To the Housestaff, Faculty, Fellows, and Students,
As our year with you ends, we want to take a moment to thank you. Thank you for your dedication, your professionalism, your curiosity, and your commitment to the best quality patient care. Thank you for being expert clinicians, accomplished researchers, and outspoken advocates for your patients. Thank you for helping open a brand new hospital, for being the best recruiters in the world, and for rising to the challenge of potpourri. Thank you for supporting us, for standing by our side, for voting on the case challenges, and for reading this blog! In short, thank you, for an amazing year – we know that next year will be even better!
– Your 2014-2015 Chief Residents (Punag, John, and Kate)
Case challenge #19 presented a 57 year old with HCV and years of recurrent epistaxis. He also noted generalized weakness, DOE, and chest pressure with exertion. His father also had recurrent nosebleeds. Exam reveals tachycardia and a flow murmur. Endoscopy reveals the following:
What is the most likely diagnosis?
You’re right, its Rendu-Osler-Weber Syndrome, also known as Hereditary Hemorrhagic Telangiectasia!
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a disorder of development of the vasculature characterized by telangiectases and arteriovenous malformations in specific locations. It is one of most common monogenic disorders, but affected individuals are frequently not diagnosed. The most common features of the disorder, nosebleeds, and telangiectases on the lips, hands, and oral mucosa are often quite subtle. Optimal management requires an understanding of the specific presentations of these vascular malformations, especially their locations and timing during life. Telangiectases in the nasal and gastrointestinal mucosa and brain arteriovenous malformations generally present with hemorrhage. However, complications of arteriovenous malformations in the lungs and liver are generally the consequence of blood shunting through these abnormal blood vessels, which lack a capillary bed and thus result in a direct artery-to-vein connection. Mutations in at least five genes are thought to result in hereditary hemorrhagic telangiectasia, but mutations in two genes (ENG and ACVRL1/ALK1) cause approximately 85% of cases. The frequency of arteriovenous malformations in particular organs and the occurrence of certain rare symptoms are dependent on the gene involved. Molecular genetic testing is used to establish the genetic subtype of hereditary hemorrhagic telangiectasia in a clinically affected individual and family, and for early diagnosis to allow for appropriate screening and preventive treatment.
Although hemorrhage is usually the presenting symptom of mucosal telangiectases and cerebral AVMs, most visceral AVMs present as a consequence of blood shunting through the abnormal vessel and bypassing the capillary bed. Shunting of air, thrombi, and bacteria through PAVMs, thus bypassing the filtering capabilities of the lungs, may cause transient ischemic attacks, embolic stroke, and cerebral and other abscesses. Migraine headache, polycythemia, and hypoxemia with cyanosis and clubbing of the nails are other complications of PAVMs Hepatic AVMs can present as high-output heart failure, portal hypertension, or biliary disease.
Optimal medical management for HHT requires distinguishing between organ locations where telangiectases and AVMs are best managed symptomatically/expectantly, versus those in which lesions should be detected and treated before the onset of symptoms. International Clinical Management Guidelines for HHT were published as a result of a consensus conference.In general, telangiectases of the skin, oral and GI mucosa, and liver are treated when symptoms dictate, but AVMs of the lungs and brain are treated in patients without symptoms given their often sudden and catastrophic presentation. The HHT Foundation International (www.hht.org) lists HHT Centers in the United States and elsewhere, as well as information regarding current management for both patients and clinicians.
You are young and healthy, meaning that you must be “low-risk,” right? But what does this mean in terms of long-term survival?
In a 2012 study in the Journal of the American Heart Association, a group of authors, including our own Jarret Barry, sought to answer this question. They sought to establish whether cardiorespiratory fitness had important implications for long-term cardiovascular risk among individuals classified as low risk by the Framingham Risk Score (10-year coronary heart disease risk <10%).
The study population was composed of men and women, 30 to 50 years of age in our own city of Dallas, Texas. Eligible individuals were defined as being at low risk for coronary heart disease by Framingham Risk Score at the time of study entry and had no history of diabetes (n=11 190). Cardiorespiratory fitness was determined by maximum graded exercise treadmill tests. Over an average 27±2-year period, 15% of low-fit (quintile 1) compared to 6% of high-fit (quintile 5) individuals died (P<0.001).
The study noted that a 1–metabolic equivalent level increase in baseline fitness was associated with an 11% reduction in all-cause deaths and an 18% reduction in deaths due to cardiovascular disease (CVD) after adjustment for age, sex, body mass index, systolic blood pressure, total cholesterol, blood glucose levels, smoking, and early family history of coronary disease. There was an incremental decrease in CVD risk with increasing fitness quintile, such that the high fit had the lowest adjusted 30-year CVD mortality rate compared to the low fit.
Cardiorespiratory fitness is associated with a significant reduction in long-term CVD among individuals identified as low risk by Framingham Risk Score. These data suggest that preventive lifestyle interventions geared to optimize cardiorespiratory fitness, even among a “low-risk” subset, should be considered to improve CVD-free survival.
See you on the Katy Trail!
- Family of heterogeneous enzymes, 100s of different types
- Mostly seen in E. coli, Klebsiella spp. but other GNR may produce
- Causes resistance to PCN, cephalosporins and aztreonam
- Do not inactivate carbapenems
- Do not affect non-beta lactams abx, but co-resistance common
- Cystitis: Fosfomycin, Nitrofurantoin, Bactrim, FQ if sensitive
- Serious infections: Carbapenems preferred
- Rx failures seen with Cefepime (? inoculum effect) but may be able to overcome with higher doses and continuous infusion based on MIC
History of Present Illness
A 57 year old with HCV, GERD, and chronic anemia presents with years of recurrent epistaxis. He has nosebleeds several times per week that last 10 mins and resolve spontaneously. He also notes generalized weakness, DOE, and chest pressure with exertion. In the past, he complained of dark colored stool. He denies using any nasal medications or drugs. He is not on blood thinners, aspirin or NSAIDs. He otherwise denies cough, SOB at rest, hemoptysis.
PMH: HCV , GERD
SHx: occ ETOH, 30 pack year smoking hx
FHx: Father had recurrent nosebleeds due to an underlying illness but he is unaware of the diagnosis
Meds: Ranitidine and prn tylenol
Vitals: 96.7F, 104 bpm, 111/68, RR 18, 100% RA
HEENT: EOMI, PERRLA, pale conjunctiva and mucous membranes, Nasal cavity with dried blood, no active bleeding seen, no nasal polyps
CV: 2/6 systolic murmur without radiation. Regular tachycardia.
Pulm: CTAB, normal effort, no wheezes/rales/rhonchi
GI: Normal bowel sounds, soft, tender to palpation of lower abdomen and mid-epigastric region, no masses
Low levels of physical capability in middle age may signal poorer chances of survival over the next 13 years, according to a cohort study of 1355 men and 1411 women in the United Kingdom.
Nurses assessed grip strength, chair rise speed, and standing balance time of participants at age 53 years.Participants with lower physical capability scores tended to have lower socioeconomic position; less healthy lifestyles; and higher prevalence of self reported cardiovascular disease,diabetes, and severe respiratory symptoms when compared with those with higher scores.
During the follow-up period, there were 177 deaths (88 from cancer, 47 from cardiovascular disease, and 42 from other causes). The fully adjusted hazard ratio of all-cause mortality for participants in the lowest vs highest quintiles of physical capability was 3.68. Those who could not complete any of the 3 tests had death rates more than 12 times higher than those who were able to complete the tests.
Subpar performance on the tests in middle age likely reflects subclinical disease and aging processes rather than manifest diseases, making this population an important one for interventions, said the investigators. What does all of this mean? Time to hit the gym!
- GI or GU infections in patients with prior abx
- Bacteremia, endocarditis in those with extensive HC exposure
- E. faecalis: Often remains sensitive to ampicillin, beta-lactams
- E. faecium: Often multi-drug resistant
- Consider Nitrofurantoin or Fosfomycin
Invasive infections Rx
- Amp-sens VRE faecalis: Amp, Amp/Sulb, Pip/Tazo, Imi/Meropenem active
- Linezolid, High dose Daptomycin (8-12 mg/kg daily), Tigecycline à Consult ID for assistance
You have a fitbit, or an up, or moov, or misfit, or SOMETHING to help track your steps and encourage your to take the fabled 10,000 steps. Companies around the country are giving their employees FREE fitness trackers to promote wellness (and perhaps reduce health insurance claims in the long-term?). But the question remains, does this strategy actually work?! Does counting steps encourage fitness and improve health? Before we tackle the modern fitness trackers, with heart rate monitoring, smartphone apps, frequent notifications, etc., let’s start at the beginning, with the basic pedometer.
In 2007, before the smartphone era, a group out of Stanford asked if pedometers increase physical activity and improve health. In the systematic review of 26 studies with over 2700 participants with a median age of 49, they found that the use of a pedometer increased physical activity by a statistically significant 27%! In the randomized-controlled trials, particiapnts averaged about 2500 additional steps. In observational studies, they increased their steps about 2200 above baseline. Importantly, this increase in steps per day led to a significant decrease in both BMI and systolic blood pressure! The study suggested that simple pedometers have the potential to significantly improve health. To read the full study, click here to visit the Journal of the American Medical Association.
- Must meet all of following: No IE (by TEE); No prostheses; Negative f/u blood cultures at 2-4 days; Defervescence within 72 h of effective therapy; No metastatic infection
- Vancomycin or Daptomycin for minimum 2 weeks
Complicated Bacteremia or Endocarditis
- 4-6 weeks at minimum
- No benefit to adding gentamicin or rifampin for native valve IE
- Generally defined as persistent bacteremia around day 7 of therapy (median time to clearance of MRSA bacteremia is 7-9 days)
- May also define failure as patient getting worse on current tx
- Remember SOURCE CONTROL!!!
- PO options acceptable for SSTI or completion of osteo Rx; IV preferred for invasive disease
- Vancomycin is the empiric drug of choice in most serious infections (duh!)
- Vanc MIC ≥ 2 associated with higher rates of Rx failure so consider alternative agents
- If vancomycin intolerance or failure:
- PNA: Linezolid, Ceftaroline
- Bacteremia/Endocarditis: Daptomycin, Ceftaroline
- CNS: Linezolid
- Osteo: Dapto, Ceftaroline