The commencement of congenital heart diseases registry in Isfahan, Iran: Methodology and design.

BACKGROUND: Reported prevalence of congenital heart diseases (CHDs) varies widely among studies worldwide. The incidence of CHD, total number of pediatric and adult grown-up congenital heart disease (GUCH), is not determined in Iran. Therefore, we have designed a system to register the information of patients with CHD for the first time in our country. METHODS: CHD registry is a database in which the patients' data are collected by five pediatric cardiologists from three referral hospitals affiliated to Isfahan University of Medical Sciences, Isfahan, Iran, and five outpatient clinics. We enrolled patients with CHD either as new cases who were referred for evaluation of potential CHD or those who were being followed within the outpatient clinics and entered their whole information in a website specifically designed for it. All the information was collected from checklist by those pediatric cardiologists. RESULTS: From April 2017 to April 2020, after developing the forms and website, the Quality Control Committee evaluated the first 558 files. 73 files (13%) needed major revisions. Among them, 34 (46%) files were omitted totally and the 39 remaining files were revised and completed. After that revision, we changed our checklist and gathered about 1600 patients accordingly. CONCLUSION: Registry of CHDs not only improves epidemiologic studies but also assists researchers to understand how much a disease management is useful and how to raise the quality of cares and outcomes. Moreover, this provides a better insight for policymakers to understand the extent of health-related problems as well as the issues related to the prevention and management of CHDs all around the world.

Assessing Iranians' parental competence: Development and psychometric properties of the Children's Sexual Behavior Questionnaire (CSBQ-IR), Iranian Version.

Background: Parents are the first line mediators in sexuality development of children. However, the majority of parents tend to have close supervision on children sexual behaviors, particularly in the conservative cultures. This article describes the development and psychometric evaluation of an instrument to measure Iranian parents' competence in caring and nurturing their children sexually. Methods: The questionnaire was designed based on the principles in measurement, Waltz'stheory. The Iranian version of Children's Sexual Behavior Questionnaire (CSBQ-IR) was developed and completed by 386 mothers and 101 fathers who participated in a community based sexuality education program in Tehran, capital of Iran. Reliability was assessed by Kuder-Richardson reliability coefficient and Split half. CSBQ-IR was evaluated for its construct, inclusiveness and content validity by principal component analysis. Results: The Kuder-Richardson reliability coefficient and Split half reliability were found 0.425 and 0.457 that was on acceptable range. Meaning, grammar, wording and item allocation of the questionnaire were found to be appropriate with (content validity ratio [CVR]=0.99) and(content validity index [CVI]=0.8) respectively. Conclusion: CSBQ-IR provides a valid assessment of the parents' competency or incompetency in nurturing, communication, and managing their children's sexual behaviors.

The effect of tadalafil on functional capacity and echocardiographic parameters in patients with repaired Tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital heart diseases (CHD) in children. Various surgical procedures including palliative shunts and TOF total correction (TFTC) were done with some complications, of which, the most common is pulmonary valve regurgitation (PR). Tadalafil is a phosphodiesterase 5 inhibitor which reduces pulmonary vascular resistance, and improves right ventricular function and vascular endothelium, and may have some beneficial effects after TFTC. METHODS: We studied 18 patients with TOF and PR, with some impaired right ventricular function after TFTC. Tadalafil tablets at a dose of 1 mg per kg (maximum 40 mg) per day as a single dose was administered orally for 8 weeks. In all patients, before and after taking tadalafil, functional class assessment, electrocardiography (ECG) changes, some echocardiographic and endothelial function parameters [flow-mediated dilation (FMD) and intima-media thickness (IMT) of carotid artery], and exercise test were determined. RESULTS: The patient's mean age was 10.11 ± 4.03 years, and the mean age of operation was 2.52 ± 1.12 years. The effect of tadalafil on different echocardiographic parameters and also on tricuspid valve regurgitation (TR) and PR severity and gradient was not significant. Moreover, it had no effects on QRS duration. Tadalafil had a significant effect on improving FMD and exercise test (P = 0.01). The effect of tadalafil on echocardiographic parameters, carotid artery IMT, and ECG parameters was not significant (P > 0.05). Tadalafil was tolerated well, and the most common side effects were headache and myalgia. CONCLUSION: This study showed that tadalafil is a safe and well-tolerated drug. It might improve exercise performance, endothelial function, and functional class, and possibly could allow patients a longer period of well-being and could possibly delay the need for pulmonary valve replacement (PVR).

Comparison of the therapeutic effects and side effects of tadalafil and sildenafil after surgery in young infants with pulmonary arterial hypertension due to systemic-to-pulmonary shunts.

Young children with CHD and large systemic-to-pulmonary shunts eventually develop pulmonary hypertension. At present, phosphodiesterase type-5 inhibitors such as sildenafil have been used to control pulmonary pressure before and after cardiac surgery. Recently, tadalafil has been utilised in older children with similar efficacy, but it has been used to a lesser extent in young infants. From April, 2015 to June, 2016, 42 patients aged 3-24 months with a large septal defect and pulmonary arterial hypertension were randomly divided into two equal groups: one group received oral sildenafil (1-3 mg/kg/day every 8 hours), whereas the other group received oral tadalafil (1 mg/kg once a day) from 7-10 days before surgery to 3-4 weeks after surgery. During the first 48 hours after surgery, pulmonary artery-to-aortic pressure ratio and recorded systolic pulmonary artery pressures were not significantly different between the two groups (p>0.05); moreover, there were no differences in paediatric ICU length of stay, mechanical ventilation time, clinical findings of low cardiac output state, and echocardiographic data between the two groups (p>0.05). Most of the patients had no side effects, and only five patients had a minor with no significant difference in both groups (p=0.371). Tadalafil can be considered as an effective oral therapy for preoperative and postoperative pulmonary hypertension in young infants. It can be administered at a once-daily dose with an appropriate efficacy and safety profile as sildenafil, and therefore it can be considered as an alternative to sildenafil in young children.

Persian Registry Of cardioVascular diseasE (PROVE): Design and methodology.

BACKGROUND: Our aim was to create and establish a database called "Persian Registry Of cardioVascular diseasE (PROVE)" in order to be used for future research and in addition, as a tool to develop national guidelines for diagnosis, treatment, and prevention of cardiovascular disease (CVD). In this paper, the design and methodology of the PROVE pilot study will be discussed, launched in Isfahan, Iran, in 2015-2016. METHODS: Through establishing PROVE, patients' data were collected from hospitals and outpatient clinics prospectively or retrospectively and followed up for a maximum of three years based on the type of CVDs. The inclusion criteria were as patients with acute coronary syndrome (ACS), ST elevation myocardial infarction (STEMI), stroke, atrial fibrillation (AF), heart failure (HF), congenital heart disease (CHD), percutaneous coronary intervention (PCI), and chronic ischemic cardiovascular disease (CICD). Specific protocols, questionnaires, and glossaries were developed for each registry. In order to ensure the validation of the protocols, questionnaires, data collection, management, and analysis, a well-established quality control (QC) protocol was developed and implemented. Data confidentiality was considered. RESULTS: In order to register patients with ACS, STEMI, stroke, HF, PCI, and CICD, the hospital recorded data were used, whereas, in case of AF and CHD registries, the data were collected from hospitals and outpatient clinics. During the pilot phase of the study in Isfahan, from March 2015 to September 2016, 9427 patients were registered as ACS including 809 as STEMI, 1195 patients with HF, 363 with AF, 761 with stroke, 1136 with CHD, 1200 with PCI, and 9 with CICD. Data collection and management were performed under the supervision of the QC group. CONCLUSION: PROVE was developed and implemented in Isfahan as a pilot study, in order to be implemented at national level in future. It provides a valuable source of valid data that could be used for future research, re-evaluation of current CVD management and more specifically, gap analysis and as a tool for assessment of the type of CVDs, prevention, treatment, and control by health care decision makers.

Assessment of immunogenic characteristics of Hemiscorpius lepturus venom and its cross-reactivity with venoms from Androctonus crassicauda and Mesobuthus eupeus.

Hemiscorpius lepturus (H. lepturus), one of the most venomous scorpions in tropical and sub-tropical areas, belongs to the Hemiscorpiidae family. Studies of antibodies in sera against the protein component of the venom from this organism can be of great use for the development of engineered variants of proteins for eventual use in the diagnosis/treatment of, and prevention of reactions to, stings. In the present in vitro study, the proteins of H. lepturus venom, which could specifically activate the production of immunoglobulin G (IgG) in victims accidently exposed to the venom from this scorpion, were evaluated and their cross-reactivity with venoms from two other important scorpion species including Androctonus crassicauda and Mesobuthus eupeus assessed. H. lepturus venom was analyzed with respect to its protein composition and its antigenic properties against antibodies found in sera collected from victims exposed to the venom of this scorpion within a previous 2-month period. The cross-reactivity of the H. lepturus venom with those from A. crassicauda and M. eupeus was assessed using ELISA and immunoblotting. Electrophoretic analysis of the venom of H. lepturus revealed several protein bands with weights of 8-116 KDa. The most frequent IgG-reactive bands in the test sera had weights of 34, 50, and 116 kDa. A weak cross-reactivity H. lepturus of venom with venoms from A. crassicauda and M. eupeus was detected. The results of immunoblotting and ELISA experiments revealed that H. lepturus venom activated the host immune response, leading to the production of a high titer of antibodies. Clearly, a determination of the major immunogenic components of H. lepturus venom could be valuable for future studies and ultimately of great importance for the potential production of recombinant or hypo-venom variants of these proteins.

Municipal solid waste landfill site selection with geographic information systems and analytical hierarchy process: a case study in Mahshahr County, Iran.

Landfill siting is a complicated process because it must combine social, environmental and technical factors. In this study, in order to consider all factors and rating criteria, a combination of geographic information systems and analytical hierarchy process (AHP) was used to determine the best sites for disposal of municipal solid waste (MSW) in Mahshahr County, Iran. In order to the decision making for landfill siting a structural hierarchy formed and the most important criteria: surface water, sensitive ecosystems, land cover, urban and rural areas, land uses, distance to roads, slope and land type were chosen according to standards and regulations. Each criterion was evaluated by rating methods. In the next step the relative importance of criteria to each other was determined by AHP. Land suitability for landfill was evaluated by simple additive weighting method. According to the landfill suitability map, the study area classified to four categories: high, moderate, low and very low suitability areas, which represented 18.6%, 20.3%, 1.6 and 0.8% of the study area respectively. The other 58.7% of the study area was determined to be completely unsuitable for landfill. By considering the parameters, such as the required area for landfill, distance to MSW generation points, and political and management issues, and consulting with municipalities managers in the study area, six sites were chosen for site visiting. The result of field study showed that it is a supplementary, and necessary, step in finding the best candidate landfill site from land with high suitability.