RESUMO
This evidence- and consensus-based practical guideline for the diagnosis and treatment of Guillain-Barré Syndrome (GBS) in childhood and adolescence has been developed by a group of delegates from relevant specialist societies and organisations; it is the result of an initiative by the German-Speaking Society of Neuropediatrics (GNP), and is supported by the Association of Scientific Medical Societies (AWMF, Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften). A systematic analysis of the literature revealed that only a few adequately-controlled studies exist for this particular age group, while none carries a low risk of bias. For this reason, the diagnostic and therapeutic recommendations largely rely on findings in adult patients with GBS, for which there are a higher number of suitable studies available. Consensus was established using a written, multi-step Delphi process. A high level of consensus could be reached for the crucial steps in diagnosis and treatment. We recommend basing the diagnostic approach on the clinical criteria of GBS and deriving support from CSF and electrophysiological findings. Repetition of invasive procedures that yield ambiguous results is only recommended if the diagnosis cannot be ascertained from the other criteria. For severe or persistently-progressive GBS treatment with intravenous immunoglobulin (IVIG) is recommended, whereas in cases of IVIG intolerance or inefficacy we recommended treatment with plasmapheresis. Corticosteroids are ineffective for GBS but can be considered when acute onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is suspected due to a prolonged disease course. The full German version of the Guideline is available on the AWMF website (https://www.awmf.org/leitlinien/detail/ll/022-008.html).
Assuntos
Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Adolescente , Criança , Técnica Delphi , Progressão da Doença , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Troca PlasmáticaRESUMO
BACKGROUND: Intestinal ischaemia is quite rare among the cardiovascular diseases. However, it is increasingly diagnosed. The aim of this selective but representative short overview is to assess the impact of intestinal ischaemia in vascular and visceral medicine from a vascularsurgical perspective. MATERIAL AND METHODS: A literature search and selection in relevant online services of the medical scientific literature was performed, in particular, of the last decade on the competent management of intestinal ischaemia combined with the clinical expertise obtained in daily vascular surgical practice including didactically prepared demonstrable casesâ/âcase reports related to typicalâ/âspecific clinical problems and situations. RESULTS AND DISCUSSION: Although the superior mesenteric artery (SMA) is most frequently responsible for the clinical presentation, usually 2 or 3 âmajor arterial trunks are involved for a relevant clinical symptomatology. These disorders of the intestinal circulation are most frequently caused by progressive atherosclerotic occlusive disease. In chronic progressive disease, the visceral arteries show the ability to enlarge typical collateral circulation pathways, which may not always lead to a complete compensation. With a degree of stenosis of more than 70â%, mesenteric ischaemic pain and physical prostration are the major clinical findings. Intestinal infarction with a mortality rate of 60-80â% is the endpoint of the chronically progressive intestinal ischaemia. There-fore, an urgent medical treatment is highly required. CT angiography is the diagnostic procedure of choice in patients with suspected chronic intestinal ischaemia. Mesenteric angiography is subject to specific questions andâ/âor to endovascular arteriographic treatment. Duplex scanning has been advocated as a non-invasive method of pre- and post-interventional screening. Treatment is indicated in symptomatic intestinal vascular disease. Due to the high morbidity of the majority of patients and the enormous invasivity associated with conventional surgery, arteriographic intervention is the treatment of choice, even though quality improvement is required. Surgical reconstructions are highly standardised and should be associated with perioperative mortality less than 3â%. We recommended the reconstruction of 2â vessels, for which antegrade supracoeliacal revascularisation techniques are favourable. In (threatening) septic conditions, autologous reconstructions are required. Intestinal infarction is the most serious complication of all visceral revascularisations. In recurrent occlusions of visceral arteries, it is recommended to favour and finally use a different therapeutic modality. Post-therapeutic care includes second-look operation as well as clinical examination and diagnostic imaging. Antithrombotic therapy should be initiated. The further screening of patients after intestinal revascularisation should be performed by duplex scanning. CONCLUSION: Chronically progressive occlusive disease of intestinal arteries is considered as a complex disease with challenging diagnostic and therapeutic management, in which an interdisciplinary, partly finding- and stage-dependent (also with regard to the frequency and recurrency of the specific local finding) sequential therapeutic approach (e.âg., endovascular vs. open procedure; interventionalistâ/âendovascular specialistâ/âvascular surgeon) becomes more and more relevant requiring a competent center of vascular medicine.
Assuntos
Intestinos/irrigação sanguínea , Isquemia/cirurgia , Oclusão Vascular Mesentérica/cirurgia , Procedimentos Cirúrgicos Vasculares/métodos , Angiografia , Doença Crônica , Circulação Colateral/fisiologia , Comportamento Cooperativo , Progressão da Doença , Fibrinolíticos/administração & dosagem , Humanos , Infarto/diagnóstico , Infarto/mortalidade , Infarto/cirurgia , Comunicação Interdisciplinar , Isquemia/diagnóstico , Isquemia/mortalidade , Artéria Mesentérica Superior/cirurgia , Oclusão Vascular Mesentérica/diagnóstico , Oclusão Vascular Mesentérica/mortalidade , Reoperação , Taxa de Sobrevida , Tomografia Computadorizada por Raios XRESUMO
In 550 patients with the clinical features of acute abdomen a surgical laparoscopy was performed. In 121 cases there was found an unspecific reason of the acute abdominal disease that did not require surgical therapy. In 349 cases a regional peritonitis was found, 80 times a diffuse peritonitis. The diagnostic validity of laparoscopy was 96% as compared to 42% for sonography. The laparoscopic access resulted in a complication rate of 0.2%. In 239 cases (43%) the disease could be managed laparoscopically, 190 cases (35%) required open surgery.
Assuntos
Abdome Agudo/diagnóstico , Infecções/diagnóstico , Laparoscopia , Peritonite/diagnóstico , Abdome Agudo/diagnóstico por imagem , Abdome Agudo/cirurgia , Humanos , Infecções/cirurgia , Peritonite/cirurgia , UltrassonografiaRESUMO
In 582 patients, laparoscopy was performed for acute abdominal pain and symptoms of acute abdomen. It allowed a clear diagnosis in 96% as compared to 42% by sonography and 25% by X-ray. In 134 cases (22%) laparotomy (i.e. unnecessary appendectomy) could be avoided. In 42% surgery could be performed laparoscopically, and 36% required surgery by laparotomy.