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排序方式: 共有188条查询结果,搜索用时 15 毫秒
1.
Health care providers have begun to use critical pathways and order sets to standardize clinical care in order to improve clinical resource management. This article describes ways that critical pathways and order sets can be developed and implemented in a coordinated manner. This approach allows for a process that facilitates automation of critical pathways variance analysis and continuous feedback to clinicians. 相似文献
2.
Banu Aygun Nicole A. Mortier Matthew P. Smeltzer Barry L. Shulkin Jane S. Hankins Russell E. Ware 《American journal of hematology》2013,88(2):116-119
Glomerular hyperfiltration and microalbuminuria/proteinuria are early manifestations of sickle nephropathy. The effects of hydroxyurea therapy on these renal manifestations of sickle cell anemia (SCA) are not well defined. Our objective was to investigate the effects of hydroxyurea on glomerular filtration rate (GFR) measured by 99mTc‐DTPA clearance, and on microalbuminuria/proteinuria in children with SCA. Hydroxyurea study of long‐term effects (HUSTLE) is a prospective study (NCT00305175) with the goal of describing the long‐term cellular, molecular, and clinical effects of hydroxyurea therapy in SCA. Glomerular filtration rate, urine microalbumin, and serum cystatin C were measured before initiating hydroxyurea therapy and then repeated after 3 years. Baseline and Year 3 values for HUSTLE subjects were compared using the Wilcoxon Signed Rank test. Associations between continuous variables were evaluated using Spearman correlation coefficient. Twenty‐three children with SCA (median age 7.5 years, range, 2.5–14.0 years) received hydroxyurea at maximum tolerated dose (MTD, 24.4 ± 4.5 mg/kg/day, range, 15.3–30.6 mg/kg/day). After 3 years of treatment, GFR measured by 99mTc‐DTPA decreased significantly from 167 ± 46 mL/min/1.73 m2 to 145 ± 27 mL/min/1.73 m2 (P = 0.016). This decrease in GFR was significantly associated with increase in fetal hemoglobin (P = 0.042) and decrease in lactate dehydrogenase levels (P = 0.035). Urine microalbumin and cystatin C levels did not change significantly. Hydroxyurea at MTD is associated with a decrease in hyperfiltration in young children with SCA. Am. J. Hematol., 88:116–119, 2013. © 2012 Wiley Periodicals, Inc. 相似文献
3.
The effect of caloric restriction, as a model of nonthyroid illness, on serum thyroid hormone and TSH concentrations in hypothyroid patients was studied to determine if pituitary-thyroid function is altered in such patients, as it is in euthyroid subjects. Serum T4, T3, and TSH concentrations and serum TSH responses to TRH were measured in 5 untreated hypothyroid patients and 10 hypothyroid patients receiving T4 replacement therapy before and after restriction of caloric intake to 500 cal daily for 7 days. In 5 untreated hypothyroid patients, the mean serum T3 concentration declined 17%, from 75 +/- 14 (+/- SE) to 62 +/- 11 ng/dl. The mean basal serum TSH concentrations were 154 +/- 67 (+/- SE) microU/ml before and 161 +/- 75 microU/ml at the end of the period of caloric restriction, and the serum TSH responses to TRH were similar on both occasions. In 10 T4-treated hypothyroid patients, the mean serum T3 concentration declined 35%, from 110 +/- 8 to 71 +/- 8 ng/dl. In this group, mean basal serum TSH concentrations were 17 +/- 5.1 microU/ml before and 18.2 +/- 7.0 microU/ml at the end of the period of caloric restriction, and as in the untreated hypothyroid patients, the serum TSH responses to TRH were similar on both occasions. Mean serum T4 concentrations and serum free T4 index values did not change in either group. These results indicate that caloric restriction in both untreated and T4-treated hypothyroid patients is accompanied by 1) reduced serum T3 concentrations, as it is euthyroid subjects, and 2) no alterations in basal or TRH-stimulated TSH secretion. 相似文献
4.
Eisenhofer G Bornstein SR Brouwers FM Cheung NK Dahia PL de Krijger RR Giordano TJ Greene LA Goldstein DS Lehnert H Manger WM Maris JM Neumann HP Pacak K Shulkin BL Smith DI Tischler AS Young WF 《Endocrine-related cancer》2004,11(3):423-436
Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors that are usually benign, but which may also present as or develop into a malignancy. Predicting such behavior is notoriously difficult and there are currently no curative treatments for malignant tumors. This report follows from a workshop at the Banbury Conference Center, Cold Spring Harbor, New York, on the 16th-18th November 2003, held to review the state of science and to facilitate future progress in the diagnosis and treatment of malignant pheochromocytoma. The rarity of the tumor and the resulting fragmented nature of studies, typically involving small numbers of patients, represent limiting factors to the development of effective treatments and diagnostic or prognostic markers for malignant disease. Such development is being facilitated by the availability of new genomics-based tools, but for such approaches to succeed ultimately requires comprehensive clinical studies involving large numbers of patients, stringently collected clinical data and tumor samples, and interdisciplinary collaborations among multiple specialist centers. Nevertheless, the well-characterized hereditary basis and the unique functional nature of these neuroendocrine tumors provide a useful framework that offers advantages for establishing the pathways of tumorigenesis and malignancy. Such findings may have relevance for understanding the basis of other more common malignancies where similar frameworks are not available. As the relevant pathways leading to pheochromocytoma are established it should be possible to take advantage of the new generation of drugs being developed to target specific pathways in other malignancies. Again the success of this will require well-designed and coordinated multi-center studies. 相似文献
5.
Mitchell A. Rees Cara E. Morin Gerald G. Behr J. Christopher Davis Hollie Lai Ajaykumar C. Morani Marguerite T. Parisi Gaurav Saigal Sudha Singh Sireesha Yedururi Alexander J. Towbin Barry L. Shulkin 《Pediatric blood & cancer》2023,70(Z4):e29973
Adrenal tumors other than neuroblastoma are uncommon in children. The most frequently encountered are adrenocortical carcinoma and pheochromocytoma. This paper offers consensus recommendations for imaging of pediatric patients with a known or suspected primary adrenal malignancy other than neuroblastoma at diagnosis and during follow-up. 相似文献
6.
Keri A. Streby Marguerite T. Parisi Barry L. Shulkin Brian LaBarre Rochelle Bagatell Lisa Diller Stephan A. Grupp Katherine K. Matthay Stephan D. Voss Alice L. Yu Wendy B. London Julie R. Park Gregory A. Yanik Arlene Naranjo 《Pediatric blood & cancer》2023,70(8):e30418
Background
Diagnostic mIBG (meta-iodobenzylguanidine) scans are an integral component of response assessment in children with high-risk neuroblastoma. The role of end-of-induction (EOI) Curie scores (CS) was previously described in patients undergoing a single course of high-dose chemotherapy (HDC) and autologous hematopoietic cell transplant (AHCT) as consolidation therapy.Objective
We now examine the prognostic significance of CS in patients randomized to tandem HDC and AHCT on the Children's Oncology Group (COG) trial ANBL0532.Study design
A retrospective analysis of mIBG scans obtained from patients enrolled in COG ANBL0532 was performed. Evaluable patients had mIBG-avid, International Neuroblastoma Staging System (INSS) stage 4 disease, did not progress during induction therapy, consented to consolidation randomization, and received either single or tandem HDC (n = 80). Optimal CS cut points maximized the outcome difference (≤CS vs. >CS cut-off) according to the Youden index.Results
For recipients of tandem HDC, the optimal cut point at diagnosis was CS = 12, with superior event-free survival (EFS) from study enrollment for patients with CS ≤ 12 (3-year EFS 74.2% ± 7.9%) versus CS > 12 (59.2% ± 7.1%) (p = .002). At EOI, the optimal cut point was CS = 0, with superior EOI EFS for patients with CS = 0 (72.9% ± 6.4%) versus CS > 0 (46.5% ± 9.1%) (p = .002).Conclusion
In the setting of tandem transplantation for children with high-risk neuroblastoma, CS at diagnosis and EOI may identify a more favorable patient group. Patients treated with tandem HDC who exhibited a CS ≤ 12 at diagnosis or CS = 0 at EOI had superior EFS compared to those with CS above these cut points. 相似文献7.
Adel Abdallah Alberto Pappo Ulrike Reiss Barry L. Shulkin Zhengping Zhuang Karel Pacak Armita Bahrami 《Pediatric blood & cancer》2020,67(4)
We report an index case of a male patient who presented with all clinical manifestations of Pacak‐Zhuang syndrome, including early‐age polycythemia, multiple pheochromocytomas/paragangliomas, duodenal somatostatinoma, and ocular findings. Sequencing analysis detected an EPAS1 mutation in all tumors tested, but not in the germline. 相似文献
8.
MIBG detection of hepatic neuroblastoma: Correlation with CT,US and surgical findings 总被引:2,自引:0,他引:2
Metaiodobenzylguanidine (MIBG) imaging is used in the diagnosis, staging and follow-up of virtually every case of neuroblastoma seen at our institution. Normal sites of MIBG uptake include the liver and therefore difficulties have been predicted and encountered in the diagnosis of hepatic neuroblastoma due to inability to separate abnormally increased tracer deposition from normal hepatic activity. We reviewed every MIBG (I123 and I131) study performed at our pediatric hospital over a 4 year period encompassing 88 patients, 67 of whom had biopsy proven neuroblastoma. Hepatic findings on MIBG studies were compared with concurrent abdominal CT and US studies in all 67 patients. The clinical records of all patients with abnormal MIBG scans or abnormal CT or US studies of the liver were also reviewed. Eight patients were found to have abnormal liver findings on one or more imaging studies (MIBG, CT, or US). There were 3 true positive MIBG studies, one of which was an early study in a patient who later went on to have one of the false positive studies. Two patients had false positive MIBG scans for liver neuroblastoma. MIBG failed to detect liver involvement in 4 patients. 相似文献
9.
Thoracic neural crest tumors in Beckwith-Wiedemann syndrome 总被引:1,自引:0,他引:1
Thornburg CD Shulkin BL Castle VP McAllister-Lucas LM 《Medical and pediatric oncology》2003,41(5):468-469
10.