Holmium laser resection of the prostate

Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormal ciliary structure and function, impaired mucociliary clearance, and chronic middle ear, sinus, and lung disease. PCD is associated with situs inversus in approximately 50% of the patients. One proposed explanation for this relationship is that normal ciliary function plays a role in normal organ orientation, whereas organ orientation in PCD is a random event because of dysfunctional cilia in early embryonic development. Another hypothesis for the association between PCD and situs inversus is that mutated genes in PCD not only cause defective cilia, but are also linked to the control of organ laterality, such that abnormalities in this molecular pathway result in random left-right asymmetry. We report on a set of monozygotic twin women with PCD. In both patients, deficiency of the inner dynein arms was noted on ciliary ultrastructural analysis, associated with a clinical syndrome of bronchiectasis, chronic sinusitis, and middle ear disease. One of the twins has situs solitus, the other has situs inversus totalis. DNA analysis confirmed that the twins are monozygotic. This is consistent with the hypothesis that situs inversus occurring in patients with primary ciliary dyskinesia is a random but "complete" event in the fetal development of patients with PCD.     

Primary ciliary dyskinesia: diagnosis and standards of care

Primary ciliary dyskinesia (PCD) is characterized by disease of the upper and lower respiratory tract, in association with visceral mirror image arrangement in 50% of cases, due to abnormal structure and/or function of cilia. The purpose of this paper is to review the clinical features, diagnosis and management of PCD. Presentations include neonatal respiratory distress, recurrent lower respiratory tract infection, chronic rhinosinusitis and male infertility. PCD enters the differential diagnosis of bronchiectasis, atypical asthma, and unusually severe upper airway disease. Diagnosis is by a cascade of investigations, starting with the saccharin test in patients older than 10 yrs; ciliary beat frequency and pattern on light microscopy; and electron microscopy to assess ciliary morphology and orientation. It is important not to confuse primary and secondary ciliary abnormalities. Nasal nitric oxide is low in PCD, and this measurement shows promise as a screening test for PCD. Diagnosis is important, in order to prevent the development of bronchiectasis and to avoid any unnecessary otorhinolaryngological procedures. Regular follow-up is essential, and management should be multidisciplinary, with input from centres with a special interest in PCD, having access to paediatric and adult chest physicians, otolaryngologists and audiological physicians, physiotherapists, counselling services and fertility clinics. The prognosis is good, but morbidity can be considerable if PCD is incorrectly managed.     

Do nasal ciliary changes reflect bronchial changes? An ultrastructural study

Ciliary dyskinesia is characterized by recurrent respiratory tract infections secondary to abnormal ciliary structure and function. The diagnosis of ciliary dyskinesia is based on the detection of axonemal ultrastructural abnormalities (AUA) is respiratory mucosa samples. In most cases, the diagnosis of AUA is made on samples obtained from nasal ciliated cells with little discomfort to the patient. However, no studies have been performed in the same patient to confirm whether nasal samples reflect bronchial ciliary changes. To answer this question and to determine whether it is necessary to sample bronchial cells for the diagnosis of ciliary dyskinesia, we investigated 12 patients (between the age of 5 and 63 yr) with chronic sputum production. The presence of situs inversus, bronchiectasis, chronic sinusitis, and sterility was investigated to determine an inherited disorder. Two groups were established: Group 1 = six patients with an inherited disorder and Group 2 = six patients without evidence of an inherited disorder. Samples were obtained by brushing or biopsy of nasal and bronchial mucosa and were processed for transmission electron microscopy. In Group 1, the mean AUA was 65.2 +/- 11.4%. The following predominant axonemal defects were found: absence of dynein arms (DA) (four patients), central complex abnormalities (CC) (one patient), and various AUA (one patient). Nasal and bronchial samples correlated significantly for total AUA (r' = 1, p < 0.01) and for outer DA defects (r' = 0.96, p < 0.05). A good but not significant correlation was found for inner DA (r' = 0.83) and peripheral microtubule (PM) defects (r' = 0.71). In Group 2, the mean AUA was 9.6 +/- 2.3%.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fine particulate organic material in the Los Angeles Basin-I: assessment of the high-volume Brigham Young University organic sampling system, BIG BOSS

BACKGROUND AND OBJECTIVE: Acute generalized, widespread bleeding is often related to disseminated intravascular coagulation (DIC), a pathologic process which complicates the clinical course of many diseases and is characterized by huge amounts of thrombin and plasmin within the circulation. The final result is the consumption of platelets, coagulation factors and inhibitors, as well as secondary hyperfibrinolysis, all leading to diffuse hemorrhage and microthromboses. This review article examines the present attitudes to the diagnosis and treatment of overt DIC in clinical practice, emphasizing the importance of an accurate differential diagnosis from some other processes characterized by acute generalized, widespread bleeding. INFORMATION SOURCES: The authors have been working in this field, both at experimental and clinical levels, contributing original papers for many years. In addition, material examined in this review includes articles published in journals covered by MedLine, recent reviews in journals with high impact factor and in relevant books on hemostasis and thrombosis. STATE OF ART AND PERSPECTIVES: DIC is an intermediary mechanism of disease which complicates the clinical course of many well-known disorders. Although the systemic hemorrhagic syndrome is the predominant clinical manifestation, massive intravascular thrombosis frequently occurs contributing to ischemia and associated organ damage, making the mortality rate of this condition high. Current concepts on the pathophysiology, laboratory diagnosis and management of DIC are presented. Complex pathophysiological interrelations make the diagnosis of the etiology of the DIC difficult in clinical practice, although simple tests are useful for identification of patients with the process. Laboratory diagnosis of DIC is mainly based on screening assays, which allow a rapid diagnosis, whereas some other highly sensitive but more complex assays are not always available to routine clinical laboratories. The management of DIC is based on the treatment of the underlying disease, supportive and replacement therapies and the control of the coagulation mechanisms. Although some advances have been achieved, management decisions are still controversial, so that therapy should be highly individualized depending on the nature of the DIC and severity of clinical symptoms. Many syndromes sharing common findings with DIC, such as primary hyperfibrinolysis or thrombotic thrombocytopenic purpura, should be excluded. Finally, new therapeutic approaches to the management of this potentially catastrophic syndrome are required.     

Psychiatric perspective of pediatric human immunodeficiency virus infection

BACKGROUND: Infection due to human immunodeficiency virus (HIV) has become a chronic disease of childhood, with increasing rates among adolescents and longer survival of those infected. This illness and its victims present a continuing challenge to the medical community. METHODS: We used computerized literature searches (MEDLINE and AIDSline) to identify research and review papers from medical, psychiatric, and psychology journals; we obtained statistics directly from the Centers for Disease Control and Prevention, National AIDS Clearinghouse. RESULTS: This overview of the topic includes epidemiology, transmission, diagnosis, psychiatric and neuropsychiatric manifestations, intervention, and the impact on families, caretakers, and health care workers. CONCLUSIONS: To psychiatrists, this disease presents the epitome of the biopsychosocial model, encompassing the biology of a viral disease with psychiatric manifestations, complicated by the societal place in which it has become entrenched.     

Clinical observation on 206 cases with lower limb deep venous thrombosis treated by integrated traditional Chinese and Western medicine]

Primary psoas abscess is an uncommon disease. In children, clinical manifestations is often inspecific leading to diagnostic delay. The authors relate a primary pyogenic abscess of the psoas muscle in a 7-year-old girl with secondary septic arthritis of the hip. Confirmation of diagnosis was established by computerized tomography (CT) of the abdominal and pelvic areas. A review of the literature is presented about clinical manifestations, pathogenesis, differential diagnosis, etiology, diagnostic and therapeutic management of this infrequent entity.     

Hepatic encephalopathy

The syndrome of hepatic encephalopathy has mystified physicians since the time of Hippocrates, and it continues to do so. It is difficult to succinctly define, diagnose with certainty, or attempt to explore its pathogenesis. The literature on this topic is confusing and often contradictory. Nevertheless, very effective empirical therapy has been devised for this syndrome. We discuss selected aspects of diagnosis, pathogenesis, clinical manifestations, and treatment of hepatic encephalopathy. Emphasis is placed on the three-pronged clinical strategy to manage hepatic encephalopathy that encompasses both diagnostic and treatment measures. The burgeoning area of subclinical hepatic encephalopathy is discussed in more detail than in most reviews of this topic. We also propose a new term for the field-acute liver failure-associated hepatic encephalopathy (ALFA-HE)-to replace the unsatisfactory older term, fulminant hepatic failure.     

A case of atypical ectopic pregnancy

The authors hospitalized and treated in 1990-1994 at their Clinic 127 patients where giardiasis was the main or secondary diagnosis. They wanted to draw attention to the increasing prevalence of the disease, to clinical manifestations and problems associated with treatment. Clinicians should consider the possible presence of this disease in different gastrointestinal manifestations, chronic hepatitis, hepatopathies as well as in acute IgM anti-HAV, HBsAg and HCV negative hepatitis.     

Inflammatory bowel disease

Although ulcerative colitis and Crohn's disease are relatively uncommon disorders, most primary care practices include a number of individuals with these diagnoses. Much of the initial evaluation and long-term care of these patients is managed or coordinated by their primary care physicians. A familiarity with current principles of diagnosis and treatment is essential. Ulcerative colitis and Crohn's disease are related, immunologically mediated disorders of unknown cause. Both are characterized by chronic relapsing courses, frequent need for surgical intervention, and increased colorectal cancer risk. Significant differences are seen between these two inflammatory bowel disease syndromes, in their histopathologic features, clinical manifestations, and response to treatment. This review focuses on the colorectal manifestations of inflammatory bowel disease, emphasizing clinical presentation, approach to diagnosis, medical and surgical management, and long-term prognosis.     

The effects of vasculitis on the gastrointestinal tract and liver

Vasculitis can affect every organ of the digestive system. In many cases, it may first present with gastrointestinal symptoms. In several forms of vasculitis, including Churg Strauss syndrome, Henoch-Sch?nlein purpura, and lupus, the majority of patients have gastrointestinal involvement. The astute gastroenterologist should consider vasculitic causes of the symptoms seen in many patients. Making the correct diagnosis requires a thorough understanding of the potential role of vasculitis in causing these symptoms and the appropriate path to making a diagnosis. This article reviews the variety of manifestations of vasculitis on the digestive system, and emphasizes diagnosis and clinical manifestations.     

Autoimmune interstitial nephritis and hepatitis in polyglandular autoimmune syndrome

A 6-year-old female with polyglandular autoimmune syndrome type I, chronic active hepatitis, and renal failure is described. The renal biopsy demonstrated advanced tubulointerstitial disease with antibodies directed against tubular basement membranes. The patient's serum contained circulating antibodies directed against both renal and hepatic parenchyma. Renal disease culminating in renal failure and anti-tubular basement membrane disease have not been previously reported in association with polyglandular autoimmune disease. We describe for the first time a patient with polyglandular autoimmune syndrome, chronic active hepatitis, circulating antibodies directed against both renal and hepatic parenchyma, and primary tubulointerstitial disease culminating in renal failure.     

Hepatopulmonary syndrome: the paradigm of liver-induced hypoxaemia

The current chapter deals with the concept, clinical manifestations and diagnostic tools of the hepatopulmonary syndrome (HPS) and highlights its most salient pathophysiological, mechanistic and therapeutic aspects. Defined as a clinical triad, including a chronic liver disorder, pulmonary gas exchange abnormalities and generalized pulmonary vascular dilatations, in the absence of intrinsic cardiopulmonary disease, this entity is currently growing in interest with both clinicians and surgeons. The combination of arterial hypoxaemia, high cardiac output with normal or low pulmonary artery pressure, and finger clubbing in a patient with advanced liver disease should strongly suggest the diagnosis of HPS. Its potential high prevalence together with failure of numerous therapeutic approaches depicts a life-threatening unique clinical condition that may dramatically benefit with an elective indication of liver transplantation (LT). A better orchestration of the concepts of the pathophysiology of this lung-liver interplay may foster our knowledge and improve the clinical management and indications of LT.     

Imaging findings in hypertensive encephalopathy

Hypertensive encephalopathy is a neurologic syndrome caused by a marked and rapid rise of blood pressure above baseline levels. Efficacy of current anti-hypertensive drugs greatly diminished the frequency of this situation in which diagnosis is essentially based on clinical parameters. This can justify the few papers found in radiologic literature. Nevertheless it is sometimes important to exclude ischemic or hemorrhagic complications or establish a differential diagnosis with other neuropathologic conditions. Then a striking imagiologic picture of focal or diffuse reversible edema of cerebral white matter can be found. We present three clinical cases of hypertensive encephalopathy. Imagiologic findings are reviewed and correlated with the pathophysiologic basis of the disease.     

Systemic calciphylaxis revisited

A syndrome characterized by rapidly progressive ischemic necrosis involving large areas of the skin and muscle, and by peripheral gangrene associated with extensive vascular calcifications was observed in a patient with end-stage renal failure on chronic hemodialysis. In an effort to control the disease, parathyroidectomy was performed which resulted in rapid improvement of tissue perfusion. However, the patient eventually died from sepsis within 2 months after admission. This case presents the typical features of the syndrome of systemic calciphylaxis. The literature is reviewed searching for similar cases of this poorly recognized, but life-threatening, clinical syndrome. The pathogenesis, clinical manifestations, and therapy of this unusual and rapidly progressive, but potentially reversible, condition are reviewed with emphasis on its prompt recognition and appropriate management.     

Clinical judgement and decision making in practice. A patient with hemoptysis

A 43-year-old man was admitted because of severe and recurring haemoptysis, which was eventually ascribed to Goodpasture's syndrome. In case of an aspecific sign such as haemoptysis experienced clinicians will try to restrict the differential diagnosis by concentrating on other signs that are relevant to the main problem. In haemoptysis these other signs are general illness, fever, shortness of breath, painful respiration, chronic productive cough, and signs or a history of cardiac or pulmonary disease. If the differential diagnosis consists mainly of rare disorders or rare manifestations of common disorders, all possibilities should be considered. If Goodpasture's syndrome is suspected, further specific diagnostic tests should be done without delay.     

Adams-Oliver syndrome: a case with juvenile chronic myelogenous leukemia and chylothorax

We report on a patient with Adams-Oliver syndrome and report new findings: a chylous pleural effusion and juvenile chronic myelogenous leukemia. Also, our patient had congenital heart disease, confirming that heart lesions are a manifestation in this syndrome. The major manifestations of this disorder are summarized. Included are cases not previously recognized as having Adams-Oliver syndrome identified in a literature survey. Distal limb deficiency is commonest with more frequent and more severe involvement of the lower limbs. Scalp defects are the second commonest manifestation, while an underlying skull defect is not infrequent. Cutis marmorata telangiectatica and dilated scalp veins are significant signs of this condition. This review highlights unresolved questions about Adams-Oliver syndrome.     

Reviewing the reviews: the example of chronic fatigue syndrome

OBJECTIVE: To test the hypothesis that the selection of literature in review articles is unsystematic and is influenced by the authors' discipline and country of residence. DATA SOURCES: Reviews in English published between 1980 and March 1996 in MEDLINE, EMBASE (BIDS), PSYCHLIT, and Current Contents were searched. STUDY SELECTION: Reviews of chronic fatigue syndrome (CFS) were selected. Articles explicitly concerned with a specialty aspect of CFS and unattributed, unreferenced, or insufficiently referenced articles were discarded. DATA EXTRACTION: Record of data sources in each review was noted as was the departmental specialty of the first author and his or her country of residence. The references cited in each index paper were tabulated by assigning them to 6 specialty categories, by article title, and by assigning them to 8 categories, by country of journal publication. DATA SYNTHESIS: Of 89 reviews, 3 (3.4%) reported on literature search and described search method. Authors from laboratory-based disciplines preferentially cited laboratory references, while psychiatry-based disciplines preferentially cited psychiatric literature (P = .01). A total of 71.6% of references cited by US authors were from US journals, while 54.9% of references cited by United Kingdom authors were published in United Kingdom journals (P = .001). CONCLUSION: Citation of the literature is influenced by review authors' discipline and nationality.     

Usefulness of cytopathology and histology in the evaluation of Barrett''s esophagus in a community hospital

BACKGROUND: Beh?et's syndrome is an immune-mediated connective tissue disorder, and its primary manifestations are oral and genital ulcerations. To our knowledge no cases of adverse fetal outcome have been reported in pregnancies complicated by this disease. CASE: A 27-year-old primigravid woman with a diagnosis of Beh?et's disease came to our institution during the first trimester. Her pregnancy was complicated by several exacerbations of her disease including vaginal and oral ulcerations and abdominal pain. She was treated with steroids throughout her pregnancy. She had ruptured membranes and evidence of fetal distress at 361/2 weeks and subsequently delivered a severely growth-restricted fetus (< 3rd percentile). CONCLUSION: Pregnancies complicated by Beh?et's disease should be monitored closely for evidence of intrauterine growth restriction and fetal compromise, as are pregnancies complicated by similar connective tissue disorders.     

Parkinsonism after a wasp sting

A 49-year-old man had mild parkinsonism after being stung by a wasp, a member of the Hymenoptera order. His clinical course was stable for 6 months after which his condition rapidly progressed to a severe akinetic-rigid syndrome with evidence, on a magnetic resonance imaging brain scan, of marked destruction of the basal ganglia. The symptoms did not respond to standard antiparkinsonian medications. Repeated courses of plasmapheresis followed by monthly intravenous infusions of immunoglobulin and long-term administration of azathioprine halted and appeared to partially reverse his deterioration. The literature on the neurologic, particularly the extrapyramidal, manifestations of stings by insects of the Hymenoptera order is reviewed and the possible pathophysiological mechanisms of injury are discussed. Hymenoptera stings should be included in the differential diagnosis of acute and chronic extrapyramidal syndromes.     

1998年云南冶金分析年评

评述了云南学者１９９８年期间在国内外发表的有关冶金分析的研究论文。内容包括：综述、学术会议、分离富集方法、滴定分析、分子光谱分析、原子光谱分析、电分析化学和其它分析方法等。引用文献１５５篇