ABSTRACT
Pulmonary arterial hypertension (PAH) can be found in patients suffering from a loss-of-function mutation of the gene encoding for the activin receptor-like kinase 1 (ALK-1), a bone morphogenetic protein (BMP) type 1 receptor. Interestingly, ALK-1 mutations also lead to hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant disease characterized by arteriovenous malformations (AVMs) leading to potentially life-threatening bleeding complications such as epistaxis. Current therapeutic options for both diseases are limited and often only temporary or accompanied by severe side effects. Here, we report of a patient with a mutation of the ALK-1 gene suffering from both HHT and PAH. Recently, it was shown that tacrolimus increased ALK-1 signaling and had beneficial effects in selected end-stage PAH patients. We thus hypothesized that treatment with tacrolimus may prevent disease progression in this patient. Surprisingly, treatment with low-dose tacrolimus dramatically improved his HHT-associated epistaxis but did not attenuate progression of PAH.
ABSTRACT
This paper describes the ElePhant (Electronic Phantom)-an anatomical correct simulation system based on 3D rapid prototyping models for the otologic intervention "Mastoidectomy". The anatomical structures of the head are created with plaster as base material using 3D-printing as rapid prototyping technology (RPT). Structures at risk, represented by electrically conductible material and fiber optics, are realized as an electric circuit and can be detected during the simulation of the surgical procedure. An accuracy study of 15 identical RPT-models compared to the 3D reconstructed CT-dataset of the patient showed that the mean accuracy is lower than the reconstructed CT layer thickness of 0.5 mm. An evaluation study of the ElePhant-system for "Mastoidectomy" was performed by 7 ENT-surgeons. The mean value of the study questionnaire (evaluation range from -2 (not at all) to +2 (very good)) was +1.2. The results showed that the ElePhant can simulate "Mastoidectomy" realistically. It is especially suitable for the simulation of the correct representation and position of the anatomical structures, realistic operation setting, and realistic milling properties of the bone structure. Furthermore it is applicable for training of surgeons.
Subject(s)
Ear/surgery , Models, Anatomic , Surgical Procedures, Operative , Algorithms , Computer Simulation , Electric Conductivity , Equipment Design , Fiber Optic Technology , Humans , Image Processing, Computer-Assisted , Imaging, Three-Dimensional , Microcomputers , Optical Fibers , Reproducibility of Results , Risk , Skull/pathologyABSTRACT
The anticonvulsant activities of several indoline derivatives were tested in mice using the pentetrazole (PTZ) and electroshock seizure model. Furthermore, the toxicity and the influence on hexobarbitone anaesthesia were determined. 2-oxoindoline--the most potent anticonvulsant compound--shows a characteristic modification of PTZ-induced seizures, especially a suppression of the tonic phase and a marked prolongation of survival time at lethal PTZ-doses as well as in doses of 100 mg/kg a complete protection effect to maximum electroshock. The majority of indolines possess also a marked sedative effect. The LD50 values of the derivatives under investigation are differing considerably, however, majority of compounds being within the range of 300 to 800 mg/kg (i.p.).
Subject(s)
Anticonvulsants , Indoles/pharmacology , Animals , Electroshock , Hexobarbital/antagonists & inhibitors , Mice , Pentylenetetrazole/antagonists & inhibitorsABSTRACT
Computed tomography was employed in 37 patients suspected of having an aortic aneurysm: it was demonstrated in 32. Additional angiography was performed in 13, in eight of whom thoracic or thoracic-abdominal aneurysm was diagnosed, in five with dissection. Computed tomography gave a more exact measure of length and width of the aneurysm than angiography. The renal arteries were involved in four instances, pelvic arteries in seven. Both arteriography and computed tomography demonstrated dissection of the thoracic aneurysm.
Subject(s)
Aortic Aneurysm/diagnosis , Adult , Aged , Aortic Dissection/diagnosis , Aorta, Abdominal , Aorta, Thoracic , Aortography , Female , Humans , Iliac Artery , Male , Middle Aged , Renal Artery , Tomography, X-Ray ComputedABSTRACT
A standardized stirred-batch experiment has been performed to determine the maximum adsorptive capacities of three activated carbons (R4, ASO and walnut coal) produced in the GDR for acetylsalicylic acid, methylsalicylate, phenobarbital, crotylbarbital, hexobarbital, pentobarbital, glutethimide, chlorophenethazine and methaqualone from aqueous and low-percentage ethanolic solutions. The activated carbon ASO and the walnut coal have always been found to be markedly superior to the activeated coal R 4. The maximum adsorptive capacities so far determined compare with data on activated carbons produced abroad, so that further developmental work seems worthwhile.20
