HLA Class II (DRB,DQA1 and DQB1) Allele and Haplotype Frequencies in the Patients with Pemphigus Vulgaris

Introduction  Pemphigus vulgaris (PV), an autoimmune disease affecting the skin and mucous membranes, is associated with some human leukocyte antigen (HLA) class II alleles and haplotypes. Materials and Methods  In order to evaluate the association of HLA-DR and DQ alleles and haplotypes in Iranian non-Jewish patients with PV, 52 patients with PV and 180 normal subjects as control group were investigated in this study. Results and Discussion  HLA-DRB1*04, -DRB1*1401, -DRB4, -DQA1*0104, -DQA1*03011, -DQB1*0302, and -DQB1*0502 alleles have been significantly increased in our patients group. Moreover, the haplotypes HLA-DRB1*04/-DQA1*03011/-DQB1*0302 and HLA-DRB1*1401/-DQA1*0104/-DQB1*0502 increased significantly in our patients. In contrast, the following alleles decreased significantly in our patients: HLA-DRB1*15, -DRB1*0301, -DRB1*07, -DRB1*11, -DRB5, -DQA1*0101, -DQA1*0103, -DQA1*201, -DQA1*05, -DQB1*0201, -DQB1*0301, -DQB1*06011, and -DQB1*0602. In addition, HLA-DRB1*15/-DQA1*0103/-DQB1*06011, HLA-DRB1*0301/-DQA1*05011/-DQB1*0201, HLA-DRB1*07/-DQA1*0201/-DQB1*0201, and HLA-DRB1*11/-DQA1*05/-DQB1*03011 decreased significantly in our patients. Genetic factors are involved in the occurrence of PV; HLA-DRB1*04 and -DRB1*1401 alleles and the related haplotypes are suggestive to be two major PV susceptibility factors in our population study.     

Surgical resection for elderly patient with skin invasion of breast cancer

We report a locally advanced elderly breast carcinoma with skin invasion. The patient was a 96-year-old woman who had a breast lump. The palpable tumor was 3 .5 cm in diameter. Ultrasonography revealed a low echoic mass. A core needle biopsy for the breast tumor led to a diagnosis of an invasive ductal carcinoma positive for estrogen receptor and progesteron receptor, and negative for HER2/neu protein expression. She underwent a tumorectomy including the cancer invasive skin by local anesthesia. Because her respiratory function was unbearable to perform a muscle-preserving mastectomy with general anesthesia. The surgical margins of the resected specimen were negative. The clinicopathological stage, according to the UICC-pTNM classification, was Stage III C (T4b, N0, M0). After the operation, she was administered aromatase inhibitor. The patient has been well and remained disease-free during a follow-up period of 3 years. The surgical excision with local anesthesia was useful for locally advanced super senior breast cancer patients who were impossible to perform general anesthesia by various kinds of factors.     

Abnormal Axon Reflex-Mediated Sweating Correlates with High State of Anxiety in Atopic Dermatitis

BackgroundSweating plays a key role in skin homeostasis, including antimicrobial and moisturizing effects, and regulation of skin surface pH. Impaired axon reflex-mediated (AXR) sweating has been observed in patients with atopic dermatitis (AD). However, the mechanism of such abnormal sudomotor axon reflex remains to be revealed.MethodsTo investigate this mechanism, sudomotor function was analyzed using a quantitative sudomotor axon reflex test (acetylcholine iontophoresis) in patients with AD (n = 26) and healthy volunteers (n = 12). Correlation between sudomotor function and certain background factors, including Spielberger State Trait Anxiety Inventory score, Severity Scoring of Atopic Dermatitis (SCORAD) score, number of circulating eosinophils, and serum concentrations of thymus and activation-regulated chemokine and immunoglobulin E radioimmunosorbent test, was validated.ResultsLatency time was significantly prolonged in AD (p = 0.0352), and AXR sweating volume (mg/0-5 min) was significantly lower in AD patients than in healthy controls (p = 0.0441). Direct sweating volume (mg/0-5 min) was comparable in AD patients and healthy controls. A significant correlation between the evaluation results of quantitative sudomotor axon reflex tests and certain background factors was not observed. The latency time in non-lesioned and lesioned areas for AD patients versus continuous anxiety value in the Spielberger State Trait Anxiety Inventory and the AXR versus SCORAD showed significant correlations (p = 0.0424, p = 0.0169, and p = 0.0523, respectively).ConclusionsAlthough the number of study subjects was little, abnormal AXR sweating in patients with AD was observed. Correlative analysis suggests possible involvement of continuous anxiety and the immune system in such abnormal sudomotor function.     

Expression of CD markers' in immune thrombocytopenic purpura: prognostic approaches

Immune Thrombocytopenic Purpura (ITP) is a common autoimmune bleeding disorder characterized by a reduction in peripheral blood platelet counts. In this disease, autoantibodies (Auto‐Abs) are produced against platelet GPIIb/GPIIIa by B cells, which require interaction with T cells. In this review, the importance of B and T lymphocytes in ITP prognosis has been studied. Relevant literature was identified by a PubMed search (1990–2016) of English‐language papers using the terms B and T lymphocyte, platelet, CD markers and immune thrombocytopenic purpura. T and B lymphocytes are the main immune cells in the body. Defective function causes disrupted balance of different subgroups of lymphocytes, and abnormal expression of surface markers of these cells results in self‐tolerance dysfunction, as well as induction of Auto‐Abs against platelet glycoproteins (PG). Given the role of B and T cells in production of autoantibodies against PG, it can be stated that the detection of changes in CD markers' expression in these cells can be a good approach for assessing prognosis in ITP patients.     

Progress and Promise Towards Safe Induced Pluripotent Stem Cells for Therapy

The recent generation of induced pluripotent stem cells (iPSCs) from somatic cells provides an invaluable resource for drug or toxicology screening, medical research, and patient-specific cell therapy. However, there are currently a number of obstacles including virus integration and the genetic alteration of iPSCs that will need to be overcome before these cells may be considered safe for clinical applications. Here, we highlight the potential and challenges of iPSC research and review advances in reprogramming methods that have rapidly moved the field closer to realizing the goal of generating safe iPSCs for transplantation.     

Whole body MRI for detecting metastatic bone tumor: comparison with bone scintigrams.

PURPOSE: To compare the effectiveness of whole body MRI (WB-MRI [magnetic resonance imaging]) and bone scintigram (BS) at detecting bone metastasis. MATERIALS AND METHODS: WB-MRI was performed on 16 patients for detecting bone metastasis (6 breast carcinoma, 7 prostatic carcinoma, 1 renal cell carcinoma [RCC], 1 hepatocellular carcinoma [HCC], and 1 primary unknown). BS was also performed in all cases. Patients were placed on a table top extender (Philips Medical Systems). The maximal longitudinal field of view (FOV) was 200 cm. At first, the total spine was imaged in the sagittal plane with a three-station approach for two image sets (fast spin-echo [SE] T1-weighted images [T1WI] and short tau inversion recovery [STIR] images). The whole body was then imaged in the coronal plane with a seven-station approach for two image sets (fast field echo [FFE] T1WI and STIR). Total examination time, including patient positioning, was within 40 min. Three independent radiologists interpreted the imaging data. RESULTS: WB-MRI identified 5 cases of 24 lesions as bone metastasis, while BS identified 3 cases of 25 lesions. Concordance between WB-MRI and BS was seen in 3 cases of 22 lesions (81%). For two cases of 2 lesions, which were identified only with WB-MRI, the lesions were located in the sacrum and thoracic spine. For one case of 3 lesions, which was identified only with BS, the lesions were located in the skull and rib. CONCLUSION: WB-MRI was an excellent method for screening bone metastasis, especially the vertebral body.     

Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study

Disorders of sex development (DSD) are congenital conditions in which the typical genetic and hormonal profiles are affected and thereby the usual process of sexual differentiation. Most of these studies, however, have been conducted in Western countries. In the present study, preschool sex-typed activities of Iranian individuals with DSD and their age-matched non-affected male and female relatives were assessed using the Pre-School Activities Inventory (PSAI) modified for retrospective self-report. A total of 192 individuals participated in our study, including 33 46,XX individuals with congenital adrenal hyperplasia (CAH; M age?=?10.36, SD?=?5.52), 15 46,XY individuals with complete androgen insensitivity syndrome (CAIS; M age?=?19.8, SD?=?7.14), and 16 46,XY individuals with 5-alpha reductase deficiency type-2 (5α-RD-2; M age?=?17.31, SD?=?7.28), as well as one age-matched non-affected male and female relative for each patient. With regard to PSAI scores, male-identifying participants with 5α-RD-2 and male controls reported similar levels of male-typical childhood play. Female-identifying participants with 5α-RD-2 and CAH showed comparable scores: significantly less masculine and more feminine than male controls, but significantly more masculine and less feminine than females with CAIS and female controls. These findings support the role of androgens in the development of sex-typical childhood play behavior, with those being exposed to higher levels of fetal functional androgens expressing more masculine behavior at preschool ages.