Die Glykogenose Typ IV als seltene Ursache einer Kardiomyopathie – Bericht einer erfolgreichen Herztransplantation

Zusammenfassung Wir berichten über einen 17j?hrigen Patienten, der an einer Glykogenose Typ IV (Morbus Andersen) und einem Immunglobulin-(Ig)-G-II-Subklassenmangel erkrankt war. Anamnestisch bedeutsam ist, da? bei gesunden Eltern des Patienten zwei jüngere Brüder im Alter von neun bzw. zehn Jahren an einer dilatativen Kardiomyopathie verstarben. Im Alter von zw?lf Jahren traten erstmals Symptome einer Herzinsuffizienz auf, wobei histologisch eine Kardiomyopathie auf der Basis der Glykogenose gesichert wurde. Das Krankheitsbild des Patienten war zus?tzlich durch rezidivierende pulmonale Infektionen mit Bildung von Bronchiektasen im Bereich des linken Unterlappens gekennzeichnet. In dieser Region entwickelte sich seit etwa dem elften Lebensjahr eine Atelektase. Bei rezidivierenden katecholaminpflichtigen kardialen Dekompensationen akzeptierten wir den Patienten trotz relativer Kontraindikationen (ausgepr?gte Kachexie, generalisierte Myopathie, Bronchiektasen bei Immundefekt) zur orthotopen Herztransplantation (HTX), welche erfolgreich durchgeführt wurde. Die ausgepr?gte Myopathie führte in der postoperativen Phase zu einer prolongierten Dauer der Mobilisation und einer verl?ngerten Beatmungszeit. Bei vorbestehenden Bronchiektasen kam es in der frühen Phase nach der Transplantation zur abszedierenden pulmonalen Infektion. Der Patient weist, über ein Jahr nach HTX, einen klinisch und funktionell stabilen Zustand auf. Die im Verlauf durchgeführten rechtsventrikul?ren Myokardbiopsien zeigten kein Anhalt für eine Rekurrenz der Glykogenose im Transplantat. Eingegangen: 12. April 1999, Akzeptiert: 22. Juni 1999     

Automobilisation und Automanipulation – Lifestyle einer modernen Gesellschaft?

Manuelle Medizin - Die Selbstbehandlung von reversiblen segmentalen Dysfunktionen ist ein zunehmendes Problem in der täglichen Praxis. Automobilisationen und -manipulationen der...     

Anonymisieren oder personalisieren?

Background

The subjective state of health with respect to pain and psyche was surveyed utilizing validated pain questionnaires in patients undergoing special pain therapy and represents the basis for targeted treatment measures.

Objective

The purpose of this study was to investigate the possible distortion of answers due to social desirability of responses in chronic pain patients.

Material and methods

During two survey periods assessing patient satisfaction using both anonymized and personalized questionnaires, the effects arising from socially desirable response patterns were analyzed. The sample consisted of chronic pain patients being treated in an inpatient therapy setting.

Results

In both periods of observation no significant impact on the response behavior of chronic pain patients was found in personalized or anonymized questionnaires.

Conclusion

The results of the study suggest that the responses of chronic pain patients with respect to their subjective state of health are not influenced by social desirability. Thus, scoring systems such as the German pain questionnaire will not be influenced by social desirability in chronic pain patients and can therefore be used as a part of diagnostics and therapy planning.

     

Kortikale Dysbalance des Migränikerhirns – Hyperexzitabilität als Folge einer Sensitisierung?

A cortical dysbalance has a pivotal role in the pathophysiology of migraine. Numerous electrophysiological and transcranial magnetic stimulation (TMS) studies have investigated the interictal excitability level in migraineurs and have shown a consistent lack of habituation during repetitive stimulation. There is some controversy in the current literature over whether this deficit is based on a lowered or an elevated preactivation level. However, the current discussion may be misguided. It seems that multiple external and intrinsic factors influence the level of cortical excitability and the frequency and intensity of attacks: Habituation is specific neither to migraine nor even to pain; the same phenomenon is found in tinnitus patients, for example. Cortical hyperexcitability is presumably the result of chronicity and the concomitant central sensitisation process.     

Schmerzakzeptanz – Konzept und Überprüfung einer deutschen Fassung des Chronic Pain Acceptance Questionnaire

Background

Up to now, only an English version of the Chronic Pain Acceptance Questionnaire (CPAQ) has been available for measuring the acceptance of chronic pain. This paper presents and analyzes a German adaptation of this instrument.

Methods

The German scale was tested on 150 patients at the DRK Pain Center in Mainz. Validity was assessed by means of various indicators of pain and psychosocial impairment. In a subgroup (n=50) the association with the heat pain threshold was determined.

Results

The factor structure of the German CPAQ scale is largely concordant with the theoretical model. The internal consistency of the total and subscales is 0.84–0.87 (Cronbach’s alpha). The factors are closely related to indicators of psychosocial functioning. Associations with the affective dimension of pain are moderate and with the sensory dimension low. No association is found with heat pain thresholds (thermal sensory analyzer).

Conclusions

The German CPAQ scale is a useful German-language instrument for the measurement of acceptance and shows good psychometric properties. The study confirms that acceptance is not an expression of a physiologically based indolence.     

Die Bestimmung des i.m. Morphin-Äquivalents zur Therapie des Krebsschmerzes mit verschiedenen Opioiden oder beim Wechsel des Verabreichungsweges

During the long-term treatment with opioids it is sometimes important to switch the opioid or change the route of administration. The estimation of morphine-equivalents can be helpful in this range because it clarifies the dose in milligramm required for different clinical situations. The basis of this estimation is the equianalgesic potency of opioids. One i.m. morphine-equivalent is the analgesic dose of an opioid (i.m. injected) equal to the analgesic effect of 1 mg morphine (i.m.). The relationships between equianalgesic doses and intramuscular and oral routes of applications are listed in tables. The cross-tolerance between different opioids during long-term treatment is not complete. To avoid an overdose, we suggest a reduction in the calculated opioid dose of 50%. Additional "rescue doses" can be used during the period immediately the change to provied satisfactory pain control. A new opioid dosage should be calculated every 24 hours based on the basaline dose plus the total quantity of "rescue" medication required by the patient. Useful starting point for calculation an effective dose when changing from one opioid or route of administration to another can result in improved pain control that is more responsive to patient need. The limitations are 1. individual differences in the response to opioids, especially during long-term treatment and in the development of analgesic tolerance, 2. individual differences in the response to alternatives routes of administration, and 3. the unknown degree of cross tolerance among opioid drugs. The scientific meaning of the estimation of i.m. morphine-equivalent is discussed.     

Aspekte einer liaisonpsychiatrischen Betreuung von Patienten einer universitären Schmerzambulanz

Background

Owing to a rise of psychosomatic comorbidities, the treatment of psychological disorders, which may negatively impact prognosis and therapy, is increasingly becoming a focus of attention for pain outpatient clinics.

Aim

This study investigates and discusses the advantages of liaison psychiatric care in a university pain clinic.

Methods

In this retrospective study, we investigated all patients who presented to an anaesthesiologically led pain clinic between January and June 2014. The psychiatric history was taken by the liaison psychiatrist of the pain clinic.

Results

In the period investigated, 485 patients were treated as outpatients. A psychiatric diagnosis was present 351 patients (72.4%). The distribution of the diagnoses was comparable with that of a consultation service. Adaptation and affective disorders dominated. The patients were preferentially treated with new generation antidepressants.

Conclusion

The constant presence of a liaison psychiatrist allows for timely, specialised care of pain patients in terms of a multimodal therapeutic approach.