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Hot Seat Readers,

The Hot Seat will be going on a summer vacation for renovation and upgrading. 

We will be back with more cases, a different layout and URL, and other PEM learning tools. 

Here's what we've done over the last few years:
Pageviews: Over 13,000

40 Hot Seat Cases

26 Different attendings on the Hot Seat

199 total comments

3 Institutions (Children's National, INOVA Fairfax, and Johns Hopkins)

Most frequent participants: Dave Mathison, Paul Mullan, Jennifer Chapman, Dewesh Agrawal, Rasha Sawaya, Sabah Iqbal, Jamil Madati

Future site will include:
Hot Seat
ECG
Best Articles
Article of the Week
Ultrasound
C-MAC
Potential for Author Profiles/Portfolios
Sky's the limit!  What do you want it to include?

The goal is to have a real-time, user-friendly educational tool.

If you have comments or suggestions, please include them below in the comment section.

Thanks for your participation and check back later this summer for our new look.

Please share your opinions by clicking on “comments” below. It’s easiest if you’re also logging into your gmail account but you can do it without as well. Just select “Name/URL” from the drop down menu, write your name, and click “submit.” You can also post anonymously although this seems less fun. To read posted comments, click on “comments” below and scroll up.

About the Hot Seat. Hot Seat cases are written by PEM fellows at Children’s National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series. 

Hot Seat Case #40 Denouement: 3 yo with bloody stools

By Sonny Tat, Children's National
with Shilpa Patel, Children's National

The Case
This is a case of a 3 year-old healthy female with abdominal pain and bloody stools who presents with blood at the anus and anemia.  The challenge in this case is how to approach this patient with an ultrasound negative for intussusception and how to address access issues (a common problem). For a complete case presentation, please click here.
 
Here's How You Answered Our Questions
What is your next diagnostic step in the ED?
Less than 3 years PEM experience (fellows, PAs/NPs, pediatricians) n=9

More than 3 years PEM experience (PEM attendings, pediatricians) n=7

Attendings and fellows agree that the next diagnostic step would be an abdominal x-ray.  Interestingly, 22% of fellows would get an abdominal CT, while none of the attendings thought a CT the next step.  Approximately 14% of attendings thought a barium enema might be beneficial, while none of the fellows chose that imaging modality.  

You have been unable to establish IV access despite multiple attempts.  What is your access plan?

Less than 3 years PEM experience (fellows, PAs/NPs, pediatricians) n=8

More than 3 years PEM experience (PEM attendings, pediatricians) n=7

The majority of providers would continue to attempt a non-EJ IV, followed by an EJ IV.  This patient is sick, but stably so at the moment.  She definitely needs access, but the majority of providers felt they could take the time to get an IV.  Only 12.5% of fellows (and no attendings) felt an IO was necessary.  No one wanted PICU admission for a central line.  At this time, the patient doesn’t truly need ICU care.

Clinical Reasoning 
Dave Mathison and Shilpa Patel (Hot Seat Attending) gave excellent responses to this case. For their complete comments, click here.

“it's super, super important to differentiate painless versus painful rectal bleeding. and make sure it's actually blood.”

“typically, PAINLESS rectal bleeding is either juvenile polyp or a Meckels. period. in this patient who has a drop in Hb with normal indices yet is well appearing....sounds like a Meckel's. and if this were the case, i would admit for a meckel's scan since the patient is minimally tachycardic with a low Hb and question of active bleeding. always good idea to get a PT and PTT to look for other coagulopathies, although this is not a presentation of acute hemophilia. and never a bad idea to put in an EJ, although probably not necessary if BP is normal and kid looks fine.”

“now PAINFUL rectal bleeding… think about infectious colitis versus inflammatory bowel disease versus vasculitis etc. so send the culture, ESR/CRP. If inflammatory markers are negative, this points more towards anatomic (meckels, polyp). If positive this supports one of these inflammatory or infectious conditions… I do like to get an abdominal XR, especially in a 3yo where ingestion of foreign material is possible… rectal obstruction can cause gaseous distention (pain) and bleeding from local trauma. same goes with constipation, although pretty HARD (no pun intended) to get your Hb down so much with constipation/tears.”

-Dave Mathison, Children's National 

From the Hot Seat
by Shilpa Patel, Children's National Medical Center

Although lower GI bleeding in children may be indicative of a serious illness, usually it is not.  Our very own Stephen Teach published a retrospective review of 109 cases (?his fellow’s project – pretty awesome) in which only four had life threatening diagnoses (https://www.dropbox.com/s/b53127f2haoqj43/1-s2.0-S0196064494703507-main.pdf) – three cases of intussusception and a single case of Meckel’s diverticulum.  

The first step in the approach to GI bleed is to determine if it is truly blood.  In our case, given the low hemoglobin and gross blood at the anus on exam, we should assume it is blood.  The second step is to try to distinguish between upper and lower GI bleeding.  Classic teaching is that bright red blood in/on stool is usually lower, however in an infant (short intestinal transit time), bright red blood could be from a massive upper GI bleed and quickly lead to hemodynamic compromise).  Whereas melena, or dark blood is classically more proximal (this could be blood that is swallowed from the nose/ingested). Red stool (though it is dark) with gross blood at the anus in our patient is probably lower GI.

I’m going to use SPI(T) (which Sonny introduced to us a few weeks ago as a teaching technique in the ED) to organize my DDx and will try to limit it to younger children since our patient is 3 years old.

S          Serious

P          Probable

I           Interesting

T         Treatable

DDX:

S: Serious

Intussusception – Though this has already been ruled out (US has a negative predictive value of 99.5% with a good sonographer), our patient's presentation, a 3 yo with intermittent severe abdominal pain sounds concerning.  Blood in stool is a late finding in intussusception. The currant jelly stool is associated with necrotic bowel and I would expect the patient to appear more ill.  Thinking creatively, the bloody stools in our scenario could be related to a lead point for intussusception (a bleeding polyp or diverticulum) causing the bleeding and the intermittent abdominal pain AND the telescoping could be intermittent (giving us a negative ultrasound when the child is comfortable).

Meckel’s diverticulum – usually described as “painless” bleeding (just blood and no stool), though our child reports abdominal pain. It can also be associated with complications (obstruction or perforation) and majority of the patients are younger than 2 years old. As Meckel’s can lead to rapid painless blood loss and hemodynamic compromise, ensuring that the patient is hemodynamically stable is very important. Our patient is anemic and should be watched.  At this point, I don't think I would get a Meckel scan on our patient.

Underlying coagulopathy – normal INR is reassuring along with normal platelets. HUS can present with GI bleed but is unlikely given normal platelets and normal Creatinine.

Volvulus (w/Malro) – would expect the child to be sicker with an abnormal aXR, or have bilious emesis (though she did have emesis x 2 earlier in the day).  If she had bilious emesis, would consider an AXR.

P: Probable

Infectious colitis – Our patient has crampy abdominal pain, it is associated with multiple stools and had vomiting earlier -fits the bill.  We should ask a travel/exposure history, send a second set of cultures and follow up on the stool cultures sent by the primary care doctor.  Would also be good to know if she was on antibiotics  (C. Diff colitis)  Something that goes against this is the lack of fever and perhaps a normal wbc….

Mnemonic for infections that cause bloody stools: YEECCSS!

Y – Yersinia

E – Ecoli

E -Entamoeba histolytica

C – Cdiff

C- Campylobacter

S- Shigella

S – Salmonella

HSP – Our patient had a URI two weeks ago, has abdominal pain with LGI bleed, is the right age (majority of cases present between 3-5) and has normal renal function labs (at least for now, as 50% of HSP eventually have renal involvement).  But…our patient does not have rash…which is fairly common…however I don’t think lack of rash rules it out…we need a scope.

IBD – 3 is a little young for onset of IBD though not impossible; the child has crampy abdominal pain with multiple blood stools (and classic relief of pain with bloody stool); rectal UC (more than Crohns) presents with LGI bleeding like this.  The borderline low MCV of 79, microcytic anemia, could be suggestive of chronic GI blood loss or could be some underlying iron deficiency anemia.  Also, there are no skin tags on rectal exam.

Juvenile  (Rectal) Polyps – usually presents with bright red blood and is often painless. Can be a lead point for intussusception or volvulus so therefore could have abdominal pain with presentation.  These can be anywhere in the GI tract (upper or lower) and hence it could be dark red blood if a bit more proximal.  A rectal exam may allow the palpation of a rectal polyp (and these can pop out – often confused with rectal prolapse - I recently saw a case).

Gastric ulcer – could cause pain and bleeding; would be worried about perforation with significant bleed, but our patient looks well.  Usually eating makes the pain worse (compared to better after eating with a duodenal ulcer). Would be good to know if she was on NSAIDs for any other reason.

Anal fissures  - common but not likely in our patient given dark red stools and anemia…usually more benign

Allergic colitis: Eosinophillic proctitis can cause lower GI bleed (or upper GI if severe eosinophillic/allergic esophagitis)…would expect this to be more chronic

I: Interesting

Portal hypertension: There was a case report of a child with undiagnosed idiopathic portal hypertension presenting with an esophageal varices and bleed – highly unusual but interesting – though unlikely in our well appearing patient with intermittent bleeding and no HSM.  Variceal bleeds are more rapid and quickly destabilize. 

Gastrointestinal Duplication – can ulcerate, perforate or form fistulas, causing lower GI bleed.  Very uncommon…we need a scope to diagnose this.  I guess this could cause pain if ischemic.

Tumors/Malignant polyps and Vascular Malformations …very rare, again would need a scope

T: Treatable

Of the list above, many are treatable.  I would use this question....to assist me in my management.

Management

For our patient, I agree with the testing that has already been done.  I would follow up with the PCP regarding the stool cultures and probably send another set. I would send a type and cross, make sure I have solid access and admit the patient for observation given the low hemoglobin and ongoing blood loss.  If IV access was difficult I would consider an EJ.  Also, I would discuss this patient with the PICU as GI bleed can lead to rapid hemodynamic instability; and with GI to consider scoping.

The Denouement
by Sonny Tat, Children's National

The patient continued to have intermittent pain so underwent a repeat abdominal ultrasound four hours later to evaluate the possibility of intermittent intussusception. This ultrasound demonstrated a thick walled cystic structure below the umbilicus with mild hyperemia without signs of intussusception. There was point tenderness at this location on ultrasound exam.

This patient remained hemodynamically stable and was admitted to the surgical service for further observation and diagnostic evaluation. 

The patient had a nuclear medicine study showing radiotracer uptake below the umbilicus (figure 2). She then underwent a diagnostic laprascopy that was converted to an open resection of a Meckel’s Diverticulum. The patient had an uncomplicated post-operative course. Her final pathology showed heterotopic gastric tissue, consistent with Meckel’s Diverticulum.

Please answer the two quick questions below. The data helps inform our future case design. Thanks for participating.

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The Hot Seat is a recurrent online case series aimed at facilitating asynchronous sharing of PEM knowledge and experience. Faculty are placed on The Hot Seat and publish their opinions without knowing the case or its outcomes. Cases are based on real cases and written by PEM fellows at Children's National in DC, Johns Hopkins in MD, and INOVA Fairfax in VA to highlight teaching points. Emily Willner is the faculty advisor for the Hot Seat.

Hot Seat Case #40 Response by Shilpa Patel

Although lower GI bleeding in children may be indicative of a serious illness, usually it is not.  Our very own Stephen Teach published a retrospective review of 109 cases (?his fellow’s project – pretty awesome) in which only four had life threatening diagnoses (https://www.dropbox.com/s/b53127f2haoqj43/1-s2.0-S0196064494703507-main.pdf) – three cases of intussusception and a single case of Meckel’s diverticulum.  

The first step in the approach to GI bleed is to determine if it is truly blood.  In our case, given the low hemoglobin and gross blood at the anus on exam, we should assume it is blood.  The second step is to try to distinguish between upper and lower GI bleeding.  Classic teaching is that bright red blood in/on stool is usually lower, however in an infant (short intestinal transit time), bright red blood could be from a massive upper GI bleed and quickly lead to hemodynamic compromise).  Whereas melena, or dark blood is classically more proximal (this could be blood that is swallowed from the nose/ingested). Red stool (though it is dark) with gross blood at the anus in our patient is probably lower GI.

I’m going to use SPI(T) (which Sonny introduced to us a few weeks ago as a teaching technique in the ED) to organize my DDx and will try to limit it to younger children since our patient is 3 years old.

S          Serious

P          Probable

I           Interesting

T         Treatable

DDX:

S: Serious

Intussusception – Though this has already been ruled out (US has a negative predictive value of 99.5% with a good sonographer), our patient's presentation, a 3 yo with intermittent severe abdominal pain sounds concerning.  Blood in stool is a late finding in intussusception. The currant jelly stool is associated with necrotic bowel and I would expect the patient to appear more ill.  Thinking creatively, the bloody stools in our scenario could be related to a lead point for intussusception (a bleeding polyp or diverticulum) causing the bleeding and the intermittent abdominal pain AND the telescoping could be intermittent (giving us a negative ultrasound when the child is comfortable).

Meckel’s diverticulum – usually described as “painless” bleeding (just blood and no stool), though our child reports abdominal pain. It can also be associated with complications (obstruction or perforation) and majority of the patients are younger than 2 years old. As Meckel’s can lead to rapid painless blood loss and hemodynamic compromise, ensuring that the patient is hemodynamically stable is very important. Our patient is anemic and should be watched.  At this point, I don't think I would get a Meckel scan on our patient.

Underlying coagulopathy – normal INR is reassuring along with normal platelets. HUS can present with GI bleed but is unlikely given normal platelets and normal Creatinine.

Volvulus (w/Malro) – would expect the child to be sicker with an abnormal aXR, or have bilious emesis (though she did have emesis x 2 earlier in the day).  If she had bilious emesis, would consider an AXR.

P: Probable

Infectious colitis – Our patient has crampy abdominal pain, it is associated with multiple stools and had vomiting earlier -fits the bill.  We should ask a travel/exposure history, send a second set of cultures and follow up on the stool cultures sent by the primary care doctor.  Would also be good to know if she was on antibiotics  (C. Diff colitis)  Something that goes against this is the lack of fever and perhaps a normal wbc….

Mnemonic for infections that cause bloody stools: YEECCSS!

Y – Yersinia

E – Ecoli

E -Entamoeba histolytica

C – Cdiff

C- Campylobacter

S- Shigella

S – Salmonella

HSP – Our patient had a URI two weeks ago, has abdominal pain with LGI bleed, is the right age (majority of cases present between 3-5) and has normal renal function labs (at least for now, as 50% of HSP eventually have renal involvement).  But…our patient does not have rash…which is fairly common…however I don’t think lack of rash rules it out…we need a scope.

IBD – 3 is a little young for onset of IBD though not impossible; the child has crampy abdominal pain with multiple blood stools (and classic relief of pain with bloody stool); rectal UC (more than Crohns) presents with LGI bleeding like this.  The borderline low MCV of 79, microcytic anemia, could be suggestive of chronic GI blood loss or could be some underlying iron deficiency anemia.  Also, there are no skin tags on rectal exam.

Juvenile  (Rectal) Polyps – usually presents with bright red blood and is often painless. Can be a lead point for intussusception or volvulus so therefore could have abdominal pain with presentation.  These can be anywhere in the GI tract (upper or lower) and hence it could be dark red blood if a bit more proximal.  A rectal exam may allow the palpation of a rectal polyp (and these can pop out – often confused with rectal prolapse - I recently saw a case).

Gastric ulcer – could cause pain and bleeding; would be worried about perforation with significant bleed, but our patient looks well.  Usually eating makes the pain worse (compared to better after eating with a duodenal ulcer). Would be good to know if she was on NSAIDs for any other reason.

Anal fissures  - common but not likely in our patient given dark red stools and anemia…usually more benign

Allergic colitis: Eosinophillic proctitis can cause lower GI bleed (or upper GI if severe eosinophillic/allergic esophagitis)…would expect this to be more chronic

I: Interesting

Portal hypertension: There was a case report of a child with undiagnosed idiopathic portal hypertension presenting with an esophageal varices and bleed – highly unusual but interesting – though unlikely in our well appearing patient with intermittent bleeding and no HSM.  Variceal bleeds are more rapid and quickly destabilize. 

Gastrointestinal Duplication – can ulcerate, perforate or form fistulas, causing lower GI bleed.  Very uncommon…we need a scope to diagnose this.  I guess this could cause pain if ischemic.

Tumors/Malignant polyps and Vascular Malformations …very rare, again would need a scope

T: Treatable

Of the list above, many are treatable.  I would use this question....to assist me in my management.

Management

For our patient, I agree with the testing that has already been done.  I would follow up with the PCP regarding the stool cultures and probably send another set. I would send a type and cross, make sure I have solid access and admit the patient for observation given the low hemoglobin and ongoing blood loss.  If IV access was difficult I would consider an EJ.  Also, I would discuss this patient with the PICU as GI bleed can lead to rapid hemodynamic instability; and with GI to consider scoping.

Hot Seat #40: 3 yo with bloody stools

By Sonny Tat, Children's National
with Shilpa Patel, Children's National

The Case

3 year-old previously healthy girl presents to your ER with abdominal pain and bloody stools. The patient had abdominal pain starting yesterday. After a few hours, the patient had a bowel movement with dark red blood, which may have eased the pain. She had two or three more episodes of bloody stool and intermittent abdominal pain yesterday. Today she went to the PMD who sent stool studies and sent her home. She is in the ED today because of persistent abdominal pain that puts her in the fetal position that is temporarily relieved by bowel movements that are associated with dark blood. She has had emesis x 2 today that is non-bloody, non-bilious.

ROS: No fevers, rash, constipation, sick contacts, or change in diet. URI two weeks ago.

Exam: VS Afebrile, HR 119, RR 22. BP 97/57

Well-appearing, active, smiling, no distress

Chest CTAB, heart RR, no murmurs or gallops

Abd ND/NT, no masses. Rectal exam shows gross blood at the anus

Labs/Rad:

Hemoglobin 8.8 WBC 11.7 Platelets 302 MCV 79

Na 139 K 3.4 Cl 106 CO2 22 BUN 11 Cr 0.4

INR 1.1

Ultrasound is negative for intussusception

Questions for you:

How would you approach this case? Please share your opinions by clicking on “comments” below. It’s easiest if you’re also logging into your gmail account but you can do it without as well. Just select “Name/URL” from the drop down menu, write your name, and click “submit.” You can also post anonymously although this seems less fun. To read posted comments, click on “comments” below and scroll up.

Come back later for the denouement of this case 

About the Hot Seat. Hot Seat cases are written by PEM fellows at Children’s National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series. 

Hot Seat Case #39 Denouement: 5 yo at OSH who “doesn't look right”

by Katie Donnelly, Children’s National
with Paul Mullan, Children's National, on the Hot Seat

The Case
This is a case of a 5 yo girl being transferred from an OSH because she “doesn’t look right” who has tachypnea and emesis.  The challenge in this case is how to approach a patient with initial findings of hepatosplenomegaly and leukocytosis. 

For a complete case presentation, please click here. 

Here's How You Answered Our Questions 

In light of this CXR, would you order any additional studies while awaiting the results of the bloodwork? 

Less than 3 years PEM experience (fellows, PAs/NPs, pediatricians) n=10

More than 3 years PEM experience (PEM attendings, pediatricians) n=12

PEM providers of all years experience agreed.  The majority would obtain an EKG.  Several would also get an ECHO with Cardiology consult.

Case continues: You receive a call from the lab with a critical WBC 800,000. The CMP is still pending. You go to assess the patient, who is clinically unchanged.  

One more question for you

What is your next management priority?

Less than 3 years PEM experience (fellows, PAs/NPs, pediatricians) n=9

More than 3 years PEM experience (PEM attendings, pediatricians) n=12

PEM providers with less than 3 years experience gave a normal saline bolus and started 1.5 x MIVFs (no potassium, as worried about tumor lysis).  One person stated that "other" for tumor lysis labs.  PEM providers with more than 3 years experience ordered 1.5x MIVFs followed by NS bolus/"other".  Three people put "other" for "more labs (LFTs, LDH, uric acid)," "I-stat electrolytes and gas," and "consider rasburicase."

Clinical Reasoning 

Thank you to the several attendings that left very insightful comments about this case!  Overall themes include the need to obtain tumor lysis labs (iStat lytes, gas, LDH, uric acid, Ca, Phos, Mg, CMP).  Additional excellent discussion topics include the use of rasburicase vs allopurinol.  Also, in the setting of hyper-leukocytosis, one should think about PICU admission for central line access and leukapheresis.  For complete comments, click here.

“If the WBC is really 800K (not 80K), with huge hepatosplenomegaly, this is leukemia until proven otherwise… With WBC that high, the serum K level is much more accurate on a non-spun specimen (such as from an I-Stat instead of sending to the lab), as leukemic WBCs are typically "leaky". Rasburicase is better than allopurinol in this setting in getting uric acid under control”. - Dewesh Agrawal, Children's National

“One of the big risks of hyper-leukocytosis is sludging, which can lead to high-output cardiac failure or stroke. My next step would be to get the kid to a place where he/she could get a big honkin' 7F line in the groin for an exchange transfusion [plasmapheresis]…You can do some hemodilution with IVF but have to be careful not to give any bulky fluids (PRBCs) since they can precipitate stroke.  Rasburicase is super $$$$, so not sure the role if the patient is getting exchanged regardless.” – David Mathison, Children's National

“Plasmapheresis is when plasma is removed and donor plasma is given. Used in TTP, HUS, Autoimmune diseases , Good pastures etc.”  VS.  “Exchange transfusion is where you remove blood (not plasma) and replace it with good donor blood example : sickle cell with acute chest syndrome where blood removed from sickler and transfused again with normal blood” – Breanna Barger, Johns Hopkins

“The highest priority is plasmapheresis, so moving her to the PICU is the focused goal… no longer recommended to alkalinize the urine because of the risk of precipitating Ca-phos product.” – Jennifer Chapman, Children's National

“Can't ignore a WBC of 800k… If blasts are seen, you have your diagnosis. While waiting for plasmapheresis and transfer to the PICU, giving a NS bolus would probably be beneficial for the marked leukocytosis. Perhaps check an EKG (+/- ECHO) before doing so to make sure you're not fluid overloading a 'sick' heart… and calls to the Hem/Onc, Cardiology.” – Jamil Madati, Children's National

From the Hot Seat
by Paul Mullan, Children's National

DDx for HSM that's likely cancer-related with that WBC and mediastinal mass:

- Leukemia, Lymphoma

- Neuroblastoma

- Hepatic cancer (less common than above types; screen with alpha-fetoprotein and CT).

- Histiocytic (these are hard to remember but I have an assortment of specific findings I look for and if I don't see at least a couple of these, this falls lower on ddx:  cytopenias or cytopenic suggested symptoms noted, skin findings papular or seborrheic, diabetes insipidus symptoms, lymphadenopathy, loose teeth - although that picture suggests maybe some loose ones!). 

The hyperleukocytosis (>50-100K WBC) pushes me toward the dx of ALL in a major way (esp Tcell ALL with that mediastinal mass ugliness on CXR), so the oncology fellow is on their way in from home to look at the slide, but in the ED at this moment I'm worried about potential for leukostasis (that is, when hyperleukocytosis causes symptoms which can affect many organ systems) - so by system, what to worry about, and what to do about it...

1. CNS:  increase ICP, bleed, seizures, AMS, focal deficits, CVA, coma; no signs of this now in this patient; continue to monitor.

2. Blood/Lytes:  

a.Tumor lysis syndrome (TLS) ; this usually begins after chemo starts but can occur prior to chemo. CMP might be pending, but double check that it's not being done and in meantime add some more labs:  Istat with lytes, uric acid, LDH, CBC, T&S, DIC panel.  Reducing wbc can happen with chemo, leucopheresis, or hydroxyurea but this will happen in ICU so not really our acute issue right now. Prophylaxis and treatment for TLS (according to most recent American Society of Clinical Oncology guidelines - by Coiffier B,et al JCO 2008;26(16): 2767-2778.) is two fold: 

1st: Hydration  with D5 1/4 NS at 3L/m2/day with goal of equal ins and outs; if already oliguric or in renal failure, d/w renal/onco on how they prefer to hydrate. 

2nd: Uric acid reduction. Two choices: allopurinol and rasburicase. The ASCO guidelines recommend first determining if patient is low risk, intermediate risk, or high risk; another fun article (Brit J Hem 2010;149:578-586) details this risk classification, but assuming this is leukemia, the >100k puts this pt in high risk zone regardless of leukemic subtype or LDH level. High risk patients get rasburicase (medium risk gets allopurinol or rasburicase; low risk gets nothing but monitoring); given contraindication to rasburicase is G6PD, definitive testing for this condition is recommended. Rasburicase is expensive but cost effectiveness studies show that it decreases ICU days, renal failure incidence, etc. and ends up saving money.  

- As Jen stated, alkalinization is no longer recommended - it was never proven effective and can increase precipitation of CaPhos crystals in urine.

- Hyperuricemia: can lead to uric acid crystallization in kidneys  -->  acute renal failure; see uric acid reduction tx above.

- Hyperkalemia (can also be spurious from leukemic blast degradation; Istat is most accurate), worry about EKG changes that lead to VT/VF/arrest; treat if >6 with standard hyperK tx and NO iv or po potassium intake. 

- Hyperphosphatemia: leads to n/v/d (worsens hydration state which is key for TLS) and can cause lethargy/seizures which makes one think of CNS leucostasis symptoms and thus complicates the workup; if Ca x Phos is >70, can get CaPho precipitation in kidneys;  therapy is hydration and phosphate binders (calcium carbonate, aluminum hydroxide) ; if severe or not decreasing, then hemodialysis. 

- Hypocalcemia: can lead to cramps/tetany and worse cases hypotension/arrhythmias.  If symptomatic, given ca gluc 50 mg/kg IV slowly with ekg monitoring.

b. DIC and thrombocytopenia; except for the photo that shows bleeding around the gums (vs tartar?), no indication of this for now, but have sent the labs above.

2. Lungs: hypoxemia (can get false low pO2 on ABG because leucocytes use up the O2), respiratory distress (made worse in her by this mediastinal mass on CXR - I would NOT get a chest CT as laying these patients down can precipitate resp failure - I would sit her up in bed, add O2 by NRB facemask, and see if this helps her tachypneic state.

3. Renal failure: from the uric acid or ca-phos deposition; if oliguric or in renal failure, discuss with renal about IVF and diuretic management

4. Cardiac failure (get an EKG; heart failure is still a possibility with the HSM and tachypnea, but I would not necessarily get cards to the ED unless EKG was abnormal; myocarditis a possibility as a secondary infection, but unlikely as a primary one with >800k wbc, unless lab called and said white count was a lab error)

5. Less common in ED: GI (bleeds, abd pain), priapism, clitoral enlargement, dactylitis, many others.   

Disposition is certainly to the ICU in this symptomatic child with high interventional needs. 

The Denouement
by Katie Donnelly, Children’s National

The case was modified somewhat for the Hot Seat presentation and the actual case follows:

This 5 year old female initially presented to an referring hospital with a WBC count of 300 and a chest x-ray  that was concerning for cardiomegaly.  Repeat CBC at our institution (less then 8 hours later!) was notable for a WBC of 803 with 81% blasts, a hemoglobin of 6.4 and platelets of 30. Her uric acid was 8.6 and her LDH was 3,522.  Her potassium was initially reported as 20, repeat of 4, the erroneous result was attributed to her massive leukocytosis.  She had a bedside ultrasound which showed normal heart size and function and hepatosplenomegaly. Her repeat chest XR above was concerning for an anterior mediastinal mass. She was started on hyperhydration with one and a half times maintenance fluids without potassium  and rasburicase for hyperuricemia. She was admitted to the PICU.  Hematology and Transfusion Medicine were consulted and the patient underwent leukapheresis given her significant risk for stroke which reduced her WBC count to 500.  Her peripheral flow cytometry was consistent with Acute T-cell Lymphoma and she has started chemotherapy.  

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The Hot Seat is a recurrent online case series aimed at facilitating asynchronous sharing of PEM knowledge and experience. Faculty are placed on The Hot Seat and publish their opinions without knowing the case or its outcomes. Cases are based on real cases and written by PEM fellows at Children's National in DC, Johns Hopkins in MD, and INOVA Fairfax in VA to highlight teaching points. Emily Willner is the faculty advisor for the Hot Seat.

Hot Seat Case #39: 5 yo at OSH who “doesn't look right”

by Katie Donnelly, Children’s National
with Paul Mullan, Children's National, on the Hot Seat

The Case

You receive a call from a referring hospital asking about transferring a 5 year old child.  She had been seen at the hospital 2 weeks prior for fever and started on amoxicillin for AOM.  She returned to the ED because her mother noted she was breathing faster than normal and she was having some vomiting.  She is no longer having fevers. On their exam her respiratory rate is in the 40’s but her lungs are clear and her abdomen is mildly distended. They have tried but were unable to obtain blood, though they do have IV access. A CXR was obtained which they report shows no pneumonia. They are worried that she just “doesn’t look right” and ask for transfer to your institution for further evaluation.

On arrival to your ED:

T of 37.1 HR 121 RR 40 BP 94/60 O2 sat 98% on room air

GEN: Alert, well developed for age. Seems uncomfortable, moving around the bed

SKIN: No petechiae or ecchymosis noted

HEENT: Normocephalic, atraumatic, neck supple, mucus membranes moist

CV: Regular rate and rhythm, no murmur

RESP: Lungs clear to auscultation bilaterally

ABD: Soft but distended abdomen, palpable hepatosplenomegaly

NEURO:  Alert, moves all extremities

LYMPH: No LAD

You send labs and then review the single view chest XR obtained at the outside hospital (shown below).

Questions for you (answer according to your PEM experience)

Case continues: You receive a call from the lab with a critical WBC 800,000. The CMP is still pending. You go to assess the patient, who is clinically unchanged.  

One more question for you

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About the Hot Seat. Hot Seat cases are written by PEM fellows at Children's National, Inova Fairfax, and Johns Hopkins. The Hot Seat Attending is a selected faculty member who comments on the case without knowing the outcome. Emily Willner is the faculty mentor for the series.