Reviews


New Attention to Vesicoureteral Reflux In Children: A Review Article

Mohsen Akhavan Sepahi, Mohammad Hossein Akhavan Sepahi

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.42652

Background and Aim: Vesicoureteral reflux (VUR) as a known cause of urinary tract
infection (UTI), renal scarring, and nephropathy, is congenital and often familial. The
prevalence of VUR is unclear, although most cases of VUR resolves spontaneously, the
management of children with VUR is controversial. The purpose of this review was to
evaluate VUR in order to provide an update on management to improve its prognosis.
Methods: The articles from several sources, including PubMed, Scopus, Embase, Google
Scholar, Web of Science, and the Directory of Open Access Journals, were included.
Conclusion: Due to various complications, VUR is very scary for patients or families, and
special attention is needed. A challenge for pediatric nephrologists is the early diagnosis
of VUR and the progressive complications of kidney disease. There is no internationally
accepted, uniform, evidence-based algorithm for the assessment of reflux anywhere.

Original Research Papers


Anti-HBs Titer in Children With Nephrotic Syndrome Admitted to a Tertiary Care Hospital

Asma Akter, Delwar Hossain, Wahida Khanam, Mohammad Ahad Adnan, Khaleda Zaman, Afiya Sultana

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.42987

Background and Aim: Nephrotic syndrome (NS) is the most common pediatric renal
disease. Immune dysregulation, prolonged immunosuppressive treatment, and recurrent
prolonged proteinuria in NS cause alterations in serum immunoglobulins, especially
hypogammaglobulinemia. Thus, anti-HBs titer may be reduced in NS patients. We assessed
anti-HBs titer among hepatitis B-vaccinated children with NS.
Methods: This case-control study was conducted at the Department of Paediatrics of the
Institute of Child & Mother Health, Dhaka, from July 2020 to June 2021. Sixty-one children
with primary and recurrent NS previously vaccinated according to the expanded programme
on immunization program were evaluated for anti-HBs titer and compared with 61 age- and
sex-matched healthy children.
Results: Protective anti-HBs titer was found in 29(47.5%) and 40(65.6%) cases in the case and
control groups, respectively. The mean anti-HBs titer was 37.2±35.5 IU/L in the case group
and 55.7±28.3 IU/L in the control group, which showed a significant difference between the
groups. The mean anti-HBs titer was 52.9±35.5 IU/L in the first attack, 33.9±36.8 IU/L in the
infrequent relapse nephrotic syndrome (IFRNS), and 22.2±27.41 IU/L in the frequent relapse
nephrotic syndrome (FRNS), respectively. The difference was also significant statistically.
The mean anti-HBs titer was lower in the FRNS and IFRNS and significant in the FRNS
compared to the first attack. The mean anti-HBs titer was significantly (P<0.05) lower in the
IFRNS and FRNS compared to the controls.
Conclusion: Anti-HBs titer was found significantly lower than the protective level in the first
attack and relapse cases of NS.

Correlation Between Biodemographic Parameters and the Size of Inferior Vena Cava and Collapsibility Index Using Ultrasound in Children

Hamidreza Badeli, Jahangir Kamalpour, Mojgan Pejman, Shabnam Aghajanpour, Golnar Mortaz Hejri, Afagh Hassanzadeh Rad, Zahra Atrkar Roshan

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.43202

Background and Aim:There is a concern regarding the relationship between biodemographic
parameters at different ages and the size of inferior vena cava (IVC) and the collapsibility
index (CI). Due to the lack of normative data on these parameters in children, we aimed to
use ultrasound to determine the mean sizes of IVC in healthy children and calculate the CI.
Methods: In this analytical cross-sectional study, we measured the IVC diameter in euvolemic
children aged four weeks to 12 years. The maximum IVC diameter was recorded during
the exhalation phase of the respiratory cycle, while the minimum diameter was recorded
during the inhalation phase using M-mode. Additionally, we calculated the CI by dividing the
difference between the maximum and minimum IVC diameters by the maximum diameter.
Results: In this study, 534 euvolemic healthy children with a mean age of 6.77±3.22 years
were assessed. The mean diameter of the maximum IVC during exhalation was 5.26±4.70
and the mean diameter of the minimum IVC during inspiration was 2.96±2.89 mm. The
mean CI in the present study was 0.5±0.13. Ultrasound measurements of IVC diameter
during exhalation, unlike IVC diameter during inhalation, were positively correlated with
age, weight, and height. The mean IVC and CI had a direct and significant correlation with
biodemographic parameters, such as age, height, weight, and body mass index.
Conclusion: Evaluating intravascular volume status holds significant clinical relevance,
particularly in pediatric patients. Utilizing ultrasound to assess the IVC allows for swift and noninvasive analysis of an individual’s hemodynamics, impacting clinical decision-making positively.
Establishing normative IVC measurements in healthy and euvolemic children can serve as valuable
reference data for clinicians and help them accurately assess fluid status in unwell pediatric patients.

Background and Aim: The spike glycoprotein is a prime focal point for vaccine development
due to its possession of numerous T-cell and B-cell epitopes. In this study, we investigated
the effects of some important mutations (K444T, N460K, F490S, L452R, and T478R) on the
immunogenicity of the spike protein in the Omicron variant. Additionally, we forecasted the
effects of these mutations on the spike protein’s solubility, allergenicity, and immunogenicity.
Methods: In this research, we obtained 100 SARS-CoV-2 spike sequences from two
databases, namely the Global Initiative on Sharing All Influenza Data (GISAID) EpiCoV and
NCBI. We conducted a comparative analysis between the wild-type spike protein (Wuhan
accession number: NC_045512.2) and the mutated spike proteins. The analysis focused on
solubility, allergenicity, and immunogenicity. It was carried out using various bioinformatics
servers, such as Dynamut, toxin pred, soluprot, Allertop, IEDB, and Vaxigen, as well as tools,
like Mega XI and Pymol II.V.II visualizer.
Results: According to the prediction of the IEDB server, the K444T mutation is likely to
decrease the humoral immune response. In addition, spike proteins in wild types and mutants
do not have allergenic properties, and these proteins are soluble and can be expressed in
Escherichia coli.
Conclusion: Vaccines formulated using spike protein design are effective. These findings
indicate the potential for developing pan-coronavirus vaccines that offer protection not only
against SARS-CoV-2 but also against a range of other coronaviruses in the future

Humoral Immunity Status in Pediatric Patients with Familial Mediterranean Fever: Exploring the Role of IgA

Gökçe Mergan Çetiner, Yasar Kandur, Derya Beyza Sayın Kocakap

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v11i3.43855

Background and Aim:Research on the role of the adaptive immune system in familial Mediterranean fever (FMF) is limited. The aim of this study was to investigate the levels of immunoglobulins (Ig) IgG, IgM, IgA and absolute lymphocyte count (ALC) in FMF patients compared to normal subjects.

Methods: Patients diagnosed with FMF were included in the study. At the time of data entry, we recorded demographic information, mutation type, ALC, eosinophils and levels of IgA, IgG, IgM and IgE. We compared these variables with those of the control group.

Results: Thirty-five FMF patients (M/F=9/26) were included in this study. There was no significant difference between the mean quantitative levels of the immunoglobulins (IgA, IgG and IgM) and Ig E between the patient and control groups (P>0.05). Compared with the biallelic and monoallelic mutations, we found higher Ig A levels in the biallelic group (1.5±0.7 vs 0.9±0.3 mg/dL, respectively P=0.008). An interesting finding was that three patients with homozygous M694 mutation had an Ig A level above the upper limit while.

Conclusion: No significant difference was found in terms of humoral immunity between FMF patients and healthy individuals. However, we believe that IgA levels should be examined in patients with severe FMF manifestations; the finding of high IgA levels in three patients with a homozygous M694V mutation supports our hypothesis.

Background and Aim: The congenital anomalies of the kidney and urinary tract (CAKUT)
are the most common findings in the pediatric age group with congenital malformations. The
aim of this study was to evaluate clinical characteristics and follow-up results of children with
unilateral solitary kidney (SK)/hypoplasia/atrophy and nephrectomized kidney.
Methods: We retrospectively reviewed the medical records of patients with unilateral atrophic/
hypoplastic/SK and nephrectomized kidney who presented to the pediatric nephrology clinic
between January 2010 and January 2023. The review included demographic data, laboratory
findings, imaging results, and the clinical course of these patients.
Results: Eighty-nine patients were included in the study (M/F=52/37). 42(47.2%) patients
had SK, 29(32.6%) patients had atrophy, 6(6.7%) patients had hypoplasia, and 12(13.5%)
patients had undergone nephrectomy. At the last examination, hypertension was present in
a total of 8 patients (9%). Hypertension was observed in 16.7% of nephrectomized patients
and 11.9% of SK patients. Proteinuria was detected in 15 patients (16.9%) at the last followup
visit, with the highest frequency observed in nephrectomized patients (33.3%). The four
groups exhibited notable variations in terms of serum creatinine and phosphorus levels,
with the nephrectomized group showing a higher creatinine level (P=0.004) and a lower
phosphorus level (P=0.027) compared to the SK group.
Conclusion: Nephrectomy patients exhibit relatively high rates of proteinuria, hypertension,
and increased creatinine levels, indicating the need for close monitoring.

Case Reports


Glomerulocystic Kidney Disease and End-stage Renal Disease in a Child: A Rare Case Report and Literature Review

Shanjida Sharmim, Syed Saimul Huque, Golam Muin Uddin, Afroza Begum, Romana Akbar, Delwar Hossain

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.41961

Glomerulocystic kidney disease (GCKD) is characterized by cystic dilatation of the
Bowman’s capsule and adjacent tubules. It is a rare form of cystic kidney disease and can
occur at any age. Sometimes GCKD may not be differentiated from other cystic kidney
diseases. It usually presents with renal failure. Here, we reported a case of a four-year-old
girl presented with end-stage renal disease who had glomerulocystic kidney disease in renal
histopathology.

Heavy Metal Exposure in Alport Syndrome in an Adolescent: A Case Report

Emre Leventoğlu, Bahriye Uzun Kenan, Betül Öğüt, Bahar Büyükkaragöz, İpek Işık Gönül, Kibriya Fidan

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.42208

Alport syndrome is an inherited glomerular disease characterized by hematuria, proteinuria,
hypertension, progressive kidney failure, hearing loss, and ocular pathologies. It is caused
by a mutation in COL4A3, COL4A4, or COL4A5 genes. A lamellar or uniformly thinned
glomerular basement membrane is a pathognomonic histologic appearance for Alport
syndrome. Light microscopy shows nonspecific findings, including mesangial matrix
expansion and hypercellularity. Renal tubules are other main components of the kidney and the
major sites in response to injuries. They are vulnerable to various conditions, such as hypoxia,
proteinuria, and nephrotoxic substances, including heavy metals, like lead and mercury.
We demonstrated that a patient with asymptomatic Alport syndrome may have accelerated
worsening of kidney functions due to occupational exposure to lead and mercury. Regarding
the initial diagnosis with current clinical and laboratory findings in patients, it is noteworthy
that there is always the possibility of another pathology, and additional investigations may
be needed. Besides, when considering public health issues and the financial burden due to
occupational diseases, we desired to draw attention to the importance and need to create safer
work environments and make frequent inspections.

IgA-dominant Infection-related Glomerulonephritis in a 13-year-old Child: A Case Report and Brief Review of Literature

Ipshita Magh , Lipsa Priyadarshini, Subal Kumar Pradhan

Journal of Pediatric Nephrology, Vol. 11 No. 2 (2023), 7 February 2024,
https://doi.org/10.22037/jpn.v12i2.43027

There has been a history of various bacterial infections and glomerular injuries, which are
now pooled under the terminology of infection-related glomerulonephritis (IRGN). IRGN
is an immune complex-mediated glomerulonephritis (GN) preceded by infection with
subsequent recovery of renal function after the resolution of the infection. Pediatric IgAdominant infection associated with GN is rare and generally has a favorable prognosis
compared to adults with severe comorbidities, like diabetic nephropathy. We reported a
13-year-old girl presented with acute kidney injury with nephritic features with no history of
concurrent illness.

Letter to Editor