MRCPH/FCPS MCQS: Learn Nocturnal Enuresis, Nuerofibromitosis type 1, Bartter syndrome, Liddle syndrome, Polycystic kidney disease, Lactose intolerance

Nocturnal Enuresis, Nuerofibromitosis type 1, Bartter syndrome, Liddle syndrome, Polycystic kidney disease, Lactose intolerance

LEARN MRCPH/FCPS PEDIATRICS MCQS

PEDIATRIc MCQs: Learn about Nocturnal enuresis, Polycystic kidney disease, Bartter syndrome, Neurofibrosis 1

Quiz

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1. The father of a 6-year-old girl brings his daughter in for evaluation of bed wetting. He states that she wets the bed approximately 3 nights per week and has never consistently remained dry throughout the night. A urinalysis is performed in the clinic and has a specific gravity of 1.020 and is negative for glucose, nitrates, or leukocyte esterase.
   Which of the following is appropriate advice to give to the patient’s father?
   1.   Counsel that nocturnal enuresis is unlikely to resolve without pharmacologic intervention
   2.   Instruct him to restrict his daughter’s intake of beverages prior to bedtime
   3.   Advise him against obtaining a bed wetting alarm, as the alarms have not been shown to be effective
   4.   Suggest that the child void frequently during the daytime
   5.   Counsel him to punish his daughter by having her change her bed sheets alone after every accident
2. You are seeing a 12-year-old girl with neurofibromatosis type 1 (NF-1) in follow-up in your clinic. She was diagnosed with NF-1 at 3 years of age based on the presence of multiple café au lait macules and axillary freckling. She is followed regularly by ophthalmology for optic gliomas and has an individualized education plan (IEP) for learning difficulties. She is in clinic with her mother, who wants to know if there are any other features of NF-1 that she should be monitoring for.
   Which of the following findings typically do not appear until after puberty in patients with NF-1?
   1.   Dermal neurofibromas
   2.   Plexiform neurofibromas
   3.   Lisch nodules
   4.   Sphenoid dysplasia
   5.   Central nervous system tumors
3. A 9-month-old male presents to clinic with feeding difficulties and change in growth curve percentile from the 50th% at 4 months of age to 10th% at present. On physical examination, he is noted to be irritable with a slightly sunken fontanelle. You obtain the following laboratory values: Na 141 mEq/L (normal: 135–145), K 2.1 mEq/L (normal: 3.5–5.0), Cl 97 mmol/L (normal: 98–108), HCO 3 11 mmol/L (normal: 22–30), BUN 5 mg/dL (normal: 6–22 mg/dL), Cr < 0.3 mg/dL (normal: 0.2–0.4), Ph 1.2 mg/dL (normal: 3.2–6.3), Mg 1.1 mEq/L (normal: 1.3–2.0). Urinalysis is notable for pH of 5 and the presence of glucose.
   Which of the following is the most likely underlying diagnosis in this patient?
   1.   Acute gastroenteritis
   2.   Bartter syndrome
   3.   Liddle syndrome
   4.   Cystinosis
   5.   Lithium intoxication
4. Which of the following is correct regarding autosomal recessive polycystic kidney disease (ARPKD)?
   1.   Enlargement of fetal kidneys is evident on firsttrimester
      ultrasound
   2.   Liver dysfunction from congenital hepatic fibrosis
      frequently develops prior to renal dysfunction
   3.   A negative renal ultrasound in both parents makes
      the diagnosis of ARPKD more likely than autosomal
      dominant polycystic kidney disease (ADPKD)
   4.   The majority of patient’s with ARPKD will be
      diagnosed prenatally
   5.   ARPKD is more common than ADPKD
5. A 20-month-old female is seen in clinic with a 4-month history of 3 to 5 watery stools per day. She is in the 60th
   percentile for weight and 75th percentile for height. Her mother describes her as a playful child and has no concerns other than the diarrhea. Dietary history reveals that the girl eats 3 meals and at least 2 small snacks and drinks 2 to 3 cups of apple juice in addition to 12 oz of cow’s milk each day.
   Which of the following is the next appropriate step in management of this patient?
   1.   Determination of stool electrolytes and osmolality
   2.   Determination of stool pH and reducing substances
   3.   Stool culture
   4.   Two-week trial of lactose-free diet
   5.   Two-week trial of juice-free diet