Pulmonary artery distensibility in pulmonary arterial hypertension: an MRI pilot study.

Pulmonary arterial hypertension (PAH) is a disease of the small vessels in which there is a substantial increase in pulmonary vascular resistance leading to right ventricle failure and death. Invasive haemodynamic evaluation is mandatory not only for diagnosis confirmation but also to address prognosis and eligibility for the use of calcium-channel blockers through an acute vasodilator challenge. Noninvasive surrogate response markers to the acute vasodilator test have been sought. In the present study, the relationship between pulmonary artery distensibility, assessed using magnetic resonance imaging (MRI), and response to acute vasodilator tests was investigated. In total, 19 patients diagnosed with idiopathic PAH without any specific treatment were evaluated. Within a 48-h window after pulmonary artery catheterisation, patients underwent cardiac MRI. Cardiac index, calculated after the determination (invasively and noninvasively) of cardiac output, showed excellent correlation, as did right atrial pressure and right ventricle ejection fraction. Pulmonary artery distensibility was significantly higher in responders. A receiver operating characteristic curve analysis has shown that 10% distensibility was able to differentiate responders from nonresponders with 100% sensitivity and 56% specificity. The present findings suggest that magnetic resonance imaging and pulmonary artery distensibility may be useful noninvasive tools for the evaluation of patients with pulmonary hypertension.     

Common bile duct calculi: updated experience with dissolution with methyl tertiary butyl ether

The authors describe their experience with methyl tertiary butyl ether (MTBE) in a larger series of patients than previously reported in order to acquaint physicians with both its effectiveness for dissolution of common bile duct calculi and the limitations of its use. Ten patients with 13 biliary calculi underwent percutaneous stone dissolution treatment with the experimental cholesterol solvent, MTBE. Three stones completely dissolved within 30 minutes, seven were reduced in size, and three were visibly unaffected. All stones not completely dissolved were easily extracted by means of a stone basket except for one in a patient taken to surgery. Although MTBE perfusion is an effective technique for management of biliary calculi, practitioners should be aware that its use is quite time consuming and its odor difficult to control.     

Segmental intestinal muscular thinning: a possible cause of intestinal obstruction in the newborn

Intestinal obstruction proximal to a transition zone without an interposed physical barrier usually indicates Hirschsprung disease. The authors report one case of focal small bowel muscular thinning just distal to a transition zone that produced clinical and radiographic findings that simulated long-segment Hirschsprung disease in a 2-day-old infant.     

Image-directed percutaneous biopsies with a biopsy gun

Core tissue for histologic study is believed by many pathologists to be more diagnostic than material from needle aspiration. Recently, a biopsy "gun" has been introduced, which simplifies core biopsies. With this device, 182 biopsies of multiple anatomic sites were performed with ultrasonic, computed tomographic, and fluoroscopic guidance and 18-gauge needles. High-quality histopathologic specimens were obtained in 177 of the biopsies, and diagnostic target tissue was obtained in 167. Only three significant complications occurred: one bleeding complication that required transfusion and two cases of pneumothorax that necessitated placement of chest tubes. The biopsy gun eliminated the disjointed movements of conventional "skinny" needle biopsies, and none of the samples demonstrated significant "crush" artifact or obscuring blood, problems that are commonly associated with manual biopsy techniques. Patient discomfort was decreased with this system compared with that of manual biopsies, and the total procedure time was reduced. Because of these distinct advantages, the authors now use the biopsy gun exclusively for all percutaneous biopsies and recommend that other institutions consider the use of this biopsy method.     

颅内蛛网膜囊肿34例

1　临床资料1.1　一般资料　 1986 / 1997收治颅内蛛网膜囊肿 34(男 2 8,女 6 )例 .年龄 3～ 6 8岁 ,其中 16岁以下 17例 .有头部外伤史者 15例 ,无明显诱因者 19例 .病程 :2 m o～ 14a.头部外伤至发病时间 :7d～ 2 a. 34例中幕上 30例 ,幕下 4例 ,其中侧裂池 15例 .1.2　临床症状　表现头疼、头昏或伴呕吐、视力下降、视乳头水肿等颅内压增高的症状 18例 ,癫痫发作 7例 ,偏瘫 6例 ,行走不稳 2例 ,失语 1例 .1.3　辅助检查　本组 2 8例作 CT检查 ,6例行 MRI检查证实 .头颅 CT扫描特征 :脑实质外有一边界清楚的低密度病灶 ,CT值与脑脊液…     

Management of a case of maple syrup urine disease--the use of gluco-insulinotherapy

We report here the treatment and poor outcome of a case of Maple Syrup Urine Disease with late diagnosis and retrieval (2 and 5 months, respectively). As the proband had quite high levels of plasmatic leucine (1956 micromol/L for a normal upper limit of 77), we started immediately with a gluco-insulin therapy to produce anabolism in the infant. When leucine has fallen to 275.3 micromol/L, we instituted feeding with branched chain amino acid-free protein and high energy from carbohydrates. After reviewing briefly the clinical, biochemical and therapeutic aspects of this disorder, we comment on the great difficulties of making early diagnosis and of obtaining the specific dietetic formulas to Maple Syrup Urine Disease, in Brazil.     

Efficacy of Zoladex LA (goserelin) in the treatment of girls with central precocious or early puberty

OBJECTIVE: To assess the efficacy of a longer acting preparation of the gonadotrophin releasing hormone (GnRH) analogue goserelin (Zoladex LA, 10.8 mg) in 12 girls with central precocious or early puberty. METHODS: Two girls started treatment de novo; the remainder had been on suppressive treatment for a median duration of 1.5 (range, 0.2-5.6) years. Assessment comprising auxology, pubertal staging, and pelvic ultrasound examination was carried out at weeks 0, 4, 8, 10, and 12 (first cycle) and weeks 8, 10, and 12 (second cycle) to evaluate the required injection frequency. Thereafter, assessment was performed on the day of injection. Zoladex LA was given every 12 weeks unless pubertal progression occurred. RESULTS: Satisfactory control was achieved in eight patients using this regimen, and three patients required more frequent injections. One girl was removed from the study because of clinical progression and extreme mood swings. No serious adverse effects occurred. Mean height velocity during the study period was 4.5 cm/year (range, 3.1-6.6) compared with 6.5 cm/year (range, 3.8-9.6) before treatment in nine patients for whom data were available. CONCLUSIONS: Zoladex LA was effective in controlling precocious puberty in girls when given at intervals of 9-12 weeks and it is recommended that an initial assessment is made eight weeks after beginning treatment.     

Characterization and induction of cementoblast cell proliferation by bioactive glass nanoparticles

Cementum is a mineralized tissue that lines the surface of the tooth root enabling attachment of the periodontal ligament to the root and surrounding alveolar bone. Studies examining the mechanisms involved in the formation of root cementum have been hindered by an inability to isolate and culture the cells required for cementum production (cementoblasts). This study isolated and characterized cementoblast cells derived from rat molar periodontal ligament. It was observed that the isolated cells expressed F‐Spondin, a cementoblast marker, while F‐Spondin expression was not observed in the cells of other tissues such as gingival fibroblasts and osteoblasts. As expected, the isolated cementoblast cells also expressed osteocalcin (OC), bone sialoprotein (BSP), alkaline phosphatase (ALP), and type I collagen, demonstrating the presence of mineralized tissues genes in cementoblast cells. These cells showed high ALP activity and calcified nodule formation in vitro. Since cementogenesis could be a critical event for regeneration of periodontal tissues, this study investigated whether bioactive glass particles could affect the proliferation of cementoblasts since they are known to enhance osteoblast proliferation. It was found that the ionic products from bioactive glass nanoparticles increased cementoblast viability, mitochondrial activity, and induced cell proliferation. Together, these results show the characterization of cementoblast cells from rat molar periodontal ligament. Additionally, it was shown that bioactive glass nanoparticles induced cementoblast to proliferate, indicating that they could be a potential material for use in cement regeneration through tissue engineering. Copyright © 2011 John Wiley & Sons, Ltd.