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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2013  |  Volume : 24  |  Issue : 3  |  Page : 546-548
Severe acute post-streptococcal glomerulonephritis in an infant

1 Department of Pediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia
2 Department of Pathology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

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Date of Web Publication24-Apr-2013


Acute post-streptococcal glomerulonephritis (APSGN) is very rare below the age of two years. We report a 14-month-old girl who presented with frank hematuria and nephrotic syndrome following group A streptococcal pharyngitis (GAS), which was confirmed by laboratory investigations. The patient underwent a renal biopsy to confirm the diagnosis and was treated with prednisolone. The proteinuria and hematuria resolved completely in eight weeks. Our case demonstrates that APSGN should be considered in evaluating hematuria and nephrotic syndrome in infants and children below two years of age.

How to cite this article:
Kari JA, Bamagai A, Jalalah SM. Severe acute post-streptococcal glomerulonephritis in an infant. Saudi J Kidney Dis Transpl 2013;24:546-8

How to cite this URL:
Kari JA, Bamagai A, Jalalah SM. Severe acute post-streptococcal glomerulonephritis in an infant. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2019 May 23];24:546-8. Available from: http://www.sjkdt.org/text.asp?2013/24/3/546/111061

   Introduction Top

Acute post-streptococcal glomerulonephritis (APSGN) is the most common cause of post-infectious glomerulonephritis in children. [1] How­ever, it is rare in children below two years of age [2],[3],[4] and that is explained by their low immunogenicity, which results in less robust immune complex formation and, hence, less APSGN. [2],[5]

There is no definite treatment for APSGN apart from penicillin to eradicate group A streptococcal (GAS) carriage. [4] The use of steroid was reported in severe cases in adults, [6],[7] but not in children.

We describe here a 14-month-old girl with typical clinical and laboratory findings of severe APSGN. She was treated with prednisolone that resulted in rapid recovery.

   Case Report Top

A previously healthy 14-month-old girl pre­sented to a private clinic with 4-day history of progressive periorbital puffiness and edema in lower limbs, associated with tea-colored urine that was noticed one day prior to presentation. Urinalysis showed protein of more than 300 mg/dL, numerous RBCs, and negative WBCs. A 24-h urine collection revealed a nephrotic range proteinuria of 2.5 g. She was diagnosed as a case of nephrotic syndrome and referred to our institution for further evaluation.

At presentation, she was a febrile and had generalized edema and hypertension (110/70 mm Hg). There was a history of upper respira­tory tract infection three weeks prior to her first presentation. Further laboratory investi­gations at our institution revealed 8.7 × 10/L of WBCs in blood, with 66.5% neutrophils and 30.3% lymphocytes. Hemoglobin was 9.1 g/dL, hematocrit 27.1%, mean corpuscular volume (MCV) 77.2% fL, and platelet count 1038 × 10/L. Her serum sodium was 135 mmol/L, potassium 5.7 mmol/L, chloride 107 mmol/L, blood urea nitrogen (BUN) 5 mmol/L, creatinine 32 μmol/L, glucose 5.6 mmol/L, calcium 2.32 mmol/L, and phosphate 1.85 mmol/L. Her total protein was 64 g/L, albumin 21 g/L, and complements C3 0.169 g/L (0.75-1.65) and C4 0.216 g/L. The anti-streptolysin O (ASO) titer was highly elevated at 3320 (0-200). Hepatitis profile was negative and urinalysis revealed +4 blood and +3 protein.

Renal ultrasound revealed kidneys of normal shape and size. A true-cut renal biopsy was obtained from the left kidney. A core of renal cortical tissue containing sufficient number of glomeruli was divided and further studied by light microscopy, immunofluorescence, and electron microscopy. The results of the histo­logy studies showed hypercellular glomeruli with endothelial cells' and mesangial cells' proliferation and neutrophils' infiltration. An epithelial cell crescent was observed in one glomerulus. Electron microscopy revealed the presence of numerous subepithelial hump-like deposits [Figure 1] and [Figure 2]; immunofluorescence results demonstrated the presence of immune complexes along the glomerular capillary wall, which were positive for IgG and C3. Therefore, the tissue studies revealed characteristic features compatible with the diagnosis of APSGN.
Figure 1: Low magnification transmission electron micrograph of a glomerulus showing hypercellular tuft with endothelial proliferation and neutrophils' infiltration resulting in obliteration of capillary lumen.

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Figure 2: Transmission electron micrograph showing numerous large hump-like dense deposits located in subepithelial areas (arrows).
CL: capillary lumen, US: urinary space

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The patient was started initially on prednisolone (2 mg/kg/day) before confirming the diagnosis. Then, prednisolone was continued for four weeks, followed by 1.5 mg/kg on alternate days for another four weeks. Gross hematuria resolved within ten days. At a follow-up at eight weeks and five months later, normal blood pressure was recorded (102/60), with completely negative proteinuria and hematuria. There was also increase in the C3 level toward normal level [0.69 (0.75-1.65) g/L], reduction in ASO titter [776 (0-200)], and normalization of serum albumin to 36 g/L.

   Discussion Top

We report a case of APSGN in a 14-month-old girl. APSGN is a rare entity in infants; there are only two confirmed previous reports in the literature: a 14-month-old boy [2] and an 8-month-old girl. [8] Another case was reported in an infant at four months of age [9] early in 1950 when the relation between glomerulonephritis (GN) and streptococcal infection was just being discovered; however, the accuracy of the diagnosis in this report could be doubted. [2] The case in our report further emphasizes the possibility of APSGN presentation in infants with hematuria and/or nephrotic syndrome.

APSGN is a common glomerulonephritis in developing countries. Over 470,000 cases occur annually, resulting in approximately 5000 deaths; 97% of these cases occur in less well-developed countries. [4],[10] It is most common in children aged five to 12 years and uncommon before the age of three years. [1],[2],[3] APSGN can be found in children less than three years old, [1],[2] but rarely in infants.

Recently, Elyas et al reported that pharyngitis has replaced impetigo as the predominant cause of APSGN in Florida, [3] and they attributed it to the changes of the etiological agent for impe­tigo over the last decade, which has affected the incidence, racial distribution, seasonal variation, and severity of APSGN. Similarly, Becquet et al reported upper respiratory infec­tion as the most common predisposing infec­tion in APSGN. [4]

Becquet et al reported also that the latent period from infection to the first symptoms of APSGN was 9.2 ± 8.4 days for the respiratory infections and 9.5 ± 4.2 days for the skin infections. [4] They reported variable clinical presentation: macroscopic hematuria (64%), microscopic hematuria (32%), arterial hyper­tension (64%), mild renal failure (43.7%), and nephrotic syndrome (25%). In addition, 22% of the cases presented with severe symptoms of congestive heart failure, severe hypertension, and/or encephalopathy. [4]

APSGN is a self-limiting disease with complete recovery in the majority of patients. [4] The use of steroid was reported in severe adult cases of APSGN. Results of these studies showed that further progress toward more serious sequels of the disease was significantly decreased by the steroid treatment. [6],[7] The use of steroids in children with severe APSGN was not reported before. In our case, prednisolone was started initially, before diagnosis was confirmed, and it was continued for a total of eight weeks. The treatment with prednisolone in our patient re­sulted in fast improvement and rapid recovery. In conclusion, our case report illustrates that APSGN should be considered in the evaluation of hematuria and nephrotic syndrome in infants, and that the use of steroid in these cases can result in a favorable outcome and rapid recovery.

   References Top

1.Sanjad S, Tolaymat A, Whitworth J, Levin S. Acute glomerulonephritis in children: A review of 153 cases. South Med J 1977;70:1202-6.  Back to cited text no. 1
2.Bingler MA, Ellis D, Moritz ML. Acute post-streptococcal glomerulonephritis in a 14-month-old boy: Why is this uncommon? Pediatr Nephrol 2007;22:448-50.  Back to cited text no. 2
3.Ilyas M, Tolaymat A. Changing epidemiology of acute post-streptococcal glomerulonephritis in Northeast Florida: A comparative study. Pediatr Nephrol 2008;23:1101-6.  Back to cited text no. 3
4.Becquet O, Pasche J, Gatti H, et al. Acute post-streptococcal glomerulonephritis in children of French Polynesia: A 3-year retrospective study. Pediatr Nephrol 2010;25:275-80.  Back to cited text no. 4
5.Adkins B, Leclerc C, Marshall-Clarke S. Neonatal adaptive immunity comes of age. Nat Rev Immunol 2004;4:553-64.  Back to cited text no. 5
6.Jovanovic D, Kovacevic Z, Rabrenovic V, Skataric V. Acute post-streptococcal glomerulonephritis in adults--corticosteroid therapy, yes or no?. Vojnosanit Pregl 2001;58:161-6.  Back to cited text no. 6
7.Raff A, Hebert T, Pullman J, Coco M. Crescentic post-streptococcal glomerulonephritis with nephrotic syndrome in the adult: Is aggressive therapy warranted? Clin Nephrol 2005;63:375-80.  Back to cited text no. 7
8.Li VS, Furnari ML, Garozzo R, Santangelo G, Mollica F. Acute post-streptococcal glomerulonephritis in an 8-month-old girl. Pediatr Nephrol 1993;7:737-8.  Back to cited text no. 8
9.FISON TN. Acute glomerulonephritis in infancy. Arch Dis Child 1956;31:101-9.  Back to cited text no. 9
10.Steer AC, Danchin MH, Carapetis JR. Group A streptococcal infections in children. J Paediatr Child Health 2007;43:203-13.  Back to cited text no. 10

Correspondence Address:
Jameela A Kari
Professor of Pediatrics & Consultant Pediatric Nephrologist, Pediatrics Department, King Abdulaziz University Hospital, P. O. Box 13042, Jeddah 21943
Saudi Arabia
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DOI: 10.4103/1319-2442.111061

PMID: 23640628

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