Special Reports


Training in pediatric nephrology officially started in 1990 in Iran, and since then, 143 pediatric
nephrologists have been trained. The first curriculum was published in 2009 and has been
revised twice. The timetable consists of a two-year program to train them by anticipating the
prerequisite clinical skills and problem-solving thinking to apply in their upcoming careers.
The training program accepts one fellow per year for each qualified training center. Until
now, the ratio of pediatric nephrology is 5.6 per one million children.

Original Research Papers


Background and Aim: Tapering alternate-day doses of corticosteroid forms the cornerstone
of the management of steroid-dependent nephrotic syndrome (SDNS) and frequently relapsing
nephrotic syndrome nephrotic syndrome (FRNS). This study compares the reduction of
relapses over a one-year follow-up, using equivalent steroid doses given as either daily or
alternate-day therapy.
Methods: This was an open-label, randomized controlled trial. The participants were children
with SDNS or FRNS, aged 2-10 years. After remission, steroid doses were tapered until a
threshold below 0.75 mg/kg/day. Then, subjects were randomized to one of two arms as
follows: Daily prednisolone at 0.15-0.30 mg/kg/day (intervention arm) or alternate-day dose
of 0.5-0.75 mg/kg (control arm). Both groups were compared after 12 months for a reduction
in relapse frequency. Secondary outcome measures included time to first relapse, proportion
of relapse-free patients, mean steroid dose used, infection episodes, need for alternative
medications, and side effects of corticosteroids.
Results: Median (interquartile range [IQR]) changes in relapse frequency did not differ
between the groups, 0 (IQR: -1.0, 0.25) vs 0 (IQR: 0.0, 1.0) in intervention and control groups,
respectively (P=0.46). The mean percentage change in relapse frequency was -18.1±42.4%
(negative denotes decreased) in the intervention group and 6.0±26.9% in the control group
(P=0.05). The median relapse frequencies during the trial period were 3 (IQR: 2, 3) and 3
(IQR: 3, 4) in the intervention group and control groups, respectively (P=0.021). At study
completion, prednisolone dose was lower in the intervention group, 0.33±0.12 vs 0.40±0.05
(P=0.02). Both groups did not differ by other secondary outcome variables.
Conclusion: In patients with SDNS and FRNS, daily low dose (0.15-0.30 mg/kg/day)
administration of prednisolone is not superior to conventional alternate-day dosing.

Evaluating Diaphragm Muscle Function in Children With Urinary Incontinence

Sümeyra Gölgeli̇kay, Meral Sertel, Yaşar Kandur

Journal of Pediatric Nephrology, Vol. 11 No. 3 (2023), 16 October 2024,
https://doi.org/10.22037/jpn.v11i3.44219

Background and Aim: This study evaluates the respiratory muscle strength, quality of life (QoL),
respiratory function of the diaphragm, participation in postural stabilization and functionality of
active diaphragmatic breathing in postural stability in children with urine incontinence symptoms
and lower urinary tract dysfunction and compares them with healthy children.
Methods: This study included 49 healthy and 49 children with lower urinary tract dysfunction
and urinary incontinence symptoms who applied to Kırıkkale University Pediatric Urology.
Children’s QoL was evaluated using the pediatric urinary incontinence QoL scale. Their
symptom severity was evaluated with the voiding disorders symptom score questionnaire,
their respiratory muscle strength was evaluated with POWER breathe K5 and the diaphragm
was evaluated with the dynamic neuromuscular stabilization method.
Results: When comparing the diaphragm’s respiratory function, participation of the diaphragm
in postural stabilization, and active breathing of the diaphragm in postural stabilization of the
children, a significant difference was observed (P<0.05). Accordingly, asymptomatic children
had greater inspiratory muscular strength, maximal inspiratory pressure and volume values
than children with urinary system dysfunction. There was a difference in the pediatric urinary
incontinence QoL scale and voiding disorders symptom score and it was due to the patient
groups (P<0.05).
Conclusion: Children with urinary incontinence symptoms and lower urinary tract dysfunction
were found to have insufficient respiratory function, use of the diaphragm, and participation in
postural stabilization of the diaphragm compared to asymptomatic children. Their respiratory
muscle strength was lower.

Background and Aim: Limited data exists on health-related quality of life (HRQOL) in
children with chronic kidney disease (CKD) stage 5D and the results often differ. This study
assesses the HRQOL in children with CKD stage 5D using the Hindi version of the paediatric
quality of life (PedsQL) 3.0 end-stage renal disease module.
Methods: This was a cross-sectional, questionnaire-based study conducted between February
2018 and January 2019 at a tertiary-care center in New Delhi, India. HRQOL was assessed in
45 children (age=5-18 years) receiving maintenance dialysis for at least 2 months, using both
child self-report and parent-proxy report.
Results: Enrolled children had poor HRQOL, with the worst scores observed in the treatmentproblems
(TP) domain (child and parent-proxy report). Patients who were on hemodialysis
had lower quality of life (QOL) scores compared to subjects on peritoneal dialysis, especially
in the TP domain. We found a significant negative impact of adolescent age group (general
fatigue [GF], about-my-kidney-disease [AMKD], family and peer interaction [F/PI]), female
gender (perceived physical appearance [PPA]), rural residence (total, GF, AMKD), separated
parents (AMKD, worry), lower maternal education (GF, communication), higher number of
medication intake (AMKD, F/PI), low hemoglobin (communication) and more number of
hospitalizations in last six months (F/PI) on HRQOL of enrolled children. Furthermore, the
child-reported mean total scores in the domains of GF, TP, F/PI and worry were less favorable
compared to parent-reported scores. School attendance was seen in only 15 % of the children.
Conclusion: End-stage kidney disease exerts a significant impact on the HRQOL of affected
children; therefore, comprehensive management of this disease in children should include
QOL assessment.

Investigating Steroid-associated Peptic Symptoms in Patients With Primary Nephrotic Syndrome

Shabnam Khansari, Behnaz Bazargani, Arash Abbasi, Daryoosh Fahimi, Fahimeh Askarian, Mastaneh Moghtaderi

Journal of Pediatric Nephrology, Vol. 11 No. 3 (2023), 16 October 2024,
https://doi.org/10.22037/jpn.v11i3.43949

Background and Aim: Nephrotic syndrome is the most common glomerulopathy among children
aged 2-10 years old and high doses of corticosteroids are the cornerstone of its management.
Whether corticosteroid use induces peptic ulcer disease in these patients remains uncertain. This
study explores any relation between steroids and peptic symptoms in these children.
Methods: A total of 100 nephrotic syndrome patients aged 1 to 15 years old treated with oral
prednisolone in our Outpatient Department were studied. In this study, we compared nephrotic
syndrome patients receiving 2 mg/kg prednisolone daily for 1 month and afterward as every
other day until tapering off in about 3-6 months. They were divided into groups. One received
aluminum Mg (Al-Mg) or proton pump inhibitors (PPIs) as prophylaxis and the other 50 patients
did not receive any prophylaxis. These two groups are investigated for any gastro-intestinal
complications. Heartburn, hematochezia, nausea, and dyspepsia are the four complications
that were studied.
Results: In this study, the data from 100 patients (61 male and 39 female) were analyzed.
Accordingly, 46% of the patients consumed 0.5 mg/kg prednisolone every other day and 19% of
the patients consumed 2 mg/kg prednisolone daily. In 51% of patients the duration of treatment
was more than 6 months and in 19% of cases it was less than a month. Also, among the 68%
of the patients who did not take PPIs, none experienced any digestive symptoms, including
hematochezia. Among those patients who received prophylactic, PPIs one patient with less
than a month of prednisolone taking and one who took prednisolone for more than six months
contracted hematochezia. 

Conclusion: One-month therapy regimen of 2 mg/kg daily prednisolone and continuation
of it until tapering off the steroids causes few gastrointestinal symptoms. The use of PPIs or
Al-Mg did not affect the incidence of gastrointestinal symptoms significantly. Accordingly,
corticosteroid therapy does not increase peptic ulcers in nephrotic syndrome patients.

Investigating Renal Dysfunction and Dyslipidemia in Obese and Overweight Children

Beheshteh Olang, Shiva Fatollahierad, Mahmoud Hajipour, Amirhossein Hosseini, Zahra Fazeli Farsani, Aliakbar Sayyari

Journal of Pediatric Nephrology, Vol. 11 No. 3 (2023), 16 October 2024,
https://doi.org/10.22037/jpn.v11i3.45591

Background and Aim: Childhood obesity and chronic kidney disease (CKD) are both substantial
public health concerns. Obesity is known to contribute directly and indirectly to the development
of CKD. This study evaluates kidney damage related to obesity and metabolic comorbidities in
overweight and obese children.
Methods: A prospective cross-sectional study was conducted in the Obesity Clinic of a
Children’s Hospital in Iran from 2017 to 2020 on obese and overweight children aged 2 to 18
years. Biochemical parameters, such as total cholesterol, high-density lipoprotein (HDL), lowdensity
lipoprotein (LDL), triglyceride (TG), creatinine, estimated glomerular filtration rate and
fasting glucose were measured. A binary logistic regression was used to assess the association
between body mass index (BMI) Z-score and biochemical parameters. Two-sided P<0.05 was
considered significant.
Results: The participants exhibited a mean BMI Z-score of 2.39±0.70. The prevalence of obesity
was 78.6% among boys and 52.9% among girls. Additionally, 78.9% of them had an estimated
glomerular filtration rate of less than 90 mL/min/1.73 m2. Impaired fasting glucose was present in
15.6% of the participants, while hypercholesterolemia was noted in 66.7% of them.
Conclusion: Significant percentages of overweight and obese children have decreased estimated
glomerular filtration rate. In addition, dyslipidemia, in the forms of high cholesterol, high TG,
and low HDL is found in these children.

Case Reports


Membranous glomerulonephritis (MGN) is the most common cause of nephrotic syndrome
in adults worldwide; however, it accounts for only 1.5% to 9% of cases of pediatric
nephrotic syndrome. Detecting phospholipase A2 receptor (PLA2R) and thrombospondin
domain containing 7A (THSD7A) among primary MGN patients has greatly transformed
the diagnosis, treatment monitoring, and prognosis. These biomarkers are rarely undetected
in the serum; however, renal tissue sent for histopathology is stained positive. Most of the
previous reports of pediatric MGN are from adolescents approaching adulthood. We report
a 9-year-old boy presenting with PLA2R-positive primary MGN who was seronegative and
responded very well to 2 doses of rituximab after a failed steroid course.

Co-occurrence of Immunoglobin A Vaculitis and Immunoglobulin A Nephropathy Suggesting Common Pathogenesis: A Case Report

Sweta Mishra, Lipsa Priyadarshini, Subal Kumar Pradhan

Journal of Pediatric Nephrology, Vol. 11 No. 3 (2023), 16 October 2024,
https://doi.org/10.22037/jpn.v11i3.45211

Immunoglobulin A (IgA) vasculitis, also known as Henoch-Schönlein purpura, and
IgA nephropathy are two different diseases of the same clinical spectrum. They may
occur sequentially in the same patient and exhibit overlapping clinical presentations,
immunological characteristics, and pathological features. The clinical presentation and the
degree of severity are different for individual cases. In this study, we report a girl who initially
presented with Henoch-Schönlein purpura, and on follow-up, renal biopsy was suggestive of
IgA nephropathy. Accordingly, this presentation suggests the common pathogenesis of both
diseases and the need for long-term follow-up of patients with Henoch-Schönlein purpura.