Fatih Erbey

The use of a protein and energy dense eicosapentaenoic acid containing supplement for malignancy-related weight loss in children.

The aim of nutritional therapy in cancer patients is to prevent weight loss and to improve functional capacity and quality of life. Clinical studies however, have continued to demonstrate that a reduction in body weight loss is difficult to achieve in cancer cachexia. Several studies have shown that supplementation with eicosapentaenoic acid (EPA), an omega‐3 fatty acid, has anti‐cachectic effects in adult cancer patients. This study evaluated the clinical effects of a protein and energy dense EPA containing nutritional supplement in a group of pediatric cancer patients receiving active chemotherapy treatment.

Evaluation of nine children with reversible posterior encephalopathy syndrome.

BACKGROUND Reversible posterior leukoencephalopathy syndrome (PRES) is a neurological disorder characterized by signs of posterior cerebral edema upon radiographic examination. MATERIALS AND METHODS We retrospectively analyzed the records of nine children with the diagnosis of PRES. RESULTS Of the nine patients, seven were receiving immunosuppressive therapy and two were acute hypertensive crisis associated with renal disease. Immunosupressive drugs were intrathecal methotrexate in two patients, cyclosporine in two patients, intrathecal cytarabine in one patient, cyclophasphamide in one patient, and intravenous immunoglobulin (IVIg) in another one patient. The most presenting symptoms were seizure, headache, and altered consciousness. Six patients had seizures. Altered consciousness was present in four patients. Headache and nausea or vomiting was present also in six patients. Visual abnormalities were noted in two patients. Magnetic resonance imaging (MRI) studies showed white-matter abnormalities suggestive of edema in the posterior regions of the cerebral hemispheres, but the changes often involved other cerebral areas, the brain stem, basal ganglia or the cerebellum. The patients were treated with antihypertensive medications, and immunosuppressive therapy was withdrawn. In all the patients, the clinical and radiological findings resolved more completely. CONCLUSION Reversible posterior leukoencephalopathy may develop in patients who have renal insufficiency or hypertension or who are immunosuppressed. This syndrome should be recognized immediately and trigger agents can be discontinued to prevent long-term sequelae.

Mesenchymal Stem Cell Treatment for Steroid Refractory Graft-versus-Host Disease in Children: A Pilot and First Study from Turkey.

This study evaluated the efficacy of mesenchymal stem cells (MSCs) from bone marrow of a third-party donor for refractory aGVHD. We report the first experience using MSCs to treat refractory aGVHD in 33 pediatric patients undergoing allogeneic HSCT from Turkey. Totally, 68 doses of bone marrow derived MSCs were infused. The median dose of MSC was 1.18 × 106 cells per kg body weight. Overall, complete response (CR) was documented in 18 patients, partial response (PR) was documented in 7 patients, and no response (NR) was documented in 8 patients. The 2-year estimated probability of overall survival (OS) for patients achieving CR and PR/NR was 63.8% and 29.4%, respectively (p = 0.0002). While the cumulative incidence of transplant related mortality (TRM) at day 100 after first MSC infusion was 46.6% in PR/NR patients, there was no any TRM at day 100 after first MSC infusion in CR patients (p = 0.001). Twelve patients developed chronic GVHD (cGVHD); eight of them were alive, with five having extensive disease and three having limited disease. In conclusion, MSCs appear to be safe and effective treatment option for pediatric patients with steroid refractory aGVHD. But the efficacy of MSCs on cGVHD in aGVHD patients treated with MSCs seems to be limited.

Report of a Non-Hodgkin lymphoma case presenting with pleural effusion.

Initial presentation of Non-Hodgkin Lymphoma (NHL) as primary pleural lymphoma is extremely rare. We report a case with NHL, who was initially presented with pleural effusion and pleural thickening. Our patients at first received intensive treatment of broad spectrum antibiotics for 10 days with no change in the clinical status, and then were diagnosed with T-lymphoblastic lymphoma with the examination of pleural fluid by using cytologic and flow cytometric methods, and treated with LMT89 group B treatment protocol. Our case points out the necessity for considering the NHL within the differential diagnosis of any pediatric patient presenting with sterile pleural effusion and pleural thickening who does not respond to antimicrobial therapy.

The Diagnostic Value of Hepatic Arterial Velocity in Venoocclusive Disease After Pediatric Hematopoietic Stem Cell Transplantation

The aim of this study was to determine usefulness of measurements of maximal systolic velocity of the hepatic artery with Doppler ultrasonography in the diagnosis of venoocclusive disease (VOD) after hematopoietic stem cell transplantation. We prospectively obtained 5 sonograms per patient: pretransplantation, day +1, +7, +14, and +28 on 36 nonconsecutive children who underwent hematopoietic stem cell transplantation. We examined the hepatic artery, the portal, hepatic and splenic veins, the thickness of the gallbladder wall, the presence of ascites, and the liver and spleen size. The diagnosis of VOD was based on clinical and laboratory data. Patients were divided into 2 groups: those with VOD (n=18) and those without VOD (n=18). The variance of 2 groups was analyzed. Vmax of the hepatic artery had a strong correlation with clinical VOD diagnosis (P<0.001). There was no statistically significant difference in the other Doppler parameters. The results of our study showed that the measurement of Vmax of the hepatic artery can provide important support in the diagnosis of VOD and can be useful in the follow-up of treatment response.

Oral Valganciclovir as Preemptive Therapy for Cytomegalovirus Reactivation in Pediatric Hematopoietic Stem Cell Transplant Patients.

Cytomegalovirus (CMV) infection is one of the most common complications after allogeneic hematopoietic stem cell transplantations (HSCT). Valganciclovir (VGC) has increasingly been used as prophylaxis against CMV infection after solid organ transplantation, but data on the efficacy and safety of VGC in pediatric HSCT patients are limited. We present our experience with VGC following ganciclovir (GCV) as preemptive therapy in pediatric HSCT patients. A total of 46 patients (38% patients) were found to be positive for CMV reactivation. Patients were treated with GCV (group I, n: 22) or GCV followed by VGC (GCV+VGC, group II, n: 24). VGC was preferred in the treatment of outpatients, whereas inpatients were treated with GCV. There was no significant difference in CMV clearance (P=0.78), treatment duration (P=0.087), and second CMV infection (P=0.3) between the 2 groups. The length of hospital stay was 21 days in GCV group, 14 days in VGC following GCV group (P=0.07). There were no treatment-related side effect in both groups. In conclusion, oral administration of VGC as preemptive therapy was found to be safe and effective. It is also a more suitable application for pediatric patients instead of an intravenous route. It could reduce the duration of inpatient stay and cost of hospitalization.

Brain Natriuretic Peptide Levels in Pediatric Cancer Patients with Febrile Neutropenia

Brain natriuretic peptide (BNP) is considered as a prognostic marker in patients with sepsis, but no data are available on BNP in pediatric cancer patients with febrile neutropenia (FN). Twenty-five pediatric cancer patients with FN were included in this study. Serum BNP level was measured. The mean BNP level was 330.8 ± 765.3 pg/mL (5.9–3806 pg/mL). BNP levels of 12 patients were found over the normal level. High BNP levels were related to some conditions of the patients, and these were statistically significant (P < .05). These conditions were required erythrocyte suspension, had pneumonia, time stayed in hospital, and neutropenia time. When regression test was done, required erythrocyte suspension for anemia and had pneumonia were found to be statistically significant. In conclusion, this is one of the first studies on BNP levels in pediatric cancer patients with FN. However, further studies with large sample sizes are needed to confirm the results and provide new data about this issue.

Isolated pulmonary Langerhans cell histiocytosis in a 3-year-old girl with atypical radiologic findings.

We performed lung biopsy and diagnosed Langerhans cell histiocytosis (LCH). German-Austrian-Dutch group HX-83 chemotherapy protocol was started after the diagnosis. During the follow-up of the patient, a bilateral pneumothorax developed and a thorax tube was placed. In the follow-up, the pneumothorax got better and the thorax tubes were taken out. In the control tomography taken during the third month of chemotherapy, it was determined that there was a remission in the number and volume of cysts. As there was a distinct remission in the tomography of the patient after a total of 12 months of chemotherapy, the chemotherapy sessions were ended. The patient is now asymptomatic. Computed tomography (CT) remain abnormal but with significant improvement of interstitial pulmonary involvement and without pneumothorax after 28 months of follow-up (Fig. 1).

Coexistence of non-Hodgkin lymphoma and cyst hydatid disease and successful medical treatment.

ANSWER The patient’s percutaneous Tru-cut biopsy was performed from the solid mass in the abdomen, and the result was reported as Burkitt lymphoma (BL). The patient had a positive serum cyst hydatid antibody at 1:640 dilution detected by indirect hemagglutination (IHA). He was negative for other parasitic infections. The patient was diagnosed to have a stage II BL pulse cyst hydatid disease (CHD), and an NHL-BFM-90 chemotherapy protocol and albendazole (10mg/kg/d) was started. Chemotherapy was stopped after 3 cycles, as no masses were observable in the control tomographies of the patient. In the same tomographies, involution of the cystic lesion mass and peripheral calcifications were detected. The patient had a decrease in the amount of cyst hydatid antibody to a titre of 1:60 one month after the stop of chemotherapy, and he had no active hydatid cysts. His albendazole therapy was terminated at 8 months. He has been followed up for 3 years without treatment, and with no relapse or reinfection for neither of the 2 conditions.

Pediatric Parapharyngeal Castleman's Disease: a Case Report

Castleman hastaligi, nedeni ve etyolojisi belli olmayan nadir bir lenfoproliferatif hastaliktir. Iki klinik formu vardir. Lokalize form; genellikle yavas buyuyen kitle seklinde kendini gosterir. Multisentrik form ise, birden fazla anatomik bolgeyi tutar. Hastalik en sik mediastinal bolgede yerlesir. Parafarengeal yerlesim ise cok nadir olup, literature bakildiginda bizim ulasabildigimiz kadariyla cocukluk caginda toplam bes parafarengeal yerlesimli Castleman hastaligi vakasi bildirilmistir. Bu yazida, parafarengeal yerlesimli Castleman hastaligi tanisi alan, 14 yasinda bir kiz hasta sunulmustur.