of the tool was evaluated by calculating Cronbach's alpha. Data were collected in 2 rounds in 2010 and 2014. The required data were collected and cross-validated from different sources in medical universities and in the Ministry of Health. Results: The analysis yielded 2 factors, explaining a total of 61.6% of the variance for the entire set of variables. The calculated Cronbach's alpha was 0.94. The correlation coefficient for all items was 0.88. The comparison of the results of the 2 rounds showed a significant improvement in the scores of universities in all domains. Conclusion: The results showed a relatively fitted model with acceptable reliability. In addition, it seems that the ranking of medical universities has improved their performance in the study indictors. It seems that in the long-term, monitoring these indicators and the visibility of their scores may improve the educational performance of universities.

Social Accountable Medical Education: A concept analysis.

INTRODUCTION: Considering the pervasiveness of social accountable medical education concept around the world and the growing trend of literature in this regard as well as various interpretations made about this concept, we found it necessary to analyze the concept of social accountable medical education. METHODS: In this study, the modified version of McKenna's approach to concept analysis was used to determine the concept, explain structures and substructures and determine the border concepts neighboring and against social accountability in medical education. RESULTS: By studying the selected sources,the components of the concept were obtained to identify it and express an analytic definition of social accountability in medical education system. Then, a model case with all attributes of the given concept and the contrary and related concepts were mentioned to determine the boundary between the main concept and auxiliary ones. CONCLUSION: According to the results of this study in the field of social accountability, the detailed and transparent analytical definition of social accountable medical education can be used in future studies as well as the function and evaluation of medical education system.