I will present the case step by step with additional information provided every ~24-72h. Please leave your thoughts in the comments, or can submit to the anonymous link.
CASE: A 59yo with severe ischemic CM (most recent EF <20%) presents to CUH ED with DOE, orthopnea, bendopnea, and PND. He has had 3 hospitalizations in the last 4 months for heart failure exacerbations. His initial lab work is notable for normal K, CO2, AG, and creatinine, CBC is wnl, normal lactate, TSH. He is afebrile, BP 140/75, HR paced at 70, and satting well on RA. he has 2+ pitting edema b/l, JVP 15cm, +HJR, his extremities are moderately cool to touch. Overall he appears chronically ill but stable for the floor.
Assumed Fick: cardiac output 2.8 L/min, index 1.37 L/min/m2
Thermodilution: cardiac output 2.8 L/min, index 1.36 L/min/m2
Day 1 questions- What Stevenson profile is this patient? What are your treatment options and how do his RHC numbers inform your decisions? What would you do with his home medicines? What conditions make thermodilution less accurate? What kind of device does have in place?
If you have further questions of the patient’s history or initial presentation please ask as well
answer below the break, lots of correct answers, chocolate goes to the intern brando(e)ns
Hi all, welcome to a new blog format. The basics- an interesting case is presented once a month on the blog. Basic information will be provided, you can request additional testing and imaging via the survey link provided. Additional testing you request will be updated twice during the week. Answer and explanations will be posted the following week. When you feel comfortable making a diagnosis, provide it with your name in the same survey link (or if feeling confident comment on the blog itself) for a chance at reducing your EROC burden (kidding, but seriously chocolate for correct answers).
Without further ado
85yo white male with a history of prostate cancer (1990s, s/p radiation and chemo), CAD, HTN, HLD, CHF (NICM, previously reduced EF, now in 50s), AVR (5 years ago, bioprosthetic, not on AC) presents with severe back pain.
3 months prior to admission patient seen in OSF ED for new onset, severe left inguinal pain which was much worse with movement and hip flexion, the pain radiated towards the back with movement but not at rest. He described the pain as a muscle pull, he had not had similar pain previously. Workup for hernia was negative, patient unclear on what was performed but believes he received blood products. He was discharged from the ED with gradual resolution of the pain over 3 weeks.
From ED visit until 2 weeks ago he had no significant complaints other than mild fatigue. Starting 2 weeks prior to admission patient presented to a local ED 6 times for minor cuts on hands that would not stop. Bleeding was stopped with dermabond and he was discharged home each time.
2 days PTA he was feeling well and went for “a long walk”. on the morning of admission he awoke with significant pain in his right inguinal area, similar in quality and intensity to previous left inguinal pain. At this point he came into our ED for evaluation.
ROS notable for bleeding as described, easy bruising starting 4 months ago, mild fatigue, moderate nausea with pain, no NS/chills/v/d.
Past Medical Hx:
Prostate CA- diagnosed in mid 1990s, s/p surgery radiaton and chemotherapy.
CAD- s/p CABG 10 years ago
CHF- apparently had reduced EF in past, now normalized. Per patient EF “in 50s” recently, AICD in place
Aortic valve replacement- 5 years ago, bioprosthetic, no AC, followed closely
Do not know if he has had c-scope
Past Surgical Hx:
CABG- no issues with post-op bleeding
Hypertension mom, DM2 dad
Lives in abilene w/ wife, no bad habits
Lasix 20mg BID
Metformin 500mg BID
Metop XL 25mg qday
Diazepam 5mg prn
lyrica 75mg qhs
On arrival he was found to be tachycardic to the 120s, BP 95/65, RR 20, SaO2 98% on ambient air with T of 36.8 C. He appeared uncomfortable though not in significant distress. His HEENT exam was normal including no gingival/mucosal findings, he was tachycardic but regular w/o m/r/g. Lung exam was normal. Abdominal exam was notable for tenderness in the lower right quadrant that was referring to groin. GU revealed no hernia, no swelling, no erythema, no TTP. Extremities notable for 10cm resolving ecchymoses on RUE. Flexion of right hip caused significant pain in groin area. Palpation of lower right back caused moderate pain in right groin. No dermatologic changes were found other than ecchymoses previously descried and healing wounds on b/l hands which had previously been dermabonded. No petechiae seen.
BMP 145/3.4/106/25 (gap 16)/ 16/.88
T bili 3.5 (no fractionation in ED). AST 25 ALT 45 alk phos 100
D dimer 6
Retic ct 8.2
mixing study: PTT 85 pre, 65 after mixing (did not correct)
Factor VIII activity: <1
Factor VIII inhibitor: 58 (high titer identified)
Factor IX activity: normal
Factor X activity: normal
vWF activity: normal
ANA 1:160 homogenous
SSA/ SSB negative
CT head no abnormalities
CT abd/pelvis with contrast: There is mild rotatory scoliosis of the lumbar spine, associated with multilevel degenerative changes of the endplates and facet joints. No worrisome lytic or blastic skeletal lesions are seen. Subcutaneous edema is present within the right flank.
The right psoas muscle is enlarged with ill-defined intramuscular hemorrhage. As the right psoas muscle measures approximately 6.2 x 7.2 cm in axial cross-section on axial image 38, and comparison to the left psoas muscle, which measures 2.4 x 4.3 cm at the same level.
Last week we presented a case of a 50 year-old woman presenting with left-sided facial pain, and a fixed and dilated left pupil. A contrast-enhanced MRI was provided and we asked you all for the next most appropriate step in management. Here’s how you answered:
HPI: A 50 year old woman presents to the ER complaining of severe left-sided facial pain and swelling. The symptoms began 2 weeks prior with URI symptoms (fevers, runny nose, sinus congestion) and she was seen at another hospital and given augmentin, levofloxacin and dexamethasone with minimal improvement in symptoms. The pain and swelling have progressed in intensity and now she reports periorbital pain and blurry vision.
A 27-year-old woman presents with sudden onset agitation and delirium. In the emergency department, she has incoherent speech and lashes out violently at staff. She is initially febrile (38.7ºC). She is admitted to the ICU for observation and after two days is intubated for deteriorating mental status. Prior to intubation, she is noted to have labile blood pressure alternating between hypertension and hypotension.
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.
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
Case challenge #18 presented a68 year old Asian female with HIV admitted for fever and SOB. Three days prior to admission, she developed watery diarrhea, approximately 5-6 BM a day. The work-up revealed 40% Eos and stool O&P with larval forms.
The correct answer is: Strongyloides Stercoralis!
Strongyloidiasis is a chronic parasitic infection of humans caused by Strongyloides stercoralis.
Transmission occurs mainly in tropical and subtropical regions but also in countries with temperate climates.
An estimated 30–100 million people are infected worldwide
Infection is acquired through direct contact with contaminated soil during agricultural, domestic and recreational activities.
Like other soil-transmitted helminthiases, the risk of infection is associated with hygiene, making children especially vulnerable.
Strongyloidiasis is frequently underdiagnosed because many cases are asymptomatic
Strongyloidiasis may cause intermittent symptoms that mostly affect the intestine (abdominal pain and intermittent or persistent diarrhea), the lungs (cough, wheezing, chronic bronchitis) or skin (pruritus, urticaria).
Infection may be severe and even life-threatening in cases of immunodeficiency.
Without appropriate therapy, the infection does not resolve and may persist for life.
Most diagnostic methods lack sensitivity.
Locating juvenile larvae, either rhabditiform or filariform, in recent stool samples will confirm the presence of this parasite.
Ascaris, Necator, and Schistosoma will have eggs in the fecal smear, not larvae
Trichinella will have larvae in the muscle
Other techniques used include direct fecal smears, serodiagnosis through ELISA, and duodenal fumigation.
Ivermectin is the drug of choice, but is not available in all endemic countries.
Albendazole is also an option, but is considered less effective.
For more information, as Nico Barros or Fernando Woll, our resident Strongy scientists!