Summer Break

PediatricEducation.org is taking a summer break. The next case will be published in on August 27th. In the meantime, please take a look at the different Differential Diagnoses, Symptom and Disease listed at the top of the page.

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What Causes School Failure?

Patient Presentation
A 10-year-old female came to clinic for her health supervision evaluation. She was having problems in school (getting D’s and F’s with an occasional C) and was struggling in all her subjects but especially reading. She was particularly vocal that she did not like school and didn’t understand why she had to go to school. “I don’t really want to do anything when I grow up so why should I go to school,” she said. Her mother was not concerned about her learning and thought that she was doing “just fine. She’s like the rest of us.” The family had moved multiple times within one location and between locations to live with relatives and friends while the mother tried to maintain employment. The past medical history was positive for mild persistent asthma that was not well-controlled and she did not have any of her medications available. The family history was positive for asthma, diabetes, attention problems and school problems. Her mother and maternal aunt had dropped out of school and her father had struggled but finished high school.

The pertinent physical exam showed a pleasant but vocal school-ager. Her vital signs were normal and her growth parameters were at the 25-50% but there were no previous visits for comparison. She had mild rhinorrhea and her lungs had some mild wheezing. The rest of her examination was normal.

The diagnosis of of a child with uncontrolled, mild persistent asthma who was struggling in school, and with social problems of home insecurity and poverty was made. The pediatrician worked with the clinic’s social worker to help secure asthma medication for the family to go home with and refills at the local pharmacy. The social worker also offered to help the mother to work with the school but the mother refused. She did accept help in finding more permanent housing and child care for other children though. The family did not followup for their asthma appointment 2 months later. The social worker contacted the cellphone and the mother said they had moved again out of the area.

Discussion
Literacy is traditionally meant as the ability to read and write. The modern term’s meaning has been expanded to include the ability to use language, numbers, images, computers, and other basic means to understand, communicate, gain useful knowledge, solve mathematical problems and use the dominant symbol systems of a culture.”

The earliest written communication was in 3500-3000 BCE, with the earliest alphabet being from 1200-750 BCE. Although the percentage of the world’s adult literacy rate is increasing each decade by ~5%, “…from 55.7 per cent in 1950 to 86.2 per cent in 2015,…” the total number of adults who are illiterate is increasing because of increased population. Universal, compulsory education is viewed as the mainstay of developed and developing nations to building a literate population. However there are many reasons that children, teenagers and adults may not succeed in a school including individual and family issues, the general social and community environment, and specific school issues.

School failure is defined as grade retention (i.e. repeating a grade, 10% of Kindergarten-8th grade students), expulsion (0.2% of students) and dropping out of school (i.e. 16-24 year old not enrolled and do not have a general equivalency degree, 9% which is a decline but total number of students is increasing because of increased enrollment). Even students that graduate from high school may not be sufficiently literate in the broader sense. Some studies cite, 80% of high school students graduating but only50% are ready for the world and only 50% are proficient in math and reading.

Students with learning disabilities (LD) make up ~10% of enrolled students (about the same % as those who have asthma). Reading disability accounts for ~6.5% which is the most common form of LD. LD is often thought to cause relatively low morbidity but students with LDs often struggle in school and have school failure. Early signs may be students struggling and putting in more time than peers or acting out in some way. Students may be completing homework and performing academically appropriately, but to do this may be expending a great deal of time and mental effort to keep up. Other signs of LD include class clown behavior, school anxiety, frequent failing grades, frequent absences or detention/suspension, and aggressive and bullying behaviors.
Co-morbidities are common with students with LD having more than 1 LD, attention or other problem. Students with LDs may also be intelligently gifted too and are sometimes called “twice-exceptional.”

Students who are struggling or failing in school should be appropriately evaluated which may include an assessment of their academic problems, behavior and mental health evaluation, general intelligence testing, social evaluation, hearing and vision testing and other testing as appropriate. Students may be evaluated by a “RTI or response to intervention” evaluation with students having specific educational interventions started with them and then the outcome of the intervention is part of the student’s evaluation. Students may qualify for special education through an Individualized Education Plan or a 504 plan.

A review of special education can be found here.
A review of how gifted children present can be found here.
A review of risk factors for grade retention can be found here.
A review of causes of school absenteeism can be found here.
A review of causes of attentional problems can be found here.

Learning Point
Causes of school failure include:

  • Child and Family
    • Absenteeism
    • Attention problem
    • Cognitive problem
    • English as a second language
    • Health issues – acute or chronic
      • Anemia
      • Allergies
      • Asthma
      • Autism
      • Brain injury – traumatic, infectious or other
      • Crohn’s disease
      • Cystic fibrosis
      • Genetics – Fragile X
      • Hypo- or hyperthyroid
      • Obstructive sleep apnea
      • Prematurity
        • In-utero drug exposure
      • Phenylketonuria
      • Seizures
    • Home environment
      • Low level of family support for student/attitudes toward school
        • Inadequate study space/time
        • Not interested in the classes
        • Don’t have specific study skills/ Behind and can’t catch up
        • Perceived inadequate rewards for effort expended
      • Competing priorities / excessive extramural activities
      • Divorce/separation/conflict
      • Neglect/abuse/violence
    • Learning disabilities
      • Language disorder – oral or listening
      • Reading disorder
      • Mathematics disorder
      • Writing disorder
      • Others or combination
    • Mental health problems
      • Anxiety
      • Conduct disorder
      • Depression
      • Excessive gaming
      • Mood disorder
      • Oppositional defiant disorder
      • Obsessive compulsive disorder
      • Panic attacks
      • Phobia
      • Separation anxiety disorder
      • Shyness
    • Sensory impairment – vision, hearing
    • Sleep problems
  • Social/Community
    • Food
    • Housing including frequent moves
    • Substance abuse
    • Violence
    • Poverty
    • Peer pressure for low performance
  • School
    • Under-resourced learning environment
      • Inadequate overall funding, facilities, books, materials, personnel, etc.
      • Classroom size
      • Environmental hostility, prejudice, or bullying, violence, safety issues
    • Under-, not-appropriately educated teachers
      • Mismatch between student/teacher
      • Curriculum inadequate or unrealistic expectations
      • Curriculum inadequate for specific individual’s needs
      • Excessive testing
      • Increased testing standards without increased educational support
      • School transitions

Questions for Further Discussion
1. What are the potential causes of school problems for this student?
2. What are the social and economic impacts of school failure?
3. What are the health impacts of school failure?
4. How many students end up in prison who have school failure and what are the health impacts of prison? A review can be found here.
5. What are the health impacts of homelessness? A review can be found here.

Related Cases

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: School Health and Asthma in Children and Homeless Health Concerns.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Byrd RS. School failure: assessment, intervention, and prevention in primary pediatric care. Pediatr Rev. 2005 Jul;26(7):233-43.

Kamal M, Bener A. Factors contributing to school failure among school children in very fast developing Arabian Society. Oman Med J. 2009 Jul;24(3):212-7.

Bradshaw CP, O’Brennan LM, McNeely CA. Core competencies and the prevention of school failure and early school leaving. New Dir Child Adolesc Dev. 2008 Winter;2008(122):19-32.

Rimrodt SL, Lipkin PH. Learning disabilities and school failure. Pediatr Rev. 2011 Aug;32(8):315-24.

Klein, J. The Failure of American Schools. The Atlantic June 2011. Available from the Internet at: https://www.theatlantic.com/magazine/archive/2011/06/the-failure-of-american-schools/308497/ (cited 6/4/18)

Welsh EC, Appana S, Anderson HA, Zierold KM. The association between school-to-work programs and school performance. J Adolesc Health. 2014 Feb;54(2):221-7.

Ibabe I. Academic Failure and Child-to-Parent Violence: Family Protective Factors.
Front Psychol. 2016 Oct 7;7:1538.

Chacon Fuertes F, Huertas Hurtado CA.
The Causes of School Failure in Secondary School Students: Validation of a Psychosocial Model with Structural Equations. Span J Psychol. 2017 Nov 20;20:E62.

Literacy. Wikipedia. Available from the Internet at: https://en.wikipedia.org/wiki/Literacy (cited 6/4/18).

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

How are Malformations of the Cerebral Cortex Grouped?

Patient Presentation
A 3-year-old male came to the inpatient floor because of intractable emesis of non-bilious fluid and stomach contents and some watery diarrhea for 36 hours after exposure to a particularly virulent form gastroenteritis at his daycare. He was not tolerating any oral or gastrotube feedings and had not urinated for 10 hours. He also had a 10-15 second generalized tonic-clonic seizure in the emergency room that was similar to his usual seizures that generally occurred 2-3 times/week. He also had a bout of watery diarrhea in the emergency room. The past medical history showed him known to have microcephaly with some areas of polymicrogyri diagnosed as an infant, and seizures that were reasonably well-controlled on phenytoin.

The pertinent physical exam showed a male in mild distress with dry mucous membranes. His weight was down 1040 grams from a recent clinic visit. His heart rate was 112 beats/minute and respirations were 26/minute. His blood pressure was 76/54. His abdomen was very mildly tender diffusely without guarding or rebound tenderness. His gastric tube was in place. There was no organomegaly and his bowel sounds were hyperactive. Neurologically he was semi-noncommunicative. His cranial nerves were normal, DTRs were slightly hyperreflexic and his tone was good. This was his normal neurological examination and the rest of his examination was normal. The work-up showed a normal abdominal radiograph, electrolytes and urinalysis were consistent with mild dehydration, and his complete blood count was normal. His phenytoin level was therapeutic. The diagnosis of gastroenteritis and dehydration was made. In the emergency room he was given ondansteron and intravenous fluids but he still could not tolerate fluids and was admitted. Over the next 24 hours he began to tolerate fluids. He had another similar seizure but again had therapeutic levels and his parents and neurologist were comfortable monitoring him at home and potentially making medication changes by telephone.

Discussion
Cerebral cortex development is an extremely complex process overall. Our current understanding is incomplete and constantly changing as our understanding of genetics and the processes each gene controls emerges from scientific research.
Neural cells are produced in the subventicular zone of the pallial or dorsal germinal epithelium. They then differentiate and migrate radially or tangentially to the cerebral cortex and organize themselves to function.

Malformations of cortical development (MCD) is a heterogeneneous group of disorders that cause defective cortical development. These disorders are broadly categorized into three areas of cerebral cortical development: neural cell proliferation, neural cell migration and cortical organization. There is wide variation in the radiographic features and clinical problems seen and individuals may have more than one of these broad classifications (i.e. have elements 1 or all 3 classifications such as polymicrogyri). Additionally, the brain may be affected with these malformations globally or focally (focal cortical development). Specific wording and classifications for MCDs are necessarily changing as more information is learned.

Malformations of cortical development presenting signs and prognosis predictors are shown in this table, modified from Guerrini and Dobyns (see To Learn More below). MCDs that are more global or that are due to neural cell proliferation problems are often more severe.

Characteristic Most Severe Intermediate Less Severe
MCD distribution Diffuse Frontal-perisylvian Posterior or other
MCD symmetry Bilateral symmetrical Bilateral asymmetrical Unilateral
Head Size Microcephaly Megalencephaly Normal head size
Tone Spascity Hypotonia Normal
Seizure Onset Early, 0-3 months Infancy, 3-12 months Late, > 12 months
Seizure Type Early infantile epileptic encephalopathy,
Infantile spasms,
Lennox-Gastaut syndrome
Non-specific generalized Focal, other types

Learning Point

  • Neural cell proliferation
    • Neural cells need to proliferate, grow and differentiate. Neural cells can divide symmetrically to expand their numbers or asymmetrically to self-renew or generate progenitor cells.
      • Examples:
        • Microcephaly
          • Microcephaly vera or true microcephaly is due to MCD problem, not due to damage or atrophy. It is a small head usually < 2 S.D. for gender and age.
            A review of cases of microcephaly can be found here. This includes non-MCD causes.
        • Megalencephaly
          • A large head usually defined as > 2 S.D. for gender and age (some people are more restrictive as benign familial macrocephaly may be > 2 S.D.) which is caused by too many neural cells.
            “Megalencephaly is different from macrocephaly (also called megacephaly or megalocephaly), which describes a big head, and which doesn’t necessarily indicate abnormality.”
        • Cortical dysgenesis
          • These can be more focal as in focal cortical dysgenesis or hemimegalencephaly where atypical gyri are seen in 1 or more locations
  • Neural cell migration
      Cellular movement is obviously complex involving nuclear and microtubule transport at the cellular level and other processes to direct and allow migration to the cortex. Neural cells can undermigrate, overmigrate or have migration defects.

        Examples:

        • Lissencephaly
          • Classically an undermigration problem, usually seen with a grossly smooth brain surface, can affect 1 or several layers of the cortex
        • Pachygyri
          • Usually an undermigration problem where there are few or large gyri grossly
        • Heterotopia
          • Groups of neurons in abnormal locations
            • Periventricular nodular heterotopia
            • Subcortical heterotopia – sometimes known as double cortex syndrome
          • Cobblestoning malformation
            • Polymicrogyri – see below
  • Cortical organization
    • Neurons that arrive at the proper location must then organize to work together to perform the appropriate tasks and link with other neuronal structures. This is done through folding and maturational changes.
    • Examples:
      • Polymicrogyri
        • Overmigration problem that appears to look like cobblestones or pebbles, can be associated with megancephaly, maybe related to lissencephaly
      • Schizencephaly
        • Cleft in the brain wall from the ventricle to the surface potentially genetic but also thought to be caused by intrauterine injury similar to porencephaly (or cysts/cavities within the brain result from intrauterine injuries such as strokes). The walls of the cleft are lined with polymicrogyri.

Questions for Further Discussion
1. What are causes of macrocephaly?
2. What are the clinical phenotypes caused by Zika virus?
3. What are the classifications of seizures? A review can be found here.

Related Cases

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for these topics: Dehydration and Brain Malformations.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Squier W, Jansen A. Abnormal development of the human cerebral cortex. J Anat. 2010 Oct;217(4):312-23.

Barkovich AJ, Guerrini R, Kuzniecky RI, Jackson GD, Dobyns WB. A developmental and genetic classification for malformations of cortical development: update 2012.
Brain. 2012 May;135(Pt 5):1348-69.

Guerrini R, Dobyns WB. Malformations of cortical development: clinical features and genetic causes. Lancet Neurol. 2014 Jul;13(7):710-26.

National Institute of Neurological Disorders and Stroke. Megalencephaly Information Page.
Available from the Internet at https://www.ninds.nih.gov/Disorders/All-Disorders/Megalencephaly-Information-Page (rev. 5/25/17, cited 5/29/18).

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

How Common is Recurrent Kawasaki Disease?

Patient Presentation
A 3.5-year-old female came to clinic with a 2-day history fever up to 101°F. The evening before she had complained of a sore throat but had no rhinorrhea, cough or rash. She was drinking reasonably well and was urinating frequently. She had been to a birthday party where an older child had had strep throat. The past medical history was positive for Kawasaki Disease diagnosed at 17 months of age and treated with intravenous immunoglobulin (IVIG) on day 6 of fever. Her echocardiograms had been negative and she was being appropriately monitored by cardiology. The family history was positive for heart disease and hypertension in grandparents in the 70s. The review of systems was otherwise negative.

The pertinent physical exam showed a preschooler who was febrile to 39.3°C, heart rate of 104 beats/minute, and respiratory rate of 24. Capillary refill was brisk and her mucous membranes were moist. She had palatal petechiae but no exudates in her throat. She had a few shotty anterior cervical and inguinal nodes that were all < 0.5 cm and freely mobile. Her ears were normal. Cardiac examination showed no murmurs with normal S1 and S2. Her abdominal and lung examination were negative and her skin examination showed no rashes.

The diagnosis of possible strep throat was made. The laboratory evaluation of a rapid strep test confirmed the diagnosis and the parents were educated regarding giving her the penicillin and reasons to call if she was not improving. “Oh we’ll call all right. After her having Kawasaki Disease we’re probably always a little worried about any fever and it coming back. We know that it’s rare to begin with and even less likely to come back, but yet we know it can. I’m not glad she’s sick but glad it is something that we know what it is and can treat it,” her father explained.

Discussion
Kawasaki Disease (KD) was first described in 1967 by Dr. Tomisaku Kawasaki, and since has been identified world-wide. It is an acute, self-limited, febrile illness seen usually in children < 5 years. In North America the incidence is ~25/100,000 children < 5 years. Highest prevalence is seen in Asian children, particularly those of Japanese ancestry. Males are more common than females (~1.5:1). In Japan, case fatality rate is < 0.1%.

The cause is not identified although strong data suggests an interaction between infectious agents and genetic susceptibility. KD is a clinical diagnosis as is recurrent KD. Without treatment at the initial episode, 15-25% of pediatric KD patients may develop cardiac aneurysms but this is decreased to 4% with IVIG treatment. It is the most common reason for acquired heart disease in developed countries.

Treatment for KD changed in 2004, and was updated by the American Heart Association in 2017 (See To Learn More for review).
Primary treatment for initial KD is IVIG (2 mg/kg x 1 infusion) within 10 days of symptoms onset but may be given later also. Aspirin can also be given until the patient is afebrile (moderate dose = 30-50 mg/kg/day to high dose = 80 mg/kg/day). Corticosteroids are sometimes used if patients do not respond to initial therapy.

KD is defined as:

  • Fever of at least 5 days (often > 39-40°C, can last 1-3 weeks without treatment)
  • Plus 4 or more principal clinical features following:
    • Changes in lips and oral cavity – erythema, cracked lips, strawberry tongue, diffuse injection
    • Bilateral bulbar conjunctival injection without exudate
    • Polymorphous exantham – maculopapular, diffuse erythroderma or erythema multiforme-like
    • Cervical lymphadenopathy – more than 1.5 cm, usually unilateral, and in the anterior cervical triangle but may be in other locations

    Note: Patients with 4 or more of the 5 principal features, especially extremity changes, can have the diagnosis made on day 4 of fever.

Laboratory findings associated with KD include:

    During acute phase

    • Leukocytosis with neutrophilia and immature forms
    • Elevated C-reactive protein
    • Elevated erythrocyte sedimentation rate
    • May also be present: sterile pyuria, hyponatremia and hypoalbuminemia

    During 2nd week after fever onset

    • Thrombocytosis

Evaluation for other possible diseases with clinical findings includes:

  • Viral infections – especially adenovirus, enterovirus, Epstein-Barr virus and measles
  • Scarlet fever
  • Staphylococcal scalded skin syndrome
  • Toxic Shock Syndrome
  • Bacterial cervical lymphadenitis
  • Drug hypersensitivity reactions
  • Stevens-Johnson syndrome
  • Juvenile rheumatoid arthritis, systemic onset
  • Rocky Mountain spotted fever
  • Leptospirosis

Learning Point
Recurrent KD is rare with the incidence rate basically unchanging over the last 30 years in Japan. Overall the incidence rate for patients reviewed between 2003-2012 in Japan, was 3.89 /1000 person-years. The authors also note that all deaths in the cohort (N=17) occurred in nonrecurrent KD patients. Risk factors in this study were age < 3 years, male gender and being resistant to initial IVIG therapy. Cardiac problems during the initial event was not a risk factor. Other studies have found other risk factors but these vary by study.

Overall, recurrent KD occurs in ~2-3% of all patients who developed initial KD. Recurrent KD is even rarer in adult patients with a 4th case being described in 2016 in a 23-year-old male who had initial KD at age 4 years.

Questions for Further Discussion
1. What is the clinical definition of incomplete KD and its treatment?
2. What are indications for a cardiology consultation?

Related Cases