The midaortic syndrome: diagnosis and treatment

The midaortic syndrome is an unusual entity seen in children and adolescents. It is characterized by severe narrowing of the abdominal aorta with progressive involvement of the renal and visceral branches. Eleven patients (aged 5 months to 15 years) suspected of having midaortic syndrome were examined preoperatively and postoperatively. All patients had hypertension and were examined with midstream aortography. All aortograms showed a smooth, segmental stenosis of the abdominal aorta and severe bilateral proximal renal artery stenosis. In three patients, percutaneous transluminal angioplasty of the renal artery was attempted, two preoperatively and one for a postoperative stricture. None showed long-term success, presumably due to the progressive nature of the disease. Grafts were surgically placed in ten patients and produced successful results in nine.     

Phosphorylation of the oxidase-related 48K phosphoprotein family in the unusual autosomal cytochrome-negative and X-linked cytochrome-positive types of chronic granulomatous disease

Activation of 32P-loaded neutrophils with phorbol myristate acetate causes the labeling of a family of three 48K proteins that focus near neutral pH. The relationship between these phosphoproteins and the activation of the respiratory burst has been supported by the previous finding that phosphorylation was defective in the two most common types of chronic granulomatous disease (CGD): X-linked cytochrome-negative (X/-) and autosomal cytochrome-positive (A/+). In this report, these studies have now been extended to the rare A/- and X/+ forms of the disease. In all three patients with A/- CGD examined, the two most acidic 48K proteins failed to undergo enhanced phosphorylation in response to phorbol stimulation, a finding similar to that seen in X/- patients. In contrast, neutrophils from two patients with X/+ CGD appeared to phosphorylate the neutral 48K proteins in a normal fashion. It thus appears that the different phosphorylation patterns seen in chronic granulomatous disease are a reflection of the genetic heterogeneity of this disorder. These findings lend further support to the conclusion that the 48K phosphoprotein family is related to the respiratory burst, although not necessarily in a straightforward manner.     

Normal brachial plexus: MR imaging

Magnetic resonance (MR) imaging of the brachial plexus was performed in the axial, coronal, and sagittal planes in seven volunteers. Normal structures were delineated by comparison with axial and sagittal cadaver sections and with gross dissection. Differentiation of soft tissues with MR imaging enabled the brachial plexus to be defined from surrounding muscle and vascular structures. Multiplanar imaging demonstrated anatomic detail not previously demonstrated with other radiologic modalities and provided excellent delineation of the components of the brachial plexus from the ventral rami to the peripheral nerve branches.     

IA～ⅡB霍奇金病：纵隔广泛受累病例的处理和结果

ＩＡ～ⅡＢ霍奇金病：纵隔广泛受累病例的处理和结果ＬｕｋｅＨＤ，ｅｔａｌ．ＩｎｔＪＲａｄｉａｔＯｎｃｏｌＢｉｏｌＰｈｙｓ，１９９７，３９：３６１．纵隔广泛受侵一直被认为是Ⅰ～Ⅱ期霍奇金病（ＨＤ）单纯放疗及较晚期ＨＤ单纯化疗的不利因素。本文作者分析１７２...