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Biomedical subjects

A Lampe

Publications and source records attributed to A Lampe.

12 recordsLinked to original sources

Congenital muscular dystrophy with short stature, proximal contractures and distal laxity.

We report 5 cases (2 familial and 3 sporadic) who share a diagnosis of congenital muscular dystrophy (CMD) in association with short stature, proximal contractures, rigidity of the spine and distal joint laxity as well as early respiratory failure and mild to moderate mental retardation. The expression of collagen VI was confirmed to be normal on muscle biopsies of all 5 patients and in the informative family linkage to any of the three COL6 A loci was excluded. These findings extend the phenotypes within the CMD classification.

Adolescent↗

Daily psychosocial stressors and cyclic response patterns in urine cortisol and neopterin in a patient with systemic lupus erythematosus.

This study investigated the complex biochemical responses to personally meaningful everyday stressors in a patient with systemic lupus erythematosus (SLE). For this purpose, a 52 year-old woman with SLE collected her entire urine for 56 days on a 12-h basis for the determination of cortisol as well as neopterin, a cellular immune parameter. Additionally, using questionnaires, daily notes and interviews, extensive psychosocial and psychological time-series data were collected every 12 h. Cross-correlational analyses of the resulting time-series revealed that stressful incidents were associated with cyclic fluctuations in both urine cortisol and urine neopterin. Specifically, whenever the patient anticipated a moderately stressful incident, urine cortisol initially increased 24 h before the incident and then decreased 12 h before the incident. Moderate stressors not anticipated by the patient were associated with an initial increase 24 h following the incident and then with a decrease after a total of 36 h. Moreover, stressors having to do with the patient's extramarital relationship were followed initially by a decrease in urine neopterin after 36 h and then by an increase after a total of 60 h. Our findings indicate that when investigating the relationship between psychosocial stressors and biochemical activity in SLE, appropriate consideration of the data's dynamic nature may be necessary to avoid flawed conclusions.

Activities of Daily Living↗

Borjeson-Forssman-Lehmann syndrome and multiple pituitary hormone deficiency.

We describe two brothers with Borjeson-Forssman-Lehmann syndrome and the 22A-->T (Lys8X) PHF6 mutation, who presented with the symptoms and signs of multiple pituitary hormone deficiency. Biochemical investigations and radiology confirmed growth hormone (GH), thyroid stimulating hormone (TSH) and adrenocorticotrophic hormone (ACTH) as well as gonadotrophin deficiency. They were also found to have optic nerve hypoplasia. This family suggests that the BFL gene product may play an important role in midline neuro-development including the hypothalamo-pituitary axis.

Abnormalities, Multiple↗

[Quality criteria for psychosomatic consultation-liaison service].

Besides the integrated psychosomatic models in various medical specialities (e.g. Internal medicine, Obstetrics and Gynaecology or Paediatrics), specific models of cooperation in the form of consultation-liaison (C-L) service between specialised psychosomatic-psychotherapeutic departments and other medical departments play a crucial role in providing psychosomatic care to patients. The concept 'liaison' expresses a particularly close form of cooperative activity, going far beyond the classical model of consultation services. In this context, the psychosomatic consultant has not only a fixed organised presence in the provision of patient care, he/she also participates in case discussions, hospital rounds, ward discussions and continuing education programmes of the 'partner' department. Thus, the liaison service is not only patient-centred, but also team-centred. Such consultation-liaison services, in Austria, were first established at the University Hospitals. As a consequence of laws regulating psychotherapeutic care, according to which such care is to be offered in general hospitals, increasingly C-L services are becoming part of regular care provision in more and more hospitals. Consensus groups, international as well as in German-speaking countries, are currently working on establishing quality standards in the area of psychosomatic C-L services. A working group of the scientific medical representatives has recently published guidelines for the structure, process and results of quality control. A second group, of the European Association of Consultation Liaison Psychiatry and Psychosomatics, is currently working on guidelines for training consultation-liaison service providers. In the present article, an overview of these projects will be presented and the results discussed against the background of the specific health policy situation in Austria.

Austria↗

[The influence of daily psychosocial stressors and associated emotions on the dynamic course of urine cortisol and urine neopterin in systemic lupus erythematosus: Experience taken from two "integrative single-case studies"].

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by flare-ups, the cause of which is unknown. According to new stress concepts, two "integrative single-case studies" have been conducted in order to gather evidence about whether daily stressful incidents and associated emotions interfere with the dynamics of urine cortisol and urine neopterin in SLE. Patients under study collected their urine at home, for a period of at least 50 days, on a daily basis, divided into day and night urine. Additionally, patients filled out questionnaires twice a day to determine their emotional state, life style and disease activity. Each week, patients were examined clinically and interviewed to identify the past week's stressors using the Incidents and Hassles Inventory (IHI, Brown and Harris). Statistical analysis of the serial data was performed using time-series analysis according to Box and Jenkins. In both "integrative single-case studies" we were able to demonstrate that stressful incidents predicted an increase in urine neopterin 36 hours (Case 1) to 60 hours (Case 2) later (p < 0.05). Additionally, in Case 1 the neopterin levels were highly associated with stress resulting from the weekly examinations and interviews. Furthermore, in Case 2 it turned out that depending on their predictability stressful incidents were preceded by a decrease in urine cortisol 12 hours earlier or were followed by a decrease in urine cortisol 36 hours later. And finally, emotional irritation was highly correlated with the course of urine-neopterin. In Case 2 irritation led to an increase in urine neopterin 84 hours later. There were no clinical signs of SLE during both prospective studies. In conclusion, our results validate the idea of "integrative single-case studies" as a new "bio-psycho-social" approach in psychoneuroimmunology. Further studies with SLE patients as well as with healthy probands will be necessary in order to both strengthen and generalize these results.

Adult↗

Chronic pelvic pain and previous sexual abuse.

OBJECTIVE: To determine whether there is an association between history of sexual abuse and development of chronic pelvic pain. METHODS: We studied 36 women with chronic pelvic pain, 23 with chronic low back pain, and 20 healthy women with reference to experience of sexual abuse, physical violence, and emotional neglect in childhood. Semistructured interviews were used to collect data. Assessment of severity of sexual abuse was based on Russell's criteria, and emotional and physical abuse were determined according to Briere's and Adler's definitions, respectively. RESULTS: In terms of experience of sexual abuse in general, the three groups did not differ statistically significantly from each other (P =.128). However, 22% of patients with chronic pelvic pain were sexually abused before their 15th birthdays, significantly more frequently than the other two groups (chronic low back pain 0%, P =.019, pain-free control 0%, P =.028). Women with chronic pelvic pain were exposed more frequently to physical violence (38%) and suffered more emotional neglect (25%) in their childhoods than women in the pain-free control group (physical abuse 5%, P =.012; emotional abuse 0%, P =.018). With regard to physical abuse and emotional neglect, the women with chronic pelvic pain did not differ from those with chronic low back pain (physical abuse 30.4%, P =.385; emotional abuse 21.7%, P =.571). CONCLUSION: Besides physical and emotional trauma, there is a significant association between sexual victimization before age 15 years and later chronic pelvic pain.

Adolescent↗

The impact of stressful life events on exacerbation of chronic low-back pain.

The impact of stressful life events on the development and onset of chronicity of low-back pain is not yet fully understood. Sixty-four consecutive patients with chronic low-back pain treated at the orthopedic out-patient unit of the Innsbruck University Hospital were investigated with regard to stressful life events. Patients were classified into two groups: one group consisting of patients whose pain had an organic etiology (n= 16), and another group consisting of those with pain of uncertain origin (idiopathic group, n=48). A method combining a semistructured interview with a self-assessment of the severity of stress caused by life events was employed for assessing the impact of such events on chronic low-back pain. In comparison to the patient group having organic causes of pain, the idiopathic group showed significantly more patients having at least one highly stressful event preceding the last substantial aggravation of pain (p=0.028). The latter group experienced significantly more exhaustion (p=0.016) and significantly more difficulties in active coping (p=0.011) when confronted with stressful life events. Stressful life events that arouse feelings of helplessness may contribute to the development of chronic idiopathic low-back pain. Our results emphasize the importance of investigating the subjective meaning and appraisal of stressful life events, taking into consideration both individual predisposition and psychosocial resources available to the patient. Finally, it is also important that the attending physician be aware of any life event that is particularly stressful for the patient so that psychosocial help be made available at the appropriate time.

Adult↗

Septicaemia with a dysgonic fermenter-2 (DF-2) bacterium in a compromised host.

Dysgonic fermenter-2 (DF-2) is a newly described gram-negative bacterium that can produce serious infections in the compromised host. We are reporting a case of DF-2 septicaemia in a splenectomized patient with chronic lymphocytic leukaemia. The clinical spectrum of infections due to the DF-2 bacterium, the difficulties in establishing the diagnosis and the preferential treatment are discussed.

Agammaglobulinemia↗

Daily psychosocial stressors interfere with the dynamics of urine neopterin in a patient with systemic lupus erythematosus: an integrative single-case study.

OBJECTIVE: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by flare-ups, the causes of which are not known. In accordance with new concepts in stress research, this study investigated whether daily psychosocial stressors interfere with immunological processes in SLE. Because such processes are unique to each individual, single-case design using time-series analysis (Box and Jenkins) was applied. METHODS: A 40-year-old woman with SLE (last flare-up September 1995) was interviewed initially to determine major life events and difficulties (using the Life Events and Difficulties Schedule) in the previous 2 years. She was then observed for 63 days. Urine neopterin, an immunological parameter demonstrated to parallel disease activity in SLE patients, was measured in daily overnight urine. Daily incidents were identified weekly by the Incidents and Hassles Inventory and independently rated. Intervening factors, including infections, medication, and lifestyle, were controlled. RESULTS: Retrospectively, data obtained from the Life Events and Difficulties Schedule indicated that major life events and difficulties had preceded the patient's last flare-up in 1995. Although there were no clinical signs of SLE during this prospective study of 63 days, cross-correlational analyses revealed that "moderately" stressful incidents associated with higher levels of emotional irritation (lag 0: +0.271, p < .05) predicted an increase in urine neopterin the following day (lag 1: +0.441, p < .05). Moreover, a 7-day cyclicity in neopterin levels that corresponded to the weekly examinations and interviews was found. CONCLUSIONS: This study showed a causal relationship between psychosocial stressors and urine neopterin concentrations that may be related to SLE disease activity. Furthermore, the workability of an integrative approach using single-case design and time-series analysis in psychoneuroimmunology was demonstrated for the first time.

Adult↗