Pathology of the lung in leukaemia and lymphoma: a study of 87 autopsies

Histopathological findings in the lungs in a series of autopsies on 87 patients suffering from various types of leukaemia or lymphoma who had received no treatment, or various combinations of radiotherapy, chemotherapy and bone marrow transplantation were reviewed. Thirteen untreated patients showed neoplastic infiltration (4), thromboembolism (4), infection (5) or amyloidosis (1). Seventy-two treated cases showed malignant infiltration (14), vascular damage (21), infections (32) and/or diffuse alveolar damage (47). One patient treated with local irradiation for myeloma had acute bronchopneumonia alone and another treated with [32P] for polycythaemia rubra vera had extensive thrombo-embolism of the large pulmonary vessels. Clinical and autopsy evidence of infection correlated very poorly. Non-infective pulmonary disease was a frequent finding. Bacterial, fungal or pneumocystis pneumonia particularly affected the chemotherapy and radiotherapy groups, while cytomegalovirus infection was seen only in the bone marrow transplant group. This study shows that diffuse alveolar damage is a common and important problem in patients treated with radiotherapy and chemotherapy.     

患有氯痤疮的三氯苯酚和五氯苯酚接触工人血清二恶英和呋喃的水平

[目的]调查患有或不患有氯痤疮的三氯苯酚（trichlorophenol,TCP）或五氯苯酚（pentachlomphenol,PCP）接触工人血清的二恶英水平,并观察患氯痤疮工人的特征。[方法]收集26名PCP工人、12名TCP工人和36名没有PCP和TCP暴露史工人的血样,并测定二恶英含量。氯痤疮的确定根据工厂医疗记录。[结果]PCP工人中,患氯痤疮与未患氯痤疮的工人相比具有更高毒性当量（根据世界卫生组织）的2378-TCDD,12378-PeCDD,123678-H6CDD,123789-H6CDD和123678-H6CDF水平。而在TCP工人中,虽然曾患有氯痤疮的工人血清中2378-TCDD水平较高,但与未患有氯痤疮者的血清水平上并无明显差异。氯痤疮的公认危险因素有首次暴露年龄较小和距第一次暴露的时间较长等,但仅在接触TPC工人中得到证实。PCP工人患氯痤疮和诊断前较长的接触时间相关。[结论]氯痤疮是二恶英高暴露的良好指标。TCP和PCP接触工人的血清二恶英同系物具有明显不同的特点。     

Acute febrile neutrophilic dermatosis in childhood (Sweet''s syndrome)

We report a case of Sweet's syndrome in childhood. Our patients suffered from repeated fevers, chest infections and had characteristic erythematous plaques. The severity of his illness increased with age and warranted oral steroid treatment. We review 16 cases reported to date in the literature and comment on the management and prognosis of the disease in childhood.     

SQUAMOUS CELL CARCINOMA IN SOUTHERN AND NORTHERN AUSTRALIA

Squamous cell carcinoma (scc) of the skin was studied in two similar populations, one living in the temperate zone of Australia, the other living in the tropics. In the tropics, the patients were significantly younger, the man to woman ratio approached unity, and women had significantly more sccs on the legs. In the temperate zone, men had significantly more on the head and neck, but women had significantly more on the upper and lower limbs.     

Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2)

Summary. Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large‐scale, pan‐European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty‐seven per cent of the non‐pregnancy‐related cases were male. Four hundred and seventy‐four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy‐seven patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.     

Rituximab and immune tolerance in severe hemophilia A: a consecutive national cohort

Summary.  Background and objectives:  he management of patients with severe hemophilia A and inhibitors to factor VIII (FVIII) resistant to standard immune tolerance is challenging. There have been recent case reports of the successful use of rituximab in up to 57% of patients as part of rescue immune tolerance regimens. Because case reports and small series are prone to the potential bias of reporting good outcomes and relatively short follow up, a consecutive cohort of all patients treated in the UK with prolonged follow up was analyzed. Methods:  A national survey of all Comprehensive Care Haemophilia Cente in the UK. Results:  A total of 15 patients were reported of whom six (40%) achieved a negative inhibitor titer by Bethedsa assay. Durable responses were unusual, observed in only 14% of cases. Clinically significant responses with either a negative inhibitor or an inhibitor titer < 5 BU mL⁻¹ and no spontaneous bleeding with FVIII replacement were observed in seven (47%) cases. Concomitant use of FVIII appeared to be important. Of the 12 patients treated with rituximab and FVIII, six (50%) achieved a negative inhibitor titer and seven (58%) had a clinically beneficial response. None of the three patients treated without FVIII responded. Conclusions:  hese data suggest that the use of rituximab combined with FVIII is a potentially useful treatment for patients with inhibitors resistant to standard immune tolerance, although sustained inhibitor eradication is uncommon.     

Future challenges for behavioural research in adolescent health

Abstract Risk to health during adolescence is more likely due to behaviour than disease. Problems arise because behaviour is distally removed from its consequences and negative reinforcement is not contiguous with it. The paper reviews recent research and the problems faced by health workers in devising educational programmes when behaviour, in the cultural value system of adolescents, is present-oriented and outcomes are future-oriented. The challenge to the behavioural scientist is to develop health education programmes which have personal meaning to adolescents. Working against this aim are such processes as adolescent taboos, locus of control, the personal fable, risk behaviours and social-cognitive immaturity. Suggestions are made for research scientists to devise and evaluate programmes to change the operational liberalism of adolescent behaviour by installing more ideologically conservative attitudes in the hope that behavioural change will follow.     

The Role of the Nurse in Labor and Delivery as Perceived by Nurses and Patients

A study was conducted to examine the role of the labor and delivery room nurse as perceived by expectant mothers trained in the Lamaze method of preparation for childbirth (L/RC), expectant mothers not trained in the Lamaze method (RC), and labor and delivery room nurses. All expectant mothers received baseline prenatal education by attending Red Cross Preparation for Parenthood. Perceptions were measured through a Q-sort of 50 items constructed for this study. Each item described a nursing behavior in labor and delivery. Differences between Q-sorts of expectant mothers and labor and delivery room nurses were insignificant. The collected data showed that labor and delivery room nurses were meeting the expectations of expectant mothers, both trained and not trained in the Lamaze method. Perceptions of the nurse's role in labor and delivery differed slightly so that individualization of care is important.     

Integration of gene maps: updating chromosome 1

The first integrated map of chromosome 1 was published in 1992. We present an updated summary map of 371 loci constructed from a location database that includes physical and genetic data. The summary map subsumes a composite physical location, sex-specific genetic location, cytogenetic assignment, mouse homology, rank and references to physical maps. The genetic length is 208 cM for the male map, in close agreement with the chiasma map, and 371 cM for the female map. There is evidence for a high level of interference on chromosome 1. The location database comprising both data and analytical software is discussed in relation to alternative approaches and possible enhancements.