Showing papers by "Primary Children's Hospital published in 1996"

Ultrasound guidance for placement of central venous catheters: a meta-analysis of the literature.

Abstract: ObjectiveTo evaluate the effect of real-time ultrasound guidance using a regular or Doppler ultrasound technique for placement of central venous catheters.Data SourcesWe searched for published and unpublished research using MEDLINE, citation review of relevant primary and review articles, conference

Mapping a cardiomyopathy locus to chromosome 3p22-p25

Abstract: Dilated cardiomyopathy (DCM) is a common disorder characterized by cardiac dilation and reduced systolic function. To identify a cardiomyopathy gene, we studied a family with DCM associated with sinus node dysfunction, supraventricular tachyarrhythmias, conduction delay, and stroke. A general linkage approach was used to localize the disease gene in this family. Linkage to D3S2303 was identified with a two-point lod score of 6.09 at a recombination fraction of 0.00. Haplotype analyses mapped this locus to a 30 cM region of chromosome 3p22-p25, excluding candidate genes encoding a G-protein (GNAI2), calcium channel (CACNL1A2), sodium channel (SCN5A), and inositol triphosphate receptor (ITPR1). These data indicate that a gene causing DCM associated with rhythm and conduction abnormalities is located on chromosome 3p, and represent the first step toward disease gene identification.

The utility of head computed tomographic scanning in pediatric patients with normal neurologic examination in the emergency department.

Abstract: Head injury is a frequent cause of morbidity and mortality in pediatric trauma. Guidelines for obtaining computed tomographic (CT) scans in the child with mild head injury are poorly defined. This study investigated the utility of head CT scanning in the pediatric patient presenting with normal neurologic examination. All patients undergoing head CT scanning for trauma in the emergency department (ED) at a tertiary care pediatric trauma center during 1992 were identified (508). Charts were reviewed for historical and physical examination findings, CT results, and need for neurosurgical intervention. Patients were excluded if they had an abnormal neurologic examination (179), known depressed skull fracture (11), bleeding diathesis (3), age older than 18 years (1), or developmental delay (1). Included were 313 patients (median 5.5 years) who presented with clinical variables including sleepiness (38%), vomiting (34%), headache (30%), loss of consciousness (LOC) (25%), irritability (22%), amnesia (20%), and seizures (8%). An abnormal head CT was noted in 88 cases (28%); 79 (25%) were traumatic abnormalities involving the skull and/or contents. Thirteen patients (4%) had intracranial injuries (ICI); all had either a linear (10), basilar (2), or depressed (1) skull fracture noted on CT. Four patients required neurosurgery, three for epidural hematoma, and one for a complicated orbital fracture (without ICI). No clinical variables (seizure, LOC, vomiting, headache, confusion, irritability, sleepiness, amnesia) were associated with ICI (P > 0.05). In pediatric head trauma patients, with normal neurologic examinations in the ED, ICI occurs < 5% of the time and neurosurgery is needed in 1% of the cases. Commonly used clinical variables are not associated with ICI in these children.

Outcome of cardiac transplantation in children: Survival in a contemporary multi-institutional experience

Abstract: Background Meaningful analysis of survival and risk factors for death in children who undergo heart transplantation is problematic because of the small number of heart transplantations performed at individual institutions. Methods and Results To more accurately examine survival and risk factors for death in children undergoing heart transplantation, we analyzed 191 patients between 1 and 18 years old who received transplants at 22 centers in the Pediatric Heart Transplant Study between January 1, 1993, and December 31, 1994. Cardiac diagnosis was congenital heart disease in 74 patients (39%), dilated cardiomyopathy in 73 (38%), and other in 44 (23%). Actuarial survival was 93% at 1 month, 82% at 1 year, and 81% at 2 years after transplantation. The major causes of death (n=31) were rejection (29% of deaths), early graft failure (19%), infection (16%), sudden death (13%), and other causes (23%). By multivariate analysis, risk factors for death were assist devices (P=.02), nonidentical ABO blood types (P=.05), and younger age (P=.10). Conclusions Contemporary survival for pediatric heart transplant recipients ≥1 year old is comparable to survival after adult heart transplantation. Risk factors for death are the need for assist devices, nonidentical ABO blood types, and younger age. Rejection is the most common cause of death after pediatric heart transplantation.

Acute response to inhaled nitric oxide in newborns with respiratory failure and pulmonary hypertension.

Abstract: Objective Systemic oxygenation is improved by inhaled nitric oxide therapy in some newborns with respiratory failure and pulmonary hypertension. Our results with inhaled nitric oxide were reviewed to determine factors associated with an acute improvement in systemic oxygenation. Methods Newborns with oxygenation indices of 25 to 40 were prospectively randomized to receive conventional therapy with or without 20 ppm inhaled nitric oxide. All newborns with oxygenation indices greater than 40 were treated with inhaled nitric oxide. Hemodynamic, blood gas, and Doppler ultrasound measurements were performed before and after 30 to 60 minutes of observation or therapy. The severity of lung disease was classified by the chest radiograph as: (1) normal or focal disease; (2) moderate diffuse disease-diffuse lung disease with well-defined heart borders; or (3) severe diffuse disease-diffuse lung opacification with indistinct heart borders. Results Heart rate, blood pressure, and ductal diameters did not change. Blood gases and ductal shunting acutely improved only in patients treated with inhaled nitric oxide. Patients with normal lung fields or focal disease had the greatest degree of improvement in systemic oxygenation. Changes in oxygenation were not influenced by gestational age, baseline blood gases, the proportion of right-to-left ductal shunting, prior treatment with a surfactant, or the use of conventional or high-frequency jet ventilation. Collectively, blood gases and ductal shunting did not improve with inhaled nitric oxide in patients with lung hypoplasia or severe diffuse lung disease. Sustained improvement in oxygenation occurred in 87% of patients with oxygenation indices greater than 40 in whom oxygenation indices less than 40 acutely developed after exposure to nitric oxide, whereas 90% of patients in whom oxygenation indices less than 40 did not acutely develop were treated with extracorporeal membrane oxygenation or ultimately died. Conclusions Inhaled nitric oxide acutely improves systemic oxygenation in many newborns with respiratory failure and pulmonary hypertension. The diagnosis and chest radiograph are helpful in identifying patients who will have favorable acute responses to therapy. In patients with severe hypoxemia, the need for invasive support with extracorporeal membrane oxygenation may be determined by an acute trial of inhaled nitric oxide.

Inhaled nitric oxide in children with severe lung disease: results of acute and prolonged therapy with two concentrations.

Abstract: Objectives To evaluate the acute effects of 11 and 60 parts per million (ppm) inhaled nitric oxide on the pulmonary vascular resistance and systemic oxygenation of children with severe lung disease, and to compare the outcome of prolonged therapy with approximate 10 and 40 ppm inhaled nitric oxide. Design Prospective, randomized study. Setting A 26-bed pediatric intensive care unit in a tertiary children's hospital. Patients Nineteen patients (median age 11 yrs, range 7 months to 16 yrs) with acute bilateral lung disease requiring a positive end-expiratory pressure (PEEP) of more than 6 cm H2 O and an FIO2 of more than 0.5 for more than 12 hrs were treated with inhaled nitric oxide. One patient was treated twice during the same hospitalization. Interventions Acute hemodynamic and blood gas effects of 11 and 60 ppm inhaled nitric oxide were studied, while delivering these concentrations in random order for intervals of 20 to 30 mins. Each interval was preceded by an interval of 20 to 30 mins without nitric oxide. Patients were then randomized and treated for a prolonged period with approximate 10 or 40 ppm inhaled nitric oxide independent of their initial acute responses to 11 and 60 ppm. Nitric oxide was discontinued when ventilatory support was decreased to a PEEP of less than equals 6 cm H2 O and an FIO2 of less than equals 0.5. Measurements and Main Results Inhaled nitric oxide selectively decreased pulmonary vascular resistance and improved systemic oxygenation. Acute hemodynamic and blood gas effects of 11 and 60 ppm nitric oxide were similar. Systemic oxygenation improved to a greater extent in patients with radiographic evidence of residual aerated lung regions than in patients with diffuse bilateral lung disease. Maximum methemoglobin concentrations were greater in patients treated for a prolonged period with 40 ppm nitric oxide. The mortality and duration of therapy were similar for patients treated with 10 and 40 ppm inhaled nitric oxide. Conclusions Pulmonary vascular resistance and systemic oxygenation are acutely improved to a similar extent by 11 and 60 ppm inhaled nitric oxide, and concentrations in excess of 10 ppm are probably not needed for prolonged therapy of children with severe lung disease.

Invited article: helical/spiral CT scanning: a pediatric radiology perspective.

Abstract: Helical/spiral CT technology has several potential benefits for scanning pediatric patients. These benefits include reduced sedation rates, decreased radiation exposure with scanning at extended pitch, improved image quality, and better three-dimensional and reformatted images. This paper reviews the technical and clinical considerations relevant to scanning the pediatric patient and offers suggestions for protocol development.

Immunohistochemical analysis of platelet-derived growth factor and basic fibroblast growth factor in cardiac biopsy and autopsy specimens of heart transplant patients

Abstract: The purposes of this study were to examine 250 heart biopsy specimens and 20 autopsy specimens from heart transplant patients for the presence and localization of platelet-derived growth factor (PDGF)and basic fibroblast growth factor (bFGF) and to correlate these findings with the histologic features of rejection and the autopsy findings of graft coronary vasculopathy and global ischemia. Positive specimen staining was significantly more prevalent for PDGF (78% of specimens) than for bFGF (54% of specimens) (p< 0.001). PDGF was distributed more in an interstitial (53%) than a vascular (28%) pattern and was associated with macrophages, whereas bFGF was distributed more in a vascular (50%) than an interstitial (12%) pattern. The prevalence of PDGF (but not bFGF) staining was significantly greater in biopsy specimens with at least grade 2 vascular rejection changes (81%) than in those without vascular rejection changes (58%) (p<0.001). In autopsy specimens, PDGF staining was present in the hearts of all 5 patients (100%) who died of graft failure from coronary vasculopathy and was present in all 11 hearts (100%) with global ischemic changes, but in only 4 of 9 (44%) of the hearts without global ischemia (p<0.01). PDGF staining was absent in nontransplanted heart specimens, whereas bFGF staining in nontransplanted heart specimen was similar to that in transplanted hearts. We conclude that PDGF is increased in transplanted hearts, is distributed more in an interstitial pattern, and is associated with macrophages. Furthermore, PDGF staining is increased in transplanted hearts with evidence of vascular rejection, coronary vasculopathy, or global ischemia.

Outcome of cardiac transplantation in children. Survival in a contemporary multi-institutional experience. Pediatric Heart Transplant Study.

Abstract: BACKGROUND Meaningful analysis of survival and risk factors for death in children who undergo heart transplantation is problematic because of the small number of heart transplantations performed at individual institutions. METHODS AND RESULTS To more accurately examine survival and risk factors for death in children undergoing heart transplantation, we analyzed 191 patients between 1 and 18 years old who received transplants at 22 centers in the Pediatric Heart Transplant Study between January 1, 1993, and December 31, 1994. Cardiac diagnosis was congenital heart disease in 74 patients (39%), dilated cardiomyopathy in 73 (38%), and other in 44 (23%). Actuarial survival was 93% at 1 month, 82% at 1 year, and 81% at 2 years after transplantation. The major causes of death (n = 31) were rejection (29% of deaths), early graft failure (19%), infection (16%), sudden death (13%), and other causes (23%). By multivariate analysis, risk factors for death were assist devices (P = .02), nonidentical ABO blood types (P = .05), and younger age (P = .10). CONCLUSIONS Contemporary survival for pediatric heart transplant recipients > or = 1 year old is comparable to survival after adult heart transplantation. Risk factors for death are the need for assist devices, nonidentical ABO blood types, and younger age. Rejection is the most common cause of death after pediatric heart transplantation.

The Solubility of Calcium and Phosphate in Two Specialty Amino Acid Solutions

Abstract: Background The purpose of this study was to determine precipitation limits and construct curves for calcium and phosphorus in parenteral nutrition solutions compounded with specialty amino acid solutions. Methods The effect of temperature, concentration of amino acids, and pH on the solubility of calcium and phosphate was studied for two specialized amino acid solutions: NephrAmine and HepatAmine. Ten amino acid solutions were manufactured with NephrAmine and 12 with HepatAmine at amino acid concentrations of 0.8%, 1.5%, and 2%. The final dextrose concentration was 10%. Some of the solutions were tested with the additive cysteine hydrochloride, 40 mg cysteine/g of protein, and some were buffered with sodium bicarbonate or hydrochloric acid to model the pH of a combined fat emulsion and total parenteral nutrient (TPN) solution. Calcium gluconate and potassium phosphate were added to test samples of each TPN solution. A range of calcium concentrations of 2.5 to 40 mEq/L and phosphate concentrations of 2.5 to 40 mmol/L were tested. After storage at room temperature (25 degrees C) for 18 hours, solutions were inspected for precipitation. At the end of the 18-hour period, the solutions were held in a water bath at 37 degrees C for 30 minutes and inspected for precipitation and microcrystallization. Results Solubility curves were plotted to represent Ca-PO4 solubility limits before visual or microscopic precipitation. These curves depend on the variables of time, temperature, concentration, and pH. Conclusions These data and graphs will help the clinical pharmacist estimate Ca-PO4 solubility limits and prevent precipitation of TPN solutions formulated with NephrAmine or Hepatamine.

Comparison of ossification of demineralized bone, hydroxyapatite, Gelfoam, and bone wax in cranial defect repair.

Abstract: Demineralized bone allografts in the repair of calvarial defects are compared with other common bone fillers. This study uses a video-digitizing radiographic analysis of calvarial defect ossification to determine calcification of bone defects and its relation to postoperative clinical examination and regional controls. The postoperative clinical results at 3 months demonstrated that bony healing was greatest in bur holes filled with demineralized bone and hydroxyapatite. Radiographic analysis demonstrated calcification of demineralized bone-filled defects compared to bone wax- and Gelfoam-filled regions. Hydroxyapatite granules are radiographically dense, thus not allowing accurate measurement of true bone healing. The results suggest that demineralized bone and hydroxyapatite provide better structural support via bone healing to defined calvarial defects than do Gelfoam and bone wax.

Cyclizine abuse by teenagers in Utah

Abstract: Substance abuse by teenagers is common, often involving use of alcohol and illicit drugs. Ingestion of cyclizine hydrochloride, a nonprescription medication, was noted to occur frequently in Utah for abuse reasons. A retrospective review was conducted of patients younger than 18 years of age over a 3-year period who intentionally ingested cyclizine identified from Utah Poison Control Center records. Eighty patients were included; 42 patients underwent hospital record review. Abuse accounted for 89% of cyclizine ingestions; hallucinations (70%) and confusion/disorientation (40%) were the most notable symptoms. Tachycardia (52%) and systolic hypertension (69%) were frequently present in patients who presented to a hospital. No serious complications occurred. This study illustrates teenage abuse of one nonprescription antihistamine presumably to induce hallucinations. Abuse of over-the-counter medications by adolescents may be more appealing than illicit drug use for numerous reasons, and may be more common than appreciated.

What is a juvenile polyp? An analysis based on 21 patients with solitary and multiple polyps.

Abstract: Background Juvenile polyps, the most common pediatric gastrointestinal polyp, have been typically characterized as either hamartomatous overgrowths or reactive inflammatory proliferations. Recent observations of excessive colonic and gastric carcinoma and dysplasia in juvenile polyposis have prompted reclassification of this entity as a premalignant condition. The relationship between solitary or multiple juvenile polyps and malignancy is less clear. Patients and methods To further investigate the frequency and significance of dysplasia in juvenile polyps, we analyzed 28 polyps from 21 patients histologically and immunohistochemically for substances previously associated with neoplastic transformation in the colorectal adenomacarcinoma sequence. Results Fifteen patients had a solitary polyp, two had 2 to 9 polyps, and four had polyposis with 10 or more polyps. Most polyps exhibited inflammatory or regenerative atypia. Foci of dysplasia were noted in polyps from 11 patients, and immunoreactivity for p53 and human chorionic gonadotropin was present in 12 of the 28 polyps each. These findings were all more frequent in the polyposis specimens than in solitary polyps. Conclusions These observations, in combination with reports of an increased risk of carcinoma in juvenile polyposis, suggest that juvenile polyps are lesions with a potential for neoplastic and malignant transformation, although they share features of an inflammatory reactive process. The implications for clinical management of patients and pathologic evaluation of juvenile polyps warrant further investigation.

Colorectal adenocarcinoma as a second malignant neoplasm following Wilms' tumor and rhabdomyosarcoma.

Abstract: Colorectal carcinoma is one of the most common primary malignancies in adults and occurs in older patients after pelvic radiation. It is rare in children and young adults. We report two cases of colonic adenocarcinoma which were second malignant neoplasms following treatment for early childhood malignancies. One child had Wilms' tumor at 9 months of age treated with preoperative radiation and surgery. He developed radiation colitis and multifocal intestinal adenocarcinomas 42 years later and died with abdominal carcinomatosis. The second child had retroperitoneal embryonal rhabdomyosarcoma at age 1 year and was treated with preoperative radiation, surgery, and chemotherapy. At age 2 years he had radiation colitis; at age 11 years he had rectal adenocarcinoma associated with adenomatous polyps, focal adenomatous change and radiation colitis. Immunohistochemical studies revealed p53 positivity in both adenocarcinomas and in adenomas from the second patient, suggesting that p53 mutation was involved in carcinogenesis. The history of high-dose radiation in early childhood and the multifocal lesions suggest the adenocarcinomas in both patients were second malignant neoplasms, with associated reactive and benign neoplastic and premalignant lesions well documented in one case. These two cases document the phenomenon of early onset of adult type tumors in survivors of childhood cancer and emphasize the need for continued clinical evaluation of patients at risk for second malignant neoplasms.

Fatal Air Bag Mediated Craniocervical Trauma in a Child

Abstract: This case report describes a 5-year-old child who sustained fatal craniocervical injuries as the result of an inflating automobile air bag. Although air bags have in general been shown to reduce the severity of injury to occupants of vehicles involved in front-end collisions, a growing number of incidents suggests that in some instances the air bag system itself may cause injuries, some of which can be serious or even fatal. Small children and infants in infant seats travelling in the front passenger seat appear to be particularly at risk. The increasing number of reports of air bag mediated injuries highlights the need for changes in both system design and possibly the threshold speed required for air bag deployment.

Frontonasal malformation and the oculoauriculovertebral spectrum: the oculoauriculofrontonasal syndrome.

Abstract: Frontonasal malformation (FNM) is a developmental field defect representing abnormal morphogenesis of the frontonasal eminence. The oculoauriculovertebral spectrum (OAVS) has been used to describe a broader range of first and second branchial-arch defects including hemifacial microsomia and Goldenhar's syndrome. A combination of FNM and OAVS has been described in the literature in 13 cases. This condition has been labeled as the oculoauriculofrontonasal syndrome, as well as ophthalmofrontonasal dysplasia. We have evaluated four patients with both FNM and OAVS. The pattern of malformation involves only the craniofacies: they have no vertebral defects, heart disease, or encephaloceles. The categorization of these four individuals and those in the literature raises interesting issues regarding syndrome classification. Originally, it was suggested that perhaps this disorder was a variation of Goldenhar's syndrome. However, now that it has become evident that FNM and OAVS are malformation patterns of ...

A new approach to reproducibility assessment : Clinical evaluation of SpaceLabs Medical oscillometric blood pressure monitor

Abstract: The SpaceLabs Medical oscillometric blood pressure monitor was evaluated in both neonatal and adult populations according to the 1992 AAMI SP-10 standard for electronic or automated sphygmomanometers. For the neonatal study, the umbilical artery pressure was used as a reference. Fifteen newborn ICU patients were enrolled in the study, yielding a total of 150 pairs of comparison readings. For the adult study, the first and fifth phases of the Korotkoff sounds were used as a reference. One hundred twelve adults were enrolled in the study, yielding a total of 336 pairs of comparison readings. Accuracy was analyzed by the AAMI SP-10 standard. For systolic and diastolic pressures, respectively, the mean differences +/- standard deviations of oscillometric accuracy were 0.1 +/- 4.3 and 2.7 +/- 4.8 mmHg for the neonatal study and -0.6 +/- 5.9 and 0.9 +/- 6.4 mmHg for the adult study, which are within the AAMI SP-10 accuracy standard. Reproducibility was analyzed by a new approach developed from the sequential technique of the 1990 British Hypertension Society protocol. For systolic and diastolic pressures, respectively, the mean differences +/- standard deviations of the oscillometric reproducibility were estimated to be -0.1 +/- 3.3 and 0.1 +/- 3.4 mmHg for the neonatal study and -0.3 +/- 3.2 and 0.1 +/- 3.5 mmHg for the adult study.

Spinal stenosis after total lumbar laminectomy for selective dorsal rhizotomy

Abstract: Knowledge of long-term outcome and complications of selective dorsal rhizotomy is limited due to the relatively recent introduction of the procedure We describe 2 patients with cerebral palsy who developed lumbar spinal stenosis several years after selective dorsal rhizotomy These patients also had substantial lateral trunk sway during gait and walked for several years with limited assistive devices This abnormal gait pattern in combination with the changes from the selective dorsal rhizotomy may lead to the development of spinal stenosis

Cuff bulk of tracheal tubes in adolescence

Abstract: Purpose To investigate cuff types for potential use in late childhood and early adolescence for appropriate fit.

Sinus node dysfunction in tuberous sclerosis

Abstract: Tuberous sclerosis is an autosomal dominant disease with potential for involvement of the skin, brain, lungs, kidneys, and heart. A case of prolonged sinus node pause and episodes of unresponsiveness in a 21-week-old infant with tuberous sclerosis is described. The infant subsequently underwent placement of an epicardial pacemaker. This case illustrates the importance of electrocar-diographic studies in infants with tuberous sclerosis and seizure-like episodes.

Doppler systemic venous flow patterns: Changes in children with mild/moderate pulmonic stenosis

Abstract: To determine the usefulness of systemic venous flow patterns in patients with mild/moderate right ventricular hypertension, 17 patients with isolated mild/moderate pulmonic stenosis and 17 age-matched normal children were evaluated with pulsed Doppler echocardiography. Tricuspid valve, superior vena caval, and hepatic vein pulsed Doppler recordings were obtained with simultaneous respirometry and electrocardiography. Peak velocities and velocity-time integrals were measured for Doppler signals corresponding with ventricular systole, ventricular diastole, ventricular end systole, and atrial systole. The groups were similar in weight, heart rate, tricuspid inflow Doppler echocardiograms, and cardiac indexes. Compared with normal subjects, patients showed changes in respiratory variation for some superior vena caval and hepatic vein indexes. In addition, hepatic vein measurements made at ventricular end systole were significantly lower and measurements made at atrial systole were significantly higher in patients than in normal subjects. These changes in systemic venous flow patterns may provide a sensitive indicator of early right-sided heart dysfunction.

Etiology and Outcome of Outpatient Fevers in Pediatric Heart Transplant Patients

Abstract: We reviewed 74 outpatient febrile episodes in 22 pediatric heart transplant patients in order to determine etiologies, rates of serious and nonserious illness, and factors predictive of serious disease. Twenty-two febrile episodes (30%) resulted in hospital admission. Only three variables were predictive of serious illness: longer duration of fever, shorter time since transplant, and lower febrile episode number. We conclude that at least 70% of outpatient febrile episodes are nonserious and can be managed safely in an outpatient setting. The duration of fever may be predictive of serious disease but is not useful at initial presentation.

Intracardiac yolk sac tumor : echocardiographic evaluation

Abstract: To our knowledge, this case of an abdominal yolk sac tumor with extensive cardiac involvement is the first to be reported in a pediatric patient. The report describes the echocardiographic findings of the tumor and the role of echocardiography in providing important information for patient management decisions.