In our center, 289 children with von Willebrand’s disease (vWD) have been diagnosed since 1982. The majority of cases (n = 198) were congenital vWD whereas 91 patients suffered from vWD induced by valproate (VPA). We overview bleeding episodes in 45 children and 64 operative procedures requiring therapeutic intervention. The aim of therapeutic and prophylactic procedures in vWD is correcting the hemostatic disorder and normalization of bleeding time. This can be achieved by application of Haemate P leading to an elevation of plasma levels of von Willebrand parameters together with normalization of bleeding time. In patients with vWD type I, DDAVP will be preferred if contraindications can be excluded and efficacy has been shown. Severe bleeding complications could be prevented in a total of 50 surgical procedures in children with vWD type I by prophylactic treatment with DDAVP or Haemate P. Two children initially treated with DDAVP had to be substituted with Haemate P in the follow-up because of continuous bleeding. In type IIa and type III vWD as well as in VPA-induced vWD, the use of Haemate P was essential for sufficient hemostasis in all bleeding and operations. We conclude that Haemate P provides effective bleeding prophylaxis and treatment in all types of vWD except platelet-type.
Weaned hairless rats were fed a diet deficient in fat, magnesium and folacin. After approximately 1 week, an erythematous dermatitis developed which was associated with extreme generalized pruritus. Scratching led to excoriations and hemorrhagic crusting. The acute stage (pruritic rash) resolved after several days and was followed by sporadic non-itching relapses. Subsequent to the onset of symptoms, rats were treated orally, once daily for 3 days with CyA, CyH or FK506. The immunosuppressants CyA and FK506 caused a dose-dependent inhibition of symptoms in contrast to CyH. The immediate clinical response was associated with changes in blood histamine, white blood cell counts and histological parameters. Since CyH is known to lack immunosuppressive activity, these results may indicate that the cutaneous changes induced by the nutritional deficiency are associated with immunological abnormalities. The results may also indicate mechanisms influenced by CyA and FK506 but not by CyH; for example, release of chemical mediators from inflammatory cells.
Anterior approaches for the stabilization of anterior and also posterior B and C type instability of the pelvic ring were the standard procedures before minimally invasive percutaneous methods supported by image intensifiers or navigation devices were established. Anterior approaches are currently still of high relevance for difficult or impossible closed reductions in multiple trauma surgery where the patient must remain in a supine position. They are also used for stabilization of an increasing number of osteoporotic fractures in the elderly which are no longer only treated in specialized pelvic trauma centres. The anterior as well as the posterior part of the pelvic ring can be stabilized via various anterior approaches. A Pfannenstiel incision is appropriate for plating of ruptures of the pubic symphysis and can be extended to a modified Stoppa approach if necessary. Fractures of the iliac wings can be approached either laterally or less traumatically, via an anterolateral approach. The latter equates the lateral window of the ilioinguinal approach to the acetabulum, allows visualization of the entire sacroiliac joint and therefore stabilization of not only iliosacral luxation but also luxated fractures with a small iliac fragment. By a combination of the different approaches it is possible to simultaneously stabilize ventral and dorsal instabilities in type C fractures of the pelvic ring with a minimal amount of iatrogenic soft tissue trauma. Although the described anterior approaches are considered as simple exact knowledge of the endangered structures and general risks for each approach are essential for a safe exposure of the anatomical region addressed.
Melasma, also known as chloasma or mask of pregnancy, presents clinically as hyperpigmented skin areas, which develop mostly in the face as a consequence of increased synthesis of melanin. The established treatment options, including topically applied agents and the use of various laser systems, mostly result in improvement but not in complete remission of the lesions. Because of its significant impact on quality of life and the limited effectivity of available treatment options, the management of melasma is challenging for the treating physician. Although many risk factors, including pregnancy and UV exposure, have been identified, the pathogenesis is not yet fully understood. Avoiding solar or artificial UV exposure is of high importance both for the prevention of melasma and for the clinical outcome of existing lesions. In order to avoid vitamin D deficiency, oral vitamin D supplementation should be recommended. In this review, we give an update on clinical aspects, epidemiology, pathogenesis and therapy of melasma and give an outlook on future developments.
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