McCance 31. Book copy questions

During fetal development, what is the primary site of erythropoiesis approximately 2 weeks after gestation?

At approximately what gestational age does the production of leukocytes and platelets begin in the developing fetus?

The liver's role in fetal erythropoiesis peaks around 4 months of gestation. What happens to hepatic blood formation thereafter?

When does hematopoiesis begin in the bone marrow during fetal development, eventually filling the entire bone marrow space?

Which of the following conditions is classified as an acquired disorder affecting erythrocytes in children?

Which of the following is classified as an inherited hemorrhagic disease affecting coagulation and platelets?

Among the neoplastic disorders discussed, which category includes both non-Hodgkin lymphoma and Hodgkin lymphoma?

A child is diagnosed with a condition affecting their red blood cells. Genetic testing reveals a mutation leading to abnormal hemoglobin structure. Which type of disorder is most likely present?

What is the primary cause of iron deficiency anemia (IDA) in toddlers, according to the information provided?

Which factor is MOST likely to contribute to iron deficiency anemia (IDA) in adolescent girls and women of childbearing age?

A child is diagnosed with iron deficiency anemia (IDA) and a suspected cow's milk allergy. What physiological mechanism BEST explains the link between these two conditions?

How does the bioavailability of iron differ between breast milk and cow's milk?

In developing countries, what is a significant contributor to iron deficiency anemia (IDA) in children?

What is a key characteristic that distinguishes hemolytic anemias caused by intrinsic erythrocyte abnormalities from those caused by extra-erythrocytic factors?

What is the underlying mechanism of hemolytic disease of the fetus and newborn (HDFN)?

Why is iron deficiency anemia (IDA) in children a significant concern, especially during early development?

Which of the following best describes the role of the fetal mononuclear phagocyte system in hemolytic disease of the fetus and newborn (HDFN)?

A child presents with refractory iron deficiency anemia (IDA), and a familial component is suspected. What underlying factor should be considered?

What is the primary reason for the abrupt decrease in reticulocytes during the first few days after a full-term neonate's birth?

How does the erythrocyte lifespan typically differ between full-term infants, premature infants, and adults?

What factor primarily contributes to the higher hemoglobin levels observed in mature males compared to females?

Why do erythrocytes in neonates consume greater quantities of glucose than erythrocytes in adults?

What is a possible explanation for why children's lymphocytes tend to have more cytoplasm and less compact nuclear chromatin compared to adult lymphocytes?

When does the neutrophil count in healthy neonates typically peak after birth?

Until approximately what age does the monocyte count remain elevated relative to adult levels?

Which statement accurately compares platelet counts in full-term neonates, infants, children, and adults?

Besides infections and toxins, what is another cause of acquired hemolytic anemias in children?

What category of intrinsic defects can lead to inherited forms of hemolytic anemia in children?

What is the most common blood disorder observed in children?

Which factor can affect the degree of postnatal decrease in hemoglobin and hematocrit values, causing it to be more marked?

At what stage of development do hemoglobin, hematocrit and red blood cell (RBC) count values start to show divergence between males and females?

What structural defects can cause inherited hemolytic anemia?

In what way do metabolic processes within the erythrocytes of neonates differ from those of erythrocytes in the normal adult?

In early stages of iron deficiency anemia (IDA), why might anemia not be immediately apparent despite depleting iron stores?

What is the primary reason for restricting cow's milk intake in children with iron deficiency anemia (IDA)?

A child with chronic iron deficiency anemia (IDA) exhibits pica, decreased physical growth, and developmental delays. What other clinical manifestation is most likely present?

Why is vitamin C often administered alongside ferrous salts in the treatment of iron deficiency anemia (IDA)?

A mother is Rh-negative and her fetus is Rh-positive. What condition can occur due to this maternal-fetal incompatibility?

A child is diagnosed with normocytic-normochromic anemia. Which of the following could be a potential cause?

Which of the following inherited defects leads to hemolytic anemia due to abnormalities in the red blood cell membrane structure?

Which laboratory finding is most indicative of iron deficiency anemia?

What is the rationale behind continuing iron therapy for 2 months after erythrocyte indexes have returned to normal in a child with IDA?

Which of the following infections is least likely to directly cause hemolytic anemia?

A child presents with lethargy, listlessness, and a hemoglobin level of 7 g/dL. What compensatory mechanism is most likely helping maintain tissue oxygenation?

Which genetic factor plays a role in determining whether a person is Rh-positive or Rh-negative?

A child with glucose-6-phosphate dehydrogenase (G6PD) deficiency is at increased risk for which type of anemia?

In a child experiencing chronic hemolysis, where would you expect to find active hematopoiesis?

What is the most important initial step in evaluating a child suspected of having iron deficiency anemia (IDA)?

Why is extramedullary hematopoiesis more commonly observed in children compared to adults?

Which of the following conditions is least likely to cause hemolytic anemia?

What triggers the decrease in erythropoietin levels and the rate of blood cell formation immediately after birth?

How does fetal hemoglobin (HbF) facilitate oxygen transport in the relatively hypoxic uterine environment?

What changes in erythrocyte characteristics occur during the second trimester of gestation?

If a fetus at 20 weeks gestation is suspected of having thalassemia major, what hemoglobin type would be analyzed to confirm this suspicion?

Why do blood cell counts tend to be higher in newborns than in adults?

What stimulates erythropoietin production in the fetus?

How does the distribution of hematopoietic marrow change from infancy to adulthood under normal conditions?

What is the approximate blood volume of a premature infant weighing 2 kg?

At birth, a neonate's hemoglobin composition typically consists of which percentages of HbF, HbA, and HbA2?

What is the primary reason there is a gradual decline in the number of immature blood cells in an infant's peripheral blood during the first 2 to 3 months of life?

What is the approximate blood volume of a 4-year-old child weighing 16 kg?

If a child has a condition causing significantly increased erythrocyte production, and their erythropoietin levels rise, what initial change would be observed in their bone marrow?

After birth, which regulatory mechanism shifts to favor the production of adult hemoglobins (HbA and HbA2) over fetal hemoglobin (HbF)?

What is thought to be the mechanism of anemia in some cases of congenital infections?

Which factor does NOT contribute to anemia in critically ill children?

What is the primary focus of ongoing research regarding blood transfusions in critically ill children?

Inherited erythrocyte defects can lead to hemolytic disease through which of the following mechanisms?

What is the function of Glucose-6-phosphate dehydrogenase (G6PD) in red blood cells?

Which of the following is the primary mechanism by which G6PD deficiency leads to hemolysis?

Why do heterozygous females sometimes exhibit partial expression of G6PD deficiency?

How does G6PD protect erythrocytes from oxidative stress?

Which of the following stressors will NOT typically initiate hemolysis in individuals with G6PD deficiency?

Why should laboratory evaluation for G6PD activity be performed shortly after a hemolytic crisis?

What is the role of NADPH in red blood cells, particularly in the context of G6PD deficiency?

How does the presence of Heinz bodies contribute to hemolysis in G6PD deficiency?

Why might a pregnant woman ingesting salicylates (aspirin) cause hemolysis in her fetus if the fetus has G6PD deficiency?

What causes erythrocyte damage in children affected by from G6PD when they are exposed to stressors?

In the diagnosis of G6PD deficiency, a low normal range of G6PD activity accompanied by a high reticulocyte count should suggest?

What is the primary concern regarding elevated levels of unconjugated bilirubin in a neonate experiencing extensive hemolysis?

In Rh incompatibility, why does the first incompatible fetus typically remain unaffected or only mildly affected by HDFN?

A fetus with severe anemia exhibits gross edema throughout the entire body. What condition is this indicative of?

What is the primary mechanism by which maternal anti-Rh antibodies cause harm to the Rh-positive fetus in subsequent pregnancies?

What clinical signs in a neonate would indicate a severe case of Hemolytic Disease of the Fetus and Newborn (HDFN)?

A newborn is diagnosed with icterus neonatorum shortly after birth. What underlying process is the primary cause of this condition?

Why does hyperbilirubinemia occur in the neonate after birth in cases of HDFN?

Without timely replacement transfusions for a child with Rh incompatibility, deposition of bilirubin in the brain can occur, leading to which specific condition?

Why is Rh incompatibility generally considered more severe than ABO incompatibility in the context of HDFN?

What does the direct Coombs test primarily measure in the context of Hemolytic Disease of the Fetus and Newborn (HDFN)?

How does erythroblastosis fetalis develop in the fetus as a result of HDFN?

Why is Rh immune globulin (RhoGAM) administered to Rh-negative mothers?

What is the primary reason why ABO incompatibility can sometimes cause HDFN even during the first incompatible pregnancy?

Which class of immunoglobulin is primarily responsible for crossing the placenta and causing HDFN?

If anti-D Ig is not administered within 72 hours of exposure to Rh-positive erythrocytes, what is the updated recommendation regarding its administration?

Why must an Rh-negative mother receive Rh immune globulin after the birth of each Rh-positive baby or after a miscarriage?

What factors influence the capacity of the mother’s immune system to produce anti-Rh antibodies?

If a mother is blood type A and the fetus is blood type B, against which fetal erythrocytes may the mother's blood contain preformed antibodies?

In cases of HDFN when Rh immune globulin was not administered, what immediate treatment is typically initiated within the first 24 hours of the neonate's life?

How does phototherapy reduce the toxic effects of unconjugated bilirubin in neonates?

What is the underlying mechanism by which IgG-coated fetal erythrocytes are destroyed in HDFN?

What factors significantly influence the effectiveness of phototherapy in lowering serum bilirubin levels?

Which statement accurately describes the development of anti-Rh antibodies in an Rh-negative mother?

Infections acquired by the mother and transmitted to the fetus can sometimes cause hemolytic anemia. Which of the following is a viral infection known to potentially lead to hemolytic anemia in neonates?

Unconjugated bilirubin is formed during the breakdown of hemoglobin. How is this substance removed from the fetal circulation?

In which scenario is ABO incompatibility most likely to cause HDFN?

Which of the following infections of the newborn, when acquired from the mother during pregnancy, may lead to hemolytic anemia with symptoms resembling those of HDFN?

What is the underlying mechanism by which autosensitization occurs during phototherapy for hyperbilirubinemia?

What are the potential sources of exposure to incompatible Rh-positive erythrocytes that can lead to the development of anti-Rh antibodies in an Rh-negative mother?

Why do anti-O antibodies not exist?

What is the primary therapeutic intervention emphasized for managing Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency?

In areas endemic for malaria, what specific action does the World Health Organization (WHO) recommend before administering antimalarial medications, regarding G6PD deficiency?

What is the underlying cause of hereditary spherocytosis (HS)?

Which characteristic distinguishes spherocytes from normal red blood cells, contributing to their removal from circulation?

What is the typical inheritance pattern observed in approximately 75% of hereditary spherocytosis (HS) cases?

Which presenting signs are most commonly associated with hereditary spherocytosis (HS)?

A newborn presents with hemolytic anemia and hyperbilirubinemia. Which condition should be suspected?

Which laboratory finding is characteristic of hereditary spherocytosis (HS)?

What test is used to assess red blood cell fragility in hereditary spherocytosis (HS)?

What is the recommended initial treatment for children under 5 years of age diagnosed with hereditary spherocytosis (HS)?

For children with severe hereditary spherocytosis (HS) or those who develop symptomatic gallstones, what is the recommended treatment approach?

What genetic mutation characterizes sickle cell disease (SCD)?

Which structural components are affected by mutations in the HbB gene in sickle cell disease?

What is the direct consequence of the preferential adhesion of sickled cells to endothelial cell surfaces?

What role do macrophages play in the context of hemolytic sickled cells?

In sickle cell disease (SCD), what is the primary mechanism by which cycles of deoxygenation and oxygenation lead to pathological consequences?

Which of the following factors contributes to the sickling of erythrocytes in individuals with sickle cell disease (SCD) by increasing the mean cell hemoglobin concentration (MCHC)?

How does inflammation in the microcirculation contribute to the pathophysiology of sickle cell disease (SCD)?

What is the most likely consequence of the influx of Ca++ ions into erythrocytes damaged by sickling in sickle cell disease (SCD)?

In sickle cell disease (SCD), what is the underlying mechanism by which low temperatures can precipitate a sickle crisis?

Which factor is LEAST likely to directly trigger or sustain sickling in individuals with sickle cell disease (SCD)?

How does the presence of other types of hemoglobin (Hb) in heterozygotes with sickle cell trait (HbAS) typically affect sickling?

What is a frequent outcome of irreversibly sickled cells in the microcirculation of individuals with sickle cell disease (SCD)?

What is the rationale behind ongoing research to identify optimal intravenous fluids for individuals with sickle cell disease (SCD)?

Which of the following genotypes of sickle cell disease (SCD) is typically associated with the most severe clinical manifestations?

In the context of sickle cell disease (SCD), what effect does a decrease in blood pH have on hemoglobin's affinity for oxygen and the likelihood of sickling?

What change relating to ionic flow occurs in erythrocytes as they are damaged by sickling in sickle cell disease (SCD)?

What is the primary reason that hematopoietic stem cell transplantation (HSCT) is infrequently performed as a cure for sickle cell disease (SCD)?

What is the significance of macrophage-sheathed capillaries in the context of sickle cell disease (SCD)?

What is the estimated number of individuals worldwide who are heterozygous carriers for the sickle cell trait (HbAS)?

What is the definitive cure for thalassemia major?

For individuals with milder forms of thalassemia who do not require frequent transfusions, what is a potential risk?

A woman with thalassemia intermedia, who has had minimal or no prior blood transfusions, is at risk for what during pregnancy if transfusions become necessary?

What is the underlying cause of the varied clinical manifestations observed in individuals with sickle cell disease (SCD)?

What is the primary genetic characteristic of hemophilia A and hemophilia B?

What percentage of hemophilia cases occur in individuals with no family history of the condition?

Why might clinical manifestations of sickle cell disease (SCD) first appear between 6 to 12 months of age?

Which of the following factors can precipitate a vaso-occlusive crisis in individuals with sickle cell disease?

A patient with hemophilia A has a concentration of clotting factor VIII that is 2% of normal. How would this patient's hemophilia be classified?

An adolescent with sickle cell disease presents with fever, cough, chest pain, and lung infiltrates. Which type of crisis is the MOST likely cause of these symptoms?

Which coagulation factor is deficient in hemophilia B (Christmas disease)?

What is the primary mechanism behind an aplastic crisis in sickle cell disease (SCD)?

Which of the following genetic mutations is most frequently observed in severe cases of hemophilia A?

A child with sickle cell disease (SCD) suddenly develops rapid splenic enlargement, hypovolemia, and shock. Which type of crisis is the MOST likely cause?

A male child is diagnosed with hemophilia A. His parents have no known family history of the disease. What is the most likely explanation for his condition?

What role does factor VIII play in the coagulation cascade?

What laboratory finding is MOST indicative of hyperhemolytic crisis in a child with sickle cell disease?

Which of the following is a potential outcome of ineffective erythropoiesis in milder forms of thalassemia?

Why are children with sickle cell disease (SCD) particularly susceptible to infections from Pneumococcus pneumoniae and Haemophilus influenzae?

What direct effect does increased sphingosine-1-phosphate (S1P) production have on red blood cells in sickle cell disease (SCD)?

Which of the following describes hemophilia C?

What is the earliest manifestation of sickle cell nephropathy in the kidneys?

Why does hemophilia primarily affect males?

How does the polymerization of sickled hemoglobin (HbS) contribute to the pathophysiology of sickle cell disease (SCD)?

How does hemolysis contribute to the development of cholecystitis in individuals with sickle cell disease (SCD)?

What is the primary mechanism by which sickled erythrocytes are removed from circulation, contributing to anemia in sickle cell disease (SCD)?

Which factor deficiency is associated with hemophilia A?

Which of the following best explains why sickle cell–hemoglobin C (HbC) disease is typically milder than sickle cell anemia?

What is the significance of genetic counseling for families with thalassemia?

In sickle cell disease (SCD), microvascular occlusions are NOT directly caused by:

In older children with sickle cell–hemoglobin C (HbC) disease, which of the following complications is MOST likely to occur?

How does the release of hemoglobin from lysed sickle erythrocytes affect vascular function?

How does the presence of normal hemoglobins (HbA and HbF) in individuals with sickle cell–thalassemia impact the sickling process?

Why are microcytic erythrocytes, common in sickle cell disease (SCD), less likely to clog the microcirculation?

How does decreased blood pH affect hemoglobin's (HbS) affinity for oxygen in individuals with sickle cell disease (SCD), and what is the consequence?

What is the underlying cause of proteinuria as an early manifestation of sickle nephropathy?

A child with sickle cell disease presents with painful swelling of the hands and feet. What is the MOST likely diagnosis?

What information does hemoglobin electrophoresis provide when HbS is confirmed in blood?

Why does homozygous inheritance of HbS typically result in a more severe form of sickle cell disease (SCD) compared to heterozygous inheritance?

At what gestational age can prenatal diagnosis for sickle cell disease (SCD) be performed using chorionic villus sampling?

In sickle cell disease (SCD), what is the significance of increased adhesion molecule expression on sickle red blood cells (RBCs) and endothelial cells?

Why are young children with sickle cell anemia (SCA) at a high risk of infection, septicemia, and meningitis?

What is the rationale behind the investigation of microvasculature models in sickle cell disease (SCD) research?

How does sickle cell trait (heterozygous inheritance of SCD) typically manifest clinically?

What is the primary goal of supportive care in the treatment of sickle cell disease (SCD)?

Why is immediate correction of acidosis and dehydration crucial in managing a sickle cell crisis?

What critical role does oxygen tension play in the pathophysiology of sickle cell disease (SCD)?

How does hydroxyurea treatment work in sickle cell disease (SCD)?

What is the primary mechanism by which the drug 5C may offer therapeutic benefits in sickle cell disease (SCD)?

What is the primary mechanism by which antiplatelet autoantibodies cause thrombocytopenia in primary immune thrombocytopenia (ITP)?

What genetic pattern do α- and β-thalassemias follow?

A child diagnosed with ITP has been experiencing symptoms for 10 months without achieving remission. According to the updated definitions, how would this phase of ITP be classified?

What is the underlying cause of the distorted crescent-like sickle shape observed in erythrocytes of individuals with sickle cell disease?

A child with ITP presents with significant bleeding requiring immediate intervention. How would you classify this ITP presentation according to the updated definitions?

Why was thalassemia named after the Greek word for 'sea'?

In sickle cell disease (SCD), what is a key factor that influences the extent, severity, and clinical manifestations of sickling?

What is the most common viral infection implicated in triggering primary immune thrombocytopenia (ITP) in children?

What characterizes the anemia associated with thalassemia?

In sickle cell disease, why do red blood cells remain sickled, even under fully oxygenated conditions?

A child presents with acute onset of bruising, petechiae, and epistaxis one week after recovering from a viral infection. Which condition is most likely?

What genetic defect causes β-thalassemias?

Which physical finding, in addition to bleeding manifestations, is commonly observed in children with primary immune thrombocytopenia (ITP)?

How are mutations in β-thalassemia classified?

A child with suspected ITP has a low platelet count. What additional finding on a peripheral blood smear would support this diagnosis?

What happens to free α chains when β-chain production is depressed in β-thalassemia?

Which diagnostic procedure is generally avoided in children presenting with typical clinical features of ITP?

What is the primary mechanism by which α-thalassemias arise?

What is the consequence of the destruction of precipitate-carrying cells in the spleen in β-thalassemia?

A child with ITP has a platelet count of 15,000/µL but no active bleeding. What is the most appropriate initial management strategy?

What is the most appropriate use of intravenous immune globulin (IVIG) in children newly diagnosed with primary immune thrombocytopenia (ITP)?

Which of the following best describes the underlying cause of the anemia seen in both Hb Bart syndrome and HbH disease?

How does the altered hemoglobin, present in Hb Bart syndrome cases, affect oxygen delivery to fetal tissues?

What is the primary cause of death in individuals with β-thalassemia major who receive regular blood transfusions?

A patient is diagnosed with β-thalassemia minor. Which set of clinical manifestations is MOST likely to be observed?

Which of the following genetic scenarios results in α-thalassemia silent carrier status?

What laboratory finding is most consistent with a diagnosis of thalassemia?

What is the purpose of chelation therapy in the treatment of thalassemia?

Which of the following conditions is characterized by hydrops fetalis in its most severe form?

A patient with thalassemia presents with skeletal deformities including a widened nasal bridge and maxilla. What is the underlying mechanism causing these changes?

Why might serum iron levels be elevated in a person with β-thalassemia minor, even in the absence of iron supplementation?

What is the rationale of increased blood transfusions to treat beta-thalassemia major?

Which of the following diagnostic procedures is used for prenatal screening of α-thalassemia major?

α-Thalassemia trait and β-thalassemia minor share similar clinical presentations. Which of the following is a shared characteristic?

What is the long-term consequence of increased iron absorption in both α and β thalassemia?

What is the underlying cause of ineffective erythropoiesis in patients with beta-thalassemia major?

A 16-year-old presents with a first-time episode of deep venous thrombosis. Genetic testing reveals a mutation affecting the synthesis of a vitamin K-dependent coagulation inhibitor in the liver. Which condition is most likely?

A neonate presents with extensive ecchymosis and skin necrosis shortly after birth. Further investigation reveals the neonate is homozygous for a protein C deficiency. What is the most likely diagnosis?

Which genetic inheritance pattern is associated with protein C deficiency?

A patient is diagnosed with Type I protein S deficiency. What laboratory findings would be expected?

A 20-year-old female with protein S deficiency is considering oral contraceptives. What is the most important consideration regarding her thrombotic risk?

In Type III protein S deficiency, which of the following laboratory results would be expected?

What is the most common mode of inheritance for antithrombin III (AT III) deficiency?

A patient with antithrombin III (AT III) deficiency is undergoing elective surgery. What is the recommended initial treatment to prevent thrombosis?

How do Type I and Type II antithrombin III (AT III) deficiencies differ?

Which of the following is a recognized cause of inherited thrombophilia that is NOT dependent on vitamin K?

Why might a heterozygous individual with protein C deficiency experience a thrombotic event in their late teens or early twenties?

While heparin is often the initial treatment for acute thrombotic events related to Protein C deficiency, what is a significant concern when initiating Warfarin for long-term therapy in these patients?

What is the significance of 'free' versus 'total' protein S levels in diagnosing and classifying Protein S deficiency?

A young patient with a family history of thrombophilia presents with iliofemoral deep vein thrombosis. Testing reveals decreased functional activity of antithrombin III (AT III), but normal antigen levels. Which type of AT III deficiency is most likely?

How does the likelihood of arterial thrombosis differ between Protein C deficiency and ATIII Deficiency?

What is the most likely outcome when a point mutation in a coagulation factor gene results in a nonsense mutation?

Why might a newborn with hemophilia not exhibit excessive bleeding during circumcision?

What is the primary goal of primary prophylaxis in children with severe hemophilia?

How does von Willebrand factor (vWF) contribute to the clotting process?

Why might intracranial bleeds or internal organ hemorrhages be particularly life-threatening complications in individuals with hemophilia?

In evaluating a child for a suspected bleeding disorder, what is the significance of a normal prothrombin time (PT) result alongside a prolonged partial thromboplastin time (PTT)?

What is the underlying cause of inherited thrombophilic conditions?

How can point mutations affect the function of coagulation proteins in hemophilia?

A suspected carrier mother undergoes chorionic villus sampling (CVS) during pregnancy. What is the primary purpose of this test in the context of hemophilia?

A child with hemophilia experiences recurrent bleeding into the joints. What is a potential long-term consequence of this repeated hemarthrosis?

Recent trials have demonstrated the use of pegylated recombinant factors VIII and IX. What is the primary benefit of using these pegylated products compared to traditional recombinant factors?

A patient is diagnosed with von Willebrand disease following an evaluation for prolonged bleeding. What hematologic finding is MOST likely to be present in this patient?

How does the inheritance pattern of von Willebrand disease (vWD) typically present?

Hageman factor (Factor XII) deficiency is described as a condition with a profound laboratory deficiency yet no clinical defects in humans. Why doesn't this deficiency typically result in bleeding disorders?

A child is diagnosed with hemophilia and starts receiving regular infusions of recombinant factor. What is a potential complication of this treatment?