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Burki T.,Royal London Hospital | Kiho L.,Royal London Hospital | Scheimberg I.,Royal London Hospital | Phelps S.,Royal London Hospital | And 3 more authors.
Pediatric Surgery International | Year: 2011

Purpose: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. Methods: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. Results: We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma. Conclusion: Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine. © 2011 Springer-Verlag.

Gonorazky M.,Nino Jesus Childrens Hospital | Gamio S.,The Ricardo Gutierrez Childrens Hospital
Binocular Vision and Strabology Quarterly | Year: 2011

Anterior plagiocephaly is a craniofacial anomaly related to premature unilateral synostosis. We present a case of anterior plagiocephaly with vertical strabismus, overaction of the contralateral superior oblique muscle and an A pattern. A detailed ophthalmic examination and radio-imaging were done. The patient underwent strabismus surgery and resolution of the strabismus was obtained. Plagiocephaly has been reported to simulate ipsilateral superior oblique muscle paresis. We report a rare occurrence of contralateral superior oblique muscle overaction in an adult with anterior plagiocephaly.

Rabusin M.,Institute of Maternal and Child Health | Snowden J.A.,University of Sheffield | Veys P.,Great Ormond Street Hospital | Quartier P.,University of Paris Descartes | And 20 more authors.
Biology of Blood and Marrow Transplantation | Year: 2013

We analyzed the long-term outcomes of pediatric patients registered in the European Group for Blood and Marrow Transplantation database who underwent hematopoietic stem cell transplantation (HSCT) for severe treatment refractory autoimmune cytopenia. With a median follow-up of 100 months, event-free survival was 54% overall, with no significant difference between allogeneic HSCT (n = 15) and autologous HSCT (n = 7) recipients (58% versus 42%; P = .50). Despite a trend toward failure of response or relapse after autologous HSCT compared with allogeneic HSCT, the difference was not significant (43% versus 13%; P = .30). Treatment-related mortality was high in both HSCT groups (29% and 16%; P = .09). Based on the limited numbers of subjects in this retrospective analysis, both allogeneic and autologous HSCT may induce complete and persistent responses in approximately one-half of pediatric patients with severe refractory autoimmune cytopenia, although treatment-related toxicity is high. © 2013 American Society for Blood and Marrow Transplantation.

Styczynski J.,Nicolaus Copernicus University | Balduzzi A.,University of Milan Bicocca | Gil L.,Poznan University of Medical Sciences | Labopin M.,University Pierre and Marie Curie | And 20 more authors.
Blood | Year: 2012

We investigated prospectively factors influencing the safety of hematopoietic stem cell (HSC) collection in 453 pediatric donors. The children in the study donated either BM or peripheral blood stem cells (PBSCs) according to center policy. A large variability in approach to donor issues was observed between the participating centers. Significant differences were observed between BM and PBSC donors regarding pain, blood allotransfusion, duration of hospital stay, and iron supplementation; however, differences between the groups undergoing BM vs PBSC donation preclude direct risk comparisons between the 2 procedures. The most common adverse event was pain, reported mainly by older children after BM harvest, but also observed after central venous catheter (CVC) placement for PBSC collection. With regard to severe adverse events, one patient (0.7%) developed a pneumothorax with hydrothorax after CVC placement for PBSC collection. The risk of allotransfusion after BM harvest was associated with a donor age of < 4 years and a BM harvest volume of > 20 mL/kg. Children < 4 years were at higher risk than older children for allotransfusion after BM harvest and there was a higher risk of complications from CVC placement before apheresis.We conclude that PBSC and BM collection are safe procedures in children. © 2012 by The American Society of Hematology.

Styczynski J.,Nicolaus Copernicus University | Gil L.,Poznan University of Medical Sciences | Tridello G.,Policlinico G. B. Rossi | Ljungman P.,Karolinska University Hospital | And 24 more authors.
Clinical Infectious Diseases | Year: 2013

Background. The objective of this analysis was to investigate prognostic factors that influence the outcome of Epstein-Barr virus (EBV)-related posttransplant lymphoproliferative disorder (PTLD) after a rituximab-based treatment in the allogeneic hematopoietic stem cell transplant (HSCT) setting.Methods. A total of 4466 allogeneic HSCTs performed between 1999 and 2011 in 19 European Group for Blood and Marrow Transplantation centers were retrospectively analyzed for PTLD, either biopsy-proven or probable disease.Results. One hundred forty-four cases of PTLD were identified, indicating an overall EBV-related PTLD frequency of 3.22%, ranging from 1.16% for matched-family donor, 2.86% for mismatched family donor, 3.97% in matched unrelated donors, and 11.24% in mismatched unrelated donor recipients. In total, 69.4% patients survived PTLD. Multivariable analysis showed that a poor response of PTLD to rituximab was associated with an age ≥30 years, involvement of extralymphoid tissue, acute GVHD, and a lack of reduction of immunosuppression upon PTLD diagnosis. In the prognostic model, the PTLD mortality increased with the increasing number of factors: 0-1, 2, or 3 factors being associated with mortality of 7%, 37%, and 72%, respectively (P <. 0001). Immunosuppression tapering was associated with a lower PTLD mortality (16% vs 39%), and a decrease of EBV DNAemia in peripheral blood during therapy was predictive of better survival.Conclusions. More than two-thirds of patients with EBV-related PTLD survived after rituximab-based treatment. Reduction of immunosuppression was associated with improved outcome, whereas older age, extranodal disease, and acute graft-vs-host disease predicted poor outcome. © 2013 The Author.

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