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Tampa, FL, United States

Kardashian G.,New York University | Strongwater A.M.,St Josephs Childrens Hospital
Journal of Pediatric Orthopaedics Part B | Year: 2010

This report is a case of slipped capital femoral epiphysis in a nonambulatory patient with spastic quadriplegic type of cerebral palsy. Such a case is unusual as no weight-bearing forces were acting on the hip; however, spasticity may have played a role. To the best of our knowledge no earlier cases have been reported in the literature. © 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins.

Ringewald J.M.,St Josephs Childrens Hospital
Current Opinion in Cardiology | Year: 2016

Purpose of review: Transcatheter pulmonary valve replacement has only been both approved and widely available for most congenital heart disease centers for a few years; its use and familiarity for interventionalists have greatly expanded our knowledge of its applicability to a multitude of clinical situations. Expanded worldwide use and longer time from implant have both served to better understand procedural limits and uncommon late adverse events. Recent findings: Although currently approved for implantation in the USA only in dysfunctional and circumferential right ventricle to pulmonary artery conduits, with expanded experience operators have been able to adapt the delivery of this valve in a large number of additional clinical scenarios. Rare technical limitations of the procedure, most importantly coronary compression, are now being better defined. Although not frequent, a significant number of infective endocarditis episodes have been reported, but more recently several studies have deepened our understanding of this late adverse event for the most commonly implanted transcatheter pulmonary valve prosthesis. Summary: Expanded and widened use has extended our understanding of who may benefit from transcatheter pulmonary valve implantation (TPVI), the current limits of TPVI, and uncommon but important late issues following TPVI. Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

Rosenberg P.S.,U.S. National Cancer Institute | Zeidler C.,Kinderklinik | Bolyard A.A.,University of Washington | Alter B.P.,U.S. National Cancer Institute | And 9 more authors.
British Journal of Haematology | Year: 2010

In severe congenital neutropenia (SCN), long-term therapy with granulocyte colony-stimulating factor (G-CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long-term risk remains uncertain. We updated a prospective study of 374 SCN patients on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. Long-term, the annual risk of MDS/AML attained a plateau (2·3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita. © Published 2010. This article is a US Government work and is in the public domain in the USA.

Kuo D.J.,University of San Diego | Menell J.S.,St Josephs Childrens Hospital | Bender J.L.G.,Columbia University
Journal of Pediatric Hematology/Oncology | Year: 2016

Background: Alveolar soft part sarcoma is a rare soft tissue sarcoma that is characterized by a pattern of slow growth with metastases to the lung, bone, and brain that is not responsive to conventional cytotoxic chemotherapy. Observations: We describe 2 patients, with a combined 19 years of treatment experience including multiple different chemotherapeutic and targeted therapy regimens, surgery, and radiotherapy. We also present a review of the literature regarding treatment options to highlight recent findings. Conclusions: Alveolar soft part sarcoma is an indolent, but persistently progressive disease. Novel therapeutic agents hold promise in its management. © 2016 Wolters Kluwer Health, Inc. All rights reserved.

Ghersin Z.J.,St Josephs Childrens Hospital | Kuo D.J.,Rady Childrens Hospital San Diego
Journal of Pediatric Hematology/Oncology | Year: 2016

Melanotic neuroectodermal tumor of infancy is a rare benign tumor of neural crest origin. The tumor generally presents in the jawbones; however, it occasionally occurs in extracranial sites. Although 95% of these tumors present within the first year of life and 15% in extracranial locations, we report an unusual case of a 15-month-old male with melanotic neuroectodermal tumor of infancy of the epididymis. The patient underwent orchiectomy without adjuvant chemotherapy or radiation. Twenty months later, there was no sign of recurrence. In addition, we discuss the role of chemotherapy and radiation and the potential importance of molecular genetics in establishing guidelines for management. © 2016 Wolters Kluwer Health, Inc. All rights reserved.

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