The importance of genetic susceptibility in Dupuytren's disease.

Dupuytren's disease (DD) is a progressive fibromatosis that causes the formation of nodules and cords in the palmar aponeurosis leading to flexion contracture of affected fingers. The etiopathogenesis is multifactorial with a strong genetic predisposition. It is the most frequent genetic disorder of connective tissues. We have collected clinical data from 736 unrelated individuals with DD who underwent surgical treatment from Germany and Switzerland. We evaluated a standardised questionnaire, assessed the importance of different risk factors and compared subgroups with and without positive family history. We found that family history clearly had the strongest influence on the age at first surgery compared to environmental factors, followed by male sex. Participants with a positive family history were on average 55.9 years of age at the first surgical intervention, 5.2 years younger than probands without known family history (p = 6.7 × 10(-8) ). The percentage of familial cases decreased with age of onset from 55% in the 40-49 years old to 17% at age 80 years or older. Further risk factors analysed were cigarettes, alcohol, diabetes, hypertension, and epilepsy. Our data pinpoint the importance of genetic susceptibility for DD, which has long been underestimated.

c-kit/CD 117 positive cells in the myometrium of pregnant women and those with uterine endometriosis.

PURPOSE: Abnormal myometrial motility may play a role in the pathogenesis of endometriosis. Uterine contractility is a major contribution to labour. Myometrial motility might be controlled by CD 117-positive uterine smooth muscle cells. METHODS: Myometrial tissues from 8 cases with uterine endometriosis, 9 pregnant uteri (31.1 ± 8.7 weeks of gestation), 10 cases from non-pregnant pre-menopausal and 9 cases from post-menopausal women were immunohistochemically evaluated using a polyclonal antibody against c-kit/CD 117. The number of CD 117 positive cells was counted within 10 microscopic high power fields (× 400) and compared with the clinical diagnoses. RESULTS: Overall, a mean number of 15.7 (range 0-43) CD 117-positive cells within the myometrium was seen. Significant highest count occurred in the myometrium of non-pregnant pre-menopausal women without uterine endometriosis (30.78 ± 9.52), followed by post-menopausal women (15.5 ± 8.37) and those with uterine endometriosis (9.98 ± 4.9; p ≤ 0.01). The lowest count of CD 117-positive cells was seen in pregnant uteri (4.09 ± 2.33; p < 0.001). CONCLUSIONS: The lowest count of CD 117-positive cells was seen in the myometrium of pregnant women suggesting a role of preventing premature uterine contractility. There is no increase of CD 117-positive cells in the myometrium of women affected by uterine endometriosis.

Volcanic sunset-glow stratum: origin.

Reexamination of the phenomenon of volcanic-dust sunsets, as typified by the Krakatoa event, supports a theory that the scattering layer is produced by the interaction of ozone and sulfur dioxide in much the same manner as is the normal "Junge"aerosol layer at 20 kilometers.

Stratospheric dust-aerosol event of november 1974.

A strong incursion of dust and aerosol at an altitude of 20 kilometers was noted over Baja California and southern Arizona in mid-November 1974, as indicated by bluish-ashen daylight skies and colorful twilight glows of the type usually associated with volcanic eruptions. Infrared satellite observations and reports from other sources eliminated a possible oceanic origin in the eastern Pacific. The stratum is probably from the extensive eruption of Volcan de Fuego in Gautemala in October 1974.

Trajectory of the mt. St. Augustine 1976 eruption ash cloud.

The ash clouds from the 23 January 1976 eruption of Mt. St. Augustine in Alaska drifted over Arizona on 25 January at an altitude of 6 kilometers as deduced from air trajectories and the observation of an unusual cloud.

[Dupuytren contracture: new aspects on form pathogenesis and surgical principle]. / Morbus Dupuytren: Neue Aspekte zu Formalgenese und Operationsprinzip.

More than 20 years ago, the author formulated the proposition "extension inhibition instead of contraction" to explain the pathogenesis of Dupuytren's disease. This concept and the limited surgery derived from it are presented with preliminary long-term results. The tissue dynamics at the palmodigital zone were investigated. Subcutaneous microdissection and the first slices of adult hands (plastination) served to investigate the intact skin-covered subcutaneous tissue layer. On the flexor side, the hand has a complex skin anchoring system which effects mobile attachment of the digital subcutaneous tissue and stationary attachment of the palmar subcutaneous tissue. The disease fixes the flexion position of the tissue layer and impedes extension of the subcutaneous tissue and fingers. It is not the open but the closed hand from which fibromatosis starts. The principle "retention of flexion position" does not imply a contraction mechanism. The pathological substrate undergoes tendinous straightening by traction. There is secondary hypertrophy of the longitudinal fascicles of aponeurosis. The derived principle of limited surgery leaves the aponeurosis in situ. The preliminary results of follow-up examinations after five years and more show that there is no increased danger of recurrence when the aponeurosis is not removed. Out of 72 hands, 34 (47%) proved to be free of disease. Recurrences were found in 17 (24%) hands.

[Changes in the hand in Ehlers-Danlos syndrome type IV]. / Veränderungen an der Hand beim Ehlers-Danlos-Syndrom Typ IV.

The authors report a lethal course of Ehlers-Danlos syndrome type IV. In this patient hypermobility of the finger joints had been seen. The patho-biochemical structure of fibrous tissue is discussed.

[Long-term static overnight extension splinting following percutaneous needle fasciotomy]. / Die langfristige statische Extensions-Nachtschiene nach perkutaner Nadelfasziotomie.

The author reports on his experience with static night splinting after percutaneous needle fasciotomy (PNF). A conventional glove splint and custom made silicone bed splints were used. According to the experience of the author 3 typical case studies demonstrate that static night splinting of the Dupuytren fingers can achieve 2 things: reduction of the risk of recurrent curvature and remodeling of the Dupuytren tissue.

Decreased cytosolic glucocorticoid receptor levels in critically ill patients.

The immune system and the hypothalamic-pituitary-adrenal axis are linked by several mechanisms. Intracellular glucocorticoid receptors represent one important connection. The aim of this study was to examine the coherence between the number of glucocorticoid receptors, activation of the hypothalamic-pituitary-adrenal axis, inflammatory cytokine levels and the severity of illness in critically ill patients. In a prospective study, blood was collected from 20 healthy members of the hospital staff (control group) and 50 ventilated patients (sample group) within the first 24 hours after intubation and within three days of extubation. 3H-dexamethasone-binding assay was used to assess cytoplasmatic free glucocorticoid receptors levels. ACTH, cortisol, IL-6 and TNFa levels were measured by ELISA. In the sample group, specific binding of 3H-dexamethasone was significantly decreased compared to the control group. Glucocorticoid receptor levels tended to be lower in more severely ill patients. Plasma cortisol and ACTH levels were significantly different from the control group after extubation but not at intubation. Severe illness is associated with rapid down-regulation of 3H-dexamethasone binding. This decrease occurs before elevation of plasma cortisol. Therefore, down-regulation of cortisol binding may be directly associated with the stress response and not due to feedback regulation following increase in plasma cortisol levels.

[Dupuytren disease--formal pathogenesis without contraction and a new surgery concept]. / Morbus Dupuytren--Formalpathogenese ohne Kontraktion und ein neues operationstaktisches Konzept.

The palmar fibromatosis holds the finger in the flexed position--extension inhibition rather than contraction. According to this extrinsic concept, only a passive, secondarily reactive effect can be attributed to the palmar aponeurosis. Out of 72 hands examined 5 or more years after an operation in which the aponeurosis had been left intact, 47% proved to be clear of disease.

Aperture synthesis using independent telescopes.

Study of a design for a large infrared telescope, consisting of six independent optical systems shows that it is possible to control the optical path differences and phases such that aperture synthesis can be achieved. A thin compensating prism in each optical path can compensate simultaneously the path errors for all objects in the field of view of the telescope. Design parameters for a 5.6-m system are given.

A 1.8-m lightweight Doubly Asymmetric Equatorial Telescope Design.

A configuration applicable to large astronomical telescopes has been developed specifically for lightweight monolithic mirrors in which the primary mirror is located on the same side of the declination axis as the secondary, placing the cassegrain focus very close to the declination axis. This configuration permits both a large instrument space and a single south polar tyne, resulting in a doubly asymmetric system. It is also shown that a passive closed-system air flotation support system can be used for both lateral and back support of the primary mirror, as contrasted to more complex active systems currently in widespread use.

The effect of storage at - 80 degrees C on the activities of cytoplasmic, mitochondrial and microsomal enzymes in rat liver.

The effect of storage at - 80 degrees C for 1-28 days on the activity of 12 enzymes in intact liver tissue, liver extract and isolated hepatic microsomes was investigated. To find optimal conditions for tissue homogenization for this study the effects of three types of homogenization on the activity of 10 enzymes from different cell compartments were compared. The activities of glucokinase and phosphofructokinase decreased markedly during storage of both supernatant and liver tissue. Storage of liver tissue increased the activity of mitochondrial enzymes or isoenzymes. While this effect can be explained by additional disintegration of liver tissue caused by freezing and thawing for enzymes like glutamine dehydrogenase, other mechanisms may be involved in the prolonged increase observed in the activity of citrate synthase and xanthine oxidase during storage. The activity of a number of enzymes from the cytosol, mitochondria and microsomes decreased more markedly in the stored liver samples than in the stored supernatant or in the stored microsomal pellet. Cytochrome P 450 content remained stable throughout the whole storage period in both intact liver tissue and isolated microsomes.