Related Experiment Video

Updated: Jun 10, 2025

Induction of Nephrotic Syndrome in Mice by Retrobulbar Injection of Doxorubicin and Prevention of Volume Retention by Sustained Release Aprotinin

Induction of Nephrotic Syndrome in Mice by Retrobulbar Injection of Doxorubicin and Prevention of Volume Retention by Sustained Release Aprotinin

Published on: May 6, 2018

Recovery from rituximab-associated persistent hypogammaglobulinaemia in children with nephrotic syndrome

Daishi Hirano¹, Takuya Fujimaru², Mayumi Sako³

¹Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association

|October 17, 2024

Insights

Rituximab treatment for childhood nephrotic syndrome can cause persistent hypogammaglobulinaemia. Many children do not recover IgG levels, and steroid-resistant cases have poorer outcomes, with agranulocytosis increasing infection risk.

Area of Science:

Pediatric Nephrology
Immunology
Pharmacology

Background:

Limited data exist on long-term outcomes of rituximab (RTX)-associated persistent hypogammaglobulinaemia in childhood idiopathic nephrotic syndrome (NS).
Understanding risk factors for non-recovery and serious infections is crucial for managing this condition.

Purpose of the Study:

To investigate the prognosis of childhood-onset idiopathic NS patients who developed persistent hypogammaglobulinaemia after RTX administration.
To identify predictors of immunoglobulin G (IgG) level recovery and risk factors for serious infections.

Main Methods:

A nationwide Japanese survey was conducted on patients with childhood-onset idiopathic NS treated with RTX.
Prognosis, IgG recovery predictors, and serious infection risk factors were analyzed.
Statistical analysis included hazard ratios and confidence intervals.

Main Results:

Of 121 patients with persistent hypogammaglobulinaemia, only 26.3% recovered within a median 2.8-year follow-up.
Steroid-resistant NS was a significant predictor of lower recovery probability (HR 0.28).
15.3% of patients experienced serious infections; agranulocytosis was a major risk factor during hypogammaglobulinaemia.

Conclusions:

A substantial proportion of children with RTX-associated hypogammaglobulinaemia do not recover IgG levels.
Patients with prior steroid-resistant NS have a significantly lower chance of recovery.
Agranulocytosis in hypogammaglobulinaemic patients is linked to increased serious infection risk.

Read full analysis

Abstract

Background:

There are limited data on the long-term outcomes and risk factors for non-recovery after development of rituximab (RTX)-associated persistent hypogammaglobulinaemia among children with idiopathic nephrotic syndrome (NS).

Methods:

A nationwide Japanese survey was conducted to determine the prognosis of patients with childhood-onset idiopathic NS who developed persistent hypogammaglobulinaemia after RTX administration. Specifically, predictors of IgG level recovery and risk factors for serious infection were examined.

Results:

The cohort comprised 118 patients (66.1% boys; median age at initial RTX administration, 7.5 years). Among the 121 patients diagnosed with persistent hypogammaglobulinaemia, only 31 (26.3%) recovered within a median observation period of 2.8 years; approximately 70% of patients continued to exhibit persistent hypogammaglobulinaemia. Among the patients who recovered from hypogammaglobulinaemia, the median time to recovery was 14.1 months. Patients with a history of steroid-resistant NS were less likely to recover from persistent hypogammaglobulinaemia (hazard ratio 0.28; 95% confidence interval 0.09-0.87). In addition, of the 118 eligible patients, 18 (15.3%) developed serious infections requiring hospitalization, and the main risk factor for infection during hypogammaglobulinaemia was agranulocytosis (a well-known adverse effect of RTX in children).

Conclusions:

A significant portion of patients with RTX-associated persistent hypogammaglobulinaemia did not exhibit recovery even after 1 year. Moreover, the data indicate that patients with a history of steroid-resistant NS have a significantly lower probability of recovering from this condition. Agranulocytosis under hypogammaglobulinaemia was significantly associated with an elevated risk of serious infections.

Keywords:

children hypogammaglobulinaemia nephrotic syndrome recovery rituximab

More Related Videos

Development of an IFN-γ ELISpot Assay to Assess Varicella-Zoster Virus-specific Cell-mediated Immunity Following Umbilical Cord Blood Transplantation

Development of an IFN-γ ELISpot Assay to Assess Varicella-Zoster Virus-specific Cell-mediated Immunity Following Umbilical Cord Blood Transplantation

Published on: July 9, 2014

Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis

Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis

Published on: September 7, 2022

Related Concept Videos

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...

Nephrotic Syndrome III : Nursing Management

Nephrotic Syndrome III : Nursing Management

Nursing management for nephrotic syndrome adapts as the disease progresses, with strategies evolving to address advancing symptoms and complications.Early-Stage Management In the early stages, nursing interventions for nephrotic syndrome resemble those used in managing acute glomerulonephritis, focusing on symptom monitoring, fluid balance, and managing mild to moderate edema.Vital Signs: Regularly monitor blood pressure, pulse, respiratory rate, and temperature to promptly identify...

Nephrotic Syndrome II : Assessment and Medical Management

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...

Acute Kidney Injury III: Clinical Manifestations

Acute Kidney Injury III: Clinical Manifestations

Acute Kidney Injury (AKI) progresses through distinct clinical phases: the oliguric, diuretic, and recovery phases, each marked by unique manifestations and challenges.Oliguric Phase:The oliguric phase is the initial stage of AKI, typically lasting 10 to 14 days. This phase is marked by a significant reduction in urine output, usually less than 400 mL per day, indicating decreased kidney function. Fluid retention is a prominent feature, leading to symptoms such as edema, hypertension, and...

Kidney Transplant II: Surgical Procedure

Kidney Transplant II: Surgical Procedure

Preoperative ManagementThe primary goals of preoperative management in kidney transplantation are to optimize the patient’s metabolic state and prepare them for surgery through diet adjustments, necessary dialysis, and tailored medical treatment. This phase also involves comprehensive infection screening and patient education about the surgical procedure and postoperative care to improve outcomes and adherence.Medical ManagementA comprehensive evaluation is required for both the living...

Articles linked to this work by shared authors, journal, and citation graph.

Acute kidney diseases and disorders in children with bloodstream infection: frequency, predictors, and outcomes.

Pediatric nephrology (Berlin, Germany)·2026

An N-terminal CDC42 T43I variant reveals the mechanism of pyrin inflammasome activation.

Science immunology·2026

Hypnotics use during pregnancy and the risks of preterm birth, low birth weight, and major congenital malformations: The Japan Environment and Children's Study.

Journal of epidemiology·2026

A multi-national study examined the response sustainability to calcineurin inhibitors and long-term kidney survivals in children with genetic podocytopathies.

Kidney international·2026

Two novel compounds (lovastatin analog Y and lovastatin analog Z) isolated from health foods containing beni-koji (red yeast rice) with adverse events report.

Journal of natural medicines·2026

Kidney Survival in Children With Steroid-Resistant Nephrotic Syndrome Treated by Rituximab.

Kidney international reports·2026

See all related articles