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Paeds Casesnephrology-urology-fluids-and-electrolytes

Paeds Cases · nephrology-urology-fluids-and-electrolytes

Post-infectious glomerulonephritis: Case

Clinical case of a six-year-old with post-streptococcal glomerulonephritis two weeks after a sore throat, covering the acute nephritic presentation, the low C3 with normal C4 recovering within eight weeks, the supportive management with fluid restriction, frusemide, and amlodipine plus streptococcal eradication, and the transition to an atypical scenario in which persistent hypocomplementaemia mandates biopsy.

paediatric nephrology long case4 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A previously well 6-year-old girl presents to the emergency department with three days of smoky brown urine and morning periorbital swelling. Her father reports that she had a sore throat and fever two weeks ago which settled without antibiotics. On examination she is afebrile, has periorbital and ankle oedema, and her blood pressure is 126 over 83. Urinalysis shows blood 3 plus and protein 2 plus, with dysmorphic red cells and red cell casts on microscopy. Her creatinine is 88 micromoles per litre, her C3 is low at 0.42 grams per litre, her C4 is normal, and her antistreptolysin O titre is raised. Throat swab is negative.

This girl has typical post-streptococcal glomerulonephritis. The diagnosis rests on the acute nephritic syndrome of haematuria, oedema, and hypertension, arising two weeks after an untreated sore throat, with dysmorphic red cells and red cell casts, a low C3 with a normal C4, and a raised antistreptolysin O titre. The latency of two weeks after pharyngitis and the low-C3-normal-C4 pattern together confirm the diagnosis and distinguish it from IgA nephropathy and from the other hypocomplementaemic glomerulonephritides. [1]

Clinical findings

The pattern is unequivocally that of pharyngitis-associated PSGN. The temporal sequence is classic: a sore throat two weeks before the haematuria, which is the expected one to three week latency after throat infection. The smoky brown urine reflects glomerular bleeding with dysmorphic red cells and red cell casts, the periorbital and ankle oedema reflects salt and water retention from the nephritic syndrome, and the hypertension is volume-dependent and renin-mediated from the renal injury. The mild acute kidney injury with a creatinine of 88 is consistent with the typical course. [2]

The low C3 with a normal C4 is the serological fingerprint of PSGN. It reflects consumption of C3 downstream of the alternative-pathway C3 convertase, with the lectin pathway also contributing, while the classical pathway is relatively spared so that C4 is preserved. The raised antistreptolysin O titre confirms a recent group A streptococcal throat infection, even though the throat swab is negative, because the organism is often cleared by the time PSGN declares itself. The negative throat swab therefore does not exclude the diagnosis. [3]

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References3ShowHide
  1. [1]Rodriguez-Iturbe B, Musser JM The current state of poststreptococcal glomerulonephritis J Am Soc Nephrol, 2008.PMID 18667731
  2. [2]Brant Pinheiro SV, et al Acute Post-Streptococcal Glomerulonephritis in Children: A Comprehensive Review Curr Med Chem, 2022.PMID 35702785
  3. [3]Hisano S, et al Activation of the lectin complement pathway in post-streptococcal acute glomerulonephritis Pathol Int, 2007.PMID 17539966
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