Critical Cases - Swollen 2 year old!

Tue, 02/02/2021 - 5:11am

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HPI

2 yo female p/w "whole body swelling"
Seen previously at urgent care and diagnosed with an allergic reaction, rx for diphenhydramine and prednisone which she has been taking
No fever, no trouble swallowing or breathing, no wheezing, and no rash
No new exposures (such as chemicals or pets)

Physical Exam

VS: Pulse 122, BP: 120/88, Temp 98.6F rectally, RR: 30, Wt. 15kg, SpO2 100%

HEENT: Notable facial swelling with periorbital edema, without erythema, conjunctiva clear

Oropharynx: Clear, no stridor, no cervical lymphadenopathy

Lungs: clear, no wheezing, no crackles

Abd: Distended abdomen, no fluid wave

GU: Labia swollen

Extrem: All 4 extremities + notable non-pitting edema without tenderness, no warmth, erythema, bruising, or rashes. Strong pulses.

Differential:

Nephrotic syndrome vs other acute renal failure most likely
Cardiomyopathy such as myocarditis or other cause of CHF, although less likely given clear lungs, no significant tachycardia, no dyspnea, no prior infectious symptoms.
Less likely cirrhosis, no risk factors for this, no bruising, no jaundice, no encephalopathy

Lab results:

Management and Case Conclusion:

Pediatric nephrologist recommended 10g of 25% albumin over 3 hours followed by IV Lasix and transfer to a pediatric specialty hospital for further management
The patient was a admitted for 4 days and treated with IV solumedrol, diuresis with Lasix, and infusions.
On discharge she had lost 2kg and -4 liters of fluid.
The patient had multiple follow up visit with a nephrologist, her swelling had resolved, her BP improved to normal range
Pt continued to still have proteinuria despite a prolonged course of high dose steroids
2 months later, she was noted to have swelling again of her face and abdomen, so she was readmitted for a renal biopsy and genetic testing (results pending), as she seems to have steroid-resistant nephrotic syndrome.

NEPHROTIC SYNDROME:

Nephrotic syndrome is diagnosed by the following 4 criteria:

1.    Proteinuria (cut offs are generally >3-4g in 24 hours or protein: creatinine ratio >300-350mg/mmol)

2.    Hypoalbuminemia (<2.5-3g/dL)

3.    Hyperlipidemia (often >350mg/dL)

4.    Peripheral edema. 

Many patients also have hypertension, but this is not necessary to make the diagnosis.
Remember on average, a normal systolic BP in a child is 90mmHg + (2 x age in years), this pt had systolic of 124 (expected 94)
There are many causes of primary nephrotic syndrome, such as: focal segmental glomerulosclerosis, membranous nephropathy, and minimal change disease.
Minimal change disease is the most common nephrotic syndrome in children and accounts for 70-90% of nephrotic syndromes in children <10 years old.
The gold standard for diagnosis is a renal biopsy, however this is not usually necessary with first presentation of nephrotic syndrome

Sources:

https://bestpractice.bmj.com/topics/en-us/356