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Multiple inflammatory syndrome in children (MIS-C) is a multisystem inflammatory disease following COVID-19 in children. This disease occurs a few weeks after COVID-19. A child with this condition develops a cytokine release cascade that results in organ damage. The involved organs include the heart, lungs, brain, gastrointestinal tract, and central nervous system. Fever is present in all patients and Kawasaki-like symptoms are one of the common features in these children. In this article, we introduce a child with MIS-C who has skin, gastrointestinal, neurological, and renal symptoms and has been hospitalized in the PICU.

Background:Congenital hydronephrosis is one of the most common abnormalities of the upper urinary tract, which can be exacerbated by a variety of intrinsic or extrinsic triggers. The urinary tract system is one of the major organs complicated by COVID-19 infection. Case presentations:Here we report five patients with an established diagnosis of congenital hydronephrosis, who presented with acute abdominal pain and fever and an abrupt increase in the anteroposterior pelvic diameter (APD). Patients had a previous stable course and were under regular follow-up with serial ultra-sonographic studies. They underwent surgery or supportive treatment due to the later exacerbation of hydronephrosis. Based on the clinical and imaging findings, no plausible etiologies for these exacerbation episodes, including infection, nephrolithiasis or abdominal masses, could be postulated. The common aspect in all these patients was the evidence of a COVID-19 infection. Conclusions:Infection with COVID-19 in children with antenatal hydronephrosis may exacerbate the degree of hydronephrosis and renal APD in ultrasonography, which itself may be mediated by the increase in inflammatory mediators.

Background:Renal disorders are reported as both underlying conditions and complications in critical pediatric COVID-19 infection. Herein, we investigated the pattern of renal involvement, particularly acute kidney injury (AKI), among pediatric patients with COVID-19.MethodsWe prospectively enrolled hospitalized pediatric patients with the clinical diagnosis of COVID-19 infection. Demographic, clinical, and laboratory findings were gathered and analyzed, using a mixed method of qualitative and quantitative approaches and descriptive statistics.Results187 patients including 120 (64.2%) male and 67 (35.8%) female with COVID-19 infection at a median (interquartile range) of 60 (24-114) months were enrolled in this study.Most patients (n=108, 58.1%) were suffered from one or two underlying comorbidities, mainly malnutrition (77.4%), neurologic/learning disorders (21.4%) and malignancy (10.2%). Kidney was the third organ involved in 30 patients (16%) during hospital admission, after lung (53.5%) and gastrointestinal tract (39%). According to the KDIGO definition and classification, AKI was detected in 38.5% of patients (stage 1: 55.6%, stage 2: 36.1%, stage 3: 8.3%) at presentation or during follow up. Nine patients (4.8%) required hemodialysis and 16 (8.6%) patients were eventually died. There was no significant association between AKI and pediatric intensive care unit (PICU) admission (p=0.079), multisystem inflammatory syndrome in children (MIS-C) (p=0.051), comorbidities (p=0.067), and mortality rate (p=0.789).ConclusionsKidneys are one of the major organs affected by COVID-19 infection. Although kidney abnormalities resolve in the majority of pediatric COVID-19 infection, particular attention should be given to kidney involvement in COVID-19 patients, particularly children with history of malnutrition and renal disorders.

BackgroundMendelian susceptibility to mycobacterial disease (MSMD) is an inborn error of immunity, resulting in susceptibility to weakly virulent mycobacteria and other intramacrophagic pathogens. Rheumatologic manifestations and vasculitis are considered rare manifestations in MSMD patients.Case presentationIn this study, we reported a 20-year-old female who was presented with recurrent lymphadenitis following bacillus Calmette-Guérin (BCG) vaccination and a history of recurrent disseminated rash diagnosed as leukocytoclastic vasculitis (LCV). A slight reduction in lymphocyte subsets including CD4+, CD19+, and CD 16 + 56 T-cell count, as well as an elevation in immunoglobulins level (IgG, IgA, IgM, IgE), were observed in the patient. Whole exome sequencing revealed a homozygous Indel-frameshift mutation, c.527_528delCT (p. S176Cfs*12), at the exon 5 of theIL12Bgene. She experienced symptom resolution after treatment with anti-mycobacterial agents and subcutaneous IFN-γ. We conducted a manual literature search for MSMD patients reported with vasculitis in PubMed, Web of Science, and Scopus databases. A total of 18 MSMD patients were found to be affected by a variety of vasculitis phenotypes mainly including LCV and Henoch-Schönlein purpura (HSP) with often skin involvement. Patients were all involved with vasculitis at the median age of 6.8 (2.6–7.7) years, nearly 6.1 years after the initial presentations. Sixteen patients (88.9%) hadIL12RB1defects and concurrentSalmonellainfection was reported in 15 (88.2%) patients.ConclusionThe lack of IL-12 and IL-23 signaling/activity/function and salmonella infection may be triggering factors for the development of leukocytoclastic vasculitis. IL12B or IL12RB1 deficiency and salmonellosis should be considered in MSMD patients with vasculitis.

Background: Information about renal involvement in pediatric patients with COVID-19 is limited, and there is not enough data about renal and urinary tract involvement in children infected with this novel virus. Objectives: This study aimed to determine the spectrum of kidney diseases in pediatric patients with COVID-19, admitted to a tertiary children’s hospital. Methods: This cross-sectional study was conducted on 71 pediatric patients with COVID-19 infection. Diagnosis of COVID-19 was established based on the guidelines by the Iranian Ministry of Health. The patients’ demographic characteristics, clinical symptoms, laboratory results, and renal ultrasonography findings were extracted from the hospital medical records. Results: On admission, 10% of patients had oliguria, 7.7% had edema, and 3% had hypertension. The first urinalysis indicated proteinuria, leukocyturia, and hematuria in 46%, 24%, and 23% of the patients, respectively. Overall, 40.7% of the patients showed some degree of renal involvement. During hospitalization, acute kidney injury (AKI) occurred in 34.5% of the patients. Based on the pediatric risk, injury, failure, loss of kidney function, and end-stage kidney disease (pRIFLE) classification, stage I (risk group) was found in 20% of patients, stage II (injury group) in 25% of patients, and stage III (failure group) in 55% of patients with AKI. The total mortality rate was estimated at 12.67%, and the incidence of in-hospital death was 30% in pediatric patients with severe COVID-19 infection associated with AKI. Conclusions: The prevalence of AKI was high in patients with COVID-19 infection hospitalized in our tertiary hospital. We also found that a decrease in renal function was associated with a higher risk of mortality. Overall, early detection of AKI and effective treatment may help reduce mortality in patients with COVID-19.

Introduction Hypercalcemia, defined as the total serum calcium concentration two standard deviations above the mean normal level, may be an emergency condition requiring proper initial diagnosis and immediate management. In association with nephrocalcinosis and hypercalciuria, differential diagnosis will be narrowed to limited diseases such as idiopathic hypercalciuria, hypervitaminosis A and D, and also renal tubular acidosis (RTA). Hypercalcemia is rarely presented in RTA with a few cases reported to date in the literature. Case presentation We reported a seven-month-old boy with complaints of refractory vomiting, restlessness, polyuria, polydipsia, and hypotonia. Laboratory tests revealed persistent hypercalcemia, hypokalemia, hypercalciuria, and metabolic acidosis with a normal anion gap. Bilateral nephrocalcinosis was detected in imaging studies. Based on these findings he was diagnosed with distal RTA and hypercalcemia and treated with polycitrate and Jaulie's solution. Conclusion Distal RTA should be considered as a differential diagnosis of hypercalcemia. Still, more studies are required for determining the pathway resulting in this rare association.

Food protein-induced enterocolitis syndrome (FPIES), is a non-IgE mediated food allergy presenting in infants younger than 12 months. Diagnostic delay may occur due to overlapping clinical symptoms with several conditions. Here, we present two cases of FPIES, mistakenly diagnosed and treated as Bartter syndrome. This study aims to emphasize the several features of this syndrome that may mimic other diagnoses and sometimes leading to near-death events due to delay in the diagnosis and improper treatment. The first patient was a 30-month-old boy with multiple episodes of profuse vomiting and diarrhea within 1 hour after breastfeeding, beginning from the first month of life progressing to hypokalemia and metabolic alkalosis at the age of 5 months leading to the diagnosis of Bartter syndrome. The second patient had a history of unremitting diarrhea which had been started soon after his first breastfeeding followed by biliary vomiting on the 7th day of life. He was treated in another hospital for neonatal sepsis, however, without an appropriate response to treatment. To conclude, despite the current belief on the rarity of FPIES, it is a more prevalent disease than expected with various non-specific manifestations imitating other conditions which may result in diagnostic delay and sometimes fatalities. To shed light on the importance of the physicians’ awareness of this syndrome, these two cases are presented here as examples of FPIES imitating other disorders.

Background: TNF-? is one of the most potent cytokines in proinflammatory reactions. Objectives: This article contributes to evaluate the correlation between urinary TNF-? and inflammatory response markers in children with acute pyelonephritis. Patients and Methods: This cross sectional study, was performed on children with acute pyelonephritis. Fresh random urine samples were obtained before treatment of pyelonephritis. Urine samples were tested for TNF-? and creatinine. We also evaluated our patients with routine biochemical studies. Results: One hundred and twenty children with acute pyelonephritis were evaluated. Urinary TNF-?/Cr was 0.0051 0.00083 Pg/g creatinine. The authors found out a significant difference in urinary TNF-?/Cr between patients who have normal and abnormal levels of urine leukocytes, urine protein, urine culture, erythrocyte sedimentation rate and C- reactive protein. This study revealed a significant correlation between urinary TNF-alpha and urine WBC (r = 0.36, P value = 0.02), ESR (r = 0.75, P value = 0.03) and CRP (r = 0.58, P value = 0.02). Conclusions: We concluded that urinary TNF-?/Cr might be a good indicator for inflammatory response in children with acute pyelonephritis.

Background: Urinary tract infection (UTI) is common in children. UTI with or without vesico-ureteral reflux (VUR) may result in renal scarring. Severe renal scarring impairs renal function and may result in hypertension, renal insufficiency, and end stage renal disease requiring dialyses or transplantation. Beta 2 micro-globulin (2MG) is a low molecular weight protein freely filtered by the glomeruli and then actively reabsorbed normally up to 99.9% in the proximal tubules; its urinary excretion is an indication of proximal tubular cell dysfunction. Objectives: The current study aimed to determine whether urinary 2MG excretion would be elevated in patients with various grades of renal scar, and also its relationship with renal outcome in long term follow-up. Materials and Methods: Urinary 2MG and Creatinine (Cr) were measured in 83 spot urine samples of patients that 53 of them did DMSA renal scan both at the time of admission to confirm pyelonephritis, and 6 month later to detect scars. 2MG was measured by radioimmunoassay method using 2MG 96-test kit (RADIM Company; Germany), and the creatinine was measurd by spectrophotometry and was recorded as microgram per mg creatinine. Twenty children had various grades of renal scars.Results were compared with the ratios of 19 children with low uptake scanning, 14 children with normal scanning after recovery from pyelonephritis, and 30 normal children served as controls. EXCEL and SPSS softwares were employed to compare the mean urinary 2MG in groups by student t-test, ANOVA, and Unpaired t-test at P,0.05 significance level. Subsequently patients were followed up for 6 years.\\r\\n Results: The mean urinary 2MG/Cr ratio was significantly higher in the scarring group (5.23 10.6) than in the normal group (0.19 0.2), and in low uptake group (0.49 0.86) (P < 0.05). When mean 2MG/Cr ratios were compared for each grade of scarring; patients with sever scar (grade III) had higher values (14.69 15.82) than grades I (0.36 0.35) and II (3.37 5.20) (P < 0.05). Patients without renal scar had a 2MG/Cr ratio below 0.46 microgram/mg Cr. The mean 2MG was also higher in the refluxing group (3.45 7.97) than nonrefluxing group (0.23 0.24) ug/mgCr (P = 0.01). Three patients who had the highest 2MG/Cr ratio values (33.3, 27, and 26.6 microgram/mg Cr) had sever scar that rapidly progressed to ESRD. They were transplanted 2 years later; after transplantation they still had recurrent UTIs. 2nd patient underwent native nephrectomy for renal abscess.\\r\\n Conclusions: Results of the current study revealed that mean 2MG/Cr ratio was higher in patients with renal scar and poor outcome. Measurement of Urinary 2MG may be useful in the early detection of tubular damage in refluxing patients and patients with renal scars and has prognostic significance.

Background: Renal scintigraphy with technetium 99m labeled dimercaptosuccinic acid (99mTc-DMSA) is a traditional imaging technique commonly used to detect renal scar in patients with probable vesicoureteral reflux (VUR) and/or urinary tract infection (UTIs). We determined whether normal results of DMSA renal scan obviate the need for voiding cystourethrography (VCUG) in evaluating children with UTIs. Materials and Methods: We observed medical records from June 2006 to April 2007 retrospectively of 208 children admitted with community acquired UTIs to Mofid children hospital (Tehran, IR/Iran) a teaching hospital in Tehran in which their age was between 2-120 months. The association between DMSA renal scan results and VCUG findings performed 48 hours and 1 month after the diagnosis was evaluated. To examine the accuracy of abnormal DMSA results in predicting VUR, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and negative and positive likelihood ratio (LRs) were calculated. Results: VUR was seen in 14.0% of renal units with normal results of DMSA and 17.3% of renal units with abnormal DMSA findings. High grade VUR (grade IIIV) was seen in 18 (7.1%) of the abnormal findings of DMSA group and in (2.8%) 1 of the normal DMSA results group (P = 0.56). In the group with previous UTI (n = 68), the sensitivity and NPV of abnormalities on DMSA renal scan for detecting the presence of VUR (grade IIIV) were 100%, and100%, respectively. In the group without evidence of previous UTI, the sensitivity and NPV of abnormalities on DMSA renal scan for detecting the presence of VUR (grade IIIV) were 93% and 97%, respectively. Totally the sensitivity and NPV of abnormalities on DMSA renal scan to detect the presence of VUR (grade IIIV) were 94% and 97%, respectively. Conclusions: As a screening test, DMSA renal scan is a high sensitive technique to assess VUR (grade IIIV) in children with UTI.

Background: The presence of vesicoureteral reflux (VUR) has been documented in 1.3% of the general population, 70% of infants with urinary tract infection (UTI), and 15–25% of children with UTI. Objectives: The main aims of this prospective cohort study were to compare the efficiency of different imaging techniques [renal ultrasonography, cortical scintigraphy with technetium-99m dimercaptosuccinic acid (99mTc DMSA)] in detecting VUR in patients with acute pyelonephritis. Patients and Methods: Between June 2003 and March 2010, we recruited a prospective cohort of patients aged 1 month to 14 years. Pediatric patients with documented urinary tract infections and imaging evidence of upper tract involvement were examined by DMSA scintigraphy, renal ultrasonography, and voiding cystourethrography (VCUG). The assessments included UTI profiling, kidney ultrasonography, and DMSA scans. Data were expressed as mean ± SD. Statistical tests were two-tailed and were considered significant when P ≤ 0.05. Results: A total of 1500 pediatric patients were eligible to receive treatment for pyelonephritis. DMSA scans were normal in 20.2% of the patients and abnormal in the remaining 79.8%, and the kidney ultrasonographies yielded normal results in 68.5% of the patients and abnormal results in the remaining 31.5%. There was a significant difference between the ultrasonography reports of patients with normal and abnormal DMSA scans. The VCUG results were normal in 74.1% of the patients and indicated VUR in the remaining 25.9% (VUR grade I in 10.7%, grade II in 7.3%, grade III in 4.7%, grade IV in 1.7%, and grade V in 1.5%). The refluxes were unilateral in 62.9% of the patients and bilateral in the remaining 37.1%. We found a significant correlation between the presence of VUR in VCUG and urological abnormality in ultrasonography (r = 0.14, P < 0.001). The incidence of VUR among patients showing severe abnormalities in DMSA scintigraphy, was significantly higher than that among patients with normal DMSA results or those showing mild to moderate changes in DMSA scintigraphy. Conclusions: We concluded that kidney ultrasonography and DMSA scans can be performed before VCUG in children with UTI. In addition, we recommend performing VCUG in cases of pyelonephritis only when the patients show abnormal kidney ultrasonography or DMSA scan results.

Studies of renal involvement in thalassemia syndromes have been varied and few. The most important cause of mortality and morbidity in these patients is organ failure due to iron deposition. We report here a cross-sectional study carried out between February 2005 and February 2006 on all beta-thalassemia major patients being treated in Mofid Children's hospital, Tehran. The aim of the study was to detect renal dysfunction in these patients. The patient cohort consisted of 103 patients with various disease severities. Fresh first morning urine samples were collected and analyzed for sodium (Na), potassium (K), calcium (Ca), creatinine (Cr), phosphate, uric acid (UA), N-acetyl beta-D-glucosaminidase (NAG) and amino acids. We also carried out a complete blood count evaluation and assayed fasting blood sugar and serum ferritin, sodium, potassium, creatinine, uric acid and amino acids in all patients. The mean age of our patient cohort was 12.5+/-5.53 years and 53.4% were female. Abnormal levels of urinary NAG were detected in 35.9% of patients (confidence interval 26-45%). Abnormal levels of fractional excretion (FE)-Na, FE-K and FE-UA and abnormal urine protein Pr/Cr and urine Ca/Cr ratios were present in 29.1, 7.8, 52.4, 0.3 and 22.3% of the patients, respectively. There was a significant relationship between urinary NAG and the age of the patient (R=0.35), duration of deferoxamine therapy (R= 0.31), duration of receiving blood transfusions (R=0.34) and level of fasting blood sugar (R=0.2). We concluded that renal disorders are not rare in patients with beta-thalassemia major and that they may increase in terms of frequency with age, increased duration of transfusion and deferoxamine usage and high levels of blood sugar.