Hemophagocytic lymphohistiocytosis mimicking surgical symptoms and complications: lessons learned from four cases.

Objective: Hemophagocytic lymphohistiocytosis (HLH) is a severe immunological disorder that leads to a massive inflammatory reaction that may prove rapidly fatal. We show that HLH may present by masquerading as surgical disease or as a postoperative complication leading to delays in diagnosis and treatment.

Study Design: A case series of four children with acute surgical presentation and prolonged unexplained postoperative sepsis, who were diagnosed with HLH.

Effects of early atipamezole reversal of medetomidine-ketamine anesthesia in mice.

Rodents are often anesthetized by using ketamine and medetomidine, with reversal by atipamezole. Methods vary for times of administration of the atipamezole, and literature is lacking regarding appropriate reversal time. We investigated the recovery of mice reversed with atipamezole 10 min (early) or 40 min (late) after induction of anesthesia.

Early UK experience in the use of clofarabine in the treatment of relapsed and refractory paediatric acute lymphoblastic leukaemia.

Clofarabine is a second-generation purine nucleoside analogue, which has shown promising activity in relapsed and refractory paediatric acute lymphoblastic leukaemia (ALL). This report summarizes the early United Kingdom experience of clofarabine for the treatment of paediatric ALL in 23 patients, outside of the context of a clinical trial. Our results demonstrated that clofarabine-based chemotherapy regimes were effective and well-tolerated in this heavily pre-treated group, with an overall response rate of 67% when used in combination regimes.

Reducing blood loss in synostosis surgery: the Liverpool experience.

Correction of craniosynostosis represents much of the workload in the pediatric designated UK craniofacial units. We reviewed recent operations as part of an ongoing unit audit cycle with outcome determined as blood use and complications or readmissions within 6 weeks of surgery. A pro forma was designed, and information from a chart search was collated on a Microsoft Excel (Microsoft, Seattle, WA) spreadsheet.

Chickenpox in varicella IgG positive patients: experience of a regional paediatric oncology centre.

Aciclovir prophylaxis was previously given to all immunocompromised patients treated by our unit, following contact with varicella zoster. In 2003, we changed practice according to National Guidelines, giving prophylaxis only to patients without serum varicella zoster immunoglobulin G antibody (VZ IgG) at diagnosis of their malignancy. Since then we have seen nine patients with acute lymphoblastic leukaemia (ALL) and VZ IgG positivity at diagnosis of their malignancy develop chickenpox.

Non-myeloablative transplantation for severe congenital neutropenia.

Severe Congenital Neutropenia is a rare condition characterized by a very low neutrophil count, which pre-disposes the affected child to recurrent bacterial infections. Treatment with granulocyte colony stimulating factor (G-CSF) has dramatically improved the prognosis of these children; but in patients who have become G-CSF refractory, hematopoeitic stem cell transplant is still the only effective curative treatment. We describe a patient who was unresponsive to escalating doses of G-CSF and underwent a successful reduced intensity conditioning, matched unrelated donor allograft resulting in cure.

Factor X deficiency presenting as a pseudotumor. Case report.

The authors report their experience in successfully treating a 15-week-old child who became comatose following a spontaneous intracerebral hemorrhage. It was initially believed that a tumor in the right frontal lobe caused the hemorrhage. Coagulation studies revealed abnormal results on presentation, and the problem was only partially corrected after an infusion of fresh frozen plasma.