Arefeh Zahmatkesh

and 8 more

Case reportEcthyma Gangrenosum in Three Unrelated Patients with Combined Immunodeficiency Arefeh zahmatkesh1, Amirhossein Hajialigoli2, Mahnaz Jamee3, Zahra Chavoshzadeh4 1Pediatric Nephrology Research Center, Research Institute for Children’s Health, Shahid Beheshti     University of Medical Sciences, Tehran, Iran2Alborz Office of Universal Scientific Education and Research Network (USERN), Alborz University of      Medical Sciences, Karaj, Iran3Pediatric Nephrology Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran4Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Correspondence should be addressed to Arefeh Zahmatkesh: arefeh.zhmtksh@gmail.com.  AbstractBackground:Ecthyma Gangrenosum(EG) is a necrotizing vasculitis characterized by cutaneous manifestation ranging from a nodule or papule to necrotic ulceration with surrounding erythema, especially with black eschar or central crust. The most common pathogen that causes EG is Pseudomonas aeruginosa(PA). PA is an opportunistic pathogen with predominance incidence among patients with primary immunodeficiency with hypo or agammaglobulinemia, malignancies, and acquired immune deficiency.Case presentation:In this case study, we present three unrelated patients (three male toddlers, mean age: 0.75 years) with the primary manifestation of EG, who underwent immunological assessment and were diagnosed with combined immunodeficiency. all patients were alive although EG has a high mortality rate, the prognosis depends on the host and the degree of immunosuppression.  A history of Persistent fever followed by skin lesions was common in our cases. Surprisingly, the initial immunological assessment reported different cellular and humoral immune deficiencies with the overall diagnosis of combined immunodeficiency based on the ESID criteria. For patients suspected of EG, early diagnosis and administration of appropriate systemic antibiotic therapy can considerably reduce morbidity and potential mortality.Conclusions:This case report illustrates the importance of immunodeficiency evaluation in patients with a skin lesion and considers pseudomonas aeruginosa culture for initiating appropriate antipseudomonal antibiotic therapy. Although recent studies show high EG-related mortality with predisposing factors, hopefully, due to appropriate intravenous antibiotics and immunoglobulin therapy, all patients remained alive.Key-words:Ecthyma Gangrenosum, combined immunodeficiency, Immunoglobulins I. Introduction Ecthyma gangrenous (EG) is a rare skin disorder, that usually starts as an erythematous macule, which developed into a vesicle. These Lesions can rapidly indurate and develop pustules or bullae, which slough and leave an eschar. (1) The first case of EG was reported by L. Borker. Hitschman and Kreibichin 1897, which accompany Pseudomonas septicemia. (2) although several studies show more pathogens associated with EG, Pseudomonas aeruginosa(Pa) is the most reported cause. (3). Pa as an opportunistic bacterium can detect from the skin, nose, throat, and stool. (4) The most common site for superinfection of the skin with Pa includes the gluteal and anogenital region, the extremities, the trunk, and the face. (5). The pathogenesis of skin lesions due to Pa is mainly dependent on the vessel walls invasion interfering with the toxin followed by ischemic necrosis resulting in necrotic ulcer with a black/gray eschar surrounded by an erythematous halo. (6) Since the first report of EG, several studies show the EG incidence among patients with predisposing risk factors like pre-existent viral infections, weak mechanical skin barriers, especially immunocompromised individuals, and even previously healthy patients. Also, malignancies like leukemia (7) and primary immunodeficiency disorders like leukocyte adhesion deficiency-1(LAD) (8), and X-linked agammaglobulinemia(XLA) are reported related to EG. (9) However, skin necrosis is a symptom of a broad range of pathologies, (1) the diagnoses of EG are based on clinical findings, that are confirmed by the skin and blood culture. (2) Besides, early appropriate treatment e.g., antibiotics, and surgical debride reduce EG-related mortality rates, also understanding the patient’s immunological status and underlying disease affect the prognosis, and initiation of appropriate treatment can reduce mortality rates. (3)This manuscript summarizes three cases of EG who were admitted to a tertiary hospital with pursuing chief complaints, initial evaluation, and treatment.II.   Case reportsA. Case 1A 16-month-old male was admitted to the hospital with a one-week history of fever and lesions on his left groin, neck, and left axilla. his fever started 3 days after influenza vaccine and didn’t respond to initial treatment(Acetaminophen). He hospitalized due to persistent fever and then Bullae lesions began 2 days after admission and progressed to a necrotic lesion with infectious secretion with a positive lesions culture which showed pseudomonas aeruginosa, he received appropriate antibiotics (Amikacin and Imipenem) based on the culture and G-CSF due to leukopenia with no response. He had a diarrhea history due to milk powder allergy when he was 2 months. His vaccination was upon the routine plan.  His parents were relative and did not report any recent travel and allergic reaction. The physical examination showed vesicular dark lesions on his left leg, axillary region, and neck. His lower extremities were swollen and tender, especially on left foot. The left foot was warmer than the right and had more lesions compared to other sites. The liver was enlarged up to 3 cm below the right costal margin, and the spleen was impalpable. In chest examination, coarse crackles at both lungs and the right lung consolidation on the chest X-ray (CXR) were found. The laboratory findings are summarized in Table 1. In the first days of admission, neutropenia and thrombocytopenia was detected, which improving during hospitalization.On the second day of administration, he was admitted to the intensive care unit (ICU) due to his unstable condition. He suffered extensive ulcerative lesion with necrotic center on his left foot which was more tender and warmer than the opposite site. Following surgery consultation, Doppler sonography and X-ray of lower extremities were performed, which showed subcutaneous and intermuscular adenoma, which suspected to myositis and necrotizing fasciitis was ruled out.In this center bacterial blood culture was negative. we started antibiotics therapy with Meropenem, Vancomycin, and Clindamycin, then changed the regimen to Ceftazidime and Clindamycin after 22 days of administration. In addition, human serum albumin and calcium with heart monitoring, were prescribed and because of low hemoglobin, packed red blood cells was infused. He received Ribavirin suspected for Crimean Congo Hemorrhagic Fever. On hospital day 3, the necrotic lesion was debrided and drained surgically. Finally, based on immunology consultation, he started receiving 5-gram intravenous immunoglobulin (IVIG). He was continued on antibiotics and finally discharged from the hospital in a stable condition with receiving monthly IVIG. B. Case 2A 5 month years old male was admitted to this center with four days’ history of fever and chilling, which did not respond to medical treatment with cephalexin. The fever was complicated with several lesions on the face after one day. Erythematous plaques without pus were seen on the cheek, under the right eye, and genitalia Picture 1. His vaccination was upon the routine plan. His parent were not relatives and did not report any recent travel or contact with the ill person. His sibling was healthy. In his physical examination, multiple hemorrhagic plaques were observed on the right leg and left thigh. Lower extremities and scalp were swallowed. He did not have organomegaly on the abdominal examination. The chest examination was normal. He was under observation in the pediatric intensive care unit and received 2 gr intravenous immunoglobulin (IVIG) because of his severe edema (suspicious of Kawasaki disease) on the first day of admission. He was also started on abroad-spectrum systematic antibiotic regimen (Meropenem, Vancomycin, and Amikacin) after admission. On hospital day 5, his fever was reduced and skin lesions improved, while his wound culture grew Pseudomonas aeruginosa. Because of the immunodeficiency suspicion, bone marrow aspiration(BMA) was performed, which reported maturation arrest in the myeloid series. For agammaglobulinemia assessment, immunologic workup was performed which yielded in Table 1. and compare with other cases.With Ecthyma Gangrenosum impression, he received intravenous methylprednisolone 1 mg/kg/BD and Mometasone ointment twice a day. He continued on antibiotics and underwent surgical debridement of the necrotic lesion and skin graft. His temperature gradually returned to normal after surgical and medical treatment on hospital day 15, he was discharged from the hospital in stable condition with prophylactic antibiotic (Cotrimoxazole) and repeating complete blood count every 2 weeks.C. Case 3:A 6months old male was admitted to this center with a history of a two-week fever. The fever was accompanied by erythematous papule which transformed into necrotic, bullous, and gangrene lesions and spread through his thigh, posterior conjunctiva, and left flunk Picture 2. One week before referral to this center, he was hospitalized and received antibiotics (Meropenem, Vancomycin, and Ciprofloxacin) upon a positive pseudomonas wound culture and positive Klebsiella cerebrospinal fluid (CSF) culture. He had a history of acholic defecation two days before hospitalization. His parents were not relatives and his older siblings were healthy. He did not have a history of recent travel or contact with a contiguous disease. In the physical examination, a few gangrenous 2*3 mm lesions with surrounding blisters were scattered on his left flank, thigh, and posterior conjunctiva. The right testis was larger than normal and bilateral hydroceles were noted. The sclera was icteric. Pulmonary and heart examinations were normal. The abdomen was distended with a bilateral erythematous lesion, without tenderness, rebound, or guarding. The liver was enlarged to 3 Cm below the right costal margin and the spleen was impalpable.The broad-spectrum antibiotic (Amikacin, Meropenem, and Vancomycin) were administrated as well as two doses of IVIG to provide passive antimicrobial coverage. In addition, abdominal sonography and surgical debridement were performed. Sonography showed mild pulmonary edema, cellulitis, myositis, and hydrocele. On hospital day 2 regarding the abnormal liver function test (LFT), he received Ursobil, and LFT was checked two times every week. Vitamins and fresh frozen plasma (FFP) were also prescribed to correct international normalized ratio(INR). The immunologic function was tested. the lymphocyte subtypes detail notice in the Table 1 and compared with others. The T CD8+ cells were reduced and the lymphocyte transformation test (LTT) was dysfunctional Table, further confirming the diagnosis of combined Immunodeficiency. He was discharged with 5 mg IVIG, antibiotics, and antifungal prophylaxis.The first step of Biochemical diagnosis of CID suspected patients is complete blood count which gives clues of immunological alteration. despite, the assessment of absolute neutrophil and lymphocyte count of our cases was influenced by Pa toxin, after human immunodeficiency virus (HIV) ruled out the specific evaluation of immunological parameters was performed. this evaluation included: i. Measurement of immunoglobulins (IgA/IgG/IgM/IgE) which is summarized in Table 1-D. Except for generalized hypogammaglobinemia in case 1, reduced IgE and IgA were seen in cases 2&3. ii. Vaccinal response which done when maternal antibodies transferred via placenta decrease (6 month years old) Table 1-E. based on our data we detected less than 1 Iu/mL Anti-Diphtheria IgG in case 2. iii. Measurement of leukocyte subtypes by flow cytometry (Immunophenotyping of CD3/CD4/CD8/CD19 and NK) summarized at Table 1-C. CD3+ as a general lymphocyte and CD19+ as a B lymphocyte marker reduced in case1&3.  Reduced CD3+/CD4+  in case 2&3 shows lymphocyte impairment, and high CD3+/CD8+ indicate low CD8+ in all cases. More CD information provide in Table 1-F. iv. Lymphocyte function assessment by Lymphocyte Transformation Test (LTT) can be measured by lymph proliferation after stimulation with phytohemagglutinin(PHA). Table 1-B. In our study all patients had normal PHA level, however cell proliferation against BCG in case 1 was slightly low and also cell proliferation against Candida in case 2&3 were reduced. II.  Discussion and conclusion:  Ecthyma Gangrenosum is a rare skin disorder, which is an ulcerative variant of septic vasculitis. EG is characterized by sharply circumscribed “pinched out” deep ulceration. These lesions usually start as an erythematous macule, which subsequently forms a vesicle, pustules, bullae with debris, and necrotic material within the ulcer. (1) since the first case report of EG, several studies show different causative pathogens. (2) In a study of 164 patients diagnosed with EG  between 1974 to 2014, PA was detected in 73.65% of cases, whereas other bacteria and even Candida albicans, were detected in only 17.35% and 9% of cases respectively. (3) Although in case 3 we isolated Klebsiella from CSF culture , Pa was detected from all lesions culture. However, Klebsiella can cause EG.(4) A case report from Isezuo K.O in 2018, shows different isolated organisms from CSF(S.aureus) and lesions(E.coli) culture in the concept of co-infection at EG pathogenesis. (5) Pa species are a normal part of the skin flora and usually found in the anogenital, axillae, and external ear canal. (6) clinical manifestations related to PA  are mainly due to the vessels walls invasion mediated by the toxin, (7) and usually affects the lower extremities, especially gluteal and genital areas. fever, diarrhea, pneumonia, shock are the most relevant associated symptoms especially in PA sepsis. (5) As CCHF was suspected in case 2, several studies show infections that can mimic EG, like mycobacterial ulcer, cutaneous leishmaniasis cutaneous tuberculosis, and even deep fungal infections thus, EG must be considered in any necrotic lesions that are unresponsive to prolonged antibiotics. (8) Another important differential diagnosis is necrotizing fasciitis which was ruled out by MRI in case3. (9)Despite, EG usually being described in immunocompromised patients, it also has been seen in patients suffering from malnutrition, underlying (hematological) malignancy, and even previously healthy individuals. (10)A literature review by Danel J Lewis et al in 2019, shows cutaneous manifestations of primary immune deficiency disorders (PID). (11) several studies show EG in patients who suffer from X-linked agammaglobulinemia (XLA) (Burton hypogammaglobinemia), which is characterized by the complete absence of circulating B-cells and plasma cells, with decreased(IgG) or absent (IgA and IgM) levels of immunoglobulins. (12) Leukocyte Adhesion Deficiency (LAD) is also reported with EG. these disorders involve an absence of ß2-integrin subunit(CD18), which prevents neutrophils aggregation. (11)  neutrophils are a vital part of the cellular host defense against bacterial infections, whereby neutrophil count bellow 500/mm3 cause the greatest risk of bacterial infections. (13) two cases of our study (Case1&2) had neutropenia on the first days of admission which resolved after treatment. In addition to several case reports of neutropenia with EG, PA toxins can produce neutropenia through bone marrow suppression and inhibition of granulocyte migration. (2). In 2003 Maria Baro et all reported X-linked agammaglobulinemia with EG, that neutropenia with bone marrow granulocyte arrest resolved when infection was treated. They also postulated that neutropenia could result from a neutrophil-altered response to stress due to mutations in the Btk gene that is expressed in myeloid series. (14) unlike other cases, case 3 had neutrophil predominance. in a case report at a tertiary hospital in Sokoto, the index patients also had neutrophil predominance as usual neutropenia associated with PA. However, our patient’s immunological comparison didn’t show obvious differences, this may be explained by their relatively good immunity. (8) another primary immune disease that leads to neutrophil dysfunctions is chronic granulomatous disease(CGD), it is caused by gene mutations encoding essential subunits of NADPH oxidase complex subsequently neutrophils fail to increase oxygen consumption for the destruction of phagocytes bacteria (e.g.; Klebsiella, Pseudomonas, Candida) and fungi. (15)  Nitro blue tetrazolium Blood Test(NBT) was normal (100%) in all our cases, thus we rollout CGDs. Besides underlying immunodeficiency diseases, some studies suggest that the immunoglobulin level and B cell percentage were decreased in the disease process and are transient.(10) However hypogammaglobinemia and reduced B cell were observed in our cases, CID was applied based on other flow cytometry data and ESID criteria. However it should be considered molecular diagnosis such as next-generation sequencing(NGS) is the exact diagnostic modality.(16)Combined immunodeficiency (CID) is an Inborn Error of Immunities(IEIs) characterized by defects in both humoral and cellular limbs of the immune system. the main clinical manifestations of IEIs are susceptible to unusual or recurrent infections that are difficult to treat. (17)  Previous studies show variable skin manifestations in CID patients with syndromic features (e.g.: atopic dermatitis in Wiskott-Aldrich syndrome (18), bulbar telangiectasia and ataxia in Ataxia-telangiectasia (19), Café au lait macules and telangiectasia in sun-exposed are in Bloom syndrome (20), dermatitis, prominent papulopustular eruption and recurrent staphylococcal infections with abscess formation in Hyper-IgE syndrome (Job syndrome) (21).The first step of Biochemical diagnosis of CID-suspected patients is a complete blood count which gives clues of immunological alteration. (22) despite, the assessment of absolute neutrophil and lymphocyte count of our cases being influenced by Pa toxin after HIV was ruled out the specific evaluation of immunological parameters was performed. this evaluation included (23, 24):In our study all patients were alive. although EG has a high mortality rate, prognosis depends on the host and the degree of immunosuppression. in patients with EG and septicemia secondary to pseudomonas it ranged from 38%-77%, and in patients without sepsis is about 15%. (25) Notably, neutropenia below 500 cells can predispose a patient to severe PA infection and this seems to be associated with a higher mortality rate even in a previously healthy child. (13) EG’s high mortality rate emphasizes the importance of early suspicion and proper treatment even when the diagnosis has not been confirmed. While awaiting culture results, empirical antimicrobial therapy with anti-pseudomonas penicillin and aminoglycoside should be started, and adjusted based on culture results. Administration of GCSF along with antibiotics should be considered to shorten the duration of neutropenia, to help resolve the EG, and to minimize the risk of septicemia in immunocompromised patients.  In conclusion, EG is a rare skin disorder, usually caused by PA, and commonly occurs in immunocompromised individuals. despite, the previously healthy and immunocompetent individuals, may become affected, we must consider an immune assessment for all patients, especially in early childhood. furthermore, early diagnosis of an underlying illness, notably primary immunodeficiency, leads to using the appropriate treatment and preventing EG-related mortality.Data Availability: Not applicable.Ethical Approval: Informed consent was obtained from the patient and parents of the patient prior to being included in the study.Consent: Written informed consent for publication was obtained from the parents of the patients prior to being included in the study.Conflicts of Interest: The authors have no conflicts of interest.Acknowledgments The authors thank the patients and their families for their participation in this study.    Ⅳ. Table 1Laboratory data: A.       Complete blood count:                           CBC1 Case number WBC 1000/ul Neutrophils 1000/µl Lymphocyte 1000/µl HB g/dl Platlet cell/ul 1 2700 1215 1405 10.7 306000 2 7980 1516 5426 9 381000 3 7900 5925 1422 11.9 219000 1.CBC: Complete Blood Count B.       Lymphocyte transformation test:                 LTT1    Case PHA2 (normal range≥3) BCG (normal range≥2.5) Candidia (normal range≥2.5) 1 4 2.2 4.4 2 3.2 3.1 2.2 3 4.3 3.1 1 1.LTT: Lymphocyte Transformation Test, 2.PHA: Phytohemagglutinin, C.       Flow cytometry:             CD markers1   Case   CD3+    CD4+   CD3+/CD4+    CD8+      CD3+/CD8+      CD16     CD19     CD56         1     88% Absolut=1236×103/µl (range=1400-8000)               58%        1.51 (range=0.9-5.5)                31%                2.84 (range=0.4-2.3)             4%      7% Absolut=98×103/µl (range=600-3100)         4%         2         70.1% Absolut=3798 (range=2400-6900)      55.2%       1.27 (range=1.4-5.1)      18.2%          3.84 (range=0.6-2.2)        -           14.2% Absolut=770 (range=700-2500)       -          3     68% Absolut=967 (range=2400-6900)      62%        1.09 (range=1.4-5.1)      6%           11.37 (range=0.6-2.2)       1.5%          14% Absolut=199 (range=700-2500)                3%  1.CD3: T-cells(general), CD4: T helper, CD8: T cytotoxic, CD16: Granulocytes/natural killer cells(NK), CD19: B-cells, CD56: NK D.       Immunoglobulins             Igs Case       IgG mg/dl        IgM mg/dl        IgA mg/dl            IgE mg/dl             1         110 Normal range for age:(666-1340)            18 Normal range for age:(76-233)          <3 Normal range for age:(24-116)           1 Normal range for age:()             2         820 Normal range for age:(377-774)           65  Normal range for age:(40-141)          <37 Normal range for age:(13-56)           3 Normal range for age:()             3         698   Normal range for age:(363-1690)           86 Normal range for age:(48-249)          7 Normal range for age:(7-78)           0.7 Normal range: 70-400)   E.        Immunoglobulin response:  Case Anti-Tetanus IgG Anti-Diphtheria IgG                 1                 1.5                0.26                 2                 2.12                >1                 3                 -                 -  1.NBT: Nitroblue Tetrazolium Test F.         Further flow cytometry information: Case2: flow cytometry lymph phenotyping analysis, Gate: Lymph (21.0%), Viability:>90% CD1 0.1 CD34 2.3 CD2 69.6 CD11 2.5 CD3 70.1 CD9 0.3 CD4 55.2 CD10 0.1 CD5 68.2 CD19 14.2 CD7 69.2 CD20 13.5 CD8 18.2 CD22 12.9 CD13 0.1 CD45 95.1 CD33 2.5 CD18 90  rang(60-90) CD14 1.3 CD11a 99 rang(Total=50-90,Lymph=50-90) CD15 97 rang(60-90) CD11b 90 rang(Total=40-85,Lymph=5-20)   CD11c 97 rang(Total=10-30%,Lymph=2-8%)    Case3: flow cytometry lymph phenotyping analysis, Gate: Lymph (39%), Viability:90%, Specimen: PB   CD2 77.4 CD20 21.3 CD56 0.3 CD3 84.3 CD38 0.1     CD4 67.2 CD4/CD8(dual) 0.4     CD8 7.9 CD2/CD19(dual) 0.2     CD10 1 CD3/HLADR(dual) 2.1     CD19 19.1 CD16 1.5        V. PicturesPictures-1:Case B:·         Initial lesions

Paniz Pourpashang

and 3 more

Rare Post Streptococcal Glomerulonephritis presentations include back pain and constipation.Paniz Pourpashang,1 Fatemeh Nili,2 Masoumeh Mohkam,3 Arefeh Zahmatkesh41 Department of pediatric nephrology, Shahid Beheshti Medical University, Tehran, Iran2 Department of pathology, Imam Khomeini hospital complex, Tehran university of medical sciences3Pediatric Nephrology Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran4School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran Correspondence should be addressed to Arefeh Zahmatkesh; arefeh.zhmtksh@gmail.com Abstract:Background: Post-streptococcal glomerulonephritis (PSGN) is a common disease that occurs after pharyngitis or dermatitis involvement with streptococcus. The disease complications are vary including rare neurological disorder like, posterior reversible encephalopathy syndrome (PRES). Although the disease has some common symptoms like hypertension, oliguria or anuria, and neurological defects, there have been no reports of a child having severe back pain or constipation up until this point. Case presentation: Here, we represent a child with severe back pain and constipation that was diagnosed as PRES syndrome followed PSGN. Conclusion: Despite several studies on PSGN, attention to different and rare associated symptoms could help practitioners with accurate diagnoses. In addition, made us investigate the disease entity. Keywords: Post-streptococcal glomerulonephritis, back-pain, constipation, posterior reversible encephalopathy syndrome.Introduction The most common cause of acute glomerulonephritis in children is post-streptococcal glomerulonephritis (PSGN), which typically affects children between the ages of 3 and 12 years and is uncommon in children under the age of 3 year. Hematuria, oliguria, edema, and hypertension are the most typical symptoms of PSGN. [1, 2]  The severity of PSGN can vary, and patients may present with subclinical symptoms or require dialysis, and the majority of cases follow pharyngitis caused by streptococci infections.[3, 4] One of the uncommon side effects of PSGN is posterior reversible encephalopathy syndrome (PRES), which typically manifests as headache, vision loss, and seizures. The etiology and clinical manifestation of PRES in children differ from those in adults, but renal diseases predominated in a large systematic review. [5] There are many different PSGN manifestations, but back pain and constipation have not yet received much attention, despite the PRES syndrome diagnosis.  In this study, we described a 10-year-old boy who complained of back pain and constipation and was ultimately diagnosed with PSGN and PRES syndrome together.Case presentation: A 10-year-old boy was referred to our clinic complaining of severe constipation, oliguria, and edema. He had previously undergone evaluations and had a raised serum creatinine level. He also had a history of visiting a crowded area and later experiencing fever and other flu-like symptoms. The symptoms were treated with conservative treatment. In his past medical history, he had been completely healthy, had no medical or familial disorders, and had completed his series of vaccinations.He was referred to our clinic with peri-orbital edema, congestion during a chest exam, hypertension, and anuria. His parents also reported that he had normal urination in the previous two days before a sudden onset of low-volume urination. He was given Lasix with normal saline, but he did not respond to this treatment, and laboratory tests revealed elevated creatinine levels. Table 1. Large kidneys were seen during an abdominal ultrasound study, and as a result of his high creatinine level, anuria, and lack of response to Lasix therapy, the patient underwent sessions of emergent dialysis.On the second day of hospitalization, the patient started experiencing severe low back pain, which persisted throughout dialysis. Considering the laboratory results and the requirement for daily dialysis, a kidney biopsy was performed with the intention of confirming PSGN (Figure 1); however, the biopsy report revealed PSGN without crescentic features. On the third day of his hospital stay, the patient experienced a seizure that was controlled with phenytoin and carbamazepine. As a result, magnetic resonance imaging (MRI) without contrast was carried out, and the results revealed PRES (Figure 2) He also received rasburicase for hyperuricemia, adjusted doses of ciprofloxacin, meropenem, and cefazoline, as well as five doses of methyl prednisolone for the treatment of PSGN. Following the methylprednisolone prescription, the patient's symptoms improved noticeably, and PSGN therapy was used to treat the patient's back pain and constipation.The patient was given a clean bill of health and was referred for follow-up exams. At the most recent checkup, his kidney size and creatinine level were both within normal ranges.ranges.Discussion: Post streptococcal glomerulonephritis (PSGN) is known as rapid deterioration of kidney functions because of an inflammatory process that included type III hypersensitivity reaction after streptococcal infection. This condition is primarily caused by a beta-hemolytic streptococcus known as nephrogenic streptococci, which affects the glomeruli of the kidneys and small blood vessels. PSGN typically appears in children 1–2 weeks after a sore throat.[4, 6] In our case, a 10-year-old boy had edema, hypertension, and other typical PSGN presentations after experiencing flu-like symptom. The most frequent side effect of PSGN is hypertension, which has a presentation rate of about 64%. After receiving full treatment, hypertension may recur in 3 to 6% of PSGN patients. [7, 8] Children with PSGN involvement may exhibit abnormal neurological symptoms like generalized seizures because of severe hypertension, which affects about 30 to 35 percent of children with PSGN. [4, 9] Additionally, hypertension can result from PRES, a term for hypertensive encephalopathy that was first used in 1996 and PRES causes seizures in patients. [10] One widely accepted PRES mechanism theory links the rise in blood pressure to a hypertensive emergency that causes a breakdown in cerebral autoregulation.[11] despite the fact that the child in our case had one seizure, which was stopped by carbamazepine, it is crucial for pediatricians to be aware of the seizure occurrence in kids with PSGN because it can negatively affect neurological function. In general, PRES with PSGN usually has a favorable prognosis. According to prior studies, clinical findings fully resolve if it is quickly diagnosed and treated.[5] Although reports on the association between back pain and PSGN on children are limited, some studies reported back pain presentation with different glomerulonephritis diagnosis in adult after infections. Study by Kadapia et al. reported back pain in a 39-year-old man with post infectious glomerulonephritis, who well responded to corticosteroid therapy. [12] Another study showed Minimal change nephrotic syndrome (MCNS) after B type influenza in a 50-years-man with progressive back pain, which spontaneous remission accrued without corticosteroid therapy.[13] However, in our study back pain resolved after corticosteroid administrations, because of less information, more case reports and studies need to investigate the treatment. In addition, no study was reported constipation in PSGN. Although constipation is highly frequent among patients with chronic kidney disease and uremic toxin could affect intestinal motility, [14] there is no reported on constipation in PSGN. Therefor more studies needed.   The predictors of poor long-term prognosis of PSGN include the presence of nephrotic syndrome, crescent formation on biopsy findings, and renal insufficiency at onset. [15] Wong et al. evaluated 27 PSGN patients who required renal biopsies due to acute severe glomerulonephritis, anuric renal failure, and nephrotic nephritic syndrome. They found that 12 patients required acute dialysis and that 11 patients had crescents that were higher than 50% on the biopsies, and that patients with crescentic glomerulonephritis had a greater need for acute dialysis.[3] Kidney damage may persist years after PSGN because of secondary inflammation after infection due to the nephron’s damages.[16] However, our patient was anuric, after daily dialysis, pathology specimen didn’t reported cresentric and his symptoms start regretting.In most cases, PSGN is a self-limiting condition, so supportive treatment for managing the effects of overload like edema and hypertension should be carried out. Only symptomatic treatment is advised. [15]  Antihypertensive medications in cases with uncontrolled blood pressure, the use of calcium channel blockers, angiotensin-converting enzyme inhibitors (ACEI), angiotensin receptor blockers (ARBs) are recommended. There is no evidence to support the use of immunosuppressive medications in PSGN patients, but they may be helpful in those with progressive renal failure. [17] Immunosuppressive and antihypertensive medications were used to control the disease in our patient, and with these medications, all patient manifestations were resolved. In fact, after receiving methylprednisolone, all patient manifestations regressed.conclusionIn conclusion, PSGN is a self-limiting disease that can manifest as hypertension, neurological disorders. However, we can explain these manifestations due to PRES, back pain and constipation were not previously reported in studies with this syndrome. pediatricians and physicians should be aware of these manifestations in PSGN. If there is any doubt about PSGN and the patient exhibits either of these symptoms, PSGN treatment should be initiated. If the constipation or back pain persists, doctors should be evaluated.Author’s contributionConceptualization: PPValidation: MM and FNInvestigation: PPResource:  PPData correction: MM and FNWriting original draft preparation: PPWriting review and editing: AZVisualization: PP and MMSupervision: MMProject administration: AZEthics approval and consent to participate: Informed consent was obtained from the patient and parents of the patient prior to being included in the study.Consent for publication: Written consent for publication was taken from the patient and her parents.Availability of data and materials: Not applicable.Competing interests: The authors declare that they have no conflict of interest.Funding: The authors received no specific funding for this research.Acknowledgments: We thank the patient and her family for their contribution to this studyReferences 1.            Kari, J.A., A. Bamagai, and S.M. Jalalah, Severe acute post-streptococcal glomerulonephritis in an infant. 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