PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS OF MCQS PUBLISHED ON 30/8/2019

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS OF MCQS PUBLISHED ON 30/8/2019: ANSWERS MCQS#01 (D).  Foley (balloon) catheter should not be used, because the balloon can cause  severe bladder spasm, which can...

ANSWERS OF MCQS PUBLISHED ON 30/8/2019

ANSWERS

MCQS#01 (D). 

Foley (balloon) catheter should not be used, because the balloon can cause severe bladder spasm, which can produce severe ureteral obstruction, affecting 1 in 8,000 boys. Vesicoureteral reflux occurs in 50% of patients and the urinary stream is weak.The urethral valves are tissue leaflets fanning distally from the prostatic urethra to the external urinary sphincter.

MCQS#02 (C).

MCQS#3 (B). 

This form of therapy has a reported success of 30-60%, although the relapse rate is significant, while motivational therapy; there is no evidence that this approach is beneficial, Pharmacologic therapy is intended to treat the symptom of enuresis and thus is regarded as second line and is not curative.

MCQS#4 (A). Others syndromes associated with syndactyly.

MCQS#5 (B). Retractile testes may be misdiagnosed as undescended testes.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC MCQS

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: PEDIATRIC MCQS: PEDIATRIC MCQS MCQS#01 Posterior urethral valves, is the most common cause of severe obstructive  uropathies in children. 0f the fo...

PEDIATRIC MCQS

PEDIATRIC MCQS

MCQS#01

Posterior urethral valves, is the most common cause of severe obstructive uropathies in children.

0f the following, the MOST appropriate statement

A.  Its incidence 1 in 40,000 boys

B.  Vesicoureteral reflux occurs in 5% of patients

C.  The urinary stream is strong

D.  Foley catheter should be avoided

E.  It is adiverticulum in the penile urethra

MCQS#02

Family history in nocturnal enuresis is positive in

A. 10%
B. 30%
C. 50%
D. 70%
E. 90%

MCQS#03

The MOST effective way of treatment in older children with nocturnal enuresis is

A. Motivational therapy

B. Conditioning therapy

C. Desmopressin acetate

D. Oxybutynin chloride

E.  Imipramine

MCQS#04

The most common cause of micropenis is failure of the hypothalamus to produce an adequate amount of gonadotropin-releasing hormone.

0f the following, the syndrome associated with micropenis is

A. Kallmann syndrome

B. Fetal hydantoin syndrome

C. Apert syndrome

D. de Lange syndrome

E. Holt-Oram syndrome

MCQS#05

The consequences of cryptorchidism include all the following EXCEPT

A. Infertility

B. Hydrocele

C. Inguinal hernia

D. Testicular malignancy

E. Poor testicular growth

ANSWERS Dated: 31/8/2019

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS of MCQS PUBLISHED ON 28/8/2019

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS of MCQS PUBLISHED ON 28/8/2019: ANSWERS of MCQS PUBLISHED ON 28/8/2019 MCQS#01 E. Half of the neonates born to mothers with Graves disease develop hyperthyroidism ...

ANSWERS of MCQS PUBLISHED ON 28/8/2019

ANSWERS of MCQS PUBLISHED ON 28/8/2019

MCQS#01

E. Half of the neonates born to mothers with Graves disease develop hyperthyroidism

Explanation:

Thyroid disease is a common entity in pregnancy. Graves disease occurs in 0.1% to 0.4% of all pregnancies, although only about 1% of neonates born to women with Graves will be clinically affected. The fetus/neonate is affected as a result of transplacental passage of thyroid-stimulating hormone (TSH) receptor-stimulating and receptor-blocking antibodies during the 2nd half of pregnancy. Because stimulating antibodies are more often produced, most affected neonates will develop hyperthyroidism. However, a small number of infants may develop hypothyroidism if the amount of blocking antibodies crossing the placenta is greater than the amount of stimulating antibodies. Evidence of fetal disease can be apparent even if the pregnant woman has inactive Graves disease (e.g., following removal or destruction of the thyroid gland) because the fetus is still exposed to maternal antibodies. Intrauterine signs of fetal disease include fetal tachycardia, growth restriction, and fetal hydrops. A fetal goiter may also be present. Post-birth, the symptoms of hyperthyroidism in a newborn are usually apparent within the first 10 days of life, although clinical symptoms can present up to 4 to 6 weeks of life. Thyrotoxicosis usually resolves by 2 months of age but may last as long as 5 months of life.

The signs and symptoms of neonatal hyperthyroidism is variable and include the following:

•Increased irritability and jitteriness

•Periorbital edema and exophthalmos

•Tachycardia

•Pulmonary hypertension

•Weight loss

•Diarrhea

•Sweating and flushing

•Advanced bone age

•Hepatosplenomegaly

•Bruising and petechiae

•Goiter

Infants with evidence of thyrotoxicosis should be managed immediately with anti-thyroid therapies and symptomatic relief can be provided by beta-blockade. Anti-thyroid options include propylthiouracil and carbimazole.

Reference:

Hernandez MI, Lee KW. Neonatal Graves disease caused by transplacental antibodies. NeoReviews. 2008; 9(7):e 305-e 208.

MCQS#02

C. There is a TSH surge after birth, with markedly elevated TSH concentrations compared to older infants

Explanation:

The timing of the newborn state screen is critical to interpret the results of thyroid function studies. Typically the newborn screen is performed 36 to 72 hours after birth in a healthy term infant. Because the state screen of the infant in this vignette was obtained shortly after birth, it may reflect normal physiologic changes that would be considered abnormal in a different situation. After birth, there is a dramatic increase in serum thyroid-stimulating hormone (TSH) concentrations with levels as high as 60 to 70 mU/L. While not completely understood, this process is thought to result from the infant’s initial exposure to the relatively cold atmosphere compared with the intrauterine environment. This TSH surge results in an increase in serum thyroxine (T4) and

triiodothyronine (T3) concentrations. The concentration of T4 in the first week of life is usually the highest than at any other time during life. The T3 levels tend to rise after the first week of life and continue to increase during the first month of life. In contrast, concentrations of reverse T3 (rT3) tend to decrease postnatally because of the increased action of deiodinase D, as well as loss of placental deiodinase D3. Free T4 and thyroid-binding globulin follow a similar path as T4, usually exhibiting a peak in the first week of life followed by a gradual decline. While an abnormally high TSH value would be concerning for congenital hypothyroidism, the infant in this vignette most likely has an elevated TSH because the screen was performed immediately after birth when the TSH is expected to be high because of normal physiologic adaptation.

Reference:

Feingold SB, Brown RS. Neonatal thyroid function. NeoReviews. 2010;11(11):e640-e645

MCQS#03

B. Hypercalcemia

Explanation:

Infants of a diabetic mother (IDM) are at increased risk for multiple problems after birth. Hypoglycemia typically occurs as a result of attenuation of the maternal supply of glucose once the umbilical cord is clamped. The fetal hyperinsulinemic state continues in the short-term and lack of maturity of the counter-regulatory hormones may result in persistent neonatal hypoglycemia. In

addition, the effect of insulin as a growth factor may result in intracardiac septal and ventricular wall thickening, which can lead to a transient cardiomyopathy. IDMs are at increased risk of having respiratory distress syndrome, although the risk has decreased over the years; this is probably because of more accurate fetal assessment of gestational age. The mechanism for surfactant deficiency may result from increased fetal insulin inhibitory action

on fibroblast-pneumocyte factor, which normally acts on type II alveolar cells to produce surfactant. IDMs are at increased risk of polycythemia, although the mechanism is not understood. In addition. IDMs are also at an increased risk for hypocalcemia (NOT HYPERcalcemia), possibly as a result of a delay in the neonatal parathyroid hormone surge due to urinary magnesium losses (which can decrease parathyroid hormone secretion, thus resulting in hypocalcemia)

References:

Dailey TL, Coustan DR. Diabetes in pregnancy. NeoReviews. 2010;11(11):e619-e625

Ogata, ES. Problems of the infant of the diabetic mother. NeoReviews. 2010;11(11):e627-e630

MCQS#04

B. Maternal thyroid-stimulating hormone (TSH)

Explanation:

Thyroid gland embryogenesis is completed by 10 to 12 weeks of gestation and the gland begins to secrete thyroid hormone at approximately 12 weeks’ gestation. The fetal thyroid-stimulating hormone (TSH) receptors, however, do not become responsive to TSH and TSH receptor antibodies (TRAbs) until ~20 weeks’ gestation. There is a progressive increase in thyroxine (T4) and thyroxine-binding globulin (TBG) in the fetus, as well as an increase in free T4 (fT4) between 18 and 36 weeks’ gestation. In the first half of pregnancy, small amounts of maternal T4 cross the placenta when fetal T4 remains low. Additionally, maternal thyroid-releasing hormone (TRH) can cross the placenta but only in small amounts because the maternal serum TRH concentration is low. However, maternal TSH does not cross the placenta. TRAb (both stimulating and blocking) are immunoglobulin G antibodies that readily cross the placenta. Additionally, anti-thyroid medications and iodide both can cross the placental barrier. Iodide placental passage can affect the fetus in the first half of pregnancy when the fetal thyroid hormone production is increasing.

Reference:

Hernandez MI, Lee KW. Neonatal Graves disease caused by transplacental antibodies. NeoReviews. 2008;9(7):e305-e208

MCQS#05

A. 5-alpha reductase deficiency

Explanation:

5-alpha reductase deficiency is an autosomal recessive disorder that limits the conversion of testosterone to dihydrotesterone. Males with 5-alpha reductase deficiency have ambiguous genitalia with appropriately differentiated Wolffian structures, absence of Müllerian-derived structures, small phallus, urogenital sinus with perineal hypospadias, and a blind vaginal pouch. Later in life, males have progressive virilization with decreased facial hair and small prostates. “Testicles at twelve” is sometimes used in reference to 5-alpha reductase deficiency because of virilization and descent of testes to the labial location at the time of puberty. Females have a normal phenotype. Thus, the infant in this vignette with a 46 XX chromosomal analysis is not likely to have 5-alpha reductase deficiency.

Aromatase deficiency prevents conversion of testosterone to estradiol, thus androstenedione is not ultimately converted to estrone (see Figure above). Affected females have Müllerian duct structures and absent Wolffian duct structures, evident by ambiguous genitalia or cliteromegaly. Affected females may also have multicystic ovaries, tall stature, virilization at puberty, and delayed bone age. If the fetus is exposed to maternal androgen and progesterone therapy between 8 to 13 weeks’ gestation, the female fetus is at risk for ambiguous genitalia, including posterior fusion of the vagina, scrotalization of the labia and some fusion of the urethral folds. If the female fetus is exposed to these maternal hormones after 13 weeks’ gestation, the fetus may develop cliteromegaly.

Congenital adrenal hyperplasia encompasses a group of enzymatic disorders that leads to ambiguous genitalia. 

Deficiencies in 21-hydroxylase, 11 beta-hydroxylase, and 3 beta-hydroxysteroid dehydrogenase lead to ambiguous external genitalia in females.

References:

Brodsky D, Martin C. Neonatology Review. 2nd edition. Lulu. 2010.

Fanaroff AA, Martin RJ, Walsh MC (eds). Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant. 8th edition. Philadelphia: Mosby-Elsevier; 2006.

Sperling MA. Pediatric Endocrinology. 3rd edition. Philadelphia: WB Saunders; 2008.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: NEONATOLOGY MCQS

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: NEONATOLOGY MCQS: NEONATOLOGY MCQS MCQS#01 A neonatologist meets with a pregnant woman at 36 weeks’ gestation with Graves disease. Her  condition has...

NEONATOLOGY MCQS

NEONATOLOGY MCQS

MCQS#01

A neonatologist meets with a pregnant woman at 36 weeks’ gestation with Graves disease. Her condition has been well-controlled and there have been no signs of fetal distress.

Which of the following statements about the effects of maternal Graves disease on the fetus or infant is FALSE?

A.  A small number of infants may develop primary hypothyroidism

B.  Exophthalmos can occur in affected infants

C.  Fetal hydrops can occur in affected fetuses

D.  Fetal hyperthyroidism typically develops during the 2nd half of gestation

E.  Half of the neonates born to mothers with Graves disease develop                      hyperthyroidism

MCQS#02

You are asked to evaluate an otherwise healthy, well appearing 4-day old term newborn because of an abnormal thyroid-stimulating hormone (TSH) concentration measured on the infant’s newborn state screen. The screen had been erroneously sent shortly after birth. The infant is breastfeeding well, with normal voiding and stooling patterns.

You speak with the family and tell them that you plan to repeat the newborn screen but are not worried because:

A.  The infant is well appearing, without clinical signs of hypothyroidism

B.  The infant’s reverse triiodothyronine (rT3) is also elevated

C.  There is a TSH surge after birth, with markedly elevated TSH concentrations        compared to older infants

D.  The TSH concentration is suppressed at birth and takes several days to              reach a normal level

E. The TSH measurement is not as reliable as measuring thyroxine (T4)

MCQS#03

A neonatologist is evaluating an infant of a diabetic mother who was born at term weighing 4.6 kg. The infant appears plethoric and is admitted to the NICU for management of hypoglycemia. The family asks the neonatologist to discuss neonatal complications of maternal diabetes.

Which of the following findings in the newborn is NOT associated with maternal diabetes?

A.Hypoglycemia

B. Hypercalcemia

C. Increased intracardiac septal thickening

D.Mild surfactant deficiency

E. Polycythemia

MCQS#04
A neonatologist is asked to consult with a pregnant woman with hyperthyroidism. The woman inquires if her own thyroid hormone crosses the placenta to the fetus.

All of the following can cross the placenta, EXCEPT for:

A. Maternal thyroid-releasing hormone (TRH)

B. Maternal thyroid-stimulating hormone (TSH)

C. Maternal thyroxine (T4)

D. Radioactive iodide

E. TSH receptor antibodies (TRAb)

MCQS#05
A neonatologist is called to the Delivery Room of a term infant with respiratory distress. The infant’s initial physical examination reveals mild respiratory distress and an unexpected finding of ambiguous external genitalia. Review of the maternal records reveals that an amniocentesis had been done showing a 46 XX karyotype.

Which of the following etiologies is LEAST likely to be attributed to an over virilized female?

A. 5-alpha reductase deficiency

B. 11-beta hydroxylase deficiency

C. 21-hydroxylase deficiency

D. Aromatase deficiency

E. Maternal androgen and progesterone therapy

ANSWERS TOMORROW

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Answers Pediatric Neonatology MCQS Published on 24...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Answers Pediatric Neonatology MCQS Published on 24...: ANSWERS MCQS#01 C. 11 beta-hydroxylase Explanation: 11 beta-hydroxylase deficiency is the second most common cause of congenita...

Answers Pediatric Neonatology MCQS Published on 24/8/2019

ANSWERS

MCQS#01

C. 11 beta-hydroxylase

Explanation:

11 beta-hydroxylase deficiency is the second most common cause of congenital adrenal hyperplasia, occurring particularly in individuals of Middle Eastern descent. It results from an inability to convert deoxycorticosterone to aldosterone and 11-deoxycortisol to cortisol. There is a resulting excess of 17 hydroxy-progesterone, which leads to increased serum androgen production. There is no salt-wasting because deoxycorticosterone acts as a mineralocorticoid. Male infants have normal external genitalia at birth while females may have ambiguous genitalia of variable severity because of increased androgen exposure. Diagnosis is confirmed with increased deoxycorticosterone

and deoxycortisol concentrations. Therapy includes glucocorticoid replacement and genital reconstruction.

References:

Brodsky D, Martin C. Neonatology Review. 2nd edition. Lulu. 2010

Kliegman RM, Behrman RE, Jenson HB, Stanton B (eds). Nelson Textbook of Pediatrics. 18th edition. Philadelphia: Saunders, 2007.

MCQS#02

C. Transposition of the great vessels

Explanation:

Effects of maternal diabetes on the fetus are well-known and include congenital heart disease (hypertrophic cardiomyopathy, ventricular septal defect, transposition of the great vessels), renal anomalies, caudal regression, neural tube defects, central nervous system anomalies, and small left colon. The risk of congenital malformations correlates with the degree of uncontrolled maternal diabetes. If a woman with diabetes achieves glycemic control after conception, the risk of fetal anomalies is 7.8%; however, if glycemic control is attained prior to pregnancy, the risk of fetal anomalies decreases to 2.5%.

Reference: Brodsky D, Martin C. Neonatology Review. 2nd edition. Lulu. 2010.

MCQS#03

D. Thyroid dysgenesis

Explanation:

Congenital hypothyroidism can present with prolonged jaundice, a large posterior fontanel, umbilical hernia, macroglossia, hoarse cry, abdominal distention, hypotonia, feeding difficulties, lethargy, mottled skin, hypothermia, and goiter. Long-term consequences include delayed growth, cognitive deficits, and delayed puberty. The most common etiology of congenital hypothyroidism is

thyroid dysgenesis (occurring in ~75% of cases), which results from partial or complete absence of the thyroid gland.

Thyroid dyshormonogenesis occurs in ~10% of infants with congenital hypothyroidism and leads to inadequate thyroid hormone production. It can be caused by thyroid-stimulating hormone resistance, defects in iodide transport, thyroglobulin abnormality, deiodase deficiency, or an organification defect.

Defects in the hypothalamic-pituitary axis, such as panhypopituitarism, are much less common, occurring in ~5% of individuals affected by congenital hypothyroidism. It is associated with other hormone deficiencies.

Neonatal hypothyroidism can also be caused by transient hypothyroidism (~10%), which is attributable to maternal medications, maternal antibodies, or neonatal iodine exposure. Preterm infants commonly have transient hypothyroxinemia of prematurity of unknown etiology but may be related to an immature hypothalamic-pituitary axis. Sick euthyroid presents as temporarily low thyroid hormone levels with normal thyroid-stimulating hormone in the setting of an acute or chronic illness.

References:

Belfort MB, Brown RS. Hypothyroidism in the preterm infants. In: Primary Care of the Premature Infant. Brodsky D, Ouellette MA (eds). Philadelphia: WB Saunders, 2008.

Brodsky D, Martin C. Neonatology Review. 2nd edition. Lulu. 2010.

Grasberger H, Refetoff S. Genetic causes of congenital hypothyroidism due to dyshormonogenesis. Curr Opin Pediatr. 2011;23:421-428.

MCQS#04

B. Decrease in serum osmolality and increase in urine osmolality

Explanation:

The infant in this vignette likely has neurogenic or central diabetes insipidus (DI) caused by a decrease in anti-diuretic hormone (ADH) production as a result of his intracerebral hemorrhage. Other intracranial lesions, such as tumors and arterio-venous malformations, can also lead to central DI. About 10% of cases are idiopathic. The inadequate ADH production causes free water loss from the kidney resulting in hypernatremia, an increase in serum osmolality, and inappropriately dilute urine. Treatment with exogenous ADH will lead to a decrease in serum osmolality and an increase in urine osmolality.

In contrast to central DI, individuals affected by nephrogenic DI have an inadequate renal response to ADH. Similar to central DI, the disease is associated with increased serum osmolality and decreased urine osmolality. However, serum ADH levels are normal or elevated. After administration of exogenous ADH, serum and urine osmolalities are not altered.

References:

Kliegman RM, Behrman RE, Jenson HB, Stanton B (eds). Nelson Textbook of Pediatrics. 18th edition. Philadelphia: Saunders, 2007.

Knoers N. Nephrogenic diabetes insipidus. In: GeneReviews. Pagon RA, Bird TD, Dolan CR, Stephens K (eds). Seattle (WA): University of Washington, Seattle, 2010.

Porterfield SP. Endocrine Physiology. 2nd edition. St Louis: Mosby; 2001.

MCQS#05

D. Hypocalcemia

Explanation:

Maternal diabetes can lead to neonatal hypocalcemia, hypoglycemia and hypomagnesemia. Although jitteriness most commonly reflects hypoglycemia in an infant of a diabetic mother, the infant in this vignette has a normal glucose concentration, heightening suspicion for another electrolyte problem. Although infants with hypocalcemia and hypomagnesemia may be asymptomatic, both can induce jitteriness. Classic signs of hypocalcemia include Chvostek and Trousseau signs, irritability, laryngospasm, tetany, seizures, and prolonged QT interval. Infants with hypomagnesemia may have muscle weakness, increased deep tendon reflexes, irritability, jitteriness, seizures, and a prolonged QT interval. Hypocalcemia and hypomagnesemia are both treated with careful monitoring and replacement of the deficient electrolyte.

Reference:

Kliegman RM, Behrman RE, Jenson HB, Stanton B (eds). Nelson Textbook of Pediatrics. 18th edition. Philadelphia: Saunders, 2007.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: NEONATOLOGY MCQS

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: NEONATOLOGY MCQS: PEDIATRICS MCQS MCQS#01 A full-term infant born is noted to have ambiguous genitalia and  elevated blood pressures. The rest of the p...

NEONATOLOGY MCQS

PEDIATRICS MCQS

MCQS#01

A full-term infant born is noted to have ambiguous genitalia and elevated blood pressures. The rest of the physical examination is unremarkable. Laboratory evaluation reveals normal serum electrolytes with elevated serum androgens and deoxycorticosterone.

Of the following, which enzymatic defect is responsible for this infant’s congenital adrenal hyperplasia?

A. Aromatase

B. 5 alpha-reductase

C. 11 beta-hydroxylase

D. 17 alpha-hydroxylase

E. 21-hydroxylase

MCQS#02

Of the following, the most likely congenital cardiac defect in an infant of a diabetic mother is:

A. Ebstein’s anomaly

B. Tetrology of Fallot

C. Transposition of the great vessels

D. Tricuspid atresia

E. Truncus arteriosus

MCQS#03

A full-term male infant has prolonged indirect hyperbilirubinemia, a large posterior fontanel, hypotonia, and feeding difficulties. The neonatology fellow suspects that the infant has congenital hypothyroidism. Laboratory evaluation reveals a low thyroxine concentration and elevated thyroid-stimulating hormone.

The most likely cause for this infant’s hypothyroidism is:

A.  Deiodase deficiency

B.  Organification defect

C.  Panhypopituitarism

D.  Thyroid dysgenesis

E.  Thyroid-stimulating hormone resistance

MCQS#04

A 1-week old full-term infant has an intraparenchymal cerebral hemorrhage. His urine output is 10 mL/kg/hour. Laboratory evaluation reveals Na+=158 mEq/L, K+=4.1 mEq/L, Cl-=118 mEq/L, HCO3-=30 mEq/L, and serum and urine osmolality of 310 mOsm and 125 mOsm, respectively.

Upon administration of exogenous vasopressin (anti-diuretic hormone), the MOST likely impact on this infant’s osmolality is:

A.  Decrease in serum and urine osmolality

B.  Decrease in serum osmolality and increase in urine osmolality

C.  Increase in serum and urine osmolality

D.  Increase in serum osmolality and decrease in urine osmolality

E.  No change in serum or urine osmolality

MCQS#05

A female infant of a diabetic mother is admitted to the Neonatal Intensive Care Unit with irritability, tremulousness, and concern for seizure activity. Physical examination reveals a jittery full-term infant with laryngospasm and episodes of rhythmic left lower extremity jerking. Her chest radiograph reveals a normal cardiothymic silhouette. Her electrocardiogram reveals a prolonged QT interval. Her blood glucose is 80 mg/dL.

Serum electrolyte evaluation of this infant would most likely reveal:

A.  Hypercalcemia

B.  Hyperkalemia

C.  Hypermagnesemia

D.  Hypocalcemia

E.  Hypomagnesemia

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PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS OF MCQS PUBLISHED YESTERDAY MCQS#01C. Noon...

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: ANSWERS OF MCQS PUBLISHED YESTERDAY MCQS#01C. Noon...: ANSWERS OF MCQS PUBLISHED YESTERDAY  MCQS#01 C. Noonan syndrome Hypertrophic cardiomyopathy is characterized by a disorganized arrang...

ANSWERS OF MCQS PUBLISHED YESTERDAY 

MCQS#01

C. Noonan syndrome

Hypertrophic cardiomyopathy is characterized by a disorganized arrangement of myocardial fibers. Noonan syndrome is the most common cause of hypertrophic cardiomyopathy in neonates and children under age 4 years. It is an autosomal dominant disorder with an incidence of approximately 1 in 1,000 to 2,500 live births. In addition to hypertrophic cardiomyopathy, features of Noonan syndrome include the following:

•Pulmonic stenosis (60% incidence)

•Hypertelorism

•Downward slanting palpebral fissures

•Low-set ears

•Short stature

•Short webbed neck

•Pectus excavatum

•Cryptorchidism

•Cognitive deficits

•Bleeding disorders

•Lymphedema

Beckwith-Wiedemann syndrome, trisomy 21, Costello syndrome (also known as fasciocutaneoskeletal syndrome), and Eagle-Barrett syndrome (also known as prune belly syndrome) have also been associated with hypertrophic cardiomyopathy. The other options listed in this question are not typically associated with hypertrophic cardiomyopathy.

Reference: Wallis G, Fricker F. Neonatal cardiomyopathy. NeoReviews. 2012; 13:e711-e723

MCQS#02

D. The majority of mothers of neonates diagnosed with congenital heart block have symptoms of systemic lupus erythematosus

Neonatal lupus erythematosus (NLE) results from transfer of anti-SSA/Ro and anti-SSB/La antibodies from a pregnant woman to her fetus, potentially injuring fetal tissue. These antibodies are nearly universally found in the serum of women whose fetuses are diagnosed in utero with congenital heart block. One of the most devastating fetal/neonatal consequences of this passively acquired autoimmune disorder is involvement of the cardiac conducting system with subsequent rhythm abnormalities such as congenital heart block. This abnormal cardiac rhythm is typically diagnosed between 16 and 24 weeks’ gestation and is associated with a 20% mortality rate in the fetus/newborn. Complete congenital heart block is generally irreversible and surviving children typically require pacing. Fewer than one-third of mothers of affected infants have a diagnosis of systemic lupus erythematosus and many women are asymptomatic.

Reference: Buyon J, Nugent D, Mellins, E, Sandborg C. Maternal immunologic diseases and neonatal disorders. NeoReviews. 2002; 3:e3-e10

MCQS#03

E. Rashkind balloon septostomy

D-Transposition of the great arteries (D-TGA) is the most common cyanotic lesion to present in the first week of life and accounts for 5% to 10% of all congenital heart disease. The severe cyanosis results from having two circulations in parallel with poor mixing. Affected patients typically do not have a murmur unless a ventricular septal defect or pulmonary stenosis (PS) is present. Commonly, a single loud S2 is auscultated because the pulmonary valve is further from the chest wall, limiting the ability to detect a P2 sound. A typically chest radiograph of patients with D-TGA include a normal heart size, increased pulmonary vascular markings, and an “egg on a string” narrow mediastinum appearance that results from the anterior-posterior aorta and main pulmonary artery relationship.

Initial management of a patient with D-TGA includes prostaglandin therapy and in the setting of inadequate mixing (as described above), a palliative emergent Rashkind procedure is necessary to improve mixing. An arterial switch procedure is the definitive corrective operation for D-TGA but is not done until adequate mixing is ensured.

The Blalock-Taussig shunt is used to ensure adequate pulmonary blood flow for lesions such as tetralogy of Fallot/severe PS, tricuspid atresia, or pulmonary atresia. Pulmonary artery banding can be required if a patient has increased pulmonary blood flow with lesions such as tricuspid atresia and a single ventricle. Inhaled nitric oxide therapy leads to dilation of the pulmonary arteries and can increase pulmonary blood flow. In patients with D-TGA, inhaled nitric oxide therapy would not be helpful because the cyanosis results from inadequate mixing of the two circulations rather than inadequate pulmonary blood flow.

References:

Brodsky D, Martin C. Neonatology Review. 2nd Edition. Lulu. 2010

Keane JF, Fyler DC, Lock JE (ed). Nadas’ Pediatric Cardiology. 2nd edition. Philadelphia: WB

Saunders Co; 2006.

MCQS#04

C. Endocarditis

Complications of an untreated PDA depend largely on the size and degree of blood flow. Congestive heart failure and pulmonary hypertension may result from over circulation as a result of left-to-right shunting. Endarteritis is an increasingly rare complication, though infective vegetations may be seen in the pulmonary artery and PDA. A ductal aneurysm may also occur, most likely after surgical closure, coiling, or an infective arteritis. About a quarter of cases of aneurysm have been found in conjunction with aneuploidy or soft-tissue disorders such as Marfan syndrome.

Reference: Schneider DJ, Moore JW. Congenital heart disease for the adult cardiologist. Circ. 2006; 114:1873-1882

MCQS#05

E. All of the above

Congenital heart defects are more prevalent in the recipient twin of twin-to-twin transfusion syndrome. Studies report a three-fold increase in the frequency of congenital heart defects. The most frequent defects are ventricular septal defects, atrial septal defects and pulmonary stenosis.

References:

Bahtiyar MO, Dulay AT, Weeks BP, et al. Prevalence of congenital heart defects in monochorionic/diamniotic twin gestations: a systematic literature review. J Ultrasound Med.

2007;26:1491–1498

Karatza AA, Wolfenden JL, Taylor MJ, et al. Influence of twin–twin transfusion syndrome on fetal cardiovascular structure and function: prospective case–control study of 136 monochorionic twin pregnancies. Heart. 2002; 88:271–277

PEDIATRICS MCQS: NEONATOLOGY

MCQS

MCQS#01

Which of the following syndromes is the most common cause of hypertrophic cardiomyopathy in neonates and children under age 4 years?

A. Apert syndrome

B. CHARGE association

C. Noonan syndrome

D. Trisomy 18

E. Turner syndrome

MCQS#02

A fetus at 20 weeks’ gestation is diagnosed with congenital heart block.

Which of the following statements about neonatal lupus erythematosus (NLE) is FALSE?

A.  Anti-SSA/Ro and anti-SSB/La antibodies are nearly universally found in the        serum of women whose fetuses are diagnosed in utero with congenital heart       block

B.  Complete congenital heart block is irreversible and surviving children                 typically   require pacing

C.  Congenital heart block resulting from NLE is typically diagnosed between 16       and 24 weeks’ gestation

D.  The majority of mothers of neonates diagnosed with congenital heart block         have symptoms of systemic lupus erythematosus

E.  The mortality rate of congenital heart block in NLE is approximately 20%

MCQS#03

You are called to the Delivery Room to evaluate a cyanotic term neonate. On arrival, you note a severely cyanotic female neonate without respiratory distress. The infant has 2+ distal pulses bilaterally, no murmur, and a loud, single S2. The chest radiograph shows a normal heart size and a narrow mediastinum. You contact a cardiologist and order intravenous prostaglandin therapy. An echocardiogram confirms the diagnosis.

What is the likely next step in the management of this infant?

A. Blalock-Taussig shunt procedure

B. Inhaled nitric oxide therapy

C. Jatene arterial switch procedure

D. Pulmonary artery banding

E. Rashkind balloon septostomy

MCQS#04

The patent ductus arteriosus (PDA) is considered to be a common form of congenital heart disease, with an incidence of about 1 per 2,000 live births.

Even if asymptomatic in childhood, an untreated PDA may cause all of the following EXCEPT:

A. Congestive heart failure

B. Endarteritis

C. Endocarditis

D. PDA aneurysm

E. Pulmonary hypertension

MCQS#05

During a routine physical examination, you hear a cardiac murmur in a 3-day old girl, the recipient of twins diagnosed prenatally with twin-to-twin transfusion syndrome (TTTS).

Which of the following statements about this scenario is TRUE?

A. Congenital heart defects are more prevalent in monochorionic infants with          TTTS.

B. The recipient twin is more frequently symptomatic.

C. There is a three-fold increase in the frequency of congenital heart defects.

D. Ventricular septal defects, atrial septal defects and pulmonary stenosis are         seen most frequently.

E.  All of the above are true

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Pediatrics Facts: Celiac Disease

20 Things You Might Not Know About Celiac Disease

1. Celiac disease is a chronic autoimmune disease, which means that you cannot “grow out” of it.

2. 1 in 100 people worldwide have celiac disease.

3. Celiac disease affects an estimated three million Americans.

4. 80% of Americans with celiac disease are not diagnosed and are needlessly suffering.

5. People with a first degree relative with celiac disease have a 1 in 10 chance of developing celiac disease themselves.

6. More children have celiac disease than Crohn’s, Ulcerative Colitis, and Cystic Fibrosis combined.

7. Celiac disease can affect every organ in your body.

8. Lifelong adherence to the gluten-free diet is the only treatment for celiac disease.

9.      Approximately 20% of people with celiac disease do not respond to the gluten-free diet.

10.   There is an average delay of 6-10 years for an accurate celiac disease diagnosis.

11.    Without a timely diagnosis, celiac disease can lead to intestinal cancers, type 1 diabetes, osteoporosis, thyroid disease, multiple sclerosis, anemia, infertility and miscarriage, epilepsy, and more.

12. There are over 300 symptoms associated with celiac           disease.

13. Approximately 20% of people with celiac disease are asymptomatic, meaning they don’t experience any external symptoms at all. However, everyone with celiac disease is still at risk for long-term complications.

14.   Celiac disease can develop at any age after people start eating foods or medications that contain gluten.

15.   The later the age of celiac disease diagnosis, the greater the chance of developing another autoimmune disorder.

16.   There are two steps to being diagnosed with celiac disease: the blood test and the endoscopy.

17.   People with celiac disease have an increased incidence of microscopic colitis and inflammatory bowel disease (Crohn’s disease and ulcerative colitis).

18.   People with celiac disease may have lactose and/or fructose intolerance, both of which can be diagnosed by a hydrogen breadth test.

19. People recently diagnosed with celiac disease are commonly deficient in fiber, iron, calcium, magnesium, zinc, folate, niacin, riboflavin, vitamin B12, and vitamin D.

20. Any food product that is labeled “gluten-free” cannot contain more than 20 parts per million of gluten, which is the safe threshold of gluten consumption for people with celiac disease.

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric Diagnosis?

PEDIATRICS MCQS, TOACS, PEARLS & UPDATES: Pediatric Diagnosis?: This is the arterial blood gas result from a 14 year old girl who presented with a  history  of having an overdose of some undisclosed tabl...