16 Dec - TTP

Here are the links to articles mentioned in todays CPC about a case of TTP:

NEJM Case Records Dec 9, 2010 - 16 y/o F with lupus nephritis and ADAMTS-13 antibody mediated TTP.

How I Treat TTP - Blood, Nov 18, 2010

Blood Smear Review fron  NEJM - Aug 4, 2005

8 Dec - Endocarditis

This AM - patient with a bioprosthetic valve in the aortic position presented with a few weeks of "flu like illness" and neck pain and muscle spasms.  Imaging of the neck was unremarkable but abdominal imaging revealed splenomegaly and probable splenic infarcts.  This constellation of findings is very worrisome for prosthetic valve endocarditis.  Cultures were noted to be positive for GPCs later in the day.

Prosthetic valve endocarditis is a challenging disease to treat, and difficult to cure without surgery.  PCN susceptible strains of streptococci respond better and may be treated without gentamicin with good cure rates,  Staphylococcal endocarditis requires the addition of gentamicin and rifampin and is more likely to require surgery. 

Culture negative endocarditis is a special case.  Most commonly, prior outpatient antibiotics are the culprit.  Always check blood cultures in patients who are at risk for endocarditis when they present with undifferentiated febrile illness.  This table helps narrow the choices in patients with culture negative endocarditis based on thier comorbid illness or other risk factors.

3 Dec - Dysphagia

Friday's case was a 60ish male who presented with insidious onset of dysphagia without odynophagia.  Swallowing solids but not liquids produced the symptoms.  The problem had been slowly pregressive over a couple months.  He had a history of CAD, atrial fibrillation and prior tobacco use.  CXR revealed an enlarged heart but no masses.  Barium swallow revealed slow transit and delayed passage of a capsule at the distal esophagus. 

The proper evaluation of dysphagia is important both to relive symptoms and to diagnose potentially lethal conditions like malignancy.  Oropharyngeal dysphagia is usually due to motor dysfunction and esophageal has a more even distribution of motor and structural causes.  In general, dysphagia to solids and liquids suggests motor causes and solids only suggests a structural lesion.

This article from Gastroenterology Clinics - Volume 32, Issue 2 (June 2003) is available through MD consult and reviews the common causes as well as a suggested diagnostic approach.  In general the first test is a barium swallow, followed by endoscopy if structural lesions or esophagitis are suggested.  If motor processes such as achalasia or esophageal spasm are suspected then manometry is the next test.

Questions about achalasia have a habit of showing up on boards.  This NEJM image case shows a fairly dramatic example.  Treatment is either surgical (heller myotomy) or medical (botox) in poor surgical candidates.  Botox does not have as durable of effect as surgery. 

2 Dec - Hypercalcemia

This AMs case was a 62 y/o female who presented with progressive muscle weakness of several weeks duration.  There was also some complaints of numbness.  She had lost a significant amount of weight and was not eating well due to some abdominal pain.  She was brought to the hospital when she was no longer able to stand.  Her past history was remarkable for MS, but this was significantly different from any of her prior flares.  She did report a 40 pack year history of smoking.

On initial lab evaluation she was found to have an elevated calcium at ~ 17.  Phosphorus was elevated as well.  Her PTH was 20, and PTHrP is pending at this time.  Her CXR revealed a large perihilar lung mass and follow up CT of the chest revealed the mass and assocaited mediastinal adenopathy.

She recieved IV fluids, furosemide, dexamethasone, calcitonin, and pamidronate.  Her muscle weakness improved as her calcium levels fell.

Hypercalcemia is one of the most common oncologic emergencies.  It is either humoral in origin from ectopic PTHrP secretion by the tumor, due to overexpression of 1,25 vit D by lymphomas, or due to direct bony destruction such as in myeloma.  It usually occurs in patients with known cancer but can be a presenting symptom of the disease.  Patients are often quite volume depleted and require significant amounts of IV fluids in the first few hours.  If calcitonin is given, it must be IM or IV, as the intranasal formulation is not active. 

This article from Mayo Clinic Proceedings summarizes hypercalcemia and other common oncologic emergencies and should be reviewed.

Contrast this presentation with an earlier discussion of hyperparathyroidism.  Remember that in general calcium levels over 12 are almost always due to malignancy. 

Dec 1 - ECG Review

No case today due to team transitions, focused on ECG review with Dr Hirsh.  Below is the wave maven site from Harvard med school.  Great to review.  We will do this from time to time at Wednesday morning report.

ECG Wave Maven - Beth Israel Deconess Med Center.

Nov 29 - Patient Safety Conference

Todays conference further reviewed data regarding a recent Medicare inspector general report on medical errors and harm events in US hospitals.  Their study identified serious harm events in ~14% of patients, with another 14% suffering some type of temporary harm.  Overall the physician reviewers found ~44% of these events to be preventable.  The most common causes of harm were medication related - with high risk drugs such as anticoagulants, insulin, narcotics, and sedatives accounting for the largest numbers of medication related harm events.  Substandard care and failure to monitor accounted for most of the other significant harm events.  The cost of these harm events was estimated at over $300 million in October 2008 alone for an annual cost of $4.4 billion annually. 

We will focus the next few months of patient safety conferences working improving high risk medication safety - starting with insulin.

There are a variety of patietn safety organizations that we will use in upcoming patient safety conferences.  Some of the best are linked below.  As you can see - a lot of folks are paying attention.

Institute for Health Care Improvement (IHI)
Agency for Healthcare Research and Quality
National Patient Safety Foundation
Society of Hospital Medicine Quality Initiatives
National Quality Forum
Leapfrog
Premier, Inc.

Nov 22 - Inpatient DM management

The topic of the day - optimizing inpatient management of hyperglycemia.  Linked here are the guidelines for management of diabetes per the American Association of Clinical Endocrinologists.  The section on hospital mamagement is below.  The full document covers how to dx and tx diabetes in the outpatient setting, as well as optimum strategies to manage risk factors such as HTN and hyperlipidemia.  Regardless of the regimen used, the plan should be reviewed and modified on a daily basis of the glucose remains uncontrolled or if hypoglycemia events occur.

Some adverse events reviewed on AHRQ's Web m&M are linked here and here.  The second link goes into more detail about hypoglycemia prevention and pitfalls even when optimum order sets are available.  As with any complex treatment algorithm in medicine these days, a multidisciplinary approach is key.

Finally - the Society of Hospital Medicine has an extensive and well resourced toolkit to enable rapid adoption of current best practices.

From AACE Guidelines:

• If appropriate for the patient, use intravenous insulin infusion (grade A)

• If hyperglycemia is reproducibly present and intravenous insulin infusion is not necessary, order scheduled subcutaneous insulin (grade B)

• For subcutaneous management, order amounts of insulin sufficient to cover basal and nutritional needs (grade B)

• Plan the patterns of glucose monitoring and delivery of insulin to match carbohydrate exposure (grade B)

• Revise the amounts of scheduled insulin daily or more frequently based on patient response (grade B)

• For patients receiving scheduled insulin, order an as needed correction dose of subcutaneous insulin with dosing that is: (a) proportionate to blood glucose elevation and insulin sensitivity of the patient and (b) appropriate to time of day; specify the times or mealtimes to which the order applies (grade B)

19 Nov - Syncope with funky neurological symptoms on recovery

Today's case was a previously healthy 71 y/o male who sustained a witnessed syncopal attach where he fell and hit is face on the ground.  He rapidly regained consciousness, but when he awakened he could not use his hands or feet and had bilateral upper and lower extremity numbness.  Distal muscle weakness was more prononced than proximal muscle weakness.  On arrival to the hospital he was bradycardic with normal blood pressure and denied light headedness.  ECG revealed sinus bradycardia, rate ~48 with an LBBB.  Due to his neurologic signs and symptoms MRI of the cervical spine was performed which revealed cord contusion with siginificant canal stenosis and osteophytes.

Spinal cord contusions are typically seen in contact sports injuries and motor vehicle crashes.  Such an injury from a low velocity fall is unusual.  An ovid search turned up no articles reporting this occurence. 

Steroid treatment is controversial but may be effective if started within a few hours of injury.  This article reviewed current recommendations - SPINE Volume 28, Number 9, pp 941–947 and is available on Ovid full text.

As far as the bradycardia goes - he is felt to need permanent pacemaker - the AHA and ACC recently revised guidelines for permanent caridiac pacing and antiarrhythmia devices and it would be useful to be familiar with these gudelines (especially class I indications) for board purposes.

18 Nov - Graves

Today's case was a 33 y/o F that presented with symptoms of emotional lability and weight loss over two months.  The symptoms began shortly following a flu like illness.  Family members had noted increased tearfulness that had progressively worsened.  There was no previous history of depression.  She did have a history of low back pain managed with prescription opiates, but no recent changes in pain or med dosages were noted.  Family members had noted neck swelling.  On examination she had marked nodular thyromegaly with an audible bruit.  There was no evidence of exophthalmos.  She was tremulous.  Heart rate was 120's to 130's.  TSH was 0.02 and T4 was 4.  She was hypercalcemic.  She was diagnosed with Grave's disease with thyrotoxicosis and started on beta blockers and PTU.

Clinical Pearls:

- Imaging and antibody tests are not necessary to establish the diagnosis in typical cases such as this.
- methimazole is more convenient and probably superior to PTU in terms of effectiveness.
- PTU is the drug of choice in pregnancy
- agranulocytosis and hepatotoxicity are potential side effects of PTU
- elderly patietns with graves can present with "apathetic hyperthyroidism" with wt loss and depression.

This Clinical Practice article reviews a case remarkably similar to the one discussed in MR and provides a good diagnostic stragety for cases that are not as typical in presentation.

Hypercalcemia in graves has been linked to PTHrP (usually seen in hypercalcemia of malignancy) as a cause of non-PTH dependent hypercalcemia. 

Graves ophthalmopathy has been reviewed here and here.  It is clinically evident in ~1/3 of Graves patients, but can be seen in imaging in 70-80% of cases.  Treatment involves immunosuppressive therapy with steroids and potentially TNF inhibitors among other treatments.

17 Nov Septic Arthritis

Long break from posting for a thousand reasons - but wanted to add some info from today's discussion on septic arthritis.  The case was a pt with previous history of total knee arthroplasty (bilateral) and diabetes mellitus.  He as admitted with fevers with swelling and tenderness of his knee.  Arthrocentesis revealed bloody synovial fluid with ~ 50 000 leukocytes.  Only 50% or so were polys.  Cultures of blood and synovial fluid were both noted to be positive for group G streptococcus.

Acute monoarthritis must be evaluated rapidly.  Arthrocentesis is quick, easy, and safe and is the key to differentiating septic arthritis from other causes of monoarthritis.  Do not rely on elevated uric acid levels in serum analysis to make a diagnosis of gout.  Gout and septic arthritis can and often do coexist.  Hematogenous infections of prosthetic joints usually result in loss of the joint.  In some cases of acute postoperative prosthetic joint infection the prosthesis can be salvaged with joint washout and prolonged (6 months) antibiotic therapy.

Linked here is the seminal article from NEJM on the approach to monoarthritis.  Still the ideal approach to this situation.

Also - diabetes mellitus is a significant risk factor for invasive streptococcal infections, and there seems to be an increasing indicence of Groups B, C, and G streptococcus.  These organisms are exquisitely susceptible to penicillin.

1 and 2 Sep - Stroke potpourri

Two cases this week have been of unusual nerologic deficits in young patients.  One patient presented with an episode of syncope and awakened with a headache and residual hemipareisis.  There was significant sinus bradycardia present and CNS imaging with MRI revealed multifocal lesions consistent with "emboli or vasculitis or small vessell ischemic disease."  The second case was a 27 year old male with R sided weakness and expressive aphasia who was found to have a L MCA distribution stroke.  TTE suggested PFO with shunt, TEE did not reveal any PFO, shunt, or thrombi.  In both patients classic stroke risk factors were not present.

The impact of an atrial septal defect on the risk of recurrent stroke is debated, but the current practice guidelines review from the Neurology journal suggest that an isolated ASD has no impact on recurrent stroke risk.  An ASD associated with an atrial septal aneurysm does appear to increase recurrent stroke risk.  The choice between antiplatelet and anticoagulant therapy is currently weighted towards antiplatelet therapy.  Anticoagulation with warfarin does not clearly improve outcomes and does increase the incidence of minor bleeding.

The evaluation of stroke in young patients (15 - 45 years) encompasses a search for risk factors that are not typical of stroke in older adults.  In a recent review of the topic, atherosclerosis accounted for 21%, cardioembolism in 17%, non-atherosclerotic vascular (migraine, dissection, etc) in 17%, but 36% had undetermined cause. 

Some zebras to consider:

Sneddon syndrome - livedo reticularis associated with ischemic cerebrovascular disease and hypertension.  Antiphospholipid and anticardiolipin antibodies are commonly found.  Usually seen in adult women and the rash follows the stroke.

Moyamoya syndrome (reviewed in May 2009 NEJM) - progressive stenosis of internal carotids in young patients.  Leads to development of distal small vessell collateral ciculation that gives a "puff of smoke" (moyamoya in Japanese) appearance on angiography.  Can lead to ischemic or hemorrhagic stroke.  Cranial XRT, down syndrome, and NF1 predispose to this condition.  Rule of thumb - if a disease is reviewed in NEJM it is fair game for an ABIM examniation question.

Aug 20 - vertebral osteomyelitis and epidural abscess

This mornings case was a 51 year old man presenting with one month of progressive back pain (thoracic) associated with low grade fevers, leukocytosis, and weight loss.  Imaging via CT at an outside facility revealed degenerative changes, spinal stenosis, and not much else.  He presented here febrile with some lower extremity spasticity but no bowel or bladder dysfunction.  he did have decreased sensation in the lower legs.  There was point tenderness over the mid thoracic spine.

There are multiple red-flag symptoms in this back pain presentation.  This recent evidence based guideline from Annals identifies some common alert signs and presents an algorithm for workup:

In this case the patient was found to have vertebral osteomyelitis an epidural abscess due to methicillin susceptible Staphylococcus aureus and was taken for surgical decompression.  He is in the early stages of an 8 week course of IV antibiotics. 

Some key points:

Medical therapy with IV antibiotics alone is appropriate therapy for select patients with epidural abscess according to this Arch Int Med study.  This is a bit of a change from previous dogma.

There are no practice guidelies dictating duration of therapy, with differing opinions regarding duration of therapy.  I generally choose 8 weeks, but this NEJM review from March 2010 suggests a duration of 6 weeks for most patients.

Regarding the efficacy of vancomycin compared to nafcillin for MSSA - see these articles:

AAC Jan 2008 - mortality with vanco 37% compared with 18% treated with beta-lactams.
Medicine Sep 2003 - study of MSSA bacteremia without endocarditis - failure rate with vanco near 20% compared with no failures in the nafcillin group.

The bottom line is beta lactams are clearly superior to vancomycin in S aureus infections, and in the case of serious or life threatening S aureus infections I feel it is worthwhile to desensitize to beta lactams in order to use these agents if at all possible.

Aug 19 Syncope and Pulmonary Hypertension

This case was an elderly female with previously repaired aortic stenosis (mechanical AVR 15 years prior) who presented with recurrent syncope over the past 1 - 2 years.  The syncope occured with exertion and was increasing in frequency.  She carried a diagnosis of hypoxemic "COPD" but no PFT records were available.  PaO2 was low, and there was no evidence of CO2 retention.  She was suspected of having pulmonary hypertension by ECHO but had refused R heart catheterization to confirm. 

Will not go into discussion of syncope workup here - see this post from July 7.

Effort syncope has been described as a common symptom in primary pulmonary hypertension for quite some time.  An early effiort to identify the mechanism found progressive decreases in blood pressure ultimately resulting in acute R heart failure accounted for the syncopal attacks.  Tachy or brady arrhtyhmias occured but did not seem to be responsible for the acute drop in BP.

A nice review of primary pulmonary hypertension from AAFP is here.

Aug 18 - Lupus Flare

Today's case was a patient with a known prior history of SLE that presented with multiple complications - including fevers, arthralgias and myalgias, and a rash on her face and abdomen.  Workup revealed evidence of multisystem disease, with elevated cardiac enzymes, hematuria, anemia, and thrombocytopenia.  Complicating her picture was a recent hospital stay where she rec'd heparin, and anti-heparin antibodies were positive. 

Peripheral blood smear revealed schistocytes:

Click this link for a nice review of mircoangiopathyc hemolytic anemia from the American Society of Hematology Image Bank

The working differential was HIT/HAT vs TTP vs other thrombotic microangiopathy.  TTP incidence is elevated in patients with SLE, and autoantibodies to ADAMTS-13 seem to play a role in pathogenesis.

Here is a link to another review of SLE pathogenesis in general from a recent NEJM article.

11 August - cholangitis

Running behind on blog updates due to a recent plague visitation at the Rasnake house. 

Two cases recently presented of patients with biliary tract complications.  One was a patient with fever, jaundice, and leukocytosis with a history of previously instrumented biliary tract due to carcinoma - diagnosis was cholangitis. 

Charcot's triad: rever, RUQ pain, and jaundice
Reynold's pentad: above three plus mental status change and hypotension.

Broad spectrum antibiotics to cover gram negative rods and anaerobes are important, but the key to management is decompression of the biliary tree via ERCP or other invasive methods.

The other case was a patient with a progressive mass in the vicinity of the pancreas but separate from pancreatic tissue that had been slowly enlarging over many months.  Most likely diagnosis in this case is lymphoma pending tissue biopsy.

Friday I was out due to monthly residency review meeting - regular updates will resume this week.

23 Jul - Pleural Effusion

This AMs case was a patient who presented with dyspnea and R chest pain 6 weeks following an episode of pneumonia.  CXR revealed a large R sided pleural effusion.

Thoracentesis was performed with findings of cloudy fluid with a protein ratio <0.5 but an LDH ration >0.6, consistent with an exudate.  No organisms were seen on gram stain and the glucose and cell counts were unremarkable.

Since we are not doing thoracentesis yet in the sim lab - you all should at least review the video here at the NEJM website before peforming this procedure.  Common ommisions that I have witnessed include not having the patient fully exhale and hold their breath when removing the catheter from the chest at the completion of the procedure.

To interpred the results of the pleural fluid analysis, consult these articles here and here by the guru of pleural fluid analysis Dr Light.  His criteria show up frequently on exams and challenge bowls:

pleural fluid / serum protein ratio >0.5
pleural fluid / serum LDH ratio >0.6
pleural fluid LDH > 2/3 the upper limit of serum normal value

Any one is sufficient to diagnose an exudative effusion, but the specificity is only 82 - 83%

Light's criteria are the most sensitive (98%), but a serum albumin - pleural albumin of <= 1.2 or a pleural fluid cholesterol >= 60 are both more specific at 92%

22 Jul - Pulmonary Embolism

This AMs case was a patient admitted with subacute onset of dyspnea.  Initial evaluation revealed an elevated D-dimer, swollen LLE and normal CXR.  CT PE study revealed extensive bilateral PEs with proximal involvemetn and R heart strain.  He had made several recent long car trips but had no other known predisposition to thromboembolism. 

For a recent summary of acute PE management see this NEJM review from this past week's issue.

Regarding selection of PE patients for outpatient treatment or early discharge - know that the data to support this practice is of poor quality and that patients with hypoxemia, RV dysfunction, large proximal PEs, and obesity (>110 kg) were excluded from the studies of outpatient treatment.  These individuals should be managed as inpatients until oral anticouagulants are at therapeutic levels and clinical stability is achieved.  This recent review should be noted before you consider early discharge of a PE patient.

21 July - Mechanical Complications of Acute MI

F/u from Dr Sullivan's CPC of a patient who presented with an acute MI and sustained a septal rupture developing an acute VSD.  Despite odds against it, this patient survived with emergent CT surgical intervention.

Mechanical complications of AMI (free wall rupture, septal rupture, papillary muscle rupture w/ acute MR, RVMI, and cardiogenic shoc) are all favorites of medicine test writers.  I will refer you to this recent Cleveland Clinic CME website for the latest and greatest - review this for boards / inservice!

Steroid therapy, which this patient had recieved, is an identified risk factor for myocardial rupture in some reports, but this link has been debated.

July 16 - Epilepsy

"For when the breath does not find entrance to him, he foams and sputters like a dying person. And the bowels are evacuated in consequence of the violent suffocation; and the suffocation is produced when the liver and stomach ascend to the diaphragm, and the mouth of the stomach is shut up; this takes place when the breath does not enter by the mouth, as it is wont. The patient kicks with his feet when the air is shut up in the lungs and cannot find an outlet, owing to the phlegm; and rushing by the blood upward and downward, it occasions convulsions and pain, and therefore he kicks with his feet."
-Hippocrates, "On the Sacred Disease" ca 400BCE

Late entry here. Pt was a pregnant female who presented with recent seizure activity and had recurrent seizures in the hospital.  She had been off anticonvulsant therapy for some time.  Seizures become more common in about 25% of pregnant women with epilepsy, and a variety of reasons may play a role:

1- increase clearance and larger VD for antiepileptic drugs (AEDs)
2 - noncompliance or cessation of AEDs due to concern for fetal risk
3 - possible lower sz threshold related to stress, sleep loss, and hormonal changes

Key poitns in managing epilepsy in women of childbearing age:

Ideally have effective seizure control before conception

Supplement folate

Use monotherapy at lowest effective dose

Avoid valproate if possible - this drug has the strongest link to teratogenicity.

The newer agents lamotrigine and carbamazepine may have an increase risk of cleft lip and palate.

This article from the Neurology journal reviews the current literature and concludes that no antiepileptic drug has been shown to be conclusively "safe" but registry data indicates that most agents can be used with a low incidence of fetal complications.

Jul 15 - DVT

Todays case was a 60 y/o patient with a 9 year history of hemipareisis from a prior hemorrhagic stroke.  He was admitted with acute onset RLE pain and swelling and found to have an acute DVT.  His D-dimer test was positive and a doppler US revealed a partially occlusive distal thrombus.

Review this Annals In The Clinic article about DVT management.  Also has some slidesets that can be used for student teaching.

Whe establishing your pretest probability to determine diagnostic testing - know that there are different Well's criteria for DVT and Well's criteria for PE.

Regarding testing for thrombophilia - there are no clinical trials to support this practice according to a recent Cochrane review of the topic.  This extensive review in the American Journal of Medicine contradicts the MKSAP answer from today, in that it proposes that there are very few indications for thrombophilia testing, and there is little evidence to back up the effectiveness of testing in these circumstances to prevent clinical events.

Jul 14 - Chest Pain

Don't tell my heart, my achy breaky heart
I just don't think it'd understand
Cause if you tell my heart, my achy breaky heart
It might blow up and kill this man...
-B. Cyrus






This mornings case was a patient admitted with substernal chest pain and pressure radiating to the left arm and jaw.  The symptoms developed shortly after hearing some traumatic news about a family member.  She had a known history of CAD with a prior MI, CABG, and later Stent to an occluded SVG.  Her initial ECG revealed antero-septal q waves and ST elevations (similar to old ECGs)

Her initial cardiac enzymes were negative, but the second set at 6 hours revealed a significantly elevated troponin of 22.  Urgent cardiac catheterization was peformed which revealed intact bypass grafts and no obstructive coronary disease.  LV-gram revealed apical ballooning sonsistent with Tako-tsubo cardiomyopathy.

Teaching points:
Review this article on the evaluation of acute chest pain.  All acute chest pain presentations must be rapidly screened for immediately life threatening diagnoses - MI, PE, dissection, and pneumothorax.  The differential diagnosis of chest pain is vast, but concentrate initial efforts at ruling in or out the four mentioned above.  Most ED chest pain protocols trigger immediate ECGs, chest X-rays, cardiac enzymes, and oxygen saturation measurements that can give you clues to PE, MI, and PTX.  Dissection is a little more tricky, and high clinical suspicion is key.

Takotsubo cardiomyopathy, also known as apical ballooning syndrome, stress induced cardiomyopathy, and broken heart syndrome, is reviewed in this NEJM Clinical Decision Making article from 2009 - their case has many features similar to the one discussed today.

The optimum evaluation and treatment of acute coronary syndromes is constantly in evolution, and this editorial summarizes the current optimum approach to patient management.  Key point - risk stratify patients using a valid tool such as the TIMI risk score, and reserve coronary angiography for those in the high risk category.

Finally - use caution in treating women with heart disease the same as men.  This study and review of prior trials suggests that using a routine invasive diagnostic strategy in women is of no benefit and may in fact be harmful.  This data is in clear contradiction to results demonstrated in men.

Jul 9 Encephalitis

Was not there for the presentation, but am very familiar with the patient discussed.  Middle aged gentleman with 3 - 4 weeks of altered mental status, headaches, and jerky limb movements.  MRI negative, but CSF persistently abnormal with elevated WBC (all lymphos), elevated RBC, and high protein.  Rec'd an initial diagnosis of aseptic meningitis but was readmitted when symptoms worsened.  This time he improved substantially with empiric acyclovir treatment for suspected HSV encephalitis.  Some key points:

There is only 1 type of encephalitis that is readily treatable, and that is HSV-1.  Be sure to test for it - and the test of choice is PCR of spinal fluid.  Do not order "HSV serology" because you will not get the answer the patient needs.  You can check for other things like West Nile Virus or the equine encephalitis viruses, but there is not much you can do for the patient.  So if you only have a couple drops of CSF - do the HSV PCR.

There is a reseource to be aware of: the Tennessee Unexplained Encephalitis Survey (TUES) study: They will enroll your patient in an ongoing study that will test any leftover CSF and blood samples for an extensive panel of encephalitis agents. 

The IDSA has published a comprehensive practice guideline that briefly reviews the exposure risks, testing, and treatment for pretty much any cause of encephalitis.

Jul 8 - MMP

MMP in todays's case coud mean multiple medical problems or multiple myeloma patient.  This case demonstrated how to take a rational approach to evaluating a complex patient with multiple simultaneous diseases presenting with a varied symptoms.  It shows that Occam's Razor is sometimes dull.  Never forget Saint's triad, or the famous Hickam's dictum - "A patient can have as many diagnoses as he damn well pleases!"

Some of this patients several diagnoses:

Obstructive Sleep Apnea:  given the growing waistlines of a growing population this is a growing problem.  OSA patients can have the same impairment driving as an intoxicated patient.  Treatment with CPAP improves quality of life and decreases car accidents.  Effect on other outcomes is not as well defined.  It is criticial to differentiate OSA from Central Sleep Apnea as CPAP might not be of much benefit in the latter.  One form of this is a rare genetic disease called Ondine's curse where patietns simply lack the respiratory drive when asleep.  So don't ever cheat on water nymphs.

Multiple myeloma was an almost indicental diagnosis in this patient.  Classic findings of bone pain and hypercalcemia were absent in this case, and the renal failure seemed to be largely due to obstruction in this case.  Nevertheless, SPEP and UPEP revealed a monoclonal spike and the bone marrow biopsuy revealed elevated plasma cells.  Skeletal survey had a few small lytic lesions in the femur and humerus.

Cardiac Exam Skills - Online Resources

Below are some links to cardiac auscultation aides.  All have free content, some direct you to optional purchases.  I am thinking of getting some of the blaufuss tools for the sim lab - post comments if you find their free stuff useful.  Looks like they have a device to pipe the audio from the murmurs into your own stethoscope. 

Mulimedia cardiac auscultation tools - matches sounds with videos of physiologic process to enhance learning:
http://www.blaufuss.org/

Large database of free heart sounds from Texas Heart Institute.  Free is good!
http://www.texasheartinstitute.org/education/cme/explore/events/eventdetail_5469.cfm

Murmur learning tool - 5 free murmurs, the rest cost ~$150:
http://www.cardiosource.org/Certified-Education/eLearning-and-Products/Heart-Songs-3.aspx

7 July - Evaluation of Syncope

The first MR of the academic year focused on a 78 year old patient with a history of syncope.  He had two prior episodes over the preceeding three years.  The most recent event had a few second prodrome followed by loss of consciousness and a fall to the floor.  He had a history of DM and treated hypertension, but blood glucose and blood pressure checked by family were normal.  ECHO and ECG were normal.  Postural blood pressure measurements were normal.  The most likely diagnosis in this case was neurocardiogenic syncope.

The evaluation of syncope usually results in unnecessary testing that rarely leads to a diagnosis.  The frequent use of carotid ultrasound is unsupported by evidence and rarely if ever alters patient management.  Even routine lab testing, including cardiac enzymes, are almost always a wast of money unless there are clinical symptoms other than syncope to suggest their use.  This Archives of Internal Medicine article summarizes the diagnostic yield of most commonly used tests.  The highest yield test was also the cheapest - postural blood pressure management.  This 2000 NEJM review directed at primary care physicians walks you through the preferred, rational evaluation of this condition.

The evaluation of neurocardiogenic syncope is summarized in this 2005 NEJM article.  Tilt table testing remains the gold standard for evaluation of this condition.  Treatment has traditionally been with beta blockers, however there is little evidence to show they work.  Midodrine, fludrocortisone, and SSRIs have all been shown to be effective.

Remember - syncope is a finding, not a diagnosis, but in most cases you need little more than your brain, ears, and hands to make the diagnosis.

30 June - Orientation Reference

Welcome, welcome!

During orientation, Dr. Rasnake mentioned a reference for practice called the "Checklist Manifesto" by Atul Gawande. This is an excellent book! The same author wrote a piece for the New Yorker entitled The Checklist in 2007 which contains many of the highlights and thoughts which were the basis for the book.

Here is the weblink:
http://www.newyorker.com/reporting/2007/12/10/071210fa_fact_gawande

21 June - recurrent vesicular rash

Teaching rounds today - 32 y/o female with several medical problems, to include celiac disease (? Dx) and a history of recurrent vesicular lesions on her forehead diagnosed as zoster.

The differential diagnosis of vesicular rash includes a variety of infectious and noninfectious causes - some benign and some potentially fatal.  Varicella, HSV, vaccinia virus, variola virus, enterovirus, rickettsialpox, impetigo, dermatitis herpetiformis, phytodermatitis, and phytophotodermatitis all need to be considered in the correct clinical setting.

Scraping of the vesicle base to obtain cells for DFA is my preferred method for rapid HSV diagnosis, and probes are available for HSV1, HSV2, and VZV.  This article has a summary of the collection procedure as well as other diagnostic methods for herpes virus infections.

Yes, phytophotodermatitis exists - so beware all those mojitos, gin&tonics, coronas, margaritas, and any other lime containing beverage out in the sun this summer.

One recent case in the ID literature emphasizes the importance of making a definite diagnosis - Kaposi varicelliform eruption - generalized vesicular rash that mimics chickenpox but is caused by HSV or coxsackie virus.  Pts with a history of eczema are at significant risk for this.

18 June - Myoclonus

87 y/o female admitted with episodic myoclonus.  She had an underlying history of dementia, Alzheimer's type on treatment with memanting and several other medications.  Her symptoms were rhythmic movements of the bilateral upper extremities and neck muscles.

Pearls:

Myoclonus can occur as part of the natural progression of Alzheimer's, but if it is early and pronounced other diagnoses such as CJD should be considered.

Certain medications (including memantine) can cause myoclonus when drug levels become high.

Clonazepam is an effective intervention for myoclonic symptoms.

17 June - ITP

Today's case was a college student that has been followed for thrombocytopenia for ~ 1 year now.  His initial presentation was with a brief illness with subjective fevers and diarrhea and a transient, possibly petechial rash.  No bleeding occured.  CBC at student health revealed a PLT count of 23K.  He was admitted for workup and observation and was noted to have a prompt increase in PLT count (>50K at discharge).  Peripheral smear was unremarkable, HIV serology was negative, and overall clinical status was good.  He was diagnosed with ITP and discharged.  Over the following year his PLT count has fluctuated but never normalized.  He underwent bone marrow biopsy which was unremarkable.  He has had no bleeding events.

The ASH Image Bank has a complete case presentation with several representative images.  Nice review of clinical aspects of the topic.  Note the large platelet in the center of this image.

Pearls:

ITP is a diagnosis of exclusion - rule out TTP, leukemia, MDS, sepsis, etc.

ITP = immune thrombocytopenic purpura, not necessarily idiopathic...

There was a recently published international concensus statement on the w/u and management of ITP - published in: Blood, Vol. 115, Issue 2, 168-186, January 14, 2010

Some pearls:

Recommended testing for all adult patients includes peripheral smear, coombs test, Rh type, and serology for HCV, HIV, and H pylori. (I guess only infections that start with H cause ITP?)

Treatment rarely indicated for PLT counts >50K and no bleeding
First line therapy: anti-D immune globulin, steroids, and IVIG
Second line therapy: azathioprine, cyclosporine, cytoxan, vincristine, danazol, dapsone, rituxan, splenectomy
Salvage Therapy:  TPO receptor agonists, campath, chemo, HSCT

16 Jun - Hypertrophic Cardiomyopathy and Abnormal Vaginal Bleeding

Coindidenatl admission with abnormal uterine bleeding and newly diagnosed hypertrophic cardiomyopathy.  Probably unrelated?  Possibly not - this study describes functional vWF deficiency in patients with HCM and associated spontaneous bleeding events.  Hmm.

Hypertrophic cardiomyopathy is reviewed in this JAMA article.  Myosin heavy chain, troponin T, and myosin binding proteins are all described to be mutated in HCM - mode of inheritance is autosomal dominant.  This patient presented with evidence of LVOT obstruction, but only 25% of HCM patients have obstructive physiology.  Given this, murmur may be absent in the majority of patients with this disease.  To better differentiate the HCM murmur from other systolic murmurs, some maneuvers can be attempted (in general, things that decrease LV filling will increase HCM murmur):

Valsalva: increases HCM
Standing: increases HCM
Hand Grip: decreases HCM

Found this neat quiz for working up murmurs online - answers are in the back.

Management consists of preventing sudden cardiac death in high risk individuals, treatment of heart failure symptoms (possibly surgical myomectomy) and management of A-fib which is a common comorbid comdition.

With regards to the abnormal menstrual bleeding in this case, one should be aware of the basics of a primary care approach to this condition.  First - rule out pregnancy.  Second - screen by hx and exam for signs or symptoms consistent with PCOS, other hormone disorders, or bleeding disorders like vWF deficiency.  Any uterine bleeding in a postmenopausal woman should worry you for endometrial carcinoma.

14 June COPD

Teaching rounds today reviewed current data regarding treatment of COPD.  The case discussed was a patient who had been admitted for the first time with a COPD exacerbation.  Several recent studies have better defined the role of various treatments for this common and debilitating condition.

First: smoking cessation and long term oxygen therapy (when appropriate) remain the most beneficial treatments available. 

Second: tiotropium appears to be superior to ipratropium and has been shown to decrease mortality.

Third: antimicrobial therapy is beneficial in patients with moderate to severe exacerbations of COPD.  In general, having 2/3 or 3/3 of the following critieria indicate a moderate or severe exacerbations: increased sputum volume, increased sputum purulence, and increased dyspnea.

Fourth: Benefits of inhaled steroids are less clear for COPD, and when used should be limited to those with moderate to severe disease only.

The ACP In the Clinic series recently reviewed COPD and this article is a quick read that highlights the state of the art in COPD management.

11 Jun - Guillain-Barre syndrome

This AMs case was a 40ish year old man admitted with 4 days of progressive ascending weakness with paresthesias.  Mental status was normal.  He had a recent illness with low grade fevers and myalgias but no other significant symptoms or rashes.  He did have a tick bite about two weeks prior to onset, but did not have any attached ticks at time of admission.  Significant flaccid paralysis of the arms and legs was present and reflexes were absent.  No cranial nerve defects were evident.  He rapidly developed respiratory insufficiency requiring intubation.  CSF evaluation revealed significantly elevated protein and a clinical diagnosis of GBS was made.  He has been treated for ~8 days, initially with IVIG and subsequently plasmapheresis but is still ventilator dependent.

Differential for acute flaccid paralysis includes GBS, myasthenia gravis, botulism, tick paralysis, toxins, polio, non polio enteroviruses, and hypokalemia.  The review article linked here has a nice table that helps differentiate clinical symptoms.

A recent review of GBS is in this issue of the BMJ.

Treatment cosists of observation for mild cases, IVIG or plasmapheresis for moderate to severe.  Plasmapheresis has been shown to be effective vs placebo in clinical trials, and IVIG has been shown to be equivalent to plasmapheresis but has not been compared directly to placebo.

Pearls:

You must measure NIF and/or vital capacity - ABG and O2 sats are usually normal right up until total respiratory failure occurs

Autonomic instability (tachycardia, hypertension) is common.

Elevated CSF WBCs should make you consider an alternate diagnosis.

10 June - Acetaminophen poisoning

This AMs case was a 30ish year old female who had been injured in a car crash approximately 2 weeks prior to admission.   On this occasion she was admitted with significant nausea, vomiting, and abdominal pain.  She had a history of heavy alcohol consumption for several years prior to admit.  In the three days leading to this admission she had taken over 30 acetaminophen tablets (dose unspecified).  On admit her transaminases were elevated >2000, her INR was 1.7, and the bilirubin was 3.  There was no evidence of encephalopathy.  Her admit acetaminophen level was 0.9.

Pearls:

Acetaminophen toxicity occurs when liver sores of glutathione are depleted and the toxic metabolite NAPQI accumulates.  Administration of N-acetyl cysteine (NAC) replenishes glutathione and allows clearance of NAPQI.  Alcohol consumption is a well established risk factor for more severe toxicity.

The Rumack-Matthew nomogram helps predict when toxicity will occur, but this nomogram is only valid for single large ingestions with a known time.  Ingestion of multiple doses over several days or ingestion of extended release formulations can provide misleading results.  Also watch your units - confusing micrograms/ml with micromoles/ml when plotting on the nomogram will throw you off significantly!

Ideally, NAC should be given before the LFTs are elevated to be effective.  Not all poison control centers recommend its use once the acetaminophen level has dropped <20 or is undetectable.  This article from NEJM calls this into question.  They recommend more liberal use of NAC, especially when LFTs are elevated.  They refer to 2 studies that show reductions in mortality on the order of 20% in the setting of fulminant liver failure when intravenous NAC is used.  The cost of NAC is modest - only $50 for a full 72 hour treatment course. 

The FDA has taken steps to relable acetaminophen, remove higher dose formilations from the OTC market, and eliminate drugs such as Vicodin and Percocet that have combinations of high dose acetaminophen and opioids.  They have yet to set formal recommendations for max dosage, but anticipate doses of 3.25 grams for healthy individuals and <2.5 grams or outright avoidance for those who consume ETOH or have liver disease. 

9 June - Hyperparathyroidism

This morniongs case was a quite unusual presentation.  A 28 y/o male was admitted with complaints of dysphagia and odynophagia and had had some concomitant symptoms of headache and photophobia.  He had some neck swelling on examination.  Serum chemistries revealed an unexpected finding of hypercalcemia (Ca >13).  PTH was measured at over 900.  Sestamibi scan revealed significant uptake in the R inferior parathyroid area.

Imortant to differentiate parathyroid carcinoma (rare) from parathyroid adenoma (common).  Features that suggest carcinoma in this case include young age, very high calcium (usually 12 or less with adenoma) and markedly elevated PTH (usually only mildly elevated with adenoma).  This article is a comprehensive review of this rare condition. 

Remember to check PTH on patients with even marginal elevations of serum calcium.  If the PTH is not maximally supressed then you are dealing with hyperparathyroidism.  Undetectable PTH in the setting of hypercalcemia has a more broad differential.  In these cases a search for more rare causes of hypercalcemia can be considered. 

Other pearls from this AM:

Elevated alk phos with normal calcium, phosphate, and LFTs suggests Pagets

Hypercalcemia can cause a short QT interval primarily through shortening of the ST segment.  This can produce findings worrisome for early repolarization vs ST elevation and apparently can mimic MI.

7 June - Anticoagulation in the Elderly

In which patients can warfarin be safely resumed after a major GI hemorrhage?  Multiple factors must be considered:  what is the risk of not anticoagulating?  Prosthetic mitral valves may not be forgiving, but a minor DVT several months ago may not pose much risk.  How many CHADS2 points does the A-fib patient have?  How significant was the bleed?  Did it occur at therapeutic INR levels or only at significantly elevated ones?  No simple algorithm for this, but these guidelines from the ACC/AHA give some pointers.

At least with regards to elderly patients with A-fib, physicians tend to overestimate the risk and clinical impact of gastrointestinal hemorrhage or hemorrhagic stroke and underestimate the benefit of anticoagulant therapy. Annals of Pharmacotherapy on Medscape reviews warfarin in the elderly in detail.

Here is a nice review of the literature that largely focuses on antiplatelet therapy in patients with a history of GI hemorrhage.  Key points:  Clopidogrel has lower bleeding risk than ASA, but not by much.  ASA plus PPI has significantly lower bleeding risk than clopidogrel.  Clopidogrel is not as active when given with omeprazole, but other PPIs do not seem to exhibit this effect.

2 Jun MR

Today's case was a patient that was admitted with orbital cellulitis.  He had been treated for an eye infection - "pink eye" per his report, with two oral abx for 2 weeks without response.  Significant proptosis was present on the left.  He was an ESRD patient on dialysis with poorly controlled diabetes.  CT revealed significant orbiral cellulitis with bony erosions.  On admission he did not appear to have necrosis or eschar in the oral cavity or nares and he was treated with vancomycin, zosyn, and cipro.  At surgery however he was found to have eschar in the sinuses and pathology revealed aseptate hyphae consistent with mucormycosis.

This NEJM case has lots of similar to this feature, and breaks down fungal sinusitis into acute, subacute, and chronic.  This patent likely fit into the subacute category.

Pearls: 
Bad DKA with rapidly progressive rhinocerebral infection.  Look for eschar and necrosis.

To differentiate orbital cellulitis from preseptal cellulitis you must evaluate for proptosis.  Physical exam is helpful, measurement with a proptometer is better, and CT imaging with orbit protocol is best. 

Mucormycosis is a group of fungal infections (Mucor, Rhizopus, Absidia, others) that produce rapidly developing infections in immunosuppressed hosts (marrow transplant, AML, DKA, high dose steroids).  The only effective antifungal options for thse infections are amphotericin B and posaconazole.

Rapid consultation of ophtho and ENT/facial plastics is key to preserving vision in orbital cellulitis.  Blindness may occur abruptly and is typically not reversible.  Orbital decompression will allow time for antibiotic therapy to be effective in cases of bacterial infection, but invasive fungal disease usualy requires significant debridement.

MR 24 - 28 May

Teaching rounds with Dr Panella covered a case of head and neck cancer with an unusual neuroendocrine tumor located in the tonsillar fossa.  Some key points of evaluating a patient with a neck mass were discussed.  If the mass is tender and infection is a possibility, a two week trial of antibiotics is a reasonable first step.  If the mass persists beyond that point, invasive testing with FNA is appropriate as well as consideration of CT imaging.  This AAFP article has a nice review of the topic.

Another case discussed this week was a patient that was admitted with progressive pain and malaise and was found to have a markedly elevated WBC at her oupatient visit (>80K).  Her peripheral smear supported a diagnosis of CLL but this alone did not completely explain her multifocal pain.  A bone scan was requested that revealed multifocal osteoblastic disease.  Bone marrow aspirate revealed metastatic adenocarcinoma, indicating the simultaneous presentation of 2 malignancies.

Pearls:
CLL is the most common type of leukemia in adults.  Many patients are diagnosed incidentally when a CBC is checked for other reasons.  Other cases are identified during evaluation of patients for unexplained lymphadenopathy.  Very few cases are symptomatic, and when symptoms occur it usually consists of fevers, chills, and night sweats (B symptoms) and not pain.  This disease is usually managed with watchful waiting, but new advances in prognostic markers may push some patients towards earlier treatment if they have high risk features.  See this NEJM review for more details.  Alemtuzumab is a monoclonal antibody therapy (anti CD52) approved for therapy of CLL.

CLL patients seem to be at risk of developing other malignancies, particularly other leukemias, lymphomas, melanomas and sarcomas.  The fact that CLL is often asymptomatic makes it more likely that the CLL will not be discovered until workup for the other malignancy, making them appear to be "simultaneous", though the CLL may have been present for quite some time.

Good pictures from American Society of Hematology Atlas of CLL shown here.

MR 19 - 21 May

Pseudoseizures:  Difficult and challenging condition for patients and physicians alike.  Suspect this in patients with epilepsy that is refractory to antiepileptic drugs or in patients with significant emotional distress or personality disorders.  Do not forget that up to 1/3 of patients that have pseudoseizures will also have true epilepsy, making management particularly difficult in these folks.  Cornerstone of diagnosis is video ECG monitoring.

MR 17 May 2010

Impromptu teaching rounds for me this AM.  Complex case involving a hispanic female with a pain flare in multiple joints, recent prolonged travel in Mexico complicated by diarrhea, and a history of unexplained iron deficiency anemia.  Important turning point in this case was measurement of ESR of >115.  XRAY of spine and pelvis revealed fused SI joints and enthesopathic changes of the vertebra consistent with ankylosing spondylitis vs enteropathic arthropathy.

Seronegative spondyloarthropathy:  constellation of diseases that include ankylosing spondylitis, reactive arthritis (Reiter's), psoriatic arthritis, enteropathic arthritis, and undifferentiated spondys.  These diseases are often associated with HLA-B27.  Enteropathic arthritis can be associated with a variety of infections to include traveler's diarrhea and brucellosis.  IBD associated spondy can preceed onset of bowel symptoms by months / years.  Treatment of these disease ranges from anti-inflammatories, corticosteroids, to TNF alph inhibitors. 

MR Potpourri Through May 14

No MR blog updates in recent weeks due to ACP and vacation.  Had a few rare diseases recently - here are some recent pearls:

Neurofibromatosis:  Autosomal dominant disease, but half of cases arise from spontaneous mutations.  NF 1 has prominent cutaneous manifestations (fibromas, axillary freckling, cafe-au-lait spots).  You can observe lisch nodules in the iris of affected individuals.  NF2 has predominant central manifestations and less cutaneous disease.  Think this dx in a patient with bilateral acoustic neuromas.  Differentiate from tuberous sclerosis, another AD disease.  Predominant facial lesions known as adenoma sebaceum, central nervous system tumors, renal tumors (angiomyolipomas), developmental delay, and seizures are prominent findings.  Some cutaneous lesions likely to show up in challenge bowl include periungual fibromas and ash leaf macules.

Marfan Syndrome:  multisystem disorder with prominent aortic pathology (aortic regurgitation).  Primary defect is mutation in the fibrillin gene.  Patients are usually tall with unusually long limbs and fingers for height.  This disorder is a cause of sudden death in young athletes.  Differentiate from Ehlers-Danlos syndrome - multiple genetic defects with Type V and III collagen being the most prominent.  Skin and tendon laxity are prominent in this condition.  Mitral valve prolapse and regurgitation is the most common cardiac manifestation.  Osteogenesis imperfecta is caused by Type I collagen deficiency, with hallmark findings of fragile bones and blue sclerae.  (Aside - the villain played by Samuel L Jackson in Unbreakable was an OI sufferer) 

HIV Infection:  Covered recently in a couple morning reports, but one relatively new development in the field:  The new treatment initiation threshold is recommend at CD4 counts less than 500, and the guidelines indicate that treatment can be considered at any CD4 count on a case by case basis with highly motivated patients.  The guidelines panel was split 50/50 regarding making this latter threshold a formal recommendation.

Narrative Medicine:  Dr Lands' Grand Rounds on narrative medicine was one to remember.  The stories we tell tie us together as a profession and give practice deeper meaning than simple facts, stats, and p values.  A poem in the sudden death article linked above brings that home, and I close this entry with it:

The time you won your town the race / We chaired you through the market-place;

Man and boy stood cheering by, / And home we brought you shoulder-high.
To-day, the road all runners come, / Shoulder-high we bring you home,
And set you at your threshold down, / Townsman of a stiller town.
— A.E. Housman, "To an Athlete Dying Young" (1895)

MR Wrapup April 12 - 16

Friday: Hypoglycemia and Hypothermia:
54 y/o F with long history of diabetes and renal failure with three previous failed kidney transplants was admitted with temperatures of 87 degrees and a field glucose measurement of 30.  She rapidly improved with passive rewarming and dextrose.  Possible causes included excessive beta blocker dosage as well as excessive insulin, though her low stated dose of 6 units BID make the latter unlikely.  The workup appropriately included evaluation of possible adrenal insufficiency due to her previous history of transplant related corticosteroid use.

Teaching pearls:
Osborn waves and hypothermia:  This particular patients ECG revealed only sinus bradycardia and QT prolongation at 600msec, consistent with hypothermia, but Osborn waves were not manifest on the initial ECG.  This NEJM Case Record has an excellent review of both hypothermia and frostbite management. 

Thursday: Cardiac Device Infection:
A 52 y/o F presented with a constellation of vague complaints to include hands and fingers turning blue associated with some chills.  These symptoms had been present for >2 years off and on.  She also had hematuria of at least 2 years duration without clear cause.  A pulmonary embolism had been documented in the previous year, but no venous thrombus was found in the lower extremities.  She has a history of congenital heart block and had a pacemaker reimplantation approximately 2 years ago prior to the onset of the current symptoms.  On this particular admission blood cultures and an echocardiogram were obtained.  Cultures were positive for coagulase negative staphylococcus in multiple specimens and echocardiography revealed presence of a vegation in the right ventricle.  She improved clinically with intravenous antibiotics and is scheduled for elective surgery to remove the infected pacemaker leads and reimplant a device with epicardial leads.

Teaching Points:
Evaluation of hematuria:  Hematuria is always abnormal in men, and is abnormal in women when not menstruating.  Presence of hematuria during menstrual bleeding should be confirmed with follow up testing after the cycle has ended.  Hematuria is an early warning of many occult, potentially fatal diseases such as carcinoma (bladder or kidney), endocarditis, or glomerulonephritis.  Examination of urine sediment, CT imaging of the urinary tract, and referral for urologic evaluation are all appropriate steps to take.  To quote from a NEJM review of microscopic hematuria: "The single most important test in the evaluation of hematuria is the microscopical analysis of urine, because it often distinguishes glomerular from nonglomerular bleeding."  It appears that this cheap and easily performed test was not performed on several previous episodes of care of this patient. 

Treatment of cardiac device infections:  Huge topic.  Usually involves removal of device in conjunction with a course of antibiotic therapy similar to treatment for endocarditis.  A recent update by former UT ID doc Larry Baddour was published this year in Circulation.  Nice reference if  you ever have to deal with these infections.

Wednesday:  Carbon monoxide poisoning - an elderly female with dementia left her car running in her garage and fell asleep at home.  When she awoke she complained of "smothering".  CO levels were approximately 30% on initial evaluation, and improved promptly with oxygen therapy.  This Clinical Practice article from NEJM is a must read, and covers topics of acute and chronic CO exposure.  Remember that CO induces toxicity both from hypoxic mechanisms but also inflammatory mechanisms, and the latter effect accounts for much of the late toxicity.

MR wrapup for Apr 5 - 9

Diogenes Syndrome Case:

An elderly female admitted with progressive ulcerations over her body.  House was in disarray, she was covered in feces, and had experienced a similar episode one year prior.  She likely has Diogenes Syndrome:  Adult self neglect syndrome, also known as messy house syndrome.  She refused all attempts at placement for skilled nursing care, and denies that any help is needed.  This is a difficult condition to address and there does not appear to be any pharmacologic or behavioral therapy of any great effect.

Sick Sinus Syndrome / Tachy Brady:

A patient with syncope had some episodes of flutter/RVR alternating with sinus pause / sinus bradycardia.  His bradyarrhythmias were only occasionally present on telemetry.  This syndrome is common in older adults with other risk factors for CAD.  Sick sinus syndrome and tachy brady are felt by some to be variants of the same disorder, but in pure sick sinus syndrome the tachycardia is not often seen. 

Other interesting features of this case was the presence of sotalol toxicity.  In this case the patient was a hemodialysis patient and was taking sotalol.  His admit ECG was bizarre with alternating wide and narrow complexes and a prolonged QT > 600msec.  Sotalol toxicity often produces torsades, but this was not seen in this patient and his QT interval normalized with dialysis and discontinuation of sotalol.

Toxicology Website (has nomograms)

http://www.ncemi.org/

Documentation for DC Dictations when Someone Dies/ Doing Death Certificates

A discharge summary done when someone passes should be done with the same criteria as any discharge summary - within 48 hours or less (stat), diagnosis list including primary and secondary causes of death, list of significant procedures, history/physical exam, hospital course with major highlights. Documentation of code discussions, family discussions, and mode of passing/code/events should be included. Instead of "discharge planning" a discussion of counseling done and ancillary staff (chaplaincy called) should also be included.

By TN law, a licensed physician should be completing the death certificate within 48 hours but the TN Office of Vital Records acknowledges the difficulty of this. Death certificates which are signed later than 4 days are reviewedby the state. Therefore, it is important for residents and faculty to discuss the appropriate listing of the cause of death and that it is consistent between the death summary and death certificate. Cardiopulmonary arrest, for instance, is a mode of death but not the cause. Myocardial infarction is a cause of death. This can be due to 1 or more factors (coronary artery disease, for example). The death certificate asks for approximate amount of time of the diagnosis prior to death (days, weeks, months, years can be listed generally). The death certificate also asks for contributing diagnosis or significant diagnosis that may not have been direct cause of death (diabetes, prostate cancer, etc). It would be beneficial to include this level of detail in the dictation.

An online module providing education about death certificates and deciding on the cause of death is available through UT Memphis for free: http://www.uthsc.edu/cme/TN_death_certificate/index.php

11 March - CML presenting as priapism

Forgot to include this one last week.  The case was a young male who presented to hospital with priapism.  He had significant leukocytosis on evaluation and was ultimately determined to have CML.  This is an uncommon but not rare clinical presentation in adults with leukemia.  CML treatment has been revolutionized by the introduction of imatinib, a specific inhibitor tyrosine kinase.

Review this disease for boards - everything from the blood smear to the BCR-ABL tyrosine kinase to the Philadelphia chromosome.

For a good reference on blood smears that you can use in teaching, see the American Society of Hematology Image Bank.  Easily searchable database of pretty much any blood smear you could ever want.

For student teaching, they also have these interactive teaching cases designed for medical students.

12 March - New Onset CHF

Today's case was an interesting presentation of a 51 year old Korean male presenting with new onset congestive heart failure.  In this case, dialted cardiomyopathy was diagnosed and untreated hypertension was felt to be the most likely cause.  The significant elevation in liver enzymes in this case was striking, somewhat above what would be expected with congestive hepatopathy.  This Cleveland Clinic Journal review article has a nice summary of the differential and workup of this disease. 

An interesting historical footnote is an outbreak of cardiomyopathy in Canada and the US Midwest associated with cobalt containing beer.  Cobalt had been added to certain brands of beer as a foam stabilizer and was felt to be non-toxic.  Apparently they did not consider the "volume" consumed by certain Canucks and Midwesterners, especially during hockey season...  This was favorite challenge bowl fodder where I trained.

As for heart failure management in general, the ACP In the Clinic series has an excellent review of the topic linked here.  The In The Clinic site also have slide sets of their topics that you can use for teaching interns / students / etc.  Make use of it!

10 March - Obstructive Jaundice

This morning's case was a brief discussion of a patient that presented with painless jaundice.  In this case the workup eventually led to ERCP with with biopsy specimen positive for adenocarcinoma at the ampulla.  Importantly, a recent CT scan did not reveal a mass in this case, which is often the case with cholangiocardinoma.  MRCP/ERCP is essential to diagnose these lesions.  The first step in the differential is to determine whether the bilirubin is primarily unconjugated or conjugated.  Unconjugated suggests diseases such as Gilbert's or hemolysis, whereas conjugated suggests hepatocyte dysfunction or obstruction. 

Read this NEJM case discussion for a nice breakdown of how to evaluate painless jaundice in a systematic way.  Had this one in my file from when I was an R2.... If you want to learn diagnostic medicine and refine your illness scripts and differential diagnoses you should be reading the Case Records of the Massachusetts General Hospital every week!

8 March - Abnormal CXR Teaching Rounds

Densities in the lung fields seen on CXR can be lumped into 4 broad categories - mnemonic AVID: Alveolar, Vascular, Interstitial, and Damage.  Vascular markings are self explanatory.  Damage = anything that causes cavities / abscesses and has its own differential (Clinical Microbiology Reviews, April 2008).  Alveolar infiltrates can be pus, fluid, protein, or cells.  One CXR to remember the appearance of is bronchoalveolar cell carcinoma.  It can present as pulmonary infiltrates that mimic pneumonia.  As for the alphabet soup of ILD - a nice review can be found here. (MD Consult login or on campus access required).

One thing that is new on my radar - eosinophilic pneumonia and chronic pneumonitis induced by daptomycin therapy.  This was reported in the 1 March 2010 issue of Clinical Infectious Diseases and cases have been previously reported elsewhere, but this is the first time I have noticed it.

3 Mar - Febrile Illness in Dialysis Patient

Always respect febrile illnesses in dialysis patients and poorly controlled diabetics.  Signs of significant pathology are often muted in these populations and clinical suspicion is key (isn't it always...)

One study showed hemodialysis access and chills to be strong predictors of S aureus bacteremia, a finding that has been described by other clinicians. 

In this case the gallbladder was felt to be the likely source.  Diabetic patients are at risk for empysematous cholecystitis, a rapidly progressive and often fatal infection.  Clostridium perfringens accounts for a significant number of these infections.  Surgical or percutaneous drainage is essential.

Regarding the atriall fibrillation portion of the case - patients with Afib/RVR that are in shock or are experiencing ischemic chest pain should be promptly cardioverted (synchronized shock, 100J).  If still awake enough to feel it, see if anesthesia can get there quickly and give some sort of conscious sedation beforehand.  If the BP is marginal and they are not in shock, try digoxin for rate control.  0.5mg IV bolus often has prompt effects on the AV node and can slow the rate within minutes.  As you do not see digoxin used much these days, it would be useful to review this article covering the contemporary use of digoxin.

2 March - Cards board review

Posted verbatim - Dr Yarbrough's clarification of murmur maneuvers.  Anyone still confused??

Reminders based on today’s board review:


In general: rIght sided murmurs are louder during Inspiration
lEft sided murmurs are louder during Expiration
a – wave: atrial pressure during atrial contraction
c – wave: increased pressure from tricuspid valve bulge into the RV during isovolumetric ventricular contraction
x –descent: results from downward movement of ventricle during ventricular contraction and atrial relaxation
v – wave: atrial filling
y – descent: decreased pressure from opening of the TV

we usually don’t worry about h and z or a more simplistic view

Standing and Valsalva decrease right and left cardiac filling and lead to decrease murmur intensity except for MVP and HCM

Squatting and Supine position increase ventricular filling and lead to increase murmur intensity except for MVP and HCM

In short: MVP and HCM are increased by standing and valsalva and decreased by squatting and supine position

To differentiate between AS, MVP and HCM use sustained hand grip: this will increase MVP; decrease HCM and AS

1 March - Quiz Bowl Follow Up

No MR today or Friday, but I did want to post a link to an article mentioned in quiz bowl Friday.  This one looked at an EMR that flagged orders that deviated from guidelines, and allowed the ordering doc to indicate whether the deviation was appropriate or not.  All in all, the deviations were correct  94% of the time.  Something to consider as the hospital implements expert system support once order entry goes online.

25 Feb - Fever and Rash

     Fever and rash in an otherwise healthy male patient with pneumonia.  When working up fever and rash, "Its probably a virus" will be correct much of the time, but there are some never miss life threatening diagnoses to remember.  Unfortunately, the differential diagnosis of fever and rash is only slightly less broad than that of fever alone.  Of all the rashes, petechial / purpuric rash with fever is the most ominous.  Think meningococcemia, RMSF, endocarditis, and fulminant gram negative sepsis.  Ask about the spleen!  I think I will make this article from ID Clinics of North America (Vol 10:1, 1996) required reading for the ID rotation.  It gives a few ways to lump the diagnoses by appearance and determine the treatable, untreatable, and non-infectious possibilities.
     In this patient, the prominent maculopapular rash sparing the palms and soles in conjunction with pneumonia suggest mycoplasma as a diagnosis.  Though generally benign, mycoplasma infections can be severe and even fatal, especially if encephalitis, erythema multiforme, aplastic anemia, or DIC occurs.  This article by Schalock in International Journal of Dermatology 2009,48, 673–681 is a great review of mycoplasma infections covering all the systemic manifestations and focusing on the dermatologic findings.  Up to 1/3 of mycoplasma cases are complicated by an exanthematous eruption according to their review. 
     Finally, when working up a patient with suspected acute retroviral syndrome (fever, rash, sore throat, adenopathy), the plasma viral load is the test of choice.  p24 antigen testing used to be the correct answer, but viral load testing is now preferred.  Low titer viral loads (1000 or less) probably represent false positive tests in this setting.  You can expect a Step 3 / ABIM question on this topic.

24 Feb - Syncope, MI, and LBBB

     Todays case was a patient with a significant underlying seizure history (poorly controlled) who now presents with several episodes of loss of consciousness not associated with convulsions.  Echocardiography revealed a rare congenital malformation - cor triatriatum.  This was likely unrelated to the syncopal event.  It is condition where atrium is divided into two chambers by a septum, which can produce symptoms the functional equivalent of mitral stenosis.
     A brief case discussion focused on patients with chest pain and left bundle branch block.  Symptoms suggestive of cardiac ischemia with a new or unknown duration LBBB should be managed as ST elevation MIs.  In patients with old LBBB and chest pain, some features suggest the presence of MI:  1mm concordant ST elevation, 5mm discordant ST elevation, or 1mm ST depression in leads V1, V2, or V3.  This data came from a substudy of the landmark GUSTO trial that established the usefulness of TPA for treatment of acute MI.  You can expect a question about MI in setting of LBBB on boards.

23 Feb - In the News

High profile stories about aspirin and rosiglitazone sure to stimulate conversations with your patients.  Be sure to calculate a Framingham risk score to help counsel patients about the risk / benefit of daily aspirin.   Based on the above story, those with higher Framingham risk scores may not be good candidates for rosiglitazone until the heart disease risk issue is resolved.

19 Feb - Progressive Multifocal Leukoencephalopathy

     An elderly male with a long history of HIV disease presents with a rapidly progressing dementing illness associated with visaul loss.  His MRI revealed extensive disease predominantly in the white matter but also involving gray matter.  The differential of CNS lesions in AIDS patients is broad, but most cases are attributable to lymphoma, toxoplasmosis, PML, cryptococcus, and rarely (in the US) TB.  In this case CSF was remarkable for high protein and 2 WBCs.  This is a typical bland CSF appearance for PML.  PCR for JC virus was positive.
     PML is a rapidly progressive demyelinating disease caused by the JC polyoma virus that is usually fatal.  Most cases are associated with AIDS, however cases have been reported in patients recieving natalizumab for treatment of MS.  There is no treatment (other than retroviral drugs to treat HIV disease).
     The other major human disease caused a polyomavirus is the BK virus induced nephropathy in kidney transplant patients. 
     Remember the most up to date management strategy for differentiating toxoplasmosis from lymphoma is to perform Thallium 201 SPECT with early brain biopsy for lesions suspicious for lymphoma.  The old strategy of treat for toxo for 2 weeks and biopsy those that do not improve is not as favored given the peformance of SPECT.

18 Feb - glands run amok

     Todays discussion of a patient admitted with atrial fibrillation with RVR brought up several important points.  Will not dwell on A-fib here as it was discussed extensively last week.  One is proper dosing and adjustment of thryoid hormone supplements.  Start low and go slow, especially in the elderly and patients with cardiac disease.  Also remind patients to take their mediciation in the AM on an empty stomach.  Erratic dosing produces erratic results. 
     Interpreting results of parathyroid hormone testing can be a challenge.  Values in the "normal" range represent hyperparathyroidism if the calcium is elevated.  Don't forget to evaluate for vitamin D deficiency as a cause of secondary hyperparathyroidism, but in those cases serum calcium should be low, not high.  This July 11, 1999 Lancet review covers all you every wanted to know about hyperparathyroidism. 
     Pearl - hypothyroid patients often have elevated lipids, and thyroid hormone supplementation reduces LDL cholesterol but has no effect on HDL, triglycerides, apo AI or Lp(a).  Expect to see something about this on boards.  See here and here for more info.

17 Feb - cryptococcal meningitis in an immunocompetent patient

     Todays case was a young otherwise healthy male with chronic meningitis.  Cerebrospinal fluid analysis revealed abundant mononuclear cells, high protein, and low glucose.  In the developing world this strongly suggests TB.  In the US, most likely it is cryptococcus or histoplasmosis.  There are several other causes of the chronic meningitis syndrome, nicely summarized in this Nov 1999 Neurological Clinics article available here at MD Consult.  Though often associated with HIV/AIDS, cryptococcal disease occurs in immunocompetent patients.  There is an emerging outbreak of a novel pathogen, Cryptococcus gattii, in the Pacific Northwest, especially Vancouver Island - so watch for it in travelers returning from the Olympics.  This has caused significant morbidity and mortality in both immunosuppressed and immunocompetent patients.  Recently updated treatment guidelines for cryptococcal disease can be found here.  Traditional teaching is that all patients with cryptococcus isolated from sputum require lumbar puncture to exclude cryptococcal meningitis.  The recent guidelines indicate that select immunocompetent patients with negative serum cryptococcal antigen titers and no signs of meningitis can be managed without lumbar puncture.  All immunosuppressed patients require LP, whether they have meningitis symptoms or not.
     On an related note, this recent JAMA article on performance and interpretation of lumbar puncture lists the "normal" opening pressure as 6 - 14 mm Hg or 6 - 18 cm H2O. 
 

16 Feb - CPC on Pulmonary Embolism

     Code in the middle of the night, rhtyhm other than v-tach (especially PEA) think PE.  Frequently overlooked, often preventable, PE remains a leading killer of hospitalized patients.  It is missed much less often today than in the past, some speculate due to widely available CT angiography and increased clinical awareness of this condition.  Here is a recent Mayo Clinic study that found PE to an uncommon cause of Type I autopsy findings (Type I = diagnosis missed pre-mortem that would have altered outcome if treated).
     The role of using thrombolytics to treat suspected PE in the setting of a code is controversial.  The British Thoracic Society recommends its use for massive PE with hemodynamic collapse.  In these cases your diagnosis has to be clinical.  Patients stable enough to go to the scanner are too stable to use thrombolytics.  The dose, if you so choose, is 50mg alteplase given as a bolus.  In a small randomized trial of heparin vs lytics for massive PE, all 4 patients given heparin died and all given lytics lived.  This is the extent of the "high quality" data for this condition.  The way I see it, the outcome of PEA code is so bad that if PE is high on the differential push the lytics, because you might help and you can't really make things much worse at that point.
    For further reading on management and prevention of thrombosis in hospitalized patients, review the Venous Thromboembolism Prophylasis module on the Johns Hopkins hospitalist curriculum.  Requires login, but it is free.  Also linked on the links section of this blog.