Primary sclerosing cholangitis: diagnostic and therapeutic problems

Progressive destruction of bile ducts in primary sclerosing cholangitis (PSC) may lead to end-stage liver disease, portal hypertension and liver failure. The diagnosis of PSC is made by characteristic multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts observed by cholangiography. Magnetic resonance cholangiography is considered to have comparable accuracy to endoscopic retrograde cholangiography (ERC) in the diagnosis of PSC, but its accuracy is limited in early stages of PSC. Up to 60% of patients with PSC develop a dominant stricture of the intra- or extrahepatic biliary tree. Patients may present with jaundice, pruritus or ascending cholangitis. Therefore, in patients with an increase in serum bilirubin and/or worsening pruritus, progressive bile duct dilation on imaging studies and/or cholangitis seen via ERC, it is recommended to exclude a dominant stricture. However, in a considerable number of patients without symptoms, a dominant stricture can be detected on the cholangiogram. The cholangiographic findings and the clinical presentation make it difficult to distinguish PSC from cholangiocarcinoma. The accuracy in the distinction between these two conditions is still rather disappointing, despite the combined use of imaging, endoscopic biopsy, and cytology. Medical, endoscopic, and surgical therapies aim to slow the progression of the disease. It is generally agreed upon that patients with symptoms from dominant strictures like cholangitis, jaundice, pruritus or worsening biochemical indices are candidates for endoscopic therapy. Although the best therapeutic endoscopic approach for these patients is still under discussion, a number of reports have documented clinical and radiographic improvement in patients following endoscopic dilation with or without placement of a biliary stent. Furthermore, indirect evidence by retrospective studies suggests that endoscopic therapy may improve survival.     

Brown bowel syndrome: diagnostic and therapeutic problems]

A new case of a brown bowel syndrome is presented in a 27 year-old man, clinically with severe esteatorrhea of many years of duration, with moderate pancreatic exocrine insufficiency and intestinal motility disorders suggesting a functional change secondary to a deposit of lipofucsin. We comment the pathogenic, diagnostic and therapeutic implications related to this circumstance.     

Idiopathic anaphylaxis: variants as diagnostic and therapeutic problems.

Idiopathic anaphylaxis presents a problem requiring rapid diagnosis and initiation of therapy. Some cases are complex and difficult to assess. We present four cases of unusual complexity to illustrate diagnostic and therapeutic problems. Two cases were found not to be idiopathic anaphylaxis, one being undifferentiated somatoform idiopathic anaphylaxis and the other very severe urticaria. Various conditions can be or mimic idiopathic anaphylaxis, and patience and observation can result in reasonable outcomes.     

Acquired von Willebrand syndrome: diagnostic problems and therapeutic options

We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Willebrand factor (VWF) propeptide level and a very short half-life of VWF after a test infusion with factor VIII/VWF concentrate confirmed the diagnosis AVWS. Two major surgical procedures were successfully managed using high-dose intravenous immunoglobulin. The differential diagnosis with congenital VWD and the diagnostic and therapeutic approaches of AVWS are discussed. We conclude that the diagnosis of AVWS relies primarily on clinical suspicion and a careful bleeding history. A correct diagnosis is essential for optimal perioperative management and treatment of bleeding episodes.     

上海市梅毒病例诊断治疗现状调查

目的 了解上海市梅毒诊断、治疗和随访的情况.方法 采用统一设计的调查问卷,对性病门诊保存的梅毒病史进行回顾性调查.结果 在5个医院的性病门诊共调查了1 669份梅毒病历,潜伏梅毒病例和非潜伏病例诊断依据中,分别仅有15.0%和13.6%有梅毒接触史的记录.1 413例(84.7%)梅毒患者接受梅毒治疗,其中1 220人(86.3%)接受青霉素类药物治疗,203人(14.4%)使用非青霉素类药物治疗,没有未进行青霉素皮试的病史记录.13.2%(221/1669)人接受梅毒随访.结论 应当努力提高和完善上海市梅毒诊断、治疗和随访工作,以遏制梅毒的流行.     

Dysphagia, diagnostic and therapeutic measures]

Dysphagia is a symptom of organically or functionally conditioned diseases of the oesophagus. In all circumstances it demands an immediate clarification of the etiology considering appropriate therapeutical measures and above all may be first reference to the carcinoma of the oesophagus.     

Clinical, diagnostic, and therapeutic aspects of familial hypercholesterolemia

Heterozygous familial hypercholesterolemia (FH) is a common inherited disorder of lipoprotein metabolism. FH is characterized by elevated levels of low-density lipoprotein cholesterol, the presence of tendon xanthomas, and premature cardiovascular disease. The underlying molecular defect of FH consists of mutations in the gene coding for the low-density-lipoprotein-receptor protein, detection of which provides the only unequivocal diagnosis. Although the cause of FH is monogenic, there is wide variation in the onset and severity of atherosclerotic disease in these patients. Additional atherogenic risk factors of environmental, metabolic, and genetic origin are presumed to influence the clinical phenotype in FH. Criteria used to identify individuals with FH include a combination of clinical characteristics, personal and family history of early coronary artery disease, and biochemical parameters. Since the introduction in 1989 of statins, which have been shown to be effective and to delay or prevent the onset of cardiovascular disease, drug treatment of FH has greatly improved. New lipid-lowering agents are presently being developed for clinical use. This review provides an update on the clinical, diagnostic, and therapeutic aspects of heterozygous familial hypercholesterolemia.     

Tuberculous meningiomyeloradiculitis--a report of two cases

Two cases of tuberculous meningitis complicated by spread to the spinal cord and nerve roots are described. Recognition of impending paraplegia by a rising cerebrospinal fluid protein content and manometric block should prompt steroid therapy as this may prevent irreversible neurological deficit.     

乳头预切开术在内窥镜逆行胰胆管造影术中的应用

目的　对乳头括约肌预切开术在内窥镜逆行胰胆管造影术（ＥＲＣＰ） 诊断和治疗中的作用及其安全性作回顾性评价．方法　７３ 例患者在行ＥＲＣＰ 诊疗时,当常规操作不能使胆系显影或胆道深部插管困难时,即用犁状拉式刀行乳头预切开,观察预切开的效果和近期并发症．结果　全组胆道造影成功率９５－９ ％ , 胆管深部插管成功率７２－９ ％ ,其中第一次操作胆道显影率９３－２ ％ , 深部插管率６２－９ ％ ． 术后并发症５ 例（６－８ ％ ） ,包括轻度胰腺炎２ 例,发热３ 例．结论　乳头括约肌预切开术是ＥＲＣＰ 诊疗中成功进入胆道的一项极有效的方法,但需熟练的内镜医师操作． 采用犁状刀进行预切开安全性高,并发症少．     

Central hypothyroidism: pathogenic, diagnostic, and therapeutic challenges

Context: Central hypothyroidism (CH) is a particular hypothyroid condition due to an insufficient stimulation by TSH of an otherwise normal thyroid gland. This condition raises several challenges for clinicians; therefore, a review of the most relevant findings on CH epidemiology, pathogenesis, and clinical management has been performed. Methodology: The relevant papers were selected by a PubMed search using appropriate key words. Main Findings: CH can be the consequence of various disorders affecting either the pituitary gland or the hypothalamus, but most frequently affecting both of them. CH is about 1000-fold rarer than primary hypothyroidism. Except for the neonatal CH due to biallelic TSHβ mutations, the thyroid hormone defect is rarely as profound as can be observed in some primary forms. In contrast with primary hypothyroidism, CH is most frequently characterized by low/normal TSH levels, and adequate thyroid hormone replacement is associated with the suppression of residual TSH secretion. Thus, CH often represents a clinical challenge because physicians cannot rely on the systematic use of the "reflex TSH strategy." The clinical management of CH is further complicated by the frequent combination with other pituitary deficiencies and their substitution.     

Pathophysiologic, diagnostic, and therapeutic aspects of the metabolic syndrome

The metabolic syndrome, characterized by increases in waist circumference, blood pressure, and triglyceride concentrations combined with reduced high-density lipoprotein and evidence of glucose intolerance, results from the interaction of visceral or central obesity with insulin resistance. This syndrome presents a clinical situation of systemic inflammation and increased cardiovascular risk. Blood pressure, even if only in the "prehypertensive" range, plays an important role in increasing the risk of cardiovascular disease. Recognition and treatment of each individual component of the metabolic syndrome is critical in reducing cardiovascular risk. Treatment should begin with lifestyle changes, including diet, exercise, and weight reduction. Antihypertensive therapy should be directed toward reduction of blood pressure to levels as close to optimal (<120/80 mm Hg) as feasible, and treatment protocols that do not cause worsening of glucose intolerance should be selected. Therapy for dyslipidemia should be directed at reducing triglycerides and increasing high-density lipoprotein. Glucose-lowering agents may be indicated, and drugs such as metformin and thiazolidinediones, which reduce insulin resistance, should form the basis of therapy. Carefully chosen therapy will effectively improve cardiovascular outcomes.     

Unproven diagnostic and therapeutic techniques

Over the years, there have been many procedures that either have no diagnostic value for any allergic disease or are inappropriate for the diagnosis and treatment of allergy. These procedures fall into the category of unproven diagnostic and therapeutic techniques for allergy. Unfortunately, there are a very limited number of well-controlled investigations examining these various methods. While these tests may provide a superficial appearance of valid test, they have not been shown by controlled clinical trials to be reliable in the diagnosis and treatment of any allergic diseases. After reviewing the data, it is very clear that there is a need for more well-controlled scientific investigations examining all of these techniques. Until that data becomes available for scientific review and critique, these unproven diagnostic and therapeutic techniques should not be used in the evaluation of patients with suspected allergic disease.