Musculoskeletal

Question 1

Form of polyarthritis that affects females more often with associated swelling and pain in 5 or more joints

Answer 1

RF - Polyarthritis

Question 2

Chronic, autoimmune idiopathic arthritis characterized by presence of chronic synovial inflammation with associated swelling, pain, heat, and/or limited ROM. Etiology is unknown; however, it is the most common autoimmune inflammatory disease of childhood.

Answer 2

Juvenile Idiopathic Arthritis

Question 3

Used in severe, progressive JIA resistant to therapy and indicated for resistant or life-threatening disease. Ophthalmic administration is used for chronic uveitis. Toxicities include Cushing syndrome and growth retardation

Answer 3

Glucocorticoid drugs

Question 4

Oligoarthritis with onset around 5 years of age associated with the following: 1. Asymmetric involvement 2. Large joints commonly affected 3. Hips and sacroiliac joints spared 4. Systemic symptoms not present

Answer 4

Early-Onset Oligoarthritis

Question 5

Myelomeningocele associated with bowel and bladder incontinence and normal motor function

Answer 5

Lower-Sacral Myelomeningocele

Question 6

Painful inflammation of synovial membrane, with fluctuating swelling. May develop insidiously, existing months-years without joint destruction. It may also cause joint damage in relatively short time

Answer 6

Synovitis

Question 7

May be used once JIA symptoms disappear to slow joint damage; however, are not usually effective in early stages

Answer 7

DMARDS

Question 8

Used in severe, progressive JIA resistant to therapy as a IM or oral compound. Must be constantly monitored during treatment due to hematologic, renal, and hepatic toxicities

Answer 8

Gold salts

Question 9

Signs and symptoms include fever, malaise, weight loss, malar facial rash, and arthralgias

Answer 9

SLE

Question 10

Treated with NSAIDs in most children to suppress inflammation and fever

Answer 10

Juvenile Idiopathic Arthritis

Question 11

Synthetic proteins that block high levels of inflammatory proteins that are indicated for moderate to severe arthritis that has not responded well to other therapies (IL-1, IL-6). These carry serious risk of infection; however, safety over time is unknown.

Answer 11

Biologic agents

Question 12

Physical findings may include: 1. Sacral dimple, hairy patch at base of spine, uneven gluteal folds 2. Hydrocephalus, difficulty swallowing, hypoventilation, apnea 3. Widely spaced cranial sutures, bulging fontanelle, macrocephaly 4. Lack of typical lower extremity function, sometimes with orthopedic deformity 5. Abnormal deep tendon reflexes in lower extremities 6. Abnormal neonatal reflexes in lower extremities 7. Decreased or absent anal wink 8. Atrophied lower extremity/hip muscles 9. Scoliosis/kyphosis 10. Obesity in older children and adolescents 11. Neurogenic bowel and bladder 12. Latex sensitivity

Answer 12

Spina Bifida

Question 13

JIA subtype that may affect children with psoriasis or a close relative. Finger and toenails may be affected.

Answer 13

Psoriatic Arthritis

Question 14

Caused when the spinal cord becomes bound near sacral level causing spinal cord to be pulled during growth. May cause neurologic damage as tightness increases, such as gait changes and foot deformities. Diagnosis made by CT or MRI after symptoms identified; however, surgical repair is only completed if severe or neurologic changes identified.

Answer 14

Tethered Cord Syndrome

Question 15

Myelomeningocele associated with ability to walk for short distances using long-leg braces. By adolescence, most individuals will use wheelchair for distances.

Answer 15

High Lumbar-Thoracic Myelomeningocele

Question 16

Myelomeningocele associated with flaccid paralysis of lower extremities, absence of deep tendon reflexes, no response to pain, postural abnormalities of feet and legs, urinary dribbling, and relaxed anal sphincter.

Answer 16

Mid-Lumbar Myelomeningocele

Question 17

Multisystem autoimmune disorder that is characterized by widespread inflammatory involvement of connective tissues with immune complex vasculitis; unknown etiology, presenting most commonly in female adolescents

Answer 17

Systemic Lupus Erythematosus

Question 18

Form of polyarthritis that is more adult-like and causes a higher risk of joint damage

Answer 18

RF + Polyarthritis

Question 19

All women of childbearing age should consume 0.8 mg folic acid daily to help prevent these defects; Folic acid should also be consumed 1 month before and during first trimester of pregnancy.

Answer 19

Neural Tube Defects

Question 20

Myelomeningocele associated with ability to walk with short-leg braces and forearm crutches.

Answer 20

Low Lumbar Myelomeningocele

Question 21

JIA subtype in which symptoms do not fit into any other category or fit into more than 1 category.

Answer 21

Undifferentiated

Question 22

JIA subtype related most often with leg and spine caused by an inflammation of entheses (where tendons join bones). It is also associated with IBD.

Answer 22

Enthesitis

Question 23

Sulfa drug 1 salicylate that is indicated for JIA and may take 6 weeks to work

Answer 23

Sulfasalazine

Question 24

Used in severe, progressive JIA resistant to therapy that is most successful and safe drug administered 1x/week. There is no oncogenic potential or risk of sterility

Answer 24

Methotrexate

Question 25

Signs and symptoms include: 1. hydrocephalus, Chiari malformation, hydromelia, tethered cord 2. hip dislocation, knee contractures, spinal deformities 3. urinary dribbling, unable to achieve urinary continence, frequent urinary tract infections, ureteral reflux with renal damage 4. chronic constipation, difficulty with bowel continence 5. motor developmental delays 6. latex allergies 7. normal intelligence with learning disorders, often with ADHD

Answer 25

Spina Bifida

Question 26

Myelomeningocele associated with ability to walk with a gluteal lurch using braces to stabilize ankles and feet. Walking ability is usually retained during adolescence.

Answer 26

High Sacral Myelomeningocele

Question 27

Oligoarthritis associated with the following: 1. Asymmetric involvement 2. Hips and SI joint involvement present 3. Chronic involvement can result in atrophy of extensor muscles in the thigh, tight hamstring ligaments, and knee flexion contractures

Answer 27

Late-Onset Oligoarthritis

Question 28

Spina bifida caused when meninges and CSF herniate through a defect in the posterior vertebral arches in which the spinal cord is typically normal and a cystic sac is evident. Cystic sac is usually well-covered with skin and may transilluminate

Answer 28

Meningocele

Question 29

Occurs when cerebellum protrudes into the spinal canal causing a life-threatening eventAssociated with progressive hydrocephalus, difficulty swallowing, hypoventilation, vocal cord paralysis, spasticity of upper extremities, and apnea in many children with myelodysplasia. May not be revealed until adulthood.

Answer 29

Arnold-Chiari Type II CNS Malformation

Question 30

Spina bifida with signs at birth that include lesion at some point along thoraco-lumbar-sacral spine, often with a cystlike structure protruding containing spinal cord and meninges. Neurological deficits depend upon location.

Answer 30

Myelomeningocele

Question 31

50% of cases of JIA that includes: 1. 4 or fewer joints affected (usually large joints like knees, ankles, and wrists) 2. Gradual onset 3. Can be painless 4. Hip monoarticular arthritis unusual 5. Usually no systemic signs, except for chronic uveitis

Answer 31

Oligoarthritis

Question 32

Condition in which onset is usually acute, with 75% diagnosed within 6 months of symptoms; diagnosis delayed for others 4-5 years. Early diagnostic suspicion is based on episodic, multisystem constellation of clinical disease and associated with persistent antinuclear antibody seropositivity

Answer 32

SLE

Question 33

Physical findings include: 1. Malar erythematous rash associated with acute onset or exacerbation, with butterfly distribution across bridge of nose and over each cheek; discoid rash over sun-exposed areas 2. Transient and migratory arthritis of small joints; never erosive 3. Pericarditis - most common manifestation of cardiac involvement 4. Recurrent headaches, seizures, chorea, frank psychosis 5. Proteinuria, hypertension

Answer 33

SLE

Question 34

Prenatal diagnosis of this condition is possible by maternal serum screening for elevated alpha-fetoprotein, followed by ultrasound diagnostics for spinal anomaly and head “lemon sign;” postnatal diagnosis made on clinical basis.

Answer 34

Spina Bifida

Question 35

Myelomeningocele associated with minimal neurologic deficit.

Answer 35

Upper Thoracic or Cerivcal Myelomeningocele

Question 36

Diagnosis: No specific laboratory studies for diagnosis; however, abnormalities may be found: 1. Human leukocyte antigen B27 2. Moderately severe anemia (7-10), leukocytosis, and thrombocytosis 3. Elevated ESR 4. Elevated ferritin 5. Elevated synovial fluid WBC count 6. Usually RF - 7. Usually ANA - 8. X-rays are rarely helpful; CT, MRI, and bone scans may better evaluate joint disease

Answer 36

Juvenile Idiopathic Arthritis

Question 37

Diagnostic test findings include: 1. Leukopenia, common at onset 2. Positive ANA, anti-double-stranded DNA, anti-Smith antibody, lupus anticoagulant, and anti-phospholipid antibody panel 3. Positive Coombs test 4. Often positive RF and other anti-tissue antibodies 5. Tissue biopsy to confirm diagnosis and distinguish disease severity

Answer 37

SLE

Question 38

Results from Chiari II malformation and obstruction of the 4th ventricle. Management includes routine measurement of head circumference, baseline head CT scan for those with VP shunt.

Answer 38

Hydrocephalus

Question 39

10-20% of JIA cases that occurs in late childhood that is associated with the following: 1. High, daily intermittent spiking fevers of 39 or greater with quick return to baseline temperature or lower 2. Linear evanescent rash present that is salmon-colored nonpruritic macular lesions commonly found on trunk and proximal extremities (transient) 3. Painful multiple joint involvement with morning stiffness, limp, refusal to walk 4. Hepatosplenomegaly, lymphadenopathy, visceral disease, pulmonary involvement, CNS,

Answer 39

Systemic JIA

Question 40

Diagnostic criteria includes: 1. Age of onset < 16 years 2. Arthritis in one or more joints OR presence of 2 or more of these signs (ROM limitation, tenderness, pain with movement, increased heat) 3. Duration of disease 6 weeks or longer 4. Further classified by onset type during 1st 6 months

Answer 40

Juvenile Idiopathic Arthritis

Question 41

Congenital abnormality of the CNS or the spine resulting from failure of neural tube to close during early embryonic development; risk factors for neural tube defect include maternal folic acid deficiency, maternal use of valproic acid or carbamazepine, or maternal diabetes

Answer 41

Spina Bifida

Question 42

Used in severe, progressive JIA resistant to therapy as a useful adjunctive agent requiring frequent ophthalmologic examinations due to possible retinopathy

Answer 42

Hydroxychloroquine

Question 43

Complication of JIA leading to massive systemic inflammatory response associated with high constant fever, headache, easy bruising/bleeding, seizures, and enlarged liver. Condition is fatal and must be treated with corticosteroids.

Answer 43

Macrophage Activation Syndrome

Question 44

40-50% of all cases of JIA that includes: 1. 5 or more inflamed joints 2. Acute or gradual onset 3. Symmetric pattern 4. Commonly affecting large joints (knees, wrists, elbows, ankles) 5. May not be painful

Answer 44

Polyarthritis

Question 45

Short segment thoracic-lumbar kyphosis resulting in sharp angulation

Answer 45

Gibbus

Question 46

Usually benign form of spina bifida; may have sacral dimple, hairy patch at base of spine, and uneven gluteal folds. Caused by failure of one or more of the vertebral arches to fuse and close during week 4 of gestation, most commonly at lumbosacral area. Spinal cord and meninges are unharmed and do not protrude.

Answer 46

Spina Bifida Occulta