Acquired von Willebrand's disease in association with Wilm's tumor: regression following treatment

A 9-yr-old female presented with a Wilm's tumor and a coagulopathy consistent with von Willebrand's disease. Factor VIII procoagulant activity (VIII C), factor VIII related antigen (VIIIR:Ag), and von Willebrand factor activity (VIII:vWf) were decreased. There was no evidence for a circulating inhibitor of the factor VIII molecular complex. von Willebrand's antigen II (vW AgII), which is deficient in hereditary von Willebrand's disease, was decreased below detectable levels in this patient. The coagulation studies, VIIIR:Ag, and vW AgII levels returned to normal following therapy of the Wilm's tumor. Wilm's tumor must be included as one of the malignancies associated with acquired von Willebrand's disease. Immunofluorescent studies of the tumor specimen showed normal endothelial staining of VIIIR:Ag by semiquantitative techniques and a lack of specific tumor adsorption of VIIIR:Ag The presence of normal amounts of tissue VIIIR:Ag has not previously been demonstrated in acquired von Willebrand's disease. Since we failed to demonstrate an inhibitor in the plasma in this patient, the etiology of the acquired von Willebrand's disease in this patient appears to differ from other cases of acquired von Willebrand's disease. The finding that vW AgII is decreased in this patient, similar to that reported in hereditary von Willebrand's disease, supports the close association of vW AgII to VIIIR:Ag, even though they are immunologically and biochemically distinct.     

Preliminary note on biometric data relating to the human coraco-acromial arch

A biometric study based on 20 human scapulae made it possible to specify the variations in the gap of the coraco-acromial arch in relation to its depth and height. A graphic representation in rectangular coordinates, then in spatial representation in relation to the three planes of reference, leads to the following findings: the bony variations in the arch occur essentially: at the coracoid apophysis, and two types of arch can be distinguished depending on the predominance of bony or of ligamentous components.     

Cardiac arrhythmias mimicking primary neurological disorders: a difficult diagnostic situation

Cardiac arrhythmias can present with the signs and symptoms of a seizure disorder. This potentially life–threatening underlying cause of non–febrile seizures should be recognized early, since successful specific treatment is possible. The purpose of this retrospective study was to examine common features in such patients. Over a period of 25 years, eight patients were initially treated for up to 5 years at our institution for a seizure disorder until dysrhythmia as the underlying cause of the seizures was disclosed. The main symptom was drop attacks coinciding with physical activity or emotional stress. Convulsions were only rarely observed. In five of the eight patients the underlying disorder was the long–QT–syndrome (Romano–Ward syndrome). In one patient intermittent complete atrioventricular block was found, another patient showed ventricular tachydysrhythmia of unknown etiology and the last patient suffered from hypertrophic cardiomyopathy.     

Variation in the determination of follicular diameter: an inter-unit pilot study using an ultrasonic phantom

Ultrasound operators in assisted reproductive technology units in New South Wales, Australia cooperated in a study that was conducted to assess their ability to measure fixed objects embedded in an ultrasound phantom. The results have demonstrated a large variation ranging between 10 and 25% coefficient of variation on distances between 10 and 32 mm. As ultrasonic imaging is the only component of assisted reproductive technology not currently controlled by an external quality assurance scheme, the authors suggest there may be a need to establish a programme.      

萘磺酰胺衍生物对细菌脂多糖诱导小鼠腹腔巨噬细胞分泌肿瘤坏死因子的影响

研究了6种氯代萘磺酰胺衍生物对细菌脂多糖(LPS)诱导经卡西霉素A_(23187)体外激活的巨噬细胞分泌肿瘤坏死因子(TNF)作用的影响。表明:将氯代萘磺酰胺的氨基接以亲水性不同长度碳链的胺基,所得衍生物可明显抑制LPS诱生TNF的作用;接以疏水性烷基,则可明显增强亚适剂量的LPS诱生TNF的作用,且碳链长度对化合物的作用强度有一定的影响。     

Transesophageal examination of the left coronary artery with a 7.5 MHz annular array two-dimensional color flow Doppler transducer

To determine the value of a recently developed 7.5 MHz annular array two-dimensional and color flow Doppler transducer for examination of the proximal parts of the left coronary artery, 25 patients were studied immediately after coronary artery bypass surgery, and the findings were compared with preoperative coronary angiograms. With two-dimensional imaging, the left main coronary artery was visualized in 22 patients (88%), left anterior descending in 13 patients (52%), and circumflex in 22 patients (88%). Stenoses were correctly identified in four of four left main coronary arteries, in five of seven left anterior descending arteries, and in none of four left circumflex arteries. Adding color flow to two-dimensional imaging in 20 patients, we found a disturbed flow signal, as a sign of stenosis, in five of five left anterior descending artery and two of four left circumflex artery stenoses. We concluded that these initial findings with this new 7.5 MHz annular array two-dimensional and color flow Doppler transducer, are promising in imaging and detection of stenoses in the proximal parts of the left coronary artery.