基于慢性肾脏病管理的精准分级诊疗应用实践

分级诊疗制度是优化基本医疗卫生制度的重要步骤,但落地实施时遇到阻碍,因存在转诊标准不一、空间不连贯和时间滞后等问题,医联体模式推进存在难度。上海市浦东新区人民医院联合7家社区卫生服务中心,构建了一套以信息化为支撑的川沙医联体慢性肾脏病（CKD）专病精准分级诊疗管理方案。以CKD病种为例,基于指南梳理CKD患者的疾病管理规律,建立医疗信息联通共享、转诊规则标准的CKD专病分级诊疗知识库,设计区域CKD专病分级诊疗系统,构建了基于医院-社区联动管理的专病分级诊疗一体化管理模型。且实证应用评价显示,基于CKD知识库的专病分级诊疗模式,可以精准定位易发和高危人群,及时筛查评估CKD早期患者,提升CKD患者的健康管理和诊疗效率。     

Treatment of craniopharyngiomas—the stereotactic approach in a ten to twenty-three years' perspective

Summary Fourty-two consecutive patients with craniopharyngioma were treated by stereotactic approach, i.e. preferentially stereotactic puncture and installation of colloid isotope into cystic tumours and external stereotactic single dose irradiation to solid tumour parts. In a minority of cases, such treatment was less suitable, and surgical removal and/or radiotherapy was used. There was no peroperative mortality. A long-term follow up (observation time 10–23 years) of the 31 patients alive indicated that they were socially well adapted with a high rate of fulltime work and a low rate of intercurrent disease. In spite of substitution therapy for pituitary insufficiency in most cases, the patients were subjectively seldom disturbed by their disease.Our results support a change in the choice of therapy for craniopharyngioma patients, from open neurosurgery to the less invasive stereotactic techniques.     

Early clinical evaluation of the Cosgrove-Edwards prosthetic heart valve ring

 We discuss the usefulness of the Cosgrove-Edwards ring from our early clinical results from 25 rings in 24 patients who underwent mitral annuloplasty (MAP) or tricuspid annuloplasty (TAP) between June 1999 and December 2000. In the MAP group, the posterior mitral annulus between the anterior and posterior fibrous trigones was reinforced with the prosthetic ring. In the TAP group, the annuli of the anterior and posterior leaflets were splinted with the ring. The prosthetic ring was attached by pledgeted U-sutures. Cardiologists performed echocardiography pre- and postoperatively. Thirteen of the 14 in the MAP group showed mitral valve regurgitation of grade 0 or I. Six of the 11 in the TAP group showed tricuspid regurgitation of grade 0 or I, and 5 patients with regurgitation equal to or greater than grade II who remained in atrial fibrillation postoperatively recovered without further clinical symptoms. No patient has required reoperation during a follow-up period of up to 2 years. Cosgrove-Edwards ring-related complications, such as valve stenosis, ring detachment, and arrhythmia, have been not recognized in these patients. In conclusion, for mitral and tricuspid annuloplasty, the Cosgrove-Edwards prosthetic ring showed excellent early clinical results, particularly in patients maintained in sinus rhythm. Received: November 5, 2001 / Accepted: May 30, 2002 Correspondence to:Y. Misawa     

Isolation of a polymorphic CA repeat sequence at the human progesterone receptor (PGR) locus

We isolated a polymorphic dinucleotide (CA) repeat sequence from a genomic clone containing the human progesterone receptor (PGR) gene. This polymorphism will be a useful marker in the genetic study of disorders affecting female endocrine systems, such as progesterone resistance and breast, uterine, and ovarian cancers. Received: July 27, 1998 / Accepted: July 29, 1998     

Ultrastructure of Nuclear Compartment in Cardiomyocytes during Regenerative and Plastic Insufficiency of the Myocardium

We studied ultrastructure of nuclear compartment in cardiomyocytes during regenerative and plastic insufficiency of the myocardium induced by anthracycline antibiotic daunomycin. A peculiarity of ultrastructural organization of cardiomyocyte nuclei under these conditions is almost complete disappearance of the heterochromatin lumps. The earliest changes in nucleoli under conditions of disturbed DNA-dependent RNA synthesis are segregation of the granular and fibrillar nucleolonema components. Deep alterations in the nucleoli manifested by fragmentation and annulation correlate with pronounced changes in cardiomyocytes ultrastructure, intensive lysis of the myofilaments, reduction of the organelles, and enhanced autophagocytosis.     

High levels of Lp(a) lipoprotein in a family ith cases of severe pre-eclampsia

We report a family with two cases of severe pre-eclampsia/eclampsia in which very high levels of Lp(a) lipoprotein were found. The serum level of Lp(a) lipoprotein is genetically determined and the Lp(a) apolipoprotein has a close homology to plasminogen. Very high levels of Lp(a) lipoprotein might interfere with the fibrinolytic/thrombolytic process in man. A previous report suggested that a high maternal serum Lp(a) lipoprotein level can cause fetal growth retardation, and it is proposed that very high levels might lead to increased deposition of fibrin in the uterine spiral arteries in pregnancy, which is central in the pathogenesis of pre-eclampsia. If confirmed, a very high Lp(a) lipoprotein level could be one risk factor for pre-eclampsia that is genetically determined.     

Triple A syndrome: genotype-phenotype assessment

The triple A or Allgrove syndrome is an autosomal-recessive disease (MIM*231550) characterized by the triad of achalasia, alacrima and adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency. Associated features of the syndrome are neurological and dermatological abnormalities. Until the discovery of the AAAS gene as the responsible gene in triple A syndrome, the diagnosis was based on characteristic clinical features. Here we present the clinical and molecular genetic data which demonstrated the marked phenotypic variability in three unrelated patients with triple A syndrome. The final diagnosis of triple A syndrome was confirmed by molecular analysis. In one patient with isolated achalasia, the diagnosis of triple A syndrome could only be made on the basis of the molecular genetic analysis of the AAAS gene. We therefore suggest that the diagnosis of triple A syndrome should be considered in patients who exhibit only one or two of the main symptoms (i.e. alacrima, achalasia or adrenal insufficiency). These patients require careful neurological investigation, and mutation analysis of the AAAS gene should be performed.     

瞬目反射、脑干听觉诱发电位、经颅多普勒超声对椎基底动脉供血不足的诊断价值

目的：观察瞬目反射（ＢＲ）、脑干听觉诱发电位（ＢＡＥＰ）、经颅多普勒超声（ＴＣＤ）对椎基底动脉供血不足（ＶＢＩ）的诊断意义。方法：对４１例已经临床确诊的ＶＢＩ病人在间歇期进行ＢＲ、ＢＡＥＰ及ＴＣＤ检查。结果：ＴＣＤ、ＢＲ、ＢＡＥＰ异常率分别为８３％、８０％、６８％。ＢＲ提示脑桥损害１例、延髓损害１５例、广泛性脑干损害１７例；ＢＡＥＰ发现内耳听力减退２０例、脑干病变１３例；ＴＣＤ发现多血管流速异常１４例、一支流速改变１６例，有或伴有血管张力异常１３例。结论：由于ＢＲ、ＢＡＥＰ反射路径不同，检测结果不尽一致。联合检查有助于对病损部位进行定位。ＴＣＤ则有助于定性诊断     

Experimental osteodystrophy of chronic renal failure induced by aluminum- and ferric-nitrilotriacetate in Wistar rats

The aluminum (Al) and iron (Fe) chelate complexes of nitrilotriacetate (NTA) cause renal insufficiency when they are administered intraperitoneally to rats. Their effects on bone metabolism were studied in 4 week old Wistar rats. Daily intraperitoneal administration of Al-NTA (3 mg Al/kg for 11 weeks) induced osteomalacia, impaired bone growth, decreased bone mineral density, lower serum PTH levels than normal as well as renal insufficiency. Al staining showed diffuse deposition in the trabecula and a strong linear band of aluminum deposited at the mineralization front and along the cement line. The osteoid seen markedly within the trabecula was probably the decalcified portion of the bone, the calcium apatite of which was defectively fabricated because of diffuse Al deposition in the trabecula. Al deposition along the cement line would make it much more susceptible to external shear stress than normal. Although daily intraperitoneal administration of Fe-NTA (6 mg Fe/kg for 11 weeks) caused impaired bone growth, decreased bone mineral content and renal insufficiency, the osteoid volume did not increase. Fe staining showed that Fe was deposited diffusely in the cytoplasm of osteoblasts. The results of this study demonstrated that during renal insufficiency, different minerals exhibi different modes of action on bone metabolism, and that AI-NTA is useful for experimental animal models of Al-induced osteomalacia in renal insufficiency.