Pharmacology books and videos 2021


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Educational objective: Tay-Sachs disease is an autosomal recessive disorder caused by [3-hexosaminidase A deficiency, which results in GM2 ganglioside accumulation. Key clinical features include progressive neurodegeneration and a cherry-red macular spot. In contrast to patients with Niemann-Pick disease, those with Tay Sachs disease have no hepatosplenomegaly.


Accumulation of which of the following metabolites is most likely present in this patient's tissues?
Poll
  •   A. Galactocerebroside
  •   B. Globotriaosylceramide
  •   C. Glucocerebroside
  •   D.Glycogen
  •   E. GM2 ganglioside
  •   F. Heparan sulfate
  •   G .Sphingomyelin
829 votes


An 8-month-old girl is brought to the office for evaluation of irritability and regression of motor skills. Her birth was unremarkable and she appeared to develop normally, but she can no longer sit or roll over. Her parents have also noticed that she startles easily with loud noises. Head circumference measurement is consistent with macrocephaly. Bilateral funduscopic evaluation shows a bright red fovea centralis that is surrounded by a contrasting white macula. Peripheral vision is decreased. Abdominal examination is normal.




Educational objective: Neisseria meningitidis can cause sepsis and circulatory collapse in previously healthy young individuals. Lipooligosaccharide, a virulence factor in the pathogens outer membrane, is the major underlying cause of disease severity.


Which of the following microbial components is directly responsible for the severity of disease in this patient?
Poll
  •   A. Capsular polysaccharide
  •   B. lmmunoglobulin protease
  •   C. Lipo-oligosaccharide
  •   D.Lipoteichoic acid
  •   E. Superantigen exotoxin
548 votes


A 16-year-old girl is brought to the emergency department after she became unresponsive. Temperature is 39.7 C (103.5 F), blood pressure is 70/40 mm Hg, and pulse is 130/min and thready. There is a diffuse petechial and ecchymotic skin rash. Laboratory testing reveals leukocytosis with left shift and evidence of disseminated intravascular coagulation and multiorgan failure. Despite aggressive interventions, the patient dies several hours after admission. Autopsy reveals hemorrhagic necrosis of many internal organs, including the bilateral adrenal glands.


Educational objective: Amyotrophic lateral sclerosis (ALS) causes both upper and lower motor neuron lesions. Loss of neurons of the anterior horns of the spinal cord (LMN lesion) causes muscle weakness and atrophy. Demyelination of the lateral corticospinal tract (UMN lesion) leads to spasticity and hyperretlexia.


The patient most likely suffered from which of the following?
Poll
  •   A. Amyotrophic lateral sclerosis
  •   B. Poliomyelitis
  •   C.Rabies
  •   D.Huntington disease
  •   E. Friedreich ataxia
  •   F. Vitamin 812 deficiency
549 votes


A Caucasian male who suffers from a severe neurological disease dies of an overwhelming respiratory infection. Autopsy shows an atrophic precentral gyrus and thin anterior roots of the spinal cord. Light microscopy reveals a severe loss of neurons in the anterior horn of the spinal cord and in the hypoglossal and ambiguous cranial nerve nuclei; corticospinal tracts stain only faintly, indicating demyelinization.


Educational objective: Amyotrophic lateral sclerosis (ALS) causes both upper and lower motor neuron lesions. Loss of neurons of the anterior horns of the spinal cord (LMN lesion) causes muscle weakness and atrophy. Demyelination of the lateral corticospinal tract (UMN lesion) leads to spasticity and hyperretlexia.


The patient most likely suffered from which of the following?
Poll
  •   A. Amyotrophic lateral sclerosis
  •   B. Poliomyelitis
  •   C.Rabies
  •   D.Huntington disease
  •   E. Friedreich ataxia
  •   F. Vitamin 812 deficiency
435 votes


A Caucasian male who suffers from a severe neurological disease dies of an overwhelming respiratory infection. Autopsy shows an atrophic precentral gyrus and thin anterior roots of the spinal cord. Light microscopy reveals a severe loss of neurons in the anterior horn of the spinal cord and in the hypoglossal and ambiguous cranial nerve nuclei; corticospinal tracts stain only faintly, indicating demyelinization.


Educational objective: Botulinum toxin blocks the presynaptic exocytosis of ACh vesicles, causing impaired muscarinic and nicotinic neurotransmission; it characteristically presents as a symmetric, descending paralysis that first manifests with cranial nerve abnormalities, often following consumption of home-canned foods.




Which of the following steps in neuromuscular transmission is most likely affected in this patient?
Poll
  •   A
  •   B
  •   C
  •   D
  •   E
  •   F
393 votes


A 46-year-old man comes to the emergency department due to worsening blurry vision, difficulty swallowing, and dry mouth for the past hour. He has also been having nausea and abdominal discomfort but has no headache or fever. The patient has no prior medical conditions and takes no medications. He smokes a pack of cigarettes daily but does not use alcohol or illicit drugs. The patient has not traveled recently but attended a community potluck event yesterday where he sampled home-canned foods. Physical examination shows bilateral ptosis and sluggish pupillary response.


Educational objective: Neurofibromatosis type I (von Recklinghausen disease) is an autosomal dominant disorder caused by mutations in the NF1 tumor suppressor gene. Patients characteristically develop numerous cutaneous neurofibromas comprised mostly of Schwann cells, which are embryologically derived from the neural crest.


The predominant cells forming these skin tumors most likely originated from which of the following structures?
Poll
  •   A.Endoderm
  •   B.Mesoderm
  •   C.Neural crest
  •   D.Neural tube
  •   E. Notochord
  •   F. Surface ectoderm
342 votes


A 22-year-old man comes to the office due to occasional dull headaches that awaken him from sleep. The patient has no associated neurologic deficits. There is no history of migraines in the family. His temperature is 37.2 C (99 F), blood pressure is 140/80 mm Hg, pulse is 60/min, and respirations are 12/min. On physical examination, there are several 3- to 5-cm flat, pigmented spots on his trunk. There are also multiple, subcentimeter, soft, fleshy, cutaneous tumors located on his trunk and neck.

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