Choreiform activity in hemoglobin SC disease.
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Biomedical subjects
Publications and source records attributed to J Wilimas.
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We report diagnostic imaging and histopathologic findings in four children with different cystic renal neoplasms. The features discerned with computed tomography (CT), as well as ultrasound, correlated well with gross histologic findings, although a definitive diagnosis could not be made on the basis of CT and ultrasound alone. Because the most malignant variants of these lesions cannot be excluded preoperatively, we advocate complete diagnostic imaging studies, followed by nephrectomy, for all cases of cystic renal neoplasms in infants and children.
The purpose of this study was to explore, using grounded theory, the process experienced by parents who are dealing with the first recurrence of cancer in their child. The sample of 33 guardians (27 mothers, 1 grandmother, and 5 fathers) was drawn from three pediatric oncology settings. Data were collected through interviews, observations, and medical record review. Thirteen parents were interviewed to validate first the evolving and, later, the complete study findings. Four interactive components emerged: regulating shock, situation monitoring, alternating realizations, and eyeing care-limiting decisions. The overall organizing construct induced from these components was labeled "coming to terms." This construct represents the parents' efforts to overcome shock and despair to make wise decisions about treatment while accepting that the outcome if beyond their control, and to help their child have the optimal chance for cure while preparing for the child's possible death.
We describe a case of an adolescent with sickle-beta+ thalassemia who developed fatal fat embolism syndrome. After presenting with bone pain, the patient developed mental status changes, hypoxemia, and died following cardiorespiratory arrest.
PURPOSE/OBJECTIVES: To determine whether infusion method influences the quality of platelets transfused. DESIGN: Linked in vitro and in vivo study. Quasi-experimental design for in vitro and cross-over design with balanced randomization for in vivo. SETTING: Pediatric cancer center in the midsouthern United States. SAMPLE: Pheresed/pooled platelet units in vitro (n = 12). In vivo convenience sample of 26 children, ages 2-19 years, with cancer and thrombocytopenia who required platelet transfusion. METHODS: Two infusion pumps (IMED 980 and Gemini, IMED Corp., San Diego, CA) versus gravity flow for in vitro platelet infusion. Gemini infusion pump versus gravity flow for in vivo platelet transfusion. MAIN OUTCOME MEASURES: Platelet count, morphology score, and corrected count increment. FINDINGS: No significant differences noted in platelet count or morphology score among or across the three infusion methods in vitro. No significant differences noted between the two infusion methods in platelet count or corrected count increment in vivo. CONCLUSIONS: Although limited to a specific patient population, setting, and infusion device, findings revealed that the pump was clinically acceptable because it did not negatively affect platelet recovery. Replication of this study with other infusion devices is recommended. IMPLICATIONS FOR NURSING PRACTICE: Study findings validate the current nursing procedure for the administration of platelets at the study setting. Use of infusion pumps for platelet transfusions is time-efficient and energy-efficient for nurses because the pumps offer a well-controlled infusion rate, accurate volume measurement, and an alarm system for monitoring the infusion.
PURPOSE/OBJECTIVES: To determine the influence of infusion rate on quality of transfused platelets and patients' physical and subjective responses. DESIGN: Linked in vitro and in vivo studies with repeated measures and crossover designs, respectively. SETTING: A 52-bed pediatric cancer center in the midsouthern United States. SAMPLE: In vitro: 12 randomly selected platelet units. in vivo: convenience sample of 26 children, ages 3-20 years, with cancer and thrombocytopenia requiring platelet transfusion. METHODS: Four infusion rates studied in vitro: two infusion rates studied in vivo. MAIN RESEARCH VARIABLES: Platelet count, morphology score, corrected count increment, and patients' physical and subjective responses. FINDINGS: No clinically significant differences were found in outcomes across the four rates in vitro. Similarly, no statistically significant differences were found between the two in vivo rates on objective or subjective outcomes. CONCLUSIONS: In this setting, the more rapid infusion rate is clinically preferable because it does not negatively affect platelet recovery or patient outcomes and it decreases the infusion time by half. IMPLICATIONS FOR NURSING PRACTICE: Findings provided a basis for altering platelet infusion rates at the study setting. Benefits of the faster infusion rate include more rapid correction of thrombocytopenia, decreased time that outpatients must remain in the clinic for transfusion. Increased time available for other parenteral infusions in the impatient setting, and substantial savings in nursing time and associated costs.
An extremely aggressive malignant epithelial neoplasm of the kidney has recently been described and named renal medullary carcinoma. The finding of this tumor is highly predictive of drepanocytes (sickle cells) in tissue sections and thus the presence of sickle hemoglobin, specifically sickle cell trait, in the patient. We present a case report of this rare tumor in a 10-year-old male. The tumor displayed a variable histologic architecture including gland-like areas with intra- and extracytoplasmic material resembling mucin with hematoxylin and eosin stain. This material was negative with periodic acid-Schiff and mucicarmine stains, stained only weakly with Alcian Blue, and was positive using antibodies against peanut agglutinin. Tumor cells stained positively with antibodies to epithelial membrane antigen, cytokeratin, vimentin, and Ulex europaeus lectin. The luminal face of tumor cells stained with peanut agglutinin. Stains using antibodies against carcinoembryonic antigen and alpha-fetoprotein were negative. Ultrastructurally, the tumor cells were characterized by short microvilli lining the luminal surface and lateral complex infoldings of adjacent plasma membranes. We discuss the relationship of this neoplasm to another renal pelvic neoplasm, collecting duct carcinoma, which may rarely occur in children. Renal medullary carcinoma should be included in the differential diagnosis of gross hematuria, which is most commonly benign self-limited hematuria, in young patients with sickle cell trait.