Epidemiology, clinical presentation, treatment and prognosis of a regional series of 26 anaplastic thyroid carcinomas (ATC). Comparison with the literature

Objective

The aim of this study is to retrospectively describe the epidemiological and clinical features, therapeutic modalities, prognostic factors and survival figures in a population of patients with anaplastic thyroid carcinoma (ATC) observed in Auvergne, France. We compared these data with those in the literature.

Material and methods

The analysis was conducted based on a computer database containing a regional register recorded by health professionals treating ATC.

Results

Of the 1500 cancers observed over 16 years, 26 were identified as ATC. The male/female ratio was 1/2.7 and the average age: 72.1; 76.9% of the cases had thyroid medical history, average tumor size at diagnosis was 7.35 cm with N1 in the course of illness in 61.5% of cases, M1 in 34.6% of cases.Surgery was performed in 84.6% of cases, radiotherapy in 53.8% and chemotherapy in 19.2%. The average survival was 9 months, the survival median: 4 months.

Conclusion

Our results show that, in univariate analysis, age above 75, capsular invasion, lymph nodes metastasis, tumor residue after surgery and lack of multimodal treatment (particularly radiotherapy in patients without tumor residue) are factors of poor prognosis. In a multivariate analysis only age above 75, followed by node invasion, capsular invasion, and finally female gender are factors of poor prognosis.     

Neurosarcoidosis : Report of 30 cases and a literature survey

Introduction

Neurosarcoidosis accounts for approximately 5% of cases of sarcoidosis.

Objective

To determine the frequency of Neurosarcoidosis in our setting and analyze the clinical-radiological findings and evolution of 30 patients consecutively diagnosed.

Methods

The medical records of patients with a diagnosis of Neurosarcoidosis were reviewed, and data regarding the clinical features, ancillary tests performed, treatment, and outcome were recorded. We revised the literature to summarize and discuss the previous clinical series of Neurosarcoidosis.

Results

It accounted for 6.7% of all cases of sarcoidosis. Seven patients had definite diagnosis and 23 had probable diagnosis. The mean age at onset of Neurosarcoidosis was 48.3 years and 66.7% of patients were women. Neurologic clinical features were the first manifestation of Neurosarcoidosis in 70% of cases. Cranial neuropathy was present in 17 patients and 14 of them had facial palsy. The central nervous system was affected in 10 patients and the peripheral nervous system in 5. Chest disease, the most common extraneurologic manifestation, was present in 20 patients. All patients were treated with corticosteroids, and all those with central nervous system involvement had poor outcome.

Conclusion

Neurosarcoidosis requires a high degree of suspicion to establish the diagnosis. Central nervous system involvement is associated with a poor prognosis.     

Late onset Becker muscular dystrophy. A case report and literature review

Introduction

The first manifestations of Becker's muscular dystrophy usually occur during childhood, as Duchenne's muscular dystrophy, a related disease. However, clinical presentation is usually more heterogeneous and less severe.

Case report

We report a 54-year-old man who presented with a late onset of Becker's muscular dystrophy, and review 12 additional similar cases previously reported in the literature. The disease onset ranged from 30 to 65 years old. Genetic deletion involved mainly exons 45 to 55 with variable phenotype: distal muscular hypertrophy in four cases, cardiac involvement (electrocardiography, radiography or echocardiography) in six out of the 12 studied cases. Compared to the paediatric form, functional impairment was milder, as only three patients after seven to 20 years of follow-up were wheelchair bound.

Conclusion

The first manifestations of Becker's muscular dystrophy may occur after the age of 30 with heterogeneous phenotypes. Severity and limb disability is milder in these patients.     

Changing epidemiology of native valve infective endocarditis

Introduction and objectives

The aim of our study is to assess changes in the epidemiologic features of patients with native valve infective endocarditis.

Methods

We analyzed a prospective series of 228 cases of native valve infective endocarditis in non-intravenous drug users attending our center between 1987 and 2009. We compared three subperiods: 1987-1994 (67 cases), 1995-2002 (74 cases) and 2003-2009 (87 cases).

Results

The mean age of patients has progressively increased (38±22 years in the first subperiod vs 60±16 years in the third; P<.001), as has the proportion of cases without predisposing heart disease (25%, 46% and 67%; P<.001). Incidence of mitral valve prolapse remained stable (12%, 18% and 11%). Percentages of patients with predisposing heart disease and who were aware of their condition have fallen in recent years (45%, 27% and 21%; P<.001). A portal of entry for the infection could not be identified in 64%. Overall, Staphylococcus aureus is the most frequent causative organism (26%) whereas the percentage of cases caused by Streptococcus viridans remains unaltered (22%, 20% and 24%).

Conclusions

We found significant changes in the epidemiology of native valve infective endocarditis. The incidence of patients without predisposing heart disease has increased significantly and staphylococci are the most frequent causative organisms.Full English text available from: www.revespcardiol.org     

Use of gadolinium as contrast agent in endoscopic retrograde cholangiopancreatography in patients with iodine allergy

Introduction

Radiologic contrasts are required during endoscopic retrograde cholangiopancreatography (ERCP). The most frequently used are iodine-based contrast media. Controversy still surrounds the optimal strategy in patients with previous adverse reactions to iodine contrasts that need to undergo an ERCP.

Objective

To evaluate the safety and efficacy of a gadolinium-derived contrast medium in patients with previous reactions to iodine-derived agents during ERCP.

Material and methods

Thirteen ERCP were performed in 11 patients with well-established adverse reactions to iodine compounds. ERCP was carried out with gadobutrol, a non-ionic gadolinium compound and without prophylaxis.

Results

In all patients, ERCP were satisfactorily completed. Thirteen cholangiograms and one pancreatogram were obtained. All procedures were technically successful, allowing diagnosis and endotherapy. The quality of the images was good, similar to those obtained with standard contrast media, and did not represent a limitation. No contrast-related adverse events were observed, and there were no post-ERCP complications.

Conclusions

Gadolinium-derived agents are a safe and effective alternative in iodine-allergic patients.     

Le profil de risque dans l’infarctus du myocarde : une évaluation indispensable pour la comparaison des populations

Objective

The aim of this study is to show that differences of mortality, in acute myocardial infarction, observed between hospitals are not necessarily linked to a bad application of guidelines but can be linked to differences in the risk profile of the populations.

Methods

Two populations admitted for ST and non-ST elevation myocardial infarction in the same region in 2006 were compared: the population of Chalon-sur-Saône’s hospital with a standard population from the observatoire des Infarctus de Côte d’Or (RICO). The risk profile of the two populations has been realised with the risk scores GRACE, EMMACE and the Simple Risk Index (SRI).

Results

The three scores are applicable for our populations according to the “C statistic”. Moreover, there is a significant difference of in-hospital mortality between Chalon-sur-Saône and RICO. But, the population of Chalon-sur-Saône presents a higher risk. Finally, in-hospital rate mortality expected by the three scores is not different from the actual mortality.

Conclusion

GRACE, EMMACE and SRI are valid scores for the comparison of risk profile of populations in acute myocardial infarction. Comparisons between hospitals are only possible after risk adjustment of the populations.     

Magnetic resonance imaging of the peripheral vasculature

Background

Atherosclerotic disease of the peripheral vasculature is a prevalent condition for which catheter-based techniques have been considered to be the gold standard for diagnosis. However, because of their invasive nature, these techniques inherently have the potential for complications. Non-invasive diagnostic techniques have historically been limited by low accuracy and high operator dependence. Magnetic resonance angiography (MRA) is a new approach that has diagnostic accuracy comparable with invasive angiography.

Methods

The literature on MRA for evaluation of carotid, mesenteric, renal, and lower-extremity arterial disease was extensively reviewed. Helpful diagnostic algorithms on the basis of the literature are also provided.

Results

MRA is both sensitive and specific when compared with invasive angiography for the evaluation of peripheral arterial disease and avoids the potential for complications resulting from arterial puncture and use of iodinated contrast.

Conclusion

Current MRA techniques are diagnostically robust and have proven to be a highly accurate, safe, and convenient means of diagnosing atherosclerotic disease of the peripheral vascular system.     

Signet ring cell carcinoma of ampulla of Vater in a young adult.

Signet ring cell carcinoma of the ampulla of Vater is extremely rare. The 7 cases reported earlier have been in older patients. We report a 32-year-old lady with this condition, who also had metastases in the bone marrow, vertebrae, lungs and liver.     

Takotsubo cardiomyopathy and QT interval prolongation: who are the patients at risk for torsades de pointes?

Objectives

QT interval prolongation is prevalent among patients with Takotsubo cardiomyopathy (TC), whereas torsades de pointes (TdP) has rarely been reported in these patients. We studied all peer-reviewed reports on TC-associated QT interval prolongation and all peer-reviewed reports on TC-associated TdP to characterize the clinical circumstances leading to TdP in patients with TC.

Methods

The literature search yielded 14 reports on TC-associated TdP and 26 reports on TC-associated QT interval prolongation. Overall, 15 patients with TC-associated TdP and 86 patients with TC-associated QT interval prolongation were reported. We systematically reviewed each report and recorded the risk factors for TdP as well as the clinical circumstances of TC.

Results

The prevalence of the male sex was higher among patients with TC-associated TdP relative to patients with TC-associated QT interval prolongation (26.7% vs 5.8%; P = .01). There was a trend in the mean maximal corrected QT interval being longer among patients with TC-associated TdP relative to patients with TC-associated QT interval prolongation (679.9 ± 230.6 vs 555.9 ± 63.8 milliseconds; P = .06). There were no differences between patients with TC-associated TdP and patients with TC-associated QT interval prolongation in mean age, maximal troponin levels, and lowest ejection fraction. Overall, 12 (80.0%) patients with TC-associated TdP had risk factors for TdP other than the female sex and systolic dysfunction, including suspicion of congenital long QT syndrome, bradycardia, hypokalemia, recent conversion from atrial fibrillation to sinus rhythm, and using QT prolonging agents.

Conclusions

Men with TC-associated QT interval prolongation are at risk for TdP. Most patients with TC-associated TdP have risk factors for TdP other than the female sex and systolic dysfunction.     

Survey of general practitioners management of erysipelas

Purpose

A few studies only have focused on ambulatory management of erysipelas.

Methods

To assess the diagnostic and therapeutic management of erysipelas by general practitioners, and their adherence to the French Society of Infectious Diseases and Dermatology joint 2000 recommendations, we surveyed 114 general practitioners during a 1 year period (from May 1st, 2005 to April 30th, 2006).

Results

Seventy-three general practitioners accepted to participate to the study and 54 cases of erysipelas were reported. Median age of patients was 63 years (range, 18-94) and sex ratio was 0.77. Lower limbs were affected in 83% out of the cases. A skin lesion was reported in 65% of the cases. None of the 15 doppler ultrasonography that were performed identified deep vein thrombosis. Five patients (9%) were initially hospitalized. Only 18% out of the patients were treated by amoxicillin. Most prescribed antimicrobial agents were pristinamycin (31%) and amoxicillin-clavulanate (27%). Median duration of treatment was 10 days. Six patients received an anti-inflammatory drug. Among the 44 patients who had a follow-up visit, 37 patients (84%) recovered and two patients were hospitalized after this follow-up assessment. Two patients experienced a recurrence of erysipelas during the study.

Conclusion

As previously reported in the literature, outcome of erysipelas after ambulatory management remains excellent, although recommendations are poorly followed.     

Tubercular mastitis in men: case report and systematic review

Background

Tuberculous mastitis is usually reported in multiparous postpartum women and is an unusual cause of gynecomastia in adolescent men.

Methods

We describe an illustrative case and review all cases of tubercular mastitis reported in men in the English literature.

Results

Our search yielded 24 cases of male tubercular mastitis. Most presented with an isolated breast lump. Constitutional symptoms were rare (21.4%), and associated tuberculosis foci, mostly inactive, were seen in 43.8%. Breast parenchymal involvement, chest wall masses, pectoral muscle abscesses, cold abscesses tracking through the chest wall, and empyema necessitatis presented as breast lumps. Fine-needle aspiration cytology was the most common diagnostic modality, and acid-fast bacilli were demonstrable in a minority (33%). A combination of granulomatous mastitis, necrosis, and clinical response to antitubercular therapy was the most common mode of establishing the diagnosis. Most patients responded to antitubercular therapy alone.

Conclusion

Tubercular mastitis is a rare cause of breast masses in men, and a high index of suspicion can prevent diagnostic delays. Most patients respond to antitubercular therapy alone.     

Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States

BackgroundSignet ring cell carcinoma (SRCC) of the ampulla of Vater is poorly understood, with approximately 22 reported cases. Our study sought to create a comprehensive review of cases in the United States.MethodsWe used the Surveillance, Epidemiology, and End Results Program to collect all cases of ampullary adenocarcinoma diagnosed between 2010 and 2015.ResultsThe age-adjusted incidence rate of SRCC of the ampulla of Vater was 1.2 cases per 10,000,000 persons per year, with 50% more cases in males than females. We identified 3448 cases of adenocarcinoma of the ampulla of Vater, 81 of which were SRCC (2.3%). SRCC tended to present a later stage than other ampullary cancers, with median survival times of 17 vs. 25 months, (p = 0.07). Survival was significantly worse for SRCC when accounting for other clinical features (HR 1.46, p = 0.01). Factors portending worse prognosis in SRCC of the ampulla of Vater were advanced age, late stage and lack of surgical intervention.ConclusionOur study represents the largest study of SRCC of the ampulla of Vater to date. SRCC has a poorer prognosis compared with other ampullary cancers. Optimal treatment regimen is the most important future area of study.     

Whipple's disease: a curable encephalitis

Introduction

Whipple's disease is a rare systemic infectious disorder. It may present with a wide range of clinical manifestations and therefore its diagnosis may be challenging.

Case report

We report a 45-year-old man who presented with acute encephalitis related to Whipple's disease. Despite the negativity of the polymerase chain reaction (PCR) test in cerebrospinal fluid, diagnosis was obtained by histopathology, PCR tests and immunohistochemistry in multiple samples. The outcome with antibiotherapy was considered as “spectacular”.

Conclusion

Whipple's disease is a rare cause of encephalitis. Its diagnosis needs the confrontation of histology and PCR tests in multiple samples. The outcome with an adapted antibiotherapy may be very successful.     

Tumor desmoide intraabdominal simulando una tumoración pancreática en una paciente con poliposis colónica familiar

Introduction

Fibromatosis consists of a benign fibroblastic proliferation with local infiltrative growth. Two types are recognized: a superficial and a deep form, also known as desmoid tumor. The latter may occur sporadically or in association with familial adenomatous polyposis and Gardner's syndrome. Pancreatic presentation is exceptional and only eight cases have been described in the literature.

Case report

We report the case of a 29-year-old woman with a history of familial colonic polyposis and two pancreatic lesions. In the surgical specimen, two poorly defined pancreatic lesions were observed with infiltration of neighboring organs. Histologically, the lesions corresponded to mesenchymal proliferation with a fusocellular pattern without cytological atypica, which were diagnosed as desmoid tumors.

Discussion

The etiology of fibromatosis is unknown. In patients with familial colonic polyposis, the most common localization of desmoid tumor is intra-abdominal. Pancreatic presentation is unusual, requiring differential diagnosis with other pancreatic neoplasms.     

Characteristics and outcomes of the hyperglycemic hyperosmolar non-ketotic syndrome in a cohort of 51 consecutive cases at a single center

Aims

The hyperglycemic hyperosmolar syndrome (HHS) is a life-threatening diabetic complication. We aimed to portrait the short and long term outcome after a HHS episode and to describe features associated with increased early mortality.

Methods

We collected data from consecutive HHS cases, defined based on rigorous glucose and osmolality criteria. We retrieved anthropometric measures, history of diabetes, other cardiovascular risk factors and chronic co-morbidity. Clinical and biochemical parameters were recorded at admission, after 24 h and at discharge. We assessed incidence of complications, as well as short (≤30 days) and long term mortality.

Results

Patients were about 80-year old. Fifty-one cases were included, characterized by severe hyperglycemia (55.5 mosm/L) and hyperosmolality (385 mosm/L). Thirty-three percent developed at least one complication. Short term mortality was 16%; lower Glasgow Coma Scale, higher plasma glucose and mild acidosis were predictive of short term mortality. The long term mortality (median follow-up 1.27 years) was not significantly different from historical mortality data after hypoglycemic coma.

Conclusion

In this study, early mortality of HHS was 16% and some clinical features at presentation were predictive of an adverse short term outcome. Long term survival after a HHS episode did not appear to be severely impaired.     

Emergency care of patients with upper gastrointestinal bleeding in regional hospitals in Catalonia

Objective

To evaluate the resources available in Catalan regional hospitals for the emergency care of upper gastrointestinal hemorrhage.

Methods

We analyzed a survey sent to 32 hospitals on the availability, composition and resources of a duty endoscopy service for the year 2009.

Results

Responses were obtained from 24 centers, covering 3,954,000 inhabitants. Duty endoscopists were available in 12 hospitals. A total of 1,483,000 inhabitants were unable to access a duty endoscopist in the referral center. Centers with duty endoscopists had more beds and had a larger catchment area. Duty services were composed of 4.5 endoscopists (range 2-11), covering 82.1 (33.2-182.5) duty shifts/year. Seventeen centers reported 1,571 episodes (51%, range: 3-280, 39.68/100,000 inhabitants). Centers with a duty service reported a greater number of cases (76 vs. 43, p = 0.047). Centers without this service referred a greater number of patients (147 vs. 17, p = 0.001). Patients in the emergency department were under the care of the internal medicine department in four centers, the surgery department in 14 centers and under the care of both departments in six. Admitted patients were under the care of the gastroenterology department in only six hospitals. The most widely used procedures were ligation of varicose bleeding and injection therapies in non-varicose bleeding. Twenty-one percent of centers did not perform combined treatment.

Conclusions

A significant proportion of the population does not have access to a duty endoscopist in referral centers. Duty shifts represent significant workload in regional hospitals. Coordination among health professionals and centers would allow the efficient application of therapeutic resources and a duty endoscopy service to be established in centers lacking this resource.     

Impact of comorbidities on in-hospital mortality from acute myocardial infarction, 2003-2009

Introduction and objectives

Treatment of acute myocardial infarction has changed notably in recent years. The objective of this study was to analyze trends in in-hospital mortality during the period 2003-2009 and to examine how changes in comorbidity indices affected mortality prediction models for acute myocardial infarction using the minimum basic data set.

Methods

During the study period, 5275 cases of acute myocardial infarction were admitted. Mortality rates were calculated by age and sex and Charlson and Elixhauser comorbidity index scores were obtained on admission for every patient. Trends were analyzed and their validity studied. Multivariate models predictive of mortality were derived and compared.

Results

Mean age and comorbidities increased in all patients over the period 2003-2009. In spite of these trends, acute myocardial infarction mortality decreased. Comorbidity indices remained valid when the criterion “present on admission” was applied. Multivariate predictive models included age, sex, medical treatment, coronary revascularization and a comorbidity index or specific comorbidities. The model with specific comorbidities showed the best predictive ability. All models found that age and comorbidities increased the risk of death, and that coronary revascularization and treatment with anticoagulants, fibrinolytics, and platelet antiaggregants were protective factors.

Conclusions

Despite the fact that the mean age and number of comorbidities in acute myocardial infarction patients has increased year over year, acute myocardial infarction mortality has decreased, probably because of more frequent reperfusion and revascularization therapy and better medical treatment.Full English text available from:www.revespcardiol.org     

Risk factor associations with clinical distal symmetrical polyneuropathy and various neuropathy screening instruments and protocols in type 1 diabetes

Objective

To determine whether DSP and neuropathy-assessment instruments used by non-physicians have similar risk factors.

Research design and methods

Analyses were cross-sectional (n = 176).

Results

Risk factors were similar for DSP and screening devices.

Conclusions

These data support the clinical utility of neuropathy screening devices used by non-physician personnel.     

Complications and Quality of Life Following Urinary Diversion After Cystectomy

Objective:

The aim of this review is to outline specific surgical complications, metabolic consequences and quality of life (QOL) following urinary diversion in patients undergoing radical cystectomy.

Methods:

Based on a comprehensive literature search (MEDLINE) the published knowledge on urinary diversion was examined regarding the research question with an emphasis on contemporary cystectomy series.

Results:

Despite the fact, that urinary diversion is commonplace in these days and the existing literature is rather extensive, the vast majority of assumptions are based on low level evidence (retrospective, single-institutional case series with small sample sizes and short-term followup). There are few randomized trials in this field.Early and late surgical complications following radical cystectomy have decreased over the past three decades for both incontinent and continent diversion, but are still significant. While metabolic disturbances are common after continent forms of urinary diversion, the problems can be minimized in most cases. Most reports testify that QOL is high after cystectomy irrespective of type of urinary diversion although urinary and sexual problems are common.

Conclusion:

Careful patient selection, strict adherence to proper surgical technique and appropriate life-long follow-up are of paramount importance in the successful management of patients undergoing radical cystectomy for bladder cancer.     

Troponin I levels in patients with preeclampsia

Introduction

Preeclampsia involves a diffuse inflammatory state and elevated levels of troponins in patients with preeclampsia have been anecdotally reported. It is, however, unknown whether it is attributable to the preeclampsia.

Objective

We sought to determine the troponin I levels at the time of delivery in pregnant women with and without preeclampsia.

Methods

Plasma samples were obtained at the time of delivery and serum troponin I was measured by ELISA method.

Results

Thirty-nine women were included (20 with preeclampsia and 19 without). Mean troponin I level was 0.008 ng/mL in patients with preeclampsia and 0.01 ng/mL in controls (P =.59). The highest troponin I level was 0.04 ng/mL for both patients with and without preeclampsia.

Conclusions

Preeclampsia was not associated with a rise in troponin I levels in our study. Patients with preeclampsia and elevated troponin levels should have further cardiac investigations.