Pace 3 Part 1(Pharmacy)

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Pace 3 Part 1(Pharmacy) - Quiz

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Questions and Answers
  • 1. 

    Dr. Butler A 45-year-old man in a gastroenterology clinic with symptoms of epigastric burning pains after eating is shown by a blood antibody test to have Helicobacter pylori infection.  Which of the following events in pathogenesis predisposed him to develop these symptoms?

    • A.

      Increase in acidity of mucous layer by splitting of urea by urease

    • B.

      Increase in mutations in gastric mucosa during gastritis

    • C.

      Development of atrophic gastritis

    • D.

      Antigenic stimulation of lymphoid tissue in the stomach

    • E.

      Actions of cytotoxin and ammonia to damage gastric mucosa

    Correct Answer
    E. Actions of cytotoxin and ammonia to damage gastric mucosa
    Explanation
    The correct answer is "Actions of cytotoxin and ammonia to damage gastric mucosa." Helicobacter pylori infection can lead to the production of cytotoxins and ammonia by the bacteria, which can damage the gastric mucosa. This damage can result in symptoms such as epigastric burning pains after eating. The other options listed do not directly relate to the damage caused by the cytotoxins and ammonia.

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  • 2. 

    A 35-year-old man went on a camping trip and drank stream water. A week later, he started having cramps and diarrhea that occurred 4-6 times a day. His stools were liquid, greasy, and floated on the toilet water. Stools were passed soon after eating and were worsened by eating dairy products.  How should this patient be managed?

    • A.

      Collect a stool specimen for ova and parasites

    • B.

      Advise him to avoid greasy food and dairy products

    • C.

      Order a blood test for HIV antibodies

    • D.

      Order a barium x-ray of his intestines

    • E.

      Perform a colonoscopy

    Correct Answer
    A. Collect a stool specimen for ova and parasites
    Explanation
    The patient's symptoms, including cramps, diarrhea, and greasy floating stools, are consistent with a possible parasitic infection. Collecting a stool specimen for ova and parasites would allow for laboratory testing to identify any potential parasites causing the symptoms. This would help in determining the appropriate treatment for the patient. The other options, such as advising him to avoid certain foods or ordering different tests, may not directly address the underlying cause of the symptoms.

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  • 3. 

    A 44-year-old male immigrant from Honduras  presented to the clinic for an immigration check up. Specimens of blood, urine, and stool were obtained and sent to the parasitology laboratory. The technician reported that eggs of Taenia solium were found in his stool specimen. Which of the following best describes associated symptoms that were most likely present?

    • A.

      Hematemesis and jaundice

    • B.

      None because of asymptomatic carriage

    • C.

      Bloody diarrhea and enlarged liver

    • D.

      Skin rash and hematuria

    • E.

      Mental confusion and focal seizures

    Correct Answer
    B. None because of asymptomatic carriage
    Explanation
    Called pork tapeworm, also human tapeworm because human is definitive host. Larval cysts are in pigs
    Eggs in human feces infect pigs or infect other humans which develop larval cysts but not adults

    2 clinical forms: Adult tapeworm- no symptoms**************
    Cysticercosis- brain inflammation
    Rarely a human with pork tapeworm can "autoinfect” with eggs and develop cysticercosis

    The cyst form (in brain or other ogans) do not produce eggs and therefore do not release ova into the intestine.

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  • 4. 

    Dr. Bergeron During a food safety conference held on a cruise ship, 164 attendees and crew members are struck with a sudden onset of vomiting (average of 5 times/day), abdominal cramps, and watery diarrhea (average of 8 times/day) within a 48-hour period. At the next scheduled stop, water, food, and stool samples are sent for analysis by local health authorities. Results for the stool samples are as follows:   Watery with no mucus Negative for blood Negative for leukocytes Negative for parasites Negative for viral enterotoxins Negative for bacterial enterotoxins     What is the most likely source of this outbreak?

    • A.

      Whipped cream

    • B.

      Garden salad

    • C.

      Undercooked hamburger meat

    • D.

      Deviled eggs

    • E.

      Undercooked chicken

    Correct Answer
    B. Garden salad
    Explanation
    Caliciviruses (noroviruses)
    Caliciviruses are naked, (+) single-stranded RNA viruses. They are roughly 25 nm to 40 nm in diameter and possess an icosahedral symmetry. Two human calicivirus (HuCV) genera belonging to this family are responsible for human gastroenteritis: Norovirus and Sapovirus. Noroviruses (NoV) include the Norwalk (NV) and Norwalk-like viruses (NLV), whereas sapoviruses (SaV) include the Sapporo-like viruses (SLV); all these viruses are currently grouped under the term noroviruses (I mention this because many people use all of these names!). Because these viruses cannot be grown in culture, there are as of yet no serological groupings possible; they are however divided into genogroups (5 NoV genogroups & 5 SaV genogroups) and genotypes. All these viruses present very narrow host specificity (humans; experimental infections in other primates has been difficult).
    Noroviruses occur throughout the world and represent 60 to over 90% of non-bacterial gastroenteritis outbreaks (developing & developed world), which occur as outbreaks mostly during the cooler months of the year3. In fact, NoV are the most important cause of waterborne and foodborne diseases. Norovirus infections affect young children and adults alike, normally as community-wide outbreaks. Noroviruses are highly infectious and infections spread very rapidly [secondary contact infection is common (> 50 to 90%)]. Outbreaks also often occur in institutionalized settings (school, day care, hospitals, orphanages, hotels, cruise ships, nursing homes, the military, camps etc.). Transmission occurs primarily through faecal-oral route, although airborne and fomite transmission may also likely occur (being in the same room as someone with projectile vomiting is an important risk factor, and the virus can be recovered from the vomitus).

    Contaminated water (drinking, swimming & ice) and food (undercooked shellfish, *****FRUITS & VEGETABLES****, frosting, pasta, sandwiches & cold cuts) are the main culprits so far identified. Viral particles are resistant to a wide variety of detrimental treatments including chlorination, low pH, solvents, heat, cold, ethanol, detergents and quaternary ammonium; they can however be inactivated by glutaraldehyde, iodine-based disinfectants and fresh bleach. Surfaces can be efficiently disinfected using a combination of detergent and bleach.
    Norovirus infection spans a wide spectrum of presentations varying from asymptomatic to severe dehydration. When compared with childhood acute gastroenteritis, especially when compared to rotavirus acute gastroenteritis, NoV gastroenteritis usually presents as a milder, self-limiting disease; this is generally (but not always) a distinguishing feature of NoV infection.
    ****THE INCUBATION PERIOD IS LESS THAN 50 HOURS, FOLLOWED BY A SUDDEN ONSET OF VOMITING AND/OR WATERY DIARRHEA (USUALLY BOTH). STOOLS USUALLY SHOW NO BLOOD, NO MUCOUS AND NO LEUKOCYTES.****
    The symptomatic period is short, lasting 1 to 3 days. Other symptoms may include nausea, malaise, abdominal cramps and dehydration; there is usually no fever. As previously mentioned, disease is self-limited. Symptomatic illness is linked with carbohydrate malabsorption and steatorrhea due to a decrease in microvilli enzymatic activity [alkaline phosphatase (regulation of fat absorption; bi-directionnal), sucrase (sucrose  fructose + glucose; leads to malabsorption, gas & diarrhoea) & trehalase (decreased trehalose hydrolysis diarrhoea)], and correlates with blunting and broadening of the intestinal villi, crypt cell hyperplasia, cytoplasmic vacuolization (fat?), as well as polymorphonuclear and mononuclear infiltration into the lamina propria.

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  • 5. 

    A 27-year-old female sex worker presents to a free clinic complaining of tea-colored urine and clay-colored stools. Patient history reveals a recent trip to Mexico and non-specific systemic symptoms such as fatigue, nausea, and low-grade fever. She admits to alcohol and injection drug abuse. Physical examination reveals epigastric discomfort and yellow-tinged sclerae. Blood work shows the following: Three years later, a new panel is performed on the same patient, with the following results: This patient would have been completely protected from HDV infection had she been seropositive for which antibody?

    • A.

      Anti-HAV

    • B.

      Anti-HCV

    • C.

      Anti-HDV

    • D.

      Anti-HEV

    • E.

      Anti-HBsAg

    Correct Answer
    E. Anti-HBsAg
    Explanation
    The correct answer is Anti-HBsAg because it indicates the presence of antibodies against the hepatitis B surface antigen. Hepatitis B virus (HBV) infection is a risk factor for hepatitis D virus (HDV) infection, as HDV requires HBV for replication. Therefore, if the patient had been seropositive for Anti-HBsAg, it would indicate that she had been previously infected with HBV and developed immunity, thus providing complete protection against HDV infection.

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  • 6. 

    Using the graph below depicting the progression of a hepatitis A infection, which week is most likely to correspond to the peak CTL response?

    • A.

      4

    • B.

      6

    • C.

      8

    • D.

      10

    • E.

      12

    Correct Answer
    A. 4
    Explanation
    The peak CTL response is most likely to occur during the fourth week of the hepatitis A infection. This is because the graph shows a steady increase in the CTL response until the fourth week, after which it starts to decline. Therefore, the highest point on the graph is likely to correspond to the peak CTL response, which occurs at week 4.

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  • 7. 

    Dr. Kelley A retired 65-year-old female physician is hospitalized with a 2-day history of headache, weakness, loss of appetite, and a progressively worsening high fever. She had just returned yesterday from a month-long trip to provide medical care in rural India. Although 10 days prior to admission she had a diarrheal illness that lasted for 2-3 days, now she reports that she has been constipated for the past 3 days. Physical examination is unremarkable except for numerous erythematous, maculopapular lesions on her trunk. Blood and stool cultures grow a Gram-negative bacillus which produces colorless colonies on MacConkey agar and colonies with a black center on Hektoen enteric agar. What is the most likely causative agent?

    • A.

      Shigella dysenteriae

    • B.

      Enterohemorrhagic Escherichia coli

    • C.

      Salmonella Typhimurium

    • D.

      Campylobacter jejuni

    • E.

      Salmonella typhi

    Correct Answer
    E. Salmonella typhi
    Explanation
    The most likely causative agent in this case is Salmonella typhi. The patient's symptoms of headache, weakness, loss of appetite, and high fever, along with a recent trip to rural India, are consistent with typhoid fever, which is caused by Salmonella typhi. The presence of erythematous, maculopapular lesions on her trunk further supports this diagnosis. The growth of a Gram-negative bacillus on culture, which produces colorless colonies on MacConkey agar and colonies with a black center on Hektoen enteric agar, is also characteristic of Salmonella typhi.

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  • 8. 

    A large outbreak of gastroenteritis (watery diarrhea and abdominal cramping) occurred among factory workers 10-12 hours after eating at a buffet luncheon. The meal included roast beef, mashed potatoes, and gravy. It had been prepared 12-24 hours before serving, improperly cooled at room temperature, and quickly reheated just before serving. The illness resolved within 24-48 hours. What are the key characteristics of the most likely causative agent?

    • A.

      Aerobic, sporeforming, Gram-positive bacillus

    • B.

      Facultative, coagulase-positive, Gram-positive cocci in clusters

    • C.

      Anaerobic, nonsporeforming, Gram-positive bacillus

    • D.

      Anaerobic, sporeforming, Gram-positive bacillus

    • E.

      Aerobic, oxidase-positive, Gram-negative bacillus

    Correct Answer
    D. Anaerobic, sporeforming, Gram-positive bacillus
    Explanation
    The key characteristics of the most likely causative agent in this scenario are anaerobic, sporeforming, and Gram-positive bacillus. This is because the outbreak of gastroenteritis occurred after consuming improperly cooled and reheated food, which is a common cause of foodborne illnesses caused by anaerobic bacteria. Sporeforming bacteria have the ability to survive harsh conditions, such as improper food handling and reheating. Gram-positive bacillus indicates the type of bacterial cell wall composition, which is commonly associated with foodborne pathogens.

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  • 9. 

    Dr. Loy A 50-year-old woman expresses concern that she might develop esophageal adenocarcinoma because her mother had it.  What is the most common cause of esophageal adenocarcinoma in the United States?

    • A.

      Candida esophagitis

    • B.

      Herpes esophagitis

    • C.

      Helicobacter pylori infection

    • D.

      Spicy food

    • E.

      Reflux esophagitis

    Correct Answer
    E. Reflux esophagitis
    Explanation
    Reflux esophagitis is the most common cause of esophageal adenocarcinoma in the United States. Chronic gastroesophageal reflux disease (GERD) can lead to the development of Barrett's esophagus, a condition in which the normal lining of the esophagus is replaced by abnormal cells. Over time, these abnormal cells can progress to esophageal adenocarcinoma. This is why the woman is concerned about her risk, as reflux esophagitis is a known risk factor for esophageal adenocarcinoma.

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  • 10. 

    A 60-year-old man undergoes biopsy of a 1-cm gastric mass (shown below). Chromogranin stain is strongly and diffusely positive. (see colored image) What is the most likely diagnosis?

    • A.

      Adenocarcinoma

    • B.

      Adenoma

    • C.

      Carcinoid tumor

    • D.

      GIST tumor

    • E.

      Lymphoma

    Correct Answer
    C. Carcinoid tumor
    Explanation
    The most likely diagnosis in this case is a carcinoid tumor. This is suggested by the strong and diffuse positive staining of the chromogranin stain. Carcinoid tumors are neuroendocrine tumors that commonly occur in the gastrointestinal tract, including the stomach. They can secrete various hormones and bioactive substances, which can cause symptoms such as flushing, diarrhea, and wheezing. The positive chromogranin stain indicates the presence of neuroendocrine cells, which is a characteristic feature of carcinoid tumors. Adenocarcinoma, adenoma, GIST tumor, and lymphoma are less likely based on the staining pattern described.

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  • 11. 

    A 30-year-old woman with malabsorption undergoes a duodenal biopsy which shows villous atrophy and increased intraepithelial lymphocytes.  Avoiding what item would most improve her symptoms?

    • A.

      NSAIDS

    • B.

      Alcohol

    • C.

      Tobacco

    • D.

      Meat

    • E.

      Gluten

    Correct Answer
    E. Gluten
    Explanation
    The woman's duodenal biopsy shows villous atrophy and increased intraepithelial lymphocytes, which are characteristic findings of celiac disease. Celiac disease is an autoimmune disorder triggered by the consumption of gluten, a protein found in wheat, barley, and rye. Avoiding gluten in her diet would most improve her symptoms and help her manage her malabsorption.

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  • 12. 

    A 23-year-old man presents with bloody diarrhea and weight loss.  Colonoscopy shows patchy areas of ulceration involving the rectum, colon and terminal ileum.  Biopsy of the rectum shows glandular disorder, crypt abscesses, paneth cell metaplasia, and granulomas.  What is the most likely diagnosis?

    • A.

      Crohn disease

    • B.

      Irritable bowel disease

    • C.

      Cholera

    • D.

      Escherichia coli infection

    • E.

      Ulcerative colitis

    Correct Answer
    A. Crohn disease
    Explanation
    The presentation of bloody diarrhea, weight loss, and patchy areas of ulceration involving multiple parts of the gastrointestinal tract, along with the histological findings of glandular disorder, crypt abscesses, paneth cell metaplasia, and granulomas, are consistent with Crohn's disease. This chronic inflammatory bowel disease commonly affects the terminal ileum and colon, but can involve any part of the gastrointestinal tract. Irritable bowel disease is not a recognized medical condition, Cholera and Escherichia coli infection typically present with watery diarrhea, and Ulcerative colitis primarily involves the rectum and colon without granulomas.

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  • 13. 

    A 19-year-old lady sees you for rectal bleeding.  Colonoscopy shows numerous (> 100) colon polyps.  Biopsies of 10 of the polyps all show tubular adenomas.  History reveals the patient’s father developed colon cancer at age 30.  She wants testing to see if her baby daughter will be affected with this disease.  What molecular abnormality would you look for in the daughter?

    • A.

      Kras mutation

    • B.

      Trisomy 21

    • C.

      P53 mutation

    • D.

      Mutation of APC (adenomatous polyposis coli) gene

    • E.

      DNA mismatch repair mutations

    Correct Answer
    D. Mutation of APC (adenomatous polyposis coli) gene
    Explanation
    The correct answer is Mutation of APC (adenomatous polyposis coli) gene. This is because the patient has multiple colon polyps, which are characteristic of familial adenomatous polyposis (FAP). FAP is caused by a mutation in the APC gene, which is an important tumor suppressor gene involved in regulating cell growth and division. Since the patient's father also had colon cancer at a young age, there is a high likelihood of an inherited genetic mutation. Testing the baby daughter for the APC gene mutation can help determine if she is at risk for developing FAP and colon cancer in the future.

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  • 14. 

    Dr. Mallik.G A 57-year-old woman had profuse menstrual bleeding for the last two years. Now, she is feeling weak and gets palpitations even with light work. She came to the doctor’s office with lightheadedness and vertigo. The physician found her anemic with spoon-shaped finger nails. Her investigations showed Hemoglobin 5 gm/dl and microcytic hypochromic anemia. The mean corpuscular hemoglobin concentration was also reduced. On further questioning, she stated that she has developed some difficulty in swallowing over the last month. What is a dangerous complication in this case?  

    • A.

      Esophageal perforation

    • B.

      Esophageal stricture

    • C.

      Primary lung cancer

    • D.

      Esophageal spasm

    • E.

      Esophageal malignancy

    Correct Answer
    E. Esophageal malignancy
    Explanation
    The dangerous complication in this case is esophageal malignancy. The patient's symptoms of difficulty in swallowing, along with the presence of anemia and spoon-shaped finger nails, suggest the possibility of an underlying esophageal malignancy. Esophageal malignancy can cause obstruction or narrowing of the esophagus, leading to difficulty in swallowing. The patient's anemia and microcytic hypochromic blood picture may be related to chronic bleeding from the malignancy. It is important to investigate further and confirm the diagnosis to initiate appropriate treatment.

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  • 15. 

    A 20-year-old woman complains of increased bowel frequency and recurrent abdominal pain over the last 6 months. The pain is typically relieved by passing motion. She also has liquid stools with the onset of pain. On examination the patient is not anemic; her abdomen is soft, with slight tenderness to the lower right abdomen. Guarding and rebound were absent.  She has no history of vomiting, bleeding per rectum or weight loss. What is the most likely diagnosis?  

    • A.

      Crohn disease

    • B.

      Ulcerative colitis

    • C.

      Small bowel lymphoma

    • D.

      Cancer ascending colon

    • E.

      Irritable bowel syndrome

    Correct Answer
    E. Irritable bowel syndrome
    Explanation
    Based on the given information, the most likely diagnosis for the 20-year-old woman is irritable bowel syndrome (IBS). IBS is a common gastrointestinal disorder characterized by abdominal pain or discomfort, changes in bowel habits (such as increased frequency or diarrhea), and relief of symptoms after passing stool. The absence of anemia, presence of soft abdomen with slight tenderness to the lower right abdomen, and the absence of other symptoms like vomiting, rectal bleeding, or weight loss suggest a functional disorder like IBS rather than an organic cause like Crohn's disease, ulcerative colitis, small bowel lymphoma, or cancer of the ascending colon.

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  • 16. 

    A 76-year-old dehydrated woman with a long history of gallstone disease was brought to the hospital with severe central abdominal pain, bilious vomiting, abdominal distension and absolute constipation. Her abdomen was soft without guarding and rebound. Peristaltic sounds were loud. Abdominal X-ray showed a severely dilated small gut with a collapsed large gut (see image). If the pathology is due to gallstones what was the likely diagnosis?

    • A.

      Acute cholecystitis

    • B.

      Gallstone ileus

    • C.

      Cancer colon due to gallstone

    • D.

      Intussusception due to gallstone

    • E.

      Obstruction of bile duct by the stone

    Correct Answer
    B. Gallstone ileus
    Explanation
    GALLSTONE ILEUS is an important, though infrequent cause of mechanical bowel obstruction, affecting elderly patients who often have other significant medical conditions. It is caused by impaction of a gallstone in the ileum after being passed through a BILIARY-ENTERIC FISTULA. The diagnosis is often delayed since symptoms may be intermittent and investigations fail to identify the cause of the obstruction. The mainstay of treatment is removal of the obstructing stone after resuscitating the patient. Gallstone ileus continues to be associated with relatively high rates of morbidity and mortality.

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  • 17. 

    A 35-year-old man with a strong family history of bowel cancer presents with blood thoroughly mixed with stool. On physical examination he is found to have multiple dark pigmentations on his buccal mucosa and lips. The DRE showed altered blood on the finger. Colonoscopy showed some polyps in the large gut; the one which was bleeding was in the ascending colon and was about 2 cm x 1.5 cm in size. The biopsy of the polyp showed a hamartomatous lesion. What is the likely diagnosis?

    • A.

      Turcot syndrome

    • B.

      Gardner syndrome

    • C.

      Peutz Jeghers syndrome

    • D.

      Familial adenomatous polyposis

    • E.

      Cowden syndrome

    Correct Answer
    C. Peutz Jeghers syndrome
    Explanation
    The likely diagnosis in this case is Peutz Jeghers syndrome. This is supported by the patient's presentation of blood mixed with stool, multiple dark pigmentations on the buccal mucosa and lips, and the presence of polyps in the large intestine. Peutz Jeghers syndrome is a rare genetic disorder characterized by the development of hamartomatous polyps in the gastrointestinal tract, as well as mucocutaneous pigmentation. It is associated with an increased risk of developing various types of cancer, including bowel cancer.

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  • 18. 

    A first-born 6-week-old male baby is brought to the physician for projectile non-bilious vomiting. The mother said that the child had occasionally been retching and throwing up over the last week, but the vomiting is now severe and occurs after each feed. The baby appears very hungry after vomiting. Upon questioning, the mother said that she herself had an abdominal surgery in childhood, but she cannot remember its exact nature. On examination the physician found the baby to be dehydrated, and palpated an olive-shaped lump in the upper abdomen. What is the most likely diagnosis?  

    • A.

      Congenital pyloric stenosis

    • B.

      Necrotizing enterocolitis

    • C.

      Meckel diverticululitis

    • D.

      Hirschsprung’s disease

    • E.

      Lower duodenal atresia

    Correct Answer
    A. Congenital pyloric stenosis
    Explanation
    The most likely diagnosis in this case is congenital pyloric stenosis. The symptoms of projectile non-bilious vomiting, severe vomiting after each feed, and a palpable olive-shaped lump in the upper abdomen are consistent with this condition. Congenital pyloric stenosis is a condition in which the muscle at the outlet of the stomach (pylorus) becomes thickened, leading to a blockage of food from leaving the stomach. This condition is more common in first-born males and can cause dehydration and hunger after vomiting. The mother's history of abdominal surgery in childhood may be a risk factor for the development of pyloric stenosis in the baby.

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  • 19. 

    A 7-day-old formula-fed premature neonate was brought with lethargy, bilious vomiting, abdominal distention and bloody stools. On examination, the abdominal wall had erythema, edema, and crepitus; the abdomen was distended with guarding but bowel sounds were absent.  Abdominal X-ray showed free air within the peritoneal cavity and also in the layers of bowel wall. What is the most likely cause of his presentation?

    • A.

      Meckel diverticululitis

    • B.

      Hirschsprung’s disease

    • C.

      Necrotizing enterocolitis

    • D.

      Lower duodenal atresia

    • E.

      Congenital pyloric stenosis

    Correct Answer
    C. Necrotizing enterocolitis
    Explanation
    Necrotizing enterocolitis (NEC) is one of the most common gastrointestinal emergencies in the newborn infant. It is a disorder characterized by ischemic necrosis of the intestinal mucosa, which is associated with inflammation, invasion of enteric gas forming organisms, and dissection of gas into the muscularis and portal venous system. Although early recognition and aggressive treatment of this disorder has improved clinical outcomes, NEC accounts for substantial long-term morbidity in survivors of neonatal intensive care, particularly in premature very low birth weight infants (birth weight below 1500 g).

    The incidence decreases with increasing gestational age (GA) and birth weight (BW), and is about 6 to 7 percent in infants with very low birth weight (VLBW) infants (BW less than 1500 g)

    CLINICAL PRESENTATION — The majority of premature infants who develop NEC are healthy, feeding well, and growing. A change in feeding tolerance with gastric retention is a frequent early sign. The timing of the onset of symptoms varies and appears to be inversely related to gestational age . Overall, 25 percent of cases present later than 30 days after birth. The median age at onset in infants with a gestational age of less than 26 weeks was 23 days, and for those with a gestational age of greater than 31 weeks, the median age at onset was 11 days.

    DIAGNOSIS — The diagnosis of NEC is based on the presence of the characteristic clinical features of abdominal distention and rectal bleeding (heme-positive or grossly bloody stools), and the abdominal radiographic finding of pneumatosis intestinalis.

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  • 20. 

    A 40-year-old alcoholic woman presents with fatigue and intense itching all over her body. About 5 years earlier she had a history of vomiting blood after binge drinking. On examination she has painless, low-midline neck swelling that moves up and down with swallowing. She also appeared jaundiced, with liver enlargement and xanthomas. Lab investigations showed elevated serum cholesterol, serum alkaline phosphatase, GGT and antimitochondrial antibody. Which of the following is the most likely diagnosis?

    • A.

      Primary biliary cirrhosis

    • B.

      Hepatoma

    • C.

      Cholangiocarcinoma

    • D.

      Hepatocellular carcinoma

    • E.

      Sclerosing cholangitis

    Correct Answer
    A. Primary biliary cirrhosis
    Explanation
    INTRODUCTION — Primary biliary cirrhosis (PBC) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually results in cirrhosis and liver failure.
    The signs and symptoms of PBC differ from those of most other liver diseases; thus, the diagnosis may be strongly suspected based upon the clinical findings. As an example, pruritus, hyperpigmentation of the skin, and hepatomegaly in a woman with a cholestatic pattern of liver function tests are common in PBC but unusual in other liver disorders such as acute and chronic hepatitis and alcoholic liver disease
    EPIDEMIOLOGY — In contrast to other types of liver disease, 95 percent of patients with PBC are women. PBC is unique among the autoimmune diseases in that it rarely occurs in childhood or before age 30. The onset is usually between the ages of 30 to 65, but the disease has been reported in women as young as 15 and as old as 93. PBC is more common in Caucasian women, but can be seen in other ethnic groups. One study found that disease severity at presentation was higher in non-Caucasians for reasons that were unclear

    CLINICAL MANIFESTATIONS AT PRESENTATION — Fatigue and pruritus used to be the most common presenting symptoms of PBC . However, now that the disease is more widely recognized and diagnosed at its earlier stages, approximately 50 to 60 percent of patients are asymptomatic at diagnosis. In series published in the 1970s, for example, up to 78 percent of patients had fatigue at presentation. More recent reports have found that, in newly diagnosed patients, approximately one-half complain of fatigue and one-third pruritus

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