not

Lower gastrointestinal cancer patients like other immunosuppressed patients are vulnerable to develop more severe infections. Iran is one of the countries with high incidence of COVID-19 and some modifications are needed to adjust international protocols to deal with this pandemic. Therefore, our cancer institute has implemented some changes in the current treatment guidelines. In each specialty, all members agreed to choose minimal intervention. The members know that some recommendations may interfere with the routine best-practice recommendations and decrease the quality measures in the patient's outcome. Therefore, these recommendations are valid just in epidemic COVID-19 situation in the country. According to the consensus of colorectal and cancer surgery professors, if a patient is a new case of rectal cancer, he or she should be referred to undergo neoadjuvant chemoradiotherapy. But if the patient comes eight to twelve weeks after receiving chemoradiotherapy in accordance with COVID-19 epidemic phase, it may be possible to delay surgery. A stent can be implanted if a patient with rectal cancer presents with obstructive symptoms and surgery can be postponed until resolving the crisis of the COVID-19 epidemic or its downward trend. For colon cancer, we will request a thoracic spiral CT scan. If there was no evidence of pulmonary involvement with COVID-19, the patient will undergo open surgery considering patient's health and protection tips.

Background: Cardiomyopathies are characterized by structural and functional abnormalities of myocardium that will probably lead to premature death from arrhythmia and/or progressive heart failure. Myocarditis is an inflammatory disease of the myocardium diagnosed by histological criteria definitively. To date, most information about the natural history and management of cardiomyopathies and myocarditis has derived from cohort studies in a small number of specialized centers.

Aim: The prevalence of patients with cardiomyopathies who referred to Rajaei Cardiovascular medical and research center from all over the country is remarkable.  Rajaie Cardiomyopathy and myocarditis Registry (RCMR) study is an observational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM) as well as myocarditis designed to determine clinical characteristics, natural history, current therapeutic approaches, response to treatment and long-term outcomes of patients with cardiomyopathy and myocarditis. Prediction of mortality and response to different treatments in these patients using artificial intelligence is another aim of this registry. Covid 19 Myocarditis and its sequence as cardiomyopathy seems a new challenge in forthcoming years.

Methods: Patients with a history of documented cardiomyopathy/ myocarditis fulfilling standard diagnostic criteria based on the ESC guideline for cardiomyopathy/ myocarditis and willing and able to give informed consent or in the case of child consent from a parent will be included. At baseline visit , past medical history, clinical signs/ symptoms, risk factors, physical examination and family history of cardiomyopathy, as well current standards for diagnostic workup and clinical follow-up and where relevant electrocardiogram, echocardiography, cardiac magnetic resonance, Holter monitoring, or  biomarker analyses will be checked. Based on the type of disease and the physician's opinion, patients will undergo exercise test, electrophysiology study or endomyocardial biopsy. Outcome and results of various therapeutic approaches currently employed for patients including implantable cardioverter defibrillator, cardiac resynchronization therapy, septal myectomy, ablation, cardiac transplantation and medications will be assessed. Long-term outcomes including the benefits and complications of therapeutic interventions will be collected. Follow-up visit will be scheduled after 12 months for all patients and survival status, hospitalizations, co-morbidities, medications will be assessed. Data will be collected on a research electronic data source (Regitory) and will be analyzed at regular intervals based on study objectives and also by using artificial intelligence.

Conclusion: RCMR aims to make a major contribution to understanding the natural history of the cardiomyopathy/ myocarditis, as well as guiding evidence-based decision making. Potential gaps with existing recommendations will be discussed as well as some suggestions for improvement of health care provision.

Rheumatoid arthritis (RA) is a progressive and common autoimmune disease with multifactorial etiology. Several pieces of research show that genetic factors play a major role in the incidence of RA. Several genome-wide association studies (GWAS) have identified the autoimmune regulator (AIRE) gene as one of the candidate loci. This gene encodes a transcription factor, which is involved in the presentation of self-antigens and negative selection of self-reactive T-cells in the thymus. Studies have indicated that single nucleotide polymorphisms (SNPs) in the AIRE gene can change the gene expression and/or function. In the present study, we assessed the possible association between SNP rs2075876 (intronic variant) in the AIRE gene with RA risk in the Iranian population. A case-control study using 56 RA patients and 58 control subjects was undertaken to evaluate rs2075876 genotypes using real-time PCR high resolution melting method (HRM). Logistic regression analysis demonstrates that homozygous AA and heterozygous AG genotypes compared with GG genotype increase the risk of RA (AA vs GG; OR= 16.43; 95%CI [5.33-50.71] and AG vs GG; OR= 3.21; 95%CI [1.22-8.45]). Also, individuals with allele A were more frequently affected with RA than subjects with G allele (OR= 5.81; 95%CI [3.28-10.30]). Furthermore, in the patient group, we found a significant correlation between erythrocyte sedimentation rate and C-reactive protein concentration with rs2075876 polymorphism (P< 0.05). Our findings propose a substantial correlation between rs2075876 polymorphism and RA risk.

HOIL-1/RBCK1 deficiency is a new autosomal receive disorder with unstable cellular responses to pro-inflammatory cytokines, resulting in auto-inflammation, pyogenic bacterial disease, as well as the development of muscular amylopectinosis. This study explored the molecular mechanisms of RBCK1 deficiency with integrated bioinformatics analyses of the feature genes and the correlative gene functions. The expression profile of GSE24519 was downloaded from the Gene Expression Omnibus database. Differentially expressed genes (DEGs) between RBCK1, MYDK88, NEMO deficient fibroblast, and healthy fibroblast specimens were identified. Gene ontology (GO) enrichment analysis on gene functions and the Kyoto Encyclopedia of Gene and Genome (KEGG) pathway analysis were performed by using Database for Annotation, Visualization, and Integrated Discovery (DAVID). Cytoscape was used to visualize the protein-protein interaction (PPI) of these DEGs. GO analysis revealed that the “Skeletal system development, Extracellular matrix organization, Positive regulation of cell migration, Negative regulation of canonical Wnt signaling pathway, Cell adhesion, Angiogenesis and Negative regulation of BMP signaling pathway, Serine-type carboxypeptidase activity, Polysaccharide binding, Calcium ion binding, frizzled binding, Neuropilin binding, and cell adhesion molecule binding, extracellular exosome, extracellular space, extracellular region, lysosomal lumen, endoplasmic reticulum lumen, cell surface and focal adhesion to BP, MF, and CC, respectively. The KEGG pathway analysis showed that the complement and coagulation cascade, ECM receptor interactions, PI3K- Akt signaling pathway, PPAR signaling pathway, TGF-beta signaling pathway, Pathway in Cancer, Viral carcinogenesis and Focal adhesion pathway were closely associated with RBCK1 deficiency occurrence. Importantly, TK1, AURKB, CDCA2, UBE2C, KIFC1, CEP55, CDCA3, GINS2, MCM6, and CDC45 were predicted to be significantly related to RCBK1 deficiency. Our discovery provides a registry of genes and pathways that are disrupted in RCBK1, which will enhance in understanding the pathogenesis of RBCK1 deficiency and other innate immunodeficiency diseases. This study has the potential to be used in the clinic for diagnosis and targeted therapy of RCBKI and other innate immunodeficiencies in the future.

Purpose: Down Syndrome (DS) is a genetic impairment has comorbidities such as Congenital Heart Disease (CHD). Pulmonary Atrial Hypertension (PAH) is a complication of CHD in these patients; however, there are no sufficient documents about the prevalence of PAH in CHD-DS patients in Iran. The purpose of this study is to investigate the prevalence of (PAH) in CHD-DS patients.

Method: This is a cross-sectional study that conducted on CHD-DS patients referred to Imam Reza hospital, Mashhad, Iran (April 2015- February 2016). The comparison group includes non-DS (NDS) children with CHD. Both groups were same in terms of age and gender. Echocardiography was run for all patients to determine CHD types and pulmonary arterial pressure (PAP).

Results: the cohort of 77 patients (including 47 in DS group and 30 in comparison group) was studied. It was observed that 66% of the DS group and 56.7% of the NDS group were female (P=0.412). The average age of case and comparison group were 15.1±25.83 and 36.6±44.17 month, respectively (p=0.108).

Most of the CHD-DS patients (63.8%) had multiple heart disease. The most common impairments among the DS-CHD group were Ventricular Septal Defect (VSD), Atrial Septal Defect (ASD), and Patent Ductus Arteriosus (PDA).48.9% of the DS patients and 23.3% of NDS group had developed PAH which demonstrates a significant higher rate among DS patients (p=0.025).

Conclusions: Our findings denote higher prevalence of PAH among CHD-DS patients. Such an observation is a meaningful warning for the studied patients to take necessary medical therapies in order to prevent complications and irreversible pulmonary vascular disease.

Background: Deformities of burn scars in chin and upper neck are a tragedy for patients and pose a great challenge to plastic surgeons due to limited available methods for reconstruction of three-dimensional appearance. In this paper we present a new technique for correction of cervicomental angle with a dermal fat flap.

Methods: Fourteen patients ages from 18 to 24 years old with extensive neck deformities were enrolled to the study. During the operation, first we deepithelialized a triangular scar area in the upper neck, then created a dermal fat flap and flip it up to the chin, restoring the chin shape and volume, after that for subsequent reconstruction of cervicomental angle, after releasing skin from lateral sides of the triangle defect, we approximated the AB and AC line to the upper edge of the defect by V-Y advancement. The remaining defect covered with a full-thickness skin graft.

Result: In all cases, the chins were reshaped with a dermal fat flap. The mean cervicomental angle was 144 + 14.6° preoperatively that was reduced to 108.9+6.15° postoperatively. The neck and chin preserved with better contours.

Conclusion: This is a new technique which has never been described before in the literature and it is innovative compared to the traditional techniques. Good cosmetic outcomes make this technique preferable for all burn neck deformity with obtunded cervicomental angle.

Key word: burns scar, neck, flaps.

Objective: Colorectal cancer is one of the most common cancers in Iran. Regarding the prevalence of this cancer and its mortality and morbidity, in this study, 5 Year Survival Rate and its Effective Factors of patients with colorectal cancer investigated.

Material and Methods: This study was conducted using retrospective cohort method. All patients diagnosed with colorectal cancer during the 2006-2011 years in Hamadan Imam Khomeini Clinic of Hematology and Oncology and Mahdieh Oncology Center were studied. Require data were extracted from their medical records. To obtain information about patients, telephone calls were made. The data were analyzed by SPPS version 16 and assessment of survival rates was conducted using Kaplan-Meier methods and Cox regression method.

Results: A total number of 108 patients with colorectal cancer were included in the study. The status of 74 patients was determined at the end of the study by follow up call   . The one, two, three, four and five overall were 77, 66, 50, 45 and 42%, respectively. The median overall survival was 46.8 months (1.3-135.6 months). Cox regression analysis showed that Metastatic tumor (p value= 0.001), lymphatic involvement (p value= 0.043) and being associated with underlying disease (p value= 0.025) was accompanied by increased risk. Multivariate cox regression test showed that metastasis was associated with increasing risk of death significantly (HR=2.83, p value= 0.013).

 Conclusion: According to the findings of the study, early screening for increasing the survival rate is recommended for people with greater risk.

In order to induce sedation during cataract surgery, various medications such as midazolam propofol and narcotics with different side effects are used in separation or in combination. Dexmedetomidine has no effect on the respiratory  system, but being dependent on dosage, it may cause cardiovasculardisorders . The present study aims to compare hemodynamic and sedative effects of dexmedetomidine and the combination of sufentanil-midazolam on patients undergoing cataract surgery. In a randomized clinical trial study, 60 patients were randomly divided into two dexmedetomidine and sufentanil-midazolam groups. In sufentanil – midazolam group,  dexmedetomidine  (DEX infusion at 0.5 μg/ kg for 10 minutes, then adjusted to 0.2 µg/kg/h) was prescribed.  In the sulfentanil-midazolam group,  sufentanil (0.1 μg /kg for 5  minutes )  and midazolam(0.2µg/kg) was injected five minutes before the operation.  Hemodynamic variables (Systolic blood pressure, diastolic blood pressure, heart rate), complications (nausea, vomiting, hypoxia), sedation level and pain intensity were recorded  (in the beginning of the study, 5, 10 minutes after anesthesia, at the start of surgery, 5, 10, 15 minutes after the surgery) as well as patient’s satisfaction, surgeon's satisfaction and complications.Results suggest that apart from gender, other primary characteristics of patients including age, history of blood pressure, diabetes history, ASA score, mean of systolic, diastolic blood pressure, heart rate and SPO2 levels were similar in both groups (P> 0.05). Systolic blood pressure   patients receiving dexmedetomidine  declined significantly more than that of patients receiving sufentanil-midazolam (P> 0.5). Diastolic blood pressure suddenly fell 5 minutes after the infusion of sufentanil-midazolam (P> 0.05), but then a relative increase and finally a relative decrease occurred  , while diastolic blood pressure in patients receiving dexmedetomidine decreased steadily . The mean heart rate in patients receiving dexmedetomidine and sufentanil-midazolam declined gently (P> 0.05). SPO2 reduced significantly in the sufentanil-midazolam group (P <0.05). drugs used  in both groups reduced pain intensity equally  (P> 0.05). From the beginning of the study, dexmedetomidine produced a relatively stable sedation level (score 2) based on Ramsay's criteria, while the combination of sufentanil-midazolam-medications cause deeper sedation (score 3) in patients (P <0.05). Despite this fact , 23.33% of the patient receiving sufentanil-midazolam could have movements during the surgerywhich was 6.66%  higher in patients receiving dexmedetomidine (P = 0.071). The satisfaction of patients receiving dexmedetomidine was significantly higher (P = 0.044), while the surgeon's satisfaction was almost identical in both anesthesia procedures (P = 0.94).

In the end, the results of the present study showed that although dexmedetomidine is associated with few respiratory problems and higher satisfaction of patients, it decreases blood pressure and heart rate progressively. However, it seems that this medicine is more effective than a combination of midazolam-sufentanil  because of more patient satisfaction, lack of hypoxia, fewer complications, and more suitable immobility.

Introduction: Bilious vomiting is highly suggestive of an acute and emergent condition among neonates. The aim of this study was to investigate the non-surgical causes of bilious vomiting in neonates admitted to a tertiary center and to compare them with the surgical causes.

Methods: This cross-sectional study was performed on 80 infants with bilious vomiting who were admitted at the neonatal intensive care unit of a pediatric tertiary center over 2 years. The demographic characteristics, clinical symptoms and signs, diagnostic assessments and therapeutic approaches were recorded.

Results: The mean age of neonates was 9.07 ± 8.84 days and 55% of them were males. The most common final diagnosis was: Necrotizing Enterocolitis (NEC), duodenal atresia, Hirschsprung’s disease, gastroesophageal reflux disease (GERD), volvulus, sepsis, meconium plug, isolated mal-rotation, metabolic abnormalities, imperforate anus, and Ladd’s bands, respectively. Abnormal findings in ultrasound and X-rays were detected in 35% and 46.3%, respectively. About half of the affected neonates were treated non-surgically. Overall, 17.5% of the patients died.  Most deaths were seen in infants with NEC. Using multivariable logistic regression analysis, the presence of NEC as an underlying etiology was the only predictor of neonatal death in neonates with bilious vomiting (OR = 12.455, 95%CI: 1.365 – 113.618, P = 0.025).

Conclusion: The most common cause of bilious vomiting was NEC followed by duodenal atresia. Half of the neonates with bilious vomiting were treated medically without operation