Background and Aim:

Gastroenteritis is a common disease in children that can lead to various complications and dehydration. Vitamin E is a fat-soluble vitamin and antioxidant. The aim of this study was to investigate the effect of vitamin E on reducing the duration of treatment in children with gastroenteritis.

Methods:

This randomized interventional study was conducted on 60 children aged 12 months to 13 years with gastroenteritis admitted to Amirkabir Hospital in Arak. The patients were divided into two equal groups (30 children each) using block randomization: an intervention group receiving vitamin E for 10 days along with standard gastroenteritis treatment (fluid therapy and ORS) and a control group receiving only standard treatment (fluid therapy and ORS). After the treatment period, both groups were evaluated for clinical improvement based on symptoms, including diarrhea, vomiting, abdominal pain, and dehydration, which were recorded in a checklist for each patient. The data from both groups were analyzed statistically using SPSS software.

Results:

The mean age of children in the vitamin E and control groups was 5.20 and 5.67 years, respectively. The two groups showed no significant differences in age or gender (P=0.106 and P=0.437, respectively). The mean duration of diarrhea, abdominal pain, and anorexia was significantly lower in the vitamin E group compared to the control group (P<0.0001). However, there were no significant differences between the two groups in the duration of fever, dehydration, or vomiting (P=0.810, P=0.536, and P=0.685, respectively).

Conclusion:

Overall, the findings indicate that vitamin E reduces the duration of diarrhea, abdominal pain, and anorexia in children with gastroenteritis but has no effect on the duration of fever, dehydration, or vomiting.

Background and Aim:

      Hypercalciuria, hyperoxaluria, hereditary renal tubular disorders, and drug intoxication are the etiologies of nephrocalcinosis in children. This study was conducted to determine the etiologies and short-outcome (resolution rate) of nephrocalcinosis.

Methods

Children aged ≤18 years old with nephrocalcinosis who were referred to the nephrology clinic of an academic center from March 2003 to 2019 were considered for the study. Measurement of serum creatinine, sodium, potassium, calcium, phosphorous, blood gas analysis and random or 24-hour urine samplings for creatinine and calcium levels were the inclusion criteria.

Results

Of 49 cases, 43 met the inclusion criteria. They included 21 girls (48.8%). The median age of patients was two years (10 months - eight years). They followed up for a median of 9 months (1-34 months). Medullary nephrocalcinosis grades III, II, and I were reported in nine (20.9%), one (2.3%), and 33(76.8%) patients, respectively. The etiologies of nephrocalcinosis were unknown (41.8%), idiopathic hypercalciuria (34.9%), idiopathic hyperoxaluria (18.6%), distal RTA (9.3%). Hypophosphatemic rickets, Dent disease, and Bartter syndrome were found each in one patient. Seven cases (16.3%) had mixed abnormalities. Resolution of nephrocalcinosis at a follow-up of ≥ six months was uncommon (19.2%). The median weight-for-age Z score at presentation was -0.8 which reached to -0.36 at the last follow-up (P =0.011).

Conclusion

We found idiopathic hypercalciuria and idiopathic hyperoxaluria as the most common identified etiologies, respectively. Hereditary tubular disorders accounted for 16.3% of the cases. Resolution of nephrocalcinosis was uncommon even in nephrocalcinosis grades I, at a follow-up of ≥ six months.

Keywords: Medullary nephrocalcinosis; Hypercalciuria; Hyperoxaluria; Distal RTA; Outcome

Background and Aim: Pediatric acute kidney injury (AKI)’s incidence, etiology, and
outcomes are very diverse and influenced by factors, such as age, location, and clinical
situation. Emphasizing regional epidemiology and its underlying causes is crucial for
improving the identification and management of this insidious issue.
Methods: This retrospective study was done at the Pediatric Nephrology Department
of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh, a
developing country in South Asia. It included AKI patients aged 28 days to 18 years who
attended outpatient and inpatient departments between July 1, 2022, and August 31, 2024.
Results: In a 24-month span, 356(27.05%) instances of AKI were detected, with 5 to 15
years (55.06%) being the most prevalent age range. The etiology of AKI was identified to be
prerenal in 154 cases (46.26%), renal in 170 instances (47.75%), and postrenal in 32 cases
(8.98%). Of the total number of patients, 290(81.46%) were managed with conservative
treatment, while 20(5.62%) required hemodialysis and 46(12.92%) required peritoneal
dialysis. Over a three-month period, 302 children (84.83%) remained alive, with 17(4.78%)
developing CKD, 22(6.18%) developing end-stage renal disease (ESRD), and 15(4.21%)
passed away (P<0.0001). The presence of anuria, volume overload, and primary renal
disease were identified as significant risk factors for beginning renal replacement therapy
(hemodialysis and peritoneal dialysis).
Conclusion: Pediatric AKI is mainly caused by gastroenteritis, sepsis, and rapidly progressive
glomerulonephritis (RPGN), which recover well at a single tertiary care center. Oliguria/
anuria, anemia, volume overload, failure (pRIFLE category) at presentation, and dialysis
necessity were correlated with unfavorable renal outcomes.

Background: Nephrotic syndrome (NS) affects 1.5 to 16.9% of children, with idiopathic cases categorized into minimal change and other types. Elevated serum immunoglobulin E (IgE) levels have been linked to frequent relapses and steroid dependency in both children and adults with minimal change NS.

Materials and Methods: This prospective cohort study included patients with NS. Serum samples for IgE levels were collected prior to initiating steroid therapy (2 mg/kg/day). Patients were discharged after achieving remission, following institutional protocols and ISPN guidelines. They were then categorized into high IgE and normal IgE groups, with characteristics compared. The number of relapses, complications, and any additional medications used during the study period were recorded.

Results: A total of 72 patients were included in the study. The mean serum IgE level was higher in the frequent relapser, steroid resistant, steroid dependent group as compared to the infrequent relapser and non-relapser group. All patients with poor response to steroid therapy and needing second line drugs had high serum IgE. In patients with a higher serum immunoglobulin E level the mean hospital stay duration was higher than in patients with a normal serum immunoglobulin E level (P=0.046). Patients with a higher serum IgE level had a higher incidence of infections, complications, poor response to therapy and use of second-line drugs (immunosuppressants), took longer time for remission. We got a significant correlation between the serum IgE levels and outcome (P<0.05)

Conclusion: Serum IgE levels were shown to be associated with disease activity and outcome in children with  primary NS.

Background and Aim: A renal length discrepancy (RLD) of more than 10 mm by ultrasound
(US) is accepted as a potential indicator of an underlying renal pathology. This study aimed to
determine the predictive value of RLD detected by US for identifying abnormal findings on
kidney radioisotope scans, specifically dimercaptosuccinic acid (DMSA) scans, in children.
Methods: The study involved a prospective data analysis of children who underwent renal
US and DMSA scans. The positive and negative predictive values for renal RLD were
calculated for values ranging from 6 to >10 mm.
Results: The left kidney was longer in 51.4% of cases, whereas the right kidney was longer in
35.7%; their lengths were equal in 12.9%. The results indicated that a specific degree of RLD
in the US could predict abnormalities on DMSA scans with varying degrees of accuracy,
depending on the age group. The cutoff ultrasound RLDs that resulted in the best specificity
and sensitivity for an abnormality on DMSA scan were ≥5 mm in children whose right kidney
was longer than the left, and ≥6 mm for children whose left kidney was longer than the right
(specificity of 88% and 84%, respectively).
Conclusion: Renal length discrepancy observed via ultrasound can be a valuable, noninvasive
tool in predicting renal abnormalities detectable by DMSA scan in children, aiding
clinical decision-making and potentially reducing the need for more invasive procedures.
However, ultrasound has moderate sensitivity and reasonable specificity for predicting
positive DMSA scan results and detecting high-grade vesicoureteral reflux (VUR).

Background and Aim:
Children with chronic kidney disease (CKD) undergoing maintenance hemodialysis (HD) are highly susceptible to infections and disturbances in mineral metabolism, including elevated calcium–phosphate (Ca×P) product. Intradialytic exercise has been proposed as a safe non-pharmacologic intervention, but evidence in pediatric HD populations is limited. This pilot study aimed to evaluate the feasibility of an eight-week combined intradialytic exercise program and its effects on infection rate and Ca×P product in children receiving HD.

Methods:
This quasi-experimental single-group pre–post pilot study included children aged 8–18 years undergoing maintenance HD. Participants performed supervised combined resistance and aerobic exercise during HD sessions three times per week for eight weeks. Infection events were recorded using standardized clinical and laboratory criteria. Serum calcium and phosphate were measured before and after the intervention, and Ca×P product was calculated. Pre- and post-intervention values were compared using paired statistical analyses, and infection rates during exercise and non-exercise periods were compared.

Results:
Nine children initiated the program and five completed the intervention. Mean Ca×P increased from 52.70 ± 17.71 to 59.43 ± 8.43 mg²/dL², with no statistically significant difference (p = 0.36). Mean infection rate was 10% during exercise months and 16% during non-exercise months, with no significant difference between periods (p = 0.673). The exercise protocol was implemented safely and without major adverse events.

Conclusion:
An eight-week combined intradialytic exercise program was feasible and safe in pediatric HD patients, but did not significantly affect infection rate or Ca×P product in this small pilot study. Larger controlled trials are required to clarify the potential clinical benefits of intradialytic exercise in this population.

Background and Aim: Urinary tract infections (UTIs) are among the most common
bacterial infections in children and adolescents. Despite standard antibiotic therapy, symptom
resolution can be delayed due to ongoing inflammation. Melatonin, a neurohormone with
known antioxidant and immunomodulatory properties, may offer therapeutic benefits in this
context. This study aimed to evaluate the clinical efficacy of adjunctive melatonin therapy in
pediatric patients with UTIs, focusing on symptom resolution and potential anti-inflammatory
effects.
Methods: In this double-blind, randomized, placebo-controlled trial, 100 children aged
4–18 years with confirmed UTI and concurrent sleep disturbances were assigned to receive
either melatonin (3 mg/day) plus antibiotics (intervention group) or antibiotics plus placebo
(control group) for 10 days. Primary outcomes included time to resolution of fever, dysuria,
abdominal pain, urinary frequency, and incontinence.
Results: Baseline characteristics were similar between groups. The melatonin group showed
significantly faster resolution of fever (1.3±0.49 vs 3.7±0.69 days, P=0.001), abdominal pain
(1.2±0.54 vs 3.62±0.67 days, P=0.001), and dysuria (1.5±0.57 vs 3.6±0.67 days, P=0.001).
No significant differences were observed in urinary frequency or incontinence outcomes. No
adverse effects related to melatonin were reported.
Conclusion: Adjunctive melatonin therapy appears to accelerate the resolution of key UTI
symptoms in children when combined with antibiotics, likely due to its anti-inflammatory
and antioxidant properties. These findings suggest a potential role for melatonin in pediatric
UTI management and warrant further investigation in larger, multicenter trials.

Abstract:

Introduction:

Nephrotic syndrome is a disease requiring long duration of steroids as  treatment. In this study we are analysing various factors, in children with this condition, which would predict delayed remission, hence requiring longer courses of steroids.

Methods:

This is a retrospective study, in children under 18 years, with nephrotic syndrome. Two hundred and three admissions were analysed. Age, course of nephrotic syndrome, presence of hypertension, cholesterol levels >400mg/dl, neutrophil to lymphocyte ratio, platelet to lymphocyte ratio, hyponatraemia and hypoalbuminemia were considered. Correlation of these parameters to delay in achieving remission (> 7 days) were done, followed by multinomial logistic regression. The score formulated from the resulting coefficients, was then tested on 63 separate cases using Receiver Operator Curve (ROC), and Area Under Receiver Operator Curve (AUROC) .

Results:

Significant spearman correlation (rho) were seen with - presence of hypertension (0.347), course of nephrotic syndrome being frequently relapsing or steroid resistant (0.319) , neutrophil to lymphocyte ratio >1.5 (0.312), cholesterol levels > 400mg/dl (0.201) and  age > 10 years (0.201.

The multinomial logistic regression model showed coefficients of 2.81, 2.81, 1.98, 1.198, 1.063 for presence of hypertension, course of nephrotic syndrome, cholesterol level, Neutrophil to lymphocyte ratio and age, respectively.

A score of more than or equal to 4  predicted delayed remission, with a sensitivity and specificity of 85.6% and 83.7%. The AUROC of this score on a separate group of 63 children showed to be 0.835

Conclusion:

This score helps us to predict the cases of nephrotic syndrome, where delayed remissions occur, requiring prolonged courses of steroids, and hence leading to the ill effects of steroids.

Background and Aim:

Vesicoureteral reflux is the abnormal return of urine from the bladder to the urethra and kidney. Muscle weakness means the decrease in muscle force. Due to witnessing uremic myopathy in patients with kidney diseases we will study the relation between muscle weakness and reflux nephropathy in this article.

Methods:

In this case control study, a total number of 118 patients referred to Amirkabir hospital in the city of Arak were divided into two groups. The first group, patients with reflux disease(N=59) and the second group, healthy patients(N=59) based on VCUG results approved by urologists. The two groups were then examined by a neurologist for hypotonia and muscle weakness. The information obtained was recorded in the checklist

Results:

There was a significant difference in regards to the gender of the case and control groups, however no significant difference was observed regarding their age, birth weight, birth rate and neonatal situation. None of the patients had a history of hypotonia or muscle weakness. Out of the patients who had reflux, 16(22.7%) had right side reflux, 13(22.4%) had left side reflux and 29(50%) had double sided reflux.

Conclusion:

No patient from either group had signs of hypotonia or muscle weakness.

Background and Aim: Renal Angina Index (RAI), which combines risk strata and clinical
injury signs of acute kidney injury (AKI) helps identify sick paediatrics at risk of developing
severe AKI. This study aimed to estimate the proportion of critically ill children with a
positive RAI score at admission who develop severe AKI on day 3 of paediatric intensive
care unit (PICU) admission.
Methods: This prospective follow-up study was done among 100 children without
preexisting kidney disease and with at least 3 days of stay in the pediatric intensive care unit
of a tertiary care teaching hospital in South India. The RAI score was calculated at admission.
The KDIGO score was applied on day 3 of admission. The proportion of children with a
positive RAI score (8 or more) who developed severe AKI (the kidney disease: Improving
global outcomes [KDIGO] stages 2 and 3) was calculated. Short-term outcomes in terms of
duration of hospital stay and mortality were also studied.
Results: A total of 100 children aged 29 days to 12 years were included in the study. Fifty-one
percent of them were found to be RAI-positive at admission. Also, 41.2% of RAI-positive
children developed severe AKI on day 3 of admission. Among the 31% of children who were
diagnosed with severe AKI by day 3 of admission, 67.7% had a positive RAI score at the
time of admission (P<0.05). AKI was found to be associated with increased median duration
of hospital stay.
Conclusion: The RAI score can predict AKI in critically ill children.

Background and Aim: Wide variations exist regarding different histopathological types
and treatment outcomes of idiopathic steroid-resistant nephrotic syndrome (iSRNS) in
children. This study has been conducted to evaluate clinicopathological profile and shortterm
outcomes of iSRNS in children and to analyze predictive factors for outcomes.
Methods: This cross-sectional observational study was conducted at a tertiary care hospital
in eastern India over a period of 18 months. Children with iSRNS aged 2–12 years, with
an available renal biopsy report, for whom treatment was started within six months, were
included. Data were collected at baseline and at 6 and 12-24 months.
Results: Of the 40 children with iSRNS (mean age: 1 year and 4 months; male: female
ratio: 1:1), 30% and 70% respectively had hematuria and hypertension at the onset of
nephrotic syndrome. Late SRNS was more common (55%) than initial SRNS (45%).
Before the development of late SRNS, the percentages of steroid-dependent, infrequent
relapsing and frequent relapsing nephrotic syndrome were 45.4%, 41% and 13.6%,
respectively. Focal segmental glomerulosclerosis (62.5%), minimal change nephrotic
syndrome (32.5%), and IgM nephropathy (5%) were the prevalent renal biopsy findings,
with no association to the clinical features or renal parameters. 75% of participants
received cyclosporine, and the rest received tacrolimus. The percentages of complete
remission, partial remission and no response at 6 months were 70%, 12.5% and 17.5%,
respectively; and at the end of 12–24 months they were 80%, 0% and 20%, respectively.
No predictive factors for outcome were found.
Conclusion: The present study explored the complex nature of iSRNS in children.
Development of SRNS cannot be predicted by any clinicopathological factors. Early age at
onset of NS and the presence of hypertension are likely indicators of subsequent development
of SRNS. A large percentage of iSRNS achieved complete remission with effective therapies
available in the era of modern medicine.

Background and Aim: Childhood idiopathic nephrotic syndrome (INS) needs long-term
treatment as well as frequent hospitalization. This study aimed to assess the economic impact
on families with childhood INS.
Methods: This cross‐sectional study was conducted on children aged 1–17 years with INS
in the Department of Pediatric Nephrology from January 2021 to December 2021. Baseline
demographic characteristics including age, gender, type of nephrotic syndrome, duration
of disease and treatment, number of hospitalizations, treatment costs, educational status of
parents, and socio-economic status using modified Kuppuswamy’s socio-economic status
scale 2021 were recorded. The economic impact of nephrotic syndrome on families was
assessed during parental interviews. Data were analyzed using SPSS software, version 19.
Results: Among the 63 families, most were in the upper-lower economic class (55.6%),
91.8% had to disrupt work, 61.9% had to bear expenses from more than one source, 65.6% in
which one family member needed to take on an additional job, 87.3% had to travel to another
district, 76.2% needed to spend money on accommodation for the accompanying person,
41% of families had to face moderate financial hardship and 14.8% had to face great financial
hardship, 28.15% had to take out a loan. There was no significant difference in the economic
impact on families among various INS subtypes.
Conclusion: Most families disrupted work, spent money from more than one source, had
family members take on additional jobs, faced moderate to great financial hardship, and
needed to take out loans.

Background and Aim: Chronic kidney disease (CKD) in children poses significant
psychological challenges for parents, potentially leading to elevated levels of stress, anxiety,
and depression. This study aimed to compare levels of stress, anxiety, health anxiety, and
depression between parents of children diagnosed with CKD and parents of healthy children.
Methods: This cross-sectional comparative study included 70 parents (35 parents of
children with chronic kidney disease and 35 parents of healthy children) recruited from Amir
Kabir Children’s Hospital in Arak, Iran. Psychological outcomes, including stress, anxiety,
depression, and health anxiety were assessed using the depression, anxiety, and stress scale
(DASS-21) and the 18-item health anxiety inventory (HAI-18). Statistical analyses was
performed using SPSS software, version 26.
Results: Parents of children with CKD reported significantly higher levels of anxiety
(P=0.044) and stress (P=0.047) compared to the control group. However, there were no
statistically significant differences in depression levels (P=0.117) or health anxiety (P=0.840).
Demographic variables showed no significant correlation with psychological outcomes.
Conclusion: Parents of children with CKD experience elevated levels of stress and anxiety,
underscoring the need for targeted psychological interventions to support their mental health.

Background and Aim: Nephrotic syndrome ranks as a prevalent chronic renal disorder
among children, leading to various challenges, including low blood albumin levels, swelling,
heightened infection risks, and dietary issues. The pivotal role of nursing interventions in
mitigating and managing these complications is undeniable. This research evaluated the
impact of nursing practices on managing nephrotic syndrome-related complications in
pediatric patients.
Methods: For this narrative review, studies from 2013 to 2025 were examined, utilizing
databases, such as Web of Science, PubMed, Scopus, SID, Magiran, and Google Scholar with
appropriate search terms.
Results: The findings indicated that critical nursing measures, such as educating parents,
consistent monitoring of fluid and electrolyte balance, skin care, dietary modifications, and
ongoing psychosocial support significantly contribute to lessening complication severity and
enhancing life quality for affected children.
Conclusion: The study underscores the critical importance of nurses in the holistic
management of nephrotic syndrome in children.

Pediatric chronic kidney disease (CKD) represents a complex and evolving clinical entity with
profound implications for long-term morbidity and quality of life. Unlike adult CKD, where
the therapeutic focus often centers on dialysis adequacy and cardiovascular comorbidities,
pediatric CKD care necessitates a developmentally sensitive and growth-oriented approach.
One of the most critical, yet under-recognized, metabolic complications in this population is
persistent hyperphosphatemia—a state of phosphate overload that exerts pathophysiological
effects across skeletal, cardiovascular, endocrine, and neurological systems.
Phosphate is essential for multiple physiological processes, including skeletal mineralization,
energy metabolism via ATP synthesis, cellular signaling pathways, and the regulation
of acid-base balance. These specialized formulas
are designed to meet growing children’s full caloric, protein, and micronutrient needs while
minimizing the intake of inorganic and highly absorbable phosphate. This systematic review
explored the biochemical basis and clinical relevance of phosphate dysregulation in pediatric
CKD, delineated its systemic complications with an emphasis on growth and bone health,
and critically appraised the emerging role of hypophosphatemic formulas as a cornerstone therapeutic intervention. It synthesized findings from the current international clinical
guidelines—including KDIGO, KDOQI, and ESPEN—while identifying critical gaps in
pediatric-specific evidence. Moreover, it discussed the practical aspects of implementing
hypophosphatemic strategies, such as regional availability, cultural adaptability, formulation
palatability, and caregiver education.

Background and Aim: Hematuria in an infant is not very uncommon. Nephrocalcinosis
and renal stones frequently cause hematuria and urinary tract infections in early infancy.
Furthermore, xanthinuria is a rare metabolic disorder that can lead to the abnormal
accumulation of xanthine, resulting in nephrocalcinosis and the subsequent formation
of renal stones. It is an autosomal recessive genetic disorder caused by a lack of xanthine
dehydrogenase, which breaks down hypoxanthine and xanthine into uric acid in the final two
steps of the purine degradation process. Only a few instances have been noted worldwide.
Furthermore, pediatric xanthinuria has never been reported from Bangladesh.
Case Presentation: Herein, we discussed a 2-month-old male infant who had symptoms of
excessive crying during micturition and microscopic hematuria, later diagnosed as a case of
xanthinuria type 1.
Conclusion: Hereditary xanthinuria may present with hematuria and nephrocalcinosis.
Confirmation by genetic testing is useful for identifying the type of hereditary xanthinuria
and predicting the outcomes.

Background: Multisystem inflammatory syndrome in children is a serious state in which a patient (typically infected with SARS-CoV-2) manifests symptoms of multi-organ failure a few weeks after exposure due to broad adaptive and innate immune activation.

            Case presentation: A 13-year-old boy with history of multiple trauma and subsequent lower limb fracture followed by orthopedic surgery, presented to the emergency department with surgical site infection (SSI). After 6 days of admission, patient represented symptoms of multisystem inflammatory syndrome including renal shutdown and blood creatinine rise. As a result, hemodialysis was performed. Patient's blood culture was negative and all of the similar clinical situations (like sepsis, toxic shock, etc.) was ruled out during orthopedic surgery and infectious disease specialties' consultations.

            Conclusion: MIS-C is a syndrome which can be emerged not only secondary to SARS-CoV-2 infection, but also can be developed by surgical site infections secondary to orthopedic surgeries. Regular organ function monitoring in patients with SSI complications can be very beneficial in early diagnosis and preventing complications.

Background and Aim: Kidney biopsies are seldom performed these days in neonates,
especially after the advent of advanced antenatal imaging and the widespread availability of
genetic studies.
Case Presentation: We described a newborn with kidney failure, for whom a biopsy was
conducted, leading to a diagnosis of glomerulo cystic kidney disease.
Conclusion: Sporadic glomerulocystic kidney disease can manifest as renal failure during
the newborn period. Renal biopsy may be used to diagnose glomerulocystic kidney disease
in this setting.

Background and Aim: Chronic kidney disease (CKD) is one of the most important problems
in children, sometimes associated with many co-morbid problems, such as intractable pain.
The pharmacokinetics of almost all analgesics are very different in CKD patients compared
to the normal population. Because of the accumulation of toxic metabolites, many of the
commonly used pain medications have more adverse effects and increased potential for
central nervous system toxicity.
Case Presentation: We reported an eleven-year-old girl affected by end-stage renal disease
(ESRD) due to focal segmental glomerolosclerosis (FSGS) nephrotic syndrome who
presented with decreased level of consciousness, behavioral dysfunction, and neurological
complications following a small dose of codeine consumption.
Conclusion: Even small doses of codeine can lead to severe, prolonged neurotoxicity in
pediatric ESRD. Appropriate drug selection and dosing in patients with CKD is necessary
to ensure optimal outcomes and avoid unwanted complications. Prescribing a standard dose
of analgesics without consideration of the patient’s renal function state may cause serious
complications.

Membrane attack complex (MCP) deficiency drives 10-15% of complement dysregulation hemolytic uremic syndrome (cHUS). We report a seven-year-old girl presented hemolytic uremic syndrome. Complement pathway assessment showed persistently very low level of CD46. She was on kidney replacement therapy (KRT) for a while and because of partial recovery of kidney function, she was off for few years. She admitted several time for pneumonia, crisis of hypertension, and relapse that treated accordingly. The whole exome sequencing was in favor of Membrane cofactor protein (MCP; CD46) mutation. At age 12.5 years she admitted with fever and dyspnea.  A coronary artery aneurysm detected by echocardiography. Intravenous immunoglobulin, steroid, and aspirin were given to prevent thrombosis. Afterward, she passed away after three -year irregular and inconsistent follow up.  This complication has never reported in the literature.