Pediatrics COMAT Part 2

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X-linked recessive B cell deficiency found only in boys. Symptoms begin after 6 months of age.
The classic presentation is an infant less than 6 months of age with posttussive emesis and apnea. Give Erythromycin for 14 days to patients and close contacts.
All Ig levels are low. Normal B cell numbers. Decreased plasma cells. Treat with IVIG
'Cough, Coryza, and Conjunctivitis', Koplik Spots.
'Slapped Cheek' caused by Parvovirus B19.
Classic 'steeple sign' from subglottic narrowing
Untreated Kawasaki disease can lead to ______
An acute viral inflammatory disease of the larynx, primarily within the subglottic space. Pathogens include parainfluenza virus type 1. Barking cough.
Recurrent skin, mucosal, and pulmonary infections. Delayed separation of the umbilical cords.
_____ Jaundice: Not present until 72 hours after birth. Resolves by 1 week in term infants and 2 weeks in preterm infants.
Coarse Facies, Abscesses, Retained Primary Teeth, Hyper-IgE, Dermatologic
_____ Jaundice: Present in the first 24 hours of life. Bilirubin increases to greater than 15mg per dL.
_____ abscess: Acute onset high fever with sore throat, a muffled 'hot potato' voice. Six months to 6 years of age.
Subglottic narrowing
Herniation of the intestine only through the abdominal wall next to the umbilicus with no sac.
Presents as an asymptomatic, nontender, smooth abdominal mass that does not usually cross the midline.
Severe lack of B and T cells due to a defect in stem cell maturation and decreased adenosine deaminase
Inability to form membrane attack complex (MAC). Recurrent Neisseria infections, meningococcal or gonococcal.
_____ sign: Reluctance of knee extension when the hip is flexed.
A range of nonhereditary, nonprogressive disorders of movement and posture. Often results from perinatal neurologic insult, but in most cases the cause is unknown.
Patients sit with the neck hyperextended and the chin protruding and lean forward in a 'tripod' position to maximize air entry.
The most common immunodeficiency. Low IgA levels only.
______ syndrome: Cardiac abnormalities, Abnormal facies, Thymic aplasia, Cleft palate, Hypocalcemia, and 22q11 deletion
'Thumbprint sign' on lateral film
Caused by Paramyxovirus. An erythematous maculopapular rash spreads from head to toe.
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Apgar Score ____: Indicate the need for immediate resuscitation.
A suppurative infection of the middle ear cavity that is common in children. Pathogens are S. pneumonia, H. influenza, and Moraxella.
An autoimmune disorder manifesting as arthritis with 'morning stiffness' and gradual loss of motion that is present for at least 6 weeks in a patient
Pruritic 'teardrop' vesicular rash in a dermatomal distribution. Uncommon unless the patient is immunocompromised.
Progressive cerebellar ataxia and oculocutaneous telangiectasia. Caused by DNA repair defect. Increased incidence of malignancies.
An autosomal dominant disorder with recurrent episodes of angioedema lasting 2-72 hours and provoked by stress or trauma.
Congenital infection associated with PDA, deafness, cataracts, and mental retardation.
The most common cause of respiratory failure in preterm infants (affects more than 70% of infants born at 28-30 weeks)
Absent tonsils and other lymphoid tissues may provide a clue
Caused by Coxsackie A virus. Oral ulcers. Maculopapular vesicular rash on the hands and feet and sometimes on the buttocks.
Bilious emesis within hours after the first feeding. Associated with Down Syndrome.
Caused by HHV-6 and HHV-7. A maculopapular rash appears as fever breaks.
Anaphylactic transfusion reaction. Do not give IVIG, as it can lead to the production of anti-IgA antibodies.
An acute inflammatory illness of the small airways in the upper and lower respiratory tracts that primarily affects infants and children
Respiratory distress, sunken abdomen, bowel sounds over the left hemithorax.
'Ground-glass' appearance, diffuse atelectasis, and air bronchograms on CXR.
An X-linked recessive disorder seen only in males. Classic presentation involves bleeding, eczema, and recurrent otitis media. Patients rarely survive to adulthood.
Herniation of the abdominal viscera through the abdominal wall at the umbilicus into a sac. Seen in Beckwith-Wiedemann syndome and trisomies.
_____ sign: Pain with passive neck flexion.
Recurrent infections of catalase + organisms. The nitroblue tetrazolium test is diagnostic.
_____ abscess: Sore throat, a muffled 'hot potato' voice, truisms, drooling, uvula displaced to opposite side. Group A streptococcus.
_____ Disease: Conjunctivitis, Rash, Adenopathy, Strawberry Tongue, Hands and Feet are red, swollen, and flaky skin
Presents with tetany secondary to hypocalcemia in the first days of life. Infections with viruses, fungi, and PCP pneumonia. X-ray may show absent thymic shadow.
Generalized, pruritic, 'teardrop' vesicular periphery. Lesions are often at different stages of healing.
The syndrome includes partial oculocutaneous albinism, peripheral neuropathy, and neutropenia. Blood smear shows neutrophils with giant granules.

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Created Oct 20, 2012ReportNominate
Tags:pediatric