Coexistence of Fabry disease and IgA nephropathy: a report of two cases

Background

Coexistence of Fabry disease and IgA nephropathy is rare. Moreover, the coexisting Fabry disease may be unrecognized due to unapparent clinical manifestations.

Method

We described two cases with coexisting Fabry disease and IgA nephropathy. The clinicopathological features of these two patients were studied.

Results

A 54-year-old male presented with proteinuria, hematuria, and hypertension, and a 33-year-old male presented with proteinuria without clinical signs or family history of Fabry disease. Both of them were diagnosed with IgA nephropathy at admission, whereas Fabry disease was not suspected. Subsequent immunofluorescent study confirmed the diagnosis of IgA nephropathy by showing positive staining for IgA and complement C3 in the mesangium. Meanwhile, light microscopy showed remarkable vacuolation of podocytes with mild mesangial expansion, which was characteristic of Fabry nephropathy. Further examination of toluidine blue-stained semi-thin sections and electron microscopy demonstrated blue bodies and myelin figures in the cytoplasm of podocytes, respectively. The diagnosis of coexisting Fabry disease was finally established based on deficient α-galactosidase A activity in both patients.

Conclusion

This case study is an important reminder of the role of kidney biopsy as an indicator of Fabry disease and its rare coexistence with IgA nephropathy.     

Data base management for nursing education

The data base management system (DBMS) is one tool that can greatly facilitate the organization and retrieval of such information. An overview of DBMS is presented for nurse educators with considerations for using and selecting a DBMS. System 1022 (produced by Software House, 1983), is discussed in terms of the process and problems encountered.     

Diagnostic values of carcinoembryonic antigen in predicting peritoneal recurrence after curative resection of gastric cancer: a meta-analysis

Aim

A meta-analysis was performed to assess the diagnostic values of carcinoembryonic antigen (CEA) in predicting the peritoneal recurrence after curative resection of gastric cancer.

Methods

The Medline, Embase, Web of Science, Ovid and Cochrane databases, Google Scholar and Vivisimo engines were searched to identify studies reporting on the accuracy of CEA protein or CEA mRNA in predicting the postoperative peritoneal recurrence of gastric cancer. Publication bias was demonstrated by Funnel plots and Egger test. The sensitivity, specificity, and diagnostic odds ratio (DOR) were calculated and summary receiver operating characteristic curves were generated.

Results

Seven and eight studies fulfilled the inclusion criteria for CEA protein and mRNA determination, including 635 and 849 patients, respectively. The pooled sensitivity, specificity and DOR of CEA protein for predicting the peritoneal recurrence were 0.77 (95 % CI 0.69–0.84), 0.89 (95 % CI 0.86–0.92), 29.71 (95 % CI 10.27–85.92), respectively. Similarly, the values for CEA mRNA were 0.82 (95 % CI 0.75–0.88), 0.82 (95 % CI 0.79–0.85) and 22.97 (95 % CI 10.90–48.41). Meanwhile, the sensitivity and DOR of CEA protein or mRNA were higher than those of cytology, while higher specificity was noted in cytology assay.

Conclusion

CEA protein and mRNA levels in peritoneal lavage show a high diagnostic accuracy and may help accurately predict the peritoneal recurrence after curative resection of gastric cancer.     

Time-effect Relationship of Extracts from Ginseng, Notoginseng and Chuanxiong on Vascular Endothelial Cells Senescence

Objective:To observe the time-effect relation of extracts from ginseng,notoginseng and chuanxiong on angiotensin II(AngⅡ)-induced senescence of vascular endothelial cells and explore the feature of Chinese medicine against vascular diseases.Methods:Human umbilical vein endothelial cells(HUVECs)cultured in vitro were stimulated with 10~(-6) mol/L Ang Ⅱ to induce cell senescence,which were divided into 4groups,the blank control group,the Ang Ⅱ model group,the extracts group and the telmisartan group.The(J-gal was used to identify senescence of cells,the cell counting kit-8 method was applied to assess the cell viability,the cell function was examined with the level of endothelial nitric oxide synthase(eNOS) and the flow cytometry was used for analyzing the cell cycle changes.Results:Compared with the control cells,the cells positive for β-gal staining was significantly increased in the Ang Ⅱ model group,and showed cell cycle arrest at G_0/G_1 phase with decreased S and G_2/M phase ceil percentage,eNOS expression and cell viability(P0.05).The extracts and telmisartan treatment of Ang II-induced cells resulted in decreased β-gal positive cells with a reduction in G_0/G_1phase cells and an increasing in S,G_2/M phase ceils and eNOS expression(P0.05).At 24 h,the extracts were more effective than telmisartan(P0.05);while telmisartan was more effective at 48 h(P0.05).Conclusion:Extracts from ginseng,notoginseng and chuanxiong can delay Ang II-induced aging of HUVECs and may play an important role in early senescence.     

The locus of control: It’s significance in the irritable bowel syndrome

PSYCHOLOGICAL factors have a role in Irritable Bowel Syndrome symptomatology. The locus of control test determines an individual’s degree of internality or externality. The degree of internality or externality does not influence the site of major Irritable Bowel Syndrome symptomatology. Externalizers have a worse prognosis than internalizers. This effect on prognosis should be borne in mind when testing for diet and or drug efficacy.     

The organization of health services research in the Veterans Administration

The VA health services research, development, and education efforts are organized in four main programs: (1) the Intramural Research Program, (2) the VA-University Health Services Research Affiliation Program, (3) the Health Services Research Training Program, and (4) the Contract Research Program. This report first describes the administrative location and structure of the Health Services Research and Development Service within the VA Department of Medicine and Surgery. Then the goals, organizational structure, and major activities of each program are presented.     

Market model addresses hospitals' need for decision support

A Decision Support System should be approached, not as “starting over,” but as a natural extension of the design and development of a hospital's current HIS. Integrated, real-time HISs generate reams of information that, when combined with relevant external data, provide the essential information base for a hospital DDS. The Travenol Market Model is discussed as an example of a DSS specific to hospital needs.     

Health services research in alcoholism

Alcoholism has become an important health care problem for the United States and the VA medical care system. The percentage of inpatients in Veterans Administration hospitals with a diagnosis of alcoholism rose from 13.0% in 1970 to a high of 15.6% in 1977. Health services research work in alcoholism has generally fallen into four major areas: community diagnosis; utilization of services by alcoholics; the effectiveness, efficiency, and quality of services; and the organization of information systems and their applicability to alcoholism. Obstacles to research include a poor understanding of the prevalence of the disease among the veteran's population, but the system offers many more opportunities than obstacles. A new information system. SATAR (Substance Abuse Treatment Automated Records), offers a chance for large-scale investigation of the problem of alcoholism among VA patients when combined with other information systems and allows for special comparisons through its large, integrated network of hospitals and clinics.     

Service Oriented Architecture for Clinical Decision Support: A Systematic Review and Future Directions

The use of a service-oriented architecture (SOA) has been identified as a promising approach for improving health care by facilitating reliable clinical decision support (CDS). A review of the literature through October 2013 identified 44 articles on this topic. The review suggests that SOA related technologies such as Business Process Model and Notation (BPMN) and Service Component Architecture (SCA) have not been generally adopted to impact health IT systems’ performance for better care solutions. Additionally, technologies such as Enterprise Service Bus (ESB) and architectural approaches like Service Choreography have not been generally exploited among researchers and developers. Based on the experience of other industries and our observation of the evolution of SOA, we found that the greater use of these approaches have the potential to significantly impact SOA implementations for CDS     

Assessment of variables contributing to Cyclosporine distribution in blood

Factors which can account for the poor correlation between whole blood and plasma Cyclosporine (CsA) levels in patients on CsA prophylaxis are evaluated. The study took account of the influence of plasma separation procedures, and the sample haematocrit on CsA distribution in the blood of renal transplant patients (n=35). CsA was measured using both specific and non-specific CsA radioimmunoassays. Significant negative correlations occurred between CsA distribution and the haematocrit, independently of the plasma separation procedure or the specificity of the assay. All results were lower when using the specific assay but a significantly higher percentage of CsA was measured in the plasma by specific assay compared to nonspecific assay when plasma was separated at both 22°C (t-test, p<0.02) and at 37°C, p<0.01). This may relate to the selective binding of CsA and its analogues by blood cells. This study is a prelude to the development of more consistent plasma separation procedures in the monitoring of this drug.     

Studies on six patients with isolated hypogonadotropic hypogonadism — The response to a short course of pulsatile LHRH

SIX patients with idiopathic hypogonadotropic hypogonadism, five males and one female, aged 20–26 years, were studied. All therapy with either testos terone or oestrogen was stopped for at least two months prior to the study. Basal levels of serum LH, FSH, oestradiol and testosterone were low and the response to 100 ug LHRH intravenously was vari able, ranging from a flat response to a normal response. Pulsatile doses of LHRH 25 ng/kg were administered sub cutaneously every two hours over a 72 hour period. The serum levels of testos terone and oestradiol remained at the previous low values but the levels of LH and FSH rose significantly in all patients. The response to 100 ug LHRH intravenously immediately after this treatment was unchanged. If there is not a consistent rise in LH in the early days after initiation of LHRH pulsatile therapy, changes to the dose and/or route of administration should be made.     

Legal constraints and alternatives in the Veterans Administration

The Veterans Administration is a federal system that has functioned, so far, independently of state and local laws and rules. The system has proven flexible, however, as it has adjusted to meet needs perceived within its organization where legal justification has been found. The impact of the overall health care system in the United States is beginning to be felt within the Veterans Administration as the possibility of national health insurance implementation may greatly affect the number and kinds of patients seen in the hospitals. The response of the Veterans Administration system will have to be within its legal framework and an understanding of the relationship between the actual operation of the system and the law will be necessary. More and better understanding of the practical impact of external programs such as comprehensive health planning, manpower training and licensing laws will be necessary for the Veterans Administration to successfully adjust within the United States' health care framework.     

Reactive Airways Dysfunction Syndrome (RADS) due to chlorine gas exposure

Reactive Airways Dysfunction Syndrome (RADS) has been described following exposure to various irritant gases⁽¹⁾. We describe a case of RADS occurring following exposure to chlorine gas and which has persisted at 6 years follow-up.     

Is Z-Plasty closure reasonable in pilonidal disease?

A PROSPECTIVE, randomised, controlled trial of radical excision of pilonidal disease with primary closure versus Z-plasty closure was carried out. The operator was a general surgeon, and no case was excluded because of infection. Twenty patients were operated upon of which twelve had abscesses. The results of surgery were compared and finally reviewed by an independent plastic surgeon after six months. No patient who was operated on during an infective episode developed a wound infection. Eleven patients had a Z-plasty closure and one recurred. Nine patients had a simple closure and two recurred.     

The use of X-terminals as clinical workstations

The Medical Computer Facility at the Fox Chase Cancer Center has installed X-terminals in patient examination rooms and at nursing stations for clinical data acess by physicians and nurses. The X-terminals are connected to UNIX operating system RISC processors via Ethernet. The RISC processors communicate with databases on a minicomputer cluster. Simultaneous presentation of textual (e.g., pathology and radiology reports) and graphical (e.g., clinical laboratory results) clinical data is provided under X-Windows. CT and MRI images can also be displayed in windows. Our experiences implementing X-terminal clinical workstations in a production environment will be discussed.     

Automation: The competitive edge for HMOs and other Alternative Delivery Systems

Until recently, many, if not most, Health Maintenance Organizations (HMO) were not automated. Moreover, HMOs that were automated tended to be automated only on a limited basis. Recently, however, the highly competitive marketplace within which HMOs and other Alternative Delivery Systems (ADS) exist has required that they operate at a maximum effectiveness and efficiency. Given the complex nature of ADSs, the volume of transactions in ADSs, the large number of members served by ADSs, and the numerous providers who are paid at different rates and on different bases by ADSs, it is impossible for an ADS to operate effectively or efficiently, let alone show optimal performance, without a sophisticated, comprehensive automated system. Reliable automated systems designed specifically to address ADS functions such as enrollment and premium billing, finance and accounting, medical information and patient management, and marketing have recently become available at a reasonable cost.     

Surgical unit time utilization review: Resource utilization and management implications

As health care providers seek ways to reduce the cost of health care services, hospital operating rooms (ORs) have been identified as potential areas for cost reduction efforts. Cost containment efforts which have shifted significant portions of the inpatient population to ambulatory areas have resulted in an inpatient population which is sicker and more procedure-intensive. Efficient management of operating rooms has assumed even greater importance in this environment. Inefficient or inaccurate scheduling of OR time often results in delays of surgery or cancellations of procedures, which are costly to the patient and the hospital. Approaches to efficient use of ORs include computerized scheduling, utilization monitoring, and refinement of scheduling policies and procedures. In the absence of commercially avaiable software to meet operating room management information needs, Johns Hopkins developed its own system in 1983. This software provides detailed information for daily OR management and long-term planning. The computerized operating room scheduling and monitoring system is described in this article and an operational measure of scheduling accuracy is proposed. Suggestions are made for incorporating this measure into planning and allocation decisions.     

The Computerized Severity Index

We describe the new Computerized Severity Index (CSI) that is obtained from an expanded discharge abstract data set, based on a 6th-digit severity addition to the ICD-9-CM coding system. The new 6-digit code book (called ICD-9-CMSA) is used to label existence and severity of each principal and secondary diagnosis. It can be used to produce an overall severity of illness level for each hospital inpatient. The impact of severity-adjusted DRGs on prospective payment and uses of the CSI for assessing quality of care, efficiency, physician practice profiles, and prediction of posthospital resource needs are discussed.     

Neuropathologic findings in AIDS and human immunodeficiency virus infection — Report on 30 patients

Central nervous system findings in 30 fatal cases of human immune deficiency viral (HIV) infection are described. Twenty seven patients had acquired immune deficiency syndrome (AIDS) and three patients had serological evidence of HIV infection only (HIV seropositive). Twenty nine patients had neuropathologic abnormalities at autopsy and frequently had more than one neuropathologic process. Neurologic disease was the dominant clinical feature in nineteen patients. The spectrum of neuropathologic disease is similar to that described in other series encompassing direct HIV infection of brain; indirect CNS involvement by opportunistic pathogens differing slightly from other series in their relative frequency; lymphoma and neurovascular disease. This, the first report which serves to document the level of neurologic disease in Irish patients with AIDS and HIV infection should help to provide meaningful information for use in the planning of neurologic and rehabilitation services for these patients.     

A chartless record—Is it adequate?

The computerized medical record supported by The Medical Record (TMR) has been the only record of physician-patient encounters on the nephrology service of the Durham Veterans Administration Medical Center since April 1981. Physicians using the system evaluated the adequacy of the computerized record as a replacement for the paper chart. The computerized record was able to capture and display all pertinent data. Manual or computerized narratives provided a useful supplement to the core computerized record only in those rare instances that a physician needed to point out which of the data in the record were important to his decision making.