LEARNING OBJECTIVES: 1. Use the physical examination to determine cost-effective use of echocardiography 2. Use an understanding of physiology to guide diagnostic procedures 3. Diagnose infiltrative cardiomyopathy. CASE INFORMATION: A 73 year-old man presented with right-sided chest pain and shortness of breath. The shortness of breath had worsened over two weeks, with new onset three-pillow orthopnea and lower extremity edema. His vital signs were normal. He had a soft S1, a normal S2, and an S4. Although he had bilateral crackles and an elevated JVP, there was no S3 or inferiorly displaced PMI. His edema was initially ascribed to hypo-albuminemia (Albumin = 2.) although his pit recovery time was greater than 90 seconds. Based upon a suspicion of a restrictive cardiomyopathy, an echocardiogram was obtained. This showed left ventricular hypertrophy and an E-to-A mitral-flow reversal consistent with diastolic dysfunction. His ejection fraction was 20% despite ventricular hypertrophy. A bone marrow biopsy was performed that confirmed the diagnosis of Waldenstrom's macroglobulinemia. IMPLICATIONS/DISCUSSION: The physical examination in concert with an understanding of the physiology underlying disease is the foundation for cost-effective identification of occult diagnoses. The silent S1 suggested a low ejection fraction, and while the S4 without an S3 or inferiorly displaced PMI suggested a non-compliant hypertrophic ventricle. Both of these conclusions were confirmed by echo. A low ejection fraction despite ventricular hypertrophy suggested an infiltration of the ventricular wall. LaPlace's law states that wall tension is a function of the pressure and radius of a chamber, divided by the thickness of the chamber's wall. Because wall tension is proportional to afterload (like a spring, the ventricle must overcome the weight that has stretched it) a thick ventricle wall should lower wall tension, thereby decreasing the ventricular afterload. The ejection fraction should increase proportional with the wall thickness. Failure to do so suggests that the thickness is due to infiltration with a non-contractile substance. The history of chronic renal insufficiency and anemia suggested the diagnosis of amyloidosis due to Waldenstrom's macroglobulinemia. This clue was instrumental in prompting the bone marrow biopsy that confirming the diagnosis. The finding of a speckled myocardium on echocardiogram is a classic, though late state finding of the disease. A 54-year-old male with a history of leukocytoclastic vasculitis was noted to have a diffuse erythematous rash while being admitted for an unrelated endoscopic procedure. The rash first developed two weeks prior in the patient's right groin and gradually spread to include both legs and his right arm. It had not responded to a course of levofloxacin or over the counter topical ointments. In the month prior to admission the patient had been tapered from 15 milligrams to 12.5 milligrams of prednisone. On admission the patient had warmth, erythema, and mild tenderness to the affected areas. Numerous 1 to 5 millimeter pustules also were present on the erythematous base. The patient was afebrile and had a mildly elevated white count of 11 thousand. Gram stain and culture of the pustules revealed no bacteria. Fungal elements were not seen. A punch biopsy was taken and returned consistent with pustular psoriasis. Acitretin therapy was initiated. IMPLICATIONS/DISCUSSION: Psoriasis is a relatively common dermatologic disorder that occurs in approximately 1% of the North American population. While generally benign, psoriasis can present in an atypical and potentially life threatening manner. Pustular psoriasis can occur in varying degrees of severity with distinct subtypes. These include the relatively limited form of palmoplantar pustulosis to the more severe generalized von Zumbush type. If untreated, disruption of the skin's barrier can lead to large amounts of volume loss and secondary bacterial infections. Systemic involvement including arthritis, heart, liver, and kidney failure has been associated with advanced cases. Triggering events are often unknown, but there has been an association with pregnancy, concurrent infections, and medications. Given the nonspecific somatic complaints and laboratory abnormalities, successful diagnosis requires recognition of the pustular areas on the erythematous base. Our patient had two associated medication risk factors of hydroxychloroquine therapy and corticosteroid withdrawal that likely acted as the inciting event. IMPLICATIONS/DISCUSSION: t is a challenge to manage pregnant patients who require anticoagulation for prosthetic valves. Coumadin is the drug of choice for anticoagulation, however because it freely crosses the placenta it is not considered safe to use in pregnant patients. The teratogenic effects of coumadin usually occur between the sixth and ninth weeks of gestation. The most common abnormalities occur in bone and cartilage development. In the second and third trimesters one can develop abnormalities of the fetal central nervous system, optic atrophy, microcephaly, mental retardation, spastisity and hypotonia. Unfractionated heparin does not cross the placenta because of its high molecular weight. Since it does not cross the transplacental barrier it lacks teratogenic effects and will not anticoagulate the fetus. Low molecular weight heparin is another anticoagulant that should be considered in these patients. However, its use in pregnancy is not well studied. The ideal treatment for a pregnant woman requiring anticoagulation would be to switch from coumadin to heparin prior to conception. The patient shold be hospitalized until she is therapeutic on heparin. The three approaches that are recommended include: 1. Dose adjusted unfractionated heparin throughout pregnancy. Heparin is given subcutaneously every 12 hours and then dose adjusted to maintain two times the normal PTT, or anti-Xa levels of 0.35 to 0.70 U/ml. 2. Dose adjusted low molecular weight heparin throughout the pregnancy so as to keep a four hour post injection anti-Xa level at 1.0 U/ml. 3. Unfractionated or low molecular weight heparin therapy until the thirteenth week, with a change to coumadin until the middle of the third trimester and then restarted on unfractionated or low molecular weight heparin until delivery. LEARNING OBJECTIVES: 1) Recognize the cutaneous manifestations of sarcoidosis 2) Review the differential diagnosis of a nodular cutaneous lesion in a immigrant from subsaharan africa 3)Review the pathophysiology of cutaneous sarcoidosis CASE INFORMATION: A healthy 21 year old South Sudanese woman, 4 weeks post-partum, who had spent 2 years in an Ethiopian refugee camp before immigrating to the U.S one year ago, presented with a rash on her left cheek of over 2 years duration. She had noticed the rash first back in Sudan and had persisted during her refugee camp stay. She reported that the rash had been non-pruritic, non-exudative, indolent and asymptomatic. She had no history of photosensitivity, exposure to chemicals or insect bite. A week's trial of anti-fungal topical application was unsuccessful. Her recent pregnancy was uneventful including negative HIV, Hep.BSAg and VDRL serology. Her PMH was negative for major medical illnesses and prior eruptive lesions. She did have a history of positive PPD 1 year ago with negative CXR following immigration to the U.S. She had not been treated with anti-tuberculous agents. Her ROS was otherwise unremarkable. Exam revealed a hyperpigmented nodular lesion approximately 1cm wide and 4 cms long in a semi-circular distribution over the left malar area. The rash was further noted to be non-tender, normothermic with mild induration. Physical exam revealed a healthy 21 year old woman with normal cardiac, pulmonary, gastrointestinal and neurological exam, with no evidence of lymphadenopathy and a similar rash elsewhere. Her CBC was within normal limits. Given the patient's ethnicity and travel history, leishmaniasis and cutaneous mycobacterial infections were included in the differential. Consideration was also given to Sarcoid and variant discoid lupus. (Syphilis was considered unlikely, given recent negative serology). A diagnostic excisional biopsy revealed sarcoid-like granulomas surrounded by mild chronic lymphocytic infiltration. Special stains (including AFB, giemsa, PAS, Fite, Giemza and warthin-starry) did not reveal pathogenic microorganisms. Patient was initiated on a local topical steroid application (clobetasol propionate) for two weeks with dramatic resolution of the rash. A diagnosis of Sarcoidosis was therefore made by exclusion and dramatic response to treatment. IMPLICATIONS/DISCUSSION: The protean manifestations of Sarcoidosis include a) Erythema nodosum (most common, although non-specific lesion). b)Lupus pernio (classically involving the rim of the nose and associated with infiltration of the nasal mucosa causing ulcerations or even fatal airway obstructions). c) Papules, nodules or plaques (as seen in the above patient). d) Psoriasis like rash. e) lesions in scars and tattoos. The learning objectives of this presentation are to review the cutaneous manifestations of Sarcoidosis, the differential diagnosis of nodular cutaneous lesions in an imigrant population and the pathophysiology of Cutaneous Sarcoidosis. Considering the growing immigrant population in the U.S, the differential of an indolent chronic rash should include parasitic infestations. However, in patients of African decent, the extremely variable masquerading rash of sarcoidosis should be considered as an important part of the differential. Hemochromatosis is the most common adult genetic disorder. Early diagnosis enables rapid treatment and an improved prognosis. The first clinical manifestation is represented by arthropathy in 45% of the cases. The articular features of are often nonspecific and associated with a delay in diagnosis that may compromise outcome. Treatment of the arthritis with analgesics is often disappointing, but identification of the underlying disease permits institution of life-saving phlebotomy therapy. The aim of this report is to highlight the fact that patients with premature osteoarthritis should alert the physician to screen for hemochromatosis in order to formulate the correct diagnosis before the development of severe internal organ involvement. MRI of the head was done to rule out pituitary masses and was normal. After extensive review it was found that she received an injection of Kenalog 40 mg to the right shoulder from another MD six weeks prior to presentation. Without any other exogenous sources of steroid, it was concluded the pt had experienced cushingoid symptoms from the Kenalog injection followed by secondary adrenocortical insufficiency at presentation. IMPLICATIONS/DISCUSSION: Therapy with pharmacological doses of glucocorticoids is the most frequent cause of secondary adrenocortical insufficiency. The usual presentation is similar to that of primary adrenocortical insufficiency, with two important exceptions. Since pituitary secretion of ACTH is deficient, the characteristic hyperpigmentation of Addisons disease is absent. The clinical features of mineralocorticoid deficiency are usually absent in the unstressed state, and therefore volume depletion, dehydration, and electrolyte abnormalities are usually absent. Hypotension is also less severe, except in acute presentations. Hyponatremia can be present, and is usually due to water retention and inability to excrete a water load rather than to sodium loss. The clinical features of ACTH and glucocorticoid deficiency are nonspecific and consist predominantly of weakness, lethargy, easy fatigue, anorexia, nausea, and occasionally, vomiting. Patients may also describe arthralgias, myalgias, and exacerbation of allergic responses. With acute decompensation, severe hypotension or shock may occur and be unresponsive to vasopressors unless glucocorticoids are administered. At that time he also described shortness of breath, insomnia, anorexia, and a sense of anxiety. The exam revealed an agitated but otherwise well-appearing man with tachycardia. Mr J had a positive urine cocaine screen. He was admitted and ruled-out for myocaridal infarction (MI) with serial troponins and electrocardiogram. Two months later, Mr J returned with similar complaints. Again his urine was positive for cocaine and his serum was negative for troponins. Over the next three months he returned to the ED four more times with a new resting tremor and a weight loss of over sixty pounds. Cocaine screens remained positive. The patient refused treatment for substance abuse and expressed dissatisfaction with the quality of care he received. Finally in September of 2002 a Thyroid work-up was performed, demonstrating a TSH <0.1 and a free thyroxine >6.5. A radioiodide uptake scan confirmed the diagnosis of Graves' disease. The patient had symptomatic relief with beta blockade and propylthiouracil. He is currently scheduled for thyroid ablation. IMPLICATIONS/DISCUSSION: An estimated 1.5 million Americans (0.7%) abuse cocaine chronically. In 1998, cocaine contributed to Emergency Department visits on 152,000 occasions (1). Cocaine abuse is a potentially life-long chronic disease. Acute cocaine intoxication may mimic other diseases, preventing or delaying their diagnosis. An easy explanation for Mr. J's tremor, weight loss and agitation were already available, thus a thyroid work-up was delayed. Cocaine abuse may draw the physician's attention to conditions caused by chronic abuse rather than unrelated medical problems. MI should be ruled out, but not without considering hyperthyroidism. In one study of 165 patients with cocaine intoxication and psychotic symptoms, only 18% were given the proper diagnosis of schizophrenia on the initial evaluation (2) . Finally, some physicians may perceive substance abuse as a primary and overriding health care problem. Mr. J did not wish to address his cocaine abuse and still rightly expected treatment of his other medical problems. In conclusion, many misperceptions may cloud the diagnostic process with patients who abuse cocaine: confusion of symptoms with drug effects, distraction from unrelated medical conditions and prioritizing abstinence above other medical care. LEARNING OBJECTIVES: Recognize the potential for endocarditis after an endometrial sampling procedure. CASE INFORMATION: Antibiotic prophylaxis is not recommended prior to endometrial sampling procedures or even dilation and curettage because of the very low risk of infectious complications. We report a case of acute bacterial endocarditis caused by Streptococcus agalactiae following endometrial sampling in a woman with no known valvular abnormalities. A 59-year-old woman with a history of abnormal uterine bleeding who had undergone office based endometrial biopsy about two weeks earlier was found unresponsive at home and was brought to the hospital. She was noted to have a temperature of 102.5 degrees Fahrenheit; she was awake, but did not speak and withdrew her left side more than her right side to painful stimuli. She also had petechiae in her conjunctiva, fingers and toes. Laboratory evaluation showed white blood cell count of 21,400 cells/mm3, normal electrolytes and glucose, and normal liver enzymes. CT scan of the head was normal at presentation. On hospital Day 3, blood cultures from admission grew beta-hemolytic Group B Streptococcus from all samples. Transthoracic and transesophageal echocardiograms demonstrated a 4Â3 cm lesion on the posterior leaflet of the mitral valve with moderate mitral regurgitation. Repeat CT of the head demonstrated a large left middle cerebral artery infarction, but no evidence of abscess. The patient responded to antibiotics, but died of complications before valve replacement surgery could be performed. IMPLICATIONS/DISCUSSION: Endometrial assessment by means of biopsy or sampling of endometrial cells is a minimally invasive alternative for dilation and curettage or hysteroscopy. Techniques using the Pipelle endometrial sampling device are the most popular due to high sensitivity and specificity compared to other office based procedures, and low rates of complications. In dilation and curettage, studies show transient bacteremia in 5% of patients and up to 0.34 in 100 patients have fever complicating their procedure. We hypothesize that our patient's endocarditis was caused by bacteremia resulting from her endometrial sampling, making this a very unusual complication of a common procedure. There is no data supporting the use of antibiotic prophylaxis for office based (i.e. Pipelle) or operating room (i.e. dilation and curettage) endometrial sampling. Unfortunately for our patient, mortality rates for Group B Streptococcus endocarditis approach 40%, similar to those associated with staphylococcal species.