Endocrine System Pediatric

ATI RN · Endocrine System Pediatric

Question 1 of 63

Which of the following mucopolysaccharidoses have normal intelligence?

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ATI RN · Endocrine System Pediatric

Question 2 of 63

Inborn errors of metabolism (IEM) are hereditary biochemical disorders caused by single-gene mutations that result in alteration of

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ATI RN · Endocrine System Pediatric

Question 3 of 63

Hypoglycemia may be a manifestation of child abuse or Munchausen syndrome by proxy induced by exogenous administration of insulin (i.e., factitious hyperinsulinemia). Of the following, the MOST reliable test that confirms the diagnosis of factitious hyperinsulinemia is

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ATI RN · Endocrine System Pediatric

Question 4 of 63

All the following should be restricted in hereditary fructose intolerance EXCEPT

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ATI RN · Endocrine System Pediatric

Question 5 of 63

A 1-week-old infant presents with poor feeding, vomiting, lethargy, and opisthotonus posturing. The critical impression is meningitis, but all cultures are negative. Seizures occur and hypoglycemia is documented; however, the seizures do not stop after the hypoglycemia is corrected with intravenous glucose. The arterial blood gas is 7.10 PCO2 23, PO2 90, with an anion gap of 35. A CT scan demonstrates cerebral edema. The most likely etiology is

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ATI RN · Endocrine System Pediatric

Question 6 of 63

All the following are true in premature thelarche EXCEPT

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ATI RN · Endocrine System Pediatric

Question 7 of 63

Protein intake is typically restricted in which of the following populations?

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ATI RN · Endocrine System Pediatric

Question 8 of 63

Plasma very-long-chain fatty acids are elevated in all of the following peroxisomal disorders EXCEPT

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ATI RN · Endocrine System Pediatric

Question 9 of 63

Matching: Glycogen storage diseases - Fanconi-Bickel

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ATI RN · Endocrine System Pediatric

Question 10 of 63

Familial isolated gonadotropin deficiency associated with anosmia is

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ATI RN · Endocrine System Pediatric

Question 11 of 63

The major causes of incidentalomas are

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ATI RN · Endocrine System Pediatric

Question 12 of 63

Individuals homozygous for galactokinase deficiency usually develop

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ATI RN · Endocrine System Pediatric

Question 13 of 63

Subjects with type 1 DM have evidence for celiac disease in

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ATI RN · Endocrine System Pediatric

Question 14 of 63

Nonketotic hyperosmolar coma is characterized by the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 15 of 63

The best approach to the diagnosis of the patient in the previous question is

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ATI RN · Endocrine System Pediatric

Question 16 of 63

What percent of newly diagnosed diabetics present with symptoms of diabetic ketoacidosis (DKA)?

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ATI RN · Endocrine System Pediatric

Question 17 of 63

Matching: Hemizygotic male has complete syndrome

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ATI RN · Endocrine System Pediatric

Question 18 of 63

Cystinuria is a disorder of renal tubular transport of the following amino acids EXCEPT

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ATI RN · Endocrine System Pediatric

Question 19 of 63

The MOST definitive test for adrenal insufficiency is measurement of

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ATI RN · Endocrine System Pediatric

Question 20 of 63

A 2-year-old girl presented with rapid deep breathing, hypoglycemia, recurrent epistaxis, easy bruising, failure to thrive, fat cheek, and hepatomegaly. Of the following, the MOST likely cause of bleeding tendency is

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ATI RN · Endocrine System Pediatric

Question 21 of 63

Antithyroid antibodies develop and subclinical or overt hypothyroidism occurs in the following conditions EXCEPT

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ATI RN · Endocrine System Pediatric

Question 22 of 63

A 3-week-old neonate develops repeated vomiting and dehydration. Examination reveals ambiguous genitalia with fusion of the anterior portion of the labioscrotal folds. Ultrasonography reveals presence of uterus, ovaries, and fallopian tubes. Of the following, the MOST valuable test to establish the diagnosis is

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ATI RN · Endocrine System Pediatric

Question 23 of 63

Physiologic factors play a role in stimulating and inhibiting growth hormone (GH). One of the following inhibit GH release

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ATI RN · Endocrine System Pediatric

Question 24 of 63

Smith Lemli-Opitz Syndrome (SLOS) is characterized by all the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 25 of 63

Signs of insulin resistance or conditions associated with insulin resistance in T2 DM in children include the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 26 of 63

In Edwards syndrome (trisomy 18), characteristic features include

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ATI RN · Endocrine System Pediatric

Question 27 of 63

Significant gynecomastia is seen in

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ATI RN · Endocrine System Pediatric

Question 28 of 63

Acquired nephrogenic diabetes insipidus (NDI) is associated with the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 29 of 63

At 10:00 AM Lylaa's mother brought her 3-year-old daughter to emergency department with difficult arousal from sleep, low grade fever, and cough for the last 2 days. Fasting blood sugar was 36 mg/dL with ketonuria. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatric

Question 30 of 63

All the following are characteristic features of infantile Refsum disease EXCEPT

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ATI RN · Endocrine System Pediatric

Question 31 of 63

Any organ or system can be injured by toxic accumulation of any of the metabolites involved in inborn errors. Of the following, the metabolic disease causing hepatic failure is

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ATI RN · Endocrine System Pediatric

Question 32 of 63

All the following are causes of infantile hypercalcemia EXCEPT

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ATI RN · Endocrine System Pediatric

Question 33 of 63

The evaluation of the child in the previous question is best accomplished by determining

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ATI RN · Endocrine System Pediatric

Question 34 of 63

Hyperkalemia in severe diabetic ketoacidosis is due to

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ATI RN · Endocrine System Pediatric

Question 35 of 63

Matching: Match the disease with its enzyme - ÃŽÂ²-Hexosaminidase A

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ATI RN · Endocrine System Pediatric

Question 36 of 63

A 12-year-old female has muscle cramps and tingling of her hands and feet unrelated to exertion. When she grabs a door handle to open the door, she is unable to release her grasp because her hand is in spasm. The most important laboratory test is

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ATI RN · Endocrine System Pediatric

Question 37 of 63

A 6-year-old boy with eczematoid rash, abnormal face, and prominent maxilla complains from deterioration in school performance, hyperactivity, and seizures for the last 2 years. Of the following, the MOST likely enzymatic deficiency is

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ATI RN · Endocrine System Pediatric

Question 38 of 63

A 9-year-old boy admitted to the hospital because of fracture of left femur due to a minor trauma. Past history revealed recurrent attacks of epistaxis and intermittent generalized bone pain. On examination, there is massive splenomegaly. Of the following, the MOST appropriate practical treatment of this disease is

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ATI RN · Endocrine System Pediatric

Question 39 of 63

A 15-month-old boy had recurrent hospital admissions because of fever, jaundice, subcutaneous bleeding, and hypoglycemic fits; on examination, there is hepatomegaly. In the last attack, the child developed severe legs pain associated with retraction of the neck and trunk. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatric

Question 40 of 63

A 7-month-old girl presented with recurrent chest infection, chocking during feeding, large protruded tongue, persistent head lag, and hepatomegaly. Chest X-ray showed cardiomegaly. All the following are beneficial in the treatment of this patient EXCEPT

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ATI RN · Endocrine System Pediatric

Question 41 of 63

Mucopolysaccharidoses (MPS) affect many solid organs, hepatosplenomegaly or hepatomegaly is usual finding, but the liver and spleen are normal in

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ATI RN · Endocrine System Pediatric

Question 42 of 63

A 15-year-old boy is brought to clinic by his parents because of delayed puberty. His growth chart reveals a height just below but parallel to the 5th centile. Examination is unremarkable. Bone age is consistent with a 12 year chronological age. Lab investigations including hormonal study are normal. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatric

Question 43 of 63

Matching: Lipidosis - Tay-Sachs

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ATI RN · Endocrine System Pediatric

Question 44 of 63

A 6-year-old has a chronic rash involving all of the nails of the fingers and toes. Last year she had a seizure and was noted to have a low serum ionized calcium. Currently she has weakness and hypotension, and she presents with a hypoglycemic seizure. The most likely diagnosis causing this seizure is

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ATI RN · Endocrine System Pediatric

Question 45 of 63

A 6-year-old female presents with breast enlargement and pubic hair development. Otherwise, she is asymptomatic. Her serum LH level is elevated. The most likely diagnosis is

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ATI RN · Endocrine System Pediatric

Question 46 of 63

Cortisol deficiency in congenital adrenal hyperplasia (salt wasting form of the disease) is treated with hydrocortisone

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ATI RN · Endocrine System Pediatric

Question 47 of 63

A 10-year-old girl is noted on a routine physical examination to have splenomegaly. Laboratory testing reveals thrombocytopenia and moderate edema, and she is referred to a hematologist for further evaluation. Bone marrow aspiration is performed and reveals the presence of Gaucher cells. When discussing therapeutic options with the parents of this child, it would be most appropriate to include which of the following statements?

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ATI RN · Endocrine System Pediatric

Question 48 of 63

The cord serum T4 is decreased in proportion to gestational age and birthweight. The serum T4 gradually increases and enters the T4 range seen in term infants by the age of

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ATI RN · Endocrine System Pediatric

Question 49 of 63

Chronic therapy for the patient in the previous question is best characterized by

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ATI RN · Endocrine System Pediatric

Question 50 of 63

Endemic cretinism is the most serious consequence of iodine deficiency; it includes two different but overlapping syndromes: a neurologic type and a myxedematous type. The neurologic syndrome is characterized by the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 51 of 63

A mother and her 14Ã‚Â½-year-old daughter come to you because the girl has not begun to menstruate. Findings on her medical history and complete physical examination are normal. Breast development and pubic hair have been present for 18 months and are normal. Which would be the most appropriate?

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ATI RN · Endocrine System Pediatric

Question 52 of 63

All the following are features of congenital erythropoietic porphyria (CEP) EXCEPT

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ATI RN · Endocrine System Pediatric

Question 53 of 63

An adolescent female develops fever, palpitation and disorientation. Examination shows exophthalmos, goiter, tachycardia and elevated blood pressure. Lab tests reveal elevated T4 and T3 with low TSH. Of the following, the BEST initial treatment is

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ATI RN · Endocrine System Pediatric

Question 54 of 63

In DKA, electrolyte abnormalities occurs secondary to hyperglycemia and acidosis. Which of the following electrolytes is artificially measured low in DKA?

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ATI RN · Endocrine System Pediatric

Question 55 of 63

A definitive diagnosis of mucopolysaccharidoses disorder is established by

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ATI RN · Endocrine System Pediatric

Question 56 of 63

Matching: Dietary biotin deficiency

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ATI RN · Endocrine System Pediatric

Question 57 of 63

Children with growth hormone deficiency are best characterized by all of the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 58 of 63

The best diagnostic test for the patient in the previous question is

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ATI RN · Endocrine System Pediatric

Question 59 of 63

A 5-day-old infant manifests tachypnea, poor feeding, vomiting, and decreased responsiveness. A sepsis workup is performed, and the child is started on a D10W infusion and receives ampicillin and cefotaxime. The condition worsens, and the child becomes unresponsive to stimuli. All cultures are negative after 48 hours. The arterial blood gas is 7.45, PCO2 36, PO2 100, the anion gap is normal, the blood glucose is 75 mg/dL, and serum ammonia is 500 ÃŽÂ¼M. The most likely diagnosis is

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ATI RN · Endocrine System Pediatric

Question 60 of 63

There is a spectrum of parathyroid deficiencies with clinical manifestations varying from no symptoms to those of complete and longstanding deficiency. Early manifestations include

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ATI RN · Endocrine System Pediatric

Question 61 of 63

A 6-year-old girl that looks tall and thin with light skin. On examination she has a peculiar malar flush, subluxation of the ocular lens, developmental delay, and severe hypertension. Of the following, the MOST likely cause of hypertension is

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ATI RN · Endocrine System Pediatric

Question 62 of 63

Xanthomas are a feature of all the following EXCEPT

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ATI RN · Endocrine System Pediatric

Question 63 of 63

Matching: Least severe

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