Acute Poststreptococcal Glomerulonephritis Research Articles

High burden and seasonal variation of paediatric scabies and pyoderma prevalence in The Gambia: A cross-sectional study.

BackgroundScabies is a WHO neglected tropical disease common in children in low- and middle-income countries. Excoriation of scabies lesions can lead to secondary pyoderma infection, most commonly by Staphyloccocus aureus and Streptococcus pyogenes (group A streptococcus, GAS), with the latter linked to acute post-streptococcal glomerulonephritis (APSGN) and potentially rheumatic heart disease (RHD). There is a paucity of data on the prevalence of these skin infections and their bacterial aetiology from Africa.Methodology/Principal findingsA cross-sectional study, conducted over a four-month period that included the dry and rainy season, was conducted to determine the prevalence of common skin infections in Sukuta, a peri-urban settlement in western Gambia, in children <5 years. Swabs from pyoderma lesions were cultured for S. aureus and GAS. Of 1441 children examined, 15.9% had scabies (95% CI 12.2–20.4), 17.4% had pyoderma (95% CI 10.4–27.7) and 9.7% had fungal infections (95% CI 6.6–14.0). Scabies was significantly associated with pyoderma (aOR 2.74, 95% CI 1.61–4.67). Of 250 pyoderma swabs, 80.8% were culture-positive for S. aureus, and 50.8% for GAS. Participants examined after the first rains were significantly more likely to have pyoderma than those examined before (aRR 2.42, 95% CI 1.38–4.23), whereas no difference in scabies prevalence was seen (aRR 1.08, 95% CI 0.70–1.67). Swab positivity was not affected by the season.Conclusions/SignificanceHigh prevalence of scabies and pyoderma were observed. Pyoderma increased significantly during the rainy season. Given the high prevalence of GAS pyoderma among children, further research on the association with RHD in West Africa is warranted.

Extrarenal Immune-Mediated Disorders Linked with Acute Poststreptococcal Glomerulonephritis: a Systematic Review.

Streptococcus A infections have been associated with immune-mediated sequelae including acute glomerulonephritis, acute rheumatic fever, thrombocytopenia, hemolytic anemia, Henoch-Schönlein purpura, arthritis, uveitis, guttate psoriasis, and erythema nodosum. Available reviews do not report the occurrence of acute poststreptococcal glomerulonephritis in association with one of the mentioned conditions. We performed a systematic review of the literature on extrarenal immune-mediated disorders associated with acute poststreptococcal glomerulonephritis. The principles recommended by the Economic and Social Research Council guidance on the conduct of narrative synthesis and on the Preferred Reporting Items for Meta-Analyses and Systematic Reviews were used. We identified 41 original articles, published after 1965, which reported on 52 patients (34 males and 18 females aged from 1.7 to 57years, median 9) affected by acute poststreptococcal glomerulonephritis associated with a further poststreptococcal disease: 29 cases with rheumatic fever (17 males and 12 females aged 3.0 to 57, median 17years), 16 with hematologic diseases such as thrombocytopenia or hemolytic anemia (13 males and 3 females aged 1.8 to 13, median 6.0years) and seven with Henoch-Schönlein syndrome, reactive arthritis or uveitis (4 males and 3 females aged 1.7 to 14, median 7.0years). Patients affected by acute poststreptococcal glomerulonephritis associated with acute rheumatic fever were on the average older (P < 0.05) than patients with acute poststreptococcal glomerulonephritis associated with thrombocytopenia, hemolytic anemia, Henoch-Schönlein syndrome, reactive arthritis or uveitis. Five large case series describing 2058 patients affected by acute poststreptococcal glomerulonephritis did not mention its occurrence in association with further immune-mediated disorders. This systematic review points out that acute poststreptococcal glomerulonephritis can be associated, albeit rarely, with rheumatic fever, thrombocytopenia, hemolytic anemia, Henoch-Schönlein syndrome, reactive arthritis, or uveitis.

A Presumed Etiology of Kawasaki Disease Based on Epidemiological Comparison With Infectious or Immune-Mediated Diseases.

Background: Kawasaki disease (KD) may be associated with infection of unknown pathogen(s). For predicting of the etiology of KD, we evaluated epidemiological characteristics in KD, common infectious diseases and immune-mediated diseases in childhood.Methods: We respectively, reviewed the data of patients with KD, influenza, aseptic meningitis, exanthem subitum (ES), Mycoplasma pneumoniae (MP) pneumonia, acute pyelonephritis (APN), Henoch-Schönlein purpura (HSP), acute poststreptococcal glomerulonephritis (APSGN), and childhood asthma. We compared and interpreted epidemiological data across the groups.Results: In age distribution, KD, APN, and ES showed a similar pattern in that majority of patients were infants or young children, and other diseases showed a relatively even age-distribution which had a peak age, mainly 5–6 years, with bell-shape patterns. In annual-case pattern, there were epidemic years in aseptic meningitis and MP pneumonia, and the fluctuated annual cases were seen in other diseases. The trends of decreasing cases were seen in APSGN, HSP, and childhood asthma in recent years. In seasonal frequency, influenza or aseptic meningitis occurred in mainly winter or summer season, respectively. HSP and APSGN cases had less in summer, and KD, APN, and ES showed relatively even occurrence throughout a year without significant seasonal variations.Conclusions: Our results suggest that KD agents may be associated with normal flora that are influenced by environmental changes, since pathogens of APN and ES could be regarded as normal flora that originate from the host itself or ubiquitously existing human reservoirs.

Systematic Review of Group A Streptococcal emm Types Associated with Acute Post-Streptococcal Glomerulonephritis.

Acute post-streptococcal glomerulonephritis (APSGN) is a postinfectious immune-mediated kidney disease associated with group A Streptococcus (GAS). The prevalence of APSGN varies within and between countries and is influenced by socioeconomic, host, and bacterial factors. The disease is more prevalent in developing countries and resource-poor settings of developed countries, such as the Indigenous populations residing in tropical Australia. The M-protein is a universally present GAS surface antigen that is the focus of molecular typing and vaccine research. Early reports suggested that some M-proteins (emm types) are more likely to cause APSGN than others. Here, we present the first systematic review of the global distribution of APSGN-associated GAS emm types. There were 46 emm types among the 676 cases described in 15 reviewed articles. Only 43% APSGN cases would have had theoretical coverage from the experimental M protein-based GAS vaccine. Vaccine coverage was higher in regions such as North America (97%) and the United Kingdom (98%) than Africa (67%) and Australia (38%). Variable vaccine coverage against APSGN- associated emm types highlights the need for further research into this disease, particularly in settings of poverty, where APSGN prevalence is higher. Three GAS emm types (emm49, emm60, and emm55) consistently occur in APSGN cases around the world. Future studies would therefore benefit from examining the genomic epidemiology of these emm types to unravel potential markers of APSGN.

Posterior Reversible Encephalopathy Syndrome in an Adolescent with Acute Poststreptococcal Glomerulonephritis

AbstractPosterior reversible encephalopathy syndrome (PRES) is a rare but severe complication of acute poststreptococcal glomerulonephritis (APSGN) in children. A 13-year old boy was diagnosed with APSGN based on clinical, laboratory, and renal biopsy findings. On the third hospital day, his blood pressure (BP) was 130/80 mm Hg on antihypertensive and diuretic treatment. He then developed confusion, blurry vision, and changes in speech and behavior. Over the next few hours, his BP increased to 160/90 to 170/90 mm Hg (99th percentile, 135/90 mm Hg). Magnetic resonance imaging of the brain revealed prominent hyperintensities in the right temporal, right temporo-occipital, and right parieto-occipital gray, and white matter on T2-weighted and fluid-attenuated inversion recovery images, compatible with PRES (posterior reversible encephalopathy syndrome). Electroencephalography showed generalized epileptiform activity. After treatment with anticonvulsant and additional antihypertensive medications, his clinical symptoms and neuroimaging findings were resolved. Posterior reversible encephalopathy syndrome should be considered in the differential diagnosis of patients with APSGN developing sudden neurological symptoms without severe increases in BP.

A Controlled Human Infection Model of Group A Streptococcus Pharyngitis: Which Strain and Why?

Group A Streptococcus (GAS) is a major cause of global infection-related morbidity and mortality. A modern controlled human infection model (CHIM) of GAS pharyngitis can accelerate vaccine development and pathogenesis research. A robust rationale for strain selection is central to meeting ethical, scientific, and regulatory requirements. Multifaceted characterization studies were done to compare a preferred candidate emm75 (M75) GAS strain to three other strains: an alternative candidate emm12 (M12) strain, an M1 strain used in 1970s pharyngitis CHIM studies (SS-496), and a representative (5448) of the globally disseminated M1T1 clone. A range of approaches were used to explore strain growth, adherence, invasion, delivery characteristics, short- and long-term viability, phylogeny, virulence factors, vaccine antigens, resistance to killing by human neutrophils, and lethality in a murine invasive model. The strains grew reliably in a medium without animal-derived components, were consistently transferred using a swab method simulating the CHIM protocol, remained viable at -80°C, and carried genes for most candidate vaccine antigens. Considering GAS molecular epidemiology, virulence factors, in vitro assays, and results from the murine model, the contemporary strains show a spectrum of virulence, with M75 appearing the least virulent and 5448 the most. The virulence profile of SS-496, used safely in 1970s CHIM studies, was similar to that of 5448 in the animal model and virulence gene carriage. The results of this multifaceted characterization confirm the M75 strain as an appropriate choice for initial deployment in the CHIM, with the aim of safely and successfully causing pharyngitis in healthy adult volunteers.IMPORTANCE GAS (Streptococcus pyogenes) is a leading global cause of infection-related morbidity and mortality. A modern CHIM of GAS pharyngitis could help to accelerate vaccine development and drive pathogenesis research. Challenge strain selection is critical to the safety and success of any CHIM and especially so for an organism such as GAS, with its wide strain diversity and potential to cause severe life-threatening acute infections (e.g., toxic shock syndrome and necrotizing fasciitis) and postinfectious complications (e.g., acute rheumatic fever, rheumatic heart disease, and acute poststreptococcal glomerulonephritis). In this paper, we outline the rationale for selecting an emm75 strain for initial use in a GAS pharyngitis CHIM in healthy adult volunteers, drawing on the findings of a broad characterization effort spanning molecular epidemiology, in vitro assays, whole-genome sequencing, and animal model studies.

DIAGNOSTIC PROBLEMS IN A PATIENT WITH NEPHRITIC SYNDROME

Acute nephritic syndrome is a collection of clinical symptoms due to a sudden decrease in glomerular filtration rate (GFR) accompanied by water and salt retention, positive erythrocytes, erythrocytes cast and albumin in urinalysis. Information about nephritic syndrome is important to know for accurate diagnose and treatment. A 13-year-old adolescent with nephritic syndrome has been reported with rapid progressive glomerulonephritis (RPGN). He has been treated with corticosteroid, antibiotic, immunosuppressant, antihypertensives, and diuretic followed by improvement of clinical condition and laboratory of the patient. Biopsy was done for definitive diagnosis. The biopsy results showed a picture of IgA nephropathy (IgAN) and resolving stage of acute poststreptococcal glomerulonephritis (APSGN). Acute poststreptococcal glomerulonephritis (APSGN) was suspected due to finding of apparent hematuria, swollen and acute renal failure after streptococcal infection. To support the diagnosis, evidence of laboratory streptococcal infections and low levels of C3 complement are needed. Whereas IgAN known by examining the IgA serum. Management of patients were including supportive and symptomatic therapy. Giving corticosteroids or cytotoxic agents is needed for RPGN therapy. The prognosis is generally good, with improvement more than 90% of cases. Long-term observation is needed to observe the possibility of the disease being chronic. In this case, the symptoms and results of the laboratory examination were in accordance with the results of the biopsy, namely IgAN and resolving APSGN. Immediate and proper management provides an improvement in general and laboratory conditions.

Clinical Audit on Management of Acute Poststreptococcal Glomerulonephritis in Children Admitted to Assiut University Children Hospital

Background: acute poststreptococcal glomerulonephritis (APSGN) is the most common glomerulopathy among children in our community. Aim: to assess degree of adherence of medical physicians to Assiut University Children Hospital protocol for management of acute poststreptococcal glomerulonephritis. Patients and Methods: fifty eight patients with APSGN admitted to Assiut University Children Hospital were included in this study. The unit´s protocol wasfollowed for the management of these cases. Results: evaluation of the studied patients were done perfectly except for 12% of patients not asked about symptoms suggestive ofhypertension (HTN) and 14% not checked for oliguria and some laboratory investigations with less importance in diagnosis. Management was perfectly done except for general and supportive measures including daily weighting, calculating fluid input and output, which showed some defect. Putting patients on blood pressure percentiles was not done at all. Conclusion: evaluation of the APSGN patients includes taking proper history of nephritic symptoms and careful clinical examination. Main treatment includes monitoring fluid status and HTN, and proper management to avoid complications. Recommendation: stress on general and supportive measures including weighting patients daily, calculating fluid input and output, checking patients for oliguria, and follow up proper dietary measures especially for hypertensive and hyperkalemic patients. Stress in putting patients on blood pressure levels according to age and height percentiles for accurate detection of HTN.

Clinical Audit on Management of Acute Poststreptococcal Glomerulonephritis in Children Admitted to Assiut University Children Hospital

Clinical Audit on Management of Acute Poststreptococcal Glomerulonephritis in Children Admitted to Assiut University Children Hospital

Post-Streptococcal Acute Glomerulonephritis in a 7- Year-Old Boy: Islamic Perspective on the Prevention of the Disease

Acute glomerulonephritis is inflammation of the kidney mediated by an immunological process. Group A βhaemolytic streptococcal infection is common in children aged 5-12 years old and can lead to acute poststreptococcal glomerulonephritis which may develop after recovery from a streptococcal throat infection or skin infection (impetigo). 97% of cases occur in less developed country. The incidence has decreased in industrialized nations due to improved hygienic condition. A 7-year-old boy, the eldest child of 4 siblings from a divorced parent, was admitted to Hospital Tengku Ampuan Afzan Kuantan in March 2015 due to facial puffiness for 1 day duration. There was also dark colored urine. Patient had history of productive cough for 3 days and fever for 2 days. Father also complained of skin ulcer on patient’s right feet for about 1 month. There was hypertension (198/80 mmHg). Urine examination showed hematuria, proteinuria, and leucocyturia. Anti-streptolysin O titre was high (1:800) and complement C3 level was low (0.29). Patient was treated with antibiotic, diuretic, and anti-hypertension. The child is entrusted to the parents who will be called by the Almighty, who will ask them about this trust. Parents play vital role in maintaining and promoting the good health of the children. Parents should also pay attention to cleanliness, personal hygiene and the prevention of disease, especially infection. Islam paid attention to washing hands and cleansing the air inhaled into the lungs by regular cleaning of the nose as part of ablution before performing prayer. Parents are responsible in preserving life, health and growth of their children. Caring for cleanliness and hygiene is not only deemed a good habit in Islam but also rendered into rituals that constitute part of the faith itself.

Post-streptococcal glomerulonephritis: Some reduction in a disease of disparities.

A retrospective Auckland-wide (total population approximately 1.4 million) study of hospital admissions from 2007 to 2015 was conducted to assess trends in admissions for acute post-streptococcal glomerulonephritis (APSGN) in children aged 0-14 years. International Statistical Classification of Diseases (ICD10) discharge codes were used to identify potential cases of APSGN, and electronic clinical records and laboratory data were compared with established case definitions for definite or probable APSGN. A total of 430 cases of APSGN were identified (definite n = 337, probable n = 93), with a mean annual incidence of 15.2/100 000 (95% confidence interval (CI) 14.9-15.6). Incidence (0-14 years) was 17 times higher in Pacific peoples (50.2/100 000, 95% CI 48.6-51.8) and almost 7 times higher in Māori (19.6/100 000, 95% CI 18.6-20.7) than European/other populations (2.9/100 000, 95% CI 2.7-3.1). Multivariate analysis found ethnicity, deprivation, male gender, age (peak 3-8 years) and season (summer/autumn) to be associated with admission risk. Admission rates showed a significant change of -9.0% (95% CI -10.4, 7.4%) per year, with 2011 being an exception. Low C3 complement, hypertension, elevated streptococcal titres, oedema and heavy proteinuria were present in 94, 65, 67, 52 and 49% of cases, respectively. Relying on ICD10 codes without further review of clinical notes would result in an overcount of cases by 25%. There is severe disparity in APSGN admission rates, with a disproportionate burden of disease for Pacific and Māori children and those living in deprived circumstances. Rates trended downward from 2007 to 2015.

Profile and outcomes of children presenting with infection-related glomerulonephritis

Poststreptococcal acute glomerulonephritis (PSGN) was reported as the most common cause of GN in children. There has been, however, a marked shift in epidemiology in recent years with the decline in poststreptococcal cases. Various other bacteria and rarely viral and fungal infections are associated with GN. More cases are now being reported with ongoing infection at the time GN is diagnosed. Therefore, the term infection-related GN (IRGN) is now being used increasingly. We describe the clinical profile and outcomes of children presenting with IRGN at a tertiary care center in the past 1 year. 5 children presented with features of GN. Only 1 of the 5 had the course typically described in PSGN. Two patients also had a post-infectious course but with some unusual features. Another patient had an ongoing systemic infection in the form of pneumonia at the time of onset of features GN, while our fifth patient developed an infection-related GN with dengue illness.

β-HEMOLYTIC STREPTOCOCCUS GROUP A CARRIER IN CHILDREN: THE PROBLEM OF DIFFERENTIAL DIAGNOSIS

Streptococcal infection is characterized by a variety of manifestations from asymptomatic carriage of the pathogen to manifest forms. Recently, in addition to the bacteriological method for confirming streptococcal etiology, the rapid test for β-hemolytic streptococcus group A is increasingly being used. Isolation of streptococci does not always indicate their involvement in pathology, quite often a person is a healthy carrier of the pathogen. The share of carrier is 10—28% of cases. However, in the practical activities of a physician, positive tests for β -hemolytic streptococcus group A (rapid test or bacteriological culture) are often treated as acute streptococcal infection even in children without any clinical manifestations of acute tonsillopharyngitis and, as a result, antibiotic therapy is prescribed. For differential diagnosis, a correct evaluation of epidemiological and clinical data with a mandatory serological test — the determination of ASO in paired sera with an interval of 7—10 days is required. Absence of an increase in antibodies indicates carrier. b-hemolytic streptococcus group A carriers in most cases do not need antibiotic therapy. However, if there is a history or risk of developing rheumatic fever, acute poststreptococcal glomerulonephritis, antibiotic therapy is necessary. The authors proposed an algorithm for managing patients with the release of b-hemolytic streptococcus group A from the oropharynx.

A case of glomerulonephritis showing remarkable segmental extracapillary proliferation; Is this a new category disease or not?

Background: Significant capillary proliferation is common in post-streptococcal acute glomerulonephritis (PSAGN) after streptococci and is a prognostic disease. Focal segmental glomerulosclerosis is a disease characterized by segmental sclerosis although it may have a poor prognosis Case Presentation: A 50-year-old man with nephrotic syndrome underwent renal biopsy, which showed marked endocapillary proliferation due to enlarged vascular endothelial cells and infiltration of lymphocytes. However, there was no mesangial cell proliferation, mesangial matrix increase, or crescent formation. Electron microscopy showed fusion of podocytes without any electron-dense deposits. Immunostaining for CD68 and CD3 was positive, and the presence of macrophages and T-cells was suggested. Steroid therapy, including pulse therapy, was performed, and then cyclosporine was added to steroid therapy. Although urinary protein decreased, his renal function did not respond well to steroid therapy, and the patient initiated dialysis 2 years later. Conclusions: We report this case considering that it was nephritis of unknown origin showing segmental endocapillary proliferation that had not been recognized previously.

Pediatric post-streptococcal glomerulonephritis: Clinical and laboratory data.

Acute post-streptococcal glomerulonephritis (APSGN) is the most common post-infectious glomerulonephritis in childhood. The aim of this study was therefore to identify the possible risk factor(s) responsible for decreased glomerular filtration rate (GFR) in APSGN. The data of patients followed up with a diagnosis of APSGN in the Pediatric Nephrology Clinic of Gaziantep University Hospital between October 2014 and October 2016 were retrospectively evaluated. The total number of subjects was 75 (male/female, 42/33) with a mean age of 8.20 ± 3.25 years. The most common presentations were edema (86.7%), macroscopic hematuria (82.7%) and hypertension (73.3%, n = 55). On laboratory examination, 28 children (37.3%) had hypoalbuminemia, 58 (77.3%) had proteinuria, 20 (26.7%) had increased C-reactive protein (CRP), while 74 (98.7%) and 12 (16%) had decreased complement (C)3 and C4, respectively. The number of children with GFR <90 mL/min/1.73 m2 was 22 (29.3%). The risk of decreased GFR was significantly higher in patients with increased CRP (P = 0.001; OR, 3.58), hypoalbuminemia (P = 0.006; OR, 4.83), and decreased C4 (P = 0.010; OR, 11.53). Additionally, white blood cell (WBC) count, neutrophil count, and neutrophil/lymphocyte ratio (NLR) were significantly higher (P = 0.02, P = 0.006, P = 0.004, respectively) in patients with low GFR. Although the prognosis of APSGN in children is good, severe systemic complications and renal failure may develop during the follow-up period. Decreased C4, presence of hypoalbuminemia, and increased inflammatory markers (WBC, CRP, neutrophil count and NLR) might be possible risk factors for severity of renal involvement. Decreased C4, in particular, may be a risk factor for decreased GFR in those children.

Study of some electrocardiographic and echocardiographic data in toddelers and children with primary glomerulonephropathy

Background and Objective: Primary glomerulonephropathy mainly acute poststreptococcal glomerulonephritis (APSGN) is the most common cause of hypertensive heart failure in the pediatric age as APSGN may involve various systems including cardiovascular system. There are few research publications on electrocardiographic (ECG) and echocardiographic (two-dimensional [2D]-echo) data in childrenwith primary glomerulonephropathy. The aim is to study some ECG and 2D-echo data in Egyptian pediatric patients with ANS). Subjects and Methods: Sixty children with ANS were included and subjected to clinical, laboratory, ECG for corrected QT (QTc) interval and 2D echocardiographic study on admission and repeated at 6 and 12 weeks using GE Vivid 7 (GE Medical System, Horten, Norway with a 3.5-MHz multifrequency transducer) to measure left ventricular ejection fraction (LVEF), left atrium to aorta ratio and E/A ratio. Results: Prolonged QTc interval was reported in 22 patients (37%), of whom 18 had hypertension. Fourteen of the 60 children had LVEF 2 and E/A ratio more than two. LVEF became within normal values by 1½ month (6 weeks) in 12 patients, two become normal by three months of follow-up. Four (28.6%) from 14 children with low LVEF had normal arterial blood pressure. All the 14 children had completely recovered on follow-up period of 3 months. Conclusion: Changes in ECG and echo data which were reported in the acute phase of ANS appear to be temporary (transient) as they became within normal values in almost all children by 12 weeks period of follow-up. Although elevated blood pressure was the most common etiology of congestive heart failure in children with ANS, the impact of primary myocardial functional disturbance could also be put into consideration.

Pediatric Pharyngitis : Etiology and Management Approaches

Background: Acute pharyngitis is infection of the tonsils and the pharynx and is very common among children and adolescents. They can be viral or bacterial in origin. The causative organism varies from geography, age, and season of infection. Although viral pharyngitis is more common, the complications of bacterial pharyngitis can be severe, and thus proper identification of the cause and management is very important. Methodology: We conducted this review using a comprehensive search of MEDLINE, PubMed, and EMBASE, January 1985, through February 2017. The following search terms were used: acute pharyngitis, pediatric pharyngitis, bacterial infection, streptococcus pharyngitis, viral pharyngitis, rapid strept test, throat culture Aim: In this review, we aim to study the common causative organisms of acute pharyngitis among pediatric population and study the best course of management that must be followed. Conclusion: Acute pharyngitis is a relatively common disease, with a viral cause most likely. An etiology due to a streptococcal infection is a major concern and should always be ruled out due to its association with significant morbidity and mortality. Long-term sequelae of streptococcal pharyngitis include rheumatic fever, rheumatic heart disease, and acute post-streptococcal glomerulonephritis

A case of acute poststreptococcal glomerulonephritis following surgery for biliary atresia in a patient with biliary cirrhosis

A case of acute poststreptococcal glomerulonephritis following surgery for biliary atresia in a patient with biliary cirrhosis

Poststreptococcal glomerulonephritis with atypical hemolytic uremic syndrome: An unusual presentation

A 14-year-old female presented with oliguric dialysis requiring kidney injury due to acute poststreptococcal glomerulonephritis (PSGN) with hypertension strongly suggestive of atypical hemolytic uremic syndrome (aHUS) with microangiopathic hemolytic anemia and elevated factor H antibody levels. Renal biopsy revealed crescentic glomerulonephritis with typical subepithelial, intramembranous and mesangial electron-dense deposits (humps) on electron microscopy. She was treated with glucocorticoids following which she recovered, remained dialysis free and her Factor H antibody levels and depressed complement 3 levels normalized. PSGN-associated HUS has rarely been described, with this patient being the 11th case reported, to the best of our knowledge. This case is unique as we describe the course and management of the first patient with PSGN-associated HUS in the era of eculizumab, without eculizumab, and plasmapheresis. This patient presented with clinical and histological features of PSGN as well as anemia and thrombocytopenia consistent with aHUS. Given that these diseases are both mediated through the alternate complement pathway, it is tempting to speculate that blockade of the terminal complement pathway through the use of eculizumab might improve outcomes. Temporally, the hematological parameters in our patient seemed to improve soon after treatment was initiated; however, none of the prior cases in the literature experienced any long-term hematological issues, suggesting that supportive management can be a reasonable alternative.

Group A streptococcal pharyngitis: Immune responses involved in bacterial clearance and GAS-associated immunopathologies.

Streptococcus pyogenes, the Group A Streptococcus (GAS), is the most common cause of bacterial pharyngitis in children and adults. Innate and adaptive host immune responses are fundamental for defense against streptococcal pharyngitis and are central to the clinical manifestation of disease. Host immune responses also contribute to the severe poststreptococcal immune diseases that constitute the major disease burden for this organism. However, until recently, little was known about the host responses elicited during infection. Cellular mediators of innate immunity used during host defense against GAS include epithelial cells, neutrophils, macrophages, and dendritic cells (DCs), which are reported to secrete a number of soluble inflammatory mediators, such as antimicrobial peptides (AMPs); eicosanoids, including PGE2 and leukotriene B4 (LTB4 ); chemokines; and proinflammatory cytokines. Th1 and Th17 responses play significant roles in adaptive immunity in both murine models of GAS pharyngitis and in human tonsil tissue. A number of inflammatory complications are associated with GAS pharyngitis, which can lead to chronic disease in patients. These include scarlet fever, tonsillar hypertrophy, and sleep apnea, as well as postinfectious sequelae, such as acute rheumatic fever (ARF), poststreptococcal glomerulonephritis, and guttate psoriasis (GP). This review aims to present the current state of knowledge on innate and adaptive immune responses elicited during GAS pharyngitis, mechanisms by which GAS evades these responses, the emerging role of the pharyngeal microbiota, and how the interplay among these factors can influence the outcome of infection and inflammation-related complications.