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A 23-year-old African-American presents with acute-onset pain in the abdomen, back, and legs. On physical examination, his pulse is 115 bpm, respiratory rate is 20, blood pressure is 100/70 mmHg, and temperature is 101°F. There is scleral icterus, a s ystolic ejection murmur at the right upper sternal border, bilateral rhonchi, a right upper quadrant abdominal scar from a cholecystectomy, and a diffusely tender abdomen without rebound. A neurologic examination is normal. A peripheral blood smear is shown in Figure

.



The patient develops pain in both hips and an MRI shows avascular necrosis. This is likely due to which of the following?

  1. osteomyelitis due to Salmonella infection
  2. HIV infection
  3. a pituitary tumor causing Cushing syndrome
  4. associated arthritis
  5. chronic ischemia to the head of the femur

Answer(s): E

Explanation:

This patient has sickle cell anemia. This is evident from sickle cells forms on the peripheral blood smear in Figure 1-13. Pain medication is an important initial concern. It is often difficult to determine whether a patient in sickle cell crisis has an ongoing infection. Infections can precipitate sickle cell crisis. With respect to community-acquired pneumonia, the diagnosis is difficult. A patient with sickle cell crisis can have fever as a result of the sickle crisis. They can have an increased respiratory rate, physical examination, and CXR findings which suggest pneumonia as a result of pulmonary infarctions. A white blood count can be elevated due to marrow stimulation. In the presence of the acute chest syndrome, characterized by chest pain, hypoxia, and CXR infiltrates, antibiotics would be indicated. Without further information, it is hard to decide to empirically start broad-spectrum antibiotics for community-acquired pneumonia. Transfusions should generally be avoided in patients with sickle cell anemia who are not symptomatic due to the anemia. Since patients in sickle cell crisis have intravascular hemolysis, their reticulocyte counts are usually high and they can replace their blood quickly. Repeated small transfusions will lead to autoantibodies that will make further transfusions difficult. Arterial blood gas determination should not be the first step, given the above information. A CT scan of the abdomen is not indicated given the nonspecific nature of the patient's abdominal findings. Parvovirus B19 can cause aplastic crisis in patients with hemoglobinopathies, including sickle cell disease. A tip to this diagnosis is the decreased reticulocyte count in a patient who normally would have a high reticulocyte count. Given the information listed above, there is no indication for broad- spectrum antibiotics. Splenectomy is not a reasonable alternative at this point. Patients with sickle cell disease have autosplenectomy by the time they are adults. A bone marrow biopsy maybe indicated because of the low platelet count, but not initially. GnCSF is not indicated because the patient does not have neutropenia.
Patients with sickle cell disease typically have isosthenuria.

This is due to repeated infarction of the renal papilli. This causes destruction and interference with the counter current mechanism that causes urine concentration. As a result, patients with sickle cell anemia have the inability to concentrate their urine. This results in fluid and electrolyte abnormalities. Patients in sickle cell crisis are usually fluid depleted. This and the sickled blood cells cause hyperviscosity and microinfarctions. There is no evidence that the patient has diabetes insipidus, which usually has a urine specific gravity less than 1.005. Patients with UTIs do not have isosthenuria due to RBCs and WBCs that increase the urine specific gravity. Sickle cell patients may have zinc deficiency, but this is not a cause of isosthenuria.



1. A22-year-old male presents to the emergency department (ED) with complaints of right-sided chest pain and dyspnea. He has no other significant medical history. There is no history of trauma. On examination, he has a pulse of 95, BP of 110/70, and SpO2 of 95% on 2 L. A chest x-ray reveals a large right pneumothorax.

Which of the following statements is true?

  1. Since the patient is hemodynamically stable, he can be observed with oxygen supplementation, pain control, and serial chest x-rays.
  2. The patient is likely to have a tall, thin habitus.
  3. This condition is probably due to small lacerations in the apex of the right lung.
  4. His risk of recurrence is 10%.
  5. Recurrences are usually on the contralateral side since adhesions prevent recurrence on the ipsilateral side.

Answer(s): B

Explanation:

Spontaneous pneumothorax is usually found in young males. A tall, thin habitus is common. Eighty- five percent of patients are found to have pulmonary blebs on the affected side. The correct management is placement of a chest tube, pain control, oxygen supplementation, and serial chest x-rays to monitor resolution. Thoracotomy is required if the pneumothorax does not resolve with a chest tube or if there is a persistent air leak. Bleb resection and pleurodesis is usually performed at the time of operation to prevent future bleb rupture and to promote adhesion of the lung to the chest wall. Thoracotomy is also offered to patients after a recurrence to prevent future episodes. Fifty percent of patients will have a recurrence on the ipsilateral side after a spontaneous pneumothorax.



A 10-month-old male presents with a 12-hour history of episodes of crying, holding his stomach, and bending over in pain. The parents report one "reddish" stool. He has no past medical history or episodes of similar events. He did have 24 hours of viral symptoms, which resolved a few days ago.
The following study was obtained.

Which of the following statements is true?

  1. The initial treatment for this child involves emergent laparotomy.
  2. Air contrast enema can be diagnostic AND therapeutic.
  3. Colonic mass is the usual source of this problem in a child.
  4. "Dance's sign" is the appearance on xray of "telescoped" intestine.
  5. Recurrence is likely after treatment.

Answer(s): B

Explanation:

Intussusception is usually seen in children 812 months of age. They present with paroxysmal, crampy abdominal pain, and sometimes emesis. "Currant-jelly" stools are sometimes seen. They usually report a history of GI viral infection in the recent past. Enlarged Peyer's patches are usually the lead point of the intussusception. Polyps, tumors, and Meckel's diverticulum are less frequent causes. On examination, these children may have a mass in the epigastrium or right upper quadrant with an absence of intestine in the right lower quadrant. This is referred to as "Dance's sign." The diagnostic tool of choice is air contrast enema. This is also therapeutic in 6090% of cases. If the intussusception cannot be reduced by the enema, laparotomy is required to prevent bowel ischemia. Compromised bowel is resected at that time. Only 5% of children have a recurrence after successful reduction.



A 4-week-old, previously healthy male presents with projectile emesis after feeds. His mother states that he has had 2 weeks of postprandial emesis, which became projectile in the past 2 days. She states that it looks like formula and has never been bilious.

Which of the following statements is true?

  1. Physical examination is almost always normal in patients with this condition.
  2. Upper gastrointestinal (GI) contrast study is the best diagnostic option.
  3. This patient should be taken emergently to the operating room (OR) once the diagnosis is made.
  4. If uncorrected, these infants will progress to complete obstruction.
  5. Surgical therapy involves bypassing the site of obstruction.

Answer(s): D

Explanation:

Pyloric stenosis usually presents in the first 48 weeks of life. Parents usually report nonbilious emesis after feeding, which progresses to a projectile nature. Untreated, this will become a complete obstruction. On examination, an "olive sign" or mass in the right upper quadrant is often found. Ultrasound is the best radiologic test. These infants may present with dehydration and metabolic abnormalities from the emesis. The most common abnormality is hypokalemic, hypochloremic metabolic alkalosis. While surgical correction is urgent, it is not emergent. These infants should be resuscitated with IV fluids and their metabolic derangements corrected before an operation. The surgical treatment is a pyloromyotomy, which involves splitting the hypertrophic muscles of the pylorus while keeping the mucosa intact. This can be performed either open or laparoscopically. Patients are usually allowedto feed within hours of their operation.






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