Endocrine System Pediatrics

ATI RN · Endocrine System Pediatrics

Question 1 of 63

The definitive diagnosis of type I glycogen storage disease GSD is by

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ATI RN · Endocrine System Pediatrics

Question 2 of 63

Matching: Match the disease with its enzyme - ÃŽÂ±-Galactosidase

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ATI RN · Endocrine System Pediatrics

Question 3 of 63

Lipidoses affect many solid organs, cherry-red spots is seen in

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ATI RN · Endocrine System Pediatrics

Question 4 of 63

Retardation of osseous development in congenitally hypothyroid infants can be shown radiographically at birth in approximately

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ATI RN · Endocrine System Pediatrics

Question 5 of 63

During a routine clinical visit, a healthy asymptomatic 6-year-old boy is found to have fasting blood glucose of 110 mg/dL; he has normal oral glucose tolerance test, no glycosuria, and HgbA1c of 6.4%. This child is MOST likely considered to have

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ATI RN · Endocrine System Pediatrics

Question 6 of 63

Sudden infant death syndrome is reported in infants with

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ATI RN · Endocrine System Pediatrics

Question 7 of 63

Indications for GH treatment to promote growth include the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 8 of 63

Main causes of hypocalcemia in nephrotic syndrome in children are the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 9 of 63

Measurement of hemoglobin A1c (HgbA1c) provides a mean for assessing long-term glycemic control for children with diabetes mellitus. In which of the following conditions this measurement is inaccurate for long-term glycemic control?

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ATI RN · Endocrine System Pediatrics

Question 10 of 63

Patients with mineralocorticoid deficiency can develop the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 11 of 63

Peak serum concentrations of TSH at birth in full-term infants reach

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ATI RN · Endocrine System Pediatrics

Question 12 of 63

A 12-year-old female has a hypoglycemic seizure, weakness, and increased cutaneous pigmentation. She is also noted to have a chronic history of mucocutaneous candidiasis, which is especially severe on her nails. In addition, she has been on thyroid replacement medication since the age of 9 years. The most likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 13 of 63

Klinefelter syndrome is the most common sex chromosomal aneuploidy in males, all the following features are true EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 14 of 63

The MOST common cardiac defect in Turner syndrome is

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ATI RN · Endocrine System Pediatrics

Question 15 of 63

One of the following is not routinely done in children with suspected hypothyroidism

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ATI RN · Endocrine System Pediatrics

Question 16 of 63

Features of Klinefelter syndrome include all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 17 of 63

Holoprosencephaly is associated with all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 18 of 63

Broad forehead, hypertelorism, small chin, long philtrum, camptodactyly, and fetal finger pads are features of the following genetic overgrowth syndrome

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ATI RN · Endocrine System Pediatrics

Question 19 of 63

A 12-year-old female adolescent is brought to the emergency room because of irritability, paresthesia, and seizure-like state after a psychological trauma. She is conscious with tachypnea and carpopedal spasm. Of the following, the MOST valuable test is the determination of

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ATI RN · Endocrine System Pediatrics

Question 20 of 63

Lysinuric protein intolerance is characterized by all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 21 of 63

A male infant is thought to have hypothyroidism because of a neonatal screening program that detected a low serum T4 level. His TSH level is normal. The most likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 22 of 63

A 9-year-old boy develops secondary sexual characteristics manifested by the parents. Examination reveals an enlarged testis of 2.7 cm and presence of pubic and axillary hair. Hormonal assessment reveals increased level of testosterone and gonadotropins. Of the following, an important NEXT step in the management is

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ATI RN · Endocrine System Pediatrics

Question 23 of 63

In general, albinism is characterized by all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 24 of 63

Appropriate treatment of the child in the previous question before the onset of serious symptoms is helpful in preventing severe retardation. Overtreatment may result in

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ATI RN · Endocrine System Pediatrics

Question 25 of 63

The mucolipidoses result from the abnormal targeting of lysosomal enzymes to the lysosome. Each statement about the mucolipidoses is correct EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 26 of 63

A 9-year-old boy presented with tiredness on exertion; cardiac auscultation reveals a murmur in aortic area; echo study showed aortic valve disease. On examination he had mild coarse facial features, corneal clouding, joint stiffness, and mild dysostosis multiplex but normal intelligence and stature. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 27 of 63

The least well-defined long-term sequelae of severe prolonged hypoglycemia is

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ATI RN · Endocrine System Pediatrics

Question 28 of 63

One of the following do not appear to be of concern in cystic fibrosis-related diabetes (CFRD)

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ATI RN · Endocrine System Pediatrics

Question 29 of 63

All the following lipidoses are inherited as an autosomal recessive traits EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 30 of 63

An 11-month-old boy presented with repeated convulsions, poor eye contact, exaggerated startle response to noise, and large head. Ophthalmic exam revealed cherry-red spot. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 31 of 63

Matching: Glycogen storage diseases - Von Gierke

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ATI RN · Endocrine System Pediatrics

Question 32 of 63

Classic 21-hydroxylase deficiency occurs in approximately 1 in 15,000-20,000 births in most populations. Of affected infants, the salt-losing form constitute about

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ATI RN · Endocrine System Pediatrics

Question 33 of 63

In addition to routine supportive care, what additional therapy would be most appropriate for the child in the previous question?

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ATI RN · Endocrine System Pediatrics

Question 34 of 63

A 5-month-old infant presented with symmetrical profound muscle weakness, tachypnea, tachycardia, and poor feeding. His chest x-ray showed cardiomegaly and his ECG revealed short PR interval. Of the following, the MOST likely glycogen storage disease is

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ATI RN · Endocrine System Pediatrics

Question 35 of 63

All the following are skeletal manifestations of Morquio disease EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 36 of 63

Matching: X-linked

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ATI RN · Endocrine System Pediatrics

Question 37 of 63

A painless nodule in the thyroid or in the neck is the usual presentation of childhood thyroid cancer. Of the following, the MOST common site of distant metastasis is

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ATI RN · Endocrine System Pediatrics

Question 38 of 63

The condition with the LEAST association with chromosome 22q deletion is

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ATI RN · Endocrine System Pediatrics

Question 39 of 63

McCune-Albright syndrome is associated with all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 40 of 63

Matching: Lipidosis - GM1-gangliosidosis

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ATI RN · Endocrine System Pediatrics

Question 41 of 63

A constant threat of homocystinuria is

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ATI RN · Endocrine System Pediatrics

Question 42 of 63

A 28-day-old girl presented with lethargy, poor feeding, and repeated vomiting for last 5 days; IV fluid and empirical antibiotics were started; later she develops repeated seizures not responding to IV calcium, glucose, B6, and anticonvulsant drugs. Septic screen is negative; serum ammonia is elevated with normal anion gap and normal pH. Family history reveals 2 siblings died with same scenario. Of the following, the MOST likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 43 of 63

The mainstay of treatment of phenylketonurea (PKU) is especial diet. All the following statements are true EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 44 of 63

In primary aldosteronism, one of the following is low

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ATI RN · Endocrine System Pediatrics

Question 45 of 63

Common features of Graves disease include all of the following EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 46 of 63

A 10-year-old presents with 1 year of polyuria and polydipsia. On voiding cystourethrogram, she is noted to have megacystis and hydroureter. Her most likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 47 of 63

A 4-year-old blue-eyed white female manifests a malar flush, mild mental retardation, subluxation of the ocular lens, iridodonesis, and Marfanoid-like features (tall, thin, arachnodactyly). The most likely diagnosis is

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ATI RN · Endocrine System Pediatrics

Question 48 of 63

In X-linked adrenoleukodystrophy (ALD), MRI shows characteristic cerebral white matter lesions mainly in

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ATI RN · Endocrine System Pediatrics

Question 49 of 63

Idiopathic familial hypercalciuria (IHC) is treated by

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ATI RN · Endocrine System Pediatrics

Question 50 of 63

Matching: Isovaleric acidemia

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ATI RN · Endocrine System Pediatrics

Question 51 of 63

Patients with heterozygous familial hypercholesterolemia have xanthomas. The best site to look for xanthomas is

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ATI RN · Endocrine System Pediatrics

Question 52 of 63

All the following are clinical features of acute intermittent porphyria EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 53 of 63

The clearest evidence of a role for viral infection in human type 1 DM is seen in

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ATI RN · Endocrine System Pediatrics

Question 54 of 63

A 12-year-old male adolescent is found to have short stature; lab tests and radiological study confirms idiopathic growth hormone (GH) deficiency. You decide to start treatment with biosynthetic recombinant DNA-derived GH. This adolescent is especially at high risk for development of

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ATI RN · Endocrine System Pediatrics

Question 55 of 63

Cushing syndrome in children has many causes, either exogenous or endogenous. Of the following, the LEAST likely endogenous cause in children is

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ATI RN · Endocrine System Pediatrics

Question 56 of 63

A 27-day-old girl presented with abnormal bicycling movement, lethargy, poor feeding, and repeated vomiting for the last 7 days, and then she developed rapid breathing, opisthotonos, and hypertonicity with bad odor urine. Of the following, the MOST effective mode of therapy is

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ATI RN · Endocrine System Pediatrics

Question 57 of 63

A 13-month-old infant is found comatose in bed after sleeping later than usual. On physical examination, the infant is afebrile and of normal size, and the liver is palpated 4 cm below the costal margin. The plasma glucose level is 15 mg/dL, the bicarbonate level is 20 mEq/L, BUN is 35 mg/dL, ammonia is 295 ÃŽÂ¼mol/L, AST is 320 U/L, ALT is 425 U/L, and bilirubin is normal. Urinalysis is negative for glucose, ketones, protein, and reducing substances. Which of the following is the most likely diagnosis?

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ATI RN · Endocrine System Pediatrics

Question 58 of 63

Type Ia accounts for the majority of patients with pseudohypoparathyroidism (PHP), they have the following skeletal abnormalities EXCEPT

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ATI RN · Endocrine System Pediatrics

Question 59 of 63

The MOST common initial manifestation of X-linked adrenoleukodystrophy (ALD) is

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ATI RN · Endocrine System Pediatrics

Question 60 of 63

A 20-day-old girl presented with prolonged neonatal jaundice, lethargy, and poor feeding treated as sepsis by empirical antibiotics and nothing by mouth. The patient got partial response, when oral feeding resumed, the condition deteriorate again. In this condition, the blood culture MOST likely reveals growth of

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ATI RN · Endocrine System Pediatrics

Question 61 of 63

Guidelines suggest that prepubertal T1DM patients should commence screening for retinopathy

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ATI RN · Endocrine System Pediatrics

Question 62 of 63

Congenital goiter is seen in which percent of newborns with congenital hypothyroidism?

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ATI RN · Endocrine System Pediatrics

Question 63 of 63

The MOST common presentation of primary carnitine deficiency is

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