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Presents information on a study on a multiblock Navier-Stokes solver which was applied to a supersonic compressor cascade flow with strong shock-wave/boundary-layer interaction. Discussion on fan and compressor designs; Methodology of the study; Results of the study.

Cardiovascular autonomic dysfunction is not uncommon in multiple sclerosis (MS) and is related to the involvement of the vegetative areas of cardiac innervations in the medulla oblongata. It has been suggested that this may contribute to the occurrence of sudden death in MS. In this case report, we present a patient with active relapsing–remitting MS who died unexpectedly due to the sudden onset of cardiac arrythmias. Post-mortem examination showed the presence of active demyelinating lesions in the medulla oblongata. As far as we know, this is the first case report clearly linking sudden cardiac death to active MS on the histopathological level. [ABSTRACT FROM AUTHOR]

Although limb girdle weakness is not part of the major diagnostic criteria of oculopharyngeal muscular dystrophy (OPMD), it has frequently been observed in the Dutch and other OPMD cohorts. In the Dutch cohort, this might be related to the relatively old age or the severity of the genetic defect. This patient-control study (14 OPMD patients and 12 controls) investigated the involvement of limb girdle muscles with a multidimensional approach in early OPMD. We assessed functional abilities, disease impact, physical activity, muscle strength, histopathology and fatty infiltration using questionnaires, actometer, functional tests, manual and quantitative muscle testing, muscle biopsy and muscle MRI. The study showed that involvement of pelvic girdle and proximal leg can be a relatively early feature of OPMD, resulting in impaired daily life activities. The fat fraction of the hip adductors and hamstrings was significantly higher in OPMD patients than in controls. Future studies should include assessment of hip flexors, hip adductors and hamstrings (muscle strength measurements and MRI), functional tests and questionnaires. These findings are important in future diagnostics, management and for the design of outcome measures in trials. [ABSTRACT FROM AUTHOR]

Mutations in PGM1 (phosphoglucomutase 1) cause Glycogen Storage Disease type XIV, which is also a congenital disorder of protein N-glycosylation. It presents throughout life as myopathy with additional systemic symptoms. We report the effect of oral galactose treatment during five months in a patient with biochemically and genetically confirmed PGM1 deficiency. The 12-minute-walking distance increased by 225 m (65%) and transferrin glycosylation was restored to near-normal levels. The exercise assessments showed a severe exercise intolerance due to a block in skeletal muscle glycogenolytic capacity and that galactose treatment tended to normalize skeletal muscle substrate use from fat to carbohydrates during exercise. [ABSTRACT FROM AUTHOR]

Purpose : We discuss the coexistence of Klippel--Trénaunay--Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. Patient : We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing his fibula. Since birth he was known as having the uncommon syndrome of Klippel--Trénaunay--Weber of his right lower leg. Methods : Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases. Results : In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months postoperatively. Discussion : This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel--Trénaunay--Weber syndrome. A case never described before in literature and probably, as in our case, difficult to diagnose at first biopsy. [ABSTRACT FROM AUTHOR]

The article describes a case of fever-induced recurrent rhabdomyolysis in a 43-year-old man. Topics discussed include the symptoms experienced by the patient including febrile throat infection with nausea, vomiting and shivering, and the common causes of rhabdomyolysis. It was found that the RYR1 mutation caused an increased susceptibility to fever-induced rhabdomyolysis in this patient.