Overt testicular disease at diagnosis of childhood acute lymphoblastic leukemia: lack of therapeutic role of local irradiation.

To assess the prognosis of overt testicular disease at diagnosis of acute lymphoblastic leukemia, and any therapeutic role of irradiation for this involvement, we reviewed the data of 811 boys treated on St Jude studies Total X--XI (early period) and Total XII-XIV (recent period). In all, 19 boys (2.3%) had testicular disease at diagnosis. In the early period, patients with testicular leukemia had a poorer overall survival (OS) (P=0.003), event-free survival (EFS) (P=0.064), and higher cumulative incidence of relapse (P=0.041) than did other patients. During the recent period, patients with and without overt testicular leukemia did not differ in OS (P=0.257), EFS (P=0.102), or cumulative incidence of relapse (P=0.51). In a multivariate analysis, OS was lower for patients with testicular disease than for those without the involvement in the early period (P=0.047) but not in the recent one (P=0.75). Both patients who received irradiation for residual testicular disease at the end of induction subsequently died of leukemia. Of the other 17 patients who did not receive irradiation, only one developed testicular relapse in combination with bone marrow relapse. In conclusion, the prognostic impact of overt testicular disease has diminished. Irradiation appears to provide no survival advantage to this patient population.

Hepatic artery thrombosis in pediatric liver transplantation.

PURPOSE: Children have been reported to be at greater risk for hepatic artery thrombosis when compared to adults due to small arterial size, nonuse of intraoperative microscope, and postoperative hypercoagulable state. METHODS: We evaluated arterial anastomosis type, intraoperative field magnification, and hepatic artery complications and how they were managed. All patients underwent ultrasound, anticoagulation consisted of 41 mg aspirin once a day, and 35 patients received alprostadil (PGE) for the first 7 days after transplantation. No patients were administered intravenous heparin following liver transplantation. RESULTS: Of the 74 livers transplanted, 36 grafts (48.6%) were whole organ transplants and 38 grafts (51.4%) were partial livers. We observed HAT in 1 of 74 (1.35%) transplants in our pediatric liver transplant population. The only patient with HAT was a young girl with a history of biliary atresia. The occurrence of a hepatic artery thrombosis on day 7 was caused by the migration of an intimal plaque dissection within the artery graft. She was emergently taken back into the operating room for graft revision. This individual currently has a survival time of 426 days following her last transplant. CONCLUSIONS: Hepatic artery thrombosis may be minimized in pediatric liver transplantation without the use of microsurgery. Anticoagulation utilizing ASA and alprostadil is sufficient to avoid HAT. Accurate use of ultrasound is crucial to avoid this complication. Graft and patient salvage is possible with expedient surgical treatment; microsurgery, anticoagulant therapy, site of arterial inflow, and recipient size and weight.

Biliary complications after pediatric liver transplantation revisited.

BACKGROUND: Biliary complications in pediatric liver transplantation (PLT) are associated with increased morbidity and mortality. METHODS: Prospectively, data was collected on 89 consecutive liver transplants performed in 82 children. Eighty-nine consecutive PLTs were tracked for transplant type (partial versus whole), recipient age/weight, duct anastomosis type, surgical technique, and biliary complications. Treatments of biliary complications (surgical versus interventional radiology) were also evaluated. RESULTS: Forty-six children (51.7%) received partial transplants and 43 (48.3%) children received whole organs. The average age for whole liver transplanted children was 8.95 +/- 6.62 years and average weight was 36.2 +/- 28.7 kg; for those receiving partial livers, 3.19 +/- 3.52 years and 14.1 +/- 13.0 kg. Duct-to-duct anastomosis was performed for 26 grafts and Roux-en-Y choledochojejunostomy for 63 grafts. Biliary complications occurred in 10 of 89 (11.2%) grafts. Complications included anastomotic strictures in four (40%), bile leak in five (50%), intraparenchymal biloma in one (10%). The complication rate for whole organs was 1/43 (2.3%) and 9/46 (19.6%) for partial organ (P =. 015). No difference in complication rates were seen in type of ductal anastomosis (7.7% vs 12.7%, P = NS). Reoperation for biliary complication was necessary in only 2/10 (20%) of grafts. CONCLUSIONS: Technical advances have reduced the incidence of biliary complications in PLT. Partial liver grafts have a statistically higher risk of biliary complication than whole grafts. Most biliary complications can be managed with radiological intervention without surgical exploration. Pediatric biliary complications are not associated with graft loss.

Early experience with the bifurcated Excluder endoprosthesis for treatment of the abdominal aortic aneurysm.

PURPOSE: This report describes our initial experience with the modular, bifurcated Excluder endoprosthesis and its safety and efficacy in the primary endovascular repair of infrarenal abdominal aortic aneurysms (AAAs). METHODS: AAAs (mean diameter, 58.2 +/- 14.3 mm) were repaired in 19 patients with this device between March 1999 and January 2000. The mean age of patients was 71.8 +/- 8.4 years (range, 57-86 years). This modular device was inserted through an 18F introducer sheath placed in one femoral artery, and the contralateral artery was cannulated with a 12F introducer sheath. All procedures were performed in a standard operating room with angiographic capabilities. RESULTS: Endograft deployment was successful in all patients. The average surgical time was 135 +/- 37 minutes, with a mean blood loss of 229 +/- 138 mL. In eight patients, the use of either aortic or iliac extenders was required for enhanced sealing or additional length. An external iliac artery dissection occurring at the time of endograft insertion was successfully treated with a Wallstent. Type II leaks initially found to be present by means of intraoperative completion angiography had spontaneously thrombosed by the 1-month follow-up computed tomography scan. There was one perioperative death (5.3%). Complications included superficial wound infections (n = 3) and a nonfatal myocardial infarction (n = 1). The mean length of hospital stay was 2.9 +/- 1.2 days, and only six patients required intensive care. Endoleaks were seen in four patients (21%) by means of the 30-day computed tomography scan; three of these endoleaks had spontaneously sealed at the time of the 6-month follow-up examination (5.5% 6-month endoleak rate). Aneurysm size did not increase in the patients with leaks. CONCLUSION: The Excluder endoprosthesis was an effective means of excluding an infrarenal AAA from the systemic circulation in this selected group of patients. The smaller sheath sizes may increase the pool of potential candidates. Further study of this device is warranted, and continued assessment of the long-term durability of the device will be necessary.

H reflex and F wave latencies to soleus normal values and side-to-side differences.

OBJECTIVE: Electromyographers must reliably differentiate between H reflexes and F waves when recording from the soleus muscle in the evaluation of S1 radiculopathy. The use of F waves in root-level injuries is questioned, whereas H reflexes have shown value in the evaluation of S1 radiculopathy. We studied the relationship between the tibial H reflex and F wave latencies in the limbs of 40 subjects. DESIGN: After recording the H wave latency, we changed the gain to 200 microV/cm and increased the stimulation to supramaximal for ten additional responses without moving the recording or stimulating electrodes. We also calculated the predicted H wave latency with the standard formula. Forty subjects, mean age 32 yr, consented and participated. RESULTS: The mean of the average F wave was 1.76 ms longer than the ipsilateral H reflex latency. The mean side-to-side difference of the average F wave was 0.56 ms. The H reflex latency side-to-side difference was 0.36 ms. CONCLUSION: The findings suggest that the average F wave latencies have a predictive value in the clinical context similar to the H reflex.

Persistent activation of mitogen-activated protein kinases p42 and p44 and ets-2 phosphorylation in response to colony-stimulating factor 1/c-fms signaling.

An antibody that specifically recognized phosphothreonine 72 in ets-2 was used to determine the phosphorylation status of endogenous ets-2 in response to colony-stimulating factor 1 (CSF-1)/c-fms signaling. Phosphorylation of ets-2 was detected in primary macrophages, cells that normally express c-fms, and in fibroblasts engineered to express human c-fms. In the former cells, ets-2 was a CSF-1 immediate-early response gene, and phosphorylated ets-2 was detected after 2 to 4 h, coincident with expression of ets-2 protein. In fibroblasts, ets-2 was constitutively expressed and rapidly became phosphorylated in response to CSF-1. In both cell systems, ets-2 phosphorylation was persistent, with maximal phosphorylation detected 8 to 24 h after CSF-1 stimulation, and was correlated with activation of the CSF-1 target urokinase plasminogen activator (uPA) gene. Kinase assays that used recombinant ets-2 protein as a substrate demonstrated that mitogen-activated protein (MAP) kinases p42 and p44 were constitutively activated in both cell types in response to CSF-1. Immune depletion experiments and the use of the MAP kinase kinase inhibitor PD98059 indicate that these two MAP kinases are the major ets-2 kinases activated in response to CSF-1/c-fms signaling. In the macrophage cell line RAW264, conditional expression of raf kinase induced ets-2 expression and phosphorylation, as well as uPA mRNA expression. Transient assays mapped ets/AP-1 response elements as critical for basal and CSF-1-stimulated uPA reporter gene activity. These results indicate that persistent activation of the raf/MAP kinase pathway by CSF-1 is necessary for both ets-2 expression and posttranslational activation in macrophages.

Percutaneous varicocele embolization versus surgical ligation for the treatment of infertility: changes in seminal parameters and pregnancy outcomes.

PURPOSE: To compare the success of percutaneous varicocele embolization to surgical ligation with regard to changes in semen characteristics and pregnancy outcome. MATERIALS AND METHODS: Infertility records from 346 men who underwent correction of their varicocele for infertility (surgical ligation 149; embolization 197) were reviewed retrospectively. Preprocedural and postprocedural semen analyses and pregnancy outcomes were obtained with use of chart and telephone follow-up. RESULTS: In men who successfully impregnated their partners, there were significant improvements in sperm density, percent total improvement, motility, and progression. Postprocedural (embolization vs surgery) percentage increases in seminal parameters were density, 156.8% versus 138.5%; total, 168.8% versus 157.91%; and motility, 2.7% versus 3.2%. The percent of individuals who had a change in sperm progression was 31% versus 41%. There was no statistical difference between the techniques based on t tests. The pregnancy rates were similar for the two groups, 39% and 34% for embolization and surgery, respectively. CONCLUSION: There is no significant statistical difference in seminal values or pregnancy outcome between the two techniques.

Pediatric enteral access center: a multidisciplinary approach.

The need for enteral access and gastrostomy placement in children has increased dramatically over the past several years. In the following article, we present a case report of a typical patient undergoing evaluation for enteral nutrition and enteral access. Following the case report, we describe our newly formed Pediatric Enteral Access Program including patient selection, the method of gastrostomy placement, risks and benefits, cost analysis, and follow-up of this ever-increasing population of children.

Orthotopic liver transplantation for inflammatory myofibroblastic tumor of the liver hilum.

Inflammatory myofibroblastic tumor is a rare gastrointestinal neoplasm. The authors report the case of a 5-year-old girl who initially presented with gastric outlet obstruction secondary to an inflammatory myofibroblastic tumor along the lesser gastric curvature. A subtotal gastrectomy and wedge resection of a left hepatic lobe nodule were performed. Obstructive jaundice developed one month postoperatively. Computerized tomography, percutaneous transhepatic cholangiography, and selective celiac and mesenteric arteriography showed a mass that involved the left hepatic lobe, with concomitant high-grade biliary obstruction and portal venous encasement. Percutaneous biliary drainage was performed. During laparotomy, the tumor was found to be unresectable, and the patient was referred to the Liver Transplant Service at The Children's Hospital of Philadelphia. A total hepatectomy with temporary portocaval shunt was performed, leaving the inferior vena cava in situ. At the back table, an ex vivo left trisegmentectomy was performed, followed by reimplantation of the posterior segment of the right hepatic lobe. The reimplanted liver segment functioned poorly, and completion hepatectomy with portocaval shunt was performed 24 hours postoperatively, because of severe coagulopathy, intraabdominal bleeding, and hemodynamic instability. The patient's condition stabilized, and she was listed for urgent liver transplantation. The anhepatic state was managed with intermittent plasmapheresis. She had transplantation 72 hours later, and was discharged from the hospital 3 weeks postoperatively after an uneventful recovery. She remains disease-free and has normal liver function 8 months after transplantation.

Transjugular intrahepatic portosystemic shunt-related arterial injuries: prospective comparison of large- and small-gauge needle systems.

PURPOSE: The authors prospectively compared the nature and incidence of hepatic arterial injuries resulting from creation of a transjugular intrahepatic portosystemic shunt (TIPS) with large- and small-gauge needle systems. PATIENTS AND METHODS: Fifty patients underwent hepatic and superior mesenteric angiography immediately before and after shunt creation. A sheathed 16-gauge needle system was used to locate and puncture the portal vein in 24 patients. A 21-gauge needle system was used in 26 patients. RESULTS: Shunts were successfully created in all patients. Three inadvertent hepatic arterial punctures were recognized during shunt placement, two with the small needle and one with the large needle system. No hepatic arterial lesions were detected in any patient. Two incidental hepatomas were identified at angiography. CONCLUSION: TIPS-related hepatic arterial injuries are rare. In this series, large and small needle systems were indistinguishable with respect to this complication.

Intentional reversible thrombosis of transjugular intrahepatic portosystemic shunts.

PURPOSE: To assess whether balloon occlusion of a transjugular intrahepatic portosystemic shunt (TIPS) will allow permanent yet reversible shunt thrombosis. MATERIALS AND METHODS: A balloon catheter was inflated in the midportion of the TIPS in two women with severe, uncontrollable encephalopathy or liver failure (aged 42 and 65 years, respectively) to allow occlusive thrombus to develop below the balloon. RESULTS: Balloon occlusion led to rapid TIPS thrombosis, which was readily reversible. CONCLUSION: Balloon thrombosis is a simple technique for complete occlusion of a TIPS. This technique may also be useful for occlusion of surgical mesocaval H-graft shunts or dialysis access shunts.

Raman spectroscopy of normal and diseased human breast tissues.

Raman spectra of histologically normal human breast biopsy samples were compared to those exhibiting infiltrating ductal carcinoma (IDC) or fibrocystic change. Experiments at 784 nm with CCD detectors reduced fluorescence interference and produced high SNR spectra with relatively low (10-200 mW) laser power. Sample to sample and patient to patient variation for normal specimens were less than 5% for the ratios of major Raman bands. The Raman spectra changed dramatically in diseased specimens, with much weaker lipid bands being evident. The spectrum of infiltrating ductal carcinoma samples is similar to that of human collagen. Differences between benign (fibrocystic) and malignant (IDC) lesions were smaller than those between normal and IDC specimens, but were still reproducible. Fiberoptic sampling through a hypodermic needle and with a remote probe were demonstrated. The possibility of rapid diagnosis with Raman spectroscopy is considered.

A 2D crossover-based map of the human X chromosome as a model for map integration.

We have constructed a two-dimensional map of 243 markers on the X chromosome. The average distance between markers ordered by two recombinants is 5.4 centiMorgans (cM), which is reduced to 3.2 cM using a less stringent criterion of one recombinant. Map resolution is enhanced by replacing the usual reference marker format with a 2D format, and the two-recombinant rule is more conservative than the lod 3.0 criterion for order. Taken together, crossover mapping and the 2D format produces maps with greater reliability and higher resolution than maps constructed using currently accepted standards. This first high-density crossover-based map of an entire human chromosome provides a model for integrating physical and genetic maps.

Characterization of human breast biopsy specimens with near-IR Raman spectroscopy.

Breast biopsy samples were examined with Raman spectroscopy with laser wavelengths ranging from 406 to 830 nm. A combination of a single-stage spectrograph, band reject filter, and CCD detector permitted low laser powers and minimal risk of sample radiation damage. Spectra of formalin-fixed human tissue revealed Raman features for lipids and carotenoids. The best defined lipid features were observed for 782- and 830-nm laser excitation, while carotenoid features were strongest in the 488-515-nm range due to resonance enhancement. Comparison of the spectra with those of fatty acid esters revealed that the major lipid component is a derivative of oleic acid. Lipid and carotenoid Raman bands were superimposed on a luminescent background which was less prominent at longer laser wavelengths. A compact, portable, diode laser spectrometer was tested in a clinical setting with fiber optic sampling. The results indicate that substantial biochemical information is available from near-IR Raman spectroscopy and the technique may have clinical applications.

Laparoscopic cholecystectomy technique for removal of the gallbladder from the peritoneal cavity.

A new laparoscopic cholecystectomy technique for removing the gallbladder from the peritoneal cavity through the umbilical incision is presented. This method is faster than the traditional technique and eliminates the necessity of transferring the camera from the umbilical port to a second port.

Young Investigator Award. Raman spectroscopy of human atherosclerotic plaque: implications for laser angioplasty.

Raman spectroscopy is a specialized technique that permits highly specific identification of specimens, in contrast to fluorescence spectroscopy with which analysis of arterial tissues generates spectra that are broad and featureless, with little difference seen between normal artery and atheroma. Various plaque types and the contributions of different arterial fluorophores were studied to determine if Raman spectroscopy could function as a potential guidance modality for laser angioplasty. Arterial specimens obtained at atherectomy and post mortem were studied in air and while immersed in blood. One hundred fifty-six Raman spectra were collected from arterial specimens and chromatographic samples of collagen, elastin, cholesterol, beta-carotene, and L-tryptophan. Analysis showed both fatty and fibrous atherosclerotic plaques to have characteristic spectral peaks at 1,002, 1,154, and 1,516 cm-1, while the Raman spectrum of normal vessel was featureless. Spectral peaks of beta-carotene were nearly identical to those of fatty plaque. The arterial fluorophores collagen, elastin, cholesterol, and L-tryptophan were non-contributory. The Raman spectrum of fatty plaque immersed in a blood field was also detectable, suggesting that this technique may be useful for in vivo plaque recognition.