Large multiple coronary artery aneurysm in adult patients: a report on three patients and a review of the literature

Three cases of large multiple coronary aneurysms, situated on both right and left coronary arteries, were seen in three middle-aged adult patients. These patients were hospitalized for myocardial infarction in two cases and for angina pectoris in the third case. On the coronary angiogram, the coronary lesions were quite unusual as there were multiple voluminous aneurysms on both coronary arteries without evidence of atherosclerotic lesions of the remainder of the coronary tree. These lesions did not seem to be congenital or atherosclerotic, and it was postulated that these lesions might have been the sequelae of a mucocutaneous lymph node syndrome although no previous history of this condition could be found in these three patients.     

patients with the description of a new scoring system and its validation on 253 other patients

Using Cox models, we established a new prognostic system based on simple clinical parameters in a training series of 232 patients whose diagnoses were made before 1989. Adverse prognostic factors for survival (P <.01) were age 65 years or older, male gender, albumin level lower than 40 g/L, hemoglobin level lower than 12 g/dL, platelet count less than 150 x 10(9)/L, white blood cell count less than 4 x 10(9)/L, high number of cytopenias, and hepatomegaly. Taking age (age 65 years or older, 1 point; younger than 65 years, 0 points), albumin (less than 40 g/L, 1 point; 40 g/L or more, 0 points), and total number of cytopenias (no cytopenia, 0 points; 1 cytopenia, 1 point; 2 or 3 cytopenias, 2 points) into account, we separated the 232 patients into 3 groups with low (score 0 or 1), intermediate (score 2), or high (score 3 or 4) risk, associated with 5-year survival rates at 87%, 62%, and 25%, respectively (P <.0001). Only the presence of 2 or 3 cytopenias was an independent prognostic factor among patients younger than 65 years (P <.0001). Albumin level lower than 40 g/L and the presence of 1 or more cytopenia defined a prognostic system for patients 65 years and older. Patients at low risk, intermediate risk, and high risk had 5-year survival rates at 92%, 63%, and 27%, respectively (P <.0001). The 3 prognostic systems separated the 167 patients of a test series in groups with significantly different survival rates. The overall scoring system retained a significant prognostic value in 86 additional patients treated between 1990 and 1996. We conclude that the combination of age, albumin level, and blood cell counts might help to select patients with Waldenstr?m macroglobulinemia for treatment and to evaluate therapeutic results.     

Stiffness of the rib cage in a subset of rheumatoid patients

In a previous investigation of lung function in patients with rheumatoid arthritis (RA), we observed that some patients had significant dysfunction of their chest wall mechanics without pleural disease that could contribute to their respiratory symptoms. To investigate further the chest wall functions of patients with RA, we selected 10 female lifetime nonsmoking patients with RA and compared them to 10 paid normal volunteers in detailed functional evaluations of lung and chest wall. Patients with significant airflow limitation were excluded from this study. We found a significant 7% reduction in total lung capacity in the RA patients without significant change in lung compliance. However, we found a significant reduction in rib cage compliance in patients with RA (0.044 +/- 0.006 L/cmH2O vs. 0.064 +/- 0.012 in controls), which was associated with a significant 38% reduction in expansion of the rib cage volume during various breathing maneuvers. These changes occurred in the absence of lung or pleural disease. This study documents that patients with RA have limited expansion of rib cage relative to abdomen, thus rib cage restriction and stiffness, which may contribute to reduced lung volume. The shift of thoracoabdominal breathing configuration observed in patients with RA will likely reduce the effectiveness of breathing muscles and thus could contribute to the dyspnea sensation experienced by patients with RA.     

Radiography of the pelvis in a series of 200 elderly patients

Review of the findings of routine radiography of the pelvis in a series of 200 elderly patients admitted to a geriatric department shows that a great number of abnormalities are detected. It is suggested that this procedure is of equal value to routine chest radiography.     

Impact of osteoarthritis on the rehabilitation of patients following a stroke

OBJECTIVE: To determine if functional gain for patients following stroke rehabilitation is adversely affected by osteoarthritis (OA). METHODS: A retrospective cohort analysis was conducted using data collected between 1994 and 2001 from a large national registry of US rehabilitation inpatients. Outcome variables were functional status (the Functional Independence Measure [FIM Instrument]) at discharge and followup, FIM gain during and after rehabilitation, length of stay, and discharge setting. The primary predictor variable was the presence of OA. Covariates were age, sex, race, other comorbidities, type of stroke, length of stay, and time to followup. Analysis was by multivariable regression. RESULTS: Data from 3,094 patients with OA and 44,943 patients without OA admitted following a stroke to inpatient rehabilitation facilities were analyzed. In unadjusted analyses, OA was associated with significantly higher FIM ratings than patients without OA at admission (mean +/- SD 65.7 +/- 19.2 versus 63.1 +/- 20.5; P < 0.001) and discharge (mean +/- SD 89.2 +/- 21.8 versus 87.7 +/- 23.0; P < 0.001), but with lower FIM ratings at followup (mean +/- SD 97.7 +/- 24.7 versus 99.7 +/- 24.9; P < 0.001). In multivariate analyses, adjusting for potential confounders, OA was associated with a 1.62-day increase in length of stay (95% confidence interval [95% CI] 1.15, 2.08) and a 1.37 smaller improvement in FIM scores between admission and followup (95% CI 0.62, 2.12). This smaller increase in FIM instrument score was caused by less improvement in FIM after discharge. CONCLUSION: OA impairs recovery from stroke. This is compensated for by longer length of stay for inpatient medical rehabilitation. Reimbursement systems may need to consider the impact of OA as a comorbid condition in patients receiving stroke rehabilitation.     

Stiffness of the rib cage in a subset of rheumatoid patients

In a previous investigation of lung function in patients with rheumatoid arthritis (RA), we observed that some patients had significant dysfunction of their chest wall mechanics without pleural disease that could contribute to their respiratory symptoms. To investigate further the chest wall functions of patients with RA, we selected 10 female lifetime nonsmoking patients with RA and compared them to 10 paid normal volunteers in detailed functional evaluations of lung and chest wall. Patients with significant airflow limitation were excluded from this study. We found a significant 7% reduction in total lung capacity in the RA patients without significant change in lung compliance. However, we found a significant reduction in rib cage compliance in patients with RA (0.044 ± 0.006 L/cmH₂O vs 0.064 ± 0.012 in controls), which was associated with a significant 38% reduction in expansion of the rib cage volume during various breathing maneuvers. These changes occurred in the absence of lung or pleural disease. This study documents that patients with RA have limited expansion of rib cage relative to abdomen, thus rib cage restriction and stiffness, which may contribute to reduced lung volume. The shift of thoracoabdominal breathing configuration observed in patients with RA will likely reduce the effectiveness of breathing muscles and thus could contribute to the dyspnea sensation experienced by patients with RA.     

The effect of a 'recall-system' in the treatment of alcoholic patients

In a service specializing in alcohol and drug abuse in Brazil, a ‘recall system’ was developed in order to decrease the drop out level, which is high in this kind of treatment. The effect of this procedure was measured in two groups: in the 82 patients with the ‘recall system’, the drop out rate after 12 months was 45%. In 227 patients without this system the drop out rate was 84%. The difference in drop out rate was significant (p < 0.01) only during the first 6 months of treatment.     

The Prader-Willi syndrome: a study of 40 patients and a review of the literature

Forty patients with the Prader-Willi syndrome have been examined. The typical features begin in gestational life with poor fetal vigor and difficulties with birth and post-partum feeding. The classical features of hypotonia, small hands and feet, cryptorchidism can be identified at this time. The delayed milestones, mental retardation and obesity become more prominent later. The average height of the patients in this series who were admitted to the Clinical Study Center was 149 cm and their weight was 114 kg. The weight and height curves show that Prader-Willi individuals are consistently shorter and heavier than normal children. Tests of endocrine function showed normal glucose tolerance. Insulin secretion was increased in relation to obesity. The rise in growth hormone (hGH) after injecting insulin to induce hypoglycemia and after the infusion of arginine was comparable to other obese individuals but was low in comparison to normal weight subjects. There was no rise in growth hormone with L-dopa administration, but there was a rise in hGH with the administration of 2-deoxy-D-glucose. The hypoglycemia produced by insulin was greater in the Prader-Willi patient than in obese controls. The rise in TRH (thyrotropin-releasing hormone) following the injection of TSH (thyrotropin stimulating hormone) was greater in the Prader-Willi patients than in the obese controls. Hypogonadism was routine in this series, and the response to LRH (luteinizing releasing hormone) was absent in all tested subjects. Treatment with clomiphene for 30 to 90 days significantly increased the response to LRH in three adult individuals who had not been treated with gonadal steroids previously and who were hypogonadal. Rectal temperature declined in three of the five Prader-Willi patients during exposure to an ambient temperature of 4 degrees C, but none of the three obese controls showed a decline. Food intake averaged 5167 kcal/d when six patients were given trays containing more food than they could eat. Food intake was not reduced when tryptophan was added to the diet. Salivary secretion was reduced in the Prader-Willi patients. A number of pulmonary function tests were significantly reduced in the study patients compared to obese or normal weight controls. The anatomic findings in four autopsied patients with the Prader-Willi syndrome showed no significant differences from those of obese subjects without this syndrome. The chromosomal pattern showed a deletion or translocation at chromosome 15 in 3 of 12 patients in whom this test was performed. These findings in 40 patients with the Prader-Willi syndrome have been compared with the information contained in 159 reports published in the medical literature.     

EDTA-dependent pseudothrombocytopenia: a clinical study of 18 patients and a review of the literature

EDTA-dependent pseudothrombocytopenia (EDTA-PTCP) is the phenomenon of a spurious low platelet count due to the appearance of antibodies that cause platelet agglutination in blood anticoagulated with EDTA. We review here the clinical features of 18 patients with EDTA-PTCP treated in our hospital from 1984 to 1987 as well as those of 34 patients reported in the literature. This phenomenon appears more frequently in severely ill patients, in association with autoimmune, neoplastic, atherosclerosis-related, and liver diseases. In the majority of our patients, EDTA-PTCP appeared during hospitalization, indicating that the antibody is an acquired one. Neither splenomegaly nor the presence of autoimmune markers were features of this entity. Unlike true thrombocytopenias, EDTA-PTCP is associated with a normal mean platelet volume. Awareness of this entity is essential since EDTA-PTCP is frequently misdiagnosed and therefore incorrectly treated.     

Malignancy in patients with haemophilia: a review of the literature

Summary. Haemophilia A and B are rare X‐linked conditions. Elevated rates of HIV and hepatitis C related malignancies in these patients are well reported, however rates of other types of cancers are not. Therefore, a retrospective literature review of cancer in patients with haemophilia was conducted. A Medline search of articles from January 1966 to July 2009 utilizing the keywords haemophilia, leukaemia, malignancy, mortality, neoplasm and cancer was performed. The articles were reviewed and additional relevant publications were located from the references. Data on age, type and severity of haemophilia, HIV status, type of malignancy and outcomes were recorded as available. Thirty‐two cases of leukaemia were identified as well as 159 malignant solid tumours. Specific incidence and prevalence rates could not be calculated due to the limited nature of the information available in the reports. Many types of malignancy have been reported in persons with haemophilia irrespective of infection with HIV and hepatitis C yet prevalence and incidence rates compared to the general population remain unknown. Patients with haemophilia can manifest non infectious related malignancies and symptomatic patients should be evaluated accordingly.     

Fracture of the pancreas in two patients after a go-kart accident

Background

After blunt abdominal trauma, an isolated injury to the pancreatic duct is uncommon. Physical signs and laboratory parameters are often inaccurate, and missing this diagnosis can cause serious clinical problems.

Case outlines

Two young women (aged 18 and 20 years) are reported who sustained isolated trauma to the pancreatic duct in go-kart accidents. Each patient sustained a fracture of the pancreas.This injury was diagnosed only after a period of clinical observation with repeated laboratory parameters, ultrasound and CT scan. Pancreatic tissue was conserved by performing a pancreaticojejunostomy.

Discussion

After any episode of blunt abdominal trauma, isolated injury to the pancreatic duct should be considered. Serum analysis, ultrasonography and CT scanning can be helpful in early diagnosis. Preservation of pancreatic tissue can be achieved with a good clinical outcome.     

Sero-conversion of HCV negative patients: a European study on the epidemiology and management of HCV haemodialysis patients

This paper reports part of the findings from a larger study reported earlier, the European study on epidemiology and the management of HCV in the haemodialysis population (1). Centres recruited to the larger study were monitored for a further one year observation period to measure and generate a deeper understanding of HCV sero-conversion. From 4724 patients who were studied at the baseline, in 68 centres, only 13 patients were found to have sero-converted. These sero-conversions occurred in 7 hospitals within 5 different countries. Possible routes of transmission and risk factors are described with respect to the individual centres and good practice recommendations based on current evidence presented.     

B-cell depletion in the treatment of patients with systemic lupus erythematosus: a longitudinal analysis of 24 patients

OBJECTIVES: To assess the clinical and basic serological consequences of B-cell depletion with rituximab in the treatment of patients with systemic lupus erythematosus (SLE) who have failed conventional immunosuppression. METHODS: An open study of 24 patients with severe SLE followed for a minimum of 3 months is reported. In the majority of patients (19 out of 24), 6 months follow-up data are described. Disease activity in these patients was assessed every 1-2 months using the British Isles Lupus Assessment Group (BILAG) system and estimates of anti-double-stranded DNA antibodies and serum C3 levels. During the follow-up period, significant side-effects were sought and the reduction in oral prednisolone was recorded. It was our general practice to stop concomitant immunosuppression (e.g. azathioprine, mycophenolate) when B-cell depletion was given (in most cases in the form of two 1 g intravenous infusions of rituximab 2 weeks apart accompanied by two 750 mg intravenous cyclophosphamide infusions and two methylprednisolone infusions of 250 mg each). RESULTS: Twenty-two patients were female and two male. At the time of B-cell depletion, the mean age was 28.9 yr (range 17-49) and the mean disease duration was 7.9 yr (range 1-18). The global BILAG score (P < 0.00001), serum C3 (P < 0.0005) and double-stranded DNA binding (P < 0.002) all improved from the time of B-cell depletion to 6 months after this treatment. Only one patient failed to achieve B-lymphocyte depletion in the peripheral blood. The period of B-lymphocyte depletion ranged from 3 to 8 months except for one patient who remains depleted at more than 4 yr. Analysis of the regular BILAG assessments showed that improvements occurred in each of the eight organs or systems. The mean daily prednisolone dose fell from 13.8 mg (s.d. 11.3) to 10 mg (s.d. 3.1). CONCLUSION: In this open study of patients who had failed conventional immunosuppressive therapy, considerable utility in the use of B-cell depletion has been demonstrated. Our data provide strong support for the performance of a full double-blind control trial.