Efficacy of Mucosamin Spray as an Adjunct for Prevention of Oral Mucositis in Children under Chemotherapy: A Two-Center Randomized Clinical Trial.

Statement of the Problem: Chemotherapy-induced mucositis is the most common complication during cancer treatment. This complication can lead to pain, increased risk of infection and malnutrition. Therefore, it is important to find a solution to reduce the severity and duration of side effects. Purpose: This study aimed to assess the efficacy of Mucosamin spray as an adjunct for prevention of oral mucositis in children under chemotherapy. Materials and Method: This parallel-design clinical trial evaluated 48 patients aged 5 to 15 years with leukemia and lymphoma presenting to the Hematology Department of Mofid and Mahak Hospitals. The patients were randomly divided into two groups (n=24). Before starting chemotherapy, all patients received oral hygiene instructions (toothbrushing without flossing). Patients in both groups were requested to use a mouthwash composed of nystatin, aluminum-magnesium hydroxide suspension (aluminum MgS), and diphenhydramine for 1 min every morning on a daily basis starting the day before treatment. Patients in the intervention group were also requested to spray their entire oral mucosa with Mucosamin spray 3 times a day in addition to using the mouthwash. Patients were requested to refrain from eating and drinking for 1h after spraying. The patients were clinically examined by a senior dental student once every other day for 20 days regarding the occurrence, severity, and duration of oral mucositis. Data were analyzed using the Chi-square and Mann-Whitney tests, Kaplan-Meier survival analysis, and log rank test. Results: No significant difference was noted between the two groups in the incidence, severity, or time of development of mucositis (p> 0.05). The 7-day non-recovery percentage was 72.7% (SE= 13.4) and 25.0% (SE= 15.3) in the control and test groups, respectively, indicating shorter duration (faster healing) of mucositis in the intervention group (p= 0.01). Conclusion: Within the limitations of this study, it seems that prophylactic application of Mucosamin spray can shorten the course of oral mucositis, in case of its occurrence.

Outcomes and survival rates of childhood osteosarcoma in Iran, A report from MAHAK Pediatric Cancer Treatment and Research Center, from 2007 to 2020.

Background: Osteosarcoma is one of the most common childhood bone malignancies. Although chemotherapy protocol including methotrexate is an effective treatment for osteosarcoma, some other regimens have excluded it because of its complications. Methods: This retrospective study was conducted on 93 children younger than 15 years old who were diagnosed with osteosarcomafrom March 2007 to January 2020. Two chemotherapy protocols were administrated for patients, namely, DCM protocol (Doxorubicin-Cisplatin-Methotrexate) and German protocol (excluding methotrexate). All statistical analysis was conducted using SPSS-25 software. Results: Among patients, 47.31% were male. Patients' age ranged from 3 to 15 with the mean of 10.41 ± 0.32 years. Femur was the most frequent primary tumor site (59.14%), followed by tibia (22.58%). Metastasis rate at diagnosis was 17.20% in our study. Furthermore, the 5-year overall survival (OS) of total patients was 37.3 ± 7.5%, whereas the 5-year OS of males and females was 33.6 ± 10.9% and 39.8 ± 10.6%, respectively. The 5-year OS of methotrexate regimen was 15.6 ± 9.6%, whereas that of methotrexate-free protocol was 50.2 ± 9.0%. Conclusions: Female patients had better survival rates than males. In addition, the chemotherapy protocol excluding methotrexate significantly increased the overall and event free survival of patients.

A Mobile Self-Management App (CanSelfMan) for Children With Cancer and Their Caregivers: Usability and Compatibility Study.

BACKGROUND: Despite the increasing development of different smartphone apps in the health care domain, most of these apps lack proper evaluation. In fact, with the rapid development of smartphones and wireless communication infrastructure, many health care systems around the world are using these apps to provide health services for people without sufficient scientific efforts to design, develop, and evaluate them. OBJECTIVE: The objective of this study was to evaluate the usability of CanSelfMan, a self-management app that provides access to reliable information to improve communication between health care providers and children with cancer and their parents/caregivers, facilitating remote monitoring and promoting medication adherence. METHODS: We performed debugging and compatibility tests in a simulated environment to identify possible errors. Then, at the end of the 3-week period of using the app, children with cancer and their parents/caregivers filled out the User Experience Questionnaire (UEQ) to evaluate the usability of the CanSelfMan app and their level of user satisfaction. RESULTS: During the 3 weeks of CanSelfMan use, 270 cases of symptom evaluation and 194 questions were recorded in the system by children and their parents/caregivers and answered by oncologists. After the end of the 3 weeks, 44 users completed the standard UEQ user experience questionnaire. According to the children's evaluations, attractiveness (mean 1.956, SD 0.547) and efficiency (mean 1.934, SD 0.499) achieved the best mean results compared with novelty (mean 1.711, SD 0.481). Parents/caregivers rated efficiency at a mean of 1.880 (SD 0.316) and attractiveness at a mean of 1.853 (SD 0.331). The lowest mean score was reported for novelty (mean 1.670, SD 0.225). CONCLUSIONS: In this study, we describe the evaluation process of a self-management system to support children with cancer and their families. Based on the feedback and scores obtained from the usability evaluation, it seems that the children and their parents find CanSelfMan to be an interesting and practical idea to provide reliable and updated information on cancer and help them manage the complications of this disease.

Targeted chemotherapy in retinoblastoma: A step forward from patient survival to globe survival.

BACKGROUND/ OBJECTIVES: Despite being the most common intraocular malignancy in childhood, there is a substantial disparity between developing and developed countries in terms of patient and globe survival in retinoblastoma (Rb). The current study intends to determine patient and globe survival before and after the introduction of the new targeted treatment modalities in a developing country. METHODS: Medical records of 350 patients (516 eyes) with retinoblastoma referred to a tertiary referral center for Rb in Tehran, Iran, were reviewed. In order to compare patient and globe survival before and after the availability of the new treatment modalities, including intra-arterial and intravitreal chemotherapy, the patients were divided into group 1 (2001-2007) and group 2 (2008-2018) based on the calendar period of diagnosis. RESULTS: Two-hundred-twenty-three eyes of 149 patients and 293 eyes of 201 patients were categorized into groups 1 and 2, respectively. The 5-year patient survival was 97% across the current survey, and the overall survival rate was 96% in group 1 and 99% in group 2 (P = 0.08). Overall, 50% of eyes with retinoblastoma underwent enucleation, which was the primary in 63% (116/184) of the unilateral and 30% (99/322) of the bilateral cases. Primary enucleation was significantly lower in group 2 (35%) in contrast to group 1 (50%) (P < 0.001). In addition, globe survival improved significantly in the International Classification of Retinoblastoma Groups D (17% in group 1 vs. 66% in group 2, P < 0.001) and E (1% in group 1 vs. 23% in group 2 P < 0.001) during the two timelines. In enucleated eyes, despite the increased rate of prelaminar involvement in group 2 (13% vs. 2% in group 1, P = 0.003), the rate of high-risk histopathologic findings was similar between the two groups. CONCLUSION: Similar to developed countries, the application of new targeted treatment modalities, including intra-arterial and intravitreal chemotherapy, has been associated with significantly improved globe survival in Rb patients. However, it should be noted that even with the availability of these novel treatment options, the decision for on-time enucleation should not be deferred.

Pediatric Cancer Registry at MAHAK Pediatric Cancer Treatment and Research Center: A Single-Center Study from Iran.

Background: The childhood cancer registry in Iran is a hospital-based system and there is not any unique and national registry system for pediatric malignancies in Iran. According to the limitations and requirements, this study was designed to clarify the aspect of childhood malignancies in Iran and promote establishing the Iranian national childhood cancer registry system. Materials and Methods: This cross-sectional longitudinal study was implied on 1500 patients younger than 20-years old diagnosed with any malignancy and admitted at MAHAK Pediatric Cancer Treatment and Research Center (MPCTRC) from 2007 to 2014. Data collection was based on a validated questionnaire with three categories including demographic data, clinical data and type of malignancy, and outcomes. Collected data were analyzed using methods for qualitative and quantitative variables (P < 0.05) by SPSS software version 22. The survival rate was calculated by the Kaplan-Meyer method. Results: This study was implied on 1500 children with a mean age of 6.1 years old. The most common malignancy was acute leukemia (30.7%) followed by central nervous system tumors (27%). At the onset of starting treatment, the rate of conferring with relapse, metastasis, and secondary malignancies was 29%, 19.5%, and 1% respectively. In addition, 52 patients had bone marrow transplantation of whom, 14 cases died. Totally, 42% of patients died and the 3-years, 5-years, and 10-years overall survival rates were 67.7% ± 0.01, 60.3% ± 0.01, and 53.8% ± 0.01, respectively. Conclusion: Establishing a population-based pediatric cancer registry in Iran is necessary and will be useful for improving the survival rate of mentioned patients.

Ocular Manifestations of Newly Diagnosed Acute Leukemia Patients.

Purpose: To present primary ocular manifestations in acute leukemia. Methods: This cross-sectional descriptive hospital-based study evaluated all newly diagnosed leukemia patients of three referral hospitals of Tehran University of Medical Sciences in 2015-2016 and Mahak Hospital in Tehran in 2017. Exclusion criteria included the patients with the previous history of chemotherapy, cases of relapsing disease, and the patients with a history of ocular disease or other systemic conditions with ophthalmic manifestations. Results: A total of 85 patients (170 eyes) were evaluated in our study, including 29 children (34.1%) and 43 females (50.6%). The mean patient age was 37.84 ± 11.91 years in the adult group and 6.28 ± 4.70 years in the pediatric category. Ophthalmic involvement was seen in 27 patients (31.8%), including 6 pediatric patients (20.7%) and 21 adult patients (37.5%). Two patients (2.3%) had direct infiltration by leukemic cells and 76 patients (89.41%) of patients were asymptomatic. There was a correlation between ophthalmic involvement and platelet count and hemoglobin level. In patients with ocular signs, higher mortality rates were observed. Conclusions: At the time of diagnosis in acute leukemia patients, complete ophthalmic evaluation including dilated fundus examination is suggested as ocular involvement in these patients is common and sometimes asymptomatic. Ophthalmic involvement in leukemic patients should be identified in a timely manner, particularly in individuals with low platelet counts and hemoglobin levels, due to the potential prognostic relevance.

An mHealth Self-management System for Support Children With Acute Lymphocytic Leukemia and Their Caregivers: Qualitative Co-design Study.

BACKGROUND: The unique features of smartphones have extended their use in different fields, especially in the health care domain. These features offer new opportunities to support patients with chronic conditions by providing them with information, education, and self-management skills. We developed a digital self-management system to support children with cancer and their caregivers in Iran (low- and middle-income country). OBJECTIVE: This study is aimed at the development and preliminary evaluation of a cancer self-management system (CanSelfMan) tailored to the needs of children with cancer and their parents or caregivers. METHODS: This study was conducted in collaboration with a multidisciplinary team between January and February 2020 at MAHAK's Pediatric Cancer Treatment and Research Center. We developed a self-management system in six stages: requirement analysis, conformity assessment, preparation of educational content, app prototyping, preliminary evaluation, and developing the final version. RESULTS: A total of 35 people (n=24, 69% parents and n=11, 31% children) volunteered to participate in the study. However, only 63% (15/24) of parents and 73% (8/11) of children were eligible to participate. By adopting a user-centered design approach, we developed a mobile app, CanSelfMan, that includes five main modules (knowledge base, self-management tips, self-assessment report, ask a question, and reminders) that provide access to reliable information about acute lymphocytic leukemia and the self-management skills required for side effect measurement and reporting. A web-based dashboard was also developed for oncologists and included a dashboard to monitor users' symptoms and answer their questions. CONCLUSIONS: The CanSelfMan app can support these groups by providing access to reliable information about cancer, facilitating communication between children or parents and health care providers, and helping promote medication adherence through a reminder function. The active participation of the target group can help identify their needs. Therefore, through the involvement of stakeholders such as patients, caregivers, and oncologists in the design process, we improved usability and ensured that the final product was useful. This app is now ready to proceed with feasibility studies.

An Overview of the Current Hepatitis B Treatment Strategies after Liver Transplantation.

Currently, liver transplantation (LT) is considered as the only option for the treatment of patients with various causes of liver failure, including patients with chronic hepatitis B virus (HBV) infections. Overall, patients with HBV who undergo LT are at increased risk of hepatitis B infection recurrence. Although the current knowledge regarding the pathophysiology of this infection has been dramatically increased over the past few decades, it is still considered a complex disease process with varying degrees of clinical characteristics and changing patterns over time. There are various treatment strategies for preventing HBV recurrence in the LT setting. Generally, these regimens include oral nucleoside/ nucleotide analogues (NAs), hepatitis B immune globulin (HBIG), and vaccines or the combination of these drugs. The treatment strategy of choice should be based on cost-effectiveness, along with other patients underlying conditions. In this case, studies indicate that potent NAs are more cost-effective than HBIG in most case scenarios. In this article, we aimed to review the general medications used in the prophylaxis of the recurrence of HBV infection after LT.

Blue Rubber Bleb Nevus Syndrome because of 12 Years of Iron Deficiency Anemia in a Patient by Double Balloon Enteroscopy; A Case Report and Review of Literature.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder comprised of venous malformation mostly involving the skin and gastrointestinal (GI) tract but can also involve other visceral organs. The most predominant site of GI tract involvement is the small bowel. In patients with GI lesions, treatment depends on the severity of bleeding, and extent of involvement. Conservative therapy with iron supplementation and blood transfusion is appropriate in cases with mild bleeding but in severe cases endoscopic and surgical interventions would be beneficial. Also, medical therapy with sirolimus significantly reduces bleeding. A 20-year-old woman was referred to our hospital after transfusion of six units of packed cell because of several episodes of lower GI bleeding within the past three months in the form of melena and a single episode of hematochezia. Her last hemoglobin level before admission was 10mg/dl. She underwent various unsuccessful investigations since she was eight years old to find the origin of refractory iron deficiency anemia. In upper endoscopy, five bleeding polypoid lesions were discovered in the jejunum. Lesions were excised by snare polypectomy. Over a six-month follow-up period, no signs of lower GI bleeding were noted and the patient had a normal hemoglobin level.

Treatment and outcomes of pediatric patients with cancer and COVID-19 at MAHAK pediatric cancer treatment and research center, Tehran, Iran.

The COVID-19 pandemic has been particularly devastating for Iran. Children with cancer are generally immunosuppressed and especially vulnerable to SARS-CoV-2 infections. We report the treatment and outcomes of pediatric oncology patients with COVID-19 at the MAHAK Pediatric Cancer Treatment and Research Center (MPCTRC) in Tehran. We enrolled pediatric oncology patients who experienced SARS-CoV-2 infections from March 18, 2020, to January 28, 2021. The COVID-19 diagnostic criteria at MPCTRC were based on imaging and clinical presentation because of specific challenges diagnosing SARS-CoV-2 infections with molecular testing, which was locally developed and conducted at centers other than MPCTRC. We enrolled nine outpatients and eight inpatients (mean age = 9 years), seven of whom had a diagnosis of leukemias, and five who had brain tumors. COVID-19 symptoms were mild in fourteen patients, and three patients were asymptomatic. Of twelve patients who received molecular testing for SARS-CoV-2 infection, eight were negative and four were positive. Of nine patients tested for IgG and IgM antibodies, one was positive. Three patients died of COVID-19, all of whom were hospitalized. Mild COVID-19 symptoms did not appear to affect the outcomes of the pediatric patients with cancer who received treatment at MPCTRC during the study period.

Successful Treatment with Intravenous and Intravascular Cidofovir for BK Virus-Associated Hemorrhagic Cystitis after Allogeneic Hematopoietic Stem Cell Transplantation: A Case Report.

BK virus rarely causes disease but is typically associated with patients who have had a transplant. The cornerstone of therapy is reduction in immunosuppression. A recent surge in BKVAN correlates with use of potent immunosuppressant drugs, such as tacrolimus and mycophenolate mofetil. Studies have not shown any correlation between BKVAN and a single immunosuppressive agent but rather the overall immunosuppressive load. A 12-year-old male with recurrent acute myeloblastic leukemia (M4) was undergoing chemotherapy regimen at MAHAK Pediatric Cancer Treatment and Research Center. Following 28 days of allogenic transplantation with protocol BU/CY/Mel from his brother, he had severe hematuria in urine. So he was screened for the reason of hematuria. The results of screening showed that he had positive BK virus in urine (viral load PCR tests: 7128037228 IU/ML). According to grade IV hemorrhagic cystic, cidofovir was administered for the first time as IV and then 2 times as intravesical. After the administration of cidofovir, the symptoms of hematuria improved and the load of BK virus decreased that finally accounted as zero. Cidofovir could be the target issue in patients' recovery. Authors suggest further evaluations of cidofovir both in allogeneic stem cell transplantation setting and in renal allograft patients to consider its impact on BKV and nephropathy.

Retinoblastoma presentation, treatment and outcome in a large referral centre in Tehran: a 10-year retrospective analysis.

BACKGROUND/OBJECTIVES: Early diagnosis, care and treatment of retinoblastoma is a challengeable issue for Iranian health system. This study was designed and conducted in a referral multidisciplinary centre in the capital city of Iran to evaluate management, care, prognosis and survival rates of paediatric patients with retinoblastoma. METHODS: In this retrospective study, a total number of 309 patients younger than 15 years, diagnosed with retinoblastoma, who referred for diagnosis and treatment to MAHAK's Pediatric Cancer Treatment and Research Center (MPCTRC) from 2007 to 2017 were evaluated. All data were analyzed via SPSS version 22 software in regard of parametric and non-parametric data. Survival rates were analyzed using the Kaplan-Meyer method. RESULTS: The mean age of patients was 20 months and the majority of patients (77%) had leukocoria as a common clinical symptom at the time of diagnosis. Primary treatment methods were systemic chemotherapy (94%), laser (35%) and primary enucleation (28%). Relapses occurred in nearly 42% of cases, and the median time from diagnosis to the first relapse was 9 months. At the time analyzing the data, 11% of patients died. Patients' 5-year OS and RFS rates were 79.6% and 41.5%, respectively. CONCLUSION: Comparing results with other conducted studies identifies that the recurrence rate was high in our considered patients. Also, OS and RFS rates in our study were not as considerable as other reports. Screening methods, updating protocols and follow-up of patients may lead to improvements in survival rates of patients with retinoblastoma.

Prediction of Cranial Radiotherapy Treatment in Pediatric Acute Lymphoblastic Leukemia Patients Using Machine Learning: A Case Study at MAHAK Hospital.

BACKGROUND: Acute Lymphoblastic Leukemia (ALL) is the most common blood disease in children and is responsible for the most deaths amongst children. Due to major improvements in the treatment protocols in the 50-years period, the survivability of this disease has witnessed dramatic rise until this date which is about 90 percent. There are many investigations tending to indicate the efficiency of cranial radiotherapy found out that without that, outcome of the patients did not change and even it improved at some cases. METHODS: the main aim of this study is predicting cranial radiotherapy treatment in pediatric acute lymphoblastic leukemia patients using machine learning. Scope of this paper is intertwined with predicting the necessity of one of the treatment modalities that has been used for many years for this group of patients named Cranial Radiotherapy (CRT). For this purpose, a case study is considered at Mahak charity hospital. In this paper, our focus is on ALL patients aged 0 to 17 treated at Mahak hospital, one of the best centers for treatment of childhood malignancies in Iran. Dataset analyzed in this study is gathered by the research team from patient's paper-based files. Our dataset consists of 241 observations on patients with 31 attributes after the data cleaning process. Our designed machine learning model for predicting cranial radiotherapy treatment in pediatric acute lymphoblastic leukemia patients is a stacked ensemble classifier of independently strong models with a meta-learner to tune the weights and parameters of the base classifiers. RESULTS: The stacked ensemble classifier show highly reasonable performance with AUC of 87.52%. Moreover, the attributes are ranked based on their predictive power and the most important variable for CRT necessity prediction is the disease relapse. CONCLUSION: In order to conclude, derived from previous studies regarding CRT it is not only cost-effective but also more healthy to eradicate the use of CRT for the treatment of childhood ALL. Furthermore, it is valuable to increase the clinical databases by creating more synthetic health databases not only for research purposes but also for physicians to keep track of their patient's status.   
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Expression of AdipoR1 and AdipoR2 and Serum Level of Adiponectin in Gastric Cancer.

BACKGROUND: Cancer is one of the major causes of death worldwide and the third leading cause of death in Iran. One of the proteins that are considered having anticancer effects is the adiponectin hormone. Adiponectin leads to programmed cell death, prevents cell growth and proliferation, and increases the expression levels of BCL2. AIM: The aim of this study was to assay the expression of adiponectin receptors (AdipoR1 and AdipoR2) genes in gastric cancer patients. MATERIALS AND METHODS: In this case-control study, 42 gastric cancer patients and 52 volunteers as healthy controls were enrolled. Total RNA was extracted. cDNA was synthesized by the reverse transcription method, and expression analysis was performed by real-time PCR. The serum level of adiponectin was also measured by ELISA. RESULTS: The expression of both AdipoR1 and AdipoR2 was significantly higher than the control group (p = 0.02). Serum adiponectin was significantly lower in gastric cancer cases when compared with normal controls (p = 0.03). CONCLUSION: We found that expression level of AdipoR1 and AdipoR2 is strongly higher; however, the level of circulating adiponectin is lower in gastric cancer. Our study suggests that the expression of AdipoR1 and AdipoR2, besides the low level of adiponectin, may play an important role in the development and/or progression of gastric cancer.

Pulmonary Complications in Candidates for Liver Transplantation.

The liver plays a pivotal role in maintaining the homeostasis of various organ systems. Also, end-stage liver disease and its complications are major causes of morbidity and mortality among adults. Individuals who develop a chronic liver disease are at increased risk of progression to multi-organ dysfunction, including the pulmonary system. The clinical complications of pulmonary problems related to the presence of liver disease range from mild (such as hypoxemia) to life-threatening diseases (such as portopulmonary hypertension and hepatopulmonary syndrome). Herein, the major pulmonary complications related to liver cirrhosis and considerations for performing liver transplantation are reviewed.

The role of artificial intelligence in colon polyps detection.

Over the past few decades, artificial intelligence (AI) has evolved dramatically and is believed to have a significant impact on all aspects of technology and daily life. The use of AI in the healthcare system has been rapidly growing, owing to the large amount of data. Various methods of AI including machine learning, deep learning and convolutional neural network (CNN) have been used in diagnostic imaging, which have helped physicians in the accurate diagnosis of diseases and determination of appropriate treatment for them. Using and collecting a huge number of digital images and medical records has led to the creation of big data over a time period. Currently, considerations regarding the diagnosis of various presentations in all endoscopic procedures and imaging findings are solely handled by endoscopists. Moreover, AI has shown to be highly effective in the field of gastroenterology in terms of diagnosis, prognosis, and image processing. Herein, this review aimed to discuss different aspects of AI use for early detection and treatment of gastroenterology diseases.

Impact of the coronavirus disease 2019 (COVID-19) pandemic on pediatric oncology care in the Middle East, North Africa, and West Asia region: A report from the Pediatric Oncology East and Mediterranean (POEM) group.

BACKGROUND: Childhood cancer is a highly curable disease when timely diagnosis and appropriate therapy are provided. A negative impact of the coronavirus disease 2019 (COVID-19) pandemic on access to care for children with cancer is likely but has not been evaluated. METHODS: A 34-item survey focusing on barriers to pediatric oncology management during the COVID-19 pandemic was distributed to heads of pediatric oncology units within the Pediatric Oncology East and Mediterranean (POEM) collaborative group, from the Middle East, North Africa, and West Asia. Responses were collected on April 11 through 22, 2020. Corresponding rates of proven COVID-19 cases and deaths were retrieved from the World Health Organization database. RESULTS: In total, 34 centers from 19 countries participated. Almost all centers applied guidelines to optimize resource utilization and safety, including delaying off-treatment visits, rotating and reducing staff, and implementing social distancing, hand hygiene measures, and personal protective equipment use. Essential treatments, including chemotherapy, surgery, and radiation therapy, were delayed in 29% to 44% of centers, and 24% of centers restricted acceptance of new patients. Clinical care delivery was reported as negatively affected in 28% of centers. Greater than 70% of centers reported shortages in blood products, and 47% to 62% reported interruptions in surgery and radiation as well as medication shortages. However, bed availability was affected in <30% of centers, reflecting the low rates of COVID-19 hospitalizations in the corresponding countries at the time of the survey. CONCLUSIONS: Mechanisms to approach childhood cancer treatment delivery during crises need to be re-evaluated, because treatment interruptions and delays are expected to affect patient outcomes in this otherwise largely curable disease.

An update on treatment options for primary sclerosing cholangitis.

Primary sclerosing cholangitis is a chronic cholestatic liver disease defined by strictures of the biliary tree which could ultimately lead to liver cirrhosis and cholangiocarcinoma. Although the exact underlying etiology of this disorder is not fully understood, the pathology is believed to be caused by immune mediated mechanisms. Growing body of evidence suggests several treatment modalities mainly focusing on the inflammation aspect of this disorder. However, there is still no consensus regarding the best treatment option for these patients. Thus, the present study aimed to review the current treatment options for patients with primary sclerosing cholangitis.

Cholangiocarcinoma: State of the Art.

BACKGROUND: Cholangiocarcinoma (CCA) is the second most frequent primary liver tumor and defined as the heterogeneous group of tumors derived from cells in the biliary tree. METHODS AND RESULTS: Based on the anatomical locations (intrahepatic, perihilar, and distal), there are various approaches to the diagnosis and treatment of CCA. Imaging modalities, staging classifications, understandings around natural behavior of CCA, and therapeutic strategies have had remarkable progress in recent years. CONCLUSIONS: This article reviews and discusses the epidemiology, clinical presentation, diagnosis, and treatment modalities of CCA; determines the appropriate inclusion and exclusion criteria for liver transplantation (LT); and defines the risk of disease progression for patients in the waiting list of LT.

Characterizing Iranian Pediatric Patients With Relapsed Acute Lymphoblastic Leukemia Through Gene Expression Profiling of Common ATP Binding Cassette Transporters Subfamily C.

BACKGROUND: The correlation between gene expression of ABCC transporters and recurrence as a treatment failure in pediatric patients with acute lymphoblastic leukemia (ALL) is an unsolved problem in scientific associations. The aim of this study was to evaluate the predictive value of ABCC1-6 gene expression pattern for estimating recurrence in Iranian pediatric patients with ALL. MATERIALS AND METHODS: Iranian pediatric patients with approved ALL enrolled in this study as 2 groups of case (relapsed ALL) and control (treated individuals who lasted for >3 years following their final treatment). Real-time polymerase chain reaction was done with GAPDH for expressing ABCC1-6 transporter genes. Cumulative doses of Vincristine, Daunorubicin, and L-Asparginase were checked for each patient. Gathered data analyzed with SPSS version 22 and REST 2009 software. RESULTS: Thirty-nine samples as 23 relapsed ALL and 16 controls enrolled. High expression of ABCC2-6 and low expression of ABCC1 were detected in pediatric patients with relapse. ABCC3 and ABCC4 had significant relation with high-risk patients of NCI group. Also, ABCC4 and ABCC6 had more expression with high doses of Daunorubicin and L-Asparginase. CONCLUSIONS: Designed expression pattern have the predictive value for estimating of conferring relapse in Iranian pediatric patients with diagnosed ALL. The authors suggest of designing a multiple childhood malignancy center project to evaluate this pattern in a cohort study.