PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC HEMATOLOGY: MCQS

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC HEMATOLOGY: MCQS: MCQ#01 A 9-year-old boy is brought to ER with high fever, poor appetite, and  irritability. His heart rate is 140/min and his blood pre...

PEDIATRIC HEMATOLOGY: MCQS

MCQ#01

A 9-year-old boy is brought to ER with high fever, poor appetite, and irritability. His heart rate is 140/min and his blood pressure is 80/60 mmHg. He has been hospitalized several times before for poorly localized abdominal pain. He also has a history of hematuria. The boy has not received several routine vaccinations because his mother is afraid that they will cause autism. His hematocrit is 22% and the reticulocyte count is 12%. The patient dies several hours after the admission. 

This patient's death may have been prevented by:

A Folic acid supplementation

B. Vaccination with a live attenuated virus

C. Vaccination with a bacterial toxoid

D. Vaccination with a conjugate capsular polysaccharide

E. Periodic blood transfusions


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MCQ#02

A 4-month-old male infant is brought to the office by his parents due to progressive lethargy, poor feeding, fatigue and increasing pallor for the past four weeks. His antenatal and birth histories are unremarkable. His diet consists mainly of breast milk. His immunizations are up-to-date. His mother's blood type is 0 +. Physical examination reveals a webbed neck, cleft lip, shielded chest, triphalangeal thumbs, and pale mucous membranes and conjunctivae. Cardiac auscultation reveals mild tachycardia and a systolic ejection murmur over the left upper sternal border.
The initial investigations reveal the following:
Hb                       8 g/dl
Ht                       26 %
WBCs                  7,000/cmm
Platelets              300,000 /cmm
Reticulocytes       0.4%
MCV                    104 fl
Blood type           A-
Bilirubin direct     0.1 mg/dl
Bilirubin total      1.0 mg/dl
What is the most likely diagnosis?
A. Wiskott-Aidrich syndrome
B. Transient erythroblastopenia of childhood
C. Fanconi's anemia
D. Diamond-Biackfan anemia

E. Rhesus incompatibility


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MCQ#03

An 8-year-old child is brought to the office for the evaluation of a 1-day history of fever and back pain. He has sickle cell disease, and has had 5 hospitalizations
for similar painful crises. His laboratory report shows normocytic anemia, reticulocytosis and leukocytosis. 
What finding is most likely to be present on this patient's peripheral smear?
A. Bite cells
B. Helmet cells
C. Howell Jolly bodies
D. Heinz bodies
E. Basophilic stippling


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MCQ#04

A 7-week-old boy is brought to the clinic for the first time since discharge from the neonatal intensive care unit (NICU). The infant was born at 32 weeks gestation weighing 1800 g (4 1b) following a pregnancy complicated only by preterm labor. In the NICU, he initially had difficulty feeding. By age 6 weeks, he was taking an appropriate volume of fortified preterm formula and was gaining weight well. His parents report no problems since discharge. On examination, the infant is slightly pale. Cardiac auscultation reveals a 2/6 systolic flow murmur, but no tachycardia or gallop. Laboratory results are as follows:
Hemoglobin                       7.8 g/dl
Hematocrit                        24.1%
White blood cells               7,000/cmm
Platelets                           230,000/cmm
Reticulocytes                    0.8%  
The peripheral smear shows normocytic, normochromic red blood cells. 
Which of the following is the most likely diagnosis?
A. Alpha thalassemia
B. Anemia of prematurity
C. Beta thalassemia
D. Glucose 6-phosphate dehydrogenase deficiency
E. Hemolytic disease of the newborn


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MCQ#05
A 7-year-old boy is brought to the emergency department due to abdominal pain and fatigue. He developed abdominal pain, vomiting. and diarrhea a week ago. A few days later. he noticed significant blood in the diarrhea. which has since resolved. His mother believed the patient was improving until he developed diffuse abdominal pain today and "didn't want to get off the couch." Despite drinking a normal amount. he has not urinated in 24 hours. Multiple family members had similar initial gastrointestinal symptoms after attending a family cookout last week, but the mother says that everyone else has recovered. On physical examination. scleral icterus. diffuse abdominal tenderness. and 2+ pedal edema are present. 
Which of the following laboratory values is most likely to be seen in this patient?
A. Decreased ferritin level
B. Decreased platelet count
C. Decreased reticulocyte count
D. Increased prothrombin time
E. Positive direct Coombs test

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PEDIATRIC HEMATOLOGY MCQS: ANSWER TO MCQ 05

MCQ: 05

CORRECT OPTION: 'B'

This patient presents with abdominal pain, fatigue, and decreased urination after a bloody diarrheal illness, which is concerning for hemolytic uremic syndrome (HUS). HUS most commonly occurs after infection with a Shiga toxin produced by a strain of Escherichia coli (01 57:H7). This toxin initially invades and destroys the colonic epithelial lining, producing bloody diarrhea and abdominal pain. Approximately 15% of children then develop the classic triad of HUS: microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (poor urine output, edema, and elevated creatinine and blood urea nitrogen).

The pathophysiology of HUS begins with systemic vascular endothelial injury and subsequent platelet microthrombi formation, which leads to thrombocytopenia and schistocytes as they flow through small vessels (including glomeruli). The resulting hemolytic anemia leads to decreased haptoglobin and elevated bilirubin causing scleral icterus and jaundice. Treatment is supportive (eg, fluid/electrolyte management, blood transfusions, dialysis).

(Choice A) Decreased ferritin levels are seen in patients with iron deficiency anemia. Patients with HUS would be expected to have normal or increased ferritin levels, as ferritin is an acute phase reactant that rises in the setting of inflammation.

(Choice C) Decreased reticulocyte counts in the setting of anemia are typically indicative of defective bone marrow (eg, viral suppression, aplastic anemia, malignancy). Reticulocyte counts are increased in the setting of hemolytic anemias.

(Choice D) Coagulation studies in HUS, including prothrombin time, are typically

normal. Thrombocytopenia and microangiopathic hemolytic anemia can be seen in both HUS a d disseminated intravascular coagulation (DIC), and abnormal coagulation studies can support the diagnosis of DIC.

(Choice E) The direct Coombs test is positive in patients with autoimmune hemolytic anemia. Because red blood cells are mechanically destroyed in HUS, the direct Coombs test will be negative.

Educational objective:

Hemolytic uremic syndrome, most commonly caused by Shiga toxin produced by Escherichia coli strain 0157:H7, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.

PEDIATRIC HEMATOLOGY MCQS: ANSWER TO MCQ 04

MCQ#04

CORRECT OPTION: 'B'

Anemia of prematurity (AOP) affects most preterm infants, and the onset and severity of anemia are proportional to the degree of prematurity. After delivery, circulating erythropoietin (EPO) normally decreases due to increased oxygen concentration in tissue. Decreased EPO causes decreased reticulocyte production in bone marrow. As a result, a physiologic red blood cell (RBC) nadir is expected and occurs at age 2-3 months in term infants. In preterm infants, however, low EPO levels are exacerbated by short RSC life span (40-50 days) and frequent phlebotomy in the neonatal intensive care unit. This can result in a significant, early-onset anemia. Most infants with AOP are asymptomatic. Those who do have symptoms generally have mild tachycardia, increased apnea, or poor weight gain. AOP often is a diagnosis of exclusion; hemolysis, enzyme defects, hemoglobinopathies, and infection should be ruled out. Laboratory studies show decreased hemoglobin and hematocrit and a low reticulocyte count relative to the degree of anemia. The RBCs appear normal under light microscopy.

Treatment includes minimizing blood draws and ensuring adequate iron intake. RBC transfusions can be given if the infant is symptomatic but will further suppress EPO levels and delay recovery. Supplemental EPO is not effective in preventing the need for transfusions.

(Choices A and C) Patients with alpha or beta thalassemia have hypochromic, microcytic RBCs and abnormal cell morphology (eg, target cells) in their peripheral smears.

(Choice D) Glucose 6-phosphate dehydrogenase deficiency can present with severe hemolytic anemia and hyperbilirubinemia. The peripheral smear shows Heinz bodies and bite cells.

(Choice E) Hemolytic disease of the newborn is due to Rh or ABO incompatibility. The anemia (and subsequent hyperbilirubinemia) generally develops within 48 hours of birth. The reticulocyte count is increased due to hemolysis.

Educational objective:

Anemia of prematurity is the most common cause of anemia in preterm infants. It is due to diminished erythropoietin levels, shortened red blood cell life span, and blood loss. Laboratory studies show decreased hemoglobin and hematocrit and a relatively low reticulocyte count.

PEDIATRIC HEMATOLOGY MCQS: ANSWER TO MCQ 03

MCQ#03
CORRECT OPTION:  'C'

Explanation: 

Sickle cell patients usually have infarcted spleens by the first 18 to 36 months of life. Repeated micro-infarctions from clumping of sickle cells can destroy tissues, thereby leading to micro-vascular beds that promote sickling. The peripheral smear characteristically reveals Howell-Jolly bodies. These bodies are nuclear remnants of red blood cells which are generally removed by a functional spleen; therefore, their presence suggests splenectomy or functional asplenia. Functional asplenia renders a patient susceptible to infections, particularly from capsulated organisms such as pneumococci.

In this case, the patient has several years history of sickle cell disease and multiple, previous, similar, painful crises. By this time, these repetitive occlusive events must have already damaged his spleen, leading to functional asplenia. Howell Jolly bodies are most likely to be seen in his peripheral smear.

(Choices A and D) Heinz bodies are aggregates of denatured hemoglobin, and are commonly seen in patients with hemolysis due to G6PD deficiency and thalassemia. When phagocytes extract this rigid precipitate, they form characteristic bite cells.

(Choice B) Helmet cells are fragmented red blood cells. Their presence is suggestive of traumatic hemolytic conditions such as DIC, HUS and TTP.

(Choice E) Basophilic stippling are ribosomal precipitates which appear as blue granules of various sizes dispersed throughout the cytoplasm of the red cell. These are often seen with thalassemias, as well as lead or heavy metal poisoning.

Educational Objective:

Howell-Jolly bodies are nuclear remnants of the red blood cells which are generally removed by a functional spleen; therefore, their presence in a peripheral smear suggests functional asplenia in sickle cell patients.

PEDIATRIC HEMATOLOGY MCQS: ANSWER TO MCQ 02

MCQ#02

CORRECT OPTION: 'D'

Explanation:

Suspect Diamond-Biackfan syndrome (DBS), also called congenital hypoplastic anemia, in a child with macrocytic anemia, low reticulocyte count, and congenital anomalies. The majority of cases are sporadic, although dominant and recessive inheritance is found in 15 percent of cases. The primary pathology is an intrinsic defect of erythroid progenitor cells which results in increased apoptosis (programmed cell death). The condition often presents with pallor in the neonatal period. Over 90 percent of cases are diagnosed within the first year of life, with the average age of diagnosis being 3 months. Congenital anomalies (as described in this case) are present in over 50 percent of cases.

The macrocytic anemia of DBS is distinct from that of megaloblastic anemia because there is no hypersegmentation of the nucleus in neutrophils and other blood cells in the former. Electrophoresis reveals elevated fetal Hb levels. Chromosomal studies are normal. Therapy is mainly corticosteroids. For unresponsive patients, transfusion therapy is indicated.

(Choice A) Wiskott-Aidrich syndrome is an X-linked disorder characterized by eczema, thrombocytopenia and hypogammaglobulinemia.

(Choice B) Transient erythroblastopenia of childhood is a pure red cell aplasia without macrocytosis. Most cases are diagnosed after 1 year of age and there are no associated congenital anomalies.

(Choice C) Fanconi's anemia is an autosomal recessive disorder characterized by

progressive pancytopenia and macrocytosis. The average age at diagnosis is 8 years. Associated deformities include cafe-au-lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys and absent thumbs (not triphalangeal thumbs).

(Choice E) The mother in this vignette is rhesus-positive; therefore, there is no risk of rhesus incompatibility for the baby.

Educational Objective:

DBS is a macrocytic pure red aplasia associated with several congenital anomalies such as short stature, webbed neck, cleft lip, shielded chest and triphalangeal thumbs.

PEDIATRIC HEMATOLOGY MCQS: ANSWER TO MCQ 01

MCQ#01

CORRECT OPTION      'D'

Explanation: 

This patient's history and presentation are consistent with sickle cell anemia complicated by an acute bacterial infection. We can assume he has sickle cell anemia based upon his anemia with a high reticulocyte count, previous admissions for pain, hematuria, and ethnicity. Patients with sickle cell anemia become functionally hyposplenic at an early age due to splenic auto-infarction. Thus, they are more susceptible than other patients to infection with encapsulated organisms, such as S. pneumoniae, H. influenzae, and N. meningitidis. Vaccination with the conjugated S. pneumoniae vaccine decreases the incidence of invasive infections caused by this organism. Twice daily administration of prophylactic penicillin should also be given to children with sickle cell disease until they reach five years of age.

(Choice A) Folic acid supplementation has often been recommended for patients

suffering from sickle cell anemia. This practice has recently come into question over fears that folic acid supplementation may conceal vitamin 812 deficiency. However, regardless of this controversy, folic acid administration would not have prevented this patient's infection.

(Choice B) The common vaccinations of childhood that use live attenuated viruses are those for measles, mumps, rubella, and chicken pox. 

(Choice C) The common vaccinations of childhood that use bacterial toxoids include tetanus and diphtheria. This patient is not presenting with signs or symptoms consistent with tetanus or diphtheria.

(Choice E) Periodic blood transfusions do not decrease the incidence of infection in patients with sickle cell anemia.

Educational Objective:

Pneumococcal vaccination plus penicillin prophylaxis can prevent almost all cases of pneumococcal sepsis in patients with sickle cell anemia.

PEDIATRIC HEPATOLOGY: MCQS

QUESTION#01

A 10-year-old child with severe acute liver failure is being cared for in the intensive care unit.

Which of the following laboratory findings best reflects worsening liver function in this patient?

A. Uptrending AST
B. Uptrending ALT
C. Hyperglycemia
D. Uptrending total bilirubin
E. Uptrending factor VII

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QUESTION#02

A 7-year-old girl is admitted with new-onset elevated serum aminotransferases and jaundice; gallstones within the gallbladder and dilation of the common bile duct are visualized on initial right upper quadrant ultrasound, as well as single stone within the common bile duct. 

Which is the next most appropriate evaluation of the hepatobiliary system to pursue?

A. Computed tomography (CT)

B. Liver biopsy

C. Percutaneous transhepatic cholangiography (PTC)

D. Hepatobiliary iminodiacetate (HIDA) scan

E. Endoscopic retrograde cholangiopancreatography (ERCP)

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QUESTION#03

A 7-year-old female is admitted to the pediatric general care floor with jaundice and fatigue. Laboratory evaluation reveals elevated AST, ALT, bilirubin, prolonged INR, IgA deficiency, and a positive anti–liver kidney microsomal type 1 (LKM-1) antibody titer. 

Which of the following is the most likely diagnosis?

A. Primary sclerosing cholangitis

B. Lupus

C. Type I autoimmune hepatitis (AIH)

D. Type II AIH

E. Intrahepatic cystic biliary dilation (Caroli disease)

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QUESTION#04

Treatment options are considered for a 14-year-old female with newly diagnosed type I autoimmune hepatitis who has not yet started on any therapy.

Which of the following is the best choice for initial treatment in this patient?

A. Prednisone

B. Cyclosporine

C. Mycophenolate

D. Tacrolimus

E. Azathioprine

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QUESTION#05

A 3-year-old girl presents to the emergency room after her mother discovered her on the bathroom floor with an open, mostly empty bottle of acetaminophen tablets. N -Acetylcysteine is administered and she is admitted to the intensive care unit for observation. 

Although each of the following might be considered appropriate investigations during the course of her illness, which is most useful for monitoring the synthetic function of this patient’s liver?

A. Serum alanine aminotransferase

B. Prothrombin time (PT)

C. Ultrasound of the abdomen

D. Serum alkaline phosphatase

E. Acetaminophen level

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PEDIATRIC HEPATOLOGY MCQS: ANSWER TO MCQ 05

Answer 5 B

Although commonly referred to as “liver function tests,” the majority of serum tests measure the enzymes that are produced within the hepatocytes or biliary system but are not measures of physiologic function.

Serum alanine aminotransferase is a marker of hepatocellular injury and may be markedly elevated after exposure to hepatotoxins such as acetaminophen. Serum alkaline phosphatase may be elevated in cholestasis and also in conditions unrelated to hepatic function such as bone injury or growth. Ultrasound of the liver is useful for detecting changes in echotexture that may be due to infiltrative diseases, as well as cystic or solid tumors. It is not useful for evaluating hepatic function.

Conjugated bilirubin, glucose, ammonia, albumin, PT/international normalization ratio (INR), factor V, factor VII, and fibrinogen are all markers of hepatic synthetic function.

Prolonged PT and INR may be seen in both acute and chronic liver injury and, when present, suggest liver dysfunction.

PEDIATRIC HEPATOLOGY MCQS: ANSWER TO MCQ 04

Answer 4 A

Autoimmune hepatitis responds very well to steroid therapy. The recommended initial therapy for AIH is 2 mg/kg/day of prednisone. Once transaminases are below 100, prednisone can be weaned to low dose. Once bilirubin levels have normalized, azathioprine may be started as maintenance therapy, preferably in concert with low-dose steroids to prevent initial relapse.

In patients who are intolerant of both corticosteroids and azathioprine, cyclosporine, tacrolimus, or mycophenolate may be considered.

PEDIATRIC HEPATOLOGY MCQS: ANSWER TO MCQ 03

Answer 3. D

This patient most likely has type II AIH, which is characterized by positive anti-LKM-1 antibody and/or anti-liver cytosol-1 antibody. Compared with patients with type I AIH, patients with type II AIH tend to present at a younger age and to have a higher incidence of partial IgA deficiency and acute liver failure at presentation (which is suggested by this patient’s coagulopathy).

Type I AIH is more common than type II AIH and is characterized by positivity for antinuclear antibody (ANA), anti–smooth muscle antibody (SMA), or anti–F-actin antibodies. Primary sclerosing cholangitis is an autoimmune inflammatory condition targeting the extrahepatic bile ducts that may be associated with other autoimmune disorders such as AIH, inflammatory bowel disease, or thyroiditis. Patients with primary sclerosing cholangitis are usually ANA, SMA, and LKM-1 negative, although these values are occasionally positive in patients with overlap syndrome, in which AIH and PSC occur together. Patients with lupus may have an elevated ANA titer, which is also seen in patients with AIH, but typically do not present with positive LKM-1 and acute liver failure. Caroli disease is a disease that presents with hyperbilirubinemia that is not associated with positive LKM-1 or IgA deficiency.

PEDIATRIC HEPATOLOGY MCQS: ANSWER TO MCQ 02

Answer 2. E

In a child with cholestasis due to extrahepatic biliary obstruction by gallstones clearly visualized on ultrasound, it is most appropriate to pursue evaluation with ERCP, which not only provides excellent images of the intrahepatic and extrahepatic biliary tree but also allows for endoscopic interventions including stone removal.

CT is useful for 3-dimensional imaging of the hepatobiliary tree but exposes the patient to ionizing radiation and does not offer the therapeutic opportunity that ERCP does. A liver biopsy is useful for evaluation of microscopic hepatic structure and would not be useful for this patient with biliary obstruction of known cause. PTC allows visualization of the biliary tract by transhepatic injection of contrast into the ductal system and is useful especially for visualization of ducts not accessible by ERCP; it does not offer therapeutic intervention as ERCP does. HIDA scan is useful in evaluating infants with cholestatic liver disease or gallbladder structure and function in older individuals.

PEDIATRIC HEPATOLOGY MCQS: ANSWER TO MCQ 01

Answer 1. D

A rising total bilirubin in the setting of acute liver failure with loss of hepatic function is an ominous sign that is likely multifactorial in nature. This may be due to reduced hepatic uptake, decreased bile conjugation, and/or decreased biliary excretion as hepatocyte death progresses.

Serum aminotransferase elevation is usually secondary to hepatocellular injury due to inflammation, toxin, or passive congestion, although there are extrahepatic causes of elevated transaminases as well. Aminotransferases levels may remain stable or trend downward even as bilirubin rises as liver failure and hepatocellular dealt progress, so uptrending AST/ALT do not necessarily reflect loss of hepatic function. Hypoglycemia, not hyperglycemia, may occur as hepatic dysfunction progresses due to loss of glycogen stores and gluconeogenesis within the liver. Factor VII is a clotting protein synthesized in the liver that, when levels decline to less than 12% of normal, may predict fulminant hepatic failure.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Question#01A healthy, 39-week gestation male wei...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Question#01

A healthy, 39-week gestation male wei...: Question#01 A healthy, 39-week gestation male weighing 3.5 kg is born to a  mother who has chronic anemia. The infant’s physical examina...

FCPS/MRCPH PEIATRICS MCQS,

Question#01

A healthy, 39-week gestation male weighing 3.5 kg is born to a mother who has chronic anemia. The infant’s physical examination findings are normal, and his hematocrit is 49% (0.49). The mother asks you about the need for vitamins and iron for her newborn son.

Of the following, the BEST response is that term babies need to begin iron therapy at

A. Birth

B. 2 weeks of age

C. 4 months of age

D. 9 months of age

E. No time

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Question#02

A 12-year-old boy comes to the clinic for a physical examination prior to attending camp. He is generally healthy. He has a reading disability and has been diagnosed with attention-deficit disorder, for which he takes medication. On physical examination, his height is 167 cm (>97th percentile), weight is 45 kg (75th percentile), and head circumference is 54 cm (50th percentile). The arm span-to-height ratio is greater than the 97th percentile, and the upper-to-lower segment ratio is less than the 10th percentile. He has five café au lait macules measuring 5 mm or more. He is prepubertal. The remainder of the physical examination findings are unremarkable. He seems shy and does not establish eye contact with you during the examination.
Of the following, the MOST appropriate next step toward providing comprehensive care for this boy is to
A. arrange for echocardiography
B. order magnetic resonance imaging of the head
C. refer him to an ophthalmologist
D. request renal ultrasonography

E. send blood for chromosome analysis

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Question#03

You are reviewing the many different formulas available for infant feeding with a group of pediatric residents.
Of the following, the metabolic disease for which feeding with a commercial soy infant formula is MOST appropriate is
A. Galactosemia
B. Hereditary tyrosinemia type 1
C. Maple syrup urine disease
D. Phenylketonuria

E. Propionic acidemia


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Question#04

You are called to the nursery to evaluate a newborn who was diagnosed in utero with pulmonary atresia and ventricular septal defect. On physical examination, she is in no distress, alert, and breathing comfortably at 40 breaths/min. Aeration of the lungs is adequate. Cardiac auscultation reveals a normal S1 and a single S2 sound, and there are no murmurs. Her pulses and perfusion are good, although she has cyanosis of the hands, feet, and perioral area. Her oxygen saturation in room air is 75%.
Of the following, the MOST likely cause of her cyanosis is
A. increased levels of fetal hemoglobin
B. left-to-right shunting at the atrial level
C. methemoglobinemia
D. right-to-left shunting at the ductus arteriosus

E. right-to-left shunting at the ventricular level

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Question#05

You diagnose necrotizing enterocolitis in a preterm neonate who has abdominal distention and blood in the stool. You decide that this infant should be placed on a 14-day regimen of parenteral nutrition.
Of the following, the micronutrient for which weekly monitoring is MOST recommended during this infant’s parenteral nutrition therapy is
A. copper
B. iron
C. phosphorus
D. selenium

E. zinc


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Correct Answer to Question#05

Question#05

You diagnose necrotizing enterocolitis in a preterm neonate who has abdominal distention and blood in the stool. You decide that this infant should be placed on a 14-day regimen of parenteral nutrition.

Of the following, the micronutrient for which weekly monitoring is MOST recommended during this infant’s parenteral nutrition therapy is

A. copper

B. iron

C. phosphorus

D. selenium

E. zinc

Correct Option to this  Question is 'C'

Explanation:

Parenteral nutrition (PN), the intravenous administration of carbohydrates, lipids, amino acids, and micronutrients, is an important component in the management of a variety of chronic disorders, including surgical conditions (eg,

omphalocele, gastroschisis, diaphragmatic hernia, short bowel syndrome), inflammatory conditions (eg, Crohn disease, ulcerative colitis, pseudomembranous colitis, pancreatitis, graft versus host disease), hypermetabolic states (eg, burns, trauma), and intestinal motility disorders (eg, pseudo-obstruction). PN is especially important in the support of very low-birthweight infants, who frequently have increased caloric requirements, decreased oral intake, and immature intestinal motility.

In addition to glucose, amino acids, sodium, potassium,

and chloride, PN provides additional mineral supplements, including calcium, phosphorus, magnesium, zinc, copper, selenium, chromium, manganese, molybdenum, and iodide. In preterm infants, the administration of both calcium and phosphorus is important to prevent metabolic bone disease.

However, the amount of calcium and phosphorus that can be administered in PN must be limited because of the risk of precipitation in the PN and formation of calcium-phosphorus complexes that could embolize. It generally is thought that

the chance of precipitation is high if the product of the concentrations (in mmol/L) of the calcium and phosphorus in PN is greater than 40. Because hypophosphatemia can have significant consequences (including impaired cardiac function, muscle weakness, and hemolysis), it is recommended that serum phosphorus concentrations be assessed at least once weekly for patients receiving PN. The serum concentrations of copper, iron, selenium, and zinc, which are trace elements, require only periodic assessment (eg, once a month or less).

Correct Option of Question#04

Question#04

You are called to the nursery to evaluate a newborn who was diagnosed in utero with pulmonary atresia and ventricular septal defect. On physical examination, she is in no distress, alert, and breathing comfortably at 40 breaths/min. Aeration of the lungs is adequate. Cardiac auscultation reveals a normal S1 and a single S2 sound, and there are no murmurs. Her pulses and perfusion are good, although she has cyanosis of the hands, feet, and perioral area. Her oxygen saturation in room air is 75%.

Of the following, the MOST likely cause of her cyanosis is

A. increased levels of fetal hemoglobin

B. left-to-right shunting at the atrial level

C. methemoglobinemia

D. right-to-left shunting at the ductus arteriosus

E. right-to-left shunting at the ventricular level

Correct Option is 'E'

Explanation:

Cyanosis (ie, bluish discoloration of the skin and mucous membranes) in the newborn may arise from a number of different conditions. Among these are pulmonary pathologies, congenital cardiovascular malformations, persistent pulmonary hypertension of the newborn, and disturbances of the hematologic and metabolic systems. Right-to-left shunting can be thought of as diversion of the desaturated blood away from the lungs and to the systemic circulation.

This can occur because blood does not perfuse the ventilated portions of the lung (intrapulmonary right-to-left shunting), does not perfuse the pulmonary artery from the heart (intracardiac right-to-left shunting), or is diverted away from the pulmonary circuit through the ductus arteriosus (extracardiac right-to-left shunting).

Methemoglobinemia is a rare condition in which the heme iron has been oxidized to the ferric state and no longer can bind oxygen reversibly. Enzyme systems reduce this oxidized hemoglobin and prevent accumulation in the normal state.

When abnormalities exist that limit this reduction, the oxidized form of heme increases, and when blood levels of methemoglobin exceed 15 g/L, the blood appears chocolate brown and the skin has a slate gray cyanotic appearance, even though the oxygen saturation may be normal. True hypoxia may occur at higher levels of methemoglobin.

For the neonate described in the vignette, the cyanosis results from right-to-left shunting at the ventricular level because she has pulmonary atresia and cannot deliver blood from her right ventricle into her lungs. All of the desaturated blood entering her right ventricle, therefore, must exit through the ventricular septal defect, thereby entering the left ventricle and aorta during systole.

The infant may, in fact, have increased levels of fetal hemoglobin, but this would serve to improve oxygen delivery, not cause cyanosis. Left-to-right shunting at the atrial level would not necessarily be expected in this infant, and even if it did occur, delivery of oxygenated left atrial blood to the right side would not produce cyanosis. Patients who have pulmonary atresia require an alternate source of pulmonaryblood flow, which may occur via the ductus arteriosus in a left-to-right flow pathway or through multiple aortopulmonary collateral arteries. Flow cannot occur from right to left in these patients because there is an obligate left-to-right shunt of blood from the system into the otherwise empty pulmonary vascular bed. In fact, when the ductus arteriosus is the only source of pulmonary blood flow in such patients, maintenance with prostaglandin E is vital to maintain pulmonary blood flow and, thus, oxygenation prior to surgical repair.

Correct option of Question#03

You are reviewing the many different formulas available for infant feeding with a group of pediatric residents.

Of the following, the metabolic disease for which feeding with a commercial soy infant formula is MOST appropriate is

A. Galactosemia

B. Hereditary tyrosinemia type 1

C. Maple syrup urine disease

D. Phenylketonuria

E. Propionic acidemia

Correct option is 'A'

Explanation: 

The improvements in the development and manufacture of infant formulas now enable physicians to treat a wide variety of chronic illnesses with specialized formulas. For example, because galactosemia is caused by defective conversion of galactose to glucose, a formula in which galactose is absent (eg, conventional soy-based formula, which does not contain lactose) should be fed to affected infants. In addition, a number of specialized formulas that have modified amino acid compositions have been developed to treat specific metabolic illnesses. These include formulas for phenylketonuria, hereditary tyrosinemia, homocystinuria, maple syrup urine disease, urea cycle disorders, and organic acidemias. Each of these metabolic disorders requires a formula that has a different composition of amino acids.

Specialized formulas also are used to treat atopic disease. Infants who have cow milk protein allergy (allergic colitis or eczema) may be treated with either a casein hydrolysate formula or an amino acid-based formula. Although some infants who have cow milk protein allergy can tolerate a soy protein-based formula, soy formulas generally are not recommended for these infants because of the high incidence of cross-reactivity.