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nbme 1 bl 4 - dnc4
#1
everyone, thanx in advance..

9. A 3-month-old boy is brought for a well-child examination. He has poor head control. Examination shows generalized hypotonia. The point of maximal impulse is at the left anterior axillary line. The liver edge is palpated 4 cm below the right costal margin. The spleen is not palpable. Which of the following is the most likely diagnosis?

A
) Congenital muscular dystrophy

B
) Glycogen storage disease, type II (Pompe's disease)

C
) GM1 gangliosidosis

D
) Infant botulism

E
) Ventricular septal defect

10. Over the past 3 months, a 30-year-old woman has had intermittent episodes of headache, palpitations, sweating, and irritability. Her blood pressure is 150/100 mm Hg while supine and 149/100 mm Hg while standing, and pulse is 90/min while supine and 110/min while standing. Examination shows no abnormalities except for pallor. Which of the following is the most likely location of this patient's lesion?

A
) Adrenal gland

B
) Brain

C
) Heart

D
) Kidney

E
) Thyroid gland

16. A 1-year study of a new drug to treat hypertension is conducted. One hundred patients with hypertension are enrolled; 50 patients are given the new drug and another 50 patients are given hydrochlorothiazide. All patients completed the trial. One noted unexpected effect is increased growth of scalp hair which occurred in those taking the new drug, a nonstatistically significant difference (p>0.10). This effect has also been reported in studies of other similar drugs in the new therapeutic class. The investigators of the study concluded that the new drug did not cause hair growth. Which of the following features of this study is most likely to affect the validity of this conclusion?

A
) Differential follow-up

B
) Lead time bias

C
) Length of the study

D
) Sample size

E
) Self-selection

19. A 6-year-old boy is brought to the physician by his mother because of progressive visual loss over the past year. Over the past 2 years, he has had deterioration of his hearing, speech, writing, and intellectual performance. His maternal uncle had similar symptoms. Visual acuity is 20/200 bilaterally. Funduscopic examination shows optic atrophy. His hearing is markedly impaired. There is weakness and spasticity of all extremities. Deep tendon reflexes are extremely hyperactive. Babinski's sign is present bilaterally. On mental status examination, he is not oriented to place, year, month, or the names of his siblings. An MRI of the brain shows marked symmetric white matter disease involving all lobes. Diagnostic studies are most likely to show which of the following?

A
) Abnormally decreased serum cholesterol level

B
) Acanthocytes on blood smear

C
) An excess of very long chain fatty acids

D
) Normal nerve conduction studies

E
) Vitamin E deficiency

22. A 5-year-old boy is brought to the emergency department 30 minutes after he fainted at home after standing up from a sitting position. His symptoms began 3 days ago with diarrhea and vomiting. He has had no urine output for 18 hours. He is alert but quiet. His temperature is 37.5 C (99.5 F), blood pressure is 75/45 mm Hg, pulse is 120/min, and respirations are 28/min. Examination shows dry lips and tenting of the skin. There is no abdominal tenderness. Bowel sounds are hyperactive. The remainder of the examination shows no abnormalities. His capillary refill time is 5 seconds. Intravenous bolus doses of 0.9% saline are administered. Bladder catheterization yields 5 mL of urine. Urinalysis is most likely to show which of the following?

A
) Blood

B
) Erythrocyte casts

C
) Hyaline casts

D
) Leukocyte casts

E
) Oxalate crystals
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#2
9.C
10A?
19c
22c.
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#3
10 yes, a, its pheochromocytoma
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#4
thanks for ur confirmation.
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#5
can u explain briefly, why ?
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#6
The first one is pheocromocytoma. as mentioned by kanita.The adrenal medulla is involved( the central portion of adrenal gland)
Q 9 :i thought abt it n chnage my mind .it looks pompe's to me.Since pompe's occurs int he first few wks of life thats y i din choose pompes.but other than the time of occurence. , the q mentions everything that cud occur in pompes.so q 9 is pompes.correct me if i am wrong pls.
Q22 is ATN due to dehydration resultuing in the excretion of hyaline casts ,peculiar to ATN.
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#7
B ,pompes disease....involving heart,liver,hypotonia.gangliosidosis picture wud b different wid hepatosplenomegaly and aeizures,coarsening of facial features,skeletal defects etc...
A , intermittent or episodic...s/o pheochromocytoma

? A/B
C ,history s/o adrenoleukodystrophy..accuulation of long chain fatty acids in nervous system myelin sheath
C , picture of hypovolemic shock wid acute renal failure wid no underlying renal,urinary tract path
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#8
How about 16? C?. When I did lab research, if I got p=0.1, I will add more samples.
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#9
So I think 16 should be D. But the answer I have is C. ???
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#10
9.B -- This is straight forward for Pompe's Disease
10.A -- Pheocromocytoma
16.A -- The question is specifically asking the validity of the results, when referring to validity it means whether there is a systematic error in the study, in other words a Bias. Before determining an adequate sample size, one must do a power analysis first prior starting.
Questions states that there is a non-statistically difference for the hair growth (p=0.10), meaning that there is no difference between the control group and the new drug group.
19.C -- Adrenoleukodistrophy (everytime I see a similar question reminds me of the movie "Lorenzo's Oil")
22.C -- Patient is dehydrated big time, hyaline casts are seen in this type of scenario
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