[Rare diseases with clinical relevance-the Silver-Russell syndrome]. / Seltene Erkrankungen mit klinischer Relevanz ­ das Silver-Russell-Syndrom.

The Silver-Russell syndrome (SRS) is a rare imprinting disease associated with pre- and postnatal growth retardation, craniofacial features, and asymmetry. Genitourinary abnormalities are seen in up to 20% of affected individuals. Apart from structural renal anomalies, cryptorchidism and hypospadias occur frequently in boys, while girls often have anomalies similar to those in Mayer-Rokitansky-Küster-Hauser syndrome with congenital hypoplasia or aplasia of the uterus and upper part of the vagina. Frequently hypospadias repair and orchiopexy are difficult because of lack of buccal mucosa due to facial dysmorphism and intraabdominal position of the testicles, respectively. Anesthetic problems with SRS children can be profound and mostly concern a difficult airway due to facial dysmorphism. Especially the young, very thin SRS patients are prone to hypoglycemia and hypothermia and require close perioperative monitoring. Children with SRS and their families face challenges from birth to adulthood. In case of urogenital abnormalities, they should receive multidisciplinary care by pediatric urologists/surgeons and pediatric anesthesiologists in a center of expertise in rare diseases.

[Age rationing : means of resource allocation in healthcare systems]. / Altersrationierung : Mittel zur Ressourcenverteilung im Gesundheitswesen.

The necessity of limiting resource in healthcare systems is becoming increasingly more evident. The population has requirements especially in the field of healthcare which are principally unlimited. However, there are only limited financial resources which can be used to satisfy the wishes of the population. For this reason rationing models are being discussed increasingly more often. One example of these models is called age rationing which means that defined services are only offered to patients up to a particular age. The aim of this article is to discuss the model of age rationing in the context of an optimized use of resources in the healthcare system.

Elevated premorbid body mass index is not associated with poor neurological outcome in the subacute state after aneurysmal subarachnoid hemorrhage.

BACKGROUND: An elevated body mass index (BMI) is suggested to be a risk factor for a poor outcome after intracranial aneurysm rupture and is considered to be associated with cerebral infarction in patients with aneurysmal subarachnoid hemorrhage (SAH). The aim of this study was to analyze the association between permorbid BMI and neurological outcome. METHODS: In this retrospective study, the patients' BMI at the time of their admission to hospital was correlated to their neurological outcome as measured by the Glasgow outcome score after two weeks and two months of treatment. RESULTS: In contrast to other studies, there were no significant correlations between premorbid BMI and neurological outcome, shunt requirement, tracheotomy requirement and duration of stay on the intensive care unit (ICU). CONCLUSIONS: Overweight patients have no higher risk of a poor neurological outcome after aneurysmal SAH if premorbid risk factors such as hypertension and hyperglycemia are carefully modified throughout the period of critical care.

Remodeling of peritoneal-like structures by mesothelial cells: its role in peritoneal healing.

BACKGROUND: Intraabdominal adhesions are a common complication following laparotomy. Since the exact mechanisms involved in this processes are unknown we have analyzed in vitro the role of mesothelial cells in peritoneal healing. MATERIAL AND METHODS: Human mesothelial cells from omental tissue were cultivated for 2 weeks in a three-dimensional culture either on or in a collagen type I matrix. The effects of blood and collagen matrix were analyzed by exposing mesothelial cells to an overlying blood clot, simulating intraperitoneal bleeding, or a second collagen layer. The production of collagen types III and IV, fibronectin, and laminin was analyzed with immunohistochemical methods. RESULTS: Mesothelial cells grown on a collagen matrix formed a monolayer of flat or cobblestone-like cells whereas those cultivated in a collagen matrix exhibited spindle-like morphology. Mesothelial cells failed to grow into an overlying collagen matrix, but did grow into a blood clot, emphasizing a potential role of blood clots in peritoneal adhesion formation. Independent of the culture systems mesothelial cells produced collagen type III, fibronectin, and laminin but not collagen type IV. CONCLUSIONS: Our experiments demonstrate remodeling of peritoneal-like structures by mesothelial cells in a three-dimensional culture reflecting their putative role in the reepithelialization after serosal defects, and also in the formation of peritoneal adhesions.

Performance evaluation of annular arrays in practice: the measurement of phase and amplitude patterns of radio-frequency deep body applicators.

An approach to a solution of two major problems in operating Annular Phased Arrays in deep body hyperthermia is presented: an E-field sensor capable of measuring phase and amplitude at 70 MHz and the concept of a power transmission factor to determine the effective amplitude of each applicator. In the four-waveguide Phased Array operating at 70 MHz, which is in clinical use at the department of Radiotherapy of the Academic Medical Center (AMC), the incident fields of the waveguides were scanned in phase and amplitude over the complete aperture midplane, inside an elliptical and a square phantom filled with saline. As a check on the application of the superposition principle, superpositions of the incident fields were compared with the electric field in the measured interference set-ups. With all four applicators radiating at equal amplitude and in phase, the maximum difference over the complete midplane of the phantom between superimposed and measured interference scans was 20% and 10 degrees in the elliptical phantom, and 20% and 30 degrees in the square phantom. After having determined nominal amplitude and phase patterns by a vector probe, any interference set-up can be superimposed from measurement of the actual incident field of each applicator. Therefore, the availability of a vector sensor as described here will contribute to solve a problem of hyperthermia quality assurance: the performance evaluation of Phased Arrays.