Publications by authors named "Sezaneh Haghpanah"

115 Publications

A cost-analysis study of using adult red cell packs and Pedi-Packs in newborn intensive care units in Southern Iran.

Cost Eff Resour Alloc 2021 Mar 6;19(1):15. Epub 2021 Mar 6.

Clinical Research Development Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Background And Objective: Saving blood products is an important public health issue especially in developing countries with limited financial resources. We aimed to suggest a new hypothetical model to make a change in the current blood transfusion policy in the newborn intensive care unit (NICU) to reduce wastage of blood supplies as well as the risk of exposure to multiple donors.

Methods: In this cross-sectional study, all transfused neonates (n = 70) who were admitted to NICU of Nemazee Hospital, a tertiary referral hospital in Southern Iran, were evaluated between March and June 2019. Based on the information of neonates' transfusion during this study period and determined transfusion indices, a specific pediatric pack was suggested and the related total costs per transfusion, as well as the donor-exposure rate of the hypothetical and the current transfusion method, were compared.

Results: Considering the mean number of transfusions per neonate: 4 and mean volume of transfused packed red cells: 20 ml per transfusion, the cost-analysis of pediatric and the adult pack was presented. Arithmetically, we proved a higher total cost per transfusion for using adult pack comparing to pediatric pack. Additionally, using a pediatric pack set leads to a 24% reduction in RBCs wastage per transfusion and a 68.13% reduction in donor-exposure rate.

Conclusions: The assignment of a dedicated pediatric pack for neonates will be able to improve the cost-effectiveness by a substantial reduction in donor-exposure rate and blood wastage. This finding should be taken into consideration to generate economic growth and make improvements in child health status.
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http://dx.doi.org/10.1186/s12962-021-00267-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7937218PMC
March 2021

Blood Transfusion Practice in Operating Rooms in Nemazee Hospital in Southern Iran.

Arch Iran Med 2021 Feb 1;24(2):107-112. Epub 2021 Feb 1.

Clinical Research Development Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: The requests for blood products in elective surgeries exceed actual use, leading to financial wastage and loss of shelf-life. In this study, we assessed the blood transfusion indices in elective surgeries performed in the operating rooms.

Methods: In this cross-sectional study, from January to June 2017, a total of 970 adult patients who underwent elective surgeries in the operating rooms of Nemazee hospital, a general referral hospital in southern Iran, were investigated. Demographic, clinical, and laboratory data, such as hemoglobin (Hb), hematocrit (Hct), platelets, prothrombin time (PT), and partial thromboplastin time (PTT) were gathered from medical records. Blood utilization was evaluated using the following indices: cross-match to transfusion ratio (C/T ratio), transfusion probability (T%), transfusion index (TI), and Maximum Surgical Blood Order Schedule (MSBOS).

Results: The overall C/T, T%, and TI ratios were 2.49, 46.6%, and 0.83 for all procedures, and the highest and lowest ratios pertained to the thoracic and cardiac surgeries, respectively. The C/T ratio was ≥2.5 for all surgical procedures except for cardiac surgeries. T% was <30 for thoracic and orthopedics surgeries and ≥30 for other surgical procedures. In all surgical procedures, TI was less than 0.5, except for cardiac surgeries. Also, the MSBOS was about 3 units for cardiac surgeries and ranged from 0.5 to 1 units in other surgeries.

Conclusion: The results of this study showed a high quality blood transfusion practice in cardiac surgeries, possibly due to more focus on this critical ward. Assessing difficulties in the process of reservation, utilization, and preparation of standard protocols and policies are required to improve the blood utilization practice in operating rooms.
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http://dx.doi.org/10.34172/aim.2021.16DOI Listing
February 2021

A cost-effectiveness analysis of the prophylaxis versus on-demand regimens in severe hemophilia A patients under 12 years old in southern Iran.

Hematology 2021 Dec;26(1):240-248

Health Human Resources Research Center, School of Management and Medical Information Sciences, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: This study aimed to compare the cost-effectiveness of these two regimens in hemophilia A patients, under-12-years-old in southern Iran.

Methods: A cost-effectiveness study comparing prophylaxis versus on-demand was conducted on 34 hemophilia patients (24 and 10 patients were on the prophylaxis and on-demand regimens respectively) in 2017. The Markov model was used to estimate the economic and clinical outcomes. The costs were collected from the societal perspective, and the utility criterion was the 'quality adjusted life year (QALY)' indicator. The required data were collected using a researcher-made cost checklist, the EQ5D standard questionnaire and Hemophilia Joint Health Score. The probabilistic sensitivity analysis (PSA) was performed to determine the robustness of the results.

Results: The means of costs, joint health score and QALY in the prophylaxis regimen were $478,963.1 purchasing power parity (PPP), 96.67, and 11.98 respectively, and in the on-demand regimen were $521,797.2 PPP, 93.46 and 10.99 respectively. The PSA confirmed the robustness of the model's results. The results of the scatter plots and acceptability curves showed that the prophylaxis regimen in 97% of the simulations for the thresholds below $20950 PPP was more cost-effective than on-demand regimen.

Conclusion: Prophylaxis regimen showed the lower costs and higher effectiveness and utility in comparison with the on-demand regimen. It is recommended that prophylaxis should be considered as the standard care for treatment of hemophilic patients.
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http://dx.doi.org/10.1080/16078454.2021.1885123DOI Listing
December 2021

Trace Elements in Children with Acute Lymphoblastic Leukemia.

Asian Pac J Cancer Prev 2021 Feb 1;22(S1):43-47. Epub 2021 Feb 1.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: Although combined chemotherapy regimen leads to 80% remission in children with acute lymphocytic leukemia (ALL), malnutrition and altered serum trace elements as a consequence of chemotherapy agents, have become the new issue to deal with. With the aim to evaluate each trace element in childhood ALL, we investiguâtes six main trace elements before and after induction chemotherapy while considering age, gender and chemotherapy protocol as confounding factors.

Methods: Thirty-six newly diagnosed ALL children were recruited, and trace elements were assessed by atomic absorption spectrometry technique. Trace elements (Zinc, Copper, Manganese, Magnesium, Chromium and Iron) decreased significantly after induction chemotherapy.

Results: Considering the confounding factors, mean difference of elements decreased significantly, except for Chromium. Its mean difference was only significant in children younger than 10 and those who had received standard risk chemotherapy.

Conclusion: In conclusion, all the studied trace elements decreased significantly after induction chemotherapy session in ALL children. This highlights the importance of complementary and supplementary management. A larger cohort study with longer follow up is warranted to elucidate the long-term effect of chemotherapy on these trace elements on the general health status, quality of life or risk of relapse in ALL children.
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http://dx.doi.org/10.31557/APJCP.2021.22.S1.43DOI Listing
February 2021

Bayesian spatial modeling of transfusion-dependent β-thalassemia incidence rate in Fars Province, Southern Iran.

Spat Spatiotemporal Epidemiol 2021 Feb 7;36:100389. Epub 2020 Nov 7.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Using maps and spatial analysis are technologies to evaluate the magnitude and spatial distribution of disease in epidemiology investigations. We aimed to conduct a Bayesian spatial analysis on epidemiologic data of transfusion-dependent β-thalassemia (TDT) patients.

Methods: In this cross-sectional study, data of all TDT patients diagnosed during 1955-2018 in all counties of Fars Province were obtained from data registry of the Organization of Special Diseases of Shiraz University of Medical Sciences in Shiraz, Fars Province, Iran. Besag, York, and Mollie's (BYM) model was used for mapping.

Results: The estimated relative risk ranged from 0.23 to 1.66 for TDT patients. The highest and lowest relative risks of TDT were observed in Larestan located in Southern and Abadeh in Northern Fars Province respectively.

Conclusions: Determining the accurate geographical distribution of a chronic disease such as β-thalassemia can be an essential prerequisite in allocation of regional health system resources.
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http://dx.doi.org/10.1016/j.sste.2020.100389DOI Listing
February 2021

Extramedullary manifestations in acute lymphoblastic leukemia in children: a systematic review and guideline-based approach of treatment.

Am J Blood Res 2020 15;10(6):360-374. Epub 2020 Dec 15.

Department of Pediatric Nephrology, Bushehr University of Medical Sciences Bushehr, Iran.

Objective: Acute lymphoblastic leukemia (ALL) may present with signs and symptoms related to extramedullary involvement, therefore, leads to delayed diagnosis of ALL in children. This study aims to consider the extramedullary manifestations of ALL in children and their proper treatment.

Method: The databases were searched for all relevant subjects including "acute lymphoblastic leukemia", "clinical presentation", "unusual presentation", "childhood acute lymphoblastic leukemia", "presenting features of ALL", "extramedullary presentation", and "atypical presentation" from April 1968 to June 2020. The Inclusion criteria for this review study were all cases reported, case series, and studies about extramedullary presentations of ALL in pediatrics. Eighty-seven studies had inclusion criteria. All reported studies were analyzed given their extramedullary presentations, age, sex, treatment option, and prognostic factors. A two-sided -value less than 0.05 was considered statistically significant.

Result: In this review study, the extramedullary initial signs and symptoms of ALL were related to musculoskeletal system 17 (19.5%) especially bony symptoms and hypercalcemia. The additional extramedullary presentations of ALL in order of frequency include; renal involvement, 17 (19.5%), hepatic symptom 12 (13.8%), orbital presentation 10 (11.5%), neurologic signs 8 (9%), dermatological manifestations 5 (5.8%), oral presentations 5 (5.8%), hypereosinophilia 5 (5.8%), abdominal manifestation 3 (3.5%), pericardial involvement 2 (2.3%), and the other miscellaneous presentations 3 (3.5%).

Conclusion: The clinicians must become familiar with these extramedullary presentations of ALL in pediatrics to avoid the delayed diagnosis of this disease and increase the probable chance of survival by early detection.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7811904PMC
December 2020

Coronavirus Disease 2019 (COVID-19) Severity in Patients with Thalassemias: A Nationwide Iranian Experience.

Mediterr J Hematol Infect Dis 2021 1;13(1):e2021008. Epub 2021 Jan 1.

Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.

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http://dx.doi.org/10.4084/MJHID.2021.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7813286PMC
January 2021

Effects of three months of treatment with vitamin E and N-acetyl cysteine on the oxidative balance in patients with transfusion-dependent β-thalassemia.

Ann Hematol 2021 Mar 20;100(3):635-644. Epub 2020 Nov 20.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Oxidative stress is a major mechanism contributing to the progression of β-thalassemia. To assess the effect of vitamin E and N-acetyl cysteine (NAC) as antioxidant agents on total oxidative stress (TOS) status and total antioxidant capacity (TAC) in patients with transfusion-dependent β-thalassemia (TDT). In this open-label randomized controlled trial, from May to August 2019, 78 eligible patients with TDT over the age of 18 were enrolled. All patients were registered at the Thalassemia Clinic of Shiraz University of Medical Sciences in Southern Iran. Patients were randomly allocated to the NAC group (10 mg/kg/day, orally), vitamin E group (10 U/kg/day, orally), and control group. The duration of the study was 3 months. The mean age of the participants was 28.5 ± 5.1 (range: 18-41) years. At the end of the study, TOS significantly decreased only in the vitamin E group (mean difference (MD), 95% confidence interval (CI): 0.27 (0.03-0.50), P = 0.026). TAC significantly decreased in both supplemented groups at the 3rd month of treatment (NAC group: MD (95% CI): 0.11 (0.04-0.18), P = 0.002 and vitamin E group: 0.09 (0.01-0.16), P = 0.022 respectively). Hemoglobin did not significantly change at the end of the study in each group (P > 0.05). Mild transient adverse events occurred in 4 patients of the NAC group and 5 patients of the vitamin E group with no need to discontinue the treatment. Vitamin E can be a safe and effective supplement in improving oxidative stress in patients with TDT. Moreover, it seems that a longer duration of using antioxidant supplements needs to make clinical hematologic improvement in TDT patients.
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http://dx.doi.org/10.1007/s00277-020-04346-2DOI Listing
March 2021

Association of Exon 14 of the Gene Sequence Variations with Response to Hydroxyurea Therapy in Patients Carrying Non Transfusion-Dependent Thalassemia.

Hemoglobin 2020 Nov 8;44(6):406-410. Epub 2020 Nov 8.

Department of Pediatric Hematology/Oncology, Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Hydroxyurea (HU) activates the γ-globin gene, resulting in increased Hb F synthesis. The gene is a member of the Sox (Sry-type HMG box) family of transcription factors, characterized by minor groove binding domain. The DNA binding domain of this gene is encoded by exon 14. We assessed the relationship between response to HU and exon 14 of the gene sequence variations in patients with non transfusion-dependent thalassemia (NTDT). One hundred NTDT patients from southern Iran underwent HU therapy randomly participated in this cross-sectional study between February 2013 and October 2014. Based on response to HU therapy, the patients were divided into two groups: good and poor responder. Sequence variations of exon 14 of the gene was assayed by the Sanger sequencing technique. From all evaluated single nucleotide polymorphisms (SNPs) as above, we found no significant association between sequence variations of exon 14 of the gene and response to HU therapy ( > 0.05). It seems that no SNPs in exon 14 of the gene is associated with response to HU in NTDT patients, but more studies are needed for further evaluation.
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http://dx.doi.org/10.1080/03630269.2020.1845722DOI Listing
November 2020

Ocular findings in patients with transfusion-dependent β-thalassemia in southern Iran.

BMC Ophthalmol 2020 Sep 22;20(1):376. Epub 2020 Sep 22.

Poostchi Ophthalmology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Ocular involvement may occur via several mechanisms in patients with transfusion-dependent β-thalassemia (TDT) mainly chronic anemia, iron overload and iron chelator toxicity. We aimed to evaluate the frequency of abnormal ocular findings and their relationship with hematologic parameters in TDT patients.

Methods: In this cross-sectional study from January 2018 to January 2019, a total of 79 patients with TDT over the age of 18 who were on iron-chelation therapy (ICT) were consecutively investigated. All patients were registered at the Thalassemia Comprehensive Center affiliated with Shiraz University of Medical Sciences, Shiraz, Southern Iran. Complete ophthalmic examination was performed by an expert ophthalmologist. Clinical and hematologic parameters were collected from the patients´ medical records.

Results: The mean age ± standard deviation (SD) of the patients was 28.4 ± 5.6 years (range: 18-43). Twenty-four patients (30.4%) were male and 29 (36.7%) were splenectomized. The mean ± SD of the best-corrected visual acuity (VA) was 0.960 ± 0.086 decimal, (range: 0.6-1), 0.016 ± 0.046 logMar, (range: 0-0.2). The frequency of patients with VA > 0.1 logMar was 3 (3.8%). The mean intraocular pressure (IOP) was 14.88 ± 3.34 (6-25) mmHg. Fundus abnormalities were observed in 8 patients (10.1%), consisting of increased cup-disk ratio (3.8%), vessel tortuosity (2.5%), retinal pigment epithelium degeneration (2.5%), myelinated nerve fiber layer (1.3%), and internal limiting membrane wrinkling (1.3%). No significant association was observed between fundus abnormalities, VA, or IOP with hematologic parameters (P > 0.05). TDT patients with diabetes mellitus had significantly higher IOP (P = 0.010) but similar frequency of fundus abnormalities with non-diabetic patients (P > 0.05).

Conclusions: The frequency of ocular abnormalities in our patients was lower than the previous reports. The frequency of fundus abnormalities were similar in diabetic and non-diabetic thalassemia patients indicating close monitoring and proper management of the disease and comorbidities in these patients.
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http://dx.doi.org/10.1186/s12886-020-01647-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7510146PMC
September 2020

Bone mineral density in transfusion-dependent thalassemia patients and its associated factors in Southern Iran.

Arch Osteoporos 2020 09 21;15(1):148. Epub 2020 Sep 21.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR, Iran.

Despite the significant improvements in the management of thalassemia, there are growing concerns regarding their long-term complications. We showed that low bone mass is one of the most prevalent complications among these patients. Insufficient physical activity and hypogonadism are the main possible associated factors followed by DM and insufficient sun exposure.

Purpose: Despite the significant improvements in the management of transfusion-dependent thalassemia (TDT), there are growing concerns regarding their long-term complications.

Methods: This cross-sectional study included 615 TDT patients who were registered and followed in a comprehensive thalassemia clinic in Southern Iran. We measured serum biochemical tests and bone mineral density in all patients. We recorded physical activity and sun exposure subjectively, and an endocrinologist visited and examined all the patients. A group of age- and gender-matched healthy volunteers participated in the study as the control group.

Results: The mean age of the studied population was 28.4 ± 7.7 years, and 55.8% were female. The prevalence of vitamin D deficiency was 45.6% and 54.4% in TDT patients and the control group. A portion of TDT patients suffered from different endocrinopathies, which included hypogonadism (49.8%), diabetes mellitus (17.2%), hypoparathyroidism (14.6%), and hypothyroidism (6.3%). The prevalence of low bone mass in patients with TDT was 48.3 and 74.6% in the femoral and lumbar bones, respectively. Low physical activity, insufficient sun exposure, diabetes mellitus, and hypogonadism were associated with low bone mass.

Conclusion: Low bone mass is highly prevalent among TDT patients in Southern Iran. Insufficient physical activity and hypogonadism are the main possible associated factors, followed by DM and insufficient sun exposure.
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http://dx.doi.org/10.1007/s11657-020-00811-7DOI Listing
September 2020

Prevalence and clinical features of COVID-19 in Iranian patients with congenital coagulation disorders.

Blood Transfus 2020 09 29;18(5):413-414. Epub 2020 Jul 29.

Haematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

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http://dx.doi.org/10.2450/2020.0223-20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7592163PMC
September 2020

Association of HFE Gene Mutations With Serum Ferritin Level and Heart and Liver Iron Overload in Patients With Transfusion-dependent Beta-Thalassemia.

J Pediatr Hematol Oncol 2021 01;43(1):e26-e28

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz.

Objective: This study was performed on patients with transfusion-dependent beta-thalassemia (TDT) to investigate the effect of HFE gene mutations of iron overload in a large group of patients with TDT major and its relationship with heart and liver T2* magnetic resonance imaging (MRI) level.

Materials And Methods: In a cross-sectional study, a total of 253 patients with TDT who had regular blood transfusion were included in this study. HFE gene mutations including H63D and C282Y were evaluated in all patients through molecular assay. Heart and liver T2* MRI results, types, duration of iron therapy, and the demographic data including age, gender, serum ferritin level, blood transfusion, and splenectomy history of the included participants were also collected, using a questionnaire.

Results: Homozygous and heterozygous H63D mutation was found in 39.5% of the patients and C282Y mutation was found only in 1 patient. Ferritin level was significantly higher in patients with H63D mutation in comparison with patients without this mutation (P=0.036). Although heart T2* MRI and also the liver T2* MRI in the patients with H63D was slightly higher, the difference was not statistically significant. No significant correlation was observed between serum ferritin level and heart and liver T2* MRI, and iron chelation regimen.

Discussion: Heart and liver iron overload was not significantly different between patients with and without H63D mutation. As for serum ferritin, it was significantly higher among patients with H63D mutation compared with patients without this mutation. Hence, it is recommended to consider HFE gene mutations among patients with thalassemia to reach a better iron overload evaluation and management.
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http://dx.doi.org/10.1097/MPH.0000000000001944DOI Listing
January 2021

Prevalence and severity of Coronavirus disease 2019 (COVID-19) in Transfusion Dependent and Non-Transfusion Dependent β-thalassemia patients and effects of associated comorbidities: an Iranian nationwide study.

Acta Biomed 2020 09 7;91(3):e2020007. Epub 2020 Sep 7.

Quisisana Hospital, Ferrara.

Background: Coronavirus disease 2019 (COVID-19) outbreak is a global and challenging disease that is accompany with mortality and morbidity.

Aim Of Study: We evaluated the prevalence and the impact of comorbidities in thalassemia Iranian patients affected by COVID-19.  Methods: A multicenter, retrospective, cross-sectional study was conducted across all comprehensive thalassemia centers in Iran, from January to June 15th, 2020.

Results: Forty-three confirmed COVID-19 thalassemia patients (32 TDT, and 11 NTDT) were detected. The mean age of patients was 35.3 ± 11.5 years (range 9 - 67); 21 females and 22 males. Overall, 78.1% of TDT and 90.9% of NTDT patients were complicated with at least one comorbidity (P: 0.656). The overall mortality rate of thalassemia patients with COVID-19 was 18.6% while 27.3% was in NTDT patients compared to 15.6% in TDT patients (P:0.401). The dead group had a non-significant higher frequency of endocrinopathies compared to the recovered group (62.5% versus 45.7% P:0.457). Ten female thalassemia patients with positive COVID-19 had hypogonadism, six patients were receiving hormone replacement therapy and all of them recovered (zero death) compared to two deaths from 4 patients who were not receiving hormone replacement therapy (P:0.133). Furthermore, the prevalence of COVID-19 in NTDT patients was significantly higher than the general population (45 per 10,000 versus 22.29 per 10,000 respectively, P:0.018) while the prevalence of TDT was almost similar to the normal population (P:0.539). The mortality rate of COVID-19 was 4.71% in the normal Iranian population compared to 18.6% in β-thalassemias (P: <0.001) at the same date.

Conclusions: It is important to acknowledge that β-thalassemia patients, especially young adults/adults, have a chronic condition which may contribute to increase susceptibility to SARS-CoV-2 infection. A higher susceptibility to the infection was observed in patients with NTDT and in untreated hypogonadal female thalassemic patients. However, to confirm these data, more accurate designed studies are needed.
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http://dx.doi.org/10.23750/abm.v91i3.10155DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7716961PMC
September 2020

Evaluation of endocrine complications in beta-thalassemia intermedia (β-TI): a cross-sectional multicenter study.

Endocrine 2020 07 18;69(1):220-227. Epub 2019 Dec 18.

Pediatric and Adolescent Outpatient Clinic, Quisisana Hospital, Ferrara, Italy.

Background: Data on the prevalence and type of endocrine disorders in β-thalassemia intermedia (β-TI) patients are scarce. This multicenter study was designed to determine the prevalence of endocrine complications and the associated risk factors in a large group of β-TI patients.

Methods: In this cross-sectional multicenter study, 726 β-TI patients, aged 2.5-80 years, registered at 12 thalassemic centers, from nine countries, were enrolled during 2017. In a subgroup of 522 patients (mean age 30.8 ± 12.1; range: 2.5-80 years) from Qatar, Iran, Oman, Cyprus, and Jordan detailed data were available.

Results: Overall, the most prevalent complications were osteopenia/osteoporosis (22.3%), hypogonadism (10.1%), and primary hypothyroidism (5.3%). In the subgroup multivariate analysis, older age was a risk factor for osteoporosis (Odds ratio: 7.870, 95% CI: 4.729-13.099, P < 0.001), hypogonadism (Odds ratio: 6.310, 95% CI: 2.944-13.521, P < 0.001), and non-insulin-dependent diabetes mellitus (NIDDM; Odds ratio: 17.67, 95% CI: 2.217-140.968, P = 0.007). Splenectomy was a risk factor for osteoporosis (Odds ratio: 1.736, 95% CI: 1.012-2.977, P = 0.045). Hydroxyurea was identified as a "protective factor" for NIDDM (Odds ratio: 0.259, 95% CI: 0.074-0.902, P = 0.034).

Conclusions: To the best of our knowledge, this is the largest cohort of β-TI patients with endocrine disorders evaluated in extremely heterogenic thalassemic populations for age, clinical, hematological, and molecular composition. The study demonstrates that endocrine complications are less common in patients with β-TI compared with β-TM patients. However, regular monitoring with timely diagnosis and proper management is crucial to prevent endocrine complications in β-TI patients.
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http://dx.doi.org/10.1007/s12020-019-02159-6DOI Listing
July 2020

Evaluation of Efficacy, Safety, and Satisfaction Taking Deferasirox Twice Daily Versus Once Daily in Patients With Transfusion-Dependent Thalassemia.

J Pediatr Hematol Oncol 2020 01;42(1):23-26

Hematology Research Center, Shiraz University of Medical Sciences, Nemazee Hospital, Shiraz.

Objective: Deferasirox is a once-daily oral iron-chelation agent approved by the US Food and Drug Administration in November 2005. The authors aimed to evaluate efficacy, safety, and satisfaction of patients regarding twice-daily dose of deferasirox in patients with thalassemia who are resistant to once-daily regimen.

Methods: In this historical cohort multicenter study, 34 patients with beta-thalassemia major resistant or intolerant to once-daily dose of deferasirox (35 mg/kg/d) were investigated in 2016. Patients were registered at 3 thalassemia referral centers in Shiraz, southern Iran and Tehran, the capital of Iran. All patients were followed for 1 year and monitored by regular physical examination, laboratory data, serum ferritin levels, and heart and liver T2 magnetic resonance imaging.

Results: Mean age of thalassemia patients was 25.6±8.1 (8 to 40) years, including 22 female individuals and 12 male individuals. Serum ferritin levels significantly decreased during the study period (2021±955 at baseline vs. 1228±894 at the end of the study, P<0.001). Liver T2 magnetic resonance imaging of the patients demonstrated a significant improvement during the study. 73.3% of patients showed normal values at the end of study compared with 28.1% at the baseline (P<0.001). Drug side effects were reported only in 2 patients (5.8%) including 1 patient with abdominal pain and 1 with leukopenia and thrombocytopenia.

Conclusions: It seems that deferasirox can be used with increased dose and twice daily with acceptable efficacy in unresponsive or intolerant thalassemia patients to once-daily dose. Close monitoring of the patients is necessary to detect and manage any possible adverse events.
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http://dx.doi.org/10.1097/MPH.0000000000001596DOI Listing
January 2020

Effect of different iron chelation regimens on bone mass in transfusion-dependent thalassemia patients.

Expert Rev Hematol 2019 11 5;12(11):997-1003. Epub 2019 Sep 5.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

: Iron overload might lead to bone loss in transfusion-dependent beta-thalassemia (TDT) patients. To investigate the role of iron chelation therapy (ICT) on bone mineral density (BMD) of TDT patients suffering from iron overload, the authors compared the efficacy of five different iron chelation regimens through assessing serum ferritin and BMD.: In 256 consecutive TDT patients, BMD was measured by dual-energy X-ray absorptiometry in lumbar spine and femoral neck regions. Treatment outcome of five iron chelation regimens including Deferoxamine (DFO), Deferiprone (DFP), Deferasirox (DFX), and combination therapy was evaluated to compare the mean differences of serum ferritin and BMD indices pre- and post-treatment during 12-months follow-up period.: No significant difference was observed in DXA characteristics and serum ferritin level changes between ICT groups, but combination of DFO and DFX had the best outcome in improving bone mass through assessing each group individually.: Combination therapy with DFX and DFO had the highest impact on reducing serum ferritin, however insignificant, and improving bone loss in both lumbar spine and femoral neck in comparison with other regimens. A randomized prospective clinical trial is advised to accurately assess the efficacy of iron chelation regimens on BMD measurements of TDT patients.
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http://dx.doi.org/10.1080/17474086.2019.1662721DOI Listing
November 2019

Changing face of Candida colonization pattern in pediatric patients with hematological malignancy during repeated hospitalizations, results of a prospective observational study (2016-2017) in shiraz, Iran.

BMC Infect Dis 2019 Aug 30;19(1):759. Epub 2019 Aug 30.

Department of Environmental Health Engineering, School of Public Health, Tehran University of Medical Sciences, Tehran, Iran.

Background: Surveillance of current changes in the epidemiology of Invasive Fungal Diseases (IFDs) as an important component of the antifungal stewardship programs (ASP), requires careful regular monitoring, especially in high-risk settings such as oncology centers. This study aimed to examine Candida colonization status and corresponding current changes in children with malignancy during repeated admissions and also investigate the possible epidemiological shifts after the implementation of ASP.

Methods: In this prospective observational study, all eligible patients younger than 18 years were recruited during 2016-2017 at Amir Medical Oncology Center (AMOC) in Shiraz, Iran. Totally, 136 patients were enrolled and 482 samples were collected from different sites (oral/nasal discharges, urine and stool). Weekly regular sampling was carried out during hospitalization. Candida colonization status and epidemiological changes were monitored during repeated admissions. Samples were cultivated on Sabouraud Dextrose agar medium and identified by Polymerase Chain Reaction -Restriction Fragment Length Polymorphism (PCR-RFLP).

Results: Estimated Candida colonization incidence was 59.9% (82/136) in our patients. Candida colonization was found to be higher in oral cavity and rectum than that in nasal cavity. Among those long-term follow ups and repetitive hospitalizations, a significant number of patients exhibited changes in their colonization patterns (37.7%). Candida colonization did not reveal any significant relationship with age, sex, oncologic diseases and degree of neutropenia. C. albicans (72.0%) was found as the most common Candida species in colonized patients, followed by C. krusei, C. kefyr, C. glabrata and C. parapsilosis.

Conclusion: Given the high incidence of Candida infections in children with cancers, close monitoring of epidemiologic changes is essential for judicious management, based on local surveillance data and improvement of overall quality of care in high risk patients.
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http://dx.doi.org/10.1186/s12879-019-4372-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6717378PMC
August 2019

Frequency of silent brain lesions and aspirin protection evaluation over 3 years follow-up in beta thalassemia patients.

Ann Hematol 2019 Oct 6;98(10):2267-2271. Epub 2019 Aug 6.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Silent brain lesions might be associated with overt cerebrovascular accident over time in beta thalassemia major (BTM) and intermediate (BTI). Aspirin may be protective in these patients. We evaluated brain magnetic resonance imaging (MRI) in thalassemia patients to see whether aspirin is protective or not. A historical cohort study was conducted on 35 thalassemia patients, 22 BTI, and 13 BTM patients at Shiraz Hematology Research Center in 2018. Median age of the patients was 32 years and ranged from 8 to 42 years. Twenty-four patients (68.6%) were females. Overall frequency of white matter lesions (WMLs) in the first MRI was 10 patients (28.6%). After 3 years, 3 patients developed new lesions and the frequency of WMLs was 13 patients (37.1%) in the second MRI. Moreover, in 3 patients, number of WMLs increased. Patients with new lesions or more lesions compared to the baseline were significantly older than the other group (median age 36.5 years vs. 31 years, P = 0.046). Regarding aspirin consumption, only 1 patient (16.7%) of patients with new lesions was using aspirin compared to 10 (34.5%) of the other group (P = 0.640). The high-risk patients with thrombocytosis, splenectomy, severe iron overload, and older age (> 30 years) should be under close follow-up and evaluated on a regular periodic basis as well as brain MRI at least once every 3 years. Aspirin could be protective against new or progressive brain lesions so that low-dose aspirin is recommended in high-risk thalassemia patients.
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http://dx.doi.org/10.1007/s00277-019-03765-0DOI Listing
October 2019

Correlation of bleeding score with frequency and severity of bleeding symptoms in FXIII deficiency assessing by the ISTH Bleeding Assessment Tool.

Transfus Apher Sci 2019 Aug 9;58(4):495-497. Epub 2019 Jul 9.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Electronic address:

Objectives: The ISTH bleeding assessment tool (ISTH-BAT) is developed for standardization of bleeding symptoms in bleeding disorders. The aim of this study is to apply this bleeding score for FXIII deficient patients and its relation to the frequency and severity of symptoms.

Methods: In this cross-sectional study, 63 patients with severe FXIII deficiency were evaluated for the assessment of bleeding score according to the standard ISTH-BAT questionnaire. All patients were registered at two major thrombosis and hemostasis centers in Iran affiliated to Zahedan University of medical sciences (50 patients) and Shiraz University of medical sciences (13 patients).

Results: Significant correlations between the bleeding score and number of symptoms (r = 0.668, P < 0.001) and with a number of severe symptoms (r = 0.938, P < 0.001) were detected. There was no significant relationship between the mean bleeding score and CNS bleeding (P = 0.390).

Conclusion: The ISTH-BAT score is an acceptable bleeding assessment tool for standardization and evaluation of patients with FXIII deficiency.
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http://dx.doi.org/10.1016/j.transci.2019.05.012DOI Listing
August 2019

A retrospective study on clinical manifestations of neonates with FXIII-A deficiency.

Blood Cells Mol Dis 2019 07 11;77:78-81. Epub 2019 Apr 11.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran. Electronic address:

We assessed clinical presentations and the rate of central nervous system (CNS) bleeding in neonates with FXIIID who exhibited bleeding diathesis in the early days of their lives. A total of 27 neonates presented bleeding or abnormal clinical symptoms, diagnosed with FXIII deficiency were evaluated. Factor XIII concentrate was initiated as the first-line of treatment, and prophylactic therapy was given to all patients. Umbilical cord bleeding, delayed detachment of umbilical stunt, seizure, hematoma, and ecchymosis were concurrent complications in 27 (100%), 5 (18.5%), 5 (18.5%), 3 (11.1%), and 1 (3.7%) of the patients, respectively. History of having CNS bleeding was detected in 13 (48.1%) patients. There was no significant association between CNS bleeding and gender, familial history of FXIIID, or other clinical presentations. Also, there was no significant difference in the mean age of the patients who had CNS bleeding (3.4 ± 0.9 days) and without CNS bleeding (2.9 ± 0.7 days). However, a near significant threshold difference between the patients with and without CNS bleeding was found regarding the mean number of suspicious FXIIID death in their family (1.8 ± 0.5 and 0.7 ± 0.1, respectively, P = 0.05). Therefore, a suggested diagnostic algorithm based on prenatal diagnosis could be useful for timely detection of FXIII deficiency in neonates.
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http://dx.doi.org/10.1016/j.bcmd.2019.04.006DOI Listing
July 2019

Comparative effectiveness of alendronate and zoledronic acid on bone mass improvement in transfusion-dependent thalassemia patients.

J Bone Miner Metab 2019 Nov 11;37(6):996-1003. Epub 2019 Apr 11.

Hematology Research Centre, Nemazee Hospital, Shiraz University of Medical Sciences, 7193635899, Shiraz, Iran.

Thalassemia, as the most prevalent genetic blood disorder, has many associated comorbidities including low bone mass. We studied the comparative effectiveness of alendronate (AL) and zoledronic acid (ZOL) on bone mass improvement in transfusion-dependent thalassemia (TDT) patients a year after treatment. Three hundred seventy-five TDT patients with low bone mass were enrolled in this study. After a year of treatment with either AL or ZOL, a second bone mineral density (BMD) test was ordered to compare the effectiveness of the two aforementioned drugs. Body mass index (BMI), physical activity, sun exposure, and biochemical laboratory data were also considered as associated factors in this study. The BMD test of both groups was almost the same at the baseline and it increased comparably after a year of treatment with AL and ZOL. However, there was a significant difference in lumbar spine BMD delta Z score between both groups of female patients. ZOL was more effective in increasing the lumbar spine BMD of female patients. The choice of bisphosphonates therapy (oral versus parenteral) should be individually selected by considering patient's preference, compliance and the physician's decision. Given the longer administration interval, and TDT patients' compliance issue, it is justified to recommend ZOL as the drug of choice for patients suffering from low bone mass.
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http://dx.doi.org/10.1007/s00774-019-01003-1DOI Listing
November 2019

Expression of antiapoptotic proteins livin and survivin in pediatric AML patients, as prognostic markers.

Pediatr Hematol Oncol 2018 May 27;35(4):250-256. Epub 2018 Dec 27.

a Hematology Research Center , Shiraz University of Medical Sciences , Shiraz , Iran.

Objective: Survivin and livin are highly expressed in various malignancies and their expression levels may be related to unfavorable prognosis. The aim was to investigate the relationships of these two markers with some prognostic factors and with survival of the children with acute myeloid leukemia (AML).

Methods: Livin and survivin expression was investigated quantitatively by immunohistochemistry staining technique in 43 primary formalin-fixed, paraffin-embedded bone marrow blocks in pediatric age group (<18 years).

Results: Both survivin and livin were expressed in 81.4% of AML patients. Livin expression showed significant positive association with high level of primary WBC (p = .002). Survivin expression showed significant positive correlations with risk of relapse (p ≤ .001) and high level of primary WBC (p = .003). The relationship of overall survival (OS) of the patients with livin and survivin expression, were investigated separately in disease subtypes. Significant association was observed between survivin expression and shorter OS regardless of subtypes including acute promyelocytic (APL) (p = .01) and nonacute promyelocytic leukemia (non-APL) (p = .008). Also, significant association of livin expression with shorter OS was detected, but only in APL subgroup (p = .046). Nevertheless, in Cox regression model after adjusting for disease subtypes, stage and cytogenetics; survivin and livin showed no significant association with OS (p > .05).

Conclusion: Livin and survivin showed significant associations with some poor prognostic factors of AML. Although survivin in both subtypes and livin in non APL subtype, showed a significant relationship with shorter OS, none of them was determined as independent prognostic factors. Further studies with larger sample size are suggested.
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http://dx.doi.org/10.1080/08880018.2018.1530702DOI Listing
May 2018

Efficacy and safety of resveratrol, an oral hemoglobin F-augmenting agent, in patients with beta-thalassemia intermedia.

Ann Hematol 2018 Oct 21;97(10):1919-1924. Epub 2018 Jun 21.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Recently, resveratrol showed induction of γ-globin mRNA synthesis in human erythroid precursors and reducing oxidative stress in red cells of thalassemia patients in many in vitro studies. We aimed to investigate the efficacy and safety of resveratrol, for the first time, in non-transfusion-dependent beta-thalassemia intermedia (B-TI) in Southern Iran. In this double-blind randomized clinical trial, 54 patients with B-TI were investigated during 6 months between October 2016 and March 2017. Patients were randomly allocated into three groups by simple randomization method. Group 1 (hydroxyurea (HU) and placebo, 18 patients), group 2 (resveratrol/piperine and placebo, 16 patients), and group 3(HU and resveratrol/piperine, 20 patients). Primary end point was considered as change in hemoglobin (Hb) levels and need for blood transfusion. Drug safety was considered as a secondary end point. Mean age of the patients was 28.2 ± 5.6 (18-42) years. Response rate was not significantly different among the three groups (P > 0.05). Higher percentages of adverse events were detected in groups 2 (31.3%) and 3 (25%) compared to group 1 (5.6%). However, the difference was not statistically significant (P > 0.05). All reported adverse events were gastrointestinal symptoms. Resveratrol showed a similar efficacy with HU in the small population of non-transfusion B-TI patients during a 6-month follow-up. Complications, mostly gastrointestinal, were observed more frequently in resveratrol groups compared to the HU group. Although it was not statistically significant, more attention should be given to safety and efficacy of resveratrol as an oral HbF-augmenting agent.
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http://dx.doi.org/10.1007/s00277-018-3392-8DOI Listing
October 2018

Effect of ursodeoxycholic acid and vitamin E in the prevention of liver injury from methotrexate in pediatric leukemia.

Turk J Gastroenterol 2018 03;29(2):203-209

Department of Gastroenterology, Shiraz University of Medical Sciences, Shiraz, Iran.

Background/aims: Ursodeoxycholic acid (UDCA) and antioxidants such as vitamin E are considered to have a protective role in preventing chemotherapy-induced liver damage. The aim of this study was to assess the efficacy of these agents for hepatoprotection in pediatric patients with B-cell acute lymphoblastic leukemia (ALL), who were treated with methotrexate in their maintenance phase of treatment.

Materials And Methods: Eighty children with B-cell ALL were randomly divided into four groups. Group 1 was administered oral vitamin E (400 mg/day); group 2 was administered oral UDCA (15 mg/kg/day); group 3 was administered a combination of the two drugs; and group 4 served as a control group and was administered no drug except their chemotherapy drugs. Complete blood count, liver function test, liver ultrasonography, and liver fibroscan were requested, and the results were compared.

Results: Group 1 showed a slight increase in total bilirubin levels compared to baseline levels during the study (P=0.036). Group 2 showed a decline in aspartate aminotransferase and alanine aminotransferase levels during the study and at 6 months after discontinuing the drug; however, these differences were not statistically significant (P=0.051 and 0.083, respectively). None of the patients showed the evidence of significant fibrosis on liver fibroscan. Eight patients showed some evidence of mild-to-moderate fibrosis (F1, F2), but the results were not different between the groups as well as between pre- and post-study periods in each group.

Conclusion: Low-dose methotrexate does not cause significant liver fibrosis in pediatric leukemia. UDCA and vitamin E have minimal roles in hepatoprotection among pediatric patients with ALL.
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http://dx.doi.org/10.5152/tjg.2018.17521DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6284693PMC
March 2018

Relationship of the Interaction Between Two Quantitative Trait Loci with γ-Globin Expression in β-Thalassemia Intermedia Patients.

Hemoglobin 2018 Mar 10;42(2):108-112. Epub 2018 May 10.

a Hematology Research Center , Shiraz University of Medical Sciences , Shiraz , Iran.

Globin switching is a significant factor on blood hemoglobin (Hb) level but its molecular mechanisms have not yet been identified, however, several quantitative trait loci (QTL) and polymorphisms involved regions on chromosomes 2p, 6q, 8q and X account for variation in the γ-globin expression level. We studied the effect of interaction between a region on intron six of the TOX gene, chromosome 8q (chr8q) and XmnI locus on the γ-globin promoter, chr11p on γ-globin expression in 150 β-thalassemia intermedia (β-TI) patients, evaluated by statistical interaction analysis. Our results showed a significant interaction between one QTL on intron six of the TOX gene (rs9693712) and XmnI locus that effect γ-globin expression. Interchromosomal interaction mediates through transcriptional machanisms to preserve true genome architectural features, chromosomes localization and DNA bending. This interaction can be a part of the unknown molecular mechanism of globin switching and regulation of gene expression.
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http://dx.doi.org/10.1080/03630269.2018.1463915DOI Listing
March 2018

The frequency of hypothyroidism and its relationship with HCV positivity in patients with thalassemia major in southern Iran.

Acta Biomed 2018 03 27;89(1):55-60. Epub 2018 Mar 27.

Hematology Research Center,Shiraz University of Medical Sciences.

Introduction: Hypothyroidism is one the most complication due to iron overload in patients with β-thalassemia major (TM). On the other hand these patients are prone to Hepatitis C virus (HCV) infection that can cause  thyroid dysfunction by itself or as the side effect of treatment with interferon (INF) or IFN plus ribavirin. The aim of this study is to evaluate the association of hypothyroidism with HCV positivity and serum ferritin levels in patients with TM.

Methods: In this cross-sectional study, 201 randomly selected patients with TM who were registered at the Thalassemia Clinic of a tertiary hospital in Shiraz, southern Iran were investigated. Thyroid function tests and serologic screening assays for HCV seropositivity (HCV Ab and HCV-RNA) were conducted for all patients.

Results: Frequency of hypothyroidism was 22.9% including 19.9% subclinical hypothyroidism, 2% primary overt hypothyroidism and 1% central hypothyroidism. Eighty six patients (42.8%) were HCV Ab positive and 60 patients (29.9%) were HCV RNA positive. No significant relationship was found between hypothyroidism and HCV positivity or receiving IFN-α (P>0.05). Hypothyroidism showed a borderline significant association with high serum ferritin levels in TM patients (P=0.055).

Conclusion: Our results showed no significant association between hypothyroidism and HCV infection in TM patients. It seems that the main mechanism of hypothyroidism in our patients is iron overload; however, for better evaluation a larger multicenter study is recommended.  Also due to the importance of consequences of HCV infection, more careful pre-transfusional screening of blood should be considered in TM patients.
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http://dx.doi.org/10.23750/abm.v89i1.5778DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6357611PMC
March 2018

The Zinc and Copper Levels in Thalassemia Major Patients, Receiving Iron Chelation Therapy.

J Pediatr Hematol Oncol 2018 04;40(3):178-181

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz.

Essential trace elements deficiency including zinc and copper are frequently reported in the literature, but the results are controversial. The aim of this study was to evaluate zinc and copper levels in thalassemia major (TM) patients who were on regular transfusion and iron chelation therapy. In a case-control cross-sectional study 43 TM patients and 43 age-matched and sex-matched healthy controls were examined. Patients were selected by convenience sampling method from TM patients who were registered in Thalassemia Clinic during 6 months. Serum zinc and copper levels were evaluated in all subjects. Zinc and copper dietary intake were also assessed. The median zinc level in the participants was significantly lower than the control group (35 [6.3 to 298] vs. 173 [3.1 to 584] μg/dL; P<0.05), but the mean copper level was significantly higher in the patients in comparison with the control group (260±118 vs. 201±69 μg/dL; P<0.05). In contrast, the mean zinc and copper dietary intake in the patient's group were significantly lower than the control group. The mean serum zinc and copper levels in the patient's group were not different according to iron chelation therapy type. Also, zinc and copper levels in the patient's group were not statistically significant based on ferritin level, age, and duration of therapy. Essential trace elements level change and deficiency might occur in TM patients. Hence, routine assessment of these elements is recommended for better management.
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http://dx.doi.org/10.1097/MPH.0000000000001102DOI Listing
April 2018

A 25-year surveillance of disseminated Bacillus Calmette-Guérin disease treatment in children in Southern Iran.

Medicine (Baltimore) 2017 Dec;96(52):e9035

Alborzi Clinical Microbiology Research Center, Shiraz University of Medical Sciences Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Disseminated Bacillus Calmette-Guérin (BCG) disease is one of the most serious complications of BCG vaccination, mainly among immunocompromised children with high morbidity and mortality.Currently, there is no any consensus with regard to the standard regimen of antituberculosis (anti-TB) agents and duration of treatment in healthy or immunocompromised host in children. The aim of this study is to investigate the effect of various combination treatment strategies for disseminated BCG disease in children.In this cross-sectional study, the outcome of 3 different combination protocols was investigated in 59 patients.All patients were younger than 6 years old. Both possible immunocompetent and proven immunodeficient children were included in a period of 25 years (1991-2014) in a Nemazee referral teaching hospital.The minimum age was 1 month and the maximum was 60 months. The average age of patients was 8 months (8.26 ± 9.73). Out of 59 cases, 32 (54.2%) were female and 27 (45.8%) were male. Based on the primary work up, 52.5% of cases were classified as definite immunodeficient and 47.5% were classified as possible immunocompetent. Overall mortality rate was 50.8%. Mortality rate of disseminated BCG disease in immunocompetent and immunodeficient children was 28.6% and 71%, respectively. The mortality rate was not statistically different between patients treated with different treatment protocols. These results were not affected by immune status and the type of immunodeficiency.More than 2 anti-TB drugs combination will not change outcome of patient with disseminated BCG disease.
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http://dx.doi.org/10.1097/MD.0000000000009035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393028PMC
December 2017