[The Stark family, dentists for 75 years]. / De familie Stark, tandmeesters en tandartsen.

In the Netherlands there are a few families that have been active from generation to generation in the profession of dentist. Although this is not the case with the Stark family, as many as 12 members of the family have been in the dental profession over a period of 75 years. In addition, a few were also very active outside dentistry, with the greatest example being the painter and toothpaste manufacturer Elias Stark (1849-1933).

[Tooth-masters and their advertisements in Dutch Newspapers from 1691 till 1876]. / Tandmeesters en hun advertenties in Nederlandse kranten van 1691 tot 1876.

By means of newspaper advertisements, we can paint a picture of tooth mastership in the 17th,18th, and 19th century The first known advertisement about dentistry, written by Dorothea Bokstal, appeared in 1691 in two Dutch newspapers (in Haarlem and Amsterdam). She claimed to have an understanding of the Science of Teeth. The first tooth-master advertised himself in 1724, but the many advertisements by Lehman Joseph and his descendants in the 18th century were the ones that gave us real insight into the way that tooth masters worked, the problems they faced and when they were allowed to practice somewhere. After 1813 (the French period) a tooth master needed to prove his/her skills. And the invention of new materials and instruments made more treatments possible. The introduction of the medical laws by Thorbecke caused a setback, but the introduction of the State Exam in 1876 brought the tooth masters further knowledge in their field.

Utilizing weak pump depletion to stabilize squeezed vacuum states.

We propose and demonstrate a pump-phase locking technique that makes use of weak pump depletion (WPD) - an unavoidable effect that is usually neglected - in a sub-threshold optical parametric oscillator (OPO). We show that the phase difference between seed and pump beam is imprinted on both light fields by the non-linear interaction in the crystal and can be read out without disturbing the squeezed output. In our experimental setup we observe squeezing levels of 1.96 ± 0.01 dB, with an anti-squeezing level of 3.78 ± 0.02 dB (for a 0.55 mW seed beam at 1064 nm and 67.8 mW of pump light at 532 nm). Our new locking technique allows for the first experimental realization of a pump-phase lock by reading out the pre-existing phase information in the pump field. There is no degradation of the detected squeezed states required to implement this scheme.

Sevoflurane therapy for life-threatening asthma in children.

BACKGROUND: Asthma is a common disease in children and often develops early in life. This multicentre retrospective case series describe the use and effectiveness of sevoflurane inhalation therapy in a series of children with severe asthma in the paediatric intensive care unit (PICU). METHODS: Seven children ranging from 4 to 13 yr of age admitted to the PICU of two tertiary care hospitals in the Netherlands were included. They all were admitted with the diagnosis of severe asthma requiring invasive mechanical ventilation and were treated with sevoflurane inhalation therapy. RESULTS: The median (range) Pco2 level at the start, after 2 h, and at the end of sevoflurane treatment were 14 (5.1-24.8), 9.8 (5.4-17.0), and 6.2 (4.5-11.4) kPa (P=0.05) while the median (range) pH was 7.02 (6.97-7.36), 7.18 (7.04-7.35), and 7.43 (7.15-7.47) kPa (P=0.01), respectively. The median (range) peak pressure values declined from 30 (23-56) to 20.4 (14-33) cm H2O (P=0.03). No severe adverse effects besides hypotension, with sufficient response to norepinephrine treatment, were seen. CONCLUSIONS: Sevoflurane inhalation corrects high levels of Pco2 and provides clinical improvement in mechanically ventilated children with life-threatening asthma who fail to respond to conventional treatment.

Phagocytosis of Mycobacterium ulcerans in the course of rifampicin and streptomycin chemotherapy in Buruli ulcer lesions.

BACKGROUND: Infection with Mycobacterium ulcerans involves a devastating skin disease called Buruli ulcer (BU). Currently, dual therapy with rifampicin and streptomycin (R/S) for 8 weeks as well as surgery are the standard treatments. OBJECTIVES: To elucidate the processes taking place in BU lesions in the course of chemotherapy we performed an in-depth histological analysis of lesions after 4 weeks of R/S treatment and compared results with findings in untreated lesions and lesions treated for 8 weeks. METHODS: Tissue specimens were collected from patients who had no treatment and from patients after 4 and 8 weeks of R/S treatment. The main features evaluated were local immune responses, histopathological alterations and bacterial distribution. RESULTS: After 4 weeks of R/S treatment we observed a large proportion of mycobacteria inside macrophages, occasionally forming globus-like aggregations. While distinct bands of inflammatory leucocytes surrounded the necrotic core in an ulcer and early granuloma formation was apparent in the healthy-appearing margins, acute cellular infiltration covering the whole lesion had developed in a nodular lesion. In contrast, ulcerative lesions after 8 weeks of chemotherapy showed intra- and extracellular bacterial debris as well as the presence of extensive chronic infiltrates forming huge granulomas. CONCLUSIONS: R/S treatment of BU results in a rapid onset of local cellular immune responses associated with phagocytosis of the extracellular M. ulcerans. This may be related to declining levels of the macrolide toxin mycolactone in the tissue, thus leading to an enhanced chemotherapy-induced clearance of the infection.

Dangerous and life-threatening drugs - practical lessons from the long QT syndrome.

The long QT syndrome (LQTS) is caused by delayed cardiac repolarisation and may be associated with ventricular arrhythmias (torsades de pointes) and sudden death. The congenital LQTS is caused by mutations in any one of many genes coding for ion channels responsible for cardiac repolarisation. The acquired LQTS is much more common and may be associated with various metabolic conditions, acquired heart disease or drugs. The apparent idiosyncratic development of QT prolongation under these circumstances may well expose a much larger population with silent genetic mutations. Attention has focused on the growing list of drugs implicated in the causation of the syndrome and this has led to the withdrawal of some drugs and new guidelines for the pre-clinical and clinical testing of new drugs. Clinicians should be aware of the drugs that may cause this syndrome and its potentially fatal arrhythmias, as well as the conditions that make patients more vulnerable. Patients should be made aware of the risk of drug interactions and precautions when prescribed these drugs. Adverse drug effects suggestive of cardiac arrhythmias should be reported to drug regulatory authorities. The LQTS has vastly expanded our knowledge of the molecular and genetic basis of cardiac repolarisation and arrhythmogenesis and its clinical significance is increasing.

Sizing of atrial septal defects to predict successful closure with transcatheter cardioSEAL device.

We conducted this retrospective study to compare methods for measuring atrial septal defects and to identify factors affecting echocardiographic measurement of such defects before transcatheter closure with the CardioSEAL'Septal Occluder. We reviewed the records of patients considered for device placement at our institution from January 1997 to April 1999. Atrial septal defect size was measured by transthoracic and transesophageal echocardiography; the stretched diameter was measured during catheterization by fluoroscopy and transesophageal echocardiography. The stretched-diameter fluoroscopic measurement was used for device size selection. Analysis of variance was used to calculate the effect of size, age, and size-by-age interaction. Thirty-one patients (3.3 to 72 years of age) underwent transthoracic and transesophageal echocardiography One patient was excluded from catheterization because of a 25-mm septal defect as indicated by transesophageal echocardiography (our maximum diameter, 15 mm). Thirty patients underwent transcatheter stretched-diameter sizing; 5 were excluded from device implantation because of defects >20 mm by stretched-diameter fluoroscopy (4) or septal length insufficient for device support (1). Implantation was successful in 23/25 patients; 2/23 had a residual shunt. In patients with available results (26/30), the stretched diameter was the same whether measured by stretched-diameter fluoroscopy or transesophageal echocardiography (P=0.007 R square=0.963). Compared with stretched-diameter fluoroscopy, precatheterization transthoracic and transesophageal echocardiography underestimated defect size by a mean of 22% and 13.2%, respectively. When data from those same tests were compared in defects of < or =0 mm and > 10 mm, transthoracic and transesophageal echocardiography were reliable predictors (P=0.003 and P=0.05, respectively) of stretched-diameter size in defects < or =0 mm.

Redefinition: coping with normal results from predictive gene testing for neurodegenerative disorders.

The purpose of this qualitative study was to describe the psychosocial impact and coping processes of normal (negative) results from predictive testing for an inherited neurodegenerative disease. Ten adults with normal results of predictive testing for the Huntington disease (HD) or the Pallido-Ponto-Nigral Degeneration (PPND) gene mutation participated in semi-structured interviews 1 month after receiving results, and seven of these participants were interviewed 6 months later. The major theme of Redefinition was derived using Knafl and Webster's analysis method (1988). People who received normal gene results experienced loss of former beliefs about themselves and developed new self definitions, relationships with family, and roles in society. This coping process evolved from a personal focus at 1 month to a broader future perspective at 6 months after testing. Identifying components of the redefinition process may be an important consideration in planning interventions to promote coping with normal gene results in persons within at-risk families.

Psychosocial impact of predictive testing for Huntington disease on support persons.

Although a support person is required by many centers during the predictive testing protocol for Huntington disease (HD), little is known about the psychosocial impact of predictive testing on persons serving in this role. Eighteen adults who were support persons during predictive HD testing in one HD testing center completed a semi-structured interview to describe their experiences. Participants also completed the Impact of Events Scale (IES) to assess perceptions of emotional distress regarding predictive testing and the State Anxiety Scale of the State Trait Anxiety Inventory (STAI) to assess anxiety regarding the interview. State anxiety scores were similar to normative values for working adults. Although support persons for individuals with a positive gene test scored higher on all measures of the IES than those who were support persons for persons with negative gene mutation results, these differences were not statistically significant. Support persons identified aspects of the protocol that did not fit their needs, perceived the testing process as extending into subsequent caregiving responsibilities when the test was positive, and were uninformed regarding specific caregiving issues for family members with the gene mutation. The impact of the testing experience appeared to be most intense for those support persons who were at-risk offspring of probands. Findings suggest that individual assessment of support person needs may allow more focused counseling of support persons during predictive genetic HD testing. Collaboration with health care providers may facilitate symptom management following testing.

Genetic testing and mental health: the model of Huntington disease.

Genetic aspects of mental health disorders are being identified through human genome and family research. Gene discovery makes diagnostic and presymptomatic testing possible. The discovery of a gene mutation for Huntington Disease (HD) enables at-risk persons to request presymptomatic genetic testing. When HD genetic testing is offered through HD testing centers, a multi-visit protocol is followed in which education and counseling are provided for persons considering the option to have HD gene testing. A case study illustrates the clinical and ethical issues regarding privacy and disclosure as well as the personal and family consequences of gene mutation knowledge. Analysis of the impact of genetic knowledge on persons being tested for HD provides a model for the integration of emerging genetic information into mental health nursing practice for other mental health disorders.

Adults seeking presymptomatic gene testing for Huntington disease.

PURPOSE: To describe the expectations of those seeking presymptomatic gene testing for Huntington disease (HD). Identification of the gene for HD makes it possible to conduct testing to determine if a healthy person with a family history of HD has a mutation in this gene. Presymptomatic gene testing reveals the likelihood that a person will develop an inherited disease in the future. Understanding expectations allows for more complete assessment and counseling before presymptomatic gene testing for genetic diseases. DESIGN: Descriptive qualitative. The population was people with a family history of HD. The sample was 17 asymptomatic adults with a positive family history of HD who requested presymptomatic gene identification at one tertiary genetic counseling program, 1995 to 1996. METHODS: Semi-structured interviews concerning expectations of adults seeking presymptomatic genetic testing were conducted by telephone. Interviews occurred after the individuals had requested presymptomatic gene identification but before results were reported. Content analysis was used to identify the expectations and questions of those who had decided to seek presymptomatic testing. FINDINGS: Common expectations included anticipating relief from uncertainty, hoping to plan for their future health care and life decisions, wanting to know if their children were at risk of developing HD, anticipating loss of family support from relatives, expecting relief from self monitoring, venturing into the unknown, and planning for disclosure. Participants attempted to avoid their loss of genetic privacy by withholding the decision to seek testing from their primary care providers. CONCLUSIONS: Participants seeking presymptomatic HD gene testing consider the effect of gene identification on themselves and their families. A desire to limit insurance or employment discrimination contributes to subjects not seeking input from health care providers in their decision making.

The impact of Alzheimer disease genetics on expert and advanced gerontological nursing practice.

Alzheimer disease (AD), a progressive neurodegenerative disorder, is the most common cause of dementia in the United States, affecting as many as 4 million people. Extensive research is under way to identify environmental and genetic risk factors for this complex disease. Currently, four genes are associated with an increased risk for AD: the amyloid precursor protein gene on chromosome 21, the Presenilin I gene on chromosome 14, the Presenilin II gene on chromosome 1, and the apolipoprotein E gene on chromosome 19. Expert and advanced practice gerontological nurses are faced with new challenges as a result of these gene discoveries. Gerontological nurses should assess for relevant environmental and genetic risk factors; obtain comprehensive family health histories recorded as pedigrees; integrate genetic information into diagnosis, intervention, and evaluation strategies; initiate and coordinate referrals to genetic specialists; and provide ongoing emotional and decision-making support for patients and families experiencing AD.

Genetic testing in Alzheimer's disease. Benefits risks and public policy.

1. Genetic research is providing new information about the structure and function of genes associated with diseases such as Alzheimer's disease. 2. The ability to perform genetic tests to diagnose or predict disease often exists before the ability to prevent or treat disease. 3. Genetic tests are associated with both benefits and risks, which likely will apply to the Alzheimer's disease population. 4. Safe and effective tests, laboratories of assured quality, competent providers, assured privacy of genetic information, and informed consumers are important prerequisites to the successful integration of genetic tests into health care services.

Prominent venous valves in hypoplastic right hearts.

To investigate the hypothesis that embryologic abnormalities in the venous valves may be associated with abnormal cardiac development, we examined the right atrial morphologic characteristics in 20 hearts with underdevelopment of the right heart and 17 normal hearts. In the study group, 16 (80%) of the patients had significantly enlarged eustachian valves, one (5%) was slightly enlarged, and three (15%) were smaller than expected. Five (25%) had cor triatriatum dexter. In comparison, eustachian valves in the control specimens were prominent in only one (6%), normal in five (29%), and almost absent in eight (47%). The thebesian valve was also more prominent in the study cohort when compared with controls (p < 0.05). No other morphologic features of the right atrium analyzed in this study differed from those found in normal specimens. We speculate that failure of the venous valves to regress appropriately may create abnormalities in fetal circulation that predispose the fetus to maldevelopment of the right heart structures.

Acute pulmonary toxicity in an infant from intravenous amiodarone.

Intravenous amiodarone is an effective treatment for supraventricular and ventricular tachyarrhythmias. We report a case of acute pulmonary toxicity in an infant from intravenous amiodarone and describe the clinical evaluation and laboratory studies leading to the diagnosis.

Benefits and burdens of genetic carrier identification.

This qualitative study examined experiences of adults requesting genetic-carrier testing for four autosomal-recessive and X-linked-recessive disorders. The sample consisted of 34 adults with a positive family history or membership in an ethnic group at risk for the inherited disorder. A semistructured interview guide was used to collect data during an interview 1 month after receipt of test results. Noncarriers experienced benefits of emotional relief and freedom to move ahead with reproductive planning. Carriers experienced burdens of sadness and loss of reproductive expectations. Some subjects in both groups experienced difficulty disclosing results to selected family members and expressed concerns regarding disclosure of testing to insurance providers.