Demographic and Clinical Findings in Pediatric Patients Affected by Organic Acidemia

How to Cite This Article: Najafi R, Hashemipour M, Mostofizadeh N, Ghazavi MR, Nasiri J, Shahsanai A, Famori F, Najafi F, Moafi M. Demographic and Clinical Findings in Pediatric Patients Affected by Organic Acidemia. Iran J Child Neurol. Spring 2016; 10(2): 74-81.AbstractObjectiveMetabolic disorders, which involve many different organs, can be ascribed to enzyme deficiency or dysfunction and manifest with a wide range of clinical symptoms. This study evaluated some of the demographic and clinical findings in pediatric patients affected by organic acidemia.Materials & MethodsThis cross-sectional study was part of a larger study conducted in patients with metabolic disorders during a period of 7 years from 2007 to 2014 in Isfahan Province, Iran. Our study covered a wide range of cases from newborn infants (one-week old) to adolescents (children up to the age of 17 years). This study evaluated patients’ demographic information, history of disease, developmental and educational status, clinical and general conditions. Phone and in-person interviews were used to gather information.ResultsOut of 5100 patients screened in this study, 392 patients were affected by one of the different metabolic disorders and 167 individuals were diagnosed as organic acidemia. Propionic acidemia/methyl malonic acidemia (PA/MMA) was the most prevalent form of this metabolic disorder. The frequency of consanguinity was 84.7% in the group of patients. The mortality rate was 18.8% in patients with organic academia.ConclusionEach of the metabolic diseases, as a separate entity, is rare; nevertheless, in aggregate they have a somewhat high overall prevalence. These diseases result in mental and developmental disorders in the absence of quick diagnosis and initiation of treatment. Furthermore, more mutations should be identified in societies affected by consanguinity. 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Design, implementation and evaluation a tool to strengthen physician- patient communication skills of medical students

Introduction: How to communicate and interact with the patient play an effective role in patient satisfaction, treatment outcome, psychological effects, medical costs, clinical competence of physician, and even patient’s complaining. these skills can be learned. Studies showed that physician did not receive enough training regarding general communication skills. this study aimed to provide a tool to strengthen communication skills of medical students in an effective and simple manner. Methods: This quasi-experimental intervention study was performed on medical students of Isfahan University of Medical Sciences in Internship period in 1388. Participants were randomized to the intervention and control groups (75 student in each group). intervention group received the tool were developed for guiding physicians to communicate with patient. After two months, physician- patient communication in two groups was assessed and compared. Finally, data analysis was performed with spss software. Results: The overall mean was 29/36±7/2. mean score in both intervention and control groups were 32/5±7 and 26/19±5/91 respectively. the mean difference between two groups was 6/35. Difference between intervention and control groups was significant (p<0/001). Conclusions: The results showed the use of educational tools are effective on communication with patients. Education about effective communication with patients should be considered in educational material for medical students

Prevention of communicable diseases after disaster: A review

Natural disasters are tragic incidents originating from atmospheric, geologic and hydrologic changes. In recent decades, millions of people have been killed by natural disasters, resulting in economic damages

Morphine mouthwash for the management of oral mucositis in patients with head and neck cancer

Background: Oral mucositis is a debilitating side effect of cancer treatment for which there is not much successful treatments at yet. We evaluated the effectiveness of topical morphine compared with a routine mouthwash in managing cancer treatment-induced mucositis. Materials and Methods: Thirty head and neck cancer patients with severe mucositis (World Health Organization Grade III or IV) were randomized into the morphine and magic mouthwash groups. Patients received morphine sulfate 2% or magic solution (contained magnesium aluminum hydroxide, viscous lidocaine, and diphenhydramine), 10 ml for every 3 h, six times a day, for 6 days. Both groups received same dietary and oral hygiene instructions and care. Mucositis was graded at baseline and every 3 days after treatment. Patients′ satisfaction and drug effect maintenance were also evaluated. Results: Twenty-eight patients (mean age of 49.5 ± 13.2 years, 63.3% female) completed the trial; 15 in the morphine group and 13 in the magic group. There was a decrease in mucositis severity in both of the morphine (P < 0.001) and magic (P = 0.049) groups. However, at the 6 th day, more reduction was observed in mucositis severity in the morphine compared with magic group (P = 0.045). Drug effect maintenance was similar between the two groups, but patients in the morphine group were more satisfied by their treatments than those in the magic group (P = 0.008). Conclusions: Topical morphine is more effective and more satisfactory to patients than the magic mouthwash in reducing severity of cancer treatment-induced oral mucositis. More studies with larger sample size and longer follow-up are required in this regard

Assessment of the Relationship between Nutritional Knowledge and Anthropometric Indices in Isfahan Children and Adolescent

Background: Anthropometric indices are one of the ways to assess physical growth and nutritional status of children and adolescents considering the importance of nutrition in children and the role of healthy nutrition in preventing diabetes, obesity and other chronic diseases, the objective of this study is determine the relationship between nutritional knowledge and anthropometric indices in children and adolescents in Isfahan. Materials and Methods: This survey conducted using multistage cluster sampling on 1776 students aged 15–7 years old in Esfahan province in 2015. Nutritional knowledge was measured by questionnaire and height, weight, and body mass index (BMI) were measured as anthropometric index. Pearson and Spearman coefficients, regression, and T Independent were used to analyze the data; regression analysis was used to adjust the variables. Results: The mean age of students were 12.6 ± 1.9 years. About 50.3% of students were male, and most of them were studying in 5th grade of elementary school (23.8%). Most of them had normal status in terms of BMI (60.6%) and height (77.8%). There was no significant relationship between the level of BMI and height of children and adolescents, and the total score of nutritional knowledge and its domains (P > 0.05), direct and significant relationship was found between nutritional knowledge in two domains of meals and food habits and age. Conclusion: As no relationship between nutritional knowledge and anthropometric index in our study, other factors are effective in this regard, and studies are required to be conducted to examine the nutritional attitude and performance, in addition to knowledge

Is Vitamin D Supplementation Effective in Prevention of Recurrent Urinary Tract Infections in the Pediatrics? A Randomized Triple-Masked Controlled Trial

Background: This study aimed to evaluate the impact of Vitamin D supplementation on prevention of recurrent urinary tract infections (UTIs) in the pediatrics. Materials and Methods: This randomized, triple-blind, placebo-controlled clinical trial was conducted in 2014 among 68 children and adolescents with recurrent UTI. They were randomly assigned to two groups, receiving either Vitamin D (1000 IU/daily) or placebo for 6 months. The serum concentration of Vitamin D before and after the study and the frequency of UTI during the study were recorded. Results: Overall 33 patients in the group of receiving Vitamin D and 32 in the placebo group completed the trial. The mean serum level of Vitamin D had a significant increase in the intervention group (15.80 ± 8.7 vs. 20.56 ± 8.30 ng/mL, P 0.05). Conclusion: The findings of this trial revealed that Vitamin D supplementation with the mentioned dose have not significant impact on preventing recurrent UTI. Future studies with higher doses of Vitamin D and longer follow-up are suggested

The impact of poor glycemic control on lipid profile variables in children with type 1 diabetes mellitus

BACKGROUND: Type 1 diabetes mellitus (T1DM) and its related comorbidities are considered an important health issue. This study aimed to evaluate the impact of glycemic control on lipid profile variables in children with T1DM. MATERIALS AND METHODS: This study included 274 children (≤19 years of age) with T1DM who had referred to the outpatient clinics of endocrinology in Emam-Hossein Hospital of Isfahan, Iran. Based on American Diabetes Association criteria, patients were divided into two groups including optimal glycemic control (OGC) and poor glycemic control (PGC). Mean lipid level and frequencies of lipid profile abnormalities between the two studied groups were compared. RESULTS: Mean age of the studied population was 13 ± 5.9 years and 133 (48.5%) were boys. A total of 162 (59.1%) and 112 (40.9%) patients had PGC and OGC, respectively. Hypercholesterolemia was the most common dyslipidemia in both groups (33 [29.1%] of OGC and 63 [39.1%] of PGC patients). The frequency of high low-density lipoprotein (LDL) was significantly higher in patients with PGC than those with OCG (P = 0.007). The frequencies of hypercholesterolemia, hypertriglyceridemia, and low levels of high-density lipoprotein were also higher in PGC group, but did not reach the significant threshold. CONCLUSION: It is suggested that glycemic control is in association with lipid profile abnormality in patients with T1DM. High LDL was significantly more frequent in patients with PGC than those with OGC. It is recommended to investigate the role of glycemic control on other cardiometabolic risk factors of T1DM patients. Our findings could be used for planning preventative strategies for reducing T1DM-related cardiovascular disease

Comparison of Interleukin-33 serum levels in asthmatic patients with a control group and relation with the severity of the disease

Background: The relation between interleukin-33 (IL-33) and asthma is not precisely known yet. The present study set to compare the serum level of IL-33 in patients with asthma and controls and study the relation with the severity of disease. Methods: The serum level of IL-33 and total IgE in 89 asthmatic patients and 57 controls were analyzed. The association of levels of IL-33 with the severity of disease, levels of total IgE, measures of spirometry (forced expiratory volume in 1 s [FEV1]), age, sex, presence or absence of other allergic diseases, and the disease duration was evaluated. Results: Higher levels of IL-33 and total IgE were detected in asthmatic patients compared with controls (P = 0.0001 and P = 0.008, respectively). In the asthmatic group, a significant direct association of IL-33 with age (P = 0.02, R = 0.23) and with total IgE level (P = 0.003, R = 0.31) were observed, but there was no relationship between other variables. Comparison of mean level of IL-33 in different asthma groups concerning the disease severity showed the statistically significant difference between them and a significant increased serum level of total IgE was observed in more severe disease. The results showed a significant negative correlation between FEV1 and total IgE (P = 0.028, R = −0.23) and IL-33 level (P = 0.0001, R = −0.83). Conclusions: IL-33 is suggested as a new inflammatory marker of severe and refractory asthma. Therefore, it may be a unique therapeutic target in these patients

The Prevalence of Asthma in Children with Type 1 Diabetes Mellitus and Relationship between Control of Diabetes and Severity of Asthma

Background: Type 1 diabetes mellitus (T1DM) is by far the most common metabolic disease in children. Asthma is the most common chronic disease in pediatric population, and its prevalence has increased in the last decades. In this study, the prevalence of asthma among these children with T1DM has been described and its association with the demographic and clinical characteristics of Iranian children has been evaluated. Materials and Methods: A cross sectional study was carried out on children with T1DM who referred to pediatric endocrinology clinics of Isfahan University of Medical Sciences. The participants were classified as a group with asthma and the second group as diabetic children without asthma. After selection and evaluation of the diabetic patients with asthma, their glycemic status was evaluated three times in the past year. All data were analyzed using the SPSS version 23.0 statistical software package. Results: A total of 419 patients (49.4% male and 50.6% female) diagnosed with T1DM were included in the study. The mean age of patients at the time of recruitment was 12.65 ± 3.9 years with a range from 3.8 to 18 years. The mean of disease duration was 5.3 ± 2.7 years. Among all participants, asthma was detected in 24 children with T1DM (5.7%). Glycemic control was significantly poorer among asthmatic patients with diabetes compared with diabetic patients without asthma. Conclusion: The study demonstrated a lower prevalence of asthma among T1DM children rather healthy ones. Hence, diabetic child patients with asthma experience poorer glycemic control in comparison with T1DM patients without asthma

Meal Frequency in Iranian Children and Adolescents at National and Sub-National Levels: The CASPIAN-IV Study

Background: This study aims to assess the pattern of meal frequency consumption of Iranian children and adolescents at national and subnational levels according to the socioeconomic status (SES) of the living region. Methods:  This multi-centric study was conducted in 2011-2012 among 14,880 students, aged 6-18 years, who were selected by multistage random cluster sampling from 30 provinces. Meal frequency was assessed by the World Health Organization Global School-based student Health Survey (WHO- GSHS) questionnaire. Data were compared at national and sub-national levels according to the SES of the living region. Results: Overall, 13486 students completed this study (participation rate: 90.6%). The frequency of breakfast, lunch, and dinner skipping was 32.08% (95% CI: 30.99, 33.2), 8.58% (95% CI: 7.71, 9.54), and 10.90% (95% CI: 10.21, 11.62), respectively. The frequency of consuming 1 meal, 2 meals, and 3 meals a day was 9.60% (95%CI: 8.94, 10.31), 29.28% (95% CI: 28.31, 30.26), and 61.12 % (95% CI: 59.89, 62.34), respectively. The mean number of days per week of breakfast consumption was 5.14 (95% CI: 5.08, 5.20), the corresponding figure was 6.39 (95%CI: 6.35, 6.42), and 6.24 (95%CI: 6.21, 6.28) for lunch and dinner, respectively. These frequencies had no significant difference according to the SES of the living region. Conclusion: Most Iranian children and adolescents consumed all three main meals, however irrespective of SES; in general skipping breakfast was more frequent than skipping lunch and dinner. Regular meal intake, notably breakfast, should be considered in health promoting programs for all children and adolescents.   Keywords: Meal frequency, Skipping meals, Children and adolescents, Prevention, Health promotion, Ira