Eculizumab in Pediatric Kidney Disorders: A Review

Mahnaz Jamee, Yeganeh Farsi, Amir Hossein Akhondi, Farnaz Kamali, Amir Hossein Hajialigol, Mahbubeh Mirzaee, Masoumeh Mohkam

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 103-111

Eculizumab is a humanized monoclonal antibody targeting the C5 (complement 5) member
of complement proteins and inhibiting its cleavage to C5a and C5b. Eculizumab has been
proven to be effective in a wide array of nephrologic, neurologic, and hematologic pediatric
disorders. Kidney disorders, particularly those with immune-mediated pathomechanism,
are the most common indications of eculizumab, including atypical hemolytic uremic
syndrome (aHUS), hemolytic uremic syndrome (HUS), membranoproliferative
glomerulonephritis (MPGN), immunoglobulin A (IgA) nephropathy, hematopoietic stem
cell transplant-associated thrombotic microangiopathy, and less commonly post-infectious
glomerulonephritis and diffuse proliferative lupus nephritis. In this review, we aimed to
summarize the current evidence on approved and off-label applications of eculizumab and
their specific considerations in pediatric kidney disorders.

Original Research Papers

Evaluation of the Clinical Findings of Pediatric Patients With Vesicoureteral Reflux to Assess Disease Severity

Ayşegül Alpcan, Yasar Kandur, Serkan Tursun

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 112-116

Background and Aim: This study aims to investigate how to benefit from clinical and
laboratory methods for further selection in the decision-making process to perform a
cystogram and assess the severity of vesicoureteral reflux (VUR).
Methods: We retrospectively reviewed the voiding cystourethrography (VCUG), ultrasound
(US), and medical records of pediatric patients with VUR. The exclusion criteria included
having neurological lesions, a posterior urethral valve, and a lack of documentation of a renal
ultrasound or voiding cystourethrography (VCUG). At the time of data entry, we retrieved
demographic findings and laboratory test results, including routine biochemical parameters,
complete blood count, and calculated blood sodium/potassium ratio
Results: Sixty-three pediatric patients with VUR were enrolled in this study. The mean (SD)
age of the patients (female/male=37/26) at the time of diagnosis was 62.0±6.5 months (range
1-195 months). Seventeen patients (26.9%) had high-grade VUR, and 46 patients (73.1%)
had low-to-moderate-grade VUR. The mean potassium level of the mean serum sodium to
potassium (Na/K) ratio was significantly lower in the high-grade VUR group (4.7±0.5 vs.
4.3±0.4 mEq/L, P=0.022, 29±3 vs. 32±3, P=0.029, respectively). The proportion of patients
with severe anterior-posterior (AP) diameter dilation was significantly higher in the lowgrade
VUR group than in the high-grade VUR group (4 [23.5%] vs. 35 [76.5%], P=0.005).
Conclusion: We conclude that the low serum sodium to potassium (Na/K) ratio allows us to
predict the VUR grade. The effect of VUR on the renin-angiotensin-aldosterone system can
be demonstrated by prospective controlled studies.

The Relationship Between the Renal Resistive Index and End Organ Damage Parameters in Hypertensive Pediatric Patients

Cihat Şanlı, Yasar Kandur, Ayşegül Alpcan, Tevfik Oğurel, Salih Koç, Serkan Tursun, Fatma Hayvacı Canbeyli

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 117-122

Background and Aim: This study aimed to investigate the strength of the relationship
between renal resistive index with left ventricular mass index, microalbuminuria, and
retinopathy to find a predictive value of the renal resistive index in early detection of
end-organ damage.
Methods: Pediatric patients diagnosed with hypertension were included in this study between
January 2020 and January 2022.
Results: A total of 41 consecutive patients who were never treated for essential hypertension
were included in this study. Microalbuminuria was detected in 10 patients (24%).
Retinopathy was detected in only 2 patients (4.9%). Echocardiographic data were available
in 25 patients. The mean left ventricular mass index was 30±10 (17-53 g/m2.7). Three male
patients (12%) had left ventricular hypertrophy but none of the females had left ventricular
hypertrophy. We categorized the patients into two groups according to the presence of
microalbuminuria and compared the two groups. The value of the renal resistive index in the
microalbuminuric group was significantly higher than in the nonalbuminuric group (0.8±0.2
vs. 0.6±0.2, P=0.047). We found a positive correlation between renal resistive index and
urine microalbumin (r=0.636, P=0.014). In regression analysis, higher resistive index values
were independently associated with microalbuminuria but not left ventricular hypertrophy.
(OR=-0.633, 95% CI 24.4-187.2, P=0.015)
Conclusion: To our knowledge, this is the first study to analyze the correlation of RI with
microalbuminuria in hypertensive children. We found a significantly higher resistive index
in patients with microalbuminuria; we also found a correlation between these variables.
This shows that this finding and an abnormal resistive index can be used as early signs of

Clinical Manifestations, Microbial Agents, and Types of Infection in Children With Urinary Tract Infection

Mitra Naseri, Elham Aghatabi, Niayesh Tafazoli, Nooshin Tafazoli

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 123-133

Background and Aim: Urinary Tract Infection (UTI) is common in children. This study
aims to evaluate demographic characteristics, etiologies, and clinical presentations of UTI
in childhood.
Methods: A cross-sectional retrospective study was conducted to investigate the available
data of the children with UTI referred to the nephrology clinic of a tertiary academic children’s
hospital from September 2002- 2016. Patients aged 0-18 years were included in the study.
Results: A total of 1245 cases, including 1084 girls (87.06%) and 161 boys (12.94%) were
included in the study. The age of patients at the first presentation was 35.43±34.94 months.
Fever was the most common manifestation (60.1%). The most and least cases of UTI were
reported in children aged 2-24 months (49.7%) and over 10 years old (2.9%), respectively.
The most common pathogens were E. coli, Klebsiella, Staphylococcus, Enterobacter,
Proteus, and Enterococcus species, respectively. The frequency of infection with E. coli in
girls was significantly higher than in boys (P=0001), while infection with Staphylococcus
and Proteus species was higher in boys (P=0.0001 and 0.002, respectively). The incidence
of pyelonephritis decreased with age. Furthermore, febrile UTIs were as common in boys as
in girls (P=0.42).
Conclusion: The prevalence of UTI is significantly higher in girls, and the frequency of UTI
varies according to age, reaching its peak in the first year of life, and the lowest frequency in
children over 10 years of age. Moreover, pyelonephritis is more common in younger children
(infants 2-24 months). Gender is a crucial factor in terms of pathogens.

Clinical Outcomes of Children With Prenatal Hydronephrosis

Milad Ghadery, Behnaz Bazargni, Arash Abbasi, Mastaneh Moghtaderi

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 134-138

Background: Fetal hydronephrosis (HY) is a frequent congenital condition that may be
detected by prenatal ultrasound studies. The main underlying abnormalities found in these
cases after birth are ureteropelvic junction stenosis, primary vesicoureteral reflux, megaureter,
duplex kidneys, and posterior urethral valves, with uretero-pelvic junction stenosis and
primary vesicoureteral reflux accounting for most cases.
Method: Neonates with a prenatal diagnosis of congenital HY born between 2017 and 2020
were referred to our center for the appropriate management and follow-up and were included
in this cross-sectional study. Ultrasonographic studies were performed in all these neonates
in the first week of birth. Prenatal HY was categorized as mild, moderate, severe, and highly
severe when the antero-posterior diameter of the renal pelvis was less than 5 mm, between 5
mm and 10 mm, between 10 mm and 15 mm, and more than 15 mm, respectively.
Results: A total of 164 children with prenatal HY were included in this study. 84 patients
(51.2%) had unilateral HY and 80 patients (48.8%) had bilateral HY. Twelve patients had
posterior ureteral valves and 64 patients (39%) had vesical-urethral reflux (VUR). no
significant relationship was observed between unilateral or bilateral reflux and outcome (P
value 0.645).
Conclusion: Prenatal screening of HY is vit

Case Reports

Idiopathic Hematuria in a Near-Term Neonate: A Rare Case and its Workup

Beau Redwood, Chia Saw, Karthikeyan Kulasekaran

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 139-142

Neonatal hematuria is a very rare occurrence and is mostly due to secondary pathology. This
case report describes a rare case of a near-term male neonate with gross Hematuria seen
immediately after birth. Urine microscopy confirmed hematuria without growth in urine
or blood cultures. A full blood count and coagulation profile showed no obvious cause for
the Hematuria, and a renal tract ultrasound showed a mobile haematoma in the bladder but
no other structural abnormalities. The newborn received an additional dose of intravenous
vitamin K and was managed conservatively. The Hematuria resolved on the second day of
life without recurrence on his 12-month follow-up, with no clear cause for the presentation.
We discuss a rare case of neonatal gross Hematuria, its differential diagnoses, and clinical

Kawasaki Disease and Pyelonephritis: A Case Report

Sayed Yousef Mojtahedi, Zahra Noparast, Amir Shahnazizadeh , Ali Talebi , Mohammad Kajiyazdi

Journal of Pediatric Nephrology, Vol. 10 No. 3 (2022), 12 December 2022, Page 143-146

Kawasaki disease is a form of vasculitis, mainly in small and medium-sized arteries of
unknown origin, and often occurs in childhood. It is the leading form of heart disease acquired
in childhood in developed countries. Pyelonephritis is not usually associated with Kawasaki,
but hereby we represent a case of Kawasaki disease associated with pyelonephritis; however,
we can’t say whether pyelonephritis had a role in stating Kawasaki and it could be a proper
and valuable topic for research.

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