Meier-Gorlin syndrome: report of eight additional cases and review

The Meier-Gorlin syndrome or ear, patella, short stature syndrome (MIM 224690) is a rare autosomal recessive disorder, characterized by the association of bilateral microtia, aplasia/hypoplasia of the patellae, and severe pre- and postnatal growth retardation. Twenty-one cases have been reported in literature thus far. Here we report on eight patients from seven families and compare them with previously described cases. One of the present cases had previously undescribed genital anomalies. There is a difference in facial characteristics between patients reported in early infancy and those described at older age; follow-up of patients is needed to substantiate this changing facial phenotype. We recommend radiographic survey of the patellae in patients at older age to investigate the weight of absent or hypoplastic patellae in the diagnosis of the syndrome.     

乙型肝炎肝组织血管病变组织及免疫组织化学的研究

乙型肝炎(HB)已成为我国危害最大的社会公共卫生问题.近年来,我们在分析、研究国内外有关病毒性肝炎文献后选择了以HB患者肝组织活检观察为主的研究方法,从肝组织学随访中研究各型HB肝实质变性坏死及肝纤维组织增生的动态变化规律[1-8],采用组织化学(组化)及免疫组织化学(免疫组化)染色方法对肝组织内HBsAg,HBcAg表达[3],不同类型纤维组织增生情况,血清HBeAg与抗-HBe转换及透明质酸,色氨酸代谢变化[9-13],进行了深入研究.     

Preoperative education for total hip and knee replacement patients

Objective. Psychoeducational preparation is known to improve postoperative outcome. We tested two common psychoeducational procedures in elderly orthopedic patients, examining how best to match interventions to patients by psychological type. Methods. Two hundred twenty-two elderly patients undergoing total hip or knee replacement were randomly assigned to (1) a slide-tape with information on the postoperative, in-hospital rehabilitation experience, or (2) training in Benson's Relaxation Response with a bedside audiotape, in a 2 × 2 factorial design. Results. The relaxation response did not influence postoperative outcomes. The educational intervention reduced length of stay and pain medication use for patients who exhibited most denial (tendency to avoid thinking about unpleasant events), and reduced postoperative anxiety and cognitive errors on the Mini-Mental State Exam for patients with most baseline anxiety. There was no effect on postoperative pain. Conclusions. The importance of attending to the patient's psychological state and level of preparation before orthopedic surgery is reinforced. Patients who exhibit most denial and highest anxiety may benefit from educational interventions, but patients' directly expressing desire for information may be a poor guide in deciding which patients would benefit, compared with more formal psychological testing for denial and anxiety.     

Braided bronchus: A previously undescribed airway anomaly

Infants with congenital heart disease frequently experience recurrent atelectasis, in many cases associated with anomalous branching of the bronchial tree. The bridging bronchus has been well described and has been associated with both left-sided obstructive lesions and a sling-like left pulmonary artery. We describe a similar, though distinct airway anomaly, the “braided bronchus,” associated with a bridging bronchus in a child with coarctation of the aorta and recurrent atelectasis. Methods used to delineate the “braided bronchus” are described. Pediatr Pulmonol. 1998; 25:348–351. © 1998 Wiley-Liss, Inc.     

Financial Analysis of Pediatric Resident Physician Primary Care Longitudinal Outpatient Experience

Objective

To determine whether residency training represents a net positive or negative cost to academic medical centers, we analyzed the cost of a residency program and clinical productivity of residents and faculty in an outpatient primary care practice with or without residents.

A review of evidence on the reliability and validity of Minimum Data Set data

This paper reviews the reliability and validity of the Minimum Data Set (MDS) assessment, which is being used increasingly in Canadian nursing homes and continuing care facilities. The central issues that surround the development and implementation of a standardized assessment such as the MDS are presented, including implications for health care managers in how to approach data quality concerns. With other sectors such as home care and inpatient psychiatry using MDS for national reporting, these issues have importance in and beyond residential care management.     

Epstein-Barr virus latent membrane protein-1 oncogene deletions: correlations with malignancy in Epstein-Barr virus--associated lymphoproliferative disorders and malignant lymphomas

LMP-1, an Epstein-Barr viral (EBV) latency protein, is considered a viral oncogene because of its ability to transform rodent fibroblasts in vivo and render them tumorigenic in nude mice. In human B cells, EBV LMP-1 induces DNA synthesis and abrogates apoptosis. LMP-1 is expressed in EBV-transformed lymphoblastoid cell lines, nasopharyngeal carcinoma (NPC), a subset of Hodgkin's disease (HD), and in EBV-associated lymphoproliferative disorders (EBV-LPDs). Recently, focused deletions near the 3' end of the LMP-1 gene (del-LMP-1, amino acids 346-355), in a region functionally related to the half-life to the LMP-1 protein, have been reported frequently in human immunodeficiency virus (HIV)- associated HD (100%) and EBV+ Malaysian and Danish peripheral T-cell lymphomas (100%, 61% respectively), but less frequently in cases of HD not associated with HIV (28%, 33%) and infectious mononucleosis (33%). To further investigate the potential relationship of del-LMP-1 to EBV- LPDs associated with immunosuppression or immunodeficiency, we studied 39 EBV-associated lymphoproliferations (10 benign, 29 malignant) from four distinct clinical settings: posttransplant (4 malignant, 1 reactive); HIV+ (18 malignant, 2 reactive); nonimmunodeficiency malignant lymphoma (ML) (7 cases); and sporadic EBV infection with lymphoid hyperplasia (7 cases). The presence of EBV within lymphoid cells was confirmed by EBV EBER1 RNA in situ hybridization or by polymerase chain reaction (PCR) analysis. EBV strain type and LMP-1 deletion status were determined by PCR. EBV strain types segregated into two distinct distributions: HIV+ (9 A; 11 B) and non-HIV (19 A, 0 B), consistent with previous reports. Overall, del-LMP-1 were found in 1 of 5 (20%) Burkitt lymphomas (BL); 17 of 24 (71%) aggressive non- Hodgkin's lymphoma (agg-NHL), and 2 of 10 (20%) reactive lymphoid proliferations. Of the agg-NHLs, del-LMP-1 were present in 4 of 4 PT-ML (100%); 10 of 15 HIV+ ML (67%); and 3 of 5 nonimmunodeficiency malignant lymphoma (ML, 60%). A total of 2 of 7 (28%) sporadic EBV- associated lymphoid hyperplasias contained a del-LMP-1. All del-LMP-1 were identical by DNA sequence analysis. No correlation was identified between the presence of del-LMP-1 and the EBV strain type observed. The high incidence of del-LMP-1 observed in agg-NHLs (71%), in contrast to the relatively low incidence observed in reactive lymphoid proliferations (28%), suggests that the deleted form may be preferentially selected in lymphomatous processes. All posttransplant agg-NHLs contained a del-LMP-1, and a similar frequency of del-LMP-1 was observed in both HIV-associated ML (66%) and nonimmunodeficiency ML (60%), suggesting that impairment of immune function alone is not a requirement for the expansion of malignant cells infected by EBV stains containing the deleted LMP-1 gene.     

Strategies for improving fixation of femoral components in total hip arthroplasty

As strategies are considered for improving fixation of femoral components in total hip arthroplasty (THA), one is challenged to exceed the standard set by contemporary cement procedures. However, despite the improved ten- to 15-year clinical results anticipated with current cementing techniques, the limited fatigue strength of polymethylmethacrylate warrants continued investigation of alternative systems, particularly for younger patients and in revision arthroplasty. Design considerations for femoral stems for cementless THA include (1) initial mechanical stability afforded by the stem shape, (2) strength and stiffness of the stem, and (3) surface features relating to biocompatibility and attachment to bone. In one approach a fit-and-fill algorithm has been implemented to design stems that maximize contact between prosthesis and cortex in priority areas to achieve stability. Titanium is recommended for the fabrication of such stems because of its corrosion resistance, its biocompatibility, and its modulus, which is lower than that of cobalt-chromium alloy. Long-term fixation of these implants will be dependent upon the maintenance of normal strain patterns in the host bone. Achievement of this goal will require additional strategies that combine optimal fit and optimal material properties of the prosthesis.