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PubMed2005

An exceptional Albanian family with seven children presenting with dysmorphic features and mental retardation: maternal phenylketonuria.

BACKGROUND: Phenylketonuria is an inborn error of amino acid metabolism which can cause severe damage to the patient or, in the case of maternal phenylketonuria, to the foetus. The maternal phenylketonuria syndrome is caused by high blood phenylalanine concentrations during pregnancy and presents with serious foetal anomalies, especially congenital heart disease, microcephaly and mental retardation. CASE PRESENTATION: We report on an affected Albanian woman and her seven children. The mother is affected by phenylketonuria and is a compound heterozygote for two pathogenetic mutations, L48S and P281L. The diagnosis was only made in the context of her children, all of whom have at least one severe organic malformation. The first child, 17 years old, has a double-chambered right ventricle, vertebral malformations and epilepsy. She is also mentally retarded, microcephalic, exhibits facial dysmorphies and small stature. The second child, a girl 15 years of age, has severe mental retardation with microcephaly, small stature and various dysmorphic features. The next sibling, a boy, died of tetralogy of Fallot at the age of three months. He also had multiple vertebral and rib malformations. The subsequent girl, now eleven years old, has mental retardation, microcephaly and epilepsy along with facial dysmorphy, partial deafness and short stature. The eight-year-old child is slightly mentally retarded and microcephalic. A five-year-old boy was a premature, dystrophic baby and exhibits mental retardation, dysmorphic facial features, brachydactyly and clinodactyly of the fifth finger on both hands. Following a miscarriage, our index case, the youngest child at two years of age, is microcephalic and mentally retarded and shows minor facial anomalies. All children exhibit features of phenylalanine embryopathy caused by maternal phenylketonuria because the mother had not been diagnosed earlier and, therefore, never received any diet. CONCLUSION: This is the largest family suffering from maternal phenylketonuria reported in the literature. Maternal phenylketonuria remains a challenge, especially in woman from countries without a neonatal screening program. Therefore, it is mandatory to be alert for the possibility of maternal phenylketonuria syndrome in case of a child with the clinical features described here to prevent foetal damage in subsequent siblings.

Abnormalities, Multiple↗
PubMedDate not supplied

Promoting choice: lessons from managed Medicaid.

Drawing on the education, enrollment, and assignment experiences of seven states with mandatory Medicaid managed care programs, this paper finds that the vast majority of enrollees will choose their own health plan if the system is explicitly designed with this in mind (as in Minnesota and Oregon). These experiences provide lessons on ways to 1) align program design with state priorities; (2) increase the level of choice (by coordinating enrollment and eligibility processes, broad-based educational strategies, and personalized attention); (3) improve the quality of choice; and (4) design state contracting processes to support choice and continuity of care.

Community Participation↗
PubMed2001

Dental device-associated problems: an analysis of FDA postmarket surveillance data.

BACKGROUND: The authors provide an analysis of dental device adverse event reports collected through the U.S. Food and Drug Administration's, or FDA's, mandatory and voluntary reporting programs between Aug. 1, 1996, and June 30, 1999. METHODS: This study includes an analysis of the total number of dental device adverse events reported during the study period and uses descriptive statistics to depict reporters' occupations, types of adverse events (deaths, injuries, malfunctions), device categories, device problems and patient problems. RESULTS: A total of 272,241 device reports were received during the 35-month study period, 28,555 (10.5 percent) of which involved dental devices. Within these reports, two deaths (0.007 percent), 18,406 injuries (64.4 percent) and 9,942 device malfunctions (34.8 percent) were reported. The most commonly reported dental devices were endosseous implants, which represented more than 90 percent of all dental device reports. Most reports (84.1 percent) provided the reporter's occupation, and the most frequently cited occupation was dentist (76.3 percent), followed by dental assistant (4.2 percent). CONCLUSIONS: Dentists and dental staff members are a vital link in the FDA's adverse event reporting system and are encouraged to report device problems to the FDA MedWatch program.

Data Collection↗
PubMed2002

Skin-test screening and tuberculosis transmission among the homeless.

We describe the implementation of a mandatory tuberculosis (TB) screening program that uses symptom screening and tuberculin skin testing in homeless shelters. We used the results of DNA fingerprinting of Mycobacterium tuberculosis isolates to evaluate the effect of the program on TB incidence and transmission. After the program was implemented, the proportion of cases among homeless persons detected by screening activities increased, and the estimated TB incidence decreased from 510 to 121 cases per 100000 population per year. Recent transmission, defined by DNA fingerprinting analysis as clustered patterns occurring within 2 years, decreased from 49% to 14% (p=0.03). Our results suggest that the shelter-based screening program decreased the incidence of TB by decreasing its transmission among the homeless.

Colorado↗
PubMed1984

Hospital returns to social security but retains alternative benefits plan.

When passage of the Social Security Amendments of 1983 required Holy Redeemer Hospital to return to the Social Security system on Jan. 1, 1984, the hospital examined alternatives to nonprofit hospitals' mandatory participation in the program. With a consortium of hospitals, it launched an unsuccessful lobbying effort to convince Congress to accept other options: deferring the return for several years, exempting hospitals that had left the system, or "grandfathering" nonprofit hospitals' employees. By Oct. 31, 1983, the hospital decided to forgo efforts to amend the legislation and made preparations for returning to the system. Employees were notified that they could continue to participate in the alternate plan (a tax-deferred annuity) or leave their account inactive until withdrawal at retirement. The plan's provisions that allowed withdrawals only under extraordinary circumstances were amended to permit withdrawals at any time. In addition, the administration introduced flexible benefits--a "cafeteria plan"--to allow participants to choose among certain taxable and nontaxable benefits that best fit each individual's life-style.

Hospital Bed Capacity, 300 to 499↗
PubMed1982

Union activity in hospitals: past, present, and future.

Between 1970 and 1980, the percentage of hospitals with one or more collective bargaining contracts increased from 15.7 percent to 27.4 percent. A substantial amount of variation exists in the extent of unionism on the basis of hospital ownership, bed size, and location. Employees are more likely to organize when hospitals in the State are regulated by a mandatory rate-setting program. Unions raise hospital employee's wages--a modal estimate for RNs is about 6 percent; the corresponding figure for nonprofessional employees is about 10 percent. Growth of union activity in hospitals has generally not been a major contributor to hospital wage inflation, and less than 10 percent of the increase in real (relative to the Consumer Price Index) spending for hospital care that occurred during the 1970s can be attributed to union growth. We project that between 45 and 50 percent of all hospitals will have at least one union by 1990.

Collective Bargaining↗
PubMed2003

Dental population health measures: supporting Army transformation.

General Eric K. Shinseki, Chief of Staff of the Army, has initiated the transformation of the Army with the goals of making the force more lethal, agile, and versatile. A key component to the transformation is deploying healthier soldiers that will result in the ability to decrease the amount of deployed medical support. This article describes two new population health measures the U.S. Army Dental Command is implementing to support the transformation. The new program will make it mandatory for dental providers to assess caries and tobacco risk at all active duty soldiers' annual dental examinations. These measures will allow the dental personnel to identify who is at risk for future disease and then to plan appropriate prevention strategies for those individual soldiers. These aggregate corporate data will allow dental leaders to target prevention services to the units with a high percentage of tobacco users. These new measures and the resultant prevention strategies can increase the readiness of our forces and can result in a healthier and mission-ready force.

Dental Caries↗
PubMedDate not supplied

Canadian experience in cytology proficiency testing.

The mandatory Laboratory Proficiency Testing Program (LPTP) of the province of Ontario as applied to cytology laboratories is briefly described. LPTP evaluates reporting of test slides to identify laboratories that have deficiencies. Such laboratories receive an on-site visit, followed by recommendations on means of improvement (especially educational) and subsequent monitoring. Most of the 18 cytology laboratories visited to date have shown an improved level of performance on later tests.

Cytodiagnosis↗
PubMed1993

[Paragliding accidents--a prospective analysis in Swiss mountain regions].

During the period from 1.1 to 31.12.90, 86 injuries associated with paragliding were analysed in a prospective study in 12 different Swiss hospitals with reference to causes, patterns, and frequencies. Spine injuries (36%) and lesions of the lower extremities (35%) were diagnosed most frequently. Surprisingly no neurological complications occurred, which is possibly explained by the solitary axial trauma. In 15 cases very severe malleolar fractures required surgical intervention. One accident was fatal due to a lung rupture. 60% of all accidents happened during the landing phase, 26% at launching and 14% at flight. Half of the pilots were affected in their primary training course. Most accidents were due to an in-flight error of judgement, such as incorrect estimation of wind conditions and a choice of unfavourable landing sites. In contrast to early reports of hang-gliding injuries, only one accident was due to an equipment failure, namely a ruptured steering line. In more than a third of all accidents, the used paraglider was not in correct correlation with the pilot's weight and experience. Inspired by the desire for a long flight, gliders of too large surface-areas were often used, leading to a more unstable flight. To reduce the frequency of paragliding injuries, an accurate choice of equipment and increased attention to environmental factors is mandatory. Furthermore education-programs should focus more on intensifying the pilot's mental and practical skills.

Adult↗
PubMed1996

Commingled grazing as a risk factor for trichomonosis in beef herds.

OBJECTIVE: To evaluate commingled grazing on public lands as a risk factor for Tritrichomonas foetus infection in beef herd bulls. DESIGN: Case-control study. SAMPLE POPULATION: Based on 1994 data from the mandatory Idaho bull testing program, all 65 infected herds (case herds), 78 randomly selected test-negative herds that tested < 10 nonvirgin bulls, and 81 randomly selected test-negative herds that tested > or = 10 nonvirgin bulls (control herds). PROCEDURE: Managers of government-owned grazing lands in Idaho identified producers who had grazing permits for an allotment under their jurisdiction in 1993 and for that allotment recorded the number of animals the producer was permitted to graze on the allotment, the number of herds with grazing permits, the total number of animals permitted, and the dates on which grazing began and ended. The number of bulls tested, number of times tested, and test results were collated from the testing database. RESULTS: The relative sensitivity of bacterial culture of preputial smegma was 81%. The attributable fraction of T foetus infection associated with commingled grazing was 33%, and the odds ratio of infection was 9.0 for herds commingled with > or = 14 other herds. The total number of animals permitted on an allotment, the use of public lands, and type of public lands used were not significantly associated with infection status. CLINICAL IMPLICATIONS: These results suggest that to control the spread of trichomonosis, the number of herds commingling on a grazing allotment should be minimized and commingled herds should be managed collectively.

Animal Husbandry↗
PubMed1995

Screening for HIV seropositive blood - a critical evaluation.

HIV screening of all donated blood is mandatory in India. The program instituted has many inherent lacunac like arbitrary identification of centres, absence of a guardian quality control program, criterion for test kit selection and absence of standard guidelines. This study was undertaken to evaluate the effects of frequent change of testing laboratory, change of testing staff, change of reagent kits and time of blood donation. All the donations at blood bank Safdarjang Hospital, during the year 1993, were taken for study. HBsAg and VDRL positivity were used as control/check value for similarity of donor population. Tests done during this period in three different laboratories show that the change of laboratory, change of reagent kit and the experience and training of the testing laboratory staff affect the test results, causing an increase in the number of false positive tests. This leads to destruction of donated blood which would have been otherwise fit for transfusion. It is suggested that a proper policy plan may be made and implemented in order to make the blood screening more effective.

AIDS Serodiagnosis↗
PubMed1998

[Unsolved questions of infectious endocarditis prophylaxis in outpatients practice].

AIM: To check practical feasibility of recommendations of prevention of infectious endocarditis (IE) and to provide more accurate figures on the occurrence of valvular lesions in working population. MATERIALS AND METHODS: Subacute IE manifestations have been analysed in 10 patients of a general outpatient clinic. Echocardiograms of 6209 patients under 60 years of age were studied retrospectively. RESULTS: In one-third of the patients IE developed in the presence of streptococcal and staphylococcal infection. In all the cases cardiac valve involvement was stated. In 4 cases changes in the valve were not accompanied my impairment of the valvular function. By echocardiography, valvular structure changes without valvular disease were detected 2.3 times more frequently that valvular disease. Difficulties presenting in outpatient IE prophylaxis are outlined. CONCLUSION: Advances in IE prevention will be achieved if echo-CG becomes a mandatory examination in the program of working population check-up, if early diagnosis of streptococcal ENT infection is made, if physicians of different specialties work as a team.

Aged↗
PubMedDate not supplied

Teaching nursing homes revisited. Survey of affiliations between American medical schools and long-term-care facilities.

A survey of American medical schools was conducted to examine the growth of teaching nursing home programs. Ninety percent (109/121) of schools surveyed reported affiliations with a nursing home(s) for teaching, research, and/or clinical care. Most affiliations occurred in the last six years and were typically with two or more nursing homes. They were usually 120 beds or more in size, and the majority were nonprofit or Veterans Administration units. Eighty-three percent (101/121) of medical schools had teaching programs involving nursing homes. Medical school courses that utilized nursing homes as teaching sites were largely selective or elective and were offered primarily in the senior year. Training programs involving residents were usually mandatory and mainly involved the departments of internal medicine and family practice. Fifty-five percent (66/121) of the schools had research programs involving nursing homes.

Interviews as Topic↗
PubMedDate not supplied

Remediation of low ABSITE scores.

PURPOSE: In 2000, a program was initiated to improve American Board of Surgery In-Training Exam (ABSITE) scores below the 40th percentile (PGY-1 and -2) and the 30th percentile (PGY-3 and -5) for categorical residents. The goal of this program is to maintain scores above this standard. METHODS: One faculty member was designated to meet with each resident with ABSITE scores below the standard described above. In April, individual study plans were designed with each resident, which emphasized practice questions and strategic planning for study, and the ABSITE test structure and results were reviewed. Two subsequent meetings were held between November and December to monitor progress. Emphasis was placed on minimizing the stigma of poor scores and on maximizing the efficiency of study time. If the resident scored above-standard that year, further support was not required. RESULTS: From 2000 to 2004, 12 (9.5%) ABSITE scores were below-standard, which resulted in 8 (20.5%) residents receiving program support 9 times. All but 1 program encounter resulted in above-standard scores the following year (improvement range, 16 to 65 percentile points; average, 34 points). Two residents had recurrent below-standard scores in subsequent years despite above-standard scores immediately after the program. One resident did not participate in the program, despite it being designated as mandatory. During the same interval, the ABSITE scores of residents not involved in the program decreased by an average of 3.7 percentile points per examination (improved scores 31 times; 39.2%, range 1 to 46, average 13.5, worse scores 45 times; 57%, range 1 to 65, average 15.2, and no change 3 times, 3.8%). CONCLUSIONS: An individualized program that minimizes the stigma of poor test results, the time commitment required by the residents, and maximizes the benefits of a question-based study system and the knowledge of approach to the ABSITE resulted in significant improvements in scores the next year. However, maintenance of these results needs continued evaluation because 3 residents had recurrent poor scores in subsequent years.

Accreditation↗
PubMed1985

The impact of training in behavioral pediatrics: a study of 24 residency programs.

Three groups of residency programs were compared: 11 externally funded to provide mandatory behavioral training, seven not externally funded but providing mandatory training, and six controls not requiring training. At the beginning and end of 1980-1981, 569 residents (70%) completed questionnaires assessing attitudes regarding behavioral disorders, physical disorders, and "mixed" disorders (with physical and behavioral aspects). Behavioral knowledge was tested by 60 multiple-choice questions. Virtually all significant effects of program type occurred in the PL-2 year. For three attitudinal measures, "competence in management," "ability to advise parents," and "future relevance," only Funded residents demonstrated higher change scores for both behavioral and mixed disorders, relative to physical disorders; Funded and Not Funded residents were superior to Controls regarding behavioral disorders. For "knowledge of resources" and "facility interest," Not Funded change was superior to Control. Given higher Funded ratings initially, Not Funded ratings approximated Funded ratings by the end of the year. Regarding knowledge, Funded and Not Funded residents showed greater improvement than Controls. These data suggest that: changes in attitudes and knowledge are related to mandatory training; Funded programs generate the most consistent changes in both behavioral and "mixed" disorders; and the impact of training is most evident in the PL-2 year.

Attitude of Health Personnel↗