How Common is Infantile Botulism?

Patient Presentation
A 7-day-old term female infant presented to the emergency room with several hours of decreased energy and poor feeding.
She is noted to be grey in color with poor perfusion, decreased tone, weak cry, and no respiratory distress but poor effort.
Resuscitation was begun. She had intravenous catheters placed with fluid boluses given and ampicillin, gentimicin and acyclovir begun.
The pertinent physical exam showed a heart rate of 100, blood pressure of 52/28, respiratory rate of 40, and a pulse oximetery was not registering.
Her pupils were 7-8 mm and not reactive to light. Her fontanelle was normal. There was no rash, or bruises. Capillary refill was 3 seconds.
Cardiac examination revealed normal S1, S2 and no murmur. Abdomen was soft with no obvious organomegaly.
Neurological examination revealed decreased tone, withdrawal to pain, and her peripheral reflexes were difficult to elicit.
The laboratory evaluation showed a first blood gas with a pH = 7.14, CO2 = 56, O2 = 80. Blood cultures were drawn. She had a normal complete blood count, andelectrolytes including calcium, magnesium and phosphorus.
The patient was then intubated and transferred to a regional children’s hospital.
Additional work-up included a normal cerebral spinal fluid, and a normal computerized tomography of the head and chest radiograph.
An electrocardiogram and echocardiogram were also normal.
She had a normal electroencephalogram but an abnormal electromyelogram which was consistent with a peripheral versus a polymyopathic neuropathy. She did not respond to a tensilon test.
The additional past medical history revealed normal labor and delivery at home for a G1P1 mother with some prenatal care.
The patient was seen by a nurse midwife at delivery, day 1 and day 2 of life and was doing well. Her neonatal screen was normal. She did not receive Hepatitis B, Vitamin K or gonococcal conjunctivitis prophylaxis.
She was breastfeeding well until several hours before coming to the emergency room.
The parents denied honey ingestion and the family history revealed no neuropathy or neurological diseases.
The diagnosis of presumed infantile botulism was made and the infant was given botulinum antitoxin.
At day 14, Clostridium botulinum type A was recovered from her stool and she remained on life support. She was discharged home after a few weeks.

Discussion
A newborn infant presenting with acute deterioration causes health care providers to quickly go through a long differential diagnosis covering all organ systems as they attempt to resuscitate and stabilize the infant.
Causes are numerous and the physicians in this case entertained some of the following in their differential:

  • Sepsis/Infection – Group B streptococcal infection, Herpes simplex, necrotizing enterocolitis, etc.
  • Congenital heart disease
  • Hematologic – bleeding secondary to sepsis, Vitamin K deficiency, etc.
  • Metabolic abnormalities – endocrinopathies, nephropathies, inborn error of metabolism, etc.
  • Neurologic – seizures, congenital or acquired neuropathies, etc.
  • Trauma – inflicted or incidental
  • Toxins/Drugs

Like most patients, the cause for this patient’s acute deterioration not immediately recognized.
This patient was resuscitated, stabilized, transported, and then was sustained with ventilatory support and other life sustaining measures for the most common problems such as sepsis and congenital abnormalities.
Concurrently, her evaluation was begun and based on those results the differential was narrowed down over time and specific treatment initiated.

Learning Point
Infantile botulism is not a common problem in the United States generally because of improved obstetrical care, general personal hygiene and education about cultural practices which could be harmful to the infant such as giving honey or placing soil or dung on the umbilical cord.

Botulism is classified into four categories – foodborne, infantile, wound and undetermined. Symptoms (except for infants) generally occur within hours and evolve over days. It is a decending, symmetric, flaccid paralysis, with cranial nerves palsies being the most common complication.
Infantile botulism occurs most often in children < 6 months and can be preceeded by constipation, poor feeding, weak cry, ocular nerve palsies, general weakness and hypotonia.
Human botulism is associated with neurotoxins A, B, E and F. Infantile botulism is almost always associated with types A and B.
Incubation in infantile botulism is 3-30 days from exposure.
About 100 cases occur annually of infantile botulism, after ingested spores of Clostridium botulinum are ingested, germinate, replicate and produce toxin in the gut. The toxin produces the paralysis.
In infantile botulism the source of the spores sometimes cannot be identified and possibly is airborne dust or soil. Honey often is not certified as free of spores and should be avoided especially in the first year of life.
Corn syrups are manufactured under sterile conditions, but are not packaged under aseptic conditions and do not receive sterilization at the end of packaging and therefore the manufacturers cannot ensure that the product is free of spotes.

Antibiotics are avoided if possible because Clostridium lysis may increase the amount of toxin. Botulinum antitoxin (human-derived immunoglobulin) is available and in studies decreases the hospitalization length. Overall mortality is ~15%.

Questions for Further Discussion
1. Clostridium botulinum is a Class A bioterrorism agent. What are the other Class A agents?
2. How is Botox® (Botulinum toxin Type A) made safe for human use?
3. How are the presentations of foodborne and wound botulism different from the infantile form?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MEDLINEplus for this topic: Botulism

To view current news articles on this topic check Google News.

American Academy of Pediatrics. Botulism and Infant Botulism, In Pickering LD, ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2003;243-245.

FrattarrelliDAC, Abdel- Haq NM. Botulism. eMedicine.
Available from the Internet at http://www.emedicine.com/ped/topic273.htm (rev. 7/9/2004, cited 6/1/06).

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competency performed.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.

  • Systems Based Practice
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    June 19, 2006

  • What are the Definitions for Different Topical Substances?

    Patient Presentation
    A 7 month-old male came to clinic with dry itchy skin that has been worsening over the past week.
    His mother says that he has had dry skin before but not this bad and she has never put anything on it.
    She has noticed that it is worse on his arms, legs and face but he has dry skin in general.
    He has been itching more and she noticed that his elbows, knees and cheeks appear more reddish.
    The family history shows that mother has dry skin, with ‘bumps’ on her upper, outer arms and thighs.
    The pertinent physical exam reveals a healthy male with generalized dry skin that is mainly papular with areas of lichenification and hypopigmentation over his body.
    He has areas of excoriation in the flexural areas of the elbows and knees, on his cheeks and behind his ears.
    These areas are also more pink-red in color than the surrounding skin. He has no areas that appear infected.
    The diagnosis of atopic dermatitis was made. His mother was educated as to the natural history of the disease.
    She was told that she could bathe him frequently with a mild ‘beauty bar” such as Dove® or a non-soap alternative such as Cetaphil®.
    She was told to pat him dry and to use “thick” emollients such as petrolatum to protect his skin. She was told to use to use the emollients every couple of hours to keep his skin moist.
    She was also told to use thinner emollients, such as a cream or lotion, if he was going to be in a warm place so he wouldn’t sweat under the emollients and irritate his skin.
    She was also instructed on the signs of infection, and the importance of applying sunscreen too.

    Discussion
    Atopic dermatitis or eczema is a common dermatological skin problem which characteristically is a pruritic, papular eruption with erythema.
    Sometimes atopic dermatitis is described as the “itch that rashes.”Rubbing and scratching can lead to excoriation and, over time lichenification.
    There can also be secondary infections or changes to the skin pigmentation (hyper- or hypo-) in affected areas.
    Emollients for skin rehydration are a mainstay of treatment. Topical steroids are commonly used to decrease inflammation in affected areas.
    Immunosuppressants such as tacrolimus are also used in some cases.

    Learning Point
    The definitions of common topical substances are:

    • Ointments- Its base is an oil (a hydrocarbon). It may have the ability to have a water incorporated into it making a water-in-oil emulsion. One example is Aquaphilic®.
    • Creams – Its base is water. It may have the ability to incorporate an oil into it making an oil-in-water emulsion. These are easier to wash from the skin and therefore people may like them better than ointments.
    • Gels – Its base is water and fine particles are more or less permanently suspended in the liquid. The number of particles is so great to render the liquid into a semi-solid state.
    • Lotions – Its base is water with fine particles more or less permanently suspended in the liquid. The particle numbers are so few that the liquid remains a liquid.

    Each of these topical substances has different properties which affect how it acts on the skin.
    How long a topical substance remains on the skin depends on many factors including the substance applied, amount applied, the location, rubbing of the location, occlusion of the area, contact with other substances such as water, sweat, etc.
    Although I cannot reference a specific amount of time, I have been taught that the approximate time topicals stay on the skin is:

    • Ointments- 2 hours
    • Creams – 1 hour
    • Gels/Lotions – 20-30 minutes

    One gram of a topical ointment or cream covers approximately 10 x 10 centimeters of skin. A 30-60 gram tube should cover the entire skin of an adult once.

    Questions for Further Discussion
    1. For liquids, what is the difference between a suspension and a solution?
    2. What are the indications for using immunosuppressants for atopic dermatitis?
    3. What is keratosis pilaris?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MEDLINEplus for this topic: Eczema

    To view current news articles on this topic check Google News.

    Stedman’s Medical Dictionary. 24th Edit. Williams &Wilkins. Baltimore, MD. 1982.

    Berkowitz CD. Pediatrics A Primary Care Approach. W.B. Saunders Co. Philadelphia, PA. 1996:392-396.

    Robertson J, Shilkofski N. The Harriet Lane Handbook. 17th. Edit. Mosby Publications: St. Louis. 2005:977,1031.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.

    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement

    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    June 12, 2006

  • What Can I Do About All These Mosquito Bites?

    Patient Presentation
    A 6-year-old female came to the office with mosquito bites that her mother was worried were infected.
    The child was outside in the early evening two days ago and was bitten several times on her legs, arms and face. Since then she has been very itchy and has scratched the bites.
    She has taken some diphenhydramine with some decrease in itching, but her mother noted that three of the bites on her lower legs look larger, redder and have a yellow discharge on top of the scratches since this morning.
    The past medical history showed that the girl had an exaggerated response to mosquito bites with large wheal reactions locally. She has not had allergic-type responses to other insects, bees or wasps according to her mother.
    She usually responds well to diphenhydramine.
    The pertinent physical exam showed a healthy female with no fever and obvious insect bites in the noted locations.
    They had been scratched and two lesions on the right leg and one on the left had 1-1.5 cm raised, red circular lesions with a diffuse border. The center had a yellowish discharge.
    She had no lymphatic streaking coming from the lesions. The rest of her physical examination was normal.
    The diagnosis of insect bites with secondary impetigo was made. She was begun on mupirocin ointment for the impetigo and hydroxyzine for the itching. She and her mother were told to return to clinic if any fever, increased swelling or streaking of the lesions occurred.
    They were also instructed on using insect repellents with less than 10% DEET in them and how use them properly. They were also instructed to wear long-sleeved clothing and to avoid early morning and early evening times outside.

    Discussion
    Impetigo is a common infection and is often caused by Group A Streptococcus including Streptococcus progenies. It can also be caused by Staphylococcus auras.
    It occurs at the site of skin breaks such as insect bites or other wounds, and is highly contagious. In healthy children and adults it can usually be treated with topical mupirocin which also may be helpful in limiting person-to-person spread.
    Oral treatment is good when there are multiple lesions in dispersed places on a single individual, or multiple family members, child care groups, or athletic teams where several individuals are affected.

    Learning Point
    Mosquitos go through four life stages – eggs which hatch when exposed to water, larvae which live in the water, pupa which occurs just before becoming an adult, and adult which emerges from the pupa and flies out of the water.
    The adult then feeds on mammals including humans. Many diseases are transmitted by mosquitos including Yellow Fever, Malaria, Dengue Fever, West Nile virus and many others.

    Insect repellent with active ingredients which have been registered with the Environmental Protection Agency (EPA) have been evaluated for effectiveness and potential side effects to humans and the environment.
    Those approved are not expected to cause unreasonable adverse effects when used according to label instructions.

    Two active ingredients with a higher-degree of efficacy and longer-lasting protection are DEET (N,N-diethyl-m-toluamide) and Picaridin (KBR 3023).
    Oil of Lemon Eucalyptus (p-methane 3,8-diol [PMD]) which is a plant based repellent, is found to have similar protection to low concentrations of DEET, but it is not labeled for children under 3 years of age.
    Permethrin is also a registered active ingredient commonly used on clothing, shoes, and camping gear. It retains its effects even after repeated laundering. Permethrin should not be used directly on the skin.

    General recommendations for repellents include:

    • Repellents should be applied before going outside even for brief time periods.
    • Repellents should be used on clothing as much as possible with the lowest concentration that is effective for the conditions. More than 50% DEET does not offer additional protection.

      For children, the highest concentration recommended is ~10% DEET.

    • Repellents should be used on (not under) clothing and on intact skin (never on cuts, wounds, irritated skin or mucus membranes). It should not be applied to children’s hands as they put them in their mouths.
    • Spray repellents should be sprayed onto hands and applied to appropriate skin areas. It should never be sprayed directly toward the face. Hands should be washed after applying the repellent. Children should not apply it themselves. An adult should do this for them.
    • A thin film of repellent is generally enough. Saturation is not required. A second thin film can be applied again if biting continues.
    • Reapply the repellent after several hours (>3-4 hours or less depending on the label instructions) and after water exposure (i.e. swimming, sweating, etc.).
    • Wash skin with soap and water after coming inside. Launder clothing also. This is important so the repellent does not build up.
    • Separate sunscreen may be used with DEET products when used according to the instructions. No data is currently available regarding the use of other active ingredients in combinations with separate sunscreen.

    The EPA does not recommend other precautions for using registered repellents on pregnant or lactating women or for children.
    The American Academy of Pediatrics states “Insect repellents containing DEET with a concentration of 10% appear to be as safe as products with a concentration of 30% when used according to the directions on the product labels.” The AAP also recommends not using DEET until older than 2 months of age.
    The AAP does not have a recommendation for Picardy or Oil of Lemon Eucalyptus use.

    Other recommendations include:

    • Keep the repellents out of children’s reach.
    • Wear clothing that covers as much of the body as possible. Use permethrin repellents on clothing only (and not on skin).
    • Use mosquito netting over the top of infants in carriers, strollers, etc.
    • Don’t let water stand around such as kiddie wading pools, buckets, bird baths, drainage ditches, etc. These represent a mosquito breeding area and also a drowning risk for children.
    • Contact the local government regarding other environmental controls for swamps, marshes, ponds, lakes, etc.

    Questions for Further Discussion
    1. What are the symptoms of West Nile Virus?
    2. What other insects do mosquito repellents also repel?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MEDLINEplus for this topic: Insect Bites and Stings
    and at Common Questions, Quick Answers for this topic: Mosquito Bites

    To view current news articles on this topic check Google News.

    American Academy of Pediatrics. Group A Streptococcal Infections In Pickering LD, Ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2003;573-584.

    American Academy of Pediatrics, Committee on Environmental Health. Follow Safety Precautions When Using DEET on Children.
    AAP News, June 2003. Available from the Internet at: http://www.aap.org/family/wnv-jun03.ht.

    Centers for Disease Control. Mosquito-borne Diseases. Available from the Internet at:
    http://www.cdc.gov/ncidod/diseases/list_mosquitoborne.htm, (rev. 4/23/2004, cited 5/22/06)

    Centers for Disease Control. What you Need to Know About Mosquito Repellent. Available from the Internet at: http://www.cdc.gov/ncidod/dvbid/westnile/mosquitorepellent.htm (cited 8/5/05,cited 5/22/06)

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.

    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    June 5, 2006

  • What Causes Color Blindness?

    Patient Presentation
    A 9-month-old female came to clinic for her health maintenance visit. As part of the interview the physician asked if the parents had any concerns about her hearing or vision.
    The father said no, but that he had a family history of color blindness and was wondering if she needed to be tested for it.
    The family history revealed that the father’s brother and maternal uncle both were red-green color blind.
    The pertinent physical exam revealed a developmentally normal female with growth parameters in the 10-50%.
    Her visual acuity was grossly normal by fixing and following objects appropriately, and in viewing a book in her lap.
    She had normal extra ocular movements bilaterally and her optic disc margins were sharp and the blood vessels appeared normal.
    The diagnosis of a healthy female was made and after reviewing a PUBMED literature search, the physician told the parents that it was most likely that the daughter did not have red-green color blindness because it was an X-linked genetic disease and the father himself usually would have to be affected.
    The parents were also told that there were potentially other inherited forms as well but that testing at this time was not possible and that as she grew older the testing would be more reliable.
    The parents were told to treat her like a normal child and to bring any concerns about her vision including possible color problems back to the physician’s attention.

    Discussion
    Color blindness or impaired color vision is common. Red-green color impairments affect 6-10% of males and 0.4 – 0.7% of females.

    Screening is often done using Ishihara charts which are pictures of various size spots with hidden wavy lines or numbers. The lines or numbers are read differently by those affected or may not be able to be read at all.
    They can be used for children as young as 4 years. The wavy line charts have a higher incidence of errors, but are also more commonly used with younger children. Five errors or more on the first 13 Ishihara plates necessitates referral for additional testing.
    Sometimes matching colors can be used in young children but it is not very reliable.

    Colors are used in all types of teaching and therefore a child or adolescent may have some problems with the instruction methods used from pre-school through secondary education.
    The impact for an individual child varies. Some studies have shown that children with other learning difficulties may have a higher or lower rate of impaired color vision.
    Even in the field of art, there are several well known artists who were affected including Carriere, Léger, Whistler and possibly Constable and Mondrian.

    Impaired color vision may have an affect on the type of career opportunities available to these children. In the United Kingdom, people are advised against certain occupations including electrical work, electronics, fabric industry, navigators, pilots, train drivers, and certain jobs in the armed forces and police.
    The British Paediatric Association recommends screening at age 5, and at 11-12 years or up to 14 years. The American Academy of Pediatrics does not have a recommendation regarding color vision screening.

    Learning Point
    There are 3 primary color cones in the eye – red, blue and green. When gradiations of the different cones are activated, different colors are seen. For example, orange light mainly stimulates red cones and stimulates some green cones.
    Depending on which cone system is defective, these are termed protanopia (red), deuteranopia (blue) and tritanopia (green). Usually one of the cone systems is affected, but there can also be a diminished response by the cones to the color stimuli.

    Impaired color vision is mainly caused by an X-linked recessive inheritance pattern. Women (8%) are carriers and males are mainly affected. There can also be complex forms where mosaicism can be found and also an incompletely autosomal dominant form.

    Questions for Further Discussion
    1. How common is blindness in children?
    2. What different methods are available for screening for visual defects in children of different ages?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MEDLINEplus for these topics: Eye Diseases and Vision Impairment and Blindness

    To view current news articles on this topic check Google News.

    Gordon M. Colour Blindness. Public Health. 1998;112:81-84.

    Committee on Practice and Ambulatory Medicine, Section on Ophthalmology
    Eye Examination and Vision Screening in Infants, Children, and Young Adults.
    Pediatrics. 1996;98:153-157. Available from the Internet at http://aappolicy.aappublications.org/cgi/reprint/pediatrics;98/1/153 (rev. 1996, cited May 11, 2006).

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.

    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.

  • Systems Based Practice

    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    May 29, 2006