Endocrine System in Pediatrics

ATI RN · Endocrine System in Pediatrics

Question 1 of 63

Weaker associations with gynecomastia are seen in

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ATI RN · Endocrine System in Pediatrics

Question 2 of 63

The MOST common cardiac defect in Noonan syndrome is

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ATI RN · Endocrine System in Pediatrics

Question 3 of 63

Matching: Match the disease with its enzyme - Sphingomyelinase

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ATI RN · Endocrine System in Pediatrics

Question 4 of 63

All the following are common features between hereditary fructose intolerance and galactosemia EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 5 of 63

A 7-month-old boy presented with recurrent attacks of hypoglycemic seizures especially during an acute illness. On examination, there are thin extremities, protuberant abdomen, and hepatomegaly. All the following are biochemical hallmarks of this disease EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 6 of 63

Transient elevation of urinary albumin can occur in the following conditions EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 7 of 63

The MOST common cause of acquired hypothyroidism is

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ATI RN · Endocrine System in Pediatrics

Question 8 of 63

The clinical presentation of adrenal insufficiency depends on the age of the patient, the usual presentation of adrenal insufficiency in infancy is

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ATI RN · Endocrine System in Pediatrics

Question 9 of 63

The following are main features of diabetic ketoacidosis (DKA) EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 10 of 63

A 10-month-old boy presented with failure to thrive, fever, jaundice, hepatomegaly, and severe rickets. Investigations revealed hypoglycemia and normal anion gap metabolic acidosis. Of the following, the MOST likely enzyme deficiency is

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ATI RN · Endocrine System in Pediatrics

Question 11 of 63

Deficiency of trimethylamine oxidase results in what symptoms?

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ATI RN · Endocrine System in Pediatrics

Question 12 of 63

The characteristic radiographic finding of Hurler disease is

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ATI RN · Endocrine System in Pediatrics

Question 13 of 63

The staging of pubertal changes differs between both genders. The onset of puberty is marked by pubarche and gonadarche. Of the following, the first normal event of puberbertal maturation in boys is

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ATI RN · Endocrine System in Pediatrics

Question 14 of 63

Renal osteodystrophy is usually due to secondary hyperparathyroidism. Of the following, the treatment incorrectly matched with the condition is

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ATI RN · Endocrine System in Pediatrics

Question 15 of 63

Type 2 DM can be managed with lifestyle modifications, including nutrition therapy, increased exercise, and oral hypoglycemic agent (metformin); however, insulin therapy is necessary in special circumstances. Of the following, insulin treatment is required when there is

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ATI RN · Endocrine System in Pediatrics

Question 16 of 63

During episodes of symptomatic hyperammonemia, all the following treatment options can be used EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 17 of 63

A 12-year-old boy presented with epistaxis, mild bruising, intermittent bone pain, and massive splenomegaly. Complete blood count shows thrombocytopenia and mild anemia. Radiological study revealed lytic lesions and Erlenmeyer flask deformity of the distal femur. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 18 of 63

A 7-month-old boy has been healthy and developing normally since birth. His mother now reports that he has decreased eye contact with her, even during feedings. The infant also startles very easily when there is a loud noise in the house. Of the following, the most appropriate diagnostic test to confirm the etiology of these findings is the measurement of

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ATI RN · Endocrine System in Pediatrics

Question 19 of 63

The following stimulate vasopressin (VP) secretion EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 20 of 63

Falsely low HbAÃ¢â€šÂC levels are noted in the following conditions EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 21 of 63

Matching: Most severe

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ATI RN · Endocrine System in Pediatrics

Question 22 of 63

The MOST consistent and characteristic radiographic finding of primary hyperparathyroidism is

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ATI RN · Endocrine System in Pediatrics

Question 23 of 63

Which of the following is true regarding Patau syndrome (trisomy 13)?

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ATI RN · Endocrine System in Pediatrics

Question 24 of 63

A 6-year-old tall and thin boy who had failed preparatory exam for school entry found to have myopia and subluxation of the ocular lens. Of the following, the MOST important challenge is to know whether he is responsive to

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ATI RN · Endocrine System in Pediatrics

Question 25 of 63

A 3-year-old boy presented to emergency unit at 9:00 AM with history of difficult arousal from sleep, flue like illness, and low grade fever; the mother stated that he had slept without having his supper. Fasting blood sugar was 42 mg/dL. The following metabolic changes are seen in this condition EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 26 of 63

All of the following are associated with hypocalcemia EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 27 of 63

Calcification within the adrenal glands may occur in a wide variety of situations, some serious and others of no obvious consequence. Adrenal calcifications are bilateral in

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ATI RN · Endocrine System in Pediatrics

Question 28 of 63

A 9-month-old boy presented with persistent head lag, rapid breathing, difficult feeding, and hepatomegaly. Chest X- ray showed cardiomegaly. Of the following, the MOST appropriate confirmatory test is

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ATI RN · Endocrine System in Pediatrics

Question 29 of 63

A 5-year-old boy develops vomiting and abdominal pain with signs of dehydration. He has been diagnosed with type 1 diabetes mellitus since the age of 4 year and he is on insulin therapy since that time. Of the following, the sign that differentiate this condition from gastroenteritis is

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ATI RN · Endocrine System in Pediatrics

Question 30 of 63

Conditions associated with growth hormone deficiency include all of the following EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 31 of 63

Peroxisomal disorders in the neonatal period are most commonly manifested by

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ATI RN · Endocrine System in Pediatrics

Question 32 of 63

Cerebral edema complicating DKA remains the major cause of morbidity and mortality in children and adolescents with T1 DM. All the following are true EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 33 of 63

Matching: Glycogen storage diseases - McArdle

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ATI RN · Endocrine System in Pediatrics

Question 34 of 63

Matching: Juvenile multiple carboxylase deficiency

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ATI RN · Endocrine System in Pediatrics

Question 35 of 63

A 2-month-old male of French Canadian heritage presents with fever, irritability, vomiting, and jaundice. Physical examination reveals an obtunded infant with hepatomegaly and jaundice. Laboratory studies reveal a blood glucose level of 10 mg/dL and elevated liver transaminases. The most likely diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 36 of 63

The best approach to therapy for patients with homozygous familial hypercholesterolemia is

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ATI RN · Endocrine System in Pediatrics

Question 37 of 63

All the following drugs are secondary causes of hypertriglyceridemia EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 38 of 63

Mucopolysaccharidoses (MPS) affect many solid organs, the cornea is clear but there are retinal degeneration and papilledema in

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ATI RN · Endocrine System in Pediatrics

Question 39 of 63

All the following mucopolysaccharidoses are inherited as autosomal recessive EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 40 of 63

A 14-year-old male has unilateral gynecomastia and is Tanner stage 3 in pubertal development. He complains of occasional episodes of breast tenderness, which last less than 30 minutes and occur once a month. His serum estradiol and prolactin levels are normal. The most likely next step in the treatment of this patient is

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ATI RN · Endocrine System in Pediatrics

Question 41 of 63

A 6-week-old infant has gained no weight since birth. She is mottled and has an indirect bilirubin level of 24 mg/dL. Her extremities are cold, and her temperature is 35Ã‚Â°C. The most likely diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 42 of 63

All the following are features of neonatal adrenoleukodystrophy (ALD) EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 43 of 63

The pituitary gland is located in a saddle-shaped cavity of the

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ATI RN · Endocrine System in Pediatrics

Question 44 of 63

A 16-year-old female adolescent develops swelling at the anterior aspect of the neck with difficulty of the swallowing. Examination reveals a firm, nontender diffuse goiter with a pebble-like surface. Her mother has had a thyroid disease in early adulthood. Of the following, the MOST valuable test to confirm the diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 45 of 63

Renal tubular dysfunction in galactosemia may be evidenced by a

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ATI RN · Endocrine System in Pediatrics

Question 46 of 63

The MOST common electrolyte abnormality in acute intermittent porphyria is

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ATI RN · Endocrine System in Pediatrics

Question 47 of 63

The MOST common brain lesion causing central precocious puberty is

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ATI RN · Endocrine System in Pediatrics

Question 48 of 63

Metabolic disorders should be considered in all neonates presenting with the following EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 49 of 63

A 14-month-old male of Northern European ancestry experienced an upper respiratory tract infection and anorexia. On the second day of the illness, he began to vomit, and by that evening he was unresponsive. On the way to the hospital, he had a generalized seizure lasting 10 minutes. At the hospital, he had a blood glucose of 10 mg/dL; his urinalysis showed no ketones. Liver function tests, anion gap, and serum ammonia were all normal. The most likely diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 50 of 63

Constitutional growth delay is characterized by all of the following EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 51 of 63

A 3-year-old boy presented with history of hyperactivity and mild mental retardation. Past history revealed repeated projectile vomiting with normal abdominal sonography. On examination, there are eczematoid rash with lighter skin, microcephaly, and mild spasticity with exaggerated tendon reflexes. Of the following, the MOST likely urine odor of this baby is

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ATI RN · Endocrine System in Pediatrics

Question 52 of 63

Precocious puberty is defined by all of the following EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 53 of 63

Thyroid peroxidase antibodies are absent in the following thyroiditis syndrome

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ATI RN · Endocrine System in Pediatrics

Question 54 of 63

The MOST common cause of permanent congenital hypothyroidism is

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ATI RN · Endocrine System in Pediatrics

Question 55 of 63

Treatment is initiated with high-dose vitamin B6 but no response is observed. The most likely explanation is

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ATI RN · Endocrine System in Pediatrics

Question 56 of 63

A 16-year-old presents with easy bruisability and chronic fatigue. He has a 3-year history of bone pain and poor growth. Physical examination reveals hepatosplenomegaly. Laboratory studies reveal normal liver enzymes but a hematocrit of 25% and a platelet count of 25,000. X-rays of the skeleton reveal long bone lytic lesions and osteosclerosis. The most likely diagnosis is

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ATI RN · Endocrine System in Pediatrics

Question 57 of 63

Hyperglycemia during diabetic ketoacidosis may be associated with

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ATI RN · Endocrine System in Pediatrics

Question 58 of 63

You are examining a 5-day-old neonate with ambiguous genitalia; there are no palpable gonads in the inguinal or genital area; the blood pressure is normal according to the age. Of the following, the NEXT step in the management of this neonate is

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ATI RN · Endocrine System in Pediatrics

Question 59 of 63

Significantly virilized females with congenital adrenal hyperplasia (CAH) usually undergo surgery between

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ATI RN · Endocrine System in Pediatrics

Question 60 of 63

Therapy with one of the following drugs usually results in significant insulin resistance leading to glucose intolerance and overt diabetes

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ATI RN · Endocrine System in Pediatrics

Question 61 of 63

Aplasia or hypoplasia of the parathyroid glands is often associated with the DiGeorge/velocardiofacial syndrome. All the following regarding this syndrome are true EXCEPT

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ATI RN · Endocrine System in Pediatrics

Question 62 of 63

The specific clue to the diagnosis of fatty acid oxidation disorders may be the finding of hypoglycemia and

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ATI RN · Endocrine System in Pediatrics

Question 63 of 63

Matching: Sensorineural deafness

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