Successful cord blood stem cell transplantation for congenital erythropoietic porphyria (Gunther's disease).

Congenital erythropoietic porphyria (Gunther's disease, GD) is a rare autosomal recessive disease. It results from the deficiency of uroporphyrinogen III synthase, the fourth enzyme on the metabolic pathway of heme synthesis. GD leads to severe scarring of the face and hands as a result of photosensitivity and fragility of the skin due to uroporphyrin I and coproporphyrin I accumulation. It also causes erythrocyte fragility leading to haemolytic anaemia. The other clinical features include hirsutism, red discolouration of teeth, finger-nails and urine and stunted growth. The outcome is poor, and the disfiguring nature of GD may partly explain the legend of the werewolf. No curative treatment was known until 1991, when the first case of BMT in GD was reported. The clinical and biological outcome after transplantation was encouraging, with an important regression of the symptoms of the disease, but the child died of CMV-infection 11 months after BMT. We report the second case of GD treated successfully by stem cell transplantation using umbilical cord blood from an HLA-identical brother in a 4-year-old girl suffering from severe GD. Our patient is very well 10 months after transplantation. We confirm that stem cell transplantation is curative for GD.

[Therapy of acute traumatic vascular injuries using covered stents]. / Therapie akuter, traumatischer Gefässläsionen mittels ummantelter Stents.

PURPOSE: Evaluation of percutaneously implanted covered stents in acute vascular bleeding as therapeutic alternative to conventional surgical treatment. MATERIALS AND METHODS: 8 patients aged 26 to 83 years with acute vascular lesions caused by traumas, and subsequent haemorrhage, were transferred to our department. Because of general inoperability or difficult surgical access, interdisciplinary evaluation favoured an interventional treatment. In 6 patients stents could be placed percutaneously to the aorta, subclavian and iliac arteries. In one case we had to implant three stents into the thoracic aorta. RESULTS: In 7 interventionally treated patients the stents could be placed exactly on the lesions (88%). The bleeding could be stopped immediately in 6 cases (75%). In one patient we had to implant successfully two more stents in reintervention (12%). In another patient the available prosthesis was too short, so that the patient had to be referred to the OR for surgical treatment (12%). There were no complications during the treatment. CONCLUSION: First results in the use of covered stents as interventional treatment of acute vascular lesions are encouraging and may represent a possible alternative to surgical therapy in locally limited bleedings, presupposing that all different types and sizes of industrially produced covered stents are available. Further investigations to compare surgical and interventional techniques are warranted.

Prediction of cranio-maxillofacial surgical planning using an inverse soft tissue modelling approach.

In cranio-maxillofacial surgery, the determination of a proper surgical plan is an important step to attain a desired aesthetic facial profile and a complete denture closure. In the present paper, we propose an efficient modeling approach to predict the surgical planning on the basis of the desired facial appearance and optimal occlusion. To evaluate the proposed planning approach, the predicted osteotomy plan of six clinical cases that underwent CMF surgery were compared to the real clinical plan. Thereafter, simulated soft-tissue outcomes were compared using the predicted and real clinical plan. This preliminary retrospective comparison of both osteotomy planning and facial outlook shows a good agreement and thereby demonstrates the potential application of the proposed approach in cranio-maxillofacial surgical planning prediction.

Vertebroplasty: experimental characterization of polymethylmethacrylate bone cement spreading as a function of viscosity, bone porosity, and flow rate.

STUDY DESIGN: This is an experimental study on an artificial vertebra model and human cadaveric spine. OBJECTIVE: Characterization of polymethylmethacrylate (PMMA) bone cement distribution in the vertebral body as a function of cement viscosity, bone porosity, and injection speed. Identification of relevant parameters for improved cement flow predictability and leak prevention in vertebroplasty. SUMMARY OF BACKGROUND DATA: Vertebroplasty is an efficient procedure to treat vertebral fractures and stabilize osteoporotic bone in the spine. Severe complications result from bone cement leakage into the spinal canal or the vascular system. Cement viscosity has been identified as an important parameter for leak prevention but the influence of bone structure and injection speed remain obscure. METHODS: An artificial vertebra model based on open porous aluminum foam was used to simulate bone of known porosity. Fifty-six vertebroplasties with 4 different starting viscosity levels and 2 different injection speeds were performed on artificial vertebrae of 3 different porosities. A validation on a human cadaveric spine was executed. The experiments were radiographically monitored and the shape of the cement clouds quantitatively described with the 2 indicators circularity and mean cement spreading distance. RESULTS: An increase in circularity and a decrease in mean cement spreading distance was observed with increasing viscosity, with the most striking change occurring between 50 and 100 Pas. Larger pores resulted in significantly reduced circularity and increased mean cement spreading distance whereas the effect of injection speed on the 2 indicators was not significant. CONCLUSION: Viscosity is the key factor for reducing the risk of PMMA cement leakage and it should be adapted to the degree of osteoporosis encountered in each patient. It may be advisable to opt for a higher starting viscosity but to inject the material at a faster rate.

A novel computational method for real-time preoperative assessment of primary dental implant stability.

A novel methodology which allows for fast and fully automatic structural analysis during preoperative planning for dental implant surgery is presented. This method integrates a fully automatic fast finite element solver within the framework of new concepts in computer-assisted preoperative planning for implant surgery. The planning system including optimized structural planning was validated by experimental results. Nine implants were placed in pig mandibles and mechanically loaded using a testing rig. The resulting displacements were measured and compared with those predicted by numerical analysis during planning. The results show that there were no statistically significant differences (P = 0.65) between the results of the models and the experiments. The results show that fast structural analysis can be integrated with surgical planning software allowing the initial axial implant stability to be predicted in real time during planning. It is believed that such a system could be used to select patients for immediate implant loading and, when further developed, be useful in other areas of preoperative surgical planning.

[Endocarditis lenta caused by Lactobacillus salivarius subsp. salicinicus (author's transl)]. / Endocarditis lenta durch Lactobacillus salivarius subsp. salicinicus

After two tooth extractions performed without antibiotic cover endocarditis lenta occurred in a ten-year-old girl. The causative organism isolated was Lactobacillus salivarius subsp. salicinicus, the first such reported case. The child has a small, haemodynamically insignificant, ventricular septal defect. A cure was achieved after long-term administration of penicillin G in high doses, at first combined with ampicillin. There were no complications.