Screening for leukemia- and clone-specific markers at birth in children with T-cell precursor ALL suggests a predominantly postnatal origin

Childhood T-cell precursor acute lymphoblastic leukemia (TCP ALL) is an aggressive disease with a presumably short latency that differs in many biologic respects from B-cell precursor (BCP) ALL. We therefore addressed the issue of in utero origin of this particular type of leukemia by tracing oncogenic mutations and clone-specific molecular markers back to birth. These markers included various first- and second-hit genetic alterations (TCRD-LMO2 breakpoint regions, n = 2; TAL1 deletions, n = 3; Notch1 mutations, n = 1) and nononcogenic T-cell receptor rearrangements (n = 13) that were derived from leukemias of 16 children who were 1.5 to 11.2 years old at diagnosis of leukemia. Despite highly sensitive polymerase chain reaction (PCR) approaches (1 cell with a specific marker among 100,000 normal cells), we identified the leukemic clone in the neonatal blood spots in only 1 young child. These data suggest that in contrast to BCP ALL most TCP ALL cases are initiated after birth.     

Comparison with computed tomography of two ultrasound devices for diagnosis of abdominal aortic aneurysm

Screening for abdominal aortic aneurysms (AAAs) in patients at risk will become more cost effective if a simple, inexpensive, and reliable ultrasound device is available. The aim of this study was to compare a 2-dimensional, handheld ultrasound device and a newly developed ultrasound volume scanner (based on bladder scan technology) with computed tomography (CT) for diagnosing AAA. A total of 146 patients (mean age 69 +/- 10 years; 127 men) were screened for the presence of AAAs (diameter >3 cm) using CT. All patients were examined with the handheld ultrasound device and the volume scanner. Maximal diameters and volumes were used for the analyses. AAAs were diagnosed by CT in 116 patients (80%). The absolute difference of aortic diameter between ultrasound and CT was <5 mm in 88% of patients. Limits of agreement between ultrasound and CT (-6.6 to 9.4 mm) exceeded the limits of clinical acceptability (+/-5 mm). An excellent correlation between ultrasound and CT was observed (r = 0.98). The correlation coefficient between the volume scanner and CT was 0.86, with agreement of 90% and kappa value of 0.73. Using an optimal cut-off value of >56 ml, defined by receiver-operating characteristic curve analysis, sensitivity, specificity, and the positive and negative predictive values of the volume scanner for detecting AAA were 90%, 90%, 97%, and 71%, respectively. In conclusion, this study shows that a 2-dimensional, handheld ultrasound device and a newly developed ultrasound volume scanner can effectively identify patients with AAAs confirmed by CT.     

Unique features of prune belly syndrome in laparoscopic surgery

BACKGROUND: The aim of this study was to evaluate the laparoscopic abdominal access modifications in children with prune belly syndrome undergoing a first stage Fowler-Stephens procedure. STUDY DESIGN: Eleven consecutive boys underwent a transperitoneal laparoscopic bilateral first stage Fowler-Stephens procedure. Patient age ranged from 1.5 to 3 years (mean age 2.2 years). In these patients, the floppy abdominal wall required a modified approach with regard to access technique, insufflation pressures, and work port stabilization methods. Duration of the procedures and intraoperative technical challenges encountered were prospectively documented. RESULTS: Mean operative time was 40 minutes (range 30 to 75 minutes), and all procedures were completed without any complications. Forceful insertion of ports was not possible, and all ports were introduced under complete open access. Larger volumes of carbon dioxide were used in the initial part of our series, when the ports were not sutured to the abdominal wall. An abdominal pressure of 8 mmHg was maintained in all patients and was considered optimal for the procedures. Short laparoscopy instruments (240 mm) were unsuitable for the procedures and had to be replaced by longer instruments (310 mm or 430 mm). CONCLUSIONS: Technical modifications are required to the approach in laparoscopic abdominal access to overcome the challenges posed by the floppy abdominal wall in prune belly patients. Open access, suture fixation of the optic and work ports, use of threaded sleeve ports, and use of proper length of laparoscopy instruments are valuable modifications to overcome the technical hurdles posed by these patients.     

Covered stent grafts for relining of chronically occluded femoro‐popliteal bypasses in frail patients

In four high‐risk patients with chronically occluded femoro‐popliteal bypass suffering from Rutherford 4‐5 chronic limb threatening ischemia we performed, as an alternative for redo surgery, endovasculair relining with covered stent grafts. During follow‐up (3, 8, 14 and 20 months) one patient had redo percutaneous intervention and eventually below‐the‐knee amputation.     

The effect of intensified lipid-lowering therapy on long-term prognosis in patients with peripheral arterial disease

BACKGROUND: The 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (statins) are associated with improved outcome in patients with peripheral arterial disease. Statins may also have beneficial properties beyond their lipid-lowering effect. METHODS: A prospective, observational cohort study was conducted at a university hospital from 1990 to 2005 to examine whether higher doses of statins and lower low-density lipoprotein (LDL) cholesterol levels are both independently associated with improved outcome in peripheral arterial disease. Enrolled were 1374 consecutive patients (age, 61 +/- 10 years, 73% male) with peripheral arterial disease (ankle-brachial index 相似文献   

Transient intradural catheter lysis in a patient with a diffuse and elongated subdural hematoma of the spine due to trauma. Case report

The often extended and elongated configuration of a diffuse subdural hematoma of the spine makes it impossible to completely evacuate with common neurosurgical approaches. The authors describe the complete evacuation of a diffuse subdural hematoma of the entire spine due to trauma in a patient who suffered myelopathy and paraplegia in succession, by using transient subdural catheter lysis. After the patient underwent a partial hemilaminectomy at T7-8 and L2-3 using a lateral transmuscular approach, a 15 cm-long intraventricular catheter was inserted at each hemilaminectomy site and connected to an external ventricular drainage system in a procedure lasting 1 hour. Subsequently, 5000 IU of urokinase was applied four times daily for 30 minutes each time over the next 5 days. Two months later, the patient presented with spastic paraparesis Manual Muscle Test Grade 4/5. Magnetic resonance (MR) imaging revealed no catheter-related complications. The authors conclude from this case that transient catheter lysis may be an effective and gentle method to treat diffuse and elongated subdural hematomas of the spine due to trauma. A larger series needs to be analyzed, however, to address the indications and limitations of the technique compared with conventional open surgery. Such evaluation should include serial MR imaging and electrophysiological examination.     

Acromioclavicular dislocation Rockwood III–V: results of early versus delayed surgical treatment

Introduction  The management of acute acromioclavicular joint (ACJ-) injuries especially of type III is still controversial. Methods  In this retrospective study the results of early ACJ reconstructions immediately after trauma (group early repair, ER) were compared with the results of delayed reconstructions in patients who first got conservative treatment and failed after some time (group delayed repair, DR). Overall, 49 patients were analysed clinically and radiographically with a mean follow-up of 53 months (range 20–92). In group ER twenty-nine patients were treated with a modified Phemister technique according to Mayr including a PDS coracoclavicular sling and temporary K- wire fixation. In group DR twenty patients were treated with a modified Weaver–Dunn-procedure with additionally transposition of the coracoacromial ligament and AC-joint resection. Results  A comparison of the overall results revealed a statistically significant better outcome in the early repair group, regarding the Constant Score, the degree of acromioclavicularjoint-reduction, numbers of complications and patient`s satisfaction. Conclusion  Our results point out that early reconstruction of ACJ-injuries in type III–V avoids the inferior clinical results of delayed reconstructions using a modified Weaver–Dunn-procedure.     

Optimization of hepatosplanchnic oxygenation in septic shock

Microcirculatory dysfunction, reduced oxygen supply and thus impaired hepatosplanchnic organ function, play a pivotal role in the clinical manifestation of sepsis and septic shock. Early correction of the perfusion mismatch is essential to maintain end-organ and gut mu-cosa barrier function and thus to prevent bacterial translocation, bacteraemia and possible multiple organ failure. This review will outline the clinical tightrope-walk optimizing hepa-tosplanchnic oxygen supply while maintaining adequate end-organ perfusion pressure and highlight the diagnostic challenges and limitations faced in clinical practice.