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View from beneath: pathology in focus. ENT presentation of Ollier's disease.

Cartilaginous tumours occurring in the head and neck are usually regarded and seen as solitary lesions. However, we present an unique case where the cartilaginous tumour in the ENT region was the presenting feature of Ollier's disease (multiple enchondromatosis). It is recommended that further investigations be carried out in patients with cartilaginous tumours presenting in the head and neck to exclude the possible presence of this unusual and interesting syndrome.

Adult↗

Results of prolonged bladder distension as treatment for detrusor instability.

Forty-six patients with urinary symptoms associated with unstable (uninhibitable) detrusor contractions have been treated by a total of 58 bladder distensions; 43 of these were fully re-evaluated urodynamically thereafter and none showed conversion from unstable to stable detrusor behaviour. Four patients with unstable bladders were symptomatically improved but 5 patients reported symptomatic deterioration. We have concluded that while over-distension may sometimes improve a patient's symptoms, it did not result in the abolition of detrusor contractions or the reversal of unstable detrusor behaviour.

Adolescent↗

Prenatal ultrasonographic diagnosis of atypical Nonne-Milroy lymphedema.

Nonne-Milroy lymphedema is a relatively rare disorder characterized by firm edema of the lower extremities either on the whole leg or limited to the feet or toes. We report a case of atypical Nonne-Milroy syndrome that presented prenatally with bilateral leg edema, bilateral hydrothorax and lung hypoplasia at 22 weeks' gestation. The differential diagnoses are discussed.

Adult↗

[Hereditary lymphedema (Nonne-Milroy-Meige syndrome) associated with chylothorax. Comments on 2 cases].

This paper presents a family case of brother and sister with unilateral lymphedema, set off spontaneously at prepuberty and earlier in the boy. They both developed a unilateral chylothorax at the age of 22-24. No congenital anomalies are present. The clinical and paraclinical data exclude forms of secondary lymphedema as well as the ones associated with gonadal dysgenesis. The family case history reveals that the parents are not kindred, appear healthy, without pulmonary complaints. Neither no other cases similar to the patients described nor other congenital anomalies or genetic diseases have been recorded in the family. The peculiarity of the cases consists in the fact that lymphedema is confined only to the left lower limb and that there are no modifications in the parents, suggesting an autosomal recessive transmission, which would be a pathogenic novelty. The association with chylothorax points to the importance of a thorough evaluation of the patient with lymphedema of the lower limb.

Adult↗

Renal adenocarcinoma in young adults.

Renal adenocarcinoma is rare in young people. The prognosis of the condition would appear to be better in young people justifying a radical treatment rationale even in cases of advanced disease. This report describes a series of patients under the age of 30 with renal adenocarcinoma and a review of the literature. The possible mechanisms for the apparent improved survival of patients in this age group is discussed.

Adenocarcinoma↗

Secondary amyloidosis in association with Aspergillus lung disease.

Three patients with amyloidosis secondary to bronchiectasis are described: in two patients bronchiectasis was secondary to allergic bronchopulmonary aspergillosis and in the third, post-tuberculous bronchiectasis was complicated by asthma and allergy to Aspergillus. We suggest that chronic Aspergillus allergy may cause amyloidosis and that some cases of amyloidosis ascribed to tuberculosis in the past may in fact have been secondary to Aspergillus allergy.

Amyloidosis↗

High dose epirubicin chemotherapy in untreated poorer prognosis small cell lung cancer.

A single agent chemotherapy regimen, comprising epirubicin 120 mg m-2 iv at 21-day intervals for a maximum of six cycles was administered to 26 patients with intermediate or poor prognosis small cell lung cancer. Staging consisted of both conventional disease extent and prognostic guidelines based on laboratory parameters. The overall response rate was 57% which predominantly occurred among the intermediate prognosis group (14 of 17 patients), compared with only 1 of 9 patients in the poor prognosis category. Seven patients survived for at least 12 months, including one 3-year survivor (remains disease free). Chemotherapy toxicity was easily managed and chemoresponse was generally accompanied by an improvement in perceived performance rating. The study confirms the activity of epirubicin against small cell lung cancer and emphasizes the importance of patient selection within the spectrum of small cell lung cancer.

Adult↗

Lung cancer: the importance of seeing a respiratory physician.

Patients with lung cancer present to and are managed by a variety of clinicians. In this study the effect of involvement by a respiratory physician on the diagnosis, staging, treatment and survival of a large unselected group of lung cancer patients was investigated. The study population was derived from the Scottish Cancer Registry. A total of 3,855 patients diagnosed during 1995 with lung cancer were studied. The data were validated and supplemented by references to medical records. The study found that a respiratory physician had been involved in the initial management of 2,901 (75.3%) patients. These patients were found more likely to have had the cancer diagnosis confirmed by histological methods and to have received active treatment with surgery, radiotherapy or chemotherapy. Survival, 1 yr after diagnosis was higher in patients who saw a respiratory physician (24.4 versus 11.1%) and benefit was found to have remained 3 yrs after diagnosis (8.1 versus 3.7%). Although the patients who had not seen a respiratory physician were generally older, and had more extensive disease, after correcting for age, stage and other prognostic factors, the relative hazard ratio of death for those not managed by a respiratory physician was 1.44. The data from this study supports the recommendations of recent lung cancer guidelines for the early involvement by a respiratory physician.

Adult↗

Necropsy study of association between sudden death and cardiac enzymes.

AIMS: To determine if cardiac enzymes measured at necropsy could be used to predict early myocardial infarction. METHODS: Cardiac enzyme activities were measured in body fluids at necropsy. Coroners' necropsies were grouped by gross and microscopic findings into cases of definite myocardial infarction, cases with occlusive coronary artery atheroma but no identifiable myocardial infarction, and non-cardiac cases. Pericardial fluid, peripheral venous blood, and blood from the right atrium were collected. Total creatine phosphokinase, creatine phosphokinase isoenzymes, aspartate aminotransferase and hydroxybutarate dehydrogenase activities were measured and the results analysed by logistic regression. RESULTS: The values of creatine phosphokinase and its isoenzymes were raised in those who had died of cardiac disease and were most discriminatory. Cases of early myocardial infarction without evidence of infarction on routine histological examination could be identified from enzyme activities. CONCLUSIONS: Measurement of cardiac enzymes in blood and pericardial fluid at necropsy can provide valuable additional information in cases of sudden death as a result of myocardial ischaemia which have occurred before macroscopic or microscopic evidence of myocardial infarction.

Arteriosclerosis↗

Quality of life in lung cancer patients: as an important prognostic factor.

Given that lung cancer is one of the common cancers world-wide, the implications of focusing on quality of life as well as survival require to be understood. We have carried out a study of the relationship between survival and quality of life in patients with lung cancer comparing patients those who lived with those who died within 3 months. The design of the study allowed every patient in a defined geographical area with a potential diagnosis of lung cancer to be studied from first outpatient consultation till after a definitive treatment has been given. Quality of life was measured using three standard questionnaires: the Nottingham Health Profile (NHP), the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) and its lung cancer supplementary questionnaire (QLQ-LC13) in addition to a study specific questionnaire collecting data on demographic, social, clinical and performance status. The contribution of quality of life in relation to survival adjusted for known prognostic factors was determined using Cox's proportional hazard model. In all 129 lung cancer patients were interviewed, and 96 patients were alive at 3-months follow-up. Only 90 of 96 patients alive at 3-months follow-up were assessable. Descriptive analyses showed that those who were dead had more perceived health problems, greater level of symptoms and significant lower physical and role functioning and global quality of life at presentation. On the other hand, univariate analyses showed that patients' aggregate scores on the NHP, the functioning scores, and global quality of life scores alone were significant predictors of survival (P<0.03, P<0.04, P<0.04, respectively ). The multivariate analyses showed that pre-diagnosis global quality of life was the most significant predictor of the length of survival even after adjusting for known prognostic factors (age, P<0.04; extent of disease, P<0.03; global quality of life, P<0.02), while performance status, sex and weight loss were not. This study confirmed that pre-diagnosis quality of life was a significant predictor of survival. Indeed, pre-diagnosis quality of life should be considered as a clinical status which has to be established by physicians before treatment starts as it is such an important predictor of survival.

Adult↗

The approach to the surgical management of cancer in four European countries.

Outcome of lung cancer appears poorer in the UK than elsewhere in Europe. This may be due to a less aggressive approach in treatment. This study investigated whether clinicians' perceptions of their approach differed between European countries. A questionnaire was circulated to cancer specialists in four countries (Belgium, Greece, Switzerland and the UK) asking about management. An aggression score was calculated using the proportion of standard cases that would proceed to operation at different ages and levels of pulmonary function. The principle problems suggested by most of the 314 respondents were inoperability before symptoms (particularly in the UK) and confounding effects of comorbidity. Surgeons particularly blamed delay in referral. The aggression scores (Belgium 54%, UK 49%, Switzerland 47% and Greece 37%) did not suggest the UK is an outlier, but the UK was more conservative in its approach to N2 disease and isolated cerebral metastasis. The aggression scores of surgeons were greater than those of the others (51% versus 42%). Lung cancer was felt to present late with potentially confounding symptoms, but delay in the clinical process was thought to be less important. Although the UK was more conservative with special cases, its approach to typical cases could account for differences in patient survival.

Data Collection↗

Ethical problems in the management of myelomeningocele and hydrocephalus. The Milroy Lecture 1975.

Within the last generation the application of major advances in drug therapy, intensive care, transfusion techniques, surgery, anaesthesia, and radiotherapy, together with a vast expansion of knowledge due to increased investigative facilities, have led to an unprecedented, dramatic and beneficial increase in the number of persons who would previously have died, or lived with severe handicaps, but can now be cured. Techniques have also been developed to prolong the lives of many people who are now able to enjoy several extra years of productive and meaningful existence, and to alleviate and improve the quality of life of many seriously handicapped persons, enabling them to become integrated as useful and contented members of the community. Choosing from numerous examples of paediatric experience, it is notable that many more extremely premature infants now survive without physical or intellectual damage; infection can almost always be cured, including neonatal and all other forms of pyogenic meningitis and the now rare cases of tuberculous meningitis and miliary tuberculosis. The few remaining new cases of Rh-haemolytic disease are also readily cured. There are outstanding successes in the treatment of childhood malignancy. Paediatric surgery has made great strides. The prognosis of congenital heart disease, of obstructions of the alimentary canal and many other conditions has improved beyond recognition. Unfortunately, the indiscriminate use of advanced techniques of all types has also kept alive those who would have died but now live with distressing physical or mental handicaps or both, often for many years, without hope of ever having an independent existence compatible with human dignity. There are many examples, including those who have sustained major brain or spinal cord injuries.

Adolescent↗

Invasive bladder cancer treated by radical external radiotherapy.

Fifty-three consecutive, unselected patients with invasive bladder cancer, Stage T2 to T3, treated by radical radiotherapy have been reviewed. Cystectomy was reserved for patients with significant worsening of disease during treatment, histologically confirmed persistent or recurrent invasive tumour after treatment, or patients with intolerable symptoms due to radiation cystitis. In 64% of our patients a favourable tumour response to radiotherapy was seen, while a further 31% showed disease progression either during or on completion of radiotherapy. Cystectomy was performed on 22% of patients, mainly for radiation cystitis, and was not associated with a significant operative mortality rate. The crude 5-year survival rate was 42%. We conclude that radical radiotherapy is as effective as other forms of treatment for invasive bladder cancer, but that there remains a need to identify those bladder tumours destined to respond poorly to radiotherapy at an earlier stage.

Adult↗

Lung cancer--management and outcome in Glasgow, 1991-92.

Current practice and outcome for patients with lung cancer were determined by retrospective case note review of a random sample of all lung cancer cases registered for a calendar year and augmented by review of all surgical and radical radiotherapy cases. A total of 262 patients - 231 patients less than 75 years of age and 31 patients more than 75 years of age - represented 83% of the random sample. Eighty-three per cent of patients were seen within 2 weeks of referral. One-third reported symptoms occurring for less than 1 month and one-third had experienced symptoms for more than 3 months. The median time interval from first hospital contact until the making of a management decision was 18 days. The median interval from first contact to surgery was 63 days, and to starting radical radiotherapy 70 days. Histological confirmation was obtained in 69% of patients. Ten per cent of all lung cancer patients were calculated to have received chemotherapy. Five per cent of the whole cohort had definitive surgery and 64% of these were judged to be free of the disease at 3 years. Overall survival was 9% at 3 years, with no differences relating to cell type or area of residence. Many areas of good practice have been identified, but the lack of tumour staging or performance status data, the low proportion receiving chemotherapy or definitive surgery and the poor outcome after radical radiotherapy indicate the need for prospective audit and feedback of results. The long time interval from management decision to surgery and radiotherapy suggests organizational issues which need attention.

Adult↗

Stricture disease: radiology of urethral stents.

Self-expanding metal stents are emerging as an effective alternative treatment in the management of urethral obstruction. The radiologic studies of 33 men with anterior urethral strictures (subprostatic, n = 11; bulbar, n = 22) that had recurred despite repeated optical urethrotomy and dilation were reported. In all patients, the stricture was successfully treated with stent insertion. Urethrography performed 1 month later in 19 patients showed an irregular intrastent lumen of varying degrees due to a hyperplastic urothelial reaction confirmed at endoscopy the same day. Available follow-up urethrograms in seven patients at 3 months showed that the hyperplasia was settling, and by 6 months, the intrastent lumen was smooth and of good caliber. Urethrography revealed postoperative structures in 14 patients. Strictures seen at 1 month (n = 6) were due to initial stent misplacement and were treated with the insertion of a second stent. Strictures seen 3 months after insertion (n = 4) occurred within the stent lumen and were considered to be significant at endoscopy in only one patient. Strictures that developed 6-12 months after stent insertion (n = 4) were not within the stent and were considered to represent genuine new strictures.

Adult↗

The UroLume stent in the management of benign prostatic hyperplasia.

There were 54 patients entered into this study of the UroLume permanent prostatic stent, most of whom were unfit for conventional prostatic surgery. The stents were inserted with the patient under local or regional anesthesia. Of the patients 34 presented in acute retention, 12 had chronic retention, 4 had severe and worsening symptoms, and 4 had symptoms and urodynamic evidence of obstruction occurring in the presence of Parkinson's disease. Following stent insertion 50 patients were able to void satisfactorily, while the remaining 4 presented with chronic retention and detrusor failure. The 40 patients who had no or minimal remaining symptoms were satisfied with the stent. Most patients experienced frequency and urgency of micturition for 1 to 3 months, which resolved in all but 9 patients with persistent severe detrusor instability. Symptom scores decreased to 6.5 (total) at 1 year for nonretention patients and 6.0 for retention patients. Stents were covered with epithelium within 6 to 9 months. However, when the stent was positioned with any part of the proximal end within the bladder or when the stent could not be epithelialized incrustation occurred (14 cases, all of which were asymptomatic). No serious urosepsis was noted in any patient in this study. Six stents were removed endoscopically without difficulty or damage to the urethra at up to 18 months. The implications of these findings to the potential role of the UroLume stent in the management of a wider range of patients with prostatic obstruction are discussed.

Aged↗

Standard of care for cancer-related anemia: improving hemoglobin levels and quality of life.

The introduction of recombinant human erythropoietin (rHuEPO) has proven to be a major advance in the therapeutic options available for managing anemia in cancer patients. The results of placebo-controlled clinical trials and large, community-based, open-label studies have confirmed that epoetin alfa, a recombinant human erythropoietin, significantly reduces transfusion requirements, and reliably increases hemoglobin (Hb) levels in anemic (Hb level <12 g/dl) cancer patients undergoing chemotherapy. Increased Hb improves patients' energy level and their ability to perform the activities of daily living, as well as their overall quality of life (QOL). These findings are independent of tumor type and disease status and are comparable in patients receiving nonplatinum- and platinum-based chemotherapeutic regimens. Furthermore, more than a decade of use in clinical trials and by physicians in routine clinical practice has demonstrated that epoetin alfa is safe and well tolerated when used to treat cancer patients with anemia. The availability of epoetin alfa as an alternative to transfusion has changed practices in anemia management; physicians can now treat anemia with the goal of achieving adequate Hb levels to relieve anemia-related fatigue, a major symptom contributing to decreased QOL in cancer patients. Incremental benefit analysis has shown that increasing Hb level from 11 g/dl to 12 g/dl yields the greatest improvement in QOL per 1 g/dl increase in Hb. The demonstrated efficacy of epoetin alfa for increasing Hb levels and improving patient QOL have made this agent a rationale choice for management of cancer-related anemia. Ongoing research will continue to provide new insights into best management of anemia with epoetin alfa in cancer patients.

Anemia, Hypochromic↗

Interviewing cancer patients in a research setting: the role of effective communication.

A case-control study of quality of life in patients with lung cancer was carried out. The investigation was conducted by means of a series of interviewer-administered instruments. As part of the study, patients' attitudes towards the interviewer-administered questionnaires were studied. Previously we reported that a high proportion of the patients found being interviewed acceptable. The present study firstly examines why patients found being interviewed acceptable and secondly investigates whether there is an association between gender, age, diagnosis, place of interview, patients' overall health status, global quality of life and patients' feelings. Two hundred and thirty-two patients attending an outpatient clinic with either lung cancer or chronic respiratory disease were interviewed by means of a short questionnaire. They were asked to indicate their feelings about interviews, and to explain the reasons why they had their particular feelings. We documented issues on communication between interviewers and patients. These include observations on interviewing cancer patients, interviewing at home, and interviewing in the clinic. Nearly all the patients (96%) indicated that they found being interviewed acceptable. A content analysis of data showed that patients' feelings can be attributed to four major themes: the interview was not disturbing, they felt relaxed and at ease, they liked to talk, and the interview was conversational. There were no significant association between gender, age, diagnosis (cases and controls), place of interview, patients' overall health status and global quality of life and the reasons expressed. Practical experience from interviewing patients at home or in the clinic reveals several issues on communication between interviewer and patients. The study results suggest that apart from communication factors, other parameters such as age, gender, diagnosis, overall health status and quality of life and interview setting do not have significant role in patients' feelings about interviews. If data on quality of life studies are to be collected by interviewers, then proper communication is essential. How to achieve this remains a major question.

Adult↗