Demographic Characteristics besides Clinical and Laboratory Manifestations of Children with Visceral Leishmaniasis in Rasht, Northern Iran.

Background: Visceral leishmaniasis (Kala-azar) is an inherently dangerous and progressive disease that can be seen in many parts of the world, including Iran. Therefore, we decided to conduct this study on children with Kala-azar in Rasht-Iran. Methods: In this descriptive study, the hospital records of all children with Kala-azar disease admitted to 17 Shahrivar Hospital, Rasht-Iran from 2009 to 2021 were reviewed. Required information were collected from patients' records and descriptive statistical analysis was done using SPSS version 24 statistical software. Results: A total of 22 children with visceral leishmaniasis were admitted to 17 Shahrivar Hospital during the study period. The average age of the children was 2.93 ± 3.32 years. Most of them (59.1%) were boys and lived in the foothills (59.1%). The most common season for children to get the disease was spring (45.5%), and the average duration of the disease until hospitalization was 16.5 ± 13.54 days. In this study, the most common clinical manifestations included fever (90.9%), splenomegaly (77.3%), hepatomegaly (50%), weakness and restlessness (27.3%), and vomiting (18.2%). The most common laboratory findings were anemia (90.9%), leukopenia (59.1%), increased erythrocyte sedimentation rate (ESR) (75%), and increased C-reactive protein (CRP) (75%). 72.7% of the children were treated with liposomal amphotericin and others with glucantime, which were all successful. Conclusion: The results of our studies were consistent with most studies in Iran and other countries. These findings can help in the diagnosis and management of children with Kalaazar and better control of the disease in the province.

An analysis of the distribution and spectrum of alpha thalassemia mutations in Rasht City, North of Iran.

Background: Alpha thalassemia is one of the most common hereditary hemoglobin disorders worldwide, particularly in the Middle East, including Iran. Therefore, determining the spectrum and distribution of alpha thalassemia mutation is a fundamental component of preventive approaches and management strategies. Methods: The present study reviews the genetic testing and blood laboratory results of 455 candidates eligible for marriage who were suspected of being thalassemia carriers and on whom genetic testing was performed from 21 March 2013 to 31 December 2020 in Rasht City. Results: A total of 114 (25.05%) alpha thalassemia cases were identified. Fifteen different alpha mutations were found. The most common mutation among the study population was -α3.7 deletion in 55 patients (48.24%), followed by Hb Constant Spring (C.S) in 21 patients (18.42%) and poly A2 in 16 (14.03%). Also, most of the patients were silent carriers. The deletion type of mutation was much more common than non-deletion mutations. Conclusion: Our study reveals genetic heterogeneity and alpha thalassemia diversity among the Rasht City population. We expect that these findings will help guide premarital screening and genetic counseling, prenatal diagnosis of thalassemia, preventive strategy development, as well as a compilation of the alpha thalassemia catalog in Guilan province.

Normal saline, the known but least-examined fluid therapy method for preventing heme-induced nephropathy in children with glucose 6 phosphate dehydrogenase deficiency: a randomized controlled clinical trial.

BACKGROUND: Glucose 6 phosphate dehydrogenase deficiency (G6PDd) is the most common enzyme deficiency in humans. Randomized clinical trials comparing the efficacy of different types of fluid therapy for prevention of acute kidney injury (AKI) following hemolysis in patients with G6PDd are lacking. The present study aimed to compare the efficacy of three different types of fluid administration, isotonic saline with or without acetazolamide versus bicarbonate solution in prevention of AKI among children with acute hemolysis due to G6PDd. METHODS: In this double-blind randomized controlled clinical trial, 120 infants and children with acute hemolysis due to G6PDd were randomly divided into three groups consisting of 40 participants in each group. Group A received normal saline. Group B received normal saline plus oral acetazolamide at a dose of 5 mg/kg/day, and group C received half saline plus 75 mEq/L sodium bicarbonate. The primary outcome of this study was the frequency of AKI among the different types of fluid administration. RESULTS: In this study, 72 (60%) patients were boys with the mean age and length of hospital stay of 3.9 ± 2.2 years and 54.4 ± 29.9 h, respectively. AKI as the primary outcome of this study occurred only in one patient in group C and the rate of AKI did not differ significantly among patients receiving different types of fluid resuscitation (P > 0.05). CONCLUSION: Normal saline was equivalent to fluids containing alkalinizing agents in preventing heme-induced nephropathy in patients with G6PDd. A higher resolution version of the Graphical abstract is available as Supplementary information.

Paediatric pre-B acute lymphoblastic leukaemia-derived exosomes regulate immune function in human T cells.

Exosomes derived from solid tumour cells are involved in immune suppression, angiogenesis and metastasis; however, the role of leukaemia-derived exosomes has less been investigated. Hence, changes in immune response-related genes and human T cells apoptosis co-incubated with exosomes isolated from patients' pre-B cell acute lymphoblastic leukaemia were evaluated in this in vitro study. Vein blood sample was obtained from each newly diagnosed acute lymphoblastic leukaemia (ALL) patient prior any therapy. ALL serum exosomes were isolated by ultrafiltration and characterized using Western blotting and transmission electron microscopy. Exosomes were then co-incubated with T lymphocytes and the gene expressions, as well as functions of human T cells were quantified by qRT-PCR. Apoptosis and caspase-3 and caspase-9 protein expression were also evaluated by flowcytometry and Western blotting analysis, respectively. Exosomes isolated from ALL patients affected T lymphocytes and elevated the apoptosis. Moreover, these exosomes altered the T cells profile into regulatory type by increasing the expression of FOXP3 and Tregs-related cytokines, including TGF-B and IL-10. The expression level of Th17-related transcription factors (RoRγt) and interleukins (IL-17 and IL-23) decreased after this treatment. According to our findings, exosomes derived from ALL patients' sera carry immunosuppressive molecules, indicating the possible effect of exosomes as liquid biomarkers for cancer staging.

Evaluation of Self-efficacy in Children and Adolescents With Thalassemia Major.

BACKGROUND: The survival of thalassemia patients has increased, but there are still challenges regarding the complications of cardiac or endocrine dysfunction and the psychological problems of these patients. Many patients with chronic disease, including thalassemia major (TM), have shown a reduction in communication skills and self-efficacy. OBJECTIVE: The aim of this study was to investigate the self-efficacy among TM patients and determine the related factors among them. MATERIALS AND METHODS: This research was a cross-sectional study and consisted of 40 TM patients 7 to 19 years of age. The control group was 80 nonthalassemia patients. Data were gathered using a 2-part questionnaire. The collected data was entered into the SPSS (version 21) and were analyzed using descriptive and analytical statistics (Mann-Whitney U, and multiple linear regression model). RESULTS: According to the Mann-Whitney test, there was no significant difference between self-efficacy score among the affected and nonthalassemia groups (P=0.62). According to the regression model, only, the frequency of monthly blood transfusion was significantly correlated with self-efficacy score, so that by 1 U increase in frequency of monthly blood transfusion, we will have 11 U decrease in self-efficacy score (ß=-11, P=0.011). CONCLUSIONS: The results of this study showed that the rate of self-efficacy in children with thalassemia was moderate (52.5%) to good (45%). The allocation of a specific ward, easy access to health care staff, and social support for patients may seem to justify the moderate to good self-efficacy in these patients.

Early Kidney Damage Markers after Deferasirox Treatment in Patients with Thalassemia Major: A Case-Control Study.

BACKGROUND: The life of patients with ß-thalassemia major depends on blood transfusion. Regular blood transfusion leads to hemosiderosis in their main organs. The aim of this study was to compare the effects of deferasirox and deferoxamine on renal damage in patients with ß-thalassemia major. METHOD: The present case-control study was conducted on 60 individuals who were referred to the 17th Shahrivar Tertiary Referral Hospital in Guilan province, Iran. In this study, patients with ß-thalassemia major who used deferasirox (n = 21) and patients who used deferoxamine (n = 19) were evaluated. The control group (n = 20) was selected from healthy individuals. Serum creatinine (CREA), blood urea nitrogen (BUN), and Cystatin C were measured from blood samples. Furthermore, urinary (U.) neutrophil gelatinase-associated lipocalin (NGAL), albumin (Alb), interleukin- (IL-) 18, and Kidney Injury Molecule-1 (KIM-1) were measured by the ELISA method and normalized for U. creatinine (CREA). RESULTS: U. NGAL, U. IL-18, and BUN biomarkers in the deferasirox group were significantly higher than those in the control group (p < 0.001). U. NGAL/CREA and U. KIM-1/CREA ratios increased in both the deferoxamine and deferasirox groups compared to the control group (p < 0.05). U. Alb was significantly higher in patients treated with deferoxamine than in healthy participants (p < 0.05). CONCLUSION: The findings of this study indicate that after taking deferasirox, there was renal damage and an increase in inflammatory factors. Also, minor renal impairment was observed after deferoxamine administration, but it was not confirmed at the molecular level (U. NGAL and KIM-1). Therefore, it seems that patients who are taking these two drugs should be monitored carefully.

The Cutoff of Ferritin for Evaluation of Hypothyroidism in Patients With Thalassemia.

BACKGROUND: Thalassemia major is a genetic disease with a recessive autosomal pattern of inheritance that occurs as a result of disorder in hemoglobin synthesis. Researchers aimed to investigate the cutoff of ferritin for the development of hypothyroidism in patients with thalassemia major. MATERIALS AND METHODS: This was a retrospective analytic cross-sectional study that was conducted on the medical records of patients with thalassemia major. Overt hypothyroidism was defined as thyroid stimulating hormone (TSH) level of >6.5 mIU/L and T4 level of < 4.2 ng/dL and subclinical hypothyroidism was defined as TSH level of >6.5 mIU/L and T4 level of >4.2 ng/dL. Data were gathered by a form including age, sex, weight, height, body mass index, thyroid test results, and the mean of last 3 consecutive hemoglobin and ferritin levels. RESULTS: In this study, 67 patients were evaluated. The mean age of the participants was 15.37±3.73 years. The frequency of subclinical hypothyroidism was 10.4%, and no cases of clinical hypothyroidism were noted as well as secondary hypothyroidism. Although there was a significant linear correlation between mean ferritin level and TSH (P=0.008), no significant correlation was noted between levels of ferritin and T4 levels. As ferritin was significantly correlated with TSH, the results showed that the mean serum level of ferritin in ß-thalassemia major patients with hypothyroidism was higher than that in ß-thalassemia major patients with normal thyroid status (P=0.013). The cutoff point for ferritin was 1953 ng/mL (sensitivity=85.7%, specificity=60%). CONCLUSION: It seems that considering the development of hypothyroidism by reaching the ferritin cutoff point, intensification of the iron chelation regimen along with a shorter interval for laboratory endocrine examinations can be recommended.

Platelet Indices Alterations in Children With Type 1 Diabetes Mellitus.

Platelet (PLT) hyperactivity is a key factor which contributes to cardiovascular complications in patients with type 2 diabetes mellitus even in preclinical stages of disease. To the best of our knowledge, there is limited researches in this regard among patients with type 1 diabetes. The aim of this study was to evaluate hematologic indices indicating PLT activity in children with type 1 diabetes. This was a case-control study which was conducted on 166 inpatients in 17 Shahrivar children hospital, Rasht, Iran during April 2016 to April 2017. Cases and controls were 83 children with type 1 diabetes mellitus and 83 children hospitalized for thorough assessment of short stature, respectively. Groups were matched for age and sex. Demographic characteristics and hematologic variables were assessed. The Shapiro-Wilk test was used to determine the normality of the distribution. Results for continuous and categorical variables were demonstrated as mean±SD and number and percent, respectively. Continuous variables without normal distribution were demonstrated as median (interquartile range). The χ/Fisher's exact test was used to compare categorical variables. The normal and non-normal distributed quantitative variables were respectively assessed by independent T-test or Mann-Whitney U test. P-value <0.05 noted statistical significance. The median (interquartile range) age of all children was 10 (6 to 13) years old. Thirty-five (42.2) of patients with diabetes and 35 (42.2) of control group were male individuals. There were positive correlation between age (r=0.370; P=0.001), hemoglobin (r=0.278; P=0.009), blood sugar (r=0.243; P=0.027), PLT distribution width (r=0.229; P=0.038), plateletcrit (PCT) (r=0.290; P=0.008), PLT to lymphocyte ratio (r=0.230; P=0.037) and glycosylated hemoglobin in children with diabetes. The cut-off point of PCT was 0.19 (sensitivity=87.8%, specificity=66.7%). Only increased PCT (>0.19) was related with poor metabolic control and can put the patients to the risk of future cardiovascular events. The authors recommend considering multiple PLT parameters, and not just one of them, and even designing a scoring system in terms of PLT parameters for type 1 diabetes mellitus management programs.

Assessment of Serum Folic Acid and Homocysteine in Thalassemia Major Patients Before and After Folic Acid Supplement Cessation.

BACKGROUND: Thalassemic patients have ineffective erythropoiesis. In recent treatment protocols, there are little data on folic acid supplementation for patients with thalassemia because it is supposed that regular blood transfusions prevent bone marrow hyperfunctioning. OBJECTIVE: Investigators aimed to assess serum folic acid and homocysteine (Hcy) in thalassemia major patients before and after folic acid supplement cessation. PATIENTS AND METHODS: This study was a before-after controlled clinical trial conducted in 17th Shahrivar Hospital, Rasht, North of Iran, during May to October 2016. The patients enrolled in this study had thalassemia major on regular blood transfusion and older than 2 years of age. They had at least a 6-month history of folic acid supplement consumption before enrollment in the study (1 mg/daily). Complete blood count, serum folic acid, and serum Hcy were measured before discontinuation of folic acid supplement. Then, patients did not receive folic acid for a month and after 1 month of folic acid cessation, the measurements were repeated. All data were entered in SPSS version 20.0 and analyzed. RESULTS: Among the 40 patients in this study, 25 (62.5%) were female. The mean age of the participants was 21.39±11.17 years old. The mean of body mass index was 21.38±3.32 kg/m. Most of the participants had used folic acid supplement >5 years (29, 72.5%). The serum Hcy level was significantly increased (5.24±2.35 vs. 5.93±2.56; P=0.008) and serum folic acid level was decreased significantly (14.74±4.20 vs. 8.80±4.16; P<0.0001) from baseline. CONCLUSIONS: Cessation of folic acid supplementations in beta thalassemia major patients can lead to a significant decrease in serum folic acid and increase in Hcy levels. According to our findings and efficacy of folic acid in patients with beta thalassemia major, it is recommended to use the supplementation in all patients.