NOTCHTOP 2023 Flashcards
Jessica, an 8-year-old girl had on and off painless, cervical lymphadenopathy. What does NOT support the decision to perform lymph node biopsy earlier?
A. Lymph node greater than 2 cm
B. Infection in the region drained by the lymph node
C. Supraclavicular lymph node adenopathy
D. Lymphadenopathy not resolving within 4-8
weeks
B. Infection in the region drained by the lymph node
Choice B would be the best answer since an enlarged lymph node can be expected in areas with infection and does not warrant immediate biopsy.
Red flags in cases of cervical lymphadenopathy would include:
Lymphadenopathy >2cm (Choice A), steady increase in size over 2-3 weeks, no improvement or decrease in size after 4-6 weeks (Choice C), supraclavicular lymphadenopathy (Choice B), hard fixed matted non-tender lymphadenopathy, persistent fever lasting more than one week, signs and symptoms suggestive of malignancy or autoimmune disease, features suggestive of Kawasaki disease, abnormalities in CBC or chest radiography.
What is done for patients diagnosed with lymphoma with an associated mediastinal disease?
A. Abdominal surgery is avoided
B. Tumor marker (LDH) is monitored
C. General anesthesia must be avoided
D. Intestinal resection avoided.
B. Tumor marker (LDH) is monitored
According to the International Pediatric Non-Hodgkin Lymphoma Staging System (IPNHLSS), any intrathoracic tumor (mediastinal, hilar, pulmonary, pleural, or thymic) is already considered Stage III. For the question, we can diagnose the patient with having a Stage III Non-Hodgkin Lymphoma since there is already an associated mediastinal disease.
The monitoring for NHL includes serum LDH.
The primary modality of treatment for childhood and adolescent NHL is multiagent systemic chemotherapy and/or immunotherapy with intrathecal chemotherapy while surgery is used mainly for diagnosis. There is no mention of contraindications for abdominal surgery (Choice A), general anesthesia (Choice B), or intestinal resection (Choice C).
Reed-Sternberg Cells seen in what condition?
A. Malignant Teratoma
B. Nephroblastoma (Wilms tumor)
C. Acute Lymphoblastic Leukemia
D. Hodgkin’s Lymphoma
D. Hodgkin’s lymphoma
Hodgkin/Reed-Sternberg (HRS) cell is a collective term for classic Reed-Sternberg (RS) cells and characteristic variant cells that are referred to as Hodgkin cells. They are germinal center B cells that have undergone malignant transformation.
Malignant teratoma (Choice A) is a benign tumor of the ovary composed of mature tissue representing at least 2 embryonic layers. Nephroblastoma/ Wilms tumor (Choice B) consists of blastemal, epithelial and stromal elements. Acute Lymphoblastic Leukemia (Choice C) consists of blasts of moderate size with soft fine chromatin and a scant amount of basophilic cytoplasm.
Tumor markers elevated in patients with neuroblastoma is ________.
A. Human chorionic gonadotrophin (HCG)
B. Alpha-feto protein (AFP)
C. Carcinoembryonic antigen (CEA)
D. Vanillylmandelic acid (VMA)
D. Vanillylmandelic acid (VMA)
• Vanillylmandelic acid is used to rule out neuroblastoma.
• Alph-afeto protein (Choice B) is used for germ cell tumors and hepatoblastomas. Elevated levels for Wilms tumors or nephroblastoma can serve as a marker of disease progression or recurrence.
• Human chorionic gonadotrophin (Choice A) as a tumor marker is for seminomatous and non-seminomatous testicular tumors, ovarian germ cell tumors, hydatidiform mole, choriocarcinoma, and non-testicular teratomas
• Carcinoembryonic antigen (Choice C) is for colon, rectum, prostate, ovary, lung, thyroid, or liver carcinomas.
What is considered as a Stage 1 Wilms tumor?
A. Tumor limited to the kidney
B. Tumor extended beyond the kidney but completely excised
C. Lymph node metastasis
D. Tumor involved blood vessels
A. Tumor limited to the kidney
• Children’s Oncology Group Staging of Wilms Tumor
o Stage I Wilms tumor – tumor is completely contained within the kidney without any breaks or spillage outside the renal capsule and no vascular invasion. Accounts for 40% to 45% of all Wilms tumors.
o Stage II-Tumor extends beyond the kidney but is completely resected with negative margins and lymph nodes. At least 1 of the following has occurred: penetration of renal capsule and/or invasion of renal sinus vessels. (Choice B, D)
o StageIII-Residual tumor present following surgery confined to the abdomen
o Stage IV - Hematogenous metastases (lung, liver, bone, brain, etc.) or lymph node metastases outside the abdominopelvic region. (Choice C)
o Stage V - Bilateral renal involvement by tumor.
What is NOT a genetic predisposition in Wilms tumor?
A. Aniridia
B. Beckwith-Wiedmann syndrome
C. Down syndrome
D. Denys-Drash syndrome
C. Down Syndrome
• In 10% of cases, Wilms tumor occurs as a part of a multiple malformations, including WAGR (Choice A), Denys-Drash (Choice D), and Beckwith-Wiedemann syndrome (Choice B). It is not known to be associated with Down Syndrome.
•WAGR Syndrome stands for Wilms tumor, aniridia, genitourinary malformations, and a range of mental disabilities.
Aside from staging, what is another prognosticating factor for Wilms tumor?
A. Tumor size
B. Symptoms
C. Age
D. Sex
C. Age
• Prognostic factors at the time of diagnosis are associated with increased risk of tumor recurrence or death and include:
o Tumor histology
o Tumor stage
o Molecular and genetic markers (loss of heterozygosity [LOH] at chromosome 16q, 1p, and 11p15 and 1q gain)
o Age >2 years (Choice C)
• Tumor size (Choice A), symptoms (Choice B), or sex (Choice D) are not considered prognosticating factors for Wilms tumor.
Histologically, what is NOT a component of a nephroblastoma?
A. Blastema
B. Epithelium
C. Stromal
D. Cord cells
D. Cord cells
Nephroblastoma or Wilms tumor is comprised of three cell types:
o Blastemal cells (Choice A) – Undifferentiated cells
o Stromal cells (Choice C) – Immature spindle cells and heterologous skeletal muscle, cartilage, osteoid, or fat
o Epithelial cells (Choice B) – Glomeruli and tubules
What are the two important parameters that aid in determining the type of initial feeding?
A. Weight and clinical stability
B. Weight and birth length
C. Absence of tachypnea and birth asphyxia
D. Gestation and absence of tachypnea
A. Weight and clinical stability
• Weight is an important parameter to determine the type of initial feeding because it will direct a clinician if the neonate would need additional caloric requirement for weight gain.
• Clinical stability is another important parameter that we should consider because if a neonate cannot consume milk per orem if they are unstable. For those cases, we would consider using parenteral nutrition.
• Choices B, C, and D - The absence of tachypnea, absence of birth asphyxia, or birth length are not the best answer since these factors will not help determine whether or not a neonate will be able to tolerate oral feeding or if they would require additional nutrition.
What is NOT a component of normal development of the heart as a child is born and grows?
A. Systemic arterial pressure increases slowly
B. Pulmonary arterial pressure decreases
C. Right ventricle wall thickens
D. Left ventricle wall thickens
C. Right ventricle wall thickens
• At birth, the right ventricle is heavier than the left ventricle. As the child grows, the right ventricle is connected to the low resistance of pulmonary circulation, therefore, its wall thickness and mass will decrease.
• Systemic vascular resistance increases due to the removal of the low-resistance placenta. As vascular resistance increases, so does pressure (Choice A).
• The decrease in pulmonary vascular resistance leads to an increase in pulmonary blood flow and decrease in pulmonary artery pressure (Choice B)
• As the systemic vascular resistance increases, there would be an increase in the force of contractility on the left side leading to left ventricle wall thickening (Choice D)
What conditions results in a narrow split second heart sound (S2)?
A. Right bundle branch block
B. Anomalous pulmonary venous return
C. Pulmonary valve stenosis
D. Pulmonary hypertension
D. Pulmonary hypertension
• Pulmonary hypertension – S2 may be narrowly split (or single), with an increased pulmonic component.
• (Choice A) Right bundle branch block – wide S2
• (Choice C) Pulmonary stenosis – ejection murmur
• (Choice B) TAPVR – systolic murmur at LSB in mild cases
When obtaining BP in a pediatric patient, the following are true, EXCEPT ______.
A. Undersized or smaller cuff may cause a falsely lowered blood pressure.
B. Bladder length >80% of the upper arm circumference
C. Patient should be in a quiet and resting state.
D. Systolic BP in the legs is 10-20% higher than the arms
A. UNDERSIZED OR SMALLER CUFF MAY CAUSE A FALSELY LOWERED BLOOD PRESSURE.
• Small cuff will result in a falsely higher BP while a large cuff would result in a falsely lower BP
• When measuring the BP of a pediatric patient, they should be seated 3-5 mins before measurement (Choice C), the correct cuff size should be used where in the bladder length >80% of the upper arm circumference (Choice B) and the bladder width >40% of the upper arm circumference, the lower part of the cuff is placed 2-3 cm above the antecubital fossa, the bell of the stethoscope is placed over the brachial artery.
• The BP of all extremities must be taken to rule out congenital cardiac diseases such as coarcation of the aorta. The systolic BP in the legs is 10-20% higher than the arms (Choice D).
Split S2 can be heard normally in children due to the asynchronous closure of which valves?
A. Aortic and Mitral
B. Mitral and Tricuspid
C. Aortic and Pulmonic
D. Tricuspid and Pulmonic
C. Aortic and pulmonic
• The first heart sound is associated with the closure of the mitral and tricuspid valves (Choice B) while the second heart sound is associated with the aortic and pulmonic valves (Choice C).
• The splitting of the second sound comes from vibrations in the aortic valve cusps and in the walls of the great vessels and their respective ventricles – the aortic and pulmonic valves.
• Choices A and D are incorrect since the paired valves are not expected to close simultaneously in any part of the cardiac cycle.
What clinical finding is NOT common in patients with biliary atresia?
A. Kernicterus
B. Pale stool
C. Dark urine
D. Jaundice
A. Kernicterus
Signs and symptoms of Biliary Atresia (BA):
o Jaundice is the first sign of biliary atresia (Choice D)
o Acholic stools often go unrecognized because stools are pale but not white and the stool color can vary (Choice B)
o Dark urine because of bilirubin excretion via the urine (Choice C)
• Kernicterus is not a common symptom of biliary atresia, rather it is a complication that may occur.
What is the definitive management of patients with biliary atresia?
A. Phototherapy
B. Antibiotics
C. Kasai operation
D. Exchange transfusion
C. Kasai operation
• If biliary atresia is confirmed via cholangiogram, a Kasai procedure or hepatoportoenterostomy should be performed. This operation attempts to restore bile flow from the liver to the proximal small bowel.
• Although phototherapy (Choice A) helps in cases of jaundice, it is not a definitive management for patients with an anatomic problem as the cause of jaundice, such as in biliary atresia. Exchange transfusion (Choice D) can also be done for patients with unconjugated hyperbilirubinemia but is not considered as a definitive management for biliary atresia. Antibiotics (Choice B) can be given pre-operatively as prophylaxis prior the Kasai procedure but will not cure the anatomic abnormality.
Which factor makes gastroenteritis common in children?
A. Improved immunity
B. Poor hand hygiene practices
C. Clean drinking water
D. Access to soap and water
B. Poor hand hygiene practices
Organisms that can cause gastroenteritis can be transmitted via the feco-oral route, therefore children with poor hygiene have an increased risk. Other choices are preventive measures.
Which of the following drugs can be useful in decreasing abdominal pain associated with gastroenteritis?
A. Metoclopramide
B. Butyl-scopolamine
C. Omeprazole
D. Ondansetron
B. Butyl-scopolamine
• Butyl-scopolamine is a peripherally acting antimuscarinic, anticholinergic agent and used to treat pain and discomfort caused by abdominal cramps, menstrual cramps, or other spasmodic activity in the digestive system.
• Metoclopramide (Choice A) block D2 receptors and is a prokinetic agent increase gastric emptying and intestinal motility
• Omeprazole (Choice C) is a proton pump inhibitor susceptible to destruction by gastric acid, which is why it is administered as enteric-coated granules that are absorbed in the duodenum.
• Ondansetron (Choice D) is a 5HT3 antagonist that blocks the chemoreceptor trigger zone and enteric nervous system 5HT3 receptors and it is used in post-chemotherapy and post- operative nausea and vomiting.
What is an example of an infectious causes of gastroenteritis?
A. Lactose
B. Rotavirus
C. Crohn’s disease
D. Gluten
B. Rotavirus
Rotavirus is the most common cause of infectious acute gastroenteritis among children in the world. Other options are no infectious causes.
What is NOT a likely clinical manifestation of GERD in babies?
A. Failure to thrive
B. Chronic cough in children
C. Distressed behavior
D. Overfeeding
D. Overfeeding
Manifestations of GERD in infants include regurgitation, excessive crying, irritability (Choice C), vomiting, food refusal, persistent hiccups, abnormal posturing, failure to thrive (Choice A), and impaired quality of life. For children, GERD presents as esophagitis, respiratory symptoms, or chronic cough (Choice B).
What is NOT a common presentation of an infant with tracheoesophageal fistula?
A. Scaphoid abdomen
B. Maternal polyhydramnios
C. Copious saliva with episodes of choking
D. Cyanosis with onset of feeding
A. Scaphoid abdomen
• If a fistula is present between the esophagus and the trachea, abdominal distention develops as air builds up in the stomach – therefore, the infant’s abdomen will be scaphoid (Choice A) if no fistula exists.
• Common findings in patients with tracheoesophageal fistula:
o Maternal Polyhydramnios (Choice B)
o Aspiration pneumonia
o Excessive salivation (Choice C)
o Early-onset respiratory distress (Choice D)
o Nasogastric tube cannot be placed in stomach
o Feeding exacerbates the symptoms, causes regurgitation, and precipitates aspiration
A newborn old baby is brought in for a chief complaint of progressive worsening of projectile vomiting, non-bilious, that occurs after feeding. Abdominal examination revealed an “olive-shaped mass” in the abdomen. What will be the expected finding on ultrasound?
A. Diaphragmatic hernia
B. Omphalocele
C. Biliary atresia
D. Pyloric stenosis
D. Pyloric stenosis
• Keywords in the case would be non-bilious vomiting after eating and olive-shaped mass. The case is referring to pyloric stenosis. The mass described is firm, movable, usually 2 cm in length, olive shaped, hard, palpated above and to the right of the umbilicus in the mid epigastrium beneath the liver’s edge.
• (Choice A) Diaphragmatic hernia would present as respiratory distress, cyanosis, decreased breath sounds on the affected side, displaced heart sounds, and scaphoid abdomen.
• (Choice B) Omphalocele would present with an anterior midline abdominal mass at the site of the umbilical cord insertion that is covered by a membrane.
• (Choice C) Biliary atresia presents as persistent jaundice, acholic stools, and hepatomegaly.
What is NOT a possible complication of pyloric stenosis?
A. Metabolic alkalosis
B. Low chloride resulting in impaired kidney excretion of bicarbonate
C. Hyperventilation due to metabolic alkalosis
D. Secondary hyperaldosteronism from decreased blood volume
C. HYPERVENTILATION DUE TO METABOLIC ALKALOSIS
• Hyperventilation should be a result of metabolic acidosis not metabolic alkalosis; therefore, best choice for this item is C.
• As vomiting continues in patients with pyloric stenosis, a progressive loss of fluid, hydrogen and chloride leads to hypochloremic metabolic alkalosis (Choice A, B). The compensatory response to the metabolic alkalosis is hypoventilation resulting in an elevated arterial pCO2.
• Secondary hyperaldosteronism can occur from decreased blood volume (Choice C) causing the kidneys retain Na+ (to correct the intravascular volume depletion), and excrete increased amounts of K+ into the urine (resulting in a low blood level of potassium).
What is the recommended surgical procedure for patients with hiatal hernia that do not respond to medical management?
A. Gastric bypass
B. Roux-and-Y anastomosis
C. Kasai procedure
D. Fundoplication
D. FUNDOPLICATION
• Fundoplication is an anti-reflux procedure following repair of the hernia defect wherein the sphincter is tightened at the top of the stomach to prevent acid from rising into the esophagus.
• Kasai procedure (Choice C) is done for biliary atresia. Roux-en-Y anastomosis gastric bypass is an end-to-side surgical anastomosis of bowel used to reconstruct the gastrointestinal tract used for weight loss (Choice A, B).
Lactose intolerance presents with feeling bloated, episodes of diarrhea, and nausea when milk is consumed because of what underlying pathology?
A. IgA deficiency
B. Abnormal immune response to milk
C. Deficiency in lactase
D. Allergy to milk
C. Deficiency in lactase
• In patients with lactase deficiency, the intestinal brush border lactase enzyme activity is lower, causing malabsorption.
• Patients with IgA deficiency (Choice A) are usually symptomatic but if severe, complications for may include asthma, diarrhea, ear and eye infections, autoimmune diseases, and pneumonia. Abnormal immune response to milk or an allergy to milk (Choice B, D) would present with more systemic findings rather than GI- specific symptoms.
In what condition is the cardiac apex displaced outwards and downwards towards the diaphragm?
A. Right ventricular hypertrophy
B. Right atrial enlargement
C. Left ventricular hypertrophy
D. Left atrial enlargement
C. Left ventricular hypertrophy
• Left ventricular hypertrophy presents with a outward and downward displacement or a “Sagging breast” or “Drooping/ Heavy heart”.
• Right ventricular hypertrophy (Choice A) presents with upward and lateral displacement.
• Right atrial enlargement (Choice B) is difficult to be identified on chest radiography due to its posterior placement. Left atrial enlargement (Choice D) can be identified on chest radiography by the double density sign.
Which is NOT a cyanotic heart disease?
A. Ventricular septal defect
B. Pulmonary atresia
C. Hypoplastic left heart syndrome
D. Tetralogy of Fallot
A. Ventricular septal defect
Acyanotic congenital heart diseases include atrial septal defect (ASD), Ventricular septal defect (VSD), and patent ductus arteriosus (PDA)
What is NOT true regarding atrial septal defect (ASD)?
A. Most common but least serious type of ASD is ostium primum defect.
B. Most common but least serious type of ASD is ostium secundum defect.
C. ASD is the 2nd most common congenital heart defect in children and adults.
D. Most children with ASD are asymptomatic.
A. MOST COMMON BUT LEAST SERIOUS TYPE OF ASD IS OSTIUM PRIMUM DEFECT.
• Using testmanship, either Choice A or B would be the answer since they are contradicting statements.
o Ostium Secundum ASD — Most common subtype of ASD, 70 to 75% of all cases. Results from poor growth of the secundum septum or excessive absorption of the septum.
o Ostium Primum ASD — 15-20% of all cases. Results from a defect in the septum secundum due to failure of the primum septum to fuse with the endocardial cushions at the base of the interatrial septum.
• Choice C is correct because VSD is the most common congenital heart disease in children, while ASD is the second most common. Choice D is correct because ASD usually is asymptomatic, especially for small lesions.
What is the term used to define a child’s heart if it is in the midline position on chest Xray?
A. Levocardia
B. Inferiocardia
C. Dextrocardia
D. Mesocardia
D. Mesocardia
Normal position would be levocardia (on the left side). Dextrocardia (Choice C) occurs when the apex of the heart points to the right and occurs in patients with situs invertus. Mesocardia (Choice D) is a condition where the longitudinal axis of the heart lies in the mid-sagittal plane and the heart has no apex. There is no such thing as inferiocardia (Choice B).
