Cases Of Acute Post-streptococcal Glomerulonephritis Research Articles

Glomerulonephritis in primary biliary cholangitis in China: an important complication.

Extrahepatic manifestations in patients with primary biliary cholangitis (PBC) are frequently observed recently. We aimed in this study to explore the clinicopathological characteristics and prognosis of glomerulonephritis in patients with PBC. Consecutive PBC patients admitted to Peking Union Medical College Hospital from January 2002 to May 2019 were retrospectively enrolled. PBC patients with other autoimmune diseases which may have nephritis were excluded. Structured interview, systemic rheumatologic examination, and laboratory tests were conducted for each patient. Literature about patients with PBC and glomerulonephritis was reviewed and summarized. Among the 330 PBC patients enrolled, glomerulonephritis were identified in 10 patients (3.0%). Eight (80.0%) were females and 2 (20.0%) were males. The mean age was 58.4 ± 9.5years old. Membranous nephropathy (MN) was revealed in 4 patients, IgA nephropathy (IgA N) in 2 patients, minimal change disease (MCD) in 2 patients, mesangial proliferative glomerulonephritis in 1 patient, and renal amyloidosis in 1 patient. Compared to the literature reviewed, 10 cases of MN, 1 case of MCD, 1 case of IgA N, and 1 case of acute poststreptococcal glomerulonephritis (APSGN) were observed. Glomerulonephritis may not be a well-recognized feature of PBC and is not a rare complication and deserved to be routinely screened in clinical practice. As MN is the most common form of glomerulonephritis in PBC patients and PBC can be asymptomatic at an early stage, patients presented with MN should be screened for PBC, so to avoid cirrhosis. Key Points • Patients with primary biliary cholangitis (PBC) can be complicated with glomerulonephritis, and membranous nephropathy (MN) is the most common form.

Myocardial dysfunction and thrombocytopenia, rare manifestations of acute post-streptococcal glomerulonephritis: case report

In this case report, we present two rare cases of acute post-streptococcal glomerulonephritis with unusual manifestations in the form of myocardial dysfunction and thrombocytopenia. APSGN typically follows Streptococcal infections and is characterized by inflammation of glomeruli. These cases, however, exhibited additional complexities. The first case involved a 12-year-old male with fever, shortness of breath, and cough. He presented with pedal edema, pallor, and hypertension. Laboratory findings revealed thrombocytopenia, anemia, and decreased C3 levels, while echocardiography indicated grade-3 diastolic dysfunction. The second case featured a 5-year-old female with icterus, fever, and body swelling. She had a palpable liver, pleural effusion, and thrombocytopenia. Both cases were diagnosed with APSGN, congestive heart failure, and thrombocytopenia. Thrombocytopenia is a rare finding in APSGN, and its etiology remains debated. Treatment included decongestive therapy and antihypertensive medication. Notably, thrombocytopenia in both cases improved without specific intervention, challenging the necessity of steroids or IVIg therapy. This report illuminates on the atypical presentation of APSGN, highlighting the potential coexistence of glomerulonephritis and thrombocytopenia. It underscores the need for further research to better understand this association and determine appropriate treatment protocols. These cases emphasize the importance of considering diverse clinical manifestations in the context of APSGN, calling for a broader understanding of this condition.

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

A Cluster of Cases of Acute Poststreptococcal Glomerulonephritis in a Family

Acute post streptococcal glomerulonephritis (APSGN) remains a common cause of acute nephritis in children. Acute poststreptococcal glomerulonephritis usually occurs as sporadic cases, occasionally an unusually high incidence of acute nephritis has been recorded. In our country we need to recognize, document and analyze epidemic attacks of acute post streptococcal glomerulonephritis. Then we can plan for it’s prevention. We have reported here a cluster of cases of APSGN prevailed in a family of a rural setting of southeast part of Bangladesh which was admitted in a subdistrict level hospital and also noticed that total admitted cases of APSGN in that hospital was more during the month of notification in comparison to other months but were not able to identify all the cases of APSGN prevailing in that area. Prophylactic antibiotic treatment is advisable in epidemic conditions and to household contacts of index cases in communities where the prevalence of the disease is high. It is expected that there are some cluster of cases of APSGN in different regions of our country during different period that is to be notified. Public awareness regarding prevention and early treatment of scabis is required.DOI: http://dx.doi.org/10.3329/cmoshmcj.v13i3.21034

Acute Poststreptococcal Glomerulonephritis: A Manifestation of Immune Reconstitution Inflammatory Syndrome

Immune reconstitution inflammatory syndrome (IRIS) is a well-described complication of initiation of highly active antiretroviral therapy in HIV-infected patients. As the immune system recovers, an inappropriate inflammatory response often occurs that causes significant disease. It is most commonly seen in patients naïve to therapy with CD4+ T-lymphocyte counts <100 cells/cmm and usually presents as a flare of mycobacterial, cytomegalovirus, or herpes zoster infections. Less commonly, this syndrome occurs in response to noninfectious triggers and results in autoimmune or malignant disease. Here we present the first case of acute poststreptococcal glomerulonephritis associated with varicella zoster virus and IRIS in an adolescent with perinatally acquired HIV and hepatitis C virus infections. Our patient was not naïve to therapy but was starting a new regimen of therapy because of virologic failure and had a relatively high CD4+ T-lymphocyte count. This case report indicates that IRIS remains a concern after initiation of a new highly active antiretroviral therapy regimen in HIV-infected patients with high viral loads, even in the presence of CD4+ T-lymphocyte counts >100 cells/cmm. It may present as infectious, malignant, or autoimmune conditions including poststreptococcal glomerulonephritis.

Genome sequence of a nephritogenic and highly transformable M49 strain of Streptococcus pyogenes.

The 1,815,783-bp genome of a serotype M49 strain of Streptococcus pyogenes (group A streptococcus [GAS]), strain NZ131, has been determined. This GAS strain (FCT type 3; emm pattern E), originally isolated from a case of acute post-streptococcal glomerulonephritis, is unusually competent for electrotransformation and has been used extensively as a model organism for both basic genetic and pathogenesis investigations. As with the previously sequenced S. pyogenes genomes, three unique prophages are a major source of genetic diversity. Two clustered regularly interspaced short palindromic repeat (CRISPR) regions were present in the genome, providing genetic information on previous prophage encounters. A unique cluster of genes was found in the pathogenicity island-like emm region that included a novel Nudix hydrolase, and, further, this cluster appears to be specific for serotype M49 and M82 strains. Nudix hydrolases eliminate potentially hazardous materials or prevent the unbalanced accumulation of normal metabolites; in bacteria, these enzymes may play a role in host cell invasion. Since M49 S. pyogenes strains have been known to be associated with skin infections, the Nudix hydrolase and its associated genes may have a role in facilitating survival in an environment that is more variable and unpredictable than the uniform warmth and moisture of the throat. The genome of NZ131 continues to shed light upon the evolutionary history of this human pathogen. Apparent horizontal transfer of genetic material has led to the existence of highly variable virulence-associated regions that are marked by multiple rearrangements and genetic diversification while other regions, even those associated with virulence, vary little between genomes. The genome regions that encode surface gene products that will interact with host targets or aid in immune avoidance are the ones that display the most sequence diversity. Thus, while natural selection favors stability in much of the genome, it favors diversity in these regions.

Acute hypocomplementemic post-infectious glomerulonephritis as a complication of sinus-related orbital cellulitis: case report

Group A beta-hemolytic streptococcus is the most common agent implicated in post-infectious acute glomerulonephritis. We report a case of acute poststreptococcal glomerulonephritis associated with sinus-related orbital abscess in an 11-year-old boy treated with surgical drainage and intravenous ceftriaxone and clindamycin. Twelve days after supportive measures, renal function was normalized. We also discuss this potentially severe nonsuppurative complication of orbital cellulitis caused by group A beta-hemolytic streptococcus.

Streptococcal pyrogenic exotoxin B antibodies in a mouse model of glomerulonephritis

Streptococcal pyrogenic exotoxin B is an extracellular cysteine protease. Only nephritis-associated strains of group A streptococci secrete this protease and this may be involved in the pathogenesis of post-streptococcal glomerulonephritis. Mice were actively immunized with a recombinant protease inactive exotoxin B mutant or passively immunized with exotoxin B antibody. Characteristics of glomerulonephritis were measured using histology, immunoglobulin deposition, complement activation, cell infiltration, and proteinuria. None of the mice given bovine serum albumin or exotoxin A as controls showed any marked changes. Immunoglobulin deposition, complement activation, and leukocyte infiltration occurred only in the glomeruli of exotoxin B-hyperimmunized mice. One particular anti-exotoxin B monoclonal antibody, 10G, was cross-reactive with kidney endothelial cells and it caused kidney injury and proteinuria when infused into mice. This cross-reactivity may be involved in the pathogenesis of glomerulonephritis following group A streptococcal infection.

Is the nephritogenic antigen in post-streptococcal glomerulonephritis pyrogenic exotoxin B (SPE B) or GAPDH?

Acute glomerulonephritis can follow infection by group A streptococci. An immune-complex pathogenesis is accepted, but the causative antigen(s) is still controversial. In recent years, 2 streptococcal antigens, the cationic cysteine proteinase exotoxin B (SPE B) and the plasmin receptor, a glyceraldehyde phosphate dehydrogenase (Plr, GAPDH) have attracted attention because: (1) they were localized in glomeruli in patients with acute post-streptococcal glomerulonephritis (APSGN); and (2) serum antibody to these antigens was associated with nephritogenic streptococcal infections. To date, putative nephritogens were always tested independently. Here, the relevance of SPE B and GAPDH was evaluated in the same renal biopsies and serum samples of well-defined APSGN patients. Renal biopsies (17 patients) and serum samples (53 patients) with APSGN and appropriate controls were examined. Immunofluorescent staining of frozen sections was performed using specific antibodies to SPE B and GAPDH. Serum antibodies were investigated by both enzyme-linked immunosorbent assay (ELISA) and Western blot methodology. Glomerular deposits of SPE B were demonstrated in 12/17 APSGN biopsies, and 2 cases were borderline; circulating antibodies were found in all instances (53/53 patients). Glomerular deposition of GAPDH was detected in 1/17 biopsies, and 2 cases were borderline; circulating antibodies were found in 5/47 patients. In 31 control biopsies, only weak staining for each antigen was found in 2 cases. In this study, glomerular deposits of and antibody response to zymogen/SPE B are more consistently present in APSGN than deposits and antibody response to GAPDH. Zymogen/SPE B is likely to be the major antigen involved in the pathogenesis of most cases of APSGN.

Five cases of acute poststreptococcal glomerulonephritis with acute renal failure and nephrotic syndrome

During the period of 27 years from 1974 to 2000, acute episodes were studied retrospectively in 130 patients under 14 years of age with poststreptococcal acute glomerulonephritis(PSAGN). PSAGN cases have a variable clinical presentation from asymptomatic to severe oliguric acute renal failure(ARF). Proteinuria is nearly always present, but is less than 3.5 g/day. Nephrotic syndrome(NS) is not commonly observed in PSAGN. Among 130 patients, 5 cases had NS with ARF. The present study investigated a spectrum of NS with ARF and the significance of clinical features in PSAGN.

Acute poststreptococcal glomerulonephritis following circumcision

A case of acute poststreptococcal glomerulonephritis following circumcision is presented. An 11-year-old boy was subjected to ritual circumcision, which was complicated by the infection of the wound and development of oliguria, edema, hematuria and hypertensive encephalopathy 2 weeks later. The diagnosis of poststreptococcal glomerulonephritis was established upon the isolation of Streptococcus pyogenes, increased antistreptolysin O (ASTO) and antiDNAse B titers and hypocomplementemia. The clinical course was uneventful with resolution of the nephritic signs, normalization of the complement and clearance of the urinary abnormalities. To the best of our knowledge this is the first case of poststreptococcal glomerulonephritis following infection of the circumcision wound.

The occurrence of nephritis plasmin binding protein (SPEB) in the extracellular products of group C nephritogenic Streptococcus zooepidemicus.

While the vast majority of cases of acute post-streptococcal glomerulonephritis (PSGN) in man are caused by infection with group A streptococci (GAS) there have been well-documented instances associated with non-GAS, in particular with the zoonotic group C Streptococcus zooepidemicus1, 2. Recent evidence (similiar molecular weight, serological response and localisation in biopsies) indicates that the nephritis strain-associated protein (NSAP)6, Vogt’s cationic protein7 and nephritis plasmin binding protein (NBPB)5 are in reality the same protein and identical to Elliot’s streptococcal proteinase3 or streptococcal pyrogenic exotoxin B (SPEB). These proteins are clearly distinct from streptokinase4. To date SPEB has not been reported in non-group A pyogenic streptococci. We have investigated both nephritogenic and apparently non-nephritogenic isolates of S. zooepidemicus for the presence of NPBP/SPEB.

Terminal complement complexes in acute poststreptococcal glomerulonephritis

Activation of the complement cascade occurs in most cases of acute poststreptococcal glomerulonephritis (APSGN) and results in the formation of the terminal complement complexes (TCC). To examine the possible role of TCC in the pathogenesis of glomerular injury in APSGN, we studied 30 patients with the clinical diagnosis of APSGN. All patients had an elevated plasma SC5b-9 concentration at the onset of clinical nephritis. Serial plasma concentrations showed an inverse linear relationship with time after onset of clinical disease (r = -0.59, P = 0.0008), while plasma C3 concentrations showed a positive linear relationship (r = 0.78, P = 0.0001). Renal biopsies of 5 patients demonstrated co-localization of C5b-9, S-protein, and C3 deposition in a glomerular capillary loop and mesangial distribution. Urinary excretion of TCC in the acute phase of APSGN was not elevated and was not a useful marker of disease activity. These data suggest that in APSGN with terminal complement pathway activation the local generation of TCC may contribute to the pathogenesis of the disease.

Acute glomerulonephritis during the third trimester of pregnancy

We report a very rare case of acute poststreptococcal glomerulonephritis (PSGN) occurring in the eighth gestational month. The pregnancy succeeded despite initially severe nephrotic syndrome, and after delivery there was a complete recovery to normal renal function. Fetal prognosis was also good. The diagnosis of PSGN was made based on serological examinations and postpartum renal biopsy. Light and electron microscopic findings showed that the glomerular lesions were different from those of typical pre-eclampsia.

Mendelian recessive ratios in acute poststreptococcal glomerulonephritis.

A prospective study of 15 families (61 siblings) of index cases of acute poststreptococcal glomerulonephritis detected a proportion of 0.260±0.016 SEM. This is an excellent fit for a single autosomal recessive trait.

Plasma factor XIII levels in children with renal disease.

Plasma levels of coagulation factor XIII determined quantitatively were found to have a tendency to be decreased in more severely affected cases of acute poststreptococcal glomerulonephritis and Henoch-Schönlein nephritis, and found to be decreased in patients with rapidly progressive glomerulonephritis and chronic renal failure in comparison with normal controls. It seems that the degree of decrease in factor XIII reflects the intensity of glomerular damage. In contrast, the patients with the nephrotic syndrome showed significant elevation of plasma factor XIII levels and it was closely correlated with the increase in serum triglycerides. The elevation of factor XIII levels in the nephrotic syndrome is suggested to be mainly attributed to enhanced protein synthesis in connection with urinary loss of proteins.

Acute poststreptococcal glomerulonephritis without proteinuria. Case report.

A case of acute poststreptococcal glomerulo-nephritis without proteinuria is reported. Mild proteinuria at a borderline level of 300 mg/24 hours was demonstrated on two days only. The course was characterized by initial transitory anuria-oliguria and severe renal insufficiency. From a review of the literature it is concluded that acute poststreptococcal glomerulonephritis without typical urinary findings does not differ bacterio-logically, serologically, histologically or clinically from typical acute poststreptococcal glomerulonephritis with proteinuria, erythrocyturia or cylindriuria. The cause of the absent proteinuria is discussed, but remains unknown.

The pathogenesis of the renal lesion in a patient with Streptococcal disease, infected ventriculoatrial shunt, cryoglobulinemia and nephritis

A case of acute poststreptococcal glomerulonephritis followed by shunt nephritis is presented. The patient manifested both persistent hypocomplementemia and cryoglobulinemia. Investigations into the nature of the glomerular deposits revealed the deposition of a staphylococcal antigen and immunoglobulin G (IgG) and β 1C globulin in a nodular pattern. No immunopathologic role for the cryoglobulins could be demonstrated, and Streptococcal products could not be identified by immunohistologic methods. The evidence supports a circulating bacterial antigen-antibacterial complex in pathogenesis, and thus the identification of an antigen in human immune complex renal disease.

Acute glomerulonephritis with normal results from urinalyses. A report of two cases and comments on four additional cases with atypical findings from urinalyses.

Two cases of acute poststreptococcal glomerulonephritis were found with paradoxically normal results from urinalyses. Three additional cases of acute glomerulonephritis with no proteinuria but with hematuria and one case with transient proteinuria and no hematuria were also found. The pitfalls of diagnosis with these unusual presentations of acute glomerulonephritis are apparent.

Acute Glomerulonephritis With Normal Results From Urinalyses

Two cases of acute poststreptococcal glomerulonephritis were found with paradoxically normal results from urinalyses. Three additional cases of acute glomerulonephritis with no proteinuria but with hematuria and one case with transient proteinuria and no hematuria were also found. The pitfalls of diagnosis with these unusual presentations of acute glomerulonephritis are apparent.