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Pediatric IgA Nephropathy Follow-up

  • Author: Mohammad Ilyas, MD, FAAP; Chief Editor: Craig B Langman, MD  more...
 
Updated: Feb 27, 2014
 

Further Outpatient Care

Routine follow up is important for monitoring the progression of disease.

Medications must be taken as prescribed.

A healthy diet and lifestyle is important.

Patients should avoid obesity, smoking, and other activities that negatively influence the cardiovascular system.

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Further Inpatient Care

Inpatient care is not necessary in patients with immunoglobulin A (IgA) nephropathy (IgAN), except for complications of renal failure, hypertension, dialysis, or renal transplantation.

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Inpatient & Outpatient Medications

No specific change of medication is necessary to transition from inpatient to outpatient therapy.

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Transfer

No transfer is necessary.

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Deterrence/Prevention

No methods for deterrence or prevention of IgA nephropathy are known.

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Complications

Primary complications include those related to uncontrolled hypertension (eg, seizure, stroke, end-organ damage), renal insufficiency (eg, growth failure, bone demineralization, anemia), and adverse reactions to one of the prescribed medications.

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Prognosis

IgA nephropathy was initially thought to be a benign disease, but it is now recognized that over 25 years of observation, it can slowly progress to end-stage renal disease in up to 50% of affected patients.[36] The remaining patients may sustain clinical remission or have persistent low-grade hematuria and/or proteinuria. The prognosis is difficult to predict with accuracy in an individual patient, but important risk factors for progressive renal disease have been identified.

Patients with IgA nephropathy who have little or no proteinuria (>500-1000 mg/d) have a low risk of progression, at least in the short term. However, proteinuria and renal insufficiency develop in a substantial proportion of patients over the long term.[37] Among patients who develop overt proteinuria and/or an elevated serum creatinine concentration, the rate of progression to end-stage renal disease is approximately 15-25% at 10 years and 20-30% at 20 years.[38]

The rate of progression is typically slow, with the glomerular filtration rate (GFR) often falling by as little as 1-3 mL/min/y, a change not associated with an elevation in the serum creatinine concentration in the short term. In one report, repeat renal biopsies were performed at 5 years in 73 patients with persistent proteinuria and a normal or near-normal initial serum creatinine value. Histologic improvement occurred in only 4%, with 41% remaining stable and 55% showing progressive glomerular and secondary vascular and tubulointerstitial injury. An increase in serum creatinine to more than 1.5 mg/dL (133 µmol/L) was associated with major pathologic lesions.[39]

The patients who develop progressive disease typically have one or more of the following clinical or laboratory findings at diagnosis, each of which is a marker for more severe disease.

  • A reduction in GFR, as manifested by an elevated serum creatinine concentration at diagnosis or during the course of the disease, is associated with a worse renal prognosis[40]
  • Hypertension (BP >95 percentile for height and sex) is predictive of a worse outcome; a higher mean arterial pressure is associated with a higher risk of progressive renal disease[13]
  • With regard to proteinuria, the rate of progression is very low among patients excreting less than 15 mg/kg/d and is greatest among those excreting more than 50 mg/kg/d[41]
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Patient Education

Inform patients about specific disease processes when possible.

Encourage patients to avoid risk factors, such as smoking, drugs, obesity, and poor medication compliance.

For patient education resources, see the Kidneys and Urinary System Center, as well as Blood in the Urine.

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Contributor Information and Disclosures
Author

Mohammad Ilyas, MD, FAAP Assistant Professor of Pediatrics, University of Florida College of Medicine; Consulting Staff, Department of Pediatrics, Section of Nephrology, Wolfson Children Hospital and Shands Hospital Jacksonville

Mohammad Ilyas, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology

Disclosure: Nothing to disclose.

Coauthor(s)

Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical and Dental Associations, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, Southwest Pediatric Nephrology Study Group

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Raptor Pharmaceuticals; Eli Lilly and Company; Dicerna<br/>Received grant/research funds from NIH for none; Received grant/research funds from Raptor Pharmaceuticals, Inc for none; Received grant/research funds from Alexion Pharmaceuticals, Inc. for none; Received consulting fee from DiCerna Pharmaceutical Inc. for none.

Additional Contributors

Deogracias Pena, MD Medical Director of Dialysis, Medical Director of Pediatric Nephrology and Transplantation, Cook Children's Medical Center; Clinical Associate Professor, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Medical Director of Pediatric Nephrology, Florida Hospital for Children

Deogracias Pena, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Pediatric Nephrology

Disclosure: Nothing to disclose.

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Glomerulus with mesangial hypercellularity and intact capillary loops. Trichrome Stain, original magnification 400x. Image courtesy of Patrick D Walker, MD.
Mesangial deposits of immunoglobulin A (IgA). Fluoresceinated Anti-IgA Antibody, Immunofluorescence microscopy, original magnification 400x. Image courtesy of Patrick D Walker, MD.
Electron photomicrograph showing mesangial electron dense deposits (arrow). Uranyl acetate and lead citrate stain, original magnification 12,000x. Image courtesy of Patrick D Walker, MD.
 
 
 
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