The Prophylaxis of Hepatic Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome With Defibrotide After Hematopoietic Stem Cell Transplantation in Children: Single Center Experience.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most severe and life-threatening complications after hematopoietic stem cell transplantation (HSCT). Defibrotide (DF) is approved for adult and pediatric patients with VOD/SOS with renal or pulmonary dysfunction after HSCT in the United States, and for severe VOD/SOS post-HSCT in patients above 1 month of age in the European Union. Several studies have examined whether DF prophylaxis can reduce the incidence of VOD/SOS in high-risk patients. A total of 334 pediatric allogeneic HSCT were included in this study. All patients received DF at the dose of 25 mg/kg/d, from the first day of the conditioning regimen to the 30th day after transplantation for VOD/SOS prophylaxis. Seventeen patients (5.08%) developed VOD/SOS; 4 of these had moderate, while 13 had mild VOD/SOS. None of the patients were developed severe or very severe VOD/SOS. In conclusion, we showed that prophylactic intervention with DF lowered the incidence of VOD/SOS in high-risk pediatric patients.

Central Nervous System Fungal Infections in Children With Leukemia and Undergoing Hematopoietic Stem Cell Transplantation: A Retrospective Multicenter Study.

BACKGROUND: Central nervous system fungal infections (CNSFI) are seen in patients with hematologic malignancies and have high morbidity and mortality. Because of their rarity, there is limited data on CNSFI in children with no established treatment protocols or guidelines. MATERIALS AND METHODS: In this multicenter retrospective study, 51 pediatric patients with leukemia, 6 of whom had undergone bone marrow transplantation, with proven or probable CNSFI were evaluated. Fungal infections were defined as proven or probable based on European Organisation for Research and Treatment of Cancer criteria. Proven CNSFI was diagnosed by appropriate central nervous system (CNS) imaging or tissue sample findings in combination with positive microbiological results of cerebrospinal fluid. A positive culture, microscopic evidence of hyphae, a positive result of the galactomannan assays are defined as positive microbiological evidence. Probable CNSFI was defined as appropriate CNS imaging findings together with proven or probable invasive fungal infections at another focus without CNS when there is no other explanatory condition. Data was collected by using the questionnaire form (Supplemental Digital Content 1, http://links.lww.com/JPHO/A541 ). RESULTS: Seventeen patients had proven, 34 patients had probable CNSFI. Headaches and seizures were the most common clinical findings. The median time between the onset of fever and diagnosis was 5 days. The most common fungal agent identified was Aspergillus . Sixteen patients received single-agent, 35 received combination antifungal therapy. Surgery was performed in 23 patients. Twenty-two patients (43%) died, 29 of the CNSFI episodes recovered with a 20% neurological sequelae. CONCLUSION: CNSFIs should be considered in the differential diagnosis in patients with leukemia and refractory/recurrent fever, headache, neurologicalocular symptoms, and a radiologic-serological evaluation should be performed immediately. Early diagnosis and prompt management, both medical and surgical, are essential for improving clinical outcomes.

Eye Movement Disorders Following Allogeneic Bone Marrow Transplantation on FK506 (Tacrolimus) and Ganciclovir.

FK506 (tacrolimus) is an immunosuppressive drug and more potent than cyclosporine. FK506 is widely used for immunosuppression in the prevention and treatment of graft-versus-host disease after allogeneic bone marrow transplantation and solid organ transplantation. Neurotoxicity is a recognized complication of FK506 therapy, but ptosis and weakness of eye abduction unilaterally has not been reported in association with FK506 administration to date. We discuss a 13-year-old male patient who developed ptosis and weakness of eye abduction unilaterally 90 days after transplantation with bone marrow from an unrelated donor, for acute lymphoblastic leukemia in this case report. FK506 therapy was administered for graft-versus-host disease prophylaxis and CMV infection was treated with ganciclovir. The physical examination findings completely resolved 72 to 96 hours after concomitant FK506 and ganciclovir treatment were terminated.

Comparison of echocardiographic findings with laboratory parameters in obese children.

UNLABELLED: Aim The purpose of our study was to evaluate the association between insulin resistance and left ventricular size and function in obese children. Material and methods A total of 79 cases aged 10-16 years and diagnosed with obesity and 79 healthy and non-obese cases as controls were included in the study. Patient and control groups were divided into three groups in terms of age as group 1 (10-12 years), group 2 (12-14 years), and group 3 (14-16 years). Fasting blood glucose, lipid profile, and fasting insulin levels of the cases were assessed. Mitral valve E and A waves, left ventricular ejection fraction, fractional shortening, end-diastolic and end-systolic diameters, left atrium diameter, and septal wall thickness were measured using echocardiography. RESULTS: Measurements of septal diastolic thicknesses, left atrium diameter, and left ventricular end-systolic diameter of all the three groups obtained by echocardiography were statistically higher compared with the controls. In all the patient groups, the mitral valve E/A ratio was >1. In groups 2 and 3, there was a positive correlation between fasting insulin levels and HOMA-IR and left ventricular end-systolic diameter, end-diastolic diameter, and septal systolic and diastolic wall thicknesses. CONCLUSION: In paediatric obesity, identification of early cardiac changes will be significant in allowing early diagnosis and treatment of cardiovascular diseases.

Early Detection of Central Nervous System Relapse of Pediatric Leukemia with Measurement of Optic Nerve Sheath Diameter on MRI.

BACKGROUND: Leukemia is the most common pediatric malignancy. Central Nervous System (CNS) is the most frequently involved extramedullary location at diagnosis and at relapse. OBJECTIVE: To determine if Magnetic Resonance Imaging (MRI) findings of optic nerves should contribute to early detection of CNS relapse in pediatric leukemia. METHODS: Twenty patients (10 boys, 10 girls; mean age 8,3 years, range 4-16 years) with proven CNS relapse of leukemia followed up between 2009 and 2017 in our institution were included. Orbital MRI exams performed before and during CNS relapse were reviewed retrospectively. Forty optic nerves with Optic Nerve Sheaths (ONS) and Optic Nerve Heads (ONH) were evaluated on fat-suppressed T2-weighted TSE axial MR images. ONS diameter was measured from the point 10 mm posterior to the globe. ONS distension and ONH configuration were graded as 0, 1 and 2. RESULTS: Before CNS relapse, right mean ONS diameter was 4.52 mm and left was 4.61 mm which were 5.68 mm and 5.66 mm respectively during CNS relapse showing a mean increase of 25% on right and 22% on left. During CNS relapse, ONS showed grade 0 distension in 15%, grade 1 in 60%, grade 2 in 25% and ONH demonstrated grade 0 configuration in 70%, grade 1 in 25% and grade 2 in 5% of the patients. CONCLUSION: MRI findings of optic nerves may contribute to diagnose CNS relapse by demonstrating elevated intracranial pressure in children with leukemia.