Glomerulonephropathies and Disorders of Tubular Function

Kelsey Richardson, Karyn Yonekawa

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Nephrotic syndrome (NS) is comprised of persistent heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. Genetic abnormalities of structural or regulatory proteins within the glomerular basement membrane and/or podocyte lead to primary congenital NS (CNS). Treatment of CNS does not involve immunosuppression and is aimed at minimizing symptoms and preventing serious complications. Infections causing secondary NS include human immunodeficiency virus, syphilis, toxoplasmosis, hepatitis B, malaria, rubella, and cytomegalovirus. Renal tubular acidosis (RTA) causes a nonanion gap metabolic acidosis. It is important to evaluate for other sources of bicarbonate (HCO3) loss before initiating a work-up for RTA and to note that immature tubular function in premature infants may cause a self-limited moderate metabolic acidosis in the first 2 weeks of life. Inherited renal tubulopathies are rare and can be distinguished from each other in part by differences in serum potassium levels, presence of metabolic acidosis or alkalosis, presence of hypertension, and urine findings (Table 91.1). Fanconi syndrome is a condition of diffuse proximal tubule dysfunction resulting in polyuria and wasting of HCO3, amino acids, uric acid, phosphate, glucose, and low-molecular-weight proteins. Fanconi syndrome may be due to an isolated defect or can be a part of a broader genetic syndrome. Primary nephrogenic diabetes insipidus may present during pregnancy as polyhydramnios. In the newborn period, treatment should focus on maintaining adequate fluid balance.

Original languageEnglish (US)
Title of host publicationAvery's Diseases of the Newborn
Subtitle of host publicationTenth Edition
PublisherElsevier Inc.
Pages1301-1307.e2
ISBN (Electronic)9780323401722
ISBN (Print)9780323401395
DOIs
StatePublished - 2018
Externally publishedYes

Keywords

  • Congenital Nephrotic Syndrome
  • Fanconi Syndrome
  • Hyperkalemia
  • Hypokalemia
  • Nephrogenic Diabetes Insipidus
  • Renal Tubular Acidosis

ASJC Scopus subject areas

  • Medicine(all)

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