An unusual presentation of familial Mediterranean fever with co-existing polyarteritis nodosa and acute post-streptococcal glomerulonephritis

The homozygous M694V mutation in the MEFV gene may cause an augmented response to the streptococcal infection that plays a role in the development of APSGN and PAN. Both clinical manifestations may occur simultaneously after streptococcal infection in a child who is previously healthy but carries a MEFV mutation.

A role of streptokinase in experimental post-streptococcal glomerulonephritis

Post-streptococcal glomerulonephritis (PSGN) refers to the sequela of the acute infection, caused by Streptococcus pyogenes (group A streptococcus, GAS). This pathology has been studied for a long time, and today attempts are being made to identify the products of their life activity, able to initiate an immunopathological process in kidneys. Most attention has been paid to streptokinase, the enzyme transforming blood plasminogen into plasmin, capable, together with the plasmin receptor (NAPlr), of damaging the glomerular tissue, as well as activating the complement system. The aim of the study was to consider two tasks: to study the ability of the GAS-obtained enzyme to transform plasminogen of different species into plasmin as well as to study its role in the development of PSGN in rabbits having subcutaneously implanted tissue chambers. The animals were infected by inoculating GAS cultures into the chambers. Materials and methods. GAS strains of M types 1, 12, 22 and their ska– isogenic mutants were used in the study. Purified plasminogen preparations were isolated from fresh human, rabbit or mouse plasma by using chromatographic column with Lysine Sepharose 4B. To reveal the ability of streptokinase to activate plasminogen into plasmin, its preparation at a concentration of 1 mg/ml was added to 10 ìg of purified human, rabbit or mouse plasminogen. The concentration of plasmin was defined photometrically using S-2251 (Chromogenix, USA). To reproduce PSGN, four chambers were implanted under the skin in each rabbit; after the complete wound healing animals were infected and observed for three weeks. On day 14, the animals were treated with benzylpenicillin. The kidneys from survived rabbits were subjected to immunohistology analysis. Results. During in vitro experiments, M1, M12 and M22 GAS streptokinase showed distinct functional activity on human plasminogen, transforming it into plasmin: optical density indicators at ë = 405 nm were 0.4–0.7 compared with the negative control (ОD < 0.001). Streptokinase did not activate mouse plasminogen (ОD = 0.001) and exerted quite a weak effect on transformation of the rabbit plasminogen into plasmin (ОD = 0.002). In experiments on PSGN induction in rabbits, we failed to detect streptokinase involvement, because no differences between initiation of glomerulonephritis by wild strains or ska– isogenic mutants were identified. Mutant strains deficient in the gene responsible for streptokinase synthesis but retained ability to bind rabbit and human IgG, caused morphological changes in kidney tissue, specific for PSGN. In addition, a comparative analysis of PSGN “rabbit” and “mouse” models developed by the same technology, was carried out and led to opposing conclusions regarding a role of streptokinase in pathogenesis of experimental glomerulonephritis. The role of IgG Fc-binding activity of GAS in development of experimental PSGN is discussed.

One Disease, Two Approaches. Acute Post-Streptococcal Glomerulonephritis – A Case Report of Two Young Patients

Objective: Acute post-streptococcal glomerulonephritis (APSGN) is the most studied immune-mediated glomerulonephritis, being caused by streptococcal infections such as pharyngotonsillitis or skin infections (impetigo, erysipelas). Globally it is the main form of glomerular suffering among pediatric patients, especially between the ages of 3 and 15, but in the developed countries, the incidence of this condition has decreased significantly in the last decades. The majority of the medical literature indicates that the treatment in such a disease is symptomatic (the major goal being controlling of the edema and hypertension) associated with antibiotics for streptococcal infection. Due to some situations where antibiotic treatment cannot be administered, this case report hypothesized that homeopathy is a possible alternative treatment method for conventional therapy. We presented two cases diagnosed with APSGN: a nine-year-old boy treated homeopathically at home and a three-year-old girl treated conventionally during hospitalization. Analyzing the two cases, we observed that the patients were cured regardless of the therapeutic method approached. This aspect has an advantage in situations where there are limitations in the administration of allopathic treatment. According to homeopathic understanding, the success rate is higher when there are clear homeopathic symptoms for choosing the right remedy. Many more cases and much more research is needed to conclude that classical homeopathy can be a treatment option for this pathology. In conclusion, it is important to find a personalized therapy (allopathy or homeopathy) for each patient that will bring the maximum benefit, depending on the particularity of the case

Posterior Reversible Encepalopathy Syndrome in A Child With Post Streptococcal Glomerulonephritis- A Rare Case Report

Posterior reversible encephalopathy syndrome is a condition occurring in majority of case of hypertensive encephalopathy mainly due to vasogenic edema in parieto occipital region in neuroimaging. It is reversible if timely treatment is done .Here we are reporting a 11 year old female child with acute post streptococal glomerulonephrtitis leading to typical clinical and radiological features of posterior reversible encephalopathy syndrome

Clinical diagnosis and treatment of scabies, a neglected tropical disease

Scabies is a parasitic infestation of the skin caused by the mite Sarcoptes scabiei var. hominis. It is common in tropical areas, including the sub-tropical areas of Southern Africa. Predisposing factors are overcrowding, poor personal hygiene, low socio-economic standards and impaired host immunity. Although it can occur at any age, scabies is commonly seen in children and young adults. It is not a fatal disease; however, it can cause severe morbidity and poor quality of life. Scabies can complicate with pyoderma which may result in post-streptococcal glomerulonephritis. There are two clinical variants, classic scabies and the much rarer crusted scabies (Norwegian scabies).

The development of a community-based public health response to an outbreak of post-streptococcal glomerulonephritis in a First Nations community

Background: Post-streptococcal glomerulonephritis (PSGN) is a rare immune-mediated condition that typically occurs in children as a result of group A streptococcus (GAS) infection. PSGN is not considered a disease of public health significance, or reportable, in Canada. Higher incidence of PSGN has been described among Indigenous people in Canada. No national or provincial guidance exists to define or manage PSGN outbreaks. Objective: To describe an outbreak of seven paediatric cases of PSGN in a remote First Nations community in northwestern Ontario and the development of a community-wide public health response. Methods: Following a literature review, an intervention was developed involving screening of all children in the community for facial or peripheral edema or skin sores, and treatment with antibiotics if noted. Case, contact and outbreak definitions were also developed. The purpose of the response was to break the chain of transmission of a possible nephritogenic strain of streptococcus circulating in the community. Relevant demographic, clinical and laboratory data were collected on all cases. Outcome: Seven paediatric cases of PSGN presented to the community nursing station between September 25 and November 29, 2017. Community-wide screening for skin sores was completed for 95% of the community’s children, including 17 household contacts, and as a result, the last of the cases was identified. Nineteen adult household contacts were also screened. Ten paediatric contacts and two adult contacts with skin sores were treated with one dose of intramuscular penicillin, and six paediatric contacts received oral cephalexin. No further cases were identified following the screening. Conclusion: PSGN continues to occur in Indigenous populations worldwide at rates higher than in the overall population. In the absence of mandatory reporting in Canada, the burden of PSGN remains underappreciated and could undermine upstream and downstream public health interventions. Evidence-based public health guidance is required to manage outbreaks in the Canadian context. The community-based response protocol developed to contain the PSGN outbreak in this First Nations community can serve as a model for the management of future PSGN outbreaks.

Acute Post Streptococcal Glomerulonephritis among Children from Sokoto, North-Western Nigeria

Aims: To determine the clinical and laboratory profile of children with acute post streptococcal glomerulonephritis (APSGN) admitted into Usmanu Danfodiyo University Teaching Hospital (UDUTH) Sokoto, the outcome, and factors associated with in-hospital mortality. Study Design: A 5- year retrospective study. Place of Study: Emergency Paediatric Unit (EPU) and Paediatric Medical Ward (PMW) of the Department of Paediatrics, UDUTH Sokoto. Methodology: The records of children aged 4 to 14 years with the diagnosis of APSGN were reviewed. Relevant history, physical examination findings, laboratory and radiologic investigation findings were extracted from their case folders and recorded into a proforma sheet. Data was analyzed using SPSS version 23. (IBM SPSS Inc., USA). The level of statistical significance was set at 5%, which is p-value < 0.05. Results: Fifty-four (0.9%) of the 6128 children were managed for APSGN giving a prevalence of 10.8 APSGN cases per 1000 children. Forty-one folders were utilized for the study. There were 21(51.2%) females and 20(48.8%) males, with F:M ratio of 1.1:1. Mean age at presentation was 9.1± 3.1 years. Majority (92.6%) were ≥5 years and mainly 22(53.7%) of low socio-economic status. The main features were body swelling 40(97.6%), fever 25(61.0%), oliguria 24(58.5%), systemic hypertension 37(90.2%), proteinuria 41(100.0%), and haematuria 41(100.0%). Acute kidney injury was the commonest 25(61.0%) complication. Thirty (73.2%) cases were discharged, 5 (12.2%) died, 6(14.6%) left against medical advice. Low social status (0.03), requirement for dialysis (p=0.003), congestive cardiac failure (p=0.01), and pulmonary oedema (p=0.04) were significantly associated with in-hospital mortality. Requirement for dialysis (p=0.005) was the independent predictor of in-hospital mortality. At three months post discharge, 20(48.8%) of the 31 cases had achieved complete resolution of APSGN. Conclusion: APSGN is common in Sokoto and similar in pattern to other reports from Nigeria. The presence of complications at presentation increases the risk of in-hospital mortality.

StreptInCor, a Group A Streptococcal Adsorbed Vaccine: Evaluation of Repeated Intramuscular Dose Toxicity Testing in Rats

Streptococcus pyogenes infections continue to be a worldwide public health problem, causing various diseases in humans, with rheumatic fever and rheumatic heart disease being the most harmful manifestations. Impetigo and post-streptococcal glomerulonephritis are also important sequelae of skin infections. We have developed a candidate vaccine epitope (StreptInCor) that presents promising results in diverse animal models. To assess whether the StreptInCor alum-adsorbed vaccine could induce undesirable effects, a certified independent company conducted a repeated intramuscular dose toxicity evaluation in Wistar rats, a choice model for toxicity studies. We did not observe significant alterations in clinical, hematological, biochemical, anatomical, or histopathological parameters due to vaccine administration, even when the animals received the highest dose. In conclusion, repeated intramuscular doses did not show signs of macroscopic or other significant changes in the clinical or histopathological parameters, indicating that StreptInCor can be considered a safe candidate vaccine.