Does a Primary Language Impairment Affect Both Languages in a Bilingual Child?

Patient Presentation
A 4-year-old Spanish-English speaking male came to clinic because his preschool teacher was concerned about his language development. His mother said that the teacher said he didn’t talk as much as the other children and seemed more shy. Using a Spanish interpreter, the mother reiterated that they had come to the United States 2 years ago because of work. Her English language skills were quite good, but she wanted a translator to make sure her concerns were understood. The child had been in the preschool for about 4 months and before this the child was taken care of at home where both parents and extended family spoke mainly Spanish. The mother said that she and the teacher had no concerns about his development otherwise. In Spanish he was able to speak 5 or more word sentences and easily tell a story, understand 2-3 step commands, follow directions and could be understood by others. Family members agreed with this assessment. The child would not use English with his family so his mother was not sure how good his English skills were. A Spanish-English teacher aide at the preschool had told the mother that she felt his Spanish was comparable to other children, but that he seemed quieter overall and hadn’t made as many friends yet at school.

The past medical history revealed a previously full-term infant with no prenatal or postnatal complications. He had no significant illnesses and had been fully vaccinated. The family history was negative for any developmental problems. The review of systems was negative.

The pertinent physical exam showed a healthy appearing male with normal vital signs and growth parameters were in the 75-90%. He had a normal neurological and general examination. He initially seemed quiet but then easily engaged with the pediatrician and was able to follow a simple game. His gross motor and fine motor skills were appropriate. He easily counted to 4 in English and named 2 colors in English and 3 in Spanish without hesitancy.

The diagnosis of a healthy child who was learning a second language was made. The pediatrician said that maybe he should be taught a way of asking for help with language in the classroom when he was working with English-speaking teachers and peers. Also having the Spanish-language teacher aide available in the classroom might also help him to be more comfortable and assist with his language acquisition. A designated consistent peer to be his “special friend” for language activities may also help. Additionally, the pediatrician recommended a hearing test which at followup during his well child check 4 months later was normal. The mother said that the teacher was now not concerned as he seemed to be learning and using more English. The mother said he seemed more comfortable at the preschool too.

Discussion
Internationally, bilingualism is the rule. Even in the US which many have considered the holdout for monolingualism, bilingualism is increasing with more than 18% of people (>5 years) speaking 2 languages and it is expected that by 2030 more than 40% of children will learn English as their second language (L2).

Children learn two or more languages in different contexts. A child may learn two language with parents speaking two different languages at home since birth, may have one language spoken at home and another in the community (such as a daycare setting) since birth, or may learn one at home since birth and a second at a later age when they have wider experiences (going to Kindergarten) with their community or immigrate to another country. There are places where bilingualism is less of an immigrant phenomenon and is an integral part of the community. Examples of stable bilingualism are French-English speaking parts of Canada, or Welsh-English speaking parts of Wales.

Children can successfully use both languages. Just because a child is young does not mean they will be more proficient in the second language (L2). There is data from children who immigrated in the year before school begins and the year afterwards. The older children who immigrated and moved directly into a school setting became more proficient. This is probably because they were older and more proficient in their primary language (L1).

Children use their languages differently depending on the audience (parents, partners, siblings, teacher, community member), and venue (home, school, Internet, work), purpose (asking for directions, explaining school work, telling stories at a family celebration) and their developmental abilities. The dominant language spoken may change across age and learning opportunities but both can be functional.

Children who learn two languages from birth have language acquisition that is comparable or greater than children who acquire only 1 language. But the growth is split between the two languages. A child may seem behind in one or both languages when looking at vocabulary and grammar development, but most children are within range of normal. There is some data that supports children’s skills ‘catching-up” to monolinguistic children by age 9-10 years.

“When both parents are minority language speakers, the children are more likely to sustain bilingual development than when only one is. Some studies also find that parents are more likely to use the minority language with daughters than with sons and that girls are more likely to develop as bilinguals than boys.”

“Language exposure in the context of book reading is particularly supportive of development in [both] language[s], and language exposure via television is not particularly supportive [of language development].”

Adolescent who speak both their home and their community language are more likely to graduate from high school, than peers who speak English only. Minority language use can be supported by continued close family and cultural connections.

Data from children who immigrate to another country have found that school age and adolescents need about 2-3 years to become conversationally fluent in their second language (L2) but it takes about 4-5 years to achieve proficiency conducive to academic achievement. Therefore adolescents who immigrate may not have enough time in the school environment to show their true academic achievement.

Learning Point
Primary language impairments in bilingual children can be difficult to discern. The main issues are to determine if the child has a global developmental issue, a primary language impairment (PLI) or learning disability, or does the child have difficulties learning the L2.

L1 proficiency and cognitive development are the key variables to L2 acquisition. A child who has a PLI, has problems in both languages. A child who has a PLI in their L1 will have problems learning a L2. This does not mean that they cannot be successful but they are less efficient in their learning than their unaffected bilingual peers.

“Poor performance on language tasks, in the face of otherwise typical development, is considered the critical marker of PLI.” There may be other cognitive weaknesses that are not as apparent such as working memory, attention, and information processing speed.

A review of indications for referral to speech therapy can be seen here.

Questions for Further Discussion
1. What services are available in your local community for bilingual education?
2. How are interpretative services best utilized?
3. How do socioeconomic factors affect second language acquisition?

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, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Speech and Language Problems in Children

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.

Kohnert K. Bilingual children with primary language impairment: issues, evidence and implications for clinical actions. J Commun Disord. 2010 Nov-Dec;43(6):456-73.

Hoff E, Core C. Input and language development in bilingually developing children. Semin Speech Lang. 2013 Nov;34(4):215-26.

Clifford V, Rhodes A, Paxton G.Learning difficulties or learning English difficulties? Additional language acquisition: an update for paediatricians. J Paediatr Child Health. 2014 Mar;50(3):175-81.

Cote LR, Bornstein MH. Productive Vocabulary among Three Groups of Bilingual American Children: Comparison and Prediction. First Lang. 2014 Dec;34(6):467-485.

Author

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

What Causes Vesicular Exanthams?

Patient Presentation
A 3-year-old male came to clinic with a history of fever and developing rash for 24 hours. The fever had been 38.2°C maximum and was responsive to antipyretics. The rash had been noticed during the night and was described as small spots on his arms and trunk. Since then they had developed a central vesicle. All the lesions looked the same, and the mother didn’t think that more were appearing. The family denied any travel. There were sick children at daycare but the family didn’t know what specific problems were occurring. There was no cough, emesis, diarrhea, pain or pruritus. He had rhinorrhea and had been drinking well but was refusing most solids. He was not particularly fatigued, lethargic or irritable. The past medical history showed a healthy child who was fully immunized. The review of systems was otherwise negative.

The pertinent physical exam showed a well-appearing child with a temperature of 38.3°C. Heart rate was 106 beats/minute and respiratory rate was 24/minute. HEENT showed no mucosal involvement, no oral lesions and his tympanic membranes were normal. He had mild clear rhinorrhea. Heart, lungs and abdominal examinations were negative. His skin had ~ 20 lesions that were distinct lesions, scattered on the upper torso and upper arms. They were 4-10 mm in size with a red, blanching base. Most had a central vesicle that was 2-3 mm in size with water-like fluid. All the lesions looked the same. There were no excoriations seen. There were no lesions on the palms or soles.

The diagnosis of a vesicular exanthem was made that was most likely due to a viral etiology such as coxsackie disease. He had a relative who had frequent contact with him and who was immunocompromised. Therefore a lesion was unroofed and a swab was sent for varicella polymerase chain reaction. The patient was started on acyclovir for possible varicella but when the test returned negative the acyclovir was stopped. When the physician contacted the mother the next day, she reported that the lesions had not progressed, his fever was lower and he was drinking well.

Discussion
Vesicles are circumscribed, elevated, fluid-filled lesions < 1 cm on the skin. They contain serious exudates or a mixture of blood and serum. They last for a short time and either break spontaneously or evolve into bullae. They can be discrete (e.g. varicella or rickettsial disease), grouped (e.g. herpes), linear (e.g. rhus dermatitis) or irregular (e.g. coxsackie) in distribution.

Associated symptoms such as pruritus, fever, myalgias, coryza and cough, along with a history of potential contact can be helpful. Vesicular rashes that are associated with systemic findings such as fever are usually due to infectious diseases (especially viruses and bacteria), while those that do not have systemic findings often are due to contact or infectious diseases that are non-respiratory contacts such as scabies or tinea.

Most patients do not need specific testing as the clinical history and physical examination will often be enough. In certain cases, scraping of the lesion to look for parasites (i.e. scabies) or multinucleated giant cells (i.e. herpes) or fungus may be indicated.

Most treatment is supportive. Topical agents such as calamine lotion or oatmeal baths may provide some relief. Medication for pain and pruritus can be helpful. Treatment for specific diseases such as acyclovir for herpes, antibiotics for bacterial disease and antifungal mediation for fungal diseases should be recommended as appropriate.

Bullae are also fluid-filled epidermal lesions that are filled with serous or seropurulent fluid. They are > 1 cm and often easily rupture due to their thin walls. The differential diagnosis is different for bullae than for vesicular lesions and is often more ominous including such diseases as Steven-Johnson, staphylococcal scalded skin and meningococcemia.

Vesicular or bullous exanthems should be investigated more extensively if there is skin sloughing, petechiae or purpura, fever and irritability, inflammation of the mucosa, urticaria, has respiratory distress, and diarrhea or abdominal pain.

Learning Point
The differential diagnosis for vesicular exanthems includes:

  • Viral
    • Coxsackie
    • Echo
    • Herpes
    • Smallpox
    • Varicella
  • Bacteria
    • Haemophilus
    • Staphylococcus
    • Streptococcus
    • Mycoplasma
  • Fungal
    • Trichophyton mentagrophytes
    • Tricophyton rubrum
  • Parasitic
    • Rickettsial diseases
    • Scabies
    • Tularemia
  • Other
    • Contact and Rhus dermatitis
    • Dishidrotic eczema
    • Kawasaki disease
    • Photosensitivity

Questions for Further Discussion
1. What is the differential diagnosis of bullous exanthems?
2. What is the difference between a pustular lesion and those that are vesicular or bullous?

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, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Rash.

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.

Sifuentes M. Vesicular Exanthems, in Pediatrics A Primary Care Approach. Berkowitz CD ed. W.B. Saudners and Co. Philadelphia, PA. 1996;400-403.

Feder HM Jr, Bennett N, Modlin JF. Atypical hand, foot, and mouth disease: a vesiculobullous eruption caused by Coxsackie virus A6. Lancet Infect Dis. 2014 Jan;14(1):83-6.

Barr KL. Evaluation of vesicular-bullous rash. ePocrates.
Available from the Internet at https://online.epocrates.com/u/2911775/Evaluation+of+vesicular-bullous+rash/Differential/Overview (rev. 10/3/14, cited 1/27/15).

Author

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

How Do You Diagnose Hypermobility?

Patient Presentation
A 16-year-old female came to clinic with a history of knee pain for 5 months. She had twisted the right knee during cheerleading practice and despite appropriate rest, exercise and strength building that was overseen by the school trainers, she still was having intermittent knee pain. The pain occurred off and on in both knees, not necessarily concurrently. It seemed worse after a heavier workout. She had pain also intermittently at night that would occasionally awaken her. She denied any swelling, erythema, difficulty walking, stiffness, weight loss, fever, skin or vision changes. Her past medical history was significant for multiple musculoskeletal pain episodes that occurred with exercise. Her mother said, “She always seems to be doing something to herself and is more clumsy. When she is running she easily trips over things.” The social history showed she was an avid cheerleader, dancer and runner. The family history was positive for rheumatoid arthritis and joint hypermobility.

The pertinent physical exam revealed a well-appearing female in no distress, normal vital signs and growth parameters in the 50% (weight) and 95% (height). Eye examination showed no obvious lens abnormalities. Cardiac examination had a regular rate and rhythm without a murmur. Abdomen was negative. Skin had no discolorations, apparent thinness, or stretch marks. Musculoskeletal examination showed no erythema or edema of any joint. She had full range of motion in all her joints and no specific pain could be elicited. She was able to oppose her thumbs to her forearm, hyperextend her 5th fingers, and could place her hands flat on the ground with forward trunk flexion. It was not clear if she could hyperextend her knees or elbows.

The diagnosis of probable generalized joint hypermobility was made. The pediatrician discussed this with the family as well as other possibilities including rheumatological diseases (later blood testing was normal). Because of the risk of Marfan syndrome, an echocardiogram was ordered (later also normal) while the patient was awaiting a genetics consultation appointment for possible Marfan syndrome or Ehlers-Danlos syndrome. The patient was also referred to orthopaedics and physical therapy for education and ongoing management of her current and probable future musculoskeletal problems.

Discussion
Hypermobility can be seen in several different clinical entities. These include generalized joint hypermobility, joint hypermobility syndrome, Marfan syndrome, Ehlers-Danlos syndrome and Osteogenesis Imperfecta. For adults, a Beighton score of at least 4 or 5 is used as a definition of hypermobility. For children a score of 5 or 6 is used as a definition. (see scoring system below).

Generalized joint hypermobility is hypermobility with few or no symptoms. If they occur, knee symptoms are the most common.

Joint hypermobility syndrome has hypermobility along with other symptoms such as pain, reduced muscle strength, and decreased proprioception and balance.

Joint hypermobility syndrome is diagnosed by:

  • 2 major criteria
  • 1 major criteria and 2 minor criteria
  • 4 minor criteria
  • 2 minor criteria and a close relative who has been diagnosed with JHS
  • Major Brighton criteria
    • Beighton score of four or more – either now or in the past
    • Joint pain for > 3 months in 4 or more joints
  • Minor criteria
    • Beighton score of 1-3, or having a Beighton score of 0-3 if age > 50 years
    • Joint pain > three months up to three joints, back pain for longer than three months, or spondylosis or spondylolisthesis
    • Dislocation or partial dislocation of > one joint, or the same joint more than once
    • > 3 soft tissue injuries – i.e. bursitis, tenosynovitis
    • Marfanoid habitus
    • Abnormal skin
    • Eye symptoms – i.e. ptosis, hyperopia
    • Varicose veins, hernia, or uterine prolapse

Learning Point
Beighton tests are clinical maneuvers that are dichotomously scored. A total score from 0-9 is calculated with a higher score being associated with hypermobility.
They have been shown to have high inter-rater reproducibility for adults and children.

Beighton scores
Items 1-4 are scored 1 point for each side, item 5 has 1 point only. Add total points for score

1. Passive dorsiflexion of the fifth metacarpophalangeal joint to > or = 90 degrees

2. Passive hyperextension of the elbow to > or = 10 degrees

3. Passive hyperextension of the knee to > or = 10 degrees

4. Passive apposition of the thumb to the flexor side of the forearm, while shoulder is flexed 90 degrees, elbow is extended, and hand is pronated

5. Forward flexion of the trunk, with the knees straight, so that the hand palms rest easily on the floor

Questions for Further Discussion
1. What defines a Marfanoid body habitus?
2. What genetic testing can be done for Marfan syndrome and Ehlers-Danlos syndrome?

3. What physical therapy can patient with hypermobility do to decrease musculoskeletal symptoms?

Related Cases

    Symptom/Presentation: Pain

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, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Joint Disorders

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.

Remvig L, Jensen DV, Ward RC. Epidemiology of general joint hypermobility and basis for the proposed criteria for benign joint hypermobility syndrome: review of the literature. J Rheumatol. 2007 Apr;34(4):804-9.

Smits-Engelsman B, Klerks M, Kirby A. Beighton score: a valid measure for generalized hypermobility in children. J Pediatr. 2011 Jan;158(1):119-23, 123.e1-4.

Scheper MC, Engelbert RH, Rameckers EA, Verbunt J, Remvig L, Juul-Kristensen B. Children with generalised joint hypermobility and musculoskeletal complaints: state of the art on diagnostics, clinical characteristics, and treatment. Biomed Res Int.2013;2013:121054

National Health System. Joint Hypermobility – Diagnosis. Medscape.
Available from the Internet at http://www.nhs.uk/Conditions/Joint-hypermobility/Pages/Diagnosis.aspx (rev. 9/18/14, cited 1/20/15).

Author

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

How Common is Gout?

Patient Presentation
A 12-year-old female came to clinic with a 3 month history of ankle pain. It initially started in the right ankle after she had turned her ankle during dancing. This got somewhat better but then her left ankle and foot also became more painful. She had continued her activities which included multiple dance activities, gymnastics, Tae Kwon Do and two weeks previously she had started cross-country running. The pain had increased in the couple of days after she started cross-country. The running occurred mainly on sidewalks and roads. She said she had no pain in the morning but it slowly increased over the day and was worse when she started her activities in the afternoon or evening. Sometime she would limp during practices. The pain decreased with rest after the activities and with warm baths. She denied any pain at night, swelling or warmth of the joint, night sweats, weight loss, rashes, fevers or chills. She denied any other joints being affected. The past medical history was non-contributory. The family history had no specific orthopaedic, rheumatologic or immune problems. The pertinent physical exam revealed a healthy female with growth parameters in the 10-50%. She was tall and thin. The ankles, feet and toes did not show any erythema or edema. No specific pain could be elicited. The joints were freely mobile. The rest of her extremities, back, and TMJ joints were negative. She had no skin changes.

The radiologic evaluation showed no bony changes in the feet or ankles. The diagnosis of general overuse resulting in ankle pain was made. The patient was instructed to significantly decrease or stop her activities and let her body rest more until the pain subsided. She then could increase her activities as tolerated. She had a health maintenance examination already scheduled in 3 weeks so followup would be done then. After the visit the medical student was reviewing the differential diagnosis of joint pain with the attending and included gout as one of the entities. “I’ve only seen hyperuricemia with patients with tumor lysis syndrome or with a metabolic problem. I’ve never seen gout. It’s really an adult disease and certainly on the differential diagnosis for adults, but we really don’t see it in children,” the attending noted.

Discussion
Purines are heterocyclic aromatic organic compounds. Common ones are adenine, guanine, xanthine, hypoxanthine, uric acid and caffeine. The most common sources of purines are meat (especially liver, kidney, and brain), certain fish (herring, mackerel, anchovies, sardines), and in lower amounts in beans, and certain plants and yeast. Purines are synthesized, used by the body, then they are degraded by a variety of enzymes. They then can be salvaged to resynthesize purines or are eliminated primarily by the kidney.

When intake or synthesis outweighs elimination then hyperuricemia can result. Hyperuricemia can occur in patients with obesity and metabolic syndrome, psoriasis, medications (thiazide diuretics), genetic diseases such as Lesch Nyhan disease, and kidney disease including transplantation. Myeloproliferative disease including leukemia, lymphoma and tumor lysis syndrome also have increased risk of hyperuricemia.

Hyperuricemia can lead to the deposition of monosodium urate crystals in the joint and periarticular tissue. Crystal deposition stimulates interleukin-1 release from monocytes that causes an inflammatory response. Painful joints and gout are the result. Overtime gouty trophi may occur that can cause bony erosions. As it can take 20 years or more for this process of hyperuricemia and crystal deposition to occur, gout generally does not occur often in the typical pediatric patient. Therefore gout is not usually considered in the differential diagnosis of joint or limb pain in the pediatric population as it is in the adult population.

The causes of leg pain can be reviewed here.

The causes of limp can be reviewed here.

Learning Point
In the adult population, gout had a prevalence rate of 1.4 – 4% in the western countries of the United States, United Kingdom, Germany and Australia.
Juvenile gouty arthritis is uncommon in the pediatric population. A review found only 66 cases of primary gout over a period from 1769-1971.

Secondary gout occurs more often but is uncommon too occurring in the diseases listed above. In adult renal transplant patients hyperuricemia (80%) and gout (10%) are frequent complications. In the pediatric renal transplant populations, one study found 50% of patients had hyperuricemia, but rarely had gout. The gout occurred > 5 years after transplant.

Questions for Further Discussion
1. What is the differential diagnosis of joint swelling?
2. What signs and symptoms are consistent with idiopathic juvenile arthritis?
3. What is pseudogout?

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, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Gout and Sports Injuries.

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.

Wikipedia. Purine.
Available from the Internet at http://en.wikipedia.org/wiki/Purine (rev. 1/2/15, cited 1/12/15).

Wikipedia. Purine metabolism.
Available from the Internet at http://en.wikipedia.org/wiki/Purine_metabolisme (rev.11/3/14 cited 1/12/15).

Treadwell BL. Juvenile gout. Ann Rheum Dis. 1971 May;30(3):279-84.

Yarom A, Rennebohm RM, Strife F, Levinson JE. Juvenile gouty arthritis. Two cases associated with mild renal insufficiency. Am J Dis Child. 1984 Oct;138(10):955-7.

Sparta G, Kemper MJ, Neuhaus TJ.
Hyperuricemia and gout following pediatric renal transplantation. Pediatr Nephrol. 2006 Dec;21(12):1884-8.

Truck J, Laube GF, von Vigier RO, Goetschel P. Gout in pediatric renal transplant recipients. Pediatr Nephrol. 2010 Dec;25(12):2535-8.

Kim HK, Zbojniewicz AM, Merrow AC, Cheon JE, Kim IO, Emery KH. MR findings of synovial disease in children and young adults: Part 2. Pediatr Radiol. 2011 Apr;41(4):512-24

Robinson PC, Taylor WJ, Merriman TR. Systematic review of the prevalence of gout and hyperuricaemia in Australia. Intern Med J. 2012 Sep;42(9):997-1007.

Author

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