Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children

P T McEnery1, A J McAdams

  • 1Division of Pediatric Nephrology, Children's Hospital Medical Center, Cincinnati, OH 45229.

Insights

Alternate-day prednisone therapy significantly reduced mesangial proliferation and improved kidney function in children with membranoproliferative glomerulonephritis Type II (MPGN II-dense deposit disease). Dense deposits were ultrastructurally lost in some patients.

Area of Science:

  • Nephrology
  • Pediatric Nephrology
  • Glomerular Diseases

Background:

  • Membranoproliferative glomerulonephritis Type II (MPGN II), also known as dense deposit disease, is a rare kidney disorder.
  • MPGN II is characterized by abnormal deposits within the glomerular basement membrane.
  • Long-term treatment outcomes for MPGN II in children are not well-established.

Purpose of the Study:

  • To evaluate the long-term effects of alternate-day prednisone on renal histology in pediatric MPGN II patients.
  • To assess changes in mesangial proliferation and capillary lumen patency over time.
  • To investigate ultrastructural changes in glomerular deposits during treatment.

Main Methods:

  • Retrospective analysis of serial renal biopsies from six children with MPGN II.
  • Patients received continuous alternate-day prednisone therapy for an average of 14 years.
  • Histopathological and ultrastructural examination of kidney biopsies.

Main Results:

  • All patients showed reduced mesangial proliferation and improved capillary lumen patency.
  • Ultrastructural analysis revealed a shift of deposits from the lamina densa to the lamina rara interna in four patients.
  • Complete loss of dense deposits was observed in the capillary walls of two patients.

Conclusions:

  • Alternate-day prednisone therapy can lead to significant histological improvements in pediatric MPGN II.
  • The observed ultrastructural changes suggest a potential mechanism for deposit clearance.
  • Long-term prednisone treatment may be a viable therapeutic option for MPGN II in children.

Related Concept Videos

Nephrotic Syndrome I : Introduction01:24

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 fluid...
Nephrotic Syndrome II : Assessment and Medical Management01:26

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 any history...
Nephrotic Syndrome III : Nursing Management01:24

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...
Acute Kidney Injury II: Pathophysiology01:29

Acute Kidney Injury II: Pathophysiology

Acute kidney injury (AKI) causes are categorized into three primary categories based on the location of the injury: prerenal, intrarenal (or intrinsic), and postrenal causes. This classification guides clinical management and illustrates how different pathways can impair kidney function.Etiology and Pathophysiology of Acute Kidney Injury1. Prerenal causesEtiology: Prerenal Acute Kidney Injury, the most common type, occurs when reduced blood flow to the kidneys decreases filtration capacity...
Acute Kidney Injury III: Clinical Manifestations01:29

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...
Chronic Kidney Disease II: Clinical Manifestations01:24

Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...