Food-borne botulism in Alaska, 1947-1985: epidemiology and clinical findings

We reviewed records of all food-borne outbreaks of botulism in Alaska from 1947 through 1985. Fifty-nine confirmed or suspected outbreaks with 156 cases were reported. All outbreaks occurred in Alaska Natives and were associated with eating traditional Alaska Native foods. Forty-four (75%) of the outbreaks were laboratory confirmed and involved 133 persons. The overall annual incidence of confirmed or suspected botulism was 8.6 cases per 100,000 population. Seventeen persons died, an overall case-fatality rate of 11%. Type E toxin accounted for 32 (73%) laboratory-confirmed outbreaks; type A, six (14%); and type B, five (11%). Forty-one cases demonstrated botulinal toxin in one or more specimens (serum, gastric contents, or stool). Of the 41 botulinal toxin-positive persons, 38 (93%) had at least three of the commonly recognized pentad of signs or symptoms--nausea and vomiting, dysphagia, diplopia, dilated and fixed pupils, or dry mouth and throat--and 20 (49%) required respiratory assistance.     

Central nervous system paracoccidioidomycosis: clinical features and laboratorial findings

OBJECTIVE: To study prospectively the clinical features and laboratorial characteristics of 24 patients with central nervous system (CNS) involvement with paracoccidioidomycosis (PCM). PCM is an infectious disease caused by the dimorphic fungus Paracoccidioides brasiliensis, endemic in subtropical areas of Central and South America. METHODS: From 173 cases of PCM, 24 (13.9%) had CNS involvement (NPCM) and were studied prospectively from 1993 to 1997. In all the patients, the diagnosis of systemic PCM was made by the demonstration of the P. brasiliensis organisms or positive serology, DID (double immunodiffusion). In seven cases the diagnosis was made by means of a CNS biopsy. CNS clinical manifestations, neuroimaging (CT or MRI) and CSF cytochemical characteristics were reported. RESULTS: The mean age was 44 years (range 25-72 years); 23 patients were male, only one was female. Neurological symptoms began before systemic symptoms in 21%; simultaneously in 33%, and after systemic symptoms in 46%. Epilepsy was the more frequent neurological presentation (44%). Twenty-three cases had parenchymatous involvement and in two of these cases there was an association with meningitis and one case had spinal cord involvement. Lesions were more frequent in the brain hemispheres (69%), in 65% there were multiple granuloma characterized by hypodense images with annular or nodular enhancing. All cases were treated with sulphamethoxazole-trimethoprin. Four patients died, while 20 patients showed a good therapeutic response. CONCLUSION: NPCM should always be considered in the differential diagnosis of expanding lesions of the CNS and meningoencephalitis. Being alert to this diagnosis depends on knowledge of epidemiology. There was good response to sulphamethoxazole-trimethoprin treatment.     

Silent atrial fibrillation: epidemiology,diagnosis, and clinical impact

Silent or subclinical asymptomatic atrial fibrillation (SAF ) has currently gained wide interest in the epidemiologic, neurologic, and cardiovascular communities. It is well known that the electrophysiological and mechanical effects of symptomatic and silent atrial fibrillation (AF ) are the same. It is probable that because “AF begets AF ,” progression from paroxysmal to persistent or permanent AF might be more rapid in patients with long‐term unrecognized and untreated SAF , because no treatment is sought by or provided to such patients. Moreover, SAF is common and has significant clinical implications. The clinical consequences of SAF , which include emboli (silent or symptomatic), heart failure, and early mortality, are of paramount importance. Consequently, SAF should be considered in estimating the prevalence of the disease and its impact on morbidity, mortality, and quality of life. Several diagnostic methods of arrhythmia detection utilizing the surface electrocardiogram (ECG ), subcutaneous ECG , or intracardiac devices have been utilized to seek meaningful arrhythmic markers of SAF . Whereas a wide range of clinical risk factors of SAF have been validated in the literature, there is an ongoing search for those arrhythmic risk factors that precisely identify and prognosticate outcome events in diverse populations at risk of SAF . Modern diagnostic modalities for the identification of SAF exist, but should be further explored, validated, and tailored to each patient needs. The scientific community should undertake the clinical challenge of identifying and treating SAF .     

HIV-TB: epidemiology, clinical features and diagnosis of smear-negativeTB

TB remains an important public health problem in the world that has been exacerbated by the HIV epidemic. In 2000, while 9% of new TB infections worldwide were attributable to HIV, in Sub-Saharan Africa--a region with higher HIV prevalence--about 31% of new TB cases were attributable to HIV. Clinical presentation of TB in HIV-infected individuals depends on the severity of the suppression of immunological functions. In patients in early stages of HIV infection, TB clinical presentation resembles that of HIV-negative individuals with more pulmonary involvement and localized lesions. With the progressive suppression of immunological functions, TB tends to be more generalized affecting more than one organ. Classic TB symptoms are non-specific and may result in delayed diagnosis or misdiagnosis. Diagnosis of smear negative pulmonary TB is based on clinical and radiological features. Sputum culture and rapid diagnostic tests based on polymerase chain reaction can be used where available.     

Miliary tuberculosis: epidemiology, clinical manifestations, diagnosis, and outcome

During the early 1970s, attention was called to the changing demographics and poor prognosis of patients with miliary tuberculosis. Thirty-eight non-AIDS patients with miliary tuberculosis seen since 1975 are reviewed. Their average age was 60 years. Two-thirds of the patients had comorbid conditions. Presenting symptoms were nonspecific; fever, anorexia, sweats, and weight loss were the most frequent. Fever, tachypnea, rales, and altered mental status were the most commonly associated signs. Chest radiographs often showed miliary disease, but the remainder of the laboratory abnormalities were nonspecific. Seventy-six percent of sputum cultures, 75% of gastric aspirate cultures, 59% of urine cultures, and 54% of bronchial washings were positive for Mycobacterium tuberculosis. Biopsy specimens, including those obtained by transbronchial biopsy, were frequently abnormal histologically but were rarely culture-positive. Mortality attributable to miliary tuberculosis was 21%. Risk factors for death included female sex and altered mental status. No patient treated initially with a regimen that included streptomycin died whereas 21% of those treated with other regimens died. These data confirm and extend the results of earlier studies and suggest that miliary tuberculosis is a disease of the elderly and immunocompromised and is associated with significant morbidity and mortality. A high index of suspicion and diagnostic persistence are required for diagnosis.     

Measles epidemiology in Mazandaran province, Iran, 2000-2002

To determine the incidence and characteristics of measles, cases reported to the Health Center for Diseases Control 2000-2002 were reviewed. Demographics data, vaccination status and its relation to occurrence the disease were analysed. A total of 729 cases (270 women,162, 269, 298, over the 3 years) were reported. The annual incidence rates over the 3 years were 7.1, 11.9 and 12.7 for each 10(5) population. In full vaccinated subjects, 46.5% of the cases occurred and 39.9% of the cases had not been vaccinated. Because of the high incidence rate of measles both in vaccinated and unvaccinated individuals, paying more attention to proper shipping and storing of vaccine, and even catch-up immunization for increasing herd immunity are recommended.     

Interruption of measles transmission in Brazil, 2000-2001

In 1992, Brazil adopted the goal of measles elimination by the year 2000; however, in 1997, after a 4-year period of good control, there was a resurgence of measles in Brazil. In 1999, to achieve the elimination goal, Brazil implemented the Supplementary Emergency Measles Action plan, with one measles surveillance technician designated to each state. Of 10,007 suspected measles cases reported during 1999, 908 (9.1%) were confirmed, and of them 378 (42%) were confirmed by laboratory analysis. Of 8358 suspected measles cases reported in 2000, 36 (0.4%) were confirmed (30 [83%] by laboratory); 92% of the discarded cases were classified on the basis of laboratory testing. In 2001, only 1 of 5599 suspected measles cases was confirmed, and it was an imported case from Japan. The last outbreak occurred in February 2000, with 15 cases. Current data suggest interruption of indigenous measles transmission in Brazil.     

Achalasia: A review of clinical diagnosis,epidemiology, treatment and outcomes

Achalasia is a neurodegenerative motility disorder of the oesophagus resulting in deranged oesophageal peristalsis and loss of lower oesophageal sphincter function.Historically,annual achalasia incidence rates were believed to be low,approximately 0.5-1.2 per 100000.More recent reports suggest that annual incidence rates have risen to 1.6 per 100000 in some populations.The aetiology of achalasia is still unclear but is likely to be multi-factorial.Suggested causes include environmental or viral exposures resulting in inflammation of the oesophageal myenteric plexus,which elicits an autoimmune response.Risk of achalasia may be elevated in a sub-group of genetically susceptible people.Improvement in the diagnosis of achalasia,through the introduction of high resolution manometry with pressure topography plotting,has resulted in the development of a novel classification system for achalasia.This classification system can evaluate patient prognosis and predict responsiveness to treatment.There is currently much debate over whether pneumatic dilatation is a superior method compared to the Heller’s myotomy procedure in the treatment of achalasia.A recent com-parative study found equal efficacy,suggesting that patient preference and local expertise should guide the choice.Although achalasia is a relatively rare condition,it carries a risk of complications,including aspiration pneumonia and oesophageal cancer.The risk of both squamous cell carcinoma and adenocarcinoma of the oesophagus is believed to be significantly increased in patients with achalasia,however the absolute excess risk is small.Therefore,it is currently unknown whether a surveillance programme in achalasia patients would be effective or cost-effective.     

Listeriosis in Iceland, 1978-2000: a description of cases and molecular epidemiology

The purpose of our study was to review all cases of listeriosis in Iceland during the period 1978-2000 and to analyse the genetic relatedness of their isolates. Case records of all patients in Iceland with listeriosis during the period were reviewed and the isolates compared using serotyping and pulsed-field gel electrophoresis (PFGE) using SmaI, AseI and ApaI restriction enzymes. Forty cases of listeriosis were diagnosed during the period, resulting in a mean annual incidence of 6.9 cases per million and a case fatality rate of 33%. In the first 5 y of the study only serotype 4b was observed; subsequently serotypes 1/2a and 1/2b appeared and serotype 4b declined in prevalence. PFGE yielded 24 different genotypes with 7 clusters of indistinguishable genotypes, each comprising 2-6 cases. During 1992-95 the annual incidence of listeriosis in Iceland rose to 15 cases per million. This was largely due to 2 clusters, 1 of 3 cases and the other of 6. No cases of listeriosis were diagnosed during 1998-2000. Our data show an increased number of cases within clusters in the latter half of the period. At the same time, food processing and distribution has become increasingly centralized in Iceland, suggesting an increased risk of listeriosis outbreaks.     

Coccidioidal meningitis: Update on epidemiology, clinical features, diagnosis, and management

Coccidioidomycosis is a fungal infection endemic to geographically restricted areas of the southwestern United States, and its incidence is increasing within endemic areas. Coccidioidal meningitis, an infrequent but very severe form of coccidioidomycosis, is associated with significant mortality and morbidity. The diagnosis depends on the demonstration of coccidioidal antibody or positive culture, histopathology, or cytology in the presence of typical abnormalities of the cerebrospinal fluid. Central nervous system complications of this infection may include hydrocephalus, vasculitis, infarctions, or parenchymal abscesses. Many patients with coccidioidal meningitis can achieve remission of infection following treatment with triazole antifungal agents. However, first-line therapy with high-dose oral fluconazole is associated with a high incidence of relapse after drug discontinuation. This article summarizes the pathogenesis, epidemiology, clinical manifestations, diagnostic studies, and treatment of coccidioidal meningitis, and highlights new information regarding this devastating infection.     

Gastrointestinal stromal tumors: epidemiology, clinical picture, diagnosis, prognosis and treatment

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract. They originate from the myenteric ganglion cells, termed the interstitial Cajal cells. The majority, i.e. 95% of GIST, show expression of the membrane receptor protein CD117 with a tyrosine kinase activity c-kit. Gastrointestinal stromal tumors constitute less than 1% of all digestive tract tumors. They may be benign or malignant (30%), and occur in every part of the gastrointestinal tract, however the stomach is the most common localization. They develop with the same prevalence in men and in women, usually above the age of 50 years. The peak incidence is observed between the fifth and the sixth decade of life. Symptoms are not typical and depend on the localization and the tumor size. About 10-30% of GIST are completely asymptomatic, and are discovered accidentally during the endoscopy or X-rays evaluation as well as during surgical interventions performed for various reasons. The malignant tumors metastasize most commonly to the liver and peritoneum. The metastases are rarely found in the lungs, pleura and bones. The detection of GIST is based on imaging, endoscopy, histological and immunohistochemical examinations. A useful and promising diagnostic procedure is positron emission tomography. The final diagnosis is mostly based on the pathologic findings of the removed tumor. The prognosis of GIST depends on its size, mitotic activity in 50 high power fields and mucosal infiltration. Radical surgery is the best treatment of GIST.     

Dysphagia lusoria: clinical aspects, manometric findings, diagnosis, and therapy

OBJECTIVE: The lusorian artery is a rare anomaly of the right subclavian artery. This artery arises from the aortic arch distal of the left subclavian artery, crossing the midline behind the esophagus. Normally this anomaly causes no symptoms. Sometimes dysphagia first appears above the age of 40 yr. METHODS: In the period of 1992-1997, the diagnosis of an aberrant right subclavian artery was made in five patients with dysphagia who were referred to a small community hospital. A sixth patient had a right-sided aorta with an aberrant left subclavian artery. RESULTS: Endoscopy revealed a pulsating impression in the esophagus of three patients. Four patients had coexisting esophageal abnormalities. Barium contrast examination of the esophagus showed a characteristic diagonal impression at the level of the fourth thoracic vertebra in all patients. Computed tomography and angiography confirmed the diagnosis and excluded aneurysms. Manometric investigation of the esophagus revealed nonspecific abnormalities in five patients. Drug treatment was sufficient in three patients (mean follow-up, 6.2 yr). Three patients were operated upon because of persistent dysphagia. Through a cervical approach the artery was ligated near its root and connected with the right carotid artery. Postoperatively two patients became symptom-free, the other patient still has intermittent dysphagia. CONCLUSION: Dysphagia can be caused by a rare anomaly of the subclavian artery. The diagnosis can be overlooked at endoscopy, but barium contrast study of the esophagus will reveal the abnormality. In patients with coexisting esophageal abnormalities the finding may be incidental and specific conservative treatment may be sufficient. Manometry cannot be used to diagnose this condition or to predict surgical outcome. When the symptoms are intractable, surgical correction should be considered even if coexisting esophageal abnormalities are present.     

Pulmonary arterial hypertension in collagenoses: clinical features, epidemiology, pathogenesis, diagnosis and treatment

Pulmonary arterial hypertension (PAH) is a severe vasculopathy, which is characterised by progressive narrowing and obliteration of the pulmonary arterioles and increased endothelin-1 levels. The increase of vascular resistance in the lung vessels leads to chronic pressure overload and to right heart failure, if untreated. PAH often occurs in association with rheumatic-inflammatory diseases (e.g., in 15% of patients with systemic sclerosis (SSc), especially in the limited form or in CREST patients) and determines their prognosis: in advanced stages, untreated patients die within a short period. Therefore all SSc patients, particularly the newly diagnosed ones, should be screened for PAH with echocardiography. If PAH is suspected, a right heart catheter should be performed, and if PAH is confirmed, adequate treatment should be initiated. While few years ago lung transplantation was the only option for patients with severe PAH, in recent years enormous progress was seen in drug treatment. Today prostanoids (Ventavis) and the endothelin receptor antagonist bosentan (Tracleer) are available for patients with PAH in WHO/NYHA stage III: they have substantially improved the prognosis of PAH in the last years. Since few months, also the phosphodiesterase inhibitor sildenafil (Revatio) is available. The combination of drugs with different mode of action will likely further improve the prognosis of PAH patients.     

Pancreatic cancer: A review of clinical diagnosis,epidemiology, treatment and outcomes

This review aims to outline the most up-to-date knowledge of pancreatic adenocarcinoma risk, diagnostics, treatment and outcomes, while identifying gaps that aim to stimulate further research in this understudied malignancy. Pancreatic adenocarcinoma is a lethal condition with a rising incidence, predicted to become the second leading cause of cancer death in some regions. It often presents at an advanced stage, which contributes to poor five-year survival rates of 2%-9%, ranking firmly last amongst all cancer sites in terms of prognostic outcomes for patients. Better understanding of the risk factors and symptoms associated with this disease is essential to inform both health professionals and the general population of potential preventive and/or early detection measures. The identification of high-risk patients who could benefit from screening to detect pre-malignant conditions such as pancreatic intraepithelial neoplasia, intraductal papillary mucinous neoplasms and mucinous cystic neoplasms is urgently required, however an acceptable screening test has yet to be identified. The management of pancreatic adenocarcinoma is evolving, with the introduction of new surgical techniques and medical therapies such as laparoscopic techniques and neo-adjuvant chemoradiotherapy, however this has only led to modest improvements in outcomes. The identification of novel biomarkers is desirable to move towards a precision medicine era, where pancreatic cancer therapy can be tailored to the individual patient, while unnecessary treatments that have negative consequences on quality of life could be prevented for others. Research efforts must also focus on the development of new agents and delivery systems. Overall, considerable progress is required to reduce the burden associated with pancreatic cancer. Recent, renewed efforts to fund large consortia and research into pancreatic adenocarcinoma are welcomed, but further streams will be necessary to facilitate the momentum needed to bring breakthroughs seen for other cancer sites.     

Mayaro fever in the city of Manaus, Brazil, 2007-2008

Mayaro Alphavirus is an arbovirus that causes outbreaks of acute febrile illness in the Amazon region of South America. We show here the cases of Mayaro fever that occurred in 2007-2008, in Manaus, a large city and capital of the Amazonas State, in Western Brazilian Amazon. IgM antibodies to Mayaro virus (MAYV) were detected by an enzyme immunoassay using infected cell cultures as antigen in the sera of 33 patients from both genera and 6-65 years old. MAYV genome was also detected by RT-PCR in the blood of 1/33 of these patients. The patients presented mainly with headache, arthralgia, myalgia, ocular pain, and rash. These cases of Mayaro fever are likely to represent the tip of an iceberg, and probably a much greater number of cases occurred in Manaus in the study period.     

Pneumonia due to Pseudomonas aeruginosa: part I: epidemiology, clinical diagnosis, and source

Pseudomonas aeruginosa is an uncommon cause of community-acquired pneumonia (CAP), but a common cause of hospital-acquired pneumonia. Controversies exist for diagnostic methods and antibiotic therapy. We review the epidemiology of CAP, including that in patients with HIV and also in hospital-acquired pneumonia, including ventilator-associated pneumonia (VAP) and bronchoscope-associated pneumonia. We performed a literature review of clinical studies involving P aeruginosa pneumonia with an emphasis on treatment and prevention. Pneumonia due to P aeruginosa occurs in several distinct syndromes: (1) CAP, usually in patients with chronic lung disease; (2) hospital-acquired pneumonia, usually occurring in the ICU; and (3) bacteremic P aeruginosa pneumonia, usually in the neutropenic host. Radiologic manifestations are nonspecific. Colonization with P aeruginosa in COPD and in hospitalized patients is a well established phenomenon such that treatment based on respiratory tract cultures may lead to overtreatment. We present circumstantial evidence that the incidence of P aeruginosa has been overestimated for hospital-acquired pneumonia and reflex administration of empirical antipseudomonal antibiotic therapy may be unnecessary. A diagnostic approach with BAL and protected specimen brush using quantitative cultures for patients with VAP led to a decrease in broad-spectrum antibiotic use and improved outcome. Endotracheal aspirate cultures with quantitative counts are commonly used, but validation is lacking. An empirical approach using the Clinical Pulmonary Infection Score is a pragmatic approach that minimizes antibiotic resistance and leads to decreased mortality in patients in the ICU. The source of the P aeruginosa may be endogenous (from respiratory or GI tract colonization) or exogenous from tap water in hospital-acquired pneumonia. The latter source is amenable to preventive measures.