Symptomatic adult polycystic liver disease in a young woman.

Adult polycystic liver disease is an entity that is striking for the presence of extraordinary hepatomegaly. Very rarely do patients under 40 years of age experience problems and only a minority of middle-aged patients manifest symptoms due to organ compression or biliary tree obstruction. The clinical course is usually benign, and no specific therapy is required. The onset of right upper quadrant pain, back pain, and shortness of breath prompted evaluation of a 32-year-old woman with progressive hepatomegaly. CT scan revealed a massive cyst-riddled liver with posterior displacement of the spleen, compression of the pancreas, and renal cysts. Prothrombin time, albumin, and liver enzyme values were normal except for a mildly elevated gamma-glutamyl transpeptidase.     

Surface movement errors in shank kinematics and knee kinetics during gait

The movement of surface mounted targets (SMT) on a shell at the mid-shank and of bone mounted targets attached to the distal shank using a Percutaneous Skeletal Tracker (PST) were simultaneously measured during free-speed walking of three adult subjects having different body types. Surface movement errors in shank kinematic estimates were determined by expressing the segmental motion derived from the SMT relative to the PST-based segment coordinate system (SCS) located at the segment center of gravity. The greatest errors were along and around the shank longitudinal axis, with peak magnitudes of 10 mm of translation and 8° of rotation in one subject. Estimates of knee joint center locations differed by less than 11 mm in each SCS direction. Differences in estimates of net knee joint forces and moments were most prominent during stance phase, with magnitudes up to 39 N in the shank mediolateral direction and 9 N.m about the mediolateral axis. The differences in kinetics were primarily related to the effect of segment position and orientation on the expression of joint forces and on the magnitude and expression of joint moments.     

Familial systematized epidermal nevus syndrome

Epidermal nevi are typically congenital but rarely familial. We report on a family in which 3 relatives have systematized epidermal nevi. The propositus also has evidence of a hemangioma and a hemangioendothelioma. Peripheral blood and skin fibroblast karyotypes of the propositus did not show evidence of mosaicism. Epidermal nevi have been associated with nondermatologic pathology, involving the nervous, vascular, and skeletal systems in sporadic cases. This report demonstrates that nondermatologic pathology can be also be associated with systematized epidermal nevi in a familial setting. The apparent skipping of generations may be explained by autosomal dominant inheritance with decreased penetrance. © 1992 Wiley-Liss, Inc.     

Adolescent Immunization: Focus on Implementation

ABSTRACT: On March 11–12, 1996, a workshop on how to implement new adolescent immunization (AI) recommendations was held in Atlanta, Ga. Sponsored by the Centers for Disease Control and Prevention, it was a collaborative effort of the National Immunization Program, the Division of Adolescent and School Health/National Center for Chronic Disease Prevention and Health Promotion, and the Hepatitis Branch/National Center for Infectious Diseases. The workshop brought together organizations and individuals interested in adolescent health and immunizations so they could address how new Al recommendations can be implemented most effectively. This article offers an overview of their discussions and suggestions, including issues of cooperation, education, legislation, and Al program development among health provider organizations, health departments, schools, community groups and various other agencies relating to adolescent health services.     

Surgically debulked malignant pleural mesothelioma: Results and prognostic factors

Background: We analyzed morbidity and mortality, sites of recurrence, and possible prognostic factors in 95 (78 male, 17 female) patients with MPM on phase I–III trials since 1990. A debulking resection to a requisite, residual tumor thickness of ≤ 5 mm was required for inclusion. Methods: Preoperative tumor volumes were determined by three-dimensional reconstruction of chest computerized tomograms. Pleurectomy (n=39) or extrapleural pneumonectomy (EPP; n=39) was performed. Seventeen patients could not be debulked. Preoperative EPP platelet counts (404,000) and mean tumor volume (491 cm³) were greater than that seen for pleurectomy (344,000, 114 cm³). Results: Median survival for all patients was 11.2 months, with that for pleurectomy 14.5 months, that for EPP 9.4 months, and that for unresectable patients 5.0 months. Arrhythmia (n=14; 15%) was the most common complication, and there were two deaths related to surgery (2.0%). Tumor volume of >100 ml, biphasic histology, male sex, and elevated platelet count were associated with decreased survival (p<0.05). Both EPP and pleurectomy had equivalent recurrence rates (27 of 39 [69%] and 31 of 39 [79%], respectively); however, 17 of 27 EPP recurrences as opposed to 28 of 31 pleurectomy recurrences were locoregional (p₂=0.013). Conclusions: Debulking resections for MPM can be performed with low operative mortality. Size and platelet count are important preoperative prognostic parameters for MPM. Patients with poor prognostic indicators should probably enter nonsurgical, innovative trials where toxicity or response to therapy can be evaluated. Presented at the 49th Annual Cancer Symposium of The Society of Surgical Oncology, Atlanta, Georgia, March 21–24, 1996.     

Project 2000: perceptions of the philosophy and practice of nursing: shifting perceptions—a new practitioner?

This is the first of two papers which address aspects of the findings of a large scale study commissioned by the English National Board which set out to examine the impact of Project 2000 on perceptions of the philosophy and practice of nursing. The findings presented here suggest that there have been fundamental shifts in perceptions of the nature and discipline of nursing. Students and diplomates of the course perceive themselves as knowledgeable doers, with their practice well grounded in theory and research. They value the interpersonal skills teaching and place the patient firmly at the centre of care delivery, viewing the patient holistically and being prepared to be fierce patient advocates where necessary. It is difficult to determine the extent to which these shifts can be attributed to the Project 2000 course, although the Project 2000 approach to education appears to be an important factor.     

Gender differences in young adults' beliefs about sunscreen use.

This study employs focus group methodology to explore gender differences in sunscreen use. Guided by the theory of reasoned action, males and females were found to differ on each of the following constructs: behavior, behavioral beliefs, and normative beliefs. Males and females differed in their sunscreen use, with females adopting a more preventive style of sunscreen use and males a more reactive style. Males and females differed in their salient beliefs that motivated their sunscreen use, many of which were related to traditional American gender roles. In addition, although males and females were aware of both positive and negative sources of normative beliefs regarding sunscreen use, females received more encouragement from their mothers and peers than males. Findings are discussed in terms of their implications for the design of future interventions.     

Multiplex PCR analysis of in vivo-arising deletion mutations in the hprt gene of human T-lymphocytes

A multiplex polymerase chain reaction (PCR) procedure was adapted for the rapid and efficient evaluation of deletions of the hypoxanthine guanine phosphoribosyltransferase (hprt) gene in human T-lymphocytes. The hprt clonal assay was used to isolate in vivo-arising hprt-deficient T-cells from six healthy males. Mutant frequencies ranged from 9-27 × 10^?6. Simple crude cellular extracts from 223 mutants were analyzed for hprt gene deletion. Sixteen (7.2%) were found to be due to total gene deletion and 22 (9.9%) were due to partial gene deletion. The relatively high frequency of total gene deletions was caused by replicate isolates of a single mutational event as shown by single-strand conformation polymorphism (SSCP) analysis of rearranged T-cell receptor (TCR)-γ genes. Eighteen of the 22 partial hprt gene deletion mutants were determined to be of independent origin based on a unique hprt mutation or SSCP-TCR-γ pattern. One-half (9/18) of the partial deletion mutants involved all or part of exon 4 alone, suggesting that this region of the hprt gene is prone to deletion. The small deletions effecting exon 1 (1 mutant), exon 2 (2 mutants), and exon 4 (6 mutants) would not have been detected by conventional Southern blot analysis and may represent a new, previously unrecognized class of mutations. The ready isolation of such intragenic deletions will allow the characterization of breakpoint junctions and may provide insights into the important processes of DNA breakage and rejoining. © 1994 Wiley-Liss, Inc.