Children's Memorial Hospital

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.

Radical resections of childhood craniopharyngiomas.

Abstract: A series of 27 children with craniopharyngiomas is presented. All the craniopharyngiomas were detected by computed tomography, and radical total resection was attempted. Postoperative evaluations were done by a series of computed tomography and magnetic resonance imaging scans. Twenty patients who had total resection confirmed by surgical and radiographic observations showed no recurrence during the follow-up period of from 1 to 12 years. It is emphasized that total resection using modern diagnostic and surgical methods is the mainstay for childhood craniopharyngiomas.

Mucopolysaccharidosis type III (Sanfilippo syndrome) and misdiagnosis of idiopathic developmental delay, attention deficit/hyperactivity disorder or autism spectrum disorder

Abstract: Mucopolysaccharidosis III is a rare genetic disease characterized by progressive cognitive decline and severe hyperactivity that does not respond to stimulants. Somatic features are relatively mild. Patients are often initially misdiagnosed as having idiopathic developmental delay, attention deficit/hyperactivity disorder and/or autism spectrum disorders, putting them at risk for unnecessary testing and treatments. Conclusion Children with developmental or speech delay, especially those with a characteristic somatic feature or behavioural abnormalities, should be screened for MPS III.

Genetic staging of unresectable or metastatic neuroblastoma in infants: a Pediatric Oncology Group study.

Abstract: Background: Current staging systems for unresectable or metastatic neuroblastoma do not reliably predict responses to chemotherapy in infants under 1 year of age. Previous studies have indicated that the DNA content, or ploidy, of malignant neuroblasts can discriminate between good and poor responders in this group of patients, but the clinical utility of ploidy assessment has remained in question. Purpose: We tested, in a prospective nonrandomized study, the hypothesis that neuroblast ploidy could be used as the sole guide for treatment selection in infants with unresectable or metastatic tumors and could differentiate between those who would respond to our previous standard regimen and those who would benefit from an immediate switch to another therapy. Methods: One hundred seventy-seven infants were enrolled in this trial. Five of these infants were subsequently excluded (two ineligible, two lacking ploidy information, and one protocol violation); therefore, 172 patients were included in the study. One hundred thirty infants with hyperdiploid tumors (DNA index >1.0; better prognosis in retrospective studies) were treated with a well-tolerated regimen of cyclophosphamide (150 mg/m 2 per day orally or intravenously on days 1-7) and doxorubicin (35 mg/m 2 intravenously on day 8). Forty-two infants with diploid tumors (DNA index =1.0; worse prognosis in retrospective studies) received cisplatin (90 mg/m 2 intravenously on day 1) and teniposide (100 mg/ m 2 intravenously on day 3) after an initial course of cyclophosphamide plus doxorubicin. Statistical end points were response and long-term survival. In addition, we assessed within each ploidy group (i.e., patients with hyperdiploid tumors and those with diploid tumors) the prognostic significance of NMYC gene copy number, tumor stage, and other variables commonly measured in this disease. Results: Of the 127 assessable infants with hyperdiploid tumors, 115 (91%) had complete responses—85 after receiving five courses of cyclophosphamide plus doxorubicin and 30 after receiving further therapy including cisplatin plus teniposide. The 3-year survival estimate for the entire hyperdiploid group was 94% (95% confidence interval [CI] = 89%-98%). Nineteen (46%) of 41 assessable infants with diploid tumors were complete responders. The overall 3-year survival estimate for this group was 55% (95% CI = 39%-70%). Prognostic factor analysis indicated that NMYC gene amplification and an elevated serum lactate dehydrogenase level were statistically significant markers of higher risk disease within the diploid group (two-sided P values of .005 and .003, respectively). Only NMYC was predictive in the hyperdiploid group (P = .003). Conclusion: Use of a prognostic staging system based on tumor cell ploidy, augmented with the NMYC gene copy number and serum level of lactate dehydrogenase, would very likely improve the treatment of infants with unresectable or metastatic neuroblastoma. Patients with diploid tumors characterized by an amplified NMYC locus represent a particularly unfavorable risk group that may benefit from innovative new therapies. [J Natl Cancer Inst 1997;89:373-80]

When do seizures usually improve with the ketogenic diet

Abstract: Summary Purpose: Parents often expect immediate seizure improvement after starting the ketogenic diet (KD) for their children The purpose of this study was to determine the typical time to seizure reduction as well as the time after which it was unlikely to be helpful in those children started on the KD Methods: Records of all children started on the KD at Johns Hopkins Hospital, Baltimore (n = 83) and Children's Memorial Hospital, Chicago (n = 35) from November 2003 to December 2006 were examined to determine the first day in which seizures were reportedly improved Results: Of the 118 children started on the KD, 99 (84%) had documented seizure reduction The overall median time to first improvement was 5 days (range: 1–65 days) Seventy-five percent of children improved within 14 days In those children who were fasted at KD onset, the time to improvement was quicker (median 5 vs 14 days, p < 001) with a higher percentage improving within 5 days (60% vs 31%, p = 001) No difference was identified between fasting and nonfasting in regards to long-term outcomes, however Discussion: The KD works quickly when effective, typically within the first 1–2 weeks Starting the KD after a fasting period may lead to a more rapid, but equivalent long-term seizure reduction, confirming prior reports If the KD has not led to seizure reduction after 2 months, it can probably be discontinued