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100
Treatment for a newborn in the NICU who's mother has immune thrombocytopenic purpura, and for which his mother received intravenous immunoglobulin, with the last dose given 3 weeks before delivery. His blood type is B–. At birth, his platelet count is 15 × 103/μL (15 × 109/L). On physical examination, he is well-appearing without any evidence of bleeding.
What is IVIG? Maternal immune thrombocytopenic purpura (ITP) can result in neonatal thrombocytopenia because of the maternal production of antibodies to her own platelets, which cross the placenta and cross-react with fetal platelets. If severe, it is treated with an infusion of intravenous immunoglobulin. If the child were experiencing acute, life-threatening bleeding, then immediate transfusion of the most available unit of platelets would be reasonable. In maternally transmitted ITP, however, the antibody is typically reactive with a common platelet antigen, so platelets transfused from an unrelated donor will likely also have an abbreviated lifespan. This would therefore not be the treatment of choice for a stable neonate with maternally transmitted ITP. Neonates with severe thrombocytopenia are at a greater risk for intracranial hemorrhage than are older children. Although it would be reasonable to observe an older child with a platelet count of 15 x 103/μL (15 × 109/L), it would not be recommended for a neonate.
100
This type of immunoglobulin crosses the placenta and causes ABO incompatibility.
What is immunoglobulin G type? Mothers with blood type O naturally have antibodies against A and B red blood cell (RBC) surface antigens. These antibodies are immunoglobulin G type and cross the placenta. Although 15% of pregnancies are at risk for ABO incompatibility, it occurs in only 4% of at risk newborns. Affected newborns commonly present with hyperbilirubinemia within the first 24 hours after birth that may progress to levels causing kernicterus if treatment is not initiated.
100
This is an embryonal tumor of the sympaticoadrenal lineage of the neural crest, and can arise anywhere in the sympathetic chain, including paraspinal ganglia, adrenal glands, and celiac ganglia.
What is a neuroblastoma?
100
It is the most common malignancy in children.
What is Leukemia? The most common malignancy in children is leukemia; ALL accounts for 80% of all childhood leukemias. There are approximately 3,000 new cases of childhood ALL per year in the United States. The peak age at diagnosis is between 2 and 5 years of age. The presentation can be variable; from an incidental finding on routine laboratory testing in an asymptomatic child to a life-threatening hemorrhage or respiratory distress (because of a mediastinal mass). The most common presenting clinical features are fever, pallor, bruising, petechiae, bone pain, hepatosplenomegaly, and lymphadenopathy. Anemia, thrombocytopenia, and an abnormal leukocyte and differential count are common, but may not be present at the time of diagnosis despite the leukemic infiltration of the bone marrow. Approximately 20% of cases of childhood ALL present with leukocytosis with a white cell count greater than 50,000/μL (50 × 109/L). In patients with leukopenia, however, blasts may not be seen on routine peripheral blood smears.
100
Just keep swimming!
Who is Dori (Finding Nemo)?
200
Transfusion of functional platelets is the treatment for life-threatening bleeding of a child with this congenital disorder of platelet adhesion.
What is Bernard-Soulier syndrome? The most common congenital platelet function disorders are Bernard-Soulier syndrome (a disorder of platelet adhesion) and Glanzmann thrombasthenia (a disorder of platelet aggregation). The treatment for bleeding in a patient with a platelet function disorder is the infusion of platelets with normal function.
200
This organism is the most frequent causative agent of sepsis in patients with sickle cell disease.
What is Streptococcus pneumoniae? Patients with sickle cell disease are functionally asplenic, and thus at risk for sepsis with encapsulated organisms. Streptococcus pneumoniae is the most common cause of sepsis in patients with sickle cell disease. Prophylactic daily penicillin in children up to 5 years of age and the use of pneumococcal vaccines have been two of the most significant medical advances for reducing the morbidity and mortality of sickle cell disease. It is important to recognize that even patients who have been taking prophylactic penicillin and have received pneumococcal vaccines can still experience pneumococcal sepsis.
200
It is the most common malignant primary brain tumor in childhood, with an incidence of 0.51 per 100,000 person-years; the incidence peaks from ages 5 to 9 years.
What is medulloblastoma? Medulloblastoma is the most common malignant primary brain tumor in childhood, with an incidence of 0.51 per 100,000 person-years; the incidence peaks from ages 5 to 9 years. Early clinical symptoms and presentation do not differ based on tumor type, so diagnosis is based on imaging characteristics and pathology. If a brain tumor or increased intracranial pressure is suspected, urgent brain imaging with either computed tomography or magnetic resonance is needed.
200
The most likely diagnosis of this 3-year-old girl with leg pain. 3 weeks ago, she had a tactile fever that resolved spontaneously after a few days; however, she has been more tired since then. Over the past week, she is refusing to walk and asking to be carried. Her current oral temperature is 38.3°C, pulse rate is 90 beats/min, respiratory rate is 24 breaths/min, and blood pressure is 100/60 mm Hg. On examination, the child is alert, playful, and in no apparent distress. The edge of her spleen is palpable 4 cm below the left costal margin. She has anicteric sclerae and shotty lymphadenopathy. There is no deformity, swelling, or tenderness of either leg. The remainder of the physical examination is unremarkable. The following are the results of the child’s complete blood cell count: White blood cell count, 3,500/µL (3.5 × 109/L), with 15% polymorphonuclear leukocytes, 75% lymphocytes, 8% monocytes, and 2% eosinophils Hemoglobin, 9.0 g/dL (90 g/L) Mean corpuscular volume, 85 µm3 (85 fL) Platelet count, 45,000 × 103/µL (45 × 109/L) Reticulocyte count, 0.5% Erythrocyte sedimentation rate, 25 mm/h (0 mm/h–20mm/h)
What is ALL or acute lymphoblastic leukemia? The most common presenting clinical features of acute lymphocytic leukemia in children are fever, pallor, bruising, petechiae, bone pain, hepatosplenomegaly, and lymphadenopathy. Anemia, thrombocytopenia, and an abnormal white blood cell (WBC) and differential count are common, but may not be present at the time of diagnosis despite the leukemic infiltration of the bone marrow. Approximately 20% of cases of childhood acute lymphocytic leukemia (ALL) present with leukocytosis with WBC counts greater than 50,000/µL (50 × 109/L); patients with ALL who present with leukopenia may not have blast cells on routine peripheral blood smears. The differential diagnoses for new-onset pancytopenia include malignancy, bone marrow failure, autoimmune disorders, infection, or peripheral destruction of hematopoietic cells.
200
Let it Go!
Who is Olaf (from Frozen)?
300
This is the platelet equivalent of neonatal Rh disease that affects the red blood cells. The fetal platelets express a paternally inherited antigen, most commonly PlA1, which is foreign to the mother. The mother produces antibodies to that antigen, which cross the placenta and cause neonatal thrombocytopenia, while having no effect on the maternal platelet count. The antibodies are maternal and not produced by the neonate, therefore their titer drops with time after birth.
What is neonatal alloimmune thrombocytopenia?
300
This medical emergency is described in a fussy 2-year-old girl with hemoglobin SS, temperature 37.4°C, heart rate 162 beats/min, blood pressure 78/52 mm Hg, oxygen saturation in room air 96%. She appears pale, her spleen tip is palpable 3 cm below the left costal margin. Her examination is otherwise normal. A CBC shows WBC of 13, Hgb of 5, MCV of 88, platelets of 95 and reticulocyte count of 35.
What is splenic sequestration? Splenic sequestration in sickle cell disease presents with severe anemia, thrombocytopenia, and a palpable spleen. Splenic sequestration in sickle cell disease is a medical emergency, and can be rapidly fatal because of very severe anemia.
300
The third most common pediatric benign bone tumor.
What is osteoid osteoma? Osteoid osteoma (OO) is the third most common benign bone tumor, after osteochondroma and nonossifying fibroma, and usually occurs in boys between 7 and 25 years of age. It usually presents with pain that is worse at night and relieved with nonsteroidal anti-inflammatory drugs (NSAIDs). The lesion may be tender to palpation and cause swelling. Swelling is common in OO and may be associated with high levels of prostaglandin production in the tumor, which is also the rationale for pain relief with NSAIDs. The tumor rarely exceeds 1.5 cm, but it can be associated with a significant amount of inflammation. The lesions are usually seen in the diaphysis or metaphysis of long bones such as the femur or tibia.
300
It is the most common site of relapse of ALL.
What is the bone marrow? The bone marrow is the most common site of relapse in pediatric ALL. Isolated bone marrow relapse has a worse prognosis than a combined or isolated extramedullary relapse. Sites of extramedullary relapse are the central nervous system and testicles. Allogeneic stem cell transplantation can be performed with a matched sibling donor or unrelated matched donor, though the former is still the preferred donor type. Various studies have shown that early relapse of acute lymphocytic leukemia (ALL) is associated with aggressive disease and a poor prognosis, with less than a third of these patients surviving, compared with a 50% survival rate for patients with late relapse. Older age (= 10 years of age) is considered a poor prognostic indicator for patients with ALL that is newly diagnosed or in relapse.
300
Be our guest! Be our guest! Put our service to the test! (2)
Who are Lumiere and Cogsworth (from Beauty and the Beast)?
400
Different types of this most common congenital bleeding disorder include types 1, 2A, 2B, 2M, 2N, and 3.
What is von Willebrand Disease? Von Willebrand disease (vWD) is the most common congenital bleeding disorder. Von Willebrand factor is a linking factor that allows functional platelets to bind to fibrin to form a clot. Von Willebrand disease is the result of decreased function or absence of von Willebrand factor; vWD has multiple phenotypes, ranging from mild to severe bleeding disorders that mirror the degree of dysfunction or absence of von Willebrand factor.
400
It is the treatment for a 2 year old girl with sickle cell disease, severe anemia, thrombocytopenia and a palpable very large spleen.
What is emergent blood transfusion, in small aliquots? Splenic sequestration in sickle cell disease presents with severe anemia, thrombocytopenia, and a palpable spleen. Splenic sequestration in sickle cell disease is a medical emergency, and can be rapidly fatal because of very severe anemia. Splenic sequestration associated with sickle cell disease should be treated with packed red blood cell transfusions, always administered slowly and in small aliquots.
400
This is the most likely diagnosis for the child below: An 18-month-old boy presents to clinic for a routine well-child visit. There is no history of serious illnesses or infections. His weight and height are at the 50th percentile for age. In the office, he is afebrile with normal vital signs. He is alert and in no apparent distress. On physical examination, you palpate a large nontender, right upper quadrant abdominal mass. The remainder of the physical examination is unremarkable. Ultrasonography shows a large mass suggestive of a malignancy in the liver. Laboratory findings show anemia, thrombocytosis, and elevated a-fetoprotein.
What is hepatoblastoma? The incidence of all pediatric liver tumors is 1.5 per million annually. Two-thirds of hepatic masses in children are malignant, and of these, two-thirds are hepatoblastoma (HB). Hepatoblastoma usually occurs in infants and very young children. The most important prognostic factor in HB is complete tumor resection. Most childhood liver tumors are found incidentally on palpation by a caretaker or pediatrician, but in cases of advanced disease, pain, weight loss, nausea, vomiting, or anorexia may also be seen. Initial radiographic evaluation for a suspected liver mass should be ultrasonography of the abdomen. Mild normocytic anemia and thrombocytosis are often seen in HB. The main tumor marker for HB is a-fetoprotein, which is elevated in more than 90% of cases.
400
It is the most common disorder of neutrophil dysfunction.
What is chronic granulomatous disease? A defect of the innate immune system should be suspected in a child presenting with frequent bacterial infections. Chronic granulomatous disease is the most common disorder of neutrophil function. Chronic granulomatous disease is diagnosed through testing of the neutrophil oxidative burst by flow cytometry and the initial management should include prophylaxis with trimethoprim and sulfamethoxazole.
400
Up on the shore they work all day, Out in the sun they slave away, While we devotin', Full time to floatin', Under the sea!
Who are Sebastian and Flounder (from Little Mermaid)?
500
Daily double: This is the preferred treatment for severe neonatal alloimmune thrombocytopenia.
What is transfusion of maternal platelets via apheresis? If the neonatal alloimmune thrombocytopenia is severe, it should be treated with a transfusion of maternal platelets collected via apheresis. The maternal platelets will not express the offending antigen, and will therefore not be destroyed by the circulating antibody.
500
This hemolytic disease is a contraindication for giving rasburicase in the setting of tumor lysis syndrome.
What is glucose-6-phosphate deficiency Rasburicase is a recombinant urate oxidase that rapidly decreases uric acid levels by degrading uric acid to allantoin, a highly soluble substance with no known adverse effects in humans. Rasburicase is contraindicated in patients with glucose-6-phosphate deficiency because of the increased risk of hemolysis. Allopurinol, a xanthine analog, is a competitive inhibitor of xanthine oxidase that blocks the conversion of xanthine and hypoxanthine to uric acid and has been shown to decrease the incidence of obstructive uropathy caused by uric acid precipitation.
500
You have to consider this syndrome and get a chest x-ray when a teenager presents on a Friday afternoon with the following symptoms and findings: Swelling of the face, neck, or upper chest Facial plethora or cyanosis Prominent neck and upper chest veins Cough, Dyspnea, Orthopnea Hoarse voice or stridor Chest pain Wheezing, Pleural effusions, Pericardial effusion
What is SVC syndrome associated with anterior mediastinal masses? Superior vena cava (SVC) syndrome is very rare in children, but most commonly results from underlying mediastinal malignancies. Onset of generalized facial swelling combined with facial plethora should prompt clinicians to consider the diagnosis of SVC syndrome. Plain radiography of the chest is effective in identifying the vast majority of mediastinal masses, and should be obtained in any child in whom a mediastinal mass is suspected.
500
This group of genetic disorders result in the failure to express proteins necessary for the normal trafficking of leukocytes to areas of infection. Without the ability to leave the blood stream and enter an area of infection, the leukocytes are unable to contain infections.
What is leukocyte adhesion deficiency? Leukocyte adhesion deficiency is a group of genetic disorders that result in the failure to express proteins necessary for the normal trafficking of leukocytes to areas of infection. Without the ability to leave the blood stream and enter an area of infection, the leukocytes are unable to contain infections.
500
Hakuna Matata! (2)
Who are Timon and Pumba (Lion King)?