Myofibroblasts defined by electron microscopy suggest the dedifferentiation of smooth muscle within the sac walls associated with congenital inguinal hernia

OBJECTIVE: To ascertain the presence of myofibroblasts in sacs associated with inguinal hernia in children, through an ultrastructural evaluation using electron microscopy. MATERIALS AND METHODS: Sacs were obtained from 10 boys and 10 girls (of similar age, approximately 45 months) with inguinal hernia and processed for electron microscopy. Thin sections were examined specifically for the presence of myofibroblasts. RESULTS: The ultrastructural evaluation showed myofibroblasts with classical electron microscopic features within all of the sacs, regardless of the gender of origin. CONCLUSION: The persistence of smooth muscle hinders the obliteration of the processus vaginalis; myofibroblasts are found in association with smooth muscle and thus such cells within the sac walls seem to originate from the smooth muscle, reflecting the dedifferentiation of smooth muscle. This dedifferentiated state may represent attempted apoptosis, which usually causes the disappearance of the smooth muscle and obliteration of the processus vaginalis after the descent of the testis into the scrotum.     

Pulmonary artery thrombi detected by echocardiography in patients with pulmonary hypertension secondary to atrial septal defect.

This report presents three patients with severe pulmonary hypertension secondary to atrial septal defect associated with thrombus and spontaneous echo contrast within the pulmonary artery diagnosed by transthoracic and transoesophageal echocardiography. Clinical and echocardiographic features seem to suggest local thrombus formation within the pulmonary arteries as a direct consequence of pulmonary hypertension rather than venous thromboembolism.     

Mutations in ANKS6 Cause a Nephronophthisis-Like Phenotype with ESRD

Nephronophthisis (NPHP) is one of the most common genetic causes of CKD; however, the underlying genetic abnormalities have been established in <50% of patients. We performed genome-wide analysis followed by targeted resequencing in a Turkish consanguineous multiplex family and identified a canonic splice site mutation in ANKS6 associated with an NPHP-like phenotype. Furthermore, we identified four additional ANKS6 variants in a cohort of 56 unrelated patients diagnosed with CKD due to nephronophthisis, chronic GN, interstitial nephritis, or unknown etiology. Immunohistochemistry in human embryonic kidney tissue demonstrated that the expression patterns of ANKS6 change substantially during development. Furthermore, we detected increased levels of both total and active β-catenin in precystic tubuli in Han:SPRD Cy/+ rats. Overall, these data indicate the importance of ANKS6 in human kidney development and suggest a mechanism by which mutations in ANKS6 may contribute to an NPHP-like phenotype in humans.     

盐酸普鲁卡因胺渗透泵片剂的研究

本文研究了盐酸普鲁卡因胺(PA·HCl)渗透泵片的处方组成、制备工艺、体外溶出;建立了PA·HCl的紫外双波长分光光度法及血清和唾液中PA及其活性代谢物N-乙酰普鲁卡因胺(NAPA)共存下的高效液相色谱法,体内PA实验数据按双室模型,用电子计算机处理求得药动学参数。药动学—药效学数据显示血药浓度和唾药浓度均与药效变化规律相关。     

Gastric adenocarcinoma: CT versus surgical staging

Seventy-five patients with gastric carcinoma underwent preoperative staging with computed tomography (CT). In 14 patients, CT failed to demonstrate lymphadenopathy despite the presence of malignant lymph nodes at surgery. In 13 patients, CT demonstrated enlarged nodes, but no malignant involvement was found at surgery. Although spread to the pancreas was correctly predicted in three patients with lack of fat plane between tumor and pancreas, five patients lacking a fat plane had no invasion, whereas eight patients with an intact fat plane had invasion. Thirty-five patients (47%) were incorrectly staged with CT:23 (31%) were understaged and 12 (16%) were overstaged. CT does not accurately display the true extent of disease in patients with gastric carcinoma and therefore should not be used routinely for staging.     

Lack of preferential Vβ usage in synovial T cells of rheumatoid arthritis patients

The T-cell receptor Vβ subfamily repertoires of synovial and peripheral T cells of 8 rheumatoid arthritis (RA) patients were determined using the polymerase chain reaction. Three normal controls were included. Some of the rheumatoid synovial samples did not express the complete range of Vβ families and lacked as many as 6 gene families. However, these patients showed considerable individual variation in expression. Overall, the data do not support preferential T-cell receptor Vβ usage in synovial T cells of RA patients either in comparison to their autochthonous peripheral T cells or to peripheral T cells of normal subjects.     

Percutaneous dilation of benign biliary strictures

Percutaneous balloon dilation of benign biliary strictures was successful in 15 of 18 patients in whom the procedure was attempted. Successful dilation was achieved in nine of 11 patients who had biliary enteric strictures, with follow-up of 22-55 months (mean, 35.4 months) after catheter removal in seven patients. Five of six strictures in the biliary tree that developed after surgery were successfully dilated, with long-term follow-up available in two patients (58 and 42 months). A patient with an inflammatory common bile duct stricture that was successfully dilated was followed up for 18 months. Percutaneous dilation of biliary tract strictures is a promising technique with good long-term results and may be the initial treatment of choice in biliary stricture management.