Gulsan Yavuz

Successful treatment of central venous catheter infection due to Candida lipolytica by caspofungin-lock therapy.

Fungal infections, mainly represented by bloodstream infections (BSI) due to Candida spp., have maintained a constant incidence rate over the past 10 years, representing around 10% of catheter-related BSI (Kojic EM et al., Clin Microbial Rev 2004; 17: 255–67). The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related BSI recommend the systemic antifungal therapy and catheter removal (Pappas PG et al., Clin Infect Dis 2009; 48: 503–35). This is warranted both by the ability of Candida spp. to form biofilms that greatly reduce antifungal activity and by the extremely high risk of metastatic infections, particularly endocarditis and retinitis (Viale P et al., J Chemother 2006; 18: 235–49). In clinical practice, catheter removal is not always easily performed, depending both on type of device (for tunnelled or totally implanted catheters, a surgical procedure is needed) and on the patient s condition (e.g. patients with severe platelet or coagulation factor deficit). For these reasons, antifungal-lock technique (ALT) alternative to the device removal is proposed (Mermel LA et al., Clin Infect Dis 2001; 32: 1249–72). ALT consists of catheter lumen replenishment by a selected antimicrobial agent and then locking it for a time to eradicate the microbes embedded in endoluminal biofilms (Angel-Moreno A et al., J Infect 2005; 51: e85–87). Treatment of catheter-related BSI due to Candida spp. with ALT has been tried in few cases (Mermel LA et al., Clin Infect Dis 2001; 32: 1249–72; Buckler BS et al., Pediatr Infect Dis J 2008; 27: 762–4; Arnow P et al., Am J Med 1991; 90: 128–30; Viale P et al., Clin Infect Dis 2001; 33: 1947–8; Benoit JL et al., Clin Infect Dis 1995; 21: 1286–8). Herein, to our knowledge, we report the first case of non-complicated Candida lipolytica fungaemia related to a Hickman catheter cured with intraluminal caspofungin in addition to systemic caspofungin therapy.

CLINICAL AND EPIDEMIOLOGICAL CHARACTERISTICS OF RETINOBLASTOMA: Correlation with Prognosis in a Turkish Pediatric Oncology Center

Advanced intraocular tumors and metastatic disease in retinoblastoma patients still occur frequently in developing countries. The aim of this retrospective study was to describe the clinical and epidemiological characteristics of patients with retinoblastoma and the effects of these features on disease prognosis in the authors’ pediatric oncology unit as a developing country profile to define the problem. A retrospective chart review of 91 patients who presented to the unit between May 1996 and December 2003 was conducted in this study. Patients with unilateral disease presented at a median age of 24 months and those with bilateral disease at a median age of 9.5 months (p <. 01). Most of the eyes with retinoblastoma (68.6%) had Reese-Ellsworth stage V disease. Metastatic disease was diagnosed in 19 (20.9%) patients. Cases with metastatic disease presented at a median age of 24 months and those without metastatic disease at a median age of 12.5 months (p <. 05). In 31 patients (34.1%) there was a delay in diagnosis. The enucleation ratio in eyes with advanced intraocular stage was significantly higher than in eyes with early intraocular stage (57.9 vs. 3.8%) (p <. 001). In patients with metastatic disease, tumor recurrence was more frequent than in the nonmetastatic patients (36.8 vs. 4.2%) (p <. 01). Seven children (7.7%) died due to central nervous system (CNS) metastasis (p <. 01). Advanced intraocular disease and distant metastases occur more frequently in Turkish children with retinoblastoma than in children in developed countries, causing a higher rate of enucleation and mortality. Late referral might account for the delayed diagnosis.

Serum levels and differential expression of CD44 in childhood leukemia and malignant lymphoma: Correlation with prognostic criteria and survival

Abstract Background : The CD44, a cell surface proteoglycan, participates in a variety of function including tumor dissemination and metastasis. However, there are no available data on the prognostic significance of CD44 expression of tumor tissue correlated with serum sCD44 level in childhood leukemias and lymphomas.

Soy Isoflavones Ameliorate the Adverse Effects of Chemotherapy in Children

Genistein sensitizes cancer cells to chemotherapy and radiation by modulating cell survival pathways. At the same time, genisteins antioxidant and anti-inflammatory effects may protect normal tissues from adverse effects of chemotherapy and radiation, which are largely due to oxygen-free radicals and inflammation. We conducted a small pilot study with a soy isoflavone mixture containing 8 mg of genistein in children receiving chemotherapy and/or radiation to investigate genisteins potential toxicity preventive effect. We monitored clinical and laboratory parameters in children with cancer who received their first cycle of chemotherapy without genistein and the subsequent cycles with genistein. Patients served as their own controls, and the clinical-laboratory data from the first cycle were compared to the data from subsequent cycles. Nine cycles of chemotherapy were administered without genistein and 57 cycles with genistein. Patients experienced less myelosuppression, mucositis, and infection when they received genistein with chemotherapy. During supplementation, serum genistein levels were 2 to 6 times higher compared to presupplementation levels. Patients who received abdominal radiation reported less pain and diarrhea when they took the genistein supplement. Further clinical investigation of soy isoflavones in pediatric cancer patients receiving chemotherapy and/or radiation should be conducted.

RIGHT ATRIAL CATHETER-RELATED COMPLICATIONS IN PEDIATRIC ONCOLOGY PATIENTS: The Situation in a Developing Country

The complications of right atrial catheters (RACs) in pediatric oncology patients are unknown for centers in developing countries. This study examined the complications of RACs at Ankara University Medical School, Turkey. A total of 90 RACs were placed in 61 children for long-term chemotherapy with a total experience of 15,536 catheter days. The rate of catheter-related sepsis was 4.9 episodes per 1000 catheter days. Coagulase-negative staphylococci and Candida species were the most common organisms, accounting for 25.0 and 13.1% of all organisms, respectively. The most common reasons for the removal of the RACs were infection (42.4%) and dislodgement (32.2%). The rates of complications were significantly higher in this study than in western studies. This increase could be explained by the differences in catheter care practices in the Turkish center. In conclusion, the use of RACs in a developing country necessitates an appraisal of the benefits and risks for each patient and improvement of catheter care procedures.

OBSTRUCTIVE JAUNDICE: An Unusual Initial Manifestation of Intra-Abdominal Non-Hodgkin Lymphoma in a Child

Obstructive jaundice is an unusual manifestation of non-Hodgkin lymphomas in children. Although surgical drainage is one of the initial treatment choices in some cases, usually lymphomatous masses rapidly response to chemotherapy and jaundice decreases due to regression of the mass, without any surgical procedure. The authors report the case of a 16-year-old girl who presented with biliary obstruction due to a neoplasm involving the duodenum. Histological examination of the specimen, which was taken from the mass by endoscopic biopsy, revealed Burkitt lymphoma infiltrating the duodenum. Chemotherapy including cyclophosphamide was started immediately. In a few days, jaundice decreased rapidly by the shrinkage of the mass. Neither surgery nor percutaneous drainage were needed. In conclusion, biliary tract obstruction due to non-Hodgkin lymphoma can be effectively treated with chemotherapy alone without any surgical procedure.

SERUM LEVELS AND DIFFERENTIAL EXPRESSION OF INTERCELLULAR ADHESION MOLECULE-1 IN CHILDHOOD LEUKEMIA AND MALIGNANT LYMPHOMA: Prognostic Importance and Relationship with Survival

Serum levels and leukemic cell-tumor tissue expression of intracellular adhesion molecule-1 (ICAM-1/CD 54) were detected in 54 children with acute leukemia and malignant lymphoma. Serum samples were obtained from all patients before treatment and after cessation of the therapy from malignant lymphoma cases and during remission from leukemic patients. Twelve age-matched healthy children were included as a control group. The serum ICAM-1 levels were significantly higher in patients with acute lymphoblastic leukemia (ALL) or Hodgkins disease (HD) than those in the control group (median values: 350.9, 286.4, and 138.4 ng/mL, respectively; P < .01 in each comparison). However, there were no significant differences concerning serum ICAM-1 levels between the control group and each of the acute myeloid leukemia (AML), non-Hodgkins lymphoma (NHL), and Burkitts lymphoma (BL) case groups (median values: 235.7, 222.7, 195.9, and 138.4 ng/mL, respectively; P > .05 in each comparison). Moreover, serum soluble ICAM-1 levels significantly declined in ALL or HD patients who were in complete remission (median values: 185.0 and 145.4 ng/mL, respectively; P < .05 in each comparison). In HD patients high levels of serum ICAM-1 could be correlated with high ESR (P < .01), whereas no statistically significant difference could be found when serum ICAM-1 titers were compared with stages, B symptoms, and histological subgroups, probably because of the inadequate number of patients in each group. Expression of ICAM-1 was mainly attributed to lymphocytes, vessels, and weakly to Hodgkins cells, and this was significantly high in patients who were in advanced stages of disease. High serum sICAM-1 level was also associated with poor outcome and survival. Determination of serum level and/or tumor tissue expression of ICAM-1 in HD and ALL might represent an additional, but probably not independent, disease-associated marker to be used in the evaluation and/or monitoring of treatment response in patients with HD and ALL.

657del5 mutation in the NBS1 gene is associated with Nijmegen breakage syndrome in a Turkish family

We report on a consanguineous Turkish family whose first son died of anal atresia and whose second son presented with severe pre‐ and post‐natal growth retardation as well as striking microcephaly, immunodeficiency, congenital heart disease, chromosomal instability and rhabdomyosarcoma in the anal region. The proband was found to carry the homozygous 657del5 mutation in the NBS1 gene, which is responsible for Nijmegen breakage syndrome (NBS) in most of the Slav populations. Our family, the first diagnosed with NBS in the Turkish population, represents one of the most severely affected examples of the syndrome, with profound pre‐ and post‐natal growth retardation associated with structural abnormalities, and expands the clinical spectrum of this rare disorder.

DOES SERUM SOLUBLE VASCULAR ENDOTHELIAL GROWTH FACTOR LEVELS HAVE DIFFERENT IMPORTANCE IN PEDIATRIC ACUTE LEUKEMIA AND MALIGNANT LYMPHOMA PATIENTS

Vascular endothelial growth factor (VEGF) seems to play a central role in angiogenesis-lymphangiogenesis in hematological malignancies. There are limited data related to childhood hematologic malignancies. The aim of the study was to evaluate soluble VEGF (sVEGF) levels in children with acute leukemia and malignant lymphoma (ML) at diagnosis and in remission. The levels of serum sVEGF were measured by enzyme-linked immunosorbent assay (ELISA) in 20 children with acute leukemia, 33 children with different histopathological subtypes of ML, and 20 healthy controls. The levels of sVEGF at diagnosis (range 2 –1040 pg/mL; median 52 pg/mL) was significantly lower than in remission (range 136 –1960 pg/mL; median 630 pg/mL) in acute myeloid leukemia (AML) group (P = .018). The sVEGF levels at diagnosis (range: 2 –640 pg/mL; median 89 pg/mL) was significantly lower compared to remission values (range: 116 –1960 pg/mL; median 136 pg/mL) in patients with acute lymphoblastic leukemia (ALL) (P = .002). In ML group, including Burkitts lymphoma (BL), T-cell non-Hodgkins lymphoma (NHL), and Hodgkins lymphoma (HL), sVEGF levels at diagnosis were higher than remission levels, but there was no statistically significant difference (P >.05). On the other hand, there were significant difference between levels in active disease and control group, ie, BL versus control, T-cell NHL versus control, and HL versus control (P = .008, P = .043, P = .007, respectively). The authors noticed that sVEGF levels showed distinct behavioral pattern in different childhood malignancies at diagnosis and in remission. In acute leukemia and ML patients, VEGF acts through different pathophysiological mechanisms, in both bone marrow (BM) angiogenesis and lymphoid tissue lymphangiogenesis.

Zinc and Selenium Status in Pediatric Malignant Lymphomas

Ninety-six untreated patients with malignant lymphoma (ML), 81 Hodgkins disease, and 15 Burkitts lymphoma were studied for zinc (Zn) status, and 21 patients also had selenium (Se) status analysis. Plasma and hair Zn and Se levels were measured by atomic absorption spectrophotometry. Chronic Zn and Se deficiencies (low plasma and low hair Zn and Se levels together) were found to be associated with ML in Turkish children. This was most likely due to the poor “nutritional environment” of the patients because majority of the ML patients were from families of low socioeconomic status. Supplementation of pediatric ML patients with Zn and Se, in addition to standard chemotherapy and radiotherapy regimen, is recommended.