Institution
Children's Memorial Hospital
Healthcare•
About: Children's Memorial Hospital is a based out in . It is known for research contribution in the topics: Population & Transplantation. The organization has 5652 authors who have published 8967 publications receiving 283837 citations.
Topics: Population, Transplantation, Medicine, Poison control, Health care
Papers published on a yearly basis
Papers
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TL;DR: Recurrence rates are increased when there are multiple preoperative infections and when there is no epithelial lining identified in the specimen.
Abstract: Objective We sought to review the presentation, evaluation, and treatment of branchial anomalies in the pediatric population and to relate these findings to recurrences and complications. Study Design and Setting We conducted a retrospective study at a tertiary care pediatric hospital. Patients Ninety-seven pediatric patients who were treated for branchial anomalies over a 10-year period were reviewed. Patients were studied if they underwent surgical treatment for the branchial anomaly and had 1 year of postoperative follow-up; 67 children met criteria, and 74 anomalies were studied. Results Patients with cysts presented at a later age than did those with branchial anomaly fistulas or sinus branchial anomalies. 32% of branchial anomalies were previously infected. Of these, 71% had more than one preoperative infection. 18% of the BA were first arch derivatives, 69% were second arch derivatives and 7% were third arch derivatives. There were 22 branchial cysts, 31 branchial sinusies and 16 branchial fistulas. The preoperative and postoperative diagnoses differed in 17 cases. None of the excised specimens that contained a cystic lining recurred; all five recurrences had multiple preoperative infections. Conclusions Recurrence rates are increased when there are multiple preoperative infections and when there is no epithelial lining identified in the specimen.
107 citations
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TL;DR: There are few pediatric data to indicate the efficacy and safety of pharmacologic or endoscopic therapies as primary prophylaxis or that prevention of a sentinel variceal bleed will ultimately improve survival; therefore, no recommendation for primary proPHylaxis with endoscopicvariceal ligation, sclerotherapy, or nonspecific beta‐blockade in children was proposed.
107 citations
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TL;DR: The two standard treatment options in children with craniopharyngioma are primary surgery and sub-total resection followed by radiotherapy, and utilization of an individualized risk-based treatment approach, that attempts to maximize cure rates without compromising long-term functional outcome.
Abstract: Background
To report the clinical outcome in children with craniopharyngioma following primary surgery and deferred radiotherapy at relapse.
Procedure
Twenty-five children with craniopharyngioma were treated with primary surgery. Total resection was achieved in 19 children (76%), while in 24% total resection was not achieved due to tumor adhesion to adjacent critical structures. None of these children received radiation therapy immediately after total or sub-total resection. Radiotherapy was delivered at the time of relapse in 11 patients (44%).
Results
The median follow-up from primary surgery was 10 years (3–16 years). The 14 year overall survival was 100%. Tumor recurrence was observed in (12/25) 48% at a median interval of 17 months. Tumor recurrence following total resection was 6/19 (32%) compared to 100% (6/6) following sub-total resection, and radiotherapy. The 2, 3, and 6 years relapse-free survival following initial surgery was 72, 55, and 50%, respectively. Univariate analysis revealed only extent of surgery to be significant for local recurrence (P < 0.0001). The sequelae observed in these patients included panhypopituitarism (100%), appetite disorders and hypothalamic obesity (32%), neuropsychological and behavioral disorders (20%), and sleep disorders (12%). Majority of children with non-endocrine complications had a local recurrence requiring further surgery and radiotherapy.
Conclusions
The two standard treatment options in children with craniopharyngioma are primary surgery and sub-total resection followed by radiotherapy. In certain subgroups of patients such as those with large tumors and hypothalamic extension, primary surgery is associated with a high incidence of complications and high failure rates. We recommend utilization of an individualized risk-based treatment approach, that attempts to maximize cure rates without compromising long-term functional outcome in children with craniopharyngiomas. Med Pediatr Oncol 2003;40:214–218. © 2003 Wiley-Liss, Inc.
107 citations
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TL;DR: The data show that MYO5B deficiency may lead to isolated cholestasis and that Myosin 5B should be considered as an additional progressive familial intrahepatic cholESTasis gene.
107 citations
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TL;DR: The controversies surrounding the potential role of pendrin in mediating apical iodide efflux into the lumen of thyroid follicles are discussed, and its functional role in the kidney and the inner ear is discussed.
Abstract: Pendred syndrome is an autosomal recessive disorder characterized by sensorineural hearing impairment, presence of goiter, and a partial defect in iodide organification, which may be associated with insufficient thyroid hormone synthesis. Goiter development and development of hypothyroidism are variable and depend on nutritional iodide intake. Pendred syndrome is caused by biallelic mutations in the SLC26A4 gene, which encodes pendrin, a transporter of chloride, bicarbonate and iodide. This review discusses the controversies surrounding the potential role of pendrin in mediating apical iodide efflux into the lumen of thyroid follicles, and discusses its functional role in the kidney and the inner ear.
107 citations
Authors
Showing all 5672 results
Name | H-index | Papers | Citations |
---|---|---|---|
Jorge E. Cortes | 163 | 2784 | 124154 |
Marc C. Hochberg | 127 | 691 | 87268 |
Michael Andreeff | 117 | 959 | 54734 |
Bharat Bhushan | 116 | 1276 | 62506 |
Donald M. Lloyd-Jones | 115 | 706 | 112655 |
David N. Herndon | 108 | 1227 | 54888 |
Frederick J. Schoen | 102 | 434 | 42611 |
Kathryn M. Edwards | 102 | 628 | 39467 |
Alan R. Dyer | 95 | 283 | 44252 |
Mark C. Willingham | 94 | 394 | 36167 |
Nicholas Katsanis | 93 | 348 | 34133 |
Peter D. Gluckman | 92 | 525 | 33375 |
Helga Refsum | 90 | 316 | 37463 |
Dale A. Schoeller | 90 | 391 | 30776 |
Shlomo Shinnar | 90 | 288 | 25621 |