Intra-tumoral T cells in pediatric brain tumors display clonal expansion and effector properties.

Brain tumors in children are a devastating disease in a high proportion of patients. Owing to inconsistent results in clinical trials in unstratified patients, the role of immunotherapy remains unclear. We performed an in-depth survey of the single-cell transcriptomes and clonal relationship of intra-tumoral T cells from children with brain tumors.

A pilot study of genomic-guided induction therapy followed by immunotherapy with difluoromethylornithine maintenance for high-risk neuroblastoma.

Background: Survival for patients with high-risk neuroblastoma (HRNB) remains poor despite aggressive multimodal therapies.

Aims: To study the feasibility and safety of incorporating a genomic-based targeted agent to induction therapy for HRNB as well as the feasibility and safety of adding difluoromethylornithine (DFMO) to anti-GD2 immunotherapy.

Methods: Twenty newly diagnosed HRNB patients were treated on this multicenter pilot trial.

Dexmedetomidine for conscious sedation in difficult awake fiberoptic intubation cases.

Currently used methods of sedation for fiberoptic intubation such as benzodiazepines, propofol, or opioids have their limitations. Dexmedetomidine (DEX) is a selective alpha-2 adrenergic agonist that has been used clinically for its sympatholytic, analgesic, and sedative properties. We report on 4 patients with particularly difficult airways who underwent successful awake fiberoptic intubation with DEX.

Methemoglobinemia associated with dapsone therapy in a child with pneumonia and chronic immune thrombocytopenic purpura.

This report describes a case of methemoglobinemia in association with dapsone therapy. The patient, an immunocompromised child with chronic immune thrombocytopenic purpura, presented with fever, cough, perioral cyanosis, bilateral lower lobe rales, and low O2 saturation by pulse oximetry (89%). His medications included prednisone and rituximab for chronic immune thrombocytopenic purpura, and dapsone for Pneumocystis carinii pneumonia prophylaxis.

Thrombocytopenia and severe hyperbilirubinemia in the neonatal period secondary to congenital thrombotic thrombocytopenic purpura and ADAMTS13 deficiency.

Congenital thrombotic thrombocytopenic purpura (TTP) is an inherited form of TTP due to the deficiency of von Willebrand factor (vWF) cleaving protease ADAMTS13. The authors describe two children with congenital TTP who presented with thrombocytopenia, hemolytic anemia, elevated LDH levels, and schistocytes on peripheral blood smear. In both children, the diagnosis of the disease was delayed despite neonatal histories significant for thrombocytopenia, anemia, and severe hyperbilirubinemia.