Pulmonary lymphangioleiomyomatosis: presentation and results of treatment

Lymphangioleiomyomatosis is a rare lung disease of unknown aetiology that affects only women. Eight premenopausal women with LAM confirmed by lung biopsy specimens were observed in 1984-2001. The most common presenting feature was exertional dyspnea (6) followed by chylous pleural effusions and pneumothoraces. In two women severe airflow obstruction was observed at presentation. HRCT revealed characteristic cysts in all cases. All women were given hormonal therapy (tamoxifen, medroxyprogesterone). The best results of treatment were achieved in cases with chylothoraces.     

Familial hypercholesterolemia: current treatment options and patient selection for low-density lipoprotein apheresis

Options for treatment of severe heterozygous and homozygous familial hypercholesterolemia prior to the statin era were limited by significant side effects and morbidity. The advent of both the statins and technology for the selective removal of LDL via apheresis have revolutionized management but challenges remain.     

Optimizing treatment options

Full and accurate diagnosis of allergic rhinitis is important as a basis for treatment decisions, as many nasal disorders have similar signs and symptoms. Optimal allergen avoidance is the starting point of treatment, so causative allergens need to be identified. Oral antihistamines are effective in relieving the majority of symptoms of allergic rhinitis and allergic conjunctivitis, but provide only partial relief from nasal congestion. Topical α-adrenergic decongestants help to relieve congestion, but prolonged use leads to rhinitis medicamentosa. Systemic decongestants are less effective than topical agents and their use is limited by systemic and central side-effects. The value of leukotriene antagonists has yet to be fully evaluated. Intranasal ipratropium bromide helps to control watery secretions, and an aerosol may be more effective than an aqueous solution. Topical glucocorticosteroids, such as triamcinolone, are the most potent and effective agents available for treating allergic rhinitis. The available evidence indicates that there is very little systemic absorption. Sodium cromoglycate is effective in allergic rhinitis, though less so than topical steroids, and has the least adverse effects among the antiallergic agents. Immunotherapy can be effective and may be indicated in individuals who cannot avoid the causative allergen. Special considerations apply to the treatment of allergic rhinitis in elderly or pregnant patients. Finally, patients with long-standing allergic conditions should be re-assessed regularly.     

A randomized trial of treatment options for alcohol-abusing workers

BACKGROUND. Employee-assistance programs sponsored by companies or labor unions identify workers who abuse alcohol and refer them for care, often to inpatient rehabilitation programs. Yet the effectiveness of inpatient treatment, as compared with a variety of less intensive alternatives, has repeatedly been called into question. In this study, anchored in the work site, we compared the effectiveness of mandatory in-hospital treatment with that of required attendance at the meetings of a self-help group and a choice of treatment options. METHODS. We randomly assigned a series of 227 workers newly identified as abusing alcohol to one of three rehabilitation regimens: compulsory inpatient treatment, compulsory attendance at Alcoholics Anonymous (AA) meetings, and a choice of options. Inpatient backup was provided if needed. The groups were compared in terms of 12 job-performance variables and 12 measures of drinking and drug use during a two-year follow-up period. RESULTS. All three groups improved, and no significant differences were found among the groups in job-related outcome variables. On seven measures of drinking and drug use, however, we found significant differences at several follow-up assessments. The hospital group fared best and that assigned to AA the least well; those allowed to choose a program had intermediate outcomes. Additional inpatient treatment was required significantly more often (P less than 0.0001) by the AA group (63 percent) and the choice group (38 percent) than by subjects assigned to initial treatment in the hospital (23 percent). The differences among the groups were especially pronounced for workers who had used cocaine within six months before study entry. The estimated costs of inpatient treatment for the AA and choice groups averaged only 10 percent less than the costs for the hospital group because of their higher rates of additional treatment. CONCLUSIONS. Even for employed problem drinkers who are not abusing drugs and who have no serious medical problems, an initial referral to AA alone or a choice of programs, although less costly than inpatient care, involves more risk than compulsory inpatient treatment and should be accompanied by close monitoring for signs of incipient relapse.     

外阴痛：病理生理因素和临床治疗新观点方面的综述

慢性外阴疼痛,简称为外阴痛,在普通妇女人群中的发病率约为16%.本文简要概述了外阴痛的病因学以及治疗现状.到目前为止,对自发性广泛性外阴痛(unprovoked generalized vulvodynia,UGVD)的研究较少,本文重点讨论了外阴痛的一个亚类,即诱发的阴道前庭痛(provoked vestibulodynia,PVD),其症状为压迫阴道前庭周围可产生严重的烧灼样痛或剧烈疼痛.以往的研究表明外周(如阴道前庭组织异常、骨盆肌张力增高)和中枢(如神经中枢冲动增多)因素都参与了PVD的发生和维持过程.此外,由于患者对疼痛的心理性反应各异,也影响了疼痛症状的表现和时程.尽管PVD的发生包含多种因素,但到目前为止,对其治疗的研究都是单一的,回顾性和不可控的.本综述着眼于对PVD的外周(如局部用药、外阴大腺切除术等)和中枢(如应用抗抑郁药、镇痛治疗等)治疗,同时还讨论了针对于痛觉传递过程中不同水平(外周和中枢)的多向治疗方案.鉴于PVD机制的复杂性,今后对PVD的治疗推荐应用生物心理学疗法.     

Idiopathic recurrent pericarditis as an immune-mediated disease: current insights into pathogenesis and emerging treatment options

Idiopathic recurrent pericarditis affects 30–50% of patients with a previous attack of pericarditis. The etiopathogenesis is incompletely understood and most cases remain idiopathic with a presumed immune-mediated pathogenesis. The mainstay of therapy is aspirin or a nonsteroidal anti-inflammatory drug plus colchicine and the possible adjunct of a low-to-moderate dose of a corticosteroid in more difficult cases. Colchicine as an adjunct to anti-inflammatory therapy reduces by 50% the subsequent recurrent rate. For true refractory cases with failure of standard combination therapies, new and emerging options especially include human intravenous immunoglobulins and biological agents (i.e., anakinra). The outcome of idiopathic recurrent pericarditis is good with a negligible risk of developing constrictive pericarditis. Thus, it is important to reassure patients on their prognosis, explaining the nature of the disease and the likely course. Moreover, therapeutic choices should include less toxic agents and favor cheaper drugs whenever possible.     

Immunotoxicity of immunotherapeutic agents

Conclusion As our knowledge of the immune system and its control expands so does the potential for the development of specific immunotherapeutic agents with few adverse or toxic actions. Selective immunosuppression to prevent graft rejection without the toxic effects of an increased susceptibility to infection or an increased incidence of cancer may be achieved if the specific cell populations responsible for each event can be well defined and then selectively affected by an immuno-therapeutic agent.Adverse toxic effects may also be avoided by carefully optimizing drug dosage and timing to affect specifically the desired immune cell population. Drug delivery to certain cell populations by specific antibody, or structural modifications of the synthetic immunosuppressive agents improve immunotherapy and result in fewer adverse effects. Toxic actions of immunotherapeutic agents such as are seen with levamisole, gold, and D-penicillamine may be related to the drug acting as an antigen and evoking an immune response to itself. Such adverse allergic reactions are much more difficult to contend with in terms of drug modification. Nonetheless, an increased knowledge of the immune system with definition of important control steps will greatly increase the potential for rational design of effective immunotherapeutic agents without immunotoxicology.     

Combined surgical and immunotherapeutic treatment of patients with fourth stage colon cancer

Sixty-five patients with the fourth stage colon cancer were subjected to the combined surgical and immunotherapy. The following conclusions are made: (1) surgical elimination of the bulk of tumor mass is a necessary prerequisite for effective immunotherapy; (2) vaccination with autological tumor cells accompanied with bacille bilié de Calmette-Guérin (BCG) as the adjuvant and with interleukin-2 as the immunostimulator effectively prevents metastasizing after successful surgery; (3) the vaccine must necessary contain living tumor cells adequately presenting tumor antigens; and (4) in some cases, immunotherapy causes undesirable autoimmune complications. They can be registered by corresponding inflammation control methods.     

肺淋巴管平滑肌瘤病临床病理学观察

目的 探讨肺淋巴管平滑肌瘤病临床、病理特征。方法 对5例肺淋巴管平滑肌瘤病临床资料进行收集分析,HE切片观察,采用免疫组织化学(SP法)检测平滑肌肌动蛋白(SMA)、HMB45、基质金属蛋白酶(MMP)2、孕激素受体(PR)、雌激素受体(ER),并进行文献复习。结果 肺淋巴管平滑肌瘤病是原因不明的肺部疾病,只发生在女性,特别是绝经前妇女。临床表现为呼吸困难,咯血,气胸和乳糜胸等。病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变。增生的平滑肌细胞免疫组织化学5例SMA、HMB45、MMP2均阳性;1例的ER和PR均阳性,1例仅ER阳性,1例仅PR阳性,1例的ER和PR均阴性。结论 育龄期妇女如反复出现自发性气胸、咯血、活动后呼吸困难应考虑肺淋巴管平滑肌瘤病的可能,病理检查可确定肺淋巴管平滑肌瘤病的诊断。     

Cyclic vomiting syndrome: treatment options

Cyclic vomiting syndrome is a disorder characterized by recurrent episodes of severe nausea and vomiting separated by symptom-free periods. Our aims were to review treatments of adult cyclic vomiting syndrome as well as to identify areas for further clinical research and the unanswered questions in this field. We conducted a PubMed search using such keywords as “cyclic vomiting syndrome,” “nausea,” “vomiting,” “treatment,” “trigger factors” and “tricyclic antidepressants” and combined this information with the knowledge and clinical research from the authors. Available data show that in adult cyclic vomiting syndrome, there is an impressive and sustained response to high-dose tricyclic antidepressants. In up to 13 % who are regarded as poor responders to tricyclic antidepressants, a predictable profile can be identified related to coexisting psychological disorders, marijuana use, poorly controlled migraine headache or chronic narcotic use. Cyclic vomiting syndrome in adults is being an increasingly recognized entity. Tricyclic antidepressants are the main treatment for controlling symptoms. Eliminating and addressing trigger factors are an essential part of management.     

Developing treatment options for metachromatic leukodystrophy

Metachromatic leukodystrophy (MLD) represents a devastating lysosomal storage disease characterized by intralysosomal accumulation of the sphingolipid sulfatide in various tissues. Three types of the disease are currently distinguished: the late-infantile, which is the most commonly observed, the juvenile and the adult type. Demyelination represents the main histopathological feature of the disorder, leading to neurological impairment with no curative treatment currently available. Nevertheless, the increased scientific interest on the disease has led to the experimental use of innovative therapeutic approaches in animal models, aiming to provide an effective therapeutic regimen for human patients, as well. This paper provides an overview of developing treatment options among patients with MLD. Apart from hematopoietic stem cell transplantation, already in use for decades, other recent data discussed includes umbilical cord blood and stem cell transplantation, enzyme replacement therapy, gene therapy and autologous hematopoietic transplantation of genetically modified stem cells. Gene therapy with oligodedroglial, neural progenitor, embryonic and microencapsulated recombinant cells represents add-on treatment options still on experimental level.