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ANTHRAX FACTS

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ANTHRAX FACTS

Anthrax is a disease caused by the organism bacillus anthracis. It derives its name from anthrakis, the Greek word for coal, because the cutaneous version of the disease can cause black skin lesions.

It is rarely seen in people and mostly affects hoofed animals, which become infected after ingesting the dormant forms of the bacteria - the spores - in soil. The spores can remain dormant in the soil for many years.
Traditionally people most at risk are those who work with animals or in industries processing animal products such as meat and wool.
Anthrax is not contagious. The only way to be infected is by being exposed to large numbers of spores.

Anthrax spores can infect humans through: 1. A cut or graze 2. In contaminated meat 3. By being inhaled.
The disease is classified by the way it is caught, so there are three types:

1. Cutaneous
2. Gastro-intestinal
3. Pulmonary or inhalation anthrax.

Evidence indicates that man is fairly resistant to anthrax. A study in the early 1960s found that mill workers inhaling up to 1300 spores over 8 hours suffered no ill effects. It is estimated that a human would have to inhale more than 10,000 spores to become infected.
Infection will only result if sufficient spores germinate and release harmful toxins.

Signs of the disease usually appear within three days, but in some cases it can be up to two months. An anthrax vaccine is available for people in high risk occupations or for members of the armed forces who may be in danger from biological warfare.

Inhalation / Pulmonary anthrax
This is the rarest form of the disease. Between 1900 and 1978 only 18 cases were recorded in the US.

Symptoms
Initially coughs and sneezes, much like a common cold. Within 36 hours chest pains, severe breathing problems and shock will develop. This type of anthrax usually results in death after about two days.

Effects
The spores are absorbed through the alveoli into the lymph system. They may not become active for up to two months. Once they germinate they release toxins which rapidly leads to hemorrhaging. Any delay administering antibiotics will reduce the chances of survival. Mortality rate for this type of anthrax is about 89%.

Treatment
The antibiotic being used to treat the current cases in the US is ciprofloxacin, known in the UK as ciproxin.

Gastrointestinal anthrax
This type of the disease is rare and develops after a person eats meat contaminated with anthrax.

Symptoms
Intestinal anthrax is characterised by acute inflammation of the gut. Initial nausea, loss of appetite, fever and vomiting is followed by abdominal pain, vomiting of blood and acute diarrhea.

Effects
Without treatment it results in death for 24% to 60% of cases.

Treatment
Anthrax can be treated with antibiotics.

Cutaneous anthrax
Most anthrax infections occur through a cut on the skin. About 2,000 cases are reported annually.



Symptoms
Skin infections begin as an itchy bump which over 2-6 days develops into a boil and then a depressed black skin lesion.

Effects
After the spores germinate they release toxins which damage the skin tissues. The disease can spread throughout the body, but deaths are rare. With treatment mortality is less than 1%.

Treatment
The antibiotic being used to treat the current cases in the US is ciprofloxacin, known in the UK as ciproxin




A 75-year-old man presents with recurrent episodes of shortness of breath on minimal exertion. He has no prior significant past medical history. Physical examination reveals blood pressure of 110/70 without pulsus paradoxus, heart rate of 110, respiratory rate of 25, and temperature of 37C (98.6F) orally. Jugular veins are distended and the heart sounds are distant, but there are third and fourth extra heart sounds. The liver is enlarged, and pedal edema is present. The electrocardiogram shows nonspecific ST-T wave changes and occasional premature ventricular contractions. The chest x-ray reveals clear lung fields and a mildly dilated cardiac silhouette. Echocardiography reveals normal systolic function and thickened ventricular walls with a 'speckled' appearance. Which of the following conditions is most consistent with the patient's clinical presentation?


A: Alcoholic cardiomyopathy
B: Hemochromatosis
C: Amyloidosis
D: Viral myocarditis
E: Tuberculosis

The correct answer is C. Amyloidosis
The restrictive cardiomyopathies are characterized pathophysiologically by an impairment to ventricular filling. The cardiac silhouette is usually mildly, if at all, enlarged. Electrocardiography typically displays low-voltage QRS complexes, atrioventricular conduction defects, and a host of nonspecific arrhythmias. Echocardiography frequently reveals normal systolic and increased left ventricular wall thickness. In amyloidosis, the left ventricular wall appears to be 'speckled.' While primary cardiac amyloidosis typically produces diastolic dysfunction or restrictive cardiomyopathy as in this question, systolic dysfunction, arrhythmias, and orthostatic hypotension may be alternative presentations. Hemochromatosis also may cause a restrictive picture, but the speckled appearance noted in the echocardiogram would be absent. Alcoholism and viral infections typically cause dilated cardiomyopathies. Chronic tuberculous pericarditis can manifest clinical symptoms similar to those seen in restrictive cardiomyopathy. Patients with constrictive pericarditis have clinical presentations similar to those of patients with restrictive cardiomyopathy but tend to have normal ventricular wall thickness on echocardiography, pericardial calcification, and the absence of third or fourth heart sounds on chest auscultation.

Each of the following patients was noted to have an abnormally high serum cholesterol and was placed on a reduced calorie, cholesterol, and fat diet for the past 3 months. None has any history of ischemic heart disease. In which of the following patients would it be most appropriate to recommend lipid-lowering drug therapy at this time?


A: A 52-year-old smoker and diabetic with an LDL cholesterol value of 3.2 mmol/L (120 mg/dL)
B: A 60-year-old hypertensive woman with an LDL cholesterol value of 3.5 mmol/L (140 mg/dL)
C: A 50-year-old man with cholesterol of 6 mmol/L (230 mg/dL)
D: A 45-year-old man with LDL cholesterol of 5 mmol/L (200 mg/dL)
E: A 58-year-old male smoker with cholesterol of 5.5 mmol/L (220 mg/dL) and LDL cholesterol of 4 mmol/L (150 mg/dL)

The correct answer is D. A 45-year-old man with LDL cholesterol of 5 mmol/L (200 mg/dL)
Given the clearly defined benefits of lipid lowering in patients at risk for ischemic heart disease, screening measurement of blood cholesterol levels (nonfasting) is recommended for all adult patients, especially young patients with a family history of premature heart disease. If hyperlipidemia is detected, secondary causes such as hypothyroidism, nephrotic syndrome, and uremia should be considered, along with stopping drugs that can aggravate the condition, including oral contraceptives, estrogens, thiazides, and beta blockers. Once these effects are considered, the primary step is attention to diet. Attempts should be made to bring the patient to normal weight and encourage the patient to undergo dietary therapy with reduced intake of calories, cholesterol, and saturated fat. However, patients who remain at high risk after 3 months of an intensive regimen of dietary therapy should be strongly considered for lipid-lowering drug therapy. Such therapy is recommended for any adult patient whose LDL cholesterol remains greater than 4.9 mmol/L (190 mg/dL) or greater than 4.1 mmol/L (160 mg/dL) in the presence of two or more risk factors. A more aggressive approach is recommended for patients with a prior history of ischemic heart disease. Other risk factors for early atherosclerosis include diabetes mellitus, hypertension, familial hyperlipidemias, hypothyroidism, systemic lupus, and homocysteinemia. Drugs that act to lower LDL cholesterol include bile acid-binding resins such as cholestyramine, nicotinic acid, and hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase inhibitors.

Chief complaint: An 8-month-old infant with fever and irritability.
History of present illness (HPI): You are in general pediatric practice. A mother brings in an 8 month old male child because of fever and irritability for five days. On the second day of illness the child was seen at a local emergency room where the physician found no source for the fever and prescribed acetaminophen. Fever and irritability persisted. The fever has been between 1 03 F and 104 F rectally. There are no other complaints and the child has been feeding well.
Physical examination reveals a febrile, irritable, circumcised male infant but is otherwise within normal limits. The child is consolable.

1. Which of the following aspects of management of this child is most important?
A. To keep the temperature below I 04 F rectally
B. To keep the temperature below 102 F rectally.
C. To determine the cause of the fever.
D. To begin your laboratory investigation.
E. Undress the baby and let the temperature come down

2. Statistically it is most likely that this child has
A. an acute viral infection.
B. occult bacteremia.
C. a urinary tract infection.
D. bacterial meningitis.
E. osteomyelitis.

3. The next day the child develops a rash. Physical examination reveals nonpitting swelling of the hands and feet, redness of the lips, bilateral bulbar conjunctivitis without exudate, an enlarged right cervical lymph node, approximately 2 x 3 cm, and a generalized, splotchy, erythematous macular rash. The pharynx is normal. Chest is clear. The child is irritable but in no distress. The neck is supple.
You expect that the CBC will reveal
A. Increased WBCs, increased neutrophiles, decreased platelets, and normal hemoglobin.
B. Increased WBCs, increased neutrophiles, increased platelets, and normal hemoglobin.
C. Elevated WBCs, normal neutrophiles, normal platelets, and normal hemoglobin.
D. Decreased WBCs, decreased neutrophiles, normal platelets, normal hemoglobin.
E. Decreased WBCs, decreased neutrophiles, decreased platelets, and decreased hemoglobin.

4. Other features of Kawasaki disease includes all of the following EXCEPT:
A. CSF pleocytosis.
B. Hydrops of the gall bladder.
C. Sterile pyuria.
D. Hematuria

5. The greatest concern for this disease regards the heart. The usual manifestation of cardiac involvement with Kawasaki syndrome is
A. Pancarditis.
B. Valvular disease.
C. Coronary artery aneurysms.
D. Endocarditis.
E. Pericarditis.
F. Dilated cardiomyopathy

6. The treatment of Kawasaki syndrome usually includes
A. Digitalis and lasix
B. Lasix and aspirin.
C. Aspirin and intravenous immunoglobulin (lgG).
D. lgG and steroids
E. Steroids and aspirin.



C is correct. Determining the cause of the fever is more important than treating the fever systematically. This is not to say that treatment of fever for the patient's comfort and the parents' reassurance is unreasonable; it is simply not nearly as important as determining the cause of the fever. The fever itself will not be harmful, and since most febrile seizures occur close to the onset of the fever, usually within the first 12 to 24 hours, it is unlikely that this child will have a febrile seizure.

2. A is correct. The most likely diagnosis for this child is an acute viral infection. Although persistence of fever for five days makes other causes somewhat more likely than if the fever had been present for only one day, acute viral infection is still the most likely explanation.

3. B is correct. You obviously recognized this child as probably having Kawasaki disease, a disorder of undetermined origin characterized by a variety of acute phase reactants including an elevated erythrocyte sedimentation rate, an elevated total white blood cell count with increased numbers of neutrophiles and a markedly elevated platelet count. While an elevated platelet count is seen in a variety of inflammatory conditions, it is especially characteristic of Kawasaki disease.

4. D is correct, Hematuria is not a feature of Kawasaki disease. Although the urethra (sterile pyuria) can be a target organ in this disorder, the kidney and the bladder are not involved. Aseptic meningitis with CSF pleocytosis is not uncommon and hydrops of the gall bladder is also a feature.

5. C is correct. Coronary artery aneurysms are the classic and most common form of cardiac involvement in children with Kawasaki syndrome. With rare exceptions, it is the only cardiac manifestation.

6. C is correct. Aspirin and intravenous immunoglobulin are the treatments of choice for Kawasaki disease. There is evidence that a regiment of both medications decreases the incidence of coronary artery aneurysms, the major cause of mortality in this condition. The mechanism of action of lgG is unclear but presumably it somehow decreases the inflammatory response. Aspirin is used for its anti-platelet effect. A low dose for several months seems to decrease the incidence of coronary artery disease.
Steroids should not be employed in the routine treatment of Kawasaki disease. Although corticosteroids do relieve the fever and signs of inflammation in Kawasaki syndrome, several studies have suggested an increased incidence of coronary artery aneurysms in children receiving corticosteroids.

A 22-year-old man presents to the emergency department with the suddenonset of left hemiparesis. His wife reports that he has not felt well forat least a month and has had a weight loss of about 9 kg. On physical examination, his temperature is 38 C (100.4 F) and his blood pressure is 116/52 mm Hg. He is somewhat cachectic and hemiparetic. The cardiac examinationshows a murmur. The physical examination is otherwise normal.

What is the most appropriate investigation to be done in this patientin order to arrive at a diagnosis?

A. Blood cultures
B. Lumbar puncture
C. Radiography of the chest
D. Complete blood count, differential, platelet count, and erythrocytesedimentation rate
E. Biopsy of inguinal lymph node

The answer is A.
The appearance of focal neurologic signs in a young person raises awide differential diagnosis. Atherosclerotic cerebrovascular disease isuncommon in the 20-year-old age group. Among other diagnostic considerationsare an embolic event from a cardiac source, such as a valvular vegetationassociated with endocarditis or an atrial myxoma, vasculitis, tuberculomatousor bacterial brain abscess, brain tumor, aneurysm or arteriovenous malformation,and coagulation disorder such as thrombotic thrombocytopenic purpura orhyperviscosity syndrome caused by multiple myeloma.
Infective endocarditis must be considered in any patient with suddenfocal neurologic deficits who has no conventional risk factors, such asatherosclerosis. Neurologic complications occur in 25% to 40% of patientswith infective endocarditis. About 15% develop cerebral emboli with associatedneurologic symptoms. The easily associated triad of new focal neurologicdeficits, fever and changing heart murmur occurs in only 33% of patients.
A lumbar puncture should not be done in a patient with focal neurologic signs before a computed tomography (CT) or magnetic resonance imaging ofthe head.
A chest radiograph is not likely to reveal a specific etiology for the sudden neurologic event. Similarly, complete blood count, differential,platelet count, and sedimentation rate could provide important clues tomany diagnostic possibilities, such as vasculitis, thrombocytopenic purpura,and multiple myeloma, but are unlikely to provide a specific etiology.
Bilateral inguinal adenopathy, especially when nodes are relativelysmall and rubbery from inconsistency, is a very common physical findingand not likely to be of significance. Biopsy of an inguinal lymph nodeshould be done only if less invasive tests fail to reveal a specific diagnosis



Mrs. Porter, who is 48 years old, presents to your office for a routine physical examination. She reports that she is healthy, takes no medications, and has no complaints. Recently, a friend of hers was diagnosed with diabetes, and she wants to know if she should be tested for diabetes, too. After conducting a careful and thorough review of her medical history, you find no symptoms of hypo- or hyperglycemia. The physical examination is entirely within normal limits.

1.What advice should you give Mrs. Porter regarding screening for diabetes?

A. Screening for diabetes is not indicated in asymptomatic patients..
B. Screening for diabetes is indicated only if she has a first-degree relative with diabetes.
C. Asymptomatic women should be screened for diabetes beginning at age 50..
D. Screening with a fasting plasma glucose (FPG) is appropriate at this time.
E. Screening with a glycosylated hemoglobin (HbA1c) is appropriate at this time.

2.An FPG is performed, and Mrs. Porter is within the normal (nondiabetic) range. What advice should you give her regarding the need for future screening?

A. Future screening is not indicated as long as she remains asymptomatic.
B. Annual FPG screening is recommended.
C. She should be screened again in 3 years.
D. She should be screened again in 5 years.
E. She should be screened again at age 60.

1.The answer is D. You should tell Mrs. Porter that screening with a fasting plasma glucose (FPG) is appropriate at this time. Undiagnosed type 2 diabetes is a common and significant health problem in the United States, with an estimated 8 million people currently undiagnosed. Vascular complications from diabetes often begin approximately four to seven years before the onset of clinical symptoms, and approximately 10% to 20% of patients have evidence of retinopathy and nephropathy at the time of diagnosis. The 1997 Report of the Expert Committee on the Diagnosis and Classification of Diabetes Mellitus strongly encourages regular screening for type 2 diabetes beginning at age 45 because there is a steep rise in the incidence of the disease after this age. The FPG is the test of choice because it is easier to perform, faster, more convenient, more reproducible, and less expensive than the oral glucose tolerance test (OGTT). Testing to determine the HbA1c concentration is not recommended for the screening or diagnosis of diabetes because methods of measuring normal ranges are not standardized among laboratories.

2.The answer is C. She should be screened again in 3 years.
The Expert Committee recommends screening asymptomatic individuals for diabetes beginning at age 45. If the results of the screening tests are normal, screening should be repeated at 3-year intervals. This interval is based on the negligible likelihood of developing complications of diabetes within 3 years of a negative screening test.
However, earlier and more frequent screening should be considered for patients with the following risk factors:
Obesity
Family history of diabetes in first-degree relative
Member of high-risk ethnic group (African, Hispanic, or Native American)
History of gestational diabetes or delivery of baby weighing > 9 lbs.
Hypertension
HDL cholesterol < 35 mg/dL and/or triglyceride level > 250 mg/dL
Impaired glucose homeostasis on previous testing

Laboratory Tests for Evaluation of Hypertension

BASIC TESTS FOR INITIAL EVALUATION
Always included
Urine for protein, blood, and glucose
Microscopic urinalysis
Hematocrit
Serum potassium
Serum creatinine and/or blood urea nitrogen
Fasting glucose
Total cholesterol
Electrocardiogram
Usually included, depending on cost and other factors
Thyroid-stimulating hormone
White blood cell count
HDL and LDL cholesterol and triglycerides
Serum calcium and phosphate
Chest x-ray; limited echocardiogram

SPECIAL STUDIES TO SCREEN FOR SECONDARY HYPERTENSION
Renovascular disease: angiotensin-converting enzyme inhibitor radionuclide renal scan, renal duplex Doppler flow studies, and MRI angiography
Pheochromocytoma: 24-h urine assay for creatinine, metanephrines, and catecholamines
Cushing's syndrome: overnight dexamethasone suppression test or 24-h urine cortisol and creatinine
Primary aldosteronism: plasma aldosterone: renin activity ratio

A healthy 28-year-old laboratory technician consults you about a test he ran 'for the heck of it.' He is asymptomatic, and the results of his physical examination are within normal limits. However, laboratory studies show a total serum bilirubin level of 1.9 mg/dL (N < 1.0) with an indirect level of 1.3 mg/dL (N < 06). Determinations of liver enzyme and serum alkaline phosphatase levels are normal. The hemoglobin level is 15.0 g/dL (N 13-18) and stable; the reticulocyte count is 1% (N 0.5-1.5).

The most likely diagnosis is

a. non-A, non-B hepatitis
b. alcoholic hepatitis
c. Gilbert's syndrome
d. Crigler-Najjar syndrome
e. Wilson's disease

C



The diagnosis of Gilbert's syndrome, a benign, hereditary glucuronyl transferase deficiency, is usually made by exclusion and should be suspected in a patient who has mild, persistent, unconjugated hyperbilirubinemia when 1) there are no systemic symptoms, 2) there is no overt or clinically recognizable hemolysis, and 3) tests of routine liver function are normal. Crigler-Najjar syndrome is associated with much higher levels of unconjugated bilirubin (6-45 mg/dL). The diagnosis of Wilson's disease should be considered in any patient under the age of 40 who has an unexplained disorder of the central nervous system, signs and symptoms of chronic active hepatitis, unexplained persistent elevations of serum transaminase, acquired hemolytic anemia, or unexplained cirrhosis. Alcoholic hepatitis is associated with variable elevations of serum alkaline phosphatase. Abnormal liver enzymes are characteristic of both alcoholic hepatitis and non-A, non-B hepatitis.

A 39-year-old white female has a 6- to 8-month history of general fatigue, myalgias, arthralgias, nausea, and constipation. She is married, has two children, and runs a successful small business. In addition to her physical complaints, she says that she has been increasingly forgetful. Past medical history includes repair of a torn meniscus 20 years ago and a bilateral tubal ligation 12 years ago. She has been taking antacids regularly for dyspepsia. Her menstrual pattern is undisturbed. A physical examination is unremarkable.

Laboratory Findings

Calcium..12.7 mg/dL (N 8.4-11.0)
Phosphorus..2.0 mg/dL (N 3.0-4.5)
Albumin..4.4 g/dL (N 3.5-5.5)
Chloride.110 mEq/L (N 95-105)
Serum uric acid5.3 mg/dL (N 3.0-8.2)
Serum creatinine..0.9 mg/dL (N 0.6-1.2)
BUN10 mg/dL (N 7-18)
After obtaining repeatedly elevated levels of parathyroid hormone (PTH), you make an appropriate diagnosis.

Optimal treatment for this patient is



a. radiation therapy
b. oral phosphate therapy
c. psychotherapy
d. intravenous plicamycin (Mithracin)
e. surgery

E

This case is a typical presentation for symptomatic primary hyperparathyroidism. The only successful treatment is surgery. Most authorities feel that parathyroidectomy should be performed for patients who are symptomatic, unless there are contraindications or the diagnosis is uncertain. Furthermore, it is appropriate to operate on young persons to avoid lifelong monitoring by time-consuming and expensive tests, particularly since surgical treatment is usually successful and carries a low risk of morbidity and mortality. Medical therapy with intravenous plicamycin is reserved for hypercalcemic emergencies, and oral phosphate therapy is merely a temporizing measure. There is no place for radiation therapy in hyperparathyroidism.

A 67-year-old white female has an intermittent history of sharp, lancinating pains at the head of the third metatarsal. She initially experienced a burning sensation with some occasional numbness of the third toe, and later found that removing her shoe would frequently alleviate the pain. Her physical examination is within normal limits except for tenderness at the head of the third metatarsal in the web space between the third and fourth metatarsals.

The most likely diagnosis is



a. improper shoe fit
b. avascular necrosis of the second metatarsal head (Freiberg's disease)
c. Morton's neuroma
d. metatarsal stress fracture
e. diabetic neuropathy

C

The presentation is that of a Morton's neuroma. The fourth toe is most commonly involved, while the third toe is the next most common site. In diabetic neuropathy, a burning dysesthesia involves the entire foot, has no trigger point, and can be completely unrelated to shoe fit. Stress fractures commonly involve the second and third metatarsals. These fractures can be caused by the repeated stress of long distance walking or running. Stress fracture tenderness is usually localized in the shaft of the mid metatarsal, while improperly fitted shoes will cause a much more generalized pain within the foot. Freiberg's disease occurs in adolescents and is an avascular necrosis of the second metatarsal head.

Parents can refuse vaccinations to their children- but what's the case of blood/products transfusion in transfusion- dependent kids e.g. thalassemics?
And what's the next step for the attending?
Comments appreciated.

Parents have legal and moral authority to make health care decisions for their children, as long as those decisions do not pose a serious threat to the child's physical well-being. Parents should not be permitted to deny their children medical care when that medical care is likely to prevent substantial harm or suffering. If necessary, the physician may need to pursue a court order in order to provide treatment against the wishes of the parents. Nevertheless, the physician must always take care to show respect for the family's beliefs and a willingness to discuss reasonable alternatives with the family.






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