Diagnostic Value of Neopterin during Neutropenic Fever and Determination of Disease Activity in Childhood Leukemias

Neopterin, a pteridine group compound that is secreted from macrophages is shown to be increased in adult leukemia; however there are few studies in childhood leukemia. This study aimed to investigate neopterin levels during childhood leukemia treatment and neutropenic fever episodes for the possibility of using as a marker for disease activity and differentiation of infections

Prognostic Factors and Long-Term Outcome in 52 Turkish Children With Hemophagocytic Lymphohistiocytosis

Albayrak, Meryem/0000-0003-2711-5150WOS: 000358289900002PubMed: 25901543Objectives: Hemophagocytic lymphohistiocytosis is a syndrome of pathologic immune activation that shares similar clinical and laboratory phenotypes with severe sepsis. Recent studies led to better recognition of hemophagocytic lymphohistiocytosis by clinicians, but no consensus exists on the criteria for high-risk patients. Design: We retrospectively reviewed the medical records of patients diagnosed with hemophagocytic lymphohistiocytosis to analyze the risk factors associated with poor outcome. Setting: Pediatric intensive care and hematology units of three tertiary hospitals in Turkey. Participants: Fifty-two children with hemophagocytic lymphohistiocytosis. Interventions: None. Measurement and Main Results: There were a total of 52 children meeting the diagnostic criteria of Histiocytic Society. Of them, 28 (54%) had a primary hemophagocytic lymphohistiocytosis. Mutation studies were performed in 18 of 28 patients (65%). Fourteen of them had PRF1, STX11, STXBP2, and UNC13D mutations, and four had Rab27a and LYST mutations. The remaining 24 patients (46%) were defined as having secondary hemophagocytic lymphohistiocytosis. Twenty-one of them had infection-associated hemophagocytic lymphohistiocytosis, and three had lysinuric protein intolerance. The mortality rate was significantly higher in primary hemophagocytic lymphohistiocytosis (64%) than in secondary hemophagocytic lymphohistiocytosis (16%) (p 0.05). Age below 2 years, hyperferritinemia, thrombocytopenia, high disseminated intravascular coagulation score at diagnosis, and no clinical response at 2 weeks of treatment were independent prognostic factors for poor prognosis. Conclusions: Our data suggest that disseminated intravascular coagulation score greater than or equal to 5 can be used in the definition of high-risk patients. Early recognition of poor risk factors has important prognostic and therapeutic implications

Severe Myelotoxicity Associated with Thiopurine S-Methyltransferase*3A/*3C Polymorphisms in a Patient with Pediatric Leukemia and the Effect of Steroid Therapy

WOS: 000348688800010PubMed: 25541649Myelosuppression is a serious complication during treatment of acute lymphoblastic leukemia and the duration of myelosuppression is affected by underlying bone marrow failure syndromes and drug pharmacogenetics caused by genetic polymorphisms. Mutations in the thiopurine S-methyltransferase (TPMT) gene causing excessive myelosuppression during 6-mercaptopurine (MP) therapy may cause excessive bone marrow toxicity. We report the case of a 15-year-old girl with T-ALL who developed severe pancytopenia during consolidation and maintenance therapy despite reduction of the dose of MP to 5% of the standard dose. Prednisolone therapy produced a remarkable but transient bone marrow recovery. Analysis of common TPMT polymorphisms revealed TPMT *3A/*3C

Remembering the Importance of an Old Friend: History Taking in Preoperative Evaluation of Healthy Children: A Single Center Experience

Albayrak, Meryem/0000-0003-2711-5150WOS: 000386045600005Objective: To investigate the consequences of routine laboratory tests that lead to surgical delay or high cost in patients with a normal medical history and physical examination who undergo minor surgical interventions. Patients and Method: Files of 1,322 patients aged between 0-16 years that had undergone elective surgical intervention within six years were reviewed. Results: Of the 1,322 patients, 1,246 (94.3%) had normal physical examination and laboratory findings. Seventy-six children who had abnormalities in laboratory findings and physical examination were referred to pediatrics. Of the 76 pediatric referees, 42 (55.3%) were reevaluated and were diagnosed with upper respiratory tract infection (n=23; 30.2%), iron deficiency anemia (n=5; 6.5%), innocent murmur (n=4; 5.3%), thalassemia minor (n=2; 2.6%), lower respiratory tract infection (n=2; 2.6%), urinary tract infection (n=1; 1.3%), mumps (n=1; 1.3%), acute gastroenteritis (n=1; 1.3%), minimal aortic and tricuspid valve insufficiency (n=1; 1.3%), minimal aortic stenosis (n=1; 1.3%), atrial septal defect (n=1; 1.3%). Surgical interventions were delayed until the recovery of the infectious diseases. In 25 of the patients, repeated tests showed normal ranges after the second test; however nine (n=9) of the patients showed increased or decreased numbers of white blood cell counts and whose medical history and physical examination revealed signs and symptoms related to infection. Conclusion: Routine laboratory tests contribute little to preoperative evaluation of children with normal history and physical examination undergoing low grade surgery

Do cerebral blood flow velocities change in iron deficiency anemia?

WOS: 000251077300006PubMed: 17984692Infants with iron deficiency had lower scores when tested for mental and motor development than their peers with better iron status. The aim of this study was to examine cerebral blood flow velocity in infants with iron deficiency anemia. Thirty-six infants (27 male, 9 female) with iron deficiency anemia, aged 6 to 36 months were divided into 2 groups according to the hemoglobin (Hb) values [group 1 (n = 23) Hb Hb >= 10g/dL]. In anterior and middle cerebral arteries only end-diastolic velocity (EDV) was increased in group 1 as compared with group 2 (P = 0.05 and P = 0.016, respectively), whereas in posterior cerebral artery both EDV and peak-systolic velocity were different between the groups (P = 0.024 and P = 0.004). Both peak-systolic velocity and EDV showed significant correlation with Hb level in the posterior cerebral artery (r = -0.38, P = 0.023 and r = - 0.35, P = 0.037) but not in the anterior and middle cerebral arteries. Increased cerebral blood flow velocities in children with lower Hb values may be due to increased cardiac output, decreased vascular resistivity caused by anemia

Could Lower Bone Turnover be a Cause of Chest Pain During Childhood?

Albayrak, Meryem/0000-0003-2711-5150WOS: 000282424800008PubMed: 20552184Chest pain, a frequent complaint during childhood, rarely originates from a cardiac pathology. Although it usually is idiopathic, it also could be associated with psychogenic, musculoskeletal, respiratory, and digestive disorders. This study aimed to investigate a possible relation between bone mineral density and chest pain in children. Bone mineral density and bone metabolism parameters were measured for 50 children with chest pain, and the findings were compared with those for 40 age- and sex-matched healthy children. Most of the cases (64%) were in the idiopathic group, and musculoskeletal chest pain was the second most frequent complaint (12%). Although bone mineral densities and osteocalcin levels did not differ significantly between the whole chest pain group and the control group, both were found to be lower in the musculoskeletal chest pain group than in other groups and the control group (p < 0.05). Musculoskeletal chest pain may be related to reduced bone mineral metabolism, and monitoring of risk factors is of particular importance.Kirikkale UniversityKirikkale University [03.08.02.01]This study was supported in part by grant no. 03.08.02.01 from Kirikkale University

Inherited coagulation disorders in Turkish children: A retrospective, single-center cohort study

Albayrak, Meryem/0000-0003-2711-5150WOS:000538124200008PubMed: 31980335Objective: This study aims to investigate the distribution, clinical characteristics and outcome of inherited coagulation disorders (ICD) in Turkish children. Subjects and methods: Data from all children (age < 18 years) with ICD examined in our center were retrospectively reviewed. Results: There were 403 children with ICD (233 males and 170 females) with a median age of four years at diagnosis. The percentages of von Willebrand disease (vWd), hemophilia and rare bleeding disorders (RBD) were 40 %, 34 % and 26 %, type-1, type-2 and type-3 vWd were 63 % 17 % and 20 %, hemophilia A and B were 84 % and 16 %, and severe, moderate and mild hemophilia were 48 %, 30 % and 22 %, respectively. Factor VII and FXI deficiencies were the most prevalent, comprising 56 % and 22 % of all children with RBD, respectively. Parental consanguinity rates were 72 % in type-3 vWd and 61 % in severe RBD. The overall prevalence of gastrointestinal bleedings was 4.5 % (18/403), intracranial bleeding (ICB) was 4.96 % (20/403), mortality from ICB was 30 % (6/20) and the overall mortality rate was 1.49 % (6/403). No life-threatening bleeding was seen during regular prophylaxis. Chronic arthropathy prevalence in severe hemophilia was 8 % with primary prophylaxis and 53 % with demand therap. Inhibitor prevalence was 14 % in hemophilia-A and 5 % in hemophiliaB. Conclusions: These data show that vWd is the most common ICD, type-3 vWd and RBD are prevalent due to frequent consanguineous marriages and diagnosis of ICD is substantially delayed in Turkish children. Prophylactic replacement therapy prevents occurrence of life-threatening bleedings and reduces the development of hemophilic arthropathy

Association of CYP3A5 Expression and Vincristine Neurotoxicity in Pediatric Malignancies in Turkish Population

Pinarli, Faruk Guclu/0000-0002-3241-2478; Albayrak, Meryem/0000-0003-2711-5150WOS: 000406231400018PubMed: 28697165Vincristine is a widely used chemotherapeutic agent in the treatment of childhood malignancies. Neuropathy is the most common adverse effect. CYP3A4 and CYP3A5 enzymes of cytochrome p450 enzyme system are responsible in vincristine metabolism. Genetic polymorphism may alter the vincristine metabolism and the neurotoxicity rate. In this study, distribution of CYP3A5 alleles among Turkish children with malignancies, relation between CYP3A5 genotype and neurotoxicity rates, as well as severity and duration of neuropathy and total vincristine doses were investigated. Patient group consisted of 115 patients (age, 1 to 17 y) with acute lymphoblastic leukemia and solid tumors, who were treated with vincristine consisting chemotherapy protocols. Control group consisted of 50 children without any neurological symptom or disorders. All patient files were reviewed for presence and severeness of neurotoxicity symptoms. Blood samples were obtained and CYP3A5 genotypes were analyzed. Neurotoxicity occurred in 20.8% of patients. Although it was found to occur more frequently after 4 doses of vincristine, and rates were higher in the low-dose vincristine group suggesting other contributing factors. Although neurotoxicity rate in the CYP3A5*1/*3 genotype was 17.6%, it was 21.6% in the CYP3A5*3/*3 genotype and the difference was not statistically significant (P<0.05). This study suggested that vincristine-related neurotoxicity is dose-independent and genotype is not the only causative factor in the occurrence of neurotoxicity in these patients.Gazi University Scientific Research Projects UnitGazi University [SBE-01/2011-72]Supported by Gazi University Scientific Research Projects Unit with a project number of SBE-01/2011-72