Explanation:

Explanation:

Subluxation of the radial head (nursemaid´s elbow) is a common childhood orthopedic problem. There is conflicting information in various textbooks on the proper technique to reduce the subluxed radial head. However, a study comparing hyperpronation to supination/flexion found that hyperpronation had a higher success rate. If a fracture is diagnosed then a sling would be helpful. Neither a wrist splint nor a long arm cast is helpful in this situation.

Explanation:

Idiopathic scoliosis can be detected in as many as 2 to 3% of children aged 10 to 16 years, 60 to 80% are girls.
Scoliosis may first be suspected when one shoulder seems higher than the other or when clothes do not hang straight, but it is often detected during routine physical examination. Patients may initially report fatigue in the lumbar region after prolonged sitting or standing.
The curve is most pronounced when patients bend forward. Most curves are convex to the right in the thoracic area and to the left in the lumbar area, so that the right shoulder is higher than the left. X-ray examination should include standing anteroposterior and lateral views of the spine. Prompt referral to an orthopedist is indicated. Moderate curves (20 to 40°) are treated conservatively (eg, with a cast or brace) to prevent further deformity. Severe curves (> 40°) can often be ameliorated surgically (eg, spinal fusion with rod placement).

Explanation:

NSAIDs are associated with a number of side effects. The most common side effects are nausea, vomiting, diarrhea, constipation, decreased appetite, rash, dizziness, headache, and drowsiness. NSAIDs may also cause tinnitus and fluid retention, leading to edema. The most serious side effects are kidney failure, liver failure, ulcers and prolonged bleeding after an injury or surgery. Some individuals are allergic to NSAIDs and may develop shortness of breath when an NSAID is administered. People with asthma are at a higher-risk for experiencing serious allergic reaction to NSAIDs.

Explanation:

Juvenile rheumatoid arthritis (JRA) should be suspected in children with symptoms of arthritis, signs of iridocyclitis, generalized adenopathy, splenomegaly, or unexplained fever lasting more than a few days, or rash. Diagnosis is primarily clinical. Patients suspected of having JRA should be tested for rheumatoid factor (RF), antinuclear antibodies (ANA), and ESR as these tests may be helpful in diagnosing JRA and distinguishing its subtypes. In Still´s disease, RF and ANA are absent. In pauciarticular-onset JRA, ANA are present in up to 75%. In polyarticular-onset JRA, RF usually is negative, but in some patients, mostly adolescent girls, it can be positive.

Explanation:

Aspiration of the joint should be done in this patient because she has clinical findings suspicious for septic arthritis. These clinical findings include redness and warmth around the knee, large effusion, and pain upon movement of the joint. Diagnostic arthrocentesis should be-performed in all patients in whom the diagnosis of septic arthritis is considered. Obtaining a 99mm technetium bone scan may aid in the diagnosis of a septic joint by demonstrating increased soft tissue uptake in the region of the joint. However, this is an aid to diagnosis; the “gold standard” is the joint aspirate. Obtaining an antinuclear antibody titer would not be helpful, as we already know that this patient has juvenile rheumatoid arthritis (JRA), and an ANA depending on the type of JRA may or may not be positive. The focus should be on determining if the knee is septic, only the joint aspirate will give us this information.

Explanation:

Intoeing, as described, is usually caused by internal tibial torsion. This problem is believed to be caused by sleeping in prone position, and sitting on the feet. In 90% of cases, internal tibial torsion gradually resolves without intervention by the age of 8. Avoiding prone sleeping enhances resolution of the problem. Night splints, orthotics, and shoe wedges are ineffective. Surgery (osteotomy) has been associated with a high complication rate, and is therefore not recommended in mild cases before the age of 8

Explanation:

Monoarticular juvenile arthritis is associated with uveitis. It is rarely deforming and seronegative. Often, there is an associated anti- nuclear antibody formation. Monoarticular arthritis should be differentiated from polyarticular juvenile arthritis, which is similar to the adult form of arthritis and does cause permanent deformities. Still´s disease is yet another form of juvenile arthritis in which patients present with high fevers, rashes, hepatosplenomegaly, and lymphadenopathy.

Explanation:

Explanation:

Explanation:

Osteogenesis imperfecta is an inheritable disorder of type I collagen. There are four types of osteogenesis imperfecta and the mildest form is type I. This is associated with nondeforming fractures in childhood, joint laxity, and hearing abnormalities. Type II is the lethal type that affects newborns. Porphyrias are inherited abnormalities in heme pathway intermediates, and clinical symptoms are manifested in adults. Pseudoxanthoma elasticum is an inheritable, generalized, progressive connective tissue disorder primarily affecting the elastic fibers. Most cases affect the skin, eyes, and the gastrointestinal system. Marfan syndrome is a dominantly inherited connective tissue disorder characterized by tall stature, scoliosis, ligamentous laxity, lens dislocation, and aortic valve insufficiency. Ehlers Danlos syndrome is an inheritable disorder of connective tissue that is characterized by hyperelasticity of skin and hypermobility of the joints.

Explanation:

The absence of fever along with a normal ESR and WBC is indicative of transient synovitis of the hip, a benign, self limiting condition that must be differentiated from septic arthritis which would be characterized with severe pain with short arc motion, fever, and elevated ESR and WBC. A normal hip should not have an effusion or pain. Perthes disease and DDH would be obvious.

Explanation:

Transient synovitis responds to bedrest, but the patient needs to be acutely reevaluated to rule out a subtle early septic arthritis. IV antibiotics and surgery are only necessary for septic arthritis and have no role in the treatment of transient synovitis. Abduction bracing is used for the treatment of Perthes disease on X-rays.

Explanation:

In the abscence of a sequestrum, the management of osteomyelitis should consist of immobilization and IV antibiotics for six weeks. While recent studies have suggested that two weeks of IV antibiotics followed by four weeks of oral antibiotics may be as effective, this answer was not one of the choices. Irrigation and debridement is not necessary in the absence of a sequestrum, and irrigation and debridement without antibiotics is not adequate treatment.

Explanation:

Without prompt control of bleeding, the patient may die before reaching an emergency department. Fat embolization to the lung, causing ARDS, can result in death, but generally over a matter of days. A responsive patient is unlikely to have a severe intracranial injury, and sciatic nerve injury is not a cause of death. Hypoxemia in this setting is a consequence of failure to control hemorrhage.

Explanation:

This clinical picture is most consistent with juvenile rheumatoid arthritis. Scarlet fever, meningitis, and otitis media do not typically affect the joints. Ankylosing spondylitis does not usually present with fevers or rash.

Explanation:

The majority of children with juvenile rheumatoid arthritis are seronegative for rheumatoid factor, in contrast with 20% seronegativity in adult patients with rheumatoid arthritis. Oligoarticular arthritis, fever, evanescent rashes, and joint deformity are all manifestations of juvenile rheumatoid arthritis.

Explanation:

Still´s disease is a form of juvenile arthritis in which patients present with high spiking fevers, splenomegaly, and rash formation. Uveitis is associated with the monoarticular or spiking oligoarticular juvenile arthritic forms, not Still´s disease.

Explanation:

Juvenile rheumatoid arthritis is the most common form of chronic arthritis in children. Although Felty´s syndrome (B) is a type of juvenile rheumatoid arthritis (which also demonstrates splenomegaly and neutropenia), juvenile rheumatoid arthritis is a more generalized description and, thus, is correct.

Explanation:

The delay in treatment associated with bruising of different ages and fractures of different ages should alert the physician to child abuse. A skeletal survey should be obtained to detect other areas of old injury. There is no role for a head CT in this patient. While parts of the clinical scenario may lead one to suspect a clotting or metabolic bone abnormality these are clearly not the best answers if the entire scenario is considered. While casting is appropriate management, it would be an error to do so and ignore obvious signs of child abuse.

Explanation:

The most important clue to the etiology of- this child´s hip pain is the preceding rubella vaccination, which is common cause of postviral synovitis. Postviral synovitis may occur 1 to 2 weeks after a respiratory, infection or rubella vaccine. It may represent either a viral infection of the joint capsule or an immune- mediated phenomenon. Moreover, the fact that the arthritis resolved spontaneously after a few days supports viral etiology. Child abuse rarely manifests as an isolated fracture of the femoral neck. Legg-Calve-Perthes disease is idiopathic avascular necrosis of the femoral head. It typically presents as hip pain and/or limp of acute or insidious onset in children between the ages of 3 and 12 years. Although this diagnosis is possible, the present patient´s history of the preceding MMR vaccination and subsequent resolution of symptoms are more suggestive of postviral synovitis.
The absence of a fever or an elevated white blood cell count makes osteomyelitis unlikely. Nonetheless, this diagnosis should be entertained if the patient does not have resolution of symptoms.
Slipped capital-femoral epiphysis is often seen in overweight children age 10 to 13 and presents with pain and a limp.
Symptoms include onset of severe pain, external rotational deformity, limited range of motion of the hip, limb-shortening, and, commonly, inability to bear weight.

Explanation:

The most important clue to the etiology of- this child´s hip pain is the preceding rubella vaccination, which is common cause of postviral synovitis. Postviral synovitis may occur 1 to 2 weeks after a respiratory, infection or rubella vaccine. It may represent either a viral infection of the joint capsule or an immune- mediated phenomenon. Moreover, the fact that the arthritis resolved spontaneously after a few days supports viral etiology. Child abuse rarely manifests as an isolated fracture of the femoral neck. Legg-Calve-Perthes disease is idiopathic avascular necrosis of the femoral head. It typically presents as hip pain and/or limp of acute or insidious onset in children between the ages of 3 and 12 years. Although this diagnosis is possible, the present patient´s history of the preceding MMR vaccination and subsequent resolution of symptoms are more suggestive of postviral synovitis.
The absence of a fever or an elevated white blood cell count makes osteomyelitis unlikely. Nonetheless, this diagnosis should be entertained if the patient does not have resolution of symptoms.
Slipped capital-femoral epiphysis is often seen in overweight children age 10 to 13 and presents with pain and a limp.
Symptoms include onset of severe pain, external rotational deformity, limited range of motion of the hip, limb-shortening, and, commonly, inability to bear weight.

Explanation:

Osteochondritis disease is a common cause of anterior knee pain in the adolescent. It is a stress reaction of the insertion of the patellar tendon into the tibial tubercle, causing osteochondritis. The condition previously was more common in adolescent boys near the time of their growth spurt; however, girls are being seen more frequently with this diagnosis owing to their increasingly active sports participation. There can be some fragmentation of the ossicles of the developing tibia tubercle or soft-tissue involvement of the patellar tendon insertion without obvious bony changes. Often there is only a tendonitis of the patellar tendon insertion and inflammation of the deep infrapatellar bursa. Clinically, a young teenager who is quite active in sports will complain of pain and swelling over the tibial tubercle. The condition may be unilateral or bilateral. On examination, there is an obviously swollen, tender, and occasionally warm area over the tibial tubercle. The radiograph and CT scan can be normal or nonspecific, or can show fragmentation of the area of patellar tendon insertion.
The natural history of untreated Osgood-Schlatter disease is relatively benign, if allowed to heal and complications are avoided. Since unusual stress at a time of rapid growth is occurring, limiting the offending sports activity is usually sufficient to relieve the symptoms. Kneeling and squatting should be limited. Physical therapy is occasionally used if there is unusual hamstring tightness.
Avascular necrosis of the hip can occur with Legg-Calve- Perthes disease and sickle-cell disease. Legg-Calve-Perthes disease is characterized by avascular (ischemic) necrosis of the femoral head. Patients frequently present with limping. When pain is present it is often mild, intermittent and referred to the thigh or knee.
Septic arthritis refers to microbial invasion of the synovial space. It presents with joint pain, swelling, and fever.
Slipped capital femoral epiphysis is a disorder of the proximal femoral metaphysis displacing anterolaterally and superiorly.
The slip can occur acutely from severe trauma, or gradually from constant force on weakened epiphyseal plate. Patients usually present with complaints of non-radiating, dull, aching pain in the thigh, groin, hip, or (occasionally) knee.
Stress fracture is a good differential because it is also associated with active sports. It is less likely to cause tenderness of the tubercle. The more likely site of tenderness is the shin. CT scan of the knee will definitely show the fracture, unlike the nonspecific findings in this question.
Systemic JRA has visceral involvements earlier that joint involvement. No such hint is present in the scenario. It can occur without sports.

Explanation:

This patient has oligoarticular juvenile rheumatoid arthritis, which has an excellent prognosis when associated with positive antinuclear antibodies. Juvenile rheumatoid arthritis is a chronic nonsuppurative inflammation of the synovium of the joints. It is characterized by joint effusions, destruction of joint cartilage, and bone deformity, destruction, and fusion. Although a specific etiology is not known, a preexisting susceptibility followed by an environmental trigger is believed to result in development of juvenile rheumatoid arthritis. Infectious triggers include viruses, Borrelia and Mycoplasma. An autoimmune origin is also suggested. Initial presentation includes morning stiffness, joint pain, and swelling. The joint is warm and has decreased range of motion. Erythema is rare. Low-grade fever may be present, together with malaise.
Fever and hepatosplenomegaly are present in systemic juvenile rheumatoid arthritis, known for its initial extra-articular manifestations. Joint manifestations appear within a few months of the fever and are similar to polyarticular juvenile rheumatoid arthritis; although a pauciarticular form also exists. The prognosis is guarded.
HLA B27 is a marker associated with ankylosing spondylitis, a highly disabling disease.
Rheumatoid factor positivity is associated with erosions, polyarthritis, and a poor prognosis.

Explanation:

Idiopathic thrombocytopenia purpura (ITP) is a diagnosis of exclusion. A patient with isolated thrombocytopenia with no other clinically apparent cause of thrombocytopenia is diagnosed as having ITP. ITP is an acquired disorder and commonly presents after a viral infection. It typically presents with bleeding, especially if the platelet count is below 10,000/mm³.
Bone marrow aspiration is typically not performed in patients suspected of having ITP unless the patient is more than 60 years of age. In elderly patients, a bone marrow aspiration and biopsy is performed to rule out myelodysplasia. If performed in a patient with ITP, this test would reveal normal erythropoiesis and myelopoiesis. Megakaryocytes are present in normal to increased numbers.
Acetaminophen is not known to cause thrombocytopenia.
Chronic liver disease can lead to thrombocytopenia because of splenomegaly and hypersplenism. However, this patient does not have any splenomegaly. Also, the patient has normal albumin. PT, and PTT levels, pointing against the presence of chronic liver disease.
Disseminated intravascular coagulation (DIC) is unlikely because the patient has normal D-dimer and Fibrinogen levels.
Both hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenia purpura (TTP) present with fever, microangiopathic hemolytic anemia, thrombocytopenia, renal failure, and neurologic abnormalities. Due to the microangiopathic hemolytic anemia, one sees schistocytes on a peripheral blood smear. This patient does not have a clinical picture to suggest HUS or TTP.

Explanation:

This child presents with pauciarticular juvenile rheumatoid arthritis (JRA). Characteristics of pauciarticular JRA include chronic asymmetric arthritis of four or less large weight- bearing joints. Systemic features are uncommon, but the most feared complication is the development of asymptomatic iridocyclitis, which can cause blindness if untreated. Iridocyclitis/uveitis may be present in all subtypes JRA, but it is particular to the pauciarticular disease of early childhood, in which approximately 30% of patients develop the complication. All children with JRA should have regular ophthalmologic examinations, including slit lamp examinations, to ensure prompt management with corticosteroids. Bone scanning is used as a means of identifying a focus of osteomyelitis or other abnormality.
However, the child in this case presents with the classic findings of pauciarticular JRA and should undergo slit lamp examination to identify possible ophthalmic complications. Complete blood count with differential and platelet count is appropriate for screening anemia in patients with JRA. However, it is not the most important screening test to perform because of the significant morbidity that may occur with undiagnosed uveitis/iridocyclitis.
Erythrocyte sedimentation rate [ESR] is often within reference range in patients with pauciarticular JRA. For this reason, ESR is not an appropriate screening test in pauciarticular JRA. It may be used to follow the success of medical treatment in patients with acute febrile JRA or polyarticular JRA.
MRI of the knee is helpful when trauma is considered in the differential diagnosis. This child has no history or trauma, and MRI is not useful as a screening test for morbidity in JRA.
Urinalysis is performed to screen for nephritis in patients with systemic lupus erythematosus. It is not a routine screening test performed in patients with JRA.

Explanation:

This patient has a hemarthrosis, with low factor VIII levels and an elevated PTT. The possible diagnoses are congenital hemophilia A and acquired anti-factor VIII antibodies. To differentiate between these two entities, a mixing study should be performed. In this test, the PTT is measured after the patient´s serum is mixed with normal serum. In the case of congenital hemophilia A, a mixing study will normalize the PU because the normal serum has factor VIII. In the acquired anti-factor VIII antibody patient, the PTT will not normalize with mixing.
A bone marrow biopsy or peripheral blood smear will not provide any information for the diagnosis of hemophilia. Moreover, with a normal CBC picture (normal cell counts) it is highly unlikely that the patient has developed any hematologic disorder that is due to any cellular abnormality. Thus, bone marrow biopsy and blood smear are not correct options here.
No further testing is incorrect because the final diagnosis of congenital versus acquired hemophilia has not yet been made.
Ristocetin cofactor activity is used to assess the von Willebrand factor (VWF) activity when von Willebrand disease (VWD) is suspected. “VW´D is a condition with defects in both platelet aggregation (as VWF acts like a glue to bind platelets to the endothelium) and clotting factor activity (as VWF is the cofactor of factor VIII). Von Willebrand disease is likely when the bleeding time is prolonged due to the platelet aggregation problem, but this patient has a normal bleeding time. VWF activity is commonly assayed as ristocetin cofactor activity, which tests the ability of VWF to agglutinate platelets by binding to its primary receptor, platelet glycoprotein lb (GP lb), in the presence of ristocetin. However hemarthrosis can also occur in VWD.

Explanation:

Scoliosis is an abnormal curvature of the spine when viewed in the coronal plane. It is also generally associated with a rotational deformity, and it is the rotational component manifested as a rib hump, prominent scapula, or lumbar fullness, that is most likely to call attention to the spinal curvature. The idiopathic type is the most common type of scoliosis and it can be further classified into infantile, juvenile, adolescent, and adult scoliosis according to the patient´s age at onset. In children and adolescents, the curvature is generally not painful. Forward bending, or the Adams test, is the best way to screen for scoliosis. Radiographs of the spine are the gold standard for diagnosis and precise evaluation. If the patient complains of pain, appropriate diagnostic tests should be performed to determine whether the curvature is secondary to the presence of a bony or spinal tumor, herniated disk, or other abnormality.
A myelogram of the spinal canal is recommended for patients with abnormal results on the neurologic examination, atypical curve patterns, rapidly progressive curvatures, or congenital scoliosis. For young patients, sedation is often required. The radiologist should be advised to look for the following:

• A syrinx (a fluid-filled cyst within the spinal cord)
• A tethered cord (a fibrous bind that is located distally and can prevent the normal cephalad migration of the cord)
• A diastematomyelia (a bony or fibrous defect that divides the spinal cord and may cause a tether)
• A diplomyelia (a reduplication of the spinal cord) If the patient has a finding of intracanal abnormalities, particularly if surgical correction of the deformity is contemplated, neurosurgical evaluation may be indicated. In many cases, the release of a tethered cord or decompression of a syrinx can be performed before or at the same time as the scoliosis surgery. Obviously, this is not an appropriate screening test but rather a very advanced evaluation along the path of a patient with confirmed scoliosis.

Patients with curves greater than 60 degrees, those with respiratory complaints, and those with scoliosis resulting from a neuromuscular cause should undergo pulmonary function testing particularly if surgery is being considered. Scoliosis can cause a restrictive lung disease, which needs to be explored by pulmonary function test especially before surgery.
Anteroposterior and lateral radiographs of the entire length of the spine should be taken for precise evaluation of a patient with suspected scoliosis, and this generally requires the use of an extra-long x-ray cassette. When the radiographs are taken, the patient should be in the standing position. If neuromuscular problems make it impossible for the patient to stand, however, radiographs can be taken with the patient sitting.

Explanation:

This patient has Legg-Calvé-Perthes disease, an avascular necrosis of the femoral head. It should be considered in any child between the ages of 4 and 12 who presents with limping. Clinically, limping is the hallmark of Legg-Clave-Perthes disease. At first it is painless, but it can proceed to pain that is worsened by activity and relieved by rest. The pain is reported in the groin, hip, thigh, and knee. Radiographs show a wide articular space initially, and then necrosis of the femoral head. Follow-up films during reconstitution reveal a flattened femoral head, which later becomes spherical. Legg-Calve-Perthes disease is a self-healing process. The goal of therapy is to maintain joint mobility through the concept of containment-maintaining the hip in the acetabulum. This is accomplished by close observation, by bracing with orthoses, or surgically with osteotomies. The most common complication of Legg-Calvé-Perthes disease is osteoarthritis.
Acute slipped capital femoral epiphysis is seen in adolescents. A stress fracture causes displacement of the femoral head from the femoral neck through the femoral capital epiphyseal plate. Typically, patients are obese with delayed skeletal maturation, or tall and thin with a recent growth spurt.
Chronic slipped capital femoral epiphysis is associated with obesity, deficient gonadal development and hypothyroidism.
Manifestations depend on the degree of slippage and its classification (preslip, acute, acute on chronic, or chronic). Chronic slipped capital femoral epiphysis is the most common type and presents with groin pain referred to the anteromedial thigh, limp, and accentuated external rotation. Radiographs show medial displacement of the epiphysis a bare upper portion of the femoral neck, and a-wide growth plate. Treatment consists of pinning, external fixation, bone grafts, and casting.
Coxarthrosis or osteoarthritis of the hip is a degenerative joint disease characterized by deterioration of cartilage with subsequent new bone formation at the articular surface. Pain is the initial complaint, followed by decreased range of motion, limp, and shortening of the affected leg due to a collapsed articular space and degenerative changes of the joint and femoral head. The disease is most common in the elderly and treatment is directed toward relief of pain and prevention of disability. Serious disability indicates the need for surgical intervention and total hip replacement.
Developmental dysplasia of the hip occurs in the newborn period but should be considered up to 3 years of age. A total of 20% of patients have a positive family history. Ligamentous laxity, maternal estrogens, breech position, and torticollis are associated with developmental dysplasia of the hip. Ultrasound is the nest diagnostic test and frog lateral radiographs in older infants and, children.
Treatment varies but is based on containment. This is achieved with double diapers, harnesses, casting, and osteotomies.

Explanation:

Explanation:

Osteomyelitis is inflammation and destruction of bone caused by bacteria, mycobacterium, or fungi. Common symptoms are localized bone pain and tenderness with constitutional symptoms (in acute osteomyelitis) or without constitutional symptoms (in chronic osteomyelitis). Trauma, ischemia, and foreign bodies predispose to osteomyelitis. Patients with acute osteomyelitis of peripheral bones usually experience weight loss, fatigue, fever, and localized warmth, swelling, erythema, and tenderness. Diagnosis is by radiography and cultures. Treatment is with antibiotics and sometimes surgery.

Explanation:

This is a case of radial head subluxations (nursemaid´s elbow). In toddlers (about 2 to 3 years old), the radial head is no wider than the radial neck and can easily slip through these ligaments (radial head subluxation). Subluxation results from traction on the forearm as when a caregiver pulls a reluctant toddler forward or catches the toddler by the wrist during a fall-actions many caregivers do not remember. Symptoms may include pain and tenderness; however, many toddlers cannot describe their symptoms and simply avoid moving the affected elbow (pseudoparalysis).
Plain x-rays are normal and considered unnecessary by some experts unless an alternate diagnosis is clinically suspected. Reduction may be diagnostic and therapeutic. The elbow is completely extended and supinated, then flexed, usually without sedation or analgesia. Reduction is often marked by a subtle palpable pop or click as the radial head resumes normal position. Children may start to move the elbow after about 20 min. Immobilization is unnecessary. If pain or dysfunction lasts longer than 24 hours, incomplete reduction or an occult fracture should be suspected.

Explanation:

Juvenile rheumatoid arthritis is a rheumatic disease that begins at or before age 16. Arthritis, fever, rash, adenopathy, splenomegaly and iridocyclitis are typical of some forms.
Patients with JRA can have joint stiffness, swelling, effusion, pain and tenderness. JRA may interfere with growth and development. Micrognathia (receded chin) due to early closure of mandibular epiphyses may occur. Iridocyclitis may develop, which may cause, conjunctival injection, pain, and photophobia but can be asymptomatic; scarring and glaucoma with band keratopathy can result. Diagnosis is clinical. Treatment involves NSAIDs and often disease modifying antirheumatic drugs.

Explanation:

Up to 14 percent of persons with Down syndrome have evidence of atlantoaxial instability on plain radiographs of the neck. From 1 to 2 percent of all persons with Down syndrome have symptoms of atlantoaxial subluxation and, in some cases, symptoms have appeared after surgery for other conditions. Family members or supervising staff should be advised to monitor for symptoms and an annual neurologic examination should be performed to look for upper motor neuron signs of spinal cord compression. To assess for spinal cord compression from atlantoaxial instability, test gait, tone, Babinski responses, deep tendon reflexes and clonus. Assess for neck pain, torticollis, gait disturbances, spasticity and weakness.

Explanation:

The lesion shown in the picture is highly characteristic of Ewing sarcoma, a bone/soft tissue malignancy that develops most commonly in adolescents and young adults. Long limbs such as the femur, tibia, and humerus are the favored sites. The tumor belongs to a heterogeneous group of malignant neoplasms known conventionally as ´small blue cell tumors.´ These include Ewing sarcoma, small cell carcinoma of the lung, lymphoma, and neuroblastoma. They all share similar anaplastic (undifferentiated) features including small uniform cell size, high nuclear/cytoplasmic ratio, and hyperchromatic nuclei. Sometimes, only special stains allow identification of the cellular origin of these tumors and a correct diagnosis. Ewing sarcoma has an aggressive course. Benign proliferation of adipocytes would result in formation of well differentiated lipomas, which are composed of lipocytes microscopically indistinguishable from normal fat cells. Clotting abnormalities such as hemopnilia, frequently lead to intramuscular hematomas. A fresh hematoma consists entirely of red blood cells, while an organizing hematoma is composed of granulation tissue. A healing fracture results in a reparative callus, formation of new bone at different stages of maturation depending on the period following the fracture. Pyogenic infection would be characterized by extensive neutrophilic infiltration with liquelactive necrosis of tissue.

Explanation:

Osgood-Schlatter disease is a common cause of anterior knee pain in the adolescent. It is a stress reaction of the insertion of the patella tendon into the tibia tubercle. The condition previously was more common in adolescent boys near the time of their growth spurt; however girls are being seen more frequently with this diagnosis owing to more active sports participation. There can be some fragmentation of the ossicles of the developing tibia tubercle or soft tissue involvement of the patella tendon insertion without obvious bony changes. Often there is only a tendonitis of the patella tendon insertion and inflammation of the deep infrapatellar bursa. Clinically, a young teenager who is quite active in sports will complain of pain and swelling over the tibial tubercle. The condition may be unilateral or bilateral.
On examination, there is an obviously swollen, tender, and occasionally warm area over the tibia tubercle. The radiograph can be normal or show fragmentation of the area of patella tendon insertion. The natural history of untreated Osgood-Schlatter disease is relatively benign, if allowed to heal and complications are avoided. Since unusual stress at a time of rapid growth is occurring, limiting the offending sports activity is usually sufficient to relieve the symptoms. Kneeling and squatting should be limited. Physical therapy is occasionally used if there is unusual hamstring tightness.

Explanation:

The boy described here has Osteogenesis Imperfecta (OI), which is a group of disorders caused by deficiencies in the synthesis of type 1 collagen. Although the hallmark of the disease is prominent skeletal deformity, other anatomic structures much in type 1 collagen-such as joints, eyes, ears, skin, and teeth-are affected as well. The clinical expression of OI constitutes a spectrum of disorders, all marked by extreme skeletal fragility. The diagnosis should be considered in patients who have bone fragility (most commonly fractures after minor or unfitness trauma) and any of the following clinical manifestations:

• Short stature
• Scoliosis
• Basilar skull deformities
• Blue sclerae
• Hearing loss
• Opalescent teeth that wear quickly
• Increased laxity of the ligaments and skin
• Wormian bones (small, irregular bones along the cranial sutures)
• Easy bruisability

The diagnosis of GI is mostly clinical and is based on the signs and symptoms described here. There is no definitive lab test for OI. Achondroplasia (choice A) is one of the chondrodystrophies that result in disproportionately short stature. Cardinal features include short – stature. shortening of the arms and legs, disproportionately long trunk, and prominent forehead (frontal bossing). The diagnosis of achondroplasia, however, does not explain the fractures, the deformities, and the hearing defect present in this patient. Similar to children with moderate to severe types of GI, battered children (choice B) have multiple fractures and bruises in various stages of healing. But again, the diagnosis of child abuse does not explain the deformities, the hearing defect the short stature, etc. Constitutional delay of growth (choice C) is suggested by a child who is growing at a normal or mildly decreased rate. The patient is delayed in pubertal development, and the bone age significantly lags behind the chronologic age. Familial short stature (choice D) is characterized by a child with short parents, by a bone age consistent with the chronologic age, and by a growth curve that follows the normal pattern even though it is significantly below the 3rd percentile. Rickets (choice Y) can cause slow growth, bone deformities, and elevation of alkaline phosphatase. However, scleral abnormalities, pathologic fractures, and hearing loss would be unexpected. Radiographic Findings in rickets are characteristic and include an increased width of the epiphyseal plate; irregular, hazy margins of the distal metaphysis; and marginal metaphyseal overgrowth that results in a “ball-in-cup´ appearance. This patient´s x-ray did not reveal any such abnormalities (except fracture), thus ruling out the diagnosis of rickets. Scurvy (choice G) presents with bone pain and swelling over long bones due to subperiosteal hemorrhage. X-ray findings include large periosteal calcium deposits due to subperiosteal hemorrhage and central epiphyseal lucency. Infants and children with scurvy typically present with irritability, pseudoparalysis (due to pain), failure to thrive, and gum bleeding. This patient does not have gum bleeding (HEENT exam is normal) or pain and swelling over long bones other than the fracture site. Moreover, scurvy does not explain the short stature, hearing loss, and scleral abnormalities (although not present in this patient).

Explanation:

Explanation:

Kawasaki disease is a systemic vasculitis of unknown origin that remains a leading cause of acquired heart disease in infants and children. It is a multisystemic disease also known as mucocutaneous lymph node syndrome. Clinical and echocardiographic features remain the basis for diagnosis. An unidentified infection origin and a T-cell immune activation play a prominent role in disease pathogenesis. Tumor necrosis factor alpha (TNF-alpha) receptor levels correlate with the degree of vascular damage and likelihood of coronary artery aneurysm formation. The cardiovascular complications account for most of the morbidity and mortality. Fever, bilateral non-exudative conjunctivitis, mucous membrane changes (injected pharynx, cracked lips, or strawberry tongue), extremity changes (edema, desquamation, erythema, or rash) and cervical adenopathy are common at presentation. The acute manifestations include myocarditis, valvular insufficiency, arrhythmias, pericardial effusion, and congestive heart failure with coronary abnormalities. These develop in 15 to 25% of patients. Leukocytosis and an elevated C-reactive protein are associated with the development of coronary artery aneurysms.
Treatment includes aspirin at 80-100 LID mg/kg/day in divided doses plus IV immune globulin (not IV immune globulin alone in high doses.
Corticosteroids should be avoided because of the potential link-with increasing the likelihood of coronary aneurysm development.
Amoxicillin is incorrect because this is not an infection of the pharynx. The erythema is due to vasculitis of the pharynx. The absence of exudate, exanthema, or stomatitis makes infection less likely.

Explanation:

Explanation:

The presentation in the question strongly suggests the diagnosis of isolated growth hormone deficiency. The basic finding is severe postnatal growth impairment (usually normal at birth) that may be specifically corrected with growth hormone (GH) therapy. GH deficiency may also occur with a deficiency of other pituitary hormones, but there is nothing in the history or examination to suggest that this is the case. There are a number of genetic forms of GH deficiency and GH resistance. Acquired OH deficiency may occur because of irradiation of cranial structures, or it may be idiopathic and usually transitory. There are also several genetic forms of multiple pituitary hormone deficiency. Acquired etiologies of this consist of tumors (e.g., craniopharyngioma), trauma, and inflammation (e.g. tuberculosis). Children with severe OH deficiency are usually more than three to four standard deviations below the mean by 1 year of age. There may then be short growth spurts alternating with lack of growth velocity. As is done in this case, a bone age (radiograph of the left hand and wrist) is obtained that will show a significant delay in bone age compared with chronologic age. The next diagnostic step is to obtain blood for IGF-l and IGE-BP3. Values that are in the upper normal range would exclude the diagnosis. The diagnosis can then be made definitively with the use of a growth hormone stimulation test (with insulin, arginine, or glucagon) where one will find absent or low levels of OH in timed collections after the administration of the OH stimulant. Recombinant growth hormone is then started as soon as possible after diagnosis. Other pituitary function also needs to be evaluated: TSH, ACTH, cortisol, gonadotropins.
The child is described as having a normal examination, and no dysmorphic features are documented, therefore a karyotype would not be the next diagnostic step. Clues to the possible diagnosis of Turner syndrome would be dysmorphic features, cubitus valgus, anomaly of the urinary tract, or heart murmur.
There is no need for skull radiographs at this time. This would be used as a screen for the possibility of a space-occupying lesion causing hypopituitarism and would be followed by an MRI. There is nothing historically or by examination in the question that suggests this diagnosis.
Constitutional delay is a variant of normal growth. Growth is generally normal for the first year, and then decelerates to or less than the third percentile, and then resumes at the normal rate but the child is small. There is usually a family history of a delayed pubertal growth spurt and delay in pubertal development. The bone age will be decreased, which eventually allows for catch-up growth and attainment of genetically programmed adult height
There is nothing in the question to suggest any chronic illness.

Explanation:

Rheumatic fever is an inflammatory disease, possibly autoimmune in nature. Immune responses to group A streptococcal antigens during pharyngitis resulting in antibody cross- reactions with myocardial antigens remain central to the pathogenesis. Rheumatic fever involves many tissues, including the heart, joints, skin, and CNS. Preceding infection with group A Streptococcus is a prerequisite to the development of acute rheumatic fever. Initially, fever, dyspnea, chest pain, and cardiac murmur develop. The Jones criteria* for diagnosis are listed below:

Major:
Carditis
Erythema marginatum and subcutaneous nodules
Sydenham´s chorea I
Arthritis

Minor:
Fever
Polyarthralgia
Reversible prolongation of the PP interval V
Elevated erythrocyte sedimentation rate
Recent beta-hemolytic streptococcal infection
History of rheumatic fever

*Two major criteria or one major and one minor criterion establish the diagnosis. The initial episode of rheumatic fever can be prevented by early treatment of streptococcal pharyngitis. Optimal therapy is intramuscular benzathine penicillin G as a single dose. Penicillin V or amoxicillin for 10 days can also be used. For the penicillin-allergic patient, either erythromycin succinate for 10 days or azithromycin for 5 days is effective.
Juvenile rheumatoid arthritis is characterized by chronic synovitis and systemic inflammatory manifestations. It does not present with carditis.
Lyme disease may begin with migratory poly arthralgias and progress to attacks of asymmetric oligoarthritis in large joints. Erythema chronica migrans the diagnostic annular red lesion at the site of the tick bite, occurs in 50% to 80% of cases during the early phase of illness. Cardiac involvement is atypical.
Septic arthritis is an infection of the synovial cavity. It does not follow a strep pharyngitis and does not cause carditis or valvular deformities.
Rheumatic fever is an inflammatory disease which develops after GAS infection (strep throat or scarlet fever) and can involve the heart (murmur), joints (swelling and pain), skin (blanching maculo-papular rash), and brain (Sydenham´s chorea).

Explanation:

This patient has developed acute osteomyelitis most likely caused by Staphylococcus aureus. The ear infection and osteomyelitis are unrelated. The causative organisms of otitis media are not responsible for infections of the bone. Osteomyelitis is an infection of the bone that may occur by acute hematogenous spread (most common in children), contiguous spread from soil tissue infection, or direct inoculation from penetrating wounds. Osteomyelitis is usually seen in children 3 to 12 years of age and is 2 times more common in boys. The most common causative organism is Staphylococcus aureus. Pasteurella multocida may be a cause of osteomyelitis that developed as the result of a bite wound inflicted by a dog or Pseudomonas aeruginosa is seen as the cause of osteomyelitis after puncture wounds of the foot through a sneaker.
Salmonella and Staphylococcus aureus are a common cause of osteomyelitis in patients with sickle-cell disease.
Group B streptococci and gram-negative enteric bacilli are important causes of osteomyelitis in the neonatal period.

Explanation:

The criteria for the diagnosis of systemic lupus erythematosus (SLE) are:

• Malar rash
• Discoid rash
• Arthritis
• Photosensitivity
• Neurologic disorder (seizure or psychosis)
• Mucocutaneous ulcers (oral or nasal)
• Renal disorder (proteinuria or cellular casts)
• Serositis
• Hematologic disorder (anemia, leucopenia, lymphopenia, thrombocytopenia)
• Immunologic disorder (+ anti-phospholipid, anti-DNA, etc.)
• Positive ANA titer

A patient who meets four or more of the above criteria (as the present patient does) is diagnosed as having definite SLE, an autoimmune inflammatory rheumatoid disease that affects multiple organ systems. Seizure is a common neurologic manifestation Of SLE that occurs due to cerebral vasculitis. Autoantibodies are produced against self-antigens, especially DNA and other nuclear antigens. Sunlight and some infections can exacerbate episodes of lupus. Antinuclear antibody test is the best screening laboratory study and should be performed whenever SLE is suspected, but it is very nonspecific. Antibodies to double-stranded DNA are highly specific and are present during active disease. Anti-Smith antibodies, which are, also highly specific, do not measure disease activity. Renal biopsy confirms lupus nephritis. Antihistone antibodies are formed in drug- induced lupus (usually with procainamide and hydralazine) erythematosus.
A neuroimaging study such as a CT of the brain should be done to exclude a structural and focal brain abnormality, such as infarcts/hypodense areas, hemorrhage, tumors, cerebral calcification, abscess, and basilar meningitis. If the present patient had local-onset´ seizures indicating a structural lesion, CT would be indicated. But neuroimaging studies are not specific for neuropsychiatric SLE. This patient´s seizure is due to SLE (the primary disorder), and thus attempts should be made to confirm the diagnosis of SLE. Even though cocaine abuse can cause seizure and septal perforation, this patient meets the diagnostic criteria for SLE.
Moreover, this patient only has nasal ulcers (and not perforation), which is a mucocutaneous presentation of SLE. In addition, drug testing is not diagnostic of cocaine abuse.
Electroencephalogram (EEG) is incorrect. Most patients with active CNS lupus will have some nonspecific changes in EEG (diffuse slow wave activity´), but these changes are very´ nonspecific and do not aid in the diagnosis of SLE. Moreover, EEG would be done if the seizure was suspected to be due to epileptic seizure disorder and not to SLE.
Rheumatoid factor is a very nonspecific test with limited diagnostic utility´ because other than rheumatoid arthritis (RA), rheumatoid factors are found in Sjogren syndrome, SLE, etc. It is also not very sensitive for RA. Moreover, RA should not be suspected in this patient because other than joint pains with morning stiffness, RA does not explain the other findings (seizure, skin changes, and nasal ulcers). Even the distribution of joint involvement (proximal and distal interphalangeal joints) in this patient is not typical for RA.
Arthritis affecting the metacarpophalangeal joints and proximal interphalangeal joints of both hands is the single most characteristic clinical feature of RA.

Explanation:

Slipped capital femoral epiphysis (SCFE) is an orthopedic emergency. The clinical picture is classic: an obese male in his early teens, who is limping, cannot rotate his leg internally, and when sitting, points the sole of the affected limb towards the other leg. Part of the classic presentation is that although the hip pathology often produces knee pain, the knee is normal on physical examination. Epiphyseal dislocation results in twisting and kinking the lateral epiphyseal vessels, resulting in compromise to the blood flow to the epiphysis. Thus, avascular necrosis (AVN) may be a complication of SCFE.
Cortico-steroid-induced avascular necrosis occurs when there is long-standing use of systemic steroid. This patient has been on inhaled steroid since a young age, and has been on systemic steroid only for the last 10 days. Thus, this is not a possibility.
Legg-Calve-Perthes (LCP) disease is the most common cause of AVN in children, more commonly occurring from ages 6 to 8 years. The vascular anatomy of the proximal femur is in a transitional stage of development in children aged 4 to 7 years, making the blood supply to the femoral head especially vulnerable. Symptoms would be insidious development of limping, decreased hip motion, knee or hip pain, and an antalgic gait. Passive motion of the hip is guarded.
Osteogenic sarcoma is incorrect because it happens in the late teens, and the clinical findings are different, with a bony mass at the area of tenderness and no limitation on the motion of the hip. Tibial torsion and foot inversion would not limit hip motion.