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Stephen S Arnon - One of the best experts on this subject based on the ideXlab platform.
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More Clinical Mimics of Infant Botulism
The Journal of Pediatrics, 2018Co-Authors: Jessica M. Khouri, Jessica R. Payne, Stephen S ArnonAbstract:Objective To ascertain the actual diagnoses of 76 patients (2005-2015) whose clinical presentations so closely resembled infant Botulism that the patients were treated with Human Botulism Immune Globulin Intravenous (BIG-IV; BabyBIG), but whose illnesses subsequently were not laboratory confirmed as infant Botulism (“clinical mimics” of infant Botulism). Study design The California Department of Public Health produces BIG-IV and distributes it nationwide as a public service (ie, not-for-profit) orphan drug to treat patients hospitalized with suspected infant Botulism. During the study period, admission records and discharge summaries for all patients treated with BIG-IV but who lacked a laboratory-confirmed diagnosis of infant Botulism were collected and abstracted. The patients' discharge diagnoses were identified, categorized, and compared with previously reported clinical mimics categories for 32 patients (1992-2005). Results From 2005 to 2015, 76 clinical mimic illnesses were identified. These illnesses were distributed into the 5 categories previously reported of (1) probable infant Botulism lacking confirmatory testing (26.3%); (2) spinal muscular atrophy (19.7%); (3) miscellaneous (15.8%); (4) metabolic disorders (11.8%); and (5) other infectious diseases (10.6%). Of the 76 clinical mimic illnesses, 15.8% had no alternate diagnosis established and were therefore categorized as undetermined. Conclusions Over the 23 years 1992-2015, patients presenting with illnesses so clinically similar to infant Botulism that they were treated with BIG-IV had actual diagnoses that were distributed into 5 main categories. These categories and their individual components constitute a working bedside differential diagnosis of infant Botulism.
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global occurrence of infant Botulism 1976 2006
Pediatrics, 2008Co-Authors: Ruth Koepke, Jeremy Sobel, Stephen S ArnonAbstract:OBJECTIVE. To summarize the worldwide occurrence of reported infant (intestinal toxemia) Botulism cases since first recognition of the disease in 1976. PATIENTS AND METHODS. We collected information on infant Botulism cases by active and passive surveillance, by provision of therapeutic Human Botulism Immune Globulin to suspected cases, and by searching the medical literature. We defined a case as laboratory-confirmed Botulism that occurred in an infant ≤12 months of age that was not caused by the ingestion of botulinum toxin in food. RESULTS. Twenty-six countries representing 5 continents reported the occurrence of at least 1 case of infant Botulism among their residents. The United States, Argentina, Australia, Canada, Italy, and Japan, in this order, reported the largest number of cases. A history of honey exposure was significantly more common among case subjects hospitalized outside of the United States than among those who were recently hospitalized in California. CONCLUSIONS. Most countries have not yet reported cases of infant Botulism. This limited reporting of the disease to date contrasts with the known global occurrence of Clostridium botulinum spores in soils and dust and suggests that infant Botulism may be underrecognized, underreported, or both. When bulbar palsies, hypotonia, and weakness are present, physicians should consider the possibility of infant Botulism even if the patient has not been fed honey. Publication of additional case reports and surveillance summaries will enhance understanding of the occurrence and extent of this underrecognized disease.
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creation and development of the public service orphan drug human Botulism immune globulin
Pediatrics, 2007Co-Authors: Stephen S ArnonAbstract:The public service orphan drug Human Botulism Immune Globulin for the treatment of infant Botulism would not have come into existence without the federal Orphan Drug Act and the funding mechanism that it provided to conduct pivotal clinical trials. Nonetheless, creating, developing, and achieving licensure of Human Botulism Immune Globulin took approximately 15 years and approximately $10.6 million (2005 dollars) to accomplish. Use of Human Botulism Immune Globulin to treat patients with infant Botulism has resulted thus far in more than 30 years of avoided hospital stay and more than $50 million (2005 dollars) of avoided hospital costs. To provide a possible paradigm for others, the circumstances that enabled a state public health department to create, test, license, and distribute an orphan drug are described here.
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Clinical Mimics of Infant Botulism
PEDIATRICS, 2007Co-Authors: Ann Marie O. Francisco, Stephen S ArnonAbstract:Since 1992, Human Botulism Immune Globulin has been provided by the California Department of Health Services to infants with probable infant Botulism, the intestinal toxemia form of human Botulism. Human Botulism Immune Globulin became available in California in 1992–1997 within a randomized, controlled, double-blinded, pivotal clinical trial and subsequently became available nationwide in 1998 –2003 in an open-label study until its licensure in October 2003 as BabyBIG. Thereafter, Human Botulism Immune Globulin remained available nationwide as an approved orphan-drug product. To achieve prompt neutralization of circulating botulinum toxin, the decision to treat with Human Botulism Immune Globulin has been based on clinical criteria that include a consistent history and physical findings of bulbar palsies, hypotonia, and weakness. After licensure, the charts of patients who did not have laboratory-confirmed infant Botulism were reviewed to identify their actual diagnoses. The 5% of 681 patients treated with Human Botulism Immune Globulin who did not have infant Botulism fell into 5 categories: spinal muscular atrophy, metabolic disorders, other infectious diseases, miscellaneous, and probable infant Botulism lacking laboratory confirmation.
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Human Botulism immune globulin for the treatment of infant Botulism.
New England Journal of Medicine, 2006Co-Authors: Stephen S Arnon, Susan E Maslanka, Robert Schechter, Nicholas P. Jewell, Charles L. HathewayAbstract:Background We created the orphan drug Human Botulism Immune Globulin Intravenous (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant Botulism, the intestinal-toxemia form of human Botulism. Methods We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant Botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant Botulism treated within 18 days after hospital admission. Results As compared with the control group in the randomized trial, infants treated with BIG-IV had a reduction in the mean length of the hospital stay, the primary efficacy outcome measure, from 5.7 weeks to 2.6 weeks (P
Susan E Maslanka - One of the best experts on this subject based on the ideXlab platform.
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Botulism as a Disease of Humans
Molecular Aspects of Botulinum Neurotoxin, 2014Co-Authors: Susan E MaslankaAbstract:Human Botulism presents in several different forms which provide unique challenges to public health. Foodborne Botulism, while still an issue with home-processed foods, is sometimes associated with restaurants and commercially produced (particularly chilled) foods. The diversity of food products available to consumers, which are widely distributed, requires an integrated, multiagency response approach to contain, evaluate and develop appropriate prevention measures. Wound and adult intestinal toxemia cases are difficult to confirm, and so the total illness burden is likely unknown. Infant Botulism highlights the diversity of the neurotoxin-producing Clostridium sp. and associated toxins which can cause Botulism worldwide. Finally, an astounding number of adverse events associated with toxin injections remind us of the hazards of botulinum toxin even as its therapeutic benefits expand.
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national outbreak of type a foodborne Botulism associated with a widely distributed commercially canned hot dog chili sauce
Clinical Infectious Diseases, 2013Co-Authors: Patricia Juliao, Janet K Dykes, Susan E Maslanka, Linda Gaul, Satish Bagdure, Lynae Granzowkibiger, Ellen Salehi, Donald Zink, Robert P Neligan, Casey BartonbehraveshAbstract:Foodborne Botulism is a rare, neuroparalytic illness caused by consumption of a botulinum neurotoxin (BoNT) produced by the bacterium Clostridium botulinum. These bacteria are found in the environment as dormant spores but can germinate and produce toxin in foods under permissive conditions of pH, water activity, anaerobic environment, and temperature. Human Botulism is usually caused by toxin types A, B, or E, and, rarely, type F. BoNT binds irreversibly to the presynaptic membranes of peripheral neuromuscular and autonomic nerve junctions, causing a symmetric, descending flaccid paralysis. Death (<5% annually) occurs from respiratory muscle paralysis [1, 2]. Most cases of foodborne Botulism in the United States are due to improperly handled (primarily home-preserved) foods [1–3]. Botulism attributed to commercially canned foods is rare. Proper commercial canning, owing to the controlled temperature and processing time, renders food commercially sterile (free of viable microorganisms, including those of public health significance such as spores of C. botulinum, capable of reproducing under normal nonrefrigerated conditions during storage and transport) [4]. A commercial canning process uses a closed vessel (known as a retort) to heat a product under pressure. Specific heating time and temperature in the retort depends on the product being sterilized. Manufacturers apply a code on all cans processed during a defined time period (sometimes described as a single retort), which identifies the production lot. During 1 week in July 2007, 4 cases of suspected Botulism were reported to the Centers for Disease Control and Prevention (CDC) from 2 states (Texas and Indiana). Given the rarity of Botulism in the continental United States, the appearance of 4 Botulism cases in different geographic locations within the same week suggested that a distributed product could be involved. Until this outbreak, no US Botulism cases were reported associated with underprocessing (failure to adequately sterilize) of commercial canned foods in >30 years [1]. We describe the investigation of this national foodborne Botulism outbreak caused by a commercially canned product.
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first report worldwide of an infant Botulism case due to clostridium botulinum type e
Journal of Clinical Microbiology, 2010Co-Authors: Carolina Luquez, Janet K Dykes, Brian H Raphael, Susan E MaslankaAbstract:Clostridium botulinum type E has been associated with Botulism in adults but never in infants. Infant Botulism type E cases have been associated with neurotoxigenic strains of C. butyricum. We report the first infant Botulism case due to C. botulinum type E worldwide.
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Human Botulism immune globulin for the treatment of infant Botulism.
New England Journal of Medicine, 2006Co-Authors: Stephen S Arnon, Susan E Maslanka, Robert Schechter, Nicholas P. Jewell, Charles L. HathewayAbstract:Background We created the orphan drug Human Botulism Immune Globulin Intravenous (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant Botulism, the intestinal-toxemia form of human Botulism. Methods We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant Botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant Botulism treated within 18 days after hospital admission. Results As compared with the control group in the randomized trial, infants treated with BIG-IV had a reduction in the mean length of the hospital stay, the primary efficacy outcome measure, from 5.7 weeks to 2.6 weeks (P
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human Botulism immune globulin for the treatment of infant Botulism
The New England Journal of Medicine, 2006Co-Authors: Stephen S Arnon, Susan E Maslanka, Robert Schechter, Nicholas P. Jewell, Charles L. HathewayAbstract:Background We created the orphan drug Human Botulism Immune Globulin Intravenous (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant Botulism, the intestinal-toxemia form of human Botulism. Methods We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant Botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant Botulism treated within 18 days after hospital admission. Results As compared with the control group in the randomized trial, infants treated with BIG-IV had a reduction in the mean length of the hospital stay, the primary efficacy outcome measure, from 5.7 weeks to 2.6 weeks (P<0.001). BIG-IV treatment also reduced the mean...
Charles L. Hatheway - One of the best experts on this subject based on the ideXlab platform.
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Human Botulism immune globulin for the treatment of infant Botulism.
New England Journal of Medicine, 2006Co-Authors: Stephen S Arnon, Susan E Maslanka, Robert Schechter, Nicholas P. Jewell, Charles L. HathewayAbstract:Background We created the orphan drug Human Botulism Immune Globulin Intravenous (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant Botulism, the intestinal-toxemia form of human Botulism. Methods We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant Botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant Botulism treated within 18 days after hospital admission. Results As compared with the control group in the randomized trial, infants treated with BIG-IV had a reduction in the mean length of the hospital stay, the primary efficacy outcome measure, from 5.7 weeks to 2.6 weeks (P
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human Botulism immune globulin for the treatment of infant Botulism
The New England Journal of Medicine, 2006Co-Authors: Stephen S Arnon, Susan E Maslanka, Robert Schechter, Nicholas P. Jewell, Charles L. HathewayAbstract:Background We created the orphan drug Human Botulism Immune Globulin Intravenous (BIG-IV), which neutralizes botulinum toxin, and evaluated its safety and efficacy in treating infant Botulism, the intestinal-toxemia form of human Botulism. Methods We performed a five-year, randomized, double-blind, placebo-controlled trial statewide, in California, of BIG-IV in 122 infants with suspected (and subsequently laboratory-confirmed) infant Botulism (75 caused by type A Clostridium botulinum toxin, and 47 by type B toxin); treatment was given within three days after hospital admission. We subsequently performed a 6-year nationwide, open-label study of 382 laboratory-confirmed cases of infant Botulism treated within 18 days after hospital admission. Results As compared with the control group in the randomized trial, infants treated with BIG-IV had a reduction in the mean length of the hospital stay, the primary efficacy outcome measure, from 5.7 weeks to 2.6 weeks (P<0.001). BIG-IV treatment also reduced the mean...
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Botulism in the United States: A Clinical and Epidemiologic Review
Annals of Internal Medicine, 1998Co-Authors: Roger L. Shapiro, Charles L. Hatheway, David L. SwerdlowAbstract:Botulism is caused by a neurotoxin produced from the anaerobic, spore-forming bacterium Clostridium botulinum. Botulism in humans is usually caused by toxin types A, B, and E. Since 1973, a median of 24 cases of foodborne Botulism, 3 cases of wound Botulism, and 71 cases of infant Botulism have been reported annually to the Centers for Disease Control and Prevention (CDC). New vehicles for transmission have emerged in recent decades, and wound Botulism associated with black tar heroin has increased dramatically since 1994. Recently, the potential terrorist use of botulinum toxin has become an important concern. Botulism is characterized by symmetric, descending, flaccid paralysis of motor and autonomic nerves, usually beginning with the cranial nerves. Blurred vision, dysphagia, and dysarthria are common initial complaints. The diagnosis of Botulism is based on compatible clinical findings; history of exposure to suspect foods; and supportive ancillary testing to rule out other causes of neurologic dysfunction that mimic Botulism, such as stroke, the Guillain-Barre syndrome, and myasthenia gravis. Laboratory confirmation of suspected cases is performed at the CDC and some state laboratories. Treatment includes supportive care and trivalent equine antitoxin, which reduces mortality if administered early. The CDC releases Botulism antitoxin through an emergency distribution system. Although rare, Botulism outbreaks are a public health emergency that require rapid recognition to prevent additional cases and to effectively treat patients. Because clinicians are the first to treat patients in any type of Botulism outbreak, they must know how to recognize, diagnose, and treat this rare but potentially lethal disease.
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Botulism surveillance and emergency response a public health strategy for a global challenge
JAMA, 1997Co-Authors: Roger L. Shapiro, Charles L. Hatheway, John Becher, David L. SwerdlowAbstract:Botulism is a neuroparalytic disease caused by a neurotoxin produced from an anaerobic spore-forming bacterium known as Clostridium botulinum . 1 The lethal potency of this toxin has mandated intensive surveillance and control measures in the United States. Recent outbreaks of Botulism have raised questions regarding the international supply and therapeutic use of Botulism antitoxin, and reports that national governments 2 and terrorist groups 3 have stockpiled Botulism toxin have increased levels of concern regarding global preparedness for an intentional episode of Botulism poisoning. It is estimated that as little as 1 g of aerosolized Botulism toxin has the potential to kill at least 1.5 million people, and modern techniques of aerosolization via tactical ballistic missiles or aeronautical spraying may be capable of disseminating up to 60% of this dosage to a target population. 4 A description of the clinical features of aerosolized Botulism toxin poisoning appears elsewhere in this issue.
Michel Popoff - One of the best experts on this subject based on the ideXlab platform.
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Public Health Risk Associated with Botulism as Foodborne Zoonoses
Toxins, 2020Co-Authors: Christine Rasetti-escargueil, Emmanuel Lemichez, Michel PopoffAbstract:Botulism is a rare but severe neurological disease in man and animals that is caused by botulinum neurotoxins (BoNTs) produced by Clostridium botulinum and atypical strains from other Clostridium and non-Clostridium species. BoNTs are divided into more than seven toxinotypes based on neutralization with specific corresponding antisera, and each toxinotype is subdivided into subtypes according to amino acid sequence variations. Animal species show variable sensitivity to the different BoNT toxinotypes. Thereby, naturally acquired animal Botulism is mainly due to BoNT/C, D and the mosaic variants CD and DC, BoNT/CD being more prevalent in birds and BoNT/DC in cattle, whereas human Botulism is more frequently in the types A, B and E, and to a lower extent, F. Botulism is not a contagious disease, since there is no direct transmission from diseased animals or man to a healthy subject. Botulism occurs via the environment, notably from food contaminated with C. botulinum spores and preserved in conditions favorable for C. botulinum growth and toxin production. The high prevalence of Botulism types C, D and variants DC and CD in farmed and wild birds, and to a lower extent in cattle, raises the risk of transmission to human beings. However, human Botulism is much rarer than animal Botulism, and Botulism types C and D are exceptional in humans. Only 15 cases or suspected cases of Botulism type C and one outbreak of Botulism type D have been reported in humans to date. In contrast, animal healthy carriers of C. botulinum group II, such as C. botulinum type E in fish of the northern hemisphere, and C. botulinum B4 in pigs, represent a more prevalent risk of Botulism transmission to human subjects. Less common Botulism types in animals but at risk of transmission to humans, can sporadically be observed, such as Botulism type E in farmed chickens in France (1998–2002), Botulism type B in cattle in The Netherlands (1977–1979), Botulism types A and B in horses, or Botulism type A in dairy cows (Egypt, 1976). In most cases, human and animal Botulisms have distinct origins, and cross transmissions between animals and human beings are rather rare, accidental events. But, due to the severity of this disease, human and animal Botulism requires a careful surveillance.
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Botulinum Neurotoxins: More and More Diverse and Fascinating Toxic Proteins
Journal of Infectious Diseases, 2013Co-Authors: Michel PopoffAbstract:Botulism is a rare but severe disease mainly resulting from food poisoning or intestinal colonization. Food poisoning, including Botulism, certainly occurred in ancient times [...]
P. Aureli - One of the best experts on this subject based on the ideXlab platform.
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Intestinal toxemia Botulism in Italy, 1984–2005
European Journal of Clinical Microbiology & Infectious Diseases, 2007Co-Authors: L. Fenicia, F. Anniballi, P. AureliAbstract:Botulism in humans is caused by botulinum neurotoxins, produced in most cases by Clostridium botulinum , although other Clostridia species are implicated as well. Of the five forms of Botulism in humans, three are referred to as “infective”: wound Botulism, infant Botulism, and adult intestinal Botulism; the latter two forms are also referred to as “intestinal toxemia Botulism” because the organism colonizes the lumen of the intestinal tract and produces botulinum neurotoxin in vivo. Twenty-three cases of infant Botulism and three cases of adult intestinal Botulism occurred in Italy between 1984 and 2005. Microbiological analyses of clinical, environmental, and food samples and analysis of clinical and epidemiological data revealed two main characteristics of intestinal toxemia Botulism in Italy that are not common in cases in other countries: the isolation of a strain of C. butyricum that produced botulinum neurotoxin type E in 6 of 26 cases, including two cases of adult intestinal toxemia Botulism, and the onset of Botulism in these cases with concomitant severe gastrointestinal symptomatology. This report summarizes the microbiological, clinical, and epidemiological data of all cases of intestinal toxemia Botulism that have occurred in Italy in the period 1984–2005.