Review Test

1.You are evaluating a 2-month-old healthy full-term male infant at a routine health care maintenance visit. His mother is concerned because he seems pale. Although your examination is normal, you draw a hemoglobin (Hgb) level to reassure the parents. Which of the following statements is correct regarding the expected Hgb concentration?

A.Evidence of nutritional iron-deficiency anemia is likely.

B.The Hgb level is likely at its physiologic lowest point.

C.Fetal Hgb has disappeared by now, and the Hgb level will be slightly lower than that at birth.

D.The Hgb level was likely low at birth and is now increasing.

E.Evidence of macrocytic anemia is likely.

2.A 2-year-old boy is brought to your office with a history of multiple bacterial infections, including six episodes of otitis media, three episodes of sinusitis, and one episode of periorbital cellulitis. He is normal-appearing with normal growth and development. Biweekly laboratory assessments for the past 3 months have revealed consistently low white blood cell counts at 2000–2500 cells/mm3, with an absolute neutrophil count of 500–1000 cells/mm3. Which one of the following is the most likely diagnosis?

A.Chédiak–Higashi syndrome

B.Shwachman–Diamond syndrome

C.Chronic benign neutropenia of childhood

D.Cyclic neutropenia

E.Kostmann syndrome

3.A 3-year-old girl is brought to the office with petechiae and bruising on the face, chest, back, and lower extremities, which her mother noticed early this morning. The mother states that her daughter has been healthy except for a viral upper respiratory illness 2 weeks ago.

Laboratory assessment reveals a platelet count of 25,000/µL. Which of the following statements regarding the likely diagnosis is correct?

A.Hemarthroses commonly occur in this disorder.

B.Spontaneous recovery within several weeks to months is expected.

C.Platelet transfusion should be urgently performed.

D.Prothrombin time and activated partial thromboplastin time are prolonged.

E.Considering the girl’s young age, this disorder will likely become chronic.

4.A 15-month-old girl has a hypochromic, microcytic anemia (hemoglobin of 10.6 g/dL) on a routine anemia screen performed in the office. History reveals a diet consisting of six 8-oz glasses of whole cow’s milk per day since the age of 9 months. Which of the following statements regarding the likely diagnosis is correct?

A.The anemia is caused by a benign disorder, and there are no physical or intellectual effects.

B.Low serum ferritin is a late finding.

C.The reticulocyte count is low, considering the impact of this disorder on the bone marrow.

D.Transferrin saturation is low.

E.Free erythrocyte protoporphyrin is low.

5.You are evaluating a term newborn female infant during a routine health maintenance evaluation at 2 weeks of age. You receive the results of a routine newborn screen performed on the second day of life. The results of the newborn screen are normal, with the exception of the sickle cell screen, which reveals that the infant has hemoglobin (Hgb) A, Hgb S, and Hgb F. Which of the following is correct regarding the diagnosis?

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A.Mild anemia is expected.

B.A vaso-occlusive crisis involving the hands and feet is likely by 6 months of age.

C.Splenic function is expected to decrease by 3 years of age.

D.Penicillin prophylaxis should be started immediately.

E.Hematuria may be the only manifestation of this disorder.

6.An 8-year-old boy with sickle cell anemia presents with severe right arm pain that began today. His pain has been unresponsive to oral acetaminophen. His mother states that he has been afebrile, and she denies any trauma. On examination, his right arm is mildly tender and is minimally warm along the humerus. Range of motion of the upper extremity is normal. There is no fever. Which of the following is the appropriate initial management?

A.Obtaining a blood culture

B.Transfusion of red blood cells with a goal of an Hgb level of 14 g/dL

C.Bolus with 40 mL/kg of normal saline

D.Administration of ibuprofen and a narcotic

E.Magnetic resonance imaging (MRI) to rule out osteomyelitis

7.A 17-year-old girl presents with concerns about her menstrual periods. Menarche occurred at 13 years of age, and for the past 2 years, her menstrual cycles have been regular but characterized by extremely heavy bleeding lasting 7–8 days. She also has a history of frequent nosebleeds since early childhood. She denies bleeding into her joints and has been otherwise healthy. She also denies medications, including aspirin. Based on her history, which of the following is the most likely diagnosis?

A.Hemophilia A

B.Hemophilia B

C.Vitamin K deficiency

D.Immune thrombocytopenic purpura (ITP)

E.von Willebrand disease

8.A 7-year-old girl comes to your office for the first time. Her parents bring her to see you because she has had widespread bruising for 3 days. They also believe that she is abnormally pale. On examination, you note that she is pale and has multiple areas of ecchymosis on her arms, legs, and trunk. Her medical history is remarkable for being born with absence of her right thumb and right radius. Laboratory studies reveal a hemoglobin level of 7 g/dL, platelet count of 30,000/µL, and white blood cell count of 800 cells/mm3. Which of the following statements regarding the likely diagnosis is correct?

A.The reticulocyte count is elevated.

B.Spontaneous recovery from this disorder is likely.

C.The growth chart likely reveals short stature.

D.Intravenous immunoglobulin should be administered for immune thrombocytopenic purpura (ITP).

E.Causes of the disorder include drugs, radiation, or chemical exposure.

9.A 10-year-old Italian boy has had chronic anemia since infancy that is characterized by severe hypochromia and microcytosis. Examination reveals a short child, an enlarged liver and spleen, and prominent facial bones, especially the maxilla, forehead, and cheekbones. Which of the following statements regarding the likely diagnosis is correct?

A.Management should include supplemental iron.

B.Hemochromatosis is a likely future complication.

C.His hemoglobin (Hgb) electrophoresis will reveal Hgb S and Hgb F.

D.The cause of his disorder is deletion of the α-globin chain.

E.His blood smear will show spherocytes.

10.A 2-year-old girl has anemia with a hemoglobin level of 9.8 g/dL on routine laboratory screening. History reveals a diet consisting of large amounts of goat’s milk. Which of the

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following statements regarding the anemia is correct?

A.The cause of the anemia is diminished intake of vitamin B12.

B.Spoon-shaped nails may be seen on examination.

C.Smooth red tongue may be seen on examination.

D.The anemia is normochromic and normocytic.

E.Management includes administration of folic acid.

The response items for questions 11–13 are the same. You will be required to select one answer for each item in the set.

A.Thrombocytopenia–absent radius (TAR) syndrome

B.Fanconi anemia

C.Wiskott–Aldrich syndrome

D.Immune thrombocytopenic purpura

E.Hemolytic uremic syndrome

F.Kasabach–Merritt syndrome

G.Glanzmann thrombasthenia

H.Bernard–Soulier syndrome

For each patient, select the likely diagnosis.

1.A 2-year-old boy with thrombocytopenia and a large hepatic hemangioma.

2.A 5-year-old boy with thrombocytopenia, moderate eczema, and both humoral and cellmediated immunodeficiency.

3.A newborn girl with thrombocytopenia, ventricular septal defect, and absence of a radius. The girl’s thumb is present.

The response items for questions 14 and 15 are the same. You will be required to select one answer for each question in the set.

A.Transient erythroblastopenia of childhood

B.Diamond–Blackfan anemia

C.Parvovirus B19 red blood cell aplasia

For each clinical description, select the likely diagnosis.

1.A 6-month-old male infant presents with rapid onset of anemia. He has triphalangeal thumbs on examination. Corticosteroids improve his anemia.

2.A 2-year-old girl develops the gradual onset of significant anemia 2 weeks after a viral upper respiratory infection. Her anemia improves spontaneously.

The response items for questions 16–19 are the same. You will be required to select one answer for each item in the set.

A.Hemophilia B

B.Platelet function defect

C.Vitamin K deficiency

D.Disseminated intravascular coagulation (DIC)

E.von Willebrand disease

F.Immune thrombocytopenic purpura

For each patient, select the most likely diagnosis.

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1.A 6-year-old boy with prolonged diarrhea lasting 2 weeks develops a hemarthrosis involving the knee. He has a prolonged prothrombin time and prolonged activated partial thromboplastin time with a normal platelet activation function assay.

2.A 2-year-old boy with newly diagnosed acute lymphocytic leukemia presents with fever, petechiae, and ecchymoses. He has thrombocytopenia, prolonged prothrombin time, prolonged activated partial thromboplastin time, and a prolonged platelet functional assay.

3.A 5-year-old boy presents with a hemarthrosis involving the knee. He has a normal prothrombin time, prolonged activated partial thromboplastin time, and normal platelet function assay.

4.An 8-year-old boy develops severe bleeding following tonsillectomy. He has a normal prothrombin time, prolonged activated partial thromboplastin time, and abnormal platelet function assay.

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