Operative findings and therapeutic results in patients with primary hyperparathyroidism

In the period from 1976 to September 1982 39 patients with the clinical diagnosis of primary hyperparathyroidism were operated on. Among then were 24 women and 14 men. The most frequent manifestation form is the renal one (n = 26). The proportion of patients with hypercalcaemic crisis was very large (n = 4). Topographically and anatomically the adenomas were most frequently found in the region of the right lower pole of the thyroid gland. 23 patients had a singular adenoma, 11 patients showed multiple adenomas and only 2 had a diffuse hyperplasia. In patients with persisting hyperparathyroidism before a repeated intervention an attempt of localization should be performed by a selective phlebography with taking of blood samples for the determination of PTH. The success of the operation is to be read off most clearly at the behaviour of the post-operative values of serum calcium. In 29 patients a normocalcaemia was achieved. 6 patients showed at first a hypocalcaemia, which however, was only temporary in 4 patients. 3 patients had a persisting hyperparathyroidism. In accordance with the literary data the formation of renal calculi clearly decreased after a successful operation.     

Teleophthalmology link between a primary health care centre and a reference hospital.

We have evaluated a teleophthalmology service linking a primary health care centre and an eye clinic at a reference hospital. General practitioners at the primary care centre serving a population of 15,000 and ophthalmologists at the reference hospital participated in this study. Eye fundus digital images were taken from 278 eye fundi of 139 consecutive patients with clinical conditions that could potentially produce fundus alterations. Fundus images were obtained with a non-mydriatic fundus camera (Canon CR6-45M) and were electronically sent reference hospital where ophthalmologist inspected the images and returned the diagnosis. In 18 patients (13%) the images did not have good enough quality to exclude eye fundus lesions. In 24 patients (17%) clear eye fundus alterations were found in at least one eye. In 14 patients (10%) there were image features suggesting retinal alterations that could not be confirmed by image inspection. Media opacity (13 eyes, 5%, seven patients, 5%) was the most common cause of poor image quality. The most difficult assessment was the evaluation of optic nerve head cupping. Retinal oedema was not observable in the digital images. In our experience teleopthalmology services seem to be an effective alternative for eye fundus diagnosis and patient follow-up.     

维持性血液透析25年回顾分析--解放军肾脏病研究所的经验

目的回顾性分析维持性血液透析(CHD)患者的人口统计学和流行病学特点。方法从1974年1月至1999年4月,在南京军区南京总医院肾脏病研究所血液净化中心接受CHD治疗的患者2004例,分析了患者的性别、年龄、原发病因、治疗持续时间和转归,及1995年以后CHD患者的死亡原因。结果2004例尿毒症患者中以中、青年为主,平均年龄41.7±13.5岁,男／女为2.21,其中38.5％患者原发病因不明,已知病因者以原发性肾小球肾炎为主,占66.1％,经肾活检明确诊断者以IgA肾病最多见;继发性肾小球疾病占17.1％,其中狼疮性肾炎占首位,糖尿病和高血压病居其次,分别占6.1％、4.9％和2.1％;777例CHD患者在本中心治疗时间超过3个月,其中18.4％仍在我院HD治疗,最长者已存活16年;31.9％患者行肾移植,30.4％转至外院治疗,3.90％改为腹膜透析,13.9％患者死亡;近年来患者的主要死因为脑血管意外(36.7％)和急性左心衰(20.0％)。CHD患者1年生存率93.2％,5年生存率64.2％,10年生存率55.1％。结论①本组患者以中、青年为主,男性多见;②IgA肾病是导致尿毒症的主要已知病因;③CHD患者1年、5年和10年生存率分别为93.2％、64.2％和55.1％,主要死亡原因为脑血管意外和心衰。     

Determinants for clinical diagnosis of hypertrophic cardiomyopathy

Although hypertrophic cardiomyopathy (HC) occurs in 1 of 500 adults, most cardiology practices treat relatively few patients with HC, suggesting that many affected patients evade clinical recognition. Determining the clinical circumstances under which HC is identified will provide clues to its under-recognition. Clinical triggers leading to diagnostic echocardiograms were analyzed in 711 consecutive patients with HC. In most (384 [54%]), HC was initially suspected only after the onset of cardiac symptoms or acute cardiac events. In a substantial minority (327 [46%]), HC was recognized while patients were asymptomatic, including 225 (32%) by routine medical evaluations, in 27 of whom (4%) HC was recognized during preparticipation examinations for competitive sports or other activities. Women, older patients (age > or =50 years), and those with outflow obstruction at rest (gradient > or =30 mm Hg) were more likely suspected to have HC by virtue of cardiac symptoms or events (p <0.0001). Conversely, patients with extreme hypertrophy (wall thickness > or =30 mm) and those at high risk for sudden death were more often asymptomatic and identified by routine or family screenings (p <0.0001 and p = 0.004, respectively). Patients who subsequently died of heart failure or experienced embolic stroke were more often identified by virtue of symptoms or acute events (p = 0.03). In conclusion, although most patients with HC were recognized clinically only after overt disease manifestations, a substantial minority were diagnosed by routine examinations while asymptomatic, including an important subset of patients with HC recognized solely because of findings on sports preparticipation screening. These data underscore the need for heightened awareness and clinical suspicion of HC to increase the number of diagnosed patients, including many who may be at high risk for sudden death.     

体位对急性脑梗死患者睡眠呼吸紊乱的影响

目的探讨睡眠体位对脑梗死患者睡眠呼吸紊乱的影响和作用机制。方法选择急性脑梗死患者53例,分为睡眠呼吸暂停综合征(SAHS)组34例,非SAHS组19例。采用视频多导睡眠监测仪,对急性脑梗死患者自然睡眠过程及不同体位睡眠呼吸状况进行全程描记。结果 53例患者中,有34例(64.2%)急性脑梗死患者睡眠呼吸暂停低通气指数(AHI)≥10。与仰卧位比较,SAHS组左、右侧卧位患者的AHI明显降低,平均血氧饱和度明显升高(P<0.05,P<0.01);且睡眠呼吸紊乱事件以阻塞型为主,仰卧位最重(P<0.01);SAHS组患者自然睡眠状态下,AHI与侧卧位/仰卧位睡眠时间比值呈负相关(r=-0.56,P<0.01)。结论脑梗死后睡眠呼吸紊乱发生率高,仰卧位时加重,体位自我调节保护睡眠呼吸的功能减弱。     

Pituitary apoplexy

Ten patients with pituitary apoplexy were treated at the Institute of Endocrinology, Gerontology and Geriatrics in Sofia over the last 5 years. They represented 5.06% of the total number of patients with pituitary adenomas treated at the Institute during the same period. The highest frequency of pituitary apoplexy was noted among patients with Nelson's syndrome. In 5 patients of this group pituitary apoplexy was the first sign of a tumor. Headaches combined with nausea and vomiting were the earliest symptoms of pituitary apoplexy, and ptosis and ophthalmoplegy were the most common symptoms in the patients. The disease was not correctly diagnosed in 7 patients who immediately after apoplexy were treated in neurological and ophthalmological departments.     

Bacteremic infection in hemodialysis

This is a retrospective study of 133 episodes of bacteremic infection in 112 hemodialysis patients. The frequency of bacteremic infection was 9.5% in patients with chronic renal failure and 10.9% in patients with acute renal failure. In patients with acute renal failure, pneumonia and intra-abdominal abscess were the most frequent sources of septicemia. Sepsis was usually due to Gram-negative organisms and mortality was high. In patients with chronic renal failure, infection of the shunt or fistula was the most common cause, was frequently due to Staphylococcus organism, and had a more favorable survival rate. Gram-negative septicemia from a nonaccess source in patients with chronic renal failure was associated with a higher mortality. Bacterial endocarditis and septic pulmonary emboli occurred in 3.6% of septic episodes and 0.35% of patients at risk and had very low mortality. A low threshold for obtaining blood cultures and early antibiotic treatment are believed to be important in the treatment of bacteremic infections in patients undergoing long-term hemodialysis.     

Hematopoietic stem cell transplantation with conditioning regimens containing melphalan in pediatric patients with acute lymphoblastic leukemia

A multicenter comparative study was carried out to investigate the efficacy and safety of hematopoietic stem cell transplantation with conditioning regimens containing melphalan in pediatric patients with acute lymphoblastic leukemia. One hundred twenty three patients at a variety of remission stages were eligible for study participation. Eighty-nine were transplanted with allogeneic grafts and 34 patients with autologous grafts (23 cases with bone marrow and 11 cases with peripheral blood stem cells). Conditioning regimens used were as follows: (A) melphalan and busulfan for 40 patients, (B) melphalan, busulfan and TBI for 44 patients, (C) other regimens for 39 patients. To accelerate engraftment G-CSF (lenograstim) was administered as a 1-hour or 24-hour drip infusion daily at 5 micrograms/kg from day 5 until hematological recovery. The five year disease free survival (DFS) was 63% for 42 patients at CR 1, 41% for 41 patients at CR 2 and 33% for 40 patients at other stages. There was no significant difference in the DFS between allogeneic-transplantation and autologous-transplantation in all disease stages. In patients at remission stage for CR 1 and CR 2, the 5-year DFS by conditioning regimen was 63% for regimen (A), 54% for regimen (B) and 54% for regimens with melphalan and TBI. There was no significant difference in the DFS between the groups. Serious complications such as renal failure were observed in 11%, veno-occlusive disease in 9%, and interstitial pneumonia in 9%. The most dominating cause of death was relapse in the disease (48% of deaths) which was most commonly observed in autologous transplantation. Contrary to that, treatment related toxic death was the most frequent cause of deaths in allogeneic-transplantation.     

Mortality in patients with ST-segment elevation myocardial infarction who do not undergo reperfusion

Reperfusion therapy reduces mortality in patients presenting with ST-segment elevation myocardial infarctions (STEMI). However, some patients may not receive thrombolytic therapy or undergo primary percutaneous coronary intervention. The decision making and clinical outcomes of these patients have not been well described. In this study, 139 patients were identified from a total of 1,126 patients with STEMI who did not undergo reperfusion therapy at a high-volume percutaneous coronary intervention center from October 2006 to March 2011. Clinical data, reasons for no reperfusion, management, and mortality were obtained by chart review. The mean age was 80 ± 13 years (61% women, 31% diabetic, and 37% known coronary artery disease). Of the 139 patients, 72 (52%) presented with primary diagnoses other than STEMI, and 39 (28%) developed STEMI >24 hours after admission. The most common reasons for no reperfusion were advanced age, co-morbid conditions, acute or chronic kidney injury, delayed presentation, advance directives precluding reperfusion, patient preference, and dementia. Eighty-four patients (60%) had ≥ 3 reasons for no reperfusion. Factors associated with hospital mortality were cardiogenic shock, intubation, and advance directives prohibiting reperfusion after physician consultation. In hospital and 1-year mortality were 53% and 69%, respectively. In conclusion, at a high-volume percutaneous coronary intervention center, most patients presenting with STEMI underwent immediate catheterization. The decision for no reperfusion was multifactorial, with advanced age reported as the most common factor. Outcomes were poor in this population, and fewer than half of these patients survived to hospital discharge.     

Bacteremia in adult patients with acquired immunodeficiency syndrome in the northeast of Thailand.

BACKGROUND: Bacteremia is a frequent complication found in HIV-infected patients and is usually associated with a poor prognosis. This study was undertaken to describe the bacterial pathogens causing bacteremia in adult Thai HIV-infected patients, and hence to give guidance in the choice of empirical antimicrobials. METHODS: Blood culture results at Srinagarind Hospital, Khon Kaen during the period January 1996 to December 2001 were retrospectively reviewed. RESULTS: In HIV-infected and HIV-uninfected patients, 172 and 4082 episodes of bacteremia occurred, respectively. In HIV-infected patients, community-acquired and nosocomial bacteremia were found in 78.5% and 21.5%, respectively and most were monomicrobial. Gram-negative bacteria were the main pathogens isolated in both groups of bacteremia. Escherichia coli and methicillin-resistant Staphylococcus aureus were more common pathogens causing nosocomial bacteremia in HIV-infected patients, whereas Acinetobacter spp were more common in HIV-uninfected patients. Salmonella spp, especially Salmonella groups D and B, were the most common (62.2%) pathogen in community-acquired bacteremia in HIV-infected patients whereas Escherichia coli was the most common in HIV-uninfected patients. Only a few episodes of community-acquired bacteremia in HIV-infected patients had identified sources. Co-trimoxazole resistance was common in community-acquired bacteremia caused by Gram-negative bacilli in HIV-infected patients, with Salmonella group B being more resistant to co-trimoxazole than Salmonella group D (statistically significant, p<0.001). However, resistance rates to ceftriaxone and ofloxacin were low. CONCLUSIONS: Bacteremia in adult HIV-infected patients was usually caused by Gram-negative bacilli in both community-acquired and nosocomial settings. Salmonella spp was the most common organism identified, especially Salmonella group B and D. Ceftriaxone or fluoroquinolones such as ofloxacin or ciprofloxacin should be used as the initial empiric therapy for HIV-infected patients with suspected bacteremia.     

European Cooperative Crohn's Disease Study (ECCDS): colonoscopy

130 patients with Crohn's disease were colonoscopied in a multicenter trial. The obtained data were analyzed with respect to gathering information on the inflammation pattern, as well as on the importance and prognostic value of special lesions in Crohn's colitis. In 52 patients a second endoscopy was performed at the end of the 2-year study period. Ulcerations and aphthous lesions were the most common lesions, followed by pseudopolyps, cobblestone lesions and stenosis. In general, there was an increasing, distal gradient in the frequency of severe lesions. patients with Crohn's colitis alone had more signs of inflammation than patients with additional involvement of the small intestine. A segmental pattern was the most common form of inflammation. The group of patients (14%) with a continuous pattern did not deviate from the whole collective in clinical activity. In patients with previous resections, inflammation near the anastomosis was accompanied more often than not by stenosis. Patients with ulcerations had a rather short time since confirmation of the diagnosis. Cobblestone lesions and pseudopolyps correlated with short symptomatology. During the follow-up of the study, patients taking steroids or a combination with prednisolone and sulfasalazine seem to have better results than those under placebo or sulfasalazine alone, as regards the more severe symptoms.     

Refusing HIV testing in an urgent care setting: results from the "Think HIV" program

Efforts to increase HIV case identification through routine, voluntary HIV testing are hindered by high refusal rates. Our objective was to identify patients most likely to refuse routine HIV testing. We developed a new HIV testing program at four Massachusetts urgent care centers. Patients were asked if they were interested in routine HIV testing. We performed analyses to assess differences in characteristics between those who refused testing and those who accepted it. Data were available for 9129/10,354 (88%) patients offered routine HIV testing from January to December 2002. Of these 9129 patients, 67% refused testing. In the crude analysis, HIV test refusal was associated with female gender, white race, older age, and higher educational level. In multivariate analysis, non-English-speaking patients who were Hispanic, Haitian, and other race were more likely to refuse testing than their English-speaking counterparts. Among all patients, "not at risk" and "already tested" were the most common reasons for test refusal. Two thirds of patients refused routine HIV testing when it was offered in a statewide urgent care-based program. If routine HIV testing programs are to be successful, strategies must be developed to increase HIV test acceptance among patients most likely to refrain from testing.     

Presentation of Pulmonary Tuberculosis

Abstract: A study was made of the presenting features of 700 consecutive Australian patients with pulmonary tuberculosis. A clinical diagnosis of pulmonary tuberculosis was suspected at the time of first presentation in only 52 patients and the initial provisional diagnosis was that of a non-tuberculous chest condition in a further 32 patients. In another 16 there was a delay in diagnosis because pulmonary tuberculosis was suspected only after chest X-rays were taken for screening purposes—for example, prior to elective surgery, A non-cavitating lesion in an upper lobe was the radiological appearance most often associated with failure to suspect tuberculosis at the time of presentation, The most common symptoms or change in pre-existing chest complaints were cough (55), loss of weight (52) and shortness of breath (43) followed by fever or night sweats (23) and haemoptysis (10) while 16 were asymptomatic .     

Do psychological factors predict outcome in both low-back pain and shoulder pain?

BACKGROUND AND OBJECTIVE: Psychological factors are assumed to predict persistent or recurrent musculoskeletal pain. The influence of psychological factors in patients with low-back pain (LBP) or shoulder pain was explored to study whether there is similarity regarding the factors that predict persisting pain and disability. METHODS: Patients presenting in primary care with a new episode of shoulder pain or non-specific (sub)acute low back pain (LBP) were enrolled in a prospective study. In both patient groups, pain catastrophising, distress, somatisation and fear-avoidance beliefs were measured at baseline. Primary outcome measures at 3 months were (1) persistent symptoms, and (2) <30% reduction in functional disability. Multivariate logistic regression analysis was used to study the associations between psychological factors and outcome. RESULTS: A total of 587 patients with shoulder pain and 171 patients with LBP were enrolled in the study. In patients with shoulder pain, most associations of psychological factors with outcome were weak and not significant. Only in patients with longer symptom duration at baseline (>or=3 months) were higher scores on catastrophising significantly associated with persistent symptoms (p = 0.04). In patients with LBP, psychological factors were more strongly associated with poor outcome, although most associations were not significant. CONCLUSION: Psychological factors, with the exception of fear-avoidance beliefs, are more strongly associated with persistent pain and disability in patients with LBP than in those with shoulder pain. This seems to indicate that in a primary care population the influence of psychological factors on outcome may vary across patients with different types of pain.     

Prevalence of hereditary thrombophilia in patients with thrombosis in different venous systems

The prevalence of hereditary thrombophilia is well known in patients with lower-extremity thrombosis but only poorly studied in patients with thrombosis at unusual sites. Consequently, it is still unclear whether such patients should generally be screened for hereditary thrombophilia. We retrospectively analyzed 260 patients with thrombosis at unusual sites including thrombosis in portal, cerebral, retinal, and upper-extremity veins with respect to the prevalence of FV Leiden, prothrombin G20210A, protein C, protein S, and antithrombin deficiency. In addition, all thrombotic episodes were analyzed for circumstantial risk factors. Used as controls, healthy volunteers (120) and patients with lower-extremity thrombosis (292) showed overall prevalence of hereditary thrombophilia of 9.1% and 39.0%, respectively. The corresponding numbers were 33.3%, 34.3%, and 39.0% in patients with portal vein, upper-extremity, and lower-extremity thrombosis, respectively. In patients with cerebral vein thrombosis, however, the prevalence was significantly lower (23.5%). Patients with retinal vein occlusion did not show an increased frequency of thrombophilia at all (5.9%). In all five groups FV Leiden was by far the most frequent defect (4.4-27.1%), while prothrombin G20210A occurred rarer (2.5-7.6%). Protein C, protein S, and antithrombin deficiency were much less prevalent (0-3.1%) except for patients with portal vein thrombosis (4.8-7.1%). Compared to healthy individuals, the relative risk of thrombosis was 4.3 (2.2-8.1), 3.8 (1.8-7.7), 2.5 (1.0-6.1), 3.7 (1.5-8.6), and 0.6 (0.2-2.1) for patients with lower-extremity, upper-extremity, cerebral vein, portal vein, and retinal vein thrombosis, respectively. Circumstantial risk factors were more frequent in patients without than with hereditary thrombophilia and were found most often in patients with upper-extremity thrombosis. In each group the most frequent circumstantial risk factor was different. However, oral contraceptives and cancer were found in all five groups. In conclusion, independent upon the presence of circumstantial risk factors, screening for hereditary thrombophilia is warranted in all patients with thrombosis at unusual sites except in those with retinal vein occlusion.     

Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome

OBJECTIVES: We sought to assess the prevalence and progression of neo-aortic root dilation and valvar regurgitation after staged reconstruction for hypoplastic left heart syndrome (HLHS). BACKGROUND: In HLHS, the pulmonary valve functions as the neo-aortic valve. Neo-aortic valve dysfunction has been observed after arterial switch operation and the Ross procedure. METHODS: Patients with HLHS born before January 1995 who had the Fontan operation and had serial echocardiograms were included. Echocardiograms were reviewed preoperatively, after each surgical reconstruction, and at most recent follow-up for neo-aortic root size and severity of neo-aortic regurgitation (AR). Potential risk factors for neo-aortic valve dysfunction were assessed. RESULTS: Fifty-three patients met inclusion criteria. Bidirectional superior cavopulmonary anastomosis as an interim procedure was performed in 39 patients (74%). Median duration of follow-up was 9.2 (range 5.1 to 21) years. During follow-up, the neo-aortic root progressively dilated out of proportion to body size over time, with 52 patients (98%) having a Z-score >2 at most recent follow-up. Neo-AR was present in 61% of patients at most recent follow-up, with progression over time in 26 patients (49%). However, neo-AR was more than mild in only three patients. Significantly larger neo-aortic root Z-scores were observed in patients with any degree of neo-AR at most recent follow-up. No other anatomic or clinical variables correlated with severity of neo-AR or root dilation. CONCLUSIONS: After staged reconstruction for HLHS, neo-aortic root dilation and neo-AR progress over time. Early volume unloading does not have a beneficial impact on dilation of the neo-aortic root. These findings raise concerns about neo-aortic valve function into adulthood.     

Clinicopathological features and prognosis of esophageal cancer in young patients. Is there a difference in outcome?

The aim of this study was to define the clinicopathological features and prognosis of esophageal cancer in patients 40 years and younger. Between 1990 and 2007, 549 patients with esophageal cancer were enrolled in a retrospective database. Patients were divided into two age groups: Group A was under 40 years old, and group B over 40 years old. The findings for 50 patients (9.1%) aged 40 years or less were compared with those of 499 patients over 40 years old. In the younger group, there were significantly more women (35/15 vs. 231/268, P  < 0.001). In the young and old groups, the most frequent histological morphology was squamous cell carcinoma (88% and 75%, respectively), although the percentages were significantly different ( P  < 0.005). In the younger group, lesions were more frequently located in the middle one-third of the esophagus than in the older group (64% vs. 28.3%, P  < 0.001). Group A was more likely to have Stage IIa than older patients. In both groups, the surgical removal of the tumor was the most frequently used choice (70% vs. 56.5%). Survival rates in younger patients at 5 years after resection were 11.4%, similar to those in older patients (16.9%, difference not significant). In the group under age 40, women predominated. Squamous cell carcinoma was the most common histology, followed by adenocarcinoma. Younger patients did not have improved 5-year survival compared with their older counterparts.     

Changes in total and local contractility of the left ventricle, imaged by radionuclide ventriculography, in patients with ischaemic heart disease during submaximal exercise

In 40 patients with chronic ischaemic heart disease [IHD) and 10 healthy subjects the total and local myocardial contractility was assessed during threshold-level exercise. The patients were subjected to radionuclide ventriculography in a modification recording the first passage of the radioindicator through the heart cavities. In the control subjects the exercise induced a 27% increase in the total ejection fraction, with increased contractility of all left ventricular segments. In the patients with IHD the reactions to exercise varied. In 40% of the patients the total ejection fraction increased adequately, in 17.5% the increase was nonadequate, in 20% no increase was observed, and in 22.5% the ejection fraction decreased. The most marked reduction in myocardial contractility was found in patients who had sustained myocardial infarction and reacted positively to the exercise test. In 34 patients there were observed left ventricular segments showing a paradoxical response of contractility to exercise--a decrease in the local ejection fraction. This pathological local reaction occurred rather in a normal function at rest than in a myocardium with hypokinetic segments at rest.     

Preoperative imaging of hilar cholangiocarcinoma: surgical evaluation of standard practises

It was the goal of this study to compare the results of the preoperative diagnostic workup (ERC, MRC, and PTC) with the tumor extent of the surgical specimen in patients with hilar cholangiocarcinoma (hilCC). PATIENTS AND METHODS: Between 9/97 and 12/2002 82 patients with hilCC were treated at our institution. In 59 patients tumor resection was feasible. Preoperative ERC, MRC and PTC - blinded for the idendity of the patients - were analysed retrospectively and compared with the surgical specimen. RESULTS: PTC resulted in significantly superior visualization of the bile ducts including the hilar lesion compared to ERC and MRC (p < 0.01). ERC, MRC and PTC were correct in predicting tumor extent in 29, 36 and 53 % of cases, respectively. The extent of the tumor was overestimated in 42 % (ERC), 41 % (MRC) and 31 % (PTC). Underestimation or wrong assessment or no decision at all occurred in 31, 23 and 16 %, respectively. In 20 patients results of all three diagnostic methods were available allowing a statistical comparison regarding the resection to be performed: PTC was superior to ERC (McNemar test:p < 0.01), but not to MRC. In the patients with overestimated tumor extent both the rate of curative resections and survival were similar to the other resected patients. DISCUSSION: In contrast to most reports in the literature, ERC and MRC were found to be of limited reliability regarding the assessment of tumor extent. PTC proved to be the most reliable approach. Overestimation of the tumor extent, which may lead to exclude the patient from potentially curative surgery, was the most common mistake in each diagnostic modality.     

Short term effects of intravenous pulses of cyclophosphamide in the treatment of connective tissue disease crisis.

A predominantly outpatient regimen of low dose intravenous cyclophosphamide was used to treat patients with serious progressive connective tissue diseases. Fifty five patients were treated with a total of 211 intravenous pulses of cyclophosphamide. Forty five patients had previously shown no response to a variety of other treatments. Low dose intravenous cyclophosphamide (500 mg) was given in 179 pulses and repeated pulses were given in most patients at weekly intervals for one to three weeks to induce disease remission. A good response was noted in 37 of 55 (67%) patients assessed four weeks after the pulses. Only 20 patients needed more than one such course of three pulses of intravenous cyclophosphamide during the observation period. The non-responders were characterised by longstanding disease and irreversible histological findings in renal and muscle biopsy samples. Patients with vasculitis, notably Wegener's granulomatosis, showed the most immediate response, and in most patients the amount of corticosteroids required was markedly reduced. In some patients steroids were completely stopped during the follow up period. The most striking observation of this effective but more conservative regimen was the low incidence of major side effects such as neutropenia and infections. It is concluded that low dose pulses of intravenous cyclophosphamide are well tolerated and are an effective treatment for patients with aggressive connective tissue diseases.