The incidence of invasive fungal infections (IFIs) has increased due to intensive chemotherapy in childhood leukemia. aim this study was evaluate the incidence, risk factors, causative pathogens, and impact on survival IFIs among pediatric leukemia patients.The hospital records 307 children with acute lymphoblastic (ALL, n=238), myeloid (AML, n=51), relapsed (n=18) between January 2010 December 2015 were retrospectively evaluated.A total 1213 febrile neutropenia episodes recorded 127 (10.4%)...

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report 4-year-old girl with probable SoJIA who presented MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). also failed intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation increased serum levels tumor necrosis factor-alpha (TNF-alpha) in...

Febrile neutropenia (FN) is an important complication that causes high rates of morbidity and mortality in patients with malignancies. We aimed to investigate the etiology, epidemiological distribution its change over years, clinical courses, outcomes FN children acute leukemia.We retrospectively analyzed demographic data, characteristics, laboratory results, severe complications, pediatric between January 2010 December 2020.In 153 patients, a total 450 episodes (FNEs) occurred. Eighty-four...

Although disseminated intravascular coagulation (DIC) has been a well-known disorder for many years, there is lack of sufficient number clinical trials about incidence, frequency underlying disorders, and prognosis DIC in children. The aim this study was to evaluate the frequency, etiologic factors, laboratory findings determine prognostic factors influencing mortality hospitalized pediatric patients. Medical records 5535 children who were investigated. Sixty-two patients diagnosed as acute...

Objective: We investigated the health-related quality of life (HRQL) in survivors pediatric acute lymphoblastic leukemia (ALL) and evaluated perceptions children, their siblings, parents.Materials Methods: Seventy ALL survivors, who were between 7 17 years age had completed therapy ≥2 years, included.The control group consisted healthy siblings.HRQL was assessed by age-specific KINDL R questionnaire.Results: No significant differences could be found among HRQL scores with respect to...

The authors overviewed 239 febrile neutropenia (FN) episodes in 82 pediatric leukemia cases treated with BFM treatment protocols. FN was observed mostly during consolidation therapy. Mucositis the most identified focus; gram-negative microorganisms were pathogens. Five patients developed invasive fungal infections. Fever resolved after mean 5.3 days and antibiotic administration time 12.7 days. Addition of G-CSF to antimicrobial therapy shortened duration neutropenia, but it did not affect...

Objective:The aim of this study is to determine the frequency dental anomalies (DAs) (microdontia, hypodontia, hyperdontia, enamel defect, root malformation) in pediatric cancer patients at ages <5 years and between 5 7 years, understand their relationship with received therapy. Materials Methods:Pediatric who were diagnosed treated before age investigated a case-control design.The included 93 whose diagnosis 9 months treatments completed 5-8 years.Group A consisted range 4 Group B 5-7...

Since a large variety of disorders may lead to lymph node enlargement determining the cause peripheral lymphadenopathy (LAP) in children can be difficult. This retrospective study evaluated 200 who were admitted an Oncology-Hematology department because and aimed determine clinical laboratory findings that valuable for differential diagnosis. A specific was documented 93 (46.5%) cases. One hundred forty (70%) classified as having benign enlargements. Fourteen (10%) these cases underwent...

In this study, we investigated whether a TATA box polymorphism in the promoter of UGT1*1 exon I, most common detected DNA Gilbert's syndrome, is contributory factor unexplained pathologic or prolonged jaundice. 38 neonates who had jaundice, 37 and 35 healthy, nonjaundiced were enrolled study. Genotypes assigned as follows: 6/6 (homozygous for normal allele bearing sequence [TA](6)TAA), 7/7 an abnormal with [TA](7)TAA), 6/7 (heterozygous one each allele). Of 110 infants, 10 (9%) 7/7, 51 (46%)...

Neonatal adrenal hemorrhage affects 0.2% of newborns and mostly due to birth-trauma, large birthweight, hypoxia, asphyxia, but it can occur spontaneously. Clinical features are varied, depending on the amount blood lost. The frequent clinical manifestations anemia, persistent jaundice, abdominal distention associated with an mass. However, some infants asymptomatic diagnosis is made only incidentally1. Only 19 cases neonatal presenting scrotal hematoma have been described in studies so far,...

The authors investigated demographics, clinical and laboratory features, treatment responses, outcomes of 93 children (median age 5 years) admitted for idiopathic thrombocytopenic purpura (ITP). therapy responses high-dose methylprednisolone (HDMP) (n = 77) intravenous immunoglobulin (IVIG) 10) treatments were similar. None the patients with hemorrhage died. Fifteen (16.1%) had progressed into chronic ITP. Seven infants a probable relationship vaccination; none these In conclusion, overall...

Munchausen syndrome by proxy (MBP) is an extreme form of child abuse where children were unnecessarily treated or investigated for medical conditions that falsified their caregivers. Here the authors report a 16-year-old female with complaints bleeding from multiple and unusual sites, including hemoptysis, hematuria, bloody tears, nipple discharge, all which are only witnessed her mother. Extensive investigation revealed no organic etiologies bleeding. The diagnosis MBP was put...

CANDLE syndrome (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) is a recently described autoinflammatory characterized by early onset, recurrent fever, skin lesions, multisystemic inflammatory manifestations. Most of the patients have been shown to mutation in PSMB8 gene. Herein, we report 2-year-old patient young onset facies, widespread generalized lymphadenopathy, hepatosplenomegaly, joint contractures, hypertrglyceridemia, lipodystrophy, autoimmune...

This study analyzes the data of thrombotic children who were followed up in different pediatric referral centers Turkey, to obtain more general on diagnosis, risk factors, management, and outcome thrombosis Turkish children. A simple two-page questionnaire was distributed among contact people from each center standardize collection. Thirteen responded invitation total number cases 271. All diagnosed with thromboembolic disease between January 1995 October 2001. Median age at time first event...

A newborn with cholestatic hepatic disease and hemophagocytic lymphohistiocytosis due to rhesus hemolytic (RHD) is reported.A 34 weeks' gestation baby RHD, who had received multiple intrauterine transfusions (IUT), developed secondary (HLH). Her serum ferritin level increased 5,527 ng/mL, liver biopsy showed severe iron overload. Treatment intravenous desferrioxamine resulted in a marked decrease levels normalization of functionWe suggest that patients have undergone IUT be evaluated for...

Summary. The development of an inhibitor against factor VIII (FVIII) is a serious complication in children with haemophilia A. Immune tolerance induction (ITI) therapy generally considered to be the best approach eradicate inhibitor. In this paper, low‐dose (≤50 IU kg −1 twice or three times weekly plasma‐derived concentrates) ITI regimen used Turkey discussed. This was given 21 A patients high titer inhibitors. median age at beginning 9 years and exposure days were 25. pre‐ITI historical...

Background In mammalian cells a regulatory mechanism, known as nonsense-mediated mRNA decay, degrades harboring premature termination codons. This mechanism is intron-dependent and functions quality control to eliminate abnormal transcripts modulates the levels of variety naturally occurring transcripts.Design Methods this study, we explored molecular ADAMTS13 deficiency in two compound heterozygous siblings carrying 29-nucleotide deletion mutation located exon 3...

Dimethyl sulfoxide (DMSO) is a cryoprotective agent used in storage of frozen stem cells cell transplantation. Central nervous system side effects DMSO such as epileptic seizures, stroke, transient global amnesia, and temporary leucoencephalopathy are rarely seen. Here, we report pediatric patient who developed seizures after DMSO-cryopreserved infusion whose magnetic resonance imaging the brain demonstrated parietal occipital focal cortical T2-signal intensity increase. toxicity should be...

Despite the use of primary prophylactic Factor VIII replacement in severe hemophilia A patients, bleeding into joints cannot be prevented completely and early diagnosis treatment joint bleedings are important for prevention permanent damage. Recent studies have shown that neoangiogenesis plays role development synovitis after recurrent bleedings. This study aimed to investigate relationship between findings levels serum angiogenic inflammatory factors patients.The patient groups consisted 10...

Objective: Rapid and effective treatment of invasive fungal infection (IFI) in patients with leukemia is important for survival.In this study, we aimed to describe variations regarding clinical features, modalities, time restarting chemotherapy, outcome children IFI acute (AL). Materials Methods: The charts all pediatric AL our clinic between the years 2001 2013 were retrospectively reviewed.All received prophylactic fluconazole during chemotherapy period.Results: was identified 25 (14%) 174...