Pediatrics COMAT Part 2

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Can you name the Pediatrics COMAT Part 2?

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

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