Correlation of Cardiac and Liver Iron Level with T2*MRI and Vitamin D3 Serum Level in Patients with Thalassemia Major.

INTRODUCTION: Thalassemia is a hypochromic microcytic anemia, which is characterized by congenital disorders. In thalassemia patients, bone diseases are one of the causes of mortality. Our goal was to investigate the association between vitamin D deficiency and increased iron uptake by cardiac myocytes and hepatocytes. MATERIALS AND METHODS: Forty patients with thalassemia major were studied in Amir Kabir Hospital, Arak, Iran. The information obtained through clinical examination. Serum ferritin level was determined by ELISA and T2*MRI performed for measuring iron content in the heart and the liver. RESULTS: The average age of the patients was 23.8 ± 10.7 years. The mean T2*MRI values were 23.7 ± 7. The vitamin D3 level in 33 patients (82.5% cases) was less than 20 ng/dl, 2 patients (5%) in the range of 20-30 ng/dl, and the others had above 30 ng/dl. Correlation between vitamin D and age was 0.611. Correlation coefficient between heart and liver T2*MRI with ferritin level in patients was 0.437 and 0.335, respectively. CONCLUSION: Due to significant associations, the periodic measurement of vitamin D, as well as PTH, is recommended for patients with thalassemia major.

Pantoprazole reduces serum ferritin in patients with thalassemia major and intermedia: A randomized, controlled study.

AIM: Complications due to iron overload exert a problematic situation in patients with thalassemia. Proton pump inhibitors (PPIs) like pantoprazole are effective agents to reduce acid gastric acid secretion and perhaps to interrupt iron absorption in conditions with increased iron absorption. Our purpose was to study effects of pantoprazole addition to chelators on iron levels in patients with thalassemia major and intermedia. METHODS: This randomized, controlled, and single center trial was performed on 60 patients with thalassemia major and intermedia in Amir Kabir hospital, Iran. Patients were randomized 1:1 to pantoprazole group (iron chelator plus pantoprazole) or control group (iron chelator) for 6 months. Serum ferritin was measured by ELISA. Iron content was measured by magnetic resonance imaging; heart T2*, and liver T2*. RESULTS: After 6 months of treatment, a significant reduction was seen in serum ferritin levels in the pantoprazole group (1444±613µg/mL to 1197±956µg/mL; P<0.001). A further reduction was seen in patients with thalassmeia intermedia. There were no significant changes in myocardial T2* values in pantoprazole group compared to control group (23.6±7.3ms to 24.1±6.4ms). Compared to the control group, pantoprazole therapy had no effect on hepatic T2* value (9.7±2.3ms to 9.8±2.6ms). However, between-group difference was significant (P<0.05). CONCLUSION: Pantoprazole therapy for 6 months has benefits for reducing serum ferritin in patients with thalassemia major and intermedia. Pantoprazole addition to iron chelators seems safe.

Vitamin A supplementation is effective for improving the clinical symptoms of urinary tract infections and reducing renal scarring in girls with acute pyelonephritis: a randomized, double-blind placebo-controlled, clinical trial study.

INTRODUCTION: It is believed that tubulointerstitial inflammation plays a role in the formation of renal scarring secondary to acute pyelonephritis (APN). Vitamin A is an anti-inflammatory agent that is involved in the re-epithelialization of damaged mucosal surfaces. OBJECTIVE: The aim of this study was to evaluate the efficacy of vitamin A supplementation in combination with antibiotics for improving urinary tract infections (UTIs) symptoms and preventing renal scarring in girls with APN. STUDY DESIGN: This randomized, double-blind, placebo-controlled clinical trial was conducted on 90 girls aged 2 to 12 years old between 2015 and 2017. Patients with UTIs and first episode of APN diagnosed based on 99 mTc-DMSA scintigraphy (uptake defect) were assessed for eligibility. Patients were randomly divided into two groups that either received 10 days of oral vitamin A (intervention group) or 10 days of placebo (control group) in addition to antibiotics during the acute phase of infection. The clinical response was considered as the primary outcome [duration (positive days) of UTI symptoms during trial treatment period] and secondary outcomes (no change, improving and or worsening of 99 mTc-DMSA scan results 6 months after treatment from baseline). P < 0.05 was considered to be statistically significant. RESULTS: Seventy-four patients (vitamin A group: 36 patients, placebo: 38 patients) were included in the analysis. The mean age was 5.25 ± 1 year old. Three patients (7.89%) in the placebo group and 2 patients (5.55%) in the vitamin A group had vesicoureteral reflux (VUR) (p = 0.114). Duration of fever (vitamin A group: 1.8 days, placebo: 3.1 days, p = 0.0026), urinary frequency (1.3 days vs. 2.8 days, p = 0.003) and poor feeding (2.3 days vs. 4.2 days, p = 0.005) were significantly lower in the vitamin A group. Following the second 99 mTc-DMSA scan, worsening of lesions was observed among 8 (22.2%) and 17 (44.7%) patients in the vitamin A and placebo groups, respectively (p = 0.003). 63.8% (23 patients) of the vitamin A group and 21% (8 patients) of placebo group showed lesion improving in the photopenic region. (P < 0.0001) There was no evidence of vitamin A intolerance. DISCUSSION: Our results show the efficacy of vitamin A supplementation on reducing renal scarring secondary to APN and on fever, urinary frequency and poor feeding duration in girls with APN. CONCLUSION: Vitamin A supplementation is effective for improving the clinical symptoms of UTI and reducing renal injury and scarring following APN in girls with first APN. However, larger randomized clinical trials (RCTs) with longer follow up are needed to confirm these effects.

A randomized, controlled study evaluating effects of amlodipine addition to chelators to reduce iron loading in patients with thalassemia major.

AIM: Cardiomyopathy due to iron overload can be fatal in patients with thalassemia major. Calcium channel blockers seem to be effective to reduce iron loading. Our goal was to study effects of amlodipine addition to chelators on iron loading in patients with thalassemia major. METHODS: This randomized, controlled, and single-center trial was performed on 56 patients with thalassemia major. Patients were randomized 1:1 to combined group (iron chelator plus amlodipine) or control group (iron chelator) for 1 year. Iron content was measured by magnetic resonance imaging; heart T2*, and liver T2*. Serum ferritin was also measured. RESULTS: After 12 months of treatment, myocardial T2* values had significant improvement in combined group (21.9 ± 8.0 ms to 24.5 ± 7.6 ms; P < .05); Difference between two groups was significant (P = .02). Combined treatment had no effect on hepatic T2* value (9.6 ± 2.8 ms to 9.5 ± 3.6 ms); difference between two groups was not significant (P = .2). In addition, a significant reduction was seen in serum ferritin levels in two groups. Mild gastrointestinal upset was the most common untoward effect. CONCLUSION: Addition of amlodipine to iron chelators has beneficial effects for reduction of iron loading in patients with thalassemia major. This combination therapy seems safe.

Incidence of obsessive-compulsive disorder in children with nonmonosymptomatic primary nocturnal enuresis.

INTRODUCTION: Enuresis is defined as the repeated voiding of urine into clothes or bed at least twice a week for at least 3 consecutive months in a child who is at least 5 years of age. The behavior is not due exclusively to the direct physiologic effect of a substance or a general medical condition. Diurnal enuresis defines wetting, whereas awake and nocturnal enuresis refers to voiding during sleep. Primary enuresis occurs in children who have never been consistently dry through the night, whereas secondary enuresis refers the resumption of wetting after at least 6 months of dryness. Monosymptomatic enuresis has no associated daytime symptoms, and nonmonosymptomatic enuresis, which is more common, often has at least one subtle daytime symptom. Monosymptomatic enuresis is rarely associated with significant organic underlying abnormalities. Obsessive-compulsive disorder (OCD) is a chronic disabling illness characterized by repetitive, ritualistic behaviors over which the patients have little or no control. The purpose of this study was to investigate the relationship between OCD and nonmonosymptomatic nocturnal enuresis (NMNE). MATERIALS AND METHODS: In this case-control study, we evaluated 186 children aged 6-17 years old who were visited in the pediatric clinics of Amir Kabir Hospital, Arak, Iran. The control group included 93 healthy children, and the case group included 93 age- and sex-matched children with stage 1-3 chronic kidney disease (CKD). Then, the children's behavioral status was evaluated using the Children's Yale-Brown Obsessive-Compulsive Scale. RESULTS: The difference in compulsion was significant (P = 0.021), whereas the difference in obsession was significant between the two groups (P = 0.013). The most common symptom in CKD children with compulsion was silent repetition of words. CONCLUSION: Compulsive and obsessive are more common in NMNE versus healthy children. The observed correlation between compulsive-obsessive and NMNE makes psychological counseling mandatory in children with NMNE.

Zinc Supplementation in Treatment of Children With Urinary Tract Infection.

INTRODUCTION: Urinary tract infection (UTI) is very common in children. Precocious diagnosis and appropriate treatment are important because of the permanent disease complications. Zinc increases the response to treatment in many infections. In this study, we explored the effect of zinc in treating UTI. MATERIALS AND METHODS: Two hundred children with UTI were divided into 2 groups of 100 who were comparable in terms of age, sex, urine laboratory profiles, and clinical signs and symptoms. The control group received a standard treatment protocol for UTI and the intervention group received oral zinc sulfate syrup plus routine treatment of UTI. RESULTS: A faster recovery was observed in the patients receiving zinc, but abdominal pain was exacerbated by zinc and lasted longer. Three months after the treatment, there was no significant difference between the two groups in the time of fever stop and negative urine culture. CONCLUSIONS: In children with UTI, zinc supplementation has a positive effect in ameliorating severe dysuria and urinary frequency while the use of this medication is not recommended in the presence of abdominal pain.

Efficacy of nebulized L-epinephrine for treatment of croup: a randomized, double-blind study.

The objective of this study was to compare the effect of L-epinephrine plus dexamethasone vs. dexamethasone for treatment of croup in children. A randomized, double-blind clinical trial was implemented on 174 patients with croup, aged from 6 months to 6 years, and admitted to the Amir Kabir Pediatric Hospital (Arak, Iran). After randomized allocation, patients were administered dexamethasone, and then, they received either saline or L-epinephrine. Westley croup scores, heart rate, respiratory rate, and blood pressure were recorded every half an hour for a total of 120 min. There was a significant difference in mean of croup scores between two groups (P < 0.009). In addition, a significant difference was seen on mean of heart rate between two groups (P < 0.026). Our results showed a considerable difference in reduction of velocity of croup scores in patients who received nebulized L-epinephrine compared to patients who received placebo.