Pediatrics COMAT Part 2

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

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