Infantile Botulism Research Articles

Neurally Adjusted Ventilatory Assist to Monitor Diaphragmatic Activity in Infantile Botulism.

Infantile botulism is a potentially life-threatening neuromuscular disorder. It presents with descending paralysis that can involve the diaphragm and cause respiratory failure. Neurally adjusted ventilatory assist (NAVA) provides synchronized bilevel positive pressure by using electrical diaphragmatic (Edi) signals. Diaphragmatic paresis is thought to be a contraindication for using NAVA. However, the use of a NAVA catheter allows continuous assessment of diaphragm activity in infantile botulism. We discuss a case of infantile botulism in an infant from central Pennsylvania who presented with poor oral feeding, hypothermia, and lethargy and progressed to develop apnea and acute respiratory failure. The infant was intubated and mechanically ventilated. A diagnosis of infantile botulism was confirmed through the detection of botulinum toxin in the infant's stool, and the infant was treated with botulism immune globulin neutralizing antibodies. During his recovery phase, a NAVA catheter was placed, which allowed monitoring of Edi signals to gauge the return of diaphragm activity and to assist with the assessment of extubation readiness. We describe the trends in this infant's Edi signals following administration of globulin neutralizing antibodies and the successful transition to invasive, and subsequently, noninvasive NAVA. Our report demonstrates the clinical utility of monitoring diaphragmatic activity using a NAVA catheter and that NAVA provided adequate respiratory support to an infant during the recovery phase of infantile botulism.

Clinical characteristics and prognosis of 8 cases of severe infant botulism

Objective: To summarize the clinical characteristics and prognosis of severe infant botulism and evaluate the therapeutic effect of botulinum antitoxin in the pediatric intensive care unit (PICU). Methods: The clinical data of 8 cases diagnosed with infantile botulism were retrospectively analyzed in the PICU of Beijing Children's Hospital from October 2019 to August 2023. Data of basic demographic information, clinical manifestations, laboratory tests, treatment and prognosis of each child were collected and analyzed using descriptive statistical methods. Results: Eight laboratory-confirmed cases of infant botulism were included in this study, all of which were male infants with an age of 6.0 (3.3,6.8) months. Three of the children were from Inner Mongolia Autonomous Region, 2 of them were from Hebei, and the other 3 were from Beijing, Shandong and Xinjiang Uyghur Autonomous Region, respectively. All the patients were previously healthy. In 4 of these cases, the possible cause was the ingestion of either honey and its products or sealed pickled food by the mother or child before the onset of the disease. The first symptom was poor milk intake (4 cases), followed by shallow shortness of breath (7 cases), limb weakness (7 cases) and so on. The typical signs were bilateral dilated pupils (8 cases) and decreased limb muscle strength (8 cases). The main subtype was type B (7 cases), and only 1 case was classified as type A. Six of the children were treated with antitoxin therapy for a duration of 24 (19, 49) d. Seven of them had invasive mechanical ventilation. All the patients survived upon discharge with a follow-up period of 29 d to 3 years and 8 months. Six patients had fully recovered, and 2 recently discharged patients were gradually recovering. Conclusions: For infants with suspected contact or ingestion of botulinum and presented with bilateral pupillary paralysis, muscle weakness and clear consciousness, the stool should be collected for diagnostic testing using a mouse bioassay as soon as possible. Type B was the most common type. The antitoxin treatment was effectiveness and the prognosis was well.

That head lag is impressive! Infantile botulism in the NICU: a case report

BackgroundInfantile botulism (IB) is a devastating and potentially life-threatening neuromuscular disorder resulting from intestinal colonization by Clostridium botulinum and the resultant toxin production. It can present with constipation, descending paralysis, and, potentially, respiratory failure. Botulism is a diagnosis that is more commonly seen in the pediatric intensive care unit (PICU) or on the general pediatric wards and would not typically be managed in the neonatal intensive care unit (NICU), and therefore requires high clinical suspicion to ensure prompt diagnosis and treatment.Case presentationWe discuss a case where an infant from central Pennsylvania presented to a Level IV NICU rather than to the PICU for an evaluation for sepsis and was uniquely diagnosed with IB. The infant presented with poor oral feeding and reduced oral intake, hypothermia, and lethargy. His symptoms progressed into hypoxia and acute respiratory failure. Interestingly, this infant had no known exposure to honey or any other identifiable sources of botulism contact. The infant’s twin brother and the other infants who attended the mother’s in-home daycare remained asymptomatic. This infant was initially evaluated and managed for a potential infectious etiology. However, a diagnosis of IB was suspected, and was later confirmed through the detection of botulinum toxin in the infant’s stools. A high level of suspicion allowed for timely treatment with Botulism Immune Globulin neutralizing antibodies (BabyBIG), even prior to confirmatory testing. We describe the process of obtaining BabyBIG, as well as the natural course of illness after treatment in our patient who ultimately made a complete recovery.ConclusionsThis case highlights the importance of considering infantile botulism as a diagnostic possibility even in the absence of risk factors, and the need for vigilance in diagnosing and treating this rare but potentially life-threatening condition. With timely recognition, subsequent treatment with BabyBIG, and supportive care, infants with infantile botulism can be expected to recover completely. This information is particularly important for neonatologists providing care for infants outside the neonatal period, especially during times of high patient census and resulting overflow of pediatric admissions in the NICU.

Neonatal Botulism: A Case Series Suggesting Varied Presentations

Infantile botulism is an uncommon diagnosis and rarer still in the neonatal period. We describe three cases of neonatal-onset botulism that presented with symptoms typically (hypotonia, constipation, facial diplegia) or atypically seen in older infants (encephalopathy, seizures, and hypothermia). Our series shows a wider spectrum of clinical presentations in patients with neonatal-onset botulism. Our report also suggests that neonatal-onset botulism should be considered more broadly in the hypotonic infant, especially as the condition is treatable with intravenous botulinum-specific immune globulin.

Inactivation of Clostridium Spores in Honey with Supercritical CO2 and in Combination with Essential Oils

The presence of tens of Clostridium botulinum spores per gram of honey can cause infantile botulism. Thermal treatment is insufficient to inactivate these resistant forms. This study explored the effectiveness of supercritical CO2 (scCO2) on its own and combined with lemon (LEO), clove (CLEO), and cinnamon (CEO) essential oils on the inactivation of Clostridium sporogenes (CECT 553) as a surrogate of Clostridium botulinum. In water, the degree of inactivation at 10 MPa after 60 min increased with the increasing temperature, reducing the population by 90% at 40 °C and by 99.7% at 80 °C. In contrast, when applied to honey, scCO2 did not inactivate Clostridium spores satisfactorily at temperatures below 70 °C, which was related to the protective effect of honey. Meanwhile, scCO2 modified with CEO (<0.4% mass) improved the inactivation degree, with a 1.3-log reduction achieved at 60 °C. With this same mixture, a reduction of 3.7 logs was accomplished in a derivative with 70% moisture. Honey was very sensitive to the temperature of the applied CO2. The obtained product could be used as a novel food, food ingredient, cosmetic, or medicine.

Feeding Intolerance in a 3-month-old.

Feeding Intolerance in a 3-month-old.

A case of infantile botulism and CMV coinfection: Red flags of an insidious diagnosis and the role intestinal dysbiosis and hypogammaglobulinemia as risk factors

Botulism is a rare pathology caused by a neurotoxin produced by bacteria of the genus Clostridium, responsible of a clinical condition characterized by flaccid paralysis and autonomic dysfunctions. Some forms, called infantile botulism, are secondary to the colonization of Clostridium botulinum spores in the gut of children and the subsequent production of the toxin. The reason why only some children develop the clinical condition is not clear.

Clinical analysis and laboratory diagnosis of three cases with infantile botulism caused by Clostridium botulinum type B

Objective: To summarize the clinical characteristics and laboratory diagnostic methods of infant botulism caused by Clostridium botulinum type B. Methods: Clinical data of 3 infants with type B botulism who were admitted to Children's Hospital Affiliated to Capital Institute of Pediatrics from May to November 2018 were retrospectively analyzed. Botulinum toxin was detected in fecal samples or fecal enrichment solution of the patients, and Clostridium botulinum was cultured and isolated from fecal samples. Results: The age of onset of the patients (two boys and one girl) was 3, 3 and 8 months old, respectively. Two cases had the onset in May and one case had the onset in November. There were two cases with mixed feeding and one case with breast feeding. One case's family members engaged in meat processing. All of them were previously healthy. All the children presented with acute flaccid paralysis, cranial nerve involvement and difficult defecation. Two cases had secondary urinary tract infection. Electromyograms of two cases showed that action potential amplitude of the motor nerve were lower than those of their peers. After treatments including intravenous human immunoglobulin, respiratory tract management, urethral catheterization, nasal feeding, etc., three cases recovered completely 2 to 4 months later. Type B botulinum toxin was detected in the fecal diluent of one patient, and the TPGYT enrichment solution and cooked meet medium of the feces of 3 patients, respectively. Clostridium botulinum B was identified from the feces of 3 infants after culture, isolation and purification. Conclusions: Combined with typical clinical manifestations including acute flaccid paralysis, cranial nerve involvement symptoms and difficult defecation examination, infant botulism can be clinically diagnosed. The detection of fecal botulinum toxin and the culture and isolation of Clostridium botulinum are helpful for the diagnosis.

Neuromuscular Junction Disorders and Floppy Infant Syndrome: A Comprehensive Review.

Floppy infant syndrome, also sometimes referred to as rag-doll syndrome, is characterized by hypotonia that could present as either peripheral hypotonia or central. Depending on the origin of hypotonia, the infant will present with different symptoms that ultimately have the characteristic feature of hypotonia. The clinical examination is crucial in diagnosing floppy infant syndrome in the neonate period, but the most critical factor is investigating and diagnosing the underlying cause of hypotonia. Regardless of whether the underlying cause of hypotonia is peripheral or central in origin, the presentation of floppy infant syndrome focuses on observing for the presence or absence of specific signs such as ‘frog-leg’ posture, significant head lag on traction or pull-to-sit maneuver, or the feeling of ‘slipping through the hands’ when the infant is held under the arms. Infantile botulism, transient neonatal myasthenia gravis, congenital myasthenia gravis, hypermagnesemia, and aminoglycoside toxicity are all neuromuscular junction disorders that are considered to be a differential diagnosis of floppy infant syndrome. These neuromuscular junction disorders ultimately impact the presence of acetylcholine within the neuromuscular junction. While some of these disorders may impact the acetylcholine receptors, others may cause a depletion within the end-plate anticholinesterase enzyme. A deficiency within the anticholinesterase deficiency may cause desensitization to acetylcholine, which could also cause present with floppy infant syndrome as well. Depending on the underlying causative disorder leading to the presence of floppy infant syndrome, the treatment will vary considerably. Treatment of the underlying causative syndrome resulting in the presentation of floppy infant syndrome deals with the symptoms of hypotonia, and as a result, the decreased muscle tone, diminished tendon reflexes, any feeding or respiratory difficulties diminish.

Classic Neuroimaging Features in a Case of Congenital Muscular Dystrophy [Fukuyama Variant] – a Rare Cause of Infantile Hypotonia in an Indian Male

The aetiological diagnosis in an infant with hypotonia is a challenging task for a clinician due to variable and long list of differentials [1]. It could be due to an insult within the central nervous system (CNS) or less commonly result from a peripheral defect at neuro-muscular level and other miscellaneous causes (rickets, hypothyroidism) [2]. Most commonly it is a central hypotonia where the muscular weakness is absent or not profound. In Indian scenario, it is mostly idiopathic central hypotonia, followed by HIE (cerebral palsy) [2]. In cases of cerebral palsy, neuro-imaging reveals the severity of affliction. The peripheral aetiologies like congenital myopathies, congenital myasthenia, infantile botulism etc. are rather rare occurrences. Infants with congenital muscular dystrophy have muscular dystrophy, central neural affliction and involvement of multiple systems (skeletal, cardiovascular, respiratory, ocular etc.). It is a rare disease with studies in Italian population showing point prevalence of 0.563 per 100,000 total population [3]. Unlike HIE, the affliction in CMD is multi-system and it is an inherited disease with variable penetration, the distinction between the two is important to a clinician for the management. CMDs generally have early fatal outcome. So, early diagnosis is important for prognostication, supportive treatment and genetic counselling. The neuro-imaging findings of CMD clearly stand out from the rest aetiologies and can guide a clinician to go in early , for an invasive test like muscle biopsy which is the gold standard diagnostic test .

Abstract P-506: INFANTILE BOTULISM IN SINGAPORE

Aims & Objectives: Infantile botulism is rare worldwide. We present the first case in Singapore. Methods A 9 month old developmentally normal and appropriately immunized boy was admitted to our hospital with a 3 day history of severe lethargy, poor suck and constipation. He was unable to sit without support or lift his head. Examination revealed generalized hypotonia, marked head lag, ptosis and drooling. There was symmetrical hypo-reflexia of all four limbs with minimal anti-gravity movements. He developed respiratory insufficiency from neuromuscular weakness on day 2 of hospitalisation. The following tests did not reveal a diagnosis; neuroimaging of brain, cerebrospinal fluid studies, electrolytes, thyroid function, muscle enzymes, metabolic and toxicology work up and antibody studies for myasthenia gravis. Stool studies were later confirmed positive for botulinum toxin A. He required non invasive ventilation, meticulous nursing care and chest physiotherapy in the intensive care unit. 50mg/kg of Botulinism Immune Globulin (BabyBIG®) procured from the Infant Botulism Treatment and Prevention Program California Department of Public Health was administered on day 16 of hospitalisation. Results Infantile botulism occurs when ingested spores of Clostridium botulinum germinate in the infant’s colon and release neurotoxins that prevent the release of acetylcholine at the synaptic cleft of motor neurons resulting in paralysis. Treatment is supportive but BabyBIG®, a human derived immunoglobulin against botulinum toxin has been shown to reduce length of hospital stay. Conclusions Botulism should be considered in any infant presenting with acute symmetrical flaccid paralysis and treated promptly with BabyBIG®.

Trends in Outcomes and Hospitalization Charges of Infant Botulism in the United States: A Comparative Analysis Between Kids' Inpatient Database and National Inpatient Sample

BackgroundNew therapeutic strategies, including immune globulin intravenous, have emerged in the past two decades for the management of botulism. However, impact on outcomes and hospitalization charges among infants (aged ≤1 year) with botulism in the United States is unknown. MethodsWe analyzed the Kids' Inpatient Database (KID) and National Inpatient Sample (NIS) for in-hospital outcomes and charges for infant botulism cases from 1997 to 2009. Demographics, discharge status, mortality, length of stay, and hospitalization charges were reported from the two databases and compared. ResultsBetween 1997 and 2009, 504 infant hospitalizations were captured in KID’, and 340 hospitalizations from NIS, for comparable years. A significant decrease was observed in mean length of stay for ’KID (P < 0.01); a similar decrease was observed for the NIS. The majority of patients were discharged to home. Despite an initial decrease after 1997, an increasing trend was observed for ’KID/NIS mean hospital charges from 2000 to 2009 (from $57,659/$56,309 to $143,171/$106,378; P < 0.001/P < 0.001). A linear increasing trend was evident when examining mean daily hospitalization charges for both databases. In conducting a subgroup analysis of the ’KID database, the youngest patients with infantile botulism (≤1.9 months) displayed the highest average number of procedures during their hospitalization (P < .001) and the highest rate of mechanical ventilation (P < .001), compared with their older counterparts. ConclusionInfant botulism cases have demonstrated a significant increase in hospitalization charges over the years despite reduced length of stay. Additionally, there were significantly higher daily adjusted hospital charges and an increased rate of routine discharges for immune globulin intravenous–treated patients. More controlled studies are needed to define the criteria for cost-effective use of intravenous immune globulin in the population with infant botulism.

PP10.13 – 2944: An unusual case of infantile botulism: Clostridium botulinum Bf (C. botulinum Bf) infection in UK

Objective We discuss a rare case of infantile Clostridium botulinum Bf infection, the associated diagnostic pitfalls and paediatric-specific management strategies. Methods A retrospective review of the patient's medical notes was undertaken. Results A previously well, weaned infant presented at 6 months with two days of reduced feeding and salivary pooling. There was no history of honey exposure. Initially, gag-reflex was absent, with preservation of other cranial nerves. Antigravity movements of limbs, reflexes, and respiratory effort, were maintained. Symptoms progressed within a week to reveal hypotonia, absent antigravity movements, with preservation of reflexes, within upper limbs. Lower limb examination was normal. Eye-movements were preserved, however pupils were slowly and asymmetrically reactive. Respiratory decompensation led to intubation and subsequent tracheostomy. Lumbar puncture, metabolic screens, and MRI brain were normal. Neurophysiology showed diffuse, severely attenuated motor nerve amplitude consistent with axonal variant Guillian-Barre Syndrome, thus IVIG was commenced. Due to the atypical presentation of descending paralysis, infantile botulism was considered. Stool specimens were positive for C. botulinum Bf. Botulinum antitoxin therapy (Baby BIG) was initiated, leading to rapid recovery. Public health investigation did not show a reservoir for this infection. Conclusion C. botulinum Bf is rare, with eight previous international cases. Our case is only the second recent presentation in UK. The case confirms that dual C.botulinum neurotoxins should be considered in diagnoses for infants presenting with isolated cranial nerve palsies, including bulbar signs. Furthermore, rapidly descending paralysis involving pupillary mydriasis and poorly reactive pupils are consistent features of infantile botulism. Additionally this case highlights that neurophysiology interpretation in infants is unreliable, with clinical suspicion of botulism guiding investigation. Treatment for infantile botulism centres on Baby BIG therapy. This immunoglobulin is specifically selected for high titres of type A and B “neutralising antibodies”, which combat the most common strains of infantile botulism.

Trends in Outcome and Hospitalization Charges of Infantile Botulism in the United States: A Comparative Analysis between Kids' Inpatient Database (KID) and National Inpatient Sample (NIS) (P4.115)

Trends in Outcome and Hospitalization Charges of Infantile Botulism in the United States: A Comparative Analysis between Kids' Inpatient Database (KID) and National Inpatient Sample (NIS) (P4.115)

“Infantile botulism: Forgotten etiology of acute hypotonia in infancy”

In Asian countries infantile botulism is a rare and often under diagnosed disease. With the intent to spread the awareness of this potentially life threatening disease we report two confirmed cases of infantile botulism and describe the way we could make our final diagnosis. Additionally since the treatment of choice of infantile botulism is Baby BIG and it is not available in our country and because of financial problems we could not afford it, we treated our patients with equine botulinum antitoxin (EqBA). We report here the effectiveness and safety of equine botulinum antitoxin (EqBA) as an alternative treatment of infantile botulism.

Muscle Disease

On the basis of strong research evidence, Duchenne muscular dystrophy (DMD), the most common severe childhood form of muscular dystrophy, is an X-linked recessive disorder caused by out-of-frame mutations of the dystrophin gene. Thus, it is classified asa dystrophinopathy. The disease onset is before age 5 years. Patients with DMD present with progressive symmetrical limb-girdle muscle weakness and become wheelchair dependent after age 12 years. (2)(3). On the basis of some research evidence,cardiomyopathy and congestive heart failure are usually seen in the late teens in patients with DMD. Progressive scoliosis and respiratory in sufficiency often develop once wheelchair dependency occurs. Respiratory failure and cardiomyopathy are common causes of death, and few survive beyond the third decade of life. (2)(3)(4)(5)(6)(7). On the basis of some research evidence, prednisone at 0.75 mg/kg daily (maximum dose, 40 mg/d) or deflazacort at 0.9 mg/kg daily (maximum dose, 39 mg/d), a derivative of prednisolone (not available in the United States), as a single morning dose is recommended for DMD patients older than 5 years, which may prolong independent walking from a few months to 2 years. (2)(3)(16)(17). Based on some research evidence, treatment with angiotensin-converting enzyme inhibitors, b-blockers, and diuretics has been reported to be beneficial in DMD patients with cardiac abnormalities. (2)(3)(5)(18). Based on expert opinion, children with muscle weakness and increased serum creatine kinase levels may be associated with either genetic or acquired muscle disorders (Tables 1 and 3). (14)(15)

Case Scenario: Hypotonia in Infancy

NFANTS with hypotonia of unknown etiology pose a unique challenge as many of the potential diagnoses have major, often conflicting, anesthetic management implications. The differential diagnosis of hypotonia is long and includes possibilities such as Duchenne muscular dystrophy, central core disease, and multiminicore disease. Intravenous anesthetic techniques are recommended because hyperkalemia or malignant hyperthermia is associated with the use of volatile anesthetics. However, the differential diagnosis of infants with hypotonia also includes mitochondrial disorders. In children with mitochondrial disorders, an intravenous anesthetic technique that includes propofol could lead to metabolic decompensation because propofol alters mitochondrial electron transfer. This dilemma is often encountered when hypotonic infants require anesthesia for diagnostic tests such as magnetic resonance imaging and muscle biopsies for definitive diagnosis.

Infantile Botulism: A Case Report and Review

BackgroundInfantile botulism is the result of ingestion of Clostridium botulinum spores, and is the most common form of infection with botulism in the United States. Ninety percent of cases occur in infants <6 months old. The infants typically present with vague symptoms such as hypotonia and poor feeding. This article reports an infant with confirmed infantile botulism that presented to the Emergency Department (ED) with complaints of decreased feeding and absence of bowel movements for >1 week. ObjectivesReview a case of infantile botulism, its diagnosis, and treatment. Case ReportA 4-month-old healthy Caucasian male presented to the ED with a 6-day history of decreased feeding after referral from the pediatrician. He had not had a bowel movement for 9 days, and his parents were also concerned about increasing weakness, as he was no longer able to hold his head up on his own. In the ED, he was minimally interactive. His vital signs were within normal limits, and he had hypoactive bowel sounds and decreased tone throughout. He was admitted to the Children's Hospital and eventually transferred to the Pediatric Intensive Care Unit requiring intubation and mechanical ventilation. The botulism immunoglobulin was administered, and a diagnosis was confirmed with positive botulinum toxin in the stool samples. Full recovery was made by the infant. ConclusionAwareness of the symptoms of botulism and a high degree of clinical suspicion is needed to make a prompt diagnosis.

How Long Can an Endotracheal Tube be Left in Place at the End-of-life?

1.Outline the viability of leaving an endotracheal tube in place for months in a pediatric palliative care patient.2.Appraise the issues surrounding current “standards of care” for how long an endotracheal tube can be left in place.3.Discuss the ethical issues surrounding decision-making and conflict between treating teams and parents. I. Background. When children intubated for an acute illness are subsequently found to have a life-limiting condition, a frequent course is to withdraw life-support technologies. For personal, cultural, or religious reasons some families may not agree with extubation but may otherwise accept nonescalation of treatment and comfort measures. When extubation is not an option, there is little information in the literature on how long an endotracheal tube can remain unchanged in situ. This case describes a 20-month-old boy with a prolonged (8 months) intubation with the same endotracheal tube in place without any mechanical or technical complications. II. Case Description. Presentation was at 8 months of age with a 2 1/2-month history of decreased tone, arrest of milestone attainment, and failure to thrive. He was initially diagnosed with infantile botulism due to a stool culture positive for viable C botulinum Type A. Subsequently, genetic testing diagnosed Thimidine Kinase 2 deficiency, a mitochondrial depletion syndrome. At the time of diagnosis, he had been ventilated via an endotracheal tube for 3 months. Due to the severity of disease and ultimately fatal prognosis the treating team felt that prolonged ventilation was not in his best interests. The parents, however, refused extubation on cultural and religious grounds, but agreed to a nonescalation of life-support technologies with a focus on comfort and quality of life. He was transferred intubated and ventilated to the medical ward where he was cared for by his parents for 3 months. After 8 months of intubation, his parents asked for him to be extubated and allowed to die. III. Conclusion. Although not a first choice, when needed, an endotracheal tube can be kept in place longer than is commonly thought without deterioration of the tube. This case also highlights several ethical issues surrounding decision-making and conflict between parents and the treating team. Physical Aspects of Care

Infantile Botulism: Clinical Manifestations, Treatment, and the Role of the Nurse Practitioner

Clostridium botulinum, a toxin most commonly found in soil and honey products, is the main source of infantile botulism. Botulinum toxin is one of the most deadly substances that can attack the neuromuscular system, resulting in a wide range of sequelae. A high index of suspicion is crucial in promptly diagnosing and treating infantile botulism because of the potential for abrupt progression into respiratory failure. This article discusses the epidemiology, pathophysiology, and clinical manifestations of infantile botulism, as well as the valuable role the nurse practitioner plays in preventing this disease within the pediatric population.