Critical Cases - Swollen 2 year old!
- 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)
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.
- 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
- UA showed 4+ protein
- Creatinine 0.25
- Albumin: 1.1 (low)
- Protein: 3.4 (low)
- Results consistent with nephrotic syndrome
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 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