Parkinson's disease. Update in diagnosis and symptom management

Parkinson's disease (PD) is a progressive neurodegenerative disorder with a high burden of morbidity. Because no diagnostic test exists for PD, clinical knowledge and skill are key to making an early, accurate diagnosis. Diagnostic criteria for PD require at least two of three motor signs: tremor, rigidity, or bradykinesia. Levodopa and the dopamine agonists are considered first-line drug therapy. Recent studies have shown a lower incidence of dyskinesia in patients who began therapy with a dopamine agonist, although levodopa may be better tolerated by patients age 70 or older. Combinations of medications and rehabilitative, alternative, and surgical therapies can often help patients achieve adequate control of PD motor symptoms and maintain a high quality of independent living.     

Kawasaki disease: diagnosis, management, and long-term implications

Kawasaki disease (KD) is an acute inflammatory vasculitis of childhood which was initially described more than 4 decades ago, yet the specific etiology remains unknown. It has become the most common cause of acquired cardiovascular disease in children in the United States. Advances in clinical therapies have reduced, but not eliminated, the incidence of coronary artery abnormalities in affected children. Pathophysiology seems to include an intense elaboration of cytokines, endothelin, and other vasoactive mediators resulting in the development of vascular endothelial changes that may leave a permanent impact on vascular integrity. Treatment with intravenous immune globulin and aspirin remains the primary management strategy and steroid therapy remains contoversial. In severe circumstances, coronary reperfusion strategies are required, and coronary artery surgery in children with KD has been required, albeit infrequently. KD may be a harbinger for early onset coronary artery disease in adults. Recently developed AHA recommendations have amended diagnostic strategies and indicated a stratified approach to the long-term follow up of this enigmatic yet widespread disease.     

von Willebrand's disease and menorrhagia: prevalence, diagnosis, and management

The reported prevalence of von Willebrand's disease (vWD) is increased in women with menorrhagia, with current estimates ranging from 5% to 20%. The consistent results of multiple studies suggest testing should be included in the evaluation of patients with menorrhagia, especially in unexplained cases and prior to surgical intervention. Although a cyclic variation in von Willebrand's factor levels has not been confirmed, several studies suggest lower levels during menses and the early follicular phase. Menorrhagia is one of the most common bleeding manifestations of von Willebrand's disease, reported by 60-95% of women afflicted with this bleeding disorder. Menorrhagia is typically severe, often resulting in anemia and interfering with quality of life. Despite the frequency of menorrhagia, there is no consensus on optimal management. Although oral contraceptives are frequently prescribed, there are no studies confirming their efficacy using objective measures of response. Desmopressin was associated with an 80-92% response rate in several uncontrolled studies relying on patient assessment of efficacy. However, a small, randomized trial found no significant reduction in menstrual blood flow compared with placebo. There are anecdotal reports of the successful use of antifibrinolytic agents alone and in combination with other therapies. There are no studies comparing the relative efficacy and safety of the available medical therapies for von Willebrand's disease associated menorrhagia. Until these studies are completed, treatment should be individualized based on von Willebrand's disease subtype, patient age, contraceptive needs, and personal preference.     

Medical management of advanced Parkinson's disease

The management of advancing Parkinson's disease (PD) is a daunting task, complicated by dynamic medication responses, side effects, and treatment-refractory symptoms in an aging patient population. The motor and nonmotor complications of advancing PD are reviewed, and practical treatment strategies are provided. Careful assessment in the context of the known natural history of advancing PD and rational treatment choices can create significant improvement in the lives of patients who have advancing PD.     

Parkinson's disease in the elderly: current management strategies

Parkinson's disease generally responds well to dopaminergic therapy, but there is no unanimity concerning the optimal time for introducing dopaminergic medication. Dose-related side effects of these drugs may respond to a drug holiday, and fluctuations in response to levodopa may sometimes be helped by addition of bromocriptine to the drug regimen. Anticholinergic drugs, tricyclic compounds, and amantadine may sometimes lead to benefit, but any antiparkinsonian effect is often disappointing. Mental side effects may occur with all of the antiparkinsonian drugs, particularly the anticholinergic agents, and especially in the elderly.     

原发性帕金森病诊断及鉴别诊断

原发性帕金森病(Idiopathic Parkinson’s disease,IPD)是一种慢性进行性的神经系统变性病,其临床表现多变,以隐匿起病、缓慢进展为特征,病理学以中脑黑质致密带含神经黑色素的细胞变性为特征。研究证明:缺少生物标记物的诊断是不精确的,可以导致相当数量的误诊。一、原发性帕金森病的诊断1.国外诊断标准:应用最广泛的是“United King-dom Parkinson’s Disease Society Brain Bank clinical di-agnostic criteria foridiopathic Parkinson Disease”,可以使诊断的精确度得到明显改善,有研究表明在该标准下最高可以达到90%的诊断精确…     

Enteral nutrition: background, indications and management

Enteral nutrition is only part of the wider field of clinical nutrition in which great advances in both theory and practice have been made over the last decade. We have attempted to summarize what we consider to be the advances that have most relevance to the clinical practice of enteral nutrition. This chapter reviews our present understanding of the processes of digestion and absorption of protein, carbohydrate and fats, and examines how this theoretical understanding can be applied to patients in the clinical situation. A broad classification of the different enteral diets is undertaken, and the reasons for the development of particular diets are discussed. The clinical value of these diets is assessed. The wide variety of indications for enteral (as opposed to parenteral) nutrition are discussed and the specific benefits of enteral nutrition for the patient are highlighted. Techniques of administration of enteral nutrition are reviewed in detail, and the methods by which enteral nutrition should be monitored are outlined. Finally, complications of enteral nutrition are summarized and advice given on how to prevent or treat them.     

Crohn's disease of the ileal pouch: reality, diagnosis, and management

Restorative proctocolectomy with ileal pouch-anal anastomosis has become the surgical treatment of choice for patients with ulcerative colitis or indeterminate colitis who require surgery. A subset of patients with ileal pouches may develop Crohn's disease or a Crohn's disease-like condition of the pouch after the surgery. Diagnosis, differential diagnosis, and management can be challenging. A combined assessment of endoscopy, histology, radiography, and examination under anesthesia is often necessary for an accurate diagnosis. A multidisciplinary approach by a medical and surgical team is advocated. It is advisable to inform ulcerative colitis patients before the surgery of the potential risk for Crohn's disease along with other inflammatory and noninflammatory complications.     

Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management

Cystic fibrosis is an autosomal recessive genetic disorder that causes dysfunction of exocrine glands, and has several clinical manifestations. Among those, sinonasal involvement is almost universal, with or without chronic sinusitis and/or nasal polyposis. This review will detail the pathophysiologic changes of the sinonasal mucosa, and the clinical manifestations, diagnosis, and treatment. Developmental anatomic abnormalities, which are identified radiologically, will also be demonstrated. Medical management is the first treatment for patients with cystic fibrosis, but effective treatment of sinonasal disease in cystic fibrosis relies heavily on surgery. In the past, nasal polyposis was the main indication for surgery, and consisted mostly of polypectomy alone. This procedure was associated with a high recurrence rate. The development of functional endoscopic sinus surgery has contributed to decreasing the morbidity of sinonasal surgery and the recurrence of nasal polyposis in cystic fibrosis. The evolution of the surgical techniques will be discussed and a review of the literature will be provided.     

Polycystic liver disease: Classification,diagnosis, treatment process,and clinical management

Polycystic liver disease(PLD) is a rare hereditary disease that independently exists in isolated PLD, or as an accompanying symptom of autosomal dominant polycystic kidney disease and autosomal recessive polycystic kidney disease with complicated mechanisms. PLD currently lacks a unified diagnostic standard. The diagnosis of PLD is usually made when the number of hepatic cysts is more than 20. Gigot classification and Schnelldorfer classification are now commonly used to define severity in PLD. Most PLD patients have no clinical symptoms, and minority with severe complications need treatments. Somatostatin analogues,mammalian target of rapamycin inhibitor, ursodeoxycholic acid and vasopressin-2 receptor antagonist are the potentially effective medical therapies, while cyst aspiration and sclerosis, transcatheter arterial embolization, fenestration, hepatic resection and liver transplantation are the options of invasion therapies.However, the effectiveness of these therapies except liver transplantation are still uncertain. Furthermore, there is no unified strategy to treat PLD between medical centers at present. In order to better understand recent study progresses on PLD for clinical practice and obtain potential directions for future researches, this review mainly focuses on the recent progress in PLD classification, clinical manifestation, diagnosis and treatment. For information, we also provided medical treatment processes of PLD in our medical center.