Great Ormond Street Hospital

About: Great Ormond Street Hospital is a healthcare organization based out in London, United Kingdom. It is known for research contribution in the topics: Transplantation & Population. The organization has 6535 authors who have published 11240 publications receiving 413647 citations. The organization is also known as: GOSH & Hospital for Sick Children.

Clinical features in 17 paediatric patients with Wegener granulomatosis.

Abstract: The aim of this report was to describe childhood patients with Wegener granulomatosis (WG) from one centre, to analyse the variety of clinical manifestations seen and compare the data with other published paediatric and adult series. The records of 17 patients with WG who were under the care of Great Ormond Street Hospital for Children (GOSH) from 1981 to 1998 were reviewed. We analysed presenting features before admittance to GOSH and the clinical signs observed whilst the children were under the care of the hospital. Of 17 patients, 13 were females and there was a male/female ratio of 1:3.25. Among the patients there were 2 sisters. The age of the patients at disease onset varied from 2 weeks to 14 years. The median/mean age was 6/6.3 years. American College of Rheumatology criteria for diagnosing WG were fulfilled in 11 of 17 patients. The frequency of different system involvement was: respiratory 87%, kidneys 53%, sinuses 35%, joints 53%, eyes 53%, nervous system 12%, skin 53%. cANCA was positive in 10 patients (59%), but pANCA was negative in all measured sera. Kidneys were involved in 2 of 8 patients (25%) with the disease onset from 0 to 5 years and in 7 of 9 patients (78%) with the disease onset from 6 to 14 years ( P<0.05). cANCA was positive in 7 of 9 patients with kidney disease (78%) and in 2 of 8 patients (25%) without kidney involvement ( P<0.05). Colchicine as a supplement to prednisolone and cytotoxic/immunosuppressant drugs was used effectively in 5 patients.

The post-transplant lymphoproliferative disorder—a literature review

Abstract: The Epstein-Barr virus (EBV)-induced post-transplant lymphoproliferative disorder (PTLD) affects 1%–10% of all paediatric renal transplant recipients. This is a heterogeneous group of conditions characterised by EBV-driven proliferation of B-lymphocytes in the face of impaired T-cell immune surveillance. The risk factors predisposing to PTLD are becoming better understood, but its pathogenesis and myriad of clinical and histological features remain poorly defined. While new treatment modalities are being tried with variable success, regular EBV surveillance and carefully monitored reduction of immunosuppression remain the mainstay of treatment. In this review, we have presented the current knowledge of this increasingly common complication in renal transplant recipients.

Consensus expert recommendations for identification and management of asparaginase hypersensitivity and silent inactivation

Abstract: L-asparaginase is an integral component of therapy for acute lymphoblastic leukemia. However, asparaginase-related complications, including the development of hypersensitivity reactions, can limit its use in individual patients. Of considerable concern in the setting of clinical allergy is the development of neutralizing antibodies and associated asparaginase inactivity. Also problematic in the use of asparaginase is the potential for the development of silent inactivation, with the formation of neutralizing antibodies and reduced asparaginase activity in the absence of a clinically evident allergic reaction. Here we present guidelines for the identification and management of clinical hypersensitivity and silent inactivation with Escherichia coli- and Erwinia chrysanthemi- derived asparaginase preparations. These guidelines were developed by a consensus panel of experts following a review of the available published data. We provide a consensus of expert opinions on the role of serum asparaginase level assessment, indications for switching asparaginase preparation, and monitoring after change in asparaginase preparation.