nNav1.5 expression is associated with glutamate level in breast cancer cells

Abstract Background: Glutamate and voltage-gated sodium channels, both have been the target of intense investigation for its involvement in carcinogenesis and progression of malignant disease. Breast cancer with increased level of glutamate often metastasize to other organs (especially bone), whilst re-expression of 'neonatal' Nav1.5, nNav1.5 in breast cancer is known to promote cell invasion in vitro, metastasis in vivo and positive lymph node metastasis in patients. Methods: In this study, the role of nNav1.5 in regulating glutamate level in human breast cancer cells was examined using pharmacological approach (VGSCs specific blocker, TTX, glutamate release inhibitor, riluzole and siRNA-nNav1.5). Effect of these agents were evaluated based on endogenous and exogenous glutamate concentration using glutamate fluorometric assay, mRNA expression of nNav1.5 using qPCR and finally, invasion using 3D culture assay. Results: Endogenous and exogenous glutamate levels were significantly higher in aggressive human breast cancer cells, MDA-MB-231 cells compared to less aggressive human breast cancer cells, MCF-7 and non-cancerous human breast epithelial cells, MCF-10A. Treatment with TTX to MDA-MB-231 cells resulted in significant reduction of endogenous and exogenous glutamate levels corresponded with significant suppression of cell invasion. Subsequently, downregulation of nNav1.5 gene was observed in TTX-treated cells. Conclusions: An interesting link between nNav1.5 expression and glutamate level in aggressive breast cancer cells was detected and requires further investigation.

Acute Myocardial Infarction Patients’ Knowledge Regarding the Modifiable Risk Factors of Heart Disease

Background: Acute myocardial infarction (heart attack) is a condition of the heart that occurs when blood flowto the heart muscles is stopped abruptly. The disruption in blood flow to the heart muscles is usually caused by ablockage of one or several coronary arteries. Heart attacks can be fatal owing to the critical functions performedby blood in the heart. Fatalities arising from heart attacks are witnessed all over the world. The difference infatality rates of heart attacks is highly dependent on the exposure to risk factors such as smoking and obesity.Healthy living populations will rarely encounter high cases of a heart attack. This study was set out to assess anddocument the knowledge level of acute myocardial infarction patients regarding the modifiable risk factors of aheart attack in Saudi Arabia. Patients suffering from a heart attack are at great danger of fatality. Knowledge ofmodifiable factors can promote healthy living habits and behaviors among them. Methods: A cross-sectionalsurvey was developed to assess knowledge levels of acute myocardial infarction patients in the three cities ofRiyadh, Jeddah, and Dammam. A total of 123 participants were recruited through convenient sampling across15 hospitals in the cities. The eligibility criteria for recruitment included a mandatory age of 18 years and SaudiArabian citizenship. Participants were issued with questionnaires containing different questions on modifiablerisk factors (fatty foods, vegetables, fruits, smoking, physical exercise, and obesity). Results: Five out of the sixmodifiable factors represented knowledge levels of less than 50%. Participants were only knowledgeable on therisk factor of obesity which saw 52.8% associating it with a heart attack. Conclusion: There is a need to boosteducation and awareness among acute myocardial infarction patients in Saudi Arabia.

Impact of blood transfusion on troponin I levels and outcomes after cardiac surgery: a cohort study

Objectives: Increased cardiac troponin I [TI] has been suggested to be a sensitive indicator of intraoperative myocardial injury. We investigated the association of transfusion on TI levels post-surgery and outcomes in patients undergoing elective cardiac surgeries

Methods: We conducted a retrospective review of 542 patients. Patients were divided into two groups based on TI levels at 24 hours [TI24] [> 6.5 microg/L vs.

Results: Red blood cell [RBC] transfusion was found to be associated with high TI levels [odds ratio [OR] = 2.33, p = 0.007, 95% confidence interval [CI]: 1.30–4.30]. A trend was observed when aortic cross-clamp time and preoperative ejection fraction were adjusted for [OR = 2.06, p = 0.080, 95% CI: 0.90–4.70]. An association was found between aortic cross-clamp time and high TI levels in the multivariable model [OR = 1.01, p = 0.028, 95% CI: 1.00–1.02]. Elevated TI levels was associated with higher mortality [OR = 4.15, p = 0.017, 95% CI: 1.29–13.08], renal failure [OR = 2.99, p = 0.004, 95% CI: 1.41-6.32], and increased length of stay in-hospital [OR = 4.50, p = 0.020, 95% CI: 0.69-8.30]

Conclusions: RBC transfusion is associated with increased TI24 post-cardiac surgery and worse outcomes, albeit a confounding effect cannot be excluded. Larger studies are required to confirm these findings

Prevalence and risk factors of fatty liver among adults

Background: Non-alcoholic fatty liver disease [NAFLD] is a heterogeneous condition that contains steatosis and non-alcoholic steatohepatitis [NASH], in the nonexistence of significant alcohol consumption, reaching 30% of the populace. The most common risk features are: age, ethnicity, gender, obesity, drugs, diabetes mellitus [DM], insulin resistance [IR], predisposition, metabolic syndrome [MS], and polycystic ovary syndrome

Materials and Methods: Patients with non-alcoholic fatty liver disease were evaluated, with medical and epidemiological data collected after informed consent at King Abdulaziz Hospital

Results: Of the 124 patients evaluated, 75.8% were women, and 88 were aged between 49 and 70 years and had no symptoms. Ultrasonography results showed steatosis in 84%. NASH was diagnosed in 75 patients of the sample. 42 patients underwent liver biopsy, of which 36% had cirrhosis, 1 had liver cancer, and 1 pure steatosis [5% each]. Risk factors were found in 70% of patients with metabolic syndrome, 87% with increased waist circumference, 63% with dyslipidemia, 61% [n=76] with high blood pressure [HBP], 28% with DM, 52% physically inactive, and 44% with insulin resistance [IR] [HOMA> 3.5]. There was an association between IR and NASH [p=0.011], IR and obesity [p=0.031], IR and MS [p=0.007], and MS and steatosis on medical ultrasound [USG] [p=0.012]

Conclusion: The results indicated that the most frequent risk factors were MS and its variables: increased waist circumference, dyslipidemia and HBP. This highlights the significance of metabolic control in non-alcoholic fatty liver disease and confirms its role as the hepatic component of metabolic syndrome

Immunophenotypic characteristics of T-acute lymphoblastic leukemia in Omani patients: a correlation with demographic factors

Objectives: To study and classify the immunophenotypic characteristics of Omani patients diagnosed with T-cell acute lymphoblastic leukemia [T-ALL] and to correlate the results with age and gender as well as biological factors [peripheral and bone marrow blast cells percentage]

Methods: Fifty cases from both genders and of all ages who fulfilled the inclusion criteria with a diagnosis of T-ALL were included in the study. Correlation of T-ALL subtypes with age, gender, and initial bone marrow and peripheral blood blast cells percentage was assessed using ANOVA

Results: Among the 50 T-ALL patients analyzed, 44 were male and six were female giving a male-to-female ratio of 7:1 [p = 0.007]. The average age of patients was 19.2 years with no significant differences in the three disease subtypes. No significant association was seen between the peripheral or bone marrow blast cell percentage and the differentiation stages of the neoplastic clone of T-ALL. All female patients were found to express an immature T-ALL phenotype

Conclusions: This study reports the subtypes of T-ALL in Oman for the first time. It is hoped that this will lead to a better understanding of the disease outcomes

Knowledge and attitudes towards clinical trial participation in Oman: a cross-sectional study

Objectives: Clinical trials are prospective studies on human subjects designed to answer various clinical questions. However, only a limited number of clinical trials have been conducted in Oman. This study aimed to assess the knowledge and attitudes of Omani patients and their relatives towards participating in clinical trials

Methods: This cross-sectional study was conducted between October 2015 and March 2016 among 174 patients and relatives attending the Haematology and Oncology Outpatient Clinics and Day Care Unit of the Sultan Qaboos University Hospital, Muscat, Oman. A self-administered questionnaire was designed to elicit participants' knowledge of and attitudes towards participation in clinical trials

Results: A total of 100 patients and relatives agreed to take part in the study [response rate: 57.5%]. The male-to-female ratio was 1:1.2. Only 31.3% of the studied population knew what clinical trials were and only 6.5% had themselves previously participated in a clinical trial. The majority agreed or strongly agreed that they would participate in clinical trials related to their own condition [59.2%]. Overall, 89.7% expected to be informed about potential clinical trials by their treating physicians

Conclusion: Omanis had a low level of knowledge of clinical trials and a very low rate of previous participation in such trials, despite a moderate level of interest. Patients should therefore be educated and informed of ongoing clinical trials in order to improve participation rates for clinical trials conducted in Oman

Impact of aberrant myeloid antigen expression on outcomes of patients with t-cell acute lymphoblastic leukemia

Objectives: To evaluate the impact of myeloid antigen expression on complete remission [CR], event-free survival [EFS], and overall survival [OS] in patients with T-cell acute lymphoblastic leukemia [T-ALL] treated with intensive chemotherapy

Methods: We retrospectively reviewed consecutive patients diagnosed with T-ALL and treated in Sultan Qaboos University Hospital and Royal Hospital in Oman between 2004 and 2010. The diagnosis of T-ALL was established using French-American-British classification or World Health Organization criteria. Patients were considered having myeloid antigen expression if they expressed CD13, CD33, or both [My+ and My-]

Results: Of the 39 patients, 38 were included in the study [25 patients with My- and median age of 18.4 years, 13 patients with My+ and median age of 22.0 years]. Median follow-up was 12 months. Thirty-two out of the total cohort were eligible for response-rate assessment. Twenty-nine patients [90.6%] achieved CR with one or two courses of chemotherapy with similar CR rates between the two groups [p = 0.880]. Twenty-five percent [5/20] of the patients with My- required two courses of induction, whereas 58.3% [7/12] of My+ required two courses of induction and the difference was statistically significant [p = 0.040]. In the multivariable analysis; age, gender, initial white blood cell count, central nervous system disease, and myeloid antigen expression were not statistically significant predictors of CR. The EFS and OS were similar between the My+ and My- groups p = 0.180 and p = 0.440, respectively

Conclusions: Patients with T-ALL with myeloid antigen expression need more courses of induction; however, rates of CR, EFS, and OS are not different from those without myeloid antigen expression. Larger prospective studies are required to confirm these findings

Therapeutic drug monitoring-guided dosing of busulfan differs from weight-based dosing in hematopoietic stem cell transplant patients

Busulfan [Bu]-based preparative regimens in hematopoietic stem cell transplantation are commonly used. Previous studies have shown that Bu at a fixed dose of 3.2 mg/kg/day [FBD] given intravenously decreases variability in drug pharmacokinetics and this decreases the dependency on therapeutic drug monitoring [TDM] of Bu. We compared the Bu dose given using TDM with the FBD of 3.2 mg/kg/day. Seventy-three patients with acute leukemia, myelodysplasia, chronic myeloid leukemia, thalassemia major, and sickle cell disease were included. The mean age at transplant was 15 years [range 2-55 years] with 57% adults. Indication for transplantation was leukemia/myelodysplastic syndrome in 46% of the patients, while the remaining 54% were transplanted for inherited blood disorders. We found that the median FBD was lower than the median TDM dose by 39 mg/day with a statistically significant difference [p < 0.001] even after adjusting for the weight [median total FBD of 349 mg, median TDM dose of 494 mg, p < 0.0001]. Age and underlying condition [malignant vs. nonmalignant] were the main factors affecting Bu clearance [p < 0.001 and p < 0.07, respectively]. TDM remains an important tool for the appropriate dosing of Bu in preparative regimens of hematopoietic stem cell transplantation, especially in populations with genetic admixture

First cytogenetic profile of Omani patients with de novo myelodysplastic syndromes - Comparison with data from Asia, Africa, Europe and North and South America

Clonal cytogenetic abnormalities have been reported among 30-80% of patients with myelodysplastic syndromes [MDS]; however, 20-70% of patients with MDS show a normal karyotype that may nevertheless harbour a cryptic genetic alteration. Earlier reports have suggested that the distribution of specific chromosomal aberrations varies among Western and Asian countries, with geographical and ethnic differences in the frequency of specific chromosomal aberrations. This article compared the cytogenetic data of 36 adult Omani patients with MDS to previously reported data from other populations. Differences were noted between the percentages of clonal aberrations and the median age of Omani subjects at presentation in comparison to individuals of different ethnicities and from various geographical locations. To the best of the authors' knowledge, this is the first report to describe the cytogenetic data of patients with MDS from Oman

Breastfeeding practice in Arar, Northern Saudi Arabia

Background: Human milk meets all the nutritional requirements of infants for the first 6 months of life, and it is associated with a lower incidence of diarrhea than partial or artificial feeding. The infant should be exclusively breastfed for at least 4 months of life and if possible for 6 months, as recommended by WHO and UNICEF

Aim of the Study: was to assess the breastfeeding practice for children under 24 months in Arar city, as well as its relation to some sociodemographic and nutrition related variables

Participants and methods: A cross-sectional study was carried out in Arar city, mothers were selected from the attendees of 6 randomly selected primary health care centers in the city. They were interviewed and filled in a questionnaire that included the key questions

Results: 11.8% of infants received exclusive breastfeeding, 39.7% received a mix of both breast and artificial feeding while 48.5% depended only on artificial feeding. More than 50% of the studied infants were males, 70% of the mothers weren't working. No significant relationship between type of child feeding and child age, sex, mother education, mother's working status, father's work or mother age group [P >0.05]. About 2 thirds of children who had mixed feeding suffered from repeated gastroenteritis, delayed teething, standing and walking

Conclusion: In accordance with the obvious deficiency of exclusive breastfeeding in Arar city which is located in Northern Saudi Arabia, we highly recommend that programs promoting exclusive breastfeeding in the first 6 months of age must be conducted to increase mothers' awareness of the exclusive breastfeeding duration is recommended

Hematopoietic stem cell transplantation in the Sultanate of Oman

The Sultanate of Oman is one of the Arabian Gulf countries with a total population of 4,414,051 as of mid 2016, of which 2,427,825 are Omanis. The gross national income per capita was 7327.7 RO [Omani rial; equivalent to US$19,033] in 2014. There are two hematopoietic stem cell transplantation [HSCT] centers in Oman: the Sultan Qaboos University Hospital [SQUH; allogeneic and autologous] and the Royal Hospital [RH; autologous]. HSCT activity in Oman started in 1995 at the SQUH center, which had only one bed, and four cases were performed in that year. The number of allogeneic HSCTs at the SQUH ranged between four and 29 cases per year, of which malignancy was the main indication for transplantation [47%]. Most of the transplants were performed from identical sibling donor. T-deplete haploidentical and recently T-replete haploidentical HSCT were also performed at the SQUH center. In the allogeneic HSCT cohort transplanted at the SQUH, the risk of acute graft-versus-host disease [Grades II-IV] was 18%, whereas the risk of extensive chronic graft-versus-host disease was 8%. The HSCT unit at the RH, which started in 2014, performs autologous HSCT procedures only. The number of autologous HSCT cases at the RH ranged between three and 16 cases per year. Limited bed availability is a frequent obstacle to HSCT in Oman. Construction of a much larger national HSCT center is about to be completed, which will likely improve access to transplant services in Oman

Unusual indolent course of a chronic active epstein-barr virus-associated natural killer cell lymphoproliferative disorder

Natural killer [NK] cell lymphoproliferative disorders are uncommon and the Epstein-Barr virus [EBV] plays an important aetiological role in their pathogenesis. We report a 20-year-old male with a chronic active EBV infection associated with a NK cell lymphoproliferative disorder which had an unusual indolent course. He presented to the Sultan Qaboos University Hospital in Muscat, Oman, in December 2011 with a history of intermittent fever and coughing. Examinations revealed generalised lymphadenopathy, hepatosplenomegaly, leukocytosis, transaminitis, diffuse bilateral lung infiltrates and bone marrow lymphocyte involvement. A polymerase chain reaction [PCR] test revealed a high EBV viral load in the peripheral blood cells. The patient received a course of piperacillin-tazobactam for Klebsiella pneumoniae, but no active treatment for the lymphoproliferative disorder. However, his lymphocyte count, serum lactate dehydrogenase and liver enzymes dropped spontaneously. In addition, EBV PCR copies fluctuated and then decreased significantly. He remained clinically asymptomatic over the following four years

First implementation of transfusion consent policy in Oman

Objectives: transfusions are a common medical intervention. Discussion of the benefits, risks and alternatives with the patient is mandated by many legislations prior to planned transfusions. At the Sultan Qaboos University Hospital [SQUH], Muscat, Oman, a written transfusion consent policy was introduced in March 2014. This was the first time such a policy was implemented in Oman. This study therefore aimed to assess adherence to this policy among different specialties within SQUH

Methods: the medical records of patients who underwent elective transfusions between June and August 2014 were reviewed to assess the presence of transfusion consent forms. If present, the consent forms were examined for completeness of patient, physician and witness information

Results: in total, the records of 446 transfused patients [299 adult and 147 paediatric patients] were assessed. Haematology patients accounted for 50% of adult patients and 71% of paediatric patients. Consent was obtained for 75% of adult and 91% of paediatric patients. The highest adherence rate was observed among adult and paediatric haematology specialists [95% and 97%, respectively]. Consent forms were correctly filled out with all details provided for 51% and 52% of adult and paediatric patients, respectively. Among inadequately completed forms, the most common error was a lack of witness details [20-25%]

Conclusion: in most cases, the pre-transfusion consent policy was successfully adhered to at SQUH. However, further work is required to ensure full compliance with the consent procedure within different specialties. Implementation of transfusion consent in other hospitals in the country is recommended

Reproducibility of manual platelet estimation following automated low platelet counts

Objectives: Manual platelet estimation is one of the methods used when automated platelet estimates are very low. However, the reproducibility of manual platelet estimation has not been adequately studied. We sought to assess the reproducibility of manual platelet estimation following automated low platelet counts and to evaluate the impact of the level of experience of the person counting on the reproducibility of manual platelet estimates

Methods: In this cross-sectional study, peripheral blood films of patients with platelet counts less than 100 × 109 /L were retrieved and given to four raters to perform manual platelet estimation independently using a predefined method [average of platelet counts in 10 fields using 100× objective multiplied by 20]. Data were analyzed using intraclass correlation coefficient [ICC] as a method of reproducibility assessment

Results: The ICC across the four raters was 0.840, indicating excellent agreement. The median difference of the two most experienced raters was 0 [range: -64 to 78]. The level of platelet estimate by the least-experienced rater predicted the disagreement [p = 0.037]. When assessing the difference between pairs of raters, there was no significant difference in the ICC [p = 0.420]

Conclusions: The agreement between different raters using manual platelet estimation was excellent. Further confirmation is necessary, with a prospective study using a gold standard method of platelet counts

role of autoantibody and antioxidant enzymes in patients with type 1 diabetes

To determine autoantibodies and antioxidant enzymes as well as the correlation between them. This study included 80 individuals, 40 patients with type 1 diabetes and 40 healthy individuals without diabetes [as a control group]. The study was carried out during the period from December 2010 to the end of December 2012 at Al-Tahreer General Hospital, Al-Basra Maternity and Pediatric Hospital, and Al-Sader Teaching Hospital. Laboratory investigations were performed to estimate glutamic acid decarboxylase antibody [GADA] and islet cell antigen-2 antibody [IA-2A] by enzyme-linked immunosorbent assay [ELISA], antioxidant enzymes [glutathione peroxidase [GPX] and superoxide dismutase [SOD]], and glycosylated hemoglobin [HbA1c] [as a marker of glycemic control] for these patient and control groups. The high prevalence of GADA and IA-2A had been demonstrated among patients with type 1 diabetes, which was significantly higher [P < 0.001] [72.5%] in comparison to 0% in the control group. These results are suggestive of the autoimmune characteristic of type 1 diabetes. The age of onset of type 1 diabetes is found to affect the frequency of these autoantibodies. The frequency was significantly higher in patients who developed the disease in early childhood [91.7% for GADA and 58.3% for IA-2A] in comparison with those who developed the disease later on [40% for GADA and 20% for IA-2A]; this probably occurred due to genetic and non-genetic factors. Although the statistical analysis of the correlation between gender and autoantibodies showed no significant difference, female patients with type 1 diabetes were found to be more affected than male patients. The frequency of these autoantibodies was found to decrease as the duration of type 1 diabetes increased. The prevalence of GADA and IA-2A in patients with duration of disease less than 5 years was 78.3% and 43.5%, respectively, and began to decrease to 0% for GADA and IA-2A in those with disease duration more than 12 years. These results are attributed to the depletion of islet cell autoantibodies with time. Additionally, HbA1c levels were significantly higher in islet cell autoantibodies-positive patients than in islet cell autoantibodies-negative patients [P < 0.001]. The difficulty in achieving glycemic control despite oral hypoglycemic drug and insulin therapy is attributed to the fact that the pathogenesis of disease in developing type 1 diabetes and latent autoimmune diabetes [LADA] in adults is due to beta-cell destruction rather than insulin resistance as in classical type 2 diabetes. The mean activity of both antioxidant enzymes [SOD and GPX] in red blood cells [RBCs] was significantly lower than the control [P < 0.001]. Also the lower mean activity of both antioxidant enzymes [SOD and GPX] in RBCs showed a higher significant value in patients who had uncontrolled diabetes [HbA1c level > 8%] [P < 0.001]. Patients with LADA who were tested positive for GAD and IA-2A showed a significant decrease in the mean activity of SOD and GPX in comparison to patients with type 2 diabetes who were tested negative to autoantibodies; most of the patients with LADA also had a higher HbA1c level > 8% [P< 0.001]. There is a strong evidence of the role of autoimmunity in the pathogenesis of type 1 diabetes. The oxidative stress SOD and GPX are depleted as well. The correlation reflects the more oxidative stress with poor diabetic patients may progress the complications

Rituximab leads to long remissions in patients with chronic immune thrombocytopenia

To assess the response rate and duration of response in patients with chronic immune thrombocytopenia [ITP] receiving rituximab. We retrospectively analyzed 32 consecutive patients with chronic ITP who were treated in two tertiary centers in Oman. Response assessment was based on the American Society of Hematology criteria. Nineteen patients [59%] had an initial response. However, six of the 19 patients lost their response leaving 13 patients with long-lasting remissions. The median age at diagnosis was 25 years [range 14-58]. The median time from diagnosis to rituximab therapy was 21 months. The median follow-up after starting rituximab was 26 months. The overall cumulative response rate was 59% [complete response 44%, partial response 15%] and the median time to respond was 30 days with a response rate of 44% at four weeks. In all responders, the cumulative rate of loss of response was 32% with a median time to lose response of 54 months. The use of rituximab in ITP achieves high response rate and long remission duration. Our study was limited by the small sample size and further larger prospective studies are recommended

Study of antibacterial activity in the local Iraqi propolis

Objective: This study includes the investigation of antibacterial activity of the local propolis against four types of bacteria isolated from patients

Methods: Bacteria were tested including Psedomonas sp, Streptococcus sp, Escherichia coli and Staphylococcus aureus. Six concentrations [0, 5, 10, 15, 20 and 25] mg/ml of propolis extracts were tested against bacteria

Results: Results revealed the presence of significant difference [P < 0.05] in the effect of propolis extract against the four types of bacteria in this study. Psedomonas sp. was the most sensitive among the others toward the propolis extract followed by Streptococcus sp., E. coli and Staphylococcus aureus at a rate of inhibition zones [14.09, 10.39, 8.78 and 8.39] mm, respectively. Results of this study also showed increasing rate of inhibition zone if the concentration of propolis extract was increased

Conclusion: This study provided that local propolis has antibacterial activity against gram positive and gram negative bacteria

[Echocardiographic evidence of early diastolic dysfunction in Asymptomatic children with Osteogenesis Imperfecta]

Objectives: Structural and functional cardiovascular abnormalities have been reported in adults with osteogenesis imperfecta [OI]; however, there is a lack of paediatric literature on this topic. This study aimed to investigate cardiovascular abnormalities in children with OI in comparison to a control group

Methods: This case-control study was conducted at the Sultan Qaboos University Hospital in Muscat, Oman, between May 2013 and August 2014. Data from eight patients with OI and 24 healthy controls were compared using conventional and tissue Doppler echocardiography [TDE]

Results: The OI group had significantly lower peak early mitral valve flow velocity [P = 0.027], peak a-wave reversal in the pulmonary vein [P = 0.030] and peak early diastolic velocity of the mitral valve and upper septum [P = 0.001 each]. The peak late diastolic velocities of the mitral valve [P = 0.002] and the upper septum [P = 0.037] were significantly higher in the OI group; however, the peak early/late diastolic velocity ratios of the mitral valve [P = 0.002] and upper septum [P = 0.001] were significantly lower. Left ventricular dimensions and aortic and pulmonary artery diameters were larger in the OI group when indexed for body surface area. Both groups had normal systolic cardiac function

Conclusion: Children with OI had normal systolic cardiac function. However, changes in myocardial tissue Doppler velocities were suggestive of early diastolic cardiac dysfunction. They also had increased left ventricular dimensions and greater vessel diameters. These findings indicate the need for early and detailed structural and functional echocardiographic assessment and follow-up of young patients with OI