A 2 year old, previously healthy female presents to the ED with diffuse lower extremity swelling and abdominal distention. According to her mother, patient had been sick with strep throat 10 days prior and was treated with a course of amoxicillin. The patient had been compliant with the medication, which was scheduled to end that evening. The previous evening, her mother had initially noted facial swelling, and given the patient Benadryl with some improvement. On the day of the ED visit, the mother had noticed the patient developed swelling in both of her lower extremities and had a distended appearance of her abdomen without any signs of pain. The patient did not have any rashes or hives. She had been having improved oral intake following her strep infection. She had no appreciable changes in her urinary or bowel habits.
On examination, the patient is afebrile, and vital signs are within normal limits. She has a distended appearance to her abdomen with normal bowel sounds, no tenderness. She exhibits symmetric bilateral lower extremity edema that extends from feet to knees. There is no erythema or rash noted. HEENT examination is unremarkable.
What is the most likely cause of her symptoms?
A. Congestive heart disease
B. Nephrotic syndrome
C. Thyroid disease
D. Liver failure
E. Post strep glomerulonephritis
Labs are significant for an elevated total protein (4.7), low albumin (1.9). No leukocytosis. Normal BUN/Cr, AST, ALT, ALP. Urinalysis is significant for protein of 500 and no blood.
With that information, what is the most likely cause of her symptoms?
Remind me, what is nephrotic syndrome again?
- Characteristics includes persistent heavy proteinuria, hypoproteinemia, hypercholesterolemia, and edema
- There is increased glomerular permeability due to alterations to normal glomerular cellular and basement membrane barrier
- These alterations cause loss of ability to restrict urinary loss of protein.
- Albumin is the protein most affected
- Edema is secondary to fluid shifts (low plasma oncotic pressure so fluid shifts from vascular to interstitial compartments) and due to increased activation of RAAS
- Elevated serum lipids and protein occur because of liver compensation for loss of proteins
- Eventually liver synthesis of albumin cannot keep up with urinary loss of proteins
- Underlying cause can be renal or due to a systemic disease
Are there different types?
- Minimal change disease
o Minimal change disease is the most common cause in children
oMore than 80% of children who are less than 7 years old with have Nephrotic syndrome due to minimal change disease.
oChildren ages 7- 16, 50% will have nephrotic syndrome due to minimal change disease
Males are affected 2:1 compared to females (in children, not adults)
- Primary Focal Segmental Glomerulosclerosis
o Focal segmental glomerulosclerosis (FSGS) is second most common
- Membranoproliferative glomerulonephritis
- diopathic membranous nephropathy (congenital)
- Lupus glomerulonephritis
- Henoch-Schonlein purpura
- Chronic infections (Hep C, HIV)
- Acute infections (viral, bacterial, or parasitic)
- Allergic reactions
- Diabetes Mellitus
- Congestive heart failure
- Renal vein thrombosis
How do these patient present?
- Patients can present with;
o Nonspecific symptoms such as fever, malaise
o Non urinary symptoms such as arthritis, rash
o Urinary symptoms such as edema, hypertension
- In the first few years of life, often present with periorbital swelling with or without generalized edema
- Edema ranges from mild periorbital swelling to severe anasarca
- Absence of hematuria, renal insufficiency
- Typically after infection or other stress. Ex. Viral illness, insect bite
What labs are important?
- Urine protein creatinine measuring with a ratio >2.0 is in the nephrotic range
o Adults, children > 2yrs – Urine P:Cr 0.2 is considered normal
o Children 6 mo – 2 yrs – Urine P:Cr 0.5 is considered normal
- Tests to order include
o UA for protein
o Serum creatinine, BUN
o Serum albumin, total protein
o Serum cholesterol, triglycerides
o Renal Ultrasound
o Serum CBC, BMP
- Expectations on aforementioned labs
o Serum albumin is decreased (albumin <3.0)
o Cholesterol levels are increased
o UA shows proteinuria with >2 g/ m2/24 hours
o Normal complement levels (in minimal change disease)
- For asymptomatic proteinuria, need to establish persistence, options include;
o AM/PM urine dipsticks
o Spot AM urine protein/creatinine ratio
- Biopsy is only performed when cause suspected to be something other than MCD
What do I do once I’ve figured out its nephrotic syndrome?
- Consult a pediatric nephrologist
- Patients will typically be started on steroids (without renal biopsy if MCD is most likely)
o Prednisone 2 mg/kg/day (max 60 mg)
o Response is typically seen within 4 weeks but treatment continues for 12 weeks
o Renal biopsy if no response to steroids
- Restricted salt intake
- Loop diuretics may be indicated for severe edema
- Beta blockers or calcium channel blockers for severe HTN (persistent HTN treat with ACEIs)
- Complications include;
o Hypercoagulable state à increased risk of thromboembolism
o Atherosclerotic heart disease
Other useful information:
- There are benign cause of proteinuria including isolated proteinuria (no other renal symptoms), transient proteinuria (can be caused by fevers or exercise), orthostatic proteinuria (protein excretion only in upright position)
- Most children go into remissions, however, nearly 80% will have relapse of minimal change disease
- Many children with FSGS will go into end stage renal failure
How’d our patient do?
Great! She was admitted overnight primarily for initial management and family teaching. She was started on course of Prednisone for presumed minimal change disease. She has had outpatient follow up with pediatric nephrology and continued her steroid course with slow improvement in her proteinuria.
Faculty Reviewer: Dr. Chris Merritt
1. O’Connel, Theodore X. Instant Work-ups: A clinical guide to pediatrics. Proteinuria. Chap 36. 245-250
2. Marcdante, Karen. Nelson’s Essentials of Pediatrics. Nephrotic syndrome and proteinuria. 7th edition. 2015. Chapter 162. Pgs 556- 558
3. Madaio, Michael. The Diagnosis of Glomerular Disease; Acute Glomerulonephritis and the Nephrotic Syndrome. Arch Internal Med. 2001; 161: 25-34
4. Pal, Abhijeet. History of Nephrotic Syndrome and Evolution of its Treatment. Frontiers in Pediatrics. May 30th 2016; 4: 56.
5. El Bakkali, Loubna. Nephrotic Syndrome in The Netherlands: a population-based cohort study and review of the literature. Pediatric Nephrology. 2011; 26: 1241-1246
HPI: An 18 month old previously healthy female with a history of mild intermittent constipation, but no prior abdominal surgeries presented to the ED with 3 days of non-bilious, non-bloody emesis and abdominal pain not responsive to laxatives and Zofran. On the day of presentation she developed multiple episodes of screaming associated with grabbing her stomach and pulling up her legs. Episodes lasted for 30 seconds to 1 minute and self-resolved. She was afebrile. Oral intake and urine output were decreased, and she had no stools on the day of presentation. Her last bowl movement was 1 day prior to presentation and was loose without blood. The patient had nasal congestion and rhinorrhea the prior week.
Vitals: Pulse 124 | Temp 98.7 °F (37.1 °C) | Resp 26 | Wt 15.7 kg | SpO2 98%
Exam: Unremarkable with the exception of diffuse abdominal tenderness and voluntary guarding, but no rebound. No hepatosplenomegaly was appreciated. Bowel sounds were diminished.
Given the story and exam, the clinical suspicion for intussusception was high, and an initial two view abdominal x-ray was obtained:
2 View Abdominal x-ray revealed a soft tissue mass in the right upper quadrant concerning for intussusception (arrows). A comprehensive ultrasound confirmed ileocolic intussusception.
A bedside point-of-care ultrasound was performed while patient was awaiting comprehensive radiographic evaluation. A donut-shaped mass was visualized in the right lower quadrant, consistent with ileo-colic intussusception.
The patient underwent a successful air-enema reduction. Post air reduction, the left lateral decubitus film shows resolution of the paucity of gas previously seen in the RUQ.
Epidemiology of Intussusception:
Intussusception is the most common abdominal emergency in early childhood, particularly among children younger than 2 years of age. Approximately 60% of children with intussusception are less than 1 year old and 80-90% are less than 2 years old. Additionally, it is the most common cause of intestinal obstruction in infants between 6 and 36 months and there is a slight male predominance, with a male:female ratio of approximately 3:2.
Intussusception in this age group most often occurs at the ileocecal junction, leading to ileocolic intussusception. The intussusceptum, a proximal segment of bowel, telescopes into the intussuscipiens, a distal segment.
Approximately 75 percent of cases of intussusception in children are considered to be idiopathic because there is no clear disease trigger or pathological lead point. Mesenteric lymph nodes may act as a lead point and may occur in the setting of gastroenteritis (both viral and bacterial), viral upper respiratory illnesses, flu-like illnesses, and adenovirus. Henoch-Schönlein purpura, HSP, is also associated with intussusception, but more commonly ileo-ileal. Non-infectious causes of lead points include Meckels, polyps, tumors (lymphoma), hematomas, vascular malformations, duplication cysts, and post-operative scarring.
The classic presentation of intussusception includes sudden onset intermittent, severe, crampy, and progressive abdominal pain, accompanied by inconsolable crying and drawing up the legs towards the abdomen, with return to baseline between episodes. As lethargy may follow episodes of abdominal pain, intussusception should be on the differential diagnosis for any young child presenting with unexplained lethargy or altered mental status. Non-bloody, non-bilious emesis is often present. The classically described triad of pain, palpable abdominal mass, and currant jelly stool occurs in <15% of children.
Abdominal plain films: A two view abdominal x-ray can be used to evaluate the bowel gas pattern and to exclude perforation in patients with suspected intussusception. It is not the imaging method of choice, as a negative plain film cannot rule out intussusception. A plain film is less sensitive and specific than ultrasonography for intussusception, but may include the following suggestive findings:
- Signs of intestinal obstruction (distended loops of bowel with absence of colonic gas)
- Target sign (two concentric circles superimposed on the right kidney)
- Crescent sign (soft tissue density projecting into the gas of the large bowel)
- Obscured liver margin (as seen in the patient in this vignette)
- Lack of air in the cecum
- Pneumoperitoneum (RARE, secondary to perforation)
Ultrasonography: Abdominal ultrasound is the modality of choice, with sensitivity and specificity approaching 100% when performed by experienced ultrasonographers. The classic imaging findings include a “target sign” or “bull’s eye” that occurs due to the layers of intestines within one another. This target is usually ≥3 cm in diameter.
Point of Care US (POCUS) for Intussusception:
Bedside ultrasound may lead to more expeditious diagnosis, particularly when pediatric radiology is not available, and may facilitate more rapid comprehensive radiographic and/or surgical evaluation. A 2011 study showed that 6 PEM physicians could perform the exam with a sensitivity of 85%, specificity of 97%, positive predictive value of 85%, and negative predictive value of 97% for diagnosing intussusception after just a 1 hour course!
Diagnostic pitfalls with ultrasonography include enlarged lymph nodes, non-pathologic ileo-ileal intussusception, the psoas muscle, colonic stool, and the kidneys, all of which may be misinterpreted as intussusception. Scan the entire abdomen and try to localize the actual kidneys. Confirm that the depth of the image and the diameter of the findings in question are consistent with the typical intussusception parameters, and observe for surrounding peristalsis to help decrease misinterpretation of findings.
Using a linear probe, start by placing the probe transversely in the RLQ and attempt to identify the psoas muscle (semicircular structure with striations and iliac vessels just medial to it). Set the depth to at least 6 cm. Slowly sweep superolaterally until the liver and gallbladder are visualized. Proceed along the course of the large bowel as the intussusception may be seen in the LLQ. If seen, an intussusception should be imaged in two planes.
The preferred method of treatment for intussusception is nonoperative, with air or barium enema reduction, which is performed by radiology. Surgery should be close by in case of complication and/or need for urgent operative intervention. Operative intervention may be needed if non-operative reduction fails or if there is concern for a mass lesion as the pathological lead point, or if there is suspected or proven bowel perforation or necrosis.
Faculty Reviewer: Dr. Erika Constantine
1. KitagawS, Miqdady M. Intussusception in children. Uptodate.com. Accessed April 17, 2016. <http://www.uptodate.com/contents/intussusception-in-children source=search_result&search=intussusception&selectedTitle=1~102>.
2. Doniger SJ, Salmon M, Lewiss RE. Point-of-Care Ultrasonography for the Rapid Diagnosis of Intussusception: A case series. Pediatr Emergency Care. 2016 Feb 15. Epub ahead of print. PMID: 26890297.
3. Alletag MJ, Riera A, Langhan ML, Chen L. Use of emergency ultrasound in the diagnostic evaluation of an infant with vomiting. Pediatr Emerg Care. 2011, Oct; 27(10):986-9.
4. Eshed I, Gorenstein A, Serour F, et al. Intussusception in children: can we rely on screening sonography performed by junior residents? Pediatr Radiol. 2004;34;134-137.
5. Ramsey KW, Halm BM. Diagnosis of intussusception using bedside ultrasound by a pediatric resident in the emergency department. Hawaii J Med Public Health. 2014 Feb;73(2):58-60.
6. Riera A, Hsiao AL, Langhan ML, Goodman TR, Chen L. Diagnosis of intussusception by physician novice sonographers in the emergency department. Ann Emerg Med. 2012 Sep;60(3):264–268. [PubMed].
7. Chang YJ, Hsia SH, Chao HC. Emergency medicine physicians performed ultrasound for pediatric intussusceptions. Biomed J. 2013 Jul-Aug;36(4):175–178. [PubMed].
8. Halm BM. Reducing the time in making the diagnosis and improving workflow with point-of-care ultrasound. Pediatr Emerg Care. 2013 Feb;29(2):218–221. [PubMed].
54 year old male with a history of advanced cirrhosis presents as a transfer from an outside hospital with upper GI bleeding. The patient had banding of esophageal varices 2 weeks prior and was at a rehab facility when he developed several episodes of gross hematemesis. He presented to an outside hospital where he was intubated for airway protection and transferred to RIH for definitive management. The bleeding had stopped prior to transfer but he then started pouring gross blood from his mouth as he was rolling in to the CC bay.
GI was called immediately but the fellow was having trouble getting in touch with her attending. The decision was made to temporize the bleeding by placing a Blakemore tube.
1. Obtain the following materials:
A: Blakemore tube: Sitting on the shelf in CC alley.
B: 2 three-way stop cocks, 2 christmas tree adapters (image 2), 1 double christmas tree adapter (image 3): Supply should have placed these in the box with the Blakemore.
C: 60cc syringe
D: Lots of lube
E: Kerlix gauze
F: Posey manometer (image 4): Call respiratory for this.
Image 2-4 (in order): Christmas tree adapter, double christmas tree adapter, posey manometer.
2. Remove the white caps (image 6) from the ports and assemble the Christmas tree adapters and triple stopcocks into the esophageal and gastric ports.
3. Test the balloons and then deflate completely and turn stop cocks so that the "off" is facing the ports. Lube up the entire length of both balloons.
1. Pass the tube through the nose if awake or the mouth if intubated. If going through the nose, you will meet a decent amount of resistance as the esophageal balloon passes through the nose.
2. Auscultate the stomach and push air through the “Gast Asp” port to ensure you are not in the lungs.
3. IMPORTANT STEP: Fill the gastric port with ONLY 50cc of air (image 8). Get an x-ray to confirm balloon is in the stomach and not the esophagus (image 9).
Update: Of note, 50 cc of air in the balloon can still damage the esophagus. We now recommend that the provider should note the distance between the top of the gastric balloon and the tip of the tube and make sure the tube is at least that far below the diaphragm before inflating (as seen on a CXR). Following this, they should now inflate the 50 cc of air (followed by another CXR to truly confirm gastric placement).
4. Once confirmed by x-ray, add another 200cc of air to the gastric balloon (image 10). This should be followed by one last CXR to truly confirm adequate placement.
5. Pull traction on the tube, tie using kerlix to a 1L bag of saline and drape over an IV pole (image 11 and 12). This pulls the gastric balloon into the GE junction to tamponade any gastric bleeding.
6. DO NOT fill the esophageal balloon unless there is continued bleeding which would indicate that the bleeding is from the esophagus. The esophageal balloon is filled to a goal pressure, not volume.
A: Connect the posey manometer to the esophageal stop cock.
B: Pump to a pressure of 40 mmHg.
C: Turn the stop cock so the "off" is facing towards the port.
7. Can use the double Christmas tree adapter to connect the gastric aspiration port to suction.
8. Congratulate yourself and get GI to come on down!