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ccs case,sickle cell crisis

medicines

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ccs case,sickle cell crisis. according to the washington manual

tx.of sickle cell anemia
1.RBC transfusion
Ix; stroke, TIA, acute chest syndrome, priapism unresponsive to supportive care and in preparation for anesthesia
2.hydroxyurea(15-35mg/kg po qd)
increase levels of fetal Hb and todecrease the incidence of vasoocclusive pain episodes by approximately 50% in adults with sickle cell anemia
3.BMT
RBC transfusion will not change the immediated course of an acute pain crisis, morphine is the drug of choice for moderate or severe pain




A 72-year-old retired salesman has had heartburn symptoms once or twice a week for the past several years. He has taken antacids on occasion with some symptom relief. He has some regurgitation once or twice per week. He denies any dysphagia or weight loss. Of note, he has worsening hypertension. A calcium channel blocker was added to his diuretic to attain better blood pressure control. Yesterday, he watched his favorite baseball team lose in the divisional playoffs, and after the Buffalo wings, chips, salsa, and 10 beers, he developed an “ache” in the substernal region with no associated symptoms. He presents to the local emergency room. The physical examination shows the following:

Vital signs: afebrile; heart rate = 84/min; respiratory rate = 16/min; blood pressure = 156/98 mm Hg

Cardiovascular examination: regular rate and rhythm; S1, S2 normal

Pulmonary examination: lungs clear to auscultation

Abdominal examination: (+) bowel sounds; soft/nontender without organomegaly

ECG: normal sinus rhythm without changes consistent with ischemia.

The emergency room physician adds nitrates to the regimen. However, the frequency and severity of the chest ache increases, lasting for minutes and occurring twice an hour. Cardiac enzymes are equivocal. The emergency room physician calls a cardiology consultation. Cardiac catheterization is performed and reveals minimal luminal irregularities in coronary arteries. There is no clinically significant coronary artery disease. The patient is considered for referral to a gastroenterologist.


What is the most likely cause of this man's symptoms?

A: Coronary artery disease
B: Pneumonia
C: Cholecystitis
D: Gastroesophageal reflux disease (GERD)
E: Musculoskeletal pain

Answer is D. With this patient’s history of heartburn and acid regurgitation coupled with a normal cardiac workup, no evidence of muscular strain, no fevers or dyspnea that would be consistent with pneumonia, and a normal ultrasound making cholecystitis unlikely, GERD is the most likely diagnosis.
GERD is one of the most common medical problems seen in clinical practice today. An estimated 4% to 7% of American adults experience GERD-related heartburn or acid regurgitation daily, 10% to 14% experience these symptoms weekly, and 15% to 44% experience them monthly. Angina-like chest pain is one of the symptoms that can occur with GERD.

A: Coronary artery disease - MI dx.: cardiac enzyme+, specific hx.+, EKG finding+ -all of these are not met, even angina, no nitrate response, cardiac catheterization-, even if variant spasm, he already took calcium channel blocker
B: Pneumonia - no fever, no resp.sx., no specific P/Ex.
C: Cholecystitis - no fever, no Murphy's sign, unfitted G-I sx.and hx.
D: Gastroesophageal reflux disease (GERD)
E: Musculoskeletal pain - no specific physical exam.

The most common bacterial pathogen involved with acute bacterial rhinosinusitis is:

A. Type B Haemophilus influenzae
B. Mycoplasma pneumoniae
C. Streptococcus pneumoniae
D. Streptococcus pyogenes
E. Moraxella catarrhalis

C

S. pneumoniae (41%)
H. influenzae (35%)
Moraxella catarrhalis (4%),
Staphylococcus aureus (3%).

Treatment

More than 70% of acute rhinosinusitis is caused by S. pneumoniae and H. influenzae.
high-dose amoxicillin

In the United States, the primary means for prevention of sporadic meningococcal disease is antimicrobial chemoprophylaxis. Indications for such prophylaxis is not indicated for


a) household members
b) day care center contacts
c) anyone directly exposed to the patient’s oral secretions (e.g., through kissing,mouth-to-mouth resuscitation, endotracheal intubation, or endotracheal tube management)
d) pregnant women

Answer is D. In the United States, the primary means for prevention of sporadic meningococcal disease is antimicrobial chemoprophylaxis of close contacts of infected persons. Close contacts include a) household members, b) day care center contacts,
and c) anyone directly exposed to the patient’s oral secretions (e.g., through kissing,
mouth-to-mouth resuscitation, endotracheal intubation, or endotracheal tube management).

The primary means for the prevention of meningococcal disease in the United States is antimicrobial prophylaxis of close contacts of persons diagnosed with meningococcal disease. Close contacts include household members, daycare center contacts and anyone directly exposed to the patient's oral secretions. a.vaccination should be considered on adjuvant ot antibiotic chemoprophylaxis for household or intimate cotacts of meningococcal disease cases
b.Chemoprophylaxis of healthcare workers is generally not indicated unless the worker has been exposed to the patient's respiratory secretions through mouth-to mouth resuscitation, endotracheal intubation or care.
Because the attack rate of meningitis in contacts of cases is highest during the first few days after onset of disease in an index case, chemoprophylaxis should be administered to appropriate contacts as soon as possible, ideally within 24 hours after identifying a case. Colonization of otherwise healthy individuals with N. meningiditis is well recognized, has been noted in up to 40% of the population at times, and is not considered an indication for chemoprophylaxis.

c.it is not live vaccine, should be considered during pregnancy



A 42-year-old man with no prior history of major illness is admitted with melena, Hb of 9.0, and coffee-ground vomitus. His stomach clears on gastric lavage. He is hemodynamically stable. On endoscopy he has a 7-mm clean-based duodenal ulcer without dark spots or visible vessel. A slide test for urease is positive on a biopsy specimen from the gastric antrum.
Which of the following would be the most appropriate treatment plan for this patient after endoscopy?

a. Intravenous (IV) H2-blockers, NPO for 24 hours, followed by clear liquid diet with progression to full diet, and then discharge within 72 hours with treatment of oral drug therapy for 8 weeks
b. Oral H2-blockers, clear liquid diet for 24 hours with progression to full diet, and then discharge within 72 hours with continued drug therapy for 8 weeks
c. Proton pump inhibitors twice daily, clear liquid diet for 24 hours, and then discharge with continued drug therapy for 1 month
d. Treatment for Helicobacter pylori for 2 weeks, followed by discharge within 24 hours on a regular diet
e. Proton pump inhibitors for 4 to 8 weeks, treatment for H. pylori for 2 weeks, regular diet, and discharge within 24 hours

The correct answer is e.
e. Patients who bleed from peptic ulcer disease and who have a clean-based ulcer at endoscopy have a less than 5% chance of rebleeding. They may be treated with any appropriate acid-reducing regimen for peptic ulcer and then be discharged within 24 hours. If gastric mucosa is tested for urease activity, implying the presence of Helicobacter pylori, the patient should be treated with an appropriate regimen.
Although proton pump inhibitors have some anti-Helicobacter activity, when used alone they are insufficient treatment for the infection. If the urease slide test of the gastric biopsy (such as the CLOTEST®) is negative, a fasting serum gastrin is warranted, especially in the absence of a history of NSAID (nonsteroidal antiinflammatory drug) usage. If on endoscopy the patient has a visible vessel or clot, the percentage of rebleeding is high and more careful monitoring is needed; endoscopic therapy may be appropriate. Acid reduction therapy with H2-blockers or proton pump inhibitors should be used to treat the ulcer(s) in addition to the anti-H. pylori antimicrobial therapy. (Yamada T et al. Textbook of Gastroenterology, 2nd ed. Philadelphia: JB Lippincott, 1995; Laine L and Peterson WL. Medical progress: Bleeding peptic ulcer. N Engl J Med 1994;331:717–727

A 67-year-old woman who is a regular patient calls the office because she has developed severe muscle weakness, muscle cramps and polyuria. She began treatment 6 weeks ago with 50 mg of chlorthalidone daily for mild-to-moderate essential hypertension. The most likely explanation for her symptoms is the development of

(A) hypokalemia
(B) hypomagnesemia
(C) hyponatremia
(D) metabolic acidosis
(E) type 2 diabetes mellitus

A

Hypokalemia is a very common side effect of non-potassium-sparing diuretics (e.g., chlorthalidone). This is often more pronounced in the older age group. Patients usually complain of muscle weakness, fatigue, and cramps. Constipation and ileus characterize the
smooth muscle involvement, whereas hyporeflexia, flaccid paralysis, and tetany are signs of severe
hypokalemia.

it is thiazide diurtics
side effect: weakness, muscle cramps, and impotence
metabolic; hypoKa, hypoMg, hyperlipidemia(increases LDL and TG),hyperCa, hypoNa, hyperglycemia,hyperuremia
Thiazide-related pancreatitis has been reported

A 50-year-old African-American man with severe chronic obstructive pulmonary disease returns to the office following a recent evaluation for possible lung transplantation in another city. He says he had been considered a suitable candidate, in all respects, but was rejected by the transplant program when a random urine test was positive for a nicotine metabolite. He had previously told you that he had stopped smoking 3 years ago. He stands by this and is at a loss to explain the positive urine test. He wants to know what he should do now. At this time you should

(A) advise him again to stop smoking and refer him to another transplant program
(B) advise him that the transplant program cannot turn him down on this basis, according to the Americans with
Disabilities Act
(C) contact the transplant program to learn their reasons for turning him down
(D) explain to the patient that transplantation is out of the question as a result of what has occurred
(E) write to the transplant program and insist that they give him another opportunity



C

Current Contraindications to Lung Transplantation:
1.Major Organ Dysfunction: especially renal or cardiac disease.
2.Infection with HIV.
3.Active Malignancy.
4.Hepatitis B antigen positive.
5.Hepatitis C with liver damage on biopsy.


Pre-Referral Investigations:
Full Pulmonary Function Studies.
Exercise Performance measurement.
Electrocardiogram and Echocardiogram.
High Resolution CT Scan of Chest.
Stress Echocardiogram or Coronary Angiography.
24-hour creatinine clearance.
Liver Function Studies.

General Medical Conditions that Effect Eligibility for Lung Transplantation:
Symptomatic Osteoporosis: Relative Contraindication.
Kyphoscoliosis: Relative Contraindication.
Progressive Neuromuscular Disease: Absolute Contraindication.
Current use of Corticosteroids: dose of 20mg prednisone or less.
Ideal Body Weight between 70 and 130% of predicted.
Psychosocial issues: Noncompliance with medical care is a Relative Contraindication.
Requirement for invasive ventilation: Relative Contraindication.
Colonization with Fungi, Atypical AFB or adequately treated MTB- not a contraindication.

A male colleague asks you to write a prescription for a narcotic analgesic for one of his female patients. You have noticed that this patient frequently has been coming by the office to see your colleague, and that several of the visits have been marked “No Charge.” When you ask your colleague why he cannot write the prescription himself, he seems defensive and says, “because I don't want anybody to get the wrong idea.” The most appropriate response to your
colleague is:

(A) “It sounds like there is more to this story than you are telling me; maybe we should talk about it.”
(B) “I wish I could help you, but I never prescribe that medication for a patient unless I have seen the patient myself.”
(C) “I will do this for you once, but I will need to see your patient in the office before I can write another prescription.”
(D) “Maybe I should see your patient in the office myself, and then decide if she needs the medication.”
(E) “You have seemed a little nervous lately. You aren't getting in over your head, are you?”

A

A 2-year-old boy is brought to the emergency department by his mother because of a large laceration on his hand. The mother says 'He is always playing with knives and is so careless. That's probably how he got hurt this time.' On physical examination the patient appears unkempt. There is a 4-cm laceration on the palmar aspect of his left hand. Child abuse is suspected. In addition to referral to the child protective services, management should include each of the following EXCEPT

A. careful review of the patient's hospital records
B. direct confrontation of the accompanying parent
C. a nonjudgmental elicitation of the circumstances of the injury
D. thorough physical examination
E. x-ray film survey of the long bones

B

A 45-year-old African-American man comes to the office for the first time because he says, 'I had blood in my urine when I went to the bathroom this morning.' He reports no other symptoms. On physical examination his kidneys are palpable bilaterally and he has mild hypertension. The information in his history that is most pertinent to his current condition is

A. chronic use of analgesics
B. cigarette smoking
C. a family history of renal disease
D. occupational exposure to carbon tetrachloride
E. recent sore throats

C

adult type(most common) PKD-A.D.
flank pain,vague abdominal complaints, symptoms of UTI,episodes of gross hematuria and the incidental discovery of hypertension are common presenting problems
in contrast, a sense of abdominal fullness due to enlarged kidney occurs relatively late in the course of the disease



A 42-year-old-sexually active female presents with low-grade fever, headache, malaise, dysuria, and vaginal discharge. Physical examination reveals several vesicular lesions on the labia bilaterally. She also has tender inguinal lymphadenopathy. All the following statements regarding the current situation are correct EXCEPT

A oral acyclovir will be effective in speeding the resolution of her symptoms
B if the patient has had prior HSV-1 infection, she will be less likely to have severe systemic symptoms
C recurrent infection will be equally likely whether the patient is infected with HSV-1 or HSV-2
D if her sexual partner uses a condom, transmission will be less likely
E prolonged acyclovir use could reduce the likelihood of recurrent infection

C

when it comes to recurrence,
80% of persons having a first episode caused by HSV-2 will have at least one recurrence
50% of persons with HSV-1 will experience a recurrence
The most common scenario is occasional recurrences (about 4 attacks per year)
Usually, the first year has the most viral activity
recurrence has also prodromal sx., less severe
one having previous HSV-1infection has less severe sx.
continuous acyclovir 400mg po qd-prophylactic use

Which of the following statements concerning the diagnosis of pheochromocytoma is correct?

A Measurement of plasma catecholamines is the preferred initial screening test
B Random urine samples are equivalent in diagnostic accuracy to the measurement of catecholamines or catecholamine metabolites in a 24-h urine collection
C After collection, the urine should be treated with dilute sodium hydroxide and refrigerated
D The ideal time to collect urine is during a period of clinical stability
E Strenuous exertion may falsely elevate the level of free urinary catecholamines

The answer is E
Since provocative testing plays a very small role in the diagnosis of pheochromocytoma, the most frequently employed assays include measurement of catecholamines or catecholamine metabolites in a single 24-h urine sample. The three assays used include measurement of vanillylmandelic acid, metanephrines, and unconjugated ('free') catecholamines. Accuracy of diagnosis depends on the collection of a full 24-h urine sample that is treated with acid and refrigerated during and after the collection. The diagnostic yield would be increased if the 24-h urine collection included a time period during which the patient experienced a hypertensive paroxysm. False-positive increases in urinary free catecholamine excretion may occur if the patient is taking methyldopa, levodopa, or sympathomimetic amines. Endogenous plasma and urinary catecholamines also may be increased during hypoglycemia, strenuous exercise, and significant central nervous system disease. Urinary metanephrines and vanillylmandelic acid are also falsely positive in situations in which endogenous catecholamines may be increased or if the patient is receiving a monoamine oxidase inhibitor. Since plasma catecholamines are highly subject to endogenous variation in catecholamine secretion, they have not been particularly useful as an initial screening test for the diagnosis of pheochromocytoma.

taken from mdconult .com


Urinary catecholamines and metabolites (24-hr sample or 2-hr sample after a paroxysm; metanephrines, the initial screening test)
Plasma catecholamines (if urinary values are equivocal; take care to obtain a basal, resting sample)

The use of repeated phlebotomy in the treatment of persons with symptomatic hemochromatosis may be expected to result in

A increased skin pigmentation
B improved cardiac function
C return of secondary sex characteristics
D decreased joint pain
E an unchanged 5-year survival rate

The answer is B
In persons with symptomatic hemochromatosis, repeated phlebotomy, by removing excessive iron stores, results in marked clinical improvement. Specifically, the liver and spleen decrease in size, liver function improves, cardiac failure is reversed, and skin pigmentation ('bronzing') diminishes. Carbohydrate intolerance may abate in up to half of all affected persons. For unknown reasons, there is no improvement in the arthropathy or hypogonadism (resulting from pituitary deposition of iron) associated with hemochromatosis. The 5-year survival rate increases from 33 to 90 percent with treatment; prolonged survival may actually increase the risk of hepatocellular carcinoma, which affects one-third of persons treated for hemochromatosis. However, if phlebotomy is begun in the precirrhotic stage, which is possible with effective genetic screening, liver cancer will not develop.

early phlebotomy :
it can prevent most late symptoms and complications. Even when started after complications have occurred, phlebotomy can decrease symptoms and improve life expectancy. early phlebotomy :
it can prevent most late symptoms and complications. Even when started after complications have occurred, phlebotomy can decrease symptoms and improve life expectancy.






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