Witch's B-Rheum
Gas-per the Friendly Ghost
Dr. Dracula
Zombie Food
Trick-or-Treat
100
A 12 year old girl with a history of Salmonella diarrhea 2 weeks ago presents with fever, conjunctivitis, oral ulcers, urethritis, and arthritis of her left knee and left hip.
Reactive Arthritis. Occurs 1-4 weeks after a GI infection with Shigella, Yersinia, Salmonella, or Campylobacter or after a GU infection caused by Chlamydia or Mycoplasma. Other organisms such as Strep, Parvovirus, C Diff, and Giardia can also trigger the illness. The classic triad is conjunctivitis, urethritis, and arthritis.
100
What is the most common cause of SERIOUS lower GI bleeding in children?
Meckel Diverticulum. Mast are asymptomatic. 2% of the population located within 2 feet of ileocecal junction, measures 2 inches in length and 2 cm in diameter. There is a 2:1 male-female ratio. Usually symptomatic before 2 years of age (if they are going to have symptoms).
100
A 4 year old girl presents with the following sequence of events: 2 weeks age she had a viral infection, now she has the acute onset of bloody nose and noticeable petechial rash, hemoglobin is normal, platelet count is 4k, peripheral smear is normal except for the lack of platelets, and the ones that are seen are large (but not giant) platelets. What is the most likely diagnosis?
Idiopathic (immune) Thrombocytopenia Purpura (ITP)
100
A 3 year old boy has the following type of seizure: 20 seconds in duration, rapid, bilateral, symmetric muscle contractions, repetitive jerks, and he fell to the ground during the seizure. What type of seizure did he have?
Myoclonic seizures. Can be isolated or occur in absence attacks or tonic-clonic attacks. In brief attacks, consciousness may be maintained. These are often associated with progressive neurogenerative genetic diseases.
100
What is the best screening test for Cushing syndrome?
24-hour Urinary Free Cortisol
200
A 16 year old African American girl presents with pericarditis, oral ulcers, hemolytic anemia, and red cell casts on UA.
Systemic Lupus Erythematosus (SLE)
200
A 15 year old girl presents with malaise, weight loss, and anorexia. She occasional has jaundice. She has anti-smooth muscle antibodies in titers 1:40 (positive). What is the most likely diagnosis?
Autoimmune Hepatitis Type 1. This is the "classic" form. It affects girls more than boys and presents between 10 and 20 years of age. ANA is almost always seen and the most specific anti-smooth muscle antibodies are seen up to 80% of cases (>1:20 in children). Family history of other autoimmune disease may be present.
200
A 14 year old girl presents with heavy menstrual flow with her first menses. Workup is done and finds the following: PT is normal, PTT is normal, bleeding time is prolonged, proportional decrease in biological activity, as measured by the ristocetin cofactor assay (rCoF)
von Willenbrand Disease (vWD) Type 1.
200
A 17 year old girl presents with optic neuritis, oculomotor disturbance, incoordination of her upper extremities with dropping of objects, sensory deficits, CSF showing increase in IgG and oligoclonal bands, and MRI with demyelination. What is the most likely diagnosis?
Multiple Sclerosis Only 5% of patients have MS begin before 10 years of age.
200
A 13 year old boy is diagnosed with ADHD. He is placed on stimulant medication. When children with ADHD are placed on stimulant meds what disorder may be unmasked?
Tic disorder
300
A 14 year old girl presents with recurrent parotitis, dry eyes, conjunctival inflammation, dryness of the mouth, and lab positives for RF, ANA, Anti-Ro.
Sjoren Syndrome (SS) Most cases present with recurrent parotits and keratoconjunctivitis sicca (dry eyes + conjunctival inflammation). The lab may show any of these antibodies.
300
What is the most common identifiable cause of chronic gastritis in children?
Helicobacter pylori
300
What is the leading cause of death in adolescents with sickle cell disease?
Acute Chest Syndrome
300
A woman undergoes a prenatal ultrasound at about 32 weeks gestation. The fetus's brain shows widely separated frontal horns with the 3rd ventricle very high between the lateral ventricles. The mother admits to using cocaine during the pregnancy. What is the diagnosis?
Agenesis of the Corpus Callosum. Can be inherited as an X-linked recessive trait or as AD trait. Recently, maternal cocaine use has been linked to this. Isolated agenesis is usually asymptomatic.
300
A 8 month olf presents with an initial history of fever for 4 days. The child had been seen 2 days ago for febrile seizure. The fever breaks today and a rash develops which is why the mother returned with the child. The rash is macular-to-maculopapular and mainly on the trunk and extremities. What is the diagnosis and organism?
Exanthem subitum (Roseola) due to Human Herpesvirus 6.
400
A 10 year old girl with known systemic onset JIA for 4 years presents with acute bloody nose, and these lab values: ALT 890, AST 799, + D-dimer, PTT prolonged at 100 sec, EBC 2k, plt 45k, ESR 8, and ferritin 2,200.
Macrophage Activation Syndrome (MAS) Occurs with sever systemic onset JIA. MAS is also known as hemophagocytic syndrome. Bone marrow may reveal hemophagocytosis. The lab findings of markedly elevated transaminases, with a positive D-dimer, prolonged PTT, and cytopenias are classic. ESR is classically depressed as well in contrast to the severity of the disease process due to the low fibrinogen.
400
A 4 month old presents with severe unremitting pruritus, diarrhea, conjugated hyperbilirubinemia, and GGT is normal. What is the most likely diagnosis?
Progressive Familial Intrahepatic Cholestasis (PFIC) Type 1. Presents between 3-6 months of age.
400
Name the syndrome: Autosomal recessive ANC < 2000 Monocytosis and eosinophilia
Kostmann Syndrome (Familial Severe Neutropenia)
400
A 13 year old girl presents with worsening muscle weakness that is exacerbated by repetitive muscle use and ocular muscle weakness and ptosis. What is the most likely diagnosis?
Juvenile Myasthenia Gravis Rare in children. 80-90% of affected children have circulating autoantibodies to acetylcholine receptors.
400
What is the classic triad of holocarboxylase synthetase or biotinidase deficiency?
alopecia, skin rash, encephalopathy
500
A 9 year old boy presents with bilateral thigh and calf pain that is worse in the evening and occasionally awakens him from sleep, no fever, no limping, no mobility problems, and no joint involvement.
Growing pains. The key is "muscle" pain and not "joint" pain.
500
Name the syndrome: macrocephaly, pigmented penile lesions, hamartomatous intestinal polyps with rectal bleeding and abdominal pain, cafe-au-lait spots, and lipomas.
Ruvalcaba-Myhre-Smith Syndrome.
500
Name the syndrome: Autosomal recessive, neutropenia, exocrine pancreas failure, short stature, metaphyseal dysostoses, recurrent infections, and FTT.
Shcwachman-Diamond Syndrome
500
A 3 month old girl presents with hypotonia and weakness that is symmetric and with more proximal muscle involvement, her legs are more affected than her arms, difficulty feeding, tongue fasciculations, and no sensory deficits are noted. What is the most likely diagnosis?
Werdnig-Hoffman Syndrome or Sever Infantile Muscular Atrophy Type 1 (SMA-1) Tongue fasciculations and proximal muscle weakness are the buzzwords.
500
What should you consider if you find eosinophils on a UA?
Allergic Interstitial Nephritis. (drug-induced)
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