Pediatric interventional radiology workforce survey summary.
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Humans↗
Biomedical subjects
Robin Kaye
Publications and source records attributed to Robin Kaye.
Successful transduction of liver in hemophilia by AAV-Factor IX and limitations imposed by the host immune response.
We have previously shown that a single portal vein infusion of a recombinant adeno-associated viral vector (rAAV) expressing canine Factor IX (F.IX) resulted in long-term expression of therapeutic levels of F.IX in dogs with severe hemophilia B. We carried out a phase 1/2 dose-escalation clinical study to extend this approach to humans with severe hemophilia B. rAAV-2 vector expressing human F.IX was infused through the hepatic artery into seven subjects. The data show that: (i) vector infusion at doses up to 2 x 10(12) vg/kg was not associated with acute or long-lasting toxicity; (ii) therapeutic levels of F.IX were achieved at the highest dose tested; (iii) duration of expression at therapeutic levels was limited to a period of approximately 8 weeks; (iv) a gradual decline in F.IX was accompanied by a transient asymptomatic elevation of liver transaminases that resolved without treatment. Further studies suggested that destruction of transduced hepatocytes by cell-mediated immunity targeting antigens of the AAV capsid caused both the decline in F.IX and the transient transaminitis. We conclude that rAAV-2 vectors can transduce human hepatocytes in vivo to result in therapeutically relevant levels of F.IX, but that future studies in humans may require immunomodulation to achieve long-term expression.
Adult↗
May-Thurner syndrome (iliac vein compression) and thrombosis in adolescents.
May-Thurner syndrome refers to anatomic compression of the left iliofemoral vein by the overriding right iliac artery. We report three adolescents who presented to our pediatric hospital with iliac vein thrombosis and were diagnosed with May-Thurner syndrome. Each received catheter-directed thrombolysis followed by balloon angioplasty to restore flow. Two patients had endovascular stents placed. The procedures were well tolerated, without major complications. Additional thrombophilic risk factors were identified in each patient. Though uncommon, pediatric hematologists should consider May-Thurner syndrome in adolescents who present with a left lower extremity thrombosis. Aggressive therapy may be warranted due to the risk of post-thrombotic syndrome.
Adolescent↗
Botulinum toxin type a injections to salivary glands: combination with single event multilevel chemoneurolysis in 2 children with severe spastic quadriplegic cerebral palsy.
We describe 2 children with severe spastic quadriplegic cerebral palsy (CP) who have significant drooling and frequent aspiration pneumonia. They underwent simultaneous botulinum toxin type A (BTX-A) injections to salivary glands for drooling and prevention of aspiration pneumonia along with single-event multilevel chemoneurolysis (SEMLC) with BTX-A and 5% phenol for severe diffuse spasticity. There was significant improvement in drooling, frequency of aspiration pneumonia, and spasticity without adverse effect. BTX-A injections into the salivary glands, in addition to SEMLC, for these 2 children with medically complicated severe spastic quadriplegic CP, were safe and highly successful procedures, which improved their health-related quality of life.
Botulinum Toxins, Type A↗
Validation of the bispectral index monitor for measuring the depth of sedation in children.
The Bispectral Index (BIS) is an empirically calibrated number derived from adult electroencephalograph data that correlates with the depth of sedation in adults. We tested the hypothesis that the BIS score is a valid measure of the depth of pediatric sedation in a study designed to avoid limitations of a previously published report. BIS values from 96 healthy ASA physical status I-II children aged 1-12 yr undergoing sedation were continually recorded and electronically transferred to a computer. Two independent observers blinded as to BIS score evaluated sedation using the Observer's Assessment of Alertness/Sedation (OAA/S) and the University of Michigan Sedation Scale (UMSS) at 3-5 min intervals. There was a significant correlation between BIS and UMSS and between BIS and OAA/S by both the Spearman's rank correlation test and by prediction probability (P < 0.001). In children <6 yr, there was a significant correlation between BIS and the clinical sedation scores for subgroups undergoing invasive and noninvasive procedures (P < 0.001). There was also good agreement between the 2 independent observers who assessed clinical sedation scores (kappa = 0.51, P < 0.001). We conclude that the BIS monitor is a quantitative, nondisruptive and easy to use depth of sedation monitor in children.
Child↗
A 14-month-old boy with hematochezia.
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Aneurysm↗
Knee pain in a 12-year-old girl.
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Child↗
Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation.
Infants with congenital hyperinsulinism often require pancreatectomy. Recessive mutations of the ATP-dependent plasma membrane potassium channel (K(ATP)) genes, SUR1 and K(ir)6.2, cause diffuse hyperinsulinism. K(ATP) channel mutations can also cause focal disease through loss of heterozygosity for maternal 11p, resulting in expression of a paternal mutation. This study evaluated whether focal vs. diffuse hyperinsulinism could be diagnosed by acute insulin response (AIR) tests and whether arterial calcium stimulation/venous sampling (ASVS) could localize focal lesions. Fifty infants with diazoxide-unresponsive hyperinsulinism were studied. Focal lesions occurred in 70% of the cases. Positive AIR calcium occurred in 17 of 30 focal and 10 of 13 diffuse cases (P < 0.04). Positive AIR tolbutamide occurred in 27 of 30 focal vs. seven of 13 diffuse cases (P < 0.02); K(ATP) channel mutations were identified in four of the latter. ASVS localized the lesion in 24 of 33 focal cases (73%) but correctly diagnosed diffuse disease in only four of 13 cases. These results indicate that preoperative AIR tests do not distinguish focal vs. diffuse disease because some K(ATP) channel mutations retain responsiveness to tolbutamide. The ASVS test can be used to localize focal lesions in infants. The combination of ASVS, careful intraoperative histologic analysis, and surgical expertise succeeded in correcting hypoglycemia in 86% of the infants with focal hyperinsulinism.
ATP-Binding Cassette Transporters↗
Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children.
Surgical management of congenital hyperinsulinism is improved by accurate localization of small, focal dysregulated pancreatic lesions using the arterial stimulation and venous sampling (ASVS) test, which can demonstrate increased hepatic venous insulin concentrations after selective arterial injections of calcium. However, anesthesia-related increases in blood glucose can induce insulin secretion, making it difficult to interpret ASVS test data. In this retrospective study, we examined the effect of anesthetic interventions on blood glucose concentrations in 68 children undergoing ASVS testing. We considered only the glucose concentrations observed before calcium stimulation in the final analysis. The choice of drugs for induction (sevoflurane, propofol, or thiopentone), maintenance inhaled anesthetics (sevoflurane, desflurane, or isoflurane), and the use of caudal epidural bupivacaine were not associated with significant differences in the mean blood glucose concentration before ASVS. However, patients receiving remifentanil infusions had smaller mean glucose concentrations (80 +/- 18 versus 100 +/- 44 mg x dl(-1), P = 0.01). These concentrations were also significantly smaller if tracheal intubation was delayed for at least 10 min after induction while patients received inhaled anesthetics via a face mask along with remifentanil infusions (79 +/- 14 for delayed intubation versus 95 +/- 39 mg x dl(-1) for early intubation, respectively, P = 0.03). The percentage increase in glucose concentrations from preintubation values was significantly smaller in these subjects (3.7% +/- 21.9% for delayed intubation versus 31.7% +/- 60.4% for early intubation, P = 0.02). We conclude that the anesthetic management protocol for these patients should include the use of remifentanil infusions and the administration of inhaled anesthetics and remifentanil infusions for a minimum of 10 min to establish a deep plane of anesthesia before tracheal intubation.
Adolescent↗
Gastrostomy tubes: facts, fallacies, fistulas, and false tracts.
The first successful gastrostomy was performed 130 years ago. Although it is difficult to ascertain the exact number of gastrostomy or jejunostomy tubes placed annually in the United States, it is estimated approximately 230,000 gastrostomy procedures were performed in U.S. hospitals in 2001. Of these, 11,000 were performed in children. Despite the many years gastrostomy tubes have been in use, they are not free from complications. The purpose of this article is to review the authors' experience with pediatric patients who have undergone radiographic placement of gastrostomy or jejunostomy tubes.
Enteral Nutrition↗