肾性骨病发病机制研究及进展

肾性骨病（renal osteopathy）,泛指继发于肾脏疾病的代谢性骨病,是慢性肾脏病晚期与血液透析患者的重要并发症。广义的肾性骨病是指一切和肾脏有关的骨病;狭义的肾病骨病也称肾性骨营养不良（ROD),即慢性肾脏病矿物质和骨代谢紊乱（CKD-MBD）,是慢性肾功能衰竭（CRF）时由于钙、磷及维生素D代谢障碍,继发甲状旁腺机能亢进,酸碱平衡紊乱等因素而引起的骨病。严重影响患者的生活质量。本文论述了肾性骨病的概念、肾性骨病的发病机制、肾性骨病的分型、与肾性骨病相关的分子通路、肾性骨病的治疗原则。旨在为临床肾性骨病的诊断、治疗提供参考。     

The association between renal stone disease and cholesterol gallstones: the easy to believe and not hard to retrieve theory of the metabolic syndrome

Renal stone disease and gallstone disease are widely prevalent and costly disease across the globe. Both renal stone disease and gallstone disease are associated with a variety of diseases including obesity, metabolic syndrome, dyslipidemia, hypertension, insulin resistance diabetes and gout. Importantly, the presence of either renal stone disease or gallstone disease is associated with an increased risk of cardiovascular disease. In a recent study of the Atherosclerosis Risk in Communities (ARIC), individuals with a history of gallstones were 54% more likely to report a history of nephrolithiasis after adjusting for age, gender, body size and other factors. Furthermore, in three large cohorts including over 240,000 subjects: the Nurses’ Health Studies (NHS) I and II and the Health Professionals Follow-up Study (HPFS), showed that gallstone disease is independently associated with nephrolithiasis. The mechanisms linking gallstone disease and renal stone disease are complex and not yet established. Insulin resistance, lithogenic diets, alterations of transporters in gallbladder and urinary system, and pH are possible potential mechanisms for future exploration. How the liver communicates with kidney in individuals with renal stone disease and gallstone disease is not well known and whether this communication is similar as in hepto-renal syndrome is subject for future research. Further research is needed to determine: (i) the underlying mechanisms of renal stone disease and gallstone disease; (ii) the potential treatment of renal stone disease and gallstone disease.     

Renovascular hypertension due to Takayasu arteritis--a case report

A 18-year old women with bilateral renal artery stenosis and renovascular hypertension as a part of Takayasu's disease is presented. Takayasu's disease is a chronic arteritis of unknown origin, primary affecting aorta and its branches. The disease is the most common in young women and is more common in the countries of eastern Asia in comparison with Western countries. Takayasu's disease can be severe and lifetreating disease. Mortality and morbidity depend on both the direct effect of the vascular lesions and the complications od disease. The mainstay of Takayasu's disease is based on the use of glucocorticoids alone or in association with cytotoxic drugs (cyclofosfamid, azatioprin, metotrexate). In the majority of cases, the partial control of disease is obtained.     

Von Hippel-Lindau disease simulating polycystic kidney disease

Polycystic kidney disease and the renal manifestations of von Hippel-Lindau disease have much in common. Making the distinction between these two diseases is important. There is a strong association of renal cell carcinoma with von Hippel-Lindau disease, whereas renal cell carcinoma is rare in polycystic kidney disease. Furthermore, the many extrarenal manifestations of von Hippel-Lindau disease are serious and can be fatal while those of polycystic kidney disease are generally benign. Early diagnosis of the lesions of von Hippel-Lindau disease could lead to effective surgical treatment and prevent death. A case of von Hippel-Lindau disease is presented which was incorrectly diagnosed as polycystic kidney disease for sixteen years. The case is instructive in that the possibility of making the correct diagnosis prior to the patient's terminal illness was only through careful assessment of the family. The case is also remarkable in that the patient suffered from progressive renal failure requiring hemodialysis, which has not been associated previously with von Hippel-Lindau disease.     

克罗恩病并发结直肠癌的研究进展

克罗恩病是一种主要累及消化道的自身免疫相关的炎症性疾病.近年来,该病的发病率和患病率在亚洲地区急剧上升,而结直肠癌是其严重的并发症之一.因此,了解克罗恩病癌变的现状与机制,加强对患者的癌症筛查与监测,对于降低克罗恩病患者的癌症病死率具有重要意义.本文对克罗恩病并发结直肠癌的倾向和分子机制,以及目前国际上对克罗恩病患者结直肠癌预防的研究进展进行综述.     

Lyme disease presenting as popliteal cyst in children

Lyme disease is the most common tick-borne disease in North America. Our review of the literature found few reports of Lyme disease presented in the orthopaedic literature. However, Lyme disease presenting as a popliteal cyst, with or without rupture, is rarely reported. We present 4 cases of Lyme disease that initially presented to our pediatric orthopaedic clinic for treatment of a popliteal cyst. The early diagnosis and treatment of Lyme disease may help prevent the often-devastating long-term sequelae of Lyme disease. The goal of this article is to increase the awareness of Lyme disease presenting in children as a popliteal cyst.     

Lyme disease and the orthopaedic implications of lyme arthritis

Lyme disease is the most common tick-borne disease in the United States and Europe. Increased awareness of the clinical manifestations of the disease is needed to improve detection and treatment. In the acute and late stages, Lyme disease may be difficult to distinguish from other disease processes. The epidemiology and pathophysiology of Lyme disease are directly related to the Borrelia burgdorferi spirochete and its effects on the integumentary, neurologic, cardiac, and musculoskeletal systems. Lyme arthritis is a common clinical manifestation of Lyme disease and should be considered in the evaluation of patients with monoarticular or pauciarticular joint complaints in a geographic area in which Lyme disease is endemic. Management of Lyme arthritis involves eradication of the spirochete with antibiotics. Generally, the prognosis is excellent. Arthroscopic synovectomy is reserved for refractory cases that do not respond to antibiotics.     

Mondor病的诊治进展

目的 探讨Mondor病的流行病学、病因、病理、临床表现、诊断治疗、复发和预后。方法 复习国内、外文献，结合我们自己的临床工作经验，对Mondor病进行全面分析。结果 Mondor病作为一种罕见病在临床上较易误诊，但预后一般较好。目前尚不清楚该病是否与乳腺癌发病有关。结论 对Mondor病的诊断和治疗应引起重视，应警惕是否伴发乳腺癌。     

The cholesterol paradox is flawed; cholesterol must be lowered in dialysis patients

In the general population, elevated cholesterol is associated with cardiovascular disease and mortality and lowering cholesterol is associated with improved outcomes. This reflects the predominance of isolated atherosclerotic coronary disease in the general population. In patients with renal disease, however, the relationship between serum lipids and cardiovascular outcomes is much less clear and even reversed. In our opinion, the relationship between cholesterol and coronary disease is obscured by high levels of co-morbid disease, malnutrition, inflammation, atypical dyslipidemia and the fact that myocardial infarction is not the typical presentation of cardiovascular disease in patients with renal disease. Thus, cholesterol lowering will still be effective in patients with chronic kidney diseases.     

Management of locally advanced prostatic carcinoma by surgery: does screening for prostatic cancer make sense?

Summary Screening for prostatic cancer would be of advantage only if removal of the small volume localized malignancy provided enhanced disease control over that seen in patients with larger volume disease. The data which is reported herein would indicate that rarely is a focal disease truly focal and that these patients may have an increased probability of subsequent disease when the focal disease is treated conservatively. A 2 disease should be staged and treated to determine if it is organ-confined and, if so, be managed in a manner similar to clinical stage B lesions which are apparently organ-confined after appropriate staging. The data presented herein would indicate that, following radical surgery, disease which is specimen confined experiences enhanced control over that which is specimen confined, with margin-positive patients having the worse ultimate prognosis. The data strongly suggests that detection of disease while it is still organ-confined provides an increased probability of control should surgery be selected for treatment. Therefore, when considering the use of surgery for control of disease, any method which would detect the disease while it is still small volume and organ-confined would provide an enhanced probability of achieving a disease-free patient after application of treatment.     

Celiac Disease and Metabolic Bone Disease

Celiac disease is a common autoimmune gastrointestinal disorder affecting multiple organs, precipitated in genetically vulnerable persons by the ingestion of gluten. Gluten is poorly digested and is presented to the intestinal mucosa as a large polypeptide. Binding to human leukocyte antigen-DQ2 and human leukocyte antigen-DQ8 molecules on antigen-presenting cells stimulates cellular and humeral immune reactions. Although common serological tests are available to diagnose celiac disease, the diagnosis of celiac disease is often delayed or missed because of lack of recognition as the disease presentation in adults is highly variable and may be asymptomatic. Celiac disease is a common secondary cause of metabolic bone disease and delayed treatment with gluten-free diet affects bone mineral density and fracture risk, so it is crucial to diagnose and treat celiac disease promptly. In this article, we will review recent studies of celiac disease in adults and provide practical, easily accessible information for busy clinicians.     

Unusual presentation of hydatid disease

Hydatid disease is endemic in most areas in Iran where sheep are raised, and diagnosis is fairly easy in a majority of cases. However, this fairly common disease may become very difficult and even impossible to diagnose. The most important factor in diagnosing hydatid disease is the awareness of its possibility. The striking clinical resemblance between hydatid disease and malignant diseases of some organs makes the correct diagnosis essential. In countries where this disease is endemic, any growing mass or tumor should arouse suspicion of hydatid disease.     

Stage IVA thymoma: patterns of spread and surgical management

Stage IVA disease can be de novo disease or more commonly represent recurrent disease. The pleura is the most common site of relapse after thymoma resection. Local pleural disease is usually simply resected. This is usually combined with either induction or adjuvant chemotherapy. The ultimate extended surgery for advanced thymic tumors is an extrapleural pneumonectomy done for extensive pleural disease. This rarely performed operation is done for both stage IVA disease found at initial presentation and for recurrent disease as a salvage procedure. Again, these advanced patients with pleural spread are probably best managed by induction chemotherapy followed by resection.     

Fabry disease

Fabry disease resulting from a deficiency of α-galactosidase A leads to the accumulation of globotriaosylceramide in various organs. Because the disease is an X-linked recessive disorder, males tend to develop more symptoms and more severe symptoms than females. There are also some variants of Fabry disease, and cardiac variant (cardiac Fabry disease) has the dysfunctions only in heart. Cardiac manifestations in Fabry disease are initially symmetrical and concentric left ventricular hypertrophy, and later progressive cardiac dysfunction with localized thinning of the basal posterior wall. In recent years, enzyme replacement therapy has been performed as a treatment for Fabry disease, and the initiation of this therapy is expected before the cardiac fibrosis develops. Therefore, early diagnosis of Fabry disease is essential, and echocardiography is an indispensable tool for clinical practice of this disease. Then, it is necessary to remember this disease as a differential diagnosis when encountering unexplained left ventricular hypertrophy.     

A comprehensive review of coronary artery disease in patients with end-stage liver disease

The prevalence of coronary artery disease has increased in patients with end stage liver disease. In the near future, non-alcoholic steatohepatitis is expected to be the leading cause of end stage liver disease and shares common risk factors with coronary artery disease such as hypertension, hyperlipidemia, obesity and diabetes mellitus. At present, liver transplantation is the only definitive treatment for end stage liver disease, with post-operative mortality associated with the presence of coronary artery disease. Given the high prevalence of cardiovascular disease and the unique balance of pro-thrombotic and antithrombotic factors in patients with end stage liver disease, we sought to discuss the non-invasive and invasive diagnosis, medical and procedural management considerations and pre-transplant evaluation of coronary artery disease in patients with end stage liver disease.     

Castleman Disease in the Parapharyngeal Space: A Case Report and Review of the Literature

Castleman disease is most commonly found in the mediastinum, while the head and neck is the second most common location. The disease exists in a unicentric and multicentric variety and is usually successfully treated with surgical resection alone. Early identification is important for treatment planning. Castleman disease has been reported to mimic other disease processes, however there has been only one report of the disease mimicking a nerve sheath tumor in the parapharyngeal space. Here we report the second case of Castleman disease mimicking a schwannoma in the parapharyngeal space.     

Ischémie rénale bilatérale par thrombis de la micro-circulation rénale dans le cadre d’une maladie de Buerger

Buerger's disease or thombo-angiitis obliterans disease is a small vessel's vasculitis, frequently observed in young and smoker's males. Diagnosis is based on both clinical and radiological arguments. There is no specific treatment designed for this disease. We report the case of 43 years old patient presenting with an acute kidney injury associated with Buerger's disease. We reviewed the different case of kidney disease in this rare disease.     

Diagnosis of Moyamoya Disease: International Standard and Regional Differences

Moyamoya disease is a chronic, occlusive cerebrovascular disease with unknown etiology characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. These diagnostic criteria of the moyamoya disease, stated by the Research Committee on Spontaneous Occlusion of the Circle of Willis (moyamoya disease) in Japan, are well established and generally accepted as the definition of this rare entity. On the contrary to the diagnosis of definitive moyamoya disease, there is some confusion in the terminology and understanding of quasi-moyamoya disease; moyamoya disease in association with various disease entities, such as atherosclerosis, autoimmune diseases, Down syndrome, etc. Although the clinical management is not affected by these semantic distinctions, terminological confusion may interfere with the international collaboration of the clinical investigation of these rare conditions. In this article, we sought to review the international standard and regional differences in the diagnosis of moyamoya disease and quasi-moyamoya disease.     

Moyamoya disease developing from unilateral moyamoya disease--case report.

The authors report a case of unilateral moyamoya disease which developed into moyamoya disease 3 years later. Unilateral moyamoya disease is generally defined as moyamoya disease, but the exact relationship is unknown. In this case, occlusive changes developed in the stenotic carotid fork, and in a similar portion contralaterally which was intact. Follow-up 4-vessel angiography is strongly recommended even for unilateral moyamoya disease.     

Comorbidity and cardiovascular risk factors in patients with chronic kidney disease

The mortality rate among dialysis patients is high. Although guidelines have been in place to improve outcomes in dialysis patients, new emphasis is being placed on better management of patients who are pre-end-stage renal disease (pre-ESRD)-patients with chronic kidney disease (CKD). Spearheaded by the National Kidney Foundation, the National Institute of Health, and the nephrology community at large, an effort is underway to improve the care of patients with kidney disease. We hope that improvement in health and outcomes of patients with kidney disease will be optimized through attention to care before the development of advanced renal disease. Cardiovascular disease (CVD) is an important comorbidity of chronic kidney disease, and reducing cardiovascular events in this population is an important goal for the people who care for chronic kidney disease patients. In this article, we review the available literature regarding certain risk factors for cardiovascular disease: proteinuria, hyperglycemia, hypertension, homocysteine, hyperlipidemia, and inflammation. When possible, recommendations for treatment are provided based on the information reviewed.