|Year : 2019 | Volume
| Issue : 1 | Page : 36-40
Pediatric acute poststreptococcal glomerulonephritis: A single-center experience
Sherif Mohamed El-Desoky1, Lujain K I. Al-Sulimani2, Manar T O. Alkhatieb2, Khalid A Alhasan3, Amr S Albanna4, Jameela A Kari1
1 Pediatric Nephrology Center of Excellence; Department of Pediatrics, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia
2 Department of Pediatrics, Children Hospital, Ministry of Health, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Kingdom of Saudi Arabia
3 Department of Pediatric, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia
4 King Abdullah International Medical Research Center, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Kingdom of Saudi Arabia
|Date of Web Publication||17-May-2019|
Jameela A Kari
Pediatric Nephrology Center of Excellence, Department of Pediatrics, King Abdulaziz University, Jeddah
Kingdom of Saudi Arabia
Source of Support: None, Conflict of Interest: None
We evaluated the frequency, severity, and outcome of children with acute poststreptococcal glomerulonephritis (PSGN) by retrospectively reviewing case records of all children diagnosed with PSGN between 2007 and 2015. The diagnosis of PSGN was based on the evidence of recent streptococcal infection, including positive streptococcal culture from the skin or throat, high or rising antistreptolysin O titer (>333 IU/ml), or high anti-deoxyribonuclease B level (>200 IU/ml). In addition, low serum complement 3 level and/or kidney biopsy results were considered. Children with evidence of chronic kidney disease (CKD) were excluded from the study. Sixty-six children (50 boys) with age 8.4 ± 3.4 years were included in the study. Ninety percent of the children had proteinuria and 79% had impaired glomerular filtration rate at presentation. One child had rapidly progressive glomerulonephritis. At 1-year follow-up, 10% of the children had renal impairment, 25% had hypertension, and 40% had proteinuria. As a considerable percentage of patients with PSGN in this series had CKD at 1-year follow-up, careful prolonged follow-up of such patients is advisable.
Keywords: Acute glomerulonephritis, nephritic syndrome, rapidly progressive glomerulonephritis
|How to cite this article:|
El-Desoky SM, I. Al-Sulimani LK, O. Alkhatieb MT, Alhasan KA, Albanna AS, Kari JA. Pediatric acute poststreptococcal glomerulonephritis: A single-center experience. Asian J Pediatr Nephrol 2019;2:36-40
|How to cite this URL:|
El-Desoky SM, I. Al-Sulimani LK, O. Alkhatieb MT, Alhasan KA, Albanna AS, Kari JA. Pediatric acute poststreptococcal glomerulonephritis: A single-center experience. Asian J Pediatr Nephrol [serial online] 2019 [cited 2019 Aug 25];2:36-40. Available from: http://www.ajpn-online.org/text.asp?2019/2/1/36/258563
| Introduction|| |
Acute poststreptococcal glomerulonephritis (PSGN) remains an important cause of pediatric acute kidney injury, particularly in the developing world. However, the short-term outcome is excellent in most cases. End-stage renal disease occurs in <1% of pediatric patients and in a slightly higher percentage of adults. The long-term complications and outcome are less clear, with significant reported variations. Despite evidence that the prevalence of PSGN is decreasing worldwide, it remains the leading cause of glomerulonephritis in children. In the Kingdom of Saudi Arabia (KSA), studies on PSGN in the pediatric population are very limited. However, there are case reports indicating that it could present at an unusual young age or with unusual presentations. In this study, we aimed to evaluate the incidence, severity, and 1-year outcome of children diagnosed with PSGN at one center in KSA.
| Methods|| |
A retrospective cohort study was conducted to evaluate all children who presented to the Pediatric Nephrology Unit at King Abdulaziz University Hospital in KSA with a diagnosis of PSGN between January 2007 and December 2015. Ethical approval was obtained from the local ethical approval committee of the Faculty of Medicine at King Abdul Aziz University, and the study was conducted according to the principles of the Helsinki Declaration. The diagnosis of PSGN was based on evidence of recent streptococcal infection, which included positive streptococcal culture from the skin or throat, high or rising anti-streptolysin O (ASO) titer (>333 IU/mL), or high anti-deoxyribonuclease B level (>200 IU). In addition, low serum complement C3 level and/or kidney biopsy results were considered. Children with evidence of chronic kidney disease (CKD) were excluded as well as children with systemic lupus erythematosus, chronic hepatitis, C3 glomerulopathy, and hereditary angioedema.
Information collected from medical records included a season at disease onset, demographic data, age at presentation, anthropometry, blood pressure, results of renal function tests, urinalysis and kidney biopsy, need for hospitalization, and details of treatment. Patients with rapidly progressive glomerulonephritis, atypical features, or unclear cause of acute nephritis underwent kidney biopsy. Data collected from follow-up visits, scheduled once in 3 months during the 1st year and annually thereafter, included evidence of CKD, including hypertension, proteinuria, and impaired glomerular filtration rate (GFR). Estimated GFR was calculated using the modified Schwartz equation. Hypertension was defined as blood pressure >95th percentile for age, sex, and height.
Supportive management included salt and fluid restriction, and antihypertensive agents, diuretics and renal replacement therapy, as required. Based on unit policy, patients with nephrotic range proteinuria received oral prednisolone, administered at 60 mg/m2/day for 4 weeks and tapered over 12 weeks, while those presenting with rapidly progressive glomerulonephritis received methylprednisolone pulses (600 mg/m2/day for 3 consecutive days) with or without oral (2–3 mg/kg daily for 8 weeks) or intravenous (500 mg/m2 monthly for 6 months) cyclophosphamide.
We used STATA software (StataCorp LLC, 2011, release 12, College Station, TX, USA) for all analyses. Categorical and continuous variables are presented as proportions and mean ± standard deviation values, respectively.
| Results|| |
Sixty-six children (50 boys) with a mean age of 8.4 ± 3.4 years were included, representing 5.3% of 1254 patients attending pediatric nephrology services during the study period. None of the patients had a recurrence of PSGN. Fifty-eight patients (78.8%) were hospitalized and five (7.6%) needed intensive care unit admission for severe presentation, including two (3%) patients with hypertensive encephalopathy or posterior reversible encephalopathy syndrome. Thirty (45.5%) patients presented in winter, 17 (25.8%) in spring, 11 (16.7%) in summer, and the remainder in autumn.
[Table 1] summarize the clinical and laboratory findings at presentation. Hypertension was common; whereas 51 (77.3%) patients had systolic hypertension, 40 (60.6%) had diastolic hypertension, and 36 (54.5%) patients had both. Gross and microscopic (>5 red blood cells/high power field) hematuria were present in 46 (69.7%) and 11 (16.6%) patients, respectively. The diagnosis in 9 patients lacking information on hematuria was based on the evidence of streptococcal infection, hypertension, low complement, and proteinuria. Proteinuria was present in 55 (90.2%) patients and was in nephrotic range (>1 g/m2/day) in 28 (42.4%) children. Leg edema and oliguria were present in 41 (62.1%) and 13 (19.7%) patients, respectively.
|Table 1: General characteristics of included subjects, plus Initial presenting features of patients diagnosed with acute post-streptococcus glomerulonephritis|
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Kidney biopsy, performed in 17 (25.7%) patients, showed no crescents in 9 (52.9%) patients, crescents in <50% of the glomeruli in 7 (41.2%) patients, and crescentic glomerulonephritis in one (5.9%) case. One child presented at 14 months of age with gross hematuria and nephrotic syndrome. Low complement C3 levels, high ASO titer, and characteristics findings on kidney biopsy enabled a diagnosis of PSGN. Following therapy with prednisone, he recovered completely by 8 weeks of follow-up.
Fifty-four (81.8%) children received oral antihypertensive medication (calcium channel blocker, amlodipine) for <6 months, and 46 (69.69%) patients required loop diuretics for <3 months. Immunosuppression included oral prednisolone in 31 (46.96%) patients, intravenous pulse methylprednisolone in 18 (27.3%) cases, and cyclophosphamide orally (n = 6, 9%) or intravenously (n = 1, 1.5%).
Information at 1-year follow-up was available for 47 (71.2%) patients. [Figure 1] shows the rates of CKD, hypertension, proteinuria, and hematuria in these patients.
|Figure 1: Long-term complications during follow-up of patients diagnosed with acute post-streptococcus glomerulonephritis. HTN: Hypertension; CRF: Chronic renal failure define as glomerular filtration rate of <90 ml/min/1.73 m2; Proteinuria: At least +1 urine protein; Hematuria: >5 red blood cells/hpf|
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| Discussion|| |
PSGN represented 5.3% of all children seen at our pediatric nephrology unit, similar to a prior report from King Faisal Specialty Hospital around two decades ago, wherein PSGN contributed to 4% to all pediatric glomerular diseases. The reported prevalence in adults living in KSA is 2.7%–2.9%. PSGN is more prevalent in developing countries compared to developed countries. Carapetis et al. estimated the global burden of PSGN-based on 11 population-based studies and found that the incidence in the developing world was around 24.3/100,000 person-years in adults and 2/100,000 person-years in children, in contrast to 6 and 0.3/100,000 person-years, respectively, in developed regions. PSGN remains the leading cause of nephritis in developing countries, where it might represent 50%–90% of pediatric acute nephritis. Further, the subclinical disease is thought to be 4–19 times as common as symptomatic disease. The epidemiology of PSGN differs from that of acute rheumatic fever. Certain strains of Group A streptococci are either rheumatogenic or nephritogenic; rarely, the two disorders occur together.
The predominance of male sex in our cohort is similar to the report by Dodge et al., in whom boys were affected by PSGN twice as often as girls, for unknown reasons. The median age of presentation in our cohort, 8.4 years, is close to previous observations. Two children presented before the age of 2 years, which is unusual as the streptococcal pharyngitis is uncommon and immune responses are immature, at this age. The diagnosis of PSGN was confirmed in our cohort by evidence of streptococcal infection and low C3 in majority of patients. Nasr et al. proposed the following five clinical and pathologic criteria to diagnose PSGN: (i) recent streptococcal infection, (ii) hypocomplementemia, (iii) light microscopic findings of proliferative glomerulonephritis, (iv) C3-dominant staining pattern on immunofluorescence, and (v) subepithelial hump-shaped deposits on electron microscopy. They suggested that the presence of three of these five features are diagnostic of PSGN with a relatively high degree of confidence. Hypocomplementemia, considered an important criterion for the diagnosis of PSGN, was lacking in 36.4% of patients in our series, potentially due to delayed presentation. This proportion is significantly higher than that reported by Qian et al. in their series of 78 patients of whom three (3.9%) presented with normal complement C3 level.
Preceding bacterial pharyngitis and skin infection were observed in 77.3% and 10.6% of cases, respectively. This is similar to a report by Becquet et al., in which upper respiratory tract infection was the most common infection preceding PSGN. A change in the epidemiology of PSGN was reported from northeast Florida, where the incidence following impetigo had declined, and the peak of incidence of PSGN had shifted from summer to spring and winter. Similarly, most of our patients presented in the winter season.
Nephrotic range proteinuria was observed in 42.4% of our patients, which is much higher than previous observation of only 2%–4% of cases. It is unclear if this reflects referral bias. Kidney biopsy, not considered necessary to make a diagnosis of PSGN, was indicated only if there were atypical features or occurrence of rapidly progressive glomerulonephritis, indicated by sustained rapid increase in serum creatinine and/or severe oliguria. In this study, kidney biopsy was indicated in one-quarter of the patients and revealed some crescentic changes in over half of these cases. While most expert groups do not recommend the use of immunosuppression, the use of corticosteroids in adult patients with severe disease was associated with satisfactory outcomes. Hence, we chose to use immunosuppression with the intent to arrest further inflammation and injury leading to disease progression to advanced CKD.
PSGN is considered a self-limiting disease with complete recovery in the majority. However, a significant proportion of patients in this series had persistent hypertension, proteinuria, and hematuria at 1-year follow-up. This is in contrast to findings from previous studies, such as the Kasahara study in 138 Japanese children with PSGN, that reported normal complement levels by 12 weeks, and resolution of proteinuria and hematuria within 3–4 years. It is possible that many patients in the present cohort comprised severe cases referred to a tertiary care center. Alternatively, the streptococcal infection might have triggered C3 glomerulopathy in a subset of patients who were genetically predisposed by disorders in complement regulation. Limitations of the study include its retrospective, single-center design; relatively small sample size, and short duration of follow-up.
PSGN among children in our geographic area is sporadic and more frequently seen in the winter. Since a considerable proportion of patients with PSGN in our population had evidence of CKD, long-term follow-up is advisable.
| Conclusion|| |
Acute Post streptococcal glomerulonephritis (APSGN) among children in our geographic area is sporadic and more frequently seen in the winter. A considerable percentage of patients with APSGN in our population had evidence of CKD; therefore, long-term follow-up advisable.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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