Publications by authors named "Ezgi Paslı Uysalol"

6 Publications

  • Page 1 of 1

Glucose 6 phosphate dehydrogenase deficiency: A single-center experience.

Turk Arch Pediatr 2021 1;56(3):245-248. Epub 2021 May 1.

Pediatric Hematology and Oncology Clinic, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.

Objective: This study aims to evaluate the demographic information, clinical and laboratory findings of patients with glucose 6 phosphate dehydrogenase deficiency.

Material And Methods: We collected data by reviewing files and electronic records of 65 patients with glucose 6 phosphate dehydrogenase deficiency under the age of 18 years who were followed up in our clinic between 2007 and 2019. Demographic, clinical, and laboratory features, family history, complications of the disease, and history of splenectomy and cholecystectomy were evaluated. Mean, standard deviation, and median values were used when descriptive analyses were presented.

Results: The age of diagnosis ranged between 1-192 months and the median age of diagnosis was two months. Fifty-nine patients (90.7%) were boys and six (9.2%) were girls. The mean value of glucose 6 phosphate dehydrogenase enzyme on admission was 1,9±1,4 U/g of hemoglobin (Hb). Family history was pesent in 40% of patients in whom information was avaliable. The most common presentation was prolonged jaundice and the most common physical finding was jaundice. Splenomegaly was detected in none of the patients. Cholelithiasis was present in one of 21 patients who were evaluated with ultrasonography. None of the patients required splenectomy, cholecystectomy, and regular erythrocyte transfusion during follow-up.

Conclusion: As G6PD variants with chronic hemolysis are not usually seen in Turkey, patients who required splenectomy, cholecystectomy, and regular erythrocyte transfusion were not detected. Although glucose 6 phosphate dehydrogenase deficiency is more common in males, it can also be seen in girls. In Turkey, glucose 6 phosphate dehydrogenase deficiency should be considered in patients presenting with prolonged jaundice.
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http://dx.doi.org/10.5152/TurkArchPediatr.2021.20042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8152643PMC
May 2021

Safety and efficacy of deferasirox in patients with transfusion-dependent thalassemia: A 4-year single-center experience.

Pediatr Hematol Oncol 2021 Mar 22:1-8. Epub 2021 Mar 22.

Pediatric Hematology Oncology, Kanuni Sultan Suleyman Training and Research Hospital, Istanbul, Turkey.

This study was organized to determine the efficacy and safety of deferasirox (DFX) in reducing the SF of patients with transfusion-dependent thalassemia (TDT). This is a retrospective, descriptive study of 101 transfusion- dependent patients with thalassemia major who were followed for 48 months. Twenty-nine patients who used an alternative chelator either alone or combined, who were not compliant to the treatment, changed the drug due to adverse reactions, and had multiple transfusions and did not complete 4 years of DFX use were excluded. A total 72 out of 101 patients completed the study. SF decreases were noted for the 6-12 and >18-year age groups, from a median of 1532 ng/mL to 1190 ng/mL, and from 1386 ng/mL to 1165 ng/mL, respectively (p > 0.05). The proportion of patients with SF concentrations >2000 ng/mL is decreased (29% at baseline decreased to 15% at the end of the study) during the 48 months. The median SF of those who used <30 mg/kg/day (n = 38) increased from 767 ng/mL to 1006 ng/mL, whereas the >30 mg/kg/day (n = 34) group's SF concentrations decreased from a median of 1575 ng/mL to 1209 ng/mL (p = 0.029). The decrease of median SF values for Syrian patients was statistically significant (p = 0.043). Most common adverse events were gastric irritation symptoms (19.4%). The total DFX discontinuation ratio was calculated as 9.7%. Although dosages between 25-30 mg/kg/day are adequate to stabilize SF concentrations higher dosages are needed to achieve a statistically significant decrease.
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http://dx.doi.org/10.1080/08880018.2021.1901809DOI Listing
March 2021

Flow Cytometry-based Absolute Blast Count on Day 8: Reliable, Fast, and Inexpensive Method.

J Pediatr Hematol Oncol 2021 01;43(1):33

Clinic of Pediatric Hematology/Oncology Istanbul Kanuni Sultan Suleyman Research and Education Hospital, Istanbul, Turkey.

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http://dx.doi.org/10.1097/MPH.0000000000001838DOI Listing
January 2021

Burkitt Leukemia With Precursor B-Cell Immunophenotype and Dual Translocation of t(14;18) and t(8;14) in a Child: Case Report and Review of the Literature.

J Pediatr Hematol Oncol 2020 01;42(1):e61-e63

Department of Pediatric Hematology and Oncology, Kanuni Sultan Suleyman Education and Research Hospital.

Background: Burkitt leukemia (BL) with the precursor B-cell immunophenotype is a rarely reported condition. The prognosis of such patients is similar to that of classic BL. However, the combination of chromosomal translocations associated with bcl-2 and c-myc rearrangement has a poor prognosis.

Observations: An 11-year-old child presented with fever and weakness. Bone marrow aspiration showed morphologically L1 type blasts and flow cytometry analysis was compatible with precursor B-cell immunophenotype. Cytogenetic analysis revealed a combination of t(8;14) and t(14;l8).

Conclusions: The combination of t(8;14) and t(14;l8) can exhibit different immunophenotypical and morphologic features in leukemias.
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http://dx.doi.org/10.1097/MPH.0000000000001354DOI Listing
January 2020

Hemophagocytic Lymphohistiocytosis Associated With Visceral Leishmaniasis.

J Pediatr Hematol Oncol 2018 07;40(5):395

Pediatric Hematology and Oncology Department, Kanuni Sultan Süleyman Training and Research Hospital, Istanbul, Turkey.

This is the report of a 2-year-old boy who presented with fever, cytopenia, and splenomegaly. The patient was diagnosed with hemophagocytic lymphohistiocytosis (HLH) and treated with HLH-2004 protocol. Repeated bone marrow aspiration showed amastigotes on follow-up. In endemic countries, visceral leishmaniasis should be considered in the differential diagnosis to avoid chemotherapy toxicity.
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http://dx.doi.org/10.1097/MPH.0000000000001178DOI Listing
July 2018

Serum level of vitamin D and trace elements in children with recurrent wheezing: a cross-sectional study.

BMC Pediatr 2014 Oct 16;14:270. Epub 2014 Oct 16.

Department of Pediatrics, Istanbul University, Istanbul Faculty of Medicine, Capa, 34390 Istanbul, Turkey.

Background: We aimed to show the relationship between recurrence of wheezing and serum levels of vitamin D, zinc, and copper in wheezy children compared with a healthy group.

Methods: In this cross sectional study, seventy-three children with wheezing and seventy-five controls were included without a follow-up period. The clinical characteristics of the children were assessed, the asthma predictive index and temporal pattern of wheeze were determined. The serum levels of vitamin D, zinc, and copper were measured. Pearson correlation analysis was used to evaluate the relationship between homogeneously distributed variables.

Results: Thirty-two of the seventy-three children (43.8%) had more than three wheezing attacks (recurrent wheezing). The Asthma Predictive Index index was positive in 26 patients (35.6%). When classified to temporal pattern of wheeze, fifty-three of the study group (72.6%) had episodic wheezing and the remainder (27.4%) was classified as multiple-trigger wheezing. We found no overall significant difference between the study and control group in terms of vitamin D and trace elements . The vitamin D and zinc levels were significantly lower and serum copper and copper/zinc ratio was significantly higher in patients with recurrent wheezing (p =0.03, p <0.01, p =0.013, p <0.01, respectively) positive Asthma Predictive Index and multiple-trigger temporal pattern of wheeze compared with patients with non- recurrent wheezing, negative Asthma Predictive Index and episodic temporal pattern of wheeze.

Conclusion: It may be postulated that for the determination of asthma risk in patients with recurrent wheezing, the serum level of vitamin D, copper and zinc can be used as a routine biomarker alongside the Asthma Predictive Index and temporal pattern of wheeze.
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http://dx.doi.org/10.1186/1471-2431-14-270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4286924PMC
October 2014